OBSERVATION ON ARRANGEMENT OF HILAR STRUCTURES IN CADAVERIC KIDNEYS AND THEIR CLINICAL SIGNIFICANCE

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Sanjay Kumar Sinha

2014-12-01

Full Text Available Hilum of an organ is a depression, pit or slit like opening through which vital structures enter or leave the organ. In addition to the kidney, hilum is also observed in the cerebellum, lung, ovary, spleen and suprarenal gland. Laparoscopic nephron-sparing surgery for solid renal masses can be achieved successfully both transperitoneally and retroperitoneally if a comprehensive knowledge of both normal and variant hilar anatomy of the kidneys is in the mind of the operating surgeon. Documented text is available on various aspects of the kidneys but an observation on variations in hilar arrangement is infrequently cited. In standard text from anterior to posterior the structures at the renal hilum are renal vein, renal artery and the renal pelvis.

Marker-Negative Pheochromocytoma Associated with Inferior Vena Cava Thrombosis

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S. Poudyal

2017-01-01

Full Text Available Pheochromocytoma associated with inferior vena cava (IVC thrombosis is very rare. A 27-year-old female presented with right flank pain and hypertensive urgency. Contrast-enhanced CT abdomen and gadolinium-contrast MRI abdomen revealed right adrenal mass suspicious of malignancy with invasion and compression to the right IVC wall along with IVC thrombus extending from the level of renal veins to the level of confluence with hepatic veins. Her routine laboratory investigations including 24-hour urine fractionated metanephrines, vanillylmandelic acid, and cortisol were normal. Right adrenalectomy with IVC thrombectomy was done. Perioperative period was uneventful. Histopathology of the mass turned out to be pheochromocytoma with thrombus revealing fibroadipose tissue with fibrin. Pheochromocytoma may present with IVC thrombus as well as normal serum and urinary markers. Thus, clinical suspicion is imperative in perioperative management of adrenal mass.

Retrograde Renal Cooling to Minimize Ischemia

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Janet L. Colli

2013-01-01

Full Text Available Objective: During partial nephrectomy, renal hypothermia has been shown to decrease ischemia induced renal damage which occurs from renal hilar clamping. In this study we investigate the infusion rate required to safely cool the entire renal unit in a porcine model using retrograde irrigation of iced saline via dual-lumen ureteral catheter. Materials and Methods: Renal cortical, renal medullary, bowel and rectal temperatures during retrograde cooling in a laparoscopic porcine model were monitored in six renal units. Iced normal saline was infused at 300 cc/hour, 600 cc/hour, 1000 cc/hour and gravity (800 cc/hour for 600 seconds with and without hilar clamping. Results: Retrograde cooling with hilar clamping provided rapid medullary renal cooling and significant hypothermia of the medulla and cortex at infusion rates ≥ 600 cc/hour. With hilar clamping, cortical temperatures decreased at -0.9° C/min. reaching a threshold temperature of 26.9° C, and medullary temperatures decreased at -0.90 C/min. reaching a temperature of 26.1° C over 600 seconds on average for combined data at infusion rates ≥ 600 cc/hour. The lowest renal temperatures were achieved with gravity infusion. Without renal hilum clamping, retrograde cooling was minimal at all infusion rates. Conclusions: Significant renal cooling by gravity infusion of iced cold saline via a duel lumen catheter with a clamped renal hilum was achieved in a porcine model. Continuous retrograde irrigation with iced saline via a two way ureteral catheter may be an effective method to induce renal hypothermia in patients undergoing robotic assisted and/or laparoscopic partial nephrectomy.

Adrenocorticotropic Hormone Secreting Pheochromocytoma Underlying Glucocorticoid Induced Pheochromocytoma Crisis

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Gil A. Geva

2018-01-01

Full Text Available Context. Pheochromocytomas are hormone secreting tumors of the medulla of the adrenal glands found in 0.1–0.5% of patients with hypertension. The vast majority of pheochromocytomas secrete catecholamines, but they have been occasionally shown to also secrete interleukins, calcitonin, testosterone, and in rare cases adrenocorticotropic hormone. Pheochromocytoma crisis is a life threatening event in which high levels of catecholamines cause a systemic reaction leading to organ failure. Case Description. A 70-year-old man was admitted with acute myocardial ischemia following glucocorticoid administration as part of an endocrine workup for an adrenal mass. Cardiac catheterization disclosed patent coronary arteries and he was discharged. A year later he returned with similar angina-like chest pain. During hospitalization, he suffered additional events of chest pain, shortness of breath, and palpitations following administration of glucocorticoids as preparation for intravenous contrast administration. Throughout his admission, the patient demonstrated both signs of Cushing’s syndrome and high catecholamine levels. Following stabilization of vital parameters and serum electrolytes, the adrenal mass was resected surgically and was found to harbor an adrenocorticotropic hormone secreting pheochromocytoma. This is the first documented case of adrenocorticotropic hormone secreting pheochromocytoma complicated by glucocorticoid induced pheochromocytoma crisis. Conclusion. Care should be taken when administering high doses of glucocorticoids to patients with suspected pheochromocytoma, even in a patient with concomitant Cushing’s syndrome.

Role of hilar resection in the treatment of hilar cholangiocarcinoma.

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Otani, Kazuhiro; Chijiiwa, Kazuo; Kai, Masahiro; Ohuchida, Jiro; Nagano, Motoaki; Kondo, Kazuhiro

2012-05-01

The aim of this study was to clarify the role of bile duct resection without hepatectomy (hilar resection) in hilar cholangiocarcinoma. We retrospectively compared surgical results for hilar cholangiocarcinoma between 8 patients treated with hilar resection and 21 patients treated with hepatectomy. All hilar resections were performed for Bismuth type I or II tumors with T2 or less lesions, whereas hepatectomy was done for type III or IV tumors excluding one type II tumor. R0 resection was equally achieved in both groups (62.5% in hilar resection group and 76.2% in hepatectomy group, p=0.469) and overall 5-year survival rates were comparable (21.9% vs. 23.6%, p=0.874). With respect to gross tumor appearance, R0 resection was achieved in all patients with papillary tumor in both groups with the excellent 5-year survivals (100% vs. 100%). In patients with nodular and flat tumors, R0 resection was achieved less frequently in the hilar resection vs. hepatectomy group (50% vs. 77.8%) mainly due to failure to clear the proximal ductal margin, resulting in poorer 5-year survival (0% vs. 18.7%). Hilar resection may be indicated for papillary T1 or 2 tumors in Bismuth type I or II cholangiocarcinoma.

CT diagnosis of abdominal ectopic pheochromocytoma

International Nuclear Information System (INIS)

Zhang Yuping; Zhao Zhiying

2010-01-01

Objective: To discuss the value of CT in diagnosis of abdominal ectopic pheochromocytoma. Methods: CT findings of 5 cases surgically and pathologically proved with ectopic pheochromocytoma were retrospectively analyzed. Results: Soft tissue mass with light asymmetry enhancement was found between the abdominal aorta and the inferior vena ca-va in one case. 1 case was completely cystic with light enhancement of the cystwall located in front of the left side of the abdominal aorta. 1 case of large solid mass occurred between the renal hilum and the tail of pancreas, with irregular shape, unclear boundary, central necrosis, calcification and obviously enhancement at the solid part. 2 cases showed as oval soft lump with even density, moderate strengthening located before the abdominal aorta. Paroxysmal hypertension occurred in 3 cases and didn't in 2 cases. Hypertension happened in 1 case during the operation because of stimulation. Blood pressure appeared in 1 case during and after operation. Blood and urinary catecholamine increased significantly in 4 cases. Conclusion: Ectopic pheochromocytoma mainly located surround the abdominal aorta with diverse CT performance. It is helpful for diagnosing when finding a lesion locates at the specified sites combined with typical clinical presentation. CT can not only depict small tumor, but also can show the relationship with surrounding structure, and it provides important information for the operation and prognosis. (authors)

Pheochromocytoma in pregnancy

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Magdalena Wyskida

2014-06-01

Full Text Available Pheochromocytoma occurs with a frequency estimated at 2-7 per 100 000 pregnant women. Unrecognized, and thus untreated pheochromocytoma is associated with very high (40-50% maternal and fetal mortality. Pheochromocytoma occurs sporadically or as a family trait. Its presence should be suspected in women with paroxysmal or established hypertension, especially before the 20th week of pregnancy, accompanied by headaches and palpitations, and excessive sweating, muscle tremors, vomiting, anxiety, vasomotor disturbances and blurred vision. The variety of clinical presentations and rarity are the cause of not including the disease in differential diagnosis of hypertension in pregnancy. Biochemical tests are essential in the diagnosis of pheochromocytoma, and involving the assessment of methoxycatecholamine urinary excretion. The second step in the diagnostics is magnetic resonance imaging of adrenal glands. Adrenalectomy is the treatment of choice for pheochromocytoma with adrenal location, which depends on the timing of the tumor diagnosis. Conservative treatment for 10-14 days with pharmacological blockade of alpha-adrenergic receptors should precede the surgery. Early diagnosis and properly planned treatment of pheochromocytoma significantly reduces the risk to the mother and fetus.

Severe polyuria after the resection of adrenal pheochromocytoma.

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Tobe, Musashi; Ito, Keiichi; Umeda, Shun; Sato, Akinori; Adaniya, Noriaki; Tanaka, Yuji; Hayakawa, Masamichi; Asano, Tomohiko

2010-12-01

A 73-year-old male patient with hypertension and hyperglycemia was referred to our hospital because of a diagnosis regarding his left adrenal tumor. Because the levels of urinary metanephrine and normetanephrine were elevated, and (131) I-MIBG scintigraphy showed intense uptake in the adrenal tumor, the tumor was diagnosed as a pheochromocytoma. An adrenalectomy was carried out. Severe polyuria, which was accompanied by a rapid decrease in central venous pressure, started 1 hour after the operation. Urine output of more than 8000 mL/day continued until the 16th postoperative day. Plasma antidiuretic hormone (ADH) levels were within the normal range. Plasma human atrial natriuretic peptide (hANP) and brain natriuretic peptide (BNP) were elevated postoperatively, and the elevation of these peptides was one possible cause for the severe polyuria. Because ADH levels in the tumor fluid were not elevated, the tumor was not an ADH-secreting tumor. Urinary β2-microglobulin was significantly elevated after the operation, thus suggesting that renal tubule dysfunction might also have been involved in the polyuria. However, the mechanism of polyuria after the resection of adrenal pheochromocytoma is not fully understood. Polyuria after the resection of adrenal pheochromocytoma is extremely rare, and the present subject is the second case to date. © 2010 The Japanese Urological Association.

[Pheochromocytoma--pathohistologic and immunohistochemical aspects].

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Tatić, Svetislav; Havelka, Marija; Paunović, Ivan; Bozić, Vesna; Diklic, Aleksandar; Brasanac, Dimitrije; Janković, Radovan; Jancić-Zguricas, Marija

2002-07-01

Pheochromocytoma originates in chromaffin cells of the adrenal medulla. Its incidence is similar in both sexes and most frequent between the ages of thirty and fifty. Multiple and bilateral pheochromocytomas constitute 5 to 10 percent of all cases. Pheochromocytoma occurs sporadically or is related to family syndromes such as: syndrome of multiple endocrine neoplasia--MEN IIA and IIB, neurofibromatosis (von Recklinghausen's disease), von Hippel-Lindau's disease, Sturge-Weber's syndrome, and tuberous sclerosis. Cases in a family usually occur at a younger age and are mostly bilateral and with more aggressive biological behaviour. The aim of the study was to make histomorphological and immunohistochemical analyses of 52 pheochromocytomas. These cases are the surgical material from the Centre of Endocrine Surgery, Institute of Endocrinology, Diabetes, and Metabolic Disorders, Clinical Centre of Serbia, Belgrade, over the period from 1974 to 1997. Frozen and fixed sections, which were cut from paraffinembedded material and stained by both hematoxylin-eosin and PAS, were used in order to make pathohistological diagnoses. The expression of chromogranin A, S-100 protein and ACTH was examined using the PAP method, while neuronspecific enolase (NSE), synaptophysin and neurofilament were examined by the APAAP method with appropriate antibodies (DAKO). The patients were between 4 and 65 years of age (average age 38.5) and there were 28 females (63.64%) and 16 males (36.36%). The largest pheochromocytoma had the diameter of 12 cm, and weight of pheochromocytomas in question was from 13.5 to 370 grams, the average weight being 83.4 grams. On gross examination, the tumours proved to be well-defined, either by fibrous capsule, or by adrenocortical tissue. The cross-sections of tumours were mainly of pale red-grayish colour, and showed numerous foci of necrosis, haemorrhage and cystic softening. Histological appearance of pheochromocytomas was with significant irregularities in

Robotic partial nephrectomy for complex renal tumors: surgical technique.

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Rogers, Craig G; Singh, Amar; Blatt, Adam M; Linehan, W Marston; Pinto, Peter A

2008-03-01

Laparoscopic partial nephrectomy requires advanced training to accomplish tumor resection and renal reconstruction while minimizing warm ischemia times. Complex renal tumors add an additional challenge to a minimally invasive approach to nephron-sparing surgery. We describe our technique, illustrated with video, of robotic partial nephrectomy for complex renal tumors, including hilar, endophytic, and multiple tumors. Robotic assistance was used to resect 14 tumors in eight patients (mean age: 50.3 yr; range: 30-68 yr). Three patients had hereditary kidney cancer. All patients had complex tumor features, including hilar tumors (n=5), endophytic tumors (n=4), and/or multiple tumors (n=3). Robotic partial nephrectomy procedures were performed successfully without complications. Hilar clamping was used with a mean warm ischemia time of 31 min (range: 24-45 min). Mean blood loss was 230 ml (range: 100-450 ml). Histopathology confirmed clear-cell renal cell carcinoma (n=3), hybrid oncocytic tumor (n=2), chromophobe renal cell carcinoma (n=2), and oncocytoma (n=1). All patients had negative surgical margins. Mean index tumor size was 3.6 cm (range: 2.6-6.4 cm). Mean hospital stay was 2.6 d. At 3-mo follow-up, no patients experienced a statistically significant change in serum creatinine or estimated glomerular filtration rate and there was no evidence of tumor recurrence. Robotic partial nephrectomy is safe and feasible for select patients with complex renal tumors, including hilar, endophytic, and multiple tumors. Robotic assistance may facilitate a minimally invasive, nephron-sparing approach for select patients with complex renal tumors who might otherwise require open surgery or total nephrectomy.

The gross anatomy of the renal sympathetic nerves revisited.

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Mompeo, Blanca; Maranillo, Eva; Garcia-Touchard, Arturo; Larkin, Theresa; Sanudo, Jose

2016-07-01

Catheter-based renal denervation techniques focus on reducing blood pressure in resistant hypertension. This procedure requires exact knowledge of the anatomical interrelation between the renal arteries and the targeted renal nervous plexus. The aim of this work was to build on classical anatomical studies and describe the gross anatomy and anatomical relationships of the renal arteries and nerve supply to the kidneys in a sample of human cadavers. Twelve human cadavers (six males and six females), age range 73 to 94 years, were dissected. The nervous fibers and renal arteries were dissected using a surgical microscope. The renal plexus along the hilar renal artery comprised a fiber-ganglionic ring surrounding the proximal third of the renal artery, a neural network along the middle and distal thirds, and smaller accessory ganglia along the course of the nerve fibers. The fibers of the neural network were mainly located on the superior (95.83%) and inferior (91.66%) surfaces of the renal artery and they were sparsely interconnected by diagonal fibers. Polar arteries were present in 33.33% of cases and the renal nerve pattern for these was similar to that of the hilar arteries. Effective renal denervation needs to target the superior and inferior surfaces of the hilar and polar arteries, where the fibers of the neural network are present. Clin. Anat. 29:660-664, 2016. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Oncological superiority of hilar en bloc resection for the treatment of hilar cholangiocarcinoma.

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Neuhaus, Peter; Thelen, Armin; Jonas, Sven; Puhl, Gero; Denecke, Timm; Veltzke-Schlieker, Wilfried; Seehofer, Daniel

2012-05-01

Long-term results after liver resection for hilar cholangiocarcinoma are still not satisfactory. Previously, we described a survival advantage of patients who undergo combined right trisectionectomy and portal vein resection, a procedure termed "hilar en bloc resection." The present study was conducted to analyze its oncological effectiveness compared to conventional hepatectomy. During hilar en bloc resection, the extrahepatic bile ducts were resected en bloc with the portal vein bifurcation, the right hepatic artery, and liver segments 1 and 4 to 8. With this "no-touch" technique, preparation of the hilar vessels in the vicinity of the tumor was avoided. The long-term outcome of 50 consecutive patients who underwent curative (R0) hilar en bloc resection between 1990 and 2004 was compared to that of 50 consecutive patients who received curative conventional major hepatectomy for hilar cholangiocarcinoma (perioperative deaths excluded). The 1-, 3-, and 5-year survival rates after hilar en bloc resection were 87%, 70%, and 58%, respectively, which was significantly higher than after conventional major hepatectomy. In the latter group, 1-, 3-, and 5-year survival rates were 79%, 40%, and 29%, respectively (P = 0.021). Tumor characteristics were comparable in both groups. A high number of pT3 and pT4 tumors and patients with positive regional lymph nodes were present in both groups. Multivariate analysis identified hilar en bloc resection as an independent prognostic factor for long-term survival (P = 0.036). In patients with central bile duct carcinomas, hilar en bloc resection is oncologically superior to conventional major hepatectomy, providing a chance of long-term survival even in advanced tumors.

Von Hippel-Lindau Syndrome: Diagnosis and Management of Hemangioblastoma and Pheochromocytoma

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P. Vaganovs

2013-01-01

Full Text Available Introduction. Von Hippel-Lindau (VHL syndrome is a pathological condition that causes various clinical symptoms and is difficult to diagnose. The most common pathological lesions are hemangioblastomas of the central nervous system, retinal angiomas, renal clear cell carcinomas, and pheochromocytomas. Case Report. A 23-year-old female had a syncope episode in 2008. Magnetic resonance imaging (MRI revealed a right temporal hemangioblastoma, which was treated surgically. Genetic screening identified a VHL gene mutation, and computed tomography (CT revealed a left adrenal mass. Since it was unclear whether the mass was a pheochromocytoma, or another benign or malignant tumors, laparoscopic adrenalectomy was performed. A month after surgery, the patient complained of general fatigue, poor concentration, loss of appetite, and insomnia. After careful clinical investigation, the patient was referred to a psychiatrist due to suspected depression, which was confirmed. Conclusions. VHL genetic screening should be performed in cases of hemangioblastoma. In VHL syndrome cases, pheochromocytoma cannot always be diagnosed by biochemical catecholamine analyses; therefore, CT or MRI scanning of the abdomen must be performed. Due to the long treatment period, some patients may develop episodes of depression, which can simulate VHL syndrome.

Catecholamine induced cardiomyopathy in pheochromocytoma

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Ron Thomas Varghese

2013-01-01

Full Text Available Catecholamine induced cardiomyopathy in the setting of pheochromocytoma is an unusual clinical entity. Earlier studies have reported left ventricular dysfunction in around 10% of subjects with pheochromocytoma. [1] Catecholamine induced vasoconstriction, direct toxic effect of byproducts of catecholamine degradation and direct receptor-mediated mechanisms are thought to contribute to cardiomyopathy in subjects with pheochromocytoma. The presentation remains a diagnostic challenge as patients may already have hypertensive heart disease and acute coronary syndrome on account of uncontrolled secondary hypertension. We report a case of a 42-year-old male, who presented with features of pheochromocytoma induced cardiomyopathy.

Magnetic resonance imaging (MRI) of the renal sinus.

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Krishna, Satheesh; Schieda, Nicola; Flood, Trevor A; Shanbhogue, Alampady Krishna; Ramanathan, Subramaniyan; Siegelman, Evan

2018-04-09

This article presents methods to improve MR imaging approach of disorders of the renal sinus which are relatively uncommon and can be technically challenging. Multi-planar Single-shot T2-weighted (T2W) Fast Spin-Echo sequences are recommended to optimally assess anatomic relations of disease. Multi-planar 3D-T1W Gradient Recalled Echo imaging before and after Gadolinium administration depicts the presence and type of enhancement and relation to arterial, venous, and collecting system structures. To improve urographic phase MRI, concentrated Gadolinium in the collecting systems should be diluted. Diffusion-Weighted Imaging (DWI) should be performed before Gadolinium administration to minimize T2* effects. Renal sinus cysts are common but can occasionally be confused for dilated collecting system or calyceal diverticula, with the latter communicating with the collecting system and filling on urographic phase imaging. Vascular lesions (e.g., aneurysm, fistulas) may mimic cystic (or solid) lesions on non-enhanced MRI but can be suspected by noting similar signal intensity to the blood pool and diagnosis can be confirmed with MR angiogram/venogram. Multilocular cystic nephroma commonly extends to the renal sinus, however, to date are indistinguishable from cystic renal cell carcinoma (RCC). Solid hilar tumors are most commonly RCC and urothelial cell carcinoma (UCC). Hilar RCC are heterogeneous, hypervascular with epicenter in the renal cortex compared to UCC which are centered in the collecting system, homogeneously hypovascular, and show profound restricted diffusion. Diagnosis of renal sinus invasion in RCC is critically important as it is the most common imaging cause of pre-operative under-staging of disease. Fat is a normal component of the renal sinus; however, amount of sinus fat correlates with cardiovascular disease and is also seen in lipomatosis. Fat-containing hilar lesions include lipomas, angiomyolipomas, and less commonly other tumors which engulf sinus

Variant anatomy of renal arteries in a Kenyan population.

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Ogeng'o, Julius A; Masaki, Charles O; Sinkeet, Simeon R; Muthoka, Johnstone M; Murunga, Acleus K

2010-01-01

Variant anatomy of renal arteries is important in renal transplant, vascular reconstruction, and uroradiological procedures. The variations show ethnic and population differences. Data from Africans are scarce and altogether absent for Kenyans. To describe patterns of origin, trajectories and branching of renal arteries in a Kenyan population. Descriptive cross-sectional study conducted in the Department of Human Anatomy, University of Nairobi. Three hundred and fifty six kidneys from 178 cadavers and postmortem specimens were used in the study. Aorta, renal arteries and kidneys were exposed by dissection. Number, trajectories, level of branching, number of branches and point of entry into the kidney were recorded. Data was analyzed using SPSS version 16.0, and presented using macrographs, tables, and bar charts. Additional arteries occurred in 14.3% of the cases. In 82.4% of these, there was one additional artery. Fifty nine point five per cent of the double renal arteries were parallel and 7.1% crossed. Of the 305 single arteries, 76.4% showed hilar, 21.6% prehilar and 2% intraparenchymal branching. In the hilar branching, ladder type was present in 65% and fork type in 35%. Bifurcation and trifurcation were present in 59.6% and 33.1% respectively. Polar arteries were present in 16.9% cases. Over 14% of the Kenyan population may have additional renal arteries while more than 20% show early branching. Several trajectories and hilar branching patterns exist which renal transplant surgeons and radiologists should be aware of to avoid inadvertent vascular injury.

The Epidemiology of Pheochromocytoma

DEFF Research Database (Denmark)

Ebbehoj, A; Søndergaard, Esben; Trolle, Christian

Pheochromocytoma is a rare disease but frequently poses a diagnostic dilemma due to the unspecific symptoms and its potentially life-threatening nature. There is a perception of an increase in the incidence of pheochromocytomas in recent years, but no data on time trends exist. We obtained data f...

Primary meningeal pheochromocytoma: a case report

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Yoon, Il Ju; Suh, Hyoung Sim [Myung-Ji St. Mary' s Hospital, Seoul (Korea, Republic of); Kim, Sung Nam [Green Cross Reference Lab, Seoul (Korea, Republic of)

2007-04-15

Pheochromocytoma is a rare endocrine tumor arising from the chromaffin tissue, and it is able to produce and secrete catecholamines. Lymph nodes, liver, lung and bone are the most frequent sites of metastasis. We report here on a case of pheochromocytoma arising from the dura in a patient who was surgically treated for bilateral pheochromocytoma five years previously.

Investigation of the gallium-67 citrate hilar accumulation

International Nuclear Information System (INIS)

Suto, Yuji

1987-01-01

To study the 67 Ga hilar accumulation, author quantitatively analyzed 67 Ga scintigrams of patients having no chest disease and normal chest roentgenograms. Relationship between hilar accumulation and smoking was quantitatively and experimentally studied. The conclusions were as follows: 1. There was significant relationship between smoking and 67 Ga hilar accumulation but there was no significant relationship between aging and the hilar accumulation. 2. The 67 Ga uptake of the hilar lymph node of smoked rat was higher than that of control group on microautoradiogram. The histological finding of the hilar lymph node of smoked rat was sinus histiocytosis. 3. Activated histiocytosis of hilar lymphatic sinus by some factors including smoking seemed to be responsible for 67 Ga hilar accumulation, of which mechanism was unknown. (author)

Pheochromocytoma

Science.gov (United States)

... foods include: Some cheeses Some beers and wines Chocolate Dried or smoked meats Certain medications that can ... blood pressure with current treatment plan A family history of pheochromocytoma A family history of a related ...

[Clinical value of "Kou mode of hepatic hilar anastomosis" in resection of type III or IV hepatic hilar cholangiocarcinoma].

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He, Xiao-dong; Liu, Wei; Tao, Lian-yuan; Zhang, Zhen-huan; Cai, Lei; Zhang, Shuang-min

2009-08-01

To evaluate the surgical technique of "Kou mode of hepatic hilar anastomosis" in the treatment for type III or IV hilar cholangiocarcinoma. The clinical data of 89 patients with type III or IV hilar cholangiocarcinoma surgically treated in our department between Jan. 1990 and Jan. 2008 were retrospectively analyzed. Since January 2000, "Kou mode of hepatic hilar anastomosis" was performed for some patients with advanced hilar cholangiocarcinoma. The patients were divided into two groups: group A treated between 1990 and 1999, group B between 2000 and 2008. The rate of resection, therapeutic efficacy and complications in these two groups were compared, respectively. Of the 37 cases with hilar cholangiocarcinoma in group A, 4 were surgically treated (10.8%), with 1 (2.7%) radical resection and 3 (8.1%) palliative resection. Among the 52 cases with hilar cholangiocarcinoma in the group B, 35 (67.3%) received surgical resection, of them 15 (28.8%) underwent radical resection and 20 (38.5%) had palliative resection. Twenty-eight of these 35 cases underwent the "Kou mode of hepatic hilar anastomosis". The resection rate of advanced hilar cholangiocarcinoma in the group B was significantly higher than that in group A (P anastomosis" developed bile leakage to a varying degree and recovered after drainage and symptomatic treatment. The resection rate of type III or IV advanced hilar cholangiocarcinoma can be remarkably improved by using a novel alternative surgical technique called "Kou mode of hepatic hilar anastomosis". However, the long-term outcome still needs to be determined by close follow-up and further observation.

Digital hilar tomography. Comparison with conventional technique

International Nuclear Information System (INIS)

Schaefer, C.B.; Braunschweig, R.; Teufl, F.; Kaiser, W.; Claussen, C.D.

1993-01-01

The aim of the following study was to compare conventional hilar tomography and digital hilar tomography. 20 patients were examined both with conventional and digital hilar tomography using the same tomographic technique and the identical exposure dose. All patients underwent computed tomography of the chest as a golden standard. The digital technique, especially the edge-enhanced image version, showed superior image quality. ROC-analysis by 4 readers found equal diagnostic performance without any statistical difference. Digital hilar tomography shows a superior and constant image quality and lowers the rate of re-exposure. Therefore, digital hilar tomography is the preferable method. (orig.) [de

Laparoscopic resection of hilar cholangiocarcinoma.

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Lee, Woohyung; Han, Ho-Seong; Yoon, Yoo-Seok; Cho, Jai Young; Choi, YoungRok; Shin, Hong Kyung; Jang, Jae Yool; Choi, Hanlim

2015-10-01

Laparoscopic resection of hilar cholangiocarcinoma is technically challenging because it involves complicated laparoscopic procedures that include laparoscopic hepatoduodenal lymphadenectomy, hemihepatectomy with caudate lobectomy, and hepaticojejunostomy. There are currently very few reports describing this type of surgery. Between August 2014 and December 2014, 5 patients underwent total laparoscopic or laparoscopic-assisted surgery for hilar cholangiocarcinoma. Two patients with type I or II hilar cholangiocarcinoma underwent radical hilar resection. Three patients with type IIIa or IIIb cholangiocarcinoma underwent extended hemihepatectomy together with caudate lobectomy. The median (range) age, operation time, blood loss, and length of hospital stay were 63 years (43-76 years), 610 minutes (410-665 minutes), 650 mL (450-1,300 mL), and 12 days (9-21 days), respectively. Four patients had a negative margin, but 1 patient was diagnosed with high-grade dysplasia on the proximal resection margin. The median tumor size was 3.0 cm. One patient experienced postoperative biliary leakage, which resolved spontaneously. Laparoscopic resection is a feasible surgical approach in selected patients with hilar cholangiocarcinoma.

Diagnostic value of MRI for hepatic hilar cholangiocarcinoma

International Nuclear Information System (INIS)

Wang Zhen; Zuo Yujiang; Sun Lihui; Zhou Jian; Shen Bingqi

2010-01-01

Objective: To investigate the value of MRI in the diagnosis of hepatic hilar cholangiocarcinoma. Methods: Sixty-four patients with hepatic hilar cholangiocarcinomas confirmed by surgery or pathology underwent MRI using a 1.5-T superconductive MR system including conventional unenhanced MRI, MRCP and dynamic contrast-enhanced MRI with Gd-DTPA. Results: Dilatation of the intrahepatic biliary tree with narrowing, occlusion or filling defects in the hepatic hilar bile ducts was noted in all 64 cases. Unenhanced MR[ showed T 1 - and T 2 -hyperintense hilar masses in 42 patients and was normal in the remaining 22 patients. The hilar masses demonstrated slow, progressive and delayed enhancement patterns. There was enhancement of the thickened bile duct wall with luminal narrowing in the 22 patients without hilar masses. Conclusion: The characteristic MRI findings of enhancing hepatic hilar mass and bile duct wall thickening together with MRCP are valuable for diagnosing hepatic hilar cholangiocarcinomas. (authors)

A patient with bilateral pheochromocytoma as part of a Von Hippel-Lindau (VHL syndrome type 2C

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Rinkes Inne

2007-10-01

Full Text Available Abstract Background Von Hippel-Lindau (VHL disease is an autosomal dominant inherited disease. It is relatively recent that type 2C was identified as a separate group solely presenting with pheochromocytomas. As an illustration, an interesting case is presented of a pregnant woman with refractory hypertension. It proved to be the first manifestation of bilateral pheochromocytomas. The family history may indicate the diagnosis, but only identification of a germ line mutation in the DNA of a patient will confirm carriership. Case presentation A 27 year pregnant patient with intra uterine growth retardation presented with hypertension and pre-eclampsia. Magnetic resonance imaging revealed bilateral adrenal pheochromocytoma. She underwent laparoscopic adrenelectomy and a missense mutation (Gly93Ser in exon 1 of the VHL gene on chromosome 3 (p25 – p26 was shown in the patient, her father and her daughter confirming the diagnosis of VHL. Conclusion In almost all VHL families molecular genetic analysis of DNA will demonstrate an inherited mutation. Because of the involvement in several organs, periodic clinical evaluation should take place in a well coordinated, multidisciplinary setting. VHL disease can be classified into several subtypes. VHL type 2C patients present with pheochromocytomas without evidence of haemangioblastomas in the central nervous system and/or retina and a low risk of renal cell carcinoma. Therefore, in such families, periodic clinical screening can be focussed on pheochromocytomas.

Pheochromocytomas / Paragangliomas and two cases

African Journals Online (AJOL)

micturition, and various drugs (gluca- gon, tyramine, tricylic antidepressants, etc). Paroxysms usually last minutes to an hour. Note. Many of the symptoms and signs associated with pheochromocytoma are fairly typical of many other clinical con- ditions (table 1). Pheochromocytoma is therefore often referred to as “the great.

Simultaneous adrenal pheochromocytoma and carotid body paraganglioma in a woman

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Han, Eun Ji; Lee, Sang Hoon; Song, In Uk; Chung, Yong An; Maeng, Lee So [The Catholic Univ. of Korea, Incheon (Korea, Republic of)

2012-03-15

Simultaneous occurrence of carotid body tumor and pheochromocytoma is rare. Most pheochromocytomas have grown on adrenal medulla, but some of the pheochromocytoma patients have multifocal paragangliomas arising from extraaderenal tissues. Pheochromocytomas and paragangliomas occur as sporadic tumors or they can be associated with several hereditary syndromes such as (1) multiple endocrine neoplasia type 2 (MEN 2), (2) Von Hippel Lindau disease (VHL) and (3) neurofibromatosis type 1 as an unusual genetic cause of pheochromocytomas. Genetic testing is recommended for patients with an apparently sporadic pheochromocytoma under the age of 20 years with a family history or features suggestive of hereditary pheochromocytoma or for patients with sympathetic paragangliomas. For individuals who do not meet these criteria, genetic testing is optional. Discovery of pheochromocytoma or paraganglioma in a patient should lead to a careful search to rule out multifocal lesions and/or hereditary syndromes. The diagnosis of pheochromocytoma and paraganglioma is made by biochemical testing, and imaging is done to localize the tumor for surgical planning. F 18 FDG PET has proved to be an effective tool in the localization of pheochromocytomas and paragangliomas.

The Epidemiology of Pheochromocytoma - Is the Incidence Increasing?

DEFF Research Database (Denmark)

Ebbehoj, A; Søndergaard, Esben; Trolle, Christian

with pheochromocytoma, but the true incidence, morbidity and mortality associated with pheochromocytoma remains to be fully elucidated. Materials and methods The cohort of potential cases of pheochromocytoma patients was identified with a data extract on relevant diagnoses from the National Patient Registry covering...

[The safety and effect of transhepatic hilar approach for the treatment of bismuth type Ⅲ and Ⅳ hilar cholangiocarcinoma].

Science.gov (United States)

He, M; Wang, H L; Yan, J Y; Xu, S W; Chen, W; Wang, J

2018-05-01

Objective: To compare the efficiency between the transhepatic hilar approach and conventional approach for the surgical treatment of Bismuth type Ⅲ and Ⅳ hilar cholangiocarcinoma. Methods: There were 42 consecutive patients with hilar cholangiocarcinoma of Bismuth type Ⅲ and Ⅳ who underwent surgical treatment at Department of Biliary-Pancreatic Surgery, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University from January 2008 to December 2013.The transhepatic hilar approach was used in 19 patients and conventional approach was performed in 23 patients.There were no differences in clinical parameters between the two groups(all P >0.05). The t-test was used to analyze the measurement data, and the χ(2) test was used to analyze the count data.Kaplan-Meier analysis was used to analyze the survival period.Multivariate COX regression analysis was used to analyze the prognosis factors. Results: Among the 19 patients who underwent transhepatic hilar approach, 3 patients changed the operative planning after reevaluated by exposing the hepatic hilus.The intraoperative blood was 300(250-400)ml in the transhepatic hilar approach group, which was significantly less than the conventional approach group, 800(450-1 300)ml( t =4.276, P =0.00 1), meanwhile, the R0 resection rate was significantly higher in the transhepatic hilar approach group than in the conventional approach group(89.4% vs . 52.2; χ(2)=6.773, P =0.009) and the 3-year and 5-year cumulative survival rate was better in the transhepatic hilar approach group than in the conventional approach group(63.2% vs . 47.8%, 26.3% vs . 0; χ(2)=66.363, 127.185, P =0.000). On univariate analysis, transhepatic hilar approach, intraoperative blood loss, intraoperative blood transfusion, R0 resection and lymph node metastasis were significant risk factors for patient survival(all P hilar approach, intraoperative blood loss, R0 resection and lymph node metastasis were significant independent risk factors for

[Malignant pheochromocytoma].

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Mornex, R; Berthezene, F; Peyrin, L; Tran Minh, V; Martin, J P; Fulchiron, D

1979-11-01

The reported incidence of malignant pheochromocytoma varies from series to series. In this series 4 cases (7.2 p. 100) were observed out of a total of 55. In two cases the tumour progressed rapidly but in the other two cases, metastases were detected 3 to 12 years after the apparent cure of a histologically benign pheochromocytoma. The urinary levels of catecholamines and their metabolites gave no indication of the underlying malignancy. The diagnosis was only made from the clinical and radiological detection of metastases (2 hepatic, 2 bone). There is no satisfactory treatment and various therapeutic methods have to be used in succession; surgery for a single metastasis, radiotherapy and antiadrenergic agents to combat clinical manifestations. The natural history of this tumour is relatively long.

Inferior hilar window on lateral chest radiographs

International Nuclear Information System (INIS)

Park, C.K.; Webb, W.R.; Klein, J.S.

1990-01-01

This paper determines the accuracy of lateral chest radiography in the detection of masses in the inferior hilar window, a normally avascular hilar region anterior to the lower lobe bronchi. Fifty patients with normal thoracic CT scans and 25 with hilar masses/adenopathy were selected retrospectively. The 75 corresponding lateral chest radiographs were blindly evaluated for visibility of the anterior walls of the lower lobe bronchi and the presence and laterality of abnormal soft tissue (>1 cm) in the inferior hilar window. Only a 7 x 7-cm square of the lateral radiograph was viewed

Pathological aspects of so called "hilar cholangiocarcinoma".

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Castellano-Megías, Víctor M; Ibarrola-de Andrés, Carolina; Colina-Ruizdelgado, Francisco

2013-07-15

Cholangiocarcinoma (CC) arising from the large intrahepatic bile ducts and extrahepatic hilar bile ducts share clinicopathological features and have been called hilar and perihilar CC as a group. However, "hilar and perihilar CC" are also used to refer exclusively to the intrahepatic hilar type CC or, more commonly, the extrahepatic hilar CC. Grossly, a major distinction can be made between papillary and non-papillary tumors. Histologically, most hilar CCs are well to moderately differentiated conventional type (biliary) carcinomas. Immunohistochemically, CK7, CK20, CEA and MUC1 are normally expressed, being MUC2 positive in less than 50% of cases. Two main premalignant lesions are known: biliary intraepithelial neoplasia (BilIN) and intraductal papillary neoplasm of the biliary tract (IPNB). IPNB includes the lesions previously named biliary papillomatosis and papillary carcinoma. A series of 29 resected hilar CC from our archives is reviewed. Most (82.8%) were conventional type adenocarcinomas, mostly well to moderately differentiated, although with a broad morphological spectrum; three cases exhibited a poorly differentiated cell component resembling signet ring cells. IPNB was observed in 5 (17.2%), four of them with an associated invasive carcinoma. A clear cell type carcinoma, an adenosquamous carcinoma and two gastric foveolar type carcinomas were observed.

Evaluation of hilar 67Ga-citrate uptake in bronchogenic carcinoma

International Nuclear Information System (INIS)

Matsuno, Noriyo; Kuyama, Junpei; Uchida, Yoshitaka; Hatano, Osamu; Imai, Yasunori; Uno, Kimiichi; Minoshima, Satoshi.

1995-01-01

We evaluated hilar uptake on 67 Ga-citrate scintigraphy and correlated with hilar lymph node metastases in one-hundred seventeen cases (two-hundred thirty four hila) of bronchogenic carcinoma. Hilar uptake was classified into three grades independently on anterior and posterior chest views: no uptake (grade 0), uptake equal to that in the thoracic vertebrae (grade 1), and uptake higher than that in the thoracic vertebrae (grade 2). If a summed grade of anterior and posterior view was larger than 3, hilar uptake was considered as positive. In forty-four cases (forty-nine hila) with hilar lymph node metastases, positive hilar uptake was found in ten cases (eleven hila), resulting in sensitivity of 22% based on cases and 23% based on hila. In seventy-three cases (one-hundred forty-six hila) without hilar lymph node metastases, positive hilar uptake was found in six cases (ten hila), resulting in specificity of 91% based on cases and 93% based on hila. In comparison to scintigraphic findings of unilaterally positive hilar uptake, findings of bilaterally positive hilar uptake showed significantly lower accuracy in determining presence of hilar lymph node metastases. No definite correlation between hilar uptake and histopathology or 67 Ga-citrate uptake in a primary tumor was observed. The results indicate that usefulness of 67 Ga-citrate scintigraphy is limited when evaluating hilar lymph node metastases in bronchogenic carcinoma. (author)

Pheochromocytoma presenting as takotsubo-like cardiomyopathy following delivery.

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Jóźwik-Plebanek, Katarzyna; Pęczkowska, Mariola; Klisiewicz, Anna; Wrzesiński, Kazimierz; Prejbisz, Aleksander; Niewada, Maciej; Kabat, Marek; Szperl, Małgorzata; Eisenhofer, Graeme; Lenders, Jacques W; Januszewicz, Andrzej

2014-12-01

Diagnosis of pheochromocytoma during pregnancy can be difficult, and the tumor carries an unfavorable prognosis if not diagnosed and treated in a timely manner. To present a case of Takotsubo-like cardiomyopathy characterized by transient left ventricular apical ballooning due to pheochromocytoma following delivery. A few hours after Caesarean section, a 32-year-old Caucasian female presented with pulmonary edema followed by cardiac arrest with echocardiographic and ventriculographic evidence of reversible acute myocardial failure characteristic of Takotsubo-like cardiomyopathy. A previously unrecognized adrenal pheochromocytoma was found during her clinical work-up. Left ventricle (LV) function normalized after surgical removal of the tumor, which was carried out after implementing an alpha-adrenoreceptor blockade. Hemorrhagic necrosis of the pheochromocytoma was seen on histopathologic analysis; this may have triggered the sequence of events leading to the development of Takotsubo-like cardiomyopathy and hemodynamic collapse. To the best of our knowledge, this is the first reported case of Takotsubo-like cardiomyopathy related to pheochromocytoma following delivery. This emphasizes the increased cardiovascular risk if pheochromocytoma is not diagnosed and treated in a timely manner, especially during pregnancy.

Hilar cholangiocarcinoma: expert consensus statement.

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Mansour, John C; Aloia, Thomas A; Crane, Christopher H; Heimbach, Julie K; Nagino, Masato; Vauthey, Jean-Nicolas

2015-08-01

An American Hepato-Pancreato-Biliary Association (AHPBA)-sponsored consensus meeting of expert panellists met on 15 January 2014 to review current evidence on the management of hilar cholangiocarcinoma in order to establish practice guidelines and to agree consensus statements. It was established that the treatment of patients with hilar cholangiocarcinoma requires a coordinated, multidisciplinary approach to optimize the chances for both durable survival and effective palliation. An adequate diagnostic and staging work-up includes high-quality cross-sectional imaging; however, pathologic confirmation is not required prior to resection or initiation of a liver transplant trimodal treatment protocol. The ideal treatment for suitable patients with resectable hilar malignancy is resection of the intra- and extrahepatic bile ducts, as well as resection of the involved ipsilateral liver. Preoperative biliary drainage is best achieved with percutaneous transhepatic approaches and may be indicated for patients with cholangitis, malnutrition or hepatic insufficiency. Portal vein embolization is a safe and effective strategy for increasing the future liver remnant (FLR) and is particularly useful for patients with an FLR of hilar cholangiocarcinoma should be evaluated for a standard trimodal protocol incorporating external beam and endoluminal radiation therapy, systemic chemotherapy and liver transplantation. Post-resection chemoradiation should be offered to patients who show high-risk features on surgical pathology. Chemoradiation is also recommended for patients with locally advanced, unresectable hilar cancers. For patients with locally recurrent or metastatic hilar cholangiocarcinoma, first-line chemotherapy with gemcitabine and cisplatin is recommended based on multiple Phase II trials and a large randomized controlled trial including a heterogeneous population of patients with biliary cancers. © 2015 International Hepato-Pancreato-Biliary Association.

Adrenal medullary hyperplasia. Hyperplasia-pheochromocytoma sequence.

Science.gov (United States)

Kurihara, K; Mizuseki, K; Kondo, T; Ohoka, H; Mannami, M; Kawai, K

1990-09-01

We present a case of unilateral adrenal medullary hyperplasia in a 63-year-old woman with clinical signs and symptoms of pheochromocytoma unassociated with multiple endocrine neoplasia. The surgically removed adrenal gland revealed diffuse medullary hyperplasia with multiple micronodules measuring up to 2 mm. The micronodules were composed of enlarged chromaffin cells with atypia, histologically similar to those of pheochromocytoma, forming small solid alveolar patterns separated by a fibrovascular stroma. Removal of the hyperplastic adrenal gland resulted in disappearance of paroxysmal nocturnal hypertension and palpitation. These results suggest that diffuse and nodular medullary hyperplasia is the precursor of pheochromocytoma.

[About the signs of malignant pheochromocytoma].

Science.gov (United States)

Simonenko, V B; Makanin, M A; Dulin, P A; Vasilchenko, M I; Lesovik, V S

2012-01-01

Morphological criteria for malignant pheochromocytoma remain to be developed According to the WHO recommendations, the sole absolute criteria is the presence of metastases in the organs normally containing no chromaffin tissue. Such signs as cellular and nuclear polymorphism, mytotic activity, vascular invasion, capsular ingrowth are not sufficient to describe a pheochromocytoma as malignant. It is equally dfficult to differentiate between malignant and benign tumours based on histological data since histologically mature neoplasms can produce metastases. Based on the results of original studies, the authors believe that such histological features as vascular and capsular invasion do not necessarily suggest unfavourable prognosis. Therefore, the conclusion of malignancy based on such features can not be regarded as absolute. Probably such neoplasms should be called "pheochromocytomas with morphological signs of malignant growths". They should be referred to the tumours with uncertain malignancy potential based on the known discrepancy between morphological structure and biological activity of neoplasms. Comparative studies of clinical and morphological features of pheochromocytomas showed that their histological type (alveolar; solid, dyscomplexed, trabecular) and morphological signs of malignant growth influence both the clinical picture and arterial hypertension. There are no significant relationship between the above morphological signs, timour mass and clinical manifestations of pheochromocytomas.

Predictors of recurrence in pheochromocytoma.

Science.gov (United States)

Press, Danielle; Akyuz, Muhammet; Dural, Cem; Aliyev, Shamil; Monteiro, Rosebel; Mino, Jeff; Mitchell, Jamie; Hamrahian, Amir; Siperstein, Allan; Berber, Eren

2014-12-01

The recurrence rate of pheochromocytoma after adrenalectomy is 6.5-16.5%. This study aims to identify predictors of recurrence and optimal biochemical testing and imaging for detecting the recurrence of pheochromocytoma. In this retrospective study we reviewed all patients who underwent adrenalectomy for pheochromocytoma during a 14-year period at a single institution. One hundred thirty-five patients had adrenalectomy for pheochromocytoma. Eight patients (6%) developed recurrent disease. The median time from initial operation to diagnosis of recurrence was 35 months. On multivariate analysis, tumor size >5 cm was an independent predictor of recurrence. One patient with recurrence died, 4 had stable disease, 2 had progression of disease, and 1 was cured. Recurrence was diagnosed by increases in plasma and/or urinary metanephrines and positive imaging in 6 patients (75%), and by positive imaging and normal biochemical levels in 2 patients (25%). Patients with large tumors (>5 cm) should be followed vigilantly for recurrence. Because 25% of patients with recurrence had normal biochemical levels, we recommend routine imaging and testing of plasma or urinary metanephrines for prompt diagnosis of recurrence. Copyright © 2014 Elsevier Inc. All rights reserved.

Anatomic renal artery branch microdissection to facilitate zero-ischemia partial nephrectomy.

Science.gov (United States)

Ng, Casey K; Gill, Inderbir S; Patil, Mukul B; Hung, Andrew J; Berger, Andre K; de Castro Abreu, Andre Luis; Nakamoto, Masahiko; Eisenberg, Manuel S; Ukimura, Osamu; Thangathurai, Duraiyah; Aron, Monish; Desai, Mihir M

2012-01-01

Robot-assisted and laparoscopic partial nephrectomies (PNs) for medial tumors are technically challenging even with the hilum clamped and, until now, were impossible to perform with the hilum unclamped. Evaluate whether targeted vascular microdissection (VMD) of renal artery branches allows zero-ischemia PN to be performed even for challenging medial tumors. A prospective cohort evaluation of 44 patients with renal masses who underwent robot-assisted or laparoscopic zero-ischemia PN either with anatomic VMD (group 1; n=22) or without anatomic VMD (group 2; n=22) performed by a single surgeon from April 2010 to January 2011. Zero-ischemia PN with VMD incorporates four maneuvers: (1) preoperative computed tomographic reconstruction of renal arterial branch anatomy, (2) anatomic dissection of targeted, tumor-specific tertiary or higher-order renal arterial branches, (3) neurosurgical aneurysm microsurgical bulldog clamp(s) for superselective tumor devascularization, and (4) transient, controlled reduction of blood pressure, if necessary. Baseline, perioperative, and postoperative data were collected prospectively. Group 1 tumors were larger (4.3 vs 2.6 cm; p=0.011), were more often hilar (41% vs 9%; p=0.09), were medial (59% and 23%; p=0.017), were closer to the hilum (1.46 vs 3.26 cm; p=0.0002), and had a lower C index score (2.1 vs 3.9; p=0.004) and higher RENAL nephrometry scores (7.7 vs 6.2; p=0.013). Despite greater complexity, no group 1 tumor required hilar clamping, and perioperative outcomes were similar to those of group 2: operating room time (4.7 and 4.1h), median blood loss (200 and 100ml), surgical margins for cancer (all negative), major complications (0% and 9%), and minor complications (18% and 14%). The median serum creatinine level was similar 2 mo postoperatively (1.2 and 1.3mg/dl). The study was limited by the relatively small sample size. Anatomic targeted dissection and superselective control of tumor-specific renal arterial branches facilitate

On the compensating filter in pulmonary hilar tomography

International Nuclear Information System (INIS)

Okayama, Akio; Nakanishi, Takashi; Fujikawa, Tsuyoshi; Inaba, Nobuo; Fujii, Shinichi

1979-01-01

In recent years, it has been an important theme for the diagnosis of pulmonary hilar cancer to evaluate whether the lesion in hilar lymph nodes and the vicinity of the hilar bronchus exists or does not. It is shown that pulmonary hilar region is from main bronchus to segmental bronchus which is separating subsegmental bronchus. It is difficult to make the optimal density for all area of the pulmonary hilar region on a same film, because there is too much difference of density between medial region under the veil of thoracic vertebra, sternum, heart and great vessels, and lateral region in conventional tomography. Therefore, a compensating filter was constructed to equalize the uneven density of pulmonary hilar region on a X-ray film. As a result, trachea, main bronchus, upper local and segmental bronchus were observed simultaneously and serially on a same film by using this filter. In addition, we could study the condition in the inside and outside of bronchial wall in detail, and have many informations compared with conventional tomogram. Particularly, this is an useful method to determine the range of lesion in the pulmonary hilar cancer. (author)

Hilar enlargement in respiratory syncytial virus pneumonia

International Nuclear Information System (INIS)

Odita, J.C.; Aghahowa, J.E.; Nwankwo, M.

1989-01-01

The clinical and radiographic features of ten children with hilar enlargement in association with proven Respiratory Syncytial Virus (RSV) infection are described. Hilar enlargement was seen in 10/35 children with RSV infection, and was invariably unilateral and right sided. It is recommended that RSV pneumonia be considered in children with unilateral hilar enlargement if tuberculosis has been excluded, and the onset of disease is rapid. (orig.)

Post-transplant Lymphoproliferative Disorder Arising from Renal Allograft Parenchyma: A Case Report

Energy Technology Data Exchange (ETDEWEB)

Park, Byung Kwan; Kim, Chan Kyo; Kwon, Ghee Young [Samsung Medical Center, Sungkyunkwan University College of Medicine, Seoul (Korea, Republic of)

2010-06-15

Post-transplant lymphoproliferative disorder (PTLD) is a rare but serious complication that occurs in patients undergoing kidney transplantation. PTLD usually manifests as a renal hilar mass comprised of histologically B-lymphocytes. We report our experience of managing a patient with PTLD arising from renal parenchyma. Ultrasonographic and MR imaging features of this unusual PTLD suggested differentiated renal cell carcinoma arising from the renal allograft

Hilar height ratio in normal Korea

International Nuclear Information System (INIS)

Yoo, Kyung Ho; Lee, Nam Joon; Seol, Hae Young; Chung, Kyoo Byung

1979-01-01

Hilar displacement is one of the significant sign of pulmonary volume change. The hilar height ratio (HHR) is a value that express the normal position of hilum in its hemithorax, and it is calculated by dividing the distance from the hilum to the lung apex by the distance from the hilum to the diaphragm. Displacement of one hilum is usually easy to detect but both are displaced in the same direction especially, recognition is more difficult. Knowledge of normal HHR allows evaluation of hilar positional change even when the relative hilar position are not altered. Normal chest PA views of 275 cases taken at Korea University Hospital during the period of April 1978 to Jun 1979 were analyzed. The right hilum is positioned in lower half of the right hemithorax, while the left hilum is situated in the upper half of left hemithorax. The difference of hilar ratio according to age group is slight, but there is significant difference between right-HHR and left-HHR. The value of right-HHR is 1.28 ± 0.14, the value of left-HHR is 0.88 ± 0.09.

CT anatomy of hilar lymphadenopathy

International Nuclear Information System (INIS)

Sone, S.; Higashihara, T.; Morimoto, S.; Ikezoe, J.; Arisawa, J.; Monden, Y.; Nahakara, K.

1983-01-01

The normal distributions of lymph nodes in the pulmonary hili is diagrammatically shown with a typical computed tomographic (CT) demonstration of hilar lymphadenopathy. On the basis of observations in anatomic cross sections of cadaver lungs, the lympth nodes in the right lung can be divided into four principal groups (right upper lobe, interlobar, middle lobe, and lower lobe) and in the left lung into three principal groups (left upper lobe, interlobar, and lower lobe). Most of the hilar lymph nodes are situated along the bronchi in close relation with the pulmonary vascular branches. Because of this close proximity, contrast-enhanced CT images are indispensable for precise CT interpretation of a hilar lymphadenopathy

Pheochromocytoma: a rare cause of childhood hypertensive encephalopathy

International Nuclear Information System (INIS)

Aftab, S.; Yasmeen, T.; Hamid, M.H.; Sarwar, M.; Sipra, H.; Sheikh, A.; Haider, N.; Hanif, G.

2012-01-01

Pheochromocytomas are rare neuroendocrine tumours of chromaffin tissues. They are catecholamine secreting tumours which cause severe hypertension and other systemic disturbances. Of all the causes of childhood hypertension, pheochromocytoma constitutes less than 1%. We report the case of a 12 years old child who presented with hypertensive encephalopathy, confirmed histologically to be secondary to pheochromocytoma, and cured with meticulous critical care and surgical resection. (author)

Pheochromocytoma as a rare cause of hypertension in a 46 X, i(X)(q10) turner syndrome: a case report and literature review.

Science.gov (United States)

Shin, Ji Yeon; Kim, Bo Hyun; Kim, Young Keum; Kim, Tae Hwa; Kim, Eun Heui; Lee, Min Jin; Kim, Jong Ho; Jeon, Yun Kyung; Kim, Sang Soo; Kim, In Joo

2018-05-10

Cardiovascular disease (CVD) presents the most serious health problems and contributes to the increased mortality in young women with Turner syndrome. Arterial hypertension in Turner syndrome patients is significantly more prevalent than that in a general age-matched control group. The aetiology of hypertension in Turner syndrome varies, even in the absence of cardiac anomalies and obvious structural renal abnormalities. Pheochromocytoma is an extremely rare cause among various etiologies for hypertension in patients with Turner syndrome. Here, we reported a pheochromocytoma as a rare cause of hypertension in Turner syndrome patient. A 21-year-old woman who has diagnosed with Turner syndrome with a karyotype of 46,X,i(X)(q10) visited for hypertension and mild headache. Transthoracic echography (TTE) showed no definite persistent ductus arteriosus shunt flow and cardiac valve abnormalities. Considering other important secondary causes like pheochromocytoma, hormonal studies were performed and the results showed increased serum norepinephrine, serum normetanephrine, and 24 h urine norepinephrine. We performed an abdominal computed tomography (CT) to confirm the location of pheochromocytoma. Abdominal CT showed a 1.9 cm right adrenal mass. I-131 meta-iodobenzylguanidine (MIBG) scintigraphy showed a right adrenal uptake. Laparoscopic adrenalectomy was performed and confirmed a pheochromocytoma. After surgery, blood pressure was within normal ranges and postoperative course was uneventful, and no recurrence developed via biochemical tests and abdominal CT until 24 months. Our case and previous literatures suggest that hypertension caused by pheochromocytoma which is a rare but important and potentially lethal cause of hypertension in Turner syndrome. This case underlines the importance of early detection of pheochromocytoma in Turner syndrome. Clinicians should keep in mind that pheochromocytoma can be a cause of hypertension in patients with Turner syndrome.

Perioperative Management of Hilar Cholangiocarcinoma.

Science.gov (United States)

Poruk, Katherine E; Pawlik, Timothy M; Weiss, Matthew J

2015-10-01

Cholangiocarcinoma is the most common primary tumor of the biliary tract although it accounts for only 2 % of all human malignancies. We herein review hilar cholangiocarcinoma including its risk factors, the main classification systems for tumors, current surgical management of the disease, and the role chemotherapy and liver transplantation may play in selected patients. We performed a comprehensive literature search using PubMed, Medline, and the Cochrane library for the period 1980-2015 using the following MeSH terms: "hilar cholangiocarcinoma", "biliary cancer", and "cholangiocarcinoma". Only recent studies that were published in English and in peer reviewed journals were included. Hilar cholangiocarcinoma is a disease of advanced age with an unclear etiology, most frequently found in Southeast Asia and relatively rare in Western countries. The best chance of long-term survival and potential cure is surgical resection with negative surgical margins, but many patients are unresectable due to locally advanced or metastatic disease at diagnosis. As a result of recent efforts, new methods of management have been identified for these patients, including preoperative portal vein embolism and biliary drainage, neoadjuvant chemotherapy with subsequent transplantation, and chemoradiation therapy. Current management of hilar cholangiocarcinoma depends on extent of the tumor at presentation and includes surgical resection, liver transplantation, portal vein embolization, and chemoradiation therapy. Our understanding of hilar cholangiocarcinoma has improved in recent years and further research offers hope to improve the outcome in patients with these rare tumors.

Nuclear medical diagnosis and therapy of pheochromocytoma

International Nuclear Information System (INIS)

Fischer, M.; Winterberg, B.; Vetter, H.; Mueller-Rensing, R.

1984-01-01

After prior labeling of the kidneys or the skeletal system for easier orientation, 0.3 - 0.7 mCi of I-131-MIBG (Henning-Berlin) were injected intravenously and scans were obtained with a gamma-camera-computer system 24 to 120 hours later. 22 patients showed clinical and biochemical evidence of pheochromocytoma; the diagnosis was confirmed surgically in 19. Two patients (16 years old) with metastatic pheochromocytoma and a female aged 73 years with intra-adrenal pheochromocytoma ineligible for surgery because of catecholamine-induced myocarditis currently undergo fractionated I-131-MIBG treatment with single doses of 65 - 135 mCi. The tumor response was assessed in 2 females by determining the volume of reference tumors with computer tomography; by 24-hour urinary catecholamine assays; and by checking drug consumption. Scintigraphy showed intra-adrenal pheochromocytoma to be present in 17 cases (left, n=11; right, n=5; bilateral, n=1). Two patients were found to have medullary hyperplasia, while 3 presented with multilocular malignant pheochromocytoma. Treatment substantially improved the clinical symptoms in 3 females. Determination of tumor volume by computer tomography showed a tumor reduction of 61% in 1 patient after a total dose of 415 mCi and of 10% after a total dose of 365 mCi in another patient. Experiences sofar available suggest that I-131-MIBG is a useful specific radionuclide for the diagnosis and treatment of pheochromocytoma. (Author)

Pheochromocytoma treated by laparoscopic surgery Feocromocitoma tratado por cirurgia laparoscópica

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Lísias Nogueira Castilho

2000-06-01

Full Text Available OBJECTIVE: To evaluate the results of the laparoscopic technique in the treatment of adrenal pheochromocytoma. METHOD: Ten patients, 7 men and 3 women, between 10 and 67 years of age (mean 48 with pheochromocytoma underwent transperitoneal laparoscopic adrenalectomy and were evaluated retrospectively, based on clinical, laboratory, and pathological diagnosis. In all cases there was a solid unilateral adrenal tumor, 5 on the left side and 5 on the right side, whose greater diameter varied from 7 to 80 mm (mean 32. Nine of the 10 patients were chronically hypertensive or had already had hypertensive crises. One patient was normotensive, but presented metabolic alterations suggestive of adrenergic hyperfunction. RESULTS: No deaths occurred in this series. There were two (20% conversions to open surgery, one due to venous bleeding and one due to the difficulty of dissection behind the vena cava in a patient presenting a partially retro-caval tumor. Surgical time in the 8 non-converted cases ranged from 70 to 215 minutes (mean 136. One patient (10% received blood transfusion, and another (10% presented two complications - acute renal failure and a subcutaneous infection. Both had been converted to open surgery. None of the non-converted cases was transfused or presented complications. Hospital discharge occurred between the 2nd and 11th post-operative day (mean 3. The pathological exam of the surgical specimens confirmed the diagnoses of pheochromocytoma in all 10 cases, one of them associated with an aldosterone-producing cortical tumor. CONCLUSIONS: Laparoscopic adrenalectomy for selected patients presenting pheochromocytoma is feasible and provides good results.OBJETIVO: Avaliar os resultados da utilização da técnica laparoscópica no tratamento do feocromocitoma de supra-renal. MÉTODO: Dez pacientes, sete homens e três mulheres, entre 10 e 67 anos de idade (média 48, com feocromocitoma, foram operados por via laparoscópica transperitoneal

Plasma metanephrines: a novel and cost-effective test for pheochromocytoma

Directory of Open Access Journals (Sweden)

G. Eisenhofer

2000-10-01

Full Text Available Pheochromocytomas are rare chromaffin cell tumors that nevertheless must be excluded in large numbers of patients who develop sustained or episodic hypertension as well as in many others with suggestive symptoms or with a familial history of pheochromocytoma. Diagnosis of pheochromocytoma depends importantly on biochemical evidence of excess catecholamine production by a tumor. Imperfect sensitivity and specificity of commonly available biochemical tests and the low incidence of the tumor among the tested population mean that considerable time and effort can be expended in confirming or ruling out pheochromocytoma in patients where the tumor is suspected. Measurements of plasma free metanephrines provide a superior test compared to other available tests for diagnosis of pheochromocytoma. In particular, the high sensitivity of plasma free metanephrines means that a normal test result reliably excludes all but the smallest of pheochromocytomas so that no other tests are necessary. Measurements of plasma free metanephrines, when systematically combined with other diagnostic procedures outlined in this review, provide a more efficient, reliable and cost-effective approach for diagnosis of pheochromocytoma than offered by previously available approaches.

Laparoscopic splenic hilar lymphadenectomy for advanced gastric cancer.

Science.gov (United States)

Hosogi, Hisahiro; Okabe, Hiroshi; Shinohara, Hisashi; Tsunoda, Shigeru; Hisamori, Shigeo; Sakai, Yoshiharu

2016-01-01

Laparoscopic distal gastrectomy has recently become accepted as a surgical option for early gastric cancer in the distal stomach, but laparoscopic total gastrectomy (LTG) has not become widespread because of technical difficulties of esophagojejunal anastomosis and splenic hilar lymphadenectomy. Splenic hilar lymphadenectomy should be employed in the treatment of advanced proximal gastric cancer to complete D2 dissection, but laparoscopically it is technically difficult even for skilled surgeons. Based on the evidence that prophylactic combined resection of spleen in total gastrectomy increased the risk of postoperative morbidity with no survival impact, surgeons have preferred laparoscopic spleen-preserving splenic hilar lymphadenectomy (LSPL) for advanced tumors without metastasis to splenic hilar nodes or invasion to the greater curvature of the stomach, and reports with LSPL have been increasing rather than LTG with splenectomy. In this paper, recent reports with laparoscopic splenic hilar lymphadenectomy were reviewed.

Metastatic pheochromocytoma in an 18-year-old

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Ali Cadili

2016-03-01

Full Text Available Pheochromocytoma most commonly presents with a combination of headache, sweating, and hypertension. This case report reviews the case of a young male patient presenting with hypertensive crisis following administration of general anesthetics and who was subsequently diagnosed with malignant pheochromocytoma. Up to date, no reliable predictive factors for malignant pheochromocytoma have been established. This case emphasizes the need for a systematic approach to the hypertensive crisis and advantages of nuclear imaging to differentiate benign from malignant disease through detection of local invasion and distant metastases. [Arch Clin Exp Surg 2016; 5(1.000: 48-51

Leiomyosarcoma of the renal pelvis

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Dhamne Sagar

2009-10-01

Full Text Available Leiomyosarcomas are rare malignant tumors of the kidney. They may arise from the renal capsule, renal vein, renal pelvic musculature or renal parenchyma. Renal pelvis is an uncommon site of occurrence, with around 10 cases reported in the literature so far. Here we present a 60-year-old male who presented with increased urinary frequency, lower limb weakness, anorexia and weight loss. Imaging showed a right renal mass. A renal cell carcinoma was suspected clinically. A right nephrectomy was performed, which showed a large circumscribed mass in the hilar region. Histology revealed a tumor mass arising from the renal pelvis. The tumor was composed of spindle cells arranged in fascicles. Immunohistochemistry showed tumor cells to be positive for smooth muscle actin (SMA and desmin (Des and negative for cytokeratin (CK, HMB 45, CD117 (C-kit, and CD34. That confirmed the diagnosis of leiomyosarcoma.

Morphometry of Hilar Ectopic Granule Cells in the Rat

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Pierce, Joseph P.; McCloskey, Daniel P.; Scharfman, Helen E.

2014-01-01

Granule cell (GC) neurogenesis in the dentate gyrus (DG) does not always proceed normally. After severe seizures (e.g., status epilepticus [SE]) and some other conditions, newborn GCs appear in the hilus. Hilar ectopic GCs (EGCs) can potentially provide insight into the effects of abnormal location and seizures on GC development. Additionally, hilar EGCs that develop after SE may contribute to epileptogenesis and cognitive impairments that follow SE. Thus, it is critical to understand how EGCs differ from normal GCs. Relatively little morphometric information is available on EGCs, especially those restricted to the hilus. This study quantitatively analyzed the structural morphology of hilar EGCs from adult male rats several months after pilocarpineinduced SE, when they are considered to have chronic epilepsy. Hilar EGCs were physiologically identified in slices, intracellularly labeled, processed for light microscopic reconstruction, and compared to GC layer GCs, from both the same post-SE tissue and the NeuroMorpho database (normal GCs). Consistently, hilar EGC and GC layer GCs had similar dendritic lengths and field sizes, and identifiable apical dendrites. However, hilar EGC dendrites were topologically more complex, with more branch points and tortuous dendritic paths. Three-dimensional analysis revealed that, remarkably, hilar EGC dendrites often extended along the longitudinal DG axis, suggesting increased capacity for septotemporal integration. Axonal reconstruction demonstrated that hilar EGCs contributed to mossy fiber sprouting. This combination of preserved and aberrant morphological features, potentially supporting convergent afferent input to EGCs and broad, divergent efferent output, could help explain why the hilar EGC population could impair DG function. PMID:21344409

Perfusion study in the pulmonary hilar region by SPECT

International Nuclear Information System (INIS)

Satoh, Katashi; Tanabe, Masatada; Kawase, Yoshiro

1990-01-01

Alveoli in the hilar region comprise the peripheral area containing daughter branches from subsegmental or one more divisional peripheral bronchi. Pulmonary perfusion in hilar region was examined by SPECT (single photon emission CT) in ten normal volunteers. ROI (region of interest) in the axial images were set in the hilar region, the upper, middle and lower lung fields with 10.8 mm thickness. Counts/one pixel (C/P) were calculated in these ROI. There was a tendency of C/P increase from upper to lower lung field. And there were no significant differences in C/P increase between hilar region and other axial fields. In the chronic obstructive pulmonary diseases, however, ventilation studies in the previous reports using Xe dynamic CT or PET (positron emission tomography) showed differences between outer region and hilar region. This method will be expected to evaluate the pulmonary perfusion not only in the whole lung but in different lung areas, including the hilar region in the chronic obstructive pulmonary diseases. (author)

Pheochromocytoma during Pregnancy

Science.gov (United States)

... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

Pheochromocytoma and gastrointestinal stromal tumors in patients with neurofibromatosis type I.

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Vlenterie, Myrella; Flucke, Uta; Hofbauer, Lorenz C; Timmers, Henri J L M; Gastmeier, Joerg; Aust, Daniela E; van der Graaf, Winette T A; Wesseling, Pieter; Eisenhofer, Graeme; Lenders, Jacques W M

2013-02-01

Neurofibromatosis I may rarely predispose to pheochromocytoma and gastrointestinal stromal tumors. A 59-year-old woman with neurofibromatosis I presented with pheochromocytoma of the left adrenal gland. During surgery, 3 gastrointestinal stromal tumors adjacent to the stomach and small intestine were removed. Despite appropriate thrombosis prophylaxis, the patient died of a pulmonary embolus 2 days postoperatively. The second patient, a 55-year-old man with neurofibromatosis I and bilateral pheochromocytomas, had several small gastrointestinal stromal tumors adjacent to the jejunum during surgery. A review of the literature was conducted to identify patients with neurofibromatosis I with concurrence of pheochromocytoma and gastrointestinal stromal tumors and to define the specific clinical features of these patients. In addition to our 2 patients, 12 other cases of neurofibromatosis I with concomitant occurrence of pheochromocytomas and gastrointestinal stromal tumors have been reported. Pheochromocytomas had adrenal locations in all patients. Two of the 14 patients had a mixed pheochromocytoma/ganglioneuroma. In 4 of the 14 patients, gastrointestinal stromal tumors were located along the stomach. The gastrointestinal stromal tumors in our 2 patients showed no somatic mutations in KIT and PDGFRA genes. A pulmonary embolism was diagnosed in 4 patients. The simultaneous occurrence of pheochromocytoma and gastrointestinal stromal tumor should be considered in all patients with neurofibromatosis I presenting with an abdominal mass with symptoms suggestive of pheochromocytoma. Therefore, a pheochromocytoma should be excluded before a patient with neurofibromatosis I undergoes surgery for a gastrointestinal stromal tumor because an undiagnosed pheochromocytoma carries a high risk of life-threatening cardiovascular complications during surgery. Finally, this combination may be associated with an increased risk for thromboembolic events, but more studies are necessary to

Differentiating immunoglobulin g4-related sclerosing cholangitis from hilar cholangiocarcinoma.

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Tabata, Taku; Kamisawa, Terumi; Hara, Seiichi; Kuruma, Sawako; Chiba, Kazuro; Kuwata, Go; Fujiwara, Takashi; Egashira, Hideto; Koizumi, Koichi; Fujiwara, Junko; Arakawa, Takeo; Momma, Kumiko; Kurata, Masanao; Honda, Goro; Tsuruta, Koji; Itoi, Takao

2013-03-01

Few studies have differentiated immunoglobulin G (IgG) 4-related sclerosing cholangitis (IgG4-SC) from hilar cholangiocarcinoma (CC). Thus, we sought to investigate useful features for differentiating IgG4-SC from hilar CC. We retrospectively compared clinical, serological, imaging, and histological features of six patients with IgG4-SC and 42 patients with hilar CC. In patients with hilar CC, obstructive jaundice was more frequent (philar CC patients (philar or hepatic duct was completely obstructed in 83% of hilar CC patients (philar bile duct stenosis, was more frequent in IgG4-SC patients (philar CC.

Pheochromocytoma in pregnancy: Case report | Ogutu | East African ...

African Journals Online (AJOL)

This is a case presentation of a 32 year old woman with pheochromocytoma diagnosed at 27 weeks of gestation, she was managed till term, induced and had assisted vaginal delivery. The pheochromocytoma was surgically re-sected successfully at six weeks postpartum.

Pheochromocytoma and paraganglioma

International Nuclear Information System (INIS)

Reckova, M.

2013-01-01

Pheochromocytoma and paraganglioma are rare tumors that originate from the cells of neural crest. Despite a wide variety of clinical features, the main remains the hypertension. Most of pheochromocytoma (FEO) and paraganglioma (PGL) represent sporadic tumors but about 20-30% of these tumors are familial. Besides standard imagining techniques, functional diagnostics plays important role, as well. The definitive treatment is surgical therapy, however in inoperable cases, radionuclide therapeutic methods are used. Systemic chemotherapy has only limited efficacy and, currently, targeted therapeutic approaches are studied. External-beam radiotherapy is used in palliative settings. The most frequent causes of morbidity and mortality are cardiovascular events, such as sudden death, heart attack, heart failure and cerebrovascular events. Early diagnosis is very important not just for prevention of disease dissemination, but for the prevention of possible complications, as well. (author)

Laparoscopic partial nephrectomy for endophytic hilar tumors

DEFF Research Database (Denmark)

Di Pierro, G B; Tartaglia, N; Aresu, L

2014-01-01

To analyze feasibility and outcomes of laparoscopic partial nephrectomy (LPN) for endophytic hilar tumors in low-intermediate (ASA I-II) risk patients.......To analyze feasibility and outcomes of laparoscopic partial nephrectomy (LPN) for endophytic hilar tumors in low-intermediate (ASA I-II) risk patients....

Graves′ disease allied with multiple pheochromocytoma

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Brahim Housni

2013-01-01

Full Text Available Pheochromocytoma is an uncommon cause of high blood pressure touching adults. The combination of severe hypertension in the triad of headache, sweating, and tachycardia should suggest this diagnosis; this clinical picture is similar to that of hyperthyroidism. We report the case of a 22-year-old patient with multiple pheochromocytoma associated with Graves′ disease revealed by malignant hypertension and discussed the difficulties of the diagnosis and the treatment approach.

Assessment of various hilar measurements on PA chest of Korean normal adults

International Nuclear Information System (INIS)

Ro, Hee Jeong; Kim, Hyun; Kang, Si Won; Park, Seog Hee; Bahk, Yong Whee

1990-01-01

Appreciation of the normal position of the hila is essential in screening and diagnosis of the diseases of the chest. Many authors assessed the hilar position in normal adults using PA chest roentgenograms. However there have been no previous publication regarding consolidated assessment of various measurements. The authors carried out the present study to evaluate various hilar measurements including hilar points, hilar distances, difference between both hilar heights, hilar height ratio, and the relationship between the level of the clavicle and hila on the 500 normal PA chest roentgenograms

Renal mass anatomic characteristics and perioperative outcomes of laparoscopic partial nephrectomy: a critical analysis.

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Tsivian, Matvey; Ulusoy, Said; Abern, Michael; Wandel, Ayelet; Sidi, A Ami; Tsivian, Alexander

2012-10-01

Anatomic parameters determining renal mass complexity have been used in a number of proposed scoring systems despite lack of a critical analysis of their independent contributions. We sought to assess the independent contribution of anatomic parameters on perioperative outcomes of laparoscopic partial nephrectomy (LPN). Preoperative imaging studies were reviewed for 147 consecutive patients undergoing LPN for a single renal mass. Renal mass anatomy was recorded: Size, growth pattern (endo-/meso-/exophytic), centrality (central/hilar/peripheral), anterior/posterior, lateral/medial, polar location. Multivariable models were used to determine associations of anatomic parameters with warm ischemia time (WIT), operative time (OT), estimated blood loss (EBL), intra- and postoperative complications, as well as renal function. All models were adjusted for the learning curve and relevant confounders. Median (range) tumor size was 3.3 cm (1.5-11 cm); 52% were central and 14% hilar. While 44% were exophytic, 23% and 33% were mesophytic and endophytic, respectively. Anatomic parameters did not uniformly predict perioperative outcomes. WIT was associated with tumor size (P=0.068), centrality (central, P=0.016; hilar, P=0.073), and endophytic growth pattern (P=0.017). OT was only associated with tumor size (Panatomic parameter predicted EBL. Tumor centrality increased the odds of overall and intraoperative complications, without reaching statistical significance. Postoperative renal function was not associated with any of the anatomic parameters considered after adjustment for baseline function and WIT. Learning curve, considered as a confounder, was independently associated with reduced WIT and OT as well as reduced odds of intraoperative complications. This study provides a detailed analysis of the independent impact of renal mass anatomic parameters on perioperative outcomes. Our findings suggest diverse independent contributions of the anatomic parameters to the

On the compensating filter in pulmonary hilar tomography

International Nuclear Information System (INIS)

Okayama, Akio; Nakanishi, Takashi; Fujikawa, Tsuyoshi

1980-01-01

It is difficult to make the optimal density for all area of the pulmonary hilar region on a same film in conventional tomography. But, it is possible to observe all area of the pulmonary hilar region on a same film by equalizing the uneven density of pulmonary hilar region with compensating filter. On this study, the filter which could compensated density in all shifts of Polytome U was constructed and discussed about diagnostic evaluation for those shifts. As a results, by using this filter, the density for all area of the pulmonary hilar region in all shifts of Polytome U could be compensated and grasp the mutral relation of its region. As a conclusion, using this filter is useful method to diagnose pulmonary hilar region. If it is possible to make a patient enough hold a breath, using circular shift is better than other's. If it is difficult to do so, it can not help using linear shift. In such a case, it shall be taken a tomograph in direction of linear shift fitting the object. (author)

A UNIQUE CASE OF PHEOCHROMOCYTOMA PRESENTING WITH HYPERTENSIVE RETINOPATHY

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Maji.S, Saha. ML, Kanwar KS, Das S, Bhagat P, Bhar P

2015-04-01

Full Text Available ABSTRACT Pheochromocytoma is an extremely uncommon tumor of childhood and there are several features that distinguish its presentation between adults and children. The incidence of pheochromocytoma in childhood is 10% of the adult incidence, occurring in approximately 1 in 500,000 children compared with 1 in 50,000 adults. Around 10% of childhood tumors are familial which is 4times the frequency in adults. Whereas only 7% of pheochromocytomas are bilateral in adults, the reported incidence of the same in children range from 24 % to as high as &70%.These tumors are known for their great diversity in clinical presentation. Greater than 50% of children present with headaches, fever, palpitation, thirst, polyuria, sweating, nausea and weight loss. However the commonest mode of presentation is sustained hypertension. Pheochromocytoma accounts for 0.5% of children with hypertension and must be considered once other causes have been eliminated. We here in report a unique case of a 13 year old girl who initially presented with bilateral hypertensive retinopathy and later found to have a pheochromocytoma on subsequent workup. Hypertensive retinopathy secondary to pheochromocytoma is itself a rare entity whose exact incidence in children is still unknown. This case highlights the importance of routine history, physical examination and measurement of bp. Prompt surgery can reverse the effect of hypertension and lead to good outcome as was evident in our case.

Spontaneous adrenal pheochromocytoma rupture complicated by intraperitoneal hemorrhage and shock

Directory of Open Access Journals (Sweden)

Kwasnik Edward

2011-08-01

Full Text Available Abstract MEN2A is a hereditary syndrome characterized by medullary thyroid carcinoma, hyperparathyroidism, and pheochromocytoma. Classically patients with a pheochromocytoma initially present with the triad of paroxysmal headaches, palpitations, and diaphoresis accompanied by marked hypertension. However, although reported as a rare presentation, spontaneous hemorrhage within a pheochromocytoma can present as an abdominal catastrophe. Unrecognized, this transformation can rapidly result in death. We report the only documented case of a thirty eight year old gentleman with MEN2A who presented to a community hospital with hemorrhagic shock and peritonitis secondary to an unrecognized hemorrhagic pheochromocytoma. The clinical course is notable for an inability to localize the source of hemorrhage during an initial damage control laparotomy that stabilized the patient sufficiently to allow emergent transfer to our facility, re-exploration for continued hemorrhage and abdominal compartment syndrome, and ultimately angiographic embolization of the left adrenal artery for control of the bleeding. Following recovery from his critical illness and appropriate medical management for pheochromocytoma, he returned for interval bilateral adrenal gland resection, from which his recovery was unremarkable. Our review of the literature highlights the high mortality associated with the undertaking of an operative intervention in the face of an unrecognized functional pheochromocytoma. This reinforces the need for maintaining a high index of suspicion for pheochromocytoma in similar cases. Our case also demonstrates the need for a mutimodal treatment approach that will often be required in these cases.

MDCT assessment of resectability in hilar cholangiocarcinoma.

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Ni, Qihong; Wang, Haolu; Zhang, Yunhe; Qian, Lijun; Chi, Jiachang; Liang, Xiaowen; Chen, Tao; Wang, Jian

2017-03-01

The purpose of this study is to investigate the value of multidetector computed tomography (MDCT) assessment of resectability in hilar cholangiocarcinoma, and to identify the factors associated with unresectability and accurate evaluation of resectability. From January 2007 to June 2015, a total of 77 consecutive patients were included. All patients had preoperative MDCT (with MPR and MinIP) and surgical treatment, and were pathologically proven with hilar cholangiocarcinoma. The MDCT images were reviewed retrospectively by two senior radiologists and one hepatobiliary surgeon. The surgical findings and pathologic results were considered to be the gold standard. The Chi square test was used to identify factors associated with unresectability and accurate evaluation of resectability. The sensitivity, specificity, and overall accuracy of MDCT assessment were 83.3 %, 75.9 %, and 80.5 %, respectively. The main causes of inaccuracy were incorrect evaluation of N2 lymph node metastasis (4/15) and distant metastasis (4/15). Bismuth type IV tumor, main or bilateral hepatic artery involvement, and main or bilateral portal vein involvement were highly associated with unresectability (P hilar cholangiocarcinoma. Bismuth type IV tumor and main or bilateral vascular involvement highly suggest the unresectability of hilar cholangiocarcinoma. Patients without biliary drainage have a more accurate MDCT evaluation of resectability. We suggest MDCT should be performed before biliary drainage to achieve an accurate evaluation of resectability in hilar cholangiocarcinoma.

Intraoperative evaluation of renal blood flow during laparoscopic partial nephrectomy with a novel Doppler system.

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Mues, Adam C; Okhunov, Zhamshid; Badani, Ketan; Gupta, Mantu; Landman, Jaime

2010-12-01

Hemostasis remains a major challenge associated with laparoscopic renal surgery. We evaluated a cost-effective novel Doppler probe (DP) for assessment of vascular control during laparoscopic partial nephrectomy (LPN). We prospectively collected data during LPN procedures. We documented tumor location and size as well as subjective quality of the hilar dissection. The DP was compared with our standard intraoperative ultrasound system (SUS) for the ability to detect blood flow during hilar dissection and to determine parenchymal ischemia around the tumor after clamping of the renal vessels. Twenty patients underwent LPN by a single surgeon. The mean tumor size was 3.0 cm (range: 1.2-6.3 cm). The times to assess the kidney using the SUS and DP were 68.6 seconds (range: 20-155) and 44.5 seconds (range: 15-180), respectively. Evaluation prior to renal hilar clamping demonstrated the presence of blood flow in all 20 patients (100%) using the SUS and in 17 of 20 (85%) using the DP. Similarly, cessation of blood flow with clamping was documented in 100% of cases with SUS and 85% with DP. Persistent flow was detected by both SUS and DP in two patients requiring further dissection and reclamping. Then, both systems detected the absence of flow before tumor resection. With blood flow interruption confirmation, no patient had significant bleeding at the time of renal parenchymal transection. Intraoperative Doppler ultrasound technologies minimize the risk of significant bleeding during LPN. The DP is a small, simple, effective probe that can be used to assess blood flow interruption to the kidney during laparoscopic renal surgery.

IgG4-Associated Cholangitis Can Mimic Hilar Cholangiocarcinoma.

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Zaydfudim, Victor M; Wang, Andrew Y; de Lange, Eduard E; Zhao, Zimin; Moskaluk, Christopher A; Bauer, Todd W; Adams, Reid B

2015-07-01

IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection.

Primary hepatic pheochromocytoma

International Nuclear Information System (INIS)

Rimmelin, A.; Hartheiser, M.; Gangi, A.; Welsch, M.; Jeung, M.Y.; Jaeck, D.; Tongio, J.; Dietemann, J.L.

1996-01-01

Pheochromocytomas are uncommon tumors that represent a potentially curable cause of hypertension. They are usually located in the adrenal glands, but 10% arise from extra-adrenal sites, located along the paravertebral sympathetic chains. We report a case of primary hepatic pheochromocytoma responsible for a severe hypertension in a 24-year-old man. Echotomography showed a lightly heterogeneous mass located in the segment 8 of the liver. Iodine 131 -metaiodobenzylguanidine scintigraphy showed a large hepatic concentration of the tracer and no other localization. This tumor appeared highly vascularized on enhanced CT scan and on aortic angiography. Magnetic resonance imaging revealed a hepatic tumor with a high signal intensity on T2-weighted images and with a signal isointense to the liver on T1-weighted images. The hepatic venous sampling contained the highest catecholamine level, whereas the adrenal venous samping was normal. After surgical resection of the hepatic tumor, the tension level and catecholamines plasmatic level normalized. No recurrent symptoms appeared during a 3-year follow-up. (orig.)

Low sensitivity of glucagon provocative testing for diagnosis of pheochromocytoma.

Science.gov (United States)

Lenders, Jacques W M; Pacak, Karel; Huynh, Thanh-Truc; Sharabi, Yehonatan; Mannelli, Massimo; Bratslavsky, Gennady; Goldstein, David S; Bornstein, Stefan R; Eisenhofer, Graeme

2010-01-01

Pheochromocytomas can usually be confirmed or excluded using currently available biochemical tests of catecholamine excess. Follow-up tests are, nevertheless, often required to distinguish false-positive from true-positive results. The glucagon stimulation test represents one such test; its diagnostic utility is, however, unclear. The aim of the study was to determine the diagnostic power of the glucagon test to exclude or confirm pheochromocytoma. Glucagon stimulation tests were carried out at three specialist referral centers in 64 patients with pheochromocytoma, 38 patients in whom the tumor was excluded, and in a reference group of 36 healthy volunteers. Plasma concentrations of norepinephrine and epinephrine were measured before and after glucagon administration. Several absolute and relative test criteria were used for calculating diagnostic sensitivity and specificity. Expression of the glucagon receptor was examined in pheochromocytoma tumor tissue from a subset of patients. Larger than 3-fold increases in plasma norepinephrine after glucagon strongly predicted the presence of a pheochromocytoma (100% specificity and positive predictive value). However, irrespective of the various criteria examined, glucagon-provoked increases in plasma catecholamines revealed the presence of the tumor in less than 50% of affected patients. Diagnostic sensitivity was particularly low in patients with pheochromocytomas due to von Hippel-Lindau syndrome. Tumors from these patients showed no significant expression of the glucagon receptor. The glucagon stimulation test offers insufficient diagnostic sensitivity for reliable exclusion or confirmation of pheochromocytoma. Because of this and the risk of hypertensive complications, the test should be abandoned in routine clinical practice.

Results of postoperative radiotherapy for resectable hilar cholangiocarcinoma

NARCIS (Netherlands)

Gerhards, Michael F.; van Gulik, Thomas M.; González González, Dioniso; Rauws, Erik A. J.; Gouma, Dirk J.

2003-01-01

The aim of this study was to assess the value of radiotherapy, and especially intraluminal brachytherapy, after resection of hilar cholangio-carcinoma by analyzing long-term complications and survival. Between 1983 and 1998, 112 patients underwent resection of a hilar cholangio-carcinoma. Of the 91

Cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy.

Science.gov (United States)

Kahraman, Hasan; Tokur, Mahmut; Sayar, Hamide; Inci, Mehmet Fatih

2013-06-10

Cryptogenic organising pneumonia is not considered in the differential diagnosis of bilateral hilar and mediastinal lymphadenopathy. We submitted a patient presenting with bilateral hilar and mediastinal lymphadenopathy. We suspected diagnosis of sarcoidosis, but the patient was diagnosed as cryptogenic organising pneumonia with the histological result. This is the second case report of cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy.

Clinical evaluation of 67Ga-citrate uptake in the hilar areas

International Nuclear Information System (INIS)

Kubota, Hisashi; Komatani, Akio; Watarai, Jiro; Yamaguchi, Koichi; Takamiya, Makoto.

1983-01-01

In the last 4 years 67 Ga-citrate scans had been performed at our department in 914 patients with various diseases. Out of these, 173 showed 67 Ga uptake of varied degree and patterns in the hilar areas. The clinical records of the 173 patients were reviewed to clarify the significance of the hilar uptake. The results were as follows : 1. On a routine 67 Ga-citrte scintigraphy, physiological hilar uptake may be seen in any disease. 2. Bilateral symmetrical hilar 67 Ga accumulation revealed no pathological significance in most cases. Such a 67 Ga uptake might be said as physiological even in the patients with malignancy, if any pathological findings in the both hila could not be noted on the chest film. Degree of the hilar uptake does not contribute much in differentiation between physiological uptake and abnormal one. 3. Primary lung cancer or metastatic lymph node from any origin at the hilar region demonstrated large and definite accumulation. Even if bilateral hilar uptake was noted in these cases, smaller and lower accumulation of the unaffected side might be diagnosed as physiological. (author)

OUTCOMES OF HILAR PEDICLE CONTROL USING SUTURE LIGATION DURING LAPAROSCOPIC SPLENECTOMY.

Science.gov (United States)

Makgoka, M

2017-06-01

Laparoscopic splenectomy is a well described gold standard procedure for various indications. One of the key steps during laparoscopic splenectomy is the hilar pedicle vessels control, which can be challenging in most cases. Most centres around the world recommend the use Ligaclib or endovascular staplers as Methods of choice for hilar pedicle control but the issue is the cost and efficiency of the laparoscopic haemostatic devices. A descriptive retrospective study of patients who had laparoscopic splenectomy from 2013 to present. Hilar splenic vessel control was done with suture ligation. We looked at outcomes of patients offered this technique, complications of this technique, and describing the technique of hilar control in laparoscopic splenectomy. Total of 27 patients had laparoscopic splenectomy with splenic hilar pedicle control with suture ligation. Mean operative time, mean blood volume loss, length of hospital stay, postoperative complications conversion to laparotomy. Laparoscopic hilar pedicle control with suture ligation is safe and effective for the patient in our hospital setting.

Hilar Inflammatory Pseudotumour with Hepatic Artery Atheroma- mimicker of Klatskin Tumour.

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Rastogi, Archana; Bihari, Chhagan; Gupta, Nalini; Deka, Pranjal; Kumar, Arvind; Negi, Sanjay Singh; Arora, Ankur

2015-03-01

Inflammatory pseudotumour of hilar biliary structures is an extremely rare benign lesion that can mimic hilar cholangiocarcinoma. Clinical presentation and imaging findings often pose diagnostic difficulties. Main histopathological findings are the presence of myofibroblastic spindle cells, plasma cells, macrophages, and lymphocytes without cellular atypia or atypical mitotic figures. We describe a case of 62 year old male who presented with surgical obstructive jaundice. Imaging revealed a mass lesion involving the biliary confluence with upstream dilatation of biliary tree. Diagnosis of hilar cholangiocarcinoma with type III hilar block was made. Intraoperately hilar mass lesion was found which was encasing right hepatic artery with no evidence of metastasis. The patient underwent Right hepatectomy with caudate lobectomy with complete common bile duct (CBD) excision with Roux en Y hepaticojejunostomy. Unexpectedly histopathological examination showed no evidence of malignancy and revealed hilar inflammatory pseudotumour with hepatic artery atherosclerosis. Preoperative imaging, operative management, pathologic diagnosis and literature review are being presented in view of rarity of the case.

Multislice helical CT in the diagnosis of hilar cholangiocarcinoma

International Nuclear Information System (INIS)

Yang Li; Zhao Shaohong; Nie Yongkang; Zhao Hong; Fang Jie; Cai Zulong; Yang Zhou; Ying Yifeng

2005-01-01

Objective: To investigate the value ofMSCT in observing the direct findings of hilar cholangiocarcinoma1Methods Multislice helical CT studies were performed on the upper abdomen in 19 consecutive patientswith painless jaundice1 Precontrast and dynamic contrast enhanced (25 s phase and 60 s phase) scanswere conducted, and 3D imageswere reconstructed using enhanced raw data in 15 cases1 The direct CT findings of hilar cholangiocarcinoma were studied by three radiologists respectively in a 32scale strategy1 The morphological features and extension of bile duct involvement by hilar cholangiocarcinoma were analyzed1 All the 19 caseswere pathologically p roved as hilar cholangiocarcinoma by surgery (15 cases) and ERCP ( 4 cases) 1 Results The direct findings and extension of hilar cholangiocarcinoma could be demonstrated in 14 out of 15 3D reconstruction images, 8 out of 19 in 25 s phase, and 7 out of 19 in 60 s phase of contrast enhancement scans, respectively ( P < 0105 ) 1 The tumor involving the bile duct was enhanced most remarkablely on 25 s phase, and the bile duct wall thickening, bile duct narrowing or occlusion were demonstrated as the p rimary findings of hilar cholangiocarcinoma1 The intraductal sp read of tumor could be demonstrated as small nodules on the bile duct wall p roximal or distal to the tumor1 Conclusion. The tumor involving the bile duct can be enhanced most remarkablely on 25 s phase after contrast injection1 Multislice helical CT, especially 3D reconstructed images, can be used to detect the direct findings of hilar cholangiocarcinomas and the extension of tumor involving the bile duct. (authors)

Single minimum incision endoscopic radical nephrectomy for renal tumors with preoperative virtual navigation using 3D-CT volume-rendering

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Shioyama Yasukazu

2010-04-01

Full Text Available Abstract Background Single minimum incision endoscopic surgery (MIES involves the use of a flexible high-definition laparoscope to facilitate open surgery. We reviewed our method of radical nephrectomy for renal tumors, which is single MIES combined with preoperative virtual surgery employing three-dimensional CT images reconstructed by the volume rendering method (3D-CT images in order to safely and appropriately approach the renal hilar vessels. We also assessed the usefulness of 3D-CT images. Methods Radical nephrectomy was done by single MIES via the translumbar approach in 80 consecutive patients. We performed the initial 20 MIES nephrectomies without preoperative 3D-CT images and the subsequent 60 MIES nephrectomies with preoperative 3D-CT images for evaluation of the renal hilar vessels and the relation of each tumor to the surrounding structures. On the basis of the 3D information, preoperative virtual surgery was performed with a computer. Results Single MIES nephrectomy was successful in all patients. In the 60 patients who underwent 3D-CT, the number of renal arteries and veins corresponded exactly with the preoperative 3D-CT data (100% sensitivity and 100% specificity. These 60 nephrectomies were completed with a shorter operating time and smaller blood loss than the initial 20 nephrectomies. Conclusions Single MIES radical nephrectomy combined with 3D-CT and virtual surgery achieved a shorter operating time and less blood loss, possibly due to safer and easier handling of the renal hilar vessels.

Bilateral superomedial hilar displacement: a unique sign of previous mediastinal radiation

International Nuclear Information System (INIS)

Harnsberger, H.R.; Armstrong, J.D. II

1983-01-01

Thirty-two per cent of a group of patients with Hodgkin disease who had been treated with mantle field radiation and had no radiographic evidence of parenchymal radiation fibrosis showed a statistically significant amount of superomedial bilateral hilar elevation. Hilar height and medial displacement ratios were used to quantitate hilar movement

Extra-adrenal Pheochromocytoma in an Adolescent

Directory of Open Access Journals (Sweden)

Abdullah, Ibrahim

2011-05-01

Full Text Available A 17-year-old male with symptoms of headache and diaphoresis presented to the emergency department. He had eight months of noted hypertension attributed to medications. On arrival his blood pressure was 229/117mmHg, and he was ill-appearing. His blood pressure was managed aggressively, and he was diagnosed with extra-adrenal pheochromocytoma by computed tomography. He eventually underwent resection of the mass. Children with severe, symptomatic hypertension should be evaluated for pheochromocytoma. Although rare, it is curable. Failure to diagnose carries a high risk of morbidity and mortality. [West J Emerg Med. 2011;12(2:258-261.

Preoperative biliary drainage in hilar cholangiocarcinoma: When and how?

Science.gov (United States)

Paik, Woo Hyun; Loganathan, Nerenthran; Hwang, Jin-Hyeok

2014-01-01

Hilar cholangiocarcinoma is a tumor of the extrahepatic bile duct involving the left main hepatic duct, the right main hepatic duct, or their confluence. Biliary drainage in hilar cholangiocarcinoma is sometimes clinically challenging because of complexities associated with the level of biliary obstruction. This may result in some adverse events, especially acute cholangitis. Hence the decision on the indication and methods of biliary drainage in patients with hilar cholangiocarcinoma should be carefully evaluated. This review focuses on the optimal method and duration of preoperative biliary drainage (PBD) in resectable hilar cholangiocarcinoma. Under certain special indications such as right lobectomy for Bismuth type IIIA or IV hilar cholangiocarcinoma, or preoperative portal vein embolization with chemoradiation therapy, PBD should be strongly recommended. Generally, selective biliary drainage is enough before surgery, however, in the cases of development of cholangitis after unilateral drainage or slow resolving hyperbilirubinemia, total biliary drainage may be considered. Although the optimal preoperative bilirubin level is still a matter of debate, the shortest possible duration of PBD is recommended. Endoscopic nasobiliary drainage seems to be the most appropriate method of PBD in terms of minimizing the risks of tract seeding and inflammatory reactions. PMID:24634710

Somatic RET mutation in a patient with pigmented adrenal pheochromocytoma

NARCIS (Netherlands)

Maison, Nicole; Korpershoek, Esther; Eisenhofer, Graeme; Robledo, Mercedes; de Krijger, Ronald; Beuschlein, Felix

UNLABELLED: Pheochromocytomas (PCC) and paraganglioma (PGL) are rare neuroendocrine tumors arising from chromaffin cells of the neural crest. Mutations in the RET-proto-oncogene are associated with sporadic pheochromocytoma, familial or sporadic medullary thyroid carcinoma (MTC) and multiple

Adrenocorticotropic hormone-producing pheochromocytoma: analysis of clinical cases

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Evgeniya Ivanovna Marova

2015-04-01

Full Text Available Ectopic secretion of ACTH from non-pituitary tumors, referred to as ectopic ACTH syndrome (EAS, accounts for about 10–20% of Cushing’s syndrome (CS. Ectopic hormone-secreting pheochromocytomas (Pheo are rare. The first publication of association between pheochromocytoma and Cushing’s syndrome by Roux is dated 1955. Pheochromocytoma represents a rare cause of hypercortisolism, accounting for less than 5 % of ectopic Cushing’s syndrome while less than 1 % of pheochromocytomas is accompanied by Cushing’s syndrome. We are reporting 4 cases of ACTH-secreting pheochromocytoma presenting as Cushing’s syndrome. Data from 4 patients were analysed. There were 4 women from 50 to 63 years old. All patients had a clinical presentation of hypercorticoidism. Their levels of adrenocorticotropic hormone in plasma, 24-hour urinary free cortisol and urinary catecholamine were high. Computed tomography scan of the abdomen in all cases revealed a mass in the left adrenal gland. Left sided adrenalectomy was performed under treatment with a-blocker doxazosin and b-blocker atenolol. Histological examination revealed in 3 cases – pheocromocytoma and in 1 case corticomedullary mixed tumor of the adrenal gland. Additional immunostaining (IHCof these tumors showed positive immunostaining for chromogranin and ACTH. The IHC search for somatostatin receptors of subtype 2 and 5 (SSTR2, SSTR5 was performed in 3 cases and showed predominately expression SSTR2. The case index of Ki-67 ranged, from 0,5 to 4%. Biochemical signs of hypercortisolism rapidly began to disappear after surgery. Follow up of the patients during the next 2 years on average was with disease remission.

Two-stage resection of a bilateral pheochromocytoma and pancreatic neuroendocrine tumor in a patient with von Hippel-Lindau disease: A case report

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Yutaka Endo

Full Text Available Introduction: von Hippel-Lindau disease (vHL disease is a hereditary disease in which tumors and cysts develop in many organs, in association with central nervous system hemangioblastomas, pheochromocytomas, and pancreatic tumors. We herein report a case of vHL disease (type 2A associated with bilateral pheochromocytomas, pancreatic neuroendocrine tumors (PNET, and cerebellar hemangioblastomas treated via pancreatectomy after adrenalectomy. Case presentation: A 51-year-old woman presented with a cerebellar tumor, bilateral hypernephroma, and pancreatic tumor detected during a medical checkup. 18F-fluorodeoxyglucose positron emission tomography–computed tomography revealed a bilateral adrenal gland tumor and a tumor in the head of the pancreas, while an abdominal computed tomography examination revealed a 30-mm tumor with strong enhancement in the head of the pancreas. Cranial magnetic resonance imaging showed a hemangioblastoma in the cerebellum. Therefore, a diagnosis of vHL disease (type 2A was made. Her family medical history included renal cell carcinoma in her father and bilateral adrenal pheochromocytoma and spinal hemangioblastoma in her brother. A detailed examination of endocrine function showed that the adrenal mass was capable of producing catecholamine. Treatment of the pheochromocytoma was prioritized, and therefore, laparoscopic left adrenalectomy and subtotal resection of the right adrenal gland were performed. Once the postoperative steroid levels were replenished, subtotal stomach-preserving pancreatoduodenectomy was performed for the PNET. After a good postoperative course, the patient was discharged in remission on the 11th day following surgery. Histopathological examination findings indicated NET G2 (MIB-1 index 10–15% pT3N0M0 Stage II A and microcystic serous cystadenoma throughout the resected specimen. The patient is scheduled to undergo treatment for the cerebellar hemangioblastoma. Conclusion: A two-staged resection

Introduction of a Case of Bilateral Pheochromocytoma Tumor

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M Zare

2009-01-01

Full Text Available Summary: Pheochromocytoma is an adrenal tumor characterized by symptoms of hypertension, headache, increased sweating, and tachycardia .In one third of patients, the tumor is discovered incidentally during radiological evaluation of the abdomen. In this article a case of bilateral pheochromocytoma is reported. The patient, a twelve years old boy, referred with complaints of headache, flushing, palpitation and blurring of vision. He had a blood pressure of 200/120 mmHg and after investigation, coarctation of aorta was excluded. On abdominal ultrasound, bilateral adrenal mass was diagnosed, followed by serial measurements of urinary vanillyl mandellic acid (VMA which was found to be elevated. Meta- Iodio benzyl glunidin (MIBG scan showed increased uptake in both adrenals. Patient underwent laparotomy and bilateral adrenalectomy with the diagnosis of pheochromocytoma. Pathologic report confirmed the diagnosis

The Epidemiology of Pheochromocytoma

DEFF Research Database (Denmark)

Ladefoged Ebbehøj, Andreas

2017-01-01

Pheochromocytoma and catecholamine secreting paraganglioma (PPGL) are exceedingly rare endocrine tumours, but remain a frequent diagnostic dilemma due to their potential life-threatening nature. Reliable data on the epidemiology of PPGL is lacking and no time trends in incidence rates (IR) have...

Hilar GABAergic interneuron activity controls spatial learning and memory retrieval.

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Yaisa Andrews-Zwilling

Full Text Available Although extensive research has demonstrated the importance of excitatory granule neurons in the dentate gyrus of the hippocampus in normal learning and memory and in the pathogenesis of amnesia in Alzheimer's disease (AD, the role of hilar GABAergic inhibitory interneurons, which control the granule neuron activity, remains unclear.We explored the function of hilar GABAergic interneurons in spatial learning and memory by inhibiting their activity through Cre-dependent viral expression of enhanced halorhodopsin (eNpHR3.0--a light-driven chloride pump. Hilar GABAergic interneuron-specific expression of eNpHR3.0 was achieved by bilaterally injecting adeno-associated virus containing a double-floxed inverted open-reading frame encoding eNpHR3.0 into the hilus of the dentate gyrus of mice expressing Cre recombinase under the control of an enhancer specific for GABAergic interneurons. In vitro and in vivo illumination with a yellow laser elicited inhibition of hilar GABAergic interneurons and consequent activation of dentate granule neurons, without affecting pyramidal neurons in the CA3 and CA1 regions of the hippocampus. We found that optogenetic inhibition of hilar GABAergic interneuron activity impaired spatial learning and memory retrieval, without affecting memory retention, as determined in the Morris water maze test. Importantly, optogenetic inhibition of hilar GABAergic interneuron activity did not alter short-term working memory, motor coordination, or exploratory activity.Our findings establish a critical role for hilar GABAergic interneuron activity in controlling spatial learning and memory retrieval and provide evidence for the potential contribution of GABAergic interneuron impairment to the pathogenesis of amnesia in AD.

Primary renal undifferentiated sarcoma as an infiltrative mass in a 12 year old boy

International Nuclear Information System (INIS)

Kim, Yong Hee; Kim, Myung Joon; Lee, Mi Jung; Kim, Se Hwa

2015-01-01

Undifferentiated sarcomas are rare tumors not classified into any sarcoma subtype. Due to their rarity, imaging findings of undifferentiated sarcomas are poorly characterized. The purpose of this report was to present imaging findings of a pathologically confirmed undifferentiated sarcoma originated from the left kidney of a 12-year-old boy. The mass was infiltrative involving the renal pelvis. It mimicked massive hilar lymphadenopathy with a preserved renal contour visible by both ultrasonography and CT. Renal vein thrombosis was also observed. Although undifferentiated sarcomas are rare, they should be considered in differential diagnosis of infiltrative renal masses with renal pelvis invasion in children

Primary renal undifferentiated sarcoma as an infiltrative mass in a 12 year old boy

Energy Technology Data Exchange (ETDEWEB)

Kim, Yong Hee; Kim, Myung Joon; Lee, Mi Jung [Dept. of Radiology and Research Institute of Radiological Science, Severance Children' s Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, Se Hwa [Dept. of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of)

2015-09-15

Undifferentiated sarcomas are rare tumors not classified into any sarcoma subtype. Due to their rarity, imaging findings of undifferentiated sarcomas are poorly characterized. The purpose of this report was to present imaging findings of a pathologically confirmed undifferentiated sarcoma originated from the left kidney of a 12-year-old boy. The mass was infiltrative involving the renal pelvis. It mimicked massive hilar lymphadenopathy with a preserved renal contour visible by both ultrasonography and CT. Renal vein thrombosis was also observed. Although undifferentiated sarcomas are rare, they should be considered in differential diagnosis of infiltrative renal masses with renal pelvis invasion in children.

A study on pulmonary hilar and mediastinal lymphoscintigraphy

International Nuclear Information System (INIS)

Matsuo, Michimasa; Ushio, Keiji; Nishiyama, Shoji; Kono, Michio; Takada, Yoshiki

1979-01-01

A routine examination of pulmonary hilar and mediastinal lymphoscintigraphy is tried in this study in order to visualize pulmonary hilar and mediastinal lymphnodes routinely. A method is as follows; 30 - 50 μCi of 198 Au-colloid (0.3 - 0.5 ml of total volume) is injected through the needle of flexible bronchofiberscope into the mucosal membrane or submucosal membrane of the bilateral B 8 or B 9 bronchi. This method was applied to 11 cases of suspected pulmonary carcinoma and gave good results. In case of bronchitis, lung abscess and chronic pneumonia, carinal lymphnodes (which are expected to be as the inferior tracheo-bronchial lymphnodes) and one or more right mediastinal lymphnodes (which are expected to be as the right superior tracheo-bronchial or right paratracheal lymphnodes) were clearly visualized from 24 to 27 hours after the injection. In these cases the pulmonary hilar and mediastinal lymphnodes were expected to be intact on x-rays and CT findings. Among cases of pulmonary carcinoma or lymphangitis carcinomatosa, on the contrary, right mediastinal lymphnodes were not visualized in case with the right hilar lymphnodes involvement, and furthermore neither carinal nor right mediastinal lymphnodes were visualized in case with carinal lymphnodes involvements on X-rays, CT, operation or autopsy findings. From these results, the pulmonary hilar and mediastinal lymphoscintigraphy may give the useful information for the treatment planning, and follow up study of the cases of pulmonary and mediastinal malignant tumors. (author)

Improved outcome of resection of hilar cholangiocarcinoma (Klatskin tumor)

NARCIS (Netherlands)

Dinant, Sander; Gerhards, Michael F.; Rauws, E. A. J.; Busch, Olivier R. C.; Gouma, Dirk J.; van Gulik, Thomas M.

2006-01-01

BACKGROUND: Treatment of hilar cholangiocarcinoma (Klatskin tumors) has changed in many aspects. A more extensive surgical approach, as proposed by Japanese surgeons, has been applied in our center over the last 5 years; it combines hilar resection with partial hepatectomy for most tumors. The aim

Serum albumin predicts survival in patients with hilar cholangiocarcinoma.

Science.gov (United States)

Waghray, Abhijeet; Sobotka, Anastasia; Marrero, Carlos Romero; Estfan, Bassam; Aucejo, Federico; Narayanan Menon, K V

2017-02-01

Hilar cholangiocarcinoma is a devastating malignancy with incidence varying by geography and other risk factors. Rapid progression of disease and delays in diagnosis restrict the number of patients eligible for curative therapy. The objective of this study was to determine prognostic factors of overall survival in all patients presenting with hilar cholangiocarcinoma. All adult patients with histologically confirmed hilar cholangiocarcinoma from 2003 to 2013 were evaluated for predictors of survival using demographic factors, laboratory data, symptoms and radiological characteristics at presentation. A total of 116 patients were identified to have pathological diagnosis of hilar cholangiocarcinoma and were included in the analysis. Patients with a serum albumin level >3.0 g/dL (P 3.0 g/dL was identified as an independent predictor of overall survival (hazard ratio 0.31; 95% confidence interval 0.14-0.70) with a survival benefit of 44 weeks. This study was the largest analysis to date of prognostic factors in patients with hilar cholangiocarcinoma. A serum albumin level >3.0 g/dL conferred an independent survival advantage with a significantly greater length of survival. © The Author(s) 2016. Published by Oxford University Press and Sixth Affiliated Hospital of Sun Yat-Sen University.

131I-MIBG Scintigraphy in the Evaluation of Suspected Pheochromocytoma

International Nuclear Information System (INIS)

Oh, Sei Jung; Yoo, Hyung Sik; Park, Chang Yun; Lee, So Jin

1992-01-01

Pheochromocytomas are catecholamine producing tumors of neuroectodermal origin. Diagnosis of pheochromocytoma is significant due to potentially curable hypertension. But they have a significant associated morbidity due to uncontrolled hypertension and mortality since 10% are malignant. From Aug. 1989 to Jul. 1992, 12 patients of our institution had 131 I-MIBG scan during work up of suspected primary or recurrent pheochromocytoma. In our studies conclude that 131 I-MIBG scan is recommended as the initial localizing study of choice (especially for the detection of extraadrenal disease and postoperative recurrence) as a guide for CT and/or MR and specific functional confirmation of their findings.

Radiation fibrosis : differentiation from true hilar mass on plain chest film

International Nuclear Information System (INIS)

Baik, Jun Hyun; Ahn, Kook Jin; Park, Seog Hee; Shinn, Kyung Sub

1996-01-01

Radiation-induced fibrotic mass might masquerade as a true hilar tumor mass on a plain chest radiograph. We attempted to differentiate radiation fibrosis from a true hilar tumor using only a plain radiograph. Plain chest radiographs were obtained from seven patients who had developed radiation fibrosis simulating hilar mass after radiation therapy for lung cancer, and from 19 patients with lung cancer, a comparison group, who had not received radiation therapy. They were reviewed for the obliteration of the overlapped mediastinal and hilar anatomical silhouettes by the mass : pulmonary artery, heart or aorta border, and paraspinal line. All seven patients with radiation-induced fibrotic mass(bilateral lesion in two patients) showed obliteration of all three overlapped anatomical silhouettes of the mediastinum and hilum on chest radiographs. In the comparison group of 19 patients with a true hilar mass, there was, however, no case which demonstrated obliteration of all landmarks, Obliteration of all anatomical landmarks at the hilum and mediastinum may be a helpful clue in the differential diagnosis of radiation-induced fibrotic mass from true hilar mass on a plain radiograph, particularly when clinical information on previous radiation therapy is unavailable

Hilar GABAergic Interneuron Activity Controls Spatial Learning and Memory Retrieval

Science.gov (United States)

Andrews-Zwilling, Yaisa; Gillespie, Anna K.; Kravitz, Alexxai V.; Nelson, Alexandra B.; Devidze, Nino; Lo, Iris; Yoon, Seo Yeon; Bien-Ly, Nga; Ring, Karen; Zwilling, Daniel; Potter, Gregory B.; Rubenstein, John L. R.; Kreitzer, Anatol C.; Huang, Yadong

2012-01-01

Background Although extensive research has demonstrated the importance of excitatory granule neurons in the dentate gyrus of the hippocampus in normal learning and memory and in the pathogenesis of amnesia in Alzheimer's disease (AD), the role of hilar GABAergic inhibitory interneurons, which control the granule neuron activity, remains unclear. Methodology and Principal Findings We explored the function of hilar GABAergic interneurons in spatial learning and memory by inhibiting their activity through Cre-dependent viral expression of enhanced halorhodopsin (eNpHR3.0)—a light-driven chloride pump. Hilar GABAergic interneuron-specific expression of eNpHR3.0 was achieved by bilaterally injecting adeno-associated virus containing a double-floxed inverted open-reading frame encoding eNpHR3.0 into the hilus of the dentate gyrus of mice expressing Cre recombinase under the control of an enhancer specific for GABAergic interneurons. In vitro and in vivo illumination with a yellow laser elicited inhibition of hilar GABAergic interneurons and consequent activation of dentate granule neurons, without affecting pyramidal neurons in the CA3 and CA1 regions of the hippocampus. We found that optogenetic inhibition of hilar GABAergic interneuron activity impaired spatial learning and memory retrieval, without affecting memory retention, as determined in the Morris water maze test. Importantly, optogenetic inhibition of hilar GABAergic interneuron activity did not alter short-term working memory, motor coordination, or exploratory activity. Conclusions and Significance Our findings establish a critical role for hilar GABAergic interneuron activity in controlling spatial learning and memory retrieval and provide evidence for the potential contribution of GABAergic interneuron impairment to the pathogenesis of amnesia in AD. PMID:22792368

Stages of Pheochromocytoma and Paraganglioma

Science.gov (United States)

... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

Silencing of CXCR4 inhibits tumor cell proliferation and neural invasion in human hilar cholangiocarcinoma.

Science.gov (United States)

Tan, Xin-Yu; Chang, Shi; Liu, Wei; Tang, Hui-Huan

2014-03-01

To evaluate the expression of CXC motif chemokine receptor 4 (CXCR4) in the tissues of patients with hilar cholangiocarcinoma (hilar-CCA) and to investigate the cell proliferation and frequency of neural invasion (NI) influenced by RNAi-mediated CXCR4 silencing. An immunohistochemical technique was used to detect the expression of CXCR4 in 41 clinical tissues, including hilar-CCA, cholangitis, and normal bile duct tissues. The effects of small interference RNA (siRNA)-mediated CXCR4 silencing were detected in the hilar-CCA cell line QBC939. Cell proliferation was determined by MTT. Expression of CXCR4 was monitored by quantitative real time polymerase chain reaction and Western blot analysis. The NI ability of hilar-CCA cells was evaluated using a perineural cell and hilar-CCA cell coculture migration assay. The expression of CXCR4 was significantly induced in clinical hilar-CCA tissue. There was a positive correlation between the expression of CXCR4 and lymph node metastasis/NI in hilar-CCA patients (philar-CCA. CXCR4 is involved in the invasion and proliferation of human hilar-CCA cell line QBC939, indicating that CXCR4 could be a promising therapeutic target for hilar-CCA.

Radiation therapy in the treatment of hilar cholangiocarcinoma

International Nuclear Information System (INIS)

Jing Jin; Zhai Renyou

2007-01-01

The incidence of hilar cholangiocarcinoma is very rare worldwide. Radical resection is the only prognostic factor for long survival in patients with hilar cholangiocarcinoma. Postoperative radiation therapy can improve local control and survival rates for patients with palliative resection, but it remains controversial in patients with radical resection. Biliary drainage can effectively release bile duct obstruction for the majority of patients with locally advanced disease, and may even prolong survival when combined with radiation therapy. Radiation therapy includes extrernal beam therapy alone, external beam therapy with intraluminal brachytheapy and new radiation technique, such as three dimentional conformal therapy and intensity modulated radiation therapy. The propective randomized clinical study is needed for further investigation in the role of combined modality therapy especially for hilar cholangiocarcinoma. (authors)

Oblique hilar tomography, computed tomography, and mediastinoscopy for prethoracotomy staging of bronchogenic carcinoma

International Nuclear Information System (INIS)

Khan, A.; Gersten, K.C.; Garvey, J.; Khan, F.A.; Steinberg, H.

1985-01-01

Preoperative oblique hilar tomography was used to evaluate hilar lymph nodes in 150 patients with clinically resectable bronchogenic carcinoma. CT was also used in the evaluation of mediastinal lymph nodes in 50 of these patients. Subsequently, all patients underwent mediastinoscopy and/or thoracotomy. Hilar and mediastinal nodes were evaluated for the presence of metastasis, and these findings were then correlated with the radiographic findings of oblique hilar tomography and CT. CT was found to be a reliable method for prethoracotomy staging of bronchogenic carcinoma and for selecting patients for mediastinoscopy. Thus patients with negative mediastinal CT need not undergo mediastinoscopy prior to thoracotomy, while mediastinoscopy and biopsy should be done in patients with enlarged mediastinal nodes on CT. Oblique hilar tomography is an accurate method for evaluation of hilar adenopathy and for predicting mediastinal involvement by extrapolation

Conventional tomographic hilar anatomy emphasizing the pulmonary veins

International Nuclear Information System (INIS)

Genereux, G.P.

1983-01-01

The pulmonary hili are a frequent site of interpretive uncertainty because of thir complex anatomy. The right hilar shadow relates primarily to the ascending and descending pulmonary arteries and the right superior pulmonary vein, whereas the left hilar density accrues from the left pulmonary artery, left descending pulmonary artery, and left superior pulmonary vein. The right and left superoir pulmonary veins are intimately associated with and inseparable from the arteries. Knowledge of the course of these veins facilitates their identification on conventional tomograms. The right and left inferior pulmonary veins lie behind the lower hili and contribute only a small increment to the normal hilar density; the horizontal course of these vessels readily distinguishes them from the vertically oriented lower lobe arteries. Prominence of both the right and left superior and inferior veins may cause hilar pseudotumors. Conventional tomograms are most helpful in understanding plain radiographs. A multiview approach in anteroposterior, 55 0 posterior oblique, and lateral projections is recommended for a complete assessment. Computed tomography in transaxial sections adds further understanding of spatial relations in the hili, including the contributions of the pulmonary veins

Hilar anatomy of the hepatic artery and surgical procedure for hilar cholangiocarcinoma

International Nuclear Information System (INIS)

Uesaka, Katsuhiko; Maeda, Atsuyuki; Kanamoto, Hideyuki; Matsunaga, Kazuya; Yuasa, Ichiro; Okamura, Yukiyasu; Yamaguchi, Shigeki; Bando, Etsuro; Furukawa, Hiroyoshi

2006-01-01

This paper describes the examination of findings by multi-detector-row CT (MDCT) and by surgery to obtain the anatomy of hilar arteries and portal vein, which is necessary for the procedure in the title. Subjects are those findings of 38 patients with hilar cholangiocarcinoma, who underwent its excision during the period of 1 year from 2002 Nov. Before operation, MDCT with 16-row detector was done 20-120 sec after infusion of a non-ionized contrast medium to compose the 3D images. The left hepatic arterial system was found to be classifiable in 3 types of common, anti-clockwise and clockwise one with the respective frequency of 63, 24 and 11%, and the right system, infra-portal (76%) and supra-portal (24%) types. It was concluded that to the arterial clockwise and supra-portal types, particular attention should be paid for the cancer invasion there and for avoidance of the artery damage during the operation. (T.I.)

Concurrent primary hyperparathyroidism and pheochromocytoma in a Chinese lady with neurofibromatosis type 1

Directory of Open Access Journals (Sweden)

Cheuk-Lik Wong

2018-04-01

Full Text Available We report a case of elderly Chinese lady with neurofibromatosis type-1 presenting with longstanding palpitation, paroxysmal hypertension and osteoporosis. Biochemical testing showed mild hypercalcaemia with non-suppressed parathyroid hormone level suggestive of primary hyperparathyroidism, and mildly elevated urinary fractionated normetanephrine and plasma-free normetanephrine pointing to a catecholamine-secreting pheochromocytoma/paraganglioma. Further scintigraphic investigation revealed evidence of a solitary parathyroid adenoma causing primary hyperparathyroidism and a left pheochromocytoma. Resection of the parathyroid adenoma and pheochromocytoma resulted in normalization of biochemical abnormalities and hypertension. The rare concurrence of primary hyperparathyroidism and pheochromocytoma in neurofibromatosis type-1 is discussed.

Metallic stent and stereotactic conformal radiotherapy for hilar cholangiocarcinoma

International Nuclear Information System (INIS)

Li Yu; Wang Ning; Tian Qihe; Guo Zhanwen; Zhang Haibo; Song Liyan

2005-01-01

Objective: To evaluate the effect of metallic stent combined with stereotactic conformal radiotherapy (SCRT) for hilar cholangiocarcinoma. Methods: Fifty-four patients with hilar cholangiocarcinoma were analyzed, including 31 treated with stent plus stereotactic conformal radiotherapy (combined group) and 23 with metallic stent alone (control group). Results: The mean survival time of combined group was 11.1 ± 4.6 months, compared with 5.1 ± 2.8 months of the control group, giving a significant difference between the two groups (P<0.01). Conclusion: The combination of metallic stent and stereotactic conformal radiotherapy is more effective than metallic stent alone for unresectable hilar cholangiocarcinoma. (authors)

Pheochromocytoma and pregnancy: A case report and review.

Science.gov (United States)

Santos, Davi Rettori Pardo Dos; Barbisan, Cinthia Callegari; Marcellini, Claudio; dos Santos, Rubia Marina Vieira Rettori

2015-01-01

Pheochromocytoma is a catecholamine-producing adrenal tumor, being a rare cause of hypertension in pregnancy. It's prevalence in hypertensive patients is 0.2%, and 0.002% of pregnancies. We follow hypertensive pregnant 24 year old on her third pregnancy, admitted to 33 weeks with hypertensive emergency cesarean section indicated by fetal distress evolving with acute pulmonary edema in the post-partum period. Indicated laparoscopy after 13 days for acute abdominal pain, with no significant finding. In the postoperative, due a severe and resistant hypertension, suspected of pheochromocytoma and confirmed by biochemical tests and imaging. Performed unilateral adrenelectomia with cure of hypertension. The pathology and immunohistochemistry confirmed the diagnosis. We conclude that atypical cases of hypertension in pregnancy should be investigated early and differentiated pre-eclampsia. Despite the low prevalence, pheochromocytoma in pregnancy increases fetal maternal morbidity and mortality and the early recognition and treatment drastically change their outcome.

Preoperative radiological approach for hilar lung cancer

International Nuclear Information System (INIS)

Ohno, Yoshiharu; Higashino, Takanori; Watanabe, Hirokazu; Yoshimura, Masahiro; Sugimura, Kazuro

2003-01-01

Recent advances in CT, MR, and nuclear medicine have made it possible to evaluate morphological and functional information in hilar lung cancer patients more accurately and quantitatively. In this review, we describe recent advances in the radiological approach to hilar lung cancer, focusing on mediastinal invasion, lymph node metastasis, and pulmonary functional imaging. We believe that further basic studies as well as clinical applications of newer MR techniques will play an important role in the management of patients with lung cancer. (author)

Expression and clinical significance of PIWIL2 in hilar cholangiocarcinoma tissues and cell lines.

Science.gov (United States)

Chen, Y J; Xiong, X F; Wen, S Q; Tian, L; Cheng, W L; Qi, Y Q

2015-06-26

The objective of this study was to explore the relationship between PIWI-like protein 2 (PIWIL2) and clinicopathological charac-teristics and prognosis after radical resection. To accomplish this, we analyzed PIWIL2 expression in hilar cholangiocarcinoma tissues and cell lines. PIWIL2 expression was detected by immunohistochemistry in 41 hilar cholangiocarcinoma samples and 10 control tissues. Western blotting and immunocytofluorescence were used to investigate PIWIL2 expression in the cholangiocarcinoma cell line QBC939 and the bile duct epithelial cell line HIBEpic. Univariate and multivariate surviv-al analyses were performed using the Kaplan-Meier method for hilar cholangiocarcinoma patients who underwent radical resection. PIWIL2 expression was significantly higher in the hilar cholangiocarcinoma tissues and QBC939 cells than in control tissues and HIBEpic cells, respectively (P hilar cholangiocarcinoma (P hilar cholangiocarcinoma.

Multicenter study of endoscopic preoperative biliary drainage for malignant hilar biliary obstruction: E-POD hilar study.

Science.gov (United States)

Nakai, Yousuke; Yamamoto, Ryuichi; Matsuyama, Masato; Sakai, Yuji; Takayama, Yukiko; Ushio, Jun; Ito, Yukiko; Kitamura, Katsuya; Ryozawa, Shomei; Imamura, Tsunao; Tsuchida, Kouhei; Hayama, Jo; Itoi, Takao; Kawaguchi, Yoshiaki; Yoshida, Yu; Sugimori, Kazuya; Shimura, Kenji; Mizuide, Masafumi; Iwai, Tomohisa; Nishikawa, Ko; Yagioka, Hiroshi; Nagahama, Masatsugu; Toda, Nobuo; Saito, Tomotaka; Yasuda, Ichiro; Hirano, Kenji; Togawa, Osamu; Nakamura, Kenji; Maetani, Iruru; Sasahira, Naoki; Isayama, Hiroyuki

2018-05-01

Endoscopic nasobiliary drainage (ENBD) is often recommended in preoperative biliary drainage (PBD) for hilar malignant biliary obstruction (MBO), but endoscopic biliary stent (EBS) is also used in the clinical practice. We conducted this large-scale multicenter study to compare ENBD and EBS in this setting. A total of 374 cases undergoing PBD including 281 ENBD and 76 EBS for hilar MBO in 29 centers were retrospectively studied. Extrahepatic cholangiocarcinoma (ECC) accounted for 69.8% and Bismuth-Corlette classification was III or more in 58.8% of the study population. Endoscopic PBD was technically successful in 94.6%, and adverse event rate was 21.9%. The rate of post-endoscopic retrograde cholangiopancreatography pancreatitis was 16.0%, and non-endoscopic sphincterotomy was the only risk factor (odds ratio [OR] 2.51). Preoperative re-intervention was performed in 61.5%: planned re-interventions in 48.4% and unplanned re-interventions in 31.0%. Percutaneous transhepatic biliary drainage was placed in 6.4% at the time of surgery. The risk factors for unplanned procedures were ECC (OR 2.64) and total bilirubin ≥ 10 mg/dL (OR 2.18). In surgically resected cases, prognostic factors were ECC (hazard ratio [HR] 0.57), predraiange magnetic resonance cholangiopancreatography (HR 1.62) and unplanned re-interventions (HR 1.81). EBS was not associated with increased adverse events, unplanned re-interventions, or a poor prognosis. Our retrospective analysis did not demonstrate the advantage of ENBD over EBS as the initial PBD for resectable hilar MBO. Although the technical success rate of endoscopic PBD was high, its re-intervention rate was not negligible, and unplanned re-intervention was associated with a poor prognosis in resected hilar MBO. © 2017 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

Hilar activity on the F-18 FDG whole-body PET studies

International Nuclear Information System (INIS)

Fujii, Hirofumi; Kitamura, Masayuki; Kubo, Atsushi; Yasuda, Seiei; Ide, Michiru; Takahashi, Wakoh; Shohtsu, Akira

1999-01-01

We evaluated the clinical characteristics of hilar activity that would be false positive findings for cancer screening on whole-body FDG PET. The cases with increased hilar activity were selected from 1,126 cases who received whole-body FDG PET between September 1996 and August 1997, and their age, sex, complication of inflammatory process in lungs, numbers of visualized mediastinal lymph nodes, frequency of smoking, blood sugar level and concentration of HbA1c were studied. FDG accumulation in the hilar regions was found in 63 cases (5.6%). The cases with increased hilar activity were older, a higher incidence of complication of pulmonary inflammation, a larger number of visualized mediastinal lymph nodes and were more frequent smokers than the control cases with normal FDG distribution. Their male-to-female ratio, blood sugar level and concentration of HbA1c were not significantly different from those of the control cases. These results suggested that an inflammatory process around the hilar region might induce this confusing FDG accumulation. (author)

Temporary targeted renal blood flow interruption using a reverse thermosensitive polymer to facilitate bloodless partial nephrectomy: a swine survival study.

Science.gov (United States)

Harty, Niall J; Laskey, Daniel H; Moinzadeh, Alireza; Flacke, Sebastian; Benn, James A; Villani, Rosanna; Kalra, Aarti; Libertino, John A; Madras, Peter N

2012-09-01

What's known on the subject? and What does the study add? Lumagel™ is a reverse thermosensitive polymer (RTP) that has previously been described in the literature as providing temporary vascular occlusion to allow for bloodless partial nephrectomy (PN) while maintaining blood flow to the untargeted portion of the kidney. At body temperature, Lumagel™ has the consistency of a viscous gel but upon cooling rapidly converts to a liquid state and does not reconstitute thereafter. This property has allowed for it to be used in situations requiring temporary vascular occlusion. Previous experience with similar RTPs in coronary arteries proved successful, with no detectable adverse events. We have previously described our technique for temporary vascular occlusion of the main renal artery, as well as segmental and sub-segmental renal branches, to allow for bloodless PN in either an open or minimally invasive approach. These experiments were performed in the acute setting. This study is a two-armed survival trial to assess whether this RTP is as safe as hilar clamping for bloodless PN. Surviving animals showed normal growth after using the RTP, absence of toxicity, no organ dysfunction, and no pathological changes attributable to the RTP. We conclude that Lumagel™ is as safe as conventional PN with hilar clamping, while adding the advantage of uninterrupted perfusion during renal resection. To examine whether randomly selected regions of the kidney could undergo temporary flow interruption with a reverse thermosensitive polymer (RTP), Lumagel™ (Pluromed, Inc., Woburn, MA, USA), followed by partial nephrectomy (PN), without adding risks beyond those encountered in the same procedure with the use of hilar clamping. A two-armed (RTP vs hilar clamp), 6-week swine survival study was performed. Four swine underwent PN using hilar clamps, while six underwent PN with flow interruption using the RTP. The RTP, administered angiographically, was used for intraluminal occlusion

Biliary stenting and anti-cancer therapy for unresectable hilar bile duct carcinomas

International Nuclear Information System (INIS)

Saito, Hiroya; Hokotate, Hirofumi; Takeuchi, Shyuhei; Takamura, Akio

2007-01-01

At present, although imaging diagnosis has been developed, most hilar bile duct cancer is still diagnosed at an advanced stage and its prognosis is generally poor. In hilar bile duct cancer, radiotherapy and other several therapies, for example-chemotherapy, arterial-infusion chemotherapy, photodynamic therapy, etc-are being performed for non-operative cases. But standard therapies for this cancer has not been established yet. On the other hand, metallic stents (MS) have been widely used to relieve biliary obstructions as an alternative to plastic prostheses and conventional drainage. The use of MS offers good palliation in hilar bile duct cancer, but patients selection is a key to obtain good results. In this article we reviewed previous studies and clinical trials regarding the anti-cancer therapy and biliary stenting for unresectable hilar bile duct cancer. And optimal therapeutic strategy for hilar bile duct cancer is proposed, primarily based on present views. (author)

Severe posterior reversible encephalopathy in pheochromocytoma: Importance of susceptibility-weighted MRI

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Serter, Asil; Alkan, Alpay; Aralasmak, Ayse; Kocakoc, Ercan [Dept. of Radiology, Bezmialem Vakif University School of Medicine, Istanbul (Turkmenistan)

2013-10-15

Pheochromocytoma is a rare cause of hypertension in children. Hypertension is one of the common reasons of posterior reversible encephalopathy. Intracerebral hemorrhage is a serious and unexpected complication of hypertensive encephalopathy due to pheochromocytoma, and very rarely seen in the childhood. Intracerebral hemorrhages should be searched if there are hypertensive reversible signal changes on the brain. Susceptibility weighted imaging (SWI) is a more sensitive method than conventional MRI when demonstrating cerebral microhemorrhagic foci. This is the first report of SWI findings on intracerebral hemorrhages in basal ganglia, brain stem and periventricular white matter due to hypertensive encephalopathy in a child with pheochromocytoma.

Spontaneous rupture of pheochromocytoma and its clinical features: a case report.

Science.gov (United States)

Maruyama, Mayumi; Sato, Haruhiro; Yagame, Mitsunori; Shoji, Sunao; Terachi, Toshiro; Osamura, Robert Yoshiyuki

2008-09-20

Rupture of adrenal pheochromocytoma is extremely rare and can be lethal because of dramatic changes in the circulation. We describe a 58-year-old Japanese man who suffered rupture of a pheochromocytoma. The patient was referred to our hospital because of severe hypertension (256/127 mmHg) and a left adrenal tumor. T2-weighted magnetic resonance imaging showed high signal intensity in the 50-mm left adrenal tumor. Endocrinological examinations showed elevated plasma and urinary catecholamine levels. These findings suggested that the left adrenal tumor was a pheochromocytoma. Phentolamine mesilate was administered intravenously. This resulted in a decrease of the systolic blood pressure to 100 mmHg. On the third hospital day, the patient complained of left back pain, and abdominal computed tomography showed rupture of the pheochromocytoma. Pulmonary congestion and effusion, and paralytic small-intestinal ileus occurred. Blood pressure was controlled, small-intestinal decompression was done with a Miller-Abbot tube, and body water was controlled by fluid replacement. After the general condition of the patient had became stable, laparoscopic adrenalectomy was performed. Phentolamine mesilate is a useful α-adrenergic blocker. However, care is needed with its administration, because rupture of pheochromocytoma may be related to a decrease in blood pressure induced by phentolamine mesilate.

Down-regulation of Gab1 inhibits cell proliferation and migration in hilar cholangiocarcinoma.

Directory of Open Access Journals (Sweden)

Haiquan Sang

Full Text Available Hilar cholangiocarcinoma is a highly aggressive malignancy originating from the hilar biliary duct epithelium. Due to few effective comprehensive treatments, the prognosis of hilar cholangiocarcinoma is poor. In this study, immunohistochemistry was first used to detect and analyze the expression of Gab1, VEGFR-2, and MMP-9 in hilar cholangiocarcinoma solid tumors and the relationships to the clinical pathological features. Furthermore, Gab1 and VEGFR-2 siRNA were used to interfere the hilar cholangiocarcinoma cell line ICBD-1 and then detect the PI3K/Akt signaling pathway, MMP-9 levels and malignant biological behaviors of tumor cells. The data showed that 1. Gab1, VEGFR-2, and MMP-9 were highly expressed and positively correlated with each other in hilar cholangiocarcinoma tissues, which were related to lymph node metastasis and differentiation. 2. After Gab1 or VEGFR-2 siRNA interference, PI3K/Akt pathway activity and MMP-9 levels were decreased in ICBD-1 cells. At the same time, cell proliferation decreased, cell cycle arrested in G1 phase, apoptosis increased and invasion decreased. These results suggest that the expression of Gab1, VEGFR-2, and MMP-9 are significantly related to the malignant biological behavior of hilar cholangiocarcinoma. Gab1 regulates growth, apoptosis and invasion through the VEGFR-2/Gab1/PI3K/Akt signaling pathway in hilar cholangiocarcinoma cells and influences the invasion of tumor cells via MMP-9.

Down-regulation of Gab1 inhibits cell proliferation and migration in hilar cholangiocarcinoma.

Science.gov (United States)

Sang, Haiquan; Li, Tingting; Li, Hangyu; Liu, Jingang

2013-01-01

Hilar cholangiocarcinoma is a highly aggressive malignancy originating from the hilar biliary duct epithelium. Due to few effective comprehensive treatments, the prognosis of hilar cholangiocarcinoma is poor. In this study, immunohistochemistry was first used to detect and analyze the expression of Gab1, VEGFR-2, and MMP-9 in hilar cholangiocarcinoma solid tumors and the relationships to the clinical pathological features. Furthermore, Gab1 and VEGFR-2 siRNA were used to interfere the hilar cholangiocarcinoma cell line ICBD-1 and then detect the PI3K/Akt signaling pathway, MMP-9 levels and malignant biological behaviors of tumor cells. The data showed that 1. Gab1, VEGFR-2, and MMP-9 were highly expressed and positively correlated with each other in hilar cholangiocarcinoma tissues, which were related to lymph node metastasis and differentiation. 2. After Gab1 or VEGFR-2 siRNA interference, PI3K/Akt pathway activity and MMP-9 levels were decreased in ICBD-1 cells. At the same time, cell proliferation decreased, cell cycle arrested in G1 phase, apoptosis increased and invasion decreased. These results suggest that the expression of Gab1, VEGFR-2, and MMP-9 are significantly related to the malignant biological behavior of hilar cholangiocarcinoma. Gab1 regulates growth, apoptosis and invasion through the VEGFR-2/Gab1/PI3K/Akt signaling pathway in hilar cholangiocarcinoma cells and influences the invasion of tumor cells via MMP-9.

Multidetector Computed Tomography in the Preoperative Workup of Hilar Cholangiocarcinoma

International Nuclear Information System (INIS)

Kim, Hyoung Jung; Lee, Dong Ho; Lim, Joo Won; Ko, Young Tae

2009-01-01

Hilar cholangiocarcinoma is associated with a dismal prognosis; however, curative resection may offer a chance of cure. Various factors should be considered in the surgical planning for curative resection. These factors include extent of bile duct involvement, relationship between portal vein and tumor involvement, diffuse hepato duodenal ligament infiltration, vascular invasion, lymph node metastasis, peritoneal seeding, and hepatic volume. Using high-quality volume data from multidetector-row computed tomography (MDCT) and adequate postprocessing images, radiologists can provide various types of information, imperative for curative resection of a hilar cholangiocarcinoma. This review illustrates the role of MDCT in the preoperative workup of hilar cholangiocarcinoma

[Acute cardiac failure in pheochromocytoma.

DEFF Research Database (Denmark)

Jønler, Morten; Munk, Kim

2008-01-01

Pheochromocytoma (P) is an endocrine catecholamine-secreting tumor. Classical symptoms like hypertension, attacks of sweating, palpitations, headache and palor are related to catecholamine discharge. We provide a case of P in a 71 year-old man presenting with acute cardiac failure, severe reduction...

TROP2 correlates with microvessel density and poor prognosis in hilar cholangiocarcinoma.

Science.gov (United States)

Ning, Shanglei; Guo, Sen; Xie, Jianjun; Xu, Yunfei; Lu, Xiaofei; Chen, Yuxin

2013-02-01

Trophoblast cell surface antigen 2 (TROP2) was found to be associated with tumor progression and poor prognosis in a variety of epithelial carcinomas. The aim of the study was to investigate TROP2 expression and its prognostic impact in hilar cholangiocarcinoma. Immunohistochemistry and quantitative real-time PCR were used to determine TROP2 expression in surgical specimens from 70 hilar cholangiocarcinoma patients receiving radical resection. The relationship between TROP2 expression and microvessel density was investigated and standard statistical analysis was used to evaluate TROP2 prognosis significance in hilar cholangiocarcinoma. High TROP2 expression by immunohistochemistry was found in 43 (61.4 %) of the 70 tumor specimens. Quantitative real-time PCR confirmed that TROP2 level in tumor was significantly higher than in non-tumoral biliary tissues (P = 0.001). Significant correlations were found between TROP2 expression and histological differentiation (P = 0.016) and tumor T stage (P = 0.031) in hilar cholangiocarcinoma. TROP2 expression correlated with microvessel density in hilar cholangiocarcinoma (P = 0.026). High TROP2 expression patients had a significantly poorer overall survival rate than those with low TROP2 expression (30 vs. 68.5 %, P = 0.001), and multivariate Cox regression analysis indicated TROP2 as an independent prognostic factor for hilar cholangiocarcinoma (P = 0.004). TROP2 expression correlates with microvessel density significantly and is an independent prognostic factor in human hilar cholangiocarcinoma.

Exploring new strategies in diagnosis and treatment of hilar cholangiocarcinoma

NARCIS (Netherlands)

Mantel, Hendrik Teunis Johannes

2016-01-01

Hilar cholangiocarcinoma is a rare form of cancer arising at the confluence of the right and left bile duct. The disease is known for its difficult diagnosis and treatment. The chapters in this thesis describe different aspects of hilar cholangiocarcinoma with the aim to improve diagnosis and

Photodynamic Therapy in Patients with Advanced Hilar Cholangiocarcinoma: Percutaneous Cholangioscopic Versus Peroral Transpapillary Approach.

Science.gov (United States)

Lee, Tae Yoon; Cheon, Young Koog; Shim, Chan Sup

2016-04-01

This study aimed to compare the clinical outcomes of patients with advanced hilar cholangiocarcinoma (CC) who underwent photodynamic therapy (PDT) with either percutaneous transhepatic cholangioscopy (PTCS) or endoscopic retrograde cholangiopancreatography (ERCP). PDT has been proposed as a promising therapy for treatment of unresectable hilar CC that is resistant to conventional standard treatment. However, few studies have compared the delivery methods of PDT in unresectable hilar CC patients. Thirty-seven adult patients with advanced hilar CC were included in this study. Twenty-four patients treated with PTCS-directed PDT and 13 patients treated with ERCP-directed PDT were analyzed retrospectively. The PTCS- and ERCP-directed PDT groups were comparable with respect to age, gender, health status, pretreatment bilirubin levels, Bismuth type, and hilar CC stage. The length of hospital stay differed significantly (p hilar CC. Lower pre-PDT bilirubin levels were associated with longer survival in all patients.

The accumulation of regulatory T cells in the hepatic hilar lymph nodes in biliary atresia.

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Sakamoto, Naoya; Muraji, Toshihiro; Ohtani, Haruo; Masumoto, Kouji

2017-10-01

A proposed etiopathogenesis of biliary atresia (BA) involves T-cell-mediated inflammatory bile duct damage and progressive hepatic fibrosis. Pediatric surgeons often observe swelling of the hepatic hilar lymph nodes during the Kasai procedure. Given the importance of regulatory mechanisms in immune responses, the present study was designed to analyze the quantitative changes of regulatory T cells (T reg cells) in the hepatic hilar lymph nodes (hepatic hilar LNs) and peripheral blood (PB) in BA. The hepatic hilar LNs and PB obtained during the Kasai procedure were analyzed by flow cytometry. The ratios of total and active Tregs to the total CD4 + cells in the PB and the hepatic hilar LNs were compared. In patients with BA, the ratios of both the total and active T reg cells in the hepatic hilar LNs were higher than those in the PB (total T reg cells: PB vs. LN; P hilar lymph nodes of BA patients. This finding could shed light on the pathogenesis of BA.

MANAGEMENT OF ENDOCRINE DISEASE: Outcome of adrenal sparing surgery in heritable pheochromocytoma.

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Castinetti, F; Taieb, D; Henry, J F; Walz, M; Guerin, C; Brue, T; Conte-Devolx, B; Neumann, H P H; Sebag, F

2016-01-01

The management of hereditary pheochromocytoma has drastically evolved in the last 20 years. Bilateral pheochromocytoma does not increase mortality in MEN2 or von Hippel-Lindau (VHL) mutation carriers who are followed regularly, but these mutations induce major morbidities if total bilateral adrenalectomy is performed. Cortical sparing adrenal surgery may be proposed to avoid definitive adrenal insufficiency. The surgical goal is to leave sufficient cortical tissue to avoid glucocorticoid replacement therapy. This approach was achieved by the progressive experience of minimally invasive surgery via the transperitoneal or retroperitoneal route. Cortical sparing adrenal surgery exhibits management of all patients with MEN2 or VHL hereditary pheochromocytoma. Hereditary pheochromocytoma is a rare disease, and a randomized trial comparing cortical sparing vs classical adrenalectomy is probably not possible. This lack of data most likely explains why cortical sparing surgery has not been adopted in most expert centers that perform at least 20 procedures per year for the treatment of this disease. This review examined recent data to provide insight into the technique, its indications, and the results and subsequent follow-up in the management of patients with hereditary pheochromocytoma with a special emphasis on MEN2. © 2016 European Society of Endocrinology.

Internal radiotherapy for hilar bile duct cancer

International Nuclear Information System (INIS)

Ryu, Munemasa; Ogino, Takashi; Konishi, Hiroshi

1999-01-01

By December 1998, 24 patients with non-resected hilar bile duct cancer (mean age of 74) had received bile duct intracavitary irradiation and 13 patients with residual cancer after resection of hilar bile duct cancer had received postoperative intracavitary irradiation. After they were externally irradiated 30 Gy in total by 15 fractions (2 Gy/time, 5 times in a week), intracavitary irradiation using 192-Ir was given 5 times in total (2 times in a week) from 3 weeks after external irradiation under the condition which dose became 8 Gy in depth of 10 mm from radiation source. The cases of postoperative irradiation had 3 times in total. As for 20 patients of non-resected hilar bile duct cancer without metastasis, 50% survival time was 265 days and there was no 5 year survivor. Fifty percents survival time of 4 patients with metastasis was 113 days. The effect of local control was recognized in 20 patients (83.3%). In 13 patients of postoperative irradiation, 50% survival time was 554 days, and survival rate of 3 years was 28%. (K.H.)

Multiple adrenal masses: MRI tissue differentiation of pheochromocytoma and adenoma at 1.5 T

International Nuclear Information System (INIS)

Slapa, R.Z.; Jakubowski, W.; Feltynowski, T.; Januszewicz, A.

1997-01-01

We present the case of 38-year-old hypertensive patient with bilateral adrenal masses and with clinical and biochemical suspicion of pheochromocytoma. Magnetic resonance imaging at 1.5 T established correct diagnosis of coexisting adrenal pheochromocytoma and adenoma (nonhyperfunctioning). The case supports the usefulness of MRI for definitive evaluation of bilateral adrenal masses in patients with suspicion of pheochromocytoma. (orig.). With 2 figs

Squamous Cell Carcinoma of the Hilar Bile Duct

Directory of Open Access Journals (Sweden)

Ippei Yamana

2011-08-01

Full Text Available We herein report a rare case of squamous cell carcinoma of the hilar bile duct. A 66-year-old Japanese male patient was admitted to our hospital because of appetite loss and jaundice. Abdominal computed tomography revealed an enhanced mass measuring 10 × 30 mm in the hilar bile duct region. After undergoing biliary drainage, the patient underwent extended right hepatic lobectomy with regional lymph nodes dissection. The tumor had invaded the right portal vein. Therefore, we also performed resection and reconstruction of the portal vein. Histopathologically, the carcinoma cells exhibited a solid structure with differentiation to squamous cell carcinoma with keratinization and intercellular bridges. Immunohistochemical staining of the tumor cells revealed positive cytokeratin staining and negative CAM 5.2 staining. Based on these findings, a definitive diagnosis of well-differentiated squamous cell carcinoma of the hilar bile duct was made.

Imaging and interventions in hilar cholangiocarcinoma: A review

Science.gov (United States)

Madhusudhan, Kumble Seetharama; Gamanagatti, Shivanand; Gupta, Arun Kumar

2015-01-01

Hilar cholangiocarcinoma is a common malignant tumor of the biliary tree. It has poor prognosis with very low 5-year survival rates. Various imaging modalities are available for detection and staging of the hilar cholangiocarcinoma. Although ultrasonography is the initial investigation of choice, imaging with contrast enhanced computed tomography scan or magnetic resonance imaging is needed prior to management. Surgery is curative wherever possible. Radiological interventions play a role in operable patients in the form of biliary drainage and/or portal vein embolization. In inoperable cases, palliative interventions include biliary drainage, biliary stenting and intra-biliary palliative treatment techniques. Complete knowledge of application of various imaging modalities available and about the possible radiological interventions is important for a radiologist to play a critical role in appropriate management of such patients.We review the various imaging techniques and appearances of hilar cholangiocarcinoma and the possible radiological interventions. PMID:25729485

Pheochromocytoma as a frequent false-positive in adrenal washout CT. A systematic review and meta-analysis

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Woo, Sungmin; Kim, Sang Youn [Seoul National University College of Medicine, Department of Radiology, Seoul (Korea, Republic of); Suh, Chong Hyun [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea, Republic of); Namwon Medical Center, Department of Radiology, Namwon-si, Jeollabuk-do (Korea, Republic of); Cho, Jeong Yeon; Kim, Seung Hyup [Seoul National University College of Medicine, Department of Radiology, Seoul (Korea, Republic of); Seoul National University Medical Research Center, Institute of Radiation Medicine and Kidney Research Institute, Seoul (Korea, Republic of)

2018-03-15

To evaluate the proportion of pheochromocytomas meeting the criteria for adenoma on adrenal washout CT and the diagnostic performance of adrenal washout CT for differentiating adenoma from pheochromocytoma. MEDLINE and EMBASE were searched to 28 March 2017. We included studies that used adrenal washout CT for characterisation of pheochromocytomas. Two independent reviewers assessed the methodological quality using Quality Assessment of Diagnostic Accuracy Studies-2. Proportions were pooled using an inverse variance method for calculating weights (random-effects). Sensitivity and specificity were pooled using hierarchical logistic regression modelling and plotted in a hierarchical summary receiver-operating-characteristics (HSROC) plot. Ten studies (114 pheochromocytomas) were included. The pooled proportion of pheochromocytomas meeting the criteria for adenomas was 35 % (95 % CI 20-51). For eight studies providing information on diagnostic performance, the pooled sensitivity and specificity for differentiating adenoma from pheochromocytoma were 0.97 (95 % CI 0.93-0.99) and 0.67 (95 % CI 0.44-0.84), respectively. The area under the HSROC curve was 0.97 (95 % CI 0.95-0.98). There was a non-negligible proportion of pheochromocytomas meeting the criteria for adenoma on adrenal washout CT. Although overall diagnostic performance was excellent for differentiating adenoma from pheochromocytoma, specificity was relatively low. (orig.)

Pheochromocytoma as a frequent false-positive in adrenal washout CT. A systematic review and meta-analysis

International Nuclear Information System (INIS)

Woo, Sungmin; Kim, Sang Youn; Suh, Chong Hyun; Cho, Jeong Yeon; Kim, Seung Hyup

2018-01-01

To evaluate the proportion of pheochromocytomas meeting the criteria for adenoma on adrenal washout CT and the diagnostic performance of adrenal washout CT for differentiating adenoma from pheochromocytoma. MEDLINE and EMBASE were searched to 28 March 2017. We included studies that used adrenal washout CT for characterisation of pheochromocytomas. Two independent reviewers assessed the methodological quality using Quality Assessment of Diagnostic Accuracy Studies-2. Proportions were pooled using an inverse variance method for calculating weights (random-effects). Sensitivity and specificity were pooled using hierarchical logistic regression modelling and plotted in a hierarchical summary receiver-operating-characteristics (HSROC) plot. Ten studies (114 pheochromocytomas) were included. The pooled proportion of pheochromocytomas meeting the criteria for adenomas was 35 % (95 % CI 20-51). For eight studies providing information on diagnostic performance, the pooled sensitivity and specificity for differentiating adenoma from pheochromocytoma were 0.97 (95 % CI 0.93-0.99) and 0.67 (95 % CI 0.44-0.84), respectively. The area under the HSROC curve was 0.97 (95 % CI 0.95-0.98). There was a non-negligible proportion of pheochromocytomas meeting the criteria for adenoma on adrenal washout CT. Although overall diagnostic performance was excellent for differentiating adenoma from pheochromocytoma, specificity was relatively low. (orig.)

Impact of splenic hilar lymph node metastasis on prognosis in patients with advanced gastric cancer.

Science.gov (United States)

Son, Taeil; Kwon, In Gyu; Lee, Joong Ho; Choi, Youn Young; Kim, Hyoung-Il; Cheong, Jae-Ho; Noh, Sung Hoon; Hyung, Woo Jin

2017-10-13

Impact of splenic hilar LN dissection during total gastrectomy for proximal advanced gastric cancer is controversial. The objective of this study was to assess the impact on prognosis of splenic hilar lymph node(LN) metastasis compared to that of metastasis to other regional LN groups. Patients who underwent total gastrectomy with D2 LN dissection from 2000 to 2010 were reviewed retrospectively. The clinicopathologic characteristics and long-term results of patients with splenic hilar LN metastasis were compared to those of patients with only metastasis to other extraperigastric LNs (stations #8a, #9, #11, or #12a). To investigate the survival benefit of performing splenic hilar LN dissection, the estimated therapeutic index for the procedure was calculated by multiplying the incidence of metastases in the hilar region by the survival rates for individuals with nodal involvement in that region. Of 602 patients, 87(14.5%) had hilar LN metastasis. The 5-year overall and relapse-free survival rates for patients with hilar LN metastasis were 24.1% and 12.1%, respectively. These rates were similar to those for patients with metastasis to other extraperigastric LNs ( P > 0.05), with similar recurrence patterns. Overall survival in the hilar LN metastasis group was better than that for patients with distant metastasis( P hilar LN dissection was 3.5, which was similar to index values for LN dissection at other extraperigastric LNs. Dissection of splenic hilar LNs during total gastrectomy for advanced gastric cancer allows for a prognosis similar to that achieved with dissection of extraperigastric LNs.

Treatment Option Overview (Pheochromocytoma and Paraganglioma)

Science.gov (United States)

... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

General Information about Pheochromocytoma and Paraganglioma

Science.gov (United States)

... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

Treatment Options for Pheochromocytoma and Paraganglioma

Science.gov (United States)

... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

Differentiating pheochromocytoma from lipid-poor adrenocortical adenoma by CT texture analysis: feasibility study.

Science.gov (United States)

Zhang, Gu-Mu-Yang; Shi, Bing; Sun, Hao; Jin, Zheng-Yu; Xue, Hua-Dan

2017-09-01

To investigate the feasibility of using CT texture analysis (CTTA) to differentiate pheochromocytoma from lipid-poor adrenocortical adenoma (lp-ACA). Ninety-eight pheochromocytomas and 66 lp-ACAs were included in this retrospective study. CTTA was performed on unenhanced and enhanced images. Receiver operating characteristic (ROC) analysis was performed, and the area under the ROC curve (AUC) was calculated for texture parameters that were significantly different for the objective. Diagnostic accuracies were evaluated using the cutoff values of texture parameters with the highest AUCs. Compared to lp-ACAs, pheochromocytomas had significantly higher mean gray-level intensity (Mean), entropy, and mean of positive pixels (MPP), but lower skewness and kurtosis on unenhanced images (P feasible to use CTTA to differentiate pheochromocytoma from lp-ACA.

Treatment of hilar cholangiocarcinoma with inserting biliary double stents

International Nuclear Information System (INIS)

Jia Guangzhi; Zhang Zidong; Wang Xuejing; Yin Hua; Li Jianming

2004-01-01

Objective: To investigate the inserting technique of biliary double stents in treating hilar cholangiocarcinoma. Methods: 6 patients with hilar cholangiocarcinoma (Bismuth IV) were treated by percutaneous transhepatic insertion of biliary stents. Double stents were inserted in each patient. Different inserting methods were adopted according to the branch angles formed by left and right hepatic ducts. Results: The jaundice of all patients alleviated or disappeared obviously after stent implantation. The average difference between post-and pre-operation in the serum total bilirubin level was (104 ± 29) μmol/L (P<0.01). Stent obstruction was found in 2 cases after 4 and 6 months respectively. Conclusion: Double stents implantation is effective for the treatment of hilar cholangiocarcinoma. Beware of the angulation between main hepatic duct and adopting different inserting methods. (authors)

High resolution CT for localization of early hilar lung carcinoma

International Nuclear Information System (INIS)

Minami, Yuko; Ishikawa, Shigemi; Saida, Yukihisa; Kajitani, Motomasa; Yamamoto, Tatsuo; Sato, Yukio; Onizuka, Masataka; Sakakibara, Yuzuru; Noguchi, Masayuki

2002-01-01

The purpose of this study was to analyse the usefulness of high resolution CT (HRCT) for the diagnosis and localization of roentgenographically occult lung cancer. HRCT was performed prospectively on chest X-ray negative patients with bloody sputum or suspicious or positive cells on sputum cytology between 1998 and 2000. After the HRCT scan, white light bronchoscopy and autofluorescence bronchoscopy were performed. HRCT depicted 19 hilar bronchial lesions in 13 cases out of 19 patients, of which 9 lesions were confirmed by white light broncoscope. Of 8 hilar squamous cell carcinomas diagnosed in this study, 7 lesions (87.5%) were depicted by HRCT. One CT-negative case (12.5%) was an in situ carcinoma in left B 1+2 . Four out of 20 lesions which showed bronchoscopic abnormality, could not be depicted by HRCT. HRCT could prospectively detect 80% of the bronchoscopic abnormalities and 87.5% of the hilar squamous cell carcinomas of the tracheobronchial lesions of the lung. Therefore, HRCT can be an effective supplemental means for screening for hilar squamous cell carcinoma. (author)

Palliative management of hilar cholangiocarcinoma

NARCIS (Netherlands)

Singhal, D.; van Gulik, T. M.; Gouma, D. J.

2005-01-01

Around 80% of the patients with hilar cholangiocarcinoma are candidates for palliative management due to extensive co-morbidity for major surgery, metastases or advanced loco-regional disease. The primary aim of treatment is to provide biliary drainage with long-term relief from pruritis,

MR imaging of an intrapericardial pheochromocytoma

International Nuclear Information System (INIS)

Fisher, M.R.; Higgins, C.B.; Andereck, W.

1985-01-01

The magnetic resonance (MR) appearance of an intrapericardial pheochromocytoma is reported, and the role of MR, compared with CT and a 131 I-metaiodobenzylguanidine scan in delineating masses from cardiac and vascular structures in the mediastinum is discussed

Is the excess cardiovascular morbidity in pheochromocytoma related to blood pressure or to catecholamines?

Science.gov (United States)

Stolk, Roeland F; Bakx, Carel; Mulder, Jan; Timmers, Henri J L M; Lenders, Jacques W M

2013-03-01

It is generally accepted that pheochromocytoma is associated with an increased cardiovascular risk. This is however not based on studies with an appropriate control group of patients with essential hypertension. We examined whether patients with pheochromocytoma have an excess cardiovascular morbidity as compared to hypertensive patients. In a retrospective case-control study we reviewed the medical charts of 109 pheochromocytoma patients for cardiovascular events within 5 years prior to the diagnosis. These patients were matched to control patients with essential hypertension for gender and year of birth and diagnosis. Outcome variables were ischemic heart disease, cerebrovascular accidents, and transient ischemic attacks. Classical cardiovascular risk factors were also assessed. A significantly higher rate of patients with pheochromocytoma suffered a cardiovascular event (13.8%; 95% confidence interval: 7.9%-21.6%) as compared to hypertensive patients (1.1%, 95% confidence interval: 0.1%-3.9%) (P risk factors. Pheochromocytoma patients have a clearly higher rate of cardiovascular events than patients with essential hypertension. This cannot be attributed to differences in blood pressure or other cardiovascular risk factors. The most likely explanation for the excess event rate is the prolonged exposure to the toxic effects of tumoral catecholamines. These data underpin the importance of a timely diagnosis and treatment of pheochromocytoma.

Duct-to-duct biliary reconstruction after radical resection of Bismuth IIIa hilar cholangiocarcinoma.

Science.gov (United States)

Wu, Wen-Guang; Gu, Jun; Dong, Ping; Lu, Jian-Hua; Li, Mao-Lan; Wu, Xiang-Song; Yang, Jia-Hua; Zhang, Lin; Ding, Qi-Chen; Weng, Hao; Ding, Qian; Liu, Ying-Bin

2013-04-21

At present, radical resection remains the only effective treatment for patients with hilar cholangiocarcinoma. The surgical approach for R0 resection of hilar cholangiocarcinoma is complex and diverse, but for the biliary reconstruction after resection, almost all surgeons use Roux-en-Y hepaticojejunostomy. A viable alternative to Roux-en-Y reconstruction after radical resection of hilar cholangiocarcinoma has not yet been proposed. We report a case of performing duct-to-duct biliary reconstruction after radical resection of Bismuth IIIa hilar cholangiocarcinoma. End-to-end anastomosis between the left hepatic duct and the distal common bile duct was used for the biliary reconstruction, and a single-layer continuous suture was performed along the bile duct using 5-0 prolene. The patient was discharged favorably without biliary fistula 2 wk later. Evidence for tumor recurrence was not found after an 18 mo follow-up. Performing bile duct end-to-end anastomosis in hilar cholangiocarcinoma can simplify the complex digestive tract reconstruction process.

A Clinical Roadmap to Investigate the Genetic Basis of Pediatric Pheochromocytoma: Which Genes Should Physicians Think About?

Directory of Open Access Journals (Sweden)

Bernardo Dias Pereira

2018-01-01

Full Text Available Pheochromocytoma is very rare at a pediatric age, and when it is present, the probability of a causative genetic mutation is high. Due to high costs of genetic surveys and an increasing number of genes associated with pheochromocytoma, a sequential genetic analysis driven by clinical and biochemical phenotypes is advised. The published literature regarding the genetic landscape of pediatric pheochromocytoma is scarce, which may hinder the establishment of genotype-phenotype correlations and the selection of appropriate genetic testing at this population. In the present review, we focus on the clinical phenotypes of pediatric patients with pheochromocytoma in an attempt to contribute to an optimized genetic testing in this clinical context. We describe epidemiological data on the prevalence of pheochromocytoma susceptibility genes, including new genes that are expanding the genetic etiology of this neuroendocrine tumor in pediatric patients. The clinical phenotypes associated with a higher pretest probability for hereditary pheochromocytoma are presented, focusing on differences between pediatric and adult patients. We also describe new syndromes, as well as rates of malignancy and multifocal disease associated with these syndromes and pheochromocytoma susceptibility genes published more recently. Finally, we discuss new tools for genetic screening of patients with pheochromocytoma, with an emphasis on its applicability in a pediatric population.

Hypertensive crisis in pregnancy due to a metamorphosing pheochromocytoma with postdelivery Cushing's syndrome.

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Langton, Katharina; Gruber, Matthias; Masjkur, Jimmy; Steenblock, Charlotte; Peitzsch, Mirko; Meinel, Jörn; Lenders, Jacques; Bornstein, Stefan; Eisenhofer, Graeme

2018-01-01

Pheochromocytomas in pregnancy are rare but potentially lethal. Even rarer is the combination of pheochromocytoma in pregnancy with subsequent development of ectopic Cushing's syndrome. We report a 36-year-old woman, previously diagnosed with essential hypertension, who developed severe hypertension in pregnancy complicated by insulin-dependent gestational diabetes. A cesarean section was performed at 32 weeks following a hypertensive crisis after routine administration of betamethasone. Postnatal persistence of signs and symptoms of catecholamine excess led to the diagnosis of a left adrenal pheochromocytoma. Between diagnosis and planned tumor removal, the patient developed signs and symptoms of Cushing's syndrome (facial edema and hirsutism, myopathy and fatigue). Biochemical testing confirmed hypercortisolism with extremely elevated levels of plasma adrenocorticotropin, urinary cortisol and multiple steroids of a plasma panel that were all normal at previous testing. The previously noradrenergic tumor also started producing epinephrine. Histopathological examination confirmed the pheochromocytoma, which was also immunohistochemically positive for adrenocorticotropin. Full post-surgical recovery was sustained with normal blood pressure and biochemical findings after one year. This report not only underlines the chameleon behavior of pheochromocytoma but also illustrates its potential for a metamorphosing presentation. Corticosteroid administration in pregnancy requires a cautious approach in patients with hypertension.

Childhood familial pheochromocytoma. Conflicting results of localization techniques

International Nuclear Information System (INIS)

Turner, M.C.; DeQuattro, V.; Falk, R.; Ansari, A.; Lieberman, E.

1986-01-01

Childhood familial pheochromocytoma was investigated in four patients by abdominal computed tomographic scan, [ 131 I]metaiodobenzylguanidine scan, and vena caval catecholamine sampling. Results conflicted with surgical findings. Computed tomographic scan identified all four adrenal tumors but missed two midline tumors in one patient. [ 131 I]metaiodobenzylguanidine scan identified two of three adrenal tumors but also suggested extra-adrenal tumors not confirmed at operation in two of three patients. Vena caval sampling for catecholamines confirmed all adrenal tumors but suggested additional tumors not verified at operation in two of three patients. All patients are asymptomatic and have normal urinary catecholamines 15 to 51 months after operation. Because of the frequency of multiple tumors in familial pheochromocytoma, different diagnostic techniques were employed. False-positive results were more frequent with [ 131 I]metaiodobenzylguanidine and vena caval sampling. Reinterpretation of the [ 131 I]metaiodobenzylguanidine scans at a later date led to less false-positive interpretation, although the false-negative rate remained unchanged. More pediatric experience with [ 131 I]metaiodobenzylguanidine scans and vena caval sampling in familial pheochromocytoma is needed. Confirmation of tumor and its localization rest with meticulous surgical exploration

MRI diagnosis and preoperative evaluation for hepatic hilar tumor

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Liu Yulin; Kong xiangquan; Xu Haibo; Xiao Xuehong; Liu Dingxi; Peng Zhenjun

2004-01-01

Objective: To investigate the value of the all-in-one MR scanning in the diagnosis and preoperative evaluation of hepatic hilar tumor. Methods: Forty-two cases of hepatic hilar tumors were examined with a 1.5 T superconductive MR system, including hepatocellular carcinoma (HCC, n=12), hilar cholangiocarcinoma (HC, n=22), and hilar metastasis (n=8). Besides the precontrast MRI and MRCP, all cases underwent consecutive dynamic contrast-enhanced MR scanning. The whole liver dynamic contrast-enhanced MR was performed with the first bolus injection of Gd-DTPA (10 ml), and 15 minutes later, 3D DCE MRA was performed with additional injection of Gd-DTPA (15-20 ml) (0.15-0.20 mmol/kg). The contrast time-signal curve of liver and tumor was drawn, and arterial and portal venous phase images were reconstructed with MIP. MR appearances were compared with surgical findings and pathology. Results: Consecutive DCE scanning was successfully performed in all cases. The contrast time-signal curve of HCC showed type I and II (10/12, 83.3%), and the curve of HC showed type III and IV (21/22, 95.4%), whereas the curve of metastasis was various. The difference of tumor peak transit time (PT) between HCC group and HC group was significant (P<0.05). The vascular invasion in HCC group appeared as arterial-portal vein fistula (2/12, 16.7%), portal vein infiltration (3/24, 12.5%), and occlusion by tumor thrombosis (4/24, 16.7%). However, the vascular invasion in HC group showed spiral artery (5/22, 22.7%), portal vein infiltration (5/44, 11.4%), portal vein central narrowing (8/44, 18.2%) and occlusion (11/44, 25.0%). All metastasis had no vascular invasion expect one gall bladder carcinoma with right portal vein infiltration. The accuracy of preoperative evaluation with MRI in HCC group and HC group was 83.3% (10/12) and 86.4% (19/22), respectively. The accuracy of preoperative evaluation in all hilar tumors was 78.6% (33/42). Conclusion: Consecutive DCE was a safe and useful technique in MR

Reversible catecholamine-induced cardiomyopathy due to pheochromocytoma: case report.

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Satendra, Milan; de Jesus, Cláudia; Bordalo e Sá, Armando L; Rosário, Luís; Rocha, José; Bicha Castelo, Henrique; Correia, Maria José; Nunes Diogo, António

2014-03-01

Pheochromocytoma is a tumor originating from chromaffin tissue. It commonly presents with symptoms and signs of catecholamine excess, such as hypertension, tachycardia, headache and sweating. Cardiovascular manifestations include catecholamine-induced cardiomyopathy, which may present as severe left ventricular dysfunction and congestive heart failure. We report a case of pheochromocytoma which was diagnosed following investigation of dilated cardiomyopathy. We highlight the dramatic symptomatic improvement and reversal of cardiomyopathy, with recovery of left ventricular function after treatment. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

Hilar biliary neurofibroma without neurofibromatosis: case report with contrast-enhanced ultrasound findings.

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Guo, Huan-Ling; Chen, Li-da; Wang, Zhu; Huang, Yang; Liu, Jin-Ya; Shan, Quan-Yuan; Xie, Xiao-Yan; Lu, Ming-de; Wang, Wei

2016-10-01

Solitary neurofibroma located in the hilum of the liver is extremely rare, particularly without neurofibromatosis. We herein report a case of hilar biliary neurofibroma without signs of von Recklinghausen's disease. A 36-year-old man was admitted to our department with progressive jaundice. The case was diagnosed as hilar cholangiocarcinoma based on preoperative imaging. The patient consequently received a Roux-en-Y hepaticojejunostomy and was confirmed with neurofibroma pathologically. This is the first reported imaging finding of hilar biliary neurofibroma using contrast-enhanced ultrasound, emphasizing the differential diagnosis of biliary tumors.

Evaluation of delayed contrast-enhanced CT scan in diagnosing hilar cholangiocarcinoma

International Nuclear Information System (INIS)

Li Jianding; Liang Chenyang; Zhang Hua; Zhang Yuezhen; Li Rui

2001-01-01

Objective: To assess the diagnostic value of delayed CT contrast enhancement patterns in hilar cholangiocarcinoma based on two-phased dynamic incremental CT scanning. Methods: Fifty-two patients with suspected hilar tumor and bile duct obstruction underwent spiral CT scan. The scan time for one revolution of the X-ray tube was 1 second. To elucidate the delay time for optimal imaging, all proved cholangiocarcinoma with delayed (6, 8, 10, 15, 20, 30 minutes) post-equilibrium-phase contrast-enhanced CT scans were acquired with unenhanced, dynamic contrast-enhanced, and delayed images. Degree of delayed enhancement was compared with that of surrounding liver parenchyma. Results: (1) 8-15 minutes after IV injection of contrast material was the delay time for optimal imaging. (2) Of 29 cholangiocarcinomas, the early CT showed hypo-attenuating (lower than that of liver parenchyma) in 23 tumors, iso-attenuating (equal to that of the liver) in 4 tumors, and hyper-attenuating (higher than that of liver) in 2 tumors. The delayed CT scan showed iso-attenuating in 8 tumors, hyper-attenuating in 21 tumors, and no hypo-attenuating. Most of delay imaging of hilar cholangiocarcinoma may appear hyper-attenuating (U = -4.3073, P 2 = 9.09, P < 0.01). Conclusion: When assessing hilar tumor, delayed CT contrast enhancement patterns based on two-phase dynamic incremental CT scans is useful in the detection and characterization of hilar cholangiocarcinoma

Rapid reversal of heart failure in a patient with pheochromocytoma and catecholamine-induced myocarditis/cardiomyopathy

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Ahmed, I.; Sankaran, R.; Al-Addad, A.

2002-01-01

Pheochromocytoma is rare and usually presents as paroxysal or sustained hypertension, nonetheless, it can also cause severe acute pulmonary edema in normotensive individuals. We present a case with pheochromocytoma and severe left ventricular failure caused by catecholamine induced myocarditis/cardiomyopathy. Severe left ventricular failure resolved rapidly and left ventricular systolic function became normal within two weeks of medical treatment. Later, patient underwent elective and uneventful surgical removal of pheochromocytoma. (author)

The HSP90 inhibitor 17-AAG exhibits potent antitumor activity for pheochromocytoma in a xenograft model.

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Xu, Yunze; Zhu, Qi; Chen, Dongning; Shen, Zhoujun; Wang, Weiqing; Ning, Guang; Zhu, Yu

2015-07-01

This study aims to investigate the effect of heat shock protein 90 (HSP90) inhibitor 17-allylamino-17-demethoxygeldanamycin (17-AAG) in the malignant pheochromocytoma using a xenograft mouse model. Treatment with 17-AAG induced a marked reduction in the volume and weight of PC12 pheochromocytoma cell tumor xenografts in mice. Furthermore, 17-AAG also significantly inhibited the expression of HSP90 and its client proteins. Our results validated HSP90 as an important target in pheochromocytoma and provided rationale for the testing of HSP90 inhibitors as a promising therapeutic agent in the antitumor therapy of pheochromocytoma.

[Pheochromocytoma in 8-year observation at a single endocrinological center in Wroclaw].

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Bednarek-Tupikowska, Grazyna; Bucyk, Barbara; Daroszewski, Jacek; Bidzińska-Speichert, Bozena; Bohdanowicz-Pawlak, Anna; Szymczak, Jadwiga; Bednorz, Włodzimierz; Podgórski, Franciszek; Zareba-Bogdał, Elzbieta; Kuliczkowska-Płaksej, Justyna; Lenarcik, Agnieszka; Filus, Alicja; Kałuzny, Marcin; Kubicka, Eliza; Syrycka, Joanna; Tupikowska, Małgorzata; Lizurej, Oskar; Bolanowski, Marek; Milewicz, Andrzej

2009-01-01

Pheochromocytoma is rare tumor with a highly variable clinical presentation. This report provides clinical picture, efficiency of diagnostics and treatment of pheochromocytoma in 8-years in the endocrinological center in Wroclaw. The records of 37 patients with pheochromocytoma were identified, who were treated in 2000-2007 in the Department of Endocrinology, Diabetology and Isotope Treatment in Wroclaw. There were 23 women (age 23-75 year) and 14 men (age 17-74). We studied frequency of clinical signs, usefulness of diagnostic methods and efficacy of treatment. The duration of the clinical history ranged from 2 months to 16 years. The most frequent symptoms were: hypertension paroxysmal and constant, palpitations, headache, sweating and anxiety. The most sensitive diagnostic method was increased concentration of urinary metanephrine in 24-hour urine. Computed tomography was the most widely used method for tumor localization. Adrenal pheochromocytoma was detecting by CT in all patients, predominated in right adrenal, in 1 case in urinary bladder. Surgery caused remission of hypertension in 59%, improvement in 26.8%, and no changes in 13.9% of patients. Malignancy was reported in 2 cases, 1 woman died after surgery. MEN 2A occur in 21.6%. The diagnosis of pheochromocytma is usually made after long duration of the disease. The study confirms that clinical presentation of pheochromocytoma is variable and nonspecific, this finding makes the diagnosis very difficult. The most typical symptom is paroxysmal hypertension, which is present only in 40%, other symptoms are nonspecific. The measurement of 24-hour urinary metanephrines was the best indicator. CT was almost always successful in localizing the tumor. Patients with pheochromocytoma should be consider for other endocrine diseases especially medullary carcinoma, primary hyperparathyroidism and other component of MEN 2A.

Extra-adrenal pheochromocytoma in pregnancy: ultrasonography and magnetic resonance imaging findings

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Carson, B.J.W.; Johnston, M.A.; Iwaniuk, G.

1995-04-01

The case of a 24-year-old woman with extra-adrenal pheochromocytoma during pregnancy was described. Initial ultrasonography (US) at 27 weeks gestational age failed to show the pheochromocytoma, which was subsequently found by US at 35 weeks gestational age. Magnetic resonance imaging (MRI) provided diagnostic confirmation, as well as important information used by the obstetrician and the surgeon in operative planning. MRI proved to be ideally suited to investigation of extra-adrenal pheochromocytomas in pregnancy because it does not use ionizing radiation, and it has multiplanar capability and high contrast resolution. In the case described, the lesion was demonstrated with all MRI pulse sequences, but was best visualized with turbo spin-echo T2-weighted imaging, as well as with fat-suppressed imaging. 7 refs., 2 figs.

Hilar cholangiocarcinoma: controversies on the extent of surgical resection aiming at cure.

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Xiang, Shuai; Lau, Wan Yee; Chen, Xiao-ping

2015-02-01

Hilar cholangiocarcinoma is the most common malignant tumor affecting the extrahepatic bile duct. Surgical treatment offers the only possibility of cure, and it requires removal of all tumoral tissues with adequate resection margins. The aims of this review are to summarize the findings and to discuss the controversies on the extent of surgical resection aiming at cure for hilar cholangiocarcinoma. The English medical literatures on hilar cholangiocarcinoma were studied to review on the relevance of adequate resection margins, routine caudate lobe resection, extent of liver resection, and combined vascular resection on perioperative and long-term survival outcomes of patients with resectable hilar cholangiocarcinoma. Complete resection of tumor represents the most important prognostic factor of long-term survival for hilar cholangiocarcinoma. The primary aim of surgery is to achieve R0 resection. When R1 resection is shown intraoperatively, further resection is recommended. Combined hepatic resection is now generally accepted as a standard procedure even for Bismuth type I/II tumors. Routine caudate lobe resection is also advocated for cure. The extent of hepatic resection remains controversial. Most surgeons recommend major hepatic resection. However, minor hepatic resection has also been advocated in most patients. The decision to carry out right- or left-sided hepatectomy is made according to the predominant site of the lesion. Portal vein resection should be considered when its involvement by tumor is suspected. The curative treatment of hilar cholangiocarcinoma remains challenging. Advances in hepatobiliary techniques have improved the perioperative and long-term survival outcomes of this tumor.

Step-by-Step robotic heminephrectomy for duplicated renal collecting system

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Homayoun Zargar

2014-08-01

Full Text Available Introduction A duplicated renal collecting system is a relatively common congenital anomaly rarely presenting in adults. Aim In this video we demonstrate our step-by-step technique of Robotic heminephrectomy in a patient with non-functioning upper pole moiety. Materials and Methods Following cystoscopy and ureteral catheter insertion the patient was placed in 600 modified flank position with the ipsilateral arm positioned at the side of the patient. A straight-line, three arm robotic port configuration was employed. The robot was docked at a 90-degree angle, perpendicular to the patient. Following mobilization the colon and identifying both ureters of the duplicated system, the ureters were followed cephalically toward, hilar vessels where the hilar anatomy was identified. The nonfunctioning pole vasculature was ligated using hem-o-lok clips. The ureter was sharply divided and the proximal ureteral stump was passed posterior the renal hilum. Ureteral stump was used as for retraction and heminephrectomy is completed along the line demarcating the upper and lower pole moieties. Renorrhaphy was performed using 0-Vicryl suture with a CT-1 needle. The nonfunctioning pole ureter was then dissected caudally toward the bladder hiatus, ligated using clips, and transected. Results The operating time was 240 minutes and blood loss was 100 cc. There was no complication post-operatively. Conclusions Wrist articulation and degree of freedom offered by robotic platform facilitates successful performance of minimally invasive heminephrectomy in the setting of an atrophic and symptomatic renal segment.

Lung cancer in hilar region: the resectability evaluation with dual phase enhanced EBCT scan

International Nuclear Information System (INIS)

Tan Guosheng; Zhou Xuhui; Li Xiangmin; Fan Miao; Meng Quanfei; Peng Qian; Tan Zhiyu

2005-01-01

Objective: To explore the clinical value of duralphase enhanced electronic beam computed tomography (EBCT) scans in resectability evaluation of lung cancer located in hilar region. Methods: Dual phase enhanced EBCT scans were available for 40 cases that were initially diagnosed as 'carcinoma of lung' in hilar region. The relations between masses and trachea, bronchi, hilar and mediastinal great vessels were analyzed and compared with operation. Results: 38 cases in our series confirmed by operation and pathological examination were divided two groups: respectable (28 cases) and non-resectable (10 cases) groups. 25 cases in the former group were consistent with operation, accounting for 89.3%, and 8 cases, in the latter group, accounting for 80%. The sensitivity, specificity and accuracy of dural-phase enhanced EBCT scan evaluating the relations between masses and hilar and mediastinal structure were as follows: 92.6%, 72.7% and 86.8%. Conclusion: Dural-phase enhanced EBCT scans can provide precise and feasible pre-operative evaluation of lung cancer in hilar region. (authors)

Pheochromocytoma and pregnancy: a deceptive connection.

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Lenders, J.W.M.

2012-01-01

A pheochromocytoma in a pregnant patient is one of the most threatening medical conditions for mother, fetus, and physician. Although extraordinarily rare with a frequency of 0.002% of all pregnancies, this tumor is notorious for its devastating consequences. As in non-pregnant patients, the signs

Feocromocitoma em criança Pheochromocytoma in childhood

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Gesmar Volga Haddad Herdy

2005-03-01

Full Text Available Feocromocitoma é uma neoplasia de células cromafins que causa sintomas e sinais decorrentes da libertação de catecolminas e é muito rara em crianças. Relatamos um caso feocromocitoma em criança, com difícil manejo clínico, confirmado também pela anatomia-patológica e curado após a ressecção cirúrgica.Pheochromocytoma is a neoplasia of chromaffin cells that is very rare in children. Its signs and symptoms result from the release of catecholamines. We report the case of a child with pheochromocytoma of difficult clinical management, confirmed on the anatomicopathological study, and cured after surgical resection.

Adrenergic crisis due to pheochromocytoma - practical aspects. A short review.

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Juszczak, Kajetan; Drewa, Tomasz

2014-01-01

The definitive therapy in case of pheochromocytoma is complete surgical resection. Improper preoperative assessment and medical management generally places the patient at risk for complications, resulting from an adrenergic crisis. Therefore, it is crucial to adequately optimize these patients before surgery. Optimal preoperative medical management significantly decreases morbidity and mortality during the tumor resection. This review addresses current knowledge in pre- and intraoperative assessment of a patient with pheochromocytoma. Before surgery the patient is conventionally prepared with α-adrenergic blockade (over 10-14 days) and subsequently, additional β-adrenergic blockade is required to treat any associated tachyarrhythmias. In preoperative assessment, it is obligatory to monitor arterial blood pressure, heart rate, and arrhythmias and to restore the blood volume to normal. In conclusion, due to the pathophysiological complexity of a pheochromocytoma, the strict cooperation between the cardiologist, endocrinologist, surgeon and the anaesthesiologist for an uneventful outcome should be achieved in patients qualified for the surgical removal of such a tumor.

Acute aortic dissection in patient with suspected pheochromocytoma

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Lešanović Jelena

2016-01-01

Full Text Available Introduction: Aortic dissection is one of the most fatal vascular emergencies. Almost 40% of the patients do not reach hospital in time while more than quarter die in the first 24 hours after the dissection begins. Case Report: A 37-year old man was admitted to our hospital with severe anterior chest pain which had lasted for over a week. Suspected aortic dissection was rapidly confirmed using imaging modalities - MDCT chest scan and TTE, followed by an urgent surgical management - Bentall procedure. MDCT chest scan also discovered adrenal incidentaloma defined as malignant, pheochromocytoma like mass. Due to the critical state of the patient, there was not enough time for further endocrinologic testing. Discussion and conclusion: When treating patients with pheochromocytoma and acute aortic disection, it is crucial to obtain a stable hemodynamic state before the surgery, since they can trigger a severe hypertensive crisis due to high levels of cathecholamines induced chronic vasoconctriction. The most vulnerable periods are the induction of anesthesia and perioperative hemodynamic oscillations, so treating patients with short acting alpha- 1 adrenergic blocking agents preoperatively has proven to be helpful - Phentolamine. Both dissection of aorta and pheochromocytoma present challenges for anesthesiologists and early recognition of symptoms is essential in establishing the diagnosis and reducing the mortality rate.

The importance of pheochromocytoma case detection in patients with neurofibromatosis type 1: A case report and review of literature.

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Tate, Joshua M; Gyorffy, Janelle B; Colburn, Jeffrey A

2017-01-01

Neurofibromatosis type 1 is a complex, multi-system genetic disorder that is associated with an increased prevalence of pheochromocytoma and paraganglioma compared to the general population, 1.0%-5.7% versus 0.2%-0.6%, respectively. A delay in pheochromocytoma and paraganglioma diagnosis or undiagnosed pheochromocytoma and paraganglioma, as seen in normotensive and asymptomatic patients, may portend a significant morbidity and mortality risk due to excess catecholamine secretion. Currently, there are no generally accepted guidelines of screening for pheochromocytoma and paragangliomas in asymptomatic individuals of this population with approaches and practices varying considerably between physicians. Emerging data suggest benefit in routine pheochromocytoma and paraganglioma screening of all individuals with neurofibromatosis type 1. Herein, we present a case to highlight how routine case detection screening would have identified pheochromocytoma earlier in an active duty military member.

The interventional treatment with two stents in malignant hilar obstruction

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Gao Kun; Dai Dingke; Zhao Renyou; Qian Xiaojun

2006-01-01

Objective: To evaluate the methodology of interventional treatment with two stents in malignant hilar obstructions. Methods: Retrospectively analyzing the two stents, interventional management of unresectable malignant hilar obstruction in 22 patients included unilateral and bilateral puncture routes for placing 'T' shape stent set and 'Y' shape stent set, respectively; together with recording the related complications. Results: The overall technical success rate was 100%, without severe complication. The total serum bilirubin reduced from (337.9 + 81.7) μmol/L before procedure to (129.1 + 51.1) μmol/L after the stent placement. Stent obstruction was found in 8 cases at 6 months; 5 cases by the time of 6-12 months and only 2 cases after 1 year. Conclusion: The method of two stents interventional treatment is a safe and useful management for malignant hilar obstructions. (authors)

Stroke and myocardial ischemia in a patient of pheochromocytoma: A rare clinical sequele

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Vivek Aggarwal

2006-01-01

Full Text Available Pheochromocytoma classically presents with paroxysms of hypertension and adrenergic symptoms including headaches, palpitation, tremor and anxiety. However, these tumors can be clinically silent and occasionally manifest only when catecholamine release is upregulated by exogenous stimuli. In addition, the clinical presentation of pheochromocytoma can mimic a number of other medical conditions including migraine, cardiac arrhythmias, myocardial infarction and stroke, thus, making the diagnosis of pheochromocytoma difficult. We present a case of a 43-year old male patient who presented for evaluation of adrenal mass in the department of endocrine surgery. This patient had a previous history of right side hemiperasis and lateral wall ischemia, with no residual clinical deficit.

Endoscopic management of hilar biliary strictures

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Singh, Rajiv Ranjan; Singh, Virendra

2015-01-01

Hilar biliary strictures are caused by various benign and malignant conditions. It is difficult to differentiate benign and malignant strictures. Postcholecystectomy benign biliary strictures are frequently encountered. Endoscopic management of these strictures is challenging. An endoscopic method has been advocated that involves placement of increasing number of stents at regular intervals to resolve the stricture. Malignant hilar strictures are mostly unresectable at the time of diagnosis and only palliation is possible.Endoscopic palliation is preferred over surgery or radiological intervention. Magnetic resonance cholangiopancreaticography is quite important in the management of these strictures. Metal stents are superior to plastic stents. The opinion is divided over the issue of unilateral or bilateral stenting.Minimal contrast or no contrast technique has been advocated during endoscopic retrograde cholangiopancreatography of these patients. The role of intraluminal brachytherapy, intraductal ablation devices, photodynamic therapy, and endoscopic ultrasound still remains to be defined. PMID:26191345

Comprehensive Molecular Characterization of Pheochromocytoma and Paraganglioma

NARCIS (Netherlands)

Fishbein, Lauren; Leshchiner, Ignaty; Walter, Vonn; Danilova, Ludmila; Robertson, A. Gordon; Johnson, Amy R.; Lichtenberg, Tara M.; Murray, Bradley A.; Ghayee, Hans K.; Else, Tobias; Ling, Shiyun; Jefferys, Stuart R.; de Cubas, Aguirre A.; Wenz, Brandon; Korpershoek, Esther; Amelio, Antonio L.; Makowski, Liza; Rathmell, W. Kimryn; Gimenez-Roqueplo, Anne Paule; Giordano, Thomas J.; Asa, Sylvia L.; Tischler, Arthur S.; Akbani, Rehan; Ally, Adrian; Amar, Laurence; Amelio, Antonio L.; Arachchi, Harindra; Asa, Sylvia L.; Auchus, Richard J.; Auman, J. Todd; Baertsch, Robert; Balasundaram, Miruna; Balu, Saianand; Bartsch, Detlef K.; Baudin, Eric; Bauer, Thomas; Beaver, Allison; Benz, Christopher; Beroukhim, Rameen; Beuschlein, Felix; Bodenheimer, Tom; Boice, Lori; Bowen, Jay; Bowlby, Reanne; Brooks, Denise; Carlsen, Rebecca; Carter, Suzie; Cassol, Clarissa A.; Cherniack, Andrew D.; Chin, Lynda; Cho, Juok; Chuah, Eric; Chudamani, Sudha; Cope, Leslie; Crain, Daniel; Curley, Erin; Danilova, Ludmila; de Cubas, Aguirre A.; de Krijger, Ronald R.; Demchok, John A.; Deutschbein, Timo; Dhalla, Noreen; Dimmock, David; Dinjens, Winand N M; Else, Tobias; Eng, Charis; Eschbacher, Jennifer; Fassnacht, Martin; Felau, Ina; Feldman, Michael; Ferguson, Martin L.; Fiddes, Ian; Fishbein, Lauren; Frazer, Scott; Gabriel, Stacey B.; Gardner, Johanna; Gastier-Foster, Julie M.; Gehlenborg, Nils; Gerken, Mark; Getz, Gad; Geurts, Jennifer; Ghayee, Hans K.; Gimenez-Roqueplo, Anne Paule; Giordano, Thomas J.; Goldman, Mary; Graim, Kiley; Gupta, Manaswi; Haan, David; Hahner, Stefanie; Hantel, Constanze; Haussler, David; Hayes, D. Neil; Heiman, David I.; Hoadley, Katherine A.; Holt, Robert A.; Hoyle, Alan P.; Huang, Mei; Hunt, Bryan; Hutter, Carolyn M.; Jefferys, Stuart R.; Johnson, Amy R.; Jones, Steven J M; Jones, Corbin D.; Kasaian, Katayoon; Kebebew, Electron; Kim, Jaegil; Kimes, Patrick; Knijnenburg, Theo; Korpershoek, Esther; Lander, Eric; Lawrence, Michael S.; Lechan, Ronald; Lee, Darlene; Leraas, Kristen M.; Lerario, Antonio; Leshchiner, Ignaty; Lichtenberg, Tara M.; Lin, Pei; Ling, Shiyun; Liu, Jia; LiVolsi, Virginia A.; Lolla, Laxmi; Lotan, Yair; Lu, Yiling; Ma, Yussanne; Maison, Nicole; Makowski, Liza; Mallery, David; Mannelli, Massimo; Marquard, Jessica; Marra, Marco A.; Matthew, Thomas; Mayo, Michael; Méatchi, Tchao; Meng, Shaowu; Merino, Maria J.; Mete, Ozgur; Meyerson, Matthew; Mieczkowski, Piotr A.; Mills, Gordon B.; Moore, Richard A.; Morozova, Olena; Morris, Scott; Mose, Lisle E.; Mungall, Andrew J.; Murray, Bradley A.; Naresh, Rashi; Nathanson, Katherine L.; Newton, Yulia; Ng, Sam; Ni, Ying; Noble, Michael S.; Nwariaku, Fiemu; Pacak, Karel; Parker, Joel S.; Paul, Evan; Penny, Robert; Perou, Charles M.; Perou, Amy H.; Pihl, Todd; Powers, James; Rabaglia, Jennifer; Radenbaugh, Amie; Ramirez, Nilsa C.; Rao, Arjun; Rathmell, W. Kimryn; Riester, Anna; Roach, Jeffrey; Robertson, A. Gordon; Sadeghi, Sara; Saksena, Gordon; Salama, Sofie; Saller, Charles; Sandusky, George; Sbiera, Silviu; Schein, Jacqueline E.; Schumacher, Steven E.; Shelton, Candace; Shelton, Troy; Sheth, Margi; Shi, Yan; Shih, Juliann; Shmulevich, Ilya; Simons, Janae V.; Sipahimalani, Payal; Skelly, Tara; Sofia, Heidi J.; Sokolov, Artem; Soloway, Matthew G.; Sougnez, Carrie; Stuart, Josh; Sun, Charlie; Swatloski, Teresa; Tam, Angela; Tan, Donghui; Tarnuzzer, Roy; Tarvin, Katherine; Thiessen, Nina; Thorne, Leigh B.; Timmers, Henri J.; Tischler, Arthur S.; Tse, Kane; Uzunangelov, Vlado; van Berkel, Anouk; Veluvolu, Umadevi; Vicha, Ales; Voet, Doug; Waldmann, Jens; Walter, Vonn; Wan, Yunhu; Wang, Zhining; Wang, Tracy S.; Weaver, Joellen; Weinstein, John N.; Weismann, Dirk; Wenz, Brandon; Wilkerson, Matthew D.; Wise, Lisa; Wong, Tina; Wong, Christopher; Wu, Ye; Yang, Liming; Zelinka, Tomas; Zenklusen, Jean C.; Zhang, Jiashan (Julia); Zhang, Wei; Zhu, Jingchun; Zinzindohoué, Franck; Zmuda, Erik; Pacak, Karel; Nathanson, Katherine L.; Wilkerson, Matthew D.

2017-01-01

We report a comprehensive molecular characterization of pheochromocytomas and paragangliomas (PCCs/PGLs), a rare tumor type. Multi-platform integration revealed that PCCs/PGLs are driven by diverse alterations affecting multiple genes and pathways. Pathogenic germline mutations occurred in eight

Pure laparoscopic radical resection for type IIIa hilar cholangiocarcinoma.

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Zhang, Cheng-Wu; Liu, Jie; Hong, De-Fei; Wang, Zhi-Fei; Hu, Zhi-Ming; Huang, Dong-Shen; Shang, Min-Jie; Yao, Wei-Feng

2018-03-01

Pure laparoscopic radical resection of hilar cholangiocarcinoma is still a challenging procedure, in which laparoscopic lymphadenectomy, hemihepatectomy with caudate lobectomy, and hepaticojejunostomy were included [1-4]. Relative report is rare in the world up to now. Hilar cholangiocarcinoma has a poor prognosis, especially when it occurs with lymph node metastasis or vessel invasion [5, 6]. We recently had a patient who underwent a pure laparoscopic extended right hepatectomy and lymph node dissection and hepaticojejunostomy for a type IIIa hilar cholangiocarcinoma. The tumor was 20 × 15 × 12 mm in diameter and located in the right bile duct and common hepatic duct. Radiological examination showed that hepatic artery and portal vein was not invaded. After the division and mutilation of the right hepatic artery and the right portal vein, short hepatic veins were divided and cut off with clip and ultrasound knife from the anterior face of the vena cava. Mobilization was performed after the devascularization of the right liver, followed by the transection of liver parenchymal with CUSA and ultrasound knife. Finally, left hepatic bile duct jejunum Roux-en-Y reconstruction was performed. This patient underwent successfully with a totally laparoscopic procedure. An extended right hepatectomy (right hemihepatectomy combined with caudate lobectomy) and complete lymph node dissection and hepaticojejunostomy were performed in this operation. The operation time was nearly 590 min, and the intraoperative blood loss was about 300 ml. No obvious complication was observed and the postoperative hospital stay was 11 days. The final diagnosis of the hilar cholangiocarcinoma with no lymph node metastasis was pT2bN0M0 stage II (American Joint Committee on Cancer, AJCC). Pure laparoscopic resection for hilar cholangiocarcinoma was proved safe and feasible, which enabled the patient to recover early and have an opportunity to receive chemotherapy as soon as possible. We

Pheochromocytoma of the urinary bladder - a case report

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Marić Predrag

2016-01-01

Full Text Available Introduction. Pheochromocytoma of the urinary bladder is a rare tumor and presents less than 0.06% of all urinary bladder tumors. Case report. We presented a 49-year-old female patient with a history of daily paroxysmal hypertension accompanied with flushing of the face and upper chest, palpitations and excessive sweating prior to micturition. Ultrasonography reported a 3 cm bladder wall tumor. The 131I-metaiodobenzylguanidine (131I-MIBG scan showed a pathological isotope accumulation in the projection of the bladder. The patient underwent a partial cystectomy. One year following the operation the patient was normotensive and without recurrence. Conclusion. The most efficient treatment option for bladder pheochromocytoma is surgical resection. The most important fact in the diagnostics is suspicion on this rare condition.

Pheochromocytomas and paragangliomas in humans and dogs

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Galac, S.; Korpershoek, E

2017-01-01

Pheochromocytomas (PCCs) and paragangliomas (PGLs) are described in several species. In humans and dogs they have many similarities: the excessive catecholamine release in hormonally active PCC causes similar clinical signs, the frequency of metastasis is similar, and they are histopathologically

Effects of partial portal vein arterialization on the hilar bile duct in a rat model.

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Guo, Shao-Hua; Li, Chong-Hui; Chen, Yong-Liang; Song, Jian-Ning; Zhang, Ai-Qun; Zhou, Cheng

2011-10-01

Liver revascularization is frequently required during the enlarged radical operation for hilar cholangiocarcinoma involving the hepatic artery. Researchers have carried out a number of experiments applying partial portal vein arterialization (PVA) in clinical practice. In this study we aimed to establish a theoretical basis for clinical application of partial PVA and to investigate the effects of partial PVA on rat hilar bile duct and hepatic functions. Thirty rats were randomly and equally assigned into 3 groups: control (group A), hepatic artery ligation+bile duct recanalization (group B), and partial PVA+bile duct recanalization (group C). Proliferation and apoptosis of rat hilar bile duct epithelial cells, arteriolar counts of the peribiliary plexus (PBP) of the bile duct wall, changes in serum biochemistry, and pathologic changes in the bile duct were assessed 1 month after operation. The proliferation of hilar bile duct epithelial cells in group B was greater than in groups A and C (Philar bile duct epithelial cells were detected in any of the groups. The PBP arteriolar counts of the hilar bile duct wall were similar in groups A and C (P>0.05), but the count was lower in group B than in group A (Philar bile duct walls were observed only in group B. Partial PVA can restore the arterial blood supply of the hilar bile duct and significantly extenuate the injury to hilar bile duct epithelial cells resulting from hepatic artery ligation.

Extent of liver resection for hilar cholangiocarcinoma (Klatskin tumor): how much is enough?

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van Gulik, Thomas M.; Ruys, Anthony T.; Busch, Oliver R. C.; Rauws, Erik A. J.; Gouma, Dirk J.

2011-01-01

Hilar resection in combination with extended liver resections has resulted in a higher rate of R0 resections and increased survival in patients with hilar cholangiocarcinoma (HCCA). This aggressive surgical approach is, however, associated with high rates of operative morbidity and mortality,

Implications and considerations during pheochromocytoma resection: A challenge to the anesthesiologist

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Sukhminder Jit Singh Bajwa

2011-01-01

Full Text Available Pheochromocytoma is a rare catecholamine secreting tumor arising commonly from adrenal medulla. It has got multidimensional challenging aspects in spite of our improved understanding of its physiological and clinical behavior during surgical resection. This neuroendocrine tumor is associated with a most unpredictable and fluctuating clinical course during anesthesia and surgical intervention. The clinical difficulties and challenges increase manifold in patients with undiagnosed or accidental diagnosis of pheochromocytoma who present to the hospital for the treatment of some other disease or emergency. The most common manifestations of this clinical spectrum include hypertension, headache, palpitations, episodic sweating, and feeling of doom. The definite and only treatment for this rare tumor is surgical resection which itself is very challenging for an anesthesiologist. This article reviews the pre-operative evaluation, pharmacological preparation, intraoperative and post-operative management of patients with pheochromocytoma especially from anesthesiologist′s perspectives.

Percutaneous transhepatic biliary drainage for hilar cholangiocarcinoma

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Qian Xiaojun; Jin Wenhui; Dai Dingke; Yu Ping; Gao Kun; Zhai Renyou

2007-01-01

Objective: To evaluate the effect of PTBD in treating malignant biliary obstruction caused by hilar cholangiocarcinoma. Methods: We retrospectively analyzed the data of 103 patients(M:62,F:41)with malignant obstructive jaundice caused by hilar cholangiocarcinoma. After taking percutaneous transhepatic cholangiography, metallic stent or plastic external catheter or external-internal catheter for drainage was deployed and then followed up was undertaken with clinical and radiographic evaluation and laboratory. examination. Results: All patients went though PTBD successfully (100%). According to Bismuth classification, all 103 cases consisted of I type(N=30), II type (N=30), III type (N=26) and IV type (N=17). Thirty-nine cases were placed with 47 stents and 64 eases with drainage tubes. 4 cases installed two stems for bilateral drainage, 2 cases installed two stents because of long segmental strictures with stent in stent, 1 case was placed with three stents, and 3 cases installed stent and plastic catheter together. Sixty-four cases received plastic catheters in this series, 35 cases installed two or more catheters for bilateral drainage, 28 cases installed external and internal drainage catheters, 12 eases installed external drainage catheters, and 24 eases installed both of them. There were 17 patients involving incorporative infection before procedure, 13 cases cured after procedure, and 15 new patients got inflammation after procedure. 13 cases showed increase of amylase (from May, 2004), 8 eases had bloody bile drainage and 1 case with pyloric obstruction. Total serum bilirubin reduced from (386 ± 162) μmol/L to (161 ± 117) μmol/L, (P<0.01) short term curative effect was related with the type of hilar cholangiocarcinoma. The survival time was 186 days(median), and 1, 3, 6, 12 month survival rate were 89.9%, 75.3%, 59.6%, 16.9%, respectively. Conclusion: Percutaneous transhepatic bile drainage is a safe and effective palliative therapy of malignant

Anesthesia Management for Pheochromocytoma Removal in a 17-Year-Old Girl: Case Report

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Gulsen Keskin

2018-03-01

Full Text Available A rare tumor of the chromaffin tissue, pheochromocytoma is characterized by increased secretion of catecholamines. Pediatric cases represent only 5% of all pheochromocytomas. In this report, we presented anesthesia management of a 17-year-old girl who would undergo right suprarenal mass excision due to pheochromocytoma accompanied with familial Mediterranean fever (FMF. Preoperative blood pressure control was achieved with phenoxybenzamine. General anesthesia was established with thiopental, vecuronium, fentanyl, and sevoflurane. Sodium nitroprusside and phentolamine was used for perioperative blood pressure control. Tramadol and diclofenac were administered for postoperative pain. The patient was discharged after a good preoperative preparation and perioperative management with uneventful treatment period. [J Contemp Med 2018; 8(1.000: 70-73

Iodine-131 MIBG scintigraphy of the extremities in metastatic pheochromocytoma and neuroblastoma

International Nuclear Information System (INIS)

Shulkin, B.L.; Shen, S.W.; Sisson, J.C.; Shapiro, B.

1987-01-01

Iodine-131 MIBG scintigraphy may be used to determine the presence or absence of metastases to the appendicular skeleton in malignant pheochromocytoma and neuroblastoma. Normal bones show no uptake of [ 131 I]MIBG and the joints are seen as photon-deficient areas surrounded by background muscle activity. Discrete concentrations of radioactivity in bone are often seen in patients with malignant pheochromocytoma and neuroblastoma. Bone marrow involvement in neuroblastoma may be indicated by diffuse uptake of [ 131 I]MIBG or focal accumulation at the metaphyses. Uncommonly, bone involvement may not be displayed by the [ 131 I]MIBG images. Since conventional bone scanning agents may also fail to detect these tumors, skeletal scintigraphy with both [ 131 I]MIBG and [/sup 99m/Tc]MDP is necessary to reliably stage malignant pheochromocytoma and neuroblastoma

Pulmonary hilar lymph nodes in lung cancer: assessment with 3D-dynamic contrast-enhanced MR imaging

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Hasegawa, Ichiro; Eguchi, Keisuke; Kohda, Ehiichi; Tanami, Yutaka; Mori, Toru; Hatabu, Hiroto; Kuribayashi, Sachio

2003-01-01

Purpose: We performed 3D-dynamic MRI on patients with primary lung cancer to identify its usefulness for detecting hilar adenopathy shown at surgery. Methods and materials: 30 consecutive patients with peripheral lung cancer underwent preoperative 3D-dynamic Gd-DTPA-enhanced MRI. Two thoracic radiologists blinded to histopathologic findings reviewed those studies independently for hilar adenopathy visualization. The results were correlated with surgical and histopathologic findings. Interreader agreement for the detection of hilar adenopathy was assessed by means of the κ statistic. Results: Dynamic MRI demonstrated hilar adenopathy, with or without metastasis revealed at surgery, in all of 15 patients. Adenopathy without metastasis was shown in four patients. Dynamic MRI also revealed metastatic adenopathy in 11 of 12 patients with pathologically proven metastasis. There was only one case with lymph node metastasis that did not have adenopathy either on MRI or even at surgery. The diagnostic accuracy of dynamic MRI for adenopathy with or without metastases revealed at surgery were as follows; sensitivity, 100%; specificity, 100%; positive predictive value, 100%; and negative predictive value, 100%, respectively. The diagnostic accuracy of dynamic MRI for hilar lymph nodes metastasis were as follows; sensitivity, 92%; specificity, 78%; positive predictive value, 73%; and negative predictive value, 93%. Interreader agreement was substantial (κ=0.73) for detection of hilar adenopathy. Conclusion: Hilar adenopathy on 3D-dynamic MRI correlated well with that of surgical finding on patients with primary lung cancer. It may have the potential to make an accurate preoperative evaluation of hilar lymph node metastasis from lung cancer

Interleukin-8 is a prognostic indicator in human hilar cholangiocarcinoma

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Sun, Qi; Li, Fanni; Sun, Fengkai; Niu, Jun

2015-01-01

Interleukin-8 (IL-8), matrix metalloproteinase-9 (MMP-9) and neovascularization have been implicated to be associated with biological processes, especially cancer progression. However, few studies have investigated the role of IL-8 in human hilar cholangiocarcinoma. In this study we detected the expression of IL-8 combined with MMP-9 and microvessel density (MVD) in hilar cholangiocarcinoma to evaluate their clinicopathological significance and prognostic value. A total of 62 patients with hilar cholangiocarcinoma who underwent curative surgery were enrolled in this study. The expression of IL-8, MMP-9 and MVD were examined immunohistochemically. The correlation of IL-8 with MMP-9 expression, MVD, clinicopathological features and survival time of patients were then analyzed. Expression of IL-8 was observed in 56.5% tumors, which was related to advanced TNM stage (P = 0.026) and tumor recurrence (P = 0.018). IL-8 had a positive correlation with MMP-9 expression and MVD. Furthermore, patients with high IL-8 expression had a significantly shorter overall survival than those with low IL-8 expression (P = 0.01). Multivariate analysis confirmed IL-8 as an independent prognostic factor (P = 0.005). In conclusion, IL-8 expression significantly correlated with MMP-9 expression and MVD, and IL-8 was a valuable prognostic factor for human hilar cholangiocarcinoma. PMID:26339407

Course of chronic hilar sarcoidosis in relation to markers of granulomatous activity

International Nuclear Information System (INIS)

Israel, H.L.; Sperber, M.; Steiner, R.M.

1983-01-01

Studies of the course of sarcoidosis have emphasized that patients with hilar or mediastinal adenopathy usually recover within several years or develop dissemination to the lungs. Chronic hilar and mediastinal adenopathy persisting with little or no change for many decades is an important subgroup that has not received adequate attention. Twelve such patients have been studied. Seven remained asymptomatic, despite persistent adenopathy, for a mean period of 16 years; two with disfiguring facial sarcoids received corticosteroids for 18 and 27 years, respectively, and three patients after ten years of stable adenopathy developed pulmonary infiltrates. Tests performed on patients with hilar adenopathy to evaluate cellular activity after a mean interval of over 16 years included Kveim reaction (positive in nine of ten), serum angiotensin converting enzyme (elevated in eight of 12), and gallium-67 scanning (hilar uptake in all eight tested). Results were similar for patients who remained well and for those who had symptomatic or progressive disease, indicating that these parameters of granulomatous activity do not reflect the duration of the disease, its outcome, or the need for treatment

Overexpression of Prdx1 in hilar cholangiocarcinoma: a predictor for recurrence and prognosis.

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Zhou, Jie; Shen, Weiwen; He, Xiaojing; Qian, Jing; Liu, Shiyuan; Yu, Guanzhen

2015-01-01

Prdx1 is an important member of peroxiredoxins (Prdxs) regulating various cellular signaling and differentiation. Prdx1 confers an aggressive survival phenotype of cancer cells and drug-resistance, yet its role in hilar cholangiocarcinoma is not fully investigated. In present study, we detected the expression profile of Prdx1 in 88 hilar cholangiocarcinoma by tissue arrays and immunohistochemistry. Prdx1 level was down-regulated by specific Prdx1-shRNA in vitro and the possible mechanism was investigated. Overexpression of Prdx1 was observed in 53 of 88 cases (60.2%). Prdx1 expression was significantly associated with tumor invasion, nodal metastasis, advanced disease stage. Down-regulation of Prdx1 inhibited cell proliferation and colony formation of QBC939 cells and reduced the level of SNAT1 expression. Patients with Prdx1 overexpression had a shorter disease-free survival and overall survival than those without Prdx1 expression. Multivariate analysis showed that Prdx1 was an independent prognostic factor for patients with hilar cholangiocarcinoma. The data indicate that Prdx1 may contribute to the development and progression of hilar cholangiocarcinoma, partially through regulating SNAT1 expression, and may be used as a biomarker in predicting the outcome of patients with hilar cholangiocarcinoma.

Local absolute alcohol ablation for the treatment of recurrent pheochromocytoma

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Shang Mingyi; Wang Peijun; Lu Ying; Ma Jun; Tang Junjun; Xi Qian; Huang Zongliang; Gao Xiaolong

2010-01-01

Objective: to assess the clinical value of local injection of absolute alcohol under CT guidance in treating recurrent pheochromocytoma. Methods: Five patients with benign recurrent pheochromocytoma were enrolled in this study. Of the five cases, the lesions were located on the right side in three, on the left in one and on both sides in one. All the lesions were pathologically proved to be benign ones. Under CT guidance the ablation therapy with local injection of absolute alcohol was performed. The therapeutic results were observed and evaluated. Results: Thirty days after the treatment, different degrees of decrease in tumor size was observed on follow-up CT scans. All the patients were followed up for 9-42 months. During the follow-up period, both the blood pressure and the vanillyl mandelic acid (VMA) level in urine remained normal and no paroxysmal dizziness, headache or syncope occurred in all patients. Conclusion: For the treatment of recurrent pheochromocytoma the ablation therapy by using local injection of absolute alcohol under CT guidance is a safe and practical therapeutic means with definite and reliable effectiveness. (authors)

Antinociceptive effect of intrathecal microencapsulated human pheochromocytoma cell in a rat model of bone cancer pain.

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Li, Xiao; Li, Guoqi; Wu, Shaoling; Zhang, Baiyu; Wan, Qing; Yu, Ding; Zhou, Ruijun; Ma, Chao

2014-07-08

Human pheochromocytoma cells, which are demonstrated to contain and release met-enkephalin and norepinephrine, may be a promising resource for cell therapy in cancer-induced intractable pain. Intrathecal injection of alginate-poly (l) lysine-alginate (APA) microencapsulated human pheochromocytoma cells leads to antinociceptive effect in a rat model of bone cancer pain, and this effect was blocked by opioid antagonist naloxone and alpha 2-adrenergic antagonist rauwolscine. Neurochemical changes of cerebrospinal fluid are in accordance with the analgesic responses. Taken together, these data support that human pheochromocytoma cell implant-induced antinociception was mediated by met-enkephalin and norepinephrine secreted from the cell implants and acting at spinal receptors. Spinal implantation of microencapsulated human pheochromocytoma cells may provide an alternative approach for the therapy of chronic intractable pain.

Neuron-specific enolase is a useful maker of neuroendocrine origin in pheochromocytoma cell culture

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Abelin, N.; Dahia, P.L.M.; Martin, R.; Kato, S.; Toledo, S.P.A.

1994-01-01

Neuron-specific enolase (NSE) has been used as a marker for neuroendocrine tumors either in immunocytochemical studies or in serum measurements. In this paper NSE levels were determined in cultured pheochromocytoma cells to test whether it is also a useful marker in cell culture of tumors derived from neuroendocrine system. Cultured pheochromocytoma cells came from a primary explant and were grown in RPMI supplemented with 20% fetal calf serum, 100 μg/mL ampicillin and 100 μ/mL streptomycin. NSE was measured in culture medium and cell homogenates. Samples from different pheochromocytoma cultures were analyzed and compared to normal cultured fibroblast cells derived from human skin. NSE was measured by a commercially available radioimmunoassay kit. NSE levels were higher in cell homogenates as compared to those in culture medium, reaching levels as high as 6-fold in the former in TE cell line (26.46 ng/mL and 4.39 ng/mL, respectively). Serial measurements in culture medium from TE cell line evidenced decreasing values in subsequential subcultures (from 9.24 ng/mL during primary explant to 1.7 ng/mL in the tenth subculture). In cultured normal fibroblasts, NSE levels in cultured media were definitely lower than those obtained from pheochromocytoma cultures. These preliminary data suggest that NSE may be a useful marker of neuroendocrine derived tumors, such as pheochromocytoma, in culture. Thus, the simplicity and availability of NSE radioimmunoassay provides an alternative to catecholamine measurement to better characterize pheochromocytoma cell lines in culture, with the advantage of faster result at lower costs. (author). 18 refs, 2 tabs

Elevated AQP1 Expression Is Associated With Unfavorable Oncologic Outcome in Patients With Hilar Cholangiocarcinoma.

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Li, Chunxiang; Li, Xiaofu; Wu, Linfeng; Jiang, Zheng

2017-08-01

Hilar cholangiocarcinomas are malignant tumors with a poor prognosis. An early prediction of prognosis for patients may help us determine treatment strategies. Aquaporin 1 is a cell membrane channel involved in water transport, cell motility, and proliferation. Increasing evidences showed that aquaporin 1 played a role in tumor prognosis and diagnosis. The purpose of this study is to evaluate the role of aquaporin 1 in hilar cholangiocarcinoma. Here, we analyzed messenger RNA expression data of genes function as bile secretion in a data set of 169 samples using the R2 bioinformatic platform ( http://r2.amc.nl ). Quantitative polymerase chain reaction was performed to verify the gene expression in 17 hilar cholangiocarcinoma samples. Immunohistochemistry was also performed in a series of specimens from 62 hilar cholangiocarcinoma tissues, and its clinical significance was assessed by clinical correlation and Kaplan-Meier analyses. All data were analyzed using the R2 web application, aquaporin 1 was selected for further analysis. The significant expression variation of aquaporin 1 among 17 cases with cholangiocarcinoma was also found using quantitative polymerase chain reaction. The expression level of aquaporin 1 protein significantly correlated with tumor-node-metastasis stage ( P = .002) and overall survival time ( P = .010). Higher aquaporin 1 expression indicated poor prognostic outcomes ( P hilar cholangiocarcinoma ( P = .002). This study highlighted the prognostic value of aquaporin 1 in hilar cholangiocarcinoma. Strong aquaporin 1 expression predicts poor survival, regardless of pathological features. Immunohistochemical detection of aquaporin 1, as a prognostic marker, may contribute to predicting clinical outcome for patients with hilar cholangiocarcinoma.

Palliation: Hilar cholangiocarcinoma

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Goenka, Mahesh Kr; Goenka, Usha

2014-01-01

Hilar cholangiocarcinomas are common tumors of the bile duct that are often unresectable at presentation. Palliation, therefore, remains the goal in the majority of these patients. Palliative treatment is particularly indicated in the presence of cholangitis and pruritus but is often also offered for high-grade jaundice and abdominal pain. Endoscopic drainage by placing stents at endoscopic retrograde cholangio-pancreatography (ERCP) is usually the preferred modality of palliation. However, for advanced disease, percutaneous stenting has been shown to be superior to endoscopic stenting. Endosonography-guided biliary drainage is emerging as an alternative technique, particularly when ERCP is not possible or fails. Metal stents are usually preferred over plastic stents, both for ERCP and for percutaneous biliary drainage. There is no consensus as to whether it is necessary to place multiple stents within advanced hilar blocks or whether unilateral stenting would suffice. However, recent data have suggested that, contrary to previous belief, it is useful to drain more than 50% of the liver volume for favorable long-term results. In the presence of cholangitis, it is beneficial to drain all of the obstructed biliary segments. Surgical bypass plays a limited role in palliation and is offered primarily as a segment III bypass if, during a laparotomy for resection, the tumor is found to be unresectable. Photodynamic therapy and, more recently, radiofrequency ablation have been used as adjuvant therapies to improve the results of biliary stenting. The exact technique to be used for palliation is guided by the extent of the biliary involvement (Bismuth class) and the availability of local expertise. PMID:25232449

Adrenal incidentaloma: A case of pheochromocytoma with sub-clinical Cushing′s syndrome

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Ashutosh Goyal

2013-01-01

Full Text Available Adrenal incidentalomas (AIs are a cluster of different pathologies, but AIs with dual functional aspects are very rare. We report a case of AI with the evidence of both pheochromocytoma and sub-clinical Cushing′s syndrome. A 42-year-old female patient presented with the history of abdominal pain. Abdominal computed tomography revealed right adrenal mass suggestive of pheochromocytoma. On endocrine evaluation, she admitted history of intermittent headache and palpitations for 4 years and was on treatment for hypertension and diabetes. There were no signs and symptoms suggestive of Cushing′s syndrome. The laboratory data demonstrated 10 times raised 24-h urinary fractionated metanephrines with non-suppressible serum cortisol after 2-day low-dose dexamethasone suppression test. She underwent right-sided adrenalectomy with subsequent resolution of both pheochromocytoma and hypercortisolism. Patient was discharged in good clinical condition.

Use of an autologous liver round ligament flap zeros postoperative bile leak after curative resection of hilar cholangiocarcinoma.

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Sun, Da-Xin; Tan, Xiao-Dong; Gao, Feng; Xu, Jin; Cui, Dong-Xu; Dai, Xian-Wei

2015-01-01

Postoperative bile leak is a major surgical morbidity after curative resection with hepaticojejunostomy for hilar cholangiocarcinoma, especially in Bismuth-Corlette types III and IV. This retrospective study assessed the effectiveness and safety of an autologous hepatic round ligament flap (AHRLF) for reducing bile leak after hilar hepaticojejunostomy. Nine type III and IV hilar cholangiocarcinoma patients were consecutively hospitalized for elective perihilar partial hepatectomy with hilar hepaticojejunostomy using an AHRLF between October 2009 and September 2013. The AHRLF was harvested to reinforce the perihilar hepaticojejunostomy. Main outcome measures included operative time, blood loss, postoperative recovery times, morbidity, bile leak, R0 resection rate, and overall survival. All patients underwent uneventful R0 resection with hilar hepaticojejunostomy. No patient experienced postoperative bile leak. The AHRLF was associated with lack of bile leak after curative perihilar hepatectomy with hepaticojejunostomy for hilar cholangiocarcinoma, without compromising oncologic safety, and is recommended in selected patients.

Antinociceptive Effect of Intrathecal Microencapsulated Human Pheochromocytoma Cell in a Rat Model of Bone Cancer Pain

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Xiao Li

2014-07-01

Full Text Available Human pheochromocytoma cells, which are demonstrated to contain and release met-enkephalin and norepinephrine, may be a promising resource for cell therapy in cancer-induced intractable pain. Intrathecal injection of alginate-poly (l lysine-alginate (APA microencapsulated human pheochromocytoma cells leads to antinociceptive effect in a rat model of bone cancer pain, and this effect was blocked by opioid antagonist naloxone and alpha 2-adrenergic antagonist rauwolscine. Neurochemical changes of cerebrospinal fluid are in accordance with the analgesic responses. Taken together, these data support that human pheochromocytoma cell implant-induced antinociception was mediated by met-enkephalin and norepinephrine secreted from the cell implants and acting at spinal receptors. Spinal implantation of microencapsulated human pheochromocytoma cells may provide an alternative approach for the therapy of chronic intractable pain.

Usefulness of thin slice target CT scan in detecting mediastinal and hilar lymphadenopathy

International Nuclear Information System (INIS)

Yoshida, Shoji; Maeda, Tomoho; Nishioka, Masatoshi

1986-01-01

Comparative study of target scan with the different slice thickness and scan modes was performed to evaluate the mediastinal and hilar lymphadenopathy. 20 cases in controls and 35 cases in lymphadenopathy were examined. To delineate mediastinal and hilar lymphadenopathy, the scan mode of standard target was most useful in contrast and sharpness. Thin slice thickness with 5 mm was necessary in detecting small lymphnode or contour and internal structure of enlarged lymphnode. Valuable estimation of 5 mm contiguous target scan was obtained in the subaortic node (no. 5), tracheobronchial node (no. 4), precarinal and subcarinal node (no. 7) and right hilar node (no. 12). (author)

Pheochromocytomas and secreting paragangliomas

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Gimenez-Roqueplo Anne-Paule

2006-12-01

Full Text Available Abstract Catecholamine-producing tumors may arise in the adrenal medulla (pheochromocytomas or in extraadrenal chromaffin cells (secreting paragangliomas. Their prevalence is about 0.1% in patients with hypertension and 4% in patients with a fortuitously discovered adrenal mass. An increase in the production of catecholamines causes symptoms (mainly headaches, palpitations and excess sweating and signs (mainly hypertension, weight loss and diabetes reflecting the effects of epinephrine and norepinephrine on α- and β-adrenergic receptors. Catecholamine-producing tumors mimic paroxysmal conditions with hypertension and/or cardiac rhythm disorders, including panic attacks, in which sympathetic activation linked to anxiety reproduces the same signs and symptoms. These tumors may be sporadic or part of any of several genetic diseases: familial pheochromocytoma-paraganglioma syndromes, multiple endocrine neoplasia type 2, neurofibromatosis 1 and von Hippel-Lindau disease. Familial cases are diagnosed earlier and are more frequently bilateral and recurring than sporadic cases. The most specific and sensitive diagnostic test for the tumor is the determination of plasma or urinary metanephrines. The tumor can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy. Treatment requires resection of the tumor, generally by laparoscopic surgery. About 10% of tumors are malignant either at first operation or during follow-up, malignancy being diagnosed by the presence of lymph node, visceral or bone metastases. Recurrences and malignancy are more frequent in cases with large or extraadrenal tumors. Patients, especially those with familial or extraadrenal tumors, should be followed-up indefinitely.

Stereotactic body radiation therapy for isolated hilar and mediastinal non-small cell lung cancers.

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Horne, Zachary D; Richman, Adam H; Dohopolski, Michael J; Clump, David A; Burton, Steven A; Heron, Dwight E

2018-01-01

The seminal phase II trial for pulmonary stereotactic body radiation therapy (SBRT) suggested that SBRT to central lesions resulted in unacceptable toxicity. Alternative dose-fractionation schemes have been proposed which may improve safety without compromise of efficacy. We report our institutional outcomes of SBRT for hilar/mediastinal non-small cell lung cancer (NSCLC). A retrospective review was conducted of patients with NSCLC in a hilar or mediastinal nodal station which was treated with SBRT. Patients presented with a lesion involving the hilum or mediastinum from primary or oligorecurrent NSCLC. Kaplan-Meier with log-rank testing and Cox analysis were utilized for outcomes analysis. From 2008-2015, 40 patients with median age of 70 were treated with SBRT for primary/oligorecurrent hilar/mediastinal NSCLC with median follow-up of 16.4 months. 85% presented with oligorecurrent disease at a median of 22.4 months following definitive therapy. The aortico-pulmonary window was the target in 40%, the hilum in 25%, lower paratracheal in 20%, subcarinal in 10%, and prevascular in 5%. The median dose was 48Gy in 4 fractions (range: 35-48Gy in 4-5 fractions). Median overall (OS) and progression-free (PFS) survivals were 22.7 and 13.1 months, respectively. Two-year local control was 87.7% and not significantly different between hilar and mediastinal targets. Median PFS was significantly improved in patients with hilar vs mediastinal nodal targets: 33.3 vs 8.4 months, respectively (p=0.031). OS was not statistically different between hilar and mediastinal targets (p=0.359). On multivariable analysis, hilar vs mediastinal target predicted for PFS (HR 3.045 95%CI [1.044-8.833], p=0.042), as did shorter time to presentation in patients with oligorecurrence (HR 0.983 [95%CI 0.967-1.000], p=0.049). Acute grade 3+ morbidity was seen in 3 patients (hemoptysis, pericardial/pleural effusion, heart failure) and late grade 3+ morbidity (hemoptysis) in 1 patient. Hilar

Hilar mossy cell circuitry controlling dentate granule cell excitability

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Seiichiro eJinde

2013-02-01

Full Text Available Glutamatergic hilar mossy cells of the dentate gyrus can either excite or inhibit distant granule cells, depending on whether their direct excitatory projections to granule cells or their projections to local inhibitory interneurons dominate. However, it remains controversial whether the net effect of mossy cell loss is granule cell excitation or inhibition. Clarifying this controversy has particular relevance to temporal lobe epilepsy, which is marked by dentate granule cell hyperexcitability and extensive loss of dentate hilar mossy cells. Two diametrically opposed hypotheses have been advanced to explain this granule cell hyperexcitability – the dormant basket cell and the irritable mossy cell hypotheses. The dormant basket cell hypothesis proposes that mossy cells normally exert a net inhibitory effect on granule cells and therefore their loss causes dentate granule cell hyperexcitability. The irritable mossy cell hypothesis takes the opposite view that mossy cells normally excite granule cells and that the surviving mossy cells in epilepsy increase their activity, causing granule cell excitation. The inability to eliminate mossy cells selectively has made it difficult to test these two opposing hypotheses. To this end, we developed a transgenic toxin-mediated, mossy cell-ablation mouse line. Using these mutants, we demonstrated that the extensive elimination of hilar mossy cells causes granule cell hyperexcitability, although the mossy cell loss observed appeared insufficient to cause clinical epilepsy. In this review, we focus on this topic and also suggest that different interneuron populations may mediate mossy cell-induced translamellar lateral inhibition and intralamellar recurrent inhibition. These unique local circuits in the dentate hilar region may be centrally involved in the functional organization of the dentate gyrus.

Right hilar mass with hemoptysis: An unusual presentation of uncommon disorder

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Asmita A Mehta

2011-01-01

Full Text Available Common differential diagnosis of lung and hilar opacity includes infectious pathology or a mitotic lesion. Behcet′s disease (BD is a rarely diagnosed disease in Indian subcontinent. BD is a multisystem inflammatory disorder that presents with recurrent orogenital ulceration, uveitis, and erythema nodosum. We present here the case of a patient who presented with recurrent hemoptysis with radiological picture of hilar mass, during the evaluation of which the diagnosis of BD was established.

No loss of hippocampal hilar somatostatinergic neurons after repeated electroconvulsive shock

DEFF Research Database (Denmark)

Dalby, Nils Ole; Tønder, N; Wolby, D P

1996-01-01

Electrically induced seizures with anesthesia and muscle relaxation (ECT) is commonly used in the therapy of psychotic depression in humans. Unmodified electroshock (ECS) is used as a model for epilepsy in the rat. In several seizure models of epilepsy, in particular the dentate hilar somatostatin......-containing (SSergic) neurons have been found to undergo degeneration. To assess the potential loss of SSergic hilar neurons after repeated ECS, 10 rats were given 110 ECS, one per day, 5 days a week. One day after the last ECS the rats were anesthesized, perfused, the brains cut on a vibratome and prepared...... for nonradioactive in situ hybridization for somatostatin along with five control rats. Like rats given 10-36 ECS in earlier studies, the ECS-treated rats displayed a markedly increased neuronal hybridization labeling when compared with control rats. The total number of dentate hilar SSergic neurons of each rat...

Scintigraphic localization of pheochromocytomas using 131 I-meta-iodobenzylguanidine. Comparison with computerized tomography

International Nuclear Information System (INIS)

Charbonnel, B.; Coornaert, S.; Tellier, J.L.; Peltier, P.; Chatal, J.F.

1984-01-01

Meta-iodobenzylguanidine was synthesized, radiolabelled with I 131 or I 123 and injected to 28 controls and 7 patients totalizing 13 foci of pheochromocytoma. The tumour was located in one adrenal gland in 3 cases, in both adrenal glands in 1 case, and between the aorta and the vena cava in 1 case; 2 were malignant with metastases. Scintigraphy was negative in all controls, whereas all pheochromocytomas were clearly demonstrated 24 h after injection, except one regarded as non functional due to necrosis. By comparison, CT readily showed the tumour in 7 cases, showed it only thanks to scintigraphic guidance in 4 cases and failed in 2 cases. It is concluded that scintigraphy with meta-iodo-benzylguanidine provides a safe and reliable means of locating a wide range of pheochromocytomas [fr

Institutional Experience in the Management of Hilar Liver Obstruction- A Series of 13 Cases.

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Pathanki, Adithya Malolan; Naragund, Adithya V; Mahadevappa, Basant

2016-12-01

Neoplastic hilar obstruction to the liver outflow presents a unique challenge to the surgeon, wherein, the balance between a curative and possibly larger resection has to be achieved against a more conservative local resection. These are often technically demanding and have thus, far produced equivocal outcomes on both ends. The present case series is on 13 patients who presented with hilar obstruction. They all underwent resections with possible curative intent. The focus of our review is on the technical nuances and the strategies we used, intra- and peri-operatively to make resections possible in these patients, who at first look were deemed inoperable. Among the 13, 10 had hilar cholangiocarcinoma (CCA) while the others had a more benign diagnosis e.g., Hydatid disease. We did not encounter any peri-operative mortality in our series. Two of our patients had to be re-explored for intra-abdominal complications. Among the 13, we encountered two deaths. The rest of the patients are still on follow-up as of April 2016. Hilar CCA continue to be rare and challenging tumours for the Hepato Pancreato Biliary (HPB) surgeon to manage. Outlooks are currently changing as we try to resect bigger and more complicated hilar liver tumours with better results.

Downregulation of the expression of HDGF attenuates malignant biological behaviors of hilar cholangiocarcinoma cells.

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Liu, Yanfeng; Sun, Jingxian; Yang, Guangyun; Liu, Zhaojian; Guo, Sen; Zhao, Rui; Xu, Kesen; Wu, Xiaopeng; Zhang, Zhaoyang

2015-09-01

Hepatoma-derived growth factor (HDGF) has been reported to be a potential predictive and prognostic marker for several types of cancer and important in malignant biological behaviors. However, its role in human hilar cholangiocarcinoma remains to be elucidated. Our previous study demonstrated that high expression levels of HDGF in hilar cholangiocarcinoma tissues correlates with tumor progression and patient outcome. The present study aimed to elucidate the detailed functions of the HDGF protein. This was performed by downregulating the protein expression of HDGF in the FRH0201 hilar cholangiocarcinoma cell line by RNA interference (RNAi) in vitro, and revealed that downregulation of the HDGF protein significantly inhibited the malignant biological behavior of the FRH0201 cells. In addition, further investigation revealed that downregulation of the protein expression of HDGF significantly decreased the secretion of vascular endothelial growth factor, which may be the mechanism partially responsible for the inhibition of malignant biological behaviors. These findings demonstrated that HDGF is important in promoting malignant biological behaviors, including proliferation, migration and invasion of hilar cholangiocarcinoma FRH0201 cells. Inhibition of the expression of HDGF downregulated the malignant biological behaviors, suggesting that downregulation of the protein expression of HDGF by RNAi may be a novel therapeutic approach to inhibit the progression of hilar cholangiocarcinoma.

Normal mediastinal and hilar lymph nodes evaluated by 5 mm slice bolus injection CT scan

International Nuclear Information System (INIS)

Yamamoto, Takako; Tsukada, Hiroshi; Koizumi, Naoya; Akita, Shinichi; Oda, Junichi; Sakai, Kunio

1995-01-01

We evaluated the number and size of normal mediastinal and hilar lymph nodes by 5 mm slice bolus injection CT (12 patients), compared with 10 mm slice CT (12 patients). More lymph nodes were clearly demonstrated by 5 mm slice CT than by 10 mm slice CT. Especially left-sided tracheobronchial (no.4), subaortic (no.5), subcarinal (no.7) and hilar lymph nodes were clearly visible. We concluded 5 mm slice bolus injection CT was useful to evaluate mediastinal and hilar lymph nodes. (author)

Prognostic significance of macrophage invasion in hilar cholangiocarcinoma

International Nuclear Information System (INIS)

Atanasov, Georgi; Hau, Hans-Michael; Dietel, Corinna; Benzing, Christian; Krenzien, Felix; Brandl, Andreas; Wiltberger, Georg; Matia, Ivan; Prager, Isabel; Schierle, Katrin; Robson, Simon C.; Reutzel-Selke, Anja; Pratschke, Johann; Schmelzle, Moritz; Jonas, Sven

2015-01-01

Tumor-associated macrophages (TAMs) promote tumor progression and have an effect on survival in human cancer. However, little is known regarding their influence on tumor progression and prognosis in human hilar cholangiocarcinoma. We analyzed surgically resected tumor specimens of hilar cholangiocarcinoma (n = 47) for distribution and localization of TAMs, as defined by expression of CD68. Abundance of TAMs was correlated with clinicopathologic characteristics, tumor recurrence and patients’ survival. Statistical analysis was performed using SPSS software. Patients with high density of TAMs in tumor invasive front (TIF) showed significantly higher local and overall tumor recurrence (both ρ < 0.05). Furthermore, high density of TAMs was associated with decreased overall (one-year 83.6 % vs. 75.1 %; three-year 61.3 % vs. 42.4 %; both ρ < 0.05) and recurrence-free survival (one-year 93.9 % vs. 57.4 %; three-year 59.8 % vs. 26.2 %; both ρ < 0.05). TAMs in TIF and tumor recurrence, were confirmed as the only independent prognostic variables in the multivariate survival analysis (all ρ < 0.05). Overall survival and recurrence free survival of patients with hilar cholangiocarcinoma significantly improved in patients with low levels of TAMs in the area of TIF, when compared to those with a high density of TAMs. These observations suggest their utilization as valuable prognostic markers in routine histopathologic evaluation, and might indicate future therapeutic approaches by targeting TAMs

Igf-I regulates pheochromocytoma cell proliferation and survival in vitro and in vivo.

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Fernández, María Celia; Venara, Marcela; Nowicki, Susana; Chemes, Héctor E; Barontini, Marta; Pennisi, Patricia A

2012-08-01

IGFs are involved in malignant transformation and growth of several tissues, including the adrenal medulla. The present study was designed to evaluate the impact of IGF-I on pheochromocytoma development. We used a murine pheochromocytoma (MPC) cell line (MPC4/30) and an animal model with a reduction of 75% in circulating IGF-I levels [liver-IGF-I-deficient (LID) mice] to perform studies in vitro and in vivo. We found that, in culture, IGF-I stimulation increases proliferation, migration, and anchorage-independent growth, whereas it inhibits apoptosis of MPC cells. When injected to control and to LID mice, MPC cells grow and form tumors with features of pheochromocytoma. Six weeks after cell inoculation, all control mice developed sc tumors. In contrast, in 73% of LID mice, tumor development was delayed to 7-12 wk, and the remaining 27% did not develop tumors up to 12 wk after inoculation. LID mice harboring MPC cells and treated with recombinant human IGF-I (LID+) developed tumors as controls. Tumors developed in control, LID, and LID+ mice had similar histology and were similarly positive for IGF-I receptor expression. The apoptotic index was higher in tumors from LID mice compared with those from control mice, whereas vascular density was decreased. In summary, our work demonstrates that IGF-I has a critical role in maintaining tumor phenotype and survival of already transformed pheochromocytoma cells and is required for the initial establishment of these tumors, providing encouragement to carry on research studies to address the IGF-I/IGF-I receptor system as a target of therapeutic strategies for pheochromocytoma treatment in the future.

Impact of Preoperative Biliary Drainage on Surgical Outcomes in Periampullary and Hilar Malignancy: A Single-Center Experience.

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Ito, Yukiko; Nakai, Yousuke; Isayama, Hiroyuki; Tsujino, Takeshi; Hamada, Tsuyoshi; Umefune, Gyotane; Akiyama, Dai; Takagi, Kaoru; Takamoto, Takeshi; Hashimoto, Takuya; Nakata, Ryo; Koike, Kazuhiko; Makuuchi, Masatoshi

2016-04-01

The role of preoperative biliary drainage (PBD) for periampullary and hilar malignancy is still controversial. We retrospectively studied consecutive 144 patients (92 periampullary and 52 hilar malignancy) undergoing surgical resection to evaluate the effects of PBD on surgical outcomes. The rate of PBD was 59% and 56%, and postoperative complications developed in 27% and 19% in periampullary and hilar malignancy, respectively. Risk factors for postoperative complications were overweight [odds ratio (OR), 7.6] and depression (OR, 8.5) in distal malignancy and American society of anesthesiologists score of 3 (OR, 6.6), depression (OR, 13.8), and portal vein embolization (OR, 6.1) in hilar malignancy. PBD was not associated with postoperative complications but reinterventions for PBD were necessary in 43% and 27% in distal and hilar biliary obstruction. In conclusion, PBD in pancreatobiliary surgery was not associated with postoperative complications, but the improvement of PBD is necessary given the high rate of reinterventions.

Clevidipine for hypertension treatment in pheochromocytoma surgery.

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Luis-García, C; Arbonés-Aran, E; Teixell-Aleu, C; Lorente-Poch, L; Trillo-Urrutia, L

2018-04-01

Pheochromocytoma is a catecholamine-producing tumour and laparoscopic adrenalectomy is its treatment of choice. During pneumoperitoneum insufflation and tumour handling there is a high risk of massive catecholamine release and hypertensive crisis. After tumour excision, severe arterial hypotension is a common effect, due to relative vasodilation and the residual effect of antihypertensive drugs. We report the case of a patient with pheochromocytoma who was treated with laparoscopic adrenalectomy. During surgical manipulation there was a sudden hypertensive peak that could be controlled quickly with clevidipine infusion. After tumour resection, clevidipine perfusion was stopped and there were no arterial hypotension episodes. Clevidipine is a new intravenous calcium antagonist with rapid onset of action and short half-life that has no residual effect and does not produce arterial hypotension after tumour resection. For these reasons, it can be a first-choice drug for this kind of surgery. Copyright © 2017 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

New strategy for treatment of carcinoma of the hilar bile duct

International Nuclear Information System (INIS)

Koyama, K.; Tanaka, J.; Kato, S.; Asanuma, Y.

1989-01-01

Surgical treatment for carcinoma of the hilar bile duct has been a challenging problem, because the five year survival rate is less than 5 per cent and the mean survival period is 17 to 24 months even in curatively resected instances. The prognostic factors of carcinoma of the hilar bile duct are remnant carcinoma at the bile duct stump and cancerous invasion into the lymphatics, veins, perineural spaces around the intrahepatic bile duct and caudate lobe of the liver. Based on these data, a new strategy for treatment of carcinoma of the hilar bile duct has been developed and applied clinically. The strategy consists of three procedures. The first is resection of the hilar bile duct with portajejunostomy; the second, specific anticancer therapy with mitomycin C (4 milligrams) adsorbed to the activated charcoal (MMC-CH) focused on the invasion of carcinoma to the periductal lymphatics, and third, intracavitary irradiation (32 to 40 gray) by 60Co using the remote after loading system through the bile duct focused on the periductal infiltration of the carcinoma and through the inferior vena cava focused on the caudate lobe of the liver. In this article, the operative procedures and theoretic background of the specific chemotherapy and irradiation are described. Seven patients have been treated using this regimen. Follow-up study ranged from seven to 38 months. All patients are alive, and five of seven are disease-free

Perioperative and long-term outcome of intrahepatic cholangiocarcinoma involving the hepatic hilus after curative-intent resection: comparison with peripheral intrahepatic cholangiocarcinoma and hilar cholangiocarcinoma.

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Zhang, Xu-Feng; Bagante, Fabio; Chen, Qinyu; Beal, Eliza W; Lv, Yi; Weiss, Matthew; Popescu, Irinel; Marques, Hugo P; Aldrighetti, Luca; Maithel, Shishir K; Pulitano, Carlo; Bauer, Todd W; Shen, Feng; Poultsides, George A; Soubrane, Olivier; Martel, Guillaume; Koerkamp, B Groot; Guglielmi, Alfredo; Itaru, Endo; Pawlik, Timothy M

2018-05-01

Intrahepatic cholangiocarcinoma with hepatic hilus involvement has been either classified as intrahepatic cholangiocarcinoma or hilar cholangiocarcinoma. The present study aimed to investigate the clinicopathologic characteristics and short- and long-term outcomes after curative resection for hilar type intrahepatic cholangiocarcinoma in comparison with peripheral intrahepatic cholangiocarcinoma and hilar cholangiocarcinoma. A total of 912 patients with mass-forming peripheral intrahepatic cholangiocarcinoma, 101 patients with hilar type intrahepatic cholangiocarcinoma, and 159 patients with hilar cholangiocarcinoma undergoing curative resection from 2000 to 2015 were included from two multi-institutional databases. Clinicopathologic characteristics and short- and long-term outcomes were compared among the 3 groups. Patients with hilar type intrahepatic cholangiocarcinoma had more aggressive tumor characteristics (eg, higher frequency of vascular invasion and lymph nodes metastasis) and experienced more extensive resections in comparison with either peripheral intrahepatic cholangiocarcinoma or hilar cholangiocarcinoma patients. The odds of lymphadenectomy and R0 resection rate among patients with hilar type intrahepatic cholangiocarcinoma were comparable with hilar cholangiocarcinoma patients, but higher than peripheral intrahepatic cholangiocarcinoma patients (lymphadenectomy incidence, 85.1% vs 42.5%, P hilar type intrahepatic cholangiocarcinoma experienced a higher rate of technical-related complications compared with peripheral intrahepatic cholangiocarcinoma patients. Of note, hilar type intrahepatic cholangiocarcinoma was associated with worse disease-specific survival and recurrence-free survival after curative resection versus peripheral intrahepatic cholangiocarcinoma (median disease-specific survival, 26.0 vs 54.0 months, P hilar cholangiocarcinoma (median disease-specific survival, 26.0 vs 49.0 months, P = .003; median recurrence-free survival

Comparison study between the MR images and pathomorphologic findings of the pulmonary hilar lymph nodes

International Nuclear Information System (INIS)

Kiyono, Kunihiro; Sakai, Fumikazu; Sone, Shusuke; Imai, Yutaka; Kawai, Takashi; Maruyama, Yuichiro; Shibata, Atsushi; Ito, Nobuo

1995-01-01

The MR images of the resected human lungs were correlated with pathomorphologic findings of the specimens to facilitate interpretation of the pulmonary hilum, specifically hilar lymph nodes. Normal hilar lymph nodes were demonstrated as low signal intensity structures on T1-, T2- and proton-density-weighted images. The walls of the pulmonary vessels and bronchi were shown as linear structures of relatively low intensity, with fatty tissue of high intensity surrounding these structures. Among these low signal intensity hilar lymph nodes were shown resulting from anthracosis, which were similar to those by fibrotic foci. (author)

Synaptic reorganization of inhibitory hilar interneuron circuitry after traumatic brain injury in mice

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Hunt, Robert F.; Scheff, Stephen W.; Smith, Bret N.

2011-01-01

Functional plasticity of synaptic networks in the dentate gyrus has been implicated in the development of posttraumatic epilepsy and in cognitive dysfunction after traumatic brain injury, but little is known about potentially pathogenic changes in inhibitory circuits. We examined synaptic inhibition of dentate granule cells and excitability of surviving GABAergic hilar interneurons 8–13 weeks after cortical contusion brain injury in transgenic mice that express enhanced green fluorescent protein in a subpopulation of inhibitory neurons. Whole-cell voltage-clamp recordings in granule cells revealed a reduction in spontaneous and miniature IPSC frequency after head injury; no concurrent change in paired-pulse ratio was found in granule cells after paired electrical stimulation of the hilus. Despite reduced inhibitory input to granule cells, action potential and EPSC frequencies were increased in hilar GABA neurons from slices ipsilateral to the injury, versus those from control or contralateral slices. Further, increased excitatory synaptic activity was detected in hilar GABA neurons ipsilateral to the injury after glutamate photostimulation of either the granule cell or CA3 pyramidal cell layers. Together, these findings suggest that excitatory drive to surviving hilar GABA neurons is enhanced by convergent input from both pyramidal and granule cells, but synaptic inhibition of granule cells is not fully restored after injury. This rewiring of circuitry regulating hilar inhibitory neurons may reflect an important compensatory mechanism, but it may also contribute to network destabilization by increasing the relative impact of surviving individual interneurons in controlling granule cell excitability in the posttraumatic dentate gyrus. PMID:21543618

99mTc-HYNIC-TOC scintigraphy is superior to 131I-MIBG imaging in the evaluation of extraadrenal pheochromocytoma.

Science.gov (United States)

Chen, Libo; Li, Fang; Zhuang, Hongming; Jing, Hongli; Du, Yanrong; Zeng, Zhengpei

2009-03-01

In this investigation, the efficacy of scintigraphy using (99m)Tc-labeled hydrazinonicotinyl-Tyr3-octreotide (HYNIC-TOC) in the evaluation of extraadrenal pheochromocytoma was assessed and compared with (131)I-labeled metaiodobenzylguanidine (MIBG) imaging. Ninety-seven patients who were suspected of having pheochromocytoma but showed no definite adrenal abnormalities on CT were evaluated by both (99m)Tc-HYNIC-TOC scintigraphy and (131)I-MIBG imaging. The results were compared with pathology findings or clinical follow-up. Of 58 patients proven to be without pheochromocytoma, (99m)Tc-HYNIC-TOC and (131)I-MIBG imaging excluded 56 and 58 patients, respectively, rendering a specificity of 96.6% for (99m)Tc-HYNIC-TOC imaging and 100% for (131)I-MIBG imaging. In the evaluation of adrenal pheochromocytoma (14 patients), the sensitivity of (99m)Tc-HYNIC-TOC scintigraphy and (131)I-MIBG imaging was 50% and 85.7%, respectively. However, in the evaluation of extraadrenal pheochromocytomas (25 patients), the sensitivity of (99m)Tc-HYNIC-TOC scintigraphy and (131)I-MIBG imaging was 96.0% and 72.0%, respectively. (99m)Tc-HYNIC-TOC scintigraphy is more sensitive than (131)I-MIBG imaging in the detection of extraadrenal pheochromocytomas.

Bilateral Vascular Variations at the Renal Hilum: A Case Report

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Naveen Kumar

2012-01-01

Full Text Available Imaging technology with its advancement in the field of urology is the boon for the patients who require minimally invasive approaches for various kidney disorders. These approaches require a precise knowledge of the normal and variant anatomy of vascular structures at the hilum of the kidney in terms of their pattern of arrangement and division. The present paper describes a bilateral anomalous arrangement of the structures at the renal hilum as well as their peculiar branching pattern which is of clinical and surgical relevance. Multiple branching of the renal vessels was observed in both kidneys due to which the hila were congested. The right renal artery immediately after its origin divided into 2 branches. The upper branch represented an aberrant artery whereas the lower branch gave 5 divisions. The left renal artery also divided into 2 branches much before the hilum as anterior and posterior divisions. The anterior branch took an arched course and gave 6 branches. The posterior branch gave 3 terminal branches before entering the renal substance. In addition to anomalous hilar structures, normal architecture of both kidneys was altered and the hilum of the left kidney was found on its anterior surface.

[sup 131]I-metaiodobenzylguanidine therapy for malignant pheochromocytoma

Energy Technology Data Exchange (ETDEWEB)

Sakahara, Harumi; Saga, Tsuneo; Hosono, Makoto; Kobayashi, Hisataka; Konishi, Junji (Kyoto Univ. (Japan). Faculty of Medicine); Endo, Keigo

1994-05-01

[sup 131]I-metaiodobenzylguanidine (MIBG) therapy was given to five patients with malignant pheochromocytoma. The patients received 1-3 doses of 3.33-4.625 GBq (total dose: 3.7 to 10.73 GBq). Partial tumor regression was observed in two patients, the tumor was unchanged in two patients, and slow progression was noted in one patient. Marked improvement in clinical symptoms was achieved in four patients. The other patients had no symptoms before [sup 131]I-MIBG treatment, but the serum epinephrine and dopamine decreased. There were no severe untoward responses in four patients. However, one patient developed transient but severe orthostatic hypotension, hypertension, and hyperglycemia from 1 week to 1 month after [sup 131]I-MIBG administration. Although complete remission was not obtained, all the patients achieved some benefit from [sup 131]I-MIBG therapy. Thus, [sup 131]I-MIBG appears to be useful for the palliation of malignant pheochromocytoma. (author).

Preventive Role of Hilar Parasympathetic Ganglia on Pulmonary Artery Vasospasm in Subarachnoid Hemorrhage: An Experimental Study.

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Araz, Omer; Aydin, Mehmet Dumlu; Gundogdu, Betul; Altas, Ender; Cakir, Murteza; Calikoglu, Cagatay; Atalay, Canan; Gundogdu, Cemal

2015-01-01

Pulmonary arteries are mainly innervated by sympathetic vasoconstrictor and parasympathetic vasodilatory fibers. We examined whether there is a relationship between the neuron densities of hilar parasympathetic ganglia and pulmonary vasospasm in subarachnoid hemorrhage (SAH). Twenty-four rabbits were divided into two groups: control (n=8) and SAH (n=16). The animals were observed for 20 days following experimental SAH. The number of hilar parasympathetic ganglia and their neuron densities were determined. Proportion of pulmonary artery ring surface to lumen surface values was accepted as vasospasm index (VSI). Neuron densities of the hilar ganglia and VSI values were compared statistically. Animals in the SAH group experienced either mild (n=6) or severe (n=10) pulmonary artery vasospasm. In the control group, the mean VSI of pulmonary arteries was 0.777±0.048 and the hilar ganglion neuron density was estimated as 12.100±2.010/mm 3 . In SAH animals with mild vasospasm, VSI=1.148±0.090 and neuron density was estimated as 10.110±1.430/mm 3 ; in animals with severe vasospasm, VSI=1.500±0.120 and neuron density was estimated as 7.340±990/mm 3 . There was an inverse correlation between quantity and neuron density of hilar ganglia and vasospasm index value. The low numbers and low density of hilar parasympathetic ganglia may be responsible for the more severe artery vasospasm in SAH.

Palliative treatment in patients with unresectable hilar cholangiocarcinoma: results of endoscopic drainage in patients with type III and IV hilar cholangiocarcinoma

NARCIS (Netherlands)

Gerhards, M. F.; den Hartog, D.; Rauws, E. A.; van Gulik, T. M.; González González, D.; Lameris, J. S.; de Wit, L. T.; Gouma, D. J.

2001-01-01

OBJECTIVE: To find out how patients fared after palliative endoscopic biliary drainage for inoperable hilar cholangiocarcinoma. DESIGN: Retrospective study. SETTING: University hospital, the Netherlands. SUBJECTS: Between 1992 and 1999, 41 patients who were referred for resection had tumours that

Adrenal and extra-adrenal pheochromocytomas presenting as life-threatening ventricular arrhythmias: Report of three cases

Directory of Open Access Journals (Sweden)

Sai Satish Oruganti

2016-05-01

Full Text Available Pheochromocytoma patients can rarely have prolonged QT interval in the ECG. We report three cases of pheochromocytoma in females presenting with ventricular arrhythmia; two had torsades de pointes and a third patient had frequent VPCs and nonsustained ventricular tachycardia. All the patients were treated with surgical removal of the tumor with complete relief of symptoms and normalization of QT interval.

Thyrotoxic and pheochromocytoma multisystem crisis: a case report.

Science.gov (United States)

Suzuki, Kodai; Miyake, Takahito; Okada, Hideshi; Yamaji, Fuminori; Kitagawa, Yuichiro; Fukuta, Tetsuya; Yasuda, Ryu; Tanaka, Yoshihito; Okamoto, Haruka; Nachi, Sho; Doi, Tomoaki; Yoshida, Takahiro; Kumada, Keisuke; Yoshida, Shozo; Ushikoshi, Hiroaki; Toyoda, Izumi; Ogura, Shinji

2017-06-23

Thyrotoxic crisis and pheochromocytoma multisystem crisis are rare, life-threatening, emergency endocrine diseases with various clinical manifestations. Here we report a case of a patient who simultaneously developed thyrotoxic crisis and pheochromocytoma multisystem crisis and required intensive cardiovascular management. A 60-year-old Asian man experienced nausea and vomiting, and subsequently developed dyspnea and cold sweats while farming. His serum free thyroxine, free triiodothyronine, and thyrotropin receptor antibody levels were elevated at 2.9 ng/dL, 7.2 pg/dL, and 4.7 IU/L, respectively. Serum thyrotropin levels were suppressed at less than 0.01 μIU/mL. Thyroid echography demonstrated no thyroid swelling (23 × 43 mm). A whole body computed tomography was performed for systemic evaluation. This revealed exophthalmos and a mass of size 57 × 64 mm in the anterior pararenal space. Based on these findings, we made an initial diagnosis of thyrotoxic crisis secondary to exacerbation of Grave's hyperthyroidism. Treatment was begun with an iodine agent at a dose of 36 mg/day, thiamazole at a dose of 30 mg/day, and hydrocortisone at a dose of 300 mg daily for 3 consecutive days. To control tachycardia, continuous intravenously administered propranolol and diltiazem infusions were given. At the same time, small doses of doxazosin and carvedilol were used for both alpha and beta adrenergic blockade. On hospital day 5, his blood pressure and serum catecholamine concentrations (adrenalin 42,365 pg/mL, dopamine 6409 pg/mL, noradrenalin 72,212 pg/mL) were still high despite higher beta blocker and calcium channel blocker doses. These findings contributed to the diagnosis of pheochromocytoma multisystem crisis with simultaneous thyrotoxic crisis. We increased the doses of doxazosin and carvedilol, which stabilized his hemodynamic status. On hospital day 16, metaiodobenzylguanidine scintigraphy showed high accumulation in the right adrenal gland tumor

Hilar cholangiocarcinoma is pathologically similar to pancreatic duct adenocarcinoma: suggestions of similar background and development.

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Nakanuma, Yasuni; Sato, Yasunori

2014-07-01

Routine experiences suggest that cholangiocarcinomas (CCAs) show different clinicopathological behaviors along the biliary tree, and hilar CCA apparently resembles pancreatic duct adenocarcinoma (PDAC). Herein, the backgrounds for these similarities were reviewed. While all cases of PDAC, hilar CCA, intrahepatic CCA (ICCA) and CCA components of combined hepatocellular-cholangiocarcinoma (cHC-CCA) were adenocarcinomas, micropapillary patterns and columnar carcinoma cells were common in PDAC and hilar CCA, and trabecular components and cuboidal carcinoma cells were common in ICCA and CCA components of cHC-CCA. Anterior gradient protein-2 and S100P were frequently expressed in perihilar CCA and PDAC, while neural cell adhesion molecule and luminal epithelial membrane antigen were common in CCA components of c-HC-CCA. Pdx1 and Hes1 were frequently and markedly expressed aberrantly in PDAC and perihilar CCA, although their expression was rare and mild in CCA components in cHC-CCA and ICCA. Hilar CCA showed a similar postoperative prognosis to PDAC but differed from ICCA and cHC-CCA. Taken together, hilar CCA may differ from ICCA and CCA components of cHC-CCA but have a similar development to PDAC. These similarities may be explained by the unique anatomical, embryological and reactive nature of the pancreatobiliary tract. Further studies of these intractable malignancies are warranted. © 2014 Japanese Society of Hepato-Biliary-Pancreatic Surgery.

Dynamic computed tomography (CT) in the rat kidney and application to acute renal failure models

International Nuclear Information System (INIS)

Ishikawa, Isao; Saito, Tadashi; Ishii, Hirofumi; Bansho, Junichi; Koyama, Yukinori; Tobita, Akira

1995-01-01

Renal dynamic CT scanning is suitable for determining the excretion of contrast medium in the cortex and medulla of the kidney, which is valuable for understanding the pathogenesis of disease processes in various conditions. This form of scanning would be convenient for use, if a method of application to the rat kidney were available. Therefore, we developed a method of applying renal dynamic CT to rats and evaluated the cortical and medullary curves, e.g., the corticomedullary junction time which is correlated to creatinine clearance, in various rat models of acute renal failure. The rat was placed in a 10deg oblique position and a bilateral hilar slice was obtained before and 5, 10, 15, 20, 25, 30, 40, 50, 60, 80, 100, 120, 140, 160 and 180 sec after administering 0.5 ml of contrast medium using Somatom DR. The width of the slice was 4 mm and the scan time was 3 sec. The corticomedullary junction time in normal rats was 23.0±10.5 sec, the peak value of the cortical curve was 286.3±76.7 Hounsfield Unit (HU) and the peak value of the medullary curve was 390.1±66.2 HU. Corticomedullary junction time after exposure of the kidney was prolonged compared to that of the unexposed kidney. In rats with acute renal failure, the excretion pattern of contrast medium was similar in both the glycerol- and HgCl2-induced acute renal failure models. The peak values of the cortical curve were maintained three hours after a clamp was placed at the hilar region of the kidney for one hour, and the peak values of the medullary curve were maintained during the administration of 10μg/kg/min of angiotensin II. Dynamic CT curves in the acute renal failure models examined were slightly different from those in human acute renal failure. These results suggest that rats do not provide an ideal model for human acute renal failure. However, the application of dynamic CT to the rat kidney models was valuable for estimating the pathogenesis of various human kidney diseases. (author)

Retrospective analysis of patients clinical manifestations before and after pheochromocytoma surgery.

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Balazovjech, I; Davidova, H; Breza, J

2004-01-01

A retrospective analysis of presurgical clinical picture and blood pressures of 34 patients with histologically verified pheochromocytoma. Assessment of clinical state, blood pressure and prognosis in the course of patients control hospitalizations from 2001 to 2002. The study was designed as a synoptic one, based on a retrospective analysis of 34 patients records with histologically verified pheochromocytoma (26 women and 8 men). Our assessments of clinical symptomatology, maximum paroxysmal hypertension values, average blood pressure values in patients with persistent arterial hypertension, arterial hypertension grade and circadian index were based on patients records. Patients epinephrectomies were followed during their control hospitalizations to assess their clinical state. Their blood pressures were evaluated by means of their circadian monitoring. From the 34 patients, 21 were diagnosed with solitary pheochromocytomas. The circadian index persistence analysis prior to the surgery was associated with circadian blood pressure monitoring in 21 patients. It was lost in 57% of patients. During their control hospitalization as many as 82% of patients preserved their circadian blood pressure variability with a more than 10% decrease in the night time, 5 patients did not preserve their circadian blood pressure variability. Seven from the original 34 patients died: Three of them died from their primary disease, one 63-year old man died from shock following tumour extirpation. The death of other 3 patients was not associated with their primary disease. Long-lasting survival of patients with pheochromocytoma after surgical treatment--except for those with malignant disease--was demonstrated. Although our assessment of the resulting treatment effects was positive, a long-term follow-up is inevitable because of a difficult pathologic-anatomical verification of the malignant nature of the disease as well as of the risk of tumour relapse assessment. (Tab. 1, Fig. 5, Ref 36.)

IgG4-associated sclerosing cholangitis masquerading as hilar cholangiocarcinoma.

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Yadav, Kamal Sunder; Sali, Priyanka Akhilesh; Mansukhani, Verushka M; Shah, Rajiv; Jagannath, P

2016-07-01

IgG4-sclerosing cholangitis (IgG4-SC) commonly presents with type 1 autoimmune pancreatitis. Isolated IgG4-SC is rare. Differentiating IgG4-SC from cholangiocarcinoma preoperatively is challenging due to overlapping radio-clinical manifestations and difficult preoperative histology. We present three cases preoperatively diagnosed and surgically treated as hilar cholangiocarcinoma. First and second cases presented with cholangiocarcinoma with portal vein involvement and third with a malignant-appearing hilar stricture. On histopathology, IgG4-SC was diagnosed in the first two cases. Third patient had raised serum IgG4, and histopathology was inconclusive for IgG4-SC and negative for malignancy. However, she responded to steroid therapy.

SPARCL1 is a novel predictor of tumor recurrence and survival in hilar cholangiocarcinoma.

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Yu, Yang; Chen, Yan; Ma, Jianxia; Yu, Xiaofeng; Yu, Guanzhen; Li, Zhaoshen

2016-03-01

Secreted protein acidic and rich in cysteines-like protein 1 (SPARCL1) has been implicated in tumor initiation, formation, and progression of various cancers, yet its role in hilar cholangiocarcinoma remains largely uncharacterized. In the present study, tissue microarrays containing resected hilar cholangiocarcinoma specimens from 92 patients were used to evaluate the expression of SPARCL1 protein by immunohistochemistry (IHC). In vitro assays were used to determine the effect of SPARCL1 overexpression on cell growth and migration. Loss of SPARCL1 expression was observed in 46 (50.0 %) of the 92 primary tumors. SPARCL1 expression is inversely associated with poorly or undifferentiation specimens (P = 0.030) in addition to lymph node metastasis (P = 0.047). Survival analysis demonstrated that SPARCL1 is an independent factor in predicting the outcome of patients with hilar cholangiocarcinoma. SPARCL1 overexpression suppressed tumor cell migration in vitro by inhibiting MMP-9, MMP-2, Vimentin, and Fibronectin expression, whereas did not inhibit cell proliferation in vitro. Our results suggest that loss of SPARCL1 is involved in the tumorigenesis of hilar cholangiocarcinoma and may serve as a novel molecular biomarker for patients' outcome.

Analysis of the placement of multiple metallic stents in the treatment of hilar cholangiocarcinoma

International Nuclear Information System (INIS)

Lu Zaiming; Liang Hongyuan; Guo Qiyong; Wen Feng; Liu Zhaoyu; Zhang Jun

2007-01-01

Objective: To evaluate the clinical efficacy of multiple stents placement in the management of hilar cholangiocarcinoma, especially in the complex cases of which the hepatic ducts are invaded. Methods: Forty-five consecutive patients with hilar cholangiocarcinoma were treated with percutaneous transhepatic placement of two or three self-expandable metallic endoprostheses. The cause of hilar obstructions in these patients were all cholangiocarcinoma, including Bismuth classification type II (n 12), IIIa (n 17), IIIb (n 10), and IV (n 6). Two or 3 stents were placed in the configuration of T, Y or X over the strictures. Results: Stent placement with 2 or 3 endoprostheses was successful in all patients. All patients showed significant decrease in serum bilirubin level. The mortality rate within 30 days of stent placement was 2.2% (1/45). The mean survival and stent patency times were 215.3 d (26- 516 d) and 181.5 d (26-473 d), respectively. Conclusion: Deploying of multiple metallic stents is an effective method to treat complex hilar cholangiocarcinoma, especially for the cases of which hepatic ducts are invaded; the hepatic ducts should be drained as much as possible. (authors)

THE PHYSIOLOGY BEHIND DIABETES MELLITUS IN PATIENTS WITH PHEOCHROMOCYTOMA: A REVIEW OF THE LITERATURE.

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Mesmar, Bayan; Poola-Kella, Silpa; Malek, Rana

2017-08-01

This paper reviews the physiologic mechanisms responsible for glucose intolerance and diabetes mellitus in patients with pheochromocytoma. Google Scholar and PubMed were searched using the following key words: "diabetes," "pheochromocytoma," "adrenoreceptors," and "hyperglycemia." All the articles that were retrieved and reviewed were in the English language. Glucose intolerance and diabetes mellitus, resulting from high circulating levels of catecholamines, are mainly the product of compromised insulin secretion from the β-cells in the pancreas, decreased glucose uptake in the peripheral tissues, and increased insulin resistance. As pheochromocytomas mainly present with cardiovascular and autonomic hyperfunctioning, it is important to understand the metabolic disorders associated with this rare disease. Hyperglycemia is an associated metabolic abnormality which can drastically improve after tumor resection, and significant downscaling of anti-hyperglycemic therapy is often required. GLUT4 = glucose transporter type 4 HbA1c = hemoglobin A1c IL = interleukin OGTT = oral glucose tolerance test.

Normal mediastinal and hilar lymph nodes in children on multi-detector row chest computed tomography

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Jong, Pim A. de; Nievelstein, Rutger-Jan A. [University Medical Center Utrecht and Wilhelmina Children' s Hospital, Department of Radiology, Utrecht (Netherlands)

2012-02-15

To study normal mediastinal and hilar lymph nodes on multi-detector chest computed tomography (CT). A cohort of 120 children aged 1-17 years underwent emergency CT, including the chest, after high-energy trauma. Axial 5-mm reconstructions were evaluated for lymph nodes at hilar and various mediastinal levels and the short-axis diameters were measured. At least one lymph node was found in 115 (96%) children, with subcarinal (69%), lower paratracheal (64%) and hilar (60%) nodes being most common. Up to 10 years of age most lymph nodes were smaller than or equal to 7 mm. In older children lymph nodes measuring up to 10-mm-short-axis diameter were found. Lymph nodes were rare along the mammary vessels, at lower oesophageal and at prevascular and posterior mediastinal levels in children. Mediastinal and hilar lymph nodes are more common than previously thought, probably because of increased detection by modern multi-detector CT. Lymph node location and age have to be taken into account when evaluating lymph nodes in the paediatric chest. (orig.)

Normal mediastinal and hilar lymph nodes in children on multi-detector row chest computed tomography

International Nuclear Information System (INIS)

Jong, Pim A. de; Nievelstein, Rutger-Jan A.

2012-01-01

To study normal mediastinal and hilar lymph nodes on multi-detector chest computed tomography (CT). A cohort of 120 children aged 1-17 years underwent emergency CT, including the chest, after high-energy trauma. Axial 5-mm reconstructions were evaluated for lymph nodes at hilar and various mediastinal levels and the short-axis diameters were measured. At least one lymph node was found in 115 (96%) children, with subcarinal (69%), lower paratracheal (64%) and hilar (60%) nodes being most common. Up to 10 years of age most lymph nodes were smaller than or equal to 7 mm. In older children lymph nodes measuring up to 10-mm-short-axis diameter were found. Lymph nodes were rare along the mammary vessels, at lower oesophageal and at prevascular and posterior mediastinal levels in children. Mediastinal and hilar lymph nodes are more common than previously thought, probably because of increased detection by modern multi-detector CT. Lymph node location and age have to be taken into account when evaluating lymph nodes in the paediatric chest. (orig.)

Pitfalls in genetic analysis of pheochromocytomas/paragangliomas-case report.

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Canu, Letizia; Rapizzi, Elena; Zampetti, Benedetta; Fucci, Rossella; Nesi, Gabriella; Richter, Susan; Qin, Nan; Giachè, Valentino; Bergamini, Carlo; Parenti, Gabriele; Valeri, Andrea; Ercolino, Tonino; Eisenhofer, Graeme; Mannelli, Massimo

2014-07-01

About 35% of patients with pheochromocytoma/paraganglioma carry a germline mutation in one of the 10 main susceptibility genes. The recent introduction of next-generation sequencing will allow the analysis of all these genes in one run. When positive, the analysis is generally unequivocal due to the association between a germline mutation and a concordant clinical presentation or positive family history. When genetic analysis reveals a novel mutation with no clinical correlates, particularly in the presence of a missense variant, the question arises whether the mutation is pathogenic or a rare polymorphism. We report the case of a 35-year-old patient operated for a pheochromocytoma who turned out to be a carrier of a novel SDHD (succinate dehydrogenase subunit D) missense mutation. With no positive family history or clinical correlates, we decided to perform additional analyses to test the clinical significance of the mutation. We performed in silico analysis, tissue loss of heterozygosity analysis, immunohistochemistry, Western blot analysis, SDH enzymatic assay, and measurement of the succinate/fumarate concentration ratio in the tumor tissue by tandem mass spectrometry. Although the in silico analysis gave contradictory results according to the different methods, all the other tests demonstrated that the SDH complex was conserved and normally active. We therefore came to the conclusion that the variant was a nonpathogenic polymorphism. Advancements in technology facilitate genetic analysis of patients with pheochromocytoma but also offer new challenges to the clinician who, in some cases, needs clinical correlates and/or functional tests to give significance to the results of the genetic assay.

Association between biliary complications and technique of hilar division (extrahepatic vs. intrahepatic in major liver resections

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Gamaletsos Evangelos

2006-08-01

Full Text Available Abstract Background Division of major vascular and biliary structures during major hepatectomies can be carried out either extrahepatically at the porta hepatic or intrahepatically during the parenchymal transection. In this retrospective study we test the hypothesis that the intrahepatic technique is associated with less early biliary complications. Methods 150 patients who underwent major hepatectomies were retrospectively allocated into an intrahepatic group (n = 100 and an extrahepatic group (n = 50 based on the technique of hilar division. The two groups were operated by two different surgical teams, each one favoring one of the two approaches for hilar dissection. Operative data (warm ischemic time, operative time, blood loss, biliary complications, morbidity and mortality rates were analyzed. Results In extrahepatic patients, operative time was longer (245 ± 50 vs 214 ± 38 min, p Conclusion Intrahepatic hilar division is as safe as extrahepatic hilar division in terms of intraoperative blood requirements, morbidity and mortality. The extrahepatic technique is associated with more severe bile leaks and biliary injuries.

Hilar somatostatin interneuron loss reduces dentate gyrus inhibition in a mouse model of temporal lobe epilepsy.

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Hofmann, Gabrielle; Balgooyen, Laura; Mattis, Joanna; Deisseroth, Karl; Buckmaster, Paul S

2016-06-01

In patients with temporal lobe epilepsy, seizures usually start in the hippocampus, and dentate granule cells are hyperexcitable. Somatostatin interneurons are a major subpopulation of inhibitory neurons in the dentate gyrus, and many are lost in patients and animal models. However, surviving somatostatin interneurons sprout axon collaterals and form new synapses, so the net effect on granule cell inhibition remains unclear. The present study uses optogenetics to activate hilar somatostatin interneurons and measure the inhibitory effect on dentate gyrus perforant path-evoked local field potential responses in a mouse model of temporal lobe epilepsy. In controls, light activation of hilar somatostatin interneurons inhibited evoked responses up to 40%. Epileptic pilocarpine-treated mice exhibited loss of hilar somatostatin interneurons and less light-induced inhibition of evoked responses. These findings suggest that severe epilepsy-related loss of hilar somatostatin interneurons can overwhelm the surviving interneurons' capacity to compensate by sprouting axon collaterals. Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.

Pheochromocytoma of posterior mediastinum (paraaortic paraganglioma). Report of a case and revision of the literature

International Nuclear Information System (INIS)

Rodriguez Sabogal, Carlos A; Pedrozo Pupo, John C; Ojeda Leon, Paulina; Garcia-Herreros, D Luis G; Fernandez, Federico

2000-01-01

Pheochromocytomas are a rare tumor, but it's important to know how to diagnose and treat them, because they are a potentially life-threatening disease that if recognized early by the physician can be managed with minimal morbidity. It constitutes one of few curable causes of arterial hypertension by performing surgical resection of the tumor. We report our experience with a female who has a pheochromocytoma in the posterior mediastinum

ADC histogram analysis for adrenal tumor histogram analysis of apparent diffusion coefficient in differentiating adrenal adenoma from pheochromocytoma.

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Umanodan, Tomokazu; Fukukura, Yoshihiko; Kumagae, Yuichi; Shindo, Toshikazu; Nakajo, Masatoyo; Takumi, Koji; Nakajo, Masanori; Hakamada, Hiroto; Umanodan, Aya; Yoshiura, Takashi

2017-04-01

To determine the diagnostic performance of apparent diffusion coefficient (ADC) histogram analysis in diffusion-weighted (DW) magnetic resonance imaging (MRI) for differentiating adrenal adenoma from pheochromocytoma. We retrospectively evaluated 52 adrenal tumors (39 adenomas and 13 pheochromocytomas) in 47 patients (21 men, 26 women; mean age, 59.3 years; range, 16-86 years) who underwent DW 3.0T MRI. Histogram parameters of ADC (b-values of 0 and 200 [ADC 200 ], 0 and 400 [ADC 400 ], and 0 and 800 s/mm 2 [ADC 800 ])-mean, variance, coefficient of variation (CV), kurtosis, skewness, and entropy-were compared between adrenal adenomas and pheochromocytomas, using the Mann-Whitney U-test. Receiver operating characteristic (ROC) curves for the histogram parameters were generated to differentiate adrenal adenomas from pheochromocytomas. Sensitivity and specificity were calculated by using a threshold criterion that would maximize the average of sensitivity and specificity. Variance and CV of ADC 800 were significantly higher in pheochromocytomas than in adrenal adenomas (P histogram parameters for diagnosing adrenal adenomas (ADC 200 , 0.82; ADC 400 , 0.87; and ADC 800 , 0.92), with sensitivity of 84.6% and specificity of 84.6% (cutoff, ≤2.82) with ADC 200 ; sensitivity of 89.7% and specificity of 84.6% (cutoff, ≤2.77) with ADC 400 ; and sensitivity of 94.9% and specificity of 92.3% (cutoff, ≤2.67) with ADC 800 . ADC histogram analysis of DW MRI can help differentiate adrenal adenoma from pheochromocytoma. 3 J. Magn. Reson. Imaging 2017;45:1195-1203. © 2016 International Society for Magnetic Resonance in Medicine.

Intrahepatic and hilar mass-forming cholangiocarcinoma: Qualitative and quantitative evaluation with diffusion-weighted MR imaging

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Fattach, Hassan El, E-mail: hassangreenmed@gmail.com [Department of Abdominal Imaging, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, 2 rue Ambroise Paré, 75010 Paris (France); Dohan, Anthony, E-mail: anthony.dohan@lrb.aphp.fr [Department of Abdominal Imaging, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, 2 rue Ambroise Paré, 75010 Paris (France); Université Paris-Diderot, Sorbonne Paris Cité, 10 Avenue de Verdun, 75010 Paris (France); UMR INSERM 965-Paris 7 “Angiogenèse et recherche translationnelle”, 2 rue Amboise Paré, 75010 Paris (France); Guerrache, Youcef, E-mail: docyoucef05@yahoo.fr [Department of Abdominal Imaging, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, 2 rue Ambroise Paré, 75010 Paris (France); Dautry, Raphael, E-mail: raphael.dautry@lrb.aphp.fr [Department of Abdominal Imaging, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, 2 rue Ambroise Paré, 75010 Paris (France); Université Paris-Diderot, Sorbonne Paris Cité, 10 Avenue de Verdun, 75010 Paris (France); and others

2015-08-15

Highlights: • DW-MR imaging helps depicts all intrahepatic or hilar mass-forming cholangiocarcinomas. • DW-MRI provides best conspicuity of intrahepatic or hilar mass-forming cholangiocarcinomas than the other MRI sequences (P < 0.001). • The use of normalized ADC using the liver as reference organ results in the most restricted distribution of ADC values of intrahepatic or hilar mass-forming cholangiocarcinomas (variation coefficient = 16.6%). - Abstract: Objective: To qualitatively and quantitatively analyze the presentation of intrahepatic and hilar mass-forming cholangiocarcinoma with diffusion-weighted magnetic resonance imaging (DW-MRI). Materials and methods: Twenty-eight patients with histopathologically proven mass-forming cholangiocarcinoma (hilar, n = 17; intrahepatic, n = 11) underwent hepatic DW-MRI at 1.5-T using free-breathing acquisition and three b-values (0,400,800 s/mm{sup 2}). Cholangiocarcinomas were evaluated qualitatively using visual analysis of DW-MR images and quantitatively with conventional ADC and normalized ADC measurements using liver and spleen as reference organs. Results: All cholangiocarcinomas (28/28; 100%) were visible on DW-MR images. DW-MRI yielded best conspicuity of cholangiocarcinomas than the other MRI sequences (P < 0.001). Seven cholangiocarcinomas (7/11; 64%) showed hypointense central area on DW-MR images. Conventional ADC value of cholangiocarcinomas (1.042 × 10{sup −3} mm{sup 2}/s ± 0.221 × 10{sup −3} mm{sup 2}/s; range: 0.616 × 10{sup −3} mm{sup 2}/s to 2.050 × 10{sup −3} mm{sup 2}/s) was significantly lower than that of apparently normal hepatic parenchyma (1.362 × 10{sup −3} mm{sup 2}/s ± 0.187 × 10{sup −3} mm{sup 2}/s) (P < 0.0001), although substantial overlap was found. No significant differences in ADC and normalized ADC values were found between intrahepatic and hilar cholangiocarcinomas. The use of normalized ADC using the liver as reference organ resulted in the most restricted

Intrahepatic and hilar mass-forming cholangiocarcinoma: Qualitative and quantitative evaluation with diffusion-weighted MR imaging

International Nuclear Information System (INIS)

Fattach, Hassan El; Dohan, Anthony; Guerrache, Youcef; Dautry, Raphael

2015-01-01

Highlights: • DW-MR imaging helps depicts all intrahepatic or hilar mass-forming cholangiocarcinomas. • DW-MRI provides best conspicuity of intrahepatic or hilar mass-forming cholangiocarcinomas than the other MRI sequences (P < 0.001). • The use of normalized ADC using the liver as reference organ results in the most restricted distribution of ADC values of intrahepatic or hilar mass-forming cholangiocarcinomas (variation coefficient = 16.6%). - Abstract: Objective: To qualitatively and quantitatively analyze the presentation of intrahepatic and hilar mass-forming cholangiocarcinoma with diffusion-weighted magnetic resonance imaging (DW-MRI). Materials and methods: Twenty-eight patients with histopathologically proven mass-forming cholangiocarcinoma (hilar, n = 17; intrahepatic, n = 11) underwent hepatic DW-MRI at 1.5-T using free-breathing acquisition and three b-values (0,400,800 s/mm 2 ). Cholangiocarcinomas were evaluated qualitatively using visual analysis of DW-MR images and quantitatively with conventional ADC and normalized ADC measurements using liver and spleen as reference organs. Results: All cholangiocarcinomas (28/28; 100%) were visible on DW-MR images. DW-MRI yielded best conspicuity of cholangiocarcinomas than the other MRI sequences (P < 0.001). Seven cholangiocarcinomas (7/11; 64%) showed hypointense central area on DW-MR images. Conventional ADC value of cholangiocarcinomas (1.042 × 10 −3 mm 2 /s ± 0.221 × 10 −3 mm 2 /s; range: 0.616 × 10 −3 mm 2 /s to 2.050 × 10 −3 mm 2 /s) was significantly lower than that of apparently normal hepatic parenchyma (1.362 × 10 −3 mm 2 /s ± 0.187 × 10 −3 mm 2 /s) (P < 0.0001), although substantial overlap was found. No significant differences in ADC and normalized ADC values were found between intrahepatic and hilar cholangiocarcinomas. The use of normalized ADC using the liver as reference organ resulted in the most restricted distribution of ADC values of cholangiocarcinomas (variation

Diagnostic ability of mediastinal and hilar lymph node metastasis of primary lung cancer

International Nuclear Information System (INIS)

Hwang, Len-Ming

1985-01-01

Preoperative thoracic CT scan and conventional radiologic procedures were performed in 68 primary lung cancer patients who underwent radical operation for intrathoracic lymph nodes. The subjects of this study consisted of 58 males and 10 females. Histologically, squamous cell carcinoma was noted in 28 patients, adenocarcinoma in 31, large cell cancer in 5 and small cell cancer in 4. According to the pTNM factor N classification, n0 was noted in 28 patients, n1 in 13 and n2 in 27. Plain and contrast enhancement CT scan were performed, using GE-made CT 9800, from apex to diaphragm with a 2-second scan time in supine position during full inspiration. As conventional radiologic procedures, posteroanterior and lateral plain roentgenography and posteroanterior and lateral tomography of the thoracic region including the hilum and mediastinum were performed on all patients, and 55 0 oblique tomography and PAG procedures were added, if necessary. Of 68 patients, 40 had metastatic lesions in the mediastinal and hilar lymph nodes, 37 in the ipsilateral hilar lymph nodes and 27 in the mediastinal lymph nodes. Of 37 patients with metastatic lesions in the ipsilateral hilar lymph nodes, 24 had such lesions in the mediastinal lymph nodes as well, and 13 only in the ipsilateral hilar lymph nodes. Three patients had mediastinal metastasis without ipsilateral hilar metastasis. In detecting mediastinal lymph node metastasis, CT showed a sensitivity of 78 %, a specificity of 73 % and an accuracy of 75 % while conventional radiologic procedures had a sensitivity of 41 %, a specificity of 78 %, and an accuracy of 63 %. In the hilum, CT also had a higher sensitivity (73 %, 62 % respectively) and lower specificity (71 %, 77 % respectively) comparing to conventional radiologic procedures. And CT had an accuracy of 72 % while conventional radiologic procedures showed 69 %. (J.P.N.)

Placement of a Newly Designed Y-Configured Bilateral Self-Expanding Metallic Stent for Hilar Biliary Obstruction: A Pilot Study.

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Jiao, Dechao; Huang, Kai; Zhu, Ming; Wu, Gang; Ren, Jianzhuang; Wang, Yanli; Han, Xinwei

2017-01-01

Whether unilateral or bilateral drainage should be performed for malignant hilar biliary obstruction remains a matter of debate. Although a Y-stent with a central wide-open mesh facilitates bilateral stent placement, it has its own limitations. This study aims to evaluate the feasibility and efficacy of a newly designed Y-configured bilateral self-expanding metallic stent (SEMS) for the treatment of hilar biliary obstruction. In this retrospective study, 14 consecutive patients with unresectable malignant hilar biliary obstruction (Bismuth type II or higher), who underwent placement of a newly designed Y-configured bilateral SEMS for hilar biliary obstruction from April 2013 to March 2015, were included into this study. Data on technical success, clinical success, stent patency, complications and patient survival were collected. Technical and clinical success was 100 and 92.9 %, respectively. Mean serum bilirubin level was significantly decreased 1 month after stent placement (P hilar biliary obstruction using port docking deployment.

New tapered metallic stent for unresectable malignant hilar bile duct obstruction.

Science.gov (United States)

Sakai, Yuji; Tsuyuguchi, Toshio; Nishikawa, Takao; Sugiyama, Harutoshi; Sasaki, Reina; Sakamoto, Dai; Watanabe, Yuto; Nakamura, Masato; Yasui, Shin; Mikata, Rintaro; Yokosuka, Osamu

2015-10-16

To examine the usefulness of a new tapered metallic stent (MS) in patients with unresectable malignant hilar bile duct obstruction. This new tapered MS was placed in 11 patients with Bismuth II or severer unresectable malignant hilar bile duct obstruction, as a prospective study. The subjects were six patients with bile duct carcinoma, three with gallbladder cancer, and two with metastatic bile duct obstruction. Stenosis morphology was Bismuth II: 7, IIIa: 3, and IV: 1. UMIN Clinical Trial Registry (UMIN000004758). MS placement was 100% (11/11) successful. There were no procedural accidents. The mean patency period was 208.401 d, the median survival period was 142.000 d, and the mean survival period was 193.273 d. Occlusion rate was 36.4% (4/11); the causes of occlusion were ingrowth and overgrowth in 2 patients each, 18.2%, respectively. Patients with occlusion underwent endoscopic treatment one more time and all were treatable. The tapered MS proved useful in patients with unresectable malignant hilar bile duct obstruction because it provided a long patency period, enabled re-treatment by re-intervention, and no procedural accidents occurred.

Photodynamic Therapy Plus Chemotherapy Compared with Photodynamic Therapy Alone in Hilar Nonresectable Cholangiocarcinoma.

Science.gov (United States)

Wentrup, Robert; Winkelmann, Nicola; Mitroshkin, Andrey; Prager, Matthias; Voderholzer, Winfried; Schachschal, Guido; Jürgensen, Christian; Büning, Carsten

2016-05-23

Standard treatments are not available for hilar nonresectable cholangiocarcinoma (NCC). It is unknown whether combination therapy of photodynamic therapy (PDT) plus systemic chemotherapy is superior to PDT alone. We retrospectively reviewed 68 patients with hilar NCC treated with either PDT plus chemotherapy (PTD-C) or PDT monotherapy (PDT-M). The primary endpoint was the mean overall survival rate. Secondary endpoints included the 1-year survival rate, risk of cholangitic complications, and outcomes, which were evaluated according to the chemotherapy protocol. More than 90% of the study population had advanced hilar NCC Bismuth type III or IV. In the PDT-M group (n=35), the mean survival time was 374 days compared with 520 days in the PDT-C group (n=33, p=0.021). The 1-year survival rate was significantly higher in the PDT-C group compared with the PDT-M group (88% vs 58%, p=0.001) with a significant reduction of mortality (hazard ratio, 0.20; 95% confidence interval, 0.07 to 0.58; p=0.003). Gemcitabine monotherapy resulted in a shorter survival time compared with the gemcitabine combination therapy (mean, 395 days vs 566 days; p=0.09). Cholangitic complications were observed at a similar frequency in the PDT-C and PDT-M groups. Combining repeated PDT with a gemcitabine-based combination therapy might offer a significant survival benefit in patients with hilar NCC.

Measurement of fractionated plasma metanephrines for exclusion of pheochromocytoma: Can specificity be improved by adjustment for age?

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Gafni Amiram

2005-02-01

Full Text Available Abstract Background Biochemical testing for pheochromocytoma by measurement of fractionated plasma metanephrines is limited by false positive rates of up to 18% in people without known genetic predisposition to the disease. The plasma normetanephrine fraction is responsible for most false positives and plasma normetanephrine increases with age. The objective of this study was to determine if we could improve the specificity of fractionated plasma measurements, by statistically adjusting for age. Methods An age-adjusted metanephrine score was derived using logistic regression from 343 subjects (including 33 people with pheochromocytoma who underwent fractionated plasma metanephrine measurements as part of investigations for suspected pheochromocytoma at Mayo Clinic Rochester (derivation set. The performance of the age-adjusted score was validated in a dataset of 158 subjects (including patients 23 with pheochromocytoma that underwent measurements of fractionated plasma metanephrines at Mayo Clinic the following year (validation dataset. None of the participants in the validation dataset had known genetic predisposition to pheochromocytoma. Results The sensitivity of the age-adjusted metanephrine score was the same as that of traditional interpretation of fractionated plasma metanephrine measurements, yielding a sensitivity of 100% (23/23, 95% confidence interval [CI] 85.7%, 100%. However, the false positive rate with traditional interpretation of fractionated plasma metanephrine measurements was 16.3% (22/135, 95% CI, 11.0%, 23.4% and that of the age-adjusted score was significantly lower at 3.0% (4/135, 95% CI, 1.2%, 7.4% (p Conclusion An adjustment for age in the interpretation of results of fractionated plasma metanephrines may significantly decrease false positives when using this test to exclude sporadic pheochromocytoma. Such improvements in false positive rate may result in savings of expenditures related to confirmatory imaging.

Pheochromocytoma complicated by intracerebral hemorrhage - a case report

International Nuclear Information System (INIS)

Nogueira, Aline Silva; Marchiori, Edson; Almeida, Fabiola Assuncao de; Martins, Renata Romano; Sales, Anderson Ribeiro; Santos, Tereza Cristina C.R.S. dos; Reis, Simone Teixeira; Silveira, Sonia Marcelino T. da

1999-01-01

The authors report a case of pheochromocytoma that was complicated by intracerebral hemorrhage in a 17-year-old female patient. Computed tomography showed a solid mass, heterogeneous, on the right adrenal. The patient underwent a right adrenalectomy. She is being observed by our out-patients clinic, presenting normal blood pressure levels and a left hemiparesis. (author)

Solid renal tumor severity in von Hippel Lindau disease is related to germline deletion length and location.

Science.gov (United States)

Maranchie, Jodi K; Afonso, Anoushka; Albert, Paul S; Kalyandrug, Sivaram; Phillips, John L; Zhou, Shubo; Peterson, James; Ghadimi, Bijan M; Hurley, Katheen; Riss, Joseph; Vasselli, James R; Ried, Thomas; Zbar, Berton; Choyke, Peter; Walther, McClellan M; Klausner, Richard D; Linehan, W Marston

2004-01-01

von Hippel Lindau disease (VHL) is an autosomal dominant familial cancer syndrome linked to alteration of the VHL tumor suppressor gene. Affected patients are predisposed to develop pheochromocytomas and cystic and solid tumors of the kidney, CNS, pancreas, retina, and epididymis. However, organ involvement varies considerably among families and has been shown to correlate with the underlying germline alteration. Clinically, we observed a paradoxically lower prevalence of renal cell carcinoma (RCC) in patients with complete germline deletion of VHL. To determine if a relationship existed between the type of VHL deletion and disease, we retrospectively evaluated 123 patients from 55 families with large germline VHL deletions, including 42 intragenic partial deletions and 13 complete VHL deletions, by history and radiographic imaging. Each individual and family was scored for cystic or solid involvement of CNS, pancreas, and kidney, and for pheochromocytoma. Germline deletions were mapped using a combination of fluorescent in situ hybridization (FISH) and quantitative Southern and Southern blot analysis. An age-adjusted comparison demonstrated a higher prevalence of RCC in patients with partial germline VHL deletions relative to complete deletions (48.9 vs. 22.6%, p=0.007). This striking phenotypic dichotomy was not seen for cystic renal lesions or for CNS (p=0.22), pancreas (p=0.72), or pheochromocytoma (p=0.34). Deletion mapping revealed that development of RCC had an even greater correlation with retention of HSPC300 (C3orf10), located within the 30-kb region of chromosome 3p, immediately telomeric to VHL (52.3 vs. 18.9%, p <0.001), suggesting the presence of a neighboring gene or genes critical to the development and maintenance of RCC. Careful correlation of genotypic data with objective phenotypic measures will provide further insight into the mechanisms of tumor formation. Copyright 2003 Wiley-Liss, Inc.

Endoscopic palliation of patients with biliary obstruction caused by nonresectable hilar cholangiocarcinoma: efficacy of self-expandable metallic Wallstents

NARCIS (Netherlands)

Cheng, John L. S.; Bruno, Marco J.; Bergman, Jacques J.; Rauws, Erik A.; Tytgat, Guido N.; Huibregtse, Kees

2002-01-01

Background: The aim of this study was to evaluate the efficacy of an endoscopically inserted self-expandable metal stent for treatment of biliary obstruction caused by nonresectable hilar cholangiocarcinoma. Methods: Data on all patients with nonresectable hilar cholangiocarcinoma receiving

A Case of Malignant Pheochromocytoma Detected during Fertility Treatment

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Kazuhisa Hagiwara

2014-01-01

Full Text Available We report a case of malignant pheochromocytoma in a 35-year-old Japanese woman during fertility treatment, successfully treated with surgical excision. The patient recovered without any postoperative problems, and plasma catecholamine levels normalized. At present, 18 months after the operation, there are no signs of relapse.

Differentiation of benign and malignant hilar bile duct stenosis.

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Liu, Xiaolei; Yang, Zhiying; Tan, Haidong; Shao, Chen; Liu, Liguo; Si, Shuang; Xu, Li; Sun, Yongliang

2016-06-15

Failure to differentiate benign and malignant hilar bile duct stenosis may lead to inappropriate treatment. We retrospectively analyzed the methods for differentiation. A total of 53 patients with hilar bile duct stenosis were included, comprising 41 malignant cases (hilar cholangiocarcinoma) and 12 benign cases (six primary sclerosing cholangitis and six IgG4-associated sclerosing cholangitis). Data of clinical histories, laboratory tests, imaging studies, and liver pathologies were collected, and comparison was made between benign and malignant groups. Compared with malignant group, patients in the benign group were more likely to have multiorgan involvement of clinical histories (P < 0.001). There was no difference on bilirubin, liver enzyme, and serum tumor marker between the two groups, whereas serum IgG4 levels were higher in the benign group (P = 0.003). Patients in the benign group were more likely to have pancreatic changes (P < 0.001) and multiple-segmental bile duct stenosis (P < 0.001) on imaging. Compared with the malignant group, patients in the benign group were more likely to show severe periportal inflammation in noninvolved liver (P < 0.001), fibrosis around intrahepatic bile duct (P < 0.001), and more IgG4-positive plasma cells (P < 0.001) on liver pathology. Benign lesion should be considered for patients with history of multiorgan involvement, pancreas changes, or multiple-segmental bile duct stenosis on imaging. Liver biopsy could be helpful for differential diagnosis before surgery. Copyright © 2016 Elsevier Inc. All rights reserved.

En Bloc Hilar Dissection of the Right Hepatic Artery in Continuity with the Bile Duct: a Technique to Reduce Biliary Complications After Adult Living-Donor Liver Transplantation.

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Abu-Gazala, Samir; Olthoff, Kim M; Goldberg, David S; Shaked, Abraham; Abt, Peter L

2016-04-01

Techniques that preserve the right hepatic artery and the common bile duct in continuity during the dissection may be associated with lower rates of biliary complications in living-donor liver transplants. This study sought to determine whether en bloc hilar dissections were associated with fewer biliary complications in living-donor liver transplants. This was a retrospective review of 41 adult LDLTs performed in a single, liver transplant center between February 2007 and September 2014. The primary outcome of interest was the occurrence of at least one of the following biliary complications: anastomotic leak, stricture, or biloma. The primary predictor of interest was the hilar dissection technique: conventional hilar dissection vs. en bloc hilar dissection. A total of 41 LDLTs were identified, 24 had a conventional, and 17 an en bloc hilar biliary dissection. The occurrence of any biliary complication was significantly more common in the conventional hilar dissection group compared to the en bloc hilar dissection group (66.7 vs. 35.3%, respectively, p = 0.047). In particularly, anastomotic strictures were significantly more common in the conventional hilar dissection group compared to the en bloc hilar dissection group (54.2 vs. 23.5%., respectively, p = 0.049). En bloc hilar dissection technique may decrease biliary complication rates in living donor liver transplants.

Refined staging in hilar bronchial neoplasms with ECG-gated multislice-CT. Case report

International Nuclear Information System (INIS)

Ohlmann, S.; Daliri, A.; Froelich, J.J.; Nowak, R.; Michulla, R.

2008-01-01

Equivocal initial CT-based staging in 2 patients with hilar bronchial neoplasms was reassessed with retrospective ECG-gated Multislice-CT and optimized examination parameters prior to definition of treatment. An initially suspected irresectable T 4 tumor with mediastinal infiltration was downstaged to T 2 in one case, while tumor infiltration into the left atrium could be confirmed in the other case. In doubtful conditions, ECG-gated multislice CT with optimized examination parameters may be helpful for refined staging in patients with hilar bronchial neoplasma, thus possibly influencing treatment strategies. (orig.)

Unexpected visitor on FDG PET/CT--brown adipose tissue (BAT) in mesentery in a case of retroperitoneal extra-adrenal pheochromocytoma: is the BAT activation secondary to catecholamine-secreting pheochromocytoma?

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Joshi, Prathamesh Vijay; Lele, Vikram Ramchandra

2012-05-01

Fused positron emission tomography-computed tomography (PET/CT) technology has enabled the determination that nonmalignant fluorodeoxyglucose (FDG) uptake is observed in brown adipose tissue (BAT). FDG uptake in BAT is a known potential source of false-positive interpretations for PET. The typical locations of BAT include neck, supraclavicular area, mediastinum, and paravertebral intercostal spaces. Examples of atypical locations for BAT include posterior neck, left paratracheal area, axillae, perirenal area, and retrocrural area. We report PET/CT findings in a young male patient with malignant retroperitoneal extra-adrenal pheochromocytoma, who demonstrated FDG uptake in BAT at multiple locations including mesenteric BAT. We also propose catecholamine-secreting pheochromocytoma as a possible cause of BAT activation in our case.

A Rare Cause of Pheochromocytoma; Neurofibromatosis Type 1-Noonan Syndrome

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Mazhar Müslüm Tuna

2014-09-01

Full Text Available Neurofibromatosis (NF Type 1 (NF-1 is an autosomal dominant disease with a prevalence of about 1/3000. NF-1 is a neurocutaneous syndrome characterized by cafe au lait macules, neurofibroma, optic glioma, lisch nodules, and symptoms involving other systems. Noonan syndrome (NS is a clinically heterogeneous disorder predominantly characterized by dysmorphic facial features, congenital heart disease, proportionate post-natal short stature, neck abnormalities, and chest deformities. NF-NS is a very rare overlapping syndrome sharing many features of both syndromes. Coexistence of pheochromocytoma, which can be life-threatening if not treated properly, is also a very rare complication of this disorder. Here, we report a patient who was admitted with a mass in the right upper quadrant and was diagnosed with pheochromocytoma and NFNS. (The Me­di­cal Bul­le­tin of Ha­se­ki 2014; 52: 227-31

Intrinsic neurophysiological properties of hilar ectopic and normotopic dentate granule cells in human temporal lobe epilepsy and a rat model.

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Althaus, A L; Sagher, O; Parent, J M; Murphy, G G

2015-02-15

Hilar ectopic dentate granule cells (DGCs) are a salient feature of aberrant plasticity in human temporal lobe epilepsy (TLE) and most rodent models of the disease. Recent evidence from rodent TLE models suggests that hilar ectopic DGCs contribute to hyperexcitability within the epileptic hippocampal network. Here we investigate the intrinsic excitability of DGCs from humans with TLE and the rat pilocarpine TLE model with the objective of comparing the neurophysiology of hilar ectopic DGCs to their normotopic counterparts in the granule cell layer (GCL). We recorded from 36 GCL and 7 hilar DGCs from human TLE tissue. Compared with GCL DGCs, hilar DGCs in patient tissue exhibited lower action potential (AP) firing rates, more depolarized AP threshold, and differed in single AP waveform, consistent with an overall decrease in excitability. To evaluate the intrinsic neurophysiology of hilar ectopic DGCs, we made recordings from retrovirus-birthdated, adult-born DGCs 2-4 mo after pilocarpine-induced status epilepticus or sham treatment in rats. Hilar DGCs from epileptic rats exhibited higher AP firing rates than normotopic DGCs from epileptic or control animals. They also displayed more depolarized resting membrane potential and wider AP waveforms, indicating an overall increase in excitability. The contrasting findings between disease and disease model may reflect differences between the late-stage disease tissue available from human surgical specimens and the earlier disease stage examined in the rat TLE model. These data represent the first neurophysiological characterization of ectopic DGCs from human hippocampus and prospectively birthdated ectopic DGCs in a rodent TLE model. Copyright © 2015 the American Physiological Society.

Value of multi-slice CT in the classification diagnosis of hilar cholangiocarcinoma

International Nuclear Information System (INIS)

Qian Yi; Zeng Mengsu; Ling Zhiqing; Rao Shengxiang; Liu Yalan

2008-01-01

Objective: To evaluate the value of multi-slice CT (MSCT) classification in the assessment of the hilar cholangiocarcinoma resectability. Methods: Thirty patients with surgically and histopathologically proved hilar cholangiocarcinomas who underwent preoperative MSCT and were diagnosed correctly were included in present study. Transverse images and reconstructed MPR images were reviewed for Bismuth-Corlette classification and morphological classification of hilar cholangiocarcinoma. Then MSCT classification was compared with findings of surgery and histopathology. Curative resectabilty of different types according to Bismuth-Corlette classification and morphological classification were analyzed with chi-square test. Results: In 30 cases, the numbers of Type I, II, IIIa, IIIb and IV according to Bismuth-Corlette classification were 1, 3, 4, 5 and 17. Seventeen patients underwent curative resections, among which 1, 2, 1, 4 and 9 belonged to Type I, II, IIIa, IIIb and IV respectively. However, there was no significant difference in curative resectability among different types of Bismuth-Corlette classification (χ 2 = 0.9875, P>0.05). In present study, the accuracy of MSCT in Bismuth-Corlette classification reached 86.7% (26/30). The numbers of periductal infiltrating, mass forming and intraductal growing type were 13, 13 and 4, while 6, 8 and 3 cases of each type underwent curative resections. There was no significant difference in curative resectability among different types of morphological classification (χ 2 =1.2583, P>0.05). The accuracy of MSCT in morphological classification was 100% (30/30) in this study group. Conclusion: MSCT can make accurate diagnosis of Bismuth-Corlette classification and morphological classification, which is helpful in preoperative respectability assessment of hilar cholangiocarcinoma. (authors)

Border between the hilar plate and the Glissonian sheath. Its clinical application

International Nuclear Information System (INIS)

Endo, Itaru; Yamaguchi, Naotaka; Masunari, Hideki

2010-01-01

Diagnosis of the border in the title, hilar plate (H)-Glissonian sheath (G) border, is important in the surgery of hilar cholangiocarcinoma as hepatectomy is needed if the disease invades over G. This paper describes definition of the border by local manual dissection and by 3D image of multi-detector row CT, both of which integration can be useful for decision of operative planning and procedure of the cancer. Authors define through the dissection of 22 dead bodies that the H-G border is the position where manual ablation of hepatic blood vessels and bile duct is impossible with tweezers. For defining the size of hilar plate, they have actually measured the distances from the confluence of right-left hepatic ducts to G in the segment 2 (G2), to G in the posterior segment (Gp), and from the outer border of U- and P- points to G2 and Gp, respectively. U- and P- points are the umbilical and posterior portal branching segment, respectively, and are used here as landmarks exhibiting the relative positioning of blood vessels and bile duct. The H-G border defined by 3DCT images of 28 patients with hepatobiliary diseases is shown to rather well agree with that by the above distances, indicating that H-G border of patients can be reasonably estimated by preoperative 3DCT imaging. Presented here are 2 cases, successfully receiving the surgery of the cancer conducted on the preoperative strategy of resection by 3DCT. Authors conclude that safe and curative resection of hilar cholangiocarcinoma can be done when their image findings are clinically applied in the planning. (T.T.)

Endoscopic Drainage of >50% of Liver in Malignant Hilar Biliary Obstruction Using Metallic or Fenestrated Plastic Stents.

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Kerdsirichairat, Tossapol; Arain, Mustafa A; Attam, Rajeev; Glessing, Brooke; Bakman, Yan; Amateau, Stuart K; Freeman, Martin L

2017-08-31

Endoscopic drainage of complex hilar tumors has generally resulted in poor outcomes. Drainage of >50% of liver volume has been proposed as optimal, but not evaluated using long multifenestrated plastic stents (MFPS) or self-expanding metal stents (SEMS). We evaluated outcomes of endoscopic drainage of malignant hilar strictures using optimal strategy and stents, and determined factors associated with stent patency, survival, and complications. Cross-sectional study was conducted at an academic center over 5 years. MFPS (10 French or 8.5 French) or open-cell SEMS were used for palliation of unresectable malignant hilar strictures, with imaging-targeted drainage of as many sectors as needed to drain >50% of viable liver volume. Risk factors were evaluated using regression analysis. The cumulative risk was assessed using Kaplan-Meier analysis. 77 patients with malignant hilar biliary strictures (median Bismuth IV) underwent targeted stenting (41 MFPS and 36 SEMS). Comparing MFPS vs. SEMS, technical success (95.1 vs. 97.2%, P=0.64), clinical success (75.6 vs. 83.3%, P=0.40), frequency of multiple stents (23/41 vs. 25/36, P=0.19), survival and adverse events were similar, but stent patency was significantly shorter (P50% of viable liver resulted in effective palliation in patients with complex malignant hilar biliary strictures. Patency was shorter in the MFPS group, but similar survival and complications were found when comparing MFPS and SEMS group.

PIXE analysis of hilar gland for the evaluation of personal history of exposure to environmental contaminants

International Nuclear Information System (INIS)

Adachi, Shuichi; Takemoto, Kazuo; Sasa, Yoshihiko; Maeda, Kunihiko.

1992-01-01

Seven autopsied cases were studied for the evaluation of individual history of exposure to environmental contaminants based on the elemental profile of hilar gland by PIXE and lung tissue by AAS. The results well characterized their occupational history, especially from Cr, Al, Si and As detection. Moreover, it might be possible that these analyses would provide data to suspect the chemical form of each element in deposited particles of lung parenchyma, since the solubility could reflect the different profile of element between hilar gland and lung parenchyma. In addition to these retrospective analyses using the materials obtained from autopsies, it is possible to assess the individual risk using small quantity of hilar gland or lung tissue obtained by biopsy or surgical resection. (author)

Hilar and mediastinal lymph node metastases from lung cancer

International Nuclear Information System (INIS)

Koganemaru, Michihiko; Fujimoto, Kiminori; Nishimura, Hiroshi; Tabuchi, Akinori; Ohtake, Hisashi; Nishimura, Yutaka

1990-01-01

Sixty-one patients with primary lung cancer, who had CT and MR imaging before surgery, were studied. MR imaging used spin-echo sequences with T1 weighted image and T2 weighted image (C-MRI), and included STIR technique (Stir-MRI). The accuracy of diagnosis of mediastinal lymphadenopathy (regarding 10 mm or larger in short transverse diameter as positive) was 89% by CT, 84% by C-MRI and 89% by Stir-MRI. The accuracy of diagnosis of hilar lymphadenopathy was 42% by CT, 67% by C-MRI, and 75% by Stir-MRI. The accuracy of diagnosis of mediastinal lymph node metastases was 89% by CT, 94% by C-MRI and 96% by Stir-MRI. The accuracy of diagnosis of hilar lymph node metastases was 78% by CT, 87% by C-MRI and 89% by Stir-MRI. The possibility of enhancement of diagnostic accuracy of lymph node metastases from lung cancer was suggested by combining MR imaging including STIR technique, with CT. (author)

Analysis of different ways of drainage for obstructive jaundice caused by hilar cholangiocarcinoma.

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Xu, Chuan; Lv, Peng-Hua; Huang, Xin-En; Wang, Shu-Xiang; Sun, Ling; Wang, Fu-An

2014-01-01

To evaluate the prognosis of different ways of drainage for patients with obstructive jaundice caused by hilar cholangiocarcinoma. During the period of January 2006- March 2012, percutaneous transhepatic catheter drainage (PTCD)/ percutaneous transhepatic biliary stenting (PTBS) were performed for 89 patients. According to percutaneous transhepatic cholangiography (PTC), external drainage was selected if the region of obstruction could not be passed by guide wire or a metallic stent was inserted if it could. External drainage was the first choice if infection was diagnosed before the procedure, and a metallic stent was inserted in one week after the infection was under control. Selection by new infections, the degree of bilirubin decrease, the change of ALT, the time of recurrence of obstruction, and the survival time of patients as the parameters was conducted to evaluate the methods of different interventional treatments regarding prognosis of patients with hilar obstruction caused by hilar cholangiocarcinoma. PTCD was conducted in 6 patients and PTBS in 7 (pPTBS was 243 days (pPTBS was found to be better than PTCD for prolonging the patient survival.

Use of the Satinsky clamp for hilar clamping during robotic partial nephrectomy: indications, technique, and multi-center outcomes.

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Abdullah, Newaj; Rahbar, Haider; Barod, Ravi; Dalela, Deepansh; Larson, Jeff; Johnson, Michael; Mass, Alon; Zargar, Homayoun; Kaouk, Jihad; Allaf, Mohamad; Bhayani, Sam; Stifelman, Michael; Rogers, Craig

2017-03-01

A Satinsky clamp may be a backup option for hilar clamping during robotic partial nephrectomy (RPN) if there are challenges with application of bulldog clamps, but there are potential safety concerns. We evaluate outcomes of RPN using Satinsky vs. bulldog clamps, and provide tips for safe use of the Satinsky as a backup option. Using a multi-center database, we identified 1073 patients who underwent RPN between 2006 and 2013, and had information available about method of hilar clamping (bulldog clamp vs. Satinsky clamp). Patient baseline characteristics, tumor features, and perioperative outcomes were compared between the Satinsky and bulldog clamp groups. A Satinsky clamp was used for hilar clamping in 94 (8.8 %) RPN cases, and bulldog clamps were used in 979 (91.2 %) cases. The use of a Satinsky clamp was associated with greater operative time (198 vs. 175 min, p hilar clamping during challenging RPN cases, but requires careful technique, and was rarely necessary.

Enhancement pattern of hilar cholangiocarcinoma: Contrast-enhanced ultrasound versus contrast-enhanced computed tomography

International Nuclear Information System (INIS)

Xu Huixiong; Chen Lida; Xie Xiaoyan; Xie Xiaohua; Xu Zuofeng; Liu Guangjian; Lin Manxia; Wang Zhu; Lu Mingde

2010-01-01

Objective: To compare the enhancement pattern of hilar cholangiocarcinoma on contrast-enhanced ultrasound (CEUS) with that on contrast-enhanced computed tomography (CECT). Methods: Thirty-two consecutive patients with pathologically proven hilar cholangiocarcinomas were evaluated by both low mechanical index CEUS and CECT. The enhancement feature of the tumor, portal vein infiltration, and lesion conspicuity on them was investigated. Results: In the arterial phase, the numbers of the lesions showing hyperenhancement, isoenhancement, and hypoenhancement, were 14 (43.8%), 14 (43.8%), and 4 (12.6%), on CEUS, and 12 (37.5%), 9 (28.1%), and 11 (34.4%), on CECT (P = 0.162). In portal phase, the numbers of the lesions showing hypoenhancement, isoenhancement, and hyperenhancement were 30 (93.8%), 1 (3.1%), and 1 (3.1%), on CEUS, and 23 (71.9%), 8 (25.0%), and 1 (3.1%), on CECT (P = 0.046). The detection rates for portal vein infiltration were 84.2% (16/19) for baseline ultrasound, 89.5% (17/19) for CEUS, and 78.9% (15/19) for CECT (all P > 0.05 between every two groups). CEUS significantly improved the lesion conspicuity in comparison with CECT. CEUS and CECT made correct diagnoses in 30 (93.8%) and 25 (78.1%) lesions prior to pathological examination (P = 0.125). Conclusion: The enhancement pattern of hilar cholangiocarcinoma on CEUS was similar with that on CECT in arterial phase, whereas in portal phase hilar cholangiocarcinoma shows hypoenhancement more likely on CEUS. CEUS and CECT lead to similar results in evaluating portal vein infiltration and diagnosis of this entity.

Enhancement pattern of hilar cholangiocarcinoma: Contrast-enhanced ultrasound versus contrast-enhanced computed tomography

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Xu Huixiong, E-mail: xuhuixiong@hotmail.co [Department of Medical Ultrasonics, The First Affiliated Hospital, Institute of Diagnostic and Interventional Ultrasound, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080 (China); Chen Lida; Xie Xiaoyan; Xie Xiaohua; Xu Zuofeng; Liu Guangjian; Lin Manxia; Wang Zhu [Department of Medical Ultrasonics, The First Affiliated Hospital, Institute of Diagnostic and Interventional Ultrasound, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080 (China); Lu Mingde, E-mail: lumd@21cn.co [Department of Hepatobiliary Surgery, First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080 (China)

2010-08-15

Objective: To compare the enhancement pattern of hilar cholangiocarcinoma on contrast-enhanced ultrasound (CEUS) with that on contrast-enhanced computed tomography (CECT). Methods: Thirty-two consecutive patients with pathologically proven hilar cholangiocarcinomas were evaluated by both low mechanical index CEUS and CECT. The enhancement feature of the tumor, portal vein infiltration, and lesion conspicuity on them was investigated. Results: In the arterial phase, the numbers of the lesions showing hyperenhancement, isoenhancement, and hypoenhancement, were 14 (43.8%), 14 (43.8%), and 4 (12.6%), on CEUS, and 12 (37.5%), 9 (28.1%), and 11 (34.4%), on CECT (P = 0.162). In portal phase, the numbers of the lesions showing hypoenhancement, isoenhancement, and hyperenhancement were 30 (93.8%), 1 (3.1%), and 1 (3.1%), on CEUS, and 23 (71.9%), 8 (25.0%), and 1 (3.1%), on CECT (P = 0.046). The detection rates for portal vein infiltration were 84.2% (16/19) for baseline ultrasound, 89.5% (17/19) for CEUS, and 78.9% (15/19) for CECT (all P > 0.05 between every two groups). CEUS significantly improved the lesion conspicuity in comparison with CECT. CEUS and CECT made correct diagnoses in 30 (93.8%) and 25 (78.1%) lesions prior to pathological examination (P = 0.125). Conclusion: The enhancement pattern of hilar cholangiocarcinoma on CEUS was similar with that on CECT in arterial phase, whereas in portal phase hilar cholangiocarcinoma shows hypoenhancement more likely on CEUS. CEUS and CECT lead to similar results in evaluating portal vein infiltration and diagnosis of this entity.

Pheochromocytoma with Markedly Abnormal Liver Function Tests and Severe Leukocytosis

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Chai Ryoung Eun

2014-03-01

Full Text Available Pheochromocytoma is a rare neuroendocrine tumor arising from the medulla of the adrenal glands, which causes an overproduction of catecholamines. The common symptoms are headache, palpitations, and sweating; however, various other clinical manifestations might also be present. Accurate diagnosis of pheochromocytoma is important because surgical treatment is usually successful, and associated clinical problems are reversible if treated early. A 49-year-old man with a history of uncontrolled hypertension and diabetes mellitus presented with chest pain, fever, and sweating. His liver function tests and white blood cell counts were markedly increased and his echocardiography results suggested stress-induced cardiomyopathy. His abdominal computed tomography showed a 5×5-cm-sized tumor in the left adrenal gland, and laboratory tests confirmed catecholamine overproduction. After surgical resection of the left adrenal gland, his liver function tests and white blood cell counts normalized, and echocardiography showed normal cardiac function. Moreover, his previous antihypertensive regimen was deescalated, and his previously uncontrolled blood glucose levels normalized without medication.

Stage of hilar cholangiocarcinoma predicts recurrence of biliary obstruction in patients with metal stents.

Science.gov (United States)

Siddiqui, Ali; Shahid, Haroon; Sarkar, Avik; Cox, Kristen; Kowalski, Thomas E; Loren, David E; Sharma, Ashish; Laing, Patrick; Birch, Madeline; Adler, Douglas G

2013-09-01

Most patients with hilar cholangiocarcinomas present with unresectable tumors, so only palliative biliary drainage with self-expanding metal stents (SEMS) is possible. Stents eventually cease to function because of tumor overgrowth and/or other causes, so it is important to identify factors that affect stent patency and failure. We examined the patency of endoscopically placed SEMS in patients with hilar cholangiocarcinoma and factors associated with patency. We performed a retrospective study of 120 consecutive patients (mean age, 67 ± 14.6 years; 74 male) who presented with obstructive jaundice from hilar cholangiocarcinoma and underwent bilateral SEMS from September 2006 through April 2012 at 2 US tertiary medical centers. We collected data on patient demographics and survival, success of stent placement and function, and immediate adverse events. The primary outcome was duration of stent patency (time from insertion to failure). Thirty-eight patients had stage 1 hilar cholangiocarcinomas, 45 had stage 2, 12 had stage 3, and 25 had stage 4. The median length of the hilar stricture was 9 mm (range, 8-50 mm). The stent was successfully passaged across the stricture in all patients and was functional in 115; its median length was 8 mm (range, 8-10 mm), and diameter was 80 mm (range, 60-100 mm). Fourteen patients had immediate adverse events, including perforation (n = 2), bleeding (n = 2), pancreatitis (n = 9), and cholangitis (n = 1). Median survival was 17 weeks (range, 1-211 weeks), and 50 patients had stent occlusion. On Kaplan-Meier analysis, the median time from stent placement to occlusion was 17 weeks (range, 1-104 weeks). More patients with stage 3 or 4 tumors (64%) had SEMS occlusion than patients with stage 1 or 2 tumors (28%) in univariate analysis (P = .017). In multivariate analysis, only cancer stage was independently and significantly associated with patency (P = .006; hazard ratio, 2.77); age, sex, length of stricture, and SEMS diameter and

Hilar granule cells of the mouse dentate gyrus: effects of age, septotemporal location, strain, and selective deletion of the proapoptotic gene BAX.

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Bermudez-Hernandez, Keria; Lu, Yi-Ling; Moretto, Jillian; Jain, Swati; LaFrancois, John J; Duffy, Aine M; Scharfman, Helen E

2017-09-01

The dentate gyrus (DG) principal cells are glutamatergic granule cells (GCs), and they are located in a compact cell layer. However, GCs are also present in the adjacent hilar region, but have been described in only a few studies. Therefore, we used the transcription factor prospero homeobox 1 (Prox1) to quantify GCs at postnatal day (PND) 16, 30, and 60 in a common mouse strain, C57BL/6J mice. At PND16, there was a large population of Prox1-immunoreactive (ir) hilar cells, with more in the septal than temporal hippocampus. At PND30 and 60, the size of the hilar Prox1-ir cell population was reduced. Similar numbers of hilar Prox1-expressing cells were observed in PND30 and 60 Swiss Webster mice. Prox1 is usually considered to be a marker of postmitotic GCs. However, many Prox1-ir hilar cells, especially at PND16, were not double-labeled with NeuN, a marker typically found in mature neurons. Most hilar Prox1-positive cells at PND16 co-expressed doublecortin (DCX) and calretinin, markers of immature GCs. Double-labeling with a marker of actively dividing cells, Ki67, was not detected. These results suggest that, surprisingly, a large population of cells in the hilus at PND16 are immature GCs (Type 2b and Type 3 cells). We also asked whether hilar Prox1-ir cell numbers are modifiable. To examine this issue, we conditionally deleted the proapoptotic gene BAX in Nestin-expressing cells at a time when there are numerous immature GCs in the hilus, PND2-8. When these mice were examined at PND60, the numbers of Prox1-ir hilar cells were significantly increased compared to control mice. However, deletion of BAX did not appear to change the proportion that co-expressed NeuN, suggesting that the size of the hilar Prox1-expressing population is modifiable. However, deleting BAX, a major developmental disruption, does not appear to change the proportion that ultimately becomes neurons.

Bone Resorption Is Increased in Pheochromocytoma Patients and Normalizes following Adrenalectomy

NARCIS (Netherlands)

Veldhuis-Vlug, A.G.; El Mahdiui, M.; Endert, E.; Heijboer, A.C.; Fliers, E.; Bisschop, P.H.

2012-01-01

Context: The sympathetic nervous system (SNS) controls bone turnover in rodents, but it is uncertain whether a similar role for the SNS exists in humans. Pheochromocytomas are catecholamine-producing neuroendocrine tumors. Because catecholamines are the neurotransmitters of the SNS, we hypothesized

Clinical and laboratory features of pheochromocytoma in a 52-year-old female patient

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Otmara Aranguren Barreto

2014-04-01

Full Text Available The article aims to describe the clinical and laboratory features of a female patient suffering from pheochromocytoma. The case is a 52-year-old female patient who presents to our healthcare center with high blood pressure, cold limbs, sweating, jitteriness, and episodes of oppressive chest pain that appear several times per day. She also reports fatigue and a 13-kilogram weight loss. The sonogram revealed a nodular image in the right adrenal gland that had low echogenicity and regular margins measuring 5 mm. The image was confirmed with a contrast-enhanced adrenal CAT scan. Urine vanillylmandelic acid levels were high and an adrenal biopsy confirmed a pheochromocytoma measuring 4.5 x 3.5 x 3 cm.

The radioenzymatic determination of adrenaline and noradrenaline in plasma and its use in the diagnostic of pheochromocytomas

International Nuclear Information System (INIS)

Neuhaus, C.P.E.

1982-01-01

The radioenzymatic determination of adrenaline and noradrenaline in human plasma for the diagnosis of pheochromocytomas was put to use after improvements were made with respect to extraction and separation steps. The plasma catecholamines at rest were distinctly higher in patients with pheochromocytomas. The plasma catecholamine level showed a significant increase as well with the glucagon test between the second and fifth minute. The method was not well suited for the localisation diagnostic where the plasma catecholamines were determined in selectively taken blood from the lower vena cava. Overall, however, the radioenzymatic determination of catecholamines in plasma proved itself to be a relatively ponderous, but exact and sensitive method for the measuring of basal catecholamine level and its changes. In the clinical area it is used as a valuable supplement to the contemporary diagnostic of pheochromocytomas. (orig./TRV) [de

Infiltration of peritumoural but tumour-free parenchyma with IgG4-positive plasma cells in hilar cholangiocarcinoma and pancreatic adenocarcinoma.

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Resheq, Yazid J; Quaas, Alexander; von Renteln, Daniel; Schramm, Christoph; Lohse, Ansgar W; Lüth, Stefan

2013-10-01

Recently, new guidelines for diagnosing IgG4-associated cholangitis have been published devaluing the diagnostic significance of IgG4-positive plasma cells and steroid trials. We sought to evaluate the utility of IgG4-positive plasma cells in discriminating IgG4-associated cholangitis from hilar cholangiocarcinoma and autoimmune pancreatitis from pancreatic adenocarcinoma under conditions when malignancy is likely to be missed. Resection specimens obtained from patients with hilar cholangiocarcinoma, pancreatic adenocarcinoma or hepatocellular carcinoma were re-evaluated for IgG4-positivity. Histological analysis focussed on peritumoural but tumour-free sections. Perioperative biochemical and clinical data were reviewed. Nineteen patients with hilar cholangiocarcinoma and 29 patients with pancreatic adenocarcinoma were eligible for histological re-evaluation. Six of 19 (32%) patients with hilar cholangiocarcinoma and 5 of 29 (17%) patients with pancreatic adenocarcinoma were IgG4-positive (≥20 IgG4-positive plasma cells per high power field). Patients with IgG4-positive hilar cholangiocarcinoma showed significantly higher levels of serum total bilirubin (3.6mg/dl vs. 1.8mg/dl; Philar cholangiocarcinoma. IgG4-positive plasma cells are of limited utility especially in distinguishing hilar cholangiocarcinoma from IgG4-associated cholangitis even when combined with clinical parameters and may be misleading under conditions when malignancy is missed. Copyright © 2013 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

A systematic review of the literature examining the diagnostic efficacy of measurement of fractionated plasma free metanephrines in the biochemical diagnosis of pheochromocytoma

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Thabane Lehana

2004-06-01

Full Text Available Abstract Background Fractionated plasma metanephrine measurements are commonly used in biochemical testing in search of pheochromocytoma. Methods We aimed to critically appraise the diagnostic efficacy of fractionated plasma free metanephrine measurements in detecting pheochromocytoma. Nine electronic databases, meeting abstracts, and the Science Citation Index were searched and supplemented with previously unpublished data. Methodologic and reporting quality was independently assessed by two endocrinologists using a checklist developed by the Standards for Reporting of Diagnostic Studies Accuracy Group and data were independently abstracted. Results Limitations in methodologic quality were noted in all studies. In all subjects (including those with genetic predisposition: the sensitivities for detection of pheochromocytoma were 96%–100% (95% CI ranged from 82% to 100%, whereas the specificities were 85%–100% (95% CI ranged from 78% to 100%. Statistical heterogeneity was noted upon pooling positive likelihood ratios when those with predisposition to disease were included (p Conclusion Negative plasma fractionated free metanephrine measurements are effective in ruling out pheochromocytoma. However, a positive test result only moderately increases suspicion of disease, particularly when screening for sporadic pheochromocytoma.

Recurrence of pheochromocytoma in 11 years old school child, diagnosed by I-131 MIBG

International Nuclear Information System (INIS)

Jara Yorg, J. A; Arias, Cohl; Gimenez, J; Rodriguez, E; Ortiz, L; Mendieta, B; Ayala, R. M; Rodriguez, C; Delgadillo, J. L

2000-01-01

Pheocromocytoma is a paraganglioma with an incidence of arterial hypertension approximately of 1%,but its diagnosis has several important issues from the clinical point of view. 1-The tumor resection frequently cure the hypertension. 2.-Its manifestations might simulates other diseases like carcinoid Sx, hypertiroidism,etc. 3.-It is a familiar disease transmitted by an autosomic dominant way. It is 10% bilateral,10%extraadrenal,10% occur in children and 10% are malignant. We present a case of pheochromocytoma recurrency in a young girl,11 y.o. operated 8 months before, at the Clinical Hospital, National University of Paraguay, School of Medicine for a right suprarenal gland pheochromocytoma of 2cms of diameter, who consults the Pediatric Department for arterial hypertension and cefalea. She also had a Von Hippel Sx and Glaucoma. Nuclear Medicine is a non invasive method that use the I-131 Methayodobencylguanidine(MIBG-I-131) with high accuracy to diagnose and treat both neuroblastoma and pheochromocytoma I-131 MIBG is the gold standard for the diagnosis of both entities with a sensitivity between 94-100%6-7 and specificity of 100% being the best method to evaluate these diseases in the pre and post operatory (Au)

Magnesium sulphate and (123)I-MIBG in pheochromocytoma: Two useful techniques for a complicated disease.

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Vendrell, M; Martín, N; Tejedor, A; Ortiz, J T; Muxí, À; Taurà, P

2016-01-01

Pheochromocytoma is a tumour of the chromaffin tissue. It may, through catecholamine release, have deleterious effects on myocardial structure. A 48-year-old woman with a history of hypertension and type II diabetes mellitus (ASA II) was diagnosed of pheochromocytoma-induced myocarditis, which caused severe cardiogenic shock, with an ejection fraction of 20%. Extreme blood pressure swings required aggressive therapy with vasoactive drugs (norepinephrine and dopamine) and an intra-aortic balloon pump, despite which severe haemodynamic instability persisted. Finally, the use of magnesium sulphate allowed for cardiovascular stabilization and weaning off vasoactive drugs prior to surgery. (123)I-metaiodobenzylguanidine scintigraphy helps not only to functionally confirm tumour tissue, but also to assess severity and prognosis of cardiac failure. Prognosis of pheochromocytoma-induced heart failure can be very poor. The use of these two well-known and relatively simple 'tools' for treatment and prognosis is a helpful option to keep in mind. Copyright © 2014 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

Unilateral versus bilateral stent insertion for malignant hilar biliary obstruction.

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Chang, Gang; Xia, Feng-Fei; Li, Hong-Fu; Niu, Su; Xu, Yuan-Shun

2017-11-01

To determine the clinical efficiency and long-term outcomes between unilateral and bilateral stent insertion in patients with malignant hilar biliary obstruction. From August 2012 to February 2016, 63 consecutive patients with malignant hilar biliary obstruction were treated with unilateral or bilateral stent insertion at our center. The bilateral stents were inserted using the side-by-side technique. The clinical efficiency and long-term outcomes were compared between the two groups. Unilateral and bilateral stent insertions were successfully performed in 31 of 33 and 27 of 30 patients, respectively (P = 0.912). No procedure-related complication occurred. Clinical success was achieved in 29 of 31 patients in the unilateral stent group and in 26 of 27 patients in the bilateral stent group (P = 0.637). During the follow-up, re-obstruction of stent occurred in five patients in the unilateral stent group and in three patients in the bilateral stent group (P = 0.58). The significant differences were not observed in the stent patency time (368 vs. 387 days, P = 0.685) and survival (200 vs. 198 days, P = 0.751) between two groups. Based on the univariate and multivariate analyses, the independent risk factors for decreasing the survival time included higher Eastern Cooperative Oncology Group performance status (P = 0.018), higher alanine aminotransferase level (P = 0.009), and absence of anticancer treatment after stent insertion (P = 0.002). Compared to bilateral stent insertion for malignant hilar biliary obstruction, unilateral stent insertion can provide comparable clinical efficiency and long-term outcomes.

Comparison of Digital Tomosynthesis and Chest Radiography for the Detection of Noncalcified Pulmonary and Hilar Lesions.

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Galea, Angela; Adlan, Tarig; Gay, David; Roobottom, Carl; Dubbins, Paul; Riordan, Richard

2015-09-01

The aim of this study was to compare the sensitivity and specificity of chest digital tomosynthesis (DTS) with chest radiography (CXR) for the detection of noncalcified pulmonary nodules and hilar lesions using computed tomography (CT) as the reference standard. A total of 78 patients with suspected noncalcified pulmonary lesions on CXR were included in the study. Two radiologists, blinded to the history and CT, analyzed the CXR and the DTS images (separately), whereas a third radiologist analyzed the CXR and DTS images together. Noncalcified intrapulmonary nodules and hilar lesions were recorded for analysis. The interobserver agreement for CXR and DTS was assessed, and the time taken to report the images was recorded. A total of 202 lesions were recorded in 78 patients. There were 111 true lesions confirmed on CT in 53 patients; in 25 patients subsequent CT excluded a lesion. The overall sensitivity was 32% for CXR and 49% for DTS. This improved to 54% when the posteroanterior CXR and DTS were reviewed together (CXR-DTS). The overall specificities for CXR, DTS, and CXR-DTS were 49%, 96%, and 98%, respectively. There were 56 suspected hilar lesions with subgroup sensitivities of 76% for CXR, 65% for DTS, and 76% for CXR-DTS. The specificity for hilar lesions was 59%, 92%, and 97% for CXR, DTS, and CXR-DTS, respectively. DTS significantly improves the detectability of noncalcified nodules when compared with and when used in combination with CXR. The specificity and interobserver agreement of DTS in the diagnosis of suspected noncalcified pulmonary nodules and hilar lesions are significantly better than those of CXR and approaches those of CT.

Lymph Node Micrometastases are Associated with Worse Survival in Patients with Otherwise Node-Negative Hilar Cholangiocarcinoma.

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Mantel, Hendrik T J; Wiggers, Jim K; Verheij, Joanne; Doff, Jan J; Sieders, Egbert; van Gulik, Thomas M; Gouw, Annette S H; Porte, Robert J

2015-12-01

Lymph node metastases on routine histology are a strong negative predictor for survival after resection of hilar cholangiocarcinoma. Additional immunohistochemistry can detect lymph node micrometastases in patients who are otherwise node negative, but the prognostic value is unsure. The objective of this study was to assess the effect on survival of immunohistochemically detected lymph node micrometastases in patients with node-negative (pN0) hilar cholangiocarcinoma on routine histology. Between 1990 and 2010, a total of 146 patients underwent curative-intent resection of hilar cholangiocarcinoma with regional lymphadenectomy at two university medical centers in the Netherlands. Ninety-one patients (62 %) without lymph node metastases at routine histology were included. Micrometastases were identified by multiple sectioning of all lymph nodes and additional immunostaining with an antibody against cytokeratin 19 (K19). The association with overall survival was assessed in univariable and multivariable analysis. Median follow-up was 48 months. Micrometastases were identified in 16 (5 %) of 324 lymph nodes, corresponding to 11 (12 %) of 91 patients. There were no differences in clinical variables between K19 lymph node-positive and -negative patients. Five-year survival rates in patients with lymph node micrometastases were significantly lower compared to patients without micrometastases (27 vs. 54 %, P = 0.01). Multivariable analysis confirmed micrometastases as an independent prognostic factor for survival (adjusted Hazard ratio 2.4, P = 0.02). Lymph node micrometastases are associated with worse survival after resection of hilar cholangiocarcinoma. Immunohistochemical detection of lymph node micrometastases leads to better staging of patients who were initially diagnosed with node-negative (pN0) hilar cholangiocarcinoma on routine histology.

Does the type of renal artery anatomic variant determine the diameter of the main vessel supplying a kidney? A study based on CT data with a particular focus on the presence of multiple renal arteries.

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Majos, Marcin; Stefańczyk, Ludomir; Szemraj-Rogucka, Zofia; Elgalal, Marcin; De Caro, Raffaele; Macchi, Veronica; Polguj, Michał

2018-04-01

An in-depth knowledge of renal vascular anatomy is essential when planning many surgical procedures; however, a few data exists regarding renal artery diameter. The aim of this study was to assess this morphological feature and to investigate whether a correlation exists between renal artery diameter and the type of arterial supply, with a particular emphasis on variant anatomy and the presence of multiple renal arteries. Computed tomography angiography (CTA) studies of 248 patients, i.e., a total of 496 kidneys, were evaluated. The mean age of the patients was 66.4 ± 15.01 years. Renal artery diameter was measured based on the type of arterial blood supply. The frequency of occurrence of three anatomic variants of renal arterial supply was established: single renal artery (RA) 43.35%, single artery with prehilar branching (pRA) 37.30%, and multiple renal artery (mRA) 19.35%. The diameter of single renal arteries, with either prehilar or hilar branching, was significantly larger than when multiple arteries were present. A detailed analysis of just the mRA variant demonstrated that the diameter of the renal arteries in men was larger (p = 0.012) than those in women and that there was no difference in diameter with regard to the side of the body (p = 0.219). The classification described in our study containing a detailed description of renal artery diameter. It may be helpful in clinical practice, especially for transplantologists, surgeons, and vascular surgeons.

Transhepatic Hilar Approach for Perihilar Cholangiocarcinoma: Significance of Early Judgment of Resectability and Safe Vascular Reconstruction.

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Kuriyama, Naohisa; Isaji, Shuji; Tanemura, Akihiro; Iizawa, Yusuke; Kato, Hiroyuki; Murata, Yasuhiro; Azumi, Yoshinori; Kishiwada, Masashi; Mizuno, Shugo; Usui, Masanobu; Sakurai, Hiroyuki

2017-03-01

In the most common surgical procedure for perihilar cholangiocarcinoma, the margin status of the proximal bile duct is determined at the final step. Our procedure, the transhepatic hilar approach, confirms a cancer-negative margin status of the proximal bile duct first. We first performed a partial hepatic parenchymal transection to expose the hilar plate, and then transected the proximal bile duct to confirm margin status. Then, divisions of the hepatic artery and portal vein of the future resected liver are performed, followed by the residual hepatic parenchymal transection. The transhepatic hilar approach offers a wide surgical field for safe resection and reconstruction of the portal vein in the middle of the hepatectomy. We reviewed 23 patients with perihilar cholangiocarcinoma who underwent major hepatectomy using our procedure from 2011 to 2015. A combined vascular resection and reconstruction was carried out in 14 patients (60.9%). R0 resection was achieved in 17 patients (73.9%), and the overall 3-year survival rate was 52.9% (median survival time 52.4 months). The transhepatic hilar approach is useful and practicable regardless of local tumor extension, enabling us to determine tumor resectability and perform safe resection and reconstruction of the portal vein early in the operation.

Percutaneous metallic self-expandable endoprostheses in malignant hilar biliary obstruction

NARCIS (Netherlands)

Stoker, J.; Laméris, J. S.; van Blankenstein, M.

1993-01-01

Forty-five patients with malignant hilar obstruction were treated with a total of 68 percutaneously inserted metallic self-expandable endoprostheses (Wallstents) for palliative biliary drainage. The stent diameter was 1 cm; the length was 3.5 to 10.5 cm. Early complications occurred in seven

How to deal with benign hilar or interlobar lymphadenopathy during video-assisted thoracoscopic surgery lobectomy.

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Yan, Shi; Lv, Chao; Wang, Xing; Wu, Nan

2016-01-01

Video-assisted thoracoscopic surgery (VATS) surgery has changed the way lobectomy procedure was performed over the past few decades. However, some difficulties impede the accomplishment of VATS lobectomy, which of them, benign lymphadenopathy may pose a threat to safety of surgery. We reported a case with enlarged hilar and interlobar lymph nodes. The video showed the instrumentation and techniques that we had adopted to deal with the complicated dilemma during the operation. Critical experience was also suggested in some hypothetical scenarios. AS techniques were further refined, successful VATS segmentectomy or lobectomy with challenging hilar or interlobar lymphadenopathy could be performed without uncontrolled bleeding or unexpected conversion. A VATS approach is acceptable in the management of benign hilar or interlobar lymphadenopathy. However, facile technique is necessary to deal with intraoperative dilemma. To those who are not sure about the practicability of the VATS procedure, planned conversion is still an effective method to ensure safety of the operation.

ACTH-producing pheochromocytoma: clinical considerations and concise review of the literature

NARCIS (Netherlands)

Nijhoff, M. F.; Dekkers, O. M.; Vleming, L. J.; Smit, J. W. A.; Romijn, J. A.; Pereira, A. M.

2009-01-01

We present a patient with a rare cause of ectopic ACTH-dependent Cushing's syndrome, caused by a pheochromocytoma. The case provides clues for a detailed discussion on the pitfalls and diagnostic difficulties in establishing the correct underlying cause of ACTH-dependent Cushing's syndrome. It

Brain Injury-Induced Synaptic Reorganization in Hilar Inhibitory Neurons Is Differentially Suppressed by Rapamycin.

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Butler, Corwin R; Boychuk, Jeffery A; Smith, Bret N

2017-01-01

Following traumatic brain injury (TBI), treatment with rapamycin suppresses mammalian (mechanistic) target of rapamycin (mTOR) activity and specific components of hippocampal synaptic reorganization associated with altered cortical excitability and seizure susceptibility. Reemergence of seizures after cessation of rapamycin treatment suggests, however, an incomplete suppression of epileptogenesis. Hilar inhibitory interneurons regulate dentate granule cell (DGC) activity, and de novo synaptic input from both DGCs and CA3 pyramidal cells after TBI increases their excitability but effects of rapamycin treatment on the injury-induced plasticity of interneurons is only partially described. Using transgenic mice in which enhanced green fluorescent protein (eGFP) is expressed in the somatostatinergic subset of hilar inhibitory interneurons, we tested the effect of daily systemic rapamycin treatment (3 mg/kg) on the excitability of hilar inhibitory interneurons after controlled cortical impact (CCI)-induced focal brain injury. Rapamycin treatment reduced, but did not normalize, the injury-induced increase in excitability of surviving eGFP+ hilar interneurons. The injury-induced increase in response to selective glutamate photostimulation of DGCs was reduced to normal levels after mTOR inhibition, but the postinjury increase in synaptic excitation arising from CA3 pyramidal cell activity was unaffected by rapamycin treatment. The incomplete suppression of synaptic reorganization in inhibitory circuits after brain injury could contribute to hippocampal hyperexcitability and the eventual reemergence of the epileptogenic process upon cessation of mTOR inhibition. Further, the cell-selective effect of mTOR inhibition on synaptic reorganization after CCI suggests possible mechanisms by which rapamycin treatment modifies epileptogenesis in some models but not others.

A retrospective study of secondary diabetes prevalence in Pheochromocytoma, Cushing and Acromegal patients

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Bastan Hagh M

1997-07-01

Full Text Available Some of the endocrinologic diseases, especially Acromegaly, Cushing and Pheochromocytoma have multiple effects on blood glucose metabolism and regulation in non-diabetic patients. In this retrospective survey, records of patients of Tehran Medical Sciences University hospitals have been reviewd. Of 124 Acromegals, GTT was performed for 51 patients, being impaired in 18%. To evaluate diabetes, FBS and BS of 90 patients were checked, overt diabetes was detected in 27%. Among 90 Cushing patients, blood glucose was checked in 60 cases, 47% of these patients had levels above the normal range, and 39% had glucosuria. Among 80 Pheochromocytoma patients, 16 cases (26.5% had overt diabetes. In comparison with other studied, we have obtained a little different results concerning diabetes and impaired GTT prevalence

Two stents insertion via single tract for treatment of hepatic hilar cholangiocarcinoma

International Nuclear Information System (INIS)

Xie Zonggui; Jin Peng; Xie Zhiyong; Yi Yuhai; Zhang Xuping

2003-01-01

Objective: To evaluate the feasibility and clinical application of two stents insertion via single tract for treatment of hepatic hilar cholangiocarcinoma. Methods: Eighteen patients with hepatic hilar cholangiocarcinoma who had left and right bile duct obstruction were treated with stents insertion via right bile duct puncturing routeway. These two stents were implanted between right and left bile duct, and between right bile duct and common bile duct. Results: Eighteen patients obtained successful two stents placement by right bile duct puncturing tract and succeeded with internal drainage for all biliary tree jaundice subsided distinctly. Conclusions: The technique of two stents insertion via single tract could predigest interventional drainage procedure of high bile duct obstruction, reduce operation trauma, shorten handling time and possess promising application value

Management of advanced hilar biliary malignancy with X-shaped stenting technique

International Nuclear Information System (INIS)

Hwang, Gyu Hyuk; You, Jin Jong; Ahn, In Oak; Na, Jae Boem; Chung, Sugn Hoon

2000-01-01

To report X-shaped stent insertion and its result in the patients with advanced hilar malignancy. X-shaped stents were inserted in six patients with advanced hilar malignancy involving segmental branches of both intrahepatic bile ducts (IHD). The causes were cholangiocarcinomas in five patients and recurrent GB cancer in one. The procedure includes three steps: 1) the insertion of two wires through three IHDs in an X configuration, using a stone basket; 2) balloon dilatation of lesions, and 3) the insertion of two stents in an as X configuration. Stents were inserted after balloon dilatation in five patients, and without balloon dilatation in one. Changes in serum bilirubin levels and procedure-related problems were reviewed. In all patients, serum bilirubin levels gradually decreased, but in two, they increased again. One of these two died of sepsis after 1 month. There was bile leakage through the puncture and bile was extracted from malignant ascites. In the other patient, occlusion of the left stent tip occurred, and additional left PTBD was performed 3 months later. Hemobilia developed in all five patients with balloon dilatation, these all experienced pain during dilatation, but afterwards this disappeared. One stent without pre-balloon dilation showed incomplete self-expansion at the crossing part and supplementary balloon dilatations were performed. In patients with advanced hilar malignancy,X-shaped stent insertion is a new palliation. Problems such as hemobilia, pain, and intraperitoneal bile leakage may, however, occur. (author)

Effect of early adrenal vein ligation on blood pressure and catecholeamine fluctuation during laparoscopic adrenalectomy for pheochromocytoma.

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Wu, Guojun; Zhang, Bo; Yu, Chuigong; Gao, Lei; Gao, Yang; Huang, Yi; Yu, Lei; Zhang, Geng; Yang, Lijun; Yuan, Jianlin

2013-09-01

To define whether previous control of the adrenal vein is a crucial procedure in laparoscopic adrenalectomy for pheochromocytoma. From January 2000 to December 2010, 114 patients with pheochromocytoma who underwent laparoscopic adrenalectomy through transperitoneal or retroperitoneal approach were included. The patients were divided into 2 groups randomly (group 1: dissection after ligation; group 2: dissection before ligation). Blood samples for the measurement of catecholamines levels using high performance liquid chromatography were taken at the following time points: t1, before anesthesia; t2, during manipulation-extraction of pheochromocytoma; t3, after removal of pheochromocytoma. The blood pressure fluctuation was recorded. Laparoscopic adrenalectomy was successfully performed on 113 patients with 1 elective open conversion because of dense peritumor adhesions. The operating time ranged from 80 to 150 minutes (mean 108, 102 in group 1, 110 in group 2). Mean blood loss ranged from 20 to 500 mL (mean 120 mL, 110 in group 1, 125 in group 2). The concentrations of plasma catecholamines between the 2 groups had no statistical differences. The blood pressure fluctuation incidence between the 2 groups had no marked difference. But the incidence increased with high functionary grade, and the difference was significant (P = .043). This study demonstrated that previous control of the adrenal vein was not a determinate factor in dealing with dangerous hypertension during laparoscopic adrenalectomies. Copyright © 2013 Elsevier Inc. All rights reserved.

Utility and safety of endobronchial ultrasound-guided transbronchial needle aspiration in patients with mediastinal and hilar lymphadenopathy: Western region experience.

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Aljohaney, Ahmed A

2018-01-01

The aim of the study was to evaluate the clinical utility and safety of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) in patients with mediastinal and hilar lymphadenopathy and to explicitly describe the utility of this procedure in patient's outcome. A retrospective review and analysis was conducted on 52 patients with mediastinal or hilar lymphadenopathy who underwent EBUS-TBNA from June 2012 to June 2016. All the patients were evaluated by computed tomography (CT) chest with contrast before EBUS examination. Enlarged mediastinal or hilar lymph node was defined as >1 cm short axis on the enhanced CT. Among the 52 patients studied, 57.7% were presented with mediastinal or hilar lymphadenopathy for diagnosis and 42.3% presented with suspected mediastinal malignancy. Paratracheal stations were the most common site for puncture in 33 lymph nodes (43%). The best diagnostic yield was obtained from subcarinal stations and the lowest yield from the hilar stations. Surgical biopsies confirmed lymphoma in six patients, tuberculosis (TB) in three, sarcoidosis in two and one had metastatic adenocarcinoma of unknown primary. The sensitivity, specificity, positive predictive value, and negative predictive value of EBUS-TBNA for diagnosis of mediastinal and hilar lymph node abnormalities were 78.6%, 100%, 100%, and 80%, respectively. The diagnostic yield of EBUS-TBNA in malignant and benign conditions was 79.0%. EBUS-TBNA is a safe and efficacious procedure which can be performed using conscious sedation with high yields. It can be used for the staging of malignancies as well as for the diagnosis of inflammatory and infectious conditions such as sarcoidosis and TB.

Predicted extracapsular invasion of hilar lymph node metastasis by fusion positron emission tomography/computed tomography in patients with lung cancer.

Science.gov (United States)

Makino, Takashi; Hata, Yoshinobu; Otsuka, Hajime; Koezuka, Satoshi; Isobe, Kazutoshi; Tochigi, Nobumi; Shiraga, Nobuyuki; Shibuya, Kazutoshi; Homma, Sakae; Iyoda, Akira

2015-09-01

Intraoperative detection of hilar lymph node metastasis, particularly with extracapsular invasion, may affect the surgical procedure in patients with lung cancer, as the preoperative estimation of hilar lymph node metastasis is unsatisfactory. The aim of this study was to investigate whether fusion positron emission tomography/computed tomography (PET/CT) is able to predict extracapsular invasion of hilar lymph node metastasis. Between April, 2007 and April, 2013, 509 patients with primary lung cancer underwent surgical resection at our institution, among whom 28 patients exhibiting hilar lymph node metastasis (at stations 10 and 11) were enrolled in this study. A maximum lymph node standardized uptake value of >2.5 in PET scans was interpreted as positive. A total of 17 patients had positive preoperative PET/CT findings in their hilar lymph nodes, while the remaining 11 had negative findings. With regard to extracapsular nodal invasion, the PET/CT findings (P=0.0005) and the histological findings (squamous cell carcinoma, P=0.05) were found to be significant predictors in the univariate analysis. In the multivariate analysis, the PET/CT findings were the only independent predictor (P=0.0004). The requirement for extensive pulmonary resection (sleeve lobectomy, bilobectomy or pneumonectomy) was significantly more frequent in the patient group with positive compared with the group with negative PET/CT findings (76 vs. 9%, respectively, P=0.01). Therefore, the PET/CT findings in the hilar lymph nodes were useful for the prediction of extracapsular invasion and, consequently, for the estimation of possible extensive pulmonary resection.

Review of Pediatric Pheochromocytoma and Paraganglioma

Directory of Open Access Journals (Sweden)

Reshma Bholah

2017-07-01

Full Text Available Pheochromocytoma (PCC and paraganglioma (PGL are rare chromaffin cell tumors which secrete catecholamines and form part of the family of neuroendocrine tumors. Although a rare cause of secondary hypertension in pediatrics, the presentation of hypertension in these patients is characteristic, and treatment is definitive. The gold standard for diagnosis is via measurement of plasma free metanephrines, with imaging studies performed for localization, identification of metastatic lesions and for surgical resection. Preoperative therapy with alpha-blocking agents, beta blockers, and potentially tyrosine hydroxylase inhibitors aid in a safe pre-, intra- and postoperative course. PCC and PGL are inherited in as much as 80% of pediatric cases, and all patients with mutations should be followed closely given the risk of recurrence and malignancy. While the presentation of chromaffin cell tumors has been well described with multiple endocrine neoplasia, NF1, and Von Hippel–Lindau syndromes, the identification of new gene mutations leading to chromaffin cell tumors at a young age is changing the landscape of how clinicians approach such cases. The paraganglioma–pheochromocytoma syndromes (SDHx comprise familial gene mutations, of which the SDHB gene mutation carries a high rate of malignancy. Since the inheritance rate of such tumors is higher than previously described, genetic screening is recommended in all patients, and lifelong follow-up for recurrent tumors is a must. A multidisciplinary team approach allows for optimal health-care delivery in such children. This review serves to provide an overview of pediatric PCC and PGL, including updates on the preferred methods of imaging, guidelines on gene testing as well as management of hypertension in such patients.

Fusion positron emission/computed tomography underestimates the presence of hilar nodal metastases in patients with resected non-small cell lung cancer.

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Carrillo, Sergio A; Daniel, Vincent C; Hall, Nathan; Hitchcock, Charles L; Ross, Patrick; Kassis, Edmund S

2012-05-01

The 5-year survival for patients with resected stage II (N1) non-small cell lung cancer ranges from 40% to 55%. No data exist addressing the benefit of neoadjuvant therapy for patients with stage II disease. This is largely in part due to the lack of a reliable, minimally invasive method to assess hilar nodes. This study is aimed at determining the ability of fusion positron emission/computed tomography (PET/CT) to identify hilar metastases in patients with resected non-small cell lung cancer. A retrospective review of surgically resected patients with fusion PET/CT within 30 days of resection was performed. The sensitivity, specificity, positive predictive value, and negative predictive value for PET/CT in detecting hilar nodal metastases was calculated for a range of maximum standardized uptake values (SUVmax). Hilar nodes from patients with falsely positive PET/CT scans were analyzed for the presence of histoplasmosis. Additionally, the impact of hilar node size greater than 1 centimeter on the calculated values was assessed. There were 119 patients evaluated. The number of lymph nodes resected ranged from 1 to 12 (X=2.98). There was decreased sensitivity and increased specificity with higher SUVmax cutoff values. At the standard SUVmax value of 2.5, the sensitivity and specificity were only 48.5% and 80.2%. The addition of size of hilar node by CT led to a modest improvement in sensitivity at all SUVmax cutoff values. Fusion PET/CT lacks sensitivity and specificity in identifying hilar nodal metastasis in patients with resected non-small cell lung cancer. Further prospective studies assessing the utility of PET/CT versus alternative sampling techniques are warranted. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Impact of specialized multi-disciplinary approach and an integrated pathway on outcomes in hilar cholangiocarcinoma.

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Gomez, D; Patel, P B; Lacasia-Purroy, C; Byrne, C; Sturgess, R P; Palmer, D; Fenwick, S; Poston, G J; Malik, H Z

2014-01-01

To assess the outcomes of patients with hilar cholangiocarcinoma following referral to a specialist multi-disciplinary team. Over an 11-year period, patients referred with hilar cholangiocarcinoma were identified from a prospectively maintained registry. Collated data included demographics, operative findings and histo-pathological data. Survival differences and prognostic factors were determined. 345 patients were referred with hilar cholangiocarcinoma, of which 57 (16.5%) patients had surgery. Prior to 2008, of 143 patients referred, only 17 (11.9%) patients underwent surgery, compared to 40 (19.8%) of 202 patients referred from 2008 onwards (p = 0.051). In the surgery group, the majority of patients underwent left hemi-hepatectomy (n = 19). In addition, portal vein (n = 5), hepatic artery (n = 2) and inferior vena cava (n = 3) resections were performed. The R0 resection rate was 73.7%. The morbidity and mortality rates were 59.6% and 14.0%, respectively. The median disease-free survival was 16 (4-101) months. The presence of lymph node metastasis (p = 0.002) was the only predictor of poorer disease-free survival. The 5-year overall survival was 39.5% and was significantly better than that of the palliative group (p hilar cholangiocarcinoma and is associated with better overall survival. Prompt referral to tertiary centres with a core team of clinicians to manage this difficult condition may allow more patients to come to potentially curative surgical resections. Copyright © 2013. Published by Elsevier Ltd.

A case of the hepatic hilar bile duct cancer with external radiation. Efficacy and severe side effect of external radiation therapy

International Nuclear Information System (INIS)

Andoh, Hideaki; Yasui, Ouki; Ise, Norihito

2003-01-01

Hepatic hilar bile duct cancer was difficult to cure by surgical treatment and its prognosis was very poor. We present the case of non-curative resection of hepatic hilar bile duct cancer, controlled with external radiation. 72 years-old-female, she complained jaundice and diagnosed hepatic hilar bile duct cancer with abdominal ultrasonography. Hepatic hilar resection was performed but curative resection could not be done, because cancer was diffusely spreaded to the hepatic and duodenal ends of the bile duct. After surgery, external radiation (1.8 Gy/day; total 50.4 Gy) was performed. Three months after operation, sometimes, cholangitis was occurred but we could not detect the intrahepatic bile duct dilatation and improved with antibiotics. After seven months, she was dead for sepsis, liver abscess and biliary cirrhosis. From autopsy findings, severe hepatic hilar fibrosis around the irradiation area, stenosis of the hepatico-jejunostomy and portal vein were existed but could not detect the remnant cancer cells. External radiation was sometimes effective, especially for this case. But we should consider the side effect of fibrosis and preventive treatments such as biliary stenting or early biliary drainage. (author)

A case of the hepatic hilar bile duct cancer with external radiation. Efficacy and severe side effect of external radiation therapy

Energy Technology Data Exchange (ETDEWEB)

Andoh, Hideaki; Yasui, Ouki; Ise, Norihito [Akita Univ. (Japan). School of Medicine

2003-04-01

Hepatic hilar bile duct cancer was difficult to cure by surgical treatment and its prognosis was very poor. We present the case of non-curative resection of hepatic hilar bile duct cancer, controlled with external radiation. 72 years-old-female, she complained jaundice and diagnosed hepatic hilar bile duct cancer with abdominal ultrasonography. Hepatic hilar resection was performed but curative resection could not be done, because cancer was diffusely spreaded to the hepatic and duodenal ends of the bile duct. After surgery, external radiation (1.8 Gy/day; total 50.4 Gy) was performed. Three months after operation, sometimes, cholangitis was occurred but we could not detect the intrahepatic bile duct dilatation and improved with antibiotics. After seven months, she was dead for sepsis, liver abscess and biliary cirrhosis. From autopsy findings, severe hepatic hilar fibrosis around the irradiation area, stenosis of the hepatico-jejunostomy and portal vein were existed but could not detect the remnant cancer cells. External radiation was sometimes effective, especially for this case. But we should consider the side effect of fibrosis and preventive treatments such as biliary stenting or early biliary drainage. (author)

Splenic hilar lymph node metastasis independently predicts poor survival for patients with gastric cancers in the upper and/or the middle third of the stomach.

Science.gov (United States)

Zhu, Guo-Lian; Sun, Zhe; Wang, Zhen-Ning; Xu, Ying-Ying; Huang, Bao-Jun; Xu, Yan; Zhu, Zhi; Xu, Hui-Mian

2012-06-15

Effectiveness of splenectomy for advanced gastric cancers occupying the upper and/or the middle third of the stomach is still in debate. The aim of the present study is to elucidate the impact of splenectomy on patient survival by investigating the pathological characteristics and prognostic significance of splenic hilar lymph node metastasis. Clinicopathologic and prognostic data of 265 patients with gastric cancer in the upper and/or the middle third of the stomach who underwent the operation of en bloc resection of primary cancer and D2/D3 lymphadenectomy combined with splenectomy were retrospectively reviewed. Multivariate analysis revealed pT category, pN category, and distant lymph node metastasis independently correlated with the presence of splenic hilar lymph node metastasis. Prognoses of patients with positive splenic hilar lymph nodes were significantly poorer than that of patients with negative splenic hilar lymph nodes for the entire study population and for those who underwent R0 resection, but not for those who underwent R1-2 resection. There was no significant difference in survival between patients who underwent R0 resection with positive splenic hilar lymph nodes and those who underwent R1-2 resection. Splenic hilar lymph node metastasis was one of independent indicators predicting worse prognosis and the presence of distant metastasis after surgery. Subset analysis according to the TNM stage revealed there were significant differences in survival between patients with and without splenic hilar lymph node metastasis. Splenic hilar lymph node metastasis should be considered as one of incurable factors. Consequently, the efficiency of splenectomy aiming at prolonging survival for patients with high risk of splenic hilar lymph nodes metastasis should be questioned, although resection of invasive organs form gastric cancers has been recommended if R0 surgery could be achieved. Copyright © 2011 Wiley Periodicals, Inc.

Magnetic Resonance Imaging Including Magnetic Resonance Cholangiopancreatography for Tumor Localization and Therapy Planning in Malignant Hilar Obstructions

International Nuclear Information System (INIS)

Haenninen, E.L.

2005-01-01

PURPOSE: To assess image quality and overall accuracy of magnetic resonance imaging (MRI), including two magnetic cholangiopancreatography (MRCP) techniques, for the diagnostics and preoperative work-up of malignant hilar obstructions. MATERIAL AND METHODS: Thirty-one patients with malignant hilar obstructions (hilar cholangiocarcinoma, n=30; hepatocellular carcinoma, n=1) received MRCP by two techniques (single-shot thick-slab and multisection thin-slice MRCP) and unenhanced and contrast material-enhanced MRI. MR assessment included the evaluation of image quality and visualization of bile ducts (5-point scale), and the classification of tumor status. MR results were subsequently correlated with the results from surgery and pathology. RESULTS: The maximum intensity projections of multisection thin-slice MRCP had significantly more artifacts compared to MRCP in the single-shot thick-slab technique, and overall image quality of single-shot thick-slab MRCP was rated significantly superior compared to multisection thin-slice MRCP (4.4 ± 0.7 and 4.1 ± 0.9, respectively). Moreover, ductal visualization of different parts of the biliary system was rated superior with single-shot thick-slab MRCP. In contrast, the original data from multisection thin slice MRCP facilitated visualization of periductal lesions and adjacent structures. Overall MR accuracy for the assessment of tumor status, periductal infiltration, and lymph node metastases was 90%, 87%, and 66%, respectively. CONCLUSION: For evaluation of malignant hilar obstructions, MRCP by the single-shot thick-slab technique had superior image quality and fewer artifacts; in contrast, besides sole biliary visualization, multisection MRCP depicted complementary adjacent parenchymal and periductal structures. We therefore recommend MRI, with a combination of both MRCP techniques, for the diagnostic work-up and therapy planning of malignant hilar obstructions

Beckwith-Wiedemann syndrome and bilateral adrenal pheochromocytoma: sonography and MRI findings

Energy Technology Data Exchange (ETDEWEB)

Baldisserotto, Matteo; Peletti, Adriana Barcellos; Araujo, Manoel Angelo de; Pertence, Ana Paula Cardoso; Dora, Marcelo Dourado; Maciel, Elines Oliva; Gaiger, Ana Maria [Hospital da Crianca Conceicao, Departamento de Radiologia, Porto Alegre, RS (Brazil)

2005-11-01

Beckwith-Wiedemann syndrome is characterized by a group of clinical abnormalities, the most frequent of which are omphalocele, macroglossia, gigantism, neonatal hypoglycemia and umbilical hernia. The association of this syndrome with malignant tumors is well documented. We report a child with this syndrome associated with bilateral adrenal pheochromocytoma. (orig.)

Beckwith-Wiedemann syndrome and bilateral adrenal pheochromocytoma: sonography and MRI findings

International Nuclear Information System (INIS)

Baldisserotto, Matteo; Peletti, Adriana Barcellos; Araujo, Manoel Angelo de; Pertence, Ana Paula Cardoso; Dora, Marcelo Dourado; Maciel, Elines Oliva; Gaiger, Ana Maria

2005-01-01

Beckwith-Wiedemann syndrome is characterized by a group of clinical abnormalities, the most frequent of which are omphalocele, macroglossia, gigantism, neonatal hypoglycemia and umbilical hernia. The association of this syndrome with malignant tumors is well documented. We report a child with this syndrome associated with bilateral adrenal pheochromocytoma. (orig.)

Mechanism of palytoxin-induced [3H]norepinephrine release from a rat pheochromocytoma cell line

International Nuclear Information System (INIS)

Tatsumi, M.; Takahashi, M.; Ohizumi, Y.

1984-01-01

Palytoxin, isolated from the zoanthid Palytoha species, is one of the most potent marine toxins. Palytoxin caused a release of [ 3 H]norepinephrine from clonal rat pheochromocytoma cells in a concentration-dependent manner. This releasing action of palytoxin was markedly inhibited or abolished by Co 2+ or Ca 2+ -free medium, but was not modified by tetrodotoxin. The release of [ 3 H]norepinephrine induced by a low concentration of palytoxin was abolished in sodium-free medium and increased as the external Na+ concentrations were increased, but the release induced by a high concentration was unaffected by varying the concentration of external Na + . The release of [ 3 H]norepinephrine induced by both concentrations of palytoxin increased with increasing Ca 2+ concentrations. Palytoxin caused a concentration-dependent increase in 22 Na and 45 Ca influxes into pheochromocytoma cells. The palytoxin-induced 45 Ca influx was markedly inhibited by Co 2+ , whereas the palytoxin-induced 22 Na influx was not affected by tetrodotoxin. These results suggest that in pheochromocytoma cells the [ 3 H]norepinephrine release induced by lower concentrations of palytoxin is primarily brought about by increasing tetrodotoxin-insensitive Na + permeability across the cell membrane, whereas that induced by higher concentrations is mainly caused by a direct increase in Ca 2+ influx into them

Mediastinal and hilar lymphadenopathy: cross-referenced anatomy on axial and coronal images displayed by using multi-detector row CT

International Nuclear Information System (INIS)

Lee, Ju Hyun; Lee, Kyung Soo; Kim, Tae Sung; Yi, Chin A; Cho, Jae Min; Lee, Min Hee

2003-01-01

The accurate evaluation of mediastinal and pulmonary hilar lymphadenopathy, especially in patients with lung cancer, is important for determining treatment options and evaluating the response to therapy. To indicate nodal location in detail, mediastinal and hilar lymph nodes have been assigned to one of 14 nodal stations. Mediastinal nodes of greater than 10 mm short-axis diameter are regarded as abnormal, irrespective of their nodal station, while hilar nodes are considered abnormal if their diameter is greater than 10 mm in any axis or they are convex compared to surrounding lung. By providing multiplanar images, multi-detector row CT allows detailed evaluation of thoracic anatomic structures more easily than in the past, when axial images only were available. At cross-referenced imaging, a lymph node depicted at axial imaging in one anatomical location can be visualized simultaneously and automatically at coronal imaging at the exactly corresponding anatomical location. Cross-referenced coincidental axial and coronal images help assess both the size and morphology of mediastinal and hilar lymph nodes

The first Dutch SDHB founder deletion in paraganglioma – pheochromocytoma patients

Directory of Open Access Journals (Sweden)

Devilee Peter

2009-04-01

Full Text Available Abstract Background Germline mutations of the tumor suppressor genes SDHB, SDHC and SDHD play a major role in hereditary paraganglioma and pheochromocytoma. These three genes encode subunits of succinate dehydrogenase (SDH, the mitochondrial tricarboxylic acid cycle enzyme and complex II component of the electron transport chain. The majority of variants of the SDH genes are missense and nonsense mutations. To date few large deletions of the SDH genes have been described. Methods We carried out gene deletion scanning using MLPA in 126 patients negative for point mutations in the SDH genes. We then proceeded to the molecular characterization of deletions, mapping breakpoints in each patient and used haplotype analysis to determine whether the deletions are due to a mutation hotspot or if a common haplotype indicated a single founder mutation. Results A novel deletion of exon 3 of the SDHB gene was identified in nine apparently unrelated Dutch patients. An identical 7905 bp deletion, c.201-4429_287-933del, was found in all patients, resulting in a frameshift and a predicted truncated protein, p.Cys68HisfsX21. Haplotype analysis demonstrated a common haplotype at the SDHB locus. Index patients presented with pheochromocytoma, extra-adrenal PGL and HN-PGL. A lack of family history was seen in seven of the nine cases. Conclusion The identical exon 3 deletions and common haplotype in nine patients indicates that this mutation is the first Dutch SDHB founder mutation. The predominantly non-familial presentation of these patients strongly suggests reduced penetrance. In this small series HN-PGL occurs as frequently as pheochromocytoma and extra-adrenal PGL.

Update on Modern Management of Pheochromocytoma and Paraganglioma

Directory of Open Access Journals (Sweden)

Jacques W. M. Lenders

2017-06-01

Full Text Available Despite all technical progress in modern diagnostic methods and treatment modalities of pheochromocytoma/paraganglioma, early consideration of the presence of these tumors remains the pivotal link towards the best possible outcome for patients. A timely diagnosis and proper treatment can prevent the wide variety of potentially catastrophic cardiovascular complications. Modern biochemical testing should include tests that offer the best available diagnostic performance, measurements of metanephrines and 3-methoxytyramine in plasma or urine. To minimize false-positive test results particular attention should be paid to pre-analytical sampling conditions. In addition to anatomical imaging by computed tomography (CT or magnetic resonance imaging, new promising functional imaging modalities of photon emission tomography/CT using with somatostatin analogues such as 68Ga-DOTATATE (68Ga-labeled DOTA(0-Tyr(3-octreotide will probably replace 123I-MIBG (iodine-123-metaiodobenzylguanidine in the near future. As nearly half of all pheochromocytoma patients harbor a mutation in one of the 14 tumor susceptibility genes, genetic testing and counseling should at least be considered in all patients with a proven tumor. Post-surgical annual follow-up of patients by measurements of plasma or urinary metanephrines should last for at least 10 years for timely detection of recurrent or metastatic disease. Patients with a high risk for recurrence or metastatic disease (paraganglioma, young age, multiple or large tumors, genetic background should be followed up lifelong.

Preoperative assessment of hilar cholangiocarcinoma using multidetector-row CT. Correlation with histopathological findings

International Nuclear Information System (INIS)

Watadani, Takeyuki; Akahane, Masaaki; Ohtomo, Kuni; Yoshikawa, Takeharu

2008-01-01

Our aim was to investigate the diagnostic reliability of multidetector-row computed tomography (MDCT) for preoperative assessment of local tumoral spread in hilar cholangiocarcinoma. Thirteen of 30 consecutive patients with hilar cholangiocarcinoma who underwent surgery, excluding 17 patients who underwent biliary drainage or preoperative portal embolization, were retrospectively evaluated. Using MDCT systems of 4 detector rows or 16 detector rows, plain and dynamic contrast-enhanced images of three phases were obtained. Extent of tumor spread and lymph node metastasis were assessed with MDCT and compared with histopathological findings. The Bismuth-Corlette classification of hilar cholangiocarcinoma with MDCT were type I, 1 patient; type IIIa, 3 patients; type IIIb, 4 patients; and type IV, 5 patients; those with histopathological findings were type I, 1 patient; type IIIa, 2 patients; type IIIb, 4 patients; and type IV, 6 patients. One patient diagnosed as type IIIa with MDCT was pathologically diagnosed as type IV. Accuracy of MDCT in tumoral spread was 92.3%, although that of lymph node metastasis was 54%. MDCT is likely to play an important role in evaluation of focal lesion spread especially in intrapancreatic tumor invasion, although a greater number of cohort cases are necessary to clearly define its role. (author)

Embolisation of pheochromocytoma to stabilise and wean a patient in cardiogenic shock from emergency extracorporeal life support

DEFF Research Database (Denmark)

Vagner, Helle; Hey, Thomas Morris; Elle, Bo

2015-01-01

Pheochromocytoma is a catecholamine-secreting tumour associated with varying symptoms ranging from episodic headache, sweating, paroxysmal hypertension and tachycardia to intractable cardiogenic shock. Cardiogenic shock is rare but well-described and the timing of correct management is crucial si...... since mortality is high. Fifty per cent of pheochromocytomas are diagnosed on autopsy. We report on a case of embolisation of the adrenal artery during ongoing extracorporeal life support (ECLS) in order to stabilise and wean the patient from ECLS as a bridge to final surgery....

Value of PET-CT and PET-CT combined with lung VCAR software in the diagnosis of hilar area lymph nodes of non-small cell lung cancer

International Nuclear Information System (INIS)

Yu Lijuan; Li Yingci; Wang Wenzhi; Wang Xin; Lu Pei'ou; Tian Mohan

2012-01-01

Objective: To explore the diagnostic value of PET-CT and PET-CT combined with lung volume computed assisted reading (Lung VCAR) software in hilar area lymph nodes. Methods: Preoperative whole body PET-CT imaging was performed in 49 patients who were highly suspicious of non-small cell lung cancer. PET-CT images of the hilar area lymph nodes and the PET-CT images of the hilar area lymph nodes from Lung VCAR software were evaluated by two experienced doctors, and then compared with the pathological diagnosis. Results: There was no significant difference between the CT values of benign and malignant lymph nodes (t=-1.40, P>0.05). But a significant difference was existed between the benign and malignant hilar lymph nodes with the density visual analysis, the lymph short diameter and the maximum of standardized uptake value (SUV max ) (χ 2 =30.37, 27.40, 20.06, all P<0.05). The sensibility,specificity and accuracy of PET-CT in diagnosis of the hilar area lymph nodes were 76.5%, 90.7%, 88.3% respectively, and the accuracy of the diagnosis was significantly higher than that of CT and PET alone (χ 2 =15.27, P<0.05) using the lymph short diameter ≥1 cm of CT, the density of lymph node is equal to (slightly lower than) the same layer vascular density and the lymph node SUV max ≥2.5 of PET as the diagnostic criteria. One hundred and three hilar area lymph nodes were diagnosed by PET-CT and four nodes were not hilar lymph nodes proved by the Lung VCAR software (3 hilar vascular uptake,1 bronchial cartilage). Conclusion: The methods of PET-CT lymph visual density analysis plus lymph node diameter and SUV max had a high diagnostic accuracy of non-small cell lung hilar lymph. For the PET-CT,the pulmonary vascular uptake was the main cause affecting the discrimination of hilar lymph nodes,while Lung VCAR software was helpful to diagnosis. (authors)

Novel Preclinical Testing Strategies for Treatment of Metastatic Pheochromocytoma

Science.gov (United States)

2015-11-01

Schleswig-Holstein Lübeck, Lübeck, Germany Abstract There is currently no effective treatment for metastatic pheochromocytomas and paragangliomas. A...Supplementary Fig. S2) and deregulated fatty acid metabolism (Fig. 2b), leading to higher levels of palmitate and linoleate, increased long chain fatty...mitochondria18. This pathway required HIF-1a, which is deregulated in SDHB-mutant tumours, and may potentially contribute to disease maintenance39. The

Pheochromocytoma presenting as an acute coronary syndrome complicated by acute heart failure: The challenge of a great mimic.

Science.gov (United States)

Sanna, Giuseppe Damiano; Talanas, Giuseppe; Fiore, Giuseppina; Canu, Antonella; Terrosu, Pierfranco

2016-10-01

Pheochromocytoma is a rare neuroendocrine tumor with a highly variable clinical presentation. The serious and potentially lethal cardiovascular complications of these tumors are related to the effects of secreted catecholamines. We describe a case of a 50-year-old woman urgently admitted to our hospital because of symptoms and clinical and instrumental findings consistent with an acute coronary syndrome complicated by acute heart failure. Urgent coronary angiography showed normal coronary arteries. During her hospital stay, the recurrence of episodes characterized by a sudden increase in blood pressure, cold sweating, and nausea allowed us to hypothesize a pheochromocytoma. The diagnosis was confirmed by elevated levels of urinary catecholamines and by the finding of a left adrenal mass on magnetic resonance imaging. The patient underwent left adrenalectomy. Therefore, the initial diagnosis was critically reappraised and reviewed as a cardiac manifestation of a pheochromocytoma during catecholaminergic crisis.

Palliative treatment with radiation-emitting metallic stents in unresectable Bismuth type III or IV hilar cholangiocarcinoma.

Science.gov (United States)

Lu, Jian; Guo, Jin-He; Zhu, Hai-Dong; Zhu, Guang-Yu; Wang, Yong; Zhang, Qi; Chen, Li; Wang, Chao; Pan, Tian-Fan; Teng, Gao-Jun

2017-01-01

The emerging data for stenting in combination with brachytherapy in unresectable hilar cholangiocarcinoma are encouraging. The aim of this study was to evaluate the efficacy and safety of radiation-emitting metallic stents (REMS) for unresectable Bismuth type III or IV hilar cholangiocarcinoma. Consecutive patients who underwent percutaneous placement with REMS or uncovered self-expandable metallic stent (SEMS) for unresectable Bismuth type III or IV hilar cholangiocarcinoma between September 2011 and April 2016 were identified into this retrospective study. Data on patient demographics and overall survival, functional success, stent patency and complications were collected at the authors' hospital. A total of 59 patients were included: 33 (55.9%) in the REMS group and 26 (44.1%) in the SEMS group. The median overall survival was 338 days in the REMS group and 141 days in the SEMS group (philar cholangiocarcinoma, and seems to prolong survival as well as patency of stent in these patients.

Pheochromocytoma of the Organ Zuckerkandl.

Science.gov (United States)

Lee, C; Chang, E; Gimenez, J; McCarron, R

2017-01-01

Pheochromocytomas (PCCs);, or intra-adrenal paragangliomas (PGLs);, are neuroendocrine tumors arising within the adrenal medulla. Extra-adrenal paragangliomas may arise in the sympathetic or parasympathetic paraganglia and more rarely in other organs. One of the most common extra-adrenal sites is in the organ of Zuckerkandl, a collection of chromaffin cells near the origin of the inferior mesenteric artery or near the aortic bifurcation. The following is a case of a patient with resistant hypertension secondary to an extra-adrenal paraganglioma in the organ of Zuckerkandl. The patient is a 43 year old man with a history of depression, type 2 diabetes mellitus, and hypertension who was sent to the emergency department by his primary care physician for severely elevated blood pressures. Patient also had diaphoresis, tachycardia, and a new, fine tremor of his left hand. Upon presentation, the patient's blood pressure was 260/120 mmHg with a heart rate of 140 beats per minute. Plasma fractionated metanephrines sent on admission revealed significantly elevated levels of total plasma metanephrines (2558 pg/mL);, free metanephrine (74 pg/ml); and free normetanephrine (2484pg/mL);. An I-123 metaiodobenzylguanidine (MIBG); scan showed abnormal uptake in the lower abdomen at the level of the aortic bifurcation. Patient was started on alpha-blockade, with subsequent addition of a beta-blocker prior to surgery. Patient underwent surgical removal of the tumor with pathology consistent with a paraganglioma. Pheochromocytomas and paragangliomas are responsible for approximately 0.5 percent of cases of secondary hypertension. Many different biochemical markers have been used to aid in the diagnosis of PCC/PGL including plasma catecholamines, plasma metanephrines, urine fractionated metanephrines, urine catecholamines, total metanephrines and vanillymandellic acid. Definitive management of a PCC and PGL involves surgical removal of the tumor. Finally, there should be a discussion

Intrahepatic and hilar mass-forming cholangiocarcinoma: Qualitative and quantitative evaluation with diffusion-weighted MR imaging.

Science.gov (United States)

Fattach, Hassan El; Dohan, Anthony; Guerrache, Youcef; Dautry, Raphael; Boudiaf, Mourad; Hoeffel, Christine; Soyer, Philippe

2015-08-01

To qualitatively and quantitatively analyze the presentation of intrahepatic and hilar mass-forming cholangiocarcinoma with diffusion-weighted magnetic resonance imaging (DW-MRI). Twenty-eight patients with histopathologically proven mass-forming cholangiocarcinoma (hilar, n=17; intrahepatic, n=11) underwent hepatic DW-MRI at 1.5-T using free-breathing acquisition and three b-values (0,400,800s/mm(2)). Cholangiocarcinomas were evaluated qualitatively using visual analysis of DW-MR images and quantitatively with conventional ADC and normalized ADC measurements using liver and spleen as reference organs. All cholangiocarcinomas (28/28; 100%) were visible on DW-MR images. DW-MRI yielded best conspicuity of cholangiocarcinomas than the other MRI sequences (Philar cholangiocarcinomas. The use of normalized ADC using the liver as reference organ resulted in the most restricted distribution of ADC values of cholangiocarcinomas (variation coefficient=16.6%). There is a trend towards a common appearance of intrahepatic and hilar mass-forming cholangiocarcinomas on DW-MRI but variations may be observed. Familiarity with these variations may improve the diagnosis of mass-forming cholangiocarcinoma. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Y-shaped bilateral self-expandable metallic stent placement for malignant hilar biliary obstruction: data from a referral center for palliative care.

Science.gov (United States)

Di Mitri, R; Mocciaro, F

2014-01-01

Malignant hilar strictures are a clinical challenge because of the current therapeutic approach and the poor prognosis. In recent years, self-expandable metallic stents have proven more effective than plastic stents for palliation of malignant hilar strictures, with the bilateral stent-in-stent technique registering a high success rate. We report our experience with Y-shaped endoscopic self-expandable metallic stents placement for treatment of advanced malignant hilar strictures. From April 2009 to August 2012, we prospectively collected data on patients treated with Y-shaped SEMS placement for advanced malignant hilar carcinoma. Data on technical success, clinical success, and complications were collected. Twenty patients (9 males) were treated (mean age 64.2 ± 15.3 years). The grade of malignant hilar strictures according to the Bismuth classification was II in 5 patients (25%), IIIa in 1 (5%), and IV in 14 (70%). The mean bilirubin level was 14.7 ± 4.9 mg/dL. Technical success was achieved in all patients, with a significant reduction in bilirubin levels (2.9 ± 1.7 mg/dL). One patient experienced cholangitis as early complication, while in 2 patients stent ingrowth was observed. No stents migration was recorded. There was no procedure-related mortality. At the end of the follow-up (7.1 ± 3.1 months), 13 of the 20 patients (65%) had died. Our experience confirms endoscopic bilateral self-expandable metallic stents placement with stent-in-stent technique (Y-shaped configuration) as a feasible, effective, and safe procedure for palliation of unresectable malignant hilar strictures.

Bilateral metal stents for hilar biliary obstruction using a 6Fr delivery system: outcomes following bilateral and side-by-side stent deployment.

Science.gov (United States)

Law, Ryan; Baron, Todd H

2013-09-01

Controversy exists on optimal endoscopic management for palliation of malignant hilar obstruction, with advocates for metal "side-by-side" (SBS) and "stent-in-stent" (SIS) techniques. We sought to evaluate the technical feasibility, efficacy, and outcomes of bilateral biliary self-expanding metal stents (SEMS) for treatment of malignant hilar obstruction using a stent with a 6Fr delivery system. This was a single-center, retrospective review of all patients who underwent bilateral placement of Zilver® biliary SEMS for malignant hilar obstruction from January 2010 to August 2012. Patients underwent endoscopic retrograde cholangiopancreatography with placement of stents using either the SIS or SBS stent techniques. Twenty-four patients (19 men, mean age 63 years) underwent bilateral stenting for malignant hilar obstruction during the study period. Seventeen and seven patients underwent the SBS and SIS technique, respectively. Cholangiocarcinoma (n=14) was the most common cause of hilar obstruction. Initial technical success was achieved in 24/24 (100%) of patients; however, 12 (50%) patients required re-intervention during the study period (median 98 days). Comparison of the SBS and SIS groups revealed no statistical difference with respect to need for re-intervention (P=0.31), successful re-intervention (P=0.60), or procedural length (P=0.89). Use of bilateral Zilver® SEMS in either the SBS or SIS configuration is safe, technically feasible, and effective for drainage of malignant hilar obstruction; however, duration of stent patency and procedure-free survival remain variable.

[Application of Da Vinci surgical robot in the dissection of splenic hilar lymph nodes for gastric cancer patients with total gastrectomy].

Science.gov (United States)

Yang, Kun; Chen, Xinzu; Zhang, Weihan; Chen, Xiaolong; Hu, Jiankun

2016-08-25

To investigate the feasibility and safety of Da Vinci surgical robot in the dissection of splenic hilar lymph nodes for gastric cancer patients with total gastrectomy. Clinical data of two cases who underwent total gastrectomy for cardia cancer at our department in January 2016 were analyzed retrospectively. Two male patients were 62 and 55 years old respectively, with preoperative diagnosis as cT2-3N0M0 and cT1-2N0M0 gastric cancer by gastroscope and biopsy, and both received robotic total gastrectomy spleen-preserving splenic hilar lymph node dissection successfully. The operative time for splenic hilar lymph node dissection was 30 min and 25 min respectively. The intraoperative estimated blood loss was both 100 ml, while the number of total harvested lymph node was 38 and 33 respectively. One dissected splenic hilar lymph node and fatty tissues in two patients were proven by pathological examinations. There were no anastomotic leakage, pancreatic fistula, splenic infarction, intraluminal bleeding, digestive tract bleeding, aneurysm of splenic artery, and other operation-associated complications. Both patients suffered from postoperative pneumonia, and were cured by conservative therapy. The robotic spleen-preserving splenic hilar lymph node dissection is feasible and safe, but its superiority needs further evaluation.

Incidence of benign lesions in patients resected for suspicious hilar obstruction

NARCIS (Netherlands)

Gerhards, M. F.; Vos, P.; van Gulik, T. M.; Rauws, E. A.; Bosma, A.; Gouma, D. J.

2001-01-01

BACKGROUND: The differentiation between benign and malignant strictures at the hepatic hilum is difficult. The aim of this study was to assess the clinical and radiographical features of hilar lesions in a series of patients resected for a presumed cholangiocarcinoma. METHODS: Of 132 consecutive

Diurnal Blood Pressure Variation in Pheochromocytoma, Primary Aldosteronism and Cushing's Syndrome

Czech Academy of Sciences Publication Activity Database

Zelinka, T.; Štrauch, B.; Pecen, Ladislav; Widimský jr., J.

Roc. 18, c. 1 (2004), s. 107-111 ISSN 0950-9240 R&D Projects: GA MŠk LN00B107 Institutional research plan: CEZ:AV0Z1030915 Keywords : primary aldosteronism * pheochromocytoma * Cushing's syndrome * cirardian blood pressure rhythm Subject RIV: BB - Applied Statistics, Operational Research Impact factor: 1.930, year: 2004

Endoscopic bilateral stent-in-stent placement for malignant hilar obstruction using a large cell type stent.

Science.gov (United States)

Park, Jin Myung; Lee, Sang Hyub; Chung, Kwang Hyun; Jang, Dong Kee; Ryu, Ji Kon; Kim, Yong-Tae; Lee, Jae Min; Paik, Woo Hyun

2016-12-01

Bilateral stent-in-stent (SIS) self-expandable metal stent placement is technically challenging for palliation of unresectable malignant hilar obstruction. In the SIS technique, the uniform large cell type biliary stent facilitates contralateral stent deployment through the mesh of the first metallic stent. This study aimed to assess the technical success and clinical effectiveness of this technique with a uniform large cell type biliary stent. Thirty-one patients who underwent bilateral SIS placement using a large cell type stent were reviewed retrospectively. All patients showed malignant hilar obstruction (Bismuth types II, III, IV) with different etiologies. Sixteen (51.6%) patients were male. The mean age of the patients was 67.0+/-14.0 years. Most patients were diagnosed as having hilar cholangiocarcinoma (58.1%) and gallbladder cancer (29.0%). Technical success rate was 83.9%. Success was achieved more frequently in patients without masses obstructing the biliary confluence (MOC) than those with MOC (95.2% vs 60.0%, P=0.03). Functional success rate was 77.4%. Complications occurred in 29.0% of the patients. These tended to occur more frequently in patients with MOC (50.0% vs 19.0%, P=0.11). Median time to recurrent biliary obstruction was 188 days and median survival was 175 days. The large cell type stent can be used efficiently for bilateral SIS placement in malignant hilar obstruction. However, the risk of technical failure increases in patients with MOC, and caution is needed to prevent complications for these patients.

Short term efficacy of interventional therapy for hilar biliary obstruction

International Nuclear Information System (INIS)

Zhai Renyou; Dai Dingke; Wang Jianfeng; Yu Ping; Wei Baojie

2006-01-01

Objective: To analyze the method and short term efficacy of interventional therapy for hilar biliary obstructive jaundice. Methods: 100 consecutive patients with perihilar biliary obstruction admitted before May 2004 were treated with percutaneous transhepatic biliary drainage (PTBD) or placement of metallic stents. Among them, 39 patients were found with bile duct cancer, 6 with adenocarcinoma of gallbladder, 22 with metastatic carcinoma, 15 with primary liver carcinoma and 18 with bile duct strait after liver transplantation. Serum total bilirubin before operation and 3-7 days, 8-14 days after procedure were analysed by t test. Results: 79 patients with PTBD (including simple external drainage and combined internal and external drainage), and 21 patients with stents placement (including 31 stents of 4 different kinds) were all carried out successfully. There were significant differences in serum total bilirubin before and 3-7 days, 8-14 days after the procedure, P<0.05 vs P<0.01. Conclusion: Interventional therapy is simple, safe, and effective for hilar biliary obstruction, the latter showed more significance than the former with short term satisfaction. (authors)

Hilar cholangiocarcinoma: Cross sectional evaluation of disease spectrum

Science.gov (United States)

Mahajan, Mangal S; Moorthy, Srikanth; Karumathil, Sreekumar P; Rajeshkannan, R; Pothera, Ramchandran

2015-01-01

Although hilar cholangiocarcinoma is relatively rare, it can be diagnosed on imaging by identifying its typical pattern. In most cases, the tumor appears to be centered on the right or left hepatic duct with involvement of the ipsilateral portal vein, atrophy of hepatic lobe on that side, and invasion of adjacent liver parenchyma. Multi-detector computed tomography (MDCT) and magnetic resonance cholangiopancreatography (MRCP) are commonly used imaging modalities to assess the longitudinal and horizontal spread of tumor. PMID:25969643

Y-Shaped Bilateral Self-Expandable Metallic Stent Placement for Malignant Hilar Biliary Obstruction: Data from a Referral Center for Palliative Care

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R. Di Mitri

2014-01-01

Full Text Available Background and Aim. Malignant hilar strictures are a clinical challenge because of the current therapeutic approach and the poor prognosis. In recent years, self-expandable metallic stents have proven more effective than plastic stents for palliation of malignant hilar strictures, with the bilateral stent-in-stent technique registering a high success rate. We report our experience with Y-shaped endoscopic self-expandable metallic stents placement for treatment of advanced malignant hilar strictures. Methods. From April 2009 to August 2012, we prospectively collected data on patients treated with Y-shaped SEMS placement for advanced malignant hilar carcinoma. Data on technical success, clinical success, and complications were collected. Results. Twenty patients (9 males were treated (mean age 64.2 ± 15.3 years. The grade of malignant hilar strictures according to the Bismuth classification was II in 5 patients (25%, IIIa in 1 (5%, and IV in 14 (70%. The mean bilirubin level was 14.7 ± 4.9 mg/dL. Technical success was achieved in all patients, with a significant reduction in bilirubin levels (2.9 ± 1.7 mg/dL. One patient experienced cholangitis as early complication, while in 2 patients stent ingrowth was observed. No stents migration was recorded. There was no procedure-related mortality. At the end of the follow-up (7.1 ± 3.1 months, 13 of the 20 patients (65% had died. Conclusions. Our experience confirms endoscopic bilateral self-expandable metallic stents placement with stent-in-stent technique (Y-shaped configuration as a feasible, effective, and safe procedure for palliation of unresectable malignant hilar strictures.

Noninvasive screening for pheochromocytoma in patients with an incidentally discovered adrenal mass. Usefulness of provocative test with metoclopramide and {sup 131}I-metaiodobenzylguanidine scintigraphy

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Yokoyama, Hiroshi; Tsuji, Yuji [Fukuoka Univ. (Japan). School of Medicine

1999-10-01

Pheochromocytoma accounts for approximately 25% of incidentally discovered adrenal masses. Certain diagnostic procedures (e.g., adrenal arteriography, needle biopsy of an adrenal mass), anesthesia and abdominal surgery may cause a sudden release of catecholamines from a pheochromocytoma and induce paroxysmal attacks of hypertension. In addition, pheochromocytoma is well known to cause unsuspected operating room deaths. Therefore, we must carefully separate this functioning neoplasm from other types of adrenal masses. In this study, we compared the results of noninvasive tests including assay of urinary catecholamines and their metabolites, a provocative pharmacologic test using metoclopramide (MCP test), and {sup 131}I-metaiodobenzylguanidine (MlBG) scintigraphy to screen for pheochromocytoma in 10 consecutive patients with an incidentally discovered adrenal mass (6 pheochromocytomas and 4 non-functioning adrenocortical adenomas). We measured the 24-hour urinary excretion of catecholamines, metanephrines and vanillyl mandelic acid in all 10 patients; 5 were positive, 4 were negative and 1 was false-negative (sensitivity=83%, specificity=100%). The MCP test was performed in 7 patients; 3 were positive, 3 were negative and 1 was false-negative (sensitivity=75%, specificity=100%). MIBG scintigraphy was performed in 7 patients; 4 were positive, 1 was negative and 2 were false-negative (sensitivity=67%, specificity=100%). According to these results, all patients with an incidentally discovered adrenal mass should undergo a determination of the 24-hour urinary excretion of catecholamines and their metabolites, including metanephrines. If this urine assay is negative, other noninvasive tests including the MCP test and MIBG scintigraphy should be considered in selected patients with radiographic characteristics of pheochromocytoma. (author)

Update on Modern Management of Pheochromocytoma and Paraganglioma.

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Lenders, Jacques W M; Eisenhofer, Graeme

2017-06-01

Despite all technical progress in modern diagnostic methods and treatment modalities of pheochromocytoma/paraganglioma, early consideration of the presence of these tumors remains the pivotal link towards the best possible outcome for patients. A timely diagnosis and proper treatment can prevent the wide variety of potentially catastrophic cardiovascular complications. Modern biochemical testing should include tests that offer the best available diagnostic performance, measurements of metanephrines and 3-methoxytyramine in plasma or urine. To minimize false-positive test results particular attention should be paid to pre-analytical sampling conditions. In addition to anatomical imaging by computed tomography (CT) or magnetic resonance imaging, new promising functional imaging modalities of photon emission tomography/CT using with somatostatin analogues such as ⁶⁸Ga-DOTATATE (⁶⁸Ga-labeled DOTA(0)-Tyr(3)-octreotide) will probably replace ¹²³I-MIBG (iodine-123-metaiodobenzylguanidine) in the near future. As nearly half of all pheochromocytoma patients harbor a mutation in one of the 14 tumor susceptibility genes, genetic testing and counseling should at least be considered in all patients with a proven tumor. Post-surgical annual follow-up of patients by measurements of plasma or urinary metanephrines should last for at least 10 years for timely detection of recurrent or metastatic disease. Patients with a high risk for recurrence or metastatic disease (paraganglioma, young age, multiple or large tumors, genetic background) should be followed up lifelong. Copyright © 2017 Korean Endocrine Society.

Lower incidence of complications in endoscopic nasobiliary drainage for hilar cholangiocarcinoma.

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Kawakubo, Kazumichi; Kawakami, Hiroshi; Kuwatani, Masaki; Haba, Shin; Kudo, Taiki; Taya, Yoko A; Kawahata, Shuhei; Kubota, Yoshimasa; Kubo, Kimitoshi; Eto, Kazunori; Ehira, Nobuyuki; Yamato, Hiroaki; Onodera, Manabu; Sakamoto, Naoya

2016-05-10

To identify the most effective endoscopic biliary drainage technique for patients with hilar cholangiocarcinoma. In total, 118 patients with hilar cholangiocarcinoma underwent endoscopic management [endoscopic nasobiliary drainage (ENBD) or endoscopic biliary stenting] as a temporary drainage in our institution between 2009 and 2014. We retrospectively evaluated all complications from initial endoscopic drainage to surgery or palliative treatment. The risk factors for biliary reintervention, post-endoscopic retrograde cholangiopancreatography (post-ERCP) pancreatitis, and percutaneous transhepatic biliary drainage (PTBD) were also analyzed using patient- and procedure-related characteristics. The risk factors for bilateral drainage were examined in a subgroup analysis of patients who underwent initial unilateral drainage. In total, 137 complications were observed in 92 (78%) patients. Biliary reintervention was required in 83 (70%) patients. ENBD was significantly associated with a low risk of biliary reintervention [odds ratio (OR) = 0.26, 95%CI: 0.08-0.76, P = 0.012]. Post-ERCP pancreatitis was observed in 19 (16%) patients. An absence of endoscopic sphincterotomy was significantly associated with post-ERCP pancreatitis (OR = 3.46, 95%CI: 1.19-10.87, P = 0.023). PTBD was required in 16 (14%) patients, and Bismuth type III or IV cholangiocarcinoma was a significant risk factor (OR = 7.88, 95%CI: 1.33-155.0, P = 0.010). Of 102 patients with initial unilateral drainage, 49 (48%) required bilateral drainage. Endoscopic sphincterotomy (OR = 3.24, 95%CI: 1.27-8.78, P = 0.004) and Bismuth II, III, or IV cholangiocarcinoma (OR = 34.69, 95%CI: 4.88-736.7, P hilar cholangiocarcinoma is challenging. ENBD should be selected as a temporary drainage method because of its low risk of complications.

Huang's three-step maneuver shortens the learning curve of laparoscopic spleen-preserving splenic hilar lymphadenectomy.

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Huang, Chang-Ming; Huang, Ze-Ning; Zheng, Chao-Hui; Li, Ping; Xie, Jian-Wei; Wang, Jia-Bin; Lin, Jian-Xian; Jun, Lu; Chen, Qi-Yue; Cao, Long-Long; Lin, Mi; Tu, Ru-Hong

2017-12-01

The goal of this study was to investigate the difference between the learning curves of different maneuvers in laparoscopic spleen-preserving splenic hilar lymphadenectomy for advanced upper gastric cancer. From January 2010 to April 2014, 53 consecutive patients who underwent laparoscopic spleen-preserving splenic hilar lymphadenectomy via the traditional-step maneuver (group A) and 53 consecutive patients via Huang's three-step maneuver (group B) were retrospectively analyzed. No significant difference in patient characteristics were found between the two groups. The learning curves of groups A and B were divided into phase 1 (1-43 cases and 1-30 cases, respectively) and phase 2 (44-53 cases and 31-53 cases, respectively). Compared with group A, the dissection time, bleeding loss and vascular injury were significantly decreased in group B. No significant differences in short-term outcomes were found between the two maneuvers. The multivariate analysis indicated that the body mass index, short gastric vessels, splenic artery type and maneuver were significantly associated with the dissection time in group B. No significant difference in the survival curve was found between the maneuvers. The learning curve of Huang's three-step maneuver was shorter than that of the traditional-step maneuver, and the former represents an ideal maneuver for laparoscopic spleen-preserving splenic hilar lymphadenectomy.To shorten the learning curve at the beginning of laparoscopic spleen-preserving splenic hilar lymphadenectomy, beginners should beneficially use Huang's three-step maneuver and select patients with advanced upper gastric cancer with a body mass index of less than 25 kg/m 2 and the concentrated type of splenic artery. Copyright © 2017. Published by Elsevier Ltd.

Multidetector row CT angiography of living related renal donors: Is there a need for venous phase imaging?

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Namasivayam, Saravanan [Department of Radiology, Division of Abdominal Imaging, Emory University School of Medicine, 1364 Clifton Road NE, Atlanta, GA 30322 (United States); Kalra, Mannudeep K. [Department of Radiology, Division of Abdominal Imaging, Emory University School of Medicine, 1364 Clifton Road NE, Atlanta, GA 30322 (United States); Waldrop, Sandra M. [Department of Radiology, Division of Abdominal Imaging, Emory University School of Medicine, 1364 Clifton Road NE, Atlanta, GA 30322 (United States); Mittal, Pardeep K. [Department of Radiology, Division of Abdominal Imaging, Emory University School of Medicine, 1364 Clifton Road NE, Atlanta, GA 30322 (United States); Small, William C. [Department of Radiology, Division of Abdominal Imaging, Emory University School of Medicine, 1364 Clifton Road NE, Atlanta, GA 30322 (United States)]. E-mail: wsmall@emory.edu

2006-09-15

Objective: To prospectively evaluate whether renal venous anatomy can be detected from arterial phase images of multidetector row CT (MDCT) of renal donors. Material and methods: Institutional review board approved our study protocol with waiver of consent. Forty-eight consecutive renal donors (age range, 21-56 years; M:F, 20:28) referred for MDCT evaluation were included. Two sub-specialty radiologists performed an independent and separate evaluation of renal venous anatomy in arterial and venous phase images. Opacification of renal venous structures was scored on a five-point scale (1-not seen; 3-minimal opacification; 5-excellent opacification). Arterial and venous phase opacification scores were compared by Wilcoxon signed rank test. Results: Both readers detected all renal venous anomalies in arterial as well as venous phase images. Each reader detected accessory right renal veins (n = 14), retroaortic left renal vein (n = 2), circumaortic left renal vein (n = 1), and left renal hilar arteriovenous malformation (n = 1) in arterial phase images. Retroaortic left renal venous branch was difficult to differentiate from lumbar vein (reader-1, n = 1; reader-2, n = 2) in both arterial and venous phase images. Sensitivity of detection of renal veins, left adrenal, gonadal and lumbar veins in arterial phase images was 100, 83-88, 100, and 85-90%, respectively. As expected, venous phase images showed significantly greater opacification of renal veins, left gonadal, adrenal and lumbar veins (p < .05). However, this did not substantially limit the evaluation of renal venous anatomy in arterial phase images. Both readers had substantial interobserver agreement (kappa coefficient, 0.7; p < 0.05). Conclusions: Arterial phase MDCT images alone can be used to detect renal venous anomalies, and to identify small left renal venous branches namely, the left gonadal, adrenal and lumbar veins in renal donors. Venous phase MDCT acquisition is not necessary for evaluation of renal

Multidetector row CT angiography of living related renal donors: Is there a need for venous phase imaging?

International Nuclear Information System (INIS)

Namasivayam, Saravanan; Kalra, Mannudeep K.; Waldrop, Sandra M.; Mittal, Pardeep K.; Small, William C.

2006-01-01

Objective: To prospectively evaluate whether renal venous anatomy can be detected from arterial phase images of multidetector row CT (MDCT) of renal donors. Material and methods: Institutional review board approved our study protocol with waiver of consent. Forty-eight consecutive renal donors (age range, 21-56 years; M:F, 20:28) referred for MDCT evaluation were included. Two sub-specialty radiologists performed an independent and separate evaluation of renal venous anatomy in arterial and venous phase images. Opacification of renal venous structures was scored on a five-point scale (1-not seen; 3-minimal opacification; 5-excellent opacification). Arterial and venous phase opacification scores were compared by Wilcoxon signed rank test. Results: Both readers detected all renal venous anomalies in arterial as well as venous phase images. Each reader detected accessory right renal veins (n = 14), retroaortic left renal vein (n = 2), circumaortic left renal vein (n = 1), and left renal hilar arteriovenous malformation (n = 1) in arterial phase images. Retroaortic left renal venous branch was difficult to differentiate from lumbar vein (reader-1, n = 1; reader-2, n = 2) in both arterial and venous phase images. Sensitivity of detection of renal veins, left adrenal, gonadal and lumbar veins in arterial phase images was 100, 83-88, 100, and 85-90%, respectively. As expected, venous phase images showed significantly greater opacification of renal veins, left gonadal, adrenal and lumbar veins (p < .05). However, this did not substantially limit the evaluation of renal venous anatomy in arterial phase images. Both readers had substantial interobserver agreement (kappa coefficient, 0.7; p < 0.05). Conclusions: Arterial phase MDCT images alone can be used to detect renal venous anomalies, and to identify small left renal venous branches namely, the left gonadal, adrenal and lumbar veins in renal donors. Venous phase MDCT acquisition is not necessary for evaluation of renal

[Technical points of laparoscopic splenic hilar lymph node dissection--The original intention of CLASS-04 research design].

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Huang, Changming; Lin, Mi

2018-02-25

According to Japanese gastric cancer treatment guidelines, the standard operation for locally advanced upper third gastric cancer is the total gastrectomy with D2 lymphadenectomy, which includes the dissection of the splenic hilar lymph nodes. With the development of minimally invasive ideas and surgical techniques, laparoscopic spleen-preserving splenic hilar lymph node dissection is gradually accepted. It needs high technical requirements and should be carried out by surgeons with rich experience of open operation and skilled laparoscopic techniques. Based on being familiar with the anatomy of splenic hilum, we should choose a reasonable surgical approach and standardized operating procedure. A favorable left-sided approach is used to perform the laparoscopic spleen-preserving splenic hilar lymph node dissection in Department of Gastric Surgery, Fujian Medical University Union Hospital. This means that the membrane of the pancreas is separated at the superior border of the pancreatic tail in order to reach the posterior pancreatic space, revealing the end of the splenic vessels' trunk. The short gastric vessels are severed at their roots. This enables complete removal of the splenic hilar lymph nodes and stomach. At the same time, based on the rich clinical practice of laparoscopic gastric cancer surgery, we have summarized an effective operating procedure called Huang's three-step maneuver. The first step is the dissection of the lymph nodes in the inferior pole region of the spleen. The second step is the dissection of the lymph nodes in the trunk of splenic artery region. The third step is the dissection of the lymph nodes in the superior pole region of the spleen. It simplifies the procedure, reduces the difficulty of the operation, improves the efficiency of the operation, and ensures the safety of the operation. To further explore the safety of laparoscopic spleen-preserving splenic hilar lymph node dissection for locally advanced upper third gastric cancer

PheoSeq : A Targeted Next-Generation Sequencing Assay for Pheochromocytoma and Paraganglioma Diagnostics

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Currás-Freixes, Maria; Piñeiro-Yañez, Elena; Montero-Conde, Cristina; Apellániz-Ruiz, María; Calsina, Bruna; Mancikova, Veronika; Remacha, Laura; Richter, Susan; Ercolino, Tonino; Rogowski-Lehmann, Natalie; Deutschbein, Timo; Calatayud, María; Guadalix, Sonsoles; Álvarez-Escolá, Cristina; Lamas, Cristina; Aller, Javier; Sastre-Marcos, Julia; Lázaro, Conxi; Galofré, Juan C.; Patiño-García, Ana; Meoro-Avilés, Amparo; Balmaña-Gelpi, Judith; De Miguel-Novoa, Paz; Balbín, Milagros; Matías-Guiu, Xavier; Letón, Rocío; Inglada-Pérez, Lucía; Torres-Pérez, Rafael; Roldán-Romero, Juan M.; Rodríguez-Antona, Cristina; Fliedner, Stephanie M J; Opocher, Giuseppe; Pacak, Karel; Korpershoek, Esther; de Krijger, Ronald R.; Vroonen, Laurent; Mannelli, Massimo; Fassnacht, Martin; Beuschlein, Felix; Eisenhofer, Graeme; Cascón, Alberto; Al-Shahrour, Fátima; Robledo, Mercedes

2017-01-01

Genetic diagnosis is recommended for all pheochromocytoma and paraganglioma (PPGL) cases, as driver mutations are identified in approximately 80% of the cases. As the list of related genes expands, genetic diagnosis becomes more time-consuming, and targeted next-generation sequencing (NGS) has

The Epidemiology of Pheochromocytoma: Increasing incidence and changing clinical presentation

DEFF Research Database (Denmark)

Ebbehoj, A; Søndergaard, Esben; Trolle, Christian

2017-01-01

–2015. In 1977–2006 paroxysmal symptoms and secondary hypertension were the primary causes leading to the diagnosis (34 and 21%, respectively), while incidentalomas were the leading cause of diagnosis 2007–2015 (57%). Patients diagnosed 2007–2015 were older (P... diagnosed in 1977–2006 in a secondary analysis using the Wilcoxon–Mann–Whitney test.We identified 183 confirmed cases of pheochromocytoma. A significant increasing trend (P

Identification of 3 novel VHL germ-line mutations in Danish VHL patients

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Dandanell, Mette; Friis-Hansen, Lennart Jan; Sunde, Lone

2012-01-01

von Hippel-Lindau (VHL) disease is a hereditary cancer syndrome in which the patients develop retinal and central nervous system hemangioblastomas, pheochromocytomas and clear-cell renal tumors. The autosomal dominant disease is caused by mutations in the VHL gene.......von Hippel-Lindau (VHL) disease is a hereditary cancer syndrome in which the patients develop retinal and central nervous system hemangioblastomas, pheochromocytomas and clear-cell renal tumors. The autosomal dominant disease is caused by mutations in the VHL gene....

Apolipoprotein E4 Causes Age- and Sex-Dependent Impairments of Hilar GABAergic Interneurons and Learning and Memory Deficits in Mice

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Leung, Laura; Andrews-Zwilling, Yaisa; Yoon, Seo Yeon; Jain, Sachi; Ring, Karen; Dai, Jessica; Wang, Max Mu; Tong, Leslie; Walker, David; Huang, Yadong

2012-01-01

Apolipoprotein (apo) E4 is the major genetic risk factor for Alzheimer's disease (AD). ApoE4 has sex-dependent effects, whereby the risk of developing AD is higher in apoE4-expressing females than males. However, the mechanism underlying the sex difference, in relation to apoE4, is unknown. Previous findings indicate that apoE4 causes age-dependent impairments of hilar GABAergic interneurons in female mice, leading to learning and memory deficits. Here, we investigate whether the detrimental effects of apoE4 on hilar GABAergic interneurons are sex-dependent using apoE knock-in (KI) mice across different ages. We found that in female apoE-KI mice, there was an age-dependent depletion of hilar GABAergic interneurons, whereby GAD67- or somatostatin-positive–but not NPY- or parvalbumin-positive–interneuron loss was exacerbated by apoE4. Loss of these neuronal populations was correlated with the severity of spatial learning deficits at 16 months of age in female apoE4-KI mice; however, this effect was not observed in female apoE3-KI mice. In contrast, we found an increase in the numbers of hilar GABAergic interneurons with advancing age in male apoE-KI mice, regardless of apoE genotype. Moreover, male apoE-KI mice showed a consistent ratio of hilar inhibitory GABAergic interneurons to excitatory mossy cells approximating 1.5 that is independent of apoE genotype and age, whereas female apoE-KI mice exhibited an age-dependent decrease in this ratio, which was exacerbated by apoE4. Interestingly, there are no apoE genotype effects on GABAergic interneurons in the CA1 and CA3 subregions of the hippocampus as well as the entorhinal and auditory cortexes. These findings suggest that the sex-dependent effects of apoE4 on developing AD is in part attributable to inherent sex-based differences in the numbers of hilar GABAergic interneurons, which is further modulated by apoE genotype. PMID:23300939

Mandibular metastasis presenting as the initial maifestation of malignant pheochromocytoma

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Yanagi, Yoshinobu; Asaumi, Jun-ichi E-mail: asaumi@md.okayama-u.ac.jp; Hisatomi, Miki; Konouchi, Hironobu; Wakasa, Toru; Kishi, Kanji

2002-10-01

A case of 59-year-old male patient presenting with metastasis to the mandible from malignant pheochromocytoma is described. The conventional radiographs and CT images suggested that the lesion was malignant osteogenic tumors or metastatic tumors due to the existence of calcification and widesoread periosteal sunburst spiculation. On MRI, an expansive mass was clearly depicted and the signal intensities of the lesion were low to intermediate on T1 weighted image with intermediate to high signal intensity on T2 weighted image. A strong enhancement of the lesion was also observed on contrast enhanced T1 weighted image. On maximum intensity projection image in the arterial phase, the mass showed exceedingly early enhancement and excessively dislocated adjacent vessels. The diagnosis of a pheochromcytoma was difficult on the basis of these imagings. The final diagnosis was based on a biopsy of the mandible and I-131 Meta-iodobenzylguanidine scintigraphy (MIBG) scintigraphy. A primary lesion of the right adrenal showed low uptake due to wide centric necrosis and metastatic lesions of liver, lumber vertebrae, ribs and sacroiliac joint showed high uptake on the I-131 MIBG scintigraphy. The final diagnosis was nonfunctioning malignant pheochromocytoma due to the absence of elevation of catecholamine or its metabolite.

Multiple variations of the renal and testicular vessels: possible embryological basis and clinical importance.

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Mazengenya, Pedzisai

2016-08-01

During routine dissection of the abdominal cavity of a 55-year-old African male cadaver, multiple anomalies including renal and testicular vessels were encountered. The right kidney was supplied by three right hilar renal arteries arising from the abdominal aorta at different vertebral levels whereas only one left renal artery supplied the left kidney. On the right three renal veins drained the kidney into the inferior vena cava. In contrast, the left kidney was drained by a single renal vein which received a large primary posterior tributary. The primary posterior tributary had three tributaries from the posterior lumbar region. The right testis had two sources of arterial supply; one from the subcostal artery and another from the abdominal aorta. The left testis was supplied normally by a single testicular artery. The right testis was drained by four testicular veins as follows: one drained into the subcostal vein, the other two drained separately for a longer course and joined shortly before draining into the right main renal vein, the fourth one drained into the anterior aspect of the inferior vena cava at the level of the second lumbar vertebra. On the left, the testicle was drained by two testicular veins which travelled separately from the deep inguinal ring and joined shortly before they drain into the left renal vein. This variation may represent an immature form of complicated development of kidneys and testes. Additionally, emphasis must be put on preoperative vascular examination to avoid surgical complications from variant vessels in this region.

Cement-augmented dorsal instrumentation of the spine as a safe adjunct to the multimodal management of metastatic pheochromocytoma: a case report

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Rittirsch Daniel

2012-01-01

Full Text Available Abstract Malignant pheochromocytoma is a neuroendocrine tumor that originates from chromaffin tissue. Although osseous metastases are common, metastatic dissemination to the spine rarely occurs. Five years after primary diagnosis of extra-adrenal, abdominal pheochromocytoma and laparoscopic extirpation, a 53-year old patient presented with recurrence of pheochromocytoma involving the spine, the pelvis, both proximal femora and the right humerus. Magnetic resonance imaging and computed tomography revealed osteolytic lesions of numerous vertebrae (T1, T5, T10, and T12. In the case of T10, total destruction of the vertebral body with involvement of the rear edge resulted in the risk of vertebral collapse and subsequent spinal stenosis. Thus, dorsal instrumentation (T8-T12 and cement augmentation of T12 was performed after perioperative alpha- and beta-adrenergic blockade with phenoxybenzamine and bisoprolol. After thorough preoperative evaluation to assess the risk for surgery and anesthesia, and appropriate perioperative management including pharmacological antihypertensive treatment, dorsal instrumentation of T8-T12 and cement augmentation of T12 prior to placing the corresponding pedicle screws did not result in hypertensive crisis or hemodynamic instability due to the release of catecholamines from metastatic lesions. To the authors' knowledge, this is the first report describing cement-augmentation in combination with dorsal instrumentation to prevent osteolytic vertebral collapse in a patient with metastatic pheochromocytoma. With appropriate preoperative measures, cement-augmented dorsal instrumentation represents a safe approach to stabilize vertebral bodies with metastatic malignant pheochromocytoma. Nevertheless, direct manipulation of metastatic lesions should be avoided as far as possible in order to minimize the risk of hemodynamic complications.

Multiple variations of the coeliac axis, hepatic and renal vasculature as incidental findings illustrated by MDCTA.

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Rafailidis, Vasileios; Papadopoulos, Georgios; Kouskouras, Konstantinos; Chryssogonidis, Ioannis; Velnidou, Anastasia; Kalogera-Fountzila, Anna

2016-08-01

Vascular anatomical variations are not uncommon and may affect any organ's arterial or venous vasculature. The coexistence of variations in different organic systems is less commonly found, but of great clinical significance in a series of clinical conditions like organ transplantation and surgical preoperative planning. Multidetector computed tomography angiography (MDCTA) has emerged as a valuable alternative to the conventional angiography for accurate evaluation of vascular anatomy and pathology. Radiologists should be familiar with each organ's vascular variations and always report them to the clinician, even if they represent an incidental finding. This case report presents a 52-year-old female patient undergoing abdominal MDCTA for characterization of a renal lesion. This examination revealed the presence of three hilar arteries on the left kidney, a main renal vein in combination with an additional renal vein in both sides along with a replaced right hepatic artery originating from the superior mesenteric artery. Moreover, both inferior phrenic arteries were found originating from the coeliac axis. 3D volume rendering technique images were used in the evaluation of vascular anatomy as illustrated in this case report.

Metastases but not cardiovascular mortality reduces life expectancy following surgical resection of apparently benign pheochromocytoma.

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Timmers, H J L M; Brouwers, F M; Hermus, A R M M; Sweep, F C G J; Verhofstad, A A J; Verbeek, A L M; Pacak, K; Lenders, J W M

2008-12-01

The treatment of choice for non-metastatic pheochromocytoma is surgical resection. Its goals are to abolish catecholamine hypersecretion, normalize blood pressure, and prevent further tumor growth or progression to metastatic disease. Data on long-term mortality and morbidity after pheochromocytoma surgery are limited. We here report a retrospective study on the long-term outcome after surgery for apparently benign pheochromocytoma at the Radboud University Nijmegen Medical Centre. Data on clinical presentation, treatment, post-surgical blood pressure and recurrence, metastasis and death were collected of 69 consecutive patients (January 1966-December 2000; follow-up: until death or January 2006). Survival was compared with survival of a matched reference population. Two patients died of surgical complications. All ten patients with metastatic disease (including three diagnosed at first surgery) died. At follow-up, 40 patients were alive and recurrence free and three patients were lost to follow up. Two patients experienced a benign recurrence. Mean+/-s.d. follow-up was 10.2+/-7.5 (median 9, range 1-38) years. Kaplan-Meier estimates for 5- and 10-year survival since surgery were 85.8% (95% CI: 77.2-94.4%) and 74.2% (95% CI: 62.0-86.4%) for patients versus 95.5 and 89.4% in the reference population (Prisk of developing metastatic disease. Only one-third becomes normotensive without antihypertensive medication. Therefore, lifelong follow-up is warranted.

Hilar accumulation of gallium-67 in patients with normal chest radiographs

International Nuclear Information System (INIS)

Hoshi, Hiroaki; Yamada, Hiroki; Kawahira, Kozaburo; Watanabe, Katsushi

1982-01-01

Gallium-67 scintigraphy is a useful screening test to detect malignant or inflammatory lesions. However, the accumulations of Gallium-67 in the normal pulmonary hilum are found in some cases. So, 277 cases with Gallium-67 scintigraphy were discussed. The hilar accumulation of Gallium-67 was classified into four grades, namely Grade 0: no Gallium-67 uptake, Grade I: low Gallium-67 uptake, Grade II: moderate Gallium-67 uptake, and Grade III: high Gallium-67 uptake. Gallium-67 uptake was found in 38 of 277 cases (14%). Thirty cases of these were estimated as Grade I (79%). Cases with Grade II were 20.3%, and only two cases were Grade III (0.7%). Gallium-67 accumulation, was bilateral in 28 cases out of 38 and cases with Gallium-67 accumulation increased with age. Twenty five of the 38 cases with Gallium-67 accumulation had such findings as suggesting old pulmonary inflammation though they had no symptoms of respiratory diseases. This study suggests that hilar Gallium-67 accumulation has no correlation with the active inflammation of the lymphnodes. (author)

Lung hilar Ga-67 uptake in patients with lymphoma following chemotherapy.

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Gunay, Emel Ceylan; Salanci, Bilge Volkan; Barista, Ibrahim; Caner, Biray

2004-07-01

Scintigraphic characteristics of lung hilar Ga-67 uptake (HU) and their relationship with the etiology (benign vs. malignant) of the hilar lesions in lymphoma patients following chemotherapy were retrospectively investigated. A total of 161 lymphoma patients were included in the study. The presence/absence of HU and if present, symmetry/asymmetry and intensity of HU (on the basis of a 3 scale grading system) were visually and semiquantitatively assessed on transaxial sections of thorax Ga-67 SPECT. By drawing ROIs over right and left hilum, asymmetry index (AI%) was also calculated. HU was categorized as benign or malignant depending on the radiological correlation and clinical follow-up. In the malignant group, the majority of patients (85.7%) had grade 2 or grade 3 uptake and all had asymmetric pattern. However, in the benign group, grade 1 uptake was more common (66%) and was mainly symmetric (94.6%) in appearance. AI% in the malignant group (73.7 +/- 36.6) was significantly higher than in the benign group (5.7 +/- 4.9) confirming the marked asymmetry in malignant patients.

Pheochromocytoma diagnosed after anticoagulation for atrial fibrillation ablation procedure: a giant in disguise.

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Galvão Braga, Carlos; Ribeiro, Sílvia; Martins, Juliana; Arantes, Carina; Ramos, Vítor; Primo, João; Magalhães, Sónia; Correia, Adelino

2014-04-01

Pheochromocytoma is a rare catecholamine-producing tumor, discovered incidentally in 50% of cases. We present the case of a 44-year-old male with a history of paroxysmal palpitations. Baseline ECG, transthoracic echocardiogram and ECG stress test showed no relevant alterations. Paroxysmal atrial fibrillation was detected on 24-hour Holter ECG. After antiarrhythmic therapy, the patient remained symptomatic, and was accordingly referred for electrophysiological study and atrial fibrillation ablation. Anticoagulation was initiated before the procedure. After ablation and still anticoagulated, he complained of hematospermia. The abdominal and pelvic imaging study showed a 10-cm left adrenal mass, predominantly cystic, compatible with pheochromocytoma, which was confirmed after biochemical tests (increased urine metanephrines and plasma catecholamines). Metaiodobenzylguanidine scintigraphy scanning confirmed localized disease in the adrenal gland, excluding other uptake foci. Following appropriate preoperative management, surgical resection of the giant mass was performed successfully and without complications. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

CT and MRT in mediastinal and hilar masses. CT und MRT bei mediastinalen und hilaeren Raumforderungen

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Prayer, L.; Kramer, J.; Stiglbauer, R.; Wimberger, D.; Imhof, H. (Vienna Univ. (Austria). Klinik fuer Radiodiagnostik Ludwig-Boltzmann-Institut fuer Radiologisch-Physikalische Tumordiagnostik, Vienna (Austria)); Metz, V. (Vienna Univ. (Austria). 2. Chirurgische Klinik)

1990-11-01

Thirty-eight patients with mediastinal and/or hilar masses were imaged by computed tomography (CT) and magnetic resonance imaging (MRI). Results were analyzed retrospectively regarding the ability to demonstrate the masses, their number, size, definition, location and tissue characteristics. CT and MRI showed equivalent results in 32 cases; additional information was obtained in two patients by CT, in four patients by MRI. In view of the specific advantages and limitations of both CT and MRI we believed that in patients with mediastinal and/or hilar masses, contrast enhanced CT remains the procedure of choice after performing plain chest radiographs; in certain cases MRI will prove useful for further evaluation. (orig.).

Quantification of Mediastinal and Hilar Lymph Node Movement Using Four-Dimensional Computed Tomography Scan: Implications for Radiation Treatment Planning

International Nuclear Information System (INIS)

Sher, David J.; Wolfgang, John A.; Niemierko, Andrzej; Choi, Noah C.

2007-01-01

Purpose: To quantitatively describe mediastinal and hilar lymph node movement in patients with lymph node-positive lung cancer. Methods and Materials: Twenty-four patients with lung cancer who underwent four-dimensional computed tomography scanning at Massachusetts General Hospital were included in the study. The maximum extent of superior motion of the superior border was measured, as well as the maximum inferior movement of the inferior border. The average of these two values is defined as the peak-to-peak movement. This process was repeated for mediolateral (ML) and anterior-posterior (AP) movement. Linear regression was used to determine lymph node characteristics associated with peak-to-peak movement. Various uniform expansions were investigated to determine the expansion margins necessary to ensure complete internal target volume (ITV) coverage. Results: The mean peak-to-peak displacements of paratracheal lymph nodes were 4 mm (craniocaudal [CC]), 2 mm (ML), and 2 mm (AP). For subcarinal lymph nodes, the mean peak-to-peak movements were 6 mm (CC), 4 mm (ML), and 2 mm (AP). The mean peak-to-peak displacements of hilar lymph nodes were 7 mm (CC), 1 mm (ML), and 4 mm (AP). On multivariate analysis, lymph node station and lymph node size were significantly related to peak-to-peak movement. Expansions of 8 mm for paratracheal nodes and 13 mm for subcarinal and hilar nodes would have been necessary to cover the ITV of 95% of these nodal masses. Conclusions: Subcarinal and hilar lymph nodes may move substantially throughout the respiratory cycle. In the absence of patient-specific information on nodal motion, expansions of at least 8 mm, 13 mm, and 13 mm should be considered to cover the ITV of paratracheal, subcarinal, and hilar lymph nodes, respectively

Left hepatectomy combined with hepatic artery resection for hilar cholangiocarcinoma: A retrospective cohort study.

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Peng, Chihan; Li, Chuan; Wen, Tianfu; Yan, Lvnan; Li, Bo

2016-08-01

To investigate the efficacy of our technique and policy on left hepatectomy (LH) with hepatic artery resection but without arterial reconstruction (HAR) in selected patients with hilar cholangiocarcinoma. From May 2005 to May 2012, 61 patients with hilar cholangiocarcinoma underwent left hepatectomy. These patients were divided into two groups: the LH with HAR group (n = 26) and the LH alone group (n = 35), based on whether hepatic artery resection was performed. We evaluated the serum total and direct bilirubin on postoperative day 7, length of hospital stay after surgery, postoperative complications, long-term postoperative survival and disease-free survival. The improvement in jaundice after surgery was comparable between the two groups (P = 0.837). There were no significant differences in the rates of postoperative complications or mortality between the LH with HAR group and the LH group (P = 0.654 and no assessment, respectively). The cumulative 1-, 2-, 3- and 5-year survival rates were 61.5%, 49%, 40.8% and 30.6% and 71.4%, 58.7%, 51.3% and 38.5%, respectively, in the LH with HAR group and the LH group (P = 0.383, including perioperative deaths). The cumulative 1-, 2-, 3- and 5-year disease-free survival rates were 61.9%, 41.6%, 29.7% and 14.8% and 58.2%, 50.7%, 44.3% and 23.6% in the LH with HAR group and the LH group, respectively (P = 0.695, including perioperative deaths). The postoperative complication rate was higher in patients with severe jaundice than those with non-severe jaundice, but no significant difference was detected (56.3% (9/16) vs. 46.7% (46.7%), P = 0.804). Similarly, 18.8% (3/16) postoperative mortality was found in patients with severe jaundice, compared to 4.4% (2/45) in those with non-severe jaundice. The difference was not significant (P = 0.139). For the cumulative 1-, 2-, 3- and 5-year survival and cumulative 1-, 2-, 3- and 5-year disease-free survival rates, patients with severe jaundice had poorer outcomes than

INFLUENCE OF INTERRUPTED PULMONARY LYMPH-DRAINAGE ON ANTIBODY-RESPONSES IN HILAR-STRIPPED LUNGS

NARCIS (Netherlands)

WANG, FZ; WINTER, JB; WILDEVUUR, CRH; PROP, J

1992-01-01

Lung transplantation interrupts hilar lymphatics. This may have an impact on immune responses to antigens entering the lung because the antigens cannot reach the lung-associated lymph nodes where the immune response is generated. We investigated the interruption and regeneration of lymphatics and

The bovine kidney as an experimental model in urology: external gross anatomy.

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Carvalho, Francismar S; Bagetti Filho, Hélio J S; Henry, Robert W; Pereira-Sampaio, Marco A

2009-01-01

The objective of this work was to obtain and record detailed and accurate measurements of the bovine kidney and to compare these new data with findings in humans. Thirty-eight bovine kidneys were used. The total number of lobes, along with the number of lobes located in the cranial polar, caudal polar and hilar regions, were recorded. Several measurements of the kidneys were made and evaluated. The hilar region presents the greatest length (mean of 76.87 mm) of the 3 renal regions of the kidney. The large area of the bovine renal hilus could make access to hilar structures easier than in the human kidney. The coefficient of variation for renal length was small (8.14%), while the coefficient of variation for the lobar number was high (26.82%). The number of renal lobes ranged from 13 to 35, with a mean of 20.62. The hilar region presents the highest number of lobes, while the cranial pole presents the lowest. The number of lobes in the cranial and caudal poles increases with the width of these regions. This is different from the hilar region, in which the lobar number increases with the length of the hilus. These data indicate that the adult bovine kidney can be used as a model for certain urologic procedures, but researchers must be aware that there are some major differences between the adult bovine kidney and the human kidney, as indicated by the data reported in this paper. (c) 2008 S. Karger AG, Basel.

CT appearance of hilar and mediastinal enlarged lymph nodes of coal worker's pneumoconiosis

International Nuclear Information System (INIS)

Ma Daqing; Guan Yansheng; Tang Hongqu; He Wen; Chen Budong; Zhang Yansong; Li Jun

2000-01-01

Objective: To study the CT appearance of the hilar and mediastinal enlarged lymph nodes in coal worker's pneumoconiosis (CWP), its pathological basis and diagnostic value for CWR complicated with lung cancer. Methods: (1) Twelve isolated lungs with CWP obtained at autopsy were inflated and fixed. CT scan was performed. The pathologic findings of enlarged lymph nodes were identified. (2) CT findings of hilar and mediastinal enlarged lymph nodes of 71 cases with CWP and 22 cases of CWP complicated with lung cancer were analyzed. Results: (1) Most of the enlarged hilar and mediastinal lymph nodes in simple CWR was in third stage of fibrosis. The fourth stage of fibrosis was only seen in lymph nodes of a case with complicated CWP. In this case the necrotic materials of lymph nodes eroded adjacent bronchi and vessels, and coalesced with progressive massive fibrosis (PMF). (2) The average number of lymph nodes in cases of complicated CWP was more than that of simple CWP (P 2 cm was 7.4%. (3) The prevalence of lymph nodes calcification in CWP was 61.1%, but egg shell calcification was only 14.7%. (4) In the cases of CWP complicated with lung cancer, lymph node > 2 cm was 20.8%, that was more than CWP (P 3 cm was 7.6%. Conclusion: Lymph nodes up to 1 cm may have dust fibrosis and coal silicosis nodules. The lymph nodes >2 cm is more common in CWP complicated with lung cancer than in simple CWP. The lymph nodes > 3 cm indicates higher probability of CWP with lung cancer than PMF

Miocardiopatía dilatada y feocromocitoma Dilated myocardiopathy and pheochromocytoma

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José Luis González González

2013-03-01

Full Text Available El feocromocitoma, un tumor de baja incidencia con un comportamiento benigno en la mayoría de los casos, puede ser el responsable de extensas manifestaciones cardiovasculares.Las manifestaciones sobre el miocardio pueden ser de profundas consecuencias, pues llegan a producir una miocardiopatía dilatada con insuficiencia cardiaca severa. La exéresis del tumor suele ser curativa en el 90 % de los casos y el manejo perioperatorio muy complejo. Se presenta un paciente joven portador de un feocromocitoma , que sometido a la larga acción de las catecolaminas llegó a desarrollar una miocardiopatía dilatada con criterio de trasplante cardiaco. La cirugía adrenal fue curativa en nuestro paciente.Pheochromocytoma, a low incidence tumor of benign behavior in most of cases, can cause extensive cardiovascular manifestations. These manifestations on the myocardium can have deep consequences since they may lead to dilated myocardiopathy with severe heart failure. The excision of tumor may be the curative solution for 90 % of cases but the perioperative management is very complex. This is the case of a young patient who carries pheochromocytoma and after a long-acting catecholemine-based treatment, he developed dilated myocardiopathy, and the medical criterion was that he required heart transplantation. The adrenal surgery was successfully healing in our patient.

Unilateral and Bilateral Adrenalectomy for Pheochromocytoma Requires Adjustment of Urinary and Plasma Metanephrine Reference Ranges

NARCIS (Netherlands)

Osinga, Thamara E.; van den Eijnden, Maartje H. A.; Kema, Ido P.; Kerstens, Michiel N.; Dullaart, Robin P. F.; de Jong, Wilhelmina H. A.; Sluiter, Wim J.; Links, Thera P.; van der Horst-Schrivers, Anouk N. A.

Context: Follow-up after adrenalectomy for pheochromocytoma is recommended because of a recurrence risk. During follow-up, plasma and/or urinary metanephrine (MN) and normetanephrine (NMN) are interpreted using reference ranges obtained in healthy subjects. Objective: Because adrenalectomy may

Correct diagnosis of vascular encasement and longitudinal extension of hilar cholangiocarcinoma by four-channel multidetector-row computed tomography

International Nuclear Information System (INIS)

Okumoto, Tadayuki; Yamada, Takayuki; Sato, Akihiro

2009-01-01

Accurate diagnosis of local invasion of hilar cholangiocarcinomas is challenging due to their small size and the anatomic complexity of the hepatic hilar region. On the other hand, the correct diagnosis of local invasion is essential for assuring the possibility of curative surgery. The purpose of this study was to evaluate the feasibility of four-channel multidetector-row computed tomography (MDCT) in the assessment of vascular and bile duct involvement, by which we could obtain useful information for the surgical management of hilar cholangiocarcinoma. The subjects were 18 patients for whom the extent of tumor invasion was surgically and pathologically confirmed. All patients underwent preoperative multiphasic CT scanning by MDCT. Arterial and portal dominant phases were acquired using a detector configuration of 1.25 mm x 4 mm, and both axial and multiplanar reconstructed images were interpreted. Longitudinal extension was evaluated up to second-order branches. Vascular invasion is considered to be the degree of tumor contiguity to the hepatic arteries and portal vein and was graded by CT. The longitudinal extension was correctly diagnosed in 14 patients (77.8%). Hepatic artery invasion was correctly diagnosed in 17 patients with sensitivity of 100% and specificity of 90%, respectively. Portal vein invasion was correctly diagnosed in 47 of 51 branches with sensitivity and specificity of 92.3% and 90.2%, respectively. Multiplanar reconstructed images contributed to the correct diagnosis for both vascular encasement and longitudinal tumor extension. In conclusion, MDCT is useful in preoperative evaluation of hilar cholangiocarcinoma, especially when combined with multiplanar reconstructed images. (author)

The importance of complete excision of the caudate lobe in resection of hilar cholangiocarcinoma

NARCIS (Netherlands)

Dinant, Sander; Gerhards, Michael F.; Busch, Olivier R. C.; Obertop, Hugo; Gouma, Dirk J.; van Gulik, Thomas M.

2005-01-01

Background: The numbers of margin-negative resections and survival times have greatly improved because of a more aggressive surgical approach to resectable hilar cholanciocarcinoma (Klatskin tumour). It was shown initially by Japanese authors that complete resection of the caudate lobe together with

Krebs cycle metabolite profiling for identification and stratification of pheochromocytomas/paragangliomas due to succinate dehydrogenase deficiency

NARCIS (Netherlands)

Richter, S; Peitzsch, M.; Rapizzi, E.; Lenders, J.W.M.; Qin, N.; Cubas, A.A. de; Schiavi, F.; Rao, J.U.; Beuschlein, F.; Quinkler, M.; Timmers, H.J.L.M.; Opocher, G.; Mannelli, M.; Pacak, K.; Robledo, M.; Eisenhofer, G.

2014-01-01

CONTEXT: Mutations of succinate dehydrogenase A/B/C/D genes (SDHx) increase susceptibility to development of pheochromocytomas and paragangliomas (PPGLs), with particularly high rates of malignancy associated with SDHB mutations. OBJECTIVE: We assessed whether altered succinate dehydrogenase

Visual Enhancement of Laparoscopic Partial Nephrectomy With 3-Charge Coupled Device Camera: Assessing Intraoperative Tissue Perfusion and Vascular Anatomy by Visible Hemoglobin Spectral Response

Science.gov (United States)

2010-10-01

reliably distinguish vascular structures during hilar dissection, and detect and monitor changes in renal tissue perfus:ion dw·ing and after warm...and in 25 patients with hilar tu- mors 16 in whom perioperative outcomes were com- parable to those of peripheral tumors. In a retro- spective study...Richstone et al also reported the safe performance of LPN for hilar tumors in 17 patients.17 Repeat partial nephrectomy for ipsilat- eral tumor has

Aortoarteritis: Could it be a form of catecholamine-induced vasculitis?

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Vijaya Sarathi

2013-01-01

Full Text Available Catecholamine-induced vasculitis is a well known but rarely described entity. However, aortoarteritis as a manifestation of catecholamine-induced vasculitis is not described in the literature. We have reported two patients in whom pheochromocytoma coexisted with aortoarteritis. Both patients were young females with history of bilateral pheochromocytomas in more than one first-degree relative. Both patients also had bilateral adrenal pheochromocytomas (second patient also had paraganglioma at left renal hilum with elevation of plasma free normetanephrine levels. We conclude that there may be an association between pheochromocytoma and aortoarteritis, and that catecholamine excess may have a role in the etiopathogenesis of aortoarteritis in these patients.

Aortoarteritis: Could it be a form of catecholamine-induced vasculitis?

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Sarathi, Vijaya; Lila, Anurag R.; Bandgar, Tushar R.; Shah, Nalini S.

2013-01-01

Catecholamine-induced vasculitis is a well known but rarely described entity. However, aortoarteritis as a manifestation of catecholamine-induced vasculitis is not described in the literature. We have reported two patients in whom pheochromocytoma coexisted with aortoarteritis. Both patients were young females with history of bilateral pheochromocytomas in more than one first-degree relative. Both patients also had bilateral adrenal pheochromocytomas (second patient also had paraganglioma at left renal hilum) with elevation of plasma free normetanephrine levels. We conclude that there may be an association between pheochromocytoma and aortoarteritis, and that catecholamine excess may have a role in the etiopathogenesis of aortoarteritis in these patients. PMID:23776874

Precision medicine in pheochromocytoma and paraganglioma: current and future concepts.

Science.gov (United States)

Björklund, P; Pacak, K; Crona, J

2016-12-01

Pheochromocytoma and paraganglioma (PPGL) are rare diseases but are also amongst the most characterized tumour types. Hence, patients with PPGL have greatly benefited from precision medicine for more than two decades. According to current molecular biology and genetics-based taxonomy, PPGL can be divided into three different clusters characterized by: Krebs cycle reprogramming with oncometabolite accumulation or depletion (group 1a); activation of the (pseudo)hypoxia signalling pathway with increased tumour cell proliferation, invasiveness and migration (group 1b); and aberrant kinase signalling causing a pro-mitogenic and anti-apoptotic state (group 2). Categorization into these clusters is highly dependent on mutation subtypes. At least 12 different syndromes with distinct genetic causes, phenotypes and outcomes have been described. Genetic screening tests have a documented benefit, as different PPGL syndromes require specific approaches for optimal diagnosis and localization of various syndrome-related tumours. Genotype-tailored treatment options, follow-up and preventive care are being investigated. Future new developments in precision medicine for PPGL will mainly focus on further identification of driver mechanisms behind both disease initiation and malignant progression. Identification of novel druggable targets and prospective validation of treatment options are eagerly awaited. To achieve these goals, we predict that collaborative large-scale studies will be needed: Pheochromocytoma may provide an example for developing precision medicine in orphan diseases that could ultimately aid in similar efforts for other rare conditions. © 2016 The Association for the Publication of the Journal of Internal Medicine.

Anesthesia for combined cesarean section and pheochromocytoma resection

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Sadhana Kulkarni

2017-01-01

Full Text Available Pheochromocytoma (PCC is a rare cause of hypertension during pregnancy [1:54000 pregnancies]. Fetomaternal morbidity and mortality is about 58% if the diagnosis is missed. Administration of anesthesia to patients with PCC is challenging. Associated pregnancy adds to the problems. This is a case report of a patient having PCC diagnosed at 26 weeks of gestation. With medical management pregnancy was continued till 34 weeks. She was posted for cesarean section and resection of PCC. Patient underwent surgery lasting for 7 h due to inferior vena cava tear and had stormy intra as well as postoperative course. Mother and baby had uneventful recovery due to continuous invasive monitoring and a good teamwork, despite limited anesthetic resources.

Management of hilar bile duct carcinoma with high-dose radiotherapy and expandable metallic stent placement

International Nuclear Information System (INIS)

Saito, Hiroya; Takamura, Akio

2000-01-01

This article describes our experience with high-dose radiotherapy in combination with the placement of expandable metallic stents (EMS) in the management of hilar bile duct carcinoma. Between 1988 and 1999, 107 consecutive patients with hilar bile duct carcinoma were treated with EMS placement either alone or in combination with high-dose radiotherapy. External beam radiotherapy (EBRT) was indicated in 101 patients, and in 86 this was combined with intraluminal 192 Ir irradiation (ILRT, 59-98 Gy) EMS were placed after the completion of radiotherapy. The 1-, 2-, 3-, and 5-year actuarial survival rates for the radiotherapy group were 66.4%, 23.4%, 15.6%, 7.8%, respectively, and the 1- and 2-year actuarial survival rates for the nonradiotherapy group were 66.4% and 0%, respectively. The placement of EMS was useful for the early establishment of an internal bile passage in radically irradiated patients and the 1-, 2-, 3-, and 5-year actuarial patency rates for the radiotherapy group were 56.3%, 45.3%, 35.2%, and 23.4%, respectively, and the 1- and 2-year actuarial patency rates for the non radiotherapy group were 50.0% and 0% respectively. High-dose radiotherapy, consisting of ILRT and EBRT, appears to be feasible in the management of hilar bile duct carcinoma, and it offers a survival advantage for patients no suited for surgical resection. The placement of EMS assists the internal bile flow and lengthens survival after high-dose radiotherapy. (author)

Renal malignant solitary fibrous tumor with single lymph node involvement: report of unusual metastasis and review of the literature

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Mearini E

2014-05-01

Full Text Available Ettore Mearini,1 Giovanni Cochetti,1 Francesco Barillaro,1 Sonia Fatigoni,2 Fausto Roila2 1Department of Medical-Surgical Specialties and Public Health, Division of Urological Andrological Surgery and Minimally Invasive Techniques, University of Perugia, Terni, Italy; 2Medical Oncology, S Maria Hospital, Terni, Italy Abstract: Solitary fibrous tumors are rare mesenchymal spindle cell neoplasms that are usually found in the pleura. The kidneys are an uncommon site and only few cases of renal solitary fibrous tumor exhibit malignant behavior metastasizing to the liver, lung, and bone through the hematogenous pathway. Purpose: To describe the first case of lymph node metastasis from renal solitary fibrous tumor in order to increase the knowledge about the malignant behavior of these tumors. Patients and methods: A 19-year-old female patient had intermittent hematuria for several months without flank pain or other symptoms. A chest and abdomen CT scan was performed and showed a multi-lobed bulky solid mass of 170 × 98 × 120 mm in the left kidney. One day before the surgery, the left renal artery was catheterized and the kidney embolization was performed using a Haemostatic Absorbable Gelatin Sponge and polyvinyl alcohol. We then performed a radical nephrectomy with hilar, para-aortic, and inter-aortocaval lymphadenectomy. Results: Estimated intraoperative blood loss was 200 mL and the operative time was 100 minutes. No postoperative complications occurred. The hospital stay was 7 days long. The histological examination was malignant solitary fibrous tumor of the kidney. Cancerous tissue showed cellular atypia, with an increased mitotic index (up to 7 × 10 hpf. Immunohistochemical analysis showed positive results for CD34, BCL2, partial expression of HBME1, and occasionally of synaptophysin. Histological evaluation confirmed the presence of metastasis in one hilar node. The patient did not receive any other therapy. At 30-month follow-up, the

Review of succinate dehydrogenase-deficient renal cell carcinoma with focus on clinical and pathobiological aspects

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Naoto Kuroda

2016-05-01

Full Text Available Succinate dehydrogenase (SDH-deficient renal cell carcinoma (RCC was first identified in 2004 and has been integrated into the 2016 WHO classification of RCC. Succinate dehydrogenase (SDH is an enzyme complex composed of four protein subunits (SDHA, SDHB, SDHC and SDHD. The tumor which presents this enzyme mutation accounts for 0.05 to 0.2% of all renal carcinomas. Multiple tumors may occur in approximately 30% of affected patients. SDHB-deficient RCC is the most frequent, and the tumor histologically consists of cuboidal cells with eosinophilic cytoplasm, vacuolization, flocculent intracytoplasmic inclusion and indistinct cell borders. Ultrastructurally, the tumor contains abundant mitochondria. Immunohistochemically, tumor cells are positive for SDHA, but negative for SDHB in SDHB-, SDHC- and SDHD-deficient RCCs. However, SDHA-deficient RCC shows negativity for both SDHA and SDHB. In molecular genetic analyses, a germline mutation in the SDHB , SDHC or SDHD gene (in keeping with most patients having germline mutations in an SDH gene has been identified in patients with or without a family history of renal tumors, paraganglioma/pheochromocytoma or gastrointestinal stromal tumor. While most tumors are low grade, some tumors may behave in an aggressive fashion, particularly if they are high nuclear grade, and have coagulative necrosis or sarcomatoid differentiation.

A modified method using a two-port approach for accessing the hilar vasculature without transferring an endostapler from camera port to utility port during thoracoscopic right upper lobectomy.

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Jiao, W; Zhao, Y; Xuan, Y; Wang, M

2015-02-01

For thoracoscopic upper lobectomies, most cutting endostaplers must be inserted through the camera port when using a two-port approach. Access to the hilar vasculature through only the utility port remains a challenge. In this study, we describe a procedure to access the hilar vasculature without transferring the endostapler site during a thoracoscopic right upper lobectomy. A 2.5-cm utility anterior incision was made in the fourth intercostal space. The posterior mediastinal visceral pleura were dissected to expose the posterior portion of the right upper bronchus and the anterior trunk of the right pulmonary artery. The pleura over the right hilar vasculature were then peeled with an electrocoagulation hook. The anterior trunk of the right pulmonary artery was then transected with a cutting endostapler through the utility port firstly. This crucial maneuver allowed the endostapler access to the right upper lobe pulmonary vein. The hilar structures were then easily handled in turn. This novel technique was performed successfully in 32 patients, with no perioperative deaths. The average operation time was 120.6 min (range 75-180 min). This novel technique permits effective control of the hilar vessels through the utility port, enabling simple, safe, quick and effective resection.

Tumor markers as a diagnostic key for hilar Cholangiocarcinoma

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Juntermanns B

2010-08-01

Full Text Available Abstract Objective Hilar cholangiocarcinoma is the fourth most common gastrointestinal malignancy. CA19-9 and CEA are helpful devices in the management of gastrointestinal malignancies and belong to clinical routine in surgical oncology. But the validity of these parameters in terms of tumor extension and prognosis of bile duct malignancies still remains unclear. Methods From 1998 to 2008, we obtained preoperative CA19-9 and CEA serum levels in 136 patients with hilar cholangiocarcinoma. We correlated tumor stage, resectability rate and survival with preoperative CA 19-9 and CEA serum levels. Results CA19-9 (UICC I: 253 ± 561 U/ml; UICC II: 742 ± 1572 U/ml; UICC III: 906 ± 1708 U/ml; UICC IV: 1707 ± 3053 U/ml and CEA levels (UICC I: 2.9 ± 3.8 U/ml; UICC II: 4.6 ± 6.5 U/ml; UICC III: 18.1 ± 29.6 U/ml; UICC IV: 22.7 ± 53.9 U/ml increase significantly with rising tumor stage. Patients with pre operative serum levels of CA19-9 (> 1000 U/ml and CEA (> 14.4 ng/ml showed a significant poorer resectability rate and survival than patients with lower CA19-9 and CEA serum levels respectively. Conclusion CA19-9 and CEA serum levels are associated with the tumor stage. If preoperatively obtained CA19-9 and CEA serum levels are highly elevated patients have an even worse survival and the frequency of irresectability is significantly higher.

Hilar Interneuron Vulnerability Distinguishes Aged Rats With Memory Impairment

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Spiegel, Amy M.; Koh, Ming Teng; Vogt, Nicholas M.; Rapp, Peter R.; Gallagher, Michela

2016-01-01

Hippocampal interneuron populations are reportedly vulnerable to normal aging. The relationship between interneuron network integrity and age-related memory impairment, however, has not been tested directly. That question was addressed in the present study using a well-characterized model in which outbred, aged, male Long-Evans rats exhibit a spectrum of individual differences in hippocampal-dependent memory. Selected interneuron populations in the hippocampus were visualized for stereological quantification with a panel of immunocytochemical markers, including glutamic acid decarboxylase-67 (GAD67), somatostatin, and neuropeptide Y. The overall pattern of results was that, although the numbers of GAD67- and somatostatin-positive interneurons declined with age across multiple fields of the hippocampus, alterations specifically related to the cognitive outcome of aging were observed exclusively in the hilus of the dentate gyrus. Because the total number of NeuN-immunoreactive hilar neurons was unaffected, the decline observed with other markers likely reflects a loss of target protein rather than neuron death. In support of that interpretation, treatment with the atypical antiepileptic levetiracetam at a low dose shown previously to improve behavioral performance fully restored hilar SOM expression in aged, memory-impaired rats. Age-related decreases in GAD67- and somatostatin-immunoreactive neuron number beyond the hilus were regionally selective and spared the CA1 field of the hippocampus entirely. Together these findings confirm the vulnerability of hippocampal interneurons to normal aging and highlight that the integrity of a specific subpopulation in the hilus is coupled with age-related memory impairment. PMID:23749483

Cytocidal activities of topoisomerase 1 inhibitors and 5-azacytidine against pheochromocytoma/paraganglioma cells in primary human tumor cultures and mouse cell lines.

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James F Powers

Full Text Available There is currently no effective treatment for metastatic pheochromocytomas and paragangliomas. A deficiency in current chemotherapy regimens is that the metastases usually grow very slowly. Drugs that target dividing tumor cells have therefore had limited success. To improve treatment, new strategies and valid experimental models are required for pre-clinical testing. However, development of models has itself been hampered by the absence of human pheochromocytoma/paraganglioma cell lines for cultures or xenografts. Topoisomerase 1 (TOP1 inhibitors are drugs that interfere with mechanisms that maintain DNA integrity during transcription in both quiescent and dividing cells. We used primary cultures of representative human tumors to establish the cytotoxicity of camptothecin, a prototypical TOP1 inhibitor, against non-dividing pheochromocytoma/paraganglioma cells, and then employed a mouse pheochromocytoma model (MPC to show that efficacy of low concentrations of camptothecin and other TOP1 inhibitors is increased by intermittent coadministration of sub-toxic concentrations of 5-azacytidine, a DNA methylation inhibitor that modulates transcription. We then tested the same drugs against a clonal MPC derivative that expresses CMV reporter-driven luciferase and GFP, intended for in vivo drug testing. Unexpectedly, luciferase expression, bioluminescence and GFP expression were paradoxically increased by both camptothecin and SN38, the active metabolite of irinotecan, thereby masking cell death. Expression of chromogranin A, a marker for neuroendocrine secretory granules, was not increased, indicating that the drug effects on levels of luciferase and GFP are specific to the GFP-luciferase construct rather than generalized cellular responses. Our findings provide proof of principle for use of TOP1 inhibitors against pheochromocytoma/paraganglioma and suggest novel strategies for enhancing efficacy and reducing toxicity by optimizing the combination and

[Clinical application of combined hepatic artery resection and reconstruction in surgical treatment for hilar cholangiocarcinoma].

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Dai, H S; Bie, P; Wang, S G; He, Y; Li, D J; Tian, F; Zhao, X; Chen, Z Y

2018-01-01

Objective: To clarify whether the surgical treatment for hilar cholangiocarcinoma combined with artery reconstruction is optimistic to the patients with hilar cholangiocarcinoma with hepatic artery invasion. Methods: There were 384 patients who received treatment in the First Affiliated Hospital to Army Medical University from January 2008 to January 2016 analyzed retrospectively. There were 27 patients underwent palliative operation, 245 patients underwent radical operation, radical resection account for 63.8%. Patients were divided into four groups according to different operation method: routine radical resection group( n =174), portal vein reconstruction group ( n =47), hepatic artery reconstruction group ( n =24), palliative group( n =27). General information of patients who underwent radical operation treatment was analyzed by chi-square test and analysis of variance. The period of operation time, blood loss, the length of hospital stay and hospitalization expenses of the radical operation patients were analyzed by one-way ANOVA. Comparison among groups was analyzed by LSD- t test. Results: The follow-up ended up in June first, 2016. Each of patients followed for 6 to 60 months, the median follow-up period was 24 months. 1-, 3-, and 5-year survival rates were 81.3%, 44.9% and 13.5% of routine radical operation group, and were 83.0%, 44.7% and 15.1% of portal vein reconstruction group, and were 70.8%, 27.7% and 6.9% of hepatic artery reconstruction group, respectively. And 1-, 3-, and 5-year survival rates of hepatic artery reconstruction group was lower than routine radical group and portal vein reconstruction group significantly ( P 0.05). The data shows that the ratio of lymphatic metastasis in hepatic artery reconstruction group (70.8%) is much higher than them in routine radical operation group (20.1%) and portal vein reconstruction group (19.1%) significantly ( P hilar cholangiocarcinoma. Cox regression analysis indicate that hepatic artery resection and

The value of digital tomosynthesis of the chest as a problem-solving tool for suspected pulmonary nodules and hilar lesions detected on chest radiography.

Science.gov (United States)

Galea, Angela; Dubbins, Paul; Riordan, Richard; Adlan, Tarig; Roobottom, Carl; Gay, David

2015-05-01

To assess the capability of digital tomosynthesis (DTS) of the chest compared to a postero-anterior (PA) and lateral chest radiograph (CXR) in the diagnosis of suspected but unconfirmed pulmonary nodules and hilar lesions detected on a CXR. Computed tomography (CT) was used as the reference standard. 78 patients with suspected non-calcified pulmonary nodules or hilar lesions on their CXR were included in the study. Two radiologists, blinded to the history and CT, prospectively analysed the CXR (PA and lateral) and the DTS images using a picture archiving and communication workstation and were asked to designate one of two outcomes: true intrapulmonary lesion or false intrapulmonary lesion. A CT of the chest performed within 4 weeks of the CXR was used as the reference standard. Inter-observer agreement and time to report the modalities were calculated for CXR and DTS. There were 34 true lesions confirmed on CT, 12 were hilar lesions and 22 were peripheral nodules. Of the 44 false lesions, 37 lesions were artefactual or due to composite shadow and 7 lesions were real but extrapulmonary simulating non-calcified intrapulmonary lesions. The PA and lateral CXR correctly classified 39/78 (50%) of the lesions, this improved to 75/78 (96%) with DTS. The sensitivity and specificity was 0.65 and 0.39 for CXR and 0.91 and 1 for DTS. Based on the DTS images, readers correctly classified all the false lesions but missed 3/34 true lesions. Two of the missed lesions were hilar in location and one was a peripheral nodule. All three missed lesions were incorrectly classified on DTS as composite shadow. DTS improves diagnostic confidence when compared to a repeat PA and lateral CXR in the diagnosis of both suspected hilar lesions and pulmonary nodules detected on CXR. DTS is able to exclude most peripheral pulmonary nodules but caution and further studies are needed to assess its ability to exclude hilar lesions. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Surgical and Palliative Management and Outcome in 184 Patients With Hilar Cholangiocarcinoma

Science.gov (United States)

Witzigmann, Helmut; Berr, Frieder; Ringel, Ulrike; Caca, Karel; Uhlmann, Dirk; Schoppmeyer, Konrad; Tannapfel, Andrea; Wittekind, Christian; Mossner, Joachim; Hauss, Johann; Wiedmann, Marcus

2006-01-01

Objective: First, to analyze the strategy for 184 patients with hilar cholangiocarcinoma seen and treated at a single interdisciplinary hepatobiliary center during a 10-year period. Second, to compare long-term outcome in patients undergoing surgical or palliative treatment, and third to evaluate the role of photodynamic therapy in this concept. Summary Background Data: Tumor resection is attainable in a minority of patients (<30%). When resection is not possible, radiotherapy and/or chemotherapy have been found to be an ineffective palliative option. Recently, photodynamic therapy (PDT) has been evaluated as a palliative and neoadjuvant modality. Methods: Treatment and outcome data of 184 patients with hilar cholangiocarcinoma were analyzed prospectively between 1994 and 2004. Sixty patients underwent resection (8 after neoadjuvant PDT); 68 had PDT in addition to stenting and 56 had stenting alone. Results: The 30-day death rate after resection was 8.3%. Major complications occurred in 52%. The overall 1-, 3-, and 5-year survival rates were 69%, 30%, and 22%, respectively. R0, R1, and R2 resection resulted in 5-year survival rates of 27%, 10%, and 0%, respectively. Multivariate analysis identified R0 resection (P < 0.01), grading (P < 0.05), and on the limit to significance venous invasion (P = 0.06) as independent prognostic factors for survival. PDT and stenting resulted in longer median survival (12 vs. 6.4 months, P < 0.01), lower serum bilirubin levels (P < 0.05), and higher Karnofsky performance status (P < 0.01) as compared with stenting alone. Median survival after PDT and stenting, but not after stenting alone, did not differ from that after both R1 and R2 resection. Conclusion: Only complete tumor resection, including hepatic resection, enables long-term survival for patients with hilar cholangiocarcinoma. Palliative PDT and subsequent stenting resulted in longer survival than stenting alone and has a similar survival time compared with incomplete R1 and

Incidental finding of pheochromocytoma after imaging exam

Directory of Open Access Journals (Sweden)

Bianca Lara Teixeira

2017-11-01

Full Text Available Pheochromocytomas — 90% of those being benign — are relatively rare tumors of the adrenal medulla, catecholamines producers. About 75% of patients manifest the sudden classic triad characterized by severe hypertension, palpitations and headache. The diagnosis is confirmed by urinary metanephrines dosage, and radiological tests such as ultrasonography, computed tomography and magnetic resonance imaging. The curative treatment consists in removing the tumor, and videolaparoscopy is the chosen surgical technique. This report describes the case of a woman, who sought the emergency unit complaining of left back, and after imaging tests, was evidenced a large adrenal mass. The patient presented as a symptom linked to the secreting tumor was only secondary hypertension. It was opted to perform an open surgery as a consequence of the tumor dimensions, and the person presents herself normotensive after left adrenectomy.

Comparison of the 68Ga-DOTATATA PET/CT, FDG PET/CT, and MIBG SPECT/CT in the Evaluation of Suspected Primary Pheochromocytomas and Paragangliomas.

Science.gov (United States)

Jing, Hongli; Li, Fang; Wang, Ling; Wang, Zhenghua; Li, Wei; Huo, Li; Zhang, Jingjing

2017-07-01

Anatomical imaging modalities including CT and MRI are the mainstay of evaluation of primary pheochromocytoma or paraganglioma. However, nuclear medicine imaging is frequently necessary to determine the nature of the lesions. The purpose of this investigation is to assess which commonly used nuclear medicine modality might have a better diagnostic value in this clinical setting. Eight patients who had been suspected of having either primary pheochromocytoma or primary paraganglioma and 1 patient with known pheochromocytoma were included in the analysis. Among the 8 patients without known diagnosis, 7 had been suggested by anatomical imaging modalities, whereas one of them presented with initial negative anatomical imaging interpretation. All of 9 patients underwent Ga-DOTATATA PET/CT, FDG PET/CT, and MIBG SPECT/CT for further evaluation. The imaging findings were compared with postsurgical pathology and follow-up. Both Ga-DOTATATA PET/CT and MIBG SPECT/CT accurately identified 9 primary tumors, whereas FDG PET/CT showed increased activity in 8 of 9 primary tumors. Both Ga-DOTATATA and FDG PET/CT are able to detect associated extra-adrenal lesions not shown on MIBG study in patients with multiple endocrine neoplasia syndrome. Ga-DOTATATA PET/CT could be the nuclear medicine imaging choice to evaluate suspected primary pheochromocytoma or paraganglioma, especially in the situation of multiple endocrine neoplasia syndrome.

Computed tomography of hilar cholangiocarcinoma: a new sign

International Nuclear Information System (INIS)

Carr, D.H.; Hadjis, N.S.; Banks, L.M.; Hemingway, A.P.; Blumgart, L.H.

1985-01-01

Thirty-seven patients with histologic proof of cholangiocarcinoma at the confluence were examined by computed tomography (CT) to determine whether this examination is of value in the assessment of these patients for surgery and whether there are any features specific to this type of tumor. Thirty-two patients showed intrahepatic duct dilatation; six of these showed dilatation of ducts in one lobe only. Eighteen patients had intrahepatic low-attenuation areas, while eight had a mass lesion in the porta hepatis. The results of this study show that CT provides useful anatomic information preoperatively but that the appearances are nonspecific. Lobar atrophy is highly suggestive of hilar cholangiocarcinoma, either of long-standing or with unilateral portal venous involvement

SYSTEME MULTISENSEUR DE PERCEPTION 3D POUR LE ROBOT MOBILE HILARE

OpenAIRE

Ferrer , Michel

1982-01-01

L'ETUDE PRESENTEE S'INSERE DANS LE VASTE DOMAINE DE LA VISION ARTIFICIELLE. ELLE CONCERNE PLUS PARTICULIEREMENT L'INTEGRATION DU SYSTEME DE PERCEPTION TROIS DIMENSIONS (3D) DU ROBOT MOBILE AUTONOME HILARE. CE SYSTEME EST COMPOSE D'UNE CAMERA MATRICIELLE A SEMICONDUCTEURS, D'UN TELEMETRE LASER ET D'UNE STRUCTURE MECANIQUE ASSURANT LA DEFLEXION DU FAISCEAU LASER. DANS CE MEMOIRE SONT DECRITS: LA CONCEPTION DE LA STRUCTURE DEFLECTRICE; LE LOGICIEL DE TRAITEMENT DES IMAGES VIDEO MULTINIVEAUX BASE...

Lymph Node Micrometastases are Associated with Worse Survival in Patients with Otherwise Node-Negative Hilar Cholangiocarcinoma

NARCIS (Netherlands)

Mantel, Hendrik T. J.; Wiggers, Jim K.; Verheij, Joanne; Doff, Jan J.; Sieders, Egbert; van Gulik, Thomas M.; Gouw, Annette S. H.; Porte, Robert J.

2015-01-01

Background. Lymph node metastases on routine histology are a strong negative predictor for survival after resection of hilar cholangiocarcinoma. Additional immunohistochemistry can detect lymph node micrometastases in patients who are otherwise node negative, but the prognostic value is unsure. The

Lymph Node Micrometastases are Associated with Worse Survival in Patients with Otherwise Node-Negative Hilar Cholangiocarcinoma

NARCIS (Netherlands)

Mantel, Hendrik T. J.; Wiggers, Jim K.; Verheij, Joanne; Doff, Jan J.; Sieders, Egbert; van Gulik, Thomas M.; Gouw, Annette S. H.; Porte, Robert J.

2015-01-01

Lymph node metastases on routine histology are a strong negative predictor for survival after resection of hilar cholangiocarcinoma. Additional immunohistochemistry can detect lymph node micrometastases in patients who are otherwise node negative, but the prognostic value is unsure. The objective of

Anti-cancer potential of MAPK pathway inhibition in paragangliomas-effect of different statins on mouse pheochromocytoma cells

NARCIS (Netherlands)

Fliedner, S.M.; Engel, T.G.P.; Lendvai, N.K.; Shankavaram, U.; Nolting, S.; Wesley, R.; Elkahloun, A.G.; Ungefroren, H.; Oldoerp, A.; Lampert, G.; Lehnert, H.; Timmers, H.J.; Pacak, K.

2014-01-01

To date, malignant pheochromocytomas and paragangliomas (PHEOs/PGLs) cannot be effectively cured and thus novel treatment strategies are urgently needed. Lovastatin has been shown to effectively induce apoptosis in mouse PHEO cells (MPC) and the more aggressive mouse tumor tissue-derived cells

Quantification of incidental mediastinal and hilar irradiation delivered during definitive stereotactic body radiation therapy for peripheral non-small cell lung cancer

Energy Technology Data Exchange (ETDEWEB)

Martin, Kate L.; Gomez, Jorge; Nazareth, Daryl P.; Warren, Graham W. [Department of Radiation Medicine, Roswell Park Cancer Institute, Buffalo, NY (United States); Singh, Anurag K., E-mail: anurag.singh@roswellpark.org [Department of Radiation Medicine, Roswell Park Cancer Institute, Buffalo, NY (United States)

2012-07-01

To determine the amount of incidental radiation dose received by the mediastinal and hilar nodes for patients with non-small cell lung cancer (NSCLC) treated with stereotactic body radiation therapy (SBRT). Fifty consecutive patients with NSCLC, treated using an SBRT technique, were identified. Of these patients, 38 had a prescription dose of 60 Gy in 20-Gy fractions and were eligible for analysis. For each patient, ipsilateral upper (level 2) and lower (level 4) paratracheal, and hilar (level 10) nodal regions were contoured on the planning computed tomography (CT) images. Using the clinical treatment plan, dose and volume calculations were performed retrospectively for each nodal region. SBRT to upper lobe tumors resulted in an average total ipsilateral mean dose of between 5.2 and 7.8 Gy for the most proximal paratracheal nodal stations (2R and 4R for right upper lobe lesions, 2L and 4L for left upper lobe lesions). SBRT to lower lobe tumors resulted in an average total ipsilateral mean dose of between 15.6 and 21.5 Gy for the most proximal hilar nodal stations (10R for right lower lobe lesions, 10 l for left lower lobe lesions). Doses to more distal nodes were substantially lower than 5 Gy. The often substantial incidental irradiation, delivered during SBRT for peripheral NSCLC of the lower lobes to the most proximal hilar lymph nodes may be therapeutic for low-volume, subclinical nodal disease. Treatment of peripheral upper lobe lung tumors delivers less incidental irradiation to the paratracheal lymph nodes with lower likelihood of therapeutic benefit.

Quantification of incidental mediastinal and hilar irradiation delivered during definitive stereotactic body radiation therapy for peripheral non–small cell lung cancer

International Nuclear Information System (INIS)

Martin, Kate L.; Gomez, Jorge; Nazareth, Daryl P.; Warren, Graham W.; Singh, Anurag K.

2012-01-01

To determine the amount of incidental radiation dose received by the mediastinal and hilar nodes for patients with non–small cell lung cancer (NSCLC) treated with stereotactic body radiation therapy (SBRT). Fifty consecutive patients with NSCLC, treated using an SBRT technique, were identified. Of these patients, 38 had a prescription dose of 60 Gy in 20-Gy fractions and were eligible for analysis. For each patient, ipsilateral upper (level 2) and lower (level 4) paratracheal, and hilar (level 10) nodal regions were contoured on the planning computed tomography (CT) images. Using the clinical treatment plan, dose and volume calculations were performed retrospectively for each nodal region. SBRT to upper lobe tumors resulted in an average total ipsilateral mean dose of between 5.2 and 7.8 Gy for the most proximal paratracheal nodal stations (2R and 4R for right upper lobe lesions, 2L and 4L for left upper lobe lesions). SBRT to lower lobe tumors resulted in an average total ipsilateral mean dose of between 15.6 and 21.5 Gy for the most proximal hilar nodal stations (10R for right lower lobe lesions, 10 l for left lower lobe lesions). Doses to more distal nodes were substantially lower than 5 Gy. The often substantial incidental irradiation, delivered during SBRT for peripheral NSCLC of the lower lobes to the most proximal hilar lymph nodes may be therapeutic for low-volume, subclinical nodal disease. Treatment of peripheral upper lobe lung tumors delivers less incidental irradiation to the paratracheal lymph nodes with lower likelihood of therapeutic benefit.

Nine pheochromocytomas in the same patient. Final mapping with ultrasound and angiography

DEFF Research Database (Denmark)

Nolsøe, C P; Jensen, L T; Torp-Pedersen, S

1988-01-01

A 37-year-old man presented with hypertension and elevated urine catecholamine. Ultrasound scanning revealed a solid tumour of the right adrenal gland and two solid tumours in the retroperitoneum. The findings were confirmed with computed tomography and abdominal angiography. At surgery only...... the tumour of the right adrenal gland was removed. The histopathologic diagnosis was pheochromocytoma. Postoperatively the symptoms and biochemistry were unchanged and the patient was referred for further treatment. At ultrasonography and abdominal aortography 6 remaining tumours were demonstrated. Surgery...

Liver parenchyma transection-first approach in hemihepatectomy with en bloc caudate lobectomy for hilar cholangiocarcinoma: A safe technique to secure favorable surgical outcomes.

Science.gov (United States)

Kawabata, Yasunari; Hayashi, Hikota; Yano, Seiji; Tajima, Yoshitsugu

2017-06-01

Although hemihepatectomy with total caudate lobectomy (hemiHx-tc) is essential for the surgical treatment of hilar cholangiocarcinoma, the advantage of an anterior approach for hemiHx-tc has not been fully discussed technically; the significance of an anterior approach without liver mobilization for preventing infectious complications also remains unknown. The liver parenchyma transection-first approach (Hp-first) technique is an early transection of the hepatic parenchyma without mobilization of the liver that utilizes a modified liver-hanging maneuver to avoid damaging the future remnant liver. Between May 2010 and August 2016, a total of 40 consecutive patients underwent surgery for hilar cholangiocarcinoma. Of these, 19 patients underwent a conventional hemihepatectomy with total caudate lobectomy (cHx), while 21 patients received a Hp-first. The patients in the Hp-first group had significantly less intraoperative blood loss (P hilar cholangiocarcinoma because it resulted in improved surgical outcomes as compared with the conventional approach. © 2017 Wiley Periodicals, Inc.

A Comparison of Y-Type and T-Type Metallic Bilateral Biliary Stents in Patients with Malignant Hilar Biliary Obstruction

International Nuclear Information System (INIS)

Koh, Esther; Jin, Gong Yong; Hwang, Seung Bae; Choi, Eun Jung; Song, Ji Soo; Han, Young Min; Kwon, Keun Sang

2013-01-01

To compare the Y type (side-by-side) and T type (stent-in-stent) bilateral biliary metal stenting in malignant hilar obstruction in terms of treatment outcomes, including post-stenting serum bilirubin level and stent patency. 41 consecutive patients with advanced hilar malignancies who underwent percutaneous placement of bilateral metallic stents - Y (n = 23) and T types (n = 18) - were retrospectively reviewed. We evaluated stent patency after the procedure by cholangiogram and abdominal CT. Pre- and post-stenting serum bilirubin level (total, direct bilirubin) at 1 week and at 1 month were compared. Student t-test and Kaplan-Meier method were used in the statistical analysis. After comparing the median stent patency according to both types, they did not differ significantly (Y: 38 days, T: 61 days; p 0.141). There was a more decrease in the total and direct bilirubin of the T type compared to the Y type after 1 week (p = 0.013, 0.025). However, no significant difference existed between the decreasing bilirubin rates of both types after 1 month (p = 0.923, 0.742). In patients with malignant hilar obstruction, both Y and T type bilateral metallic biliary stents are effective methods. Stent patency and bilirubin decrease rates were not significantly different.

A Comparison of Y-Type and T-Type Metallic Bilateral Biliary Stents in Patients with Malignant Hilar Biliary Obstruction

Energy Technology Data Exchange (ETDEWEB)

Koh, Esther; Jin, Gong Yong; Hwang, Seung Bae; Choi, Eun Jung; Song, Ji Soo; Han, Young Min; Kwon, Keun Sang [Dept. of Chonbuk National University Hospital and Medical School, Jeonju (Korea, Republic of)

2013-04-15

To compare the Y type (side-by-side) and T type (stent-in-stent) bilateral biliary metal stenting in malignant hilar obstruction in terms of treatment outcomes, including post-stenting serum bilirubin level and stent patency. 41 consecutive patients with advanced hilar malignancies who underwent percutaneous placement of bilateral metallic stents - Y (n = 23) and T types (n = 18) - were retrospectively reviewed. We evaluated stent patency after the procedure by cholangiogram and abdominal CT. Pre- and post-stenting serum bilirubin level (total, direct bilirubin) at 1 week and at 1 month were compared. Student t-test and Kaplan-Meier method were used in the statistical analysis. After comparing the median stent patency according to both types, they did not differ significantly (Y: 38 days, T: 61 days; p 0.141). There was a more decrease in the total and direct bilirubin of the T type compared to the Y type after 1 week (p = 0.013, 0.025). However, no significant difference existed between the decreasing bilirubin rates of both types after 1 month (p = 0.923, 0.742). In patients with malignant hilar obstruction, both Y and T type bilateral metallic biliary stents are effective methods. Stent patency and bilirubin decrease rates were not significantly different.

The role of Endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA) for qualitative diagnosis of mediastinal and hilar lymphadenopathy: a prospective analysis

International Nuclear Information System (INIS)

Ye, Ting; Hu, Hong; Luo, Xiaoyang; Chen, Haiquan

2011-01-01

Recently EBUS-TBNA, which has a sensitivity of 94.6%, specificity of 100% and diagnostic accuracy rate of 96.3% as previously reported, has been widely used for patients with mediastinal and hilar lymphadenopathy or suspected lung cancer to get accurate diagnosis. The purpose of the current study was to evaluate the usefulness of EBUS-TBNA in obtaining cytological and histological diagnosis of mediastinal and hilar lymph nodes compared to the results obtained with conventional mediastinoscopy as previously reported, and to assess the relationship of diagnostic accuracy and number of passes and size of lymph nodes. 101 patients with mediastinal and hilar lymphadenopathy or suspected lung cancer in our institution were included in this prospective study. EBUS-TBNA was performed in all cases. The final diagnosis was confirmed by cytology, surgical results, and/or clinical follow-up for at least 6 months. Sensitivity, specificity, accuracy, and positive and negative predictive values were calculated using standard formulas. In 101 patients, EBUS-TBNA was successfully performed to obtain samples from 225 lymph nodes, 7 lung masses, 1 mediastinal mass and 2 esophageal masses. 63 malignant tumors and 38 benign diseases were confirmed. Epidermal growth factor receptor mutation was detected in 10 biopsy samples, and epidermal growth factor receptor mutation was detected in 4 cases. With respect to the correct diagnosis of mediastinal and hilar lymphadenopathy, EBUS-TBNA had a sensitivity of 95.08%, specificity of 100%, positive predictive value of 100%, negative predictive value of 93.02%, and overall accuracy of 97.02%. The relationship of diagnostic accuracy and number of lymph node passes or size of lymph nodes was both insignificant (p = 0.27; p = 0.23). The procedure was uneventful without complications. EBUS-TBNA is an accurate and safe tool in diagnosis of mediastinal and hilar lymphadenopathy. It cannot completely replace mediastinoscopy, it may indeed reduce the

MANAGEMENT OF ENDOCRINE DISEASE: Recurrence or new tumors after complete resection of pheochromocytomas and paragangliomas: a systematic review and meta-analysis.

Science.gov (United States)

Amar, Laurence; Lussey-Lepoutre, Charlotte; Lenders, Jacques W M; Djadi-Prat, Juliette; Plouin, Pierre-Francois; Steichen, Olivier

2016-10-01

To systematically review the incidence and factors associated with recurrences or new tumors after apparent complete resection of pheochromocytoma or thoraco-abdomino-pelvic paraganglioma. A systematic review and meta-analysis of published literature was performed. Pubmed and Embase from 1980 to 2012 were searched for studies published in English on patients with non-metastatic pheochromocytoma or thoraco-abdomino-pelvic paraganglioma, complete tumor resection, postoperative follow-up exceeding 1 month, and recurrence or new tumor documented by pathology, hormonal dosages, or imaging tests. Incidence rates of new events after curative surgery were calculated for each study that had sufficient information and pooled using random-effect meta-analysis. In total, 38 studies were selected from 3518 references, of which 36 reported retrospective cohorts from the USA, Europe, and Asia. Patient follow-up was neither standardized nor exhaustive in the included studies. A clear description of patient retrieval methods was available for nine studies and the follow-up protocol and patient flow for four studies. Only two studies used multivariable methods to assess potential predictors of postoperative events.The overall rate of recurrent disease from 34 studies was 0.98 events/100 person-years (95% confidence interval 0.71, 1.25). Syndromic diseases and paragangliomas were consistently associated with a higher risk of a new event in individual studies and in meta-regression analysis. The risk of recurrent disease after complete resection of pheochromocytoma may be lower than that previously estimated, corresponding to five events for 100 patients followed up for 5 years after complete resection. Risk stratification is required to tailor the follow-up protocol after complete resection of a pheochromocytoma or paraganglioma. Large multicenter studies are needed to this end. © 2016 European Society of Endocrinology.

Diagnostic accuracy of a volume-rendered computed tomography movie and other computed tomography-based imaging methods in assessment of renal vascular anatomy for laparoscopic donor nephrectomy.

Science.gov (United States)

Yamamoto, Shingo; Tanooka, Masao; Ando, Kumiko; Yamano, Toshiko; Ishikura, Reiichi; Nojima, Michio; Hirota, Shozo; Shima, Hiroki

2009-12-01

To evaluate the diagnostic accuracy of computed tomography (CT)-based imaging methods for assessing renal vascular anatomy, imaging studies, including standard axial CT, three-dimensional volume-rendered CT (3DVR-CT), and a 3DVR-CT movie, were performed on 30 patients who underwent laparoscopic donor nephrectomy (10 right side, 20 left side) for predicting the location of the renal arteries and renal, adrenal, gonadal, and lumbar veins. These findings were compared with videos obtained during the operation. Two of 37 renal arteries observed intraoperatively were missed by standard axial CT and 3DVR-CT, whereas all arteries were identified by the 3DVR-CT movie. Two of 36 renal veins were missed by standard axial CT and 3DVR-CT, whereas 1 was missed by the 3DVR-CT movie. In 20 left renal hilar anatomical structures, 20 adrenal, 20 gonadal, and 22 lumbar veins were observed during the operation. Preoperatively, the standard axial CT, 3DVR-CT, and 3DVR-CT movie detected 11, 19, and 20 adrenal veins; 13, 14, and 19 gonadal veins; and 6, 11, and 15 lumbar veins, respectively. Overall, of 135 renal vascular structures, the standard axial CT, 3DVR-CT, and 3DVR-CT movie accurately detected 99 (73.3%), 113 (83.7%), and 126 (93.3%) vessels, respectively, which indicated that the 3DVR-CT movie demonstrated a significantly higher detection rate than other CT-based imaging methods (P renal vascular anatomy before laparoscopic donor nephrectomy.

Neoadjuvant Down-Sizing of Hilar Cholangiocarcinoma with Photodynamic Therapy—Long-Term Outcome of a Phase II Pilot Study

Directory of Open Access Journals (Sweden)

Andrej Wagner

2015-11-01

Full Text Available Hilar cholangiocarcinoma (CC is non-resectable in the majority of patients often due to intrahepatic extension along bile duct branches/segments, and even after complete resection (R0 recurrence can be as high as 70%. Photodynamic therapy (PDT is an established palliative local tumor ablative treatment for non-resectable hilar CC. We report the long-term outcome of curative resection (R0 performed after neoadjuvant PDT for downsizing of tumor margins in seven patients (median age 59 years with initially non-resectable hilar CC. Photofrin® was injected intravenously 24–48 h before laser light irradiation of the tumor stenoses and the adjacent bile duct segments. Major resective surgery was done with curative intention six weeks after PDT. All seven patients had been curatively (R0 resected and there were no undue early or late complications for the neoadjuvant PDT and surgery. Six of seven patients died from tumor recurrence at a median of 3.2 years after resection, the five-year survival rate was 43%. These results are comparable with published data for patients resected R0 without pre-treatment, indicating that neoadjuvant PDT is feasible and could improve overall survival of patients considered non-curatively resectable because of initial tumor extension in bile duct branches/segments—however, this concept needs to be validated in a larger trial.

Detection of benign hilar bile duct stenoses - A retrospective analysis in 250 patients with suspicion of Klatskin tumour.

Science.gov (United States)

Scheuermann, Uwe; Widyaningsih, Rizky; Hoppe-Lotichius, Maria; Heise, Michael; Otto, Gerd

2016-06-01

The aim of this study was to identify clinical, laboratory and radiological parameters to distinguish benign from malignant stenoses of the proximal bile duct. Between 1997 and 2011, 250 patients were referred to our clinic with hilar bile duct stenoses suspicious for Klatskin tumour. Medical histories, clinical data, pre-interventional laboratory tests, imaging findings, as well as therapeutic approach and patient outcome were compared to final histological results. All data were retrieved from our prospectively maintained database and analysed retrospectively. We found benign bile duct lesions in 34 patients (13.6%). Among the entire study population, uni- and multivariate analyses of 18 clinicopathological parameters revealed that patient age, serum alkaline phosphatase, tumour marker CA19-9 and presence of tumour mass in computed tomography were independent predictors for malignant biliary stenoses (p hilar biliary stenoses of 74.6%, 80.0% and 83.5%, respectively. Surgical resection could be avoided by preoperative work-up and surgical exploration in 10 out of 34 patients with benign lesions. Rates of major liver resections performed were 66.7% in the benign lesion group and 90.7% in the Klatskin tumour group. Despite improvements of preoperative diagnostics, it remains difficult to differentiate between benign and malignant hilar bile duct stenosis. Even explorative laparotomy was not able to safely exclude Klatskin tumour in all cases and therefore major liver resection was inevitable.

Threaded biliary inside stents are a safe and effective therapeutic option in cases of malignant hilar obstruction.

Science.gov (United States)

Inatomi, Osamu; Bamba, Shigeki; Shioya, Makoto; Mochizuki, Yosuke; Ban, Hiromitsu; Tsujikawa, Tomoyuki; Saito, Yasuharu; Andoh, Akira; Fujiyama, Yoshihide

2013-02-14

Although endoscopic biliary stents have been accepted as part of palliative therapy for cases of malignant hilar obstruction, the optimal endoscopic management regime remains controversial. In this study, we evaluated the safety and efficacy of placing a threaded stent above the sphincter of Oddi (threaded inside plastic stents, threaded PS) and compared the results with those of other stent types. Patients with malignant hilar obstruction, including those requiring biliary drainage for stent occlusion, were selected. Patients received either one of the following endoscopic indwelling stents: threaded PS, conventional plastic stents (conventional PS), or metallic stents (MS). Duration of stent patency and the incident of complication were compared in these patients. Forty-two patients underwent placement of endoscopic indwelling stents (threaded PS = 12, conventional PS = 17, MS = 13). The median duration of threaded PS patency was significantly longer than that of conventional PS patency (142 vs. 32 days; P = 0.04, logrank test). The median duration of threaded PS and MS patency was not significantly different (142 vs. 150 days, P = 0.83). Stent migration did not occur in any group. Among patients who underwent threaded PS placement as a salvage therapy after MS obstruction due to tumor ingrowth, the median duration of MS patency was significantly shorter than that of threaded PS patency (123 vs. 240 days). Threaded PS are safe and effective in cases of malignant hilar obstruction; moreover, it is a suitable therapeutic option not only for initial drainage but also for salvage therapy.

The clinical application of ultrasonography-guided percutaneous transhepatic injection of iodized oil containing chemotherapeutic agent for the treatment of hilar lymphatic metastasis

International Nuclear Information System (INIS)

Zhao Guangsheng; Zhang Yuewei; Yang Xiaohong; Li Chuang; Zhao Mu; Wang Wenqing; Wang Ruoyu

2010-01-01

Objective: To discuss the technique and the clinical effect of ultrasonography-guided percutaneous transhepatic injection of iodized oil containing chemotherapeutic agent for the treatment of hepatic hilar lymphatic metastasis. Methods: Under ultrasonographic guidance,percutaneous transhepatic injection of iodized oil containing chemotherapeutic agent, so-called chemo-ablation, into the diseased lymph nodes was performed in thirteen patients with hepatic hilar lymphatic metastasis. The therapeutic results were evaluated based on the post-operative imaging examinations as well as the alleviation of the clinical symptoms. Results: Percutaneous transhepatic injection of iodized oil containing chemotherapeutic agent into the diseased lymph nodes was successfully carried out in all thirteen patients. After the procedure,the patients were followed up for a mean period of 13.5 months. The therapeutic effectiveness was 100%, while the regression rate of the lesions was 76.9%. No operation-related complications occurred. Conclusion: Percutaneous transhepatic injection of iodized oil containing chemotherapeutic agent into the diseased lymph nodes under ultrasonographic guidance is an effective and safe treatment for hepatic hilar lymphatic metastasis with reliable effectiveness. (authors)

Endoscopic stenting for hilar cholangiocarcinoma: efficacy of unilateral and bilateral placement of plastic and metal stents in a retrospective review of 480 patients

Directory of Open Access Journals (Sweden)

Liberato Manuel José

2012-08-01

Full Text Available Abstract Background Endoscopic biliary drainage of hilar cholangiocarcinoma is controversial with respect to the optimal types of stents and the extent of drainage. This study evaluated endoscopic palliation in patients with hilar cholangiocarcinoma using self-expandable metallic stents (SEMS and plastic stents (PS.We also compared unilateral and bilateral stent placement according to the Bismuth classification. Methods Data on 480 patients receiving endoscopic biliary drainage for hilar cholangiocarcinoma between September 1995 and December 2010 were retrospectively reviewed to evaluate the following outcome parameters: technical success (TS, functional success (FS, early and late complications, stent patency and survival. Patients were followed from stent insertion until death or stent occlusion. Patients were divided into 3 groups according to the Bismuth classification (Group 1, type I; Group 2, type II; Group 3, type > III. Results The initial stent insertion was successful in 450 (93.8% patients. TS was achieved in 204 (88.3% patients treated with PS and in 246 (98.8% patients palliated with SEMS (p P P  Conclusions SEMS insertion for the palliation of hilar cholangiocarcinoma offers higher technical and clinical success rates in the ITT analysis as well as lower complication rates and a superior cumulative stent patency when compared with PS placement in all Bismuth classifications. The cumulative patency of bilateral SEMS or PS stents was significantly higher than that of unilateral SEMS or PS stents, with lower occlusion rates in Bismuth II patients.

Impact of F DOPA-PET on therapeutic decision in endocrine tumours: digestive tumours, medullary thyroid cancer or pheochromocytoma

International Nuclear Information System (INIS)

Montravers, F.; Grahek, D.; Kerrou, K.; Gutman, F.; Beco, V. de; Nataf, V.; Balard, M.; Talbot, J.N.

2006-01-01

FDOPA-PET has been proposed for a decade in oncology, in particular in endocrine tumours. To the best of our knowledge, only one impact rate has been reported: 31% in 17 patients with digestive carcinoid tumours. We did a questionnaire survey to evaluate this impact reported by the referring clinician in 87 patients who had FDOPA PET due to digestive carcinoid tumour or another type of digestive endocrine tumour or a medullary thyroid cancer or a pheochromocytoma. The response rate to the survey was 87%. The overall impact of FDOPA PET on patient's management was 36%. Its value was greater for digestive carcinoid tumour and for medullary thyroid cancer; the number of patients with pheochromocytoma is still limited. In the other digestive endocrine tumours, a change in patient management was less frequent and FDOPA PET should be performed when the other examinations are inconclusive. (author)

Assessment of Chronological Effects of Irreversible Electroporation on Hilar Bile Ducts in a Porcine Model

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Choi, Jae Woong, E-mail: cooljay@korea.ac.kr; Lu, David S. K., E-mail: dlu@mednet.ucla.edu; Osuagwu, Ferdnand, E-mail: fosuagwu@mednet.ucla.edu; Raman, Steven, E-mail: sraman@mednet.ucla.edu [David Geffen School of Medicine at UCLA, Department of Radiology (United States); Lassman, Charles, E-mail: classman@mednet.ucla.edu [David Geffen School of Medicine at UCLA, Department of Pathology (United States)

2013-11-07

PurposeTo evaluate the chronological effects of irreversible electroporation (IRE) on large hilar bile ducts in an in vivo porcine model correlated with computed tomography (CT) cholangiography and histopathology.Materials and MethodsTwelve IRE zones were made along hilar bile ducts intraoperatively under ultrasound (US)-guidance in 11 pigs. Paired electrodes were placed either on opposing sides of the bile duct (straddle [STR]) or both on one side of the bile duct (one-sided [OSD]). The shortest electrode-to-duct distance was classified as periductal (≤2 mm) or nonperiductal (>2 mm). CT cholangiography and laboratory tests were performed before IRE and again at 2 days, 4 weeks, and 8 weeks after IRE. Degree of bile duct injury were graded as follows: grade 0 = no narrowing; grade 1 = ≤50 % duct narrowing; grade 2 = >50 % narrowing without proximal duct dilatation; grade 3 = grade 2 with proximal duct dilatation; and grade 4 = grade 3 with enzyme elevation. Pigs were selected for killing and histopathology at 2 days, 4, and 8 weeks.ResultsNonperiductal electrode placement produced no long-term strictures in 5 of 5 ducts. Periductal electrode placement produced mild narrowing in 6 of 7 ducts: 5 grade 1 and 1 grade 2. None showed increased enzymes. There was no significant difference between STR versus OSD electrode placement. Histopathology showed minor but relatively greater ductal mural changes in narrowed ducts.ConclusionIn the larger hilar ducts, long-term patency and mural integrity appear resistant to IRE damage with the energy deposition used, especially if the electrode is not immediately periductal in position.

Hepatic Artery Resection for Bismuth Type III and IV Hilar Cholangiocarcinoma: Is Reconstruction Always Required?

Science.gov (United States)

Hu, Hai-Jie; Jin, Yan-Wen; Zhou, Rong-Xing; Shrestha, Anuj; Ma, Wen-Jie; Yang, Qin; Wang, Jun-Ke; Liu, Fei; Cheng, Nan-Sheng; Li, Fu-Yu

2018-03-06

The objective of the study is to examine the feasibility of hepatic artery resection (HAR) without subsequent reconstruction (RCS) in specified patients of Bismuth type III and IV hilar cholangiocarcinoma. We retrospectively reviewed 63 patients who underwent hepatic artery resection for Bismuth type III and IV hilar cholangiocarcinoma. These patients were subsequently enrolled into two groups based on whether the artery reconstruction was conducted. Postoperative morbidity and mortality, and long-term survival outcome were compared between the two groups. There were 29 patients in HAR group and 34 patients in the HAR + RCS group. Patients with hepatic artery reconstruction tended to have longer operative time (545.6 ± 143.1 min vs. 656.3 ± 192.8 min; P = 0.013) and smaller tumor size (3.0 ± 1.1 cm vs. 2.5 ± 0.9 cm; P = 0.036). The R0 resection margin was comparable between the HAR group and HAR + RCS group (86.2 vs. 85.3%; P > 0.05). Twelve patients (41.4%) with 24 complications in HAR group and 13 patients (38.2%) with 25 complications in HAR + RCS group were recorded (P = 0.799). The postoperative hepatic failure rate (13.8 vs. 5.9%) and postoperative mortality rate (3.4% vs. 2.9%) were also comparable between the two groups. In the HAR group, the overall 1-, 3-, and 5-year survival rates were 72, 41, and 19%, respectively; while in the HAR + RCS group, the overall 1-, 3-, and 5-year survival rates were 79, 45, and 25%, respectively (P = 0.928). Hepatic artery resection without reconstruction is also a safe and feasible surgical procedure for highly selected cases of Bismuth type III and IV hilar cholangiocarcinoma.

Assessment of Chronological Effects of Irreversible Electroporation on Hilar Bile Ducts in a Porcine Model

International Nuclear Information System (INIS)

Choi, Jae Woong; Lu, David S. K.; Osuagwu, Ferdnand; Raman, Steven; Lassman, Charles

2014-01-01

PurposeTo evaluate the chronological effects of irreversible electroporation (IRE) on large hilar bile ducts in an in vivo porcine model correlated with computed tomography (CT) cholangiography and histopathology.Materials and MethodsTwelve IRE zones were made along hilar bile ducts intraoperatively under ultrasound (US)-guidance in 11 pigs. Paired electrodes were placed either on opposing sides of the bile duct (straddle [STR]) or both on one side of the bile duct (one-sided [OSD]). The shortest electrode-to-duct distance was classified as periductal (≤2 mm) or nonperiductal (>2 mm). CT cholangiography and laboratory tests were performed before IRE and again at 2 days, 4 weeks, and 8 weeks after IRE. Degree of bile duct injury were graded as follows: grade 0 = no narrowing; grade 1 = ≤50 % duct narrowing; grade 2 = >50 % narrowing without proximal duct dilatation; grade 3 = grade 2 with proximal duct dilatation; and grade 4 = grade 3 with enzyme elevation. Pigs were selected for killing and histopathology at 2 days, 4, and 8 weeks.ResultsNonperiductal electrode placement produced no long-term strictures in 5 of 5 ducts. Periductal electrode placement produced mild narrowing in 6 of 7 ducts: 5 grade 1 and 1 grade 2. None showed increased enzymes. There was no significant difference between STR versus OSD electrode placement. Histopathology showed minor but relatively greater ductal mural changes in narrowed ducts.ConclusionIn the larger hilar ducts, long-term patency and mural integrity appear resistant to IRE damage with the energy deposition used, especially if the electrode is not immediately periductal in position

Effectiveness of the Benign and Malignant Diagnosis of Mediastinal and Hilar Lymph Nodes by Endobronchial Ultrasound Elastography.

Science.gov (United States)

Huang, Haidong; Huang, Zhiang; Wang, Qin; Wang, Xinan; Dong, Yuchao; Zhang, Wei; Zarogoulidis, Paul; Man, Yan-Gao; Schmidt, Wolfgang Hohenforst; Bai, Chong

2017-01-01

Background and Objectives: Endobronchial ultrasound elastography is a new technique for describing the stiffness of tissue during endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA). The aims of this study were to investigate the diagnostic value of Endobronchial ultrasound (EBUS) elastography for distinguishing the difference between benign and malignant lymph nodes among mediastinal and hilar lymph node. Materials and Methods: From June 2015 to August 2015, 47 patients confirmed of mediastinal and hilar lymph node enlargement through examination of Computed tomography (CT) were enrolled, and a total of 78 lymph nodes were evaluated by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA). EBUS-guided elastography of lymph nodes was performed prior to EBUS-TBNA. A convex probe EBUS was used with a new EBUS processor to assess elastographic patterns that were classified based on color distribution as follows: Type 1, predominantly non-blue (green, yellow and red); Type 2, part blue, part non-blue (green, yellow and red); Type 3, predominantly blue. Pathological determination of malignant or benign lymph nodes was used as the gold standard for this study. The elastographic patterns were compared with the final pathologic results from EBUS-TBNA. Results: On pathological evaluation of the lymph nodes, 45 were benign and 33 were malignant. The lymph nodes that were classified as Type 1 on endobronchial ultrasound elastography were benign in 26/27 (96.3%) and malignant in 1/27 (3.7%); for Type 2 lymph nodes, 15/20 (75.0%) were benign and 5/20 (25.0%) were malignant; Type 3 lymph nodes were benign in 4/31 (12.9%) and malignant in 27/31 (87.1%). In classifying Type 1 as 'benign' and Type 3 as 'malignant,' the sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy rates were 96.43%, 86.67%, 87.10%, 96.30%, 91.38%, respectively. Conclusion: EBUS elastography of mediastinal and

Resection of hilar cholangiocarcinoma with left hepatectomy after pre-operative embolization of the proper hepatic artery

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Yasuda, Yoshikazu; Larsen, Peter N; Ishibashi, Toshimitsu

2010-01-01

Right or right-extended hepatectomy including the caudate lobe is the most common treatment for hilar cholangiocarcinoma (HC). A 5-year survival of up to 60% can be achieved using this procedure if R0-resection is obtained. However, for some patients a left-sided liver resection is necessary...

Influence of hilar deposition in the evaluation of the alveolar epithelial permeability on 99mTc-DTPA aerosol inhaled scintigraphy

International Nuclear Information System (INIS)

Ogi, Shigeyuki; Uchiyama, Mayuki; Fukuda, Kunihiko; Urashima, Mitsuyoshi; Gotoh, Eisuke; Fukumitsu, Nobuyoshi

2009-01-01

We investigated whether hilar radioaerosol deposition affects the clearance rate of technetium-99m-labeled diethylenetriaminepentaacetic acid ( 99m Tc-DTPA) from peripheral alveolar regions. A total of 38 patients underwent 99m Tc-DTPA inhalation lung scintigraphy. Six region of interest (ROI) patterns were adopted: ROI 1 was outlined around the entire hemithorax, and ROIs 2-6 were outlined around the hemithorax but excluded square ROIs of different size in the hilar region. Half-times (T 1/2 ) were calculated with time-activity curves using one-compartment and two-compartment analyses. The T 1/2 of ROIs 1-5 were plotted against the T 1/2 of ROI 6, and regression lines were obtained with the least-squares method. The absolute values of the differences between surveyed values and regression line were calculated. The Wilcoxon test for trend and a single linear regression model were used to determine statistical significance. There were significant reductions in the absolute values of the differences between surveyed values and regression line from ROIs 1-5 by one-component analysis and the fast component of two-compartment analysis (P 99m Tc-DTPA from the alveoli in damaged lungs. The hilar region should be excluded from ROIs when alveolar epithelial permeability is evaluated. (author)

Synchronous triple urogenital cancer (renal cancer, bladder cancer, prostatic cancer). A case report

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Takada, Tsuyoshi; Honda, Masahito; Momohara, Chikahiro; Komori, Kazuhiko; Fujioka, Hideki [Osaka Police Hospital (Japan)

2002-04-01

A case of synchronous triple urogenital cancer, which was comprised of renal cell carcinoma of the left kidney, transitional cell carcinoma of the urinary bladder, and adenocarcinoma of the prostate, is reported. A 72-year-old Japanese male patient was referred to our outpatient clinic with the complaint of asymptomatic hematuria. At that time, his serum of level of PSA was elevated to 20 ng/ml. Cystourethroscopy showed a papillary bladder tumor and coagula through the left urinary orifice. Ultrasonography, computed tomography and magnetic resonance imaging showed a mass lesion measuring about 6 cm by 5 cm in the left kidney. Angiography showed a hypervascular lesion measuring about 6 cm by 5 cm at the same site. Double cancer, consisting of renal cell carcinoma and transitional cell carcinoma of the urinary bladder, was suspected and we performed left total nephroureterectomy, hilar lymphadenectomy, and transurethral rection of the bladder tumor, one month later. At the same time, we performed a biopsy of the prostate. Histological diagnosis was renal cell carcinoma, clear cell carcinoma and transitional cell carcinoma of urinary bladder. Histological diagnosis of the prostate biopsy was moderately differentiated adenocarcinoma. Since this case fulfilled the criteria of Warren and Gates, it was classified as synchronous triple urogenital cancer. A review of the literature revealed 17 authentic cases of triple urogenital cancer, of which 14 and 10 cases were reported as a combination of renal cancer, bladder cancer and prostatic cancer, in the world and in Japan, respectively. Furthermore, he had been exposed to the atomic bomb explosion in Hiroshima in 1945. This carcinogenic precursor may be related to the development of the triple cancer. (author)

Synchronous triple urogenital cancer (renal cancer, bladder cancer, prostatic cancer). A case report

International Nuclear Information System (INIS)

Takada, Tsuyoshi; Honda, Masahito; Momohara, Chikahiro; Komori, Kazuhiko; Fujioka, Hideki

2002-01-01

A case of synchronous triple urogenital cancer, which was comprised of renal cell carcinoma of the left kidney, transitional cell carcinoma of the urinary bladder, and adenocarcinoma of the prostate, is reported. A 72-year-old Japanese male patient was referred to our outpatient clinic with the complaint of asymptomatic hematuria. At that time, his serum of level of PSA was elevated to 20 ng/ml. Cystourethroscopy showed a papillary bladder tumor and coagula through the left urinary orifice. Ultrasonography, computed tomography and magnetic resonance imaging showed a mass lesion measuring about 6 cm by 5 cm in the left kidney. Angiography showed a hypervascular lesion measuring about 6 cm by 5 cm at the same site. Double cancer, consisting of renal cell carcinoma and transitional cell carcinoma of the urinary bladder, was suspected and we performed left total nephroureterectomy, hilar lymphadenectomy, and transurethral rection of the bladder tumor, one month later. At the same time, we performed a biopsy of the prostate. Histological diagnosis was renal cell carcinoma, clear cell carcinoma and transitional cell carcinoma of urinary bladder. Histological diagnosis of the prostate biopsy was moderately differentiated adenocarcinoma. Since this case fulfilled the criteria of Warren and Gates, it was classified as synchronous triple urogenital cancer. A review of the literature revealed 17 authentic cases of triple urogenital cancer, of which 14 and 10 cases were reported as a combination of renal cancer, bladder cancer and prostatic cancer, in the world and in Japan, respectively. Furthermore, he had been exposed to the atomic bomb explosion in Hiroshima in 1945. This carcinogenic precursor may be related to the development of the triple cancer. (author)

Percutaneous intraductal radiofrequency ablation in the management of unresectable Bismuth types III and IV hilar cholangiocarcinoma.

Science.gov (United States)

Wang, Yu; Cui, Wei; Fan, Wenzhe; Zhang, Yingqiang; Yao, Wang; Huang, Kunbo; Li, Jiaping

2016-08-16

To assess the feasibility and safety of percutaneous intraductal radiofrequency ablation (RFA) for unresectable Bismuth types III and IV hilar cholangiocarcinoma. Percutaneous intraductal RFA combined with metal stent placement was successful in all patients without any technical problems; the technical success rate was 100%. Chemotherapy was administered to two patients. After treatment, serum direct bilirubin levels were notably decreased. Six patients died during the follow-up period. Median stent patency from the time of the first RFA and survival from the time of diagnosis were 100 days (95% confidence interval (CI), 85-115 days) and 5.3 months (95% CI, 2.5-8.1 months), respectively. No acute pancreatitis, bile duct bleeding and perforation, bile leakage, or other severe complications occurred. Four cases of procedure-related cholangitis, three cases of postoperative abdominal pain, and five cases of asymptomatic transient increase in serum amylase were observed. One patient who presented with stent blockage 252 days' post-procedure underwent repeat ablation. Between September 2013 and May 2015, nine patients with unresectable Bismuth types III and IV hilar cholangiocarcinoma who were treated with percutaneous intraductal RFA combined with metal stent placement after the percutaneous transhepatic cholangial drainage were included in the retrospective analysis. Procedure-related complications, stent patency, and survival after treatment were investigated. Percutaneous intraductal RFA combined with metal stent placement is a technically safe and feasible therapeutic option for the palliative treatment of unresectable Bismuth types III and IV hilar cholangiocarcinoma. Its long-term efficacy and safety is promising, but needs further study via randomized and prospective trials that include a greater number of patients.

Management of full term pregnant patient with paroxysmal hypertension due to incidental pheochromocytoma

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S. Sahu*

2013-12-01

Case Description: A 25-year-old, full-term pregnant woman diagnosed with pre-eclampsia was referred to our tertiary care hospital with severe resistant hypertension. Her blood pressure remained labile despite the usual medications, which led to the suspicion of an underlying endocrinological problem. Further biochemical and radiological investigations confirmed the diagnosis of pheochromocytoma. The patient was invasively monitored and treated with alpha blockade, beta blocker, and vasodilators in ICU. On the fifth day, she went into spontaneous labour with confirmed rupture of the membranes. The labour was augmented with intravenous oxytocin 2U in 500 ml solution of Ringer’s lactate. A nitro-glycerine basal infusion was started and titrated to control BP during labour to keep the blood pressure below 160/90 mmHg. An injection of Phentolamine drip and beta blocker esmolol was kept ready, to control the wide fluctuation of blood pressure. She delivered a live, healthy, male infant weighing 2.5 Kg. She was kept in the ICU for 72 h with epidural patient-controlled analgesia (EPCA. The patient was not keen for a resection of the adrenal tumour immediately after delivery. She was discharged with medical management, with a further plan for surgery in due course. With a multidisciplinary team approach (gynaecologist, anaesthesiologist, endocrinologist, and surgeon, proper planning, and adequate preoperative medical management; pheochromocytoma in pregnancy can be managed successfully.

Inhibition of Cdc42 and Rac1 activities in pheochromocytoma, the adrenal medulla tumor.

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Croisé, Pauline; Brunaud, Laurent; Tóth, Petra; Gasman, Stéphane; Ory, Stéphane

2017-04-03

Altered Rho GTPase signaling has been linked to many types of cancer. As many small G proteins, Rho GTPases cycle between an active and inactive state thanks to specific regulators that catalyze exchange of GDP into GTP (Rho-GEF) or hydrolysis of GTP into GDP (Rho-GAP). Recent studies have shown that alteration takes place either at the level of Rho proteins themselves (expression levels, point mutations) or at the level of their regulators, mostly RhoGEFs and RhoGAPs. Most reports describe Rho GTPases gain of function that may participate to the tumorigenesis processes. In contrast, we have recently reported that decreased activities of Cdc42 and Rac1 as well as decreased expression of 2 Rho-GEFs, FARP1 and ARHGEF1, correlate with pheochromocytomas, a tumor developing in the medulla of the adrenal gland (Croisé et al., Endocrine Related Cancer, 2016). Here we highlight the major evidence and further study the correlation between Rho GTPases activities and expression levels of ARHGEF1 and FARP1. Finally we also discuss how the decrease of Cdc42 and Rac1 activities may help human pheochromocytomas to develop and comment the possible relationship between FARP1, ARHGEF1 and the 2 Rho GTPases Cdc42 and Rac1 in tumorigenesis.

Air cholangiography in endoscopic bilateral stent-in-stent placement of metallic stents for malignant hilar biliary obstruction.

Science.gov (United States)

Lee, Jae Min; Lee, Sang Hyub; Jang, Dong Kee; Chung, Kwang Hyun; Park, Jin Myung; Paik, Woo Hyun; Lee, Jun Kyu; Ryu, Ji Kon; Kim, Yong-Tae

2016-03-01

Although endoscopic bilateral stent-in-stent (SIS) placement of self-expandable metallic stents (SEMS) is one of the major palliative treatments for unresectable malignant hilar biliary obstruction, post-endoscopic retrograde cholangiopancreatography (ERCP) cholangitis can occur frequently due to inadequate drainage, especially after contrast injection into the biliary tree. The aim of this study is to evaluate the efficacy and safety of air cholangiography-assisted stenting. This study included 47 patients with malignant hilar biliary obstruction who underwent endoscopic bilateral SEMS placement using the SIS technique. They were divided into two groups, air (n = 23) or iodine contrast (n = 24) cholangiography. We retrospectively compared comprehensive clinical and laboratory data of both groups. There were no significant differences found between the two groups with respect to technical success (87% versus 87.5%, air versus contrast group, respectively), functional success (95% versus 95.2%), 30-day mortality (8.3% versus 8.7%) and stent patency. Post-ERCP adverse events occurred in 5 (21.7%) of the patients in the air group and 8 (33.3%) of the patients in the contrast group. Among these, the rate of cholangitis was significantly lower in the air group (4.8% versus 29.2%, p = 0.048). In multivariate analysis, air cholangiography, technical success and a shorter procedure time were significantly associated with a lower incidence of post-ERCP cholangitis. Air cholangiography-assisted stenting can be a safe and effective method for endoscopic bilateral SIS placement of SEMS in patients with malignant hilar biliary obstruction.

Anthropometric Renal Anatomic Alterations Between Supine and Prone Positions in Percutaneous Renal Ablation for Renal Cortical Neoplasms.

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Lusch, Achim; Fujimoto, Scott; Findeiss, Laura K; Okhunov, Zhamshid; McDougall, Elspeth M; Landman, Jaime

2016-02-01

To establish patterns of anatomic changes relevant to the kidney and colon during positional change between the supine and prone positions as noted on CT scans performed during percutaneous cryoablation for renal cortical neoplasms (RCN). Nineteen patients undergoing percutaneous cryoablation for RCN with abdominal CT scan in both the supine and prone positions were included in the study. We documented the anterior/posterior, medial/lateral, and cranial/caudal anatomic changes of the kidney, kidney rotation, and the proportion of the kidney whose access was limited by the liver, spleen, and lung. We also calculated the length of the percutaneous access tract and the distance between the colon and kidney in hilar position as well as the anterior/posterior location of the colon relative to the kidney. In the prone position, the kidney lies significantly more anteriorly on both sides: 4.7 cm vs 4.3 cm (L) and 4.4 cm vs 4.1 cm (R) (p = 0.02 and p = 0.03, respectively). On prone CT images, both kidneys are more cranial when compared with the supine position: 80.4 mm vs 60.8 mm (L) and 87.2 mm vs 57.4 mm (R) (p = 0.002 and p anatomic alterations between supine and prone CT imaging. The changes associated with the prone position modify percutaneous access, particularly for right upper pole tumors. Prone imaging before surgery may be helpful in selected cases.

Malign pheochromocytoma: importance of the scintigraphic follow-up with metaiodobenzulguanidine 131I (MIBG-131I)

International Nuclear Information System (INIS)

Kato, M.; Velhote, V.V.; Souto, F.J.P.; Long, Y.J.; Costa, P.L.A.

1989-01-01

The authors report a case of pheochromocytoma investigated with metaiodobenzylguanidine labeled with 131 I (MIBG- 131 I). The methodology identify primitive lesion, its recurrence and metastasis. The authors mention the advantage of the technique due to the high specificity, sensitivity and because it is harmless, offering optimal information about the morphology and functional nature, concerning diagnosis and follow-up of the disease. (author) [pt

Endoscopic stenting for hilar cholangiocarcinoma: efficacy of unilateral and bilateral placement of plastic and metal stents in a retrospective review of 480 patients

Science.gov (United States)

2012-01-01

Background Endoscopic biliary drainage of hilar cholangiocarcinoma is controversial with respect to the optimal types of stents and the extent of drainage. This study evaluated endoscopic palliation in patients with hilar cholangiocarcinoma using self-expandable metallic stents (SEMS) and plastic stents (PS).We also compared unilateral and bilateral stent placement according to the Bismuth classification. Methods Data on 480 patients receiving endoscopic biliary drainage for hilar cholangiocarcinoma between September 1995 and December 2010 were retrospectively reviewed to evaluate the following outcome parameters: technical success (TS), functional success (FS), early and late complications, stent patency and survival. Patients were followed from stent insertion until death or stent occlusion. Patients were divided into 3 groups according to the Bismuth classification (Group 1, type I; Group 2, type II; Group 3, type > III). Results The initial stent insertion was successful in 450 (93.8%) patients. TS was achieved in 204 (88.3%) patients treated with PS and in 246 (98.8%) patients palliated with SEMS (p stent patency in weeks (w) were as follows: 20 w in patients palliated with PS and 27 w in patients treated with SEMS (p stent patency. Conclusions SEMS insertion for the palliation of hilar cholangiocarcinoma offers higher technical and clinical success rates in the ITT analysis as well as lower complication rates and a superior cumulative stent patency when compared with PS placement in all Bismuth classifications. The cumulative patency of bilateral SEMS or PS stents was significantly higher than that of unilateral SEMS or PS stents, with lower occlusion rates in Bismuth II patients. PMID:22873816

Pheochromocytoma-paraganglioma: Biochemical and genetic diagnosis.

Science.gov (United States)

Cano Megías, Marta; Rodriguez Puyol, Diego; Fernández Rodríguez, Loreto; Sención Martinez, Gloria Lisette; Martínez Miguel, Patricia

Pheochromocytomas and paragangliomas are tumours derived from neural crest cells, which can be diagnosed by biochemical measurement of metanephrine and methoxytyramine. Advances in genetic research have identified many genes involved in the pathogenesis of these tumours, suggesting that up to 35-45% may have an underlying germline mutation. These genes have a singular transcriptional signature and can be grouped into 2 clusters (or groups): cluster 1 (VHL and SHDx), involved in angiogenesis and hypoxia pathways; and cluster 2 (MEN2 and NF1), linked to the kinase signalling pathway. In turn, these genes are associated with a characteristic biochemical phenotype (noradrenergic and adrenergic), and clinical features (location, biological behaviour, age of presentation, etc.) in a large number of cases. Early diagnosis of these tumours, accompanied by a correct genetic diagnosis, should eventually become a priority to enable better treatment, early detection of complications, proper screening of family members and related tumours, as well as an improvement in the overall prognosis of these patients. Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

Management of pediatric pheochromocytoma. A review of the literature

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Mauriello C

2016-06-01

Full Text Available Introduction Pheochromocytoma is a tumor originated from the chromaffin tissue of the adrenal medulla or from extra-adrenal paraganglionic tissue. Pheochromocytomasare extremely rare in the pediatric population, accounting for 1% of pediatric hypertension. Material and Methods The Authors conduced a systematic review of the pediatric PCC focusing on the indications and surgical technique. Results Surgery remains the mainstay of treatment of pheochromocytomain children. Prior to surgery all children must be prepared with alpha-blockade with adequate fluid and salt replacement in order to reduce surgical complications. Discussion and Conclusions Minimally invasive adrenalectomy is the gold standard for benign lesions of the adrenal gland. The lateral transperitoneal adrenalectomy is the standard approach. Laparoscopic bilateral partial adrenalectomies should be considered in children with bilateral PCC in order to avoid lifelong glucocorticoid and mineralocorticoid replacement.

Extrahepatic bile duct resection in combination with liver resection for hilar cholangiocarcinoma : A report of 42 cases

NARCIS (Netherlands)

IJitsma, AJC; Appeltans, BMG; de Jong, KP; Porte, RJ; Peeters, PMJG; Slooff, MJH

2004-01-01

From September 1986 until December 2001, 42 patients (20 males and 22 females) underwent a combined extrahepatic bile duct resection (EHBDR) and liver resection (LR) for hilar cholangiocarcinoma (HC). The aim of this study was to analyze patient survival, morbidity, and mortality as well as to seek

Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma

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Wong Nora

2006-01-01

Full Text Available Abstract Background Germline mutations of the SDHD, SDHB and SDHC genes, encoding three of the four subunits of succinate dehydrogenase, are a major cause of hereditary paraganglioma and pheochromocytoma, and demonstrate that these genes are classic tumor suppressors. Succinate dehydrogenase is a heterotetrameric protein complex and a component of both the Krebs cycle and the mitochondrial respiratory chain (succinate:ubiquinone oxidoreductase or complex II. Methods Using conformation sensitive gel electrophoresis (CSGE and direct DNA sequencing to analyse genomic DNA from peripheral blood lymphocytes, here we describe the mutation analysis of the SDHB and SDHC genes in 37 patients with sporadic (i.e. no known family history head and neck paraganglioma and five pheochromocytoma and/or paraganglioma families. Results Two sporadic patients were found to have a SDHB splice site mutation in intron 4, c.423+1G>A, which produces a mis-spliced transcript with a 54 nucleotide deletion, resulting in an 18 amino acid in-frame deletion. A third patient was found to carry the c.214C>T (p.Arg72Cys missense mutation in exon 4 of SDHC, which is situated in a highly conserved protein motif that constitutes the quinone-binding site of the succinate: ubiquinone oxidoreductase (SQR complex in E. coli. Together with our previous results, we found 27 germline mutations of SDH genes in 95 cases (28% of sporadic head and neck paraganglioma. In addition all index patients of five families showing hereditary pheochromocytoma-paraganglioma were found to carry germline mutations of SDHB: four of which were novel, c.343C>T (p.Arg115X, c.141G>A (p.Trp47X, c.281G>A (p.Arg94Lys, and c.653G>C (p.Trp218Ser, and one reported previously, c.136C>T, p.Arg46X. Conclusion In conclusion, these data indicate that germline mutations of SDHB and SDHC play a minor role in sporadic head and neck paraganglioma and further underline the importance of germline SDHB mutations in cases of

[Pheochromocytoma and paraganglioma in childhood: a report of 2 cases report].

Science.gov (United States)

Mosquera Gorostidi, A; Justo Ranera, A; Zakirian Denis, S E; González Temprano, N; Sagaseta de Ilúrdoz Uranga, M; Molina Garicano, J

2015-01-01

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors in children and most of them are sporadic. However, they represent the most common endocrine tumor in childhood, and hereditary tumor syndromes are most relevant in these age. Advances in genetic, biochemistry and imaging techniques have revised the management of these tumors; thus A biochemical study should be always initiated once the clinical diagnosis is suspected, followed by imaging and molecular studies, particularly in the context of known familial disease. The diagnostic and therapeutic features are reviewed after the presentation of two clinical cases, where the second one is a patient with type 1 Neurofibromatosis. Copyright © 2014. Published by Elsevier Espana.

Better long-term outcomes with hilar ductoplasty and a side-to-side Roux-en-Y hepaticojejunostomy.

Science.gov (United States)

Xia, Hong-Tian; Liu, Yang; Yang, Tao; Liang, Bin; Wang, Jing; Dong, Jia-Hong

2017-07-01

Whether a wide hilar hepaticojejunostomy after bile duct cyst (BDC) excision can prevent the development of postoperative complications remains an unanswered question. We compared our outcomes after a minimum of 2-y follow-up in patients with Todani type Ia choledochal cyst treated with hilar ductoplasty followed by a side-to-side Roux-en-Y hepaticojejunostomy (ductoplasty group) or radical cyst resection with an end-to-side Roux-en-Y hepaticojejunostomy (conventional group). We retrospectively reviewed the records of patients with Todani type Ia choledochal cyst who received radical cyst excision from January 1997 to December 2012, and we compared the groups' postoperative complications and surgical outcomes. The groups' baseline demographics were similar, except for age. The gender distribution and preoperative presenting symptoms were comparable in the ductoplasty (n = 72) and conventional (n = 53) groups (all P > 0.05). Average age was 37.0 y for the ductoplasty group and 41.8 y for the conventional group (P = 0.024). The short-term complication rate of the groups was not significantly different (conventional group, 13.2% [7/53]; ductoplasty group, 8.3% [6/72]; all P > 0.05). A significant between-group difference was found in the long-term complication rate of biliary-enteric anastomotic strictures (9.4% in the conventional group and 0% in the ductoplasty group, P = 0.012). The rates of satisfactory surgical outcomes were 91.1% and 77.1% in the ductoplasty and conventional groups, respectively (P = 0.036). The application of hilar ductoplasty with a side-to-side Roux-en-Y hepaticojejunostomy as the primary surgery for bile duct cyst excision significantly reduced the postoperative complication of biliary-enteric anastomotic stricture and greatly improved our patients' prognosis with regard to biliary function. Copyright © 2017 Elsevier Inc. All rights reserved.

Percutaneous Biliary Drainage Using Open Cell Stents for Malignant Biliary Hilar Obstruction

Energy Technology Data Exchange (ETDEWEB)

Ahn, Sun Jun; Bae, Jae Ik; Han, Tae Sun; Won, Je Hwan; Kim, Ji Dae; Kwack, Kyu Sung; Lee, Jae Hee; Kim, Young Chul [Dept. of Radiology, Ajou University School of Medicine, Suwon (Korea, Republic of)

2012-11-15

To evaluate the feasibility, safety and the effectiveness of the complex assembly of open cell nitinol stents for biliary hilar malignancy. During the 10 month period between January and October 2007, 26 consecutive patients with malignant biliary hilar obstruction underwent percutaneous insertion of open cell design nitinol stents. Four types of stent placement methods were used according to the patients' ductal anatomy of the hilum. We evaluated the technical feasibility of stent placement, complications, patient survival, and the duration of stent patency. Bilobar biliary stent placement was conducted in 26 patients with malignant biliary obstruction-T (n = 9), Y (n 7), crisscross (n = 6) and multiple intersecting types (n = 4). Primary technical success was obtained in 24 of 26 (93%) patients. The crushing of the 1st stent during insertion of the 2nd stent occurred in two cases. Major complications occurred in 2 of 26 patients (7.7%). One case of active bleeding from hepatic segmental artery and one case of sepsis after procedure occurred. Clinical success was achieved in 21 of 24 (87.5%) patients, who were followed for a mean of 141.5 days (range 25-354 days). The mean primary stent patency period was 191.8 days and the mean patient survival period was 299 days. Applying an open cell stent in the biliary system is feasible, and can be effective, especially in multiple intersecting stent insertions in the hepatic hilum.

Percutaneous Biliary Drainage Using Open Cell Stents for Malignant Biliary Hilar Obstruction

International Nuclear Information System (INIS)

Ahn, Sun Jun; Bae, Jae Ik; Han, Tae Sun; Won, Je Hwan; Kim, Ji Dae; Kwack, Kyu Sung; Lee, Jae Hee; Kim, Young Chul

2012-01-01

To evaluate the feasibility, safety and the effectiveness of the complex assembly of open cell nitinol stents for biliary hilar malignancy. During the 10 month period between January and October 2007, 26 consecutive patients with malignant biliary hilar obstruction underwent percutaneous insertion of open cell design nitinol stents. Four types of stent placement methods were used according to the patients' ductal anatomy of the hilum. We evaluated the technical feasibility of stent placement, complications, patient survival, and the duration of stent patency. Bilobar biliary stent placement was conducted in 26 patients with malignant biliary obstruction-T (n = 9), Y (n 7), crisscross (n = 6) and multiple intersecting types (n = 4). Primary technical success was obtained in 24 of 26 (93%) patients. The crushing of the 1st stent during insertion of the 2nd stent occurred in two cases. Major complications occurred in 2 of 26 patients (7.7%). One case of active bleeding from hepatic segmental artery and one case of sepsis after procedure occurred. Clinical success was achieved in 21 of 24 (87.5%) patients, who were followed for a mean of 141.5 days (range 25-354 days). The mean primary stent patency period was 191.8 days and the mean patient survival period was 299 days. Applying an open cell stent in the biliary system is feasible, and can be effective, especially in multiple intersecting stent insertions in the hepatic hilum.

Comparison of endoscopic ultrasonography and computed tomography in detecting mediastinal and hilar lymph nodes from bronchogenic carcinoma

International Nuclear Information System (INIS)

Imamura, Masahiro; Murata, Takashi; Yoshida, Masayuki

1990-01-01

We investigated and compared the ability of CT and endoscopic ultrasonography by radial scanning (EUS) to diagnose metasasis of lung cancer to the mediastinum and hilar lymph nodes in 27 patients undergoing resection of primary lung cancer and 6 autopsy cases. We also determined the relationship between the presence or absence of metastasis and the size of each lymph node based on the lymph node size measured at the time of resection and its histopathological findings, and we then set up a standard value that was the most accurate in evaluating the presence or absence of metastasis using a receiver operating characteristics (ROC) curve. When lymph node sizes appearing as images were compared with their actual sizes measured on resected specimens before formalin fixation, the short axis measured by either method was found to generally agree with the actual values, while the long axis was slightly smaller than the actual values, although EUS gave more accurate values. When the ability to diagnose metastasis was compared between CT and EUS using the standard value obtained from the ROC curve (a more than 8 mm short axis was defined as positive for metastasis), there were no differences in the ability to delineate the entire area of the mediastinum, including hilar lymph nodes. With respect to individual sites, although there was some difficulty delineating some regions in the mediastinum (pretracheal lymph node) with EUS, more lymph nodes in the mediastinum that were delineated by EUS histopathologically had metastatic lesions than those delineated by CT. However, both methods often failed to delineate hilar lymph nodes, with no difference between the two methods. (author)

Can preoperative and postoperative CA19-9 levels predict survival and early recurrence in patients with resectable hilar cholangiocarcinoma?

Science.gov (United States)

Wang, Jun-Ke; Hu, Hai-Jie; Shrestha, Anuj; Ma, Wen-Jie; Yang, Qin; Liu, Fei; Cheng, Nan-Sheng; Li, Fu-Yu

2017-07-11

To investigate the predictive values of preoperative and postoperative serum CA19-9 levels on survival and other prognostic factors including early recurrence in patients with resectable hilar cholangiocarcinoma. In univariate analysis, increased preoperative and postoperative CA19-9 levels in the light of different cut-off points (37, 100, 150, 200, 400, 1000 U/ml) were significantly associated with poor survival outcomes, of which the cut-off point of 150 U/ml showed the strongest predictive value (both P 150 U/ml was significantly associated with lymph node metastasis (OR = 3.471, 95% CI 1.216-9.905; P = 0.020) and early recurrence (OR = 8.280, 95% CI 2.391-28.674; P = 0.001). Meanwhile, postoperative CA19-9 level > 150 U/ml was also correlated with early recurrence (OR = 4.006, 95% CI 1.107-14.459; P = 0.034). Ninety-eight patients who had undergone curative surgery for hilar cholangiocarcinoma between 1995 and 2014 in our institution were selected for the study. The correlations of preoperative and postoperative serum CA19-9 levels on the basis of different cut-off points with survival and various tumor factors were retrospectively analyzed with univariate and multivariate methods. In patients with resectable hilar cholangiocarcinoma, serum CA19-9 predict survival and early recurrence. Patients with increased preoperative and postoperative CA19-9 levels have poor survival outcomes and higher tendency of early recurrence.

Interobserver variability in the detection of mediastinal and hilar lymph nodes on CT in children with suspected pulmonary tuberculosis

International Nuclear Information System (INIS)

Andronikou, Savvas; Brauer, Barbara; Brachmeyer, Steven; Lucas, Susan; Joseph, Elaine; Galpin, Jackie; DuToit, George; Swingler, George

2005-01-01

The presence of mediastinal or hilar adenopathy is critical for the diagnosis of pulmonary TB. Interobserver variability in the detection of lymphadenopathy on CT in children affects the usefulness of CT as a gold standard. To determine the interobserver variability for the detection of hilar and mediastinal adenopathy on CT in children. One hundred children with clinically suspected pulmonary TB were prospectively recruited for CT scanning of the chest. Four observers reviewed the scans independently for the presence of lymphadenopathy at predetermined sites. Overall Kappa statistic was determined for each recognised site of mediastinal and hilar lymphadenopathy. Kappa statistics showed that observers only agreed moderately in their detection of lymphadenopathy. The site of best agreement was the right hilum followed by the subcarinal, right paratracheal and precarinal locations. Observers differed most at the anterior mediastinum and left hilum. The best Kappa statistic was for the overall presence of lymphadenopathy taking all sites into account. Imaging techniques that are considered the gold standard for particular diseases must be validated pathologically, and if this is not possible, interobserver variability should be evaluated. CT is considered the gold standard for detecting lymphadenopathy, but we have shown only moderate agreement between readers. Readers had difficulty in distinguishing lymphadenopathy from normal thymus and were unable to distinguish normal from pathological nodes without a predetermined size threshold for abnormality. The right hilum and the sites around the carina are the most reliable for the reported presence of lymphadenopathy. (orig.)

Malignant Hilar Biliary Obstruction: Treatment by Means of Placement of a Newly Designed Y-Shaped Branched Covered Stent

Energy Technology Data Exchange (ETDEWEB)

Yun, Jong Hyouk, E-mail: xell1015@naver.com; Jung, Gyoo-Sik, E-mail: gsjung@medimail.co.kr; Park, Jung Gu [Kosin University College of Medicine, Departments of Diagnostic Radiology (Korea, Republic of); Kang, Byung Chul [Ewha Womans University College of Medicine, Departments of Radiology, Mokdong Hospital (Korea, Republic of); Shin, Dong-Hoon [Kosin University College of Medicine, Departments of General Surgery (Korea, Republic of); Yun, Byung Chul; Lee, Sang Uk [Kosin University College of Medicine, Departments of Internal Medicine (Korea, Republic of)

2016-04-15

PurposeTo evaluate the technical feasibility and clinical efficacy of placement of a newly designed Y-shaped branched covered stent for palliative treatment of malignant hilar biliary obstruction.MethodsFrom June 2011 to September 2014, 34 consecutive patients with malignant hilar biliary obstruction underwent percutaneous placement of a Y-shaped branched covered stent for palliative treatment. Technical and clinical success, complications, cumulative patient survival, and stent patency were evaluated.ResultsStent placement was technically successful in all patients. All patients showed adequate biliary drainage on the follow-up cholangiogram. Mean serum bilirubin level (10.9 mg/dl) decreased significantly 1 week (5.7 mg/dl) and 1 month (2.6 mg/dl) after stent placement (p < 0.01). Complications associated with the procedure included hemobilia (n = 3) and biloma (n = 1). During the mean follow-up period of 225 (range 12–820) days, nine patients (26.5 %) developed stent occlusion caused by tumor overgrowth (n = 8) and sludge (n = 1). Two of them underwent coaxial placement of a second stent with good results. The median survival time was 281 days and median primary stent patency was 337 days. There were no significant differences in the patient survival and stent patency rates in relation to age, sex, or Bismuth type.ConclusionPercutaneous placement of the Y-shaped branched covered stent seems to be technically feasible and clinically effective for palliative treatment of malignant hilar biliary obstruction.

Pheochromocytoma and Adrenocortical Adenoma in the Same Gland

Directory of Open Access Journals (Sweden)

Wei-Ren Hwang

2007-07-01

Full Text Available A right adrenal tumor was found incidentally during abdominal computed tomography exam in a 51-year-old female patient, who had had diabetes and hypertension for more than 10 years. The computed tomography scan was arranged for possible pancreatic lesion by a neurologist. Norepinephrine level was high in the plasma and urine. Vanillylmandelic acid level was elevated in the urine. Diurnal cortisol rhythm, plasma adrenocorticotropic hormone and urine free cortisol were all normal, but the plasma cortisol concentration could not be suppressed after a standard low-dose dexamethasone suppression test. Therefore, adrenal cortical adenoma with subclinical Cushing's syndrome was highly suspected; however, further imaging studies, including magnetic resonance image and 131I-6β-iodomethylnorcholesterol adrenal scintigraphy failed to discriminate an additional tumor. After right adrenalectomy, a small adrenal cortical adenoma and a large pheochromocytoma were noted. This is an extremely rare case of an adrenal incidentaloma consisting of both medullary and cortical tumors in the same gland.

[Clinical value of MRI united-sequences examination in diagnosis and differentiation of morphological sub-type of hilar and extrahepatic big bile duct cholangiocarcinoma].

Science.gov (United States)

Yin, Long-Lin; Song, Bin; Guan, Ying; Li, Ying-Chun; Chen, Guang-Wen; Zhao, Li-Ming; Lai, Li

2014-09-01

To investigate MRI features and associated histological and pathological changes of hilar and extrahepatic big bile duct cholangiocarcinoma with different morphological sub-types, and its value in differentiating between nodular cholangiocarcinoma (NCC) and intraductal growing cholangiocarcinoma (IDCC). Imaging data of 152 patients with pathologically confirmed hilar and extrahepatic big bile duct cholangiocarcinoma were reviewed, which included 86 periductal infiltrating cholangiocarcinoma (PDCC), 55 NCC, and 11 IDCC. Imaging features of the three morphological sub-types were compared. Each of the subtypes demonstrated its unique imaging features. Significant differences (P big bile duct cholangiocarcinoma. MRI united-sequences examination can accurately describe those imaging features for differentiation diagnosis.

Case report

African Journals Online (AJOL)

abp

2016-05-13

May 13, 2016 ... Abstract. We report a rare case of an elderly woman presented with right renal mass with invasion of renal vein and several small lymphadenopathy in the hilar area .the diagnosis of kidney cancer is suspected and the patient underwent open radical nephrectomy, surrenalectomy and lymphadenectomy ...

A novel mutation in the succinate dehydrogenase subunit D gene in siblings with the hereditary paraganglioma–pheochromocytoma syndrome

Directory of Open Access Journals (Sweden)

Chaithra Prasad

2014-10-01

Full Text Available Germline mutations in the succinate dehydrogenase complex subunit D gene are now known to be associated with hereditary paraganglioma–pheochromocytoma syndromes. Since the initial succinate dehydrogenase complex subunit D gene mutation was identified about a decade ago, more than 131 unique variants have been reported. We report the case of two siblings presenting with multiple paragangliomas and pheochromocytomas; they were both found to carry a mutation in the succinate dehydrogenase complex subunit D gene involving a substitution of thymine to guanine at nucleotide 236 in exon 3. This particular mutation of the succinate dehydrogenase complex subunit D gene has only been reported in one previous patient in Japan; this is, therefore, the first report of this pathogenic mutation in siblings and the first report of this mutation in North America. With continued screening of more individuals, we will be able to create a robust mutation database that can help us understand disease patterns associated with particular variants and may be a starting point in the development of new therapies for familial paraganglioma syndromes.

Role of preoperative PET-CT in assessing mediastinal and hilar lymph node status in early stage lung cancer

Directory of Open Access Journals (Sweden)

Wei-Yang Lin

2012-05-01

Conclusion: Integrated PET-CT is a useful tool for predicting the negativity of mediastinal LN status pre-operatively in clinically early stage (Stages I and II lung cancer but may be relatively inaccurate in predicting hilar LN status and largely confounded by false positives caused by inflammatory process.

Clinical value of preoperative serum CA 19-9 and CA 125 levels in predicting the resectability of hilar cholangiocarcinoma.

Science.gov (United States)

Hu, Hai-Jie; Mao, Hui; Tan, Yong-Qiong; Shrestha, Anuj; Ma, Wen-Jie; Yang, Qin; Wang, Jun-Ke; Cheng, Nan-Sheng; Li, Fu-Yu

2016-01-01

To examine the predictive value of tumor markers for evaluating tumor resectability in patients with hilar cholangiocarcinoma and to explore the prognostic effect of various preoperative factors on resectability in patients with potentially resectable tumors. Patients with potentially resectable tumors judged by radiologic examination were included. The receiver operating characteristic (ROC) analysis was conducted to evaluate serum carbohydrate antigenic determinant 19-9 (CA 19-9), carbohydrate antigen 125 (CA 125) and carcino embryonie antigen levels on tumor resectability. Univariate and multivariate logistic regression models were also conducted to analysis the correlation of preoperative factors with resectability. In patients with normal bilirubin levels, ROC curve analysis calculated the ideal CA 19-9 cut-off value of 203.96 U/ml in prediction of resectability, with a sensitivity of 83.7 %, specificity of 80 %, positive predictive value of 91.1 % and negative predictive value of 66.7 %. Meanwhile, the optimal cut-off value for CA 125 to predict resectability was 25.905 U/ml (sensitivity, 78.6 %; specificity, 67.5 %). In a multivariate logistic regression model, tumor size ≤3 cm (OR 4.149, 95 % CI 1.326-12.981, P = 0.015), preoperative CA 19-9 level ≤200 U/ml (OR 20.324, 95 % CI 6.509-63.467, P hilar cholangiocarcinoma. Preoperative CA 19-9 and CA 125 levels predict resectability in patients with radiological resectable hilar cholangiocarcinoma. Increased preoperative CA 19-9 levels and CA 125 levels are associated with poor resectability rate.

Novel use of F-DOPA PET/CT imaging in a child with paraganglioma/pheochromocytoma syndrome

International Nuclear Information System (INIS)

Levine, Daniel S.; Nadel, Helen R.; Metzger, Daniel L.; Oviedo, Angelica; Adam, Michael J.; Skarsgard, Erik

2011-01-01

We report the use of F-DOPA PET/CT imaging in the evaluation of a teenager with marked hypertension and right pararenal, left adrenal and left para-aortic mass lesions. The use of the modality for this clinical application has not been described previously within the pediatric imaging literature. The value of this technique relative to conventional imaging modalities is discussed and warrants consideration of its use, if available, for evaluating children with suspected paragangliomas/pheochromocytomas. (orig.)

Novel use of F-DOPA PET/CT imaging in a child with paraganglioma/pheochromocytoma syndrome

Energy Technology Data Exchange (ETDEWEB)

Levine, Daniel S.; Nadel, Helen R. [University of British Columbia, Department of Radiology and Nuclear Medicine, British Columbia Children' s Hospital, Vancouver (Canada); Metzger, Daniel L. [University of British Columbia, Department of Pediatrics, Division of Endocrinology, British Columbia Children' s Hospital, Vancouver (Canada); Oviedo, Angelica [University of British Columbia, Department of Pathology, British Columbia Children' s Hospital, Vancouver (Canada); Adam, Michael J. [University of British Columbia, Department of PET Chemistry, Tri-University Meson Facility (TRIUMF), Vancouver (Canada); Skarsgard, Erik [University of British Columbia, Department of Surgery, British Columbia Children' s Hospital, Vancouver (Canada)

2011-10-15

We report the use of F-DOPA PET/CT imaging in the evaluation of a teenager with marked hypertension and right pararenal, left adrenal and left para-aortic mass lesions. The use of the modality for this clinical application has not been described previously within the pediatric imaging literature. The value of this technique relative to conventional imaging modalities is discussed and warrants consideration of its use, if available, for evaluating children with suspected paragangliomas/pheochromocytomas. (orig.)

Malignant pheochromocytomas and paragangliomas - the importance of a multidisciplinary approach

DEFF Research Database (Denmark)

Andersen, Kim Francis; Altaf, Rahim; Krarup-Hansen, Anders

2011-01-01

-secreted with catecholamines, may indicate tumour mass and malignancy and can be used to monitor response and relapse. The secretory and non-secretory tumours can be visualised with functional (specific and non-specific) imaging as SPECT and PET using ¹²³I-MIBG, somatostatin analogues, ¹8F-DOPA, and ¹8F-FDG. These modalities...... imaging with somatostatin analogues generally has high sensitivity in malignant disease. There are no curative therapeutic options for malignant, metastatic pheochromocytomas/paragangliomas, wherefore consolidation of quality of life is essential. Adjuvant radionuclide treatment with beta......-emitting isotopes coupled to MIBG or somatostatin analogues have shown response in approximately 30%. Chemotherapy is restricted to patients not accessible for surgery and resistant to radionuclide therapy. Novel targeted therapies, which mainly through a cytostatic effect interfere with specific targeted molecules...

[Study of anatomy of the lateral attachment of the renal fascia in adult with multidetector computed tomography].

Science.gov (United States)

Qi, Rui; Zhou, Xianping; Yu, Jianqun; Chen, Weixia; Li, Zhenlin; Zhang, Chunle

2012-08-01

The present paper is aimed to observe the lateral attachment of the renal fascia (RF) in vivo with multidetector computed tomography (MDCT) scanning, and to discuss its diagnostic value. 121 healthy adults were adopted into this experiment. All images were obtained with MDCT and double phase enhancement scanning. Then we observed the lateral attachment of RF. In addition, we mad a fresh body specimen as anatomical basis. The study found that above the renal hilar plane (RHP), the anterior renal fascia laterally fused with the peritoneum of the liver on the right and the peritoneum of the spleen on the left,and the posterior renal fascia fused with the subdiaphragmatic fascia. The lateral attachment of the RF at the RHP and the lower renal pole(LRP)is divided into three types. The RF in Type I is about 47.9% (58/121) at the left RHP, while about 33.9% (41/121) at the right RHP. At the LRP of the kidney is about 55.3% (67/121) on the left, and about 42.1% (51/121) on the right. The RF in Type I is about 38.8% (47/121) on the left side at the RHP, about 26.4% (32/121) on the right side. At the LRP, left side about 27.3% (33/121), right side about 13.3%(16/121). The RF in Type III at the RHP is 13.3% (16/121) on the left side, and on the right side is about 39.7% (48/121). At the LRP, it is about 17.4% (21/121) on the left side, and about 44.6% (54/121) on the right side. MDCT can display the lateral attachment of the RF better as well as the outside connection of the retroperitoneal space.

The impact of caudate lobe resection on margin status and outcomes in patients with hilar cholangiocarcinoma: a multi-institutional analysis from the US Extrahepatic Biliary Malignancy Consortium.

Science.gov (United States)

Bhutiani, Neal; Scoggins, Charles R; McMasters, Kelly M; Ethun, Cecilia G; Poultsides, George A; Pawlik, Timothy M; Weber, Sharon M; Schmidt, Carl R; Fields, Ryan C; Idrees, Kamran; Hatzaras, Ioannis; Shen, Perry; Maithel, Shishir K; Martin, Robert C G

2018-04-01

The objective of this study was to determine the impact of caudate resection on margin status and outcomes during resection of extrahepatic hilar cholangiocarcinoma. A database of 1,092 patients treated for biliary malignancies at institutions of the Extrahepatic Biliary Malignancy Consortium was queried for individuals undergoing curative-intent resection for extrahepatic hilar cholangiocarcinoma. Patients who did versus did not undergo concomitant caudate resection were compared with regard to demographic, baseline, and tumor characteristics as well as perioperative outcomes. A total of 241 patients underwent resection for a hilar cholangiocarcinoma, of whom 85 underwent caudate resection. Patients undergoing caudate resection were less likely to have a final positive margin (P = .01). Kaplan-Meier curve of overall survival for patients undergoing caudate resection indicated no improvement over patients not undergoing caudate resection (P = .16). On multivariable analysis, caudate resection was not associated with improved overall survival or recurrence-free survival, although lymph node positivity was associated with worse overall survival and recurrence-free survival, and adjuvant chemoradiotherapy was associated with improved overall survival and recurrence-free survival. Caudate resection is associated with a greater likelihood of margin-negative resection in patients with extrahepatic hilar cholangiocarcinoma. Precise preoperative imaging is critical to assess the extent of biliary involvement, so that all degrees of hepatic resections are possible at the time of the initial operation. Copyright © 2017 Elsevier Inc. All rights reserved.

Rescue EUS-guided intrahepatic biliary drainage for malignant hilar biliary stricture after failed transpapillary re-intervention.

Science.gov (United States)

Minaga, Kosuke; Takenaka, Mamoru; Kitano, Masayuki; Chiba, Yasutaka; Imai, Hajime; Yamao, Kentaro; Kamata, Ken; Miyata, Takeshi; Omoto, Shunsuke; Sakurai, Toshiharu; Watanabe, Tomohiro; Nishida, Naoshi; Kudo, Masatoshi

2017-11-01

Treatment of unresectable malignant hilar biliary stricture (UMHBS) is challenging, especially after failure of repeated transpapillary endoscopic stenting. Endoscopic ultrasonography-guided intrahepatic biliary drainage (EUS-IBD) is a recent technique for intrahepatic biliary decompression, but indications for its use for complex hilar strictures have not been well studied. The aim of this study was to assess the feasibility and safety of EUS-IBD for UMHBS after failed transpapillary re-intervention. Retrospective analysis of all consecutive patients with UMHBS of Bismuth II grade or higher who, between December 2008 and May 2016, underwent EUS-IBD after failed repeated transpapillary interventions. The technical success, clinical success, and complication rates were evaluated. Factors associated with clinical ineffectiveness of EUS-IBD were explored. A total of 30 patients (19 women, median age 66 years [range 52-87]) underwent EUS-IBD for UMHBS during the study period. Hilar biliary stricture morphology was classified as Bismuth II, III, or IV in 5, 13, and 12 patients, respectively. The median number of preceding endoscopic interventions was 4 (range 2-14). EUS-IBD was required because the following procedures failed: duodenal scope insertion (n = 4), accessing the papilla after duodenal stent insertion (n = 5), or achieving desired intrahepatic biliary drainage (n = 21). Technical success with EUS-IBD was achieved in 29 of 30 patients (96.7%) and clinical success was attained in 22 of these 29 (75.9%). Mild peritonitis occurred in three of 30 (10%) and was managed conservatively. Stent dysfunction occurred in 23.3% (7/30). There was no procedure-related mortality. On multivariable analysis, Bismuth IV stricture predicted clinical ineffectiveness (odds ratio = 12.7, 95% CI 1.18-135.4, P = 0.035). EUS-IBD may be a feasible and effective rescue alternative with few major complications after failed transpapillary endoscopic re-intervention in patients

[Curative effect analysis of bile reinfusion combined with enteral nutrition support before surgery of hilar cholangiocarcinoma].

Science.gov (United States)

Song, P; Mao, L; Bian, X J; Zhou, T; Fan, Y Y; Zhang, J; Xie, M; Qiu, Y D

2018-05-01

Objective: To investigate the clinical effect of bile reinfusion combined with enteral nutrition support before surgery for hilar cholangiocarcinoma. Methods: A retrospective analysis of patients with hilar cholangiocarcinoma who underwent surgical treatment at Nanjing Drum Tower Hospital Hepato-biliary-pancreatic Surgery Department from July 2010 to August 2017 was completed.A total of 52 cases were finally enrolled in our study.All the patients included, on the basis of whether they received preoperative drainage and bile reinfusion, were divided into non-drainage group( n =15) and drainage group( n =37). Differences of clinical indicators, including operation time, intraoperative bleeding and serum liver function index levels at day 1, 3, 7 postoperative, postoperative complications(liver failure, biliary fistula, pleural effusion, peritoneal effusion, abdominal cavity infection, death in hospital), tumor classification, R0 resection, postoperative hospitalization time between the 2 groups were analyzed. At the same time, in the drainage group, patients were divided into non-enteral nutrition subgroup( n =13) and enteral nutrition subgroup( n =24) according to whether they received enteral nutrition before operation. The normal distribution data of the group was statistically analyzed by independent sample t test, the non-normal distribution data of the group was statistically analyzed by rank-sum test. The count data was statistically analyzed by non-calibration and correction of the square test. Results: There was no statistically significant difference in general infomation such as age, gender, and serum liver function between non-drainage group and drainage group( P >0.05). There was no statistically significant difference in general information such as age, gender, and serum liver function between non-enteral nutrition group and enteral nutrition group( P >0.05). The rate of vascular resection and reconstruction(33.3%) and operating time(10.8(2.2)h) in

Oxytocin Depolarizes Fast-Spiking Hilar Interneurons and Induces GABA Release onto Mossy Cells of the Rat Dentate Gyrus

Science.gov (United States)

Harden, Scott W.; Frazier, Charles J.

2016-01-01

Delivery of exogenous oxytocin (OXT) to central oxytocin receptors (OXT-Rs) is currently being investigated as a potential treatment for conditions such as post-traumatic stress disorder (PTSD), depression, social anxiety, and autism spectrum disorder (ASD). Despite significant research implicating central OXT signaling in modulation of mood, affect, social behavior, and stress response, relatively little is known about the cellular and synaptic mechanisms underlying these complex actions, particularly in brain regions which express the OXT-R but lie outside of the hypothalamus (where OXT-synthesizing neurons reside). We report that bath application of low concentrations of the selective OXT-R agonist Thr4,Gly7-OXT (TGOT) reliably and robustly drives GABA release in the dentate gyrus in an action potential dependent manner. Additional experiments led to identification of a small subset of small hilar interneurons that are directly depolarized by acute application of TGOT. From a physiological perspective, TGOT-responsive hilar interneurons have high input resistance, rapid repolarization velocity during an action potential, and a robust afterhyperpolarization. Further, they fire irregularly (or stutter) in response to moderate depolarization, and fire quickly with minimal spike frequency accommodation in response to large current injections. From an anatomical perspective, TGOT responsive hilar interneurons have dense axonal arborizations in the hilus that were found close proximity with mossy cell somata and/or proximal dendrites, and also invade the granule cell layer. Further, they have primary dendrites that always extend into the granule cell layer, and sometimes have clear arborizations in the molecular layer. Overall, these data reveal a novel site of action for OXT in an important limbic circuit, and represent a significant step towards better understanding how endogenous OXT may modulate flow of information in hippocampal networks. PMID:27068005

Pheochromocytoma complicated by intracerebral hemorrhage - a case report; Feocromocitoma complicado com acidente vascular encefalico hemorragico - relato de um caso

Energy Technology Data Exchange (ETDEWEB)

Nogueira, Aline Silva; Marchiori, Edson; Almeida, Fabiola Assuncao de; Martins, Renata Romano; Sales, Anderson Ribeiro; Santos, Tereza Cristina C.R.S. dos; Reis, Simone Teixeira [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia; Silveira, Sonia Marcelino T. da

1999-06-01

The authors report a case of pheochromocytoma that was complicated by intracerebral hemorrhage in a 17-year-old female patient. Computed tomography showed a solid mass, heterogeneous, on the right adrenal. The patient underwent a right adrenalectomy. She is being observed by our out-patients clinic, presenting normal blood pressure levels and a left hemiparesis. (author)

Long-term potentiation in hilar circuitry modulates gating by the dentate gyrus.

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Wright, Brandon J; Jackson, Meyer B

2014-07-16

The dentate gyrus serves as a gateway to the hippocampus, filtering and processing sensory inputs as an animal explores its environment. The hilus occupies a strategic position within the dentate gyrus from which it can play a pivotal role in these functions. Inputs from dentate granule cells converge on the hilus, and excitatory hilar mossy cells redistribute these signals back to granule cells to transform a pattern of cortical input into a new pattern of output to the hippocampal CA3 region. Using voltage-sensitive dye to image electrical activity in rat hippocampal slices, we explored how long-term potentiation (LTP) of different excitatory synapses modifies the flow of information. Theta burst stimulation of the perforant path potentiated responses throughout the molecular layer, but left responses in the CA3 region unchanged. By contrast, theta burst stimulation of the granule cell layer potentiated responses throughout the molecular layer, as well as in the CA3 region. Theta burst stimulation of the granule cell layer potentiated CA3 responses not only to granule cell layer stimulation but also to perforant path stimulation. Potentiation of responses in the CA3 region reflected NMDA receptor-dependent LTP of upstream synapses between granule cells and mossy cells, with no detectable contribution from NMDA receptor-independent LTP of local CA3 mossy fiber synapses. Potentiation of transmission to the CA3 region required LTP in both granule cell→mossy cell and mossy cell→granule cell synapses. This bidirectional plasticity enables hilar circuitry to regulate the flow of information through the dentate gyrus and on to the hippocampus. Copyright © 2014 the authors 0270-6474/14/349743-11$15.00/0.

[Retrospective analysis of 47 cases with hilar cholangiocarcinoma using T-staging system].

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Peng, Cheng-hong; Zhao, Zhi-ming; Peng, Shu-you; Liu, Ying-bin; Wu, Yü-lian; Fang, He-qing; Jiang, Xian-chuan

2005-01-01

To evaluate the clinical value of T-staging system for hilar cholangiocarcinoma which was adopted in memorial Sloan-Kettering cancer center of New York. The image data of these 47 patients were analyzed retrospectively from December 1997 to December 2002 whose data were according with our demand, and they were staged into three-stage according to the criteria of the T-staging system. The difference of respectability, ratio of tumor-free resection margin and actuarial survival rate were analyzed for different T-staging. And the coincident ratio of three different kinds of imaging methods was also analyzed. Twenty patients had T(1) tumors, twenty three had T(2) tumors and four had T(3) tumors. The resectability of the three stage was 60%, 39% and 0% respectively, and the difference was significant (P = 0.013). The likelihood of achieving tumor-free margin decreased progressively with increasing T stage (P = 0.018). The cumulative 1-year survival rates of T(1), T(2) and T(3) patients were 60%, 39% and 0% respectively, and the cumulative 3-year survival rate was 35%, 9% and 0% respectively, the survival of different stage patients differed markedly (P = 0.0103). The coincident ratio of combined using MRCP and color Doppler-ultrasonography was higher than that of combined using MRCP and B-ultrasonography or combined using CT/SCT and color Doppler-ultrasonography (P = 0.007). The T-staging system has a better value for preoperative assessment, and can be used to judge resectability and survival of hilar cholangiocarcinoma. It will be helpful to use MRCP and color Doppler-Ultrasonography combined to verdict the coverage of the tumor and the T-staging preoperatively.

Two-dimensional time-of-flight MR angiography of mediastinum and pulmonary hilar vessels

International Nuclear Information System (INIS)

Honda, Norinari; Machida, Kikuo; Mamiya, Toshio

1992-01-01

Two-dimensional time-of-flight magnetic resonance angiography (2D TOF MRA) of mediastinal and pulmonary hilar vessels was performed in 10 patients, seven men and three women with a mean age (range) of 65.7 (48-88) years. The rate of visualization of the vessels and the diagnostic ability of 2D TOF MRA were assessed in comparison with contrast-enhanced CT. A radiofrequency-spoiled gradient echo sequence (SPGR) was used during repeated breath-holding (8-27 seconds) in coronal (8 patients) and axial (2 patients) imaging planes on a 1.5 Tesla superconducting scanner under the following conditions: repetition time/echo time/flip angle/excitation: 25-33/7-8 ms/45deg/1, field-of-view: 30 x 30 cm, slice thickness: 2.5 mm, 32 slices, 256 (frequency) x 192 (phase) matrix, with gradient moment nulling technique. Visualization sufficient to enable diagnosis of the vascular lesion was obtained in 95 (52%) vessels, mere visualization in 63 (35%), and non-visualization in 24 (13%) of the 182 evaluable vessels. The rates of good visualization of pulmonary hilar vessels (26/86, 30%) and veins (26/48, 54%) were significantly lower than that of arteries (43/48, 90%, p<0.05). The sensitivity and specificity of 2D TOF MRA were 77% (10/13) and 100% (83/83), respectively, in 96 evaluable vessels of nine patients. 2D TOF MRA of mediastinum and pulmonary hili is clinically feasible, and may be useful because of its high specificity. (author)

Motion-specific internal target volumes for FDG-avid mediastinal and hilar lymph nodes

International Nuclear Information System (INIS)

Lamb, James M.; Robinson, Clifford G.; Bradley, Jeffrey D.; Low, Daniel A.

2013-01-01

Background and purpose: To quantify the benefit of motion-specific internal target volumes for FDG-avid mediastinal and hilar lymph nodes generated using 4D-PET, vs. conventional internal target volumes generated using non-respiratory gated PET and 4D-CT scans. Materials and methods: Five patients with FDG-avid tumors metastatic to 11 hilar or mediastinal lymph nodes were imaged with respiratory-correlated FDG-PET (4D-PET) and 4D-CT. FDG-avid nodes were contoured by a radiation oncologist in two ways. Standard-of-care volumes were contoured using conventional un-gated PET, 4D-CT, and breath-hold CT. A second, motion-specific, set of volumes were contoured using 4D-PET.Contours based on 4D-PET corresponded directly to an internal target volume (ITV 4D ), whereas contours based on un-gated PET were expanded by a series of exploratory isotropic margins (from 5 to 13 mm) based on literature recommendations on lymph node motion to form internal target volumes (ITV 3D ). Results: A 13 mm expansion of the un-gated PET nodal volume was needed to cover the ITV 4D for 10 of 11 nodes studied. The ITV 3D based on a 13 mm expansion included on average 45 cm 3 of tissue that was not included in the ITV 4D . Conclusions: Motion-specific lymph-node internal target volumes generated from 4D-PET imaging could be used to improve accuracy and/or reduce normal-tissue irradiation compared to the standard-of-care un-gated PET based internal target volumes

[The impact of preoperative biliary drainage on surgical morbidity in hilar cholangiocarcinoma patients].

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Li, Shao-qiang; Chen, Dong; Liang, Li-jian; Peng, Bao-gang; Yin, Xiao-yu

2009-08-01

To evaluate the impact of preoperative biliary drainage on surgical morbidity in hilar cholangiocarcinoma patients underwent surgery. One hundred and eleven consecutive patients with hilar cholangiocarcinoma whose serum total bilirubin (TBIL) level > 85 micromol/L and underwent surgery in the period from June 1998 to August 2007 were enrolled. There were 67 male and 44 female patients, aged from 26 to 82 years old with a mean of 56 years old. Fifty-five patients underwent preoperative biliary drainage with a mean of 11.4 d of drainage period (drainage group), the other (n = 56) were the non-drainage group. The preoperative TBIL level of drainage group was (154 +/- 69) micromol/L, which was significantly lower than the value of pre-drainage (256 +/- 136) micromol/L (P = 0.000) and the value of non-drainage group (268 +/- 174) micromol/L (P = 0.005). ALT and GGT levels could be lowered by preoperative biliary drainage. The postoperative complications of these two groups were comparable (36.3% vs. 28.6%, P = 0.381). Four patients in drainage group and 5 patients in non-drainage group died of liver failure. Multivariate logistic regression indicated that hepatectomy (OR = 0.284, P = 0.003) was the independent risk factor associated with postoperative morbidity. Bismuth-Corlette classification (OR = 0.211, P = 0.028) was the independent risk factor linked to postoperative mortality. Preoperative biliary drainage could alleviate liver injury due to hyperbilirubin, but it could not decrease the surgical morbidity and postoperative mortality. Concomitant hepatectomy and Bismuth-Corlette classification were independent risk factors linked to surgical risks.

Usefulness of [18F]-DA and [18F]-DOPA for PET imaging in a mouse model of pheochromocytoma

International Nuclear Information System (INIS)

Martiniova, Lucia; Cleary, Susannah; Lai, Edwin W.; Kiesewetter, Dale O.; Seidel, Jurgen; Dawson, Linda F.; Phillips, Jacqueline K.; Thomasson, David; Chen Xiaoyuan; Eisenhofer, Graeme; Powers, James F.; Kvetnansky, Richard

2012-01-01

Purpose: To evaluate the usefulness of [ 18 F]-6-fluorodopamine ([ 18 F]-DA) and [ 18 F]-L-6-fluoro-3,4-dihydroxyphenylalanine ([ 18 F]-DOPA) positron emission tomography (PET) in the detection of subcutaneous (s.c.) and metastatic pheochromocytoma in mice; to assess the expression of the norepinephrine transporter (NET) and vesicular monoamine transporters 1 and 2 (VMAT1 and VMAT2), all important for [ 18 F]-DA and [ 18 F]-DOPA uptake. Furthermore, to compare tumor detection by micro-computed tomography (microCT) to magnetic resonance imaging (MRI) in individual mouse. Methods: SUV max values were calculated from [ 18 F]-DA and [ 18 F]-DOPA PET, tumor-to-liver ratios (TLR) were obtained and expression of NET, VMAT1 and VMAT2 was evaluated. Results: [ 18 F]-DA detected less metastatic lesions compared to [ 18 F]-DOPA. TLR values for liver metastases were 2.26–2.71 for [ 18 F]-DOPA and 1.83–2.83 for [ 18 F]-DA. A limited uptake of [ 18 F]-DA was found in s.c. tumors (TLR=0.22-0.27) compared to [ 18 F]-DOPA (TLR=1.56-2.24). Overall, NET and VMAT2 were expressed in all organ and s.c. tumors. However, s.c. tumors lacked expression of VMAT1. We confirmed [ 18 F]-DA's high affinity for the NET for its uptake and VMAT1 and VMAT2 for its storage and retention in pheochromocytoma cell vesicles. In contrast, [ 18 F]-DOPA was found to utilize only VMAT2. Conclusion: MRI was superior in the detection of all organ tumors compared to microCT and PET. [ 18 F]-DOPA had overall better sensitivity than [ 18 F]-DA for the detection of metastases. Subcutaneous tumors were localized only with [ 18 F]-DOPA, a finding that may reflect differences in expression of VMAT1 and VMAT2, perhaps similar to some patients with pheochromocytoma where [ 18 F]-DOPA provides better visualization of lesions than [ 18 F]-DA.

The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency

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Devilee Peter

2005-11-01

Full Text Available Abstract Background The SDHA, SDHB, SDHC and SDHD genes encode the subunits of succinate dehydrogenase (succinate: ubiquinone oxidoreductase, a component of both the Krebs cycle and the mitochondrial respiratory chain. SDHA, a flavoprotein and SDHB, an iron-sulfur protein together constitute the catalytic domain, while SDHC and SDHD encode membrane anchors that allow the complex to participate in the respiratory chain as complex II. Germline mutations of SDHD and SDHB are a major cause of the hereditary forms of the tumors paraganglioma and pheochromocytoma. The largest subunit, SDHA, is mutated in patients with Leigh syndrome and late-onset optic atrophy, but has not as yet been identified as a factor in hereditary cancer. Description The SDH mutation database is based on the recently described Leiden Open (source Variation Database (LOVD system. The variants currently described in the database were extracted from the published literature and in some cases annotated to conform to current mutation nomenclature. Researchers can also directly submit new sequence variants online. Since the identification of SDHD, SDHC, and SDHB as classic tumor suppressor genes in 2000 and 2001, studies from research groups around the world have identified a total of 120 variants. Here we introduce all reported paraganglioma and pheochromocytoma related sequence variations in these genes, in addition to all reported mutations of SDHA. The database is now accessible online. Conclusion The SDH mutation database offers a valuable tool and resource for clinicians involved in the treatment of patients with paraganglioma-pheochromocytoma, clinical geneticists needing an overview of current knowledge, and geneticists and other researchers needing a solid foundation for further exploration of both these tumor syndromes and SDHA-related phenotypes.

A Comparison of Bilateral Side-by-Side Metal Stents Deployed Above and Across the Sphincter of Oddi in the Management of Malignant Hilar Biliary Obstruction.

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Cosgrove, Natalie; Siddiqui, Ali A; Adler, Douglas G; Shahid, Haroon; Sarkar, Avik; Sharma, Ashish; Kowalski, Thomas E; Loren, David; Warndorf, Matthew; Chennat, Jennifer; Munigala, Satish; Papachristou, Georgios I

2017-07-01

The optimal method for endoscopic placement of bilateral self-expanding metal stents (SEMS) for the management of malignant hilar biliary obstruction has not been determined. The aim of this study was to compare the efficacies and complication rates between SEMS placed above and across the sphincter of Oddi (SO) in patients with malignant hilar biliary obstruction. A retrospective review of patients with malignant hilar strictures who underwent bilateral SEMS placement at 3 centers was performed. Patients were divided into 2 groups: group A (above SO, n=52) or B (across SO, n=120). Patient demographics, technical success (successful SEMS placement across the stricture), functional success (decrease in pretreatment bilirubin level), complications, stent occlusion, and patient survival in the 2 groups were evaluated. We identified 172 patients with malignant hilar biliary obstruction (106 males, mean age 67 y). Significantly more early complications (1.9% vs. 11.7%, P=0.04) were seen in group B, mainly post-endoscopic retrograde cholangio-pancreatography pancreatitis. Mean SEMS patency periods were 33 weeks for group A and 29.6 weeks for group B (P=0.3). Occlusion rates were 50% and 45% for groups A and B (P=0.61); occlusion was due to tumor in-growth or overgrowth in all patients. SEMS occlusion was successfully treated endoscopically in 85% (22/26) patients in group A and 96% (52/54) in group B (P=0.24). The median survival time was 26 weeks in the group A and 29 weeks in group B (P=0.49). Bilateral side-by-side SEMS placement above or below the SO results in similar success rates, stent patency duration, and stent occlusion rates. Significantly fewer complications, with a trend toward lower rates of pancreatitis, were observed for SEMS placed above the SO.

The value of digital tomosynthesis of the chest as a problem-solving tool for suspected pulmonary nodules and hilar lesions detected on chest radiography

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Galea, Angela, E-mail: galeaangie@gmail.com [Peninsula Radiology Academy, William Prance Road, Plymouth PL65WR (United Kingdom); Dubbins, Paul, E-mail: Paul.dubbins@nhs.net [Plymouth Hospital NHS Trust, Plymouth PL68DH (United Kingdom); Riordan, Richard, E-mail: richardriordan@nhs.net [Plymouth Hospital NHS Trust, Plymouth PL68DH (United Kingdom); Adlan, Tarig, E-mail: tarig.adlan@nhs.net [Plymouth Hospital NHS Trust, Plymouth PL68DH (United Kingdom); Roobottom, Carl, E-mail: carl.roobotoom@nhs.net [Plymouth Hospital NHS Trust, Plymouth PL68DH (United Kingdom); Gay, David, E-mail: davegay@nhs.net [Plymouth Hospital NHS Trust, Plymouth PL68DH (United Kingdom)

2015-05-15

Graphical abstract: When compared to CXR, DTS has: • Superior resolution • Better assessment of location in the AP dimension (better at locating a pleural or intrapulmonary lesion) • Better characterisation (better at distinguishing between calcified plaque and soft tissue) • Removes composite artefact caused by overlying anatomical structures (such as the ribs or pulmonary vessels) DTS has improved sensitivity, specificity and accuracy when compared to CXR. - Highlights: • DTS is a type of limited angle tomography. Sixty coronal reconstructed images of the chest are produced that combine the superior resolution of radiography with the tomographic benefits of computed tomography. • The sensitivity for detecting a suspected lung lesions is 0.65 with CXR and 0.91 for DTS. • The high specificity of DTS (1) and the high negative predictive value (0.94) are similar to CT and suggest that if the DTS is normal patients do not need further assessment with CT with significant potential dose savings. • 50% of suspected lesions were resolved with CXR, this improved to 96% with DTS. - Abstract: Objectives: To assess the capability of digital tomosynthesis (DTS) of the chest compared to a postero-anterior (PA) and lateral chest radiograph (CXR) in the diagnosis of suspected but unconfirmed pulmonary nodules and hilar lesions detected on a CXR. Computed tomography (CT) was used as the reference standard. Materials and method: 78 patients with suspected non-calcified pulmonary nodules or hilar lesions on their CXR were included in the study. Two radiologists, blinded to the history and CT, prospectively analysed the CXR (PA and lateral) and the DTS images using a picture archiving and communication workstation and were asked to designate one of two outcomes: true intrapulmonary lesion or false intrapulmonary lesion. A CT of the chest performed within 4 weeks of the CXR was used as the reference standard. Inter-observer agreement and time to report the modalities

The value of digital tomosynthesis of the chest as a problem-solving tool for suspected pulmonary nodules and hilar lesions detected on chest radiography

International Nuclear Information System (INIS)

Galea, Angela; Dubbins, Paul; Riordan, Richard; Adlan, Tarig; Roobottom, Carl; Gay, David

2015-01-01

Graphical abstract: When compared to CXR, DTS has: • Superior resolution • Better assessment of location in the AP dimension (better at locating a pleural or intrapulmonary lesion) • Better characterisation (better at distinguishing between calcified plaque and soft tissue) • Removes composite artefact caused by overlying anatomical structures (such as the ribs or pulmonary vessels) DTS has improved sensitivity, specificity and accuracy when compared to CXR. - Highlights: • DTS is a type of limited angle tomography. Sixty coronal reconstructed images of the chest are produced that combine the superior resolution of radiography with the tomographic benefits of computed tomography. • The sensitivity for detecting a suspected lung lesions is 0.65 with CXR and 0.91 for DTS. • The high specificity of DTS (1) and the high negative predictive value (0.94) are similar to CT and suggest that if the DTS is normal patients do not need further assessment with CT with significant potential dose savings. • 50% of suspected lesions were resolved with CXR, this improved to 96% with DTS. - Abstract: Objectives: To assess the capability of digital tomosynthesis (DTS) of the chest compared to a postero-anterior (PA) and lateral chest radiograph (CXR) in the diagnosis of suspected but unconfirmed pulmonary nodules and hilar lesions detected on a CXR. Computed tomography (CT) was used as the reference standard. Materials and method: 78 patients with suspected non-calcified pulmonary nodules or hilar lesions on their CXR were included in the study. Two radiologists, blinded to the history and CT, prospectively analysed the CXR (PA and lateral) and the DTS images using a picture archiving and communication workstation and were asked to designate one of two outcomes: true intrapulmonary lesion or false intrapulmonary lesion. A CT of the chest performed within 4 weeks of the CXR was used as the reference standard. Inter-observer agreement and time to report the modalities

A case of von Hippel-Lindau disease with exudative maculopathy

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Basel T Ba′arah

2009-01-01

Full Text Available Von Hippel-Lindau (VHL disease is a rare multisystem familial tumor syndrome of autosomal dominant inheritance. Hallmark lesions include retinal, cerebellum and spinal cord hemangioblastomas, renal cell carcinomas, adrenal pheochromocytomas, angiomatous or cystic lesions of the kidneys, pancreas, and epididymis. We report a case of VHL disease in a 26-year-old patient who presented with exudative macular edema. Ocular and systemic studies revealed the presence of retinal and central nervous system hemangioblastomas, adrenal pheochromocytoma, multiple pancreatic, and kidney cysts. The retinal angiomas were successfully treated with argon laser photocoagulation and cryotherapy.

Pheochromocytoma/Paraganglioma: A Poster Child for Cancer Metabolism.

Science.gov (United States)

Tevosian, Sergei G; Ghayee, Hans K

2018-05-01

Pheochromocytomas (PCCs) are tumors that are derived from the chromaffin cells of the adrenal medulla. Extra-adrenal PCCs called paragangliomas (PGLs) are derived from the sympathetic and parasympathetic chain ganglia. PCCs secrete catecholamines, which cause hypertension and have adverse cardiovascular consequences as a result of catecholamine excess. PGLs may or may not produce catecholamines depending on their genetic type and anatomical location. The most worrisome aspect of these tumors is their ability to become aggressive and metastasize; there are no known cures for metastasized PGLs. Original articles and reviews indexed in PubMed were identified by querying with specific PCC/PGL- and Krebs cycle pathway-related terms. Additional references were selected through the in-depth analysis of the relevant publications. We primarily discuss Krebs cycle mutations that can be instrumental in helping investigators identify key biological pathways and molecules that may serve as biomarkers of or treatment targets for PCC/PGL. The mainstay of treatment of patients with PCC/PGLs is surgical. However, the tide may be turning with the discovery of new genes associated with PCC/PGLs that may shed light on oncometabolites used by these tumors.

Feocromocitoma: uma causa rara de hipertensão arterial na infância Pheochromocytoma: a rare cause of hypertension in childhood

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Flávia Carolina D. Georgetti

2007-09-01

Full Text Available OBJETIVO: Relatar o caso de um adolescente com feocromocitoma, uma causa rara de hipertensão arterial na infância. DESCRIÇÃO: Adolescente internado em unidade de terapia intensiva infantil em decorrência de emergência hipertensiva, conseqüente à presença de feocromocitoma em adrenal esquerda, diagnosticado por meio de tomografia computadorizada do abdome e pela dosagem de adrenalina e noradrenalina urinárias. O paciente foi submetido à adrenalectomia esquerda, após o uso de alfa-bloqueador para controle do quadro hipertensivo. O anatomopatológico confirmou o diagnóstico do tumor. No pós-operatório, o paciente permaneceu estável, possibilitando a suspensão dos anti-hipertensivos. COMENTÁRIOS: Os feocromocitomas são tumores capazes de produzir catecolaminas, especialmente adrenalina e/ou noradrenalina. Cerca de 85 a 95% dos tumores são únicos, benignos e encontrados na medula adrenal. O feocromocitoma é um tumor de incidência rara e apenas 10 a 20% ocorrem na infância, representando uma causa rara de hipertensão arterial. Esta última é um sinal freqüente na maioria das crianças (80%, podendo ser acompanhada por cefaléia e sudorese. A encefalopatia hipertensiva consiste em uma forma de apresentação excepcional da doença. O diagnóstico pode ser realizado, na maioria dos casos, pela tomografia de abdome e pela dosagem das catecolaminas e seus metabólitos produzidos pelo tumor. O tratamento de escolha consiste na ressecção completa do tumor após o preparo farmacológico do paciente com o uso de alfa-bloqueador. No pós-operatório, a maioria dos pacientes evolui com controle do quadro de hipertensão arterial.OBJECTIVE: Report an adolescent with pheochromocytoma, a rare cause of hypertension in childhood. CASE DESCRIPTION: Adolescent admitted to the pediatric intensive care unit due to hypertension, secondary to the presence of pheochromocytoma on the left adrenal. Diagnosis of the pheochromocytoma was made by

Lithium Improves Survival of PC12 Pheochromocytoma Cells in High-Density Cultures and after Exposure to Toxic Compounds

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Cinzia Fabrizi

2014-01-01

Full Text Available Autophagy is an evolutionary conserved mechanism that allows for the degradation of long-lived proteins and entire organelles which are driven to lysosomes for digestion. Different kinds of stressful conditions such as starvation are able to induce autophagy. Lithium and rapamycin are potent autophagy inducers with different molecular targets. Lithium stimulates autophagy by decreasing the intracellular myo-inositol-1,4,5-triphosphate levels, while rapamycin acts through the inhibition of the mammalian target of rapamycin (mTOR. The correlation between autophagy and cell death is still a matter of debate especially in transformed cells. In fact, the execution of autophagy can protect cells from death by promptly removing damaged organelles such as mitochondria. Nevertheless, an excessive use of the autophagic machinery can drive cells to death via a sort of self-cannibalism. Our data show that lithium (used within its therapeutic window stimulates the overgrowth of the rat Pheochromocytoma cell line PC12. Besides, lithium and rapamycin protect PC12 cells from toxic compounds such as thapsigargin and trimethyltin. Taken together these data indicate that pharmacological activation of autophagy allows for the survival of Pheochromocytoma cells in stressful conditions such as high-density cultures and exposure to toxins.

Local and Long-Range Circuit Connections to Hilar Mossy Cells in the Dentate Gyrus

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Sun, Yanjun; Grieco, Steven F.; Holmes, Todd C.

2017-01-01

Abstract Hilar mossy cells are the prominent glutamatergic cell type in the dentate hilus of the dentate gyrus (DG); they have been proposed to have critical roles in the DG network. To better understand how mossy cells contribute to DG function, we have applied new viral genetic and functional circuit mapping approaches to quantitatively map and compare local and long-range circuit connections of mossy cells and dentate granule cells in the mouse. The great majority of inputs to mossy cells consist of two parallel inputs from within the DG: an excitatory input pathway from dentate granule cells and an inhibitory input pathway from local DG inhibitory neurons. Mossy cells also receive a moderate degree of excitatory and inhibitory CA3 input from proximal CA3 subfields. Long range inputs to mossy cells are numerically sparse, and they are only identified readily from the medial septum and the septofimbrial nucleus. In comparison, dentate granule cells receive most of their inputs from the entorhinal cortex. The granule cells receive significant synaptic inputs from the hilus and the medial septum, and they also receive direct inputs from both distal and proximal CA3 subfields, which has been underdescribed in the existing literature. Our slice-based physiological mapping studies further supported the identified circuit connections of mossy cells and granule cells. Together, our data suggest that hilar mossy cells are major local circuit integrators and they exert modulation of the activity of dentate granule cells as well as the CA3 region through “back-projection” pathways. PMID:28451637

T-configured Dual Stent Placement in Malignant Biliary Hilar Obstruction: Technique and Clinical Application

International Nuclear Information System (INIS)

Jeon, Yong Sik; Kim, Ji Hyung

2010-01-01

To evaluate implanting techniques of T-configured dual stents in malignant hilar obstruction and investigate the clinical factors related to stent obstruction. The study included 28 patients undergoing T-configured dual stent implantation to palliate malignant biliary hilar obstruction. The unilobar approach was first attempted in the procedure, which progressed to bilobar approach when it was found that the unilobar approach was not feasible. If the stent was again obstructed, clinical palliation was attempted using stent re-insertion or PTBD. Clinical factors associated with T-configured dual stenting were examined in the patients with stent re-obstruction, which was followed by a correlation between these clinical factors and stent obstruction. Eleven of 13 patients were successfully treated by the unilobar approach. The two unsuccessful cases sustained angulation of the central large mesh stent. For the bilobar approach, 14 of 15 patients were successfully treated. For the one unsuccessful patient, the stent failed to pass through the central large mesh. Stent obstruction was revealed in 13 patients during the follow-up period. Balloon-assisted stent re-canalization was successfully performed in 5 patients. The analysis suggests that no definite correlation was found between stent obstruction and the clinical factors associated with dual stent placement. Conversely, bile containing sludge or debris was significantly correlated to early stent obstruction. Technical adoption considering the bile duct anatomy and obstruction pattern is important for T-configured dual stent implantation. The prudent evaluation of bile juice characters and cholangiographic findings is required for the appropriate clinical application of the T-configured dual stenting

A novel combined interventional radiologic and hepatobiliary surgical approach to a complex traumatic hilar biliary stricture

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Rachel E. NeMoyer

Full Text Available Introduction: Benign strictures of the biliary system are challenging and uncommon conditions requiring a multidisciplinary team for appropriate management. Presentation of case: The patient is a 32-year-old male that developed a hilar stricture as sequelae of a gunshot wound. Due to the complex nature of the stricture and scarring at the porta hepatis a combined interventional radiologic and surgical approach was carried out to approach the hilum of the right and left hepatic ducts. The location of this stricture was found by ultrasound guidance intraoperatively using a balloon tipped catheter placed under fluoroscopy in the interventional radiology suite prior to surgery. This allowed the surgeons to select the line of parenchymal transection for best visualization of the stricture. A left hepatectomy was performed, the internal stent located and the right hepatic duct opened tangentially to allow a side-to-side Roux-en-Y hepaticojejunostomy (a Puestow-like anastomosis. Discussion: Injury to the intrahepatic biliary ductal confluence is rarely fatal, however, the associated injuries lead to severe morbidity as seen in this example. Management of these injuries poses a considerable challenge to the surgeon and treating physicians. Conclusion: Here we describe an innovative multi-disciplinary approach to the repair of this rare injury. Keywords: Combined approach, Interventional radiology, Hepatobiliary surgery, Complex traumatic hilar biliary stricture, Case report

Value of dual-time-point 18FDG PET-CT imaging on involved-field radiotherapy for hilar and mediastinal metastatic lymph nodes in non-small cell lung cancer

International Nuclear Information System (INIS)

Hu Man; Sun Xindong; Liu Ningbo; Gong Heyi; Fu Zheng; Ma Li; Li Xinke; Xu Xiaoqing; Yu Jinming

2008-01-01

Objective: To discuss the value of dual-time-point 18 FDG PET-CT imaging on involved-field radiotherapy for hilar and mediastinal metastatic lymph nodes in patients with non-small cell lung cancer (NSCLC). Methods: Fifty-four patients with NSCLC were included in this analysis, including 34 men and 20 women with mean age of 59 (34-76) years. Two sequential PET-CT scans given 3-5 days before surgery were standard single-time-point imaging for the whole body and delayed imaging for the thorax. The pathologic data were used as golden standard to determine the difference between the standard single-time-point and dual-time-point PET-CT imaging in the definition of gross target volume (GTV) of involved-field radiotherapy for metastatic lymph nodes. Results: For hilar metastatic lymph nodes, the GTV defined by single-time-point imaging was consistent with pathologic GTV in 21 patients (39%), comparing with 31 patients (57%) by dual-time-point imaging. Using pathologic data as golden standard, GTV alteration defined by single-time-point imaging had statistically significant difference comparing with that defined by dual-time-point imaging( =519.00, P=0.023). For mediastinal metastatic lymph nodes, the GTV defined by single-time-point imaging was consistent with pathologic GTV in 30 patients (56%), comparing with 36 patients (67%) by dual-time-point imaging. Using pathologic data as golden standard, GTV alteration defined by single-time-point imaging had no statistically significant difference comparing with that defined by dual-time-point imaging (u= 397.50, P=0.616). Conclusions: For patients with NSCLC receiving involved-field radiotherapy, GTV definition for hilar and mediastinal metastatic lymph nodes by dual-time-point imaging is more consistent with that by pathologic data. Dual-time-point imaging has a larger value in terms of target delineation for hilar and mediastinal metastatic lymph nodes. (authors)

Segmental hepatic artery at hepatic hilar area. Analysis by 3 dimensional integrated image of artery, portal vein and bile duct

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Sakai, Hisamune; Okuda, Kouji; Yoshida, Jun; Kinoshita, Hisafumi; Aoyagi, Shigeaki [Kurume Univ., School of Medicine, Kurume, Fukuoka (Japan)

2006-11-15

Multiple individual variations in running and bifurcation of the hepatic artery, biliary duct and portal vein are known in hepatic hilar area. This paper describes the examination of such arterial variations by integrating the 3D images of those vessels obtained by multidetector-row CT (MDCT). Subjects are findings from 64 patients with cholangiocarcinoma, hepatocarcinoma or cholelithiasis. MDCT dynamic scanning, and percutaneous transhepatic biliary drainage-CT and/or drip infusion cholangiography-CT with the intravenous iopamidol and/or iotroxate megulumin, were done with GE LightSpeed Ultra 16 slice type equipment to compose the 3D images. Arterial variants of the bifurcation in the right and left lobe were found to be 18 cases/62 (29%) and 13/64 (20%), respectively. The left artery running at right side of portal venous umbilical region was seen in 9/64 (14%) and right artery running ''northward'', in 9/62 (14%). Previous realization of such individual 3D arterial variations as above is necessary for the precise microsurgery of the hilar area to preserve the essential vessel. (T.I.)

Segmental hepatic artery at hepatic hilar area. Analysis by 3 dimensional integrated image of artery, portal vein and bile duct

International Nuclear Information System (INIS)

Sakai, Hisamune; Okuda, Kouji; Yoshida, Jun; Kinoshita, Hisafumi; Aoyagi, Shigeaki

2006-01-01

Multiple individual variations in running and bifurcation of the hepatic artery, biliary duct and portal vein are known in hepatic hilar area. This paper describes the examination of such arterial variations by integrating the 3D images of those vessels obtained by multidetector-row CT (MDCT). Subjects are findings from 64 patients with cholangiocarcinoma, hepatocarcinoma or cholelithiasis. MDCT dynamic scanning, and percutaneous transhepatic biliary drainage-CT and/or drip infusion cholangiography-CT with the intravenous iopamidol and/or iotroxate megulumin, were done with GE LightSpeed Ultra 16 slice type equipment to compose the 3D images. Arterial variants of the bifurcation in the right and left lobe were found to be 18 cases/62 (29%) and 13/64 (20%), respectively. The left artery running at right side of portal venous umbilical region was seen in 9/64 (14%) and right artery running ''northward'', in 9/62 (14%). Previous realization of such individual 3D arterial variations as above is necessary for the precise microsurgery of the hilar area to preserve the essential vessel. (T.I.)

Scintigraphic localization of a disseminated malignant pheochromocytoma with the use of 131I-meta-iodobenzylguanidine

International Nuclear Information System (INIS)

Troncone, L.; Maini, L.M.; Rufini, V.; Bonifazi, N.; De Rosa, G.; Corsello, S.M.; Mattei, O.

1984-01-01

Preliminary clinical studies with 131 I-meta-iodo-benzylguanidine, a newly synthesized radiopharmaceutical and guanethidine analog capable of imaging the adrenal medulla, have led to the identification, of a case of disseminated malignant pheochromocytoma with the localization of brain and bone metastases. The result is of particular interest as the symptomatology in this case appeared rather equivocal and various investigations had led to a completely different diagnosis. This new scintigraphic technique has proved to be safe, specific and noninvasive, and it may have a clinical application as a complementary or alternative technique to conventional diagnostic tests. (orig.)

Usefulness of standardized uptake values for distinguishing adrenal glands with pheochromocytoma from normal adrenal glands by use of 6-18F-fluorodopamine PET.

NARCIS (Netherlands)

Timmers, H.J.L.M.; Carrasquillo, J.A.; Whatley, M.A.; Eisenhofer, G.; Chen, C.C.; Ling, A.; Linehan, W.M.; Pinto, P.A.; Adams, K.T.; Pacak, K.

2007-01-01

6-(18)F-Fluorodopamine ((18)F-FDA) PET is a highly sensitive tool for the localization of pheochromocytoma (PHEO). The aim of this study was to establish cutoff values for pathologic and physiologic adrenal gland tracer uptake. METHODS: (18)F-FDA PET with CT coregistration was performed in 14

Right trisectionectomy with principle en bloc portal vein resection for right-sided hilar cholangiocarcinoma: no-touch technique.

Science.gov (United States)

Machado, Marcel Autran; Makdissi, Fabio F; Surjan, Rodrigo C

2012-04-01

The most favorable long-term survival rate for hilar cholangiocarcinoma is achieved by a R0 resection. A surgical concept involving a no-touch technique, with extended right hepatic resections and principle en bloc portal vein resection was described by Neuhaus et al. According to Neuhaus et al., their technique may increase the chance of R0, because the right branch of the portal vein and hepatic artery is in close contact with the tumor and is frequently infiltrated. The left artery runs on the left margin of the hilum and often is free. The 5-year survival rate for their patients is 61% but 60-day mortality rate is 8%. Given the increased morbidity, some authors do not agree with routine resection of portal vein and may perform the resection of portal vein only on demand, after intraoperative assessment and confirmation of portal vein invasion. This video shows en bloc resection of extrahepatic bile ducts, portal vein bifurcation, and right hepatic artery, together with extended right trisectionectomy (removal of segments 1, 4, 5, 6, 7, and 8). A 75-year-old man with progressive jaundice due to right-sided hilar cholangiocarcinoma underwent percutaneous biliary drainage with metallic stents for palliation. The patient was referred for a second opinion. Serum bilirubin levels were normal, and CT scan showed a resectable tumor, but volumetry showed a small left liver remnant. Right portal vein embolization was then performed, and CT scan performed after 4 weeks showed adequate compensatory hypertrophy of the future liver remnant (segments 2 and 3). Surgical decision was to perform a right trisectionectomy with en bloc portal vein and bile duct resection using the no-touch technique. The operation began with hilar lymphadenectomy. The common bile duct is sectioned. Right hepatic artery is ligated. Left hepatic artery is encircled. Portal vein is dissected and encircled. Right liver is mobilized and detached from retrohepatic vena cava. Right and middle hepatic

Management of anesthesia in unspecified extra-adrenal pheochromocytoma patient who used beta-blocker

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Ayse Belin Ozer

2014-01-01

Full Text Available An operation was planned for a female patient aged 59 for intra-abdominal mass. The patient was using nebivolol for hypertension. Blood pressure (BP of the patient was raised to 200/130 mmHg during anesthesia induction. BP was gradually reduced by remifentanil infusion. Following the manipulation of the mass, BP began to increase (225/160 mmHg, thus nitroglycerin and followed nitroprusside infusion was started. Propofol (200 + 200 mg and furosemide (20 mg were administered intravenously. BP suddenly dropped (90/60 mmHg following the removal of the mass, nitroglycerine, and nitroprusside infusions were stopped; remifentanil dose was decreased and fluid was quickly infused. The patient was uneventually recovered. Vanilmandelic acid level was higher in the patient and pheochromocytoma was considered.

Liver function assessment using 99mTc-GSA single-photon emission computed tomography (SPECT)/CT fusion imaging in hilar bile duct cancer: A retrospective study.

Science.gov (United States)

Sumiyoshi, Tatsuaki; Shima, Yasuo; Okabayashi, Takehiro; Kozuki, Akihito; Hata, Yasuhiro; Noda, Yoshihiro; Kouno, Michihiko; Miyagawa, Kazuyuki; Tokorodani, Ryotaro; Saisaka, Yuichi; Tokumaru, Teppei; Nakamura, Toshio; Morita, Sojiro

2016-07-01

The objective of this study was to determine the utility of Tc-99m-diethylenetriamine-penta-acetic acid-galactosyl human serum albumin ((99m)Tc-GSA) single-photon emission computed tomography (SPECT)/CT fusion imaging for posthepatectomy remnant liver function assessment in hilar bile duct cancer patients. Thirty hilar bile duct cancer patients who underwent major hepatectomy with extrahepatic bile duct resection were retrospectively analyzed. Indocyanine green plasma clearance rate (KICG) value and estimated KICG by (99m)Tc-GSA scintigraphy (KGSA) and volumetric and functional rates of future remnant liver by (99m)Tc-GSA SPECT/CT fusion imaging were used to evaluate preoperative whole liver function and posthepatectomy remnant liver function, respectively. Remnant (rem) KICG (= KICG × volumetric rate) and remKGSA (= KGSA × functional rate) were used to predict future remnant liver function; major hepatectomy was considered unsafe for values liver were significantly higher than volumetric rates (median: 0.54 vs 0.46; P liver failure and mortality did not occur in the patients for whom hepatectomy was considered unsafe based on remKICG. remKGSA showed a stronger correlation with postoperative prothrombin time activity than remKICG. (99m)Tc-GSA SPECT/CT fusion imaging enables accurate assessment of future remnant liver function and suitability for hepatectomy in hilar bile duct cancer patients. Copyright © 2016 Elsevier Inc. All rights reserved.

MR imaging and MR cholangiopancreatography in the preoperative evaluation of hilar cholangiocarcinoma: correlation with surgical and pathologic findings

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Masselli, Gabriele; Gualdi, Gianfranco [Umberto I Hospital-La Sapienza University Rome, Department of Radiology, Rome (Italy); Manfredi, Riccardo [University of Verona, Department of Radiology, Verona (Italy); Vecchioli, Amorino [A. Gemelli Hospital-University of Sacred Heart, Department of Radiology, Rome (Italy)

2008-10-15

The primary aim was to evaluate delayed contrast-enhanced MRI in depicting perineural spread of hilar cholangiocarcinoma (CCC) and consequently to determine the capability of MRI/MRCP for staging CCC. Fifteen patients that underwent MRI/MRCP and surgical treatment were retrospectively included. Two radiologists evaluated MR images to assess delayed periductal enhancement, extent of bile duct stenosis, liver parenchymal and vascular involvement and presence of liver atrophy. An agreement between delayed enhancement of the bile duct walls and perineural neoplastic spread showed a very good correlation factor (0.93). The overall accuracy in detecting biliary neoplastic invasion was higher for delayed T1-weighted images (93.3%) than for the MRCP images (80%), and T1-delayed image increased the MR accuracy in assessing the neoplastic resectability (p < 0.05). MRI correctly predicted vascular involvement in 73% and liver involvement in 80% of the cases. The number of overall correctly assessed patients with regard to resectability was 11 true positive, 1 false positive and 3 true negative. The combination of MRI/MRCP is a reliable diagnostic method for staging hilar cholangiocarcinomas. Delayed periductal enhancement is accurate in the evaluation of neoplastic perineural spread, and it can improve diagnostic accuracy to identify resectable and unresectable tumours. (orig.)

Cefaléia em pacientes com feocromocitoma: influência da hipertensão arterial Headache in patients with pheochromocytoma: influence of arterial hypertension

Directory of Open Access Journals (Sweden)

ELCIO JULIATO PIOVESAN

1998-06-01

Full Text Available A cefaléia no feocromocitoma é elemento diagnóstico importante, que pode caracterizar uma reação do organismo frente a oscilações hormonais patológicas. Após averiguação da hipertensão arterial e da cefaléia em 20 pacientes durante os períodos de descompensação do feocromocitoma, sugerimos que a hipertensão arterial isolada não é o único fator desencadeante dos fenômenos álgicos cranianos. A possibilidade das variações nas catecolaminas, adrenomedulina e outras substâncias neuroendócrinas deve ser melhor esclarecida para comprovar esta hipótese.Headache is an important diagnostic element in pheochromocytoma and it may characterize a body reaction to pathological hormonal oscillations. We observed the pheochromocytoma instability in 20 patients during arterial hypertension and tried to correlate with headache. We found that isolate hypertension is not the only factor in headache pathogenesis. It is possible that changes in catecholamines, adrenomedullin and other neuropeptides may cause some of these symptoms.

Robotic surgery twice performed in the treatment of hilar cholangiocarcinoma with deep jaundice: delayed right hemihepatectomy following the right-hepatic vascular control.

Science.gov (United States)

Zhu, Zhenyu; Liu, Quanda; Chen, Junzhou; Duan, Weihong; Dong, Maosheng; Mu, Peiyuan; Cheng, Di; Che, Honglei; Zhang, Tao; Xu, Xiaoya; Zhou, Ningxin

2014-10-01

To explore and find a new method to treat hilar cholangiocarcinoma with deep jaundice assisted by Da Vinci robot. A hilar cholangiocarcinoma patient of type Bismuch-Corlette IIIa was found with deep jaundice (total bilirubin: 635 µmol/L). On the first admission, we performed Da Vinci robotic surgery including drainage of left hepatic duct, dissection of right hepatic vessels (right portal vein and right hepatic artery), and placement of right-hepatic vascular control device. Three weeks later on the second admission when the jaundice disappeared we occluded right-hepatic vascular discontinuously for 6 days and then sustained later. On the third admission after 3 weeks of right-hepatic vascular control, the right hemihepatectomy was performed by Da Vinci robot for the second time. The future liver remnant after the right-hepatic vascular control increased from 35% to 47%. The volume of left lobe increased by 368 mL. When the total bilirubin and liver function were all normal, right hemihepatectomy was performed by Da Vinci robot 10 weeks after the first operation. The removal of atrophic right hepatic lobe with tumor in bile duct was found with no pathologic cancer remaining in the margin. The patient was followed up at our outpatient clinic every 3 months and no tumor recurrence occurs by now (1 y). Under the Da Vinci robotic surgical system, a programmed treatment can be achieved: first, the hepatic vessels were controlled gradually together with biliary drainage, which results in liver's partial atrophy and compensatory hypertrophy in the other part. Then a radical hepatectomy could be achieved. Such programmed hepatectomy provides a new treatment for patients of hilar cholangiocarcinoma with deep jaundice who have the possibility of radical heptolobectomy.

Feocromocitoma bilateral: la importancia de los estudios de diagnóstico por imagen Bilateral pheochromocytoma: the importance of imaging studies

Directory of Open Access Journals (Sweden)

Igor I Bonnet

2011-10-01

Full Text Available Se presenta un caso clínico poco frecuente de feocromocitoma bilateral, en el cual los estudios de diagnóstico por imagen, tanto de información estructural como funcional, constituyeron una fuente fundamental para su detección y seguimiento.We report a rare case of bilateral pheochromocytoma, in which imaging studies, both of structural and functional information, were a major source for its detection and follow-up.

Free‑floating cancer cells in lymph node sinuses of hilar lymph node‑positive patients with non‑small cell lung cancer.

Science.gov (United States)

Nakamura, Yusuke; Mukai, Masaya; Hiraiwa, Shinichiro; Kishima, Kyoko; Sugiyama, Tomoko; Tajiri, Takuma; Yamada, Shunsuke; Iwazaki, Masayuki

2018-05-14

Previous studies demonstrated that free‑floating cancer cells (FFCCs) in the lymph node sinuses were of prognostic significance for colorectal and gastric cancer. The present study investigated the clinical significance of detecting FFCCs using Fast Red staining for cytokeratin in stage I/II non‑small cell lung cancer (NSCLC) patients and hilar lymph node positive NSCLC patients who underwent curative resection. Between 2002 and 2011, a total of 164 patients (including 22 hilar lymph node positive patients) were investigated. Resected lymph nodes were stained for cytokeratin using an anti‑cytokeratin antibody. In order to achieve a clear distinction from coal dust, an anti‑cytokeratin antibody was labeled with a secondary antibody conjugated with alkaline phosphatase, which was detected by a reaction with Fast Red/naphthol that produced a red color. Patients were considered to be positive for FFCCs (FFCCs+) if one or more than one free‑floating cytokeratin‑positive cell was detected in the lymph node sinuses, which could not be detected by hematoxylin and eosin staining. Among all 164 patients, a significant difference was observed in 5‑year relapse‑free survival (5Y‑RFS) rates, with 76.9 and 33.3% being achieved by FFCCs‑ and FFCCs+ patients, respectively (Philar lymph node‑positive patients, a significant difference was also observed in 5Y‑RFS, with 53.8 and 0.0% being achieved by FFCCs‑ and FFCCs+ patients, respectively (P=0.006). The 5Y‑OS tended to be lower in FFCCs+ patients, with 69.2 and 53.3% being achieved by FFCCs‑ and FFCCs+ patients, respectively (P=0.463). The findings of the present study suggested the presence of FFCCs in stage I/II NSCLC patients was associated with a poor prognosis. In addition, FFCCs in hilar lymph node‑positive patients may potential be a useful marker in foreseeing the recurrence of cancer.

Ectopic Expression of α6 and δ GABAA Receptor Subunits in Hilar Somatostatin Neurons Increases Tonic Inhibition and Alters Network Activity in the Dentate Gyrus

Science.gov (United States)

Tong, Xiaoping; Peng, Zechun; Zhang, Nianhui; Cetina, Yliana; Huang, Christine S.; Wallner, Martin; Otis, Thomas S.

2015-01-01

The role of GABAA receptor (GABAAR)-mediated tonic inhibition in interneurons remains unclear and may vary among subgroups. Somatostatin (SOM) interneurons in the hilus of the dentate gyrus show negligible expression of nonsynaptic GABAAR subunits and very low tonic inhibition. To determine the effects of ectopic expression of tonic GABAAR subtypes in these neurons, Cre-dependent viral vectors were used to express GFP-tagged GABAAR subunits (α6 and δ) selectively in hilar SOM neurons in SOM-Cre mice. In single-transfected animals, immunohistochemistry demonstrated strong expression of either the α6 or δ subunit; in cotransfected animals, both subunits were consistently expressed in the same neurons. Electrophysiology revealed a robust increase of tonic current, with progressively larger increases following transfection of δ, α6, and α6/δ subunits, respectively, indicating formation of functional receptors in all conditions and likely coassembly of the subunits in the same receptor following cotransfection. An in vitro model of repetitive bursting was used to determine the effects of increased tonic inhibition in hilar SOM interneurons on circuit activity in the dentate gyrus. Upon cotransfection, the frequency of GABAAR-mediated bursting in granule cells was reduced, consistent with a reduction in synchronous firing among hilar SOM interneurons. Moreover, in vivo studies of Fos expression demonstrated reduced activation of α6/δ-cotransfected neurons following acute seizure induction by pentylenetetrazole. The findings demonstrate that increasing tonic inhibition in hilar SOM interneurons can alter dentate gyrus circuit activity during strong stimulation and suggest that tonic inhibition of interneurons could play a role in regulating excessive synchrony within the network. SIGNIFICANCE STATEMENT In contrast to many hippocampal interneurons, somatostatin (SOM) neurons in the hilus of the dentate gyrus have very low levels of nonsynaptic GABAARs and exhibit

Fluorodeoxyglucose positron emission tomography-computed tomography scan in von Hippel-Lindau syndrome: a case report and review of literature

International Nuclear Information System (INIS)

Solav, Shrikant; Bhandari, Ritu

2012-01-01

Von Hippel-Lindau (VHL) syndrome is a hereditary autosomal dominant disorder caused by defective tumor suppression gene at 3p25-p26. The gene for VHL disease is found on chromosome 3, and is inherited in a dominant fashion. The VHL gene is a tumor suppressor gene. This means that its role in a normal cell is to stop the uncontrolled growth and proliferation. It is characterized by abnormal growth of blood vessels. It strikes the eyes, central nervous system, kidneys, endocrine glands, etc. It predisposes the patient to retinal angiomas, central nervous system hemangioblastoma, renal cell carcinoma (RCC), pheochromocytomas, islet cell tumor of the pancreas, endolymphatic sac tumors, renal, pancreatic, epididymal cysts. We present a case of familial VHL syndrome whose Fluorine 18-fluorodeoxyglucose positron emission tomography-computed tomography scan was truly positive for adrenal pheochromocytoma but was falsely negative for RCC. Review of literature related to this entity is made. (author)

Accuracy of recommended sampling and assay methods for the determination of plasma-free and urinary fractionated metanephrines in the diagnosis of pheochromocytoma and paraganglioma: a systematic review.

Science.gov (United States)

Därr, Roland; Kuhn, Matthias; Bode, Christoph; Bornstein, Stefan R; Pacak, Karel; Lenders, Jacques W M; Eisenhofer, Graeme

2017-06-01

To determine the accuracy of biochemical tests for the diagnosis of pheochromocytoma and paraganglioma. A search of the PubMed database was conducted for English-language articles published between October 1958 and December 2016 on the biochemical diagnosis of pheochromocytoma and paraganglioma using immunoassay methods or high-performance liquid chromatography with coulometric/electrochemical or tandem mass spectrometric detection for measurement of fractionated metanephrines in 24-h urine collections or plasma-free metanephrines obtained under seated or supine blood sampling conditions. Application of the Standards for Reporting of Diagnostic Studies Accuracy Group criteria yielded 23 suitable articles. Summary receiver operating characteristic analysis revealed sensitivities/specificities of 94/93% and 91/93% for measurement of plasma-free metanephrines and urinary fractionated metanephrines using high-performance liquid chromatography or immunoassay methods, respectively. Partial areas under the curve were 0.947 vs. 0.911. Irrespective of the analytical method, sensitivity was significantly higher for supine compared with seated sampling, 95 vs. 89% (p sampling compared with 24-h urine, 95 vs. 90% (p sampling, seated sampling, and urine. Test accuracy increased linearly from 90 to 93% for 24-h urine at prevalence rates of 0.0-1.0, decreased linearly from 94 to 89% for seated sampling and was constant at 95% for supine conditions. Current tests for the biochemical diagnosis of pheochromocytoma and paraganglioma show excellent diagnostic accuracy. Supine sampling conditions and measurement of plasma-free metanephrines using high-performance liquid chromatography with coulometric/electrochemical or tandem mass spectrometric detection provides the highest accuracy at all prevalence rates.

Complicated Horseshoe Kidney

Energy Technology Data Exchange (ETDEWEB)

Kim, K. S.; Kim, S. R.; Cha, K. S.; Park, S. S. [Chung Ang University College of Medicine, Seoul (Korea, Republic of)

2010-05-15

Horseshoe kidney is an important urological anomaly when it is complicated or accompanied by other diseases. Recently we have experienced four cases of horseshoe kidney which were complicated with hydronephrosis, renal stone and adrenal pheochromocytoma. With review of literatures, we emphasize the importance of detection of these complications.

Complicated Horseshoe Kidney

International Nuclear Information System (INIS)

Kim, K. S.; Kim, S. R.; Cha, K. S.; Park, S. S.

2010-01-01

Horseshoe kidney is an important urological anomaly when it is complicated or accompanied by other diseases. Recently we have experienced four cases of horseshoe kidney which were complicated with hydronephrosis, renal stone and adrenal pheochromocytoma. With review of literatures, we emphasize the importance of detection of these complications.

Hilar mossy cells of the dentate gyrus: a historical perspective

Directory of Open Access Journals (Sweden)

Helen E Scharfman

2013-01-01

Full Text Available The circuitry of the dentate gyrus of the hippocampus is unique compared to other hippocampal subfields because there are two glutamatergic principal cells instead of one: granule cells, which are the vast majority of the cells in the dentate gyrus, and the so-called ‘mossy cells.’ The distinctive appearance of mossy cells, the extensive divergence of their axons, and their vulnerability to excitotoxicity relative to granule cells has led to a great deal of interest in mossy cells. Nevertheless, there is no consensus about the normal functions of mossy cells and the implications of their vulnerability. There even seems to be some ambiguity about exactly what mossy cells are. Here we review initial studies of mossy cells, characteristics that define them, and suggest a practical definition to allow investigators to distinguish mossy cells from other hilar neurons even if all morphological and physiological information is unavailable due to technical limitations of their experiments. In addition, hypotheses are discussed about the role of mossy cells in the dentate gyrus network, reasons for their vulnerability and their implications for disease.

Hilar mossy cells of the dentate gyrus: a historical perspective

Science.gov (United States)

Scharfman, Helen E.; Myers, Catherine E.

2013-01-01

The circuitry of the dentate gyrus (DG) of the hippocampus is unique compared to other hippocampal subfields because there are two glutamatergic principal cells instead of one: granule cells, which are the vast majority of the cells in the DG, and the so-called “mossy cells.” The distinctive appearance of mossy cells, the extensive divergence of their axons, and their vulnerability to excitotoxicity relative to granule cells has led to a great deal of interest in mossy cells. Nevertheless, there is no consensus about the normal functions of mossy cells and the implications of their vulnerability. There even seems to be some ambiguity about exactly what mossy cells are. Here we review initial studies of mossy cells, characteristics that define them, and suggest a practical definition to allow investigators to distinguish mossy cells from other hilar neurons even if all morphological and physiological information is unavailable due to technical limitations of their experiments. In addition, hypotheses are discussed about the role of mossy cells in the DG network, reasons for their vulnerability and their implications for disease. PMID:23420672

sup(99m)Tc-DMSA renal scintigraphy in renal failure due to various renal diseases

Energy Technology Data Exchange (ETDEWEB)

Hosokawa, S; Daijo, K; Okabe, T; Kawamura, J; Hara, A [Kyoto Univ. (Japan). Hospital

1979-08-01

Renal contours in renal failure were studied by means of sup(99m)Tc-dimercaptosuccinic acid (DMSA) renoscintigraphy. Renal cortical images were obtained even in renal failure cases. Causes of renal failure were chronic glomerulonephritis in 7, bilateral renal tuberculosis in 2, chronic pyelonephritis in 3, bilateral renal calculi in 3, diabetic nephropathy in 2, polycystic kidney disease in 2 and stomach cancer in 1.

sup(99m)Tc-DMSA renal scintigraphy in renal failure due to various renal diseases

International Nuclear Information System (INIS)

Hosokawa, Shin-ichi; Daijo, Kazuyuki; Okabe, Tatsushiro; Kawamura, Juichi; Hara, Akira

1979-01-01

Renal contours in renal failure were studied by means of sup(99m)Tc-dimercaptosuccinic acid (DMSA) renoscintigraphy. Renal cortical images were obtained even in renal failure cases. Causes of renal failure were chronic glomerulonephritis in 7, bilateral renal tuberculosis in 2, chronic pyelonephritis in 3, bilateral renal calculi in 3, diabetic nephropathy in 2, polycystic kidney disease in 2 and stomach cancer in 1. (author)

Renal computed angiography. Part I: Renal CT phlebography. Renal veins variants

International Nuclear Information System (INIS)

Al-Amin, M.; Krupev, M.; Hadjidekov, V.; Plachkov, I.

2012-01-01

The changing trend in renal surgery, transplantation and minimal invasive urology implies preprocedure evaluation of renal veins. Development of imaging methods offers new possibilities for venographic visualization. The goal of this study is to present authors experience in visualization of renal veins using 64 MDCT and to evaluate the utility in assessments of their variants. 128 patients (68 females and 60 males, mean age 53,3) with urological complaints underwent 64MDCT examination including CT angiography. Contrast enhancement includes 3-4ml/sec injection flow of 90 ml contrast medium followed by 20 ml saline at the same rate. In 23 out of 128 examined patients some of the common variants of the renal vein is found. 64 MDCT angiography visualize very well renal veins and becomes method of choice in preoperative assessment of renal vein anatomy. (authors)

The roles of the liver and pancreas in renal nutcracker syndrome

Energy Technology Data Exchange (ETDEWEB)

Yun, Seong Jong, E-mail: zoomknight@naver.com [Department of Radiology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, 149 Sangil-dong, Kangdong-gu, Seoul 134-727 (Korea, Republic of); Department of Radiology, Graduate School of Medicine, Kyung Hee University, Hoegi-dong, Dongdaemun-gu, Seoul 130-701 (Korea, Republic of); Nam, Deok Ho, E-mail: namjindan@daum.net [Department of Radiology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, 149 Sangil-dong, Kangdong-gu, Seoul 134-727 (Korea, Republic of); Ryu, Jung Kyu, E-mail: oddie2@naver.com [Department of Radiology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, 149 Sangil-dong, Kangdong-gu, Seoul 134-727 (Korea, Republic of); Kim, Ji Su, E-mail: js830808@hanmail.net [Department of Radiology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, 149 Sangil-dong, Kangdong-gu, Seoul 134-727 (Korea, Republic of)

2014-10-15

Graphical abstract: - Highlights: • The presence of the liver and pancreas may influence NCS by compressing SMA against the aorta. • The presence of the liver and pancreas at the level of the LRV is not yet recognized as an independent factor for NCS but should be. • The presence of the liver and the pancreas may allow clinicians to identify NCS patients and may influence the choice of treatment options. - Abstract: Introduction: To assess the frequency and significance of presence of the liver and pancreas at the left renal vein (LRV) level in patients with suspected renal nutcracker syndrome (NCS). Materials and methods: We included 101 patients with hematuria who underwent urography three-dimensional CT between April 2009 and November 2013. These patients were divided into NCS (n = 25) and non-NCS (n = 76) patients according to the following CT criteria: (1) the presence of beak sign and (2) hilar-aortomesenteric left renal vein diameter ratio >4. Patients were grouped according to the presence of the liver and pancreas at the LRV: group LP (both liver and pancreas), group L (only liver), group P (only pancreas), and group O (neither liver nor pancreas). The difference in the frequencies of groups was analyzed between NCS and non-NCS patients. Multivariate analysis was used to determine the independent factors between NCS and non-NCS patients. Results: The frequencies of group LP, group L, group P, and group O in NCS vs. non-NCS were 88% vs. 5.3% (p < 0.001), 4.0% vs. 2.6% (p = 0.75), 4.0% vs. 11.8% (p = 0.45), 4.0% vs. 80.3% (p < 0.001), respectively. Multivariate analysis demonstrated that group was a predictor for differential diagnosis between NCS and non-NCS (p = 0.022), and group LP was an independent factor for the presence of NCS (odds ratio, 43.8; 95% confidence interval, 3.8–500.3; p < 0.002; reference, group O). Conclusion: The presence of the liver and pancreas at the level of the LRV was frequently found in NCS and was the independent factor

The roles of the liver and pancreas in renal nutcracker syndrome

International Nuclear Information System (INIS)

Yun, Seong Jong; Nam, Deok Ho; Ryu, Jung Kyu; Kim, Ji Su

2014-01-01

Graphical abstract: - Highlights: • The presence of the liver and pancreas may influence NCS by compressing SMA against the aorta. • The presence of the liver and pancreas at the level of the LRV is not yet recognized as an independent factor for NCS but should be. • The presence of the liver and the pancreas may allow clinicians to identify NCS patients and may influence the choice of treatment options. - Abstract: Introduction: To assess the frequency and significance of presence of the liver and pancreas at the left renal vein (LRV) level in patients with suspected renal nutcracker syndrome (NCS). Materials and methods: We included 101 patients with hematuria who underwent urography three-dimensional CT between April 2009 and November 2013. These patients were divided into NCS (n = 25) and non-NCS (n = 76) patients according to the following CT criteria: (1) the presence of beak sign and (2) hilar-aortomesenteric left renal vein diameter ratio >4. Patients were grouped according to the presence of the liver and pancreas at the LRV: group LP (both liver and pancreas), group L (only liver), group P (only pancreas), and group O (neither liver nor pancreas). The difference in the frequencies of groups was analyzed between NCS and non-NCS patients. Multivariate analysis was used to determine the independent factors between NCS and non-NCS patients. Results: The frequencies of group LP, group L, group P, and group O in NCS vs. non-NCS were 88% vs. 5.3% (p < 0.001), 4.0% vs. 2.6% (p = 0.75), 4.0% vs. 11.8% (p = 0.45), 4.0% vs. 80.3% (p < 0.001), respectively. Multivariate analysis demonstrated that group was a predictor for differential diagnosis between NCS and non-NCS (p = 0.022), and group LP was an independent factor for the presence of NCS (odds ratio, 43.8; 95% confidence interval, 3.8–500.3; p < 0.002; reference, group O). Conclusion: The presence of the liver and pancreas at the level of the LRV was frequently found in NCS and was the independent factor

Angiographic Assessment of the Right Hepatic Artery for Encasement by Hilar Cholangiocarcinoma: Comparison Between Antero-Posterior and Right Anterior Oblique Projections

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Furukawa, Hiroyoshi; Iwata, Ryoko; Moriyama, Noriyuki

2001-01-01

Purpose: To evaluate the usefulness of right anterior oblique (RAO) arteriography for evaluating encasement of the right hepatic artery (RHA) by hilar cholangiocarcinoma.Methods: Celiac arteriography was performed in both the antero-posterior (AP) and RAO projection in ten patients with cholangiocarcinoma. The lengths of the arteries between the bifurcation of the anterior and posterior branch of the liver and the following points were measured: (a) the bifurcation of the left and right hepatic artery (AP-LR), (b) the bifurcation of the proper hepatic artery and the gastroduodenal artery (AP-PG). Additionally, image quality in investigating the invasion of the RHA was evaluated.Results: On the AP images, the average lengths of AP-LR and AP-PG were 24.5 ± 5.1 mm and 30.0 ± 4.9 mm, respectively. On RAO images, the lengths were 28.2 ± 4.6 mm and 32.7 ± 4.8 mm, respectively. Every length was different between the two projections (p < 0.01). In 6 of 10 patients with hilar cholangiocarcinoma, images in RAO projections were superior to AP images for evaluation of encasement.Conclusion: We conclude that angiography obtained in the RAO projection yields images that are superior to those obtained in the conventional AP projection for assessment of RHA encasement

Imaging appearances of von Hippel-Lindau disease

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Ma Xiaolong; Wang Jianhua; Lu Jianping; Wang Li; Liu Qi; Jiang Hui; Wei Wei

2013-01-01

Objective: To assess imaging appearances of von Hippel-Lindau disease (VHLD). Methods: The clinical and imaging data of ten patients who met VHLD gene diagnostic criteria were retrospectively analyzed. Seven underwent CT abdominal scan, three underwent MRI abdominal scan. All patients underwent MRI cranial scan. Eight patients were treated by surgery, and three of them underwent endoscopic ultrasound guided aspiration biopsy of pancreatic tumors. Results: All patients had multiple cysts of the pancreas, eight patients suffered multiple cysts of kidneys. Three patients suffered multiple islet cell tumors of pancreas: one was accompanied with pancreatic serous cystadenoma, another was accompanied with left adrenal pheochromocytoma. Three patients had bilateral renal cell carcinoma (one of them had the right kidney cut off), one patient had right renal cell carcinoma. Two patients suffered cerebellar hemangioblastomas, and one of them was accompanied with mutiple spinal hemangioblastomas and the left retinal hemangioblastoma. Two patients with multiple cysts of pancreas and kidneys had not been found any tumor. Conclusion: Multiple cysts of pancreas and kidney are highly suggestive of VHLD. At the same time, endocrine tumors of pancreas, serous cystadenoma of pancreas, adrenal pheochromocytoma, renal carcinomas, multiple hemangioblastomas of central nervous system and retinal hemangioblastoma can also occur in VHLD. (authors)

Effects of Aroclor 1254 on dopamine and norepinephrine concentrations in pheochromocytoma (PC-12) cells

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Seegal, R.F.; Brosch, K.; Bush, B.; Ritz, M.; Shain, W.

1990-01-01

Pheochromocytoma (PC-12) cells synthesize, store, release and metabolize dopamine (DA) and norepinephrine (NE) in a manner analogous to that observed in the mammalian central nervous system. These cells were used to develop and validate an alternate method to animal testing to assess the effects of a complex environmental mixture of polychlorinated biphenyls (Aroclor 1254) on cellular catecholamine function. Aroclor 1254, at concentrations of 1 to 100 ppm, significantly decreased cellular catecholamine concentrations after 6 hrs. Exposure at 100 ppm for periods of less than an hr increased cellular catecholamine concentrations while longer exposure times (i.e., 1 to 24 hr) decreased cellular catecholamine concentrations. This in vitro depletion of catecholamines is similar to that seen in vivo. Thus, PC-12 cells may be useful for neurochemical evaluation of neurotoxicants with particular reference to effects on catecholaminergic systems

[Heredity in renal and prostatic neoplasia].

Science.gov (United States)

Prayer Galetti, T; D'Arrigo, L; De Zorzi, L; Patarnello, T

1997-09-01

There is an ever growing report of data supporting the evidence that accumulated genetic changes underlie the development of neoplasia. The paradigma of this multistep process is colon cancer were cancer onset is associated, over decades, with at least seven genetic events. The number of genetic alterations increases moving from adenomatous lesions to colon cancer and, although the genetic alterations occur according to a preferred sequence, the total accumulation of changes rather than their sequential order is responsible of tumor biological behavior. It is noteworthy that, at least for this neoplasia, carcinogenesis appears to arise as a result of the mutational activation of oncogenes coupled with the mutational inactivation of tumor suppressor genes. In some cases mutant suppressor genes appear to exert a phenotypic effect even when present in the heterozygous state thus been non "recessive" at the cellular level. The general features of this model may apply also to renal cell cancer (RCC) and prostate cancer (CaP). Extensive literature exists on the cytogenetic and molecular findings in RCC. Only 2% of RCC are familiar, but molecular genetic studies of these cancers have provided important informations on RCC pathogenesis. As with other cancers, familiar RCC is characterized by an early age of onset and frequent multicentricity. A pathological classification useful in studying these patients subdivide renal cancers in papillary (pRCC) and non papillary (RCC) neoplasms. The most common cause of inherited RCC is the Von Hippel Lindau disease (VHL) a dominantly inherited multisystem disorder characterized by retinal and cerebellar hemangioblastomas, pheochromocytomas, pancreatic cysts and RCC. Over 70% of these patients will develop an RCC by their sixth decade. In 1993 the isolation of the tumor suppressor gene in VHL disease at the level of chromosome 3p25-p26 have lead to a better understanding of RCC. Most missense mutations are associated with high risk of

Continuous renal replacement therapy improves renal recovery from acute renal failure.

Science.gov (United States)

Jacka, Michael J; Ivancinova, Xenia; Gibney, R T Noel

2005-03-01

Acute renal failure (ARF) occurs in up to 10% of critically ill patients, with significant associated morbidity and mortality. The optimal mode of renal replacement therapy (RRT) remains controversial. This retrospective study compared continuous renal replacement therapy (CRRT) and intermittent hemodialysis (IHD) for RRT in terms of intensive care unit (ICU) and hospital mortality, and renal recovery. We reviewed the records of all patients undergoing RRT for the treatment of ARF over a 12-month period. Patients were compared according to mode of RRT, demographics, physiologic characteristics, and outcomes of ICU and hospital mortality and renal recovery using the Chi square, Student's t test, and multiple logistic regression as appropriate. 116 patients with renal insufficiency underwent RRT during the study period. Of these, 93 had ARF. The severity of illness of CRRT patients was similar to that of IHD patients using APACHE II (25.1 vs 23.5, P = 0.37), but they required significantly more intensive nursing (therapeutic intervention scale 47.8 vs 37.6, P = 0.0001). Mortality was associated with lower pH at presentation (P = 0.003) and increasing age (P = 0.03). Renal recovery was significantly more frequent among patients initially treated with CRRT (21/24 vs 5/14, P = 0.0003). Further investigation to define optimal timing, dose, and duration of RRT may be beneficial. Although further study is needed, this study suggests that renal recovery may be better after CRRT than IHD for ARF. Mortality was not affected significantly by RRT mode.

Diseases of the abdomen including the pelvis

International Nuclear Information System (INIS)

Kido, C.; Tanaka, H.

1983-01-01

This book discusses the following diseases: fatty liver; cystic disease of the liver; liver abscess; liver cirrhosis; hepatic hemangioma; cholelithiasis; primary liver cancer; cholangioma; cancer of the common bile duct; pancreatic cyst; pancreatic calculi; chronic pancreatitis; pancreatic pseudocyst; chronic pancreatitis: pancreatic fatty degeneration; cancer of the pancreas; nonfunctioning kidney: chalk kidney; polycystic kidney; perirenal calcified abscess; renal infarct; cancer of the renal pelvis; adrenal pheochromocytoma; adenoma of the adrenal cortex; leiomyosarcoma of the stomach; malignant mesothelioma; intraperitoneal abscess; perityphlic abscess; retroperitoneal reticulum cell sarcoma; and retroperitoneal cyst

Renal cell carcinoma in patient with crossed fused renal ectopia

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Ozgur Cakmak

2016-01-01

Full Text Available Primary renal cell carcinomas have rarely been reported in patients with crossed fused renal ectopia. We presented a patient with right to left crossed fused kidney harbouring renal tumor. The most frequent tumor encountered in crossed fused renal ectopia is renal cell carcinoma. In this case, partial nephrectomy was performed which pave way to preservation of the uninvolved both renal units. Due to unpredictable anatomy, careful preoperative planning and meticulous delineation of renal vasculature is essential for preservation of the uninvolved renal units.

Radioimmunoassay in the diagnosis of arterial hypertension

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Slavnov, V N; Olejnik, V A; Yakovlev, A A; Yugrinov, O G; Markov, V V [Kievskij Nauchno-Issledovatel' skij Inst. Ehndokrinologii i Obmena Veshchestv (Ukrainian SSR)

1984-11-01

The paper is concerned with the results of a study of the aldosterone concentration and renin activity, the general level of catecholamines and their fractions in the peripheral blood and blood taken at selective venography from the vena cava inferior, renal and adrenal veins of 108 patients with hypertension, aldosteroma and idiopathic hyperaldosteronism, adrenal and extraadrenal pheochromocytoma, renovascular and renoparenchymatous arterial hypertension. The aldosterone concentration and renin activity were determined with radioimmunoassay, and the general content of catecholamines and their fractions with a radioenzymatic method using standard kits. It has been shown that the radioimmunoassay to determine the aldosterone concentration and renin activity makes possible differential diagnosis of hypertension, aldosteroma, idiopathic and secondary hyperaldosteronism. A considerable increase in the blood plasma renin activity on the affected side was revealed in the patients with renovascular hypertension, and in renoparenchymatous hypertension it was equal in both renal veins. The study of the total content of catecholamines and their fractions in the blood from different parts of the venous system can be used for topical diagnosis of adrenal and extraadrenal pheochromocytoma.

Radioimmunoassay in the diagnosis of arterial hypertension

International Nuclear Information System (INIS)

Slavnov, V.N.; Olejnik, V.A.; Yakovlev, A.A.; Yugrinov, O.G.; Markov, V.V.

1984-01-01

The paper is concerned with the results of a study of the aldosterone concentration and renin activity, the general level of catecholamines and their fractions in the peripheral blood and blood taken at selective venography from the vena cava inferior, renal and adrenal veins of 108 patients with hypertension, aldosteroma and idiopathic hyperaldosteronism, adrenal and extraadrenal pheochromocytoma, renovascular and renoparenchymatous arterial hypertension. The aldosterone concentration and renin activity were determined with radioimmunoassay, and the general content of catecholamines and their fractions with a radioenzymatic method using standard kits. It has been shown that the radioimmunoassay to determine the aldosterone concentration and renin activity makes it possible to resort to differential diagnosis of hypertension, aldosteroma, idiopathic and secondary hyperaldosteronism. A considerable increase in the blood plasma renin activity on the affected side was revealed in the patients with renovascular hypertension, and in renoparenchymatous hypertension it was equal in both renal veins. The study of the total content of catecholamines and their fractions in the blood from different parts of the venous system can be used for topical diagnosis of adrenal and extraadrenal pheochromocytoma

Zero ischemia laparoscopic partial thulium laser nephrectomy.

LENUS (Irish Health Repository)

Thomas, Arun Z

2013-11-01

Laser technology presents a promising alternative to achieve tumor excision and renal hemostasis with or without hilar occlusion, yet its use in partial nephrectomy has not been significantly evaluated. We prospectively evaluated the thulium:yttrium-aluminum-garnet laser in laparoscopic partial nephrectomy (LPN) in our institution over a 1-year period.

Incidentally Detected Inoperable Malignant Pheochromocytoma with Hepatic Metastasis Treated by Transcatheter Arterial Chemoembolization

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Joong Keun Kim

2014-12-01

Full Text Available Malignant pheochromocytoma (PCC is a rare condition. Although the liver is the second most frequent site of metastasis in malignant PCC, no definite treatments have been established. Herein, we report a case of liver metastasis of PCC that was successfully treated by transcatheter arterial chemoembolization (TACE. A 69-year-old man was admitted to the Department of Gastroenterology for evaluation of an incidental hepatic mass in August 2013. He had undergone right adrenalectomy in May 2005 and PCC had been confirmed on the basis of histopathological findings. Liver biopsy was performed, and metastatic PCC was diagnosed. The lesion appeared inoperable because of invasion of the portal vein and metastases in the lymph nodes along the hepatoduodenal ligament. Thus, TACE was performed instead. After TACE, symptoms including dizziness and cold sweating improved, and the patient's serum catecholamine levels decreased. On the basis of this case, we believe that TACE may be a useful treatment for liver metastasis in malignant PCC.

Do We Need to Clamp the Renal Hilum Liberally during the Initial Phase of the Learning Curve of Robot-Assisted Nephron-Sparing Surgery?

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Ömer Acar

2014-01-01

Full Text Available Objective. We aimed to compare the results of our initial robot-assisted nephron-sparing surgeries (RANSS performed with or without hilar clamping. Material and Method. Charts of the initial RANSSs (n=44, which were performed by a single surgeon, were retrospectively reviewed. R.E.N.A.L. nephrometry system, modified Clavien classification, and M.D.R.D. equation were used to record tumoral complexity, complications, and estimated glomerular filtration rate (eGFR, respectively. Outcomes of the clamped (group 1, n=14 versus off-clamp (group 2, n=30 RANSSs were compared. Results. The difference between the two groups was insignificant regarding mean patient age, mean tumor size, and mean R.E.N.A.L. nephrometry score. Mean operative time, mean estimated blood loss amount, and mean length of hospitalization were similar between groups. A total of 4 patients in each group suffered 11 Clavien grade ≥2 complications early postoperatively. Open conversion rates were similar. The difference between the 2 groups in terms of the mean postoperative change in eGFR was insignificant. We did not encounter any local recurrence after a mean follow-up of 18.9 months. Conclusions. Creating warm-ischemic conditions during RANSS should not be a liberal decision, even in the initial phases of the learning curve for a highly experienced open surgeon.

Chronic renal failure due to unilateral renal agenesis and total renal dysplasia (=aplasia)

International Nuclear Information System (INIS)

Kroepelin, T.; Ziupa, J.; Wimmer, B.

1983-01-01

Three adult patients with unilateral renal agenesis/total dysplasia (= aplasia) and with an early chronic renal failure are presented. One patient had renal agenesis without ureter bud and ureteric ostium on one side, and reflux pyelonephritis on the other; one had small compact total renal dysplasia (= aplasia) on one side, while chronic uric acid nephropathy (chronic renal disease as a cause of gout) was diagnosed on the other; the third patient had a total large multicystic dysplasia on one side, and on the other a segmental large multicystic dysplasia. Radiological steps and radiodiagnostic criteria are discussed and the combination of urogenital and extraurogenital anomalies is referred to. (orig.)

Efficacy of ultrasonography-guided renal biopsy for the evaluation of renal dysfunction following renal transplantation

International Nuclear Information System (INIS)

Kim, Young Jae; Choi, Chul Soon; Min, Seon Jeong; Lee, Gyung Kyu; Lee, Eil Seong; Kang, Ik Won; Bae, Sang Hoon

2003-01-01

To evaluate the usefulness and complications of renal biopsy under ultrasonography-guidance in renal dysfunction after renal transplantation. Ultrasonography-guided renal biopsy was done in 47 patients with the transplanted kidney. The subjects consisted of 30 males and 17 females, age ranged from 16 to 66 years (average age=38 years). Biopsies were done once in 27 patients, twice in 17 patients, three times in 3 patients, a total of 70 biopsies. The success rate of renal biopsy for the accurate pathologic diagnosis and the incidence and types of complications following biopsy were evaluated. The success rate of renal biopsy for the accurate pathologic diagnosis was 96%(67/70). Pathologic diagnosis included 27 cases of acute rejection (39%), 8 cases of acute tubular necrosis (11%), 4 cases of acute rejection and acute tubular necrosis (6%), 4 cases of cyclosporin toxicity (6%), 4 cases of primary disease recurrence (6%), 4 cases of infection (6%) and others. Complications after renal biopsy included 15 cases of microscopic hematuria (21%), 1 case of gross hematuria with spontaneous cessation and 1 case of life threatening hemorrhage. Ultrasonography-guided renal biopsy is a safe and effective diagnostic method for the evaluation of renal dysfunction following renal transplantation.

The contribution of cell blocks in the diagnosis of mediastinal masses and hilar adenopathy samples from echobronchoscopy.

Science.gov (United States)

Lourido-Cebreiro, Tamara; Leiro-Fernández, Virginia; Tardio-Baiges, Antoni; Botana-Rial, Maribel; Núñez-Delgado, Manuel; Álvarez-Martín, M Jesús; Fernández-Villar, Alberto

2014-07-01

Cell block material from puncture can be obtained with endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) in many cases. The aim of this study was to analyse the value of additional information from cell blocks obtained with EBUS-TBNA samples from mediastinal and hilar lymph nodes and masses. Review of pathology reports with a specific diagnosis obtained from EBUS-TBNA samples of mediastinal or hilar lesions, prospectively obtained over a two-year period. The generation of cell blocks from cytology needle samples, the contribution to morphological diagnosis, and the possible use of samples for immunohistochemistry were analysed. One hundred and twenty-nine samples corresponding to 110 patients were reviewed. The diagnosis was lung cancer in 81% of cases, extrapulmonary carcinoma in 10%, sarcoidosis in 4%, lymphoma in 2.7%, and tuberculosis in 0.9%. Cell blocks could be obtained in 72% of cases. Immunohistochemistry studies on the cell blocks were significantly easier to perform than on conventional smears (52.6% vs. 14%, P<.0001). In 4cases, the cell block provided an exclusive morphological diagnosis (3sarcoidosis and one metastasis from prostatic carcinoma) and in 3carcinomas, subtype and origin could be identified. Exclusive diagnoses from the cell block were significantly more frequent in benign disease than in malignant disease (25% vs 0.9%, P=.002). Cell blocks were obtained from 72% of EBUS-TBNA diagnostic procedures. The main contributions of cell blocks to pathology examinations were the possibility of carrying out immunohistochemical staining for the better classification of neoplasms, especially extrapulmonary metastatic tumours, and the improved diagnosis of benign lesions. Copyright © 2013 SEPAR. Published by Elsevier Espana. All rights reserved.

Rapid clearance of iodine-131 MIBG from the heart and liver of patients with adrenergic dysfunction and pheochromocytoma

International Nuclear Information System (INIS)

Nakajo, M.; Shimabukuro, K.; Miyaji, N.; Shimada, J.; Shirono, K.; Sakata, H.; Yoshimura, H.; Yonekura, R.; Shinohara, S.

1985-01-01

Iodine-131 MIBG, a radiolabeled adrenergic neuron-blocking agent, decreased rapidly from the heart and liver of patients with adrenergic dysfunction and pheochromocytoma when compared with eight controls. However, there was no significant difference in the rate of [ 131 I]MIBG decrease in these organs between controls and patients in the intervals subsequent to 4 hr. These findings suggest that adrenergic neuronal uptake of [ 131 I]MIBG in these organs is smaller in the patients than in the controls. Measurements of time-activity relationships of radioiodinated MIBG may be useful for assessment of adrenergic function of these organs and thus of generalized disorders of adrenergic innervation

“Transcollateral” Renal Angioplasty for a Completely Occluded Renal Artery

International Nuclear Information System (INIS)

Chandra, Subash; Chadha, Davinder S.; Swamy, Ajay

2011-01-01

Percutaneous transluminal renal angioplasty with stenting has been effective in the control of hypertension, renal function, and pulmonary edema caused by atherosclerotic renal artery stenosis. However, the role of the procedure has not been fully established in the context of chronic total occlusion of renal artery. We report the successful use of this procedure in 57-year-old male patient who reported for evaluation of a recent episode of accelerated hypertension. A renal angiogram in this patient showed ostial stenosis of the right renal artery, which was filling by way of the collateral artery. Renal angioplasty for chronic total occlusion of right renal artery was successfully performed in a retrograde fashion through a collateral artery, thereby leading to improvement of renal function and blood pressure control.

A case of multiple extra-adrenal pheochromocytoma diagnosed by [131I] meta-iodobenzylguanidine ([131I] MIGB) scintigraphy and nuclear magnetic resonance (NMR)

International Nuclear Information System (INIS)

Sasaki, Junko; Yamada, Hironori; Fujisawa, Takashi

1986-01-01