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Sample records for sclerosing epithelioid fibrosarcoma

  1. [Sclerosing epithelioid fibrosarcoma of the paravertebral column. Case report and literature review].

    Science.gov (United States)

    Puerta Roldán, Patricia; Rodríguez Rodríguez, Rodrigo; Bagué Rossell, Silvia; de Juan Delago, Manel; Molet Teixidó, Joan

    2013-01-01

    Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of low-grade fibrosarcoma, with specific histological and immunohistochemical features and a poor prognosis. We report a case of SEF of the paravertebral column in a 49-year old male who presented a paraspinal mass with extension into the L4-L5 neural foramen and invasion of the L5 nerve root. Histology of the tumourectomy specimen and its immunohistochemical study led to the diagnosis of SEF. This case was particularly unusual due to its paravertebral column location and, despite its low grade, illustrates the malignant potential of SEF. Copyright © 2012 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

  2. Sclerosing Epithelioid Fibrosarcoma of the Bone: A Case Report of High Resistance to Chemotherapy and a Survey of the Literature

    Directory of Open Access Journals (Sweden)

    Thomas G. P. Grunewald

    2010-01-01

    Full Text Available Sclerosing epithelioid fibrosarcoma (SEF is a rare soft tissue sarcoma mostly occurring in extraosseous sites. SEF represents a clinically challenging entity especially because no standardized treatment regimens are available. Intraosseous localization is an additional challenge with respect to the therapeutical approach. We report on a 16-year-old patient with SEF of the right proximal tibia. The patient underwent standardized neoadjuvant chemotherapy analogous to the EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprosthetic reconstruction. Histopathological analysis of the resected tumor showed >90% vital tumor cells suggesting no response to chemotherapy. Therefore, therapy was reassigned to the CWS 2002 High-Risk protocol for the treatment of soft tissue sarcoma. To date (22 months after diagnosis, there is no evidence of relapse or metastasis. Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin, and Methotrexate, which should be considered in planning treatment for patients with SEF.

  3. Fibrosarcoma of bone

    International Nuclear Information System (INIS)

    Taconis, W.K.

    1982-01-01

    A general clinical-radiological description of fibrosarcoma of bone, including tumours with features of malignant fibrous histiocytoma is presented. 104 patients with fibrosarcoma of the long bones are analysed in terms of age and sex distribution, symptoms, duration of symptoms and tumour localization. The radiological findings obtained in patients with fibrosarcoma of the long bones are discussed. The treatment and course of fibrosarcoma of the long bones are discussed. Data on the type of therapy given were available on 103 patients: 67 were treated by ablative surgery either immediately or within three months of preceding local surgery and/or radiotherapy. In the remaining 36 cases treatment consisted of local surgery, radiotherapy or a combination of these, or non-curative (palliative) treatment. In a few cases ablative surgery was performed at a later stage. 13 patients with fibrosarcoma of the axial skeleton and 14 with fibrosarcoma of the jaws are considered. A causistic discussion of patients with a secondary fibrosarcoma is presented. Secondary fibrosarcoma was found in a total of 19 patients (14%); 4 after irradiation. The features of significance for the course of the disease are discussed: general features such as age and sex, tumour localization in the long bones, presence or absence of a pathological fracture, and the radiological and histological characteristics of the tumour. The type of therapy and the occurrence of lung metastases in relation to the course of the disease is also discussed. (Auth.)

  4. Fibrosarcoma of the meninges

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    Ishwar Chand Premsagar

    2010-03-01

    Full Text Available Meningeal fibrosarcomas are rare tumors. Only 41 cases have been reported in the literature to date. Primary central nervous system fibrosarcomas are very aggressive neoplasms and have a poor prognosis. Hence they need to be correctly diagnosed. This is a case of a 13-year old boy with intracranial space occupying lesion. The mass was completely removed and histological examination was characteristic of meningeal fibrosarcoma. The pathological diagnosis is usually made on routine light microscopic examination; however, occasionally these may be difficult to distinguish from other malignant neoplasms such as gliomas, meningiomas and metastases. The diagnosis of fibrosarcoma is based on the identification of a predominant herringbone architectural pattern, the overall uniformity of the spindle cell population, the prominent vimentin positivity, and the presence of pericellular reticulin fibre network. IHC helps to exclude other diagnoses.

  5. Epithelioid lung haenangioendiothelioma

    International Nuclear Information System (INIS)

    Finozzi, V.; Andrade, E.; Campos, N.; Pizarrosa, C.

    2000-01-01

    The first national case of epithelioid lung haenangioendiothelioma concerned a 60 years old woman. Clinical picture and TC diagnosis showed a vascularized tumor producing persistent haenoptoic expectoration.It is necessary to be acquainted with it for the purpose of a differentiated diagnosis. It is a tumor with specific immunohistochemical markers against factor V III.Treatment consisted of surgical resection. Concerning its classification it is situated at the limit between benign and malignant, prognosis is usually good, but evolution is slow, extending for over 20 years

  6. Primary Sclerosing Cholangitis (PSC)

    Science.gov (United States)

    ... Legacy Society Make Gifts of Stock Donate Your Car Personal Fundraising Partnership & Support Share Your Story Spread the Word Give While You Shop Contact Us Donate Now Primary Sclerosing Cholangitis Back ...

  7. Gastric epithelioid haemangioendothelioma.

    Science.gov (United States)

    Tavares, A B; Almeida, A G; Viveiros, F A; Cidade, C N; Barbosa, J M

    2011-05-10

    Epithelioid haemangioendothelioma (EHE) is a rare tumour of vascular origin, characterised by celular proliferation, endotelial, epitelioid or hystiocitoid. It may develop in any organ, but it is more common in lung and liver. Surgery is the recommended treatment; however, in case of a potentially benign situation, an expectant attitude should be adopted. The case reports a 71-year-old female who underwent a laparotomy for a colonic adenocarcinoma. During surgery, a polypoid lesion in the dependency of the gastric wall was found incidentally, which was removed. Histopathology and immunohistochemical analysis confirmed the diagnosis of EHE. Gastric vascular neoplasms represent about 0.9-3.3% of all gastric tumours. Usually have a good prognosis, but due to the borderline biological behaviour of these tumours, it is important to have a detailed clinical evaluation at follow-up of these patients.

  8. Epithelioid leiomyosarcoma of uterus.

    Science.gov (United States)

    Setia, Aparna; Kanotra, Shalini; Aggarwal, Ritika; Bhavthankar, Dilip Prabhakarrao

    2012-04-02

    Fibroid most commonly presents in the reproductive age group and presence of fibroid with postmenopausal bleeding is a rare entity and all investigations and measures should be done to rule out leiomyosarcoma. A 45-year-old female had attained menopause 3 year back and developed postmenopausal bleeding since 2 months, with palpable mass, of 24 weeks size. Ultrasonography showed multiple whorled mass lesions, endometrium and myometrium could not be seen separately. Total abdominal hysterectomy with bilateral salpingoophorectomy was performed. Intraoperative findings showed 24 weeks uterine mass with size 17.5×15.5×11.5 cm and weight 1.9 kg with multiple, intramural fibroids. Cut section of removed specimen showed black and yellow necrotic and haemorrhagic areas with degenerative changes suggestive of malignancy. Histopathology reported epithelioid leiomyosarcoma.

  9. Renal epithelioid angiomyolipoma: 2 Cases report

    African Journals Online (AJOL)

    O. Boudaouara

    2017-06-29

    Jun 29, 2017 ... Abstract. Introduction: The 2004 World Health Organization Classification of Renal Neoplasms defined epithelioid angiomyolipoma as a potentially malignant mesenchymal neoplasm, characterized by a proliferation of predominantly epithelioid cells with approximately one third of patients experiencing ...

  10. Glucocorticosteroids for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Giljaca, Vanja; Poropat, Goran; Stimac, Davor

    2010-01-01

    Primary sclerosing cholangitis is a chronic cholestatic disease of intrahepatic and extrahepatic biliary ducts, characterised by chronic periductal inflammation and sclerosis of the ducts, which results in segmental stenoses of bile ducts, cholestasis, fibrosis, and ultimately, liver cirrhosis...... sclerosing cholangitis, like ursodeoxycholic acid, glucocorticosteroids, and immunomodulatory agents, but none has been successful in reversing the process of the disease. To date, liver transplantation is the only definite therapeutic solution for patients with advanced primary sclerosing cholangitis...

  11. Subacute sclerosing panencephalitis

    International Nuclear Information System (INIS)

    Modi, G.; Bill, P.; Campbell, H.

    1989-01-01

    A 19-year-old female patient presented in an acute state of akinetic mutism. Serological analysis of serum and cerebrospinal fluid demonstrated the presence of antibodies to measles virus. CT scan carried out during this acute phase of relapse demonstrated white matter enhancement affecting the cortical white matter of the frontal lobes and corpus callosum. These features indicate that active demyelination occurs during acute relapse in subacute sclerosing panencephalitis (SSPE) and suggest that immunotherapy should be considered during this acute phase. (orig.)

  12. Epithelioid hemangioendothelioma of the liver

    International Nuclear Information System (INIS)

    Ashraf, S.; Ashraf, H.M.; Mamoon, N.; Luqman, M.L.

    2007-01-01

    Epithelioid hemangioendothelioma is an intermediate grade malignant neoplasm of vascular origin. The tumor involves the liver and lungs, but other organs are affected too. The key to the diagnosis is identification of cells of endothelial origin containing Factor VIII R antigen. Surgical resection of isolated lesions is the treatment of choice; with unpredictable results reported for chemotherapy, radiotherapy, and resection of multiple lesions. The prognosis is very variable, and ranges from few months to more than 25 years. (author)

  13. Orbital Epithelioid Sarcoma: A Case Report

    NARCIS (Netherlands)

    Jurdy, Lama L.; Blank, Leo E.; Bras, Johannes; Saeed, Peerooz

    2016-01-01

    Epithelioid sarcoma is a rare but often aggressive malignancy of soft tissue that usually occurs in young adults as a superficial lesion in the distal upper limbs. To date, there are only 4 case reports of epithelioid sarcoma primarily occurring in the orbit. Two of these patients were treated with

  14. A Case of Angioleiomyoma with Epithelioid Granuloma

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    Masazumi Onishi

    2011-08-01

    Full Text Available We describe a 61-year-old Japanese woman who had been aware of a lesion on her left thigh for 10 years. Pathological examination demonstrated a well-circumscribed encapsulated nodule at the dermal-subcutaneous boundary, composed of eosinophilic spindle cell bundles, connective tissue, and numerous small vessels. Immunohistochemically, these eosinophilic cells were positive for α-smooth muscle actin. The granulomatous areas in the tumor were composed focally of epithelioid cells and lymphocytes. The epithelioid cells were negative for α-smooth muscle actin. We diagnosed this case as an angioleiomyoma with epithelioid granuloma. Malignant tumors with granulomatous change have sometimes been reported in the literature, but benign tumors with epithelioid granuloma, such as the present one, are rare. We thought that epithelioid cell granuloma might transform to angioleiomyoma through the action of IL-1 released from vascular smooth muscle cells.

  15. Primary sclerosing cholangitis

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    Chapman Roger

    2006-10-01

    Full Text Available Abstract Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9–1.31/100,000 and point prevalence of 8.5–13.6/100,000. The onset of PSC is usually insidious and many patients are asymptomatic at diagnosis or have mild symptoms only such as fatigue, abdominal discomfort and pruritus In late stages, splenomegaly and jaundice may be a feature. In most, the disease progresses to cirrhosis and liver failure. Cholangiocarcinoma develops in 8–30% of patients. PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. The disease is diagnosed on typical cholangiographic and histological findings and after exclusion of secondary sclerosing cholangitis. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.

  16. Primary sclerosing cholangitis and pregnancy

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    Casper Q. Kammeijer

    2011-08-01

    Full Text Available Primary sclerosing cholangitis is a progressive disease, and coincidentally in pregnancy it is rare. It is characterized by progressive inflammation and destruction of bile ducts finally resulting in liver failure. A rare case of primary sclerosing cholangitis in pregnancy is presented. The course of the pregnancy was marked by threatened preterm delivery and exacerbation of cholestasis. She was successfully treated with ursodeoxycholic acid (UDCA. Although, primary sclerosing cholangitis has both maternal and fetal effects on pregnancy, the overall outcome is favorable. Only few cases have been reported using high dose ursodeoxycholic acid for primary sclerosing cholangitis in pregnancy, it often improves pruritus but has no protection against stillbirth. Data on the safety to the fetus or neonate and long-term outcome are scarce.

  17. Degenrative Fibroid and Sclerosing Peritonitis

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    Michael Critchley

    2012-01-01

    Full Text Available Sclerosing peritonitis is a rare condition characterised by ascites, peritoneal and bowel wall thickening. Causes reported in the literature include luteal ovarian the comas, peritoneal dialysis, peritoneal chemotherapy and liver cirrhosis. We report an interesting case of a woman presenting with diarrhoea, abdominal distension, ascites and pleural effusion. She was subsequently diagnosed with Sclerosing Peritonitis caused by a degenerating fibroid which was successfully treated by Total Abdominal Hysterectomy and Bilateral Salpingoophrectomy.

  18. Bile acids for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Chen, Weikeng; Gluud, C

    2003-01-01

    Bile acids have been used for treating primary sclerosing cholangitis, but their beneficial and harmful effects remain unclear.......Bile acids have been used for treating primary sclerosing cholangitis, but their beneficial and harmful effects remain unclear....

  19. Penile epithelioid sarcoma: MR imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Sirikci, A.; Bayram, M.; Demirci, M. [Department of Radiology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey); Bakir, K. [Department of Pathology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey); Sarica, K. [Department of Urology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey)

    1999-10-01

    Magnetic resonance imaging findings of a 38-year-old man with epithelioid sarcoma of the penis is presented. It started as a firm, painless and slowly growing nodule at the base of his penis 6 months previously which caused pain radiating to the testis during coitus. It has been well known that sarcomas may well mimic reactive processes. Initial presentation of epithelioid sarcoma may provoke considerable diagnostic difficulty, and its differentiation from benign lesions, such as Peyronie`s disease and chronic inflammation, may be a clinical problem. In our present report the MR findings are compared with those of the epithelioid sarcomas of various locations reported in the literature and differential diagnosis of the entity is discussed. To our knowledge, this is the first report regarding the MR findings of the epithelioid sarcoma of penis. (orig.) With 3 figs., 16 refs.

  20. Subareolar Sclerosing Ductal Hyperplasia.

    Science.gov (United States)

    Cheng, Esther; D'Alfonso, Timothy M; Arafah, Maria; Marrero Rolon, Rebecca; Ginter, Paula S; Hoda, Syed A

    2017-02-01

    Subareolar sclerosing duct hyperplasia (SSDH) remains to be fully characterized nearly 20 years after initial description. Thirty-five SSDH cases diagnosed over a 16-year period (January 2000 to December 2015) were reviewed. All patients were female (mean age = 59 years, range = 18-80) who had presented with a unilateral solitary lesion (left 22, right 13) with a mean size of 1.3 cm (range = 0.4-3.0 cm), and showed florid and papillary epithelial hyperplasia with dense sclerosis without involvement of nipple or areolar epidermis. Significant lesions concurrent within SSDH included low-grade adenosquamous carcinoma (n = 1), ductal carcinoma in situ (DCIS; n = 1), lobular carcinoma in situ (LCIS; n = 1), and atypical ductal hyperplasia (ADH; n = 13). No case of SSDH recurred in a mean follow-up of 44 months (range = 6-189). Subsequent significant lesions occurred in 6 patients: DCIS (n = 3; ipsilateral 2, contralateral 1), ipsilateral ADH (n = 2), and ipsilateral atypical lobular hyperplasia (n = 1). Long-term follow-up for patients with SSDH is indicated as DCIS can occur subsequently in either breast.

  1. Primary sclerosing cholangitis

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    Marina G Silveira

    2008-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in end-stage liver disease and reduced life expectancy. PSC primarily affects young and middle-aged men, often in association with underlying inflammatory bowel disease. The etiology of PSC includes immune-mediated components and elements of undefined nature. A cholestatic picture of liver biochemistries with elevations in serum alkaline phosphatase, nonspecific autoantibodies such as perinuclear antineutrophilic antibody, antinuclear antibodies and smooth muscle antibodies, and diffuse multifocal biliary strictures, resulting in a ‘beaded’ appearance on radiographic studies, are the hallmarks of the disease. No effective medical therapy is currently available, although clinical studies are in progress. Ursodeoxycholic acid at high doses (28 mg/kg/day to 30 mg/kg/day is the most promising agent but is unproven so far. Liver transplantation is currently the only life-extending therapy for patients with end-stage disease, although recurrent disease can be observed in the transplanted liver. The multiple complications of PSC include pruritus, fatigue, vitamin deficiencies, metabolic bone disease, peristomal varices, bacterial cholangitis, dominant biliary strictures, gallbladder stones and polyps, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of PSC.

  2. Subacute sclerosing panencephalitis

    International Nuclear Information System (INIS)

    Inada, Hiroshi; Hattori, Hideji; Nakajima, Seijun; Iwamura, Chiyo; Tanaka, Akemi; Kim, Masayoshi; Matsuoka, Osamu; Murata, Ryosuke; Inoue, Yuichi

    1986-01-01

    We studied three children with subacute sclerosing panencephalitis (SSPE) who had been diagnosed between 1981 and 1983. They were treated with inosiplex and transfer factor, and one was given interferon. Clinical symptoms in all three patients sometimes improved for periods of several months. In two patients computed tomography (CT) first showed low density in the basal ganglia, which later improved and finally disappeared. In all three patients CT showed gradual enlargement of the ventricles and cerebral atrophy. Disappearance of the low-density areas may mean that some of the pathological changes of this disease, including inflammation, demyelination, and gliosis, are reversible. In two patients, we studied magnetic resonance imaging. The spin-echo images showed high intensity in the lateral portions of basal ganglia, in the parieto-occipital portions, and in the frontal portions. Inversion recovery images usually showed low intensity of the same lesions. We think that the MRI gave more useful detail than CT. We think that the improvement in the CT findings and clinical symptoms were due both to the treatment (inosiplex seemed to be especially helpful) and to the natural course of this disease. (author)

  3. Sclerosing Encapsulating Peritonitis; Review

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    Norman O. Machado

    2016-05-01

    Full Text Available Sclerosing encapsulating peritonitis (SEP is a rare chronic inflammatory condition of the peritoneum with an unknown aetiology. Also known as abdominal cocoon, the condition occurs when loops of the bowel are encased within the peritoneal cavity by a membrane, leading to intestinal obstruction. Due to its rarity and nonspecific clinical features, it is often misdiagnosed. The condition presents with recurrent episodes of small bowel obstruction and can be idiopathic or secondary; the latter is associated with predisposing factors such as peritoneal dialysis or abdominal tuberculosis. In the early stages, patients can be managed conservatively; however, surgical intervention is necessary for those with advanced stage intestinal obstruction. A literature review revealed 118 cases of SEP; the mean age of these patients was 39 years and 68.0% were male. The predominant presentation was abdominal pain (72.0%, distension (44.9% or a mass (30.5%. Almost all of the patients underwent surgical excision (99.2% without postoperative complications (88.1%.

  4. [Epithelioid hemangioendothelioma: an uncommon liver tumor].

    Science.gov (United States)

    Pareja, Eugenia; Cortés, Miriam; Rayon, Miguel; Moya, Angel; Mir, Jose

    2010-01-01

    We report the case of a female patient who was referred to our unit because of a solid liver tumor, suggestive of metastasis. After biopsy, the patient was diagnosed with epithelioid hemangioendothelioma of the liver. Epithelioid hemangioendothelioma is a rare entity with an unpredictable, potentially fatal, clinical course and outcome. Due to its rarity, this entity should be considered when a solitary hepatic lesion is detected and should be included in the differential diagnosis with liver metastases. We highlight the infrequency of this tumor, its presentation as a solitary hepatic lesion and the indication of surgical treatment. We describe the clinical and pathological characteristics of epithelioid hemangioendothelioma of the liver and report a new case of this entity. The distinct therapeutic options are discussed. Copyright 2010 Elsevier España, S.L. All rights reserved.

  5. Malignant Epithelioid Angiomyolipoma of the Kidney

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    Kuo-How Huang

    2007-01-01

    Full Text Available Angiomyolipoma (AML is a common benign renal tumor composed of thick-walled blood vessels, smooth muscle, and adipose tissue, but the malignant epithelioid variant is extremely rare. A 78-year-old woman presented with fever and left flank pain for 3 days. Computed tomography showed a heterogeneously enhanced mass without fat density in the left kidney. Radical nephrectomy was performed and pathology showed malignant epithelioid AML with regional lymph node metastases. The tumor cells were positive for human melanosome-associated protein (HMB-45 on immunohistochemical staining. The patient died of disseminated metastases (lungs and bones 5 months postoperatively. Epithelioid AML is a potentially aggressive tumor. The prognosis is poor in metastatic disease. HMB-45 immunoreactivity is a useful marker to make diagnosis. [J Formos Med Assoc 2007;106(2 Suppl:S51-S54

  6. Bile acids for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Poropat, Goran; Giljaca, Vanja; Stimac, Davor

    2011-01-01

    Primary sclerosing cholangitis is a progressive chronic cholestatic liver disease that usually leads to the development of cirrhosis. Studies evaluating bile acids in the treatment of primary sclerosing cholangitis have shown a potential benefit of their use. However, no influence on patients...

  7. Perivascular Epithelioid Cell Tumor in the Stomach

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    Sun Ah Shin

    2017-07-01

    Full Text Available Perivascular epithelioid cell tumors or PEComas can arise in any location in the body. However, a limited number of cases of gastric PEComa have been reported. We present two cases of gastric PEComas. The first case involved a 62-year-old woman who presented with a 4.2 cm gastric subepithelial mass in the prepyloric antrum, and the second case involved a 67-year-old man with a 5.0 cm mass slightly below the gastroesophageal junction. Microscopic examination revealed that both tumors were composed of perivascular epithelioid cells that were immunoreactive for melanocytic and smooth muscle markers. Prior to surgery, the clinical impression of both tumors was gastrointestinal stromal tumor (GIST, and the second case was erroneously diagnosed as GIST even after microscopic examination. Although gastric PEComa is a very rare neoplasm, it should be considered in the differential diagnosis of gastric submucosal lesions.

  8. CT features of renal epithelioid angiomyolipomas

    International Nuclear Information System (INIS)

    Hu Xiaoyun; Fang Xiangming; Hu Chunhong; Chen Hongwei; Cui Lei; Bao Jian; Yao Xuanjun

    2010-01-01

    Objective: To explore the CT and pathological features of renal epithelioid angiomyolipoma (EAML). Methods: Clinical data and CT images from ten cases with EAML proved by surgery and pathology were retrospectively analyzed. All cases were performed with plain and contrast enhanced CT scans. Results: CT features: higher pre-contrasted density than kidney, bulging from kidney, absent of fat, markedly heterogeneous enhancement (quick wash-in and slow wash-out), big size without lobular sign, complete capsule with clear margin and mild necrostic area. Pathological features: diffuse sheets of epithelioid cells were found under microscopy with immunohistochemistrical findings including positivity for HMB-45 and negativity for EMA. Conclusion: Some specific CT features, which is correlated well with the pathological findings, provide helpful information in the primary diagnosis of EAML. (authors)

  9. Chemotherapy-induced sclerosing cholangitis

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    Sandrasegaran, K.; Alazmi, W.M.; Tann, M.; Fogel, E.L.; McHenry, L.; Lehman, G.A

    2006-08-15

    Aim: To review the computed tomography (CT), magnetic resonance imaging (MRI) and cholangiographic findings of chemotherapy-induced sclerosing cholangitis (CISC). Methods: Between January 1995 and December 2004, 11 patients in the endoscopic retrograde cholangiography database were identified with CISC. Twelve CT, four MRI, 69 endoscopic and nine antegrade cholangiographic studies in these patients were reviewed. Serial change in appearance and response to endoscopic treatment were recorded. Results: CISC showed segmental irregular biliary dilatation with strictures of proximal extrahepatic bile ducts. The distal 5 cm of common bile duct was not affected in any patient. CT and MRI findings included altered vascular perfusion of one or more liver segments, liver metastases or peritoneal carcinomatosis. Biliary strictures needed repeated stenting in 10 patients (mean: every 4.7 months). Cirrhosis (n = 1) or confluent fibrosis (n = 0) were uncommon findings. Conclusion: CISC shares similar cholangiographic appearances to primary sclerosing cholangitis (PSC). Unlike PSC, biliary disease primarily involved ducts at the hepatic porta rather than intrahepatic ducts. Multiphasic contrast-enhanced CT or MRI may show evidence of perfusion abnormalities, cavitary liver lesions, or metastatic disease.

  10. Sclerosing encapsulating peritonitis: a case report

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    Candido, Paula de Castro Menezes; Werner, Andrea de Freitas; Pereira, Izabela Machado Flores; Matos, Breno Assuncao; Pfeilsticker, Rudolf Moreira; Silva Filho, Raul, E-mail: paulacmcandido@yahoo.com.br [Hospital Felicio Rocho, Belo Horizonte, MG (Brazil)

    2015-01-15

    Sclerosing encapsulating peritonitis, a rare cause of bowel obstruction, was described as a complication associated with peritoneal dialysis which is much feared because of its severity. The authors report a case where radiological findings in association with clinical symptoms have allowed for a noninvasive diagnosis of sclerosing encapsulating peritonitis, emphasizing the high sensitivity and specificity of computed tomography to demonstrate the characteristic findings of such a condition. (author)

  11. MRI in subacute sclerosing panencephalitis

    International Nuclear Information System (INIS)

    Tuncay, R.; Akman-Demir, G.; Goekyigit, A.; Eraksoy, M.; Barlas, M.; Tolun, R.; Guersoy, G.

    1996-01-01

    Subacute sclerosing panencephalitis (SSPE) is a progressive, slow virus infection of the brain, caused by the measles virus, attacking children and young adults. We investigated 15 patients with SSPE by MRI, with 5 normal and 10 pathological results. In the early period, lesions were in the grey matter and subcortical white matter. They were asymmetrical and had a predilection for the posterior parts of the hemispheres. Later, high-signal changes in deep white matter and severe cerebral atrophy were observed. Parenchymal lesions significantly correlated with the duration of disease. A significant relationship between MRI findings and clinical stage was observed in the 1st year of the disease. (orig.). With 4 figs., 1 tab

  12. MRI in subacute sclerosing panencephalitis

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    Tuncay, R. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Akman-Demir, G. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Goekyigit, A. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Eraksoy, M. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Barlas, M. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Tolun, R. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Guersoy, G. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey)

    1996-10-01

    Subacute sclerosing panencephalitis (SSPE) is a progressive, slow virus infection of the brain, caused by the measles virus, attacking children and young adults. We investigated 15 patients with SSPE by MRI, with 5 normal and 10 pathological results. In the early period, lesions were in the grey matter and subcortical white matter. They were asymmetrical and had a predilection for the posterior parts of the hemispheres. Later, high-signal changes in deep white matter and severe cerebral atrophy were observed. Parenchymal lesions significantly correlated with the duration of disease. A significant relationship between MRI findings and clinical stage was observed in the 1st year of the disease. (orig.). With 4 figs., 1 tab.

  13. Infantile fibrosarcoma: radiological and clinical features

    International Nuclear Information System (INIS)

    Vinnicombe, S.J.; Hall, C.M.

    1994-01-01

    Two cases of infantile fibrosarcoma are described. This rare childhood malignancy of mesodermal origin usually affects the lower limbs, as it did in both of our cases. Previously, the only treatment option available involved some form of radical and often mutilating surgery. More recently, combination chemotherapy has given good results, with the effect that various imaging modalities have become important in assessing both the initial extent of disease and the response to treatment. Computed tomography has the advantage of demonstrating the amount of osseous involvement, but at the expense of a considerable dose of ionizing radiation. On the other hand, magnetic resonance imaging, with its multiplanar capacity, gives superior demonstration of breaching of tissue planes, which has important implications for planning of surgery. However, as in other soft tissue tumours, changes in signal characteristics with treatment have proved less specific than was originally anticipated. (orig.)

  14. Fibrosarcoma en región geniana

    Directory of Open Access Journals (Sweden)

    Guillermo Sánchez Acuña

    2012-06-01

    Full Text Available El fibrosarcoma es la neoplasia maligna mesodérmica de más incidencia.Los autores se vieron motivados a presentar un caso clínico con esta entidad con el objetivo de enfatizar en la importancia del correcto manejo diagnóstico y terapéutico en función de la rehabilitación de estos pacientes. Se presenta un caso clínico de un paciente de 54 años de edad con antecedentes de HTA controlada, que 23 años atrás comenzó con aumento de volumen en región geniana izquierda, motivo por el que fue intervenido quirúrgicamente en los años 1987, 1991 y 2009, en otros servicios de Cirugía Máxilofacial con diagnóstico histopatológico de neurofibroma, que acudió a consulta presentando una lesión de 5 cm de diámetro, con patrón de crecimiento rápido, que sangraba espontáneamente, la cual le comprimía el globo ocular izquierdo limitándole la apertura palpebral. Luego de realizársele el estudio preoperatorio correspondiente, se decidió tratamiento quirúrgico, se obtuvo un diagnóstico concluyente a través del estudio inmunohistoquímico de fibrosarcoma de grado intermedio de diferenciación, de marcada inmunorreactividad CD34 (+, focal, Vimentina (+, focal. Se concluye que el tratamiento de esta entidad es quirúrgico, teniendo en cuenta las normas oncológicas actuales, y su enfoque terapéutico debe ser multidisciplinario en aras de lograr una correcta rehabilitación funcional y estética del paciente. La inmunohistoquímica constituye un arma diagnóstica concluyente que contribuye a instaurar tratamiento adecuado en estas lesiones.

  15. Fibroblastic osteosarcoma with epithelioid and squamous differentiation in a dog.

    Science.gov (United States)

    Jenkins, Tiffany L; Agnew, Dalen; Rissi, Daniel R

    2018-04-01

    A fibroblastic osteosarcoma with epithelioid and squamous differentiation in the distal femur of a 9-y-old spayed female Greyhound dog is described. Grossly, the tumor consisted of a pale-white, firm-to-hard mass that replaced the medullary and cortical areas of the distal end of the right femur. Histologically, the mass was composed predominantly of spindle cells admixed with areas of mineralized and non-mineralized osteoid matrix that were surrounded by stellate osteoblasts and scattered multinucleate giant cells, consistent with the diagnosis of a fibroblastic osteosarcoma. In addition, well-demarcated clusters of neoplastic epithelioid cells and foci of squamous differentiation with keratin pearls were present throughout the neoplasm. The spindle cells, epithelioid cells, and areas of squamous differentiation expressed cytoplasmic immunostaining for osteocalcin and osteonectin. The spindle cells and epithelioid cells were also immunopositive for vimentin. Epithelioid cells also expressed occasional cytoplasmic immunostaining for pancytokeratin (PCK) Lu-5, and areas of squamous differentiation were immunoreactive for PCK Lu-5 and high molecular weight CK; these areas were inconsistently immunoreactive for CK 5-6 and immunonegative for low molecular weight CK. Foci of squamous differentiation were not located within blood or lymphatic vessels, given that no immunoreactivity for factor VIII-related antigen was observed around these areas. A thorough autopsy and an evaluation of the medical history excluded a primary carcinoma or other neoplasm elsewhere in the dog. The findings were consistent with a diagnosis of fibroblastic osteosarcoma with epithelioid and squamous differentiation.

  16. File list: Oth.Oth.05.AllAg.Fibrosarcoma [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.Oth.05.AllAg.Fibrosarcoma hg19 TFs and others Others Fibrosarcoma SRX253227,SRX...253229,SRX253232,SRX253231,SRX253230 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/Oth.Oth.05.AllAg.Fibrosarcoma.bed ...

  17. File list: InP.Oth.20.AllAg.Fibrosarcoma [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available InP.Oth.20.AllAg.Fibrosarcoma hg19 Input control Others Fibrosarcoma SRX253236,SRX2...53238,SRX253237,SRX253233,SRX253235,SRX253234 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/InP.Oth.20.AllAg.Fibrosarcoma.bed ...

  18. File list: InP.Oth.50.AllAg.Fibrosarcoma [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available InP.Oth.50.AllAg.Fibrosarcoma hg19 Input control Others Fibrosarcoma SRX253236,SRX2...53238,SRX253237,SRX253233,SRX253235,SRX253234 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/InP.Oth.50.AllAg.Fibrosarcoma.bed ...

  19. File list: InP.Oth.10.AllAg.Fibrosarcoma [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available InP.Oth.10.AllAg.Fibrosarcoma hg19 Input control Others Fibrosarcoma SRX253236,SRX2...53233,SRX253238,SRX253237,SRX253234,SRX253235 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/InP.Oth.10.AllAg.Fibrosarcoma.bed ...

  20. File list: InP.Oth.05.AllAg.Fibrosarcoma [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available InP.Oth.05.AllAg.Fibrosarcoma hg19 Input control Others Fibrosarcoma SRX253236,SRX2...53233,SRX253234,SRX253235,SRX253237,SRX253238 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/InP.Oth.05.AllAg.Fibrosarcoma.bed ...

  1. File list: Oth.Oth.50.AllAg.Fibrosarcoma [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.Oth.50.AllAg.Fibrosarcoma hg19 TFs and others Others Fibrosarcoma SRX253227,SRX...253229,SRX253232,SRX253231,SRX253230 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/Oth.Oth.50.AllAg.Fibrosarcoma.bed ...

  2. File list: Oth.Oth.20.AllAg.Fibrosarcoma [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.Oth.20.AllAg.Fibrosarcoma hg19 TFs and others Others Fibrosarcoma SRX253227,SRX...253229,SRX253232,SRX253231,SRX253230 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/Oth.Oth.20.AllAg.Fibrosarcoma.bed ...

  3. File list: Oth.Oth.10.AllAg.Fibrosarcoma [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.Oth.10.AllAg.Fibrosarcoma hg19 TFs and others Others Fibrosarcoma SRX253227,SRX...253229,SRX253232,SRX253231,SRX253230 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/Oth.Oth.10.AllAg.Fibrosarcoma.bed ...

  4. Sclerosing peritonitis with gross calcification: case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Cheung Sook; Kim, Young Jae; Min, Seon Jeong; Cho, Seong Whi; Lee, Gyung Kyu; Lee, Eil Seong; Kang, Ik Won [Hallym University College of Medicine, Seoul (Korea, Republic of)

    2003-09-01

    Sclerosing peritonitis is an uncommon complication of continuous ambulatory peritoneal dialysis (CAPD) and can lead to small bowel dysfunction involving abdominal pain, progressive loss of ultrafiltration, and small intestinal obstruction. Peritoneal thickening, in which calcification can develop, often starts as al small plaque which gradually becomes larger. We report a case of CAPD-related calcifying peritonitis.

  5. Grey-Turner's sign in sclerosing peritonitis

    NARCIS (Netherlands)

    Stouthard, J. M.; Krediet, R. T.; Arisz, L.

    1989-01-01

    A 41-year-old CAPD patient developed Grey-Turner's sign during the course of bacterial peritonitis due to Pseudomonas aeruginosa. At the same time a diagnosis of sclerosing peritonitis was made by CT-scanning of the abdomen. We think that Grey-Turner's flank staining could either have been caused by

  6. A new lethal sclerosing bone dysplasia

    International Nuclear Information System (INIS)

    Kingston, H.M.; Freeman, J.S.; Hall, C.M.

    1991-01-01

    A neonate is described with a lethal sclerosing bone dysplasia associated with prenatal fractures and craniofacial abnormalities including microcephaly, exophthalmos, hypoplastic nose and mid-face, small jaw and nodular hyperplasia of the gums. Parental consanguinity suggests that an autosomal recessive mutation is the likely aetiology. (orig.)

  7. Transumbilical laparoscopic treatment of Congenital Infantile Fibrosarcoma of the Ileum

    Directory of Open Access Journals (Sweden)

    G. Scirè

    2014-08-01

    Full Text Available Congenital-Infantile Fibrosarcoma (CIF is a malignant mesenchymal tumor representing 10-20% of soft-tissue tumors. Complete surgical resection is generally the treatment of choice. The most recurrent cytogenetic abnormality was identified as the traslocation t(12;15(p13:q25, which bears the fusion of Tel gene EVT6 with TrkC gene. This study describes a case of infantile fibrosarcoma of the ileum in a female newborn examined for intestinal occlusion and its laparoscopic treatment.

  8. Fibrosarcoma en región geniana Fibrosarcoma in the genial region

    Directory of Open Access Journals (Sweden)

    Guillermo Sánchez Acuña

    2012-06-01

    Full Text Available El fibrosarcoma es la neoplasia maligna mesodérmica de más incidencia.Los autores se vieron motivados a presentar un caso clínico con esta entidad con el objetivo de enfatizar en la importancia del correcto manejo diagnóstico y terapéutico en función de la rehabilitación de estos pacientes. Se presenta un caso clínico de un paciente de 54 años de edad con antecedentes de HTA controlada, que 23 años atrás comenzó con aumento de volumen en región geniana izquierda, motivo por el que fue intervenido quirúrgicamente en los años 1987, 1991 y 2009, en otros servicios de Cirugía Máxilofacial con diagnóstico histopatológico de neurofibroma, que acudió a consulta presentando una lesión de 5 cm de diámetro, con patrón de crecimiento rápido, que sangraba espontáneamente, la cual le comprimía el globo ocular izquierdo limitándole la apertura palpebral. Luego de realizársele el estudio preoperatorio correspondiente, se decidió tratamiento quirúrgico, se obtuvo un diagnóstico concluyente a través del estudio inmunohistoquímico de fibrosarcoma de grado intermedio de diferenciación, de marcada inmunorreactividad CD34 (+, focal, Vimentina (+, focal. Se concluye que el tratamiento de esta entidad es quirúrgico, teniendo en cuenta las normas oncológicas actuales, y su enfoque terapéutico debe ser multidisciplinario en aras de lograr una correcta rehabilitación funcional y estética del paciente. La inmunohistoquímica constituye un arma diagnóstica concluyente que contribuye a instaurar tratamiento adecuado en estas lesiones.The fibrosarcoma is the more incident mesodermic malignant neoplasm. Authors present this clinical case presenting with this entity to emphasize on the significance of the appropriate diagnostic and therapeutical management for rehabilitation of these patients. This patient is aged 54 with a history of controlled high blood pressure (HBT who over the 23 past years begun to show an increase of volume in

  9. Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment.

    Science.gov (United States)

    Rosenberg, Ari; Agulnik, Mark

    2018-03-15

    Epithelioid hemangioendothelioma (EHE) is an extremely rare sarcoma, as such it can pose a clinical dilemma based solely on its rarity. Also, the spectrum of disease varies greatly between an indolent disease and aggressive disease with widespread metastases. In our clinical practice, the primary focus has been to get a handle on the aggressive nature of the disease, which will then dictate how urgently one needs to treat the patient. Pathological review with immunohistochemistry and molecular characterization is paramount. Our treatment strategy is watch-and-wait versus active therapy on clinical trial or based on results of prior clinical trials. There is evidence to support the use of chemotherapeutics and targeted therapies specifically focusing on anti-angiogenesis. The current landscape of oncology with the emergence and excitement of immunotherapy could also translate in a role for immunotherapy in this disease. While rare, there is certainly no reason that research and trials for patients with EHE should not remain on utmost importance for those of us who specialize in the treatment of sarcomas.

  10. Congenital fibrosarcoma of the chest wall: Report of a case

    Directory of Open Access Journals (Sweden)

    Shilpi Singh Gupta

    2010-01-01

    Full Text Available Congenital fibrosarcoma (CFS is a rare soft tissue tumour that usually occurs before the age of 1, and involves the distal extremities. The literature regarding the precise diagnosis and treatment of these tumours is limited. We present and discuss a case of CFS which ended fatally due to lung metastasis after 2 years.

  11. Primary fibrosarcoma im small intestine of dog - Case report

    Directory of Open Access Journals (Sweden)

    Geórgia Modé Magalhães

    2015-06-01

    Full Text Available ABSTRACT. Magalhães G.M., Santilli J., Calazans S.G., Nishimura L.T., Cerejo S.A. & Dias F.G.G. [Primary fibrosarcoma im small intestine of dog - Case report.] Fibrossarcoma primário em intestino delgado de cão - Relato de caso. Revista Brasileira de Medicina Veterinária, 37(2:145-148, 2015. Programa de Pós-Graduação em Medicina Veterinária de Pequenos Animais, Universidade de Franca, Av. Dr. Armando Salles Oliveira, 201, Cx postal 82, Parque Universitá- rio, Franca, SP 14404-600, Brasil. E-mail: georgiamode@hotmail.com Intestinal neoplasms are uncommon in dogs, and among the most frequently diagnosed are smooth muscle, lymphomas and carcinomas. The fibrosarcoma is extremely rare in the intestine of animals of this species therefore, little is known about the macroscopic and behavior of this tumor. Given this unusual intestinal disease in dogs, the present study aimed to report a case of intestinal fibrosarcoma in a poodle breed dog, 15 years old, with no apparent clinical signs. The mass was pedunculated, whitish and firm consistency. The diagnosis was made by histopathology. After four months of surgical excision, there was no recurrence and metastasis. We conclude that the intestinal fibrosarcoma has low aggressiveness, are rare and can present macroscopic pediculated.

  12. Fibrosarcoma of the vocal fold: a late complication of radiotherapy

    International Nuclear Information System (INIS)

    Nageris, B.; Elidan, J.; Sherman, Y.

    1994-01-01

    The carcinogenic effect of ionizing radiation is a well known phenomenon. However, the induction of malignancies following irradiation for head and neck cancers is quite rare. Most reported cases are osteogenic sarcomas with soft tissue sarcomas encountered less often. We report a rare case of fibrosarcoma of the larynx, following radiation therapy for glottic carcinoma. (author)

  13. Epithelioid sarcoma mimicking abscess: review of the MRI appearances

    International Nuclear Information System (INIS)

    Dion, E.; Forest, M.; Brasseur, J.L.; Grenier, P.; Amoura, Z.

    2001-01-01

    A case of epithelioid sarcoma involving the soft tissue of the ankle is presented. The tumor was a hemorrhagic, fluid-filled, multiloculated lesion with inflammatory changes in the surrounding planes. Tuberculous abscess was diagnosed on the basis of the clinical picture, ultrasound and MRI findings. Surgical exploration of the ankle mass was carried out because of lack of local healing while the patient's general and pulmonary status improved on antituberculosis treatment. This was an unusual case of epithelioid sarcoma mimicking a multilocular abscess. (orig.)

  14. Inflammatory bowel disease with primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Sørensen, Jakob Ørskov; Nielsen, Ole Haagen; Andersson, Mikael

    2018-01-01

    BACKGROUND AND AIMS: Inflammatory bowel disease (IBD) may be complicated by primary sclerosing cholangitis (PSC). We aimed to assess the characteristics of Danish PSC-IBD patients and to compare their prognosis with IBD patients without PSC. METHODS: A retrospective nationwide population-based co......BACKGROUND AND AIMS: Inflammatory bowel disease (IBD) may be complicated by primary sclerosing cholangitis (PSC). We aimed to assess the characteristics of Danish PSC-IBD patients and to compare their prognosis with IBD patients without PSC. METHODS: A retrospective nationwide population....... Among patients with PSC and Crohn's disease (CD) 91% had colonic involvement. The PSC-IBD patients had a significantly higher probability of receiving resective surgery (HR; 2.13, 95% CI: 1.50-3.03); of developing colorectal cancer (CRC) (HR; 21.4, 95% CI: 9.6-47.6), of cholangiocarcinoma (HR; 190, 95...

  15. Subacute sclerosing panencephalitis presenting as mania

    Directory of Open Access Journals (Sweden)

    Aggarwal Ashish

    2011-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a rare, invariably fatal degenerative disease of the central nervous system developing after measles infection. Besides neurological symptoms as initial presenting symptoms, rare reports of its presentation with pure psychiatric symptoms have been reported. We here report a case of 14 year old male who initially presented with manic symptoms and then subsequently diagnosed to be suffering from SSPE. Improtance of ruling our organic conditions is emphasized.

  16. Characteristics of primary sclerosing cholangitis in Japan.

    Science.gov (United States)

    Takikawa, Hajime

    2007-10-01

    At a workshop on primary sclerosing cholangitis (PSC) held during Digestive Disease Week - Japan 2003, 388 PSC cases in Japan were analyzed. Two peaks in the age distribution were also observed in this survey. Jaundice and itching, major symptoms in PSC patients included in the diagnostic criteria, were observed in only 28% and 16%, respectively. Alkaline phosphatase levels were less than twofold of the upper limit of the normal range in 35%. In this regard, the diagnostic criteria in 2003 from the Mayo Clinic, including cholestatic symptoms and two- to threefold increases in serum alkaline phosphatase, should be modified in Japan. Inflammatory bowel diseases were complicated in 37%, and autoimmune pancreatitis (AIP) in 7.2%. PSC cases with inflammatory bowel diseases were younger than the average, creating the firstpeak in age distribution, and have similar characteristics compared to patients with PSC in foreign countries. In addition, even after the exclusion of cases of sclerosing cholangitis complicated with AIP, the second peak in the age distribution was clearly evident. Recently, a concept of immunoglobulin G4-related sclerosing cholangitis has been postulated, which has a similar pathogenesis to AIP but without apparent pancreatic lesions. PSC patients without apparent involvement of the pancreas may be present in older patients and seem to be specific to Japan.

  17. Cytogenetic study of a pulmonary sclerosing hemangioma.

    Science.gov (United States)

    Pareja, María J; Vargas, María T; Sánchez, Ana; Ibáñez, José; González-Cámpora, Ricardo

    2009-11-01

    Pulmonary sclerosing hemangioma (PSH) is an uncommon benign tumor that presents as a solitary asymptomatic and slow-growing nodule. It occurs in both young and old persons; peak incidence is in the fifth decade. Both sexes are affected by this tumor, but women more frequently than men. On histological examination, PSH shows prominent sclerotization and vascularization of the tissue. Recent studies conclude that PSH derives from type II pneumocytes, but the potential for progression and histogenesis remains controversial. We report a case of pulmonary sclerosing hemangioma in a 61-year-old woman with a neoplastic node 1 cm in diameter. The karyotype was 46,XX,t(8;18),der(14;15),+14 in all the cells analyzed. PTEN (10q23) and IgH (14q32) probes were analyzed in interphase nuclei and paraffin-embedded tissues of tumor cells. These chromosome abnormalities could provide information about the relationship of genetic changes to the biological properties of sclerosing hemangioma tumors.

  18. Ultrasonographic findings of sclerosing encapsulating peritonitis

    Energy Technology Data Exchange (ETDEWEB)

    Han, Jong Kyu; Lee, Hae Kyung; Moon, Chul; Hong, Hyun Sook; Kwon, Kwi Hyang; Choi, Deuk Lin [Soonchunhyangi University College of Medicine, Seoul (Korea, Republic of)

    2001-03-15

    To evaluate the ultrasonographic findings of the patients with sclerosing encapsulating peritonitis (SEP). Thirteen patients with surgically confirmed sclerosing encapsulating peritonitis were involved in this study. Because of intestinal obstruction, all patients had received operations. Among 13 patients, 12 cases had continuous ambulatory peritoneal dialysis (CAPD) for 2 months-12 years and 4 months from (mean; 6 years and 10 months), owing to chronic renal failure and one patient had an operation due to variceal bleeding caused by liver cirrhosis. On ultrasonographic examination, all patients showed loculated ascites which were large (n=7) or small (n=6) in amount with multiple separations. The small bowel loops were tethered posteriorly perisaltic movement and covered with the thick membrane. The ultrasonographic of findings of sclerosing encapsulating peritonitis were posteriorly tethered small bowels covered with a thick membrane and loculated ascites with multiple septa. Ultrasonographic examination can detect the thin membrane covering the small bowel loops in the early phase of the disease, therefore ultrasonography would be a helpful modality to diagnose SEP early.

  19. Diagnosis, Differential Diagnosis, and Epidemiology of Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    Ponsioen, Cyriel Y.

    2015-01-01

    According to recent guidelines, primary sclerosing cholangitis (PSC) is diagnosed when a patient has a cholestatic liver enzyme profile, characteristic bile duct changes on imaging, and when secondary causes of sclerosing cholangitis are excluded. In patients with a clinical suspicion but normal

  20. Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a Newborn

    Directory of Open Access Journals (Sweden)

    Margarita Kaiser

    2017-01-01

    Full Text Available Congenital infantile fibrosarcoma (CIF is a rare malignant mesenchymal tumor and only 14 cases have been reported with gastrointestinal manifestation. We report about a female newborn delivered per emergency cesarean section at 34 weeks of gestation. Postnatally, she rapidly developed an acute abdomen and sonographic evidence of intestinal perforation requiring laparotomy on the first day of life. A perforated 2 × 3 cm sized spherical tumorous structure of the jejunum was identified. Due to unknown histopathology at this point and unclear resectional margins, she received a temporary ileostomy, which was closed two months later. Histopathology revealed a congenital intestinal fibrosarcoma without the characteristic ETV6-NTRK3 fusion transcript. In conclusion, this rare tumor must be considered as differential diagnosis of intestinal perforations in newborns.

  1. Treatment of fibrosarcoma in a maned wolf (Chrysocyon brachyurus) by rostral maxillectomy.

    Science.gov (United States)

    McNulty, E E; Gilson, S D; Houser, B S; Ouse, A

    2000-09-01

    A 12-yr-old captive intact male maned wolf (Chrysocyon brachyurus) was diagnosed with a fibrosarcoma of the incisive bones. The mass was excised by rostral maxillectomy, and the wolf remained normal and on display with good function and cosmetics for 7 mo. Subsequently, it became weak, ataxic, and dyspneic and was euthanatized. At necropsy, there was a small regrowth of the maxillary tumor, a metastatic mediastinal mass, and multiple metastatic lung masses, suggesting that oral fibrosarcoma in maned wolves behaves similarly to oral fibrosarcoma in domestic canines. Aggressive surgical treatment of oral fibrosarcoma in this species can achieve good functional and cosmetic results.

  2. Computed tomography appearances of sclerosing encapsulating peritonitis

    International Nuclear Information System (INIS)

    George, C.; Al-Zwae, K.; Nair, S.; Cast, J.E.I.

    2007-01-01

    Sclerosing encapsulating peritonitis (SEP) is a serious complication of peritoneal dialysis (PD) characterized by thickened peritoneal membranes, which lead to decreased ultra-filtration and intestinal obstruction. Its early clinical features are nonspecific, and it is often diagnosed late following laparotomy and peritoneal biopsy, when the patient develops small bowel obstruction, which can be a life-threatening complication. However, this is changing with increasing awareness of computed tomography (CT) findings in SEP. CT can yield an early, non-invasive diagnosis that may improve patient outcome. We present a review of the CT appearances of SEP

  3. Computed tomography appearances of sclerosing encapsulating peritonitis

    Energy Technology Data Exchange (ETDEWEB)

    George, C. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom)]. E-mail: cheriangeorge@hotmail.com; Al-Zwae, K. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom); Nair, S. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom); Cast, J.E.I. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom)

    2007-08-15

    Sclerosing encapsulating peritonitis (SEP) is a serious complication of peritoneal dialysis (PD) characterized by thickened peritoneal membranes, which lead to decreased ultra-filtration and intestinal obstruction. Its early clinical features are nonspecific, and it is often diagnosed late following laparotomy and peritoneal biopsy, when the patient develops small bowel obstruction, which can be a life-threatening complication. However, this is changing with increasing awareness of computed tomography (CT) findings in SEP. CT can yield an early, non-invasive diagnosis that may improve patient outcome. We present a review of the CT appearances of SEP.

  4. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon

    Directory of Open Access Journals (Sweden)

    Legakis Nikolaos

    2006-02-01

    Full Text Available Abstract Background Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. Case presentation A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Conclusion Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

  5. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon).

    Science.gov (United States)

    Serafimidis, Costas; Katsarolis, Ioannis; Vernadakis, Spyros; Rallis, George; Giannopoulos, George; Legakis, Nikolaos; Peros, George

    2006-02-13

    Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon) is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

  6. Primary sclerosing cholangitis: diagnostic and management challenges

    Directory of Open Access Journals (Sweden)

    Sirpal S

    2017-11-01

    Full Text Available Sanjeev Sirpal,1 Natasha Chandok2 1Department of Medicine, Centre Hospitalier de l’Université de Montréal (CHUM, University of Montreal, Montreal, QC, 2Department of Medicine, University of Western Ontario, London, ON, Canada Abstract: Primary sclerosing cholangitis (PSC is a chronic immune-mediated disease affecting intra- and extrahepatic bile ducts, primarily the large biliary ducts. Clinical manifestations are broad, and the spectrum encompasses asymptomatic cholestasis, icteric cholangitis with pruritis, cirrhosis, and cholangiocarcinoma. Though rare, PSC has a propensity to affect young to middle-aged males and is strongly associated with inflammatory bowel disease. There is an unmet need for effective medical treatments for PSC, and to date, the only curative therapy is liver transplantation reserved for those with end-stage liver disease. This article addresses the diagnostic and management challenges of PSC, with a succinct analysis of existing therapies, their limitations, and a glimpse into the future of the management of this multifaceted pathologic entity. Keywords: primary sclerosing cholangitis, management, PSC

  7. IgG4-Related Sclerosing Cholangitis.

    Science.gov (United States)

    Nakazawa, Takahiro; Shimizu, Shuya; Naitoh, Itaru

    2016-08-01

    More men than women develop immunoglobulin G4-related sclerosing cholangitis (IgG4-SC). Age at clinical onset is significantly older in patients with IgG4-SC. Patients with IgG4-SC appear similar to those with cholangiocarcinoma and primary sclerosing cholangitis (PSC). The association between IgG4-SC and autoimmune pancreatitis (AIP) is useful for the diagnosis of IgG4-SC. However, some IgG4-SC cases are isolated from AIP and are difficult to diagnose. The authors focus on three distinct features of IgG4-SC. First, diffuse inflammation induces a longer stenosis on cholangiography in contrast to the short stenosis of patients with PSC. Second, fibroinflammatory involvement is observed mainly in the stroma of the bile duct wall, whereas the bile duct epithelium is intact. Third, steroid therapy results in remarkable improvement. Although the prognosis of patients with IgG4-SC is good, some cases have developed portal hypertension and liver cirrhosis during their clinical course. Further study is needed to elucidate the long-term outcomes and mechanism of IgG4-SC. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  8. Sclerosing stromal tumor of the ovary: A case report

    Directory of Open Access Journals (Sweden)

    Navjot Kaur

    2014-01-01

    Full Text Available Sclerosing stromal tumors are benign ovarian neoplasms of the sex cord-stromal category, occurring predominantly in the second and third decades of life. Herein, we report a 23-year-old female who presented with pelvic pain, irregular menses but normal hormonal status and was diagnosed as having a right ovarian tumor. A right oophorectomy was performed, and microscopic examination revealed a sclerosing stromal tumor of the right ovary. We stress the importance of being familiar with sclerosing stromal tumors when evaluating ovarian neoplasms in young women, in order to contribute to the appropriate clinical management, preventing extensive and unnecessary surgery, and preserving fertility.

  9. Sclerosing Stromal Tumor of Ovary: A Case Report

    Directory of Open Access Journals (Sweden)

    Menka Khanna

    2012-01-01

    Full Text Available Sclerosing stromal tumor (SST is an extremely rare and distinctive sex cord stromal tumor which occurs predominantly in the second and third decades of life. We report a case of a 32-year-old woman who developed a sclerosing stromal tumor of ovary and presented with irregular menstruation and pelvic pain. Her hormonal status was normal but CA-125 was raised. She was suspected to have a malignant tumor on computed tomography and underwent bilateral salpingo-oopherectomy. It is therefore necessary to keep in mind the possibility of sclerosing stromal tumor in a young woman.

  10. Epithelioid Sarcoma: Opportunities for Biology-driven Targeted Therapy

    Directory of Open Access Journals (Sweden)

    Jonathan eNoujaim

    2015-08-01

    Full Text Available Epithelioid sarcoma is a soft tissue sarcoma of children and young adults for which the preferred treatment for localised disease is wide surgical resection. Medical management is to a great extent undefined, and therefore for patients with regional and distal metastases, the development of targeted therapies is greatly desired. In this review we will summarize clinically-relevant biomarkers (e.g., SMARCB1, CA125, dysadherin and others with respect to targeted therapeutic opportunities. We will also examine the role of EGFR, mTOR and polykinase inhibitors (e.g., sunitinib in the management of local and disseminated disease. Towards building a consortium of pharmaceutical, academic and non-profit collaborators, we will discuss the state of resources for investigating epithelioid sarcoma with respect to cell line resources, tissue banks, and registries so that a roadmap can be developed towards effective biology-driven therapies.

  11. Multifocal epithelioid hemangioendothelioma of the phalanges of the hand

    International Nuclear Information System (INIS)

    Bruegel, Melanie; Waldt, Simone; Woertler, Klaus; Rummeny, Ernst J.; Weirich, Gregor

    2006-01-01

    Epithelioid hemangioendothelioma (EH) of bone is a rare vascular neoplasm characterized by epithelioid endothelial cells and a variable biologic behaviour. The principal sites of occurrence of this osteolytic tumor are the lower extremity and the axial skeleton. Approximately half of the cases present with multifocal disease. The latter feature can be helpful in suggesting the diagnosis of a vascular tumor; on the other hand, it strengthens the need for a skeletal survey or whole-body MRI/CT. We report on the clinical, histologic and radiologic features - including CT and MRI findings - of EH in a case of multifocal disease of the phalanges of the hand, a very uncommon anatomic site of affliction. (orig.)

  12. Epithelioid hemangioma of brachial artery: report of a case and review of the literature

    Directory of Open Access Journals (Sweden)

    Ragazzi Moira

    2015-01-01

    Full Text Available Epithelioid hemangioma (EH is an uncommon benign vascular lesion, also known as angioblastic lymphoid (or angiolymphoid hyperplasia with eosinophilia, characterized by an unclear etiopathogenesis.

  13. A Case of Distal Epithelioid Sarcoma of the Thumb Expressing Podoplanin, TLE1 and Ca 125

    Directory of Open Access Journals (Sweden)

    George Karagkounis

    2013-01-01

    Full Text Available Distal epithelioid sarcoma is a rare and slowly growing tumor that usually develops in the upper extremities of young adults. Neoplastic cells have both spindle and epithelioid appearance and are characterized by the loss of the nuclear protein SMARCB1/INI1. We present the case of a distal epithelioid sarcoma arising in the thumb of a 14-year-old girl, which immunohistochemically was characterized by the loss of SMARCB1/INI1 protein as well as the expression of podoplanin (D2-40, TLE1, Glut1, and Ca 125; plus, we highlight the differential diagnosis of epithelioid sarcoma from its histological mimics.

  14. Epithelioid sarcoma of the penis: a case report.

    Science.gov (United States)

    Colombo, Fulvio; Franceschelli, Alessandro; Schiavina, Riccardo; Gentile, Giorgio; Passaretti, Giovanni; Martorana, Giuseppe

    2013-11-01

    Epithelioid sarcoma of the penis is a slowly growing soft tissue neoplasm that rarely arises from the penis. The aim of this case is to describe this rare pathology and to underline the importance of differential diagnosis with benign diseases such as Peyronie's disease. We report the case of a 20-year-old man who referred the onset of a progressive left dorsolateral penile curvature (about 60°) started 3 years before. The patient was evaluated with two US examinations that revealed two sites of tunical thickening with normal hemodynamic evaluation. The physical examination demonstrated a dorsal fibrotic plaque of about 2.5 cm. A juvenile form of Peyronie's Disease was diagnosed, and the patient was scheduled for surgical treatment (plaque's incision/excision and grafting). Intraoperative appearance showed that the great part of the left cavernous body was substituted by a very tough tissue which deeply involved the erectile tissue; intraoperative frozen section was suspicious for mesenchymal epithelioid neoplasm. In order to obtain definitive histological analysis and collect proper informed consent, we preferred to proceed with our original project, applying a dual graft (buccal mucosa and allograft dermal matrix) to cover the wide excised area. The final histological report confirmed the diagnosis of epithelioid sarcoma. Postoperative CT and MRI, at 3 and 6 months from surgery, were negative for metastases. The patient underwent radical intervention after 6 months. Epithelioid sarcoma of the penis and Peyronie's Disease can present with similar clinical findings although they obviously entail a different clinical progress. Since the diagnosis of neoplasm can be established only by the pathologist on biopsy specimen, in cases of unusual clinical presentation of Peyronie's disease (especially in young men suffering from a fast-growing penile induration), an early histological assessment should be performed to avoid the possibility of misdiagnosis in case of this

  15. CT imaging and histopathological features of renal epithelioid angiomyolipomas

    International Nuclear Information System (INIS)

    Cui, L.; Zhang, J.-G.; Hu, X.-Y.; Fang, X.-M.; Lerner, A.; Yao, X.-J.; Zhu, Z.-M.

    2012-01-01

    Aim: To describe computed tomography (CT) imaging and histopathological manifestations of renal epithelioid angiomyolipomas (EAMLs) for better understanding and cognition in the diagnosis of this new category of renal tumours. Materials and methods: Clinical data and CT images from 10 cases of EAML were retrospectively analysed. All patients underwent CT with and without contrast medium administration, with multiplanar reconstruction (MPR) when needed. Results: Plain CT manifestations of EAMLs were a higher density of mass (10–25 HU) than renal parenchyma, bulging contour of the involved kidney, absence of fat, distinct edges without a lobulate appearance. Contrast-enhanced CT features were markedly heterogeneous enhancement (from rapid wash-in to slow wash-out), large tumour size without lobular appearance, complete capsule with distinct margins and frequent mild necrotic areas. Histopathological features were epithelioid cells with eosinophilic cytoplasm, large and deeply stained nuclei, and dense arrangement of tumour cells with patchy necrosis; diffuse sheets of epithelioid cells were positive for HMB-45 (melanoma-associated antigen) and negative for epithelial membrane antigen (EMA) staining. Conclusion: Multiple specific CT features correlated well with the histopathology and may play an important role in the primary diagnosis of EAMLs.

  16. Infantile fibrosarcoma: Magnetic resonance imaging findings in six cases

    Energy Technology Data Exchange (ETDEWEB)

    Canale, Sandra [Department of Radiology, Institut Gustave-Roussy, 39 rue Camille Desmoulins, 94805 Villejuif (France)], E-mail: canalesandra@yahoo.com; Vanel, Daniel [Department of Radiology, Institut Gustave-Roussy, 39 rue Camille Desmoulins, 94805 Villejuif (France); Istituti Ortopedici Rizzoli, 1/10 via di Barbiano, 40136 Bologna (Italy); Couanet, Dominique [Department of Radiology, Institut Gustave-Roussy, 39 rue Camille Desmoulins, 94805 Villejuif (France); Patte, Catherine [Department of Pediatrics, Institut Gustave-Roussy, 39 rue Camille Desmoulins, 94805 Villejuif (France); Caramella, Caroline; Dromain, Clarisse [Department of Radiology, Institut Gustave-Roussy, 39 rue Camille Desmoulins, 94805 Villejuif (France)

    2009-10-15

    Purpose: To retrospectively review magnetic resonance (MR) imaging features in a series of six infantile fibrosarcomas to find out if MR can suggest this unusual diagnosis and to highlight the value of MR during and following treatment. Materials and methods: The records of six cases of histologically proven infantile fibrosarcoma were retrieved from the files of our cancer center. All imaging data available were consensually reviewed by two radiologists. Results: There were five females and one male (age range at diagnosis, 0-12 months; mean, 6 months). The most common finding was a well-circumscribed single mass in five patients (83%). All tumors had arisen on limbs; at their proximal or distal extremity or at the root of the limb. The masses were 9 cm large in mean diameter. The initial tumor signal was isointense to muscle on T1-weighted and hyperintense on T2-weighted sequences. All masses were well circumscribed and half of them contained internal fibrous septa. The internal signal was homogeneous in three patients and heterogeneous in the three others. An intense enhancement was seen in all three contrast-enhanced exams available; heterogeneous in two cases and homogeneous in one. Osseous erosion was observed in only one patient who was the only one with distant metastasis. After treatment (chemotherapy and very limited surgery), tumors had totally disappeared, leaving muscle fat infiltration in two patients and subcutaneous fat hypertrophy in one patient. Conclusion: Although imaging findings are not specific of infantile fibrosarcoma, this diagnosis could be suggested when MR imaging depicts a large well-circumscribed mass arising in a limb at birth or during the neonatal period. This mass is sometimes heterogeneous and septate and exhibits an isointense T1- and hyperintense T2-weighted signals and strong enhancement. MR is also the technique of choice for follow-up during treatment which consists nowadays almost exclusively in chemotherapy.

  17. Genetics Home Reference: polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy

    Science.gov (United States)

    ... feelings of intense happiness (euphoria), a loss of inhibition, and poor concentration. These neurologic changes cause significant ... with sclerosing leukoencephalopathy Orphanet: Nasu-Hakola disease Patient Support and Advocacy Resources (3 links) Alzheimer's Association Family ...

  18. A renal epithelioid angiomyolipoma/perivascular epithelioid cell tumor with TFE3 gene break visualized by FISH.

    Science.gov (United States)

    Ohe, Chisato; Kuroda, Naoto; Hes, Ondrej; Michal, Michal; Vanecek, Tomas; Grossmann, Petr; Tanaka, Yukichi; Tanaka, Mio; Inui, Hidekazu; Komai, Yoshihiro; Matsuda, Tadashi; Uemura, Yoshiko

    2012-12-01

    We present a case of renal epithelioid angiomyolipoma (eAML)/perivascular epithelioid cell tumor (PEComa) with a TFE3 gene break visible by fluorescence in situ hybridization (FISH). Histologically, the tumor was composed of mainly epithelioid cells forming solid arrangements with small foci of spindle cells. In a small portion of the tumor, neoplastic cells displayed nuclear pleomorphism, such as polygonal and enlarged vesicular nuclei with prominent nucleoli. Marked vascularity was noticeable in the background, and perivascular hyaline sclerosis was also seen. Immunohistochemically, neoplastic cells were diffusely positive for α-smooth muscle actin and melanosome in the cytoplasm. Nuclei of many neoplastic cells were positive for TFE3. FISH analysis of the TFE3 gene break using the Poseidon TFE3 (Xp11) Break probe revealed positive results. Reverse transcriptase-polymerase chain reactions (RT-PCR) for ASPL/TFE3, PRCC/TFE3, CLTC/TFE3, PSF/TFE3, and NonO/TFE3 gene fusions all revealed negative results. This is the first reported case of renal eAML/PEComa with a TFE3 gene break, and it has unique histological findings as compared to previously reported TFE3 gene fusion-positive PEComas. Pathologists should recognize that PEComa with TFE3 gene fusion can arise even in the kidney.

  19. Primary fibrosarcoma im small intestine of dog - Case report

    OpenAIRE

    Geórgia Modé Magalhães; Juliana Santilli; Sabryna Gouveia Calazans; Lilian Toshiko Nishimura; Sofia de Amorim Cerejo; Fernanda Gosuen Gonçalves Dias

    2015-01-01

    ABSTRACT. Magalhães G.M., Santilli J., Calazans S.G., Nishimura L.T., Cerejo S.A. & Dias F.G.G. [Primary fibrosarcoma im small intestine of dog - Case report.] Fibrossarcoma primário em intestino delgado de cão - Relato de caso. Revista Brasileira de Medicina Veterinária, 37(2):145-148, 2015. Programa de Pós-Graduação em Medicina Veterinária de Pequenos Animais, Universidade de Franca, Av. Dr. Armando Salles Oliveira, 201, Cx postal 82, Parque Universitá- rio, Franca, SP 14404-600, Brasil. E-...

  20. Endocardial fibrosarcoma in a reticulated python (Python reticularis).

    Science.gov (United States)

    Gumber, Sanjeev; Nevarez, Javier G; Cho, Doo-Youn

    2010-11-01

    A female, reticulated python (Python reticularis) of unknown age was presented with a history of lethargy, weakness, and distended coelom. Physical examination revealed severe dystocia and stomatitis. The reticulated python was euthanized due to a poor clinical prognosis. Postmortem examination revealed marked distention of the reproductive tract with 26 eggs (10-12 cm in diameter), pericardial effusion, and a slightly firm, pale tan mass (3-4 cm in diameter) adhered to the endocardium at the base of aorta. Based on histopathologic and transmission electron microscopic findings, the diagnosis of endocardial fibrosarcoma was made.

  1. A case of cutaneous scleroderma with primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    H P Nandeesh

    2014-01-01

    Full Text Available Sclerosing cholangitis comprises of a spectrum of cholestatic conditions that are characterized by patchy fibrosis, inflammation and destruction of intra hepatic and extrahepatic ducts. We report a case of a 42 year old woman who presented with darkening of skin with yellowish discolouration of the eyes. Clinical examination revealed icterus, taut skin with hepatosplenomegaly. Liver function tests showed a cholestatic picture. Skin biopsy showed features of cutaneous scleroderma. MRCP and Liver biopsy was suggestive of sclerosing cholangitis.

  2. Lymphoplasmacytic Sclerosing Pancreatitis and Retroperitoneal Fibrosis

    Directory of Open Access Journals (Sweden)

    Nigel K. F. Koo Ng

    2008-01-01

    Full Text Available Although cases of lymphoplasmacytic sclerosing pancreatitis (LSP associated with idiopathic retroperitoneal fibrosis have been reported, the association is rare. We describe a 74-year-old man who presented with obstructive jaundice and weight loss. Nineteen months earlier, he had been diagnosed with idiopathic retroperitoneal fibrosis and treated with bilateral ureteric stents. Initial investigations were suggestive of a diagnosis of LSP, however, a malignant cause could not be ruled out. He underwent an exploratory laparotomy and frozen sections confirmed the diagnosis of LSP. An internal biliary bypass was performed using a Roux loop of jejunum, and the patient made an uneventful recovery. This case illustrates the difficulty in distinguishing LSP from pancreatic carcinoma preoperatively.

  3. Bifocal sclerosing osteosarcoma: unusual presentation and course

    International Nuclear Information System (INIS)

    Abramovici, L.; Steiner, G.C.; Rosenberg, Z.; Kenan, S.

    1998-01-01

    Multifocal osteosarcoma is uncommon. Long-term survival of an incompletely treated case is exceptional. We report an unusual case of bifocal sclerosing osteosarcoma in a 38-year-old women that involved the left ilium and right proximal femur. The femoral lesion was resected. The tumor in the left ilium was not treated. She did not receive chemotherapy and has been free of metastases for 7 years. Recently, growth of the pelvic osteosarcoma has resulted in vascular compression and edema of the lower extremity. The patient's alkaline phosphatase has been elevated throughout. The tumor was HMB-45 positive, which has not been previously reported in osteosarcoma. The pathogenesis of multifocal osteosarcoma is discussed. (orig.)

  4. The Impact of Radiation on an Unusual Case of Omental Epithelioid Angiosarcoma

    Directory of Open Access Journals (Sweden)

    Sumana Narayanan

    2015-01-01

    Full Text Available Epithelioid angiosarcoma is a rare high-grade tumor with irregular vascular morphology. We report an unusual case of intra-abdominal epithelioid angiosarcoma affecting the omentum and peritoneal surfaces resulting in significant hemorrhagic and inflammatory changes. As in other cases of this tumor this patient had previously undergone radiation treatment for a history of cervical cancer.

  5. Impacts of tomato extract on the mice fibrosarcoma cells

    Directory of Open Access Journals (Sweden)

    Shirzad Hedayatollah

    2013-01-01

    Full Text Available Introduction: The anticancer effect of tomato lycopene has been approved in some cancers. This study was aimed to determine the prohibitive and therapeutic effects of tomato extract on the growth of fibrosarcoma in mice. Materials and Methods: In this experimental study 3 groups of 10 male Balb/c mice were injected subcutaneously with 5×105 WEHI-164 tumor cells in the chest area. Prevention group was fed tomato extract (5 mg for a 4 week period (from 2 weeks before tumor cell injection up to 2 weeks after injection and the treatment group was fed simultaneously with tumor cell injection up to two weeks after injection daily by an oral gastric tube. The tumors areas were measured and recorded on days 10, 12, 14, 16, 18, 20 and 22. The data were analyzed using Kruskal-Wallis and Mann-Whitney tests. Results: The results showed that the tumor areas in control group were significantly more after the intervention than two groups of treatment and prevention (p<0.05. The difference was not statistically significant between the two groups of prevention and treatment. Conclusion: With emphasize on antioxidant of tomato, it seems that tomato extract has an important role in prevention and control fibrosarcoma growth.

  6. Intensive fibrosarcoma-binding capability of the reconstituted analog and its antitumor activity.

    Science.gov (United States)

    Xu, Jian; Du, Yue; Liu, Wen-Juan; Li, Liang; Li, Yi; Wang, Xiao-Fei; Yi, Hong-Fei; Shan, Chuan-Kun; Xia, Gui-Min; Liu, Xiu-Jun; Zhen, Yong-Su

    2018-11-01

    Fibrosarcomas are highly aggressive malignant tumors. It is urgently needed to explore targeted drugs and modalities for more effective therapy. Matrix metalloproteinases (MMPs) play important roles in tumor progression and metastasis, while several MMPs are highly expressed in fibrosarcomas. In addition, tissue inhibitor of metalloproteinase 2 (TIMP2) displays specific interaction with MMPs. Therefore, TIMP2 may play an active role in the development of fibrosarcoma-targeting agents. In the current study, a TIMP2-based recombinant protein LT and its enediyne-integrated analog LTE were prepared; furthermore, the fibrosarcoma-binding intensity and antitumor activity were investigated. As shown, intense and selective binding capability of the protein LT to human fibrosarcoma specimens was confirmed by tissue microarray. Moreover, LTE, the enediyne-integrated analog of LT, exerted highly potent cytotoxicity to fibrosarcoma HT1080 cells, induced apoptosis, and caused G2/M arrest. LTE at 0.1 nM markedly suppressed the migration and invasion of HT1080 cells. LTE at tolerated dose of 0.6 mg/kg inhibited the tumor growth of fibrosarcoma xenograft in athymic mice. The study provides evidence that the TIMP2-based reconstituted analog LTE may be useful as a targeted drug for fibrosarcome therapy.

  7. Less promising results with sclerosing ethoxysclerol injections for midportion achilles tendinopathy: a retrospective study

    NARCIS (Netherlands)

    van Sterkenburg, Maayke N.; de Jonge, Milko C.; Sierevelt, Inger N.; van Dijk, C. Niek

    2010-01-01

    BACKGROUND: Local injections of the sclerosing substance polidocanol (Ethoxysclerol) have shown good clinical results in patients with chronic midportion Achilles tendinopathy. After training by the inventors of the technique, sclerosing Ethoxysclerol injections were applied on a group of patients

  8. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial

    NARCIS (Netherlands)

    Beuers, U.; Spengler, U.; Kruis, W.; AYDEMIR, U.; WIEBECKE, B.; HELDWEIN, W.; WEINZIERL, M.; Pape, G. R.; Sauerbruch, T.; Paumgartner, G.

    1992-01-01

    The efficacy and safety of ursodeoxycholic acid for the treatment of primary sclerosing cholangitis were evaluated in a prospective, randomized, double-blind, placebo-controlled trial. Fourteen patients with primary sclerosing cholangitis documented by cholestatic serum enzyme pattern, liver

  9. Epithelioid hemangioma of the colon: a case report

    Directory of Open Access Journals (Sweden)

    Ronaldo Nonose

    Full Text Available CONTEXT: Epithelioid hemangioma or angiolymphoid hyperplasia with eosinophilia is an uncommon benign vascular neoplasm that is usually located on the face or neck. Exceptionally, it has been described affecting the colon, with only two such cases described in the worldwide literature. The aim here was to present a case of primary epithelioid hemangioma of the sigmoid colon with confirmation by immunohistochemical examination. CASE REPORT: A 37-year-old woman had had a complaint of intermittent abdominal pain for six months. Two months after the condition started, she began to present changes in her intestinal habit, with evacuations containing blood and mucus and a weight loss of 4 kg over this period. At physical examination, a palpable mass was noted in the lower left quadrant of the abdomen. Neoplasia of the colon was clinically suspected and she underwent colonoscopy. This demonstrated the presence of a vegetating sessile lesion of approximately 5 cm in diameter, at a distance of 36 cm from the anal margin. It occupied 80% of the intestinal lumen. A biopsy collected during the examination suggested a diagnosis of neoplasia of vascular origin. After surgical resection, histopathological examination of the resected specimen confirmed the diagnosis of epithelioid hemangioma of the colon, which was backed up by the immunohistochemical panel (factor VIII, Ki-67, CD-34. At present, three years after the surgery, the patient is asymptomatic, she has recovered her normal weight and she has normal findings from control colonoscopy. Despite the rarity of neoplasia of vascular origin, this possibility should be considered in the differential diagnosis for colorectal tumors.

  10. Congenital fibrosarcoma of the ileum: case report with molecular confirmation and literature review.

    LENUS (Irish Health Repository)

    Rizkalla, Hala

    2012-02-01

    Congenital fibrosarcoma is a rare, soft tissue malignancy of infancy, most commonly involving the distal extremities. We report a case of congenital fibrosarcoma of the ileum in a 5-day-old boy who presented with an acute abdomen due to ileal perforation. Partial ileal resection was carried out with successful anastomosis. Grossly, the resected small bowel showed focal luminal stenosis with a thickened, indurated wall. Histology showed a transmural primitive spindle cell proliferation with a morphology consistent with congenital fibrosarcoma. The associated hallmark chromosomal translocation t(12;15)(q13;q25) was demonstrated by reverse transcriptase polymerase chain reaction.

  11. Correlation of Imaging Findings with Pathologic Findings of Sclerosing Adenosis

    International Nuclear Information System (INIS)

    Choi, Bo Bae; Shu, Kwang Sun

    2012-01-01

    The purpose of this study was to evaluate the mammographic and sonographic findings of pure sclerosing adenosis. We retrospectively reviewed the mammographic and sonographic findings in 40 cases of pure sclerosing adenosis confirmed by core needle biopsy (n = 23), vacuum-assisted biopsy (n = 7), excision biopsy (n = 9), and lumpectomy (n = 1) from January 2002 to March 2010. All imaging findings were analyzed according to the American College of Radiology (ACR) breast imaging reporting and data system (BI-RADS). Radiologic features were correlated with pathologic findings. Although most mammograms showed negative findings (57%), calcification was the most common abnormal finding of sclerosing adenosis. On sonography, the most common finding was a circumscribed oval hypoechoic mass without posterior features (78%). Most masses showed BI-RADS category 3, (75%, 27/36). Five cases showed categories 4 or 5 (14%, 5/36). Most mammographic and sonographic findings of sclerosing adenosis are non-specific and non-pathognomonic, even though sometimes sclerosing adenosis can be radiologically or histopathologically confused with malignancy

  12. A delayed presentation of ameloblastic fibrosarcoma in an African patient.

    Science.gov (United States)

    Chauke, Nkhensani Yvonne; Sofianos, Chrysis; Liakos, Dimitri; Ndobe, Elias

    2017-08-01

    A 24-year-old womanpresented with ameloblastic fibrosarcoma arising from ameloblastic fibroma. The delayed presentation accounted for the extensive destruction of the mandible and complete occlusion of her oral cavity. This resulted in an inability to eat and maintain oral hygiene. A multidisciplinary team management approach involved nutritional optimisation, segmental mandibulectomy, reconstruction with a reconstructive plate and a free anterolateral thigh flap to line the the floor of mouth. Functional and aesthetic outcome was acceptable, and the patient is planned for secondary free fibular flap bony reconstruction. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  13. Caffeine induces metformin anticancer effect on fibrosarcoma in hamsters.

    Science.gov (United States)

    Popović, D J; Lalošević, D; Miljković, D; Popović, K J; Čapo, I; Popović, J K

    2018-04-01

    We investigated the effect of metformin and caffeine on fibrosarcoma in hamsters. 32 Syrian golden hamsters of both sexes, weighing approximately 100 g, were randomly allocated to 3 experimental and 2 control groups, with a minimum of 6 animals per group. 2 x 106 BHK-21/C13 cells in 1 ml were injected subcutaneously into the animals' back in 4 groups. The first experimental group started peroral treatment with metformin 500 mg/kg daily, the second with caffeine 100 mg/kg daily and the third with a combination of metformin 500 mg/kg and caffeine 100 mg/kg daily, via a gastric probe 3 days before tumor inoculation. After 2 weeks, when the tumors were approximately 2 cm in the control group, all animals were sacrificed. The blood was collected for glucose and other analyses. The tumors were excised and weighed and their diameters were measured. The tumor samples were pathohistologically (HE) and immunohistochemically (Ki-67, CD 31, COX IV, GLUT-1, iNOS) assessed and the main organs toxicologically analyzed, including the control animals that had received metformin and caffeine. Tumor volume was determined using the formula LxS2/2, where L was the longest and S the shortest diameter. Ki-67-positive cells in the tumor samples were quantified. Images were taken and processed by software UTHSCSA Image Tools for Windows Version 3.00. Statistical significances were determined by the Student's t-test. The combination of metformin and caffeine inhibited fibrosarcoma growth in hamsters without toxicity. Administration of metformin with caffeine might be an effective and safe approach in novel nontoxic adjuvant anticancer treatment.

  14. Subacute Sclerosing Panencephalitis: Clinical and Demographic Characteristics

    International Nuclear Information System (INIS)

    Rafique, A.; Amjad, N.; Chand, P.; Ahmed, K.; Ibrahim, S.; Zaidi, S. S. Z.; Rana, M. S.

    2014-01-01

    Objective: To determine the clinical and demographic characteristics of children diagnosed with Subacute sclerosing panencephalitis (SSPE). Study Design: Case series. Place and Duration of Study: The Aga Khan University Hospital, Karachi, from January 2000 to June 2012. Methodology: A retrospective analysis was done, regarding medical charts of 43 children under the age of 16 years with a discharge diagnosis of SSPE. Demographic and clinical characteristics were recorded. Results were expressed as percentages. Results: Most of the 43 patients were male (72%). The average age at presentation was 8.7 years with average duration of symptoms being 100.6 days. History of measles was present in 17 patients (39.5%). All children had seizures at presentation and 65% had cognitive impairment. Most patients required poly therapy for control of seizures. Sodium valproate was the most commonly used anti-epileptic agent; Isoprinosine was tried in 22 (51%) patients. CSF for antimeasles antibodies was positive in approximately 86% of the 40 (93%) children. EEG showed burst suppression pattern in 36 (83.7%) cases. Forty-two patients (97.6%) were discharged home in a vegetative state. Conclusion: SSPE is progressive neurodegenerative disorder. It can be prevented by timely immunization against measles. Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis. Most patients experience a gradual but progressive decline in motor and cognitive functions. (author)

  15. Intraductal location of the sclerosing adenosis of the breast.

    Science.gov (United States)

    Unal, Bulent; Gur, A Serhat; Bhargava, Rohit; Edington, Howard; Ahrendt, Gretchen; Soran, Atilla

    2009-01-01

    Sclerosing adenosis is a benign breast disease with non-specific images on ultrasound or mammogram. It can mimic infiltrating carcinoma when the above mentioned imaging techniques are used. Herein we present a patient with breast cancer who received neoadjuvant chemotherapy and subsequently underwent mastectomy. Ductoscopy was performed to the mastectomised breast specimen as per the ductoscopy research protocol. Ductoscopy revealed several nodular lesions in the duct with no additional demonstrable intraductal pathology. The lesions were reported as sclerosing adenosis by pathologist. As to our knowledge, this is the first case in literature that demonstrates the use of ductoscopy in diagnosing the sclerosing adenosis in the breast tissue. Ductoscopy and development of ductoscopy guided biopsy techniques may be used as an early diagnostic method for the ductal breast lesions (Fig. 2, Ref. 10). Full Text (Free, PDF) www.bmj.sk.

  16. Microscopic endometrial perivascular epithelioid cell nodules: a case report with the earliest presentation of a uterine perivascular epithelioid cell tumor

    Directory of Open Access Journals (Sweden)

    Fang Chia-Lang

    2012-09-01

    Full Text Available Abstract Perivascular epithelioid cell (PEC tumors (PEComas are a family of related mesenchymal tumors composed of PECs which co-express melanocytic and smooth muscle markers. Although their distinctive histologic, immunohistochemical, ultrastructural, and genetic features have been clearly demonstrated, their histogenesis and normal counterpart remain largely unknown. Precursor lesions of PEComas have rarely been reported. We herein describe a tuberous sclerosis patient with microscopic PEC nodules in the endometrium of adenomyosis, pelvic endometriosis, an ovarian endometriotic cyst, and the endometrium of the uterine cavity. The nodules showed a mixture of spindle-shaped and epithelioid cells concentrically arranged around small arteries. The cells exhibited uniform nuclei, light eosinophilic cytoplasm, and immunoreactivity with HMB-45 and CD10. Some nodules revealed continuity with a PEComa in the myometrium. These findings support microscopic endometrial PEC nodules possibly being precursor lesions of uterine PEComas. The wide distribution of the nodules in the pelvis may be related to the multicentricity of PEComas in tuberous sclerosis patients. Owing to the immunoreactivity with CD10, microscopic endometrial PEC nodules may be misinterpreted as endothelial stromal cells unless melanocytic markers are stained. To the best of our knowledge, this is a case with the earliest manifestation of PEC lesions occurring in the endometrium. Virtual Slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/9658280017862643

  17. Diffuse sclerosing variant of papillary thyroid carcinoma: case report

    International Nuclear Information System (INIS)

    Lee, Seung Chan; Kim, Dong Wook

    2006-01-01

    Diffuse sclerosing papillary carcinoma (DSPC) is a variant of papillary thyroid carcinoma (PTC), but it shows more aggressive clinical course and a poorer prognosis than the other types of PTC. Most PTCs show a focal nodular pattern in the thyroid on the imaging modalities, but DSPC reveals a diffuse infiltrating configuration in the thyroid without any focal nodular lesion. To our knowledge, there are scant radiological reports of diffuse sclerosing variant of papillary thyroid carcinoma. In this report, we present the case of a patient with DSPC who showed the characteristic findings on sonography and computed tomography

  18. The CT appearances of sclerosing mesenteritis and associated diseases

    International Nuclear Information System (INIS)

    Wat, S.Y.J.; Harish, S.; Winterbottom, A.; Choudhary, A.K.; Freeman, A.H.

    2006-01-01

    Sclerosing mesenteritis is characterized by non-specific inflammation of the mesenteric fat associated with variable amount of fibrosis. The aetiology is unclear; the pathogenesis is obscure, and even its nomenclature remains variable. It is a rare condition with imaging features that can be mistaken either for a mesenteric neoplasm or for a wide variety of non-neoplastic inflammatory conditions. Knowledge of the imaging features of this condition may prevent unwarranted aggressive therapy. This review discusses the pathogenesis, clinical manifestations of this condition, as well as illustrating the characteristic computed tomography (CT) features of sclerosing mesenteritis. A rational approach to the differential diagnosis is discussed

  19. Sclerosing stromal tumor of the ovary in a premenarchal female

    International Nuclear Information System (INIS)

    Fefferman, Nancy R.; Pinkney, Lynne P.; Rivera, Rafael; Popiolek, Dorota; Hummel-Levine, Pascale; Cosme, Jaqueline

    2003-01-01

    Sclerosing stromal tumor (SST) is a rare benign ovarian neoplasm of stromal origin with less than 100 cases reported in the literature. Unlike the other stromal tumors, thecomas and fibromas, which tend to occur in the fifth and sixth decades, sclerosing stromal tumors predominantly affect females in the second and third decades. Computed tomography (CT), magnetic resonance imaging (MRI), and ultrasound findings have been described, but have not been reported previously in the pediatric literature. We present a case of SST of the ovary in a 10-year-old premenarchal female, the youngest patient to our knowledge reported in the literature, and describe the ultrasound and CT findings with pathologic correlation. (orig.)

  20. Sclerosing cholangitis: Clinicopathologic features, imaging spectrum, and systemic approach to differential diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Ni Eun [Dept. of Radiology, Research Institute of Radiological Science, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, So Yeon; Lee, Seung Soo; Byun, Jae Ho; Kim, Hyoung Jung; Kim, Jin Hee; Lee, Moon Gyu [Dept. of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    2016-02-15

    Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.

  1. Sclerosing Cholangitis: Clinicopathologic Features, Imaging Spectrum, and Systemic Approach to Differential Diagnosis.

    Science.gov (United States)

    Seo, Nieun; Kim, So Yeon; Lee, Seung Soo; Byun, Jae Ho; Kim, Jin Hee; Kim, Hyoung Jung; Lee, Moon-Gyu

    2016-01-01

    Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.

  2. Epithelioid sarcoma: results of conservative surgery and radiotherapy

    International Nuclear Information System (INIS)

    Callister, Matthew D.; Ballo, Matthew T. M.D.; Pisters, Peter W.T.; Patel, Shreyaskumar R.; Feig, Barry W.; Pollock, Raphael E.; Benjamin, Robert S.; Zagars, Gunar K

    2001-01-01

    Purpose: To determine the outcome and prognostic factors for patients with localized epithelioid sarcoma treated with conservative surgery and radiotherapy (RT). Methods and Materials: The medical records of 24 patients with nonmetastatic epithelioid sarcoma treated with conservative surgery and RT were reviewed. Preoperative RT was given to 3 patients (median 46.4 Gy) and postoperative RT to 21 patients (median 64.5 Gy). A local (limb-sparing) surgical procedure was performed in all patients. Results: At a median follow-up of 131 months, 14 patients had relapsed and 13 patients had died. The actuarial overall and disease-free survival rate at 10 years was 50% and 37%, respectively. Local, nodal, and metastatic failure occurred in 7, 4, and 10 patients, respectively, yielding a 10-year actuarial local, nodal, and metastatic control rate of 63%, 81%, and 56%, respectively. Univariate analysis revealed that size ≤5 cm and extremity location were favorable prognostic factors for overall, disease-free, and metastasis-free survival. The actuarial 5-year overall, disease-free, and metastasis-free survival rate was 79% vs. 25% (p=0.002), 51% vs. 13% (p=0.03), and 79% vs. 13% (p 5 cm. The actuarial 5-year overall, disease-free, and metastasis-free survival rate was 77% vs. 39% (p 0.002), 56% vs. 0% (p=0.01), and 78% vs. 17% (p=0.01), respectively, for extremity vs. nonextremity location. Multivariate analysis of the factors correlating with the overall, disease-free, and metastasis-free survival confirmed the favorable prognostic significance of small lesion size. The prognostic significance of extremity location on univariate analysis was explained by an imbalance in the mean tumor sizes. Conclusions: Epithelioid sarcoma is an aggressive soft-tissue sarcoma, with high rates of local and distant relapse. Local control with conservative surgery and RT compares favorably to published surgical series. The poor outcome for tumors ≥5 cm in size emphasizes the need for

  3. Subacute sclerosing panencephalitis: A clinical appraisal

    Directory of Open Access Journals (Sweden)

    Sujit Abajirao Abajirao

    2013-01-01

    Full Text Available Introduction: Subacute sclerosing panencephalitis (SSPE is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE. Methods: We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken′s criteria. We analyzed demographical, clinical, electrophysiological, and imaging features. Results: Study included 34 patients, 26 (76.5% males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5% followed by seizures (23.5%. Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years. Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging. Conclusion: SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.

  4. Epithelioid Sarcoma: Opportunities for Biology-Driven Targeted Therapy.

    Science.gov (United States)

    Noujaim, Jonathan; Thway, Khin; Bajwa, Zia; Bajwa, Ayeza; Maki, Robert G; Jones, Robin L; Keller, Charles

    2015-01-01

    Epithelioid sarcoma (ES) is a soft tissue sarcoma of children and young adults for which the preferred treatment for localized disease is wide surgical resection. Medical management is to a great extent undefined, and therefore for patients with regional and distal metastases, the development of targeted therapies is greatly desired. In this review, we will summarize clinically relevant biomarkers (e.g., SMARCB1, CA125, dysadherin, and others) with respect to targeted therapeutic opportunities. We will also examine the role of EGFR, mTOR, and polykinase inhibitors (e.g., sunitinib) in the management of local and disseminated disease. Toward building a consortium of pharmaceutical, academic, and non-profit collaborators, we will discuss the state of resources for investigating ES with respect to cell line resources, tissue banks, and registries so that a roadmap can be developed toward effective biology-driven therapies.

  5. Epithelioid hemangioendothelioma of the spine. Report of two cases.

    LENUS (Irish Health Repository)

    Aquilina, Kristian

    2012-02-03

    Epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin. The authors describe two cases of spinal EH, one involving the T-10 vertebra and the second involving the upper cervical spine. In the first case the patient underwent resection of the tumor; this case represents the longest reported follow-up period for spinal EH. In the second case, extensive involvement of C-2, C-3, and C-4 as well as encasement of both vertebral arteries precluded safe tumor resection, and posterior occipitocervical stabilization was performed. The patient subsequently died of metastatic disease. The findings in these two cases underscore the difficulty in predicting the clinical behavior of spinal EH based solely on histological and clinical features as well as the uncertainty of the roles of surgery, chemotherapy, and radiotherapy in the oncological management of a spinal tumor for which clinical data are very limited.

  6. Epithelioid sarcoma: clinical, MR imaging and pathologic findings

    International Nuclear Information System (INIS)

    Hanna, S.L.; Kaste, S.; Jenkins, J.J.; Hewan-Lowe, K.; Spence, J.V.; Gupta, M.; Monson, D.; Fletcher, B.D.

    2002-01-01

    Objective. To report and describe the MR imaging features of eight new cases of this rare soft tissue sarcoma and correlate them with the clinical and histologic findings.Design and patients. Retrospective analysis was carried out for the MR imaging characteristics and histologic findings of eight patients with pathologically proven epithelioid sarcoma and the literature was reviewed. Findings were correlated in each case with the patient's clinical presentation and eventual outcome.Results. The patients, whose primary tumors ranged from 2.5 cm to 19 cm in maximum dimension, were 1 to 90 years of age. Tumors involved the extremities (n=5), the scalp (n=2) and the paraspinal muscles (n=1). Five tumors presented as well-defined, frequently painful, deeply situated masses and three as subcutaneous nodules or cutaneous ulcers with no palpable mass. Four patients had associated regional lymphadenopathy and one had distant metastases at diagnosis. MR imaging showed tumor infiltration of adjacent tissues in seven patients. Signal characteristics reflected varying degrees of cellularity, and the presence of necrosis, hemorrhage, fibrosis, hyalinization and inflammation. Bone marrow involvement was demonstrated in one patient. Clinical outcomes were generally poor.Conclusions. Epithelioid sarcoma is an aggressive soft tissue sarcoma with a varied clinical presentation, growth pattern, MR signal characteristics and histologic picture. The tumor favors the distal extremities and is commonly infiltrative and accompanied by enlarged regional lymph nodes. This neoplasm may present as an intramuscular mass but should also be suspected in patients with ulcerating cutaneous nodules with or without regional lymphadenopathy. (orig.)

  7. Ultrastructural characteristics of type A epithelioid cells during BCG-granulomatosis and treatment with lysosomotropic isoniazid.

    Science.gov (United States)

    Shkurupii, V A; Kozyaev, M A; Nadeev, A P

    2006-04-01

    We studied BCG-granulomas, their cellular composition, and ultrastructure of type A epithelioid cells in the liver of male BALB/c mice with spontaneous granulomatous inflammation. The animals received free isoniazid or isoniazid conjugated with lysosomotropic intracellularly prolonged matrix (dialdehyde dextran, molecular weight 65-75 kDa). Lysosomotropic isoniazid was accumulated in the vacuolar apparatus of epithelioid cells and produced a stimulatory effect on plastic processes in these cells.

  8. MINI REVIEW ARTICLE:Immunohistochemistry of Epithelioid Soft Tissue Sarcomas, Literature Review Based on Case Studies

    Directory of Open Access Journals (Sweden)

    Megha Joshi

    2012-07-01

    Full Text Available Neoplasms with epithelioid histology may be diagnostically challenging. Immunohisto chemistry (IHC can aid in confirming thedifferential diagnosis of mesotheliomas, melanomas, lymphomas, and soft tissue sarcomas, all tumors that can present with an epithelioid histology. Immunohistochemistry can also assist in confirming the type of sarcomas. Using cases diagnosed in acommunity hospital setting over a ten year period, the use of IHC in sarcomas will be illustrated.

  9. Primary sclerosing cholangitis and disease distribution in inflammatory bowel disease.

    LENUS (Irish Health Repository)

    O'Toole, Aoibhlinn

    2012-04-01

    The relationship between site of intestinal inflammation and primary sclerosing cholangitis (PSC) development in inflammatory bowel disease (IBD) has not been studied extensively, but may be important in understanding the pathogenesis of PSC. We aimed to determine patterns of disease distribution in IBD patients with and without PSC.

  10. Sclerosing cholangitis with ulcerative colitis in a Nigerian woman ...

    African Journals Online (AJOL)

    Primary Sclerosing Cholangitis (PSC) is a relatively rare cause of chronic liver disease worldwide. It presents as chronic cholestasis associated with jaundice and pruritus. We report a middle aged Nigerian woman who presented with cholestatic jaundice and diagnosed with PSC with concurrent ulcerative colitis based on ...

  11. Epithelioid hemangioma of bone: radiologic and magnetic resonance imaging characteristics with histopathological correlation

    Energy Technology Data Exchange (ETDEWEB)

    Schenker, Kathleen; Blumer, Steven [Nemours/Alfred I. duPont Hospital for Children, Department of Radiology, Wilmington, DE (United States); Jaramillo, Diego [Nicklaus Children' s Hospital, Department of Radiology, Miami, FL (United States); Treece, Amanda L. [University of Colorado School of Medicine, Departments of Pathology and Laboratory Medicine, Children' s Hospital Colorado, Aurora, CO (United States); Bhatia, Aashim [Monroe Carell Jr. Children' s Hospital at Vanderbilt, Department of Radiology, Nashville, TN (United States)

    2017-11-15

    Epithelioid hemangioma is a rare vascular tumor that can occur in soft tissues or bone. The tumor is part of a spectrum of vascular tumors that also includes epithelioid hemangioendothelioma and angiosarcoma. When involving the bone, the tumor usually involves the metaphysis or diaphysis of the long tubular bones and most commonly occurs in adults. It has been rarely reported in pediatric patients, and in these reported patients, the tumor primarily involves the epiphysis. To review three cases of epithelioid hemangioma of bone occurring in pediatric patients involving the epiphysis and to explore the imaging features of this tumor. Retrospectively review three cases of epithelioid hemangioma occurring in skeletally immature patients. These tumors primarily involved the epiphyses or epiphyseal equivalent bones. One lesion was centered in the metaphysis but extended to the epiphysis. These are three cases presenting in an unusual location and at an unusual age. Epithelioid hemangioma, though rare, can occur in pediatric patients and appears to involve the epiphyses in these patients. This is in contrast to the usual age and location reported. Epithelioid hemangioma may be considered for an epiphyseal lesion in a skeletally immature patient. (orig.)

  12. Blood flow and blood volume in a transplanted rat fibrosarcoma

    International Nuclear Information System (INIS)

    Tozer, G.M.; Morris, C.C.

    1990-01-01

    Blood flow measurements following i.v. infusion of iodi-antipyrine labelled with 14 C ( 14 C-IAP) and blood volume measurements following i.v. injection of 125 I human serum albumin and 51 Cr-labelled red blood cells were made in a transplanted rat fibrosarcoma for comparison with various normal tissues. The tumour-blood partition co-efficient for 14 C-IAP w as found to be 0.79 ± 0.07 which is similar to most of the normal tissues studied. The solubility of 14 C-IAP in plasma was found to be higher than that in whole blood. Blood flow to tumours 3 was found to be 17.9 ± 4.0 ml blood 100 g tissue -1 xmin -1 . These values were considered to be primarily measurements of nutritive flow. Blood in the tumours was found to occupy around 1% of the tissue space which was similar to that found for normal muscle and skin. There was no direct correlation between % blood volume and blood flow for the different tissues studied. Th haematocrit of blood contained in tumour tissue was calculated to be significantly lower than that of blood contained in the normal tissues. It was suspected that permeability of tumour blood vessel walls to 125 I-HSA could have accounted for this difference. (author). 41 refs.; 2 figs.; 3 tabs

  13. CT and MRI Findings of Soft Tissue Adult Fibrosarcoma in Extremities

    Directory of Open Access Journals (Sweden)

    Hexiang Wang

    2018-01-01

    Full Text Available Objective. To characterize and evaluate CT and MRI features of extremity soft tissue adult fibrosarcoma. Methods. CT and MRI images from 10 adult patients with pathologically proven fibrosarcomas were retrospectively analyzed with regard to tumor location, size, number, shape, margins, attenuation, signal intensity, and enhancement patterns on MR images. Additionally, the relationships between lesions, deep fascia, and change in adjacent bones were also assessed. Results. Nineteen tumor lesions in 10 patients were selected for this study. Eighteen lesions were lobulated and one was oval in shape. Most cases were located under the deep fascia, including seven cases that had a nodular lump adjacent to the deep fascia and one case that had broken lesion through the deep fascia. On CT, the adult fibrosarcomas mostly showed iso-attenuated soft tissue masses (n=6. On MRI, all the cases (n=9 displayed low signal on T1-weighted imaging (T1WI and heterogeneous low and high intensity signals on T2-weighted imaging (T2WI, with band-like areas of low signal on both T1WI and T2WI. On contrast-enhanced MRI images, three cases showed heterogeneous peripheral enhancement and one case demonstrated a spoke-wheel-like enhancement. Eight cases showed muscle edema signals in the peritumoral muscle and one case involved adjacent bone. Conclusion. CT and MR imaging have respective advantages in diagnosing adult fibrosarcoma. Combined application of CT and MR is recommended for patients with suspected adult fibrosarcoma.

  14. Sclerosing lobular hyperplasia of breast: cytomorphologic and histomorphologic features: a case report

    Directory of Open Access Journals (Sweden)

    Kapur Payal

    2006-04-01

    Full Text Available Abstract Background Mammary sclerosing lobular hyperplasia is an uncommon benign lesion of adolescent and young women. Fine-needle aspiration cytology of mammary sclerosing lobular hyperplasia is said to show characteristic features that include an absence of stromal fragments. Case presentation In this article, we describe a case of sclerosing lobular hyperplasia that occurred in the right breast of a 12-year-old girl. Fine-needle aspiration cytology showed some fibroadenoma-like features including the presence of stromal fragments, while branched tubular fragments were not seen. The diagnosis of sclerosing lobular hyperplasia was made on histologic examination that showed preserved acinar architecture with lobular hyperplasia and sclerosis of intralobular and interlobular stroma. Conclusion Fine-needle aspiration cytology features of mammary sclerosing lobular hyperplasia are not diagnostic and overlap with those of fibroadenoma; however, a distinction between the two benign entities is of no clinical significance. The definitive diagnosis of sclerosing lobular hyperplasia requires histopathologic evaluation.

  15. Epithelioid hemangioendothelioma – A rare pulmonary tumor

    Directory of Open Access Journals (Sweden)

    Sandra Saleiro

    2008-05-01

    Full Text Available The authors report a case of a rare pulmonary neoplasm – epithelioid hemangioendothelioma, in a 39 year-old woman, asymptomatic until December 2003, when she developed pleuritic and right-sided chest pain. The patient presented a previous chest radiograph, performed 13 years before, which showed multiple small bilateral pulmonary nodules attributed to tuberculous sequelae. The definitive histological diagnosis was made by lung biopsy through thoracotomy. The patient developed a clinical and imagiological worsening and then therapy with interferon α-2a was started. Even with imagiological stability of pulmonary lesions the patient remained symptomatic and died nine months after the diagnosis had been established.The authors emphasise the rarity of this type of pulmonary neoplasm and discuss its clinical presentation, histological features, treatment and prognosis. Resumo: Os autores descrevem um caso de uma neoplasia pulmonar rara – hemangioendotelioma epitelióide – numa doente do sexo feminino, de 39 anos, assintomática até Dezembro de 2003, altura em que apresentou toracalgia direita de características pleuríticas. A doente era portadora de uma radiografia torácica antiga, efectuada há 13 anos, que revelava múltiplos pequenos nódulos pulmonares bilaterais, atribuídos a sequelas de tuberculose pulmonar. O diagnóstico histológico definitivo foi efectuado através de biópsia pulmonar por toracotomia. Dado a doente ter apresentado agravamento clínico e imagiológico foi iniciada terapêutica com interferão α-2a. Apesar da estabilidade imagiológica das lesões pulmonares, a doente manteve-se sintomática e faleceu nove meses depois do diagnóstico ter sido estabelecido.Os autores realçam a raridade deste tipo de neoplasia pulmonar e discutem a sua apresentação clínica, características histológicas, tratamento e prognóstico. Key-words: Epithelioid

  16. A case of immunoglobulin G-4 related sclerosing disease mimicking lung cancer

    International Nuclear Information System (INIS)

    Kwon, Soo Hee; Lee, Young Kyung; Shim, Mi Suk; Lee, Hyang Im

    2013-01-01

    Immunoglobulin (Ig) G4-related sclerosing disease is a recently described systemic fibro-inflammatory disease associated with an elevated circulating level of IgG4 and extensive IgG4-positive lymphoplasmacytic infiltration, resulting in sclerosing inflammation involving various body organs. We experienced one case where surgery confirmed IgG4-related sclerosing disease as a solitary lung mass mimicking lung cancer. We report radiologic findings including chest computed tomography and positron emission tomography computed tomography, with clinical manifestations of IgG4-related sclerosing disease.

  17. Case report 347: Dedifferentiated chondrosarcoma: Grade 4 fibrosarcoma arising in grade 1 chandrosarcoma (femur)

    International Nuclear Information System (INIS)

    Frassica, F.J.; Unni, K.K.; Sim, F.H.

    1986-01-01

    In summary, a 74-year-old woman presented with a 2-week history of pain in the left thigh. Dedifferentiated lesions have a very poor prognosis - less than 10% 5-year survivals. This figure contrasts with the 5-year survival rate of 59% for individuals with conventional chondrosarcoma. Fibrosarcoma is less common than osteosarcoma as the highly malignant, anaplastic portion of a dedifferentiated chondrosarcoma. In an unpublished series of 79 cases of dedifferentiated chondrosarcoma from the Mayo Clinic 43 showed osteosarcoma and 33 fibrosarcoma (Table 3). (orig.)

  18. CT guided puncture aspiration and sclerosing treatment of ovary cyst

    International Nuclear Information System (INIS)

    Peng Yongjun; Du Xiumei; Yuan Jinrong; Chen Chanqing

    2007-01-01

    Objective: To analyze the method and the curative effect with CT guided percutaneous puncture aspiration and sclerosing treatment of ovary cyst. Method: 22 ovary cysts in 22 patients were treated with percutaneous puncture aspiration and underwent repeated sclerotherapy with 99.7% ethanol injection. Among the 22 patients, 18 patients had solitary ovary cyst and was aspirated with an 18-22G gauge aspiration needle. The amount of aspirated fluid varied from 30ml-500ml and 25%-30% cyst volume was replaced by appropriate ethanol Post treatment follow-up were achieved every 3 months. Results All the Punctures were successfully completed. During the 3 months to one year follow-up, 16 ovary cyst disappeared, 6 cysts were small over 50%, without main complication. Conclusion CT guided percutaneous puncture aspiration and sclerosing treatment of ovary cyst is a treatment of choice because of its safety, low complication, and high curative effect. (authors)

  19. Sclerosing stromal tumor of the ovary: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Hyun Koo; Koh, Byung Hee; Rhim, Hyun Chul; Cho, On Koo; Kim, Yong Soo; Hahm, Chang Kok [School of Medicine, Hanyang Univ., Seoul (Korea, Republic of)

    2002-07-01

    Sclerosing stromal tumor of the ovary is a rare benign neoplasm, with distinctive clinical and pathologic features. It occurs predominantly in females during the second and third decades of life. Histologically, it is composed of cellular and acellular collagenized areas, and edematous stromal areas, and at ultrasonography and computed tomography is seen as a distinctive mixed solid and cystic mass lesion. We report a case of sclerosing stromal tumor of the ovary in a 15-year-old girl with a history of menorrhagia since menarche. Ultrasonography revealed the tumor as a well-defined, lobulated, heterogenous echogenic pelvic mass, while at CT, a huge pelvic mass 9 x 9 x 10 cm in size, was seen. This comprised a well-enhanced internal solid portion, a capsule, septa, and a non-enhanced cystic portion.

  20. IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    Allon Kahn

    2015-01-01

    Full Text Available IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

  1. Radiation diagnosis of patients with primary sclerosing cholangitis

    International Nuclear Information System (INIS)

    Sharipov, V.Sh.

    2001-01-01

    Results of combined examination of patients for the purpose of diagnosis of primary sclerosing cholangitis (PSC) are presented. Combined examination consisted of the following techniques: ultrasonography, routine X-ray contrast study of upper section of digestive system, relaxation duodenography, percutaneous transhepatic cholangiography, computerized tomography, endoscopic retrograde pancreatocholangiography. Peculiarities in X-ray PSC semiotics were revealed. It is shown that the combined examination and X-ray semiotics of the disease is of great significance for PSC preoperational diagnosis [ru

  2. Secondary sclerosing cholangitis in critically ill patients: current perspectives

    Directory of Open Access Journals (Sweden)

    Gudnason HO

    2017-06-01

    Full Text Available Hafsteinn O Gudnason,1 Einar S Björnsson1,2 1Division of Gastroenterology and Hepatology, Department of Internal Medicine, Landspitali, University Hospital of Iceland, 2Faculty of Medicine, University of Iceland, Reykjavik, Iceland Abstract: Secondary sclerosing cholangitis (SSC is a term used for a group of chronic cholestatic disease affecting the intra- and/or extrahepatic biliary tree with inflammation and progressive stricture formation, which can lead to biliary cirrhosis. A newly recognized form of SSC is secondary sclerosing cholangitis in critically ill patients (SSC-CIP. Pathogenesis is believed to involve ischemic injury of intrahepatic bile ducts associated with prolonged hypotension, vasopressors administration, and/or mechanical ventilation in patients treated in the intensive care unit (ICU. Patients diagnosed with SSC-CIP have no prior history of liver disease and no known pathologic process or injury responsible for bile duct obstruction prior to ICU treatment. Reasons leading to ICU treatment are many including multitrauma, burn injury, cardiac surgery, severe pneumonia, other infections, or bleeding after abdominal surgery. Patients have in common prolonged ICU admission. SSC-CIP is associated with rapid progression to liver cirrhosis and poor survival with limited treatment options except a liver transplantation. Transplant-free survival is around 17–40 months, which is lower than in other SSC patients. During the initial stages of the disease, the clinical symptoms and biochemical profile are not specific and easily missed. Biliary casts formation may be considered pathognomonic for SSC-CIP since most patients have them in early stages of the disease. Increased awareness and early detection of the disease and its complications is considered to be crucial to improve the poor prognosis. Keywords: secondary sclerosing cholangitis, SSC-CIP, chronic cholestatic disease, sclerosing cholangitis

  3. Diagnosis of IgG4-related sclerosing cholangitis

    Science.gov (United States)

    Nakazawa, Takahiro; Naitoh, Itaru; Hayashi, Kazuki; Miyabe, Katsuyuki; Simizu, Shuya; Joh, Takashi

    2013-01-01

    IgG4-related sclerosing cholangitis (IgG4-SC) is often associated with autoimmune pancreatitis. However, the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis (PSC), and the presence of segmental stenosis suggests cholangiocarcinoma (CC). IgG4-SC responds well to steroid therapy, whereas PSC is only effectively treated with liver transplantation and CC requires surgical intervention. Since IgG4-SC was first described, it has become a third distinct clinical entity of sclerosing cholangitis. The aim of this review was to introduce the diagnostic methods for IgG4-SC. IgG4-SC should be carefully diagnosed based on a combination of characteristic clinical, serological, morphological, and histopathological features after cholangiographic classification and targeting of a disease for differential diagnosis. When intrapancreatic stenosis is detected, pancreatic cancer or CC should be ruled out. If multiple intrahepatic stenoses are evident, PSC should be distinguished on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining. Associated inflammatory bowel disease is suggestive of PSC. If stenosis is demonstrated in the hepatic hilar region, CC should be discriminated by ultrasonography, intraductal ultrasonography, bile duct biopsy, and a higher cutoff serum IgG4 level of 182 mg/dL. PMID:24282356

  4. Epithelioid variant of malignant peripheral nerve sheath tumor (malignant schwannoma) of the urinary bladder.

    Science.gov (United States)

    Eltoum, I A; Moore, R J; Cook, W; Crowe, D R; Rodgers, W H; Siegal, G P

    1999-10-01

    Sarcoma represents less than 2% of all neoplasms diagnosed or recognized in effusions. Epithelioid peripheral nerve sheath tumor is a rare tumor that is difficult to differentiate from other epithelioid tumors without the use of ancillary studies. A 39-year-old paraplegic man presented with hematuria and a bladder mass that extended to involve the pelvic peritoneum. Light microscopy using hematoxylin-eosin, Papanicolaou, and immunohistochemical stains as well as transmission electron microscopy showed features of epithelioid malignant peripheral nerve sheath tumor with rhabdoid features and an accompanying eosinophilic infiltrate. Cytologic smears confirmed the similarities between the primary tumor in the bladder and the cells in the pelvic fluid and excluded the possibility of reactive changes related to postsurgical radiation. Ancillary studies were critical in narrowing the differential diagnoses and reaching the final conclusion.

  5. Radiation-induced fibrosarcoma of the mandible following treatment for bilateral retinoblastoma

    Energy Technology Data Exchange (ETDEWEB)

    Ferlito, A; Recher, G [Padua Univ. (Italy); Tomazzoli, L

    1979-10-01

    A case of fibrosarcoma of the mandible following radiotherapy for bilateral retinoblastoma and occurring in an 11-year-old female child is described. After a clinical description of the case, reported with histological documentation, problems connected with the pathogenesis of the malignancies are dealt with. Besides irradiation, genetic mutation as a carcinogenetic co-factor is taken into consideration. (author).

  6. Hemipelvectomy with laparoscopic abdominoperineal excision for epithelioid sarcoma treatment

    Directory of Open Access Journals (Sweden)

    Daniel Paulino Santana

    2017-07-01

    Full Text Available The epithelioid sarcoma (ES is an unusual variant of the sarcoma, not reaching 1% of all soft tissue sarcomas. Initially reported in 1970, it has a more aggressive variant, which was classified as the “proximal-type” in 1997. These are aggressive tumors with high rates of distant metastasis and local relapses. Isolated radio and chemotherapy responses are poor and free-margin surgical resection is the treatment of choice.This is the case report of a 25-year-old male patient diagnosed with “proximal-type” ES in the perineal region. He underwent surgical resection in another institution and was later admitted to our institution with local recurrence with rectal and left thigh muscle invasion. Neoadjuvant radio-chemotherapy was performed, followed by laparoscopic abdominoperineal rectal resection and partial left hemipelvectomy associated with left lower-limb amputation. The patient had no postoperative complications and is currently undergoing outpatient follow-up. The anatomopathological analysis showed tumor-free margins.The “proximal-type” ES is a rare soft-tissue sarcoma subtype. High local recurrence, as well as the metastasis rates, makes its treatment a challenging task. Resumo: O sarcoma epitelióide (SE é um subtipo incomum de sarcoma, não chegando a 1% dos sarcomas de partes moles. Foi inicialmente descrito em 1970, sendo que em 1997 uma variante mais agressiva foi classificada como “tipo proximal”. São tumores agressivos, com altas taxas de metástases e recidiva local, com resposta pobre à quimioterapia e radioterapia isoladas. A ressecção cirúrgica com margens livres é o tratamento padrão.Apresentamos o caso de um paciente de 25 anos com SE tipo proximal avançado em região perineal, submetido a ressecção em outro serviço, evoluindo com recidiva local com invasão do canal anal e musculatura da coxa esquerda. Foi realizado tratamento radioquimioterápico neoadjuvante seguido de ressecção cirúrgica com

  7. Low-Grade Uterine Epithelioid Hemangioendothelioma Presented as a Submucosal Leiomyoma during Labor

    Directory of Open Access Journals (Sweden)

    Anastasios V. Koutsopoulos

    2013-01-01

    Full Text Available With the exception of leiomyomas, soft tissue tumors of the uterine corpus are not common. This is particularly true for vascular neoplasms, with the epithelioid hemangioendothelioma being a curiosity; not more than twenty-two cases of malignant hemangioendotheliomas have been reported in the literature so far, all of which were high-grade hemangioendotheliomas (hemangiosarcomas. We present herewith a unique case of low-grade epithelioid hemangioendothelioma of the uterus in a pregnant woman aged 29 years. The clinical, histological, and immunohistochemical characteristics of this entity, together with its differential diagnosis, are discussed.

  8. Response rate of fibrosarcoma cells to cytotoxic drugs on the expression level correlates to the therapeutic response rate of fibrosarcomas and is mediated by regulation of apoptotic pathways

    International Nuclear Information System (INIS)

    Lehnhardt, Marcus; Mueller, Oliver; Klein-Hitpass, Ludger; Kuhnen, Cornelius; Homann, Heinz Herbert; Daigeler, Adrien; Steinau, Hans Ulrich; Roehrs, Sonja; Schnoor, Laura; Steinstraesser, Lars

    2005-01-01

    Because of the high resistance rate of fibrosarcomas against cytotoxic agents clinical chemotherapy of these tumors is not established. A better understanding of the diverse modes of tumor cell death following cytotoxic therapies will provide a molecular basis for new chemotherapeutic strategies. In this study we elucidated the response of a fibrosarcoma cell line to clinically used cytostatic agents on the level of gene expression. HT1080 fibrosarcoma cells were exposed to the chemotherapeutic agents doxorubicin, actinomycin D or vincristine. Total RNA was isolated and the gene expression patterns were analyzed by microarray analysis. Expression levels for 46 selected candidate genes were validated by quantitative real-time PCR. The analysis of the microarray data resulted in 3.309 (actinomycin D), 1.019 (doxorubicin) and 134 (vincristine) probesets that showed significant expression changes. For the RNA synthesis blocker actinomycin D, 99.4% of all differentially expressed probesets were under-represented. In comparison, probesets down-regulated by doxorubicin comprised only 37.4% of all genes effected by this agent. Closer analysis of the differentially regulated genes revealed that doxorubicin induced cell death of HT1080 fibrosarcoma cells mainly by regulating the abundance of factors mediating the mitochondrial (intrinsic) apoptosis pathway. Furthermore doxorubicin influences other pathways and crosstalk to other pathways (including to the death receptor pathway) at multiple levels. We found increased levels of cytochrome c, APAF-1 and members of the STAT-family (STAT1, STAT3), while Bcl-2 expression was decreased. Caspase-1, -3, -6, -8, and -9 were increased indicating that these proteases are key factors in the execution of doxorubicin mediated apoptosis. This study demonstrates that chemotherapy regulates the expression of apoptosis-related factors in fibrosarcoma cells. The number and the specific pattern of the genes depend on the used cytotoxic drug

  9. Sclerosing polycystic adenosis of the salivary gland: a report of 16 cases.

    NARCIS (Netherlands)

    Gnepp, D.R.; Wang, L.J.; Brandwein-Gensler, M.; Slootweg, P.J.; Gill, M.; Hille, J.

    2006-01-01

    Sclerosing polycystic adenosis is a recently described, extremely rare, reactive, sclerosing, inflammatory process somewhat similar to fibrocystic changes and adenosis tumor of the breast. To date, there have been 22 cases described in the literature. Because of the infrequency of this lesion, we

  10. Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings

    Energy Technology Data Exchange (ETDEWEB)

    Itoh, S., E-mail: shigekimiyo@luck.ocn.ne.j [Department of Technical Radiology, Nagoya University School of Health Sciences, Nagoya (Japan); Nagasaka, T. [Department of Pathology, Nagoya University Graduate School of Medicine, Nagoya (Japan); Suzuki, K.; Satake, H.; Ota, T.; Naganawa, S. [Department of Radiology, Nagoya University Graduate School of Medicine, Nagoya (Japan)

    2009-11-15

    Aim: To assess the clinical, computed tomography (CT), and pathological findings in patients with lymphoplasmacytic sclerosing cholangitis. Materials and methods: Fifteen consecutive patients (four women and 11 men, mean age 71 years) with lymphoplasmacytic sclerosing cholangitis and without the characteristic features of underlying disorders causing benign biliary strictures were retrospectively recruited. Two radiologists evaluated multiphase contrast-enhanced CT images acquired with 0.5 or 1-mm collimation. One pathologist performed all histological examinations, including IgG4 immunostaining. Results: The intrahepatic biliary ducts showed dilatation in all 15 patients, but only seven presented with jaundice. Although laboratory data were not available in all patients, serum gammaglobulin and IgG levels were elevated in five of six patients and six of eight patients, respectively. Anti-nuclear antibody was detected in three of six patients. The involved biliary ducts showed the following CT findings: involvement of the hilar biliary duct (14/15), a mean wall thickness of 4.9 mm, a smooth margin (10/15), a narrow but visible lumen (6/15), hyper-attenuation during the late arterial phase (9/15), homogeneous hyper-attenuation during the delayed phase (11/11), and no vascular invasion (14/15). Abnormal findings in the pancreas and urinary tract were detected in eight of 15 patients. In 13 patients with adequate specimens, moderate to severe lymphoplasmacytic infiltration associated with dense fibrosis was observed. Infiltration of IgG4-positive plasma cells was moderate or severe in nine patients and minimal or absent in four patients. Conclusion: Lymphoplasmacytic sclerosing cholangitis exhibits relatively characteristic clinical and CT findings, although they are not sufficiently specific for differentiation from other biliary diseases.

  11. Frequency and importance of radiologically visible coronary scleroses

    International Nuclear Information System (INIS)

    Hoyer, B.

    1981-01-01

    The importance of radiologically visible coronary sceleroses for an early diagnosing of coronary heart disease was investigated. In 3 mixed collectives of patients with and without coronary heart diseases who had had a coronary angiography were examined retrospectively for coronary sclerosis (group I: standard films, group II: old X-ray findings, group III: fluoroscopy before beginning coronary angiography). In the retrospective evaluation, the sensitivity to the recognition of coronary heart disease was low. With prospective examination by means of fluoroscopy, coronary calcification could be proven in 37.66% of the patients with coronary diseases (sensitivity); the specificity (no coronary calcification in persons without coronary disease) was 99%. The duration and extent of a calcification do not show a definite influence on the severity of the disease. Several coronary scleroses in one or several vessels mostly indicate a vascular disease. Coronary scleroses are not necessarily located in the same point as coronary stenoses: in 85.5% of the patients with coronary sclerosis of the left truncus, haemodynamically important stenoses were found only in following vascular regions. Considering the high specificity of coronary scleroses in the fluoroscopic picture and the high sensitivity under favourable conditions, this method seems to be suitable as a screening method for early recognition of coronary heart diseases in the asymptomatic stage. A proven coronary sclerosis should in any case be the cause for continuing the search for a coronary heart disease. The radiation exposure during fluoroscopy of coronary sclerosis is low if the adjustment is correct, it takes little time, the patient is not put to discomfort. (orig./MG) [de

  12. Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings

    International Nuclear Information System (INIS)

    Itoh, S.; Nagasaka, T.; Suzuki, K.; Satake, H.; Ota, T.; Naganawa, S.

    2009-01-01

    Aim: To assess the clinical, computed tomography (CT), and pathological findings in patients with lymphoplasmacytic sclerosing cholangitis. Materials and methods: Fifteen consecutive patients (four women and 11 men, mean age 71 years) with lymphoplasmacytic sclerosing cholangitis and without the characteristic features of underlying disorders causing benign biliary strictures were retrospectively recruited. Two radiologists evaluated multiphase contrast-enhanced CT images acquired with 0.5 or 1-mm collimation. One pathologist performed all histological examinations, including IgG4 immunostaining. Results: The intrahepatic biliary ducts showed dilatation in all 15 patients, but only seven presented with jaundice. Although laboratory data were not available in all patients, serum gammaglobulin and IgG levels were elevated in five of six patients and six of eight patients, respectively. Anti-nuclear antibody was detected in three of six patients. The involved biliary ducts showed the following CT findings: involvement of the hilar biliary duct (14/15), a mean wall thickness of 4.9 mm, a smooth margin (10/15), a narrow but visible lumen (6/15), hyper-attenuation during the late arterial phase (9/15), homogeneous hyper-attenuation during the delayed phase (11/11), and no vascular invasion (14/15). Abnormal findings in the pancreas and urinary tract were detected in eight of 15 patients. In 13 patients with adequate specimens, moderate to severe lymphoplasmacytic infiltration associated with dense fibrosis was observed. Infiltration of IgG4-positive plasma cells was moderate or severe in nine patients and minimal or absent in four patients. Conclusion: Lymphoplasmacytic sclerosing cholangitis exhibits relatively characteristic clinical and CT findings, although they are not sufficiently specific for differentiation from other biliary diseases.

  13. Epithelioid sarcoma and squamous cell carcinoma arising in a burn scar

    Directory of Open Access Journals (Sweden)

    Kusum D Jashnani

    2011-01-01

    Full Text Available Development of a malignant tumor is a well known complication of a chronic burn scar. Most of these tumors are squamous cell carcinomas and only 28 cases of burn scar sarcomas have been reported in literature. We report the first occurrence of the combination of squamous cell carcinoma and epithelioid sarcoma arising in a burn scar.

  14. Epithelioid angiomatosis in the acquired immunodeficiency syndrome: morphology and differential diagnosis

    NARCIS (Netherlands)

    Walford, N.; van der Wouw, P. A.; Das, P. K.; ten Velden, J. J.; Hulsebosch, H. J.

    1990-01-01

    A rare vascular proliferation found as a skin lesion in patients suffering from the acquired immunodeficiency syndrome and sometimes referred to as epithelioid angiomatosis is believed to be a manifestation of infection by the cat scratch bacillus or a related organism. We describe the histological

  15. Proximal-type epithelioid sarcoma: a new case report and literature ...

    African Journals Online (AJOL)

    Proximal-type epithelioid sarcoma is a rare soft tissue neoplasm which arises from the more proximal part of body and occurs more often in young people; the definite diagnosis depends mainly on the pathological examination; early detection and complete excision remain the foundation of treatment. Due to its aggressive ...

  16. Epithelioid Angiomyolipoma – a case report and review of the literature

    LENUS (Irish Health Repository)

    Keane, F

    2017-08-01

    Herein we present the case of a 43-year-old female in whom a left renal mass was identified incidentally on imaging performed for staging of a newly diagnosed breast carcinoma. The mass was resected and histologic examination and immunohistochemistry confirmed a diagnosis of epithelioid angiomyolipoma

  17. Proximal-Type Epithelioid Sarcoma: Report of an Unusual Case in the Uterine Cervix.

    Science.gov (United States)

    Suárez-Zamora, David Alfonso; Barrera-Herrera, Luis Eduardo; Rodríguez-Urrego, Paula Andrea; Palau-Lázaro, Mauricio Alfonso

    2017-08-01

    Epithelioid sarcoma is a rare malignant mesenchymal neoplasm (less than 1% of all sarcomas) with epithelioid morphology. Among the 2 subtypes, proximal represents only one-third of cases and commonly involves deep tissues of pelvic region, including the perineum, genital area, and groin, and occurs more frequently in older patients who present a more aggressive course. In the female genital tract, proximal-type epithelioid sarcoma (PES) mainly affects the vulva and is extremely uncommon in the uterus. To our knowledge, only a few cases of PES involving the cervix and uterine body have been previously reported in the literature. We report a 23-year-old woman who presented with abnormal vaginal bleeding. She was found to have a cervical mass, which was resected and diagnosed as a hemangioendothelioma. However, 2 months later, the mass recurred and the histopathological analysis at our institution demonstrated a PES confined to the uterine cervix. It is important to include this neoplasm in the differential diagnosis of epithelioid tumors that can involve the female genital tract because it has a significant impact on prognosis and treatment.

  18. Differentiating immunoglobulin g4-related sclerosing cholangitis from hilar cholangiocarcinoma.

    Science.gov (United States)

    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.

  19. Computed tomographic findings of early subacute sclerosing panencephalitis

    International Nuclear Information System (INIS)

    Pedersen, H.; Wulff, C.H.; Rigshospitalet, Copenhagen

    1982-01-01

    Computed tomography of the brain (CT) was carried out at the early stages of subacute sclerosing panencephalitis (SSPE) in three children. The lateral ventricles were very small and the hemispheric sulci and interhemispheric fissures were not visible in all three patients in contrast to severe atrophy found at a later stage in one patient. The early CT abnormalities were revealed at the same time as the titres of measles antibodies in blood and cerebrospinal fluid were elevated, and the characteristic periodic complexes in the electroencephalogram established the diagnosis of SSPE. The CT changes indicating brain swelling reflect the reactive changes of this slow virus infection. (orig.)

  20. An 111In-Pentetreotide Positive Sclerosing Pneumocytoma.

    Science.gov (United States)

    Savelli, Giordano; Bnà, Claudio; Zambelli, Claudia; Illuminati, Sonia; Bonello, Luke

    2017-04-01

    A 43-year-old woman had an incidental lung mass identified on shoulder x-ray performed for pain. Contrast-enhanced CT showed a 38-mm mass in the medial segment of the right middle lobe, with features suggestive of carcinoid tumor. A In-pentetreotide scan showed intense uptake; furthermore, fine needle aspiration biopsy yielded neuroendocrine cells confirming the carcinoid hypothesis. However, definitive surgical histology showed a sclerosing pneumocytoma. This could potentially suggest that such rare tumors, with metastatic potential, could respond to somatostatin analogue treatment.

  1. A Recurrence of Bilateral Diffuse Sclerosing Lobular Hyperplasia of Breast: A Case Report.

    Science.gov (United States)

    Elfituri, Osama; Sonawane, Snehal; Xu, Haoliang; Warso, Michael A; Wiley, Elizabeth

    2017-12-01

    Mammary sclerosing lobular hyperplasia is an uncommon benign fibroproliferative lesion of adolescent and young women, often of African American heritage with an incidence of ~3%. Patients generally complain of a palpable, painless, or slightly tender and well-defined lump in breast. Very rarely, this lesion may be bilateral and diffuse. The definitive diagnosis of sclerosing lobular hyperplasia requires histopathologic evaluation. Here, we describe a case of diffuse sclerosing lobular hyperplasia in a 29-year-old African American woman that required bilateral mastectomy and recurred bilaterally requiring second resections. This appears to be the first report of this phenomenon.

  2. Altered Cyclosporine Absorption in a Patient with Ulcerative Colitis, Sclerosing Cholangitis and Pancreatic Insufficiency

    Directory of Open Access Journals (Sweden)

    Mark G Swain

    1991-01-01

    Full Text Available Pancreatic insufficiency leading to altered cyclosporine absorption is reported in a 37-year-old man with ulcerative colitis and sclerosing cholangitis. Asymptomatic chronic pancreatitis occurs frequently in patients with ulcerative colitis, and even more commonly when there is coexistent sclerosing cholangitis. However, pancreatic insufficiency has been documented in only one patient previously with ulcerative colitis and sclerosing cholangitis. Pancreatic function testing can help to identify the complex etiology of malabsorption in these patients and is recommended in patients when liver transplantation is contemplated, as pancreatic insufficiency may alter the absorption of cyclosporine.

  3. Abnormal 67Ga uptake in a fibrosarcoma of the pericardium with malignant transformation

    International Nuclear Information System (INIS)

    Hayashida, Kohei; Nishimura, Tsunehiko; Kumita, Shin-ichirou; Uehara, Toshiisa; Imakita, Masami

    1990-01-01

    A 31-year-old woman presented with general malaise, back pain, and edema of the lower extremities. A chest X-ray film showed an enlarged cardiac shadow and clear lung fields. A pericardial lesion with decreased activity on blood pool imaging and increased uptake on gallium citrate Ga67 imaging displaced the heart upwards and to the left. The pericardial mass showed an inhomogeneous signal intensity on MRI and was large enough to obstruct the venous return by compressing the heart. At operation, the mass was found to originate from the pericardium and was histologically identified as a malignant fibrosarcoma. Twelve years before, the patient had undergone an operation for the removal of a pericardial tumor which was histologically identified as a benign hemangioma. In view of the rarity of pericardial tumors, the present tumor is suspected to have undergone a transformation from benign hemangioma to malignant fibrosarcoma. (orig.)

  4. Etoposide Incorporated into Camel Milk Phospholipids Liposomes Shows Increased Activity against Fibrosarcoma in a Mouse Model

    Directory of Open Access Journals (Sweden)

    Hamzah M. Maswadeh

    2015-01-01

    Full Text Available Phospholipids were isolated from camel milk and identified by using high performance liquid chromatography and gas chromatography-mass spectrometry (GC/MS. Anticancer drug etoposide (ETP was entrapped in liposomes, prepared from camel milk phospholipids, to determine its activity against fibrosarcoma in a murine model. Fibrosarcoma was induced in mice by injecting benzopyrene (BAP and tumor-bearing mice were treated with various formulations of etoposide, including etoposide entrapped camel milk phospholipids liposomes (ETP-Cam-liposomes and etoposide-loaded DPPC-liposomes (ETP-DPPC-liposomes. The tumor-bearing mice treated with ETP-Cam-liposomes showed slow progression of tumors and increased survival compared to free ETP or ETP-DPPC-liposomes. These results suggest that ETP-Cam-liposomes may prove to be a better drug delivery system for anticancer drugs.

  5. Case of fibrosarcoma of the thyroid gland. Many years after radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Nakamoto, Yasuhiko; Nagasako, Ren [Shimane Central Hospital, Shimane (Japan); Tasaka, Yasuyuki; Iwasaki, Hiroshi; Fujita, Kayoko

    1983-12-01

    Naturally occurring fibrosarcoma of the thyroid gland is very rare. Only 5 cases of this tumor have been reported in Japan. Recently, we diagnosed a thyroid fibro sarcoma in a 46-year-old woman. It started as a papillary cystadenoma 26 years ago, followed by the appearance of papillary adenocarcinoma 6 years later. At that time most of the tumor was removed, except for a small portion which had invaded the trachea, and 5,000 rads of /sup 60/Co was given postoperatively to her neck. The tumor was quiescent for 18 years and then fibrosarcoma appeared in the same region. We think that this tumor may be a radiation-induced sarcoma.

  6. INI Expressing Epithelioid Sarcoma with Osteoclastic Giant Cells in a Child: A Case Report with Summary of Prior Published Cases.

    Science.gov (United States)

    Bhattacharyya, Riju; Ghosh, Ranajoy; Saha, Koushik; Chatterjee, Uttara

    2017-08-01

    Epithelioid sarcoma is a heterogeneous tumor with 2 subtypes, classic and proximal. The proximal variant is more aggressive and occurs in proximal location in young adults. We present a proximal epithelioid sarcoma in the leg of an 8 year old girl with rhabdoid morphology and scattered osteoclastic giant cells. Nuclear INI-1 was retained. Despite wide local excision, local recurrence occurred at 8 months. Following re-excision, she developed a chest wall metastasis after 9 months. Epithelioid sarcoma, proximal type with osteoclastic giant cells in the pediatric age group has not been reported previously and should be considered in the differential diagnoses of tumors with epithelioid cell morphology and scattered osteoclastic giant cells. Retained INI expression helped to differentiate this tumor from malignant rhabdoid tumor.

  7. Hepatic fibrosarcoma incarcerated in a peritoneopericardial diaphragmatic hernia in a cat

    Directory of Open Access Journals (Sweden)

    Michael Linton

    2016-03-01

    Full Text Available Case summary A 14-year-old, female neutered domestic shorthair presented for dyspnoea. Thoracic ultrasonography and radiography showed that a heterogeneous mass was present within the pericardial sac, and the mass continued caudally with the mesenteric fat. On CT, the outline of the diaphragm was not continuous and there was an obvious defect with diaphragmatic thickening present at the mid-level of the liver. A pleural effusion and a small-volume pericardial effusion were also present. A ventral midline coeliotomy and median sternotomy revealed a 5 × 6 × 7 cm firm, irregular, tan-coloured soft tissue mass within the pericardial sac attached to both the diaphragmatic defect and liver. The mass was carefully dissected away from the heart and the diaphragmatic defect was repaired with primary closure. Postoperatively, the cat had a persistent pneumothorax that required continuous pleural suction for 41 h. The cat died 44 h postoperatively. Histopathology and immunohistochemistry confirmed the mass to be a hepatic fibrosarcoma incarcerated in a peritoneopericardial diaphragmatic hernia (PPDH. Relevance and novel information This is the first reported case of metaplastic transformation of liver into a sarcoma in a cat with PPDH. In addition, hepatic fibrosarcoma is a rarely reported location for fibrosarcoma in this species.

  8. Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis.

    Science.gov (United States)

    Moon, Sung-Hoon; Kim, Myung-Hwan; Lee, Jong Kyun; Baek, Seunghee; Woo, Young Sik; Cho, Dong Hui; Oh, Dongwook; Song, Tae Jun; Park, Do Hyun; Lee, Sang Soo; Seo, Dong Wan; Lee, Sung Koo

    2017-04-01

    Recent research has shown that a substantial number of patients with primary sclerosing cholangitis (PSC) can also have elevated serum/tissue IgG4. The aim of our study was to develop a simple scoring system for the discrimination of IgG4-related sclerosing cholangits (IgG4-SC) from PSC. Patients with IgG4-SC (n = 39) and PSC (n = 76) who had intrahepatic/hilar strictures were included. Candidate-differentiating variables included patient age, other organ involvement (OOI), inflammatory bowel disease, serum IgG4, and cholangiographic features. A scoring system was developed on the basis of these variables, and its performance was internally validated using a bootstrapping-based method. The scoring system in the final model included age (IgG4-SC. The discrimination between IgG4-SC and PSC using the scoring system was excellent (area under the receiver operating characteristic curve, 0.986). A reliable differentiation of IgG4-SC from PSC can be made using the scoring system presented here. We suggest the diagnosis of IgG4-SC at a cutoff of 7 points or higher and the indication of diagnostic steroid trial at 5 or 6 points. External validation of our scoring system is warranted.

  9. Therapeutic tests on methylcholanthrene-induced fibrosarcoma and transplanted fibrosarcoma of the mouse - clostridial oncolysis and its enhancement by high-frequency hyperthermia and X-rays

    International Nuclear Information System (INIS)

    Pirlet, T.

    1982-01-01

    Tests aimed at a conditioning of tumours by means of x-ray irradiation and high-frequency hyperthermia. Methyl cholanthrene-induced fibrosarcoma (IMCM) and its transplant version (T-IMCM) produced at the neck of NMRI-mice at a critical localization immediately next to vital organs, served as a test system since it is almost resistant to simple oncolysis. Individual therapy components were compared for their mutual enhancement of efficacy in 16 test groups, and the triple combination (Cl + X-ray + HFH) was tested in relapse application as well. A 2000-R-x-ray irradiation was followed by almost simultaneous 4 min HF heating and i.v. spore administration at 12 h intervals. Assessment criteria were tumour growth, survival rates and times, absence of recidivity and healing, analysis of post-mortem findings and dimension or duration of lyses. In addition, the two tumour types of induction and transplant tumour were compared. The typical course of clostridial oncolysis can be confirmed in principle. Results show that it is possible by means of additional physical methods as were applied, to increase the systemically apathogenic and selective therapy principle of rather negligible oncolysis to over 80% (IMCM) or 97% (T-IMCM). Concerning the MCA fibrosarcoma which is very resistant to clostridial oncolysis in this mouse-tumour model, marked prolongations of survival or healing are possible even in extremely critical localization. In principle, this provides the preconditions for including oncolysis in clinical studies in human medicine. (orig./MG) [de

  10. MMP-2 is a disease-modifying gene in primary sclerosing cholangitis

    NARCIS (Netherlands)

    Korkmaz, Kerem Sebib; de Rooij, Bert-Jan F.; van Hoek, Bart; Janse, Marcel; Coenraad, Minneke J.; van der Reijden, Johan J.; Weersma, Rinse K.; Porte, Robert J.; Voorneveld, Philip W.; Baranski, Andrzej G.; Verspaget, Hein W.

    BackgroundPrimary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the bile ducts, frequently necessitating orthotopic liver transplantation (OLT), often accompanied by inflammatory bowel disease (IBD). Matrix metalloproteinases (MMPs) are associated with fibrotic diseases caused by

  11. Mesenteric ossification in CT indicates sclerosing peritonitis in chronic bacterial infection and pancreatitis

    International Nuclear Information System (INIS)

    Kirchner, J.; Kirchner, E.M.; Kickuth, R.; Stein, A.

    2004-01-01

    Sclerosing peritonitis already has been described as a serious complication of the continuous ambulatory peritoneal dialysis. But different other affections of the pertioneum such as chronic bacterial peritonitis and pancreatitis may result in sclerosing peritonitis, too. The symptom is characterised by thickened small bowel walls and periotoneal membranes as well as peritoneal calcifications which can be shown in computed tomography. We demonstrate two cases of peritoneal ossifications due to peritonitis and pancreatitis. (orig.) [de

  12. Hepatic perivascular epithelioid cell tumor (PEComa: a case report with a review of literatures

    Directory of Open Access Journals (Sweden)

    Hyun-Jin Son

    2017-03-01

    Full Text Available Hepatic perivascular epithelioid cell tumors (PEComas are very rare. We report a primary hepatic PEComa with a review of the literature. A 56-year-old women presented with a nodular mass detected during the management of chronic renal failure and chronic hepatitis C. Diagnostic imaging studies suggested a nodular hepatocellular carcinoma in segment 5 of the liver. The patient underwent partial hepatectomy. A brown-colored expansile mass measuring 3.2×3.0 cm was relatively demarcated from the surrounding liver parenchyma. The tumor was mainly composed of epithelioid cells that were arranged in a trabecular growth pattern. Adipose tissue and thick-walled blood vessels were minimally identified. A small amount of extramedullary hematopoiesis was observed in the sinusoidal spaces between tumor cells. Tumor cells were diffusely immunoreactive for human melanoma black 45 (HMB45 and Melan A, focally immunoreactive for smooth muscle actin, but not for hepatocyte specific antigen (HSA.

  13. HMB-45 negative clear cell perivascular epithelioid cell tumor of the skin.

    Science.gov (United States)

    Pusiol, Teresa; Morichetti, Doriana; Zorzi, Maria Grazia; Dario, Surace

    2012-01-01

    The first case of cutaneous clear cell perivascular epithelioid cell tumor (PEComa) with negative HMB-45 marker is presented. The tumor was a nodule 3x2 cm in size, located on the right foot in a 60-year-old man. The lesion consisted of large irregularly shaped cells with clear cytoplasm, negative for S-100 protein, HMB-45, Melan-A, pancytokeratin, epithelial membrane antigen and CAM5.2. Multifocal positivity for desmin, microphthalmia transcription factor and tyrosinase was found. The diagnosis of cutaneous PEComa of clear cell type was made. Clear cell change is a very unusual finding in PEComa and may pose problems in diagnostic differentiation from other clear cell cutaneous lesions that may be excluded with immunohistochemistry. In our case, the HMB-45 negativity may be explained by extensive clear cell change. Additional studies are necessary to accept the clear cell cutaneous HMB-45 negative PEComa as a new variant of perivascular epithelioid cell tumor.

  14. Sentinel node biopsy as an adjunct to limb salvage surgery for epithelioid sarcoma of the hand

    Directory of Open Access Journals (Sweden)

    Hammond Alex

    2005-06-01

    Full Text Available Abstract Background Epithelioid sarcomas of the hand are rare, high-grade tumors with a propensity for regional lymphatic spread approaching 40%. Case presentation A 54-year-old male with an epithelioid sarcoma of the palm was treated with neoadjuvant radiation, wide excision, and two-stage reconstruction. Sentinel lymph node biopsy was used to stage the patient's axilla. Sentinel node biopsy results were negative. The patient has remained free of local, regional and distant disease for the follow-up time of 16 months. Conclusion The rarity of this tumor makes definitive conclusions difficult but SLN biopsy appears to be a useful adjunct in the treatment of these sarcomas.

  15. Molecular and radiological diagnosis of sclerosing bone dysplasias

    International Nuclear Information System (INIS)

    Hul, Wim van; Vanhoenacker, Filip; Balemans, Wendy; Janssens, Katrien; Schepper, A.M. de

    2001-01-01

    Bone mineral density (BMD) is a quantitative trait for which the heritability of the variance is estimated to be up to 80%, based on epidemiological and twin studies. Further illustration of the involvement of genetic factors in bone homeostasis, is the existence of an extended group of genetic conditions associated with an abnormal bone density. The group of conditions with increased bone density has long been poorly studied and understood at the molecular genetic level but recently, thanks to recent developments in molecular genetics and genomics, for some of them major breakthroughs have been made. These findings will make the molecular analysis of such patients an additional tool in diagnostics and in genetic counseling. However, the initial identification of affected patients is still largely dependent upon recognition of clinical and radiological stigmata of the disease. Therefore, in this overview of sclerosing bone dysplasias, the classical clinical and radiological signs of this group of disorders will be discussed along with the new molecular insights

  16. Molecular and radiological diagnosis of sclerosing bone dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Hul, Wim van E-mail: vhul@uia.ac.be; Vanhoenacker, Filip; Balemans, Wendy; Janssens, Katrien; Schepper, A.M. de

    2001-12-01

    Bone mineral density (BMD) is a quantitative trait for which the heritability of the variance is estimated to be up to 80%, based on epidemiological and twin studies. Further illustration of the involvement of genetic factors in bone homeostasis, is the existence of an extended group of genetic conditions associated with an abnormal bone density. The group of conditions with increased bone density has long been poorly studied and understood at the molecular genetic level but recently, thanks to recent developments in molecular genetics and genomics, for some of them major breakthroughs have been made. These findings will make the molecular analysis of such patients an additional tool in diagnostics and in genetic counseling. However, the initial identification of affected patients is still largely dependent upon recognition of clinical and radiological stigmata of the disease. Therefore, in this overview of sclerosing bone dysplasias, the classical clinical and radiological signs of this group of disorders will be discussed along with the new molecular insights.

  17. Radiologic bone changes of polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy

    International Nuclear Information System (INIS)

    Maekelae, P.; Virtama, P.

    1982-01-01

    More than 50 cases of polycystic lipomembranous osteodysplasia (PLO) with sclerosing leukoencephalopathy (SL) have been described in Finland, Sweden, Japan, and in the USA. Radiographic bone changes, including symmetrical cystic lesions in the small bones of the extremities and trabecular loss in the distal ends of the long tubular bones, represent primary abnormalities in the diagnosis of the disease. Neuropsychiatric symptoms, frontal syndrome, and pyramidal signs make the patients dangerous to themselves. They are often involved in traffic accidents are prone to multiple spontaneous or almost spontaneous fractures. PLO usually starts with slight bone pain around the age of 20 years. Progress is very slow during the next ten years, but faster after the age of 40 years. The patients usually die before the age of 50 years having total dementia and epileptiform convulsions. (orig.)

  18. Trigger mechanisms of secondary sclerosing cholangitis in critically ill patients.

    Science.gov (United States)

    Leonhardt, Silke; Veltzke-Schlieker, Wilfried; Adler, Andreas; Schott, Eckart; Hetzer, Roland; Schaffartzik, Walter; Tryba, Michael; Neuhaus, Peter; Seehofer, Daniel

    2015-03-31

    In recent years the development of secondary sclerosing cholangitis in critically ill patients (SSC-CIP) has increasingly been perceived as a separate disease entity. About possible trigger mechanisms of SSC-CIP has been speculated, systematic investigations on this issue are still lacking. The purpose of this study was to evaluate the prevalence and influence of promoting factors. Temporality, consistency and biological plausibility are essential prerequisites for causality. In this study, we investigated the temporality and consistency of possible triggers of SSC-CIP in a large case series. Biological plausibility of the individual triggers is discussed in a scientific context. SSC-CIP cases were recruited retrospectively from 2633 patients who underwent or were scheduled for liver transplantation at the University Hospital Charité, Berlin. All patients who developed secondary sclerosing cholangitis in association with intensive care treatment were included. Possible trigger factors during the course of the initial intensive care treatment were recorded. Sixteen patients (68% males, mean age 45.87 ± 14.64 years) with a confirmed diagnosis of SSC-CIP were identified. Of the 19 risk factors investigated, particularly severe hypotension with a prolonged decrease in mean arterial blood pressure (MAP) to <65 mmHg and systemic inflammatory response syndrome (SIRS) were established as possible triggers of SSC-CIP. The occurrence of severe hypotension appears to be the first and most significant step in the pathogenesis. It seems that severe hypotension has a critical effect on the blood supply of bile ducts when it occurs together with additional microcirculatory disturbances. In critically ill patients with newly acquired cholestasis the differential diagnosis of SSC-CIP should be considered when they have had an episode of haemodynamic instability with a prolonged decrease in MAP, initial need for large amounts of blood transfusions or colloids, and early

  19. Pelvic pain in a young patient: Sclerosing stromal tumor

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    Huriye Ayşe Parlakgümüş

    2013-03-01

    Full Text Available Introduction: Sclerosing stromal tumors are rare, benign sex chord stromal tumors. They are usually unilateral and are seen in second or third decades. The complaint at admission may be menstrual irregularity, pelvic pain, palpable pelvic mass, precocious puberty and postmenopausal bleeding. Because the complaint at admission and radiological findings are not specific to SSTs preoperative diagnosis is challenging. Herein we present the sonographical, intraoperative and histopathological findings of a SST diagnosed during laparoscopy in a patient who admitted with chronic pelvic pain and received pelvic inflammatory disease and endometriosis treatment and differential diagnosis of SSTs with the other ovarian tumors. Case report: 24 years old nulliparous patient first admitted to the gynecology department with the complaint of foul smelling vaginal discharge and pelvic pain. The diagnosis was pelvic inflammatory disease and the patient received antibiotics. The pelvic examination was normal except the mass in the right ovary which had similar echogenity to the ovary. Because of the pelvic pain the mass was assumed to be an endometrioma and the patient was prescribed an oral contraceptive treatment for 3 months. Because of the persistent pelvic pain a diagnostic laparoscopy was performed which revealed a 2 cm, pinkish- white, exophytic lesion originating from the right ovary. Pathological examination reported the mass to be a sclerosing stromal tumor. After the treatment the patient no longer complained of vaginal discharge but pelvic pain still persisted. After the operation the patient no longer complained of pelvic pain. Conclusion: Although SSTs are rare, they should be kept in mind when a young patient admits with menstrual irregularity, pelvic pain and hirsutism, particularly if the pain is refractory to treatment.

  20. Urothelial carcinoma of urinary bladder with exclusive heterologous component of epithelioid rhabdomyosarcoma at metastatic site.

    Science.gov (United States)

    Agarwal, Poojan; Pasricha, Sunil; Gupta, Gurudutt; Sharma, Anila; Mehta, Anurag

    2018-01-01

    Urothelial carcinoma of urinary bladder with divergent differentiation into rhabdomyosarcoma (RMS) is an extremely uncommon aggressive phenomenon. We present a case of a 74-year-old male with bladder carcinoma which metastasized to the abdominal wall as epithelioid RMS. To the best knowledge of our literature searches, an oligometastasis of exclusive heterologous component has not been described before. The clinical, radiological, and immunohistochemistry profile of the patient supported the monoclonal nature of the tumor.

  1. Epithelioid inflammatory myofibroblastic sarcoma responsive to surgery and an ALK inhibitor in a patient with panhypopituitarism.

    Science.gov (United States)

    Kurihara-Hosokawa, Kotomi; Kawasaki, Isao; Tamai, Anna; Yoshida, Yoko; Yakushiji, Yosuke; Ueno, Hiroki; Fukumoto, Mariko; Fukushima, Hiroko; Inoue, Takeshi; Hosoi, Masayuki

    2014-01-01

    We encountered a case of epithelioid inflammatory myofibroblastic sarcoma (EIMS) originating from an abdominal organ that rapidly regrew twice. The patient underwent two surgeries. Large tumors grew within three months after the second surgery. The patient subsequently received chemotherapy with an anaplastic lymphoma kinase (ALK) inhibitor. Although EIMS has a poor prognosis, the patient continues to be alive with disease 14 months after surgical treatment and the administration of the ALK inhibitor.

  2. A 3-dimensional in vitro model of epithelioid granulomas induced by high aspect ratio nanomaterials

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    Hurt Robert H

    2011-05-01

    Full Text Available Abstract Background The most common causes of granulomatous inflammation are persistent pathogens and poorly-degradable irritating materials. A characteristic pathological reaction to intratracheal instillation, pharyngeal aspiration, or inhalation of carbon nanotubes is formation of epithelioid granulomas accompanied by interstitial fibrosis in the lungs. In the mesothelium, a similar response is induced by high aspect ratio nanomaterials, including asbestos fibers, following intraperitoneal injection. This asbestos-like behaviour of some engineered nanomaterials is a concern for their potential adverse health effects in the lungs and mesothelium. We hypothesize that high aspect ratio nanomaterials will induce epithelioid granulomas in nonadherent macrophages in 3D cultures. Results Carbon black particles (Printex 90 and crocidolite asbestos fibers were used as well-characterized reference materials and compared with three commercial samples of multiwalled carbon nanotubes (MWCNTs. Doses were identified in 2D and 3D cultures in order to minimize acute toxicity and to reflect realistic occupational exposures in humans and in previous inhalation studies in rodents. Under serum-free conditions, exposure of nonadherent primary murine bone marrow-derived macrophages to 0.5 μg/ml (0.38 μg/cm2 of crocidolite asbestos fibers or MWCNTs, but not carbon black, induced macrophage differentiation into epithelioid cells and formation of stable aggregates with the characteristic morphology of granulomas. Formation of multinucleated giant cells was also induced by asbestos fibers or MWCNTs in this 3D in vitro model. After 7-14 days, macrophages exposed to high aspect ratio nanomaterials co-expressed proinflammatory (M1 as well as profibrotic (M2 phenotypic markers. Conclusions Induction of epithelioid granulomas appears to correlate with high aspect ratio and complex 3D structure of carbon nanotubes, not with their iron content or surface area. This model

  3. Pulmonary Epithelioid Hemangioendothelioma in a Patient with Crohn’s Disease

    Directory of Open Access Journals (Sweden)

    Nanda Ramchandar

    2015-01-01

    Full Text Available Pulmonary epithelioid hemangioendothelioma (PEH is a rare neoplasm, largely unresponsive to chemotherapeutic medications, and with varied prognosis. Imaging on computerized tomography may demonstrate perivascular nodules, but diagnosis is ultimately made on biopsy with immunohistochemical analysis. Here we describe a case of PEH in a 14-year-old male with Crohn’s disease, which, to our knowledge, has not previously been described in the literature.

  4. Nuclear grade and necrosis predict prognosis in malignant epithelioid pleural mesothelioma: a multi-institutional study.

    Science.gov (United States)

    Rosen, Lauren E; Karrison, Theodore; Ananthanarayanan, Vijayalakshmi; Gallan, Alexander J; Adusumilli, Prasad S; Alchami, Fouad S; Attanoos, Richard; Brcic, Luka; Butnor, Kelly J; Galateau-Sallé, Françoise; Hiroshima, Kenzo; Kadota, Kyuichi; Klampatsa, Astero; Stang, Nolween Le; Lindenmann, Joerg; Litzky, Leslie A; Marchevsky, Alberto; Medeiros, Filomena; Montero, M Angeles; Moore, David A; Nabeshima, Kazuki; Pavlisko, Elizabeth N; Roggli, Victor L; Sauter, Jennifer L; Sharma, Anupama; Sheaff, Michael; Travis, William D; Vigneswaran, Wickii T; Vrugt, Bart; Walts, Ann E; Tjota, Melissa Y; Krausz, Thomas; Husain, Aliya N

    2018-04-01

    A recently described nuclear grading system predicted survival in patients with epithelioid malignant pleural mesothelioma. The current study was undertaken to validate the grading system and to identify additional prognostic factors. We analyzed cases of epithelioid malignant pleural mesothelioma from 17 institutions across the globe from 1998 to 2014. Nuclear grade was computed combining nuclear atypia and mitotic count into a grade of I-III using the published system. Nuclear grade was assessed by one pathologist for three institutions, the remaining were scored independently. The presence or absence of necrosis and predominant growth pattern were also evaluated. Two additional scoring systems were evaluated, one combining nuclear grade and necrosis and the other mitotic count and necrosis. Median overall survival was the primary endpoint. A total of 776 cases were identified including 301 (39%) nuclear grade I tumors, 354 (45%) grade II tumors and 121 (16%) grade III tumors. The overall survival was 16 months, and correlated independently with age (P=0.006), sex (0.015), necrosis (0.030), mitotic count (0.001), nuclear atypia (0.009), nuclear grade (<0.0001), and mitosis and necrosis score (<0.0001). The addition of necrosis to nuclear grade further stratified overall survival, allowing classification of epithelioid malignant pleural mesothelioma into four distinct prognostic groups: nuclear grade I tumors without necrosis (29 months), nuclear grade I tumors with necrosis and grade II tumors without necrosis (16 months), nuclear grade II tumors with necrosis (10 months) and nuclear grade III tumors (8 months). The mitosis-necrosis score stratified patients by survival, but not as well as the combination of necrosis and nuclear grade. This study confirms that nuclear grade predicts survival in epithelioid malignant pleural mesothelioma, identifies necrosis as factor that further stratifies overall survival, and validates the grading system across multiple

  5. A case of a resectable single hepatic epithelioid hemangioendothelioma with characteristic imaging by ADC map.

    Science.gov (United States)

    Okano, Hiroshi; Nakajima, Hideki; Tochio, Tomomasa; Suga, Daisuke; Kumazawa, Hiroaki; Isono, Yoshiaki; Tanaka, Hiroki; Matsusaki, Shimpei; Sase, Tomohiro; Saito, Tomonori; Mukai, Katsumi; Nishimura, Akira; Matsushima, Nobuyoshi; Baba, Youichirou; Murata, Tetsuya; Hamada, Takashi; Taoka, Hiroki

    2015-12-01

    A 47-year-old woman with a single-nodule hepatic tumor was referred to our hospital. She had no symptoms. The tumor was located at the surface of the right lobe of the liver; it showed peripheral low signal intensity on a magnetic resonance imaging apparent diffusion coefficient (ADC) map, and an influx of blood flow into the peripheral area of the tumor at the early vascular phase on perflubutane microbubble (Sonazoid(®)) contrast-enhanced (CE) ultrasonography. Since we suspected a malignant tumor, the patient underwent surgical resection. The hepatic tumor was resected curatively. Pathological examination revealed that the tumor was composed of epithelioid cells with an epithelioid structure and/or cord-like structure. Immunohistochemical staining was positive for cluster of differentiation 34 and factor VIII-related antigen. Based on the above, a final diagnosis of hepatic epithelioid hemangioendothelioma (EHE) was made. Hepatic EHE is a rare hepatic tumor: only a few cases of hepatic EHE with curative resection have been reported. We were unable to reach a diagnosis of hepatic EHE by imaging studies; however, an ADC map was useful in showing the malignant potential of the tumor, and CE ultrasonography was useful in revealing the peripheral blood flow of the tumor. When an unusual hepatic mass is encountered, hepatic EHE should be kept in mind, and the mass should be inspected with more than one imaging modality, including an ADC map, in the process of differential diagnosis.

  6. Giant gastrointestinal stromal tumour of rare sarcomatoid epithelioid subtype: Case study and literature review

    Science.gov (United States)

    Lech, Gustaw; Korcz, Wojciech; Kowalczyk, Emilia; Guzel, Tomasz; Radoch, Marcin; Krasnodębski, Ireneusz Wojciech

    2015-01-01

    Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal tumours of the gastrointestinal tract, but they represent less than 3% of all gastrointestinal tract malignancies. This is a detailed case study of a 52-year-old male patient treated for very uncommon histological subtype of gastric GIST with atypical clinical presentation, asymptomatic progress and late diagnosis. The resected tumour, giant in diameters, was confirmed to represent the most rare histopathologic subtype of GISTs - sarcomatoid epithelioid GIST. We report this case and review the literature with a special focus on pathomorphological evaluation, biological aggressiveness and prognostic factors. To our knowledge this is the first report of giant GIST of very uncommon sarcomatoid epithelioid subtype. It is concluded that clinicians should pay attention to the fact that initial diagnosis may be delayed due to mildly asymptomatic and non-specific clinical presentation. Asymptomatic tumours diagnosed at a late stage, which is often the case, can be large on presentation. Prognosis for patients diagnosed with GIST depend on tumour size, mitotic rate, histopathologic subtype and tumour location. That is why early diagnosis and R0 resection, which is usually feasible and safe even in giant gastric sarcomatoid epithelioid subtype of GISTs, are the key factors for further treatment and good prognosis. PMID:25805949

  7. Sclerosing cholangitis with autoimmune pancreatitis versus primary sclerosing cholangitis: comparison on endoscopic retrograde cholangiography, MR cholangiography, CT, and MRI

    Energy Technology Data Exchange (ETDEWEB)

    Kim; Jin Hee; Byun, Jae Ho; Kim, So Yeon; Lee, Seung Soo; Kim, Hyoung Jung; Lee, Moon-Gyu [Dept. of Radiology and Research Inst. of Radiology, Univ. of Ulsan Coll. of Medicine, Asan Medical Center, Seoul (Korea, Republic of)], e-mail: jhbyun@amc.seoul.kr; Kim, Myung-Hwan [Dept. of Internal Medicine, Univ. of Ulsan Coll. of Medicine, Asan Medical Center, Seoul (Korea, Republic of)

    2013-07-15

    Background: It is essential to differentiate sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) from primary sclerosing cholangitis (PSC) as the treatment and prognosis of the two diseases are totally different. Purpose: To compare image findings of SC-AIP and PSC on endoscopic retrograde cholangiography (ERC), magnetic resonance cholangiography (MRC), computed tomography (CT), and magnetic resonance imaging (MRI). Material and Methods: Two radiologists retrospectively reviewed ERC, MRC, CT, and MRI in 28 SC-AIP and 23 PSC patients in consensus. Factors evaluated included the length, location, and multiplicity of bile duct stricture, the presence of characteristic cholangiographic features of PSC on ERC and MRC, and the presence, location, thickness, and pattern of bile duct wall thickening on CT and MRI. Results: On ERC, focal stricture, multifocal and intrahepatic bile duct stricture, and beaded, pruned-tree, and diverticulum-like appearance were more frequent in PSC than in SC-AIP patients (P = 0.006). On MRC, multifocal and intrahepatic bile duct stricture and pruned-tree appearance were more frequent in PSC than in SC-AIP patients (P = 0.044). On CT and MRI, the bile duct wall was thicker (5.1 mm vs. 3.1 mm; P = 0.033 and 4.3 mm vs. 3.0 mm; P = 0.01, respectively) in SC-AIP than in PSC patients. PSC was more frequently associated with intrahepatic bile duct wall thickening on both CT (93% vs. 50%; P = 0.024) and MRI (100% vs. 50%; P = 0.023) than SC-AIP. Conclusion: The combination of ERC or MRC with cross-sectional images, including CT and MRI, may be helpful in differentiating between SC-AIP and PSC.

  8. Sclerosing cholangitis with autoimmune pancreatitis versus primary sclerosing cholangitis: comparison on endoscopic retrograde cholangiography, MR cholangiography, CT, and MRI

    International Nuclear Information System (INIS)

    Kim; Jin Hee; Byun, Jae Ho; Kim, So Yeon; Lee, Seung Soo; Kim, Hyoung Jung; Lee, Moon-Gyu; Kim, Myung-Hwan

    2013-01-01

    Background: It is essential to differentiate sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) from primary sclerosing cholangitis (PSC) as the treatment and prognosis of the two diseases are totally different. Purpose: To compare image findings of SC-AIP and PSC on endoscopic retrograde cholangiography (ERC), magnetic resonance cholangiography (MRC), computed tomography (CT), and magnetic resonance imaging (MRI). Material and Methods: Two radiologists retrospectively reviewed ERC, MRC, CT, and MRI in 28 SC-AIP and 23 PSC patients in consensus. Factors evaluated included the length, location, and multiplicity of bile duct stricture, the presence of characteristic cholangiographic features of PSC on ERC and MRC, and the presence, location, thickness, and pattern of bile duct wall thickening on CT and MRI. Results: On ERC, focal stricture, multifocal and intrahepatic bile duct stricture, and beaded, pruned-tree, and diverticulum-like appearance were more frequent in PSC than in SC-AIP patients (P = 0.006). On MRC, multifocal and intrahepatic bile duct stricture and pruned-tree appearance were more frequent in PSC than in SC-AIP patients (P = 0.044). On CT and MRI, the bile duct wall was thicker (5.1 mm vs. 3.1 mm; P = 0.033 and 4.3 mm vs. 3.0 mm; P = 0.01, respectively) in SC-AIP than in PSC patients. PSC was more frequently associated with intrahepatic bile duct wall thickening on both CT (93% vs. 50%; P = 0.024) and MRI (100% vs. 50%; P = 0.023) than SC-AIP. Conclusion: The combination of ERC or MRC with cross-sectional images, including CT and MRI, may be helpful in differentiating between SC-AIP and PSC

  9. Epithelioid fibrous histiocytoma: molecular characterization of ALK fusion partners in 23 cases.

    Science.gov (United States)

    Dickson, Brendan C; Swanson, David; Charames, George S; Fletcher, Christopher Dm; Hornick, Jason L

    2018-05-01

    Epithelioid fibrous histiocytoma is a rare and distinctive cutaneous neoplasm. Most cases harbor ALK rearrangement and show ALK overexpression, which distinguish this neoplasm from conventional cutaneous fibrous histiocytoma and variants. SQSTM1 and VCL have previously been shown to partner with ALK in one case each of epithelioid fibrous histiocytoma. The purpose of this study was to examine a large cohort of epithelioid fibrous histiocytomas by next-generation sequencing to characterize the nature and prevalence of ALK fusion partners. A retrospective archival review was performed to identify cases of epithelioid fibrous histiocytoma (2012-2016). Immunohistochemistry was performed to confirm ALK expression. Targeted next-generation sequencing was applied on RNA extracted from formalin-fixed paraffin-embedded tissue to identify the fusion partners. Twenty-three cases fulfilled inclusion criteria. The mean patient age was 39 years (range, 8-74), there was no sex predilection, and >75% of cases involved the lower extremities. The most common gene fusions were SQSTM1-ALK (N=12; 52%) and VCL-ALK (N=7; 30%); the other four cases harbored novel fusion partners (DCTN1, ETV6, PPFIBP1, and SPECC1L). The pattern of ALK immunoreactivity was usually granular cytoplasmic (N=12; 52%) or granular cytoplasmic and nuclear (N=10; 43%); the case containing an ETV6 fusion partner showed nuclear staining alone. There was no apparent relationship between tumor morphology and the ALK fusion partner. In summary, SQSTM1 and VCL are the most common ALK fusion partners in epithelioid fibrous histiocytoma; DCTN1, ETV6, PPFIBP1, and SPECC1L represent rare fusion partners. The proteins encoded by these genes play diverse roles in scaffolding, cell adhesion, signaling, and transcription (among others) without clear commonalities. These findings expand the oncogenic promiscuity of many of these ALK fusion genes, which drive neoplasia in tumors of diverse lineages with widely varied clinical

  10. Antitumor effectiveness of different amounts of electrical charge in Ehrlich and fibrosarcoma Sa-37 tumors

    International Nuclear Information System (INIS)

    Ciria, HC; Quevedo, MS; Cabrales, LB; Bruzón, RP; Salas, MF; Pena, OG; González, TR; López, DS; Flores, JM

    2004-01-01

    In vivo studies were conducted to quantify the effectiveness of low-level direct electric current for different amounts of electrical charge and the survival rate in fibrosarcoma Sa-37 and Ehrlich tumors, also the effect of direct electric in Ehrlich tumor was evaluate through the measurements of tumor volume and the peritumoral and tumoral findings. BALB/c male mice, 7–8 week old and 20–22 g weight were used. Ehrlich and fibrosarcoma Sa-37 cell lines, growing in BALB/c mice. Solid and subcutaneous Ehrlich and fibrosarcoma Sa-37 tumors, located dorsolaterally in animals, were initiated by the inoculation of 5 × 10 6 and 1 × 10 5 viable tumor cells, respectively. For each type of tumor four groups (one control group and three treated groups) consisting of 10 mice randomly divided were formed. When the tumors reached approximately 0.5 cm 3 , four platinum electrodes were inserted into their bases. The electric charge delivered to the tumors was varied in the range of 5.5 to 110 C/cm 3 for a constant time of 45 minutes. An additional experiment was performed in BALB/c male mice bearing Ehrlich tumor to examine from a histolological point of view the effects of direct electric current. A control group and a treated group with 77 C/cm 3 (27.0 C in 0.35 cm 3 ) and 10 mA for 45 min were formed. In this experiment when the tumor volumes reached 0.35 cm 3 , two anodes and two cathodes were inserted into the base perpendicular to the tumor long axis. Significant tumor growth delay and survival rate were achieved after electrotherapy and both were dependent on direct electric current intensity, being more marked in fibrosarcoma Sa-37 tumor. Complete regressions for fibrosarcoma Sa-37 and Ehrlich tumors were observed for electrical charges of 80 and 92 C/cm 3 , respectively. Histopathological and peritumoral findings in Ehrlich tumor revealed in the treated group marked tumor necrosis, vascular congestion, peritumoral neutrophil infiltration, an acute inflammatory

  11. Morphological modulation of human fibrosarcoma HT-1080 cells by hydroxybenzoate compounds during apoptosis

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    Jassem G Mahdi

    2015-10-01

    Full Text Available Hydroxybenzoate (HB compounds have shown to modulate the morphology in human fibrosarcoma HT-1080 cells. The changes in HT-1080 cells showed marker signs of apoptosis, which included the condensation of nucleus, cell round, blebbing and the formation of apoptotic bodies. The different stages of apoptosis were assessed microscopically using different staining and immunohistochemical techniques, as well as scanning electron microscopy. In addition, HB compounds increased the expression of caspase-3, which is closely associated with the development of the modulation in HT-1080 cells that are undergoing the programmed cell death. Both acetyl salicylic acid (ASA and HBZn compounds were dose and treatment duration dependent.

  12. Echocardiographic evaluation of patients with subacute sclerosing panencephalitis

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    Derya Çimen

    2014-03-01

    Full Text Available Objective: Subacute sclerosing panencephalitis is a slowly progressive, inflammatory and neurodegenerative disease caused by virus infection in the central nervous system. Since there are a limited number of studies in the literature evaluating the cardiovascular functions of patients with SSPE, the present study evaluates the patients with SSPE using tissue Doppler echocardiography and compares them between the control group in order to shed some light on the subject. Methods: The study is a prospective observational study. 49 patients (17 female, 32 male with SSPE were included in the study. Patients were divided into two groups: Stage 2 (n=29 and Stage 3 (n=20. Echocardiographic data were compared with a control group of 26 which is the same average age. All children underwent a detailed echocardiography, which contained an M-mode, pulse Doppler and tissue Doppler imaging. Results: Sinus tachycardia ( >100 beats/min in children was detected in nineteen (38.7% patients. There were not significant differences between parameters of systolic and diastolic function of the heart. Stage 2 group, EF: 69.9±6.4; SF: 39.2±5.58; and MPI (mitral: 0.38±0.03 and MPI (tricuspid: 0.39±0.10. And in the Stage 3 group, EF: 68.5±7.0, SF: 37.8±5.34, MPI (mitral: 0.37±0.09 and MPI (tricuspid: 0.38±0.12. In the control group EF:70.96±5.54; SF:39.96±5.05 and MPI(mitral: 0.35±0.06 MPI (tricuspid:0.36±0.04 and statistically meaningful differences were not found between patients and control groups (p >0.05. Conclusion: Cardiac functions may be preserved and cardiac functions constitute no significant risks of mortality in the advanced stages of patients with Subacute sclerosing panencephalitis, which is a group of chronic and bedridden patients.

  13. Clinical and histological study of pituitary fibrosarcoma following radiotherapy for pituitary adenoma

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    Sato, Kazufumi; Hayashi, Minoru; Kubota, Toshihiko; Kawano, Hirokazu; Handa, Yuji [Fukui Medical School, Matsuoka (Japan); Komai, Toshio

    1990-11-01

    A 49-year old male was admitted with a history of radiotherapy for a pituitary adenoma 9 years earlier. Three weeks prior to admission, he noticed visual loss in the left eye. Computed tomography (CT) scan revealed a sellar tumor. The patient underwent craniotomy and the tumor was partially resected. The histological diagnosis was benign pituitary adenoma. Two months after surgery, he began to complain of headache and left hemiparesis. CT scan at that time showed a large parasellar tumor extending into the right temporal lobe. A second craniotomy was performed and a firm tumor was partially removed. Under light microscopy, the tumor was composed of anaplastic spindle cells showing a fascicular pattern. Ultrastructurally, the tumor cells were spindle-shaped with elongated nuclei. The cytoplasm contained numerous distended rough endoplasmic reticula and free ribosomes, Golgi apparatus as well as glycogen granules. Some desmosome-like intercellular adherents were observed. Collagen fibers were scattered in the extracellular space. There was no apparent formation of a basement membrane. These findings suggested a close morphological similarity between tumor cells and fibroblasts, conforming to ultrastructural diagnostic criteria for fibrosarcoma. In spite of intensive treatment, such as a second radiotherapy and subsequent craniotomy, the patient died 9 months after admission. The clinical course and pathological findings of the post-irradiation pituitary fibrosarcoma are discussed. (author).

  14. Enhanced metastatic potential of murine fibrosarcomas treated in vitro with ultraviolet radiation

    International Nuclear Information System (INIS)

    Fisher, M.S.; Cifone, M.A.

    1981-01-01

    The purpose of this study was to determine whether repeated treatment of tumor cells in vitro with mutagenic doses of ultraviolet (UV) radiation could influence the metastatic behavior of these cells in vivo. Three cloned lines of UV-2237, a fibrosarcoma induced in a C3H- mouse by chronic irradiation with UV, and SF-19, a spontaneous C3H- fibrosarcoma, were grown in culture. These cell lines varied from low to high metastatic potential as determined by in vivo tests. The cultures were exposed to UV radiation from an FS40 sunlamp at a dose that killed 40% of the cells. These UV radiation exposures were repeated at 3- to 5-day intervals for a total of 5 treatments. The mutation frequency was analyzed by monitoring the appearance of ouabain-resistant colonies following UV irradiation. With all four tumor lines, the frequency of conversion to ouabain resistance was increased more than 10-fold. Tumor cells given 5 UV radiation treatments and control cultures carried in parallel without exposure to UV radiation were tested for metastatic potential in an in vivo lung colony assay. Cell lines treated in vitro with UV radiation produced more experimental metastases than the counterpart unirradiated cultures. We conclude that, in all four tumor lines, exposure of tumorigenic cells to mutagenic doses of UV radiation can alter their biological behavior and that this may contribute to the progression of tumors from low to high metastatic capability

  15. Variations in primary sclerosing cholangitis across the age spectrum.

    Science.gov (United States)

    Eaton, John E; McCauley, Bryan M; Atkinson, Elizabeth J; Juran, Brian D; Schlicht, Erik M; de Andrade, Mariza; Lazaridis, Konstantinos N

    2017-10-01

    Primary sclerosing cholangitis (PSC) typically develops in middle-age adults. Little is known about phenotypic differences when PSC is diagnosed at various ages. Therefore, we sought to compare the clinical characteristics of a large PSC cohort based on the age when PSC was diagnosed. We performed a multicenter retrospective review to compare the features of PSC among those diagnosed between 1-19 (n = 95), 20-59 (n = 662), and 60-79 years (n = 102). Those with an early diagnosis (ED) of PSC were more likely to have small-duct PSC (13%) than those with a middle-age diagnosis (MD) (5%) and late diagnosis (LD) groups (2%), P early in life are more likely to have small-duct PSC and less likely to have disease-related complications. Clinicians should be vigilant for underlying cholangiocarcinoma among those with PSC diagnosed late in life. © 2017 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

  16. Sclerosing thymoma: case report Timoma esclerosante: relato de caso

    Directory of Open Access Journals (Sweden)

    Gesine Gregorio Siqueira

    2012-08-01

    Full Text Available We present a rare case of thymoma in a 36-year old woman, who was initially diagnosed with severe myasthenia gravis and subsequently undergone surgical resection. During surgery tumor was found at the anterior mediastinum, tightly attached to the phrenic nerve, pleura and pericardium. Histological assessment showed large areas of sclerosis and fibrous collagenous tissue as well as islands of epithelial and lymphoid cells. Sclerosing thymoma, which is a rare subtype of thymoma (Relatamos um caso raro de timoma em uma mulher de 36 anos de idade, com clínica e diagnóstico de miastenia gravis de difícil controle clínico, submetida à ressecção cirúrgica. No intraoperatório, observou-se tumor no mediastino anterior, firmemente aderido ao nervo frênico, à pleura e ao pericárdio. Ao exame histológico, foram evidenciadas extensas áreas de tecido fibrocolagenoso e esclerose, assim como ilhas de células epiteliais e células linfoides. Diagnosticado timoma esclerosante, subtipo raro de timoma (< 1%, sendo este o primeiro caso relatado no Brasil. A paciente apresentou melhora parcial dos sintomas associados à miastenia gravis.

  17. Papillary bile duct dysplasia in primary sclerosing cholangitis.

    Science.gov (United States)

    Ludwig, J; Wahlstrom, H E; Batts, K P; Wiesner, R H

    1992-06-01

    A 62-year-old man with a 20-year history of chronic ulcerative colitis and a 9-year history of primary sclerosing cholangitis (PSC) underwent orthotopic liver transplantation because of symptoms related to PSC and cholangiographic features compatible with a biliary neoplasm. Study of the excised liver revealed papillary mucosal lesions in the common hepatic duct and the right and left hepatic ducts as well as cholangiectases and other features typically associated with PSC. The papillary lesions consisted of abundant fibrovascular stroma covered by biliary epithelium with low-grade and high-grade dysplasia. Some periductal glands were also dysplastic. These features distinguished papillary dysplasia from classic biliary papillomatosis. Only one focus of microinvasion was found; there were no metastases. Among 60 cases of PSC in whom the entire liver could be studied after orthotopic liver transplantation, this was the only instance of unequivocal dysplasia. However, in one specimen, papillary hyperplasia was found. Detailed macroscopic and microscopic rereview of 23 livers from our patients with the longest history of PSC (range, 5-24 years) failed to reveal any additional cases with dysplasia. It is concluded that (a) papillary mucosal lesions in PSC may represent papillary dysplasia without invasion; (b) these lesions may evolve from papillary hyperplasia; (c) the process may be largely, if not entirely, in situ; and (d) the prevalence of dysplasia and carcinoma of bile ducts may be less than the 7%-9% reported in the literature for malignancies associated with PSC.

  18. Uncommon of the uncommon: Malignant Perivascular epithelioid cell tumor of the lung

    International Nuclear Information System (INIS)

    Lim, Hyun Ju; Lee, Ho Yun; Han, Joung Ho; Choi, Yong Soo; Lee, Kyung Soo

    2013-01-01

    A perivascular epithelioid cell (PEC) tumor is a rare mesenchymal tumor characterized by abundant cytoplasmic Periodic acid-Schiff positive glycogen (also called sugar tumor or clear cell tumor of the lung for this characteristic) and is mostly benign. We report a case of a 63-year-old man who presented with an enlarging mass on chest radiograph. After a thorough workup, diagnosis of malignant pulmonary PEC tumor with lung to lung metastases was established. Herein, the difficulties of diagnosis and management we confronted are described.

  19. Fra-1 induces morphological transformation and increases in vitro invasiveness and motility of epithelioid adenocarcinoma cells

    DEFF Research Database (Denmark)

    Kustikova, O.; Kramerov, D.; Grigorian, M.

    1998-01-01

    in vitro nor in vivo. CSML100 possesses all characteristics of a highly progressive carcinoma. These cells do not form tight contacts, are highly invasive in vitro, and are metastatic in vivo. AP-1 activity was considerably higher in CSML100 cells than in CSML0 cells. There was a common predominant Jun...... from tumors of epithelial origin revealed a correlation of fra-1 expression with mesenchymal characteristics of carcinoma cells. Moreover, we show here for the first time that the expression of exogenous Fra-1 in epithelioid cells results in morphological changes that resemble fibroblastoid conversion...

  20. Uncommon of the uncommon: Malignant Perivascular epithelioid cell tumor of the lung

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Hyun Ju; Lee, Ho Yun; Han, Joung Ho; Choi, Yong Soo; Lee, Kyung Soo [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2013-08-15

    A perivascular epithelioid cell (PEC) tumor is a rare mesenchymal tumor characterized by abundant cytoplasmic Periodic acid-Schiff positive glycogen (also called sugar tumor or clear cell tumor of the lung for this characteristic) and is mostly benign. We report a case of a 63-year-old man who presented with an enlarging mass on chest radiograph. After a thorough workup, diagnosis of malignant pulmonary PEC tumor with lung to lung metastases was established. Herein, the difficulties of diagnosis and management we confronted are described.

  1. Radiologic Findings of Immunoglobulin G4 Related Sclerosing Esophagitis: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Mi Sun; Kim, Su Young; Lee, Byung Hoon; Hwang, Yoon Joon; Han, Yoon Hee [Dept. of Radiology, Ilsan Paik Hospital, Inje University College of Medicine, Koyang (Korea, Republic of)

    2012-02-15

    We describe a case of immunoglobulin G4 (IgG4)-related sclerosing esophagitis occurring in a 63-year-old man with progressive dysphagia and 10-kg weight loss over 9 months. An esophagoscopy revealed significant stricture with diffuse mucosal friability and ulceration at mid esophagus level. Barium esophagogram showed diffuse stenosis at the mid and lower esophagus levels with ulcerations and irregularity of the mucosa. Multidetector computed tomography revealed diffuse edematous and circumferential thickening of the submucosa and muscle layer of this esophageal segment. Fluorine 18 fluorodeoxyglucose positron emission tomography (FDG PET) revealed diffuse mild FDG uptake in mid to lower esophagus. Although benign inflammatory lesion was suspected based on the imaging findings, the patient underwent surgery for worsening esophageal stricture and the esophageal lesion was pathologically confirmed as IgG4-related sclerosing esophagitis. Radiologic benignancy and high clinical suspicion for IgG4-related sclerosing disease may help making a proper decision and avoiding unnecessary operation.

  2. Radiologic Findings of Immunoglobulin G4 Related Sclerosing Esophagitis: A Case Report

    International Nuclear Information System (INIS)

    Kim, Mi Sun; Kim, Su Young; Lee, Byung Hoon; Hwang, Yoon Joon; Han, Yoon Hee

    2012-01-01

    We describe a case of immunoglobulin G4 (IgG4)-related sclerosing esophagitis occurring in a 63-year-old man with progressive dysphagia and 10-kg weight loss over 9 months. An esophagoscopy revealed significant stricture with diffuse mucosal friability and ulceration at mid esophagus level. Barium esophagogram showed diffuse stenosis at the mid and lower esophagus levels with ulcerations and irregularity of the mucosa. Multidetector computed tomography revealed diffuse edematous and circumferential thickening of the submucosa and muscle layer of this esophageal segment. Fluorine 18 fluorodeoxyglucose positron emission tomography (FDG PET) revealed diffuse mild FDG uptake in mid to lower esophagus. Although benign inflammatory lesion was suspected based on the imaging findings, the patient underwent surgery for worsening esophageal stricture and the esophageal lesion was pathologically confirmed as IgG4-related sclerosing esophagitis. Radiologic benignancy and high clinical suspicion for IgG4-related sclerosing disease may help making a proper decision and avoiding unnecessary operation.

  3. Sclerosing encapsulating peritonitis in chronic ambulatory peritoneal dialysis;preoperative catheter drainage : a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Tae Hoon [Dankook Univ. Hospital, Seoul (Korea, Republic of)

    1996-10-01

    Sclerosing encapsulating peritonitis is a well recognized, but uncommon, complication of chronic ambulatory peritoneal dialysis. I report a case of sclerosing encapsulating peritonitis in which percutaneous catheter drainage was performed preoperatively. Ultrasonography(US) and computed tomography(CT) showed a large multi-septated cystic mass which occupied nearly all the peritoneal cavity. Percutaneous drainage with two 8.5 French catheters was preoperatively performed under fluoroscopy and about 2100 ml of bloody fluid was drained for 20 days. On follow-up CT, the size of the cyst had significantly decreased and anoperation was performed. It is considered that percutaneous catheter drainage is useful in the preoperative decompression of sclerosing encapsulating peritonitis.

  4. Sclerosing encapsulating peritonitis in chronic ambulatory peritoneal dialysis;preoperative catheter drainage : a case report

    International Nuclear Information System (INIS)

    Kim, Tae Hoon

    1996-01-01

    Sclerosing encapsulating peritonitis is a well recognized, but uncommon, complication of chronic ambulatory peritoneal dialysis. I report a case of sclerosing encapsulating peritonitis in which percutaneous catheter drainage was performed preoperatively. Ultrasonography(US) and computed tomography(CT) showed a large multi-septated cystic mass which occupied nearly all the peritoneal cavity. Percutaneous drainage with two 8.5 French catheters was preoperatively performed under fluoroscopy and about 2100 ml of bloody fluid was drained for 20 days. On follow-up CT, the size of the cyst had significantly decreased and anoperation was performed. It is considered that percutaneous catheter drainage is useful in the preoperative decompression of sclerosing encapsulating peritonitis

  5. Clinicopathologic characteristics and prognostic factors of ovarian fibrosarcoma: the results of a multi-center retrospective study

    Directory of Open Access Journals (Sweden)

    Liao Ling-Min

    2010-10-01

    Full Text Available Abstract Background Ovarian fibrosarcomas are very rare tumors, and therefore, few case studies have evaluated the prognostic factors of this disease. To our knowledge, this study represents the largest study to evaluate the clinical and pathologic factors associated with ovarian fibrosarcoma patients. Methods Thirty-one cases of ovarian fibrosarcoma were retrospectively reviewed, which included medical records for eight patients, and 23 published case reports from 1995 through 2009. Patient treatment regimens included total hysterectomy with bilateral adnexectomy and an omentectomy (BAO (n = 9, oophorectomy (OR (n = 8, chemotherapy (CT (n = 1, BAO followed by chemotherapy (BAO+CT (n = 11, BAO followed by radiotherapy (BAO+RT (n = 1, and oophorectomy followed by radiotherapy (OR + RT (n = 1. Results The patients of this cohort were staged according to the guidelines of the Federation of Gynecology and Obstetrics (FIGO, with 15, 6, 9, and 1 stage I-IV cases identified, respectively. Mitotic count values were also evaluated from 10 high-power fields (HPFs, and 3 cases had an average mitotic count P = 0.007 and treatment (P = 0.008 were predictive of poor prognosis. Furthermore, patients with stage I tumors that received BAO+CT were associated with a better prognosis. Conclusions Mitotic activity, and cells positive for Ki-67 were identified as important factors in the diagnosis of ovarian fibrosarcoma. Furthermore, FIGO stage and treatment modalities have the potential to be prognostic factors of survival, with BAO followed by adjuvant chemotherapy associated with an improved treatment outcome.

  6. Ambiguous effect of signals transmitted by the vagus nerve on fibrosarcoma incidence and survival of tumor-bearing rats

    Czech Academy of Sciences Publication Activity Database

    Míková, L.; Horváthová, L.; Ondicova, K.; Tillinger, A.; Vannucci, Luca; Bizik, J.; Gidron, Y.; Mravec, B.

    2015-01-01

    Roč. 593, APR 2015 (2015), s. 90-94 ISSN 0304-3940 Institutional support: RVO:61388971 Keywords : BP6-TU2 fibrosarcoma cells * Subdiaphragmatic vagotomy * Vagus nerve Subject RIV: EC - Immunology Impact factor: 2.107, year: 2015

  7. Sclerosing Variant of the Bronchioloalveolar Carcinoma: Imaging Findings in an Atypical Case

    Directory of Open Access Journals (Sweden)

    Carolina Lamas Constantino

    2010-01-01

    Full Text Available Bronchioloalveolar carcinoma remains one of the most enigmatic lung cancers, demonstrating varied growth patterns, mixed histological features, and confusing clinical manifestations. This paper reports a case of an unusual form of presentation: a sclerosing type associated with desmoplastic reaction and cicatrization. A 75-year-old woman was admitted with persistent dry cough and progressive dyspnea. Physical examination showed bilateral inspiratory crackles. A chest radiograph and high-resolution computed tomography demonstrated confluent airspace nodules, forming areas of consolidation in both lungs, with signs of architectural distortion. The lung biopsy revealed a nonmucinous sclerosing bronchioloalveolar carcinoma.

  8. Genetic polymorphisms of matrix metalloproteinase 3 in primary sclerosing cholangitis

    Science.gov (United States)

    Juran, Brian D.; Atkinson, Elizabeth J.; Schlicht, Erik M.; Larson, Joseph J.; Ellinghaus, David; Franke, Andre; Lazaridis, Konstantinos N.

    2011-01-01

    Background The damaging cholestasis inherent to primary sclerosing cholangitis (PSC) results from bile duct stricturing because of progressive fibrosis. The matrix metalloproteinase 3 (MMP3) degrades a wide range of matrix components and is expressed by activated liver stellate cells, and so is a candidate for involvement with the fibrotic processes underlying PSC. Moreover, the MMP3 gene harbours polymorphisms associated with variation in its activity directly impacting clinical phenotypes. Aims We aimed to examine the influence of MMP3 polymorphisms on PSC risk and progression. Methods Nine single nucleotide polymorphisms (SNPs) tagging the common genetic variation of MMP3 were genotyped in 266 PSC patients and 407 controls. SNPs and inferred haplotypes were assessed for PSC association by logistic regression and score tests. The effect of SNPs on survival to liver transplant or death was analysed using Cox regression, and Kaplan–Meier curves were constructed. Results No association of PSC with individual SNPs or haplotypes of MMP3 was detected. However, progression to death or liver transplant was significantly associated with homozygosity for minor alleles of rs522616, rs650108 and rs683878, particularly among PSC patients with concurrent ulcerative colitis (UC) (strongest in redundant SNPs rs650108/rs683878, hazard ratio = 3.23, 95% confidence interval 1.45–7.25, P = 0.004). Conclusions Genetic variation in MMP3 influences PSC progression, possibly in the context of coexisting UC. While the functional variants and specific mechanisms remain unknown, this finding implicates the turnover of the extracellular matrix as an important and variable component of PSC pathogenesis. Efforts to understand this process could form the basis for developing effective treatments, which are currently lacking for PSC. PMID:21134112

  9. Epithelioid Sarcoma of the Forearm Arising from Perineural Sheath of Median Nerve Mimicking Carpal Tunnel Syndrome

    Directory of Open Access Journals (Sweden)

    Hiromasa Fujii

    2009-01-01

    Full Text Available We report here a case of epithelioid sarcoma in the forearm of a 33-year-old male presenting with symptoms and signs of carpal tunnel syndrome originating from the direct involvement of the median nerve. Due to the slow growing of the tumor, the patient noticed the presence of tumor mass in his forearm after several months from the initial onset of the symptoms. Magnetic resonance imaging showed an 8×4 cm mass involving the median nerve in the middle part of the forearm, and histological analysis of the biopsy specimen revealed the diagnosis of epithelioid sarcoma. Radical surgical resection was performed in conjunction with adjuvant chemotherapy. The function of the flexors were restored by the multiple tendon transfers (EIP→FDS; ECRL→FDP; BrR→FPL; EDM→opponens with superficial cutaneous branch of radial nerve transfer to the resected median nerve. The function of the affected hand showed excellent with the DASH disability/symptom score of 22.5, and both the grasp power and sensory of the median nerve area has recovered up to 50% of the normal side. The patient returned to his original vocation and alive with continuous disease free at 3.5-year follow-up since initial treatment.

  10. Epithelioid Myoepithelioma of the Accessory Parotid Gland: Pathological and Magnetic Resonance Imaging Findings

    Directory of Open Access Journals (Sweden)

    Hiroyoshi Iguchi

    2014-05-01

    Full Text Available Tumors of the accessory parotid gland (APG are rare, and pleomorphic adenoma (PA is the most common benign APG tumor subtype. Myoepithelioma of the APG is much rarer than PA, and to date, only 5 cases have been sporadically reported in the English literature. We describe the clinicopathological and MRI findings of an epithelioid myoepithelioma of the APG that was treated in our hospital. The patient's only clinical symptom was a slow-growing and painless mid-cheek mass. The tumor was suspected to be PA before surgery based on the following MRI findings: (1 a well-circumscribed and lobulated contour, (2 isointensity and hyperintensity relative to the muscle on T1- and T2-weighted images (WIs, respectively, (3 good enhancement on contrast-enhanced T1-WIs, (4 peripheral hypointensity on T2-WIs, and (5 a gradual time-signal intensity curve enhancement pattern on gadolinium-enhanced dynamic MRI. The tumor was completely resected via a standard parotidectomy approach, and the postoperative pathological examination of the tumor, including immunohistochemistry, confirmed the diagnosis of epithelioid myoepithelioma. As it is hardly possible to distinguish myoepithelioma from PA and low-grade malignant tumors preoperatively, a pathological examination using frozen sections is helpful for surgical strategy-related decisions.

  11. Disseminated Kaposi sarcoma with epithelioid morphology in an HIV/AIDS patient: A previously unreported variant.

    Science.gov (United States)

    Basra, Pukhraz; Paramo, Juan; Alexis, John

    2018-04-16

    Kaposi sarcoma is an oligoclonal HHV-8-driven vascular proliferation that was first described by a Viennese dermatologist Dr Moritz Kaposi. The disease has been seen in different clinical-epidemiological settings with a wide morphologic spectrum. We report a 52-year-old Caucasian man with HIV/AIDS and Kaposi sarcoma who presented with dyspnea and pleural effusion. He reported numerous tender subcutaneous nodules developing over the past few months on his chest, back and abdomen. An excisional biopsy of one of the nodules was performed. Touch preps revealed malignant cells in clusters. Microscopically, the neoplasm appeared undifferentiated with an epithelioid morphology, and involved the dermis and subcutaneous fat. Despite the medical history, Kaposi sarcoma was not considered foremost in the differential diagnosis. The malignant cells were positive for vimentin and negative for S100 protein, keratin AE1/3, CK7, CK20, napsin A, TTF-1 and synaptophysin. Additional stains revealed positivity for HHV-8, CD31 and D2-40, supporting the diagnosis of Kaposi sarcoma. Kaposi sarcoma has been well described with many variants that may cause diagnostic difficulty. An epithelioid variant has not been reported and consequently, may cause misinterpretation of an otherwise well-known entity that may become life threatening if appropriate treatment is not initiated in a timely manner. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. Dabrafenib Treatment in a Patient with an Epithelioid Glioblastoma and BRAF V600E Mutation

    Directory of Open Access Journals (Sweden)

    Garry Ceccon

    2018-04-01

    Full Text Available Novel therapeutic targets in malignant glioma patients are urgently needed. Point mutations of the v-Raf murine sarcoma viral oncogene homolog B (BRAF gene occur predominantly in melanoma patients, but may also occur in gliomas. Thus, this is a target of great interest for this group of patients. In a nine-year-old male patient, an anaplastic astrocytoma in the left temporoparietal region was diagnosed histologically. After first- and second-line treatment, a malignant progression to a secondary glioblastoma was observed ten years after the initial diagnosis. Within the following seven years, all other conventional treatment options were exhausted. At this time point, recurrent tumor histology revealed an epithelioid glioblastoma, without a mutation in the isocitrate dehydrogenase gene (IDH wild-type. In order to identify a potential target for an experimental salvage therapy, mutational tumor analysis showed a BRAF V600E mutation. Consecutively, dabrafenib treatment was initiated. The patient remained clinically stable, and follow-up magnetic resonance images (MRI were consistent with “Stable Disease” according to the Response Assessment in Neuro-Oncology Working Group (RANO criteria for the following ten months until tumor progression was detected. The patient died 16 months after dabrafenib treatment initiation. Particularly in younger glioma patients as well as in patients with an epithelioid glioblastoma, screening for a V600E BRAF mutation is promising since, in these cases, targeted therapy with BRAF inhibitors seems to be a useful salvage treatment option.

  13. Oncogenic transformation of rat lung epithelioid cells by SV40 DNA and restriction enzyme fragments

    International Nuclear Information System (INIS)

    Daya-Grosjean, L.; Lasne, C.; Nardeux, P.; Chouroulinkov, I.; Monier, R.

    1979-01-01

    Rat epithelioid lung cells were transformed with various preparations of SV40 DNA using the Ca 2+ -precipitation technique. The amount of SV40 genetic information integrated into transformed clones was evaluated by DNA-DNA renaturation kinetics. The growth properties on plastic and in soft-agar were examined, as well as the ability to induce tumors in syngeneic newborn animals or in adult nude mice. One particular transformed line, which had received the HpaII/BamHIA (59 per cent) fragment, was found to contain about 3 integrated copies of this fragment per cell and no significant amount of the HpaII/BamHIB (41 per cent fragment). This line which grew to high saturatio densities and efficiently formed clones in low serum on plastic, produced tumors in both syngeneic rats and nude mice. Thus the HpaII/BamHIA fragment, which mainly includes early viral information, was sufficient to impart these properties to rat epithelioid lung cells. (author)

  14. Malignant perivascular epithelioid cell tumor of the orbit: Report of a case and review of literature

    Directory of Open Access Journals (Sweden)

    Md Shahid Alam

    2017-01-01

    Full Text Available Perivascular epithelioid cell tumor (PEComa is a rare neoplasm considered to arise from myomelanocytic cell lineage. The uterus is reportedly the most common site to be involved. Orbital PEComa is extremely rare with only two cases reported till date. A 5-year-old male presented with a right medial orbital mass for the last 6 months. The patient was diagnosed with alveolar soft part sarcoma elsewhere. Magnetic resonance imaging features were suggestive of lymphangioma with bleeding. The excision biopsy revealed multiple tumor cells comprising epithelioid cells with clear cytoplasm, along with nuclear atypia and mitosis. Immunohistochemistry was positive for HMB-45, smooth muscle actin, vimentin, and CD-34. It was negative for cytokeratin, S-100, and synaptophysin, which clinched the diagnosis of malignant orbital PEComa. Neoadjuvant chemotherapy was administered. There was no recurrence at 24 months of follow-up. At present, there is no consensus on management protocol for malignant PEComa. Complete surgical excision with chemotherapy appears to offer the best prognosis.

  15. Two cases of fibrosarcoma occurring in the x-ray irradiated site

    International Nuclear Information System (INIS)

    Wakamatsu, Katsuo; Mochizuki, Masako.

    1975-01-01

    The histological examination was carried out on the following 2 cases: One case was a 46-year-old man with psoriasis vulgaris who received x-ray irradiation continually for about 10 years on the gluteal regions and the extensive sides of both elbows. An ulcer appeared on the irradiated site and about 2 weeks later a tumor appeared. The other case was a 77-year-old man with prurigo in the pudendal region. Although the dose and duration of irradiation are unknown, the tumors appeared in the irradiated site 6-7 years after the start of irradiation. The histological examination of these cases revealed the clinical findings of fibrosarcoma. In the review of literature, 8 cases of irradiation sarcoma in Japan were discussed. (Kanao, N.)

  16. Unusual case of congenital/infantile fibrosarcoma in a new born

    Directory of Open Access Journals (Sweden)

    Eddiba Tarik

    2013-01-01

    Full Text Available Congenital infantile fibrosarcoma (CIFS is a rare mesenchymal tumor that is primarily developed in the soft tissue of distal extremities and occasionally in unusual locations such as the lung and retroperitoneum. It occurs mainly in children below the age of 5 years. About 200 cases have been reported in the literature so far, very few of them in new-borns. The prognosis of this tumor is relatively good compared to adult forms. We report an unusual case of CIFS occurring in new-born mimicking an hemangioma and causing hemorrhage in the neonatal period. The tumor is located in the left arm and axilla and associated with a hand malformation. A shoulder amputation is performed after chemotherapy failure. The infant is now two-years old with no recurrence.

  17. Cytotoxic effect of chlorpromazine and its interaction with radiation on a mouse fibrosarcoma

    International Nuclear Information System (INIS)

    George, K.C.; Srinivasan, V.T.; Singh, B.B.

    1980-01-01

    Chlorpromazine hydrochloride (CPZ) was shown to exhibit a definite cytotoxic effect on mice fibrosarcomas in vivo under both normal and acutely hypoxic conditions. Administration of the drug (20 or 40 mg/kg) before exposure to 20 Gy of x rays caused a greater delay in growth than in tumours irradiated without the drug; an equal amount of growth delay was found at both doses. The effect of the combined treatment was greater than the sum of the effects of the two treatments given individually. However, since an equal delay in tumour growth was caused by CPZ given before or after irradiation, the combined effect, although synergistic, could not be described as radiosensitization. (U.K.)

  18. Pituitary fibrosarcoma secondary to radiation therapy for the treatment of chromophobe adenoma

    International Nuclear Information System (INIS)

    Shin, Hideo; Namba, Hiroki; Ishige, Naoki; Ohsato, Katsunobu; Nakamura, Takao

    1980-01-01

    A 33-year old female was seen in 1967 with about 3 year-history of diminution of visual acuity and amenorrhea, with abnormal gain of weight. At operation on April 19, 1967, typical pituitary adenoma was found and an intracapsular removal was performed. Histological sections showed a chromophobe adenoma without features of malignancy. Following operation, visual acuity and visual fields improved gradually to almost complete recovery. Radiotherapy was given with a tumor dose of 5,000 r. over a 6-week period. A second operation was done on November 29, 1973, and an intracapsular removal was repeated. The third operation on October 8, 1974 and the fourth operation on June 15, 1978 were done for saving sight, but in vain. Nevertheless, each operation showed extension of the tumor in the same way as in the previous observation. In April 1979, she was admitted by ambulance for dehydration and shock. She then developed swallowing disturbance, frontal headache and mental confusion. For the following 5 months, she had increasing deficits and subsequently developed dyspnea and left hemiparesis. Terminally, there was coma for a week. Postmortem examination disclosed neoplastic tissue, which not only filled a remarkably enlarged pituitary fossa but also extended downward as far as the mucosa of the oral cavity and upward into the frontal lobe. The optic nerves and 3rd nerves were almost obscured by the firm tumor. On slicing the brain coronally, this tumor was seen extending upward as far as the lateral ventricle. Microscopic examination showed a fibrosarcoma composed of elongated spindle cells. At the base of the tumor, islands of adenomatous tissue were present. The most likely causative factor in the development of pituitary fibrosarcoma in this patient is therapeutic radiation together with repeated surgical trauma. We would like to emphasize early awareness of such malignant alteration of clinical picture following radiation therapy. (J.P.N.)

  19. PPARγ1 phosphorylation enhances proliferation and drug resistance in human fibrosarcoma cells

    Energy Technology Data Exchange (ETDEWEB)

    Pang, Xiaojuan; Shu, Yuxin; Niu, Zhiyuan; Zheng, Wei; Wu, Haochen [State Key Laboratory of Pharmaceutical Biotechnology, Nanjing University, Nanjing (China); Lu, Yan, E-mail: luyan@nju.edu.cn [State Key Laboratory of Pharmaceutical Biotechnology, Nanjing University, Nanjing (China); Shen, Pingping, E-mail: ppshen@nju.edu.cn [State Key Laboratory of Pharmaceutical Biotechnology, Nanjing University, Nanjing (China); Model Animal Research Center (MARC), Nanjing University, Nanjing (China)

    2014-03-10

    Post-translational regulation plays a critical role in the control of cell growth and proliferation. The phosphorylation of peroxisome proliferator-activated receptor γ (PPARγ) is the most important post-translational modification. The function of PPARγ phosphorylation has been studied extensively in the past. However, the relationship between phosphorylated PPARγ1 and tumors remains unclear. Here we investigated the role of PPARγ1 phosphorylation in human fibrosarcoma HT1080 cell line. Using the nonphosphorylation (Ser84 to alanine, S84A) and phosphorylation (Ser84 to aspartic acid, S84D) mutant of PPARγ1, the results suggested that phosphorylation attenuated PPARγ1 transcriptional activity. Meanwhile, we demonstrated that phosphorylated PPARγ1 promoted HT1080 cell proliferation and this effect was dependent on the regulation of cell cycle arrest. The mRNA levels of cyclin-dependent kinase inhibitor (CKI) p21{sup Waf1/Cip1} and p27{sup Kip1} descended in PPARγ1{sup S84D} stable HT1080 cell, whereas the expression of p18{sup INK4C} was not changed. Moreover, compared to the PPARγ1{sup S84A}, PPARγ1{sup S84D} up-regulated the expression levels of cyclin D1 and cyclin A. Finally, PPARγ1 phosphorylation reduced sensitivity to agonist rosiglitazone and increased resistance to anticancer drug 5-fluorouracil (5-FU) in HT1080 cell. Our findings establish PPARγ1 phosphorylation as a critical event in human fibrosarcoma growth. These findings raise the possibility that chemical compounds that prevent the phosphorylation of PPARγ1 could act as anticancer drugs. - Highlights: • Phosphorylation attenuates PPARγ1 transcriptional activity. • Phosphorylated PPARγ1 promotes HT1080 cells proliferation. • PPARγ1 phosphorylation regulates cell cycle by mediating expression of cell cycle regulators. • PPARγ1 phosphorylation reduces sensitivity to agonist and anticancer drug. • Our findings establish PPARγ1 phosphorylation as a critical event in HT1080

  20. Applicability and prognostic value of histologic scoring systems in primary sclerosing cholangitis

    NARCIS (Netherlands)

    de Vries, Elisabeth M. G.; Verheij, Joanne; Hubscher, Stefan G.; Leeflang, Mariska M. G.; Boonstra, Kirsten; Beuers, Ulrich; Ponsioen, Cyriel Y.

    2015-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. At present, there is no appropriate histologic scoring system available for PSC, evaluating both degree of necroinflammatory activity (grade) and fibrosis (stage). The aim of this study was to assess if three scoring

  1. Value of brush cytology for dominant strictures in primary sclerosing cholangitis

    NARCIS (Netherlands)

    Ponsioen, C. Y.; Vrouenraets, S. M.; van Milligen de Wit, A. W.; Sturm, P.; Tascilar, M.; Offerhaus, G. J.; Prins, M.; Huibregtse, K.; Tytgat, G. N.

    1999-01-01

    Around 10% of patients with primary sclerosing cholangitis (PSC) develop cholangiocarcinoma, which is cholangiographically often indistinguishable from a benign dominant stricture. The aim of the present study was to assess the value of brush cytology in discriminating between benign and malignant

  2. Deterioration of cholestasis after endoscopic retrograde cholangiography in advanced primary sclerosing cholangitis

    NARCIS (Netherlands)

    Beuers, U.; Spengler, U.; Sackmann, M.; Paumgartner, G.; Sauerbruch, T.

    1992-01-01

    Complications of endoscopic retrograde cholangiography specific to patients with primary sclerosing cholangitis have not yet been reported. We observed transient rises of serum bilirubin after diagnostic endoscopic retrograde cholangiography in five of 15 patients and persistent rises in three of 15

  3. Generation of sclerosant foams by mechanical methods increases the foam temperature.

    Science.gov (United States)

    Tan, Lulu; Wong, Kaichung; Connor, David; Fakhim, Babak; Behnia, Masud; Parsi, Kurosh

    2017-08-01

    Objective To investigate the effect of agitation on foam temperature. Methods Sodium tetradecyl sulphate and polidocanol were used. Prior to foam generation, the sclerosant and all constituent equipment were cooled to 4-25℃ and compared with cooling the sclerosant only. Foam was generated using a modified Tessari method. During foam agitation, the temperature change was measured using a thermocouple for 120 s. Results Pre-cooling all the constituent equipment resulted in a cooler foam in comparison with only cooling the sclerosant. A starting temperature of 4℃ produced average foam temperatures of 12.5 and 13.2℃ for sodium tetradecyl sulphate and polidocanol, respectively. It was also found that only cooling the liquid sclerosant provided minimal cooling to the final foam temperature, with the temperature 20 and 20.5℃ for sodium tetradecyl sulphate and polidocanol, respectively. Conclusion The foam generation process has a noticeable impact on final foam temperature and needs to be taken into consideration when creating foam.

  4. A survey of infectious agents as risk factors for primary sclerosing cholangitis: are Chlamydia species involved?

    NARCIS (Netherlands)

    Ponsioen, Cyriel Y.; Defoer, Jacqueline; ten Kate, Fiebo J. W.; Weverling, Gerrit J.; Tytgat, Guido N. J.; Pannekoek, Yvonne; Wertheim-Dillen, Pauline M. E.

    2002-01-01

    Objectives The aetiology of primary sclerosing cholangitis (PSC) is unknown, and the role of micro-organisms has been studied only to a limited extent. We tested the hypothesis that past or persisting infection with common viruses or atypical bacteria might play a role in genetically susceptible

  5. Case report 834: Chronic sclerosing osteomyelitis of the mandible with long bone periostitis.

    Science.gov (United States)

    Stewart, A; Carneiro, R; Pollock, L; Shaw, D

    1994-04-01

    We present the case of a patient with primary chronic osteomyelitis of the mandible, the radiological appearance of which is compatible with a diagnosis of chronic sclerosing osteomyelitis. The accompanying femoral and tibial periosteal reactive new bone formation and the benign clinical course suggest that this presentation may represent a form of chronic multifocal osteomyelitis.

  6. Diffuse sclerosing variant of thyroid carcinoma presenting as Hashimoto thyroiditis: a case report.

    Science.gov (United States)

    Vukasović, Anamarija; Kuna, Sanja Kusacić; Ostović, Karmen Trutin; Prgomet, Drago; Banek, Tomislav

    2012-11-01

    The aim of report is to present a case of a rare diffuse sclerosing variant of a papillary thyroid carcinoma. A 15-year old girl referred for ultrasound examination because of painless thyroid swelling lasting 10 days before. An ultrasound of the neck showed diffusely changed thyroid parenchyma, without nodes, looking as lymphocytic thyroiditis Hashimoto at first, but with snow-storm appearance, predominantly in the right lobe. Positive thyroid peroxidase antibodies (TPO-AT) also suggested Hashimoto thyroiditis. Repeated US-FNAB (fine needle-aspiration biopsy) of the right lobe revealed diffuse sclerosing variant of papillary thyroid carcinoma and patient underwent total thyreoidectomy. Patohistologic finding confirmed diffuse sclerosing variant of a papillary thyroid carcinoma in the both thyroid lobes and several metastatic lymph nodes. Two months later patient recived radioablative therapy with 3700 MBq (100 mCi) of 1-131 followed by levothyroxine replacement. At the moment, patient is without evidence of local or distant metastases and next regular control is scheduled in 6 months. In conclusion, a diffuse sclerosing variant is rare form of papillary thyroid carcinoma that echographically looks similar to Hashimoto thyroiditis and sometimes could be easily overlooked.

  7. Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    Weismueller, Tobias J.; Trivedi, Palak J; Bergquist, Annika; Imam, Mohamad; Lenzen, Henrike; Ponsioen, Cyriel Y.; Holm, Kristian; Gotthardt, Daniel; Faerkkilae, Martti A.; Marschall, Hanns-Ulrich; Thorburn, Douglas; Weersma, Rinse K.; Fevery, Johan; Mueller, Tobias; Chazouilleres, Olivier; Schulze, Kornelius; Lazaridis, Konstantinos N.; Almer, Sven; Pereira, Stephen P.; Levy, Cynthia; Mason, Andrew L.; Naess, Sigrid; Bowlus, Christopher L.; Floreani, Annarosa; Halilbasic, Emina; Yimam, Kidist K.; Milkiewicz, Piotr; Beuers, Ulrich; Huynh, Dep K.; Pares, Albert; Manser, Christine N.; Dalekos, George N.; Eksteen, Bertus; Invernizzi, Pietro; Berg, Christoph P.; Kirchner, Gabi I.; Sarrazin, Christoph; Zimmer, Vincent; Fabris, Luca; Braun, Felix; Marzioni, Marco; Juran, Brian D.; Said, Karouk; Rupp, Christian; Jokelainen, Kalle; de Valle, Maria Benito; Saffioti, Francesca; Cheung, Angela; Trauner, Michael; Schramm, Christoph; Chapman, Roger W.; Karlsen, Tom H.; Schrumpf, Erik; Strassburg, Christian P.; Manns, Michael P.; Lindor, Keith D; Hirschfield, Gideon M.; Hansen, Bettina E.; Boberg, Kirsten M.

    BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large international cohort of patients with PSC. METHODS: We

  8. Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    Weismuller, Tobias J.; Trivedi, Palak J.; Bergquist, Annika; Imam, Mohamad; Lenzen, Henrike; Ponsioen, Cyriel Y.; Holm, Kristian; Gotthardt, Daniel; Farkkila, Martti A.; Marschall, Hanns-Ulrich; Thorburn, Douglas; Weersma, Rinse K.; Fevery, Johan; Mueller, Tobias; Chazouilleres, Olivier; Schulze, Kornelius; Lazaridis, Konstantinos N.; Almer, Sven; Pereira, Stephen P.; Levy, Cynthia; Mason, Andrew; Naess, Sigrid; Bowlus, Christopher L.; Floreani, Annarosa; Halilbasic, Emina; Yimam, Kidist K.; Milkiewicz, Piotr; Beuers, Ulrich; Huynh, Dep K.; Pares, Albert; Manser, Christine N.; Dalekos, George N.; Eksteen, Bertus; Invernizzi, Pietro; Berg, Christoph P.; Kirchner, Gabi I.; Sarrazin, Christoph; Zimmer, Vincent; Fabris, Luca; Braun, Felix; Marzioni, Marco; Juran, Brian D.; Said, Karouk; Rupp, Christian; Jokelainen, Kalle; Benito de Valle, Maria; Saffioti, Francesca; Cheung, Angela; Trauner, Michael; Schramm, Christoph; Chapman, Roger W.; Karlsen, Tom H.; Schrumpf, Erik; Strassburg, Christian P.; Manns, Michael P.; Lindor, Keith D.; Hirschfield, Gideon M.; Hansen, Bettina E.; Boberg, Kirsten M.

    2017-01-01

    Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large international cohort of patients with PSC. We performed a retrospective

  9. Proximal-type epithelioid sarcoma - Case report Sarcoma epitelióide tipo proximal - Relato de caso

    Directory of Open Access Journals (Sweden)

    Luciana Mendes dos Santos

    2013-06-01

    Full Text Available Epithelioid sarcoma, first described by Enzinger in 1970, is a rare soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in distal portions of the extremities of adolescents and young adults. In 1997, Guillou et al. described a different type of epithelioid sarcoma, called proximal-type epithelioid sarcoma, which is found mostly in the pelvic and perineal regions and genital tracts of young to middle-aged adults. It is characterized by a proliferation of epithelioid-like cells with rhabdoid features and the absence of a granuloma-like pattern. In this paper we present a case of proximal-type epithelioid sarcoma with an aggressive clinical course, including distant metastasis and death nine months after diagnosis.O sarcoma epitelióide, primeiramente descrito por Enzinger, em 1970, é uma neoplasia de partes moles que ocorre principalmente nas extremidades distais de adolescentes e adultos jovens. Em 1997, Guillou e cols. descreveram um tipo diferente de sarcoma epitelióide, que afetava frequentemente a região pélvica, períneo e áreas genitais de pacientes de média idade, com exame histológico caracterizado pela proliferação de células com aspecto epitelióide. Neste trabalho, descreve-se caso de paciente que apresentava há três meses duas lesões na região glútea, cujo exame histológico confirmou diagnóstico de sarcoma epitelioide do tipo proximal, já com presença de metástases pulmonares e cerebrais e que foi a óbito nove meses após o diagnóstico.

  10. Less promising results with sclerosing ethoxysclerol injections for midportion achilles tendinopathy: a retrospective study.

    Science.gov (United States)

    van Sterkenburg, Maayke N; de Jonge, Milko C; Sierevelt, Inger N; van Dijk, C Niek

    2010-11-01

    Local injections of the sclerosing substance polidocanol (Ethoxysclerol) have shown good clinical results in patients with chronic midportion Achilles tendinopathy. After training by the inventors of the technique, sclerosing Ethoxysclerol injections were applied on a group of patients in our center. Sclerosing Ethoxysclerol injections will yield good results in the majority of patients. Case series; Level of evidence, 4. In 113 patients (140 tendons) with Achilles tendinopathy, we identified 62 patients (70 tendons) showing neovascularization on color Doppler ultrasound. Fifty-three Achilles tendons (48 patients) were treated with sclerosing Ethoxysclerol injections, with intervals of 6 weeks and a maximum of 5 sessions. Treatment was completed when neovascularization or pain had disappeared, or when there was no positive treatment effect after 3 to 4 sessions. Forty-eight patients (20 women and 28 men) with a median age of 45 years, (range, 33-68 years) were treated. Median symptom duration was 23 months (range, 3-300 months). Fifty-three tendons were treated with a median of 3 sessions of Ethoxysclerol injections. Six weeks after the last injection, 35% of patients had no complaints, 9% had minimal symptoms, 42% were the same, and 14% had more complaints. Women were 3.8 times (95% confidence interval: 1.1-13.8) more likely to have unsatisfactory outcome than men. Pain correlated positively with neovessels on ultrasound (P < .01). At 2.7 to 5.1 year follow-up, 53% had received additional (surgical/conservative) treatment; 3 of these patients (7.5%) still had complaints of Achilles tendinopathy. In 6 patients, complaints that were still present 6 weeks after treatment had resolved spontaneously by final follow-up. Our study did not confirm the high beneficial value of sclerosing neovascularization in patients with midportion Achilles tendinopathy. Despite the retrospective design of our study, we consider it important to stress that injection of Ethoxysclerol may

  11. Hemagglutinin-specific neutralization of subacute sclerosing panencephalitis viruses.

    Directory of Open Access Journals (Sweden)

    Miguel Ángel Muñoz-Alía

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a progressive, lethal complication of measles caused by particular mutants of measles virus (MeV that persist in the brain despite high levels of neutralizing antibodies. We addressed the hypothesis that antigenic drift is involved in the pathogenetic mechanism of SSPE by analyzing antigenic alterations in the MeV envelope hemagglutinin protein (MeV-H found in patients with SSPE in relation to major circulating MeV genotypes. To this aim, we obtained cDNA for the MeV-H gene from tissue taken at brain autopsy from 3 deceased persons with SSPE who had short (3-4 months, SMa79, average (3.5 years, SMa84, and long (18 years, SMa94 disease courses. Recombinant MeVs with a substituted MeV-H gene were generated by a reverse genetic system. Virus neutralization assays with a panel of anti-MeV-H murine monoclonal antibodies (mAbs or vaccine-immunized mouse anti-MeV-H polyclonal sera were performed to determine the antigenic relatedness. Functional and receptor-binding analysis of the SSPE MeV-H showed activity in a SLAM/nectin-4-dependent manner. Similar to our panel of wild-type viruses, our SSPE viruses showed an altered antigenic profile. Genotypes A, G3, and F (SSPE case SMa79 were the exception, with an intact antigenic structure. Genotypes D7 and F (SSPE SMa79 showed enhanced neutralization by mAbs targeting antigenic site IIa. Genotypes H1 and the recently reported D4.2 were the most antigenically altered genotypes. Epitope mapping of neutralizing mAbs BH015 and BH130 reveal a new antigenic site on MeV-H, which we designated Φ for its intermediate position between previously defined antigenic sites Ia and Ib. We conclude that SSPE-causing viruses show similar antigenic properties to currently circulating MeV genotypes. The absence of a direct correlation between antigenic changes and predisposition of a certain genotype to cause SSPE does not lend support to the proposed antigenic drift as a

  12. Proximal-type epithelioid sarcoma of pharynx: A case report with immunohistochemical study

    Directory of Open Access Journals (Sweden)

    Tadashi Terada, M.D., Ph.D.

    2015-06-01

    Full Text Available Epithelioid sarcoma (ES usually occurs in extremities. ES shows characteristic granulomatous morphologies, and its prognosis is not so poor. Proximal-type ES (PT-ES is a variant of ES, and is characterized by central locations, severe atypical features, and poor prognosis. Both ES and PT-ES histologically show epithelial and sarcomatous patterns, and immunohistochemically express both vimentin and cytokeratins. A 77-year-old man presented with sore throat. The laryngoscope revealed a large polypoid tumor (4 × 3 × 4 cm in upper pharynx, and a large biopsy was taken. The biopsy showed malignant epithelioid and spindle tumor cells. There were no apparent transitions between normal epithelial cells and tumor cells. The tumor consisted of malignant epithelioid and spindle cells with marked anaplasia. The tumor showed marked infiltrative features and lymphovascular permiations. Mitotic figures, atypical mitosis, and necrotic areas were present. No apparent features of sarcomas were seen. No rhabdoid cells were seen. Immunohistochemically, the malignant cells were positive for vimentin (3+, diffuse, CK AE1/3 (1+, focal, CK CAM5.2 (1+, diffuse, CK8 (1+, focal, CK18 (1+, focal, CD20 (1+, focal, p53 (3+, 84%, Ki-67 (labeling = 94%, CD68 (2+, only focal, and anti-trypsin (2+, only focal. They were negative for CK34BE12, CK5, CK6, CK7, CK14, CK19, CD99, CEA, CA19-9, CA125, EMA, S100 protein, NCAM, NSE, synaptophysin, chromogranin, α-smooth muscle actin, smooth muscle actin, desmin, CD34, CD31, CD45, D2-40, factor-VIII-related antigen, HMB-45, Melan-A, myoglobin, MDM2, CDK4, KIT, and PDGFRA. The author diagnosed this pharyngeal tumor as PT-ES, but the author cannot completely exclude the possibility of sarcomatoid carcinoma. The prognosis of patient was poor; the patient died of carcinomatosis 2 years after the manifestation.

  13. Treatment of Hepatic Epithelioid Hemangioendothelioma: Finding Uses for Thalidomide in a New Era of Medicine

    Directory of Open Access Journals (Sweden)

    Matthew P. Soape

    2015-01-01

    Full Text Available Hepatic epithelioid hemangioendothelioma (HEH is extremely rare, occurring in 1 to 2 per 100,000, with chemotherapy options not well defined. Our case involved a 49-year-old female who had hepatic masses and metastasis to the lungs with a liver biopsy revealing HEH. After developing a rash from sorafenib, thalidomide was started with the progression of disease stabilized. Resection is only an option in 10% of the cases; therefore, chemotherapy is the only line of treatment. Newer chemotherapy alternatives are targeting angiogenesis via the vascular endothelial growth factor. Thalidomide was first used as an antiemetic, but, sadly, soon linked to phocomelia birth defects. Given the mechanism of action against angiogenesis, thalidomide has a valid role in vascular tumors. In conclusion, the use of thalidomide as chemotherapy is novel and promising, especially in the setting of a rare vascular liver tumor such as HEH.

  14. Primary pleural epithelioid hemangioendothelioma (EHE)--two cases and review of the literature.

    Science.gov (United States)

    Lazarus, Angeline; Fuhrer, Gregory; Malekiani, Christina; McKay, Sean; Thurber, John

    2011-01-01

    We present two cases with symptoms of progressively worsening cough, dyspnea, decreased exercise tolerance and right-sided back pain in the first case and upper respiratory symptoms characterized by cough and a low grade fever in the second case. Report of two cases. The initial chest X-ray in both the cases showed pleural effusion. Further imaging with computed tomography of the chest confirmed the effusion in both cases. Thoracentesis was done in both of them revealed an exudative effusion that did not reveal any infection or malignancy. Both cases underwent surgical biopsy and the diagnosis of primary pleural epithelioid hemangioendothelioma was made. Both the cases had progressive clinical deterioration despite chemotherapy with Taxol and Bevacizumab in one case and carboplatin, etoposide, and bevacizumab, in the second case. Both developed metastatic disease to lungs and died. Published 2010. This article is a US Government work and is in the public domain in the USA.

  15. Multifocal Epithelioid Hemangioendothelioma Derived from the Spine Region: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    G. Kerry

    2012-02-01

    Full Text Available Background: Epithelioid hemangioendothelioma (EHE is a rare vascular tumor with malignant biological behavior. It arises from endothelial cells, usually within soft tissues, and can occur in almost all locations. Case Report: We report a unique case of a 25-year-old man who presented with sudden attacks of severe back pain followed by acute non-traumatic paraplegia. Emergency diagnostics revealed a pathologic fracture of the T7 vertebra with tumor tissue invasion of the spinal canal. Furthermore, multifocal metastases were found. Results: To achieve en bloc resection, interdisciplinary surgical approaches were indicated. Despite multimodal therapy concepts, including radiotherapy and chemotherapy as well as endovascular embolization, the patient died within 8 weeks. Conclusion: Prognosis of EHE is unpredictable and mainly determined by its location. The lesions are potentially aggressive; therefore, en bloc resection should be attempted whenever possible. However, as shown in the literature, only 15% of patients are suitable for total resection.

  16. Malignant hepatic perivascular epithelioid cell tumor (PEComa)- Case report and a brief review

    International Nuclear Information System (INIS)

    Abhirup, B.; Kaushal, K.; Ganesh, N.; Sanket, M.

    2015-01-01

    Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms which can arise from almost any location in the body. Diagnosing them pre-operatively is difficult as they mimic features of other hepatic neoplasms including hepatocellular carcinoma (HCC), fibrolamellar HCC, and focal nodular hyperplasia (FNH) among others. The unique feature of these tumors is the coexpression of muscle and melanocytic markers. These are identified immunohistochemically by the expression of Human Melanin Black-45 (HMB-45), Melan-A and Smooth Muscle Antigen (SMA) which are seen in the majority of tumors. The liver is uncommonly associated with a PEComa and the approach to a patient with hepatic PEComa is not well described. There is no consensus regarding the neo-adjuvant/adjuvant therapy in these patients. The natural history of this condition is not well documented making it an unpredictable disease. Here we have discussed a case and reviewed the literature concerning these rare tumors.

  17. An immunoglobulin G-4 related sclerosing disease of the small bowel: CT and small bowel series findings

    International Nuclear Information System (INIS)

    Ko, Young Hwan; Hwang, Dae Hyun; Min, Seon Jeong; Woo, Ji Young; Kim, Jeong Won; Hong, Hye Sook; Yang, Ik; Lee, Yul

    2013-01-01

    Immunoglobulin G4 (IgG4)-related sclerosing disease is rare and is known to involve various organs. We present a case of histologically proven IgG4-related sclerosing disease of the small bowel with imaging findings on computed tomography (CT) and small bowel series. CT showed irregular wall thickening, loss of mural stratification and aneurysmal dilatation of the distal ileum. Small bowel series showed aneurysmal dilatations, interloop adhesion with traction and abrupt angulation.

  18. Effect of aged garlic extract on immune responses to experimental fibrosarcoma tumor in BALB/c mice.

    Science.gov (United States)

    Tabari, M Abouhosseini; Ebrahimpour, S

    2014-01-01

    Aged garlic extract (AGE) has many biological activities including radical scavenging, antioxidative and immunomodulative effects. In this research work, the antitumor and immunomodulatory effects of AGE against fibrosarcoma implanted tumor were studied. WEHI-164 fibrosarcoma cells were implanted subcutaneously on day 0 into the right flank of 40 BALB/c mice at age of 8 weeks. Mice were randomly categorized in two separate groups: First received AGE (100 mg/kg, IP), second group as the control group received phosphate buffered saline. Treatments were carried out 3 times/week. Tumor growth was measured and morbidity was recorded. Subpopulations of CD4+/CD8+ T cells were determined using flow cytometry. WEHI-164 cell specific cytotoxicity of splenocytes and in vitro production of interferon gamma (IFN-γ) and interleukin-4 cytokines were measured. The mice received AGE had significantly longer survival time compared with the control mice. The inhibitory effect on tumor growth was seen in AGE treated mice. The CD4+/CD8+ ratio and in vitro IFN-γ production of splenocytes were significantly increased in AGE group. WEHI-164 specific cytotoxicity of splenocytes from AGE mice was also significantly increased at 25:1 E: T ratio. Administration of AGE resulted in improved immune responses against experimentally implanted fibrosarcoma tumors in BALB/c mice. AGE showed significant effects on inhibition of tumor growth and longevity of survival times.

  19. S-phase checkpoint elements of the E2F-1 family increase radiosensitivity in fibrosarcoma cells lacking p53

    International Nuclear Information System (INIS)

    Bodis, Stephan; Pruschy, Martin; Wirbelauer, Christiane; Glanzmann, Christoph; Krek, Wilhelm

    1997-01-01

    Purpose: Correct advance of cells through the S-phase of the mammalian cell cycle depends on the timely controlled activity of the E2F-1 transcription factor by cyclin A-cdk2. We are studying the reproductive integrity and radiosensitation of isogenic mouse fibrosarcoma cells, differing only in their p53 status, after expression of E2F-1 wildtype (wt) and specific E2F-1 mutants (mt) lacking the cyclin-A-binding domain. In this tumor model system only p53 wild-type expressing tumor cells are sensitive to ionizing radiation in vitro and in vivo. Material and Methods: Either wild-type p53 or genetically engineered p53 'null' mouse embryo fibroblasts were transfected with the oncogenes E1A and ras. These otherwise isogenic fibrosarcoma cells, with a malignant phenotype and tumorigenic in nude mice, were transfected with retroviruses containing either E2F-1 wild-type or specific E2F-1 mutants lacking the cyclin-A binding domain. Reproductive integrity after E2F-1 transfection with or without ionizing radiation (RT) was tested using the clonogenic assay. Tumor cell morphology of treated cells is analyzed for cell death mechanism. Results: E2F-1 wild-type expression in fibrosarcoma cells induced a clear p53 dependent cell death. While clonogenic survival of p53 'null' tumor cells was only slightly reduced with the expression of E2F-1 wild type (survival fraction of 0.5), the clonogenic survival of p53 wild-type fibrosarcoma tumor cells was reduced by at least one logarithm (survival fraction of 0.05). However, expression of the specific E2F-1 mutant lacking the cyclin-A binding domain reduced clonogenic survival in both the p53 'null' and the p53 wild-type fibrosarcoma cells by at least 2 logarithms (survival fraction 0.01 for p53 'null' and 0.002 for p53 wild-type). The mean values of the survival fractions after 2 and 5 Gy radiation alone in p53 'null' fibrosarcoma cells (SF 2 and SF 5) were SF 2 0.7, SF 5 = 0.15, respectively. The combination of ionizing RT in the p53

  20. Fetal presentation of congenital fibrosarcoma of the meninges: case report and literature review.

    Science.gov (United States)

    Marguet, Florent; Bergogne, Lise; Laurent, Nicole; Rousseau, Thierry; Laquerrière, Annie

    2015-01-01

    Congenital infantile fibrosarcoma (CIFS) exceptionally occurs in the meninges, with no cases reported before or at birth. We report herein a meningeal CIFS diagnosed in a fetus at 40 weeks of gestation (WG). A 24-year-old pregnant woman was referred to the obstetrics department for vaginal bleeding. A severe right hydrocephalus due to a large tumor invading the left hemisphere and ventricles was discovered in the fetus, and medical termination of the pregnancy was achieved. Histological examination revealed a highly cellular spindle or ovoid shaped cell proliferation organized in interlacing bundles; it was diffusely positive for vimentin, and scarcely for SMA (smooth muscle actin). NFs (neurofilaments), NeuN, S100 protein, desmin, GFAP (glial fibrillary acidic protein), Olig2, chromogranin, synaptophysin, CD31, CD34, BCL2, and EMA (epithelial membrane antigen) antibodies were negative. Ki67 antibody labeled 20% of the nuclei. A reverse transcription polymerase chain reaction assay was performed to detect the gene fusion ETV6-NTRK3 transcript. Despite negative results, it was concluded to be a CIFS of the meninges. CIFS of the meninges during the fetal period has never been reported. Its diagnosis is based on immunohistochemistry, and, whenever possible, on the detection of the reciprocal translocation t (12;15) resulting in the gene fusion ETV6-NTRK3. Its prognosis depends on rapid growth and local invasiveness leading to cerebral structure damage.

  1. Significance of fractionation regimens in radiation and combined hyperthermia using a murine fibrosarcoma

    International Nuclear Information System (INIS)

    Hahn, E.W.; Alfieri, A.A.; Kim, J.H.

    1978-01-01

    The significance of time--dose ralationships in the use of local tumor hyperthermia (LTH) when combined with radiation (RAD) was studied in a murine fibrosarcoma. RAD, either alone or combined with LTH, was delivered in four equal fractions (total doses, 1.8 to 4.2 krad) separated by 1 to 4 days. LTH (43.1 C +- .05 C for 15 minutes, water bath) was applied immediately after RAD. In this tumor system, RAD was most effective when delivered every 2nd or 3rd day, by a factor of 1.25 over the response achieved when the four fractions were delivered every 1 or 4 days. At all levels studied, RAD + LTH produced a superior tumor response compared to RAD alone. The ratio of the RAD + LTH/RAD doses to achieve an isobiological response ranged from 1.7 to 2.5. Most significant was the finding that the RAD + LTH treatment response was independent of the fractionation scheme used and more dependent on the total RAD dose delivered

  2. Type I collagen gel protects murine fibrosarcoma L929 cells from TNFα-induced cell death

    International Nuclear Information System (INIS)

    Wang, Hong-Ju; He, Wen-Qi; Chen, Ling; Liu, Wei-Wei; Xu, Qian; Xia, Ming-Yu; Hayashi, Toshihiko; Fujisaki, Hitomi; Hattori, Shunji; Tashiro, Shin-ichi; Onodera, Satoshi; Ikejima, Takashi

    2015-01-01

    Murine fibrosarcoma L929 cells have been used to test efficacy of proinflammatory cytokine TNFα. In the present study, we reported on protective effect of type I collagen gel used as L929 cell culture. L929 cell grew and proliferated well on collagen gel. However, the L929 cells exhibited cobblestone-like morphology which was much different from the spread fusiform shape when cultured on conventional cell dishes as well as the cells tended to aggregate. On conventional cell culture dishes, the cells treated with TNFα became round in shape and eventually died in a necroptotic manner. The cells cultured on collagen gel, however, were completely unaffected. TNFα treatment was reported to induce autophagy in L929 cells on the plastic dish, and therefore we investigated the effect of collagen gel on induction of autophagy. The results indicated that autophagy induced by TNFα treatment was much reduced when the cells were cultured on collagen gel. In conclusion, type I collagen gel protected L929 cell from TNFα-induced cell death. - Highlights: • Collagen gel culture changed the morphology of L929 cells. • L929 cell cultured on collagen gel were resistant to TNFα-induced cell death. • Collagen gel culture inhibited TNFα-induced autophagy in L929 cells

  3. Successful treatment of a rare case of ameloblastic fibrosarcoma with radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Oertel, Michael; Reinartz, Gabriele; Scobioala, Sergiu; Eich, Hans Theodor [University Hospital of Muenster, Department of Radiation Oncology, Muenster (Germany)

    2017-08-15

    Sarcomas are rare diseases of the head and neck region, representing around 1% of all malignancies. Amongst them, ameloblastic fibrosarcoma (AFS) is of even greater rarity, with less than 100 cases reported in the literature. Consequently, no standard treatment or guidelines have been made available. Surgery is often performed as primary therapy, but may be limited due to anatomical or functional reasons. We present a case of AFS successfully treated by postoperative radiation therapy. A detailed case study is provided, followed by a review of the English-language literature focusing on the role of radiation therapy. (orig.) [German] Sarkome sind in der Kopf-Hals-Region seltene Tumore mit einem Anteil von ca. 1 % an allen malignen Erkrankungen. Das ameloblastische Fibrosarkom (AFS) ist wiederum von noch groesserer Seltenheit, mit weniger als 100 berichteten Faellen in der Literatur. Entsprechend konnte bis jetzt kein Standardvorgehen etabliert werden. Chirurgische Interventionen werden haeufig als Primaertherapie durchgefuehrt, sind jedoch aus anatomischen bzw. funktionalen Gruenden limitiert. Wir stellen den Fall eines AFS vor, das erfolgreich mit einer postoperativen Radiotherapie behandelt wurde. Eine detaillierte Fallstudie wird ergaenzt durch eine Uebersicht der englischsprachigen Literatur zu diesem Thema unter besonderer Beruecksichtigung der Rolle der Strahlentherapie. (orig.)

  4. Type I collagen gel protects murine fibrosarcoma L929 cells from TNFα-induced cell death

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Hong-Ju; He, Wen-Qi; Chen, Ling; Liu, Wei-Wei; Xu, Qian; Xia, Ming-Yu; Hayashi, Toshihiko [China-Japan Research Institute of Medical and Pharmaceutical Sciences, Shenyang Pharmaceutical University, Shenyang 110016 (China); Fujisaki, Hitomi; Hattori, Shunji [Nippi Research Institute of Biomatrix, Toride, Ibaraki 302-0017 (Japan); Tashiro, Shin-ichi [Institute for Clinical and Biomedical Sciences, Kyoto 603-8072 (Japan); Onodera, Satoshi [Department of Clinical and Pharmaceutical Sciences, Showa Pharmaceutical University, Tokyo 194-8543 (Japan); Ikejima, Takashi, E-mail: ikejimat@vip.sina.com [China-Japan Research Institute of Medical and Pharmaceutical Sciences, Shenyang Pharmaceutical University, Shenyang 110016 (China)

    2015-02-20

    Murine fibrosarcoma L929 cells have been used to test efficacy of proinflammatory cytokine TNFα. In the present study, we reported on protective effect of type I collagen gel used as L929 cell culture. L929 cell grew and proliferated well on collagen gel. However, the L929 cells exhibited cobblestone-like morphology which was much different from the spread fusiform shape when cultured on conventional cell dishes as well as the cells tended to aggregate. On conventional cell culture dishes, the cells treated with TNFα became round in shape and eventually died in a necroptotic manner. The cells cultured on collagen gel, however, were completely unaffected. TNFα treatment was reported to induce autophagy in L929 cells on the plastic dish, and therefore we investigated the effect of collagen gel on induction of autophagy. The results indicated that autophagy induced by TNFα treatment was much reduced when the cells were cultured on collagen gel. In conclusion, type I collagen gel protected L929 cell from TNFα-induced cell death. - Highlights: • Collagen gel culture changed the morphology of L929 cells. • L929 cell cultured on collagen gel were resistant to TNFα-induced cell death. • Collagen gel culture inhibited TNFα-induced autophagy in L929 cells.

  5. Murine liver implantation of radiation-induced fibrosarcoma: characterization with MR imaging, microangiography and histopathology

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Huaijun; Keyzer, Frederik de; Jin, Lixin; Yu, Jie; Marchal, Guy; Ni, Yicheng [Catholic University of Leuven, Department of Radiology, University Hospitals, Leuven (Belgium); Putte, Marie van de; Witte, Peter de [K.U. Leuven, Laboratory for Pharmaceutical Biology, Faculty of Pharmaceutical Sciences, Leuven (Belgium); Chen, Feng [Catholic University of Leuven, Department of Radiology, University Hospitals, Leuven (Belgium); Southeast University, Department of Radiology, Zhong Da Hospital, Nanjing, Jiangsu Province (China)

    2008-07-15

    We sought to establish and characterize a mouse liver tumor model as a platform for preclinical assessment of new diagnostics and therapeutics. Radiation-induced fibrosarcoma (RIF-1) was intrahepatically implanted in 27 C3H/Km mice. Serial in vivo magnetic resonance imaging (MRI) with a clinical 1.5-T-magnet was performed using T1- (T1WI), T2- (T2WI), and diffusion-weighted sequences (DWI), dynamic contrast-enhanced MRI (DCE-MRI), and contrast-enhanced T1WI, and validated with postmortem microangiography and histopathology. Implantation procedure succeeded in 25 mice with 2 deaths from overdosed anesthesia or hypothermia. RIF-1 grew in 21 mice with volume doubling time of 2.55{+-}0.88 days and final size of 216.2{+-}150.4 mm{sup 3} at day 14. Three mice were found without tumor growth and one only with abdominal seeding. The intrahepatic RIF-1 was hypervascularized with negligible necrosis as shown on MRI, microangiography and histology. On DCE-MRI, maximal initial slope of contrast-time curve and volume transfer constant per unit volume of tissue, K, differed between the tumor and liver with only the former significantly lower in the tumor than in the liver (P<0.05). Liver implantation of RIF-1 in mice proves a feasible and reproducible model and appears promising for use to screen new diagnostics and therapeutics under noninvasive monitoring even with a clinical MRI system. (orig.)

  6. Primary Fibrosarcoma of the Liver: We Don’t Know Much

    Directory of Open Access Journals (Sweden)

    Sadaf Ali

    2008-11-01

    Full Text Available A 60-year-old lady presented to us with a right upper abdominal mass. With a clinical diagnosis of liver tumor, she was evaluated with abdominal CT, MRI, nuclear scan, tumor markers, USG guided FNAC and other baseline investigations. On evaluation she had a massive right lobe tumor crossing the midline. In view of the ambiguous diagnosis she was subjected to laparotomy where the lesion was judged unresectable and a biopsy was taken. Histopathological examination showed the rare pathology of primary fibrosarcoma of the liver with features of homogeneous, spindle-shaped cells with abundant collagen fibers showing a classic herringbone pattern. Tissue samples were then sent to another referral cancer hospital for immunohistochemistry and immunoreactive vimentin was found in the tumor cells. Electron microscopically, the tumor cells were rich in rough endoplasmic reticulum without a basement membrane, and were surrounded by large amounts of collagen fibers. The fibroblastic character of the tumor cells was suggested by light and electron microscopy.

  7. Comparative activation states of tumor-associated and peritoneal macrophages from mice bearing an induced fibrosarcoma.

    Science.gov (United States)

    Valdez, J C; de Alderete, N; Meson, O E; Sirena, A; Perdigon, G

    1990-11-01

    Balb/c mice bearing a methylcholanthrene-induced fibrosarcoma were used to compare the activation levels of tumor-associated and peritoneal macrophages. Two stages of tumor growth were examined, namely "small" and "large" tumors, with average diameters of 10 and 30 mm, respectively. The activation state, determined by measurement of both phagocytic index and beta-glucuronidase content, was found to be markedly higher in tumor-associated macrophages than in their peritoneal counterparts and it was, in addition, independent of tumor progression. The percentage of tumor-associated macrophages, which were detected on the basis of Fc receptor expression, remained constant in the growing neoplasm, at approximately 23% of total cell population. None of these parameters were affected by inoculation with an immunopotentiating dose of heat-killed Candida albicans which, on the other hand, seemed not to alter the course of the tumor. These data suggest that within the tumor microenvironment macrophages would somehow be maintained at a constant proportion and at a highly activated state, while outside the tumor they would be at a lower activation level. Our results also suggest that TAM would not possess antitumor activity in vivo, although we have found this activity in vitro.

  8. Mechanistic investigation of toxicity of chromium oxide nanoparticles in murine fibrosarcoma cells

    Directory of Open Access Journals (Sweden)

    Alarifi S

    2016-03-01

    Full Text Available Saud Alarifi, Daoud Ali, Saad Alkahtani Department of Zoology, College of Science, King Saud University, Riyadh, Saudi Arabia Abstract: Chromium oxide nanoparticles (Cr2O3NPs are widely used in polymers and paints. In the present study, we aimed to determine the toxicity of Cr2O3NPs in murine fibrosarcoma (L929 cells. The cytotoxicity of Cr2O3NPs was measured by MTT and neutral red uptake assays; Cr2O3NPs had significant cytotoxic effects on L929 cells. Enhancement of intracellular reactive oxygen species was observed in L929 cells after exposure to Cr2O3NPs. Cr2O3NPs produced caspase-3, indicating that exposure to Cr2O3NPs induced apoptosis. After exposure to Cr2O3NPs, the cellular glutathione level decreased and lipid peroxidation, superoxide dismutase, and catalase increased in a dose- and time-dependent manner. By using single-cell gel tests, we also observed increased DNA damage in a Cr2O3NP exposure-duration- and dose-dependent fashion. Cell toxicity and DNA damage may be useful biomarkers for determining the safety of Cr2O3NPs in human and animal health. Keywords: Cr2O3NPs, L929 cells, MTT assay, oxidative stress 

  9. Murine liver implantation of radiation-induced fibrosarcoma: characterization with MR imaging, microangiography and histopathology

    International Nuclear Information System (INIS)

    Wang, Huaijun; Keyzer, Frederik de; Jin, Lixin; Yu, Jie; Marchal, Guy; Ni, Yicheng; Putte, Marie van de; Witte, Peter de; Chen, Feng

    2008-01-01

    We sought to establish and characterize a mouse liver tumor model as a platform for preclinical assessment of new diagnostics and therapeutics. Radiation-induced fibrosarcoma (RIF-1) was intrahepatically implanted in 27 C3H/Km mice. Serial in vivo magnetic resonance imaging (MRI) with a clinical 1.5-T-magnet was performed using T1- (T1WI), T2- (T2WI), and diffusion-weighted sequences (DWI), dynamic contrast-enhanced MRI (DCE-MRI), and contrast-enhanced T1WI, and validated with postmortem microangiography and histopathology. Implantation procedure succeeded in 25 mice with 2 deaths from overdosed anesthesia or hypothermia. RIF-1 grew in 21 mice with volume doubling time of 2.55±0.88 days and final size of 216.2±150.4 mm 3 at day 14. Three mice were found without tumor growth and one only with abdominal seeding. The intrahepatic RIF-1 was hypervascularized with negligible necrosis as shown on MRI, microangiography and histology. On DCE-MRI, maximal initial slope of contrast-time curve and volume transfer constant per unit volume of tissue, K, differed between the tumor and liver with only the former significantly lower in the tumor than in the liver (P<0.05). Liver implantation of RIF-1 in mice proves a feasible and reproducible model and appears promising for use to screen new diagnostics and therapeutics under noninvasive monitoring even with a clinical MRI system. (orig.)

  10. Fibrosarcoma of the jaws: two cases of primary tumors with intraosseous growth.

    Science.gov (United States)

    Angiero, Francesca; Rizzuti, Tommaso; Crippa, Rolando; Stefani, Michele

    2007-01-01

    Fibrosarcoma (FS) is a malignant mesenchymal neoplasm of the fibroblasts that rarely affects the oral cavity. Two cases of primary FS of the jaws with intraosseous growth (2 men, aged 53 and 71 years) are described. Microscopically, in one case the tumor showed an intense proliferation of spindle-shaped cells, varying little in size and shape and arranged in parallel bands, partly crossing each other, with significant mitotic activity and nuclear pleomorphism; the second case was characterized by low cellularity comprising spindle-shaped cells, deposited in a variably fibrous and myxoid stroma. On immunohistochemistry, cells in both cases were strongly immunoreactive for MIB-1 and vimentin, focally positive for CD68, and negative for S-100 protein, pancytokeratin, HMB45, CD34, desmin, smooth muscle actin (SMA) and epithelial membrane antigen (EMA). Based on clinical, histological and immunohistochemical findings, the final diagnosis was FS in the first case, myxofibrosarcoma in the second. Treatment was radical surgery with mandibular reconstruction. After two years, the first patient displayed multiple metastases and died during the third year after the initial diagnosis; the second patient was still alive and doing well five years after treatment. We discuss the differential diagnosis versus other forms of sarcoma, examining the morphological appearance that is frequently very similar, the immunohistochemical expression of MIB-1, vimentin, S-100, CD-34, CD68, EMA, as well as conventional clinicopathological features that may help to distinguish FS from other sarcomas.

  11. Influencing the radiosensitivity of a transplantable fibrosarcoma by synchronisation with hydroxyurea

    International Nuclear Information System (INIS)

    Kummermehr, J.; Trott, K.R.

    1979-01-01

    Due to its specific S-phase-toxicity and the blocking effect in G 1 /S-transition, hydroxy urea seems to be particularly suitable for testing the possible use of a synchronisation therapy in animal experiments. The injection of 0,5 mg/g, repeated four times, in a transplantable fibrosarcoma of a mouse, caused the accumulation of 70% of the surviving proliferating cells in the radio-sensitive G 1 /S-phase. A slight sensitization, corresponding to a dose modification factor of 1,3, could be manifested compared with a single dose of 600 rd. but not with higher doses. This effect could partly be repeated in franctionated treatment with 2 x 600 rd at 2-day internals. With other intervals and with an extension to triple-fractionated-synchronisation treatment, the total outcome was negative. Due to these results and the situation of human solid tumors which is, cell-kinetically, considerably less favourable, a combined clinical treatment on the basis of synchronisation seems hardly promising. (orig.) 891 MG/orig. 892 RDG [de

  12. 18-F-FDG PET-CT in Monitoring of Chemotherapeutic Effect in a Case of Metastatic Hepatic Epithelioid Hemangioendothelioma.

    Science.gov (United States)

    Shamim, Shamim Ahmed; Tripathy, Sarthak; Mukherjee, Anirban; Bal, Chandrasekhar; Roy, Shambo Guha

    2017-01-01

    Hepatic epithelioid hemangioendothelioma is a rare variant of mesenchymal tumor. Surgical resection or partial hepatectomy is the treatment of choice in the case of localized disease. However, in metastatic cases, chemotherapeutic drugs targeting the tyrosine kinase are being used. We hereby present 18-F-fludeoxyglucose positron emission tomography-computed tomography findings in a case of a 35-year old woman with metastatic HEHE showing significant response to Sorafenib therapy after 6 months.

  13. Sclerosing lymphangitis of the penis associated with marked penile oedema and skin erosions.

    Science.gov (United States)

    Karray, Mehdi; Litaiem, Noureddine; Jones, Mariem; Zeglaoui, Faten

    2017-07-27

    Sclerosing lymphangitis of the penis is a benign, under-reported condition consisting of a asymptomatic firm cord-like swelling around the coronal sulcus of the penis usually affecting men in the second or third decade of life. Penile oedema and erosions are rarely reported. Clinical signs may be remarkable contrasting with the self-limited character of the disease. We report a new case of sclerosing lymphangitis of the penis occurring in a 59-year-old patient marked by penile swelling and several overlying skin erosions, and discuss the clinical features and the pathogenesis aspects of the disease. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  14. Brain CT and MRI findings of a long-term case of subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Aoshiba, Kazunori; Ota, Kohei; Komatsuzaki, Satoshi; Kobayashi, Itsuro; Maruyama, Shoichi

    1987-11-01

    Our study involved a long-term case (ten years) of subacute sclerosing panencephalitis. The case began with a 23 year-old experiencing visual deterioration. During the course of his illness, amnesia, autism and abnormal behavior were observed without any myoclonus. On the electroencephalogram, periodic synclonous discharge was shown in the early stage of his illness and subsequently disappeared. The brain CT and the MRI disclosed diffuse lesions in both cortical and subcortical areas of the cerebral hemispheres. The location and spread of lesions were more clearly revealed by the MRI than the brain CT. These findings suggest that the MRI is more useful than the brain CT in the diagnosis of subacute sclerosing panencephalitis.

  15. Brain CT and MRI findings of a long-term case of subacute sclerosing panencephalitis

    International Nuclear Information System (INIS)

    Aoshiba, Kazunori; Ota, Kohei; Komatsuzaki, Satoshi; Kobayashi, Itsuro; Maruyama, Shoichi

    1987-01-01

    Our study involved a long-term case (ten years) of subacute sclerosing panencephalitis. The case began with a 23 year-old experiencing visual deterioration. During the course of his illness, amnesia, autism and abnormal behavior were observed without any myoclonus. On the electroencephalogram, periodic synclonous discharge was shown in the early stage of his illness and subsequently disappeared. The brain CT and the MRI disclosed diffuse lesions in both cortical and subcortical areas of the cerebral hemispheres. The location and spread of lesions were more clearly revealed by the MRI than the brain CT. These findings suggest that the MRI is more useful than the brain CT in the diagnosis of subacute sclerosing panencephalitis. (author)

  16. Air contamination in the sclerosing foam for the treatment of varicose veins.

    Science.gov (United States)

    de Franciscis, S; Nobile, Cga; Larosa, E; Montemurro, R; Serra, R

    2016-03-01

    Fluids and drugs formulated for intravenous infusion may potentially promote the growth of microorganisms that can cause infections. The aim of this study is to test the sterility of sclerosing foam. Polidocanol was used for the production of the foam. The Tessari method was used in order to generate the foam. The preparation was carried out both in the operating theater and in an outpatient room. A validation test with microorganisms was also performed. The measurements showed no evident growth of microorganisms and in the validation tests the foam appeared to even display bacteriostatic and/or bactericide properties. Sclerosing foam seems to be safe from a microbiological point of view. © The Author(s) 2014.

  17. A Radial Sclerosing Lesion Mimicking Breast Cancer on Mammography in a Young Woman

    Directory of Open Access Journals (Sweden)

    Masashi Furukawa

    2012-02-01

    Full Text Available A spiculated mass on a mammogram is highly suggestive of malignancy. We report the case of a 32-year-old woman with a radial sclerosing lesion that mimicked breast cancer on mammography. She visited her physician after palpating a lump in her left breast. Mammography showed architectural distortion in the upper inner quadrant of the left breast. Ultrasonography showed a low echoic area with an ambiguous boundary. Core needle biopsy was performed because of the suspicion of malignancy. Histological examination did not reveal any malignant cells. After 6 months, the breast lump became larger and the patient was referred to our hospital. Mammography performed in our hospital showed a spiculated mass, and therefore mammotome biopsy was performed. Histological examination revealed dense fibroelastic stroma with a wide variety of mastopathic changes, leading to a diagnosis of a radial sclerosing lesion. One year after the biopsy, the lump on her left breast had disappeared and mammography showed no spiculated mass.

  18. Sclerosing lipogranuloma of the eyelid: unusual complication following nasal packing in endoscopic sinus surgery.

    Science.gov (United States)

    Ramaswamy, Balakrishnan; Singh, Rohit; Manusrut, Manusrut; Hazarika, Manali

    2015-03-06

    An eyelid or orbital lipogranuloma can occur following nasal packing with liquid paraffin, petroleum jelly or an antibiotic-based cream. It usually presents a few weeks or months after the initial procedure. We present a report of three such cases of sclerosing lipogranuloma involving the eyelid, which occurred following a sinonasal surgery where nasal packing using petroleum jelly was performed. The typical clinical course and the classical histopathological features are highlighted. All cases were diagnosed by histopathological examination as sclerosing lipogranuloma. Complete surgical removal resulted in complete resolution on 1 month follow-up. The diagnosis is based on a high degree of suspicion following a detailed history of prior use of lipid-based products for nasal packing following endonasal surgery. Histopathology is diagnostic. Surgical excision is the treatment of choice, however, due to its infiltrative nature, it may be difficult to obtain a complete cure. 2015 BMJ Publishing Group Ltd.

  19. Segmentectomy for giant pulmonary sclerosing haemangiomas with high serum KL-6 levels

    OpenAIRE

    Kuroda, Hiroaki; Mun, Mingyon; Okumura, Sakae; Nakagawa, Ken

    2012-01-01

    We describe a 61-year old female patient with a giant pulmonary sclerosing haemangioma (PSH) and an extremely high preoperative serum KL-6 level. During an annual health screening, the patient showed a posterior mediastinal mass on chest radiography. Chest computed tomography and magnetic resonance imaging revealed a well-circumscribed 60 mm diameter nodule with a marked contrast enhancement in the left lower lobe. The preoperative serum KL-6 level was elevated to 8204 U/ml. We performed a fo...

  20. Isolated nail lichen planus with primary sclerosing cholangitis in a child

    International Nuclear Information System (INIS)

    Al-Ajroush, N.; Al-Khenaizan, S.

    2007-01-01

    Lichen planus (LP) is an uncommon, inflammatory dermatosis with characteristic lesions affecting the skin, nails and the mucous membranes. It is rare in childhood. Although nail abnormalities have been reported in 1-10% of patients with LP, the prevalence of nail involvement in affected children is unknown. Here we report a 2-year-old child with isolated nail LP, in association with primary sclerosing cholangitis. (author)

  1.  Alkaline phosphatase normalization is a biomarker of improved survival in primary sclerosing cholangitis.

    Science.gov (United States)

    Hilscher, Moira; Enders, Felicity B; Carey, Elizabeth J; Lindor, Keith D; Tabibian, James H

    2016-01-01

     Introduction. Recent studies suggest that serum alkaline phosphatase may represent a prognostic biomarker in patients with primary sclerosing cholangitis. However, this association remains poorly understood. Therefore, the aim of this study was to investigate the prognostic significance and clinical correlates of alkaline phosphatase normalization in primary sclerosing cholangitis. This was a retrospective cohort study of patients with a new diagnosis of primary sclerosing cholangitis made at an academic medical center. The primary endpoint was time to hepatobiliaryneoplasia, liver transplantation, or liver-related death. Secondary endpoints included occurrence of and time to alkaline phosphatase normalization. Patients who did and did not achieve normalization were compared with respect to clinical characteristics and endpoint-free survival, and the association between normalization and the primary endpoint was assessed with univariate and multivariate Cox proportional-hazards analyses. Eighty six patients were included in the study, with a total of 755 patient-years of follow-up. Thirty-eight patients (44%) experienced alkaline phosphatase normalization within 12 months of diagnosis. Alkaline phosphatase normalization was associated with longer primary endpoint-free survival (p = 0.0032) and decreased risk of requiring liver transplantation (p = 0.033). Persistent normalization was associated with even fewer adverse endpoints as well as longer survival. In multivariate analyses, alkaline phosphatase normalization (adjusted hazard ratio 0.21, p = 0.012) and baseline bilirubin (adjusted hazard ratio 4.87, p = 0.029) were the only significant predictors of primary endpoint-free survival. Alkaline phosphatase normalization, particularly if persistent, represents a robust biomarker of improved long-term survival and decreased risk of requiring liver transplantation in patients with primary sclerosing cholangitis.

  2. Shear wave elastography diagnosis of the diffuse sclerosing variant of papillary thyroid carcinoma: A case report

    OpenAIRE

    Xue, Nianyu; Xu, Youfeng; Huang, Pintong; Zhang, Shengmin; Wang, Hongwei; Yu, Fei

    2016-01-01

    The present study aimed to report the shear wave elastography (SWE) findings in a patient with the diffuse sclerosing variant of papillary thyroid carcinoma (DSVPTC). Since patients with DSVPTC may present with typical clinicopathological features and initially appear to have Hashimoto's thyroiditis, a thorough clinical evaluation and an early diagnosis are important. A 20-year-old female patient presented with a 1-month history of a neck mass and sore throat. Conventional ultrasound and SWE ...

  3. Case report. Sclerosing peritoneal mesothelioma in a dog: histopathological, histochemical and immunohistochemical investigations

    Directory of Open Access Journals (Sweden)

    Anna Rita D'Angelo

    2014-12-01

    Full Text Available Mesotheliomas are rare neoplasm affecting on rare occasions both animals and humans and which arise from the mesothelial cells lining the coelomic cavities. We report herein the histopathological, histochemical and immunohistochemical findings in a dog affected by sclerosing peritoneal mesothelioma, a rare variant of canine mesothelioma, and submitted to laparotomy in December 2012 (Teramo, Italy. Our data confirm that mesothelioma still represents a diagnostic challenge and that immunohistochemistry can be extremely useful as supportive diagnostic technique.

  4. Pleural epithelioid angiosarcoma with lymphatic differentiation arisen after radiometabolic therapy for thyroid carcinoma: immunohistochemical findings and review of the literature.

    Science.gov (United States)

    Cabibi, Daniela; Pipitone, Giulia; Porcasi, Rossana; Ingrao, Sabrina; Benza, Ignazio; Porrello, Calogero; Cajozzo, Massimo; Giannone, Antonino Giulio

    2017-08-15

    Pleural angiosarcoma is a rare tumor that causes diffuse pleural thickening and effusion, mimicking mesothelioma. Immunohistochemistry is needed to highlight endothelial differentiation. We describe the first case of pleural angiosarcoma with lymphatic differentiation following radiometabolic therapy for thyroid carcinoma. A 50-year-old man showed diffuse pleural thickening and effusion. Nine years earlier, he underwent thyroidectomy and radiometabolic therapy for thyroid carcinoma with lymph node metastases. Histologically, the tumor consisted of a solid proliferation of atypical epithelioid cells and anastomosed vascular spaces, lacking of red blood cells and containing Alcian blue positive material. The tumor showed positive immunostaining for Vimentin, CD31, CK7, D2-40, c-MYC, Ki67, focal positivity for PanCK, and negative immunostaining for Factor VIII, CD34, WT1, CK5/6, Calretinin, EMA, HBME-1, CEA, p63, EpCAM, Bcl-2, TTF1 and Thyroglobulin. CD99 showed a granular/paranuclear pattern of positivity. The histological and immunohistochemical features were consistent with "pleural angiosarcoma with lymphatic differentiation, epithelioid variant". Epithelioid angiosarcoma with lymphatic differentiation is very rare and aggressive. Moreover, the positivity for c-MYC suggests the relationship with radiometabolic therapy. To our knowledge, this is the first case of pleural c-MYC-positive angiosarcoma with lymphatic differentiation reported in the literature and the first one arisen after radiometabolic therapy for thyroid carcinoma.

  5. A Biphasic Pleural Tumor with Features of an Epithelioid and Small Cell Mesothelioma: Morphologic and Molecular Findings

    Directory of Open Access Journals (Sweden)

    Sarah Hackman

    2016-01-01

    Full Text Available Malignant mesotheliomas are generally classified into epithelioid, sarcomatoid, desmoplastic, and biphasic types with rare reports of a small cell form. These small cell variants display some morphologic overlap with desmoplastic small round cell tumors (DSRCTs which generally occur within the abdominal cavity of young males and are defined by a characteristic t(11;22(p13;q12 translocation. However, there are rare reports of DSRCTs lacking this translocation. We present a 78-year-old man with a pleura-based biphasic neoplasm with features of both epithelioid mesothelioma and a small cell blastema-like neoplasm. The epithelioid portion showed IHC reactivity for pan cytokeratin, CK5/6, D2-40, and calretinin and the small cell portion marked with CD99, pan cytokeratin, WT1, FLI1, S100, CD200, MyoD1, and CD15. Fluorescence in situ hybridization testing for the t(11;22(p13;q12 translocation disclosed loss of the EWSR1 gene in 94% of tumor cell nuclei, but there was no evidence of the classic translocation. Array based-comparative genomic hybridization (a-CGH confirmed the tumor had numerous chromosome copy number losses, including 11p15.5-p11.12 and 22q12.1-q13.33, with loss of the EWSR1 and WT1 gene regions. Herein, we report novel complex CGH findings in a biphasic tumor and review the molecular genetic alterations in both mesothelioma and DSRCTs.

  6. The B-mode Sonography and Sonoelastographic Features of Sclerosing Adenosis of the Breast

    International Nuclear Information System (INIS)

    Myong, Joo Hwa; Kim, Sung Hun; Kang, Bong Joo; Ahn, Young I; Yoon, Soo Kyoung; Lee, A Won; Yim, Kwang Il; Kim, Tae Eun; Song, Byung Joo

    2011-01-01

    The purpose of this study was to evaluate the B-mode sonographic and sonoelastographic features of high risk lesions of the breast. From April 2009 to February 2010, 1390 patients with breast lesions underwent US-guided core-biopsy. Among them, 13 lesions were confirmed to be pure sclerosing adenosis by subsequent surgical excision or on imaging follow-up of more than 1 year. Two radiologists retrospectively analyzed the B-mode sonography according to the Breast Imaging Reporting and Data System classification. The sonoelastographic images were classified into 5 elasticity scores according to the Itoh classification and the strain ratio between the mass and the surrounding fat tissue was reviewed. We considered the sonoelastographic patterns to be suspicious for the case with a score of 4 and 5 and a strain ratio of more than a 2.24. The common B-mode sonographic features of sclerosing adenosis were an irregular shape (69.2%, 9 of 13), an indistinct margin (92.3%, 12 of 13), hypoechogenicity (76.9%, 10 of 13) and category 4A, a low suspicion of malignancy (61.5%, 8 of 13). The common sonoelastographic features were a score of 2 (42%, 6 of 13) and a strain ratio < 2.24 (69.2%, 9 of 13). Sclerosing adenosis showed suspicious B-mode sonographic findings, but it had benign sonolastographic features

  7. The B-mode Sonography and Sonoelastographic Features of Sclerosing Adenosis of the Breast

    Energy Technology Data Exchange (ETDEWEB)

    Myong, Joo Hwa; Kim, Sung Hun; Kang, Bong Joo; Ahn, Young I; Yoon, Soo Kyoung; Lee, A Won; Yim, Kwang Il; Kim, Tae Eun; Song, Byung Joo [Seoul St. Mary' s Hospital, The Catholic University, Seoul (Korea, Republic of)

    2011-06-15

    The purpose of this study was to evaluate the B-mode sonographic and sonoelastographic features of high risk lesions of the breast. From April 2009 to February 2010, 1390 patients with breast lesions underwent US-guided core-biopsy. Among them, 13 lesions were confirmed to be pure sclerosing adenosis by subsequent surgical excision or on imaging follow-up of more than 1 year. Two radiologists retrospectively analyzed the B-mode sonography according to the Breast Imaging Reporting and Data System classification. The sonoelastographic images were classified into 5 elasticity scores according to the Itoh classification and the strain ratio between the mass and the surrounding fat tissue was reviewed. We considered the sonoelastographic patterns to be suspicious for the case with a score of 4 and 5 and a strain ratio of more than a 2.24. The common B-mode sonographic features of sclerosing adenosis were an irregular shape (69.2%, 9 of 13), an indistinct margin (92.3%, 12 of 13), hypoechogenicity (76.9%, 10 of 13) and category 4A, a low suspicion of malignancy (61.5%, 8 of 13). The common sonoelastographic features were a score of 2 (42%, 6 of 13) and a strain ratio < 2.24 (69.2%, 9 of 13). Sclerosing adenosis showed suspicious B-mode sonographic findings, but it had benign sonolastographic features

  8. MDM2 Amplification and PI3KCA Mutation in a Case of Sclerosing Rhabdomyosarcoma

    Directory of Open Access Journals (Sweden)

    Ken Kikuchi

    2013-01-01

    Full Text Available A rare sclerosing variant of rhabdomyosarcoma characterized by prominent hyalinization and pseudovascular pattern has recently been described as a subtype biologically distinct from embryonal, alveolar, and pleomorphic forms. We present cytogenetic and molecular findings as well as experimental studies of an unusual case of sclerosing rhabdomyosarcoma. The primary lesion arose within the plantar subcutaneous tissue of the left foot of an otherwise healthy 23-year-old male who eventually developed pulmonary nodules despite systemic chemotherapy. Two genetic abnormalities identified in surgical and/or autopsy samples of the tumor were introduced into 10T1/2 murine fibroblasts to determine whether these genetic changes cooperatively facilitated transformation and growth. Cytogenetic analysis revealed a complex abnormal hyperdiploid clone, and MDM2 gene amplification was confirmed by fluorescence in situ hybridization. Cancer gene mutation screening using a combination of multiplexed PCR and mass spectroscopy revealed a PIK3CA exon 20 H1047R mutation in the primary tumor, lung metastasis, and liver metastasis. However, this mutation was not cooperative with MDM2 overexpression in experimental assays for transformation or growth. Nevertheless, MDM2 and PIK3CA are genes worthy of further investigation in patients with sclerosing rhabdomyosarcoma and might be considered in the enrollment of these patients into clinical trials of targeted therapeutics.

  9. Sensitization of a transplantable murine fibrosarcoma by partial synchronization with hydroxyurea

    International Nuclear Information System (INIS)

    Kummermehr, J.; Trott, K.R.; Gesellschaft fuer Strahlen- und Umweltforschung m.b.H., Neuherberg/Muenchen

    1977-01-01

    A C3H fibrosarcoma was synchronized by an injection schedule of hydroxyurea adjusted to its proliferation kinetics. By imposing an 8 h block, 70% of the proliferating non-S-phase cells were accumulated and entered the S-phase synchronously between 2 and 3 h after the last injection. Progression of S-phase cells exposed to hydroxyurea was severely disturbed whereas progression of the synchronized population was nearly normal. Tumours were given a local irradiation with single doses of 300kV X-rays either 2 h after the last hydroxyurea injection, when the synchronized population was still at the G 1 /S-border, or 6 h after the last injection, when cells had proceeded into mid S-phase. Doses ranged from 600 rad to 2400 rad and were given under ambient or hypoxic conditions. The tumour volume was measured at regular intervals. The median regrowth delay of tumours irradiated in air without hydroxyurea displayed a typical biphasic dose/response curve. Tumours irradiated 6 h after hydroxyurea injection showed the same response. For tumours irradiated 2 h after hydroxyurea injection, a significant increase in delay time was found after 600 rad had been given in air, but not after higher doses. A similar sensitizing effect could be detected after 2400 rad had been given under hypoxic conditions. The sensitizing effect of synchronization could be repeated 2 days after a first dose of 600 rad but not after 3 or 4 days. Repeating the synchronization and 600 rad irradiation schedule three times was less effective than irradiating with 3 fractions of 600 rad in 4 days. (author)

  10. Nano-scaled particles of titanium dioxide convert benign mouse fibrosarcoma cells into aggressive tumor cells.

    Science.gov (United States)

    Onuma, Kunishige; Sato, Yu; Ogawara, Satomi; Shirasawa, Nobuyuki; Kobayashi, Masanobu; Yoshitake, Jun; Yoshimura, Tetsuhiko; Iigo, Masaaki; Fujii, Junichi; Okada, Futoshi

    2009-11-01

    Nanoparticles are prevalent in both commercial and medicinal products; however, the contribution of nanomaterials to carcinogenesis remains unclear. We therefore examined the effects of nano-sized titanium dioxide (TiO(2)) on poorly tumorigenic and nonmetastatic QR-32 fibrosarcoma cells. We found that mice that were cotransplanted subcutaneously with QR-32 cells and nano-sized TiO(2), either uncoated (TiO(2)-1, hydrophilic) or coated with stearic acid (TiO(2)-2, hydrophobic), did not form tumors. However, QR-32 cells became tumorigenic after injection into sites previously implanted with TiO(2)-1, but not TiO(2)-2, and these developing tumors acquired metastatic phenotypes. No differences were observed either histologically or in inflammatory cytokine mRNA expression between TiO(2)-1 and TiO(2)-2 treatments. However, TiO(2)-2, but not TiO(2)-1, generated high levels of reactive oxygen species (ROS) in cell-free conditions. Although both TiO(2)-1 and TiO(2)-2 resulted in intracellular ROS formation, TiO(2)-2 elicited a stronger response, resulting in cytotoxicity to the QR-32 cells. Moreover, TiO(2)-2, but not TiO(2)-1, led to the development of nuclear interstices and multinucleate cells. Cells that survived the TiO(2) toxicity acquired a tumorigenic phenotype. TiO(2)-induced ROS formation and its related cell injury were inhibited by the addition of antioxidant N-acetyl-l-cysteine. These results indicate that nano-sized TiO(2) has the potential to convert benign tumor cells into malignant ones through the generation of ROS in the target cells.

  11. Nano-Scaled Particles of Titanium Dioxide Convert Benign Mouse Fibrosarcoma Cells into Aggressive Tumor Cells

    Science.gov (United States)

    Onuma, Kunishige; Sato, Yu; Ogawara, Satomi; Shirasawa, Nobuyuki; Kobayashi, Masanobu; Yoshitake, Jun; Yoshimura, Tetsuhiko; Iigo, Masaaki; Fujii, Junichi; Okada, Futoshi

    2009-01-01

    Nanoparticles are prevalent in both commercial and medicinal products; however, the contribution of nanomaterials to carcinogenesis remains unclear. We therefore examined the effects of nano-sized titanium dioxide (TiO2) on poorly tumorigenic and nonmetastatic QR-32 fibrosarcoma cells. We found that mice that were cotransplanted subcutaneously with QR-32 cells and nano-sized TiO2, either uncoated (TiO2−1, hydrophilic) or coated with stearic acid (TiO2−2, hydrophobic), did not form tumors. However, QR-32 cells became tumorigenic after injection into sites previously implanted with TiO2−1, but not TiO2−2, and these developing tumors acquired metastatic phenotypes. No differences were observed either histologically or in inflammatory cytokine mRNA expression between TiO2−1 and TiO2−2 treatments. However, TiO2−2, but not TiO2−1, generated high levels of reactive oxygen species (ROS) in cell-free conditions. Although both TiO2−1 and TiO2−2 resulted in intracellular ROS formation, TiO2−2 elicited a stronger response, resulting in cytotoxicity to the QR-32 cells. Moreover, TiO2−2, but not TiO2−1, led to the development of nuclear interstices and multinucleate cells. Cells that survived the TiO2 toxicity acquired a tumorigenic phenotype. TiO2-induced ROS formation and its related cell injury were inhibited by the addition of antioxidant N-acetyl-l-cysteine. These results indicate that nano-sized TiO2 has the potential to convert benign tumor cells into malignant ones through the generation of ROS in the target cells. PMID:19815711

  12. The Preventive and Curative Effects of Fresh Garlic Extract And its Aerial Parts on Fibrosarcoma in Balb/c Mice

    Directory of Open Access Journals (Sweden)

    M Rafieian-kopaei

    2012-05-01

    Full Text Available Introduction: Garlic has been demonstrated to have anticancer activity in some studies; however its effect on fibrosarcoma is not evident. This study intends to examine the preventive and curative effects of fresh garlic extract and its aerial parts on the growth of WEHI-164 fibrosarcoma cells in Balb/c mice. Methods: In this preclinical study, 48 female inbred Balb/c mice(6 to 7 weeks old were divided in to 6 groups of 8 each. A single aliquot of WEHI-164 cells(5 × 106 cells/100 μl was injected subcutaneously in the chest of animal. Two weeks before or three weeks after cell injection, 0.2 cc of normal saline or 20 mg/kg extract of garlic or its aerial parts were injected intraperitoneally(IP to the Balb/c mice. The tumors sizes were compared with each other, using ANOVA test. The antioxidant potential and total phenolic compounds of the extracts were also assessed. Results: The mean sizes of tumor growth in groups which received fresh garlic extract or its aerial parts were smaller than that of control group. However this difference was significant on the 21st day only in garlic extract group(p<0.05. The antioxidant power of fresh garlic involved 35.6%, whereas for its aerial parts it was 15.3%. Moreover, the general amount of phenol in fresh garlic was 12.61 mg/g and in its aerial parts was 2.44 mg/g. Conclusion: Garlic consumption might have a crucial role in prevention and control of fibrosarcoma growth. Furthermore, the phenolic compounds and antioxidant activity of garlic aerial parts are less in comparison to garlic itself, however, higher doses might have anticancer activity

  13. Epithelioid hemangioendothelioma of the spine treated with RapidArc volumetric-modulated radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Guy, Jean-Baptiste; Trone, Jane-Chloé [Department of Radiotherapy, Institut de Cancérologie de la Loire, St Priest en Jarez (France); Chargari, Cyrus [Department of Radiation Oncology, HIA du Val de Grâce, Paris (France); Falk, Alexander Tuan [Department of Radiation Oncology, Centre Antoine Lacassagne, Nice (France); Khodri, Mustapha [Department of Physics, Institut de Cancérologie de la Loire, St Priest en Jarez (France); Magné, Nicolas, E-mail: nicolas.magne@icloire.fr [Department of Radiotherapy, Institut de Cancérologie de la Loire, St Priest en Jarez (France)

    2014-10-01

    Radiotherapy for epithelioid hemangioendothelioma (EHE) using volumetric intensity-modulated arc radiotherapy (VMAT). A 48-year-old woman was referred for curative irradiation of a vertebral EHE after failure of surgery. A comparison between VMAT and conventional conformal tridimensional (3D) dosimetry was performed and potential advantage of VMAT for sparing critical organs from irradiation's side effects was discussed. The total delivered dose on the planning target volume was 54 Gy in 27 fractions. The patient was finally treated with VMAT. The tolerance was excellent. There was no acute toxicity, including no increase in pain. With a follow-up of 18 months, no delayed toxicity was reported. The clinical response consisted of a decrease in the dorsal pain. The D{sub max} for the spinal cord was reduced from 55 Gy (3D-radiotherapy [RT]) (which would be an unacceptable dose to the spine because of the risk of myelopathy) to 42.8 Gy (VMAT), which remains below the recommended dose threshold (45 Gy). The dose delivered to 20% of organ volume (D{sub 20}) was reduced from 47 Gy (3D-RT) to 3 Gy (VMAT) for the spinal cord. The study shows that VMAT allows the delivery of curative treatment for vertebral EHEs because of critical organ sparing.

  14. Diffuse Hepatic Epithelioid Hemangioendothelioma Developed in a Patient with Hepatitis C Cirrhosis

    Directory of Open Access Journals (Sweden)

    Pedro W. Baron

    2014-01-01

    Full Text Available Hepatic epithelioid hemangioendothelioma (HEHE is an infrequent vascular tumor of endothelial origin that primarily occurs in women in the mid-fifth decade of life without underlying chronic liver disease or cirrhosis. Liver transplant should be the first-line of therapy in patients with large or diffuse unresectable tumors even in the presence of metastatic disease due to the favorable long-term outcome. We report the case of a 48-year-old female who complained of abdominal pain and weight loss. She has a history of cirrhosis secondary to chronic hepatitis C (HCV and was treated with interferon and ribavirin with sustained virological response. Her work-up revealed multiple confluent infiltrating bilobar liver masses diagnosed as HEHE. She underwent a successful liver transplant without evidence of recurrent HCV infection. She developed cervical spine (C4-C6 HEHE metastases 4 years after transplant. She underwent surgical resection and local radiotherapy after resection with good clinical response. To the best of our knowledge, this is the first report of HEHE that developed in a patient with HCV cirrhosis successfully treated with antiviral therapy before transplant and liver transplant with good allograft function without evidence of recurrent liver tumor or HCV infection but developed metastases to the cervical spine 4 years after transplant.

  15. Emmprin, released as a microvesicle in epithelioid sarcoma, interacts with fibroblasts.

    Science.gov (United States)

    Aoki, Mikiko; Koga, Kaori; Hamasaki, Makoto; Egawa, Nagayasu; Nabeshima, Kazuki

    2017-06-01

    Emmprin (extracellular matrix metalloproteinase inducer, CD147) is a glycosylated transmembrane protein, consisting of two immunoglobulin domains, that stimulates the production of matrix metalloproteinases (MMPs) by tumor-associated fibroblasts. These effects play important roles in tumor invasion and metastasis. However, the precise mechanisms by which emmprin acts on fibroblasts have not been fully elucidated, especially in sarcoma cells. Previously, we demonstrated that emmprin, expressed in conditioned medium collected from the epithelioid sarcoma cell line (FU-EPS-1), stimulates MMP-2 production via interactions with fibroblasts. In this study, we used microvesicles derived from sarcoma cells, and determined whether emmprin exists in the microvesicles, which enhance the production of MMP-2 via fibroblasts. Microvesicles released from FU-EPS-1 cells were shown to contain full-length emmprin, identified as a 45-kDa protein characterized by polylactosamine glycosylation. Microvesicles collected from FU-EPS-1 cells transfected with emmprin-specific siRNA or transduced with shRNA displayed significantly reduced MMP-2 production by fibroblasts compared with those from control-transfected cells. Our findings show that emmprin is released through microvesicle shedding in sarcoma cells, and emmprin in microvesicles regulates MMP-2 production by influencing the activity of fibroblasts located at sites distant from the tumor cells.

  16. Intrahepatic peribiliary perivascular epithelioid cell tumor (PEComa) associated with heterotopic pancreas: A case report.

    Science.gov (United States)

    Kiriyama, Yuka; Tsukamoto, Tetsuya; Mizoguchi, Yoshikazu; Ishihara, Shin; Horiguchi, Akihiko; Tokoro, Takamasa; Kato, Yutaro; Sugioka, Atsushi; Kuroda, Makoto

    2016-08-20

    Perivascular epithelioid-cell tumor (PEComa) is a group of rare mesenchymal neoplasms that express myomelanocytic-cell markers and exhibit a wide variety of histopathological features. Although heterotopic pancreas has been reported to occur in the gastrointestinal tract, intrahepatic heterotopic pancreas has been reported only rarely. We present a case of intrahepatic PEComa that showed a strong regional correlation with the presence of heterotopic pancreas. An intrahepatic tumor and biliary dilatation was incidentally discovered during a diagnostic evaluation to investigate low-back pain in a 47-year-old Japanese male. Cholangiocarcinoma was suspected and a left hemihepatectomy performed. Histological examination revealed a 3 × 3.8-mm tumor in the neighboring B2 bile duct. Histological and immunohistochemical investigations revealed the presence of a PEComa and pancreatic acini within the tumor mass. PEComa in the hepatobiliary and pancreatic regions are extremely rare. The presence of heterotopic pancreas is also relatively uncommon. The strong regional association of these 2 lesions raises the possibility of a PEComa originating from heterotopic pancreas or from an irritable response caused by heterotopic pancreas.

  17. Hepatic Epithelioid Hemangioendothelioma Presenting as an Enlarging Vascular Lesion within the Spleen

    Directory of Open Access Journals (Sweden)

    Juliet A. Emamaullee

    2018-01-01

    Full Text Available Epithelioid hemangioendothelioma (EHE is a rare vascular neoplasm with variable malignant potential that most often presents within the liver. Many patients present with bilobar or extrahepatic disease, and the current treatment paradigm involves liver transplantation, with favorable long term results. Up to 25% of patients are diagnosed incidentally following imaging for other indications, and confirmation of diagnosis requires histologic analysis, as there are no classical imaging features to distinguish hepatic EHE (HEHE from other solid hepatic lesions. Here we describe a case of microscopic HEHE that was diagnosed following splenectomy for an enlarging vascular tumor within the spleen. Due to the unexpected diagnosis of EHE within the spleen and coexisting but stable appearing liver hemangiomata, a left hepatic lobectomy was performed. Explant histology revealed benign hemangiomata and diffuse, microscopic HEHE. The patient ultimately underwent liver transplantation. HEHE can be a challenging diagnosis, and this case emphasizes that any enlarging vascular lesion, even within the spleen, should prompt a high index of suspicion for HEHE in the setting of known hemangiomata.

  18. Ameloblastic fibrosarcoma of the upper jaw: Report of a rare case with long-term follow-up

    Directory of Open Access Journals (Sweden)

    Maryam Khalili

    2013-01-01

    Full Text Available Ameloblastic fibrosarcoma (AFS is a rare malignant mixed odontogenic tumor which is usually considered as the malignant counterpart of ameloblastic fibroma. Only mesenchymal component represents sarcomatous alterations and ameloblast-like epithelial nest remains bland in AFS. Here, we report a case of AFS in a 26-year-old man in the maxilla, which was regarded as an uncommon location for this tumor. After 2 years follow up, no evidence of recurrence was noted. We also emphasize on comprehensive clinical, radiographic, and histopathologic evaluation of such patients rather than immunohistochemical staining to make an accurate diagnosis.

  19. Radiotherapy after surgery to dermato-fibrosarcoma protuberans of large dimension. Review of the literature and case report

    International Nuclear Information System (INIS)

    Bastos, Erika Malheiros; Santos, Ivan Dunshee de Abranches Oliveira; Ferreira, Lydia Masako; Paiva, Geruza Rezende; Brunstein, Flavia; Lima, Alessandra Haddad de; Segreto, Roberto

    2004-01-01

    Dermato-fibrosarcoma protuberans is a low incidence skin neoplasia of difficult management due to its high recurrence rate. Usual treatment consists of a large resection, some times causing functional and aesthetics impairment, and despite these efforts, published data point to a recurrence rate of 11% to 53%. Recently, radiotherapy has been used modifying the unreal course of this tumor, decreasing drastically the recurrence rate when compared to surgery only. We reviewed these treatment modalities and related three cases of large recurrent tumors treated in our plastic surgery department with surgery and adjuvant radiotherapy, without recurrences after 2 years follow up. (author)

  20. Correlation between expressions of hypoxia -inducible factor (HIF-1α, blood vessels density, cell proliferation, and apoptosis intensity in canine fibromas and fibrosarcomas

    Directory of Open Access Journals (Sweden)

    Madej Janusz A.

    2014-03-01

    Full Text Available The study aimed to demonstrate the expression of hypoxia-inducible factor (HIF-1α in soft tissue mesenchymal tumours (fibroma and fibrosarcoma in dogs. An attempt was made to correlate the obtained results with density of blood vessels (expression of von Willebrand Factor, vWF, expression of Ki-67 proliferation antigen, and with intensity of apoptosis in studied tumours. The study was performed on paraffin sections of 15 fibromas and 40 fibrosarcomas sampled from 55 female dogs aged 6 to 16 years. Immunohistochemical staining against HIF-1α, vWF, and Ki-67 was performed. Apoptosis was detected with the use of TUNEL reaction. A significantly higher HIF-1α expression was noted in fibrosarcomas in comparison to fibromas (P < 0.0001. HIF-1α expression in fibromas manifested strong positive correlation with tumour vascularity (r = 0.67, P = 0.007. Moreover, HIF-1α expression in fibrosarcomas manifested a moderate positive correlation with tumour malignancy grade (r = 0.44, P = 0.004, tumour vascularity (r = 0.52, P < 0.001, Ki-67 antigen expression (r = 0.42; P = 0.007, and TUNELpositive cells (r = 0.37, P = 0.017. Expression of HIF-1α was detected in 86.7% of fibroma type tumours and in 100% of fibrosarcomas. In all studied tumours expression of HIF-1α manifested positive correlation with the density of blood vessels, and in fibrosarcomas it correlated also with malignancy grade, intensity of Ki-67 expression, and with intensity of apoptosis in tumour cells.

  1. Primary (Poorly Differentiated Sclerosing Liposarcoma of Temporal Region. An Uncommon Tumor in a Rare Site: A Case Report

    Directory of Open Access Journals (Sweden)

    Anuradha CK Rao

    2015-02-01

    Full Text Available Liposarcoma (LS in the head and neck region is a rare tumor. The sclerosing variant of LS is a subtype of well-differentiated LS characterized by areas of conventional LS admixed with hypocellular areas of stromal sclerosis that show atypical lipomatous cells. The (poorly differentiated sclerosing LS, on the other hand, is more cellular with atypical, pleomorphic and often bizarre giant tumor cells admixed with atypical lipoblasts. We report a case of poorly differentiated sclerosing LS of temporal region in a 49-year-old man. Radiologically, the tumor was dumbbell shaped with intra and extra cranial extension. In this case, we discuss the clinico-radiological and pathological findings of an unusual tumor in a rare location. [J Interdiscipl Histopathol 2015; 3(1.000: 33-35

  2. Effects of HIF-1 inhibition by chetomin on hypoxia-related transcription and radiosensitivity in HT 1080 human fibrosarcoma cells

    International Nuclear Information System (INIS)

    Staab, Adrian; Einsele, Hermann; Flentje, Michael; Vordermark, Dirk; Loeffler, Jürgen; Said, Harun M; Diehlmann, Désirée; Katzer, Astrid; Beyer, Melanie; Fleischer, Markus; Schwab, Franz; Baier, Kurt

    2007-01-01

    Hypoxia-inducible factor-1 (HIF-1) overexpression has been linked to tumor progression and poor prognosis. We investigated whether targeting of HIF-1 using chetomin, a disrupter of the interaction of HIF-1 with the transcriptional coactivator p300, influences the radiosensitivity of hypoxic HT 1080 human fibrosarcoma cells. Optimal dose of chetomin was determined by EGFP-HRE gene reporter assay in stably transfected HT 1080 cells. Cells were assayed for expression of the hypoxia-inducible genes carbonic anhydrase 9 (CA9) and vascular endothelial growth factor (VEGF) by RT-PCR and for clonogenic survival after irradiation with 2, 5 or 10 Gy, under normoxic or hypoxic (0.1% O 2 , 12 h) conditions in the presence or absence of chetomin (150 nM, 12 h, pre-treatment of 4 h). Chetomin treatment significantly reduced CA9 and VEGF mRNA expression in hypoxic cells to 44.4 ± 7.2% and 39.6 ± 16.0%, respectively, of untreated hypoxic controls. Chetomin clearly reduced the modified oxygen enhancement ratio (OER') compared to untreated cells, from 2.02 to 1.27, from 1.86 to 1.22 and from 1.49 to 1.06 at the 50%, 37% and 10% clonogenic survival levels, respectively. HIF-1 inhibition by chetomin effectively reduces hypoxia-dependent transcription and radiosensitizes hypoxic HT 1080 human fibrosarcoma cells in vitro

  3. Proteomic Differences in Feline Fibrosarcomas Grown Using Doxorubicin-Sensitive and -Resistant Cell Lines in the Chick Embryo Model

    Directory of Open Access Journals (Sweden)

    Katarzyna Zabielska-Koczywąs

    2018-02-01

    Full Text Available Proteomic analyses are rapid and powerful tools that are used to increase the understanding of cancer pathogenesis, discover cancer biomarkers and predictive markers, and select and monitor novel targets for cancer therapy. Feline injection-site sarcomas (FISS are aggressive skin tumours with high recurrence rates, despite treatment with surgery, radiotherapy, and chemotherapy. Doxorubicin is a drug of choice for soft tissue sarcomas, including FISS. However, multidrug resistance is one of the major causes of chemotherapy failure. The main aim of the present study was to identify proteins that differentiate doxorubicin-resistant from doxorubicin-sensitive FISS using two-dimensional gel electrophoresis (2DE, followed by matrix-assisted laser desorption ionisation time-of-flight mass spectrometry (MALDI-TOF MS analysis. Using the three-dimensional (3D preclinical in ovo model, which resembles features of spontaneous fibrosarcomas, three significantly (p ≤ 0.05 differentially expressed proteins were identified in tumours grown from doxorubicin-resistant fibrosarcoma cell lines (FFS1 and FFS3 in comparison to the doxorubicin-sensitive one (FFS5: Annexin A5 (ANXA5, Annexin A3 (ANXA3, and meiosis-specific nuclear structural protein 1 (MNS1. Moreover, nine other proteins were significantly differentially expressed in tumours grown from the high doxorubicin-resistant cell line (FFS1 in comparison to sensitive one (FFS5. This study may be the first proteomic fingerprinting of FISS reported, identifying potential candidates for specific predictive biomarkers and research targets for doxorubicin-resistant FISS.

  4. Case report: A female case of isolated IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma.

    Science.gov (United States)

    Xiao, Jianchun; Li, Guanqiao; Yang, Gang; Jia, Congwei; Li, Binglu

    2017-04-01

    IgG4-related disease is a newly recognized fibroinflammatory disorder, characterized by tumefactive lesions, storiform fibrosis and IgG4-positive plasma cells infiltration. IgG4-related sclerosing cholangitis (IgG4-SC) is the most common extrapancreatic manifestation of IgG4-related disease, but it is frequently associated with autoimmune pancreatitis(AIP). Only few case was reported to be diagnosed with IgG4-SC in the absence of AIP, with a striking male preponderance. Here we report a female case of isolated IgG4 related sclerosing cholangitis mimicking cholangiocarcinoma. A 58-year-old woman complaint of one-month history of jaundice and right upper quadrant discomfort, and the biliary reconstruction showed full-length wall thickening and segmental stenosis. Cholangiocarcinoma was then diagnosed. Choledochoplasty was performed, followed by Roux-en-Y anastomosis. However, pathological examination revealed IgG4-related sclerosing cholangitis (IgG4-SC) and the retrospective measurement of serum IgG4 was 346 mg/dL post-operatively. The patient was followed for another nine monthswithout recurrence. The differential diagnosis between cholangiocarcinoma and IgG4-SC is challenging due to significant overlap of clinical manifestations, lab tests and imaging characteristics. However, as an afterthought of this case, typical cholangiocarcinoma rarely presents full-length wall thickening. What the case taught us was pre-operative IgG4 measurement for patients with long bile duct involvement was highly recommended in order to rule out IgG4-SC.

  5. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study.

    Science.gov (United States)

    Gregorio, G V; Portmann, B; Karani, J; Harrison, P; Donaldson, P T; Vergani, D; Mieli-Vergani, G

    2001-03-01

    To investigate whether sclerosing cholangitis with an autoimmune serology characteristic of autoimmune hepatitis (AIH) and AIH are distinct entities, we studied 55 consecutive children with clinical and/or biochemical evidence of liver disease and circulating antinuclear (ANA), anti-smooth muscle (SMA), and/or liver-kidney-microsomal type 1 (LKM1) autoantibodies. They underwent liver biopsy, direct cholangiography, sigmoidoscopy, and rectal biopsy at presentation. Twenty-eight were diagnosed as AIH in the absence and 27 autoimmune sclerosing cholangitis (ASC) in the presence of radiological features of cholangiopathy. Twenty-six ASC and 20 AIH had ANA and/or SMA; 1 ASC and 8 AIH LKM1 autoantibody. Similarities between the 2 conditions included most clinical and biochemical parameters and a lower frequency of HLA DR4. Inflammatory bowel disease and histological biliary changes were more common in ASC; coagulopathy, hypoalbuminemia, lymphocytic periportal hepatitis, and HLA DR3 were more common in AIH. Histological biliary changes were observed in 65% of ASC and 31% of AIH patients. Eighty-nine percent responded to immunosuppression. Follow-up liver biopsies from 17 ASC and 18 AIH patients had similarly reduced inflammatory activity and no progression to cirrhosis. Sixteen follow-up cholangiograms from AIH patients and 9 from ASC patients were unchanged, while 8 ASC patients showed a progressive cholangiopathy. One child with AIH and ulcerative colitis developed sclerosing cholangitis 8 years after presentation. At 2 to 16 years (median, 7 years) from presentation, all patients are alive, including 4 ASC patients who underwent liver transplantation. In conclusion, ASC and AIH are similarly prevalent in childhood; cholangiography is often needed to distinguish between these 2 entities, which are likely to lie within the same disease process.

  6. HMB-45 negative multifocal malignant perivascular epithelioid cell tumor of the soft tissue responding to sirolimus: First case report from India.

    Science.gov (United States)

    Kapoor, Akhil; Beniwal, Surender; Singhal, Mukesh Kumar; Kumar, Narender; Kumar, Vanita; Kumar, Harvindra Singh

    2015-01-01

    Perivascular epithelioid cell tumor (PEComa) is a group of sarcomas that exhibit a myomelanocytic phenotype and possess a unique cell type in the perivascular epithelioid cell. Traditionally HMB-45 immunoreactivity is the first criteria required to consider a tumor to be PEComa. We report a case of multifocal PEComa with negative HMB-45 marker. The patient presented with three big ulceroproliferative lesions; two over right thigh and one over the scalp in the right frontal region. The patient was prescribed with oral sirolimus to which good response was seen. To the best of our knowledge, this is the first case of HMB-45 negative multifocal malignant PEComa from India.

  7. Primary sclerosing cholangitis associated with increased peripheral eosinophils and serum IgE.

    Science.gov (United States)

    Shimomura, I; Takase, Y; Matsumoto, S; Kuyama, J; Nakajima, T; Maeda, H; Sugase, T; Hata, A; Hanada, M; Okuno, M

    1996-10-01

    Symptoms of cholestasis, including epigastralgia, fever, and jaundice, with marked increases in peripheral eosinophils and serum IgE in a 20-year-old man are reported here. Endoscopic retrograde cholangio-pancreatography (ERCP) detected constrictions of the bile ducts, compatible with primary sclerosing cholangitis (PSC). The symptoms and blood parameters of liver dysfunction were associated with the degree of eosinophilia and high serum IgE levels. During corticosteroid therapy, all of these parameters improved, and morphologic improvements of the bile ducts were also observed. The pathogenesis of PSC may be explained, in part, by the concept of hypereosinophilic syndrome or allergic reaction.

  8. Radiological diagnosis of primary sclerosing cholangitis: value of ERC and CT

    International Nuclear Information System (INIS)

    Kollmann, F.D.; Maeurer, J.; Hintze, R.E.; Adler, A.; Veltzke, W.; Lohmann, R.; Felix, R.

    1994-01-01

    To evaluate the use of computed tomography (CT) in primary sclerosing cholangitis (PSC) as compared to endoscopic-retrograde cholangiography (ERC), imaging studies of 24 patients were reviewed. 19 patients were studied by ERC, 19 by CT and 14 by both. In 17 cases, ERC confirmed PSC. One cholangiogram suggested a tumor. CT reflected PSC in only 11 cases, while three patients displayed a mass lesion. Thus, ERC remains the standard imaging technique for diagnosing PSC, whereas CT proves beneficial in excluding hepatic masses. (orig.) [de

  9. Garre's chronic diffuse sclerosing osteomyelitis of the sacrum: a rare condition mimicking malignancy.

    LENUS (Irish Health Repository)

    Nasir, N

    2012-02-03

    Garre\\'s chronic diffuse sclerosing osteomyelitis (DSOM) is a rare disease that occurs most commonly in the mandible. We present a case of sacral DSOM that simulated an expanding destructive sacral tumour. Treatment was conducted on the basis of the available experience with the mandibular form of the disease, with partial symptomatic relief, but progressive sclerosis of the sacral lesion. To the best of our knowledge, this is the first case initially presenting in the sacrum. As an osteolytic expanding lesion simulating malignancy, it is important to recognize this entity in the sacrum.

  10. Immunoglobulin G4-Related Sclerosing Disease Involving the Urethra: Case Report

    International Nuclear Information System (INIS)

    Choi, Jin Woo; KIm, Sang Youn; Cho, Jeong Yeon; Kim, Seung Hyup; Moon, Kyung Chul

    2012-01-01

    Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. We described the imaging findings of an IgG4-related inflammatory pseudotumor in the urethra. The urethral mass showed isoattenuation on unenhanced CT images, delayed enhancement on enhanced CT images, iso- to slight hyper-intensity on T1 and T2 weighted magnetic resonance images, diffusion restriction on diffusion weighted images, and heterogeneously low echogeneity on ultrasonography.

  11. Sclerosing adenosis: mammographic and ultrasonographic findings with clinical and histopathological correlation

    International Nuclear Information System (INIS)

    Guenhan-Bilgen, Isil; Memis, Aysenur; Uestuen, Esin Emin; Oezdemir, Necmettin; Erhan, Yildiz

    2002-01-01

    Objective: To evaluate the mammographic and ultrasonographic findings of sclerosing adenosis, a relatively uncommon entity which may sometimes mimic carcinoma. Materials and methods: A retrospective review of the records of 33700 women, who have undergone mammographic examination at our institution between January 1985 and July 2001 revealed 43 histopathologically proven sclerosing adenosis. The history, physical examination, mammographic and ultrasonographic findings were analyzed in all patients. In 30 patients, the nonpalpable lesions were preoperatively localized by the needle-hookwire system under the guidance of mammography (n=22) or ultrasonography (US) (n=8). Radiological features were correlated with histopathological findings. Results: The age of the patients varied between 32 and 55 years (mean, 43.7 years). Only two patients had a family history of breast cancer. In six patients, the presenting complaint was mastalgia. A palpable mass was present in 13 cases. The mammographic findings were; microcalcifications in 24 (55.8%) (clustered in 22, diffuse in two), mass in five (11.6%), asymmetric focal density in three (6.9%), and focal architectural distortion in three (6.9%) patients. Four of the masses were irregularly contoured, while one was well-circumscribed. On US, focal acoustic shadowing without a mass configuration was noted in the three patients who showed asymmetrical focal density on mammography. In eight patients, who showed normal mammograms, a solid mass was detected on US. Two masses had discrete well-circumscribed oval or lobulated contours, while six showed microlobulation and irregularity. In one case, the irregularly contoured mass had marked posterior acoustic shadowing. Two of the three patients, who had focal architectural distortion on mammograms, had an irregularly contoured solid mass, while the third presented as focal acoustic shadowing without a mass configuration. Conclusion: Sclerosing adenosis mostly presents as a nonpalpable

  12. Sclerosing mucoepidermoid carcinoma with eosinophilia of the thyroid: A cytological dilemma

    Directory of Open Access Journals (Sweden)

    Chayanika Pantola

    2016-01-01

    Full Text Available Sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE of the thyroid is a rare primary thyroid tumor arising in a background of Hashimoto′s/lymphocytic thyroiditis and has been recently introduced in the World Health Organization (WHO classification of thyroid tumors. It is characterized by extensive sclerosis, squamous and glandular differentiation, and inflammatory infiltrate rich in eosinophil. Here, we are discussing the cytological features of this rare case in a 35-year-old female presented with thyroid swelling and lymph-node enlargement.

  13. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor

    Directory of Open Access Journals (Sweden)

    Angela Sardaro

    2014-10-01

    Full Text Available Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers, and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease.

  14. The effect of nonsteroidal anti-inflammatory drugs ibuprofen, flurbiprofen, and diclofenac on in vitro and in vivo growth of mouse fibrosarcoma

    Czech Academy of Sciences Publication Activity Database

    Hoferová, Zuzana; Fedoročko, P.; Hofmanová, Jiřina; Hofer, Michal; Znojil, V.; Minksová, Kateřina; Souček, Karel; Egyed, A.; Kozubík, Alois

    2002-01-01

    Roč. 20, č. 4 (2002), s. 490-498 ISSN 0735-7907 R&D Projects: GA AV ČR IBS5004009 Grant - others:VEGA MŠ SR(SK) 1/6026/99 Institutional research plan: CEZ:AV0Z5004920 Keywords : cyclooxygenase (COX) inhibitors * fibrosarcoma cells Subject RIV: BO - Biophysics Impact factor: 1.850, year: 2002

  15. An experimental study for efficacy of acetic acid as a sclerosing agent

    International Nuclear Information System (INIS)

    Kim, Young Chan; Oh, Ju Hyung; Yoon, Yup; Ko, Young Tae; Choi, Woo Suk; Kim, Eui Jong; Lim, Joo Won

    1997-01-01

    To evaluate the efficacy of acetic acid as a sclerosing agent by observation of histologic change in urinary bladder epithelium after the instillation of acetic acid. Urinary bladder of the rabbit was catheterized with a Foley catheter, and acetic acid of 10%, 20%, 30%, 40% and 50% concentration was instilled for 5 minutes. After evacuation of the acid, the bladder was irrigated three times with normal saline. After two days, gross and histologic examinations of the bladder were performed. A bladder into which 10% acetic acid had been instilled revealed a nearly normal epithelium without denudation. In two cases, 20% acetic acid was instilled;one revealed partial denudation of the epithelium and the other revealed complete denudation. Mild to moderate interstitial edema and vascular congestion of the bladder wall were evident in all cases in which acid at a concentration of 30% or more had been instilled. In all cases in which the concentration of acid was greater than 30%, the epithelium was completely denuded. An acetic acid concentration of 40% or more is sufficient to completely destroy the epithelium of rabbit urinary bladder, and may be effective as a new sclerosing agent in cases of renal or hepatic cyst

  16. Pruritus is associated with severely impaired quality of life in patients with primary sclerosing cholangitis.

    Science.gov (United States)

    Gotthardt, Daniel Nils; Rupp, Christian; Bruhin, Miriam; Schellberg, Dieter; Weiss, Karl H; Stefan, Reinhard; Donnerstag, Nadine; Stremmel, Wolfgang; Löwe, Bernd; Juenger, Jana; Sauer, Peter

    2014-12-01

    Quality of life, fundamental to the individual patient, has shown a lack of correlation with severity in research on several diseases. Thus, we aimed to identify factors associated with quality of life in patients with primary sclerosing cholangitis. The Short Form Health Survey and the Patient Health Questionnaire were used to assess quality of life and depression. Complete data sets of 113 patients were analyzed for correlation with sex, age, presence of concomitant inflammatory bowel disease and dominant stenosis, frequency of pruritus, and Mayo Risk Score. Physical functioning decreased with age (P<0.001). Further, women experienced more prominent role limitations because of physical (P<0.03) and emotional (P<0.01) problems. Although patients' quality of life and depression scores were only slightly lower than normal, more frequent pruritus was associated with a considerable reduction in quality of life in terms of physical and social functioning, general and mental health, bodily pain, vitality, and roles (because of physical problems) (P<0.01). It did not differ significantly according to the Mayo Risk Score or the presence of dominant stenoses. Depression scores were only significantly affected in patients with more frequent pruritus. Pruritus severely affects quality of life in patients with primary sclerosing cholangitis and is associated with depression to varying extents, although the most commonly used parameters of disease severity do not correspond to quality of life in these patients. These findings need to be considered with respect to treatment outcomes and indications for liver transplantation.

  17. Microbiological analysis of bile and its impact in critically ill patients with secondary sclerosing cholangitis.

    Science.gov (United States)

    Voigtländer, Torsten; Leuchs, Ensieh; Vonberg, Ralf-Peter; Solbach, Philipp; Manns, Michael P; Suerbaum, Sebastian; Lankisch, Tim O

    2015-05-01

    Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is an emerging disease entity with unfavourable outcome. Our aim was to analyze the microbial spectrum in bile of patients with SSC-CIP and to evaluate the potential impact on the empiric antibiotic treatment in these patients. 169 patients (72 patients with SSC-CIP and 97 patients with primary sclerosing cholangitis (PSC)) were included in a prospective observational study between 2010 and 2013. Bile was obtained during endoscopic retrograde cholangiography (ERC) and microbiologically analyzed. Patients with SSC displayed a significantly different microbiological profile in bile. Enterococcus faecium, Pseudomonas aeruginosa and non-albicans species of Candida were more frequent in SSC compared to patients with PSC (p bile (p = 0.001). The antimicrobial therapy was adjusted in 64% of patients due to resistance or presence of microorganisms not covered by the initial therapy regimen. Patients with SSC-CIP have a distinct microbial profile in bile. Difficult to treat organisms are frequent and an ERC with bile fluid collection for microbiological analysis should be considered in case of insufficient antimicrobial treatment. Copyright © 2015 The British Infection Association. Published by Elsevier Ltd. All rights reserved.

  18. Disruption of chromosome 11 in canine fibrosarcomas highlights an unusual variability of CDKN2B in dogs

    Directory of Open Access Journals (Sweden)

    Haugland Sean

    2009-07-01

    Full Text Available Abstract Background In dogs in the western world neoplasia constitutes the most frequently diagnosed cause of death. Although there appear to be similarities between canine and human cancers, rather little is known about the cytogenetic and molecular alterations in canine tumours. Different dog breeds are susceptible to different types of cancer, but the genetic basis of the great majority of these predispositions has yet to be discovered. In some retriever breeds there is a high incidence of soft tissue sarcomas and we have previously reported alterations of chromosomes 11 and 30 in two poorly differentiated fibrosarcomas. Here we extend our observations and present a case report on detail rearrangements on chromosome 11 as well as genetic variations in a tumour suppressor gene in normal dogs. Results BAC hybridisations on metaphases of two fibrosarcomas showed complex rearrangements on chromosome 11, and loss of parts of this chromosome. Microsatellite markers on a paired tumour and blood DNA pointed to loss of heterozygosity on chromosome 11 in the CDKN2B-CDKN2A tumour suppressor gene cluster region. PCR and sequencing revealed the homozygous loss of coding sequences for these genes, except for exon 1β of CDKN2A, which codes for the N-terminus of p14ARF. For CDKN2B exon 1, two alleles were observed in DNA from blood; one of them identical to the sequence in the dog reference genome and containing 4 copies of a 12 bp repeat found only in the canine gene amongst all species so far sequenced; the other allele was shorter due to a missing copy of the repeat. Sequencing of this exon in 141 dogs from 18 different breeds revealed a polymorphic region involving a GGC triplet repeat and a GGGGACGGCGGC repeat. Seven alleles were recorded and sixteen of the eighteen breeds showed heterozygosity. Conclusion Complex chromosome rearrangements were observed on chromosome 11 in two Labrador retriever fibrosarcomas. The chromosome alterations were reflected

  19. Sonographic image of cervix epithelioid trophoblastic tumor coexisting with mucinous adenocarcinoma in a postmenopausal woman: A case report.

    Science.gov (United States)

    Zhu, Yi; Zhang, Guo-Nan; Zhang, Rui-Bo; Shi, Yu; Wang, Deng-Feng; He, Rong

    2017-09-01

    Epithelioid trophoblastic tumor (ETT) is a distinctive but rare gestational trophoblastic neoplasia (GTN) composed of chorionic-type intermediate trophoblast cells. Approximately 50% ETT arose from the uterine cervix or lower uterine segment following a previous pregnancy with vaginal bleeding. With its unusual ability to simulate an invasive epithelioid neoplasm, ETT frequently poses a diagnostic challenge, especially involving the uterine cervix. We herein report the case of a 60-year-old female with persistent vaginal bleeding and middle-level elevation of serum human chorionic gonadotropin (hCG). Ultrasound revealed a 3.0 × 2.7 cm well-circumscribed, strongly echogenic lesion in the cervix, with a peripheral pattern of Doppler signals. The enhanced pattern by contrast-enhanced ultrasound displayed strong peripheral enhancement accompanied with globular appearance, then centripetal filling completely, and fading away rapidly. The final pathological diagnosis was ETT accompanying mucinous adenocarcinoma. Due to the pre-operative evaluation of a presumed IB2 cervix mucinous adenocarcinoma, the patient was treated with 2 courses of neoadjuvant chemotherapy followed by radical hysterectomy. The patient is currently disease-free for the past 1 year. This case report demonstrates that sonographic image of tumor shapes and blood flow could be helpful in differentiating ETT from another GTN and enable more accurate diagnosis before treatment.

  20. Epithelioid osteoblastoma or aggressive osteoblastoma of the astragalus. Presentation of a case and revision of clinical and pathological symptoms

    Directory of Open Access Journals (Sweden)

    A. Alcántara-Vázquez

    2018-04-01

    Full Text Available This is the case of a 58 year-old man with a painful tumour in his right ankle, resulting from a long development. An osteolytic injury was found in an X-ray, formed by bone or osteoid trabeculae surrounded by epithelioid osteoblasts and amongst these cells there were osteoclast-type multinucleated cells. The histogenesis of the tumour, the differential diagnosis and the treatment modalities are reported. Resumen: Se presenta el caso de un hombre de 58 años de edad con un tumor doloroso en el tobillo derecho, de larga evolución. Radiológicamente se encontró una lesión osteolítica, con un anillo de esclerosis en el astrágalo. Histológicamente el tumor, está formado por trabéculas de hueso u osteoide rodeadas de osteoblastos epitelioides y entre estas células hay células multinucleadas tipo osteoclasto. Se comenta la histogénesis del tumor, el diagnóstico diferencial y las modalidades de tratamiento. Keywords: Osteoblastoma, Aggressive osteoblastoma, Epithelioid osteoblastoma, Palabras clave: Osteoblastoma, Osteoblastoma agresivo, Osteoblastoma epitelioides

  1. The effect of silver nitrate, chloroformic garlic extract and normal saline in induction of sclerosing cholangitis in rabbits

    International Nuclear Information System (INIS)

    Hosseni, Seyed V.; Mohebzadeh, J.; Mehrabani, D.; Amini, M.; Kumar, Perikala V.; Bagheri, Mohammad H.; Sadjjadi, Seyed M.; Amini, A.

    2008-01-01

    Objective was to the effects of 0.5% silver nitrate, 20% chloroformic garlic extract and 0.9% normal saline in induction of sclerosing cholangitis in the bile ducts of rabbits. During a 6-months period from April to September 2006 in Shiraz University Laboratory Animal Research Center, we selected 3 equal groups of rabbits. We injected 0.5% silver nitrate, 20% chloroformic garlic extract and 0.9% normal saline into the bile ducts of each group. The animals were euthanized and autopsied after 4 months and the liver and bile ducts were removed and studied histopathologically. Cholangiography was undertaken to evaluate the presence and extent of any sclerosing cholangitis. Animals showed sclerosing cholangitis in silver nitrate group (7 [58%]), one (8%) in chloroformic garlic extract group and one (7%) in normal saline group. The difference between silver nitrate and chloroformic garlic extract groups were statistically significant and similar results were noticed between chloroformic garlic extract and normal saline groups. Twenty percent of chloroformic garlic extract had fewer complications such as sclerosing cholangitis, compared to other materials. (author)

  2. Leukemoid reaction, a rare manifestation of autoimmune hemolytic anemia in a case of small duct primary sclerosing cholangitis.

    Science.gov (United States)

    Salagre, Kaustubh D; Sahay, Ravindra Nath; Patil, Anuja; Pati, Anuja; Joshi, Amita; Shukla, Akash

    2013-10-01

    A 48 year old lady presented with jaundice and exertional breathlesness. Her laboratory reports showed anaemia, reticulocytosis, leucocytosis, elevated Lactate Dehydrogenase (LDH), alkaline phosphatase levels, hyperbillirubinemia and positive direct Coomb's test. After ruling out all the other causes of autoimmunity and hemolytic anemia, she was diagnosed as leukemoid reaction due to autoimmune hemolytic anemia with primary sclerosing cholangitis. Patient showed immediate improvement after corticosteroids.

  3. Dense genotyping of immune-related disease regions identifies nine new risk loci for primary sclerosing cholangitis

    NARCIS (Netherlands)

    Liu, Jimmy Z.; Hov, Johannes Roksund; Folseraas, Trine; Ellinghaus, Eva; Rushbrook, Simon M.; Doncheva, Nadezhda T.; Andreassen, Ole A.; Weersma, Rinse K.; Weismüller, Tobias J.; Eksteen, Bertus; Invernizzi, Pietro; Hirschfield, Gideon M.; Gotthardt, Daniel Nils; Pares, Albert; Ellinghaus, David; Shah, Tejas; Juran, Brian D.; Milkiewicz, Piotr; Rust, Christian; Schramm, Christoph; Müller, Tobias; Srivastava, Brijesh; Dalekos, Georgios; Nöthen, Markus M.; Herms, Stefan; Winkelmann, Juliane; Mitrovic, Mitja; Braun, Felix; Ponsioen, Cyriel Y.; Croucher, Peter J. P.; Sterneck, Martina; Teufel, Andreas; Mason, Andrew L.; Saarela, Janna; Leppa, Virpi; Dorfman, Ruslan; Alvaro, Domenico; Floreani, Annarosa; Onengut-Gumuscu, Suna; Rich, Stephen S.; Thompson, Wesley K.; Schork, Andrew J.; Næss, Sigrid; Thomsen, Ingo; Mayr, Gabriele; König, Inke R.; Hveem, Kristian; Cleynen, Isabelle; Gutierrez-Achury, Javier; Ricaño-Ponce, Isis; van Heel, David; Björnsson, Einar; Sandford, Richard N.; Durie, Peter R.; Melum, Espen; Vatn, Morten H.; Silverberg, Mark S.; Duerr, Richard H.; Padyukov, Leonid; Brand, Stephan; Sans, Miquel; Annese, Vito; Achkar, Jean-Paul; Boberg, Kirsten Muri; Marschall, Hanns-Ulrich; Chazouillères, Olivier; Bowlus, Christopher L.; Wijmenga, Cisca; Schrumpf, Erik; Vermeire, Severine; Albrecht, Mario; Rioux, John D.; Alexander, Graeme; Bergquist, Annika; Cho, Judy; Schreiber, Stefan; Manns, Michael P.; Färkkilä, Martti; Dale, Anders M.; Chapman, Roger W.; Lazaridis, Konstantinos N.; Franke, Andre; Anderson, Carl A.; Karlsen, Tom H.

    2013-01-01

    Primary sclerosing cholangitis (PSC) is a severe liver disease of unknown etiology leading to fibrotic destruction of the bile ducts and ultimately to the need for liver transplantation. We compared 3,789 PSC cases of European ancestry to 25,079 population controls across 130,422 SNPs genotyped

  4. Dense genotyping of immune-related disease regions identifies nine new risk loci for primary sclerosing cholangitis

    NARCIS (Netherlands)

    Liu, Jimmy Z.; Hov, Johannes Roksund; Folseraas, Trine; Ellinghaus, Eva; Rushbrook, Simon M.; Doncheva, Nadezhda T.; Andreassen, Ole A.; Weersma, Rinse K.; Weismueller, Tobias J.; Eksteen, Bertus; Invernizzi, Pietro; Hirschfield, Gideon M.; Gotthardt, Daniel Nils; Pares, Albert; Ellinghaus, David; Shah, Tejas; Juran, Brian D.; Milkiewicz, Piotr; Rust, Christian; Schramm, Christoph; Mueller, Tobias; Srivastava, Brijesh; Dalekos, Georgios; Noethen, Markus M.; Herms, Stefan; Winkelmann, Juliane; Mitrovic, Mitja; Braun, Felix; Ponsioen, Cyriel Y.; Croucher, Peter J. P.; Sterneck, Martina; Teufel, Andreas; Mason, Andrew L.; Saarela, Janna; Leppa, Virpi; Dorfman, Ruslan; Alvaro, Domenico; Floreani, Annarosa; Onengut-Gumuscu, Suna; Rich, Stephen S.; Thompson, Wesley K.; Schork, Andrew J.; Naess, Sigrid; Thomsen, Ingo; Mayr, Gabriele; Koenig, Inke R.; Hveem, Kristian; Cleynen, Isabelle; Gutierrez-Achury, Javier; Ricano-Ponce, Isis; van Heel, David; Bjoernsson, Einar; Sandford, Richard N.; Durie, Peter R.; Melum, Espen; Vatn, Morten H.; Silverberg, Mark S.; Duerr, Richard H.; Padyukov, Leonid; Brand, Stephan; Sans, Miquel; Annese, Vito; Achkar, Jean-Paul; Boberg, Kirsten Muri; Marschall, Hanns-Ulrich; Chazouilleres, Olivier; Bowlus, Christopher L.; Wijmenga, Cisca; Schrumpf, Erik; Vermeire, Severine; Albrecht, Mario; Rioux, John D.; Alexander, Graeme; Bergquist, Annika; Cho, Judy; Schreiber, Stefan; Manns, Michael P.; Farkkila, Martti; Dale, Anders M.; Chapman, Roger W.; Lazaridis, Konstantinos N.; Franke, Andre; Anderson, Carl A.; Karlsen, Tom H.

    Primary sclerosing cholangitis (PSC) is a severe liver disease of unknown etiology leading to fibrotic destruction of the bile ducts and ultimately to the need for liver transplantation(1-3). We compared 3,789 PSC cases of European ancestry to 25,079 population controls across 130,422 SNPs genotyped

  5. Clinical point of view, imaging and neuro physiology of the case with diagnosis of neuro fibrosarcomas sacrococcigeo

    International Nuclear Information System (INIS)

    Hernandez Hernandez, Barbara Aymee; Brown Martinez, Marta

    2004-01-01

    It was considered a case with diagnosis of neuro fibrosarcomas sacrococcigeo, which was very well studied from the clinical point of view, imaging and neuro physiology; with the objective of showing the correlation degree among these studies. He/she was carried out study of nervous conduction of nerve later, reflective tibial H, electromiografia of dependent muscles of the plexo lumbosacro, potentials evoked somatosensoriales and wave F of nerve later tibial. With this the existence of a functional dysfunction was demonstrated in the medullary segments from L4-S2 with prevalence motor. He/she was also carried out mielografia and nuclear magnetic resonance that it evidenced the existence from an expansible process to the level sacrococcigeo. The utility of the neuro physiological studies was demonstrated in the diagnosis of affections tumorales, cocktails with the imagenological studies that define the anatomical alterations and the diagnosis morfofuncional of these pathologies is completed

  6. Three ulcerative colitis susceptibility loci are associated with primary sclerosing cholangitis and indicate a role for IL2, REL, and CARD9

    NARCIS (Netherlands)

    Janse, Marcel; Lamberts, Laetitia E.; Franke, Lude; Raychaudhuri, Soumya; Ellinghaus, Eva; Muri Boberg, Kirsten; Melum, Espen; Folseraas, Trine; Schrumpf, Erik; Bergquist, Annika; Björnsson, Einar; Fu, Jingyuan; Jan Westra, Harm; Groen, Harry J. M.; Fehrmann, Rudolf S. N.; Smolonska, Joanna; van den Berg, Leonard H.; Ophoff, Roel A.; Porte, Robert J.; Weismüller, Tobias J.; Wedemeyer, Jochen; Schramm, Christoph; Sterneck, Martina; Günther, Rainer; Braun, Felix; Vermeire, Severine; Henckaerts, Liesbet; Wijmenga, Cisca; Ponsioen, Cyriel Y.; Schreiber, Stefan; Karlsen, Tom H.; Franke, Andre; Weersma, Rinse K.

    2011-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts. Both environmental and genetic factors contribute to its pathogenesis. To further clarify its genetic background, we investigated susceptibility loci recently

  7. Three Ulcerative Colitis Susceptibility Loci Are Associated with Primary Sclerosing Cholangitis and Indicate a Role for IL2, REL, and CARD9

    NARCIS (Netherlands)

    Janse, Marcel; Lamberts, Laetitia E.; Franke, Lude; Raychaudhuri, Soumya; Ellinghaus, Eva; Boberg, Kirsten Muri; Melum, Espen; Folseraas, Trine; Schrumpf, Erik; Bergquist, Annika; Bjornsson, Einar; Fu, Jingyuan; Westra, Harm Jan; Groen, Harry J. M.; Fehrmann, Rudolf S. N.; Smolonska, Joanna; van den Berg, Leonard H.; Ophoff, Roel A.; Porte, Robert J.; Weismueller, Tobias J.; Wedemeyer, Jochen; Schramm, Christoph; Sterneck, Martina; Guenther, Rainer; Braun, Felix; Vermeire, Severine; Henckaerts, Liesbet; Wijmenga, Cisca; Ponsioen, Cyriel Y.; Schreiber, Stefan; Karlsen, Tom H.; Franke, Andre; Weersma, Rinse K.

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts. Both environmental and genetic factors contribute to its pathogenesis. To further clarify its genetic background, we investigated susceptibility loci recently

  8. Laparoscopic Management of Sclerosing Stromal Tumors of the Ovary Combined with Ectopic Pregnancy.

    Science.gov (United States)

    Liu, Hua-Qian; Liu, Qiang; Sun, Xue-Bing; Chang, Wen-Min

    2015-01-01

    Like other stromal-derived gynecological tumors, a sclerosing stromal tumor of the ovary (SSTO) is a rare benign tumor that is difficult to distinguish from a malignant ovarian tumor in clinical practice. An SSTO is routinely treated with laparotomy. Here, we present two extremely rare cases of SSTO with contralateral and ipsilateral tubal pregnancies, in which laparoscopic surgery was performed to remove the tumors. After surgery, one patient (case 1) became pregnant twice within 29 months, and the other patient (case 2) did not become pregnant within 6 months postoperatively. These two cases suggest that laparoscopic management is not only useful in treating SSTO and complicating diseases, but it may also help to reduce unnecessary surgical injury to the ovary. © 2015 S. Karger AG, Basel.

  9. Subacute sclerosing panencephalitis with bilateral inferior collicular hyperintensity on magnetic resonance imaging brain

    Directory of Open Access Journals (Sweden)

    Maya Thomas

    2012-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is chronic encephalitis occurring after infection with measles virus. An 8-year-old boy presented with progressive behavioral changes, cognitive decline and myoclonic jerks, progressing to a bed bound state over 2 months. Magnetic resonance imaging (MRI brain showed T2-weighted hyperintensities in the subcortical areas of the left occipital lobe and brachium of the inferior colliculus on both sides. EEG showed bilateral, synchronous periodic discharges. Serum/cerebrospinal fluid measles IgG titer was significantly positive. The overall features were suggestive of SSPE. MRI finding of bilateral inferior colliculus changes on MRI without significant involvement of other commonly involved areas suggests an uncommon/rare imaging pattern of SSPE.

  10. Segmentectomy for giant pulmonary sclerosing haemangiomas with high serum KL-6 levels

    Science.gov (United States)

    Kuroda, Hiroaki; Mun, Mingyon; Okumura, Sakae; Nakagawa, Ken

    2012-01-01

    We describe a 61-year old female patient with a giant pulmonary sclerosing haemangioma (PSH) and an extremely high preoperative serum KL-6 level. During an annual health screening, the patient showed a posterior mediastinal mass on chest radiography. Chest computed tomography and magnetic resonance imaging revealed a well-circumscribed 60 mm diameter nodule with a marked contrast enhancement in the left lower lobe. The preoperative serum KL-6 level was elevated to 8204 U/ml. We performed a four-port thoracoscopic basal segmentectomy and lymph node sampling for diagnosis and therapy. The postoperative diagnosis showed PSH. The serum KL-6 level decreased dramatically with tumour resection. To the best of our knowledge, this is the first report of a patient with PSH showing a high serum KL-6 level. PMID:22454483

  11. Regional cerebral metabolic rate for glucose and cerebrospinal fluid monoamine metabolites in subacute sclerosing panencephalitis

    International Nuclear Information System (INIS)

    Yanai, Kazuhiko; Miyabayashi, Shigeaki; Iinuma, Kazuie; Tada, Keiya; Fukuda, Hiroshi; Ito, Masatoshi; Matsuzawa, Taiju.

    1987-01-01

    Regional cerebral metabolic rate for glucose (rCMRglu) and cerebrospinal fluid monoamine metabolites were measured in two cases of subacute sclerosing panencephalitis (SSPE) with different clinical courses. A marked decrease in rCMRglu was found in the cortical gray matter of a patient with rapidly developing SSPE (3.6 - 4.2 mg/100 g brain tissue/min). However, the rCMRglu was preserved in the caudate and lenticular nuclei of the patient (7.7 mg/100 g/min). The rCMRglu in a patient with slowly developing SSPE revealed patterns and values similar to those of the control. Cerebrospinal fluid monoamine metabolites ; homovanilic acid and 5-hydroxyindoleacetic acid, were decreased in both rapidly and slowly developing SSPE. These data indicated that rCMRglu correlated better with the neurological and psychological status and that dopaminergic and serotonergic abnormalities have been implicated in pathophysiology of SSPE. (author)

  12. Sclerosing Angiomatoid Nodular Transformation: Laparoscopic Splenectomy as Therapeutic and Diagnostic Approach at the Same Time

    Directory of Open Access Journals (Sweden)

    Calogero Cipolla

    2018-01-01

    Full Text Available Introduction. Sclerosing angiomatoid nodular transformation (SANT of the spleen is a rare benign vascular lesion with unknown etiopathogenesis and with definite features of imaging, histopathology, and immunohistochemistry. It was first described by Martel et al. in 2004, and to date, only 151 cases have been reported. Case Description. We report a case of SANT of the spleen detected in a 66-year-old Caucasian, without comorbidities, presented to our department with epigastric pain. We, also, presented a review of the literature. Conclusions. SANT is a benign incidentally vascular condition in the majority of cases. The wide age and gender distribution in our review is in accordance with that in previous studies in English literature. In our opinion, splenectomy is the choice treatment because it is at the same time diagnostic and therapeutic in a definitive way.

  13. Sclerosing Angiomatoid Nodular Transformation: Laparoscopic Splenectomy as Therapeutic and Diagnostic Approach at the Same Time.

    Science.gov (United States)

    Cipolla, Calogero; Florena, Ada Maria; Ferrara, Gabriella; Di Gregorio, Riccardo; Unti, Elettra; Giannone, Antonino G; Lazzaro, Luigi A; Graceffa, Giuseppa; Pantuso, Gianni

    2018-01-01

    Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion with unknown etiopathogenesis and with definite features of imaging, histopathology, and immunohistochemistry. It was first described by Martel et al. in 2004, and to date, only 151 cases have been reported. We report a case of SANT of the spleen detected in a 66-year-old Caucasian, without comorbidities, presented to our department with epigastric pain. We, also, presented a review of the literature. SANT is a benign incidentally vascular condition in the majority of cases. The wide age and gender distribution in our review is in accordance with that in previous studies in English literature. In our opinion, splenectomy is the choice treatment because it is at the same time diagnostic and therapeutic in a definitive way.

  14. IgG4-associated sclerosing cholangitis masquerading as hilar cholangiocarcinoma.

    Science.gov (United States)

    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.

  15. IDIOPATHIC SCLEROSING ENCAPSULATING PERITONITIS CAUSING ACUTE INTESTINAL OBSTRUCTION AND GANGRENE: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Nava

    2016-04-01

    Full Text Available INTRODUCTION Sclerosing encapsulating peritonitis (SEP is a relatively rare cause of intestinal obstruction resulting from encasement of variable lengths of bowel by dense fibro-collagenous membrane. It is more common in young females, and shows tropical and sub-tropical distribution. The idiopathic cases of SEP, which lack any identifiable cause from clinical, radiological and histopathological findings, are also reported under the descriptive term “abdominal cocoon syndrome”. SEP presents with acute or sub-acute intestinal obstruction with or without a mass. In the era of laparoscopic surgery, inadvertent damage to the small bowel at insertion of the trocar and cannula can occur by being unaware of this condition resulting in unnecessary bowel resection. Persistent untreated SEP may advance to bowel gangrene or intestinal perforation, representing life threatening conditions. We report the clinical presentation of a 75-year-old female presenting with signs of intestinal obstruction whose imaging findings revealed abdominal cocoon with bowel gangrene leading to perforation and the same confirmed at surgery. Surgical excision of the fibrotic sac encasing the bowel, resection of gangrenous bowel segment and end ileostomy was performed. Histopathology of the excised membrane confirmed sclerosing encapsulating peritonitis. To our knowledge, only a few cases of abdominal cocoon with perforation have been reported in literature so far. Radiologists should be aware of this relatively rare cause of intestinal obstruction, its imaging findings and complications, as preoperative diagnosis will prevent delay and aid in treatment planning to the surgeon. Identification of soft tissue density membrane encasing congregated small bowel loops into a single area on computed-tomography gives diagnostic clue. Surgical excision of sac, release of bowel loops and adhesions with partial intestinal resection when necessary is the treatment.

  16. Secondary Sclerosing Cholangitis in Critically Ill Patients: Clinical Presentation, Cholangiographic Features, Natural History, and Outcome

    Science.gov (United States)

    Leonhardt, Silke; Veltzke-Schlieker, Wilfried; Adler, Andreas; Schott, Eckart; Eurich, Dennis; Faber, Wladimir; Neuhaus, Peter; Seehofer, Daniel

    2015-01-01

    Abstract Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is an important differential diagnosis in patients presenting with cholestasis and PSC-like cholangiographic changes in endoscopic retrograde cholangiography (ERC). As a relatively newly described entity, SSC-CIP is still underdiagnosed, and the diagnosis is often delayed. The present study aims to improve the early detection of SSC-CIP and the identification of its complications. A total of 2633 records of patients who underwent or were listed for orthotopic liver transplantation at the University Hospital Charité, Berlin, were analyzed retrospectively. The clinical presentation and outcome (mean follow-up 62.7 months) of the 16 identified SSC-CIP cases were reviewed. Cholestasis was the first sign of SSC-CIP. GGT was the predominant enzyme of cholestasis. Hypercholesterolemia occurred in at least 75% of the patients. SSC-CIP provoked a profound weight loss (mean 18 kg) in 94% of our patients. SSC-CIP was diagnosed by ERC in all patients. The 3 different cholangiographic features detected correspond roughly to the following stages: (I) evidence of biliary casts, (II) progressive destruction of intrahepatic bile ducts, and (III) picture of pruned tree. Biliary cast formation is a hallmark of SSC-CIP and was seen in 87% of our cases. In 75% of the patients, the clinical course was complicated by cholangiosepsis, cholangitic liver abscesses, acalculous cholecystitis, or gallbladder perforation. SSC-CIP was associated with worse prognosis; transplant-free survival was ∼40 months (mean). Because of its high rate of serious complications and unfavorable prognosis, it is imperative to diagnose SSC-CIP early and to differentiate SSC-CIP from other types of sclerosing cholangitis. Specific characteristics enable identification of SSC-CIP. Early cooperation with a transplant center and special attention to biliary complications are required after diagnosis of SSC-CIP. PMID:26656347

  17. Risk factors and outcome in patients with primary sclerosing cholangitis with persistent biliary candidiasis.

    Science.gov (United States)

    Rupp, Christian; Bode, Konrad Alexander; Chahoud, Fadi; Wannhoff, Andreas; Friedrich, Kilian; Weiss, Karl-Heinz; Sauer, Peter; Stremmel, Wolfgang; Gotthardt, Daniel Nils

    2014-10-23

    Candidiasis is commonly observed in patients with primary sclerosing cholangitis (PSC), but the clinical risk factors associated with its presence have not been fully investigated. In this study, we aimed to analyse the incidence, risk factors, and transplantation-free survival in primary sclerosing cholangitis (PSC) patients with persistent biliary candidiasis. We retrospectively analysed patients diagnosed with PSC who were admitted to our department during 2002 to 2012. One-hundred fifty patients whose bile cultures were tested for fungal species were selected, and their clinical and laboratory parameters were investigated. The results of endoscopic retrograde cholangiography (ERC) and bile cultures were analysed using chart reviews. The cases of biliary candidiasis were sub-classified as transient or persistent. Thirty out of 150 (20.0%) patients had biliary candidiasis. Although all patients demonstrated comparable baseline characteristics, those with biliary candidiasis showed significantly reduced transplantation-free survival (p candidiasis. A subgroup analysis showed reduced survival with a greater necessity for orthotopic liver transplantation (OLT) only in patients with persistence of Candida (p = 0.007). The survival in the patients with transient biliary candidiasis was comparable to that in candidiasis-free patients. In a multivariate regression analysis that included Mayo risk score (MRS), sex, age, dominant stenosis, inflammatory bowel disease, autoimmune hepatitis overlap syndrome, and number of times ERC was performed, biliary candidiasis was an independent risk factor for reduced survival (p = 0.008). Risk factors associated with acquisition of biliary candidiasis were age at PSC diagnosis and number of ERCs. The persistence of biliary candidiasis is associated with markedly reduced transplantation-free survival in PSC patients. By contrast, actuarial survival in patients with transient biliary candidiasis approaches that for patients without any

  18. A sclerosing stromal tumor of the ovary with masculinization in a premenarchal girl

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    Soo Min Park

    2011-05-01

    Full Text Available A sclerosing stromal tumor of the ovary is an extremely rare benign tumor; it usually is found during the second and third decades of life. Patients present with pelvic pain or a palpable abdominal mass. Hormonal effects such as masculinization are uncommon. Here, an 11-year old premenarchal girl presented with deepening of the voice. In addition, clitoromegaly and hirsutism with a male suprapubic hair pattern were observed. The laboratory findings showed that the testosterone level was elevated to 3.67 ng/mL, andostenedione to above 10 ng/mL, dehydroepiandrosterone-sulfate to 346 μg/dL and 17-hydroxy progesterone (17-OHP to 11.28 ng/mL. The chromosome evaluation revealed a 46,XX female karyotype. An adrenocorticotropic hormone stimulation test was performed. The 17-OHP to cortisol ratio in 30 minutes was 0.045, which suggested a heterozygote for the 21-hydroxylase deficiency. However, the CYP21A2 gene encoding steroid 21-hydroxylase showed normal. The pelvic ultrasound showed a heterogeneous mass consisting of predominantly solid tissue in the pelvic cavity. The pelvic magnetic resonance imaging revealed an 8.9× 6.2×6.6 cm mass of the left ovary. A left oophrectomy was performed and microscopic examination confirmed a sclerosing stromal tumor. Immunohistochemical studies showed that the tumor was positive for smooth muscle actin and vimentin, but negative for S-100 protein and cytokeratin. Following surgery, the hormone levels returned to the normal range and the hirsutism resolved.

  19. Epithelioid sarcoma of the plantar fascia mimicking Morbus Ledderhose - A severe pitfall for clinical and histopathological misinterpretation.

    Science.gov (United States)

    Toepfer, Andreas; Harrasser, Norbert; Dreyer, Florian; Mogler, Carolin; Walther, Markus; von Eisenhart-Rothe, Rüdiger

    2017-12-01

    Plantar fibromatosis, also known as Morbus Ledderhose, is a well known and frequently encountered disorder of the planta pedis. When conservative treatment fails, surgical therapy with complete resection is the therapeutical procedure of choice. Soft tissue sarcoma is a heterogeneous and rare malignant disease of the musculoskeletal system with over 50 histopathological subtypes which can potentially arise in any localization but is most commonly found at the extremities. Here, we report the case of an epithelioid sarcoma of the sole of the foot which was initially and repeatedly clinically and histopathologically misinterpreted as plantar fibromatosis, receiving insufficient resection and subsequently ending in amputation of the lower leg. Copyright © 2017 European Foot and Ankle Society. Published by Elsevier Ltd. All rights reserved.

  20. Epithelioid hemangioendothelioma of the liver with metastatic coeliac lymph nodes in an 11-year-old boy

    International Nuclear Information System (INIS)

    Ines, David da; Petitcolin, Virginie; Garcier, Jean-Marc; Joubert-Zakeyh, Juliette; Demeocq, Francois

    2010-01-01

    Epithelioid hemangioendothelioma of the liver is a very rare vascular tumour in children with intermediate malignant potential. We present a case in which the typical imaging appearances of coalescent peripheral hepatic masses with capsular retraction contributed to the diagnosis. A positron emission tomography-CT (PET-CT) procedure was performed in staging the disease with a strong suspicion of coeliac nodal involvement confirmed after laparotomy and histological analysis. Our case is unique because of the rarity of the disease, the young age of the child, and proven nodal metastases at initial diagnosis. The use of PET-CT allows better staging at initial diagnosis and thus better management with improved follow-up in these patients. (orig.)

  1. Perivascular Epithelioid Cell Tumor (PEComa of the Uterine Cervix in a Patient with Tuberous Sclerosis Complex: A Literature Review

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    Handan ÇELİK

    2018-01-01

    Full Text Available Perivascular epithelioid cell tumors (PEComa are a rare type of mesenchymal tumor arising from perivascular epithelial cells. These tumor cells are a co-expression of both melanocytic and myogenic antigens, such as HMB 45 and smooth muscle actin, and at least in some patients, are located around vessels. PEComas has been reported at various sites, including visceral organs, soft tissue, the prostate gland and broad ligaments. In the female reproductive system, the uterine corpus is the most common site of involvement. Some cases are related to tuberous sclerosis complex. Cervical PEComa with tuberous sclerosis complex is presented in the case of a 41 year-old and the literature is reviewed. There have been only eight cases of cervical PEComas and only one other case associated with tuberous sclerosis complex reported to date.

  2. Tachyphylaxis of juxtaglomerular epithelioid cells to angiotensin II. Differences between the electrical membrane response and renin secretion

    DEFF Research Database (Denmark)

    Bührle, C P; Hackenthal, E; Nobiling, R

    1987-01-01

    A study has been made of desensitization of the depolarizing response to angiotensin II of juxtaglomerular epithelioid and vascular smooth muscle cells in the mouse kidney afferent arteriole, of media cells from the mesenteric artery as well as of cultured smooth muscle and mesangial cells. In all...... cell types, desensitization to this effect of angiotensin II was observed. There was no cross-desensitization between angiotensin II and other depolarizing agonists. Hence, it is concluded that this desensitization is specific, i.e. of the tachyphylaxis type. Substances interfering with receptor...... recycling, such as chloroquine and monensin, did not block the recovery of the cells from desensitization after removal of the octapeptide. Desensitization to the action of angiotensin II was also observed with respect to its vasoconstrictor effect in the isolated perfused rat kidney. In contrast...

  3. Sclerosing cholangitis

    Science.gov (United States)

    ... gallstones in the bile duct) Infections in the liver, gallbladder, and bile ducts Symptoms The first symptoms are usually: Fatigue Itching ... varices (enlarged veins) Biliary cirrhosis (inflammation of the bile ducts) Liver failure Persistent jaundice Some people develop infections of ...

  4. Diagnosis of sclerosing hemangioma of lung: Don′t rely on fine-needle aspiration cytology diagnosis alone

    Directory of Open Access Journals (Sweden)

    Kaushik Saha

    2013-01-01

    Full Text Available Sclerosing hemangioma is a rare variety of benign pulmonary neoplasm. It usually presents as asymptomatic, solitary, peripheral, circumscribed lesions in middle-aged women. Here, we describe a 46-year-old woman presenting to us for evaluation of right parahilar lung mass. Previous chest radiography done 10 years back showed a lung mass of almost similar size. Computed Tomography (CT-guided fine-needle aspiration cytology (FNAC was suggestive of adenocarcinoma of lung. A well-circumscribed, capsulated, ovoid mass measuring 5.6 cm × 4 cm × 3 cm, adjacent to the transverse fissure of the right lung was excised by lateral thoracotomy. Histopathological examination along with immunohistochemistry was suggestive of sclerosing hemangioma of lung. A pathologist must consider the clinicoradiological features before coming to a final diagnosis of lung malignancy from FNAC. Whenever there is any confusion regarding lung mass, thoracotomy must be done for arriving at an exact diagnosis from histopathology.

  5. Raine Syndrome (OMIM #259775), Caused By FAM20C Mutation, Is Congenital Sclerosing Osteomalacia With Cerebral Calcification (OMIM 259660).

    Science.gov (United States)

    Whyte, Michael P; McAlister, William H; Fallon, Michael D; Pierpont, Mary Ella; Bijanki, Vinieth N; Duan, Shenghui; Otaify, Ghada A; Sly, William S; Mumm, Steven

    2017-04-01

    In 1985, we briefly reported infant sisters with a unique, lethal, autosomal recessive disorder designated congenital sclerosing osteomalacia with cerebral calcification. In 1986, this condition was entered into Mendelian Inheritance In Man (MIM) as osteomalacia, sclerosing, with cerebral calcification (MIM 259660). However, no attestations followed. Instead, in 1989 Raine and colleagues published an affected neonate considering unprecedented the striking clinical and radiographic features. In 1992, "Raine syndrome" entered MIM formally as osteosclerotic bone dysplasia, lethal (MIM #259775). In 2007, the etiology emerged as loss-of-function mutation of FAM20C that encodes family with sequence similarity 20, member C. FAM20C is highly expressed in embryonic calcified tissues and encodes a kinase (dentin matrix protein 4) for most of the secreted phosphoproteome including FGF23, osteopontin, and other regulators of skeletal mineralization. Herein, we detail the clinical, radiological, biochemical, histopathological, and FAM20C findings of our patients. Following premortem tetracycline labeling, the proposita's non-decalcified skeletal histopathology after autopsy indicated no rickets but documented severe osteomalacia. Archival DNA revealed the sisters were compound heterozygotes for a unique missense mutation and a novel deletion in FAM20C. Individuals heterozygous for the missense mutation seemed to prematurely fuse their metopic suture and develop a metopic ridge sometimes including trigonocephaly. Our findings clarify FAM20C's role in hard tissue formation and mineralization, and show that Raine syndrome is congenital sclerosing osteomalacia with cerebral calcification. © 2016 American Society for Bone and Mineral Research. © 2016 American Society for Bone and Mineral Research.

  6. Pazopanib for metastatic pulmonary epithelioid hemangioendothelioma—a suitable treatment option: case report and review of anti-angiogenic treatment options

    International Nuclear Information System (INIS)

    Semenisty, Valeriya; Naroditsky, Inna; Keidar, Zohar; Bar-Sela, Gil

    2015-01-01

    Epithelioid hemangioendothelioma is a rare vascular tumor of borderline or low-grade malignancy. The lungs and liver are the two common primary organs affected. Metastatic disease was reported in more than 100 cases in the literature. However, no firm conclusions can be determined for recommended treatment options. The current case presents a patient with metastatic pulmonary epithelioid hemangioendothelioma to the cervical and mediastinal lymph nodes, lungs and liver that has been treated with pazopanib for more than two years with PET avid complete metabolic response in the mediastinum and lungs, and long-lasting stable disease. Target therapies that block VEGFR have a logical base in this rare malignancy. The current case is the first to report objective, long-lasting response to pazopanib

  7. A novel branched TAT(47-57) peptide for selective Ni(2+) introduction into the human fibrosarcoma cell nucleus.

    Science.gov (United States)

    Szyrwiel, Łukasz; Shimura, Mari; Shirataki, Junko; Matsuyama, Satoshi; Matsunaga, Akihiro; Setner, Bartosz; Szczukowski, Łukasz; Szewczuk, Zbigniew; Yamauchi, Kazuto; Malinka, Wiesław; Chavatte, Laurent; Łobinski, Ryszard

    2015-07-01

    A TAT47-57 peptide was modified on the N-terminus by elongation with a 2,3-diaminopropionic acid residue and then by coupling of two histidine residues on its N-atoms. This branched peptide could bind to Ni under physiological conditions as a 1 : 1 complex. We demonstrated that the complex was quantitatively taken up by human fibrosarcoma cells, in contrast to Ni(2+) ions. Ni localization (especially at the nuclei) was confirmed by imaging using both scanning X-ray fluorescence microscopy and Newport Green fluorescence. A competitive assay with Newport Green showed that the latter displaced the peptide ligand from the Ni-complex. Ni(2+) delivered as a complex with the designed peptide induced substantially more DNA damage than when introduced as a free ion. The availability of such a construct opens up the way to investigate the importance of the nucleus as a target for the cytotoxicity, genotoxicity or carcinogenicity of Ni(2+).

  8. Growth-related variations in the glycosaminoglycan synthesis of ultraviolet light-induced murine cutaneous fibrosarcoma cells

    International Nuclear Information System (INIS)

    Piepkorn, M.; Carney, H.; Linker, A.

    1985-01-01

    Glycosaminoglycan synthesis was studied in cell populations of ultraviolet light-induced murine cutaneous fibrosarcoma cells under conditions of varying growth rates in vitro. After labeling with the precursors, 3 H-glucosamine and 35 SO 4 , sulfated glycosaminoglycans recoverable by direct proteolysis of the culture monolayers increased approximately 5-fold on a per cell basis from sparsely populated, exponential cell cultures (greater than 85% of cells in S, G2, or M phases) to stationary cultures inhibited by high cell density (greater than 50% of cells in G1). Within this cell surface-associated material, the relative ratio of heparan sulfate to the chondroitin sulfates was approximately 60/40% under conditions of exponential growth; in the growth-arrested cultures, the reverse ratio was found. The substratum attached material, obtained from the flask surface after ethyl glycol bis(beta-aminoethyl ether)-N,N'-tetraacetic acid (EGTA)-mediated detachment of the monolayers, contained relatively more hyaluronic acid, heparan sulfate, and chondroitin sulfates in the most actively proliferating cultures compared with the growth-inhibited cell populations. Furthermore, heparan sulfate and the chondroitin sulfates, which were enriched in the substratum material and in the cell pellet of exponential cultures, showed a relative shift to the cell surface-associated compartment (releasable by mild trypsinization after EGTA-mediated cell detachment) and to the compartment loosely associated with the pericellular matrix (i.e., released into the supernatant during detachment of the monolayers in the presence of EGTA)

  9. Pigmented Epithelioid Melanocytoma (PEM)/Animal Type Melanoma (ATM): Quest for an Origin. Report of One Unusual Case Indicating Follicular Origin and Another Arising in an Intradermal Nevus.

    Science.gov (United States)

    Tarasen, Ashley; Carlson, J Andrew; Leonard, M Kathryn; Merlino, Glenn; Kaetzel, David; Slominski, Andrzej T

    2017-08-15

    Pigmented epithelioid melanocytoma (PEM) is a tumor encompassing epithelioid blue nevus of Carney complex (EBN of CNC) and was previously termed animal-type melanoma. Histologically PEMs are heavily pigmented spindled and epithelioid dermal melanocytic tumors with infiltrative borders, however, their origin remains unclear. Stem cells for the epidermis and hair follicle are located in the bulge area of the hair follicle with the potential to differentiate into multiple lineages. Multiple cutaneous carcinomas, including follicular cutaneous squamous cell carcinoma (FSCC), are thought to arise from stem cells in the follicular bulge. We present two cases of PEM/ATM in a 63 year-old male on the scalp with follicular origin and a 72 year-old female on the upper back arising in an intradermal nevus. Biopsy of both cases revealed a proliferation of heavily pigmented dermal nests of melanocytes with atypia. The Case 1 tumor was in continuation with the outer root sheath of the hair follicle in the bulge region. Case 2 arose in an intradermal melanocytic nevus. Rare mitotic figures, including atypical mitotic figures, were identified in both cases. We present two cases of PEM, with histologic evidence suggesting two origins: one from the follicular bulb and one from an intradermal nevus.

  10. Osteomielitis crónica esclerosante difusa Chronic diffuse sclerosing osteomyelitis

    Directory of Open Access Journals (Sweden)

    M. Paula Aparicio M

    2008-12-01

    Full Text Available La osteomielitis esclerosante difusa se considera como una osteomielitis crónica primaria consistente en un proceso inflamatorio, doloroso y prolongado en el tiempo. Afecta solo a la mandíbula y es generalmente unilateral, compromete hueso basal y alveolar, y se localiza al nivel de cuerpo, ángulo, rama e incluso cóndilo. La causa es aún controversial, ya que algunos le atribuyen un origen infeccioso, mientras otros lo consideran una condición no infecciosa, como producto de sobrecargas o asociado con síndrome SAPHO (sinovitis, acné, pustulosis, hiperostosis y osteítis, pero la literatura no es concluyente. Con respecto al tratamiento, al igual que su causa, no está totalmente esclarecido y se describen a lo largo del tiempo distintas alternativas, que van desde lo conservador a lo más radical. Se presenta el seguimiento y tratamiento durante 7 meses de una paciente afectada por osteomielitis esclerosante difusa con 18 años de evolución aproximadamente, que ha sido refractaria a las alternativas terapéuticas convencionales.Diffuse sclerosing osteomyelitis is considered a chronic primary osteomyelitis consisting in an inflammatory, painful and prolonged process. It only affects the mandible and it is generally unilateral. It involves the basal and alveolar bone and it is located at the level of body, angle, branch and even condyle. The cause is more controversial, since some attribute an infectious origin to it, whereas others consider it as a non-infectious condition resulting from the overloads or associated with SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis, but literature is not concluding. Treatment as well as its cause are not totally clear. Different alternatives are described that go from the conservative to the most radical position. The 7-month follow-up and treatment of a female patient suffering from diffuse sclerosing osteomyelitis with approximately18 years of evolution that has been refractory

  11. MR imaging of primary sclerosing cholangitis - Additional value of diffusion-weighted imaging and ADC measurement

    Energy Technology Data Exchange (ETDEWEB)

    Djokicc Kovac, Jelena [Center for Radiology and Magnetic Resonance Imaging, Clinical Center Serbia, Belgrade (Serbia)], e-mail: jelenadjokic2003@yahoo.co.uk; Maksimovic, Ruzica [Center for Radiology and Magnetic Resonance Imaging, Clinical Center Serbia, Belgrade (Serbia); Faculty of Medicine, Univ. of Belgrade, Belgrade (Serbia); Jesic, Rada [Clinic for Gastroenterohepatology, Clinical Center Serbia, Belgrade (Serbia); Faculty of Medicine, Univ. of Belgrade, Belgrade (Serbia); Stanisavljevic, Dejana [Inst. for Statistics, Faculty of Medicine, Univ. of Belgrade, Belgrade (Serbia); Kovac, Bojan [Military Medical Academy, Belgrade (Serbia)

    2013-04-15

    Background: Primary sclerosing cholangitis (PSC) is a cholestatic liver disease with chronic inflammation and progressive destruction of biliary tree. Magnetic resonance (MR) examination with diffusion-weighted imaging (DWI) allows analysis of morphological liver parenchymal changes and non-invasive assessment of liver fibrosis. Moreover, MR cholangiopancreatography (MRCP), as a part of standard MR protocol, provides insight into bile duct irregularities. Purpose: To evaluate MR and MRCP findings in patients with primary sclerosing cholangitis and to determine the value of DWI in the assessment of liver fibrosis. Material and Methods: The following MR findings were reviewed in 38 patients: abnormalities in liver parenchyma signal intensity, changes in liver morphology, lymphadenopathy, signs of portal hypertension, and irregularities of intra- and extrahepatic bile ducts. Apparent diffusion coefficient (ADC) was calculated for six locations in the liver for b = 800 s/mm{sup 2}. Results: T2-weighted hyperintensity was seen as peripheral wedge-shaped areas in 42.1% and as periportal edema in 28.9% of patients. Increased enhancement of liver parenchyma on arterial-phase imaging was observed in six (15.8%) patients. Caudate lobe hypertrophy was present in 10 (26.3%), while spherical liver shape was noted in 7.9% of patients. Liver cirrhosis was seen in 34.2% of patients; the most common pattern was micronodular cirrhosis (61.5%). Other findings included lymphadenopathy (28.9%), signs of portal hypertension (36.7%), and bile duct irregularities (78.9%). The mean ADCs (x10{sup -3} mm{sup 2}/s) were significantly different at stage I vs. stages III and IV, and stage II vs. stage IV. No significant difference was found between stages II and III. For prediction of stage {>=}II and stage {>=}III, areas under receiver-operating characteristic curves were 0.891 and 0.887, respectively. Conclusion: MR with MRCP is a necessary diagnostic procedure for diagnosis of PSC and

  12. Perivascular epithelioid cell tumour: Dynamic CT, MRI and clinicopathological characteristics—Analysis of 32 cases and review of the literature

    International Nuclear Information System (INIS)

    Tan, Y.; Zhang, H.; Xiao, E.-H.

    2013-01-01

    Aim: To evaluate the dynamic computed tomography (CT), magnetic resonance imaging (MRI), and clinicopathological characteristics of perivascular epithelioid cell tumours (PEComas), thus improving the diagnosis of the tumour. Materials and methods: A retrospective analysis was undertaken of the dynamic CT, MRI, and clinicopathological characteristics of 32 PEComas diagnosed at histopathology during the period 1 January 2005 to 1 March 2012 at two hospitals. Results: The age of the patients ranged from 14–80 years (mean 43.3 years). There were more women in this group (19/32). Solitary tumours were identified in kidney (n = 16), liver (n = 7), gynaecological organs (n = 2), retroperitoneal soft tissue (n = 2), lung (n = 2), palate (n = 1), left groin (n = 1). One patient had multiple tumours in the liver, kidney, and retroperitoneal soft tissue. Dynamic CT (32 cases) and MRI (15 cases) demonstrated tumours that were of low density or hypointense on T1-weighted imaging (WI) and hyperintense on T2WI; some were isodense with fat (CT: 10/32; MRI: 6/15). The tumours usually had well-defined borders and were of a regular shape (CT: 26/32; MRI: 12/15). Tumour diameters ranged from 1.5–18 cm (mean 5.1 cm). Most tumours (CT: 21/32, MRI: 10/15) enhanced heterogeneously and significantly on arterial and venous phases. Tumours appeared slightly hypodense on delayed CT imaging, although some (6/32) had delayed enhancement. The expression rate of HMB-45 (human melanoma black monoclonal antibody) was 100% (32/32). Histological classification in 22 cases (22/32) was epithelioid angiomyolipoma (AML), three (3/32) were clear cell “sugar” tumours (CCSTs), two (2/32) were lymphangioleiomyomatosis (LAM), and two (2/32) were clear cell myomelanocytic tumours of the falciform ligament/ligamentum teres (CCMMT). Three tumours did not have a specific classification. Conclusion: Knowledge of dynamic CT, MRI, and clinicopathological characteristics could help improve the diagnosis of

  13. Gene Expression by PBMC in Primary Sclerosing Cholangitis: Evidence for Dysregulation of Immune Mediated Genes

    Directory of Open Access Journals (Sweden)

    Christopher A. Aoki

    2006-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is a chronic disease of the bile ducts characterized by an inflammatory infiltrate and obliterative fibrosis. The precise role of the immune system in the pathogenesis of PSC remains unknown. We used RNA microarray analysis to identify immune-related genes and pathways that are differentially expressed in PSC. Messenger RNA (mRNA from peripheral blood mononuclear cells (PBMC was isolated from both patients with PSC and age and sex matched healthy controls. Samples from 5 PSC patients and 5 controls were analyzed by microarray and based upon rigorous statistical analysis of the data, relevant genes were chosen for confirmation by RT-PCR in 10 PSC patients and 10 controls. Using unsupervised hierarchical clustering, gene expression in PSC was statistically different from our control population. Interestingly, genes within the IL-2 receptor beta, IL-6 and MAP Kinase pathways were found to be differently expressed in patients with PSC compared to controls. Further, individual genes, TNF-α induced protein 6 (TNFaip6 and membrane-spanning 4-domains, subfamily A (ms4a were found to be upregulated in PSC while similar to Mothers against decapentaplegic homolog 5 (SMAD 5 was downregulated. In conclusion, several immune-related pathways and genes were differentially expressed in PSC compared to control patients, giving further evidence that this disease is systemic and immune-mediated.

  14. Comparison of efficacy between sodium morrhuate and lauromacrogol as sclerosing agents in treatment of hepatic cyst

    Directory of Open Access Journals (Sweden)

    QIN Zuyun

    2015-07-01

    Full Text Available Objective To compare the efficacy of sodium morrhuate versus lauromacrogol in the treatment of hepatic cyst. Methods Seventy-four patients with hepatic cyst who were admitted to our hospital from January 2009 to May 2013 were enrolled as subjects and divided into two groups. After the cystic fluid was drained by percutaneous liver biopsy, sodium morrhuate solution was injected into the cystic cavity for adhesion and sclerosis in 46 patients in group A, and lauromacrogol solution was injected in 28 patients in group B. The incidence rates of pain in patients during and after surgery were compared between the two groups. The follow-up comparison of hepatic cyst recurrence rates within one year after surgery was performed between the two groups. Between-group comparison was performed by χ2 test. Results Five patients (10.87% in group A and two patients (7.14% in group B had recurrence within one year after treatment. There was no significant difference in recurrence rate between the two groups (χ2=0.283, P>0.05. The incidence of pain in group A was significantly higher than that in group B (χ2=5.258, P<0.05. Conclusion With the same efficacy as sodium morrhuate in the treatment of hepatic cyst, lauromacrogol can be routinely used as a sclerosing agent due to its mild side effects.

  15. Expression of defective measles virus genes in brain tissues of patients with subacute sclerosing panencephalitis

    International Nuclear Information System (INIS)

    Baczko, K.; Liebert, U.G.; Billeter, M.; Cattaneo, R.; Budka, H.; Ter Meulen, V.

    1986-01-01

    The persistence of measles virus in selected areas of the brains of four patients with subacute sclerosing panencephalitis (SSPE) was characterized by immunohistological and biochemical techniques. The five measles virus structural proteins were never simultaneously detectable in any of the bran sections. Nucleocapsid proteins and phosphoproteins were found in every diseased brain area, whereas hemagglutinin protein was detected in two cases, fusion protein was detected in three cases, and matrix protein was detected in only one case. Also, it could be shown that the amounts of measles virus RNA in the brains differed from patient to patient and in the different regions investigated. In all patients, plus-strand RNAs specific for these five viral genes could be detected. However, the amounts of fusion and hemagglutinin mRNAs were low compared with the amounts in lytically infected cells. The presence of particular measles virus RNAs in SSPE-infected brains did not always correlate with mRNA activity. In in vitro translations, the matrix protein was produced in only one case, and the hemagglutinin protein was produced in none. These results indicate that measles virus persistence in SSPE is correlated with different defects of several genes which probably prevent assembly of viral particles in SSPE-infected brain tissue

  16. Radial scar/complex sclerosing lesion of the breast--value of ultrasound.

    Science.gov (United States)

    Grunwald, S; Heyer, H; Kühl, A; Schwesinger, G; Schimming, A; Köhler, G; Ohlinger, R

    2007-04-01

    Although benign, radial scar/complex sclerosing adenosis is a lesion which histopathologically resembles tubular carcinoma. On physical examination, it is difficult to distinguish radial scar from a malignant tumour. Mammography cannot differentiate radial scar from malignancy. This clinical study aims to delineate the role of preoperative ultrasonography with emphasis on the question whether ultrasonography could lower the number of false-positive readings and therefore the number of open biopsies required. In this examination, we present the clinical, mammographic, ultrasonographic, and histopathological features of 6 cases of radial scars. Although most authors describe radial scars as non-palpable, 2 of 6 lesions were indeed palpable. On mammograms, radial scars have a spiculated appearance, a feature observed in all of our cases. Numerous ultrasonographic characteristics are listed in the literature, but ultrasonography is not reported to have clear-cut advantages. Although this study did not elucidate any unique ultrasonographic features to characterise these lesions, the analysis of all ultrasonographic results made us recognise a set of "nearly specific ultrasonographic features" of radial scars. Current B-mode imaging does not appear to lead to the desirable reduction of the rate of unnecessary open biopsies.

  17. De-novo cholangiocarcinoma in native common bile duct remnant following OLT for primary sclerosing cholangitis.

    Science.gov (United States)

    Landaverde, Carmen; Ng, Vivian; Sato, Alisa; Tabibian, James; Durazo, Francisco; Busuttil, Ronald

    2009-01-01

    Primary sclerosing cholangitis (PSC) is a chronic, progressive, inflammatory and obstructive disease of the intra- and extra-hepatic bile ducts of unknown etiology. Currently, orthotopic liver transplantation (OLT) is the only definitive treatment for PSC-related end-stage liver disease. However, PSC has been known to recur in the grafted liver. Roux-en-Y hepaticojejunostomy is more commonly performed than choledochocholedochostomy for PSC, although choledochocholedochostomy has been found to be safe and efficacious for PSC if the distal common bile duct is uninvolved at the time of OLT. Our case is unique in that it describes a patient who developed de-novo cholangiocarcinoma in the remnant portion of the native common bile duct six years after OLT with choledochocholedochostomy for PSC-associated end-stage liver disease without having PSC recurrence. In conclusion, our case report indicates that choledochocholedochostomy may not be desirable in PSC due to an increased risk of developing cholangiocarcinoma in the native common bile duct. This risk exists as well with a Roux-en-Y hepaticojejunostomy in the remaining intra-duodenal and intra-pancreatic biliary epithelium, although in theory to a lesser extent. Therefore, the risk of developing cholangiocarcinoma in the recipient common bile duct can only be completely eliminated by performing a Whipple procedure at the time of OLT.

  18. Diagnosis of primary sclerosing cholangitis: prospective comparison of MR cholangiography with endoscopic retrograde cholangiography

    International Nuclear Information System (INIS)

    Oberholzer, K.; Mildenberger, P.; Grebe, P.; Bantelmann, M.; Thelen, M.; Lohse, A.W.; Schadeck, T.

    1998-01-01

    Purpose: To assess the accuracy of MR cholangiography (MRC) in the diagnosis of primary sclerosing cholangitis (PSC) in comparison to endoscopic retrograde cholangiography (ERC). Method: 20 patients with PSC were examined by ERC and MRC (1.0 T. HASTE sequence). Visualization and pathologic changes of the extra- and intrahepatic bile ducts were evaluated with both methods. Results: Mural irregularities of the common bile duct were seen with MRC in 6/7 cases, stenoses and dilatation of the common bile duct were detected correctly in all patients. Diffuse, multifocal strictures of the intrahepatic bile duct were the most common intrahepatic findings and correctly diagnosed in all patients. Mural irregularities of the intrahepatic ducts in early stages may be missed by MRC because of the limited spatial resolution. MRC is superior to ERC in visualization of nonopacified intrahepatic ducts. Conclusions: MRC is a reliable, non-invasive method to detect typical diagnostic features of PSC. It should be considered as an adjunct to ERC in patients with suspected PSC for primary diagnosis and as an alternate method for follow-up studies. (orig.) [de

  19. Imaging and estimation of the prognostic features of primary sclerosing cholangitis by ultrasonography and MR cholangiography

    International Nuclear Information System (INIS)

    Oikarinen, H.; Paeaekkoe, E.; Suramo, I.; Paeivaensalo, M.; Tervonen, O.; Lehtola, J.; Aukee, J.

    2001-01-01

    Purpose: To evaluate the ability of US and MR cholangiography (MRC) to detect bile duct changes and prognostic signs of primary sclerosing cholangitis (PSC) seen at endoscopic retrograde cholangiography (ERC). Material and Methods: In a prospective study, 9 patients with PSC underwent US, MRC, MR imaging and ERC of the bile ducts and the liver. Eight age- and sex-matched control patients were examined with MRC, MR imaging and ERC. A segmental comparison was performed to assess the ability of MRC-MR and US to reveal the accurate ductal involvement in different segments of the biliary tree and the specific criteria of poor prognostic outcome in PSC. The ability of MRC-MR to detect the presence of PSC in different patients was analysed blindly. Results: MRC-MR depicted changes of PSC correctly in 9 patients (radiologist 1) and in 8 patients with 1 false-positive finding (radiologist 2) in the blinded analysis. In the segmental comparison, MRC missed especially bile duct dilatation. MRC was too pessimistic in the evaluation of the outcome. US detected features suggestive of PSC in 8 patients (radiologist 3). US was unable to show the predictors of poor outcome. Conclusion: MRC and US seem to be useful in the detection of PSC. US is unable and MRC is too pessimistic to estimate the outcome of PSC

  20. Radio-opaque ethylcellulose-ethanol is a safe and efficient sclerosing agent for venous malformations

    International Nuclear Information System (INIS)

    Dompmartin, Anne; Barrellier, Marie-Therese; Blaizot, Xavier; Chene, Yannick; Gaillard, Cathy; Theron, Jacques; Hammer, Frank; Labbe, Daniel; Leroyer, Robert; Chedru, Valerie; Ollivier, Catherine; Vikkula, Miikka; Boon, Laurence M.

    2011-01-01

    To evaluate the efficacy and safety of gelified ethanol, a newly developed sclerosing agent for slow-flow vascular malformations. Seventy-nine sclerotherapy procedures were performed on 44 patients with 37 venous malformations, 2 glomuvenous malformations, 2 lymphatic malformations, 2 lymphatico-venous malformations, and 1 Klippel-Trenaunay syndrome. The median injected volume was 1.00 mL/site of injection. Effects of sclerotherapy on pain, functional and cosmetic disturbance were statistically evaluated with a final result score. Local and systemic complications were recorded. The mean Visual Analogue Scores were 5.20 ± 2.81 before and 1.52 ± 1.25 after treatment (p < 0.001). Functional and aesthetic improvement was achieved in 31/35 patients (89%) and in 33/41 (80%), respectively. Minor local side effects included necrosis with or without issue of ethylcellulose, palpable residue, and hematoma. No systemic side-effects occurred. Per mL used, radio-opaque gelified ethanol is at least as effective as absolute ethanol. No systemic complication was observed, as only a low dose of ethanol was injected. Indications for sclerotherapy can be widened to areas with higher risk for local side effects (hands and periocular region), as ethanol is trapped in the lesion. Careful injection procedure is though necessary, because only a limited amount of ethylcellulose can be used per puncture. (orig.)

  1. Leukocytapheresis Therapy Improved Cholestasis in a Patient Suffering from Primary Sclerosing Cholangitis with Ulcerative Colitis

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    Minoru Itou

    2009-04-01

    Full Text Available Primary sclerosing cholangitis (PSC is an autoimmune disease of the hepatobiliary system for which effective therapy has not been established. Leukocytapheresis (LCAP therapy is known to effective in patients with ulcerative colitis (UC. In addition, effects of LCAP therapy were reported on some autoimmune diseases such as Crohn’s disease, rheumatoid arthritis and rapidly progressive glomerulonephritis. Here we report the case of a 29-year-old man with PSC associated with UC who was treated with LCAP therapy. He had a 16-year history of UC and a 12-year history of PSC. Although he was under treatment with prednisolone and ursodeoxycholic acid, exacerbation of UC and PSC-associated cholestasis were seen. Since he showed side effects of prednisolone, he was treated with LCAP. Not only improvement of UC, but also decreased serum alkaline phosphatase, γ-guanosine triphosphate and total bile acids, suggesting improvement of PSC-associated cholestaisis, were seen after treatment with LCAP. Our experience with this case suggests that LCAP therapy could be a new effective therapeutic strategy for patients with PSC associated with UC.

  2. Coexisting Sclerosing Angiomatoid Nodular Transformation of the Spleen with Multiple Calcifying Fibrous Pseudotumors in a Patient

    Directory of Open Access Journals (Sweden)

    Jen-Chieh Lee

    2007-01-01

    Full Text Available Primary tumor or tumor-like lesions of the spleen are rare. Among them, vascular lesions are the most common. Vascular tumor of the spleen is different from the usual hemangioma of soft tissue because the vascular structure of the spleen is unique. Sclerosing angiomatoid nodular transformation (SANT is a recently described vascular lesion of the spleen. Grossly, it is a multinodular, well-circumscribed tumor containing a hypervascular core. Microscopically, it comprises three types of vessels, and each type recapitulates the immunohistochemical characteristics of the normal vascular elements of the splenic red pulp, i.e. capillaries, sinusoids, and small veins, respectively. Because of the rarity of this entity, its actual pathogenesis is still unknown. In this study, we report a case of SANT occurring in a 43-year-old woman, in whom there were also multiple calcifying fibrous pseudotumors (CFPTs in the abdominal cavity. Both SANT and CFPT are thought to be variants of inflammatory pseudotumor. Coexistence of these two rare entities in a patient has never been reported, and this fact suggests that there might be a common mechanism contributing to the formation of these two types of lesions. [J Formos Med Assoc 2007;106(3:234-239

  3. Immunosuppressive Agents for the Treatment of Primary Sclerosing Cholangitis: A Systematic Review and Meta-Analysis.

    Science.gov (United States)

    Peng, Xia; Luo, Xin; Hou, Jing-Ying; Wu, Shu-Yun; Li, Liang-Zong; Zheng, Ming-Hua; Wang, Ling-Yun

    2017-01-01

    Currently, there are no effective therapeutic agents for patients with primary sclerosing cholangitis (PSC). This study aimed to evaluate the safety and efficiency of immunosuppressive agents (IAs) for the treatment of PSC. The literatures were searched using the following keywords singly or in combination: PSC, treatments, IAs. The primary outcome was defined as the need for liver transplantation or mortality. Two hundred sixty six patients from 7 eligible studies were analyzed. IAs had no remarkable effects on the rate of mortality or liver transplantation (relative risk, RR 1.02, 95% CI 0.58-1.62, p = 0.92). Subgroup analyses showed no significant effect of IAs co-administration therapy (IAs co-administered with ursodeoxycholic acid, IA co-administered with IA; RR 1.41, 95% CI 0.40-4.95, p = 0.60). IAs caused adverse events (AEs) such as diarrhea, abdominal pain, and pruritus (RR 1.81, 95% CI 1.07-3.07, p = 0.03). IAs therapy did not significantly improve markers of liver function except for aspartate transaminase (weighted mean difference -9.76, 95% CI -12.92 to -6.6, p IAs administrated as either monotherapy or combination therapy do not reduce the risk of mortality or liver transplantation. IAs monotherapy is associated with AEs. © 2017 S. Karger AG, Basel.

  4. Diffuse sclerosing variant of thyroid papillary carcinoma: Diagnostic challenges occur with Hashimoto's thyroiditis

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    Chien-Chin Chen

    2013-06-01

    Full Text Available Diffuse sclerosing papillary thyroid carcinoma (DSPTC is a relatively rare variant of papillary thyroid carcinoma with distinct histological features, radiological characteristics, and biological aggressiveness. Compared with conventional papillary thyroid carcinoma, DSPTC is characterized by scattered microscopic tumor islands, diffuse fibrosis, calcification, and abundant lymphocytic aggregation. A preoperative diagnosis is challenging in the absence of nodules and scanty fine needle aspiration cytology samples. We describe a unique DSPTC patient, an 18-year-old woman who presented with a neck mass that grew slowly for 2 years. The palpable neck mass was nontender, well defined, firm, and unmovable. Laboratory studies showed normal thyroid function and positive autoimmune markers: antithyroglobulin antibody = 1:1600 and antimicrosomal antibody = 1:1600. A neck ultrasound showed diffusely prominent microcalcifications with one small vague nodule. Hashimoto's thyroiditis with an accompanying malignancy was suspected. Based on the result of intraoperative pathology reports, the patient was given a total thyroidectomy. Lymph node dissection and histological analysis revealed bilateral DSPTC in addition to lymphocytic thyroiditis in nonmalignant areas of the thyroid. Clinical and histological diagnostic challenges usually occur when DSPTC presents with a diffuse thyroid enlargement, dispersed microscopic tumor islands (frequently without mass formation, extensive fibrosis, and abundant lymphocytic infiltration mimicking thyroiditis.

  5. Reduced Coffee Consumption Among Individuals with Primary Sclerosing Cholangitis but Not Primary Biliary Cirrhosis

    Science.gov (United States)

    Lammert, Craig; Juran, Brian D.; Schlicht, Erik; Xie, Xiao; Atkinson, Elizabeth J.; de Andrade, Mariza; Lazaridis, Konstantinos N.

    2014-01-01

    Background & Aims Coffee consumption has been associated with decreased risk of liver disease and related outcomes. However, coffee drinking has not been investigated among patients with cholestatic autoimmune liver diseases, primary biliary cirrhosis (PBC), or primary sclerosing cholangitis (PSC). We investigated the relationship between coffee consumption and risk of PBC and PSC in a large North American cohort. Methods Lifetime coffee drinking habits were determined from responses to questionnaires from 606 patients with PBC, 480 with PSC, and 564 healthy volunteers (controls). Patients (those with PBC or PSC) were compared to controls utilizing the Wilcoxon rank sum test for continuous variables and c2 method for discrete variables. Logistic regression was used to analyze the estimate the effects of different coffee parameters (time, frequency, and type of coffee consumption) after adjusting for age, sex, smoking status, and education level. Results Patients with PBC and controls did not differ in coffee parameters. However, 24% of patients with PSC had never drank coffee compared to 16% of controls (Pcoffee drinking coffee (46.6% vs 66.7% for controls, Pcoffee protected against proctocolectomy (hazard ratio=0.34, PCoffee consumption is lower among patients with PSC, but not PBC, compared to controls. PMID:24440215

  6. A possible case of Garre's sclerosing osteomyelitis from Medieval Tuscany (11th-12th centuries).

    Science.gov (United States)

    Giuffra, Valentina; Vitiello, Angelica; Giusiani, Sara; Caramella, Davide; Fornaciari, Gino

    2015-12-01

    Archaeological excavations carried out at the castle of Monte di Croce near Florence brought to light a small cemetery complex belonging to the castle church, dated back to the 11th-12th centuries. An elite stone tomb contained the skeletal remains of a male aged 35-45 years with obvious pathology of the right tibia. The proximal metaphysis and the upper half of the diaphysis appear massively enlarged as a result of severe chronic periostitis. A transverse section illustrates complete obliteration of the medullary cavity by new spongy bone, with some large cavitations. The primary, but completely remodeled tibial shaft is still recognizable. This finding and the strong sclerotic reaction with some central cavitations rule out any form of bone tumor and indicate a chronic inflammatory disease. The morphological and radiological picture and the tibial localization suggest a diagnosis of chronic sclerosing osteomyelitis of Garré, a rare form of chronic osteomyelitis characterized by an intense periosteal reaction with little or no suppuration. Copyright © 2015. Published by Elsevier Inc.

  7. Shear wave elastography diagnosis of the diffuse sclerosing variant of papillary thyroid carcinoma: A case report.

    Science.gov (United States)

    Xue, Nianyu; Xu, Youfeng; Huang, Pintong; Zhang, Shengmin; Wang, Hongwei; Yu, Fei

    2016-08-01

    The present study aimed to report the shear wave elastography (SWE) findings in a patient with the diffuse sclerosing variant of papillary thyroid carcinoma (DSVPTC). Since patients with DSVPTC may present with typical clinicopathological features and initially appear to have Hashimoto's thyroiditis, a thorough clinical evaluation and an early diagnosis are important. A 20-year-old female patient presented with a 1-month history of a neck mass and sore throat. Conventional ultrasound and SWE were performed using an AIXPLORER system with 14-5 MHz linear transducer. The patient had undergone total thyroidectomy and bilateral neck lymph node dissection, and an intraoperative pathology consultation to confirm the malignancy of lymph node metastasis. Pathological diagnosis was DSVPTC in both lobes, with lymph node metastases in the bilateral neck. The clinical presentation and serological findings were all indicative of Hashimoto's thyroiditis. Thyroid ultrasonography revealed diffuse enlargement of the both lobes, heterogenous echogenicity without mass formation, diffuse scattered microcalcifications and poor vascularization. SWE revealed stiff values of the thyroid: The mean stiffness was 99.7 kpa, the minimum stiffness was 59.1 kpa and the maximum stiffness was 180.1 kpa. The maximum stiffness of the DSVPTC (180.1 kpa) was higher compared with the diagnostic criteria of malignant thyroid nodules (65 kPa). SWE may be considered as a novel and valuable method to diagnose DSVPC.

  8. Impact of Microbes on the Pathogenesis of Primary Biliary Cirrhosis (PBC and Primary Sclerosing Cholangitis (PSC

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    Jochen Mattner

    2016-11-01

    Full Text Available Primary biliary cirrhosis (PBC and primary sclerosing cholangitis (PSC represent the major clinical entities of chronic cholestatic liver diseases. Both disorders are characterized by portal inflammation and slowly progress to obliterative fibrosis and eventually liver cirrhosis. Although immune-pathogenic mechanisms have been implicated in the pathogenesis of PBC and PSC, neither disorder is considered to be a classical autoimmune disease, as PSC and PBC patients do not respond to immune-suppressants. Furthermore, the decreased bile flow resulting from the immune-mediated tissue assault and the subsequent accumulation of toxic bile products in PBC and PSC not only perpetuates biliary epithelial damage, but also alters the composition of the intestinal and biliary microbiota and its mutual interactions with the host. Consistent with the close association of PSC and inflammatory bowel disease (IBD, the polyclonal hyper IgM response in PBC and (auto-antibodies which cross-react to microbial antigens in both diseases, an expansion of individual microbes leads to shifts in the composition of the intestinal or biliary microbiota and a subsequent altered integrity of epithelial layers, promoting microbial translocation. These changes have been implicated in the pathogenesis of both devastating disorders. Thus, we will discuss here these recent findings in the context of novel and alternative therapeutic options.

  9. Subacute sclerosing panencephalitis in papua new guinean children: the cost of continuing inadequate measles vaccine coverage.

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    Laurens Manning

    2011-01-01

    Full Text Available subacute sclerosing panencephalitis (SSPE is a late, rare and usually fatal complication of measles infection. Although a very high incidence of SSPE in Papua New Guinea (PNG was first recognized 20 years ago, estimated measles vaccine coverage has remained at ≤ 70% since and a large measles epidemic occurred in 2002. We report a series of 22 SSPE cases presenting between November 2007 and July 2009 in Madang Province, PNG, including localized clusters with the highest ever reported annual incidence.as part of a prospective observational study of severe childhood illness at Modilon Hospital, the provincial referral center, children presenting with evidence of meningo-encephalitis were assessed in detail including lumbar puncture in most cases. A diagnosis of SSPE was based on clinical features and presence of measles-specific IgG in cerebrospinal fluid and/or plasma. The estimated annual SSPE incidence in Madang province was 54/million population aged 100/million/year. The distribution of year of birth of the 22 children with SSPE closely matched the reported annual measles incidence in PNG, including a peak in 2002.SSPE follows measles infections in very young PNG children. Because PNG children have known low seroconversion rates to the first measles vaccine given at 6 months of age, efforts such as supplementary measles immunisation programs should continue in order to reduce the pool of non-immune people surrounding the youngest and most vulnerable members of PNG communities.

  10. Central Retinal and Posterior Ciliary Artery Occlusion After Intralesional Injection of Sclerosant to Glabellar Subcutaneous Hemangioma

    International Nuclear Information System (INIS)

    Matsuo, Toshihiko; Fujiwara, Hiroyasu; Gobara, Hideo; Mimura, Hidefumi; Kanazawa, Susumu

    2009-01-01

    The aim of this study is to describe vision loss caused by central retinal artery and posterior ciliary artery occlusion as a consequence of sclerotherapy with a polidocanol injection to a glabellar hemangioma. An 18-year-old man underwent direct injection with a 23-gauge needle of 1 mL of a polidocanol-carbon dioxide emulsion into the glabellar subcutaneous hemangioma under ultrasound visualization of the needle tip by radiologists. He developed lid swelling the next day, and 3 days later at referral, the visual acuity in the left eye was no light perception. Funduscopy revealed central retinal artery occlusion and fluorescein angiography disclosed no perfusion at all in the left fundus, indicating concurrent posterior ciliary artery occlusion. The patient also showed mydriasis, blepharoptosis, and total external ophthalmoplegia on the left side. Magnetic resonance imaging demonstrated the swollen medial rectus muscle. In a month, blepharoptosis and ophthalmoplegia resolved but the visual acuity remained no light perception. Sclerosing therapy for facial hemangioma may develop a severe complication such as permanent visual loss.

  11. Colour doppler ultrasonography and sclerosing therapy in diagnosis and treatment of tendinopathy in horses-a research model for human medicine

    DEFF Research Database (Denmark)

    Boesen, Morten Ilum; Nanni, Simone; Langberg, Henning

    2007-01-01

    Sclerosing therapy has in recent studies showed promising results in patients with clinically and ultrasonographically diagnosed tendinosis in Achilles and patellar tendons. The aim of this investigation was to study the presence of intratendinous colour Doppler (CD) flow in horses with clinically...... diagnosed chronic tendinopathy and to test if experience from human studies could be extrapolated to horses. Special interest was focused on the treatment with sclerosing therapy and whether we could obtain the same successful peroperative findings as in humans. Four horses with clinically diagnosed...... unilateral chronic tendinosis in the forelimbs were examinated with both grey-scale ultrasonography (US) and CD. The horses were to be euthanised according to standard procedure is such cases. The US findings were used for guidance of sclerosing therapy. All horses showed abnormal findings on US, especially...

  12. Multiple Bowen's disease and epithelioid malignant peripheral nerve sheath tumor in a patient who experienced chronic arsenic poisoning

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    Ching-En Chen

    2017-01-01

    Full Text Available The Southwest coastal plain of Taiwan is an endemic area of arsenic contamination. Residents who lived there before the 1970s and who used raw groundwater for drinking have a higher risk of arsenic poisoning. In 1968, Tseng et al. described Blackfoot disease as a peripheral vascular disease caused by chronic exposure to arsenic, thereby introducing the concept of arsenic-induced systemic illness in Taiwan. Multiple Bowen's disease (BD is one of the characteristic consequences of chronic arsenic poisoning and it usually presents as cutaneous carcinoma in situ. Multiple BD can also be associated with squamous cell carcinoma and basal cell carcinoma of the skin, as well as lung, liver, gastrointestinal, and bladder cancers. We encountered a 79-year-old male from Yun-Lin, a county in Southwest Taiwan, who presented with a progressing tumor in his right anterior chest wall. In addition, numerous keratoses and scaly skin lesions were noted on his trunk and extremities, some of which were combined with erosions. The patient was diagnosed with chronic arsenic poisoning with multiple BD and the huge tumor was confirmed as an epithelioid malignant peripheral nerve sheath tumor.

  13. Epithelioid hemangioendothelioma and multiple thoraco-lumbar lateral meningoceles: two rare pathological entities in a patient with NF-1

    International Nuclear Information System (INIS)

    Reis, C.; Carneiro, E.; Fonseca, J.; Salgado, A.; Pereira, P.; Vaz, R.; Pinto, R.; Capelinha, A.F.; Lopes, J.M.

    2005-01-01

    Epithelioid hemangioendothelioma (EHE) is a rare vascular soft-tissue tumour of intermediate malignancy. Neurofibromatosis type I (NF-1) is a genetic syndrome associated with soft tissue sarcoma and higher risk of developing neoplasia. Lateral meningoceles are uncommon entities, being mostly associated with NF-1. We report a case of a 31-year-old woman, with NF-1 and past history of right thalamic/peduncular astrocytoma WHO grade II, admitted to the Neurosurgery Department in December 2003 due to severe low back pain, irradiating to the left leg without a radicular pattern. Thoraco-lumbar magnetic resonance imaging (MRI) showed a large left posterior paravertebral expansive lesion, bilateral and multiple thoraco-lumbar lateral meningoceles and dural ectasias with scalloping of the vertebral bodies. Biopsy of the paravertebral mass lesion disclosed EHE. We present this case because of the novel association between NF-1 and EHE, and the unusual aggressiveness of the neoplasia. Additionally, we highlight the co-existence of bilateral and multiple lateral meningoceles. (orig.)

  14. Radioresistant cell strain of human fibrosarcoma cells obtained after long-term exposure to X-rays

    International Nuclear Information System (INIS)

    Wei, K.; Kodym, R.

    1998-01-01

    A radioresistant cell strain from human fibrosarcoma HT1080 has been obtained after prolonged exposure to x-rays for 7 months (2 Gy per day, 5 days per week). This new strain, HT1080R, differs from HT1080 in a significantly increased ability of clonogenical survival, with coefficient α decreasing from 0.161 to 0.123 Gy -1 and coefficient β decreasing from 0.0950 to 0.0565 Gy -2 . Furthermore, the radioresistance of HT1080R proved to be stable in long-term passaged cultures as well as in frozen samples. Differences between the two cell lines are also observed in the G-banded karyotype; the new cell line shows monosomy of chromosome 17 and loss of 5p + and 11q + present in the parental cells. These data suggest that the radioresistance may have been caused by radiation-induced cell mutation and that the resistant cells may have been selected by repeated irradiations. In order to characterize this new strain, the ability of the cells to rejoin DNA double-strand breaks, the cell cycle distribution and the amount of apoptosis after irradiation have been estimated; however, no differences are observed between these two cell strains. Although the mechanism of the elevated radioresistance remains unknown, this pair of cell strains can provide a new model system for further investigations with regard to the mechanisms of cellular radioresistance. The results also show that any type of irradiation similar to the schedules used in radiotherapy can lead to the formation and selection of more radioresistant cell clones in vitro, a phenomenon with possible implications for radiotherapy. (orig.)

  15. Molecular Understanding of Growth Inhibitory Effect from Irradiated to Bystander Tumor Cells in Mouse Fibrosarcoma Tumor Model

    Science.gov (United States)

    Desai, Sejal; Srambikkal, Nishad; Yadav, Hansa D.; Shetake, Neena; Balla, Murali M. S.; Kumar, Amit; Ray, Pritha; Ghosh, Anu

    2016-01-01

    Even though bystander effects pertaining to radiation risk assessment has been extensively studied, the molecular players of radiation induced bystander effect (RIBE) in the context of cancer radiotherapy are poorly known. In this regard, the present study is aimed to investigate the effect of irradiated tumor cells on the bystander counterparts in mouse fibrosarcoma (WEHI 164 cells) tumor model. Mice co-implanted with WEHI 164 cells γ-irradiated with a lethal dose of 15 Gy and unirradiated (bystander) WEHI 164 cells showed inhibited tumor growth, which was measured in terms of tumor volume and Luc+WEHI 164 cells based bioluminescence in vivo imaging. Histopathological analysis and other assays revealed decreased mitotic index, increased apoptosis and senescence in these tumor tissues. In addition, poor angiogenesis was observed in these tumor tissues, which was further confirmed by fluorescence imaging of tumor vascularisation and CD31 expression by immuno-histochemistry. Interestingly, the growth inhibitory bystander effect was exerted more prominently by soluble factors obtained from the irradiated tumor cells than the cellular fraction. Cytokine profiling of the supernatants obtained from the irradiated tumor cells showed increased levels of VEGF, Rantes, PDGF, GMCSF and IL-2 and decreased levels of IL-6 and SCF. Comparative proteomic analysis of the supernatants from the irradiated tumor cells showed differential expression of total 24 protein spots (21 up- and 3 down-regulated) when compared with the supernatant from the unirradiated control cells. The proteins which showed substantially higher level in the supernatant from the irradiated cells included diphosphate kinase B, heat shock cognate, annexin A1, angiopoietin-2, actin (cytoplasmic 1/2) and stress induced phosphoprotein 1. However, the levels of proteins like annexin A2, protein S100 A4 and cofilin was found to be lower in this supernatant. In conclusion, our results provided deeper insight about

  16. Molecular Understanding of Growth Inhibitory Effect from Irradiated to Bystander Tumor Cells in Mouse Fibrosarcoma Tumor Model.

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    Sejal Desai

    Full Text Available Even though bystander effects pertaining to radiation risk assessment has been extensively studied, the molecular players of radiation induced bystander effect (RIBE in the context of cancer radiotherapy are poorly known. In this regard, the present study is aimed to investigate the effect of irradiated tumor cells on the bystander counterparts in mouse fibrosarcoma (WEHI 164 cells tumor model. Mice co-implanted with WEHI 164 cells γ-irradiated with a lethal dose of 15 Gy and unirradiated (bystander WEHI 164 cells showed inhibited tumor growth, which was measured in terms of tumor volume and Luc+WEHI 164 cells based bioluminescence in vivo imaging. Histopathological analysis and other assays revealed decreased mitotic index, increased apoptosis and senescence in these tumor tissues. In addition, poor angiogenesis was observed in these tumor tissues, which was further confirmed by fluorescence imaging of tumor vascularisation and CD31 expression by immuno-histochemistry. Interestingly, the growth inhibitory bystander effect was exerted more prominently by soluble factors obtained from the irradiated tumor cells than the cellular fraction. Cytokine profiling of the supernatants obtained from the irradiated tumor cells showed increased levels of VEGF, Rantes, PDGF, GMCSF and IL-2 and decreased levels of IL-6 and SCF. Comparative proteomic analysis of the supernatants from the irradiated tumor cells showed differential expression of total 24 protein spots (21 up- and 3 down-regulated when compared with the supernatant from the unirradiated control cells. The proteins which showed substantially higher level in the supernatant from the irradiated cells included diphosphate kinase B, heat shock cognate, annexin A1, angiopoietin-2, actin (cytoplasmic 1/2 and stress induced phosphoprotein 1. However, the levels of proteins like annexin A2, protein S100 A4 and cofilin was found to be lower in this supernatant. In conclusion, our results provided deeper

  17. Growth-Inhibitory and Apoptosis-Inducing Effects of Punica granatum L. var. spinosa (Apple Punice) on Fibrosarcoma Cell Lines.

    Science.gov (United States)

    Sineh Sepehr, Koushan; Baradaran, Behzad; Mazandarani, Masoumeh; Yousefi, Bahman; Abdollahpour Alitappeh, Meghdad; Khori, Vahid

    2014-12-01

    Punica granatum L. var. granatum (Pomegranate), an herbaceous plant found in Iran, The aim of this study was to investigate the cytotoxic effects, induction of apoptosis, and the mechanism of cell death of ethanol extract from Punica granatum L. var. spinosa on the mouse fibrosarcoma cell line, WEHI-164. Various parts of the herbs were extracted from fruit using ethanol as the solvent, and the cytotoxicity and cell viability of the ethanolic extract were determined by the MTT assay. To determine whether necrosis or apoptosis is the predominant cause of cell death, cell death detection was performed using the ELISA method. The induction of apoptosis was confirmed using the terminal deoxynucleotidyl transferase- (TdT-) mediated dUTP nick end labeling (TUNEL) assay. Moreover, a sensitive immunoblotting technique was used to examine the production of Caspase-3 and Bcl2 proteins. Our findings suggested that the ethalonic extract of Punica granatum L. var. spinosa altered cell morphology, decreased cell viability, suppressed cell proliferation and induced cell death in a time- and dose-dependent manner in WEHI-164 cells (IC50 = 229.024μg/ml), when compared to a chemotherapeutic anticancer drug, Toxol (Vesper Pharmaceuticals), with increased nucleosome production from apoptotic cells. Induction of apoptosis by the plant extract was proved by the decrease of pro-Caspase-3 and Bcl2 proteins and quantitatively confirmed by Immunoblotting analysis. The results obtained from the present study have demonstrated the growth-inhibitory effect of Ethanol Extracts from Punica granatum L. var. spinosa, and clearly showed that apoptosis was the major mechanism of in-vitro cell death induced by the extract.

  18. Timing and modality of the sclerosing agents binding to the human proteins: laboratory analysis and clinical evidences

    Directory of Open Access Journals (Sweden)

    Lorenzo Tessari

    2014-07-01

    Full Text Available Sclerosing agents (SA are blood inactivated. Nevertheless, investigations concerning the interaction among SA and blood components have never been deeply investigated. Aim of the study is to precisely identify SA blood ligands, to determine their binding time and to highlight the clinical consequences. Thirty-one blood samples were collected from chronic venous disease patients and tested by capillary and agarose gel (AGE electrophoresis before and after adding polidocanol (POL and sodiumtetradecylsulphate (STS. The two different types of electrophoresis allowed an evaluation of the blood proteins binding with the sclerosing agents, with a reaction time lower than 8 seconds for the AGE. Subsequently six patients underwent foam sclerotherapy and then were subdivided in group A (4 patients and B (2 patients. In group A blood sample was obtained from the ipsilateral brachial vein immediately before (T0 and repeated 1, 3, 5, and 10 minutes after injection of STS 3% injection into the GSV. In group B, the same procedure was performed with the same timing from the ipsilateral femoral vein. Free STS (fSTS and total proteinbound STS (bSTS were measured. POL mainly binds to β-globulins (11%, while STS to albumin and α-globulins (62.6% and 30.7% on the protidogram, respectively. Both in the brachial and in the femoral vein, the average fSTS was always 0. STS binds to albumin (62.6% and α-globulins (30.7%, while POL is bound mainly by the b-globulins (11%. The present paper demonstrates how the vast majority of the sclerosing agent is bound to the blood proteins, suggesting the need to look for possible sclerotherapy complications factors also in the used gas and/or in the subsequent cathabolites release.

  19. Enrichment of measles virus-like RNA in the nucleocapsid fraction isolated from subacute sclerosing panencephalitis brains

    Energy Technology Data Exchange (ETDEWEB)

    Bedows, E; Payne, F E [Michigan Univ., Ann Arbor (USA). School of Public Health; Kohne, D E [Center for Neurologic Study, San Diego, CA, USA; Tourtellotte, W W [Neurology Service, V.A. Wadsworth Hospital Center, Los Angeles, CA, USA

    1982-02-01

    A procedure has been developed which facilitates the detection of measles virus RNA sequences in human brains. The procedure involves isolating subviral components (nucleocapsids) from brain tissues prior to RNA purification, followed by hybridization of these RNAs to cDNA synthesized from measles virus 50 S RNA template. Using these techniques we were able to obtain an RNA fraction which was manyfold enriched in measles virus-specific RNA, relative to unfractionated subacute sclerosing panencephalitis (SSPE) brain RNAs. 70-100% of the measles virus-specific RNA present in these SSPE brain samples were recovered in this enriched fraction.

  20. Enrichment of measles virus-like RNA in the nucleocapsid fraction isolated from subacute sclerosing panencephalitis brains

    International Nuclear Information System (INIS)

    Bedows, E.; Payne, F.E.; Kohne, D.E.; Tourtellotte, W.W.

    1982-01-01

    A procedure has been developed which facilitates the detection of measles virus RNA sequences in human brains. The procedure involves isolating subviral components (nucleocapsids) from brain tissues prior to RNA purification, followed by hybridization of these RNAs to cDNA synthesized from measles virus 50 S RNA template. Using these techniques we were able to obtain an RNA fraction which was manyfold enriched in measles virus-specific RNA, relative to unfractionated subacute sclerosing panencephalitis (SSPE) brain RNAs. 70-100% of the measles virus-specific RNA present in these SSPE brain samples were recovered in this enriched fraction. (Auth.)

  1. [Primary sclerosing cholangitis associated with Sjögren's syndrome, retroperitoneal fibrosis and chronic pancreatitis. Report of a case].

    Science.gov (United States)

    Barreda, F; Contardo, C; León, A; Navarrete, J; Figueroa, R; Attanasio, F

    1989-01-01

    Primary Sclerosing Cholangitis (PSC) is an unusual chronic, cholestatic disease of unknown etiology, more frequently seen in young adults in close relationship with Chronic Ulcerative Colitis. We report the case of a 30 year old woman, coming from the peruvian amazon with PSC associated with Sjögren Syndrome, Chronic Pancreatitis and Retroperitoneal Fibrosis, without colonic involvement. She was treated with external biliary drainage and controlled for 12 months. In this paper, clinical, biochemical, radiological, histological and therapeutic features are reviewed as well as its possible immunologie autoimmune origin.

  2. Characterization of Intestinal Microbiota in Ulcerative Colitis Patients with and without Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Kevans, D; Tyler, A D; Holm, K; Jørgensen, K K; Vatn, M H; Karlsen, T H; Kaplan, G G; Eksteen, B; Gevers, D; Hov, J R; Silverberg, M S

    2016-03-01

    There is an unexplained association between ulcerative colitis [UC] and primary sclerosing cholangitis [PSC], with the intestinal microbiota implicated as an important factor. The study aim was to compare the structure of the intestinal microbiota of patients with UC with and without PSC. UC patients with PSC [PSC-UC] and without PSC [UC] were identified from biobanks at Oslo University Hospital, Foothills Hospital Calgary and Mount Sinai Hospital Toronto. Microbial DNA was extracted from colonic tissue and sequencing performed of the V4 region of the 16S rRNA gene on Illumina MiSeq. Sequences were assigned to operational taxonomic units [OTUs] using Quantitative Insights Into Microbial Ecology [QIIME]. Microbial alpha diversity, beta diversity, and relative abundance were compared between PSC-UC and UC phenotypes. In all, 31 PSC-UC patients and 56 UC patients were included. Principal coordinate analysis [PCoA] demonstrated that city of sample collection was the strongest determinant of taxonomic profile. In the Oslo cohort, Chao 1 index was modestly decreased in PSC-UC compared with UC [p = 0.04] but did not differ significantly in the Calgary cohort. No clustering by PSC phenotype was observed using beta diversity measures. For multiple microbial genera there were nominally significant differences between UC and PSC-UC, but results were not robust to false-discovery rate correction. No strong PSC-specific microbial associations in UC patients consistent across different cohorts were identified. Recruitment centre had a strong effect on microbial composition. Future studies should include larger cohorts to increase power and the ability to control for confounding factors. Copyright © 2015 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.

  3. Combined MEG-EEG source localisation in patients with sub-acute sclerosing pan-encephalitis.

    Science.gov (United States)

    Velmurugan, J; Sinha, Sanjib; Nagappa, Madhu; Mariyappa, N; Bindu, P S; Ravi, G S; Hazra, Nandita; Thennarasu, K; Ravi, V; Taly, A B; Satishchandra, P

    2016-08-01

    To study the genesis and propagation patterns of periodic complexes (PCs) associated with myoclonic jerks in sub-acute sclerosing pan-encephalitis (SSPE) using magnetoencephalography (MEG) and electroencephalography (EEG). Simultaneous recording of MEG (306 channels) and EEG (64 channels) in five patients of SSPE (M:F = 3:2; age 10.8 ± 3.2 years; symptom-duration 6.2 ± 10 months) was carried out using Elekta Neuromag(®) TRIUX™ system. Qualitative analysis of 80-160 PCs per patient was performed. Ten isomorphic classical PCs with significant field topography per patient were analysed at the 'onset' and at 'earliest significant peak' of the burst using discrete and distributed source imaging methods. MEG background was asymmetrical in 2 and slow in 3 patients. Complexes were periodic (3) or quasi-periodic (2), occurring every 4-16 s and varied in morphology among patients. Mean source localization at onset of bursts using discrete and distributed source imaging in magnetic source imaging (MSI) was in thalami and or insula (50 and 50 %, respectively) and in electric source imaging (ESI) was also in thalami and or insula (38 and 46 %, respectively). Mean source localization at the earliest rising phase of peak in MSI was in peri-central gyrus (49 and 42 %) and in ESI it was in frontal cortex (52 and 56 %). Further analysis revealed that PCs were generated in thalami and or insula and thereafter propagated to anterolateral surface of the cortices (viz. sensori-motor cortex and frontal cortex) to same side as that of the onset. This novel MEG-EEG based case series of PCs provides newer insights for understanding the plausible generators of myoclonus in SSPE and patterns of their propagation.

  4. Surveillance of primary sclerosing cholangitis with ERC and brush cytology: risk factors for cholangiocarcinoma.

    Science.gov (United States)

    Boyd, Sonja; Mustonen, Harri; Tenca, Andrea; Jokelainen, Kalle; Arola, Johanna; Färkkilä, Martti A

    2017-02-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease leading to bile duct strictures and fibrosis, and predisposing to cholangiocarcinoma (CCA). Biliary dysplasia is a known precursor of CCA. In our unit, PSC patients undergo regular surveillance with ERC and brush cytology (BC), and liver transplantation is an option in case with biliary dysplasia. We evaluated the risk factors for biliary dysplasia and CCA based on ERC imaging, BC and liver function tests. Seven hundred and eighty-eight ERCs were performed with BC for 447 PSC patients. ERC images were evaluated using the modified Amsterdam score, neutrophilic inflammation was assessed in BC, and liver function tests were collected. Ploidy analysis with DNA flow cytometry was performed in cases with advanced PSC or previous suspicious BC/aneuploidy. The endpoint was either a benign disease course (follow-up for ≥2.4 years after the latest ERC), benign histology, biliary dysplasia or CCA. Benign disease course was seen in 424/447 (including 23 cases with biliary dysplasia), and CCA in 17 (3.8%) patients. Gallbladder carcinoma/carcinoma in situ was diagnosed in three patients. Advanced ERC findings, male gender, suspicious BC, aneuploidy in flow cytometry, inflammation, and elevation of ALP, bilirubin, ALT, AST, GGT, CEA and CA19-9 represented significant risk factors for CCA in univariate analysis. PSC patients with advanced bile duct disease and elevated liver enzymes, CEA or CA19-9, inflammation or suspicious BC are most likely to develop CCA. These patients may benefit from surveillance with BC if early liver transplantation is possible.

  5. Prospective evaluation of ursodeoxycholic acid withdrawal in patients with primary sclerosing cholangitis.

    Science.gov (United States)

    Wunsch, Ewa; Trottier, Jocelyn; Milkiewicz, Malgorzata; Raszeja-Wyszomirska, Joanna; Hirschfield, Gideon M; Barbier, Olivier; Milkiewicz, Piotr

    2014-09-01

    Ursodeoxycholic acid (UDCA) is no longer recommended for management of adult patients with primary sclerosing cholangitis (PSC). We undertook a prospective evaluation of UDCA withdrawal in a group of consecutive patients with PSC. Twenty six patients, all treated with UDCA (dose range: 10-15 mg/kg/day) were included. Paired blood samples for liver biochemistry, bile acids, and fibroblast growth factor 19 (FGF19) were collected before UDCA withdrawal and 3 months later. Liquid chromatography/tandem mass spectrometry was used for quantification of 29 plasma bile acid metabolites. Pruritus and health-related quality of life (HRQoL) were assessed with a 10-point numeric rating scale, the Medical Outcomes Study Short Form-36 (SF-36), and PBC-40 questionnaires. UDCA withdrawal resulted in a significant deterioration in liver biochemistry (increase of alkaline phosphatase of 75.6%; Pacid analysis revealed a significant decrease in lithocholic acid and its derivatives after UDCA withdrawal, but no effect on concentrations of primary bile acids aside from an increased accumulation of their taurine conjugates. After UDCA removal cholestatic parameters, taurine species of cholic acid and chenodeoxycholic acid correlated with serum FGF19 levels. No significant effect on HRQoL after UDCA withdrawal was observed; however, 42% of patients reported a deterioration in their pruritus. At 3 months, discontinuation of UDCA in patients with PSC causes significant deterioration in liver biochemistry and influences concentrations of bile acid metabolites. A proportion of patients report increased pruritus, but other short-term markers of quality of life are unaffected. © 2014 by the American Association for the Study of Liver Diseases.

  6. Complications of Sclerotherapy with Sclerosing Foam in Lower Extremity Varicose Veins

    Directory of Open Access Journals (Sweden)

    Hossein Hemmati

    2015-01-01

    Full Text Available Background: Using sclerotherapy with foam has caused a great change in treatment of varicose veins. Although, it is more than a century that it is being used, no exact and comprehensive knowledge of its complications has been published yet with regard to the existing facilities in Iran. Materials and Methods: In this cross-sectional study, Patients with varicose veins of lower extremity referred to cardiology clinic of Razi hospital, Rasht were included in the study after doppler sonography and in case of presence of varicose veins with more than 2 mm diameter in lower extremities without inadequacy of saphenofemoral and saphenopopliteal valve. They then underwent sclerosing with foam treatment. The total number of patients was 156 who were examined 2 weeks and 3 months after sclerotherapy in terms of complications such as pain, pigmentation, recurrence, phlebitis, deep vein thrombosis and skin necrosis. Results: Out of 156 patients, 47 were men and 109 women whose mean±SD age was 46.5±12.2 years. Two weeks after sclerotherapy, pain in 95 patients (65.1%, pigmentation in 79 patients (53.4%, recurrence in 5 patients (3.4%, necrosis in 8 patients (5.5% and no phlebitis was witnessed. Three months after sclerotherapy, pain in 10 patients (6.8%, pigmentation in 52 patients (35.1%, recurrence in 13 patients (8.8% and phlebitis in 13 patients (8.8% were seen; however, necrosis was not observed Conclusion: Sclerotherapy with foam is an effective, safe and inexpensive method for treatment of varicose veins of lower extremities. Thus, in case of a careful selection of patients and conduction in an equipped center, few complications will be seen.

  7. MR elastography in primary sclerosing cholangitis: correlating liver stiffness with bile duct strictures and parenchymal changes.

    Science.gov (United States)

    Bookwalter, Candice A; Venkatesh, Sudhakar K; Eaton, John E; Smyrk, Thomas D; Ehman, Richard L

    2018-04-07

    To determine correlation of liver stiffness measured by MR Elastography (MRE) with biliary abnormalities on MR Cholangiopancreatography (MRCP) and MRI parenchymal features in patients with primary sclerosing cholangitis (PSC). Fifty-five patients with PSC who underwent MRI of the liver with MRCP and MRE were retrospectively evaluated. Two board-certified abdominal radiologists in agreement reviewed the MRI, MRCP, and MRE images. The biliary tree was evaluated for stricture, dilatation, wall enhancement, and thickening at segmental duct, right main duct, left main duct, and common bile duct levels. Liver parenchyma features including signal intensity on T2W and DWI, and hyperenhancement in arterial, portal venous, and delayed phase were evaluated in nine Couinaud liver segments. Atrophy or hypertrophy of segments, cirrhotic morphology, varices, and splenomegaly were scored as present or absent. Regions of interest were placed in each of the nine segments on stiffness maps wherever available and liver stiffness (LS) was recorded. Mean segmental LS, right lobar (V-VIII), left lobar (I-III, and IVA, IVB), and global LS (average of all segments) were calculated. Spearman rank correlation analysis was performed for significant correlation. Features with significant correlation were then analyzed for significant differences in mean LS. Multiple regression analysis of MRI and MRCP features was performed for significant correlation with elevated LS. A total of 439/495 segments were evaluated and 56 segments not included in MRE slices were excluded for correlation analysis. Mean segmental LS correlated with the presence of strictures (r = 0.18, p duct strictures. Segments with increased LS show T2 hyperintensity, DWI hyperintensity, and post-contrast hyperenhancement. Global liver stiffness shows a moderate correlation with number of segmental strictures and significantly correlates with spleen stiffness, splenomegaly, and varices.

  8. IgG4-related sclerosing cholangitis overlapping with autoimmune hepatitis: Report of a case.

    Science.gov (United States)

    Li, Hongyan; Sun, Li; Brigstock, David R; Qi, Lina; Gao, Runping

    2017-05-01

    IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of IgG4-related disease (IgG4-RD) but the presence of IgG4-SC in the porta hepatis is difficult to differentiate from hilar cholangiocarcinoma (HCCA). IgG4-related autoimmune hepatitis (IgG4-related AIH) is extremely rare and it is not fully clear whether IgG4-related AIH is a hepatic manifestation of IgG4-RD or a subtype of AIH. We present a rare case of a 52-year-old male who was admitted with obstructive jaundice and itchy skin. He primarily presented a severe bile duct stricture in the porta hepatis and an elevated serum level of carbohydrate antigen 19-9 (CA19-9) mimicking HCCA. The patient underwent a surgical resection of the left hepatic lobular and cholecyst as well as common bile duct with a right hepatico-jejunostomy. He was finally diagnosed as IgG4-SC accompanied with IgG4-related AIH by immunohistochemistry, but he lacked conventional autoantibodies. The patient responded well to steroid therapy and remains healthy with no signs of recurrence at six-month follow-up. This is the first case report that hepatic portal IgG4-SC overlapping with IgG4-related AIH without the presence of conventional autoantibodies. Additionally, we suggest that IgG4-RD should be always considered in case of a bile duct stricture in the porta hepatis to avoid unnecessary surgical operation. Copyright © 2017 Elsevier GmbH. All rights reserved.

  9. Pancreatic changes in patients with primary sclerosing cholangitis: MR cholangiopancreatography and MRI findings

    International Nuclear Information System (INIS)

    Ozkavukcu, Esra; Erden, Ayse; Erden, Ilhan

    2009-01-01

    Purpose: To evaluate the possible pancreatic changes and their frequencies in patients with primary sclerosing cholangitis (PSC) on MR cholangiopancreatography (MRCP), and conventional abdominal MRI. Materials and Methods: Patient group consisted of 29 PSC (13 male, 16 female) cases, whereas cohort 1 consisted of 12 female patients with primary biliary cirrhosis, and cohort 2 consisted of 17 patients (6 male, 11 female) with non-immune chronic liver disease. Two radiologists retrospectively evaluated the MR examinations paying special attention to the pancreatic size (atrophy or enlargement), T1- and T2-signal intensity of the pancreas, focal pancreatic lesion, capsule-like rim, peripancreatic edema or fluid, fascial thickening, and pancreatic ducts (dilatation or narrowing). The results are expressed as percentages. Three groups were compared using Pearson chi-square test for each feature. However, only p-value for 'dilatation of the pancreatic duct' was determined, whereas p-value could not be calculated because of the insufficient number of subjects/sequences for the other features. Results: Twelve PSC patients (41.3%) had pancreatic abnormalities. The most common pancreatic changes in PSC patients were decreased T1-signal intensity (44%) and dilatation of the pancreatic duct (13.8%), respectively. Increased T2-signal intensity was also shown in 2 PSC patients (6.9%). Conclusion: Even PSC patients without any sign of pancreatitis, can show MR changes in the pancreatic parenchyma or the pancreatic duct. The etiologies of these changes, and whether they are unique to PSC, are still controversial. Histopathological studies bringing light to these pancreatic changes are needed.

  10. Individuals with Primary Sclerosing Cholangitis Have Elevated Levels of Biomarkers for Apoptosis but Not Necrosis.

    Science.gov (United States)

    Masuoka, Howard C; Vuppalanchi, Raj; Deppe, Ross; Bybee, Phelan; Comerford, Megan; Liangpunsakul, Suthat; Ghabril, Marwan; Chalasani, Naga

    2015-12-01

    Hepatocyte apoptosis or necrosis from accumulation of bile salts may play an important role in the disease progression of primary sclerosing cholangitis (PSC). The aim of the current study was to measure serum markers of hepatocyte apoptosis (cytokeratin-18 fragments--K18) and necrosis (high-mobility group protein B1--HMGB1) in adults with PSC and examine the relationship with disease severity. We measured serum levels of K18 and HMGB1 in well-phenotyped PSC (N = 37) and 39 control subjects (N = 39). Severity of PSC was assessed biochemically, histologically, and PSC Mayo risk score. Quantification of hepatocyte apoptosis was performed using TUNEL assay. The mean age of the study cohort was 49.7 ± 13.3 years and comprised of 67% men and 93% Caucasian. Serum K18 levels were significantly higher in the PSC patients compared to control (217.4 ± 78.1 vs. 157.0 ± 58.2 U/L, p = 0.001). However, HMGB1 levels were not different between the two groups (5.38 ± 2.99 vs. 6.28 ± 2.85 ng/mL, p = 0.15). Within the PSC group, K18 levels significantly correlated with AST (r = 0.5, p = 0.002), alkaline phosphatase (r = 0.5, p = 0.001), total bilirubin (r = 0.61, p ≤ 0.001), and albumin (r = -0.4, p = 0.02). Serum K18 levels also correlated with the level of apoptosis present on the liver biopsy (r = 0.8, p ≤ 0.001) and Mayo risk score (r = 0.4, p = 0.015). Serum K18 but not HMGB1 levels were increased in PSC and associated with severity of underlying liver disease and the degree of hepatocyte apoptosis.

  11. Perivascular epithelioid cell tumor (PEComa of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis

    Directory of Open Access Journals (Sweden)

    Ma Linglei

    2004-10-01

    Full Text Available Abstract Background The World Health Organization recently recognized a family of neoplasms showing at least partial morphological or immunohistochemical evidence of a putative perivascular epithelioid cell (PEC differentiation. These tumors include angiomyolipoma (AML, clear cell "sugar" tumors of the lung (CCST, lymphangioleiomyomatosis (LAM, clear cell myomelanocytic tumors of the falciform ligament and distinctive clear cell tumors at various other anatomic sites. Case presentation & methods A 41-year old gravida-1 para-1 with tuberous sclerosis presented with an incidentally identified 2.2 cm mass. The morphology and immunohistochemical profile was consistent with PEComa. Distinct aggregates of HMB-45 epithelioid cells were present in an occasionally distinctive perivascular distribution in the myometrium, small bowel lamina propria and ovarian hila. These distinctive aggregates, for which we propose the designation "PEComatosis" based on their intraabdominal distribution, did not display cytological atypia, mitotic activity or necrosis. CGH and DNA ploidy analysis showed a balanced chromosomal profile and diploid nuclei, respectively. There was no recurrence or metastases at 35 months' follow-up. Fifty-one previously reported cases of non-AML, LAM and CCST PEComas [perivascular epithelioid cell tumors- not otherwise specified (PEComa-NOS] are reviewed. Conclusions The lesions may be a reflection of tumor multicentricity, in which each may be a potential nidus for the development of future more well-developed tumors. Alternatively, they may be a manifestation of a poorly understood "field effect", in which there is an increased propensity to develop tumors of this type throughout the abdomen. Finally, and least likely in our opinion, they may represent tumor spread from its primary site.

  12. A novel rat fibrosarcoma cell line from transformed bone marrow-derived mesenchymal stem cells with maintained in vitro and in vivo stemness properties

    International Nuclear Information System (INIS)

    Wang, Meng-Yu; Nestvold, Janne; Rekdal, Øystein; Kvalheim, Gunnar; Fodstad, Øystein

    2017-01-01

    Increasing evidence suggests a possible relationship between mesenchymal stem cells (MSCs) and sarcoma. MSCs are hypothesized to be the cells initiating sarcomagenesis, and cancer stem cells (CSCs) sharing features of MSCs have been identified in sarcomas. Here, we report on the characteristics of a bone marrow-derived rat mesenchymal stem cell line that spontaneously transformed in long-term culture. The rat transformed mesenchymal stem cells (rTMSCs) produced soft-tissue fibrosarcomas in immunocompromised mice and immunocompetent rats. In vitro, the rTMSCs displayed increased proliferation capacity compared to the untransformed cell line. The transformed MSCs maintained the mesenchymal phenotype by expression of the stem cell marker CD 90 and the lack of hematopoietic and endothelial markers. Cytogenetic analysis detected trisomy 6 in the rTMSCs. Side population (SP) isolation and tumorsphere cultivation of the transformed cells confirmed the presence of CSCs among the rTMSCs. Importantly, the rTMSCs retained their differentiation capacity towards osteogenic and adipogenic lineages. This transformed MSC-based cell line may be valuable in examining the balance in a mixed cell population between cancer stem cell properties and the ability to differentiate to specific non-transformed cell populations. Moreover, it may also be a useful tool to evaluate the efficacy of novel targeted immunotherapies in vivo. - Highlights: • Spontaneously transformed rat MSCs (rTMSCs) share characteristics with normal MSCs. • rTMSCs possess a side population, enriched with tumorigenic cells. • rTMSCs model fibrosarcoma in vivo.

  13. A novel rat fibrosarcoma cell line from transformed bone marrow-derived mesenchymal stem cells with maintained in vitro and in vivo stemness properties

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Meng-Yu [Department of Cell Therapy, Institute for Cancer Research, Oslo University Hospital, Oslo (Norway); Nestvold, Janne, E-mail: j.m.nestvold@medisin.uio.no [Department of Molecular Medicine, Institute of Basic Medical Sciences, University of Oslo (Norway); Rekdal, Øystein [Department of Medical Biology, Faculty of Health Sciences, UiT The Arctic University of Norway, Tromsø (Norway); Kvalheim, Gunnar [Department of Cell Therapy, Institute for Cancer Research, Oslo University Hospital, Oslo (Norway); Fodstad, Øystein [Department of Tumor Biology, Institute for Cancer Research, Oslo University Hospital, Oslo (Norway)

    2017-03-15

    Increasing evidence suggests a possible relationship between mesenchymal stem cells (MSCs) and sarcoma. MSCs are hypothesized to be the cells initiating sarcomagenesis, and cancer stem cells (CSCs) sharing features of MSCs have been identified in sarcomas. Here, we report on the characteristics of a bone marrow-derived rat mesenchymal stem cell line that spontaneously transformed in long-term culture. The rat transformed mesenchymal stem cells (rTMSCs) produced soft-tissue fibrosarcomas in immunocompromised mice and immunocompetent rats. In vitro, the rTMSCs displayed increased proliferation capacity compared to the untransformed cell line. The transformed MSCs maintained the mesenchymal phenotype by expression of the stem cell marker CD 90 and the lack of hematopoietic and endothelial markers. Cytogenetic analysis detected trisomy 6 in the rTMSCs. Side population (SP) isolation and tumorsphere cultivation of the transformed cells confirmed the presence of CSCs among the rTMSCs. Importantly, the rTMSCs retained their differentiation capacity towards osteogenic and adipogenic lineages. This transformed MSC-based cell line may be valuable in examining the balance in a mixed cell population between cancer stem cell properties and the ability to differentiate to specific non-transformed cell populations. Moreover, it may also be a useful tool to evaluate the efficacy of novel targeted immunotherapies in vivo. - Highlights: • Spontaneously transformed rat MSCs (rTMSCs) share characteristics with normal MSCs. • rTMSCs possess a side population, enriched with tumorigenic cells. • rTMSCs model fibrosarcoma in vivo.

  14. Measurement of differences in pO2 in response to perfluorocarbon/carbogen in FSa and NFSa murine fibrosarcomas with low-frequency electron paramagnetic resonance oximetry.

    Science.gov (United States)

    Halpern, H J; Yu, C; Peric, M; Barth, E D; Karczmar, G S; River, J N; Grdina, D J; Teicher, B A

    1996-05-01

    We have used very low-frequency electron paramagnetic resonance (EPR) oximetry to measure the change in oxygen concentration (delta pO2) due to change in breathing atmosphere in FSa and NFSa fibrosarcomas implanted in the legs of C3H mice infused with perfluoro-octylbromine (PFOB). Measurements in each tumor were made before and after the administration of the high-density (47% v/v) perfluorocarbon PFOB, perflubron (Alliance Pharmaceutical Corporation, San Diego, CA). Measurements in each tumor were also made, after the administration of the PFOB, both before (PFOB/air) and after the administration of carbogen (95% O2 + 5% CO2, PFOB/carbogen). Large changes (delta p02) relative to PFOB/air oxygenation were seen with the administration of PFOB/carbogen. No significant difference in oxygen concentration was seen between air-breathing mice with and without PFOB. The mean delta pO2 for FSa tumors was 13 +/- 6 torr, while the mean for NFSa fibrosarcomas was 28 +/- 7 torr. There were such large intertumor differences that the trend toward a smaller change in the more hypoxic FSa tumors was not significant (P = 0.13). This paper describes a novel method of measuring differences in oxygenation in tumor tissues. The results of such measurements indicate large differences in pO2 response to different breathing atmospheres in PFOB-infused tumors of similar histology. The intertumor delta pO2 differences may correlate with differences in radiation response.

  15. Effect of radiotherapy in combination with liposomal MTP-PE or inhibitors of metabolism of eicosanoids on the growth of experimental fibrosarcoma

    International Nuclear Information System (INIS)

    Fedorocko, P.; Sedlakova, Z.; Mackova, N.; Sabol, M.; Eliasova, V.; Kocikova, A.; Matula, P.

    1998-01-01

    The immunophenotypization, tumorigenicity and immunogenicity of the tumor were characterized. The tumorigenic dose of the fibrosarcoma G5:1:13 for mice C3H/DiSn when administered s.c. is 1x10 5 per mouse. With this dose, tumors with an average volume of 64 mm 3 on day 6 developed; 98-100% mice died on day 120. The phenotype of the fibrosarcoma, which is immunogenic, is MHC-I + , HNC-II - , B7 - , B7.2 - . After administration of irradiated tumor cells (2 doses, s.c., 1x10 6 per mouse), a tumor developed in none of the animals to which the non-irradiated tumor cells had been administered. The effect of the individual factors was examined. Gamma radiation affected survival of the mice as follows: 2x8 Gy (60%), 4x6 Gy (80%), 5x5 Gy (80%), 3x7 Gy (80%), in positive correlation with the tumor size. The growth was affected similarly by MTP-PE (40%), diclofenac (40%), ibuprofen (40%) and flurbiprofen (30%). When diclofenac was combined with MTP-PE, a surprising strengthening of the tumor growth was observed. This finding is very important in view of the clinical use of MTP-PE and inhibitors of arachidonic acid metabolism in the treatment of tumorous diseases

  16. The effect of ginkgo biloba extract on the fractionated radiation therapy in C3H mouse fibrosarcoma

    International Nuclear Information System (INIS)

    Kim, Jong Hoon; Ha, Sung Whan; Park, Charn Il

    2002-01-01

    A gingko biloba extract (GBE) has been known as a hypoxic cell radiosensitizer. Its mechanisms of action are increase of the red blood cell deformability, decrease the blood viscosity, and decrease the hypoxic cell fraction in the tumor. The aims of this study were to estimate the effect of GBE on fractionated radiotherapy and to clarify the mechanism of action of the GBE by estimating the blood flow in tumor and normal muscle. Fibrosarcoma (FSall) growing in a C3H mouse leg muscle was used as the tumor model. When the tumor size reached 7 mm in diameter, the GBE was given intraperitoneally at 1 and 25 hours prior to irradiation. The tumor growth delay was measured according to the various doses of radiation (3, 6, 9, 12, Gy and 15 Gy) and to the fractionation (single and fractionated irradiation) with and without the GBE injection. The radiation dose to the tumor the response relationships and the enhancement ratio of the GBE were measured. In addition, the blood flow of a normal muscle and a tumor was compared by laser Doppler flowmetry according to the GBE treatment. When the GBE was used with single fraction irradiation with doses ranging from 3 to 12 Gy, GBE increased the tumor growth delay significantly (ρ < 0.05) and the enhancement ratio of the GBE was 1.16. In fractionated irradiation with 3 Gy per day, the relationships between the radiation dose (D) and the tumor growth delay (TGD) were TGD (days) = 0.26 x D (Gy)+0.13 in the radiation alone group, and the TGD (days) = 0.30 x D (Gy) + 0.13 in the radiation with GBE group. As a result, the enhancement ratio was 1.19 (95% confidence interval; 1.13 ∼ 1.27). Laser Doppler flowmetry was used to measure the blood flow. The mean blood flow was higher in the muscle (7.78 mL/100 g/min in tumor and the 10.15 mL/100 g/min in muscle, ρ = 0.0001) and the low blood flow fraction (less than 2 mL/100 g/min) was higher in the tumor (0.5% vs. 5.2%, ρ = 0.005). The blood flow was not changed with the GBE in normal

  17. Microbubbles in macrocysts - Contrast-enhanced ultrasound assisted sclerosant therapy of a congenital macrocystic lymphangioma: a case report.

    Science.gov (United States)

    Menendez-Castro, Carlos; Zapke, Maren; Fahlbusch, Fabian; von Goessel, Heiko; Rascher, Wolfgang; Jüngert, Jörg

    2017-07-06

    Congenital cystic lymphangiomas are benign malformations due to a developmental disorder of lymphatic vessels. Besides surgical excision, sclerosant therapy of these lesions by intracavitary injection of OK-432 (Picibanil®), a lyophilized mixture of group A Streptococcus pyogenes, is a common therapeutical option. For an appropriate application of OK-432, a detailed knowledge about the structure and composition of the congenital cystic lymphangioma is essential. SonoVue® is a commercially available contrast agent commonly used in sonography by intravenous and intracavitary application. Here we report the case of 2 month old male patient with a large thoracic congenital cystic lymphangioma. Preinterventional imaging of the malformation was performed by contrast-enhanced ultrasound after intracavitary application of SonoVue® immediately followed by a successful sclerotherapy with OK-432. Contrast agent-enhanced ultrasound imaging offers a valuable option to preinterventionally clarify the anatomic specifications of a congenital cystic lymphangioma in more detail than by single conventional sonography. By the exact knowledge about the composition and especially about the intercystic communications of the lymphangioma sclerosant therapy becomes safer and more efficient.

  18. IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis.

    Science.gov (United States)

    Detlefsen, Sönke; Klöppel, Günter

    2018-04-01

    In 2011, chronic fibroinflammatory processes occurring simultaneously or metachronously in various organs and associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4-positive plasma cells have been recognized as manifestations of a systemic disorder called IgG4-related disease (IgG4-RD). The histologic key findings are lymphoplasmacytic infiltration rich in IgG4-positive plasma cells combined with storiform fibrosis and obliterative phlebitis. Among the organs mainly affected by IgG4-RD are the pancreas and the extrahepatic bile ducts. The pancreatic and biliary alterations have been described under the terms autoimmune pancreatitis (AIP) and sclerosing cholangitis, respectively. These diseases are currently more precisely called IgG4-related pancreatitis (or type 1 AIP to distinguish it from type 2 AIP that is unrelated to IgG4-RD) and IgG4-related sclerosing cholangitis (IgG4-related SC). Clinically and grossly, both diseases commonly imitate pancreatic and biliary adenocarcinoma, tumors that are well known for their dismal prognosis. As IgG4-RD responds to steroid treatment, making a resection of a suspected tumor unnecessary, a biopsy is often required to establish the preoperative diagnosis. This review discusses the morphologic spectrum of IgG4-related pancreatitis and IgG4-related SC and focuses on the biopsy relevant histologic features for the diagnosis and differential diagnosis of these diseases.

  19. Surgical management of isolated mesenteric autoimmune disease: addressing the spectrum of IgG4-related disease and sclerosing mesenteritis.

    Science.gov (United States)

    Greenbaum, Alissa; Yadak, Nour; Perez, Steven; Rajput, Ashwani

    2017-06-08

    IgG 4 -related disease (IgG 4 -RD) is a rare form of autoimmune sclerosing disease, characterised by elevated serum IgG 4 and tissue IgG 4 levels, specific histopathological findings, multiorgan involvement and adequate response to glucocorticoid treatment. The low incidence and the heterogeneous nature of the disease has made consensus on diagnostic criteria for IgG 4 -RD difficult. Whether sclerosing mesenteritis (SM) is considered a manifestation of IgG 4 -RD is strongly debated. We present the case of a patient with a history of rheumatoid arthritis who presented with a calcified abdominal mass. She was found to have an isolated, pedunculated mesenteric mass positive for IgG 4 and concurrently elevated serum IgG 4 levels. Clinical features did not classify her disease as either SM or IgG 4 -RD as currently described in consensus statements. Concurrent diagnoses of IgG 4 -RD, SM and other autoimmune disorders, as well as postoperative recommendations for resected isolated IgG 4 -positive masses, are discussed. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  20. IgG4 related sclerosing mastitis: expanding the morphological spectrum of IgG4 related diseases.

    Science.gov (United States)

    Chougule, Abhijit; Bal, Amanjit; Das, Ashim; Singh, Gurpreet

    2015-01-01

    IgG4 related disease (IgG4RD) is a recently recognised condition characterised by mass forming lesions associated with storiform fibrosis, obliterative phlebitis, lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells and elevated serum IgG4 levels. Although rare, mammary involvement has been reported as IgG4 related sclerosing mastitis, the morphological counterpart of a growing family of IgG4 related diseases. A total of 17 cases belonging to mass forming benign inflammatory breast lesions such as plasma cell mastitis, granulomatous lobular mastitis, non-specific mastitis and inflammatory pseudotumour were investigated as a possible member of IgG4 related sclerosing mastitis. Clinical, radiological, histopathological and immunohistochemistry findings were noted in all cases. Cases diagnosed as inflammatory pseudotumour showed all the histopathological features of IgG4RD along with increased number of IgG4 positive plasma cells and IgG4/IgG ratio >40%. However, only a few IgG4 positive cells were seen in plasma cell mastitis, granulomatous lobular mastitis and non-specific mastitis cases. These cases also did not fulfill the morphological criteria for the diagnosis of IgG4 related diseases. IgG4RD should be excluded in plasma cell rich lesions diagnosed on core biopsies by IgG4 immunostaining. This can avoid unnecessary surgery as IgG4 related diseases respond to simple and effective steroid treatment.

  1. Ursodeoxycholic acid therapy for primary sclerosing cholangitis: results of a 2-year randomized controlled trial to evaluate single versus multiple daily doses

    NARCIS (Netherlands)

    van Hoogstraten, H. J.; Wolfhagen, F. H.; van de Meeberg, P. C.; Kuiper, H.; Nix, G. A.; Becx, M. C.; Hoek, A. C.; van Houte, D. P.; Rijk, M. C.; Salemans, J. M.; Scherpenisse, J.; Schrijver, M.; Smit, A. M.; Spoelstra, P.; Stadhouders, P. H.; Tan, T. G.; Hop, W. C.; ten Kate, F. J.; vanBerge-Henegouwen, G. P.; Schalm, S. W.; van Buuren, H. R.

    1998-01-01

    Ursodeoxycholic acid has been reported to be of potential benefit for primary sclerosing cholangitis but little is known about the long-term biochemical, histological and radiological efficacy or the optimum frequency of ursodeoxycholic acid administration. A 2-year multicentre randomised controlled

  2. Gut barrier failure biomarkers are associated with poor disease outcome in patients with primary sclerosing cholangitis

    Science.gov (United States)

    Tornai, Tamas; Palyu, Eszter; Vitalis, Zsuzsanna; Tornai, Istvan; Tornai, David; Antal-Szalmas, Peter; Norman, Gary L; Shums, Zakera; Veres, Gabor; Dezsofi, Antal; Par, Gabriella; Par, Alajos; Orosz, Peter; Szalay, Ferenc; Lakatos, Peter Laszlo; Papp, Maria

    2017-01-01

    AIM To assess the prevalence of a panel of serologic markers that reflect gut barrier dysfunction in a mixed cohort of pediatric and adult primary sclerosing cholangitis (PSC) patients. METHODS Sera of 67 PSC patients [median age (range): 32 (5-79) years, concomitant IBD: 67% and cirrhosis: 20%] were assayed for the presence of antibodies against to F-actin (AAA IgA/IgG) and gliadin (AGA IgA/IgG)] and for serum level of intestinal fatty acid-binding protein (I-FABP) by ELISA. Markers of lipopolysaccharide (LPS) exposure [LPS binding protein (LBP)] and various anti-microbial antibodies [anti-OMP Plus IgA and endotoxin core IgA antibody (EndoCAb)] were also determined. Poor disease outcome was defined as orthotopic liver transplantation and/or liver-related death during the follow-up [median: 99 (14-106) mo]. One hundred and fifty-three healthy subjects (HCONT) and 172 ulcerative colitis (UC) patients were the controls. RESULTS A total of 28.4%, 28.0%, 9% and 20.9% of PSC patients were positive for AAA IgA, AAA IgG, AGA IgA and AGA IgG, respectively. Frequencies of AAA IgA and AAA IgG (P < 0.001, for both) and AGA IgG (P = 0.01, for both) but not AGA IgA were significantly higher compared to both of the HCONT and the UC groups. In survival analysis, AAA IgA-positivity was revealed as an independent predictor of poor disease outcome after adjusting either for the presence of cirrhosis [HR = 5.15 (1.27-20.86), P = 0.022 or for the Mayo risk score (HR = 4.24 (0.99-18.21), P = 0.052]. AAA IgA-positivity was significantly associated with higher frequency of anti-microbial antibodies (P < 0.001 for EndoCab IgA and P = 0.012 for anti-OMP Plus IgA) and higher level of the enterocyte damage marker (median I-FABPAAA IgA pos vs neg: 365 vs 166 pg/mL, P = 0.011), but not with serum LBP level. CONCLUSION Presence of IgA type AAA identified PSC patients with progressive disease. Moreover, it is associated with enhanced mucosal immune response to various microbial antigens and

  3. Triphasic and epithelioid minimal fat renal angiomyolipoma and clear cell renal cell carcinoma: qualitative and quantitative CEUS characteristics and distinguishing features.

    Science.gov (United States)

    Lu, Qing; Li, Cui-xian; Huang, Bei-jian; Xue, Li-yun; Wang, Wen-ping

    2015-02-01

    To determine the contrast-enhanced ultrasonography (CEUS) characteristics of minimal fat renal angiomyolipoma (AML) (triphasic and epithelioid) and compare them to each other and to clear cell renal cell carcinoma (ccRCC) to explore their differential diagnostic clue. Qualitative and quantitative CEUS analyses were retrospectively conducted for epithelioid renal AMLs (EAMLs) (n = 15), triphasic minimal fat AMLs (TAMLs) (n = 25), and ccRCCs (n = 113). Enhancement patterns and features with CEUS were qualitatively evaluated. As for the quantitative parameters, rise times (RT), time to peak (TTP), and tumor-to-cortex enhancement ratio (TOC ratio) were compared among these renal tumor histotypes. No significant differences were detected on conventional ultrasound in the three histotypes of renal tumor. On qualitative CEUS analysis, centripetal enhancement in cortical phase (73.3% in EAMLs, 84.0% in TAMLs vs. 18.6% in ccRCCs, p qualitative and quantitative characteristics made no significant difference between EAMLs and TAMLs. In the differential diagnosis of EAMLs from TAMLs, pseudocapsule sign was valuable (40.0% in EAMLs vs. 0.0% in TAMLs, p 97.34% as the criteria to differentiate ccRCCs and EAMLs from TAMLs, the sensitivity and specificity were 80.0% and 87.5%, respectively. Qualitative and quantitative CEUS analyses are helpful in the differential diagnosis of ccRCCs, EAMLs, and TAMLs.

  4. Frequent deletion of 3p21.1 region carrying semaphorin 3G and aberrant expression of the genes participating in semaphorin signaling in the epithelioid type of malignant mesothelioma cells.

    Science.gov (United States)

    Yoshikawa, Yoshie; Sato, Ayuko; Tsujimura, Tohru; Morinaga, Tomonori; Fukuoka, Kazuya; Yamada, Shusai; Murakami, Aki; Kondo, Nobuyuki; Matsumoto, Seiji; Okumura, Yoshitomo; Tanaka, Fumihiro; Hasegawa, Seiki; Hashimoto-Tamaoki, Tomoko; Nakano, Takashi

    2011-12-01

    Array-based comparative genomic hybridization analysis was performed on 21 malignant mesothelioma (MM) samples (16 primary cell cultures and 5 cell lines) and two reactive mesothelial hyperplasia (RM) primary cell cultures. The RM samples did not have any genomic losses or gains. In MM samples, deletions in 1p, 3p21, 4q, 9p21, 16p13 and 22q were detected frequently. We focused on 3p21 because this deletion was specific to the epithelioid type. Especially, a deletion in 3p21.1 region carrying seven genes including SEMA3G was found in 52% of MM samples (11 of 14 epithelioid samples). The allele loss of 3p21.1 might be a good marker for the epithelioid MM. A homozygous deletion in this region was detected in two MM primary cell cultures. A heterozygous deletion detected in nine samples contained the 3p21.1 region and 3p21.31 one carrying the candidate tumor suppressor genes such as semaphorin 3F (SEMA3F), SEMA3B and Ras association (RalGDS/AF-6) domain family member 1 (RASSF1A). SEMA3B, 3F and 3G are class 3 semaphorins and inhibit growth by competing with vascular endothelial growth factor (VEGF) through binding to neuropilin. All MM samples downregulated the expression of more than one gene for SEMA3B, 3F and 3G when compared with Met5a, a normal pleura-derived cell line. Moreover, in 12 of 14 epithelioid MM samples the expression level of SEMA3A was lower than that in Met5a and the two RM samples. An augmented expression of VEGFA was detected in half of the MM samples. The expression ratio of VEGFA/SEMA3A was significantly higher in the epithelioid MMs than in Met5a, RMs and the non-epithelioid MMs. Our data suggest that the downregulated expression of SEMA3A and several SEMA3s results in a loss of inhibitory activities in tumor angiogenesis and tumor growth of VEGFA; therefore, it may play an important role on the pathogenesis of the epithelioid type of MM.

  5. Dimerization of endogenous MT1-MMP is a regulatory step in the activation of the 72-kDa gelatinase MMP-2 on fibroblasts and fibrosarcoma cells

    DEFF Research Database (Denmark)

    Ingvarsen, Signe; Madsen, Daniel H.; Hillig, Thore

    2008-01-01

    The secreted gelatinase matrix metalloprotease-2 (MMP-2) and the membrane-anchored matrix metalloprotease MT1-MMP (MMP-14), are central players in pericellular proteolysis in extracellular matrix degradation. In addition to possessing a direct collagenolytic and gelatinolytic activity......, these enzymes take part in a cascade pathway in which MT1-MMP activates the MMP-2 proenzyme. This reaction occurs in an interplay with the matrix metalloprotease inhibitor, TIMP-2, and the proposed mechanism involves two molecules of MT1-MMP in complex with one TIMP-2 molecule. We provide positive evidence...... that proMMP-2 activation is governed by dimerization of MT1-MMP on the surface of fibroblasts and fibrosarcoma cells. Even in the absence of transfection and overexpression, dimerization of MT1-MMP markedly stimulated the formation of active MMP-2 products. The effect demonstrated here was brought about...

  6. Use of Molecular Imaging Markers of Glycolysis, Hypoxia and Proliferation (18F-FDG, 64Cu-ATSM and 18F-FLT) in a Dog with Fibrosarcoma

    DEFF Research Database (Denmark)

    Zornhagen, Kamilla; Clausen, Malene; Hansen, Anders Elias

    2015-01-01

    of cancer patients. A dog with fibrosarcoma was imaged using 18F-FDG, 64Cu-ATSM, and 18F-FLT before, during, and after 10 fractions of 4.5 Gy radiotherapy. Uptake of all tracers decreased during treatment. Fluctuations in 18F-FDG and 18F-FLT PET uptakes and a heterogeneous spatial distribution of the three......Glycolysis, hypoxia, and proliferation are important factors in the tumor microenvironment contributing to treatment-resistant aggressiveness. Imaging these factors using combined functional positron emission tomography and computed tomography can potentially guide diagnosis and management...... tracers were seen. Tracer distributions partially overlapped. It appears that each tracer provides distinct information about tumor heterogeneity and treatment response....

  7. Galangin and kaempferol suppress phorbol-12-myristate-13-acetate-induced matrix metalloproteinase-9 expression in human fibrosarcoma HT-1080 cells.

    Science.gov (United States)

    Choi, Yu Jung; Lee, Young Hun; Lee, Seung-Taek

    2015-01-01

    Matrix metalloproteinase (MMP)-9 degrades type IV collagen in the basement membrane and plays crucial roles in several pathological implications, including tumorigenesis and inflammation. In this study, we analyzed the effect of flavonols on MMP-9 expression in phorbol-12-myristate-13-acetate (PMA)-induced human fibrosarcoma HT-1080 cells. Galangin and kaempferol efficiently decreased MMP-9 secretion, whereas fisetin only weakly decreased its secretion. Galangin and kaempferol did not affect cell viability at concentrations up to 30 μM. Luciferase reporter assays showed that galangin and kaempferol decrease transcription of MMP-9 mRNA. Moreover, galangin and kaempferol strongly reduce IκBα phosphorylation and significantly decrease JNK phosphorylation. These results indicate that galangin and kaempferol suppress PMA-induced MMP-9 expression by blocking activation of NF-κB and AP-1. Therefore, these flavonols could be used as chemopreventive agents to lower the risk of diseases involving MMP-9.

  8. Epithelioid schwannoma of the skin displaying unique histopathological features: a teaching case giving rise to diagnostic difficulties on a morphological examination of a resected specimen, with a brief literature review.

    Science.gov (United States)

    Yamada, Sohsuke; Kirishima, Mari; Hiraki, Tsubasa; Higashi, Michiyo; Hatanaka, Kazuhito; Tanimoto, Akihide

    2017-01-19

    Epithelioid schwannoma as a rare variant poses a challenge to all pathologists, as this uncommon entity is extremely difficult to conclusively diagnose by morphological analyses on a resected sample alone owing to its unique histopathological features. However, few papers have described the detailed clinicopathological characteristics of epithelioid schwannoma. A 65-year-old female presented with a history of a flat and slightly elevated firm and tan plaque accompanied by occasional tenderness, measuring 10 × 8 mm, in the right joint of her hand 1 year before resection. A gross examination of a locally resected specimen revealed an encapsulated nodular lesion, yellow-whitish in color, partly filled with blood. A microscopic examination showed that the tumor predominantly consisted of a solid proliferation of epithelioid cells having mildly enlarged and round to partially spindled nuclei and abundant vacuolated or clear cytoplasm with very few mitotic figures and modest nuclear size variation, associated with focal hyalinized, cystic and hemorrhagic degeneration. This well-demarcated tumor was surrounded by dense, hyalinized and layered fibrocollagenous stroma. Immunohistochemically, these tumor cells were diffusely positive for S-100 protein and had a very low MIB-1 labeling index, and type IV collagen was strongly reactive with reduplicated basal lamina of them. We ultimately made a diagnosis of cutaneous epithelioid schwannoma. We should be aware that, since pathologists might misinterpret epithelioid schwannoma as other soft tissue tumors, including its malignant counterpart, a wide panel of immunohistochemical antibodies can be powerful supplementary tools for identifying a very rare entity of conventional schwannoma.

  9. Synthesis and evaluation of Tc-99m-labeled RRL-containing peptide as a non-invasive tumor imaging agent in a mouse fibrosarcoma model.

    Science.gov (United States)

    Kim, Dae-Weung; Kim, Woo Hyoung; Kim, Myoung Hyoun; Kim, Chang Guhn

    2015-11-01

    Arginine-arginine-leucine (RRL) is considered a tumor endothelial cell-specific binding sequence. RRL-containing peptide targeting tumor vessels is an excellent candidate for tumor imaging. In this study, we developed RRL-containing hexapeptides and evaluated their feasibility as a tumor imaging agent in a HT-1080 fibrosarcoma-bearing murine model. The hexapeptide, glutamic acid-cysteine-glycine (ECG)-RRL was synthesized using Fmoc solid-phase peptide synthesis. Radiolabeling efficiency was evaluated using instant thin-layer chromatography. Uptake of Tc-99m ECG-RRL within HT-1080 cells was evaluated in vitro by confocal microscopy and cellular binding affinity was calculated. Gamma images were acquired In HT-1080 fibrosarcoma tumor-bearing mice, and the tumor-to-muscle uptake ratio was calculated. The inflammatory-to-normal muscle uptake ratio was also calculated in an inflammation mouse model. A biodistribution study was performed to calculate %ID/g. A high yield of Tc-99m ECG-RRL complexes was prepared after Tc-99m radiolabeling. Binding of Tc-99m ECG-RRL to tumor cells had was confirmed by in vitro studies. Gamma camera imaging in the murine model showed that Tc-99m ECG-RRL accumulated substantially in the subcutaneously engrafted tumor and that tumoral uptake was blocked by co-injecting excess RRL. Moreover, Tc-99m ECG-RRL accumulated minimally in inflammatory lesions. We successfully developed Tc-99m ECG-RRL as a new tumor imaging candidate. Specific tumoral uptake of Tc-99m ECG-RRL was evaluated both in vitro and in vivo, and it was determined to be a good tumor imaging candidate. Additionally, Tc-99m ECG-RRL effectively distinguished between cancerous tissue and inflammatory lesions.

  10. Small interfering RNA targeting HIF-1{alpha} reduces hypoxia-dependent transcription and radiosensitizes hypoxic HT 1080 human fibrosarcoma cells in vitro

    Energy Technology Data Exchange (ETDEWEB)

    Staab, Adrian [Wuerzburg Univ. (Germany). Dept. of Radiation Oncology; Paul Scherrer Institute (PSI), Villigen (Switzerland); Fleischer, Markus [Wuerzburg Univ. (Germany). Dept. of Radiation Oncology; Wuerzburg Univ. (Germany). Medical Clinic II; Loeffler, Juergen; Einsele, Herrmann [Wuerzburg Univ. (Germany). Medical Clinic II; Said, Harun M.; Katzer, Astrid; Flentje, Michael [Wuerzburg Univ. (Germany). Dept. of Radiation Oncology; Plathow, Christian [Freiburg Univ. (Germany). Dept. of Nuclear Medicine; Vordermark, Dirk [Wuerzburg Univ. (Germany). Dept. of Radiation Oncology; Halle-Wittenberg Univ. (Germany). Dept. of Radiation Oncology

    2011-04-15

    Background: Hypoxia inducible factor-1 has been identified as a potential target to overcome hypoxia-induced radioresistance The aim of the present study was to investigate whether selective HIF-1 inhibition via small interfering RNA (siRNA) targeting hypoxia-inducible factor 1{alpha} (HIF-1{alpha}) affects hypoxia-induced radioresistance in HT 1080 human fibrosarcoma cells. Material and Methods: HIF-1{alpha} expression in HT 1080 human fibrosarcoma cells in vitro was silenced using HIF-1{alpha} siRNA sequence primers. Quantitative real-time polymerase chain reaction assay was performed to quantify the mRNA expression of HIF-1{alpha}. HIF-1{alpha} protein levels were studied by Western blotting at 20% (air) or after 12 hours at 0.1% O{sub 2} (hypoxia). Cells were assayed for clonogenic survival after irradiation with 2, 5, or 10 Gy, under normoxic or hypoxic conditions in the presence of HIF-1{alpha}-targeted or control siRNA sequences. A modified oxygen enhancement ratio (OER') was calculated as the ratio of the doses to achieve the same survival at 0.1% O{sub 2} as at ambient oxygen tensions. OER' was obtained at cell survival levels of 50%, 37%, and 10%. Results: HIF-1{alpha}-targeted siRNA enhanced radiation treatment efficacy under severely hypoxic conditions compared to tumor cells treated with scrambled control siRNA. OER was reduced on all survival levels after treatment with HIF-1{alpha}-targeted siRNA, suggesting that inhibition of HIF-1 activation by using HIF-1{alpha}-targeted siRNA increases radiosensitivity of hypoxic tumor cells in vitro. Conclusion: Inhibition of HIF-1 activation by using HIF-1{alpha}-targeted siRNA clearly acts synergistically with radiotherapy and increase radiosensitivity of hypoxic cells in vitro. (orig.)

  11. Small interfering RNA targeting HIF-1α reduces hypoxia-dependent transcription and radiosensitizes hypoxic HT 1080 human fibrosarcoma cells in vitro

    International Nuclear Information System (INIS)

    Staab, Adrian; Fleischer, Markus; Wuerzburg Univ.; Loeffler, Juergen; Einsele, Herrmann; Said, Harun M.; Katzer, Astrid; Flentje, Michael; Plathow, Christian; Vordermark, Dirk; Halle-Wittenberg Univ.

    2011-01-01

    Background: Hypoxia inducible factor-1 has been identified as a potential target to overcome hypoxia-induced radioresistance The aim of the present study was to investigate whether selective HIF-1 inhibition via small interfering RNA (siRNA) targeting hypoxia-inducible factor 1α (HIF-1α) affects hypoxia-induced radioresistance in HT 1080 human fibrosarcoma cells. Material and Methods: HIF-1α expression in HT 1080 human fibrosarcoma cells in vitro was silenced using HIF-1α siRNA sequence primers. Quantitative real-time polymerase chain reaction assay was performed to quantify the mRNA expression of HIF-1α. HIF-1α protein levels were studied by Western blotting at 20% (air) or after 12 hours at 0.1% O 2 (hypoxia). Cells were assayed for clonogenic survival after irradiation with 2, 5, or 10 Gy, under normoxic or hypoxic conditions in the presence of HIF-1α-targeted or control siRNA sequences. A modified oxygen enhancement ratio (OER') was calculated as the ratio of the doses to achieve the same survival at 0.1% O 2 as at ambient oxygen tensions. OER' was obtained at cell survival levels of 50%, 37%, and 10%. Results: HIF-1α-targeted siRNA enhanced radiation treatment efficacy under severely hypoxic conditions compared to tumor cells treated with scrambled control siRNA. OER was reduced on all survival levels after treatment with HIF-1α-targeted siRNA, suggesting that inhibition of HIF-1 activation by using HIF-1α-targeted siRNA increases radiosensitivity of hypoxic tumor cells in vitro. Conclusion: Inhibition of HIF-1 activation by using HIF-1α-targeted siRNA clearly acts synergistically with radiotherapy and increase radiosensitivity of hypoxic cells in vitro. (orig.)

  12. Immunoglobulin G4-related sclerosing disease manifesting as bilateral tonsillar hypertrophy on MRI images: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Mee Hyun; Woo, Ji Young; Lee, Yul; Yoon, Dae Young; Hong, Hye Sook; Hong, Min Eui [Hallym University College of Medicine, Kangnam Sacred Heart Hospital, Seoul (Korea, Republic of)

    2016-02-15

    Immunoglobulin G4-related sclerosing disease (IgG4-SD) is currently recognized as a distinct systemic disease involving various organs. We reported the imaging findings of a case of pathologically confirmed IgG4-SD involving bilateral palatine tonsils. CT and MRI showed diffuse enlargement of both palatine tonsils with homogeneous contrast enhancement. Focal contour bulging was noted in the right palatine tonsil. Lesions appeared as isointense on T1-weighted and slightly hyperintense on T2-weighted MRI images, as compared with muscle. The T2-weighted MRI image showed a striated pattern in both tonsils. Despite its rare occurrence, IgG4-SD should be included in the differential diagnoses of patients with symptomatic bilateral tonsillar hypertrophy that is non-responsive to medication.

  13. Immunoglobulin G4-related sclerosing disease manifesting as bilateral tonsillar hypertrophy on MRI images: A case report

    International Nuclear Information System (INIS)

    Park, Mee Hyun; Woo, Ji Young; Lee, Yul; Yoon, Dae Young; Hong, Hye Sook; Hong, Min Eui

    2016-01-01

    Immunoglobulin G4-related sclerosing disease (IgG4-SD) is currently recognized as a distinct systemic disease involving various organs. We reported the imaging findings of a case of pathologically confirmed IgG4-SD involving bilateral palatine tonsils. CT and MRI showed diffuse enlargement of both palatine tonsils with homogeneous contrast enhancement. Focal contour bulging was noted in the right palatine tonsil. Lesions appeared as isointense on T1-weighted and slightly hyperintense on T2-weighted MRI images, as compared with muscle. The T2-weighted MRI image showed a striated pattern in both tonsils. Despite its rare occurrence, IgG4-SD should be included in the differential diagnoses of patients with symptomatic bilateral tonsillar hypertrophy that is non-responsive to medication

  14. Secondary Sclerosing Cholangitis in Critically Ill Patients: Clinical Presentation, Cholangiographic Features, Natural History, and Outcome: A Series of 16 Cases.

    Science.gov (United States)

    Leonhardt, Silke; Veltzke-Schlieker, Wilfried; Adler, Andreas; Schott, Eckart; Eurich, Dennis; Faber, Wladimir; Neuhaus, Peter; Seehofer, Daniel

    2015-12-01

    Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is an important differential diagnosis in patients presenting with cholestasis and PSC-like cholangiographic changes in endoscopic retrograde cholangiography (ERC). As a relatively newly described entity, SSC-CIP is still underdiagnosed, and the diagnosis is often delayed. The present study aims to improve the early detection of SSC-CIP and the identification of its complications.A total of 2633 records of patients who underwent or were listed for orthotopic liver transplantation at the University Hospital Charité, Berlin, were analyzed retrospectively. The clinical presentation and outcome (mean follow-up 62.7 months) of the 16 identified SSC-CIP cases were reviewed.Cholestasis was the first sign of SSC-CIP. GGT was the predominant enzyme of cholestasis. Hypercholesterolemia occurred in at least 75% of the patients. SSC-CIP provoked a profound weight loss (mean 18 kg) in 94% of our patients. SSC-CIP was diagnosed by ERC in all patients. The 3 different cholangiographic features detected correspond roughly to the following stages: (I) evidence of biliary casts, (II) progressive destruction of intrahepatic bile ducts, and (III) picture of pruned tree. Biliary cast formation is a hallmark of SSC-CIP and was seen in 87% of our cases. In 75% of the patients, the clinical course was complicated by cholangiosepsis, cholangitic liver abscesses, acalculous cholecystitis, or gallbladder perforation. SSC-CIP was associated with worse prognosis; transplant-free survival was ∼40 months (mean).Because of its high rate of serious complications and unfavorable prognosis, it is imperative to diagnose SSC-CIP early and to differentiate SSC-CIP from other types of sclerosing cholangitis. Specific characteristics enable identification of SSC-CIP. Early cooperation with a transplant center and special attention to biliary complications are required after diagnosis of SSC-CIP.

  15. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.

    Science.gov (United States)

    Knobler, R; Moinzadeh, P; Hunzelmann, N; Kreuter, A; Cozzio, A; Mouthon, L; Cutolo, M; Rongioletti, F; Denton, C P; Rudnicka, L; Frasin, L A; Smith, V; Gabrielli, A; Aberer, E; Bagot, M; Bali, G; Bouaziz, J; Braae Olesen, A; Foeldvari, I; Frances, C; Jalili, A; Just, U; Kähäri, V; Kárpáti, S; Kofoed, K; Krasowska, D; Olszewska, M; Orteu, C; Panelius, J; Parodi, A; Petit, A; Quaglino, P; Ranki, A; Sanchez Schmidt, J M; Seneschal, J; Skrok, A; Sticherling, M; Sunderkötter, C; Taieb, A; Tanew, A; Wolf, P; Worm, M; Wutte, N J; Krieg, T

    2017-09-01

    The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum. © 2017 European Academy of Dermatology and Venereology.

  16. Mesenteric ossification in CT indicates sclerosing peritonitis in chronic bacterial infection and pancreatitis; Mesenteriale Verknoecherungen als computertomographische Zeichen einer sklerosierenden Peritonitis bei chronischer Bauchfellentzuendung und Pankreatitis

    Energy Technology Data Exchange (ETDEWEB)

    Kirchner, J.; Kirchner, E.M. [Abt. fuer Diagnostische und Interventionelle Radiologie, Klinikum Niederberg Velbert (Germany); Kickuth, R. [Klinik fuer Radiologie und Nuklearmedizin, Katholisches Marienhospital Herne, Universitaetsklinik der Ruhr Univ. Bochum (Germany); Stein, A. [Klinik fuer Strahlentherapie und Onkologie, Universitaetsklinikum Frankfurt/Main (Germany)

    2004-07-01

    Sclerosing peritonitis already has been described as a serious complication of the continuous ambulatory peritoneal dialysis. But different other affections of the pertioneum such as chronic bacterial peritonitis and pancreatitis may result in sclerosing peritonitis, too. The symptom is characterised by thickened small bowel walls and periotoneal membranes as well as peritoneal calcifications which can be shown in computed tomography. We demonstrate two cases of peritoneal ossifications due to peritonitis and pancreatitis. (orig.) [German] Die sklerosierende Peritonitis wurde wiederholt als ernste Komplikation der Bauchfelldialyse beschrieben. Aber auch verschiedene andere Reizzustaende wie chronische bakterielle Peritonitis und Pankreatitis koennen eine sklerosierende Peritonitis nach sich ziehen. Hierbei zeigen sich neben einer Verdickung des Peritoneums und der Waende des Duenndarms auch computertomographisch nachweisbare Kalkeinlagerungen. Wir stellen zwei Patienten mit chronischer Peritonitis nach Sigmaperforation und Pankreatitis mit Verknoecherungen des Peritoneums vor. (orig.)

  17. Celiac Disease, Enteropathy-Associated T-Cell Lymphoma, and Primary Sclerosing Cholangitis in One Patient: A Very Rare Association and Review of the Literature

    Directory of Open Access Journals (Sweden)

    N. Majid

    2013-01-01

    Full Text Available Enteropathy-associated T-cell lymphoma (EATL is a very rare peripheral T-cell lymphoma which is mostly associated with celiac disease. However, the association of primary sclerosing cholangitis and enteropathy-associated T-cell lymphoma is uncommon. Herein we report and discuss the first case of patient who presented simultaneously with these two rare diseases. It is a 54-year-old man who stopped gluten-free diet after 15 years history of celiac disease. The diagnosis was based on the histological examination of duodenal biopsy and the diagnosis of primary sclerosing cholangitis was made on liver biopsy, as well as the magnetic resonance cholangiogram. The treatment of EATL is mainly based on chemotherapy in addition to the optimal management of complications and adverse events that impact on the response to treatment and clinical outcomes, although the prognosis remains remarkably very poor.

  18. IgG4-related retroperitoneal fibrosis and sclerosing cholangitis independent of autoimmune pancreatitis. A recurrent case after a 5-year history of spontaneous remission.

    Science.gov (United States)

    Miura, Hideaki; Miyachi, Yasutaka

    2009-07-06

    A new clinicopathological concept of IgG4-related sclerosing disease affecting various organs has recently been proposed in relation to autoimmune pancreatitis. This report describes the case of IgG4-related retroperitoneal fibrosis and sclerosing cholangitis independent of autoimmune pancreatitis, which recurred after a long period of spontaneous remission. An 80-year-old Japanese man presented with obstructive jaundice owing to a hepatic hilum bile duct stricture. Coincidentally, a soft tissue mass surrounding the abdominal aorta, suggesting retroperitoneal fibrosis, was identified. Unexpectedly, spontaneous regression of obstructive jaundice together with retroperitoneal fibrosis occurred. The presence of high serum IgG4 concentrations measured later led us to consider a possible association with autoimmune pancreatitis; however, there were no clinical features confirming autoimmune pancreatitis. After a 5-year history of spontaneous clinical remission, there was an elevation of serum IgG4 levels and renal dysfunction owing to bilateral hydronephrosis caused by a reemergence of the retroperitoneal mass. Evaluation by endoscopic retrograde cholangiopancreatography revealed a biliary stricture, suggesting sclerosing cholangitis which was observed without the presence of any pancreatic duct abnormality. The subsequent excellent results obtained using steroid therapy, namely the decrease in serum IgG4 levels and the regression of the retroperitoneal mass, strongly suggested that the present case was an IgG4-related sclerosing disease. Aside from high serum IgG4 concentrations, markedly elevated levels of serum IgE was found retrospectively, although the clinical significance remains unknown. When we encounter fibrotic diseases of unknown etiology, we should measure serum IgG4 concentrations and monitor the disease activity over long periods even after achieving clinical remission.

  19. Chitosan–Sodium Tetradecyl Sulfate Hydrogel: Characterization and Preclinical Evaluation of a Novel Sclerosing Embolizing Agent for the Treatment of Endoleaks

    Energy Technology Data Exchange (ETDEWEB)

    Zehtabi, Fatemeh [École de technologie supérieure, Department of Mechanical Engineering (Canada); Dumont-Mackay, Vincent [Centre hospitalier de l’Université de Montréal, Département de pathologie (Canada); Fatimi, Ahmed [École de technologie supérieure, Department of Mechanical Engineering (Canada); Bertrand-Grenier, Antony; Héon, Hélène; Soulez, Gilles [Centre Hospitalier de l’Université de Montréal (CRCHUM), Research Centre (Canada); Lerouge, Sophie, E-mail: Sophie.lerouge@etsmtl.ca [École de technologie supérieure, Department of Mechanical Engineering (Canada)

    2017-04-15

    PurposeTo compare the efficacy of an embolization agent with sclerosing properties (made of chitosan and sodium tetradecyl sulfate, CH–STS) with a similar embolization agent but without sclerosing properties (made of chitosan, CH) in treating endoleaks in a canine endovascular aneurysm repair model.MethodsTwo chitosan-based radiopaque hydrogels were prepared, one with STS and one without STS. Their rheological, injectability, and embolizing properties were assessed in vitro; afterwards, their efficacy in occluding endoleaks was compared in a canine bilateral aneurysm model reproducing type I endoleaks (n = 9 each). The primary endpoint was endoleak persistence at 3 or 6 months, assessed on a CT scan and macroscopic examination. Secondary endpoints were the occurrence of stent-graft (SG) thrombosis, the evolution of the aneurysm mean diameter, as well as aneurysm healing and inflammation scores in pathology examinations.ResultsIn vitro experiments showed that both products gelled rapidly and presented initial storage moduli greater than 800 Pa, which increased with time. Both gels were compatible with microcatheter injection and occlude flow up to physiological pressure in vitro. In a type I endoleak model, the injection of CH–STS sclerosing gel tended to reduce the risk of occurrence of endoleaks, compared to CH non-sclerosing agent (2/9 vs. 6/9, p = 0.069). No case of SG thrombosis was observed. Moderate inflammation was found around both gels, with a comparable intensity score in both CH and CH–STS groups (2.6 ± 0.9 and 2.7 ± 0.9, respectively; p = 0.789).ConclusionsFlow occlusion combined with chemical endothelial denudation appears promising for the treatment of endoleaks.Level of EvidenceN/A.

  20. No negative impact of serum IgG4 levels on clinical outcome in 435 patients with primary sclerosing cholangitis from Japan.

    Science.gov (United States)

    Tanaka, Atsushi; Tazuma, Susumu; Nakazawa, Takahiro; Isayama, Hiroyuki; Tsuyuguchi, Toshio; Inui, Kazuo; Takikawa, Hajime

    2017-04-01

    Several studies have demonstrated that elevated serum IgG4 levels are associated with poor outcomes of primary sclerosing cholangitis (PSC), but the impact of serum IgG4 levels on PSC remains controversial. In this study, we aimed to determine prognostic factors of patients with PSC and to investigate the association between serum IgG4 levels and the clinical features and prognosis of PSC in a Japanese cohort. We retrospectively analyzed follow-up data for 435 patients with PSC (UMIN000018438). Patients with distinct etiologies of sclerosing cholangitis including IgG4-related sclerosing cholangitis (IgG4-SC) were excluded from this study. Serum IgG4 levels were tested at the time of diagnosis in 216 of 435 patients with PSC, and were elevated in 27 patients (>134 mg/dl, 12.5%). Clinical features at diagnosis were comparable between patients with normal and elevated serum IgG4 levels, with the exception of serum albumin. The overall and liver-transplantation free survival rate was comparable between the groups. Multivariate analysis indicated that age, albumin, and bilirubin, but not IgG4, at the time of diagnosis affected PSC prognosis. The current study showed that serum IgG4 levels at diagnosis do not affect PSC prognosis in a Japanese cohort that excluded patients with IgG4-SC. © 2017 Japanese Society of Hepato-Biliary-Pancreatic Surgery.

  1. Inhibition of intestinal bile acid absorption improves cholestatic liver and bile duct injury in a mouse model of sclerosing cholangitis.

    Science.gov (United States)

    Baghdasaryan, Anna; Fuchs, Claudia D; Österreicher, Christoph H; Lemberger, Ursula J; Halilbasic, Emina; Påhlman, Ingrid; Graffner, Hans; Krones, Elisabeth; Fickert, Peter; Wahlström, Annika; Ståhlman, Marcus; Paumgartner, Gustav; Marschall, Hanns-Ulrich; Trauner, Michael

    2016-03-01

    Approximately 95% of bile acids (BAs) excreted into bile are reabsorbed in the gut and circulate back to the liver for further biliary secretion. Therefore, pharmacological inhibition of the ileal apical sodium-dependent BA transporter (ASBT/SLC10A2) may protect against BA-mediated cholestatic liver and bile duct injury. Eight week old Mdr2(-/-) (Abcb4(-/-)) mice (model of cholestatic liver injury and sclerosing cholangitis) received either a diet supplemented with A4250 (0.01% w/w) - a highly potent and selective ASBT inhibitor - or a chow diet. Liver injury was assessed biochemically and histologically after 4weeks of A4250 treatment. Expression profiles of genes involved in BA homeostasis, inflammation and fibrosis were assessed via RT-PCR from liver and ileum homogenates. Intestinal inflammation was assessed by RNA expression profiling and immunohistochemistry. Bile flow and composition, as well as biliary and fecal BA profiles were analyzed after 1week of ASBT inhibitor feeding. A4250 improved sclerosing cholangitis in Mdr2(-/-) mice and significantly reduced serum alanine aminotransferase, alkaline phosphatase and BAs levels, hepatic expression of pro-inflammatory (Tnf-α, Vcam1, Mcp-1) and pro-fibrogenic (Col1a1, Col1a2) genes and bile duct proliferation (mRNA and immunohistochemistry for cytokeratin 19 (CK19)). Furthermore, A4250 significantly reduced bile flow and biliary BA output, which correlated with reduced Bsep transcription, while Ntcp and Cyp7a1 were induced. Importantly A4250 significantly reduced biliary BA secretion but preserved HCO3(-) and biliary phospholipid secretion resulting in an increased HCO3(-)/BA and PL/BA ratio. In addition, A4250 profoundly increased fecal BA excretion without causing diarrhea and altered BA pool composition, resulting in diminished concentrations of primary BAs tauro-β-muricholic acid and taurocholic acid. Pharmacological ASBT inhibition attenuates cholestatic liver and bile duct injury by reducing biliary BA

  2. Measles Virus: Identification in the M Protein Primary Sequence of a Potential Molecular Marker for Subacute Sclerosing Panencephalitis

    Directory of Open Access Journals (Sweden)

    Hasan Kweder

    2015-01-01

    Full Text Available Subacute Sclerosing Panencephalitis (SSPE, a rare lethal disease of children and young adults due to persistence of measles virus (MeV in the brain, is caused by wild type (wt MeV. Why MeV vaccine strains never cause SSPE is completely unknown. Hypothesizing that this phenotypic difference could potentially be represented by a molecular marker, we compared glycoprotein and matrix (M genes from SSPE cases with those from the Moraten vaccine strain, searching for differential structural motifs. We observed that all known SSPE viruses have residues P64, E89, and A209 (PEA in their M proteins whereas the equivalent residues for vaccine strains are either S64, K89, and T209 (SKT as in Moraten or PKT. Through the construction of MeV recombinants, we have obtained evidence that the wt MeV-M protein PEA motif, in particular A209, is linked to increased viral spread. Importantly, for the 10 wt genotypes (of 23 that have had their M proteins sequenced, 9 have the PEA motif, the exception being B3, which has PET. Interestingly, cases of SSPE caused by genotype B3 have yet to be reported. In conclusion, our results strongly suggest that the PEA motif is a molecular marker for wt MeV at risk to cause SSPE.

  3. Ursodeoxycholic acid in patients with ulcerative colitis and primary sclerosing cholangitis for prevention of colon cancer: a meta-analysis.

    Science.gov (United States)

    Ashraf, Imran; Choudhary, Abhishek; Arif, Murtaza; Matteson, Michelle L; Hammad, Hazem T; Puli, Srinivas R; Bechtold, Matthew L

    2012-04-01

    Colon cancer risk is high in patients with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC). Ursodeoxycholic acid has been shown to have some promise as a chemopreventive agent. A meta-analysis was performed to compare the efficacy of ursodeoxycholic acid in the prevention of colonic neoplasia in patients with UC and PSC. Multiple databases were searched (January 2011). Studies examining the use of ursodeoxycholic acid vs. no ursodeoxycholic acid or placebo in adult patients with UC and PSC were included. Data were extracted in standard forms by two independent reviewers. Meta-analysis for the effect of ursodeoxycholic acid was performed by calculating pooled estimates of adenoma or colon cancer formation by odds ratio (OR) with random effects model. Heterogeneity was assessed by calculating the I (2) measure of inconsistency. RevMan 5 was utilized for statistical analysis. Four studies (n = 281) met the inclusion criteria. The studies were of adequate quality. Ursodeoxycholic acid demonstrated no overall improvement in adenoma (OR 0.53; 95 % CI: 0.19-1.48, p = 0.23) or colon cancer occurrence (OR 0.50; 95 % CI: 0.18-1.43, p = 0.20) as compared to no ursodeoxycholic acid or placebo in patients with UC and PSC. Ursodeoxycholic acid use in patients with UC and PSC does not appear to decrease the risk of adenomas or colon cancer.

  4. Bile acid changes after high-dose ursodeoxycholic acid treatment in primary sclerosing cholangitis: relation to disease progression

    Science.gov (United States)

    Sinakos, Emmanouil; Marschall, Hanns-Ulrich; Kowdley, Kris V.; Befeler, Alex; Keach, Jill; Lindor, Keith

    2010-01-01

    High-dose (28-30mg/kg/day) ursodeoxycholic acid (UDCA) treatment improves serum liver tests in patients with primary sclerosing cholangitis (PSC) but does not improve survival and is associated with increased rates of serious adverse events. The mechanism for the latter undesired effect remains unclear. High-dose UDCA could result in the production of hepatotoxic bile acids, such as lithocholic acid (LCA), due to limited small bowel absorption of UDCA and conversion of UDCA by bacteria in the colon. We determined the serum bile acid composition in 56 patients with PSC previously enrolled in a randomized, double-blind controlled trial of high dose UDCA versus placebo. Samples for analysis were obtained at baseline and at the end of treatment. The mean changes in UDCA (16.86 vs 0.05 μmol/L) and total bile acid (17.21 vs −0.55 μmol/L) levels were significantly higher in the UDCA group (n=29) compared to placebo (n=27) when pretreatment levels were compared (pacid (CA), deoxycholic acid (DCA) and chenodeoxycholic acid (CDCA). Patients (n=9) in the UDCA group who reached clinical endpoints of disease progression (development of cirrhosis, varices, liver transplantation or death) tend to have greater increase in their post-treatment total bile acid levels (34.99 vs 9.21 μmol/L) (pacid pool including lithocholic acid. PMID:20564380

  5. Analysis of MxA, IL-4, and IRF-1 genes in Filipino patients with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Pipo-Deveza, J R; Kusuhara, K; Silao, C L T; Lukban, M B; Salonga, A M; Sanchez, B C; Kira, R; Takemoto, M; Torisu, H; Hara, T

    2006-08-01

    Subacute sclerosing panencephalitis (SSPE) is a chronic and debilitating disease of the central nervous system caused by a latent measles virus infection. Three candidate genes, MxA, IL-4, and IRF-1 genes were shown to be associated with SSPE in Japanese patients. These genes have been suggested to play a role in the establishment of persistent viral infection in the central nervous system. Sixty Filipino SSPE patients and 120 healthy control subjects were included in the study. Single nucleotide polymorphisms at promoter regions ( IL-4-590C/T and MXA-88G/T) were screened using PCR-RFLP method. Genotyping was done for GT repeat polymorphism within intron 7 of IRF-1. The TT genotype of MXA, as well as the CT genotype of IL-4, were seen a little more frequently among the SSPE patients as compared to the control subjects. The values though, did not reach statistical significance. IRF-1 analysis did not differ between the two groups. Our study failed to demonstrate a significant association between IL-4, MXA, or IRF-1, and SSPE in the Filipino population. Our results might be explained by a greater contribution of environmental factors such as the socio-economic and nutritional factors in the susceptibility of Filipinos to SSPE other than genetic factors.

  6. Detection of Cholangiocarcinoma with Magnetic Resonance Spectroscopy of Bile in Patients with and without Primary Sclerosing Cholangitis

    International Nuclear Information System (INIS)

    Albiin, N.; Smith, I.C.P.; Arnelo, U.; Lindberg, B.; Bergquist, A.; Dolenko, B.; Bryksina, N.; Bezabeh, T.

    2008-01-01

    Background: Early detection of cholangiocarcinoma (CC) is very difficult, especially in patients with primary sclerosing cholangitis (PSC) who are at increased risk of developing CC. Purpose: To evaluate 1 H magnetic resonance spectroscopy ( 1 H-MRS) of bile as a diagnostic marker for CC in patients with and without PSC. Material and Methods: The institutional review board approved the study, and all patients gave informed consent. Bile from 49 patients was sampled and investigated using 1 H-MRS. MR spectra of bile samples from 45 patients (18 female; age range 22-87 years, mean age 57 years) were analyzed both conventionally and using computerized multivariate analysis. Sixteen of the patients had CC, 18 had PSC, and 11 had other benign findings. Results: The spectra of bile from CC patients differed from the benign group in the levels of phosphatidylcholine, bile acids, lipid, and cholesterol. It was possible to distinguish CC from benign conditions in all patients with malignancy. Two benign non-PSC patients were misclassified as malignant. The sensitivity, specificity, and accuracy were 88.9%, 87.1%, and 87.8%, respectively. Conclusion: With 1 H-MRS of bile, cholangiocarcinoma could be discriminated from benign biliary conditions with or without PSC

  7. Diffuse sclerosing variant of papillary carcinoma of the thyroid. Clinical importance, surgical treatment, and follow-up study

    Energy Technology Data Exchange (ETDEWEB)

    Fujimoto, Y.; Obara, T.; Ito, Y.; Kodama, T.; Aiba, M.; Yamaguchi, K. (Tokyo Women' s Medical College (Japan))

    1990-12-01

    A diffuse sclerosing variant is not very rare among papillary carcinomas of the thyroid when the patients are female and younger than 30 years of age. The variant is characterized by diffuse involvement of one or both thyroid lobes, with dense sclerosis, patchy lymphocytic infiltration, and abundant psammoma bodies. Controversy still exists concerning its prognosis. We reviewed our experience with 14 patients treated between 1958 and 1988. All patients were young females, their age being from 10 to 28 years with a mean of 19.6. Hashimoto's thyroiditis had been suspected in nine patients before they came to our clinic. Nowadays the diagnosis of this cancer is possible when we have this entity in mind and detect abundant psammoma bodies either by ultrasonography or by soft-tissue roentgenography of the neck. Total thyroidectomy with modified neck dissection was carried out in eight patients, subtotal thyroidectomy with neck dissection in five, and lobectomy with neck dissection in one. All of them are alive and well without distant metastasis at a mean follow-up of 16 years. Because most of the patients with this variant of papillary carcinoma are young women and the prognosis is favorable, a complete resection without causing later recurrence, but also cosmetic and complication-free surgery, should be considered.

  8. The Response of RIF-1 Fibrosarcomas to the Vascular-Disrupting Agent ZD6126 Assessed by In Vivo and Ex Vivo1H Magnetic Resonance Spectroscopy

    Directory of Open Access Journals (Sweden)

    Basetti Madhu

    2006-07-01

    Full Text Available The response of radiation-induced fibrosarcoma1 (RIF-1 tumors treated with the vascular-disrupting agent (VDA ZD6126 was assessed by in vivo and ex vivo1H magnetic resonance spectroscopy (MRS methods. Tumors treated with 200 mg/kg ZD6126 showed a significant reduction in total choline (tCho in vivo 24 hours after treatment, whereas control tumors showed a significant increase in tCho. This response was investigated further within both ex vivo unprocessed tumor tissues and tumor tissue metabolite extracts. Ex vivo high-resolution magic angle spinning (HRMAS and 1H MRS of metabolite extracts revealed a significant reduction in phosphocholine and glycerophosphocholine in biopsies of ZD6126-treated tumors, confirming in vivo tCho response. ZD6126-induced reduction in choline compounds is consistent with a reduction in cell membrane turnover associated with necrosis and cell death following disruption of the tumor vasculature. In vivo tumor tissue water diffusion and lactate measurements showed no significant changes in response to ZD6126. Spin-spin relaxation times (T2 of water and metabolites also remained unchanged. Noninvasive 1H MRS measurement of tCho in vivo provides a potential biomarker of tumor response to VDAs in RIF-1 tumors.

  9. T Cell Intrinsic USP15 Deficiency Promotes Excessive IFN-γ Production and an Immunosuppressive Tumor Microenvironment in MCA-Induced Fibrosarcoma

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    Qiang Zou

    2015-12-01

    Full Text Available USP15 is a deubiquitinase that negatively regulates activation of naive CD4+ T cells and generation of IFN-γ-producing T helper 1 (Th1 cells. USP15 deficiency in mice promotes antitumor T cell responses in a transplantable cancer model; however, it has remained unclear how deregulated T cell activation impacts primary tumor development during the prolonged interplay between tumors and the immune system. Here, we find that the USP15-deficient mice are hypersensitive to methylcholantrene (MCA-induced fibrosarcomas. Excessive IFN-γ production in USP15-deficient mice promotes expression of the immunosuppressive molecule PD-L1 and the chemokine CXCL12, causing accumulation of T-bet+ regulatory T cells and CD11b+Gr-1+ myeloid-derived suppressor cells at tumor site. Mixed bone marrow adoptive transfer studies further reveals a T cell-intrinsic role for USP15 in regulating IFN-γ production and tumor development. These findings suggest that T cell intrinsic USP15 deficiency causes excessive production of IFN-γ, which promotes an immunosuppressive tumor microenvironment during MCA-induced primary tumorigenesis.

  10. PATHOMORPHOLOGICAL FEATURES OF BONE LESIONS AND CORRELATION OF CLINICAL, LABORATORY AND MORPHOMETRIC CRITERIA IN PATIENTS WITH LATENT SCLEROSING HEMATOGENOUS OSTEOMYELITIS (GARRÉ

    Directory of Open Access Journals (Sweden)

    V. V. Grigorovsky

    2018-01-01

    Full Text Available Introduction. Sclerosing hematogenous osteomyelitis of Garré (SHO holds a significant place among cases of latent hematogenous osteomyelitis. Pathomorphological studies of sclerosing hematogenous osteomyelitis are needed to improve differentiated diagnostics, to prognosticate morphology specifics of nidus and to choose the optimal treatment tactics.Purpose of the study — to identify statistical differences between manifestation patterns of various disease types and correlation between clinical, laboratorial and morphometric criteria of bone lesions in patients with sclerosing hematogenous osteomyelitis.Material and methods. The authors studied tissue fragments of affected bones of 25 patients with SHO which was diagnosed by clinical, laboratorial, visualizing and morphological methods. Gradation morphometric criteria were used to reflect condition of nidus tissues. The authors made the analysis of qualitative characters and correlation analysis of dependencies between clinical and laboratorial criteria on the one hand, and with morphometric criteria — on the other, and identified association factor. Results. Pathomorphological study of SHO nidus demonstrated variances of bone lesions in separate disease cases despite the overall similarity of morphological manifestation. About 2/3 of all cases correspond to fibrosing type whereby even small exudative inflammation sites are absent. In about 1/3 of all cases apart from fibrosis, osteosclerosis and remodeling, the osteomyelitis niduses contain microabscesses indicative of suppurative inflammation as well as the authors observed small sequestration. The maximum differences in patients with various types of SHO were identified in such parameters as share of stab microphages and erythrocyte sedimentation rate (ESR. In cases of long term morbidity the fibrosing disease type is prevailing, in cases of short term lesion (1–2 years — a fibrosing type with microabscesses formation.A series of

  11. Fibrosis of the thyroid gland caused by an IgG4-related sclerosing disease: three years of follow-up.

    Science.gov (United States)

    Oriot, P; Amraoui, A; Rousseau, E; Malvaux, P; Dechambre, S; Delcourt, A

    2014-12-01

    Immunoglobulin G4-related sclerosing disease (IgG4-RSD) represents a recently identified inflammatory disorder in which infiltration of IgG4 plasma cells causes fibrosis in organs. While IgG4-RSD is well documented in the pancreas and other organs, it is poorly characterized in the thyroid gland. We report a case of a 48-year-old female with a fibrotic thyroid mass associated with a retroperitoneal fibrosis. Diagnosed early as Riedel disease, the high serum IgG4, immunohistopathology and decreased fibrosis with corticosteroid therapy, finally confirm for the first time, the origin of IgG4-RSD fibrosis of the thyroid.

  12. Treatment of Advanced Malignant Uterine Perivascular Epithelioid Cell Tumor with mTOR Inhibitors: Single-institution Experience and Review of the Literature.

    Science.gov (United States)

    Starbuck, Kristen D; Drake, Richard D; Budd, G Thomas; Rose, Peter G

    2016-11-01

    Uterine perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Many have malignant behavior, and no successful treatment strategy has been established. Identification of mutations in the tuberous sclerosis 1 (TSC1) and TSC2 genes producing constitutive activation of the mammalian target of rapamycin (mTOR) pathway presents an opportunity for targeted therapy. Patients with advanced malignant uterine PEComa treated with mTOR inhibitors were identified and records were retrospectively reviewed for treatment response based on radiographic assessment. Three patients with advanced uterine PEComas underwent debulking surgery followed by mTOR inhibitor therapy; two had a complete response to therapy and disease in one patient progressed. Given the absence of effective therapies for malignant uterine PEComas, targeting the mTOR pathway is a logical strategy to pursue given the known pathobiology involving the Tuberous Sclerosis complex. Treatment of malignant uterine PEComas with mTOR inhibitors was effective in two out of three patients after surgical resection, with durable response. Copyright© 2016 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

  13. Radiotherapy With or Without Surgery for Patients With Idiopathic Sclerosing Orbital Inflammation Refractory or Intolerant to Steroid Therapy

    International Nuclear Information System (INIS)

    Lee, Jong Hoon; Kim, Yeon-Sil; Yang, Suk Woo; Cho, Won-Kyung; Lee, Sang Nam; Lee, Kyung Ji; Ryu, Mi-Ryeong; Jang, Hong Seok

    2012-01-01

    Purpose: To evaluate the outcomes of patients with idiopathic sclerosing orbital inflammation (ISOI) treated with radiotherapy with or without surgery. Methods and Materials: We retrospectively reviewed 22 patients with histopathologically confirmed ISOI who had been refractory or intolerant to steroid therapy and treated with radiation with or without surgery. The radiation dose ranged from 20 to 40 Gy (median, 20 Gy) at 2 Gy per fraction. Presenting signs and treatment outcomes were assessed. Results: Proptosis was the most common sign at presentation, seen in 19 (86.3%) patients, followed by restriction of extraocular movements in 10 (45.4%) patients. Response to radiotherapy was complete in 15 (68.1%) patients, partial in 3 (13.6%) patients, and none in 4 (18.2%) patients. At the median follow-up of 34 months, 14 (63.6%) patients had progression-free state of symptoms and signs, with the progression-free duration ranging from 3 to 75 months (median, 41.5 months), whereas 8 (36.4%) patients had recurrent or persistent disease although they had received radiotherapy. Of the 14 progression-free patients, 6 underwent a bimodality treatment of debulking surgery of ocular disease and radiotherapy. They had had no recurrent disease. Cataract was the most common late complications, and 2 patients experienced a Grade 3 cataract. Conclusion: Our study suggests that for patients with ISOI who are refractory or intolerant to steroid therapy, 20 Gy of radiotherapy appears to be effective for the control of disease with acceptable complications, especially when it is combined with surgery.

  14. Prospective evaluation of PBC-specific health-related quality of life questionnaires in patients with primary sclerosing cholangitis.

    Science.gov (United States)

    Raszeja-Wyszomirska, Joanna; Wunsch, Ewa; Krawczyk, Marek; Rigopoulou, Eirini I; Bogdanos, Dimitrios; Milkiewicz, Piotr

    2015-06-01

    Primary biliary cirrhosis and Primary sclerosing cholangitis are autoimmune cholestatic liver diseases sharing a lot in common, including a significant impairment of patients' health-related quality of life HRQoL HRQoL in PBC is assessed with disease-specific PBC-40 and PBC-27 questionnaires. A PSC-specific questionnaire has not been developed. Neither PBC-40 nor PBC-27s applicability for PSC has been evaluated. We applied these three questionnaires for HRQoL assessment in a large homogenous cohort of PSC patients. This cross-sectional study enrolled 102 Caucasian PSCs and 53 matched healthy controls and measured HRQoL using generic SF-36, and disease-specific (PBC-40/PBC-27) questionnaires. (i) SF-36. Most SF-36 domains were significantly lower in PSCs than controls. Physical Functioning and Mental Component Summary scores were significantly lower in female patients and correlated negatively with age but not with concurrent inflammatory bowel disease. Cirrhosis was associated with lower Physical Functioning, Role Physical, General Health, Vitality and Physical Component Summary. (ii) PBC-40 and PBC-27. Both tools showed similar HRQoL impairment scoring. Fatigue and Cognitive were impaired in female patients. Several correlations existed between HRQoL and laboratory parameters, including cholestatic tests and Itch. Cirrhosis correlated with Other symptoms and Fatigue PBC-40. (iii) PBC-40 vs PBC-27. Strong correlations among most domains of both questionnaires were seen, as well as between (iv) SF-36 vs PBC-40 or SF-36 vs PBC-27. This is the first study directly comparing PBC-40, PBC-27 and SF-36 in PSC. PSC patients, especially females, show HRQoL impairment. PBC-40 and PBC-27 questionnaires could be of potential use for HRQoL assessment in PSC. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  15. Development and validation of a primary sclerosing cholangitis-specific patient-reported outcomes instrument: The PSC PRO.

    Science.gov (United States)

    Younossi, Zobair M; Afendy, Arian; Stepanova, Maria; Racila, Andrei; Nader, Fatema; Gomel, Rachel; Safer, Ricky; Lenderking, William R; Skalicky, Anne; Kleinman, Leah; Myers, Robert P; Subramanian, G Mani; McHutchison, John G; Levy, Cynthia; Bowlus, Christopher L; Kowdley, Kris; Muir, Andrew J

    2017-11-20

    Primary sclerosing cholangitis (PSC) is a chronic liver disease associated with inflammation and biliary fibrosis that leads to cholangitis, cirrhosis, and impaired quality of life. Our objective was to develop and validate a PSC-specific patient-reported outcome (PRO) instrument. We developed a 42-item PSC PRO instrument that contains two modules (Symptoms and Impact of Symptoms) and conducted an external validation. Reliability and validity were evaluated using clinical data and a battery of other validated instruments. Test-retest reliability was assessed in a subgroup of patients who repeated the PSC PRO after the first administration. One hundred two PSC subjects (44 ± 13 years; 32% male, 74% employed, 39% with cirrhosis, 14% with a history of decompensated cirrhosis, 38% history of depression, and 68% with inflammatory bowel disease [IBD]) completed PSC PRO and other PRO instruments (Short Form 36 V2 [SF-36], Chronic Liver Disease Questionnaire [CLDQ], Primary Biliary Cholangitis - 40 [PBC-40], and five dimensions [5-D Itch]). PSC PRO demonstrated excellent internal consistency (Cronbach alphas, 0.84-0.94) and discriminant validity (41 of 42 items had the highest correlations with their own domains). There were good correlations between PSC PRO domains and relevant domains of SF-36, CLDQ, and PBC-40 (R = 0.69-0.90; all P 0.05). Test-retest reliability was assessed in 53 subjects who repeated PSC PRO within a median (interquartile range) of 37 (27-47) days. There was excellent reliability for most domains with intraclass correlations (0.71-0.88; all P < 0.001). PSC PRO is a self-administered disease-specific instrument developed according to U.S. Food and Drug Administration guidelines. This preliminary validation study suggests good psychometric properties. Further validation of the instrument in a larger and more diverse sample of PSC patients is needed. (Hepatology 2017). © 2017 by the American Association for the Study of Liver Diseases.

  16. Elevation of serum IgG4 in Western patients with autoimmune sclerosing pancreatocholangitis: a word of caution.

    Science.gov (United States)

    Hochwald, Steven N; Hemming, Alan W; Draganov, Peter; Vogel, Stephen B; Dixon, Lisa R; Grobmyer, Stephen R

    2008-04-01

    Autoimmune pancreatocholangitis is characterized by sclerosing inflammation of the biliary tree or pancreatic duct and can mimic pancreaticobiliary malignancy. Serum immunoglobin (Ig) G4 values seem to be helpful in distinguishing autoimmune pancreatocholangitis from pancreatic malignancy in the Japanese population; however, its significance in the Western population has not been well studied. We report a retrospective analysis of 7 consecutive patients with autoimmune pancreatocholangitis and compare them to 23 patients with pancreatic malignancy. Clinical presentation, diagnostic tests, and preoperative IgG4 levels were reviewed in all patients. Presence of autoimmune pancreatocholangitis or pancreatic malignancy was determined by pathologic analysis in all patients and reviewed by a single pathologist. In all patients, autoimmune pancreatocholangitis manifested in a similar fashion to pancreatic malignancy. Median IgG4 levels were far lower in pancreatic cancer patients with localized, resectable disease (24 mg/dL), locally advanced disease (24 mg/dL), and metastatic disease (28 mg/dL) as compared with patients with autoimmune pancreatocholangitis (142 mg/dL, P 100 mg/dL. In contrast, all patients with autoimmune pancreatitis or cholangitis had levels >100 mg/dL. However, in five of these seven patients, IgG4 levels were below the upper limits of normal. Autoimmune pancreatocholangitis mimics pancreatobiliary malignancy. Serum IgG4 values seem to be helpful in distinguishing autoimmune pancreatocholangitis from malignancy in the Western population. However, absolute values seem to be lower in the United States compared with Japan. The upper limit of normal as reported in laboratories in the United States may not be useful in identifying abnormally high IgG4 values. A new upper limit of normal may need to be defined because IgG subclass determinations are being used more frequently in Western patients with biliary obstruction.

  17. MicroRNAs in Serum and Bile of Patients with Primary Sclerosing Cholangitis and/or Cholangiocarcinoma.

    Science.gov (United States)

    Voigtländer, Torsten; Gupta, Shashi K; Thum, Sabrina; Fendrich, Jasmin; Manns, Michael P; Lankisch, Tim O; Thum, Thomas

    2015-01-01

    Patients with primary sclerosing cholangitis (PSC) are at high risk for the development of cholangiocarcinoma (CC). Analysis of micro ribonucleic acid (MiRNA) patterns is an evolving research field in biliary pathophysiology with potential value in diagnosis and therapy. Our aim was to evaluate miRNA patterns in serum and bile of patients with PSC and/or CC. Serum and bile from consecutive patients with PSC (n = 40 (serum), n = 52 (bile)), CC (n = 31 (serum), n = 19 (bile)) and patients with CC complicating PSC (PSC/CC) (n = 12 (bile)) were analyzed in a cross-sectional study between 2009 and 2012. As additional control serum samples from healthy individuals were analyzed (n = 12). The miRNA levels in serum and bile were determined with global miRNA profiling and subsequent miRNA-specific polymerase chain reaction-mediated validation. Serum analysis revealed significant differences for miR-1281 (p = 0.001), miR-126 (p = 0.001), miR-26a (p = 0.001), miR-30b (p = 0.001) and miR-122 (p = 0.034) between patients with PSC and patients with CC. All validated miRNAs were significantly lower in healthy individuals. MiR-412 (p = 0.001), miR-640 (p = 0.001), miR-1537 (p = 0.003) and miR-3189 (p = 0.001) were significantly different between patients with PSC and PSC/CC in bile. Patients with PSC and/or CC have distinct miRNA profiles in serum and bile. Furthermore, miRNA concentrations are different in bile of patients with CC on top of PSC indicating the potential diagnostic value of these miRNAs.

  18. IgG4-Related Sclerosing Disease, an Emerging Entity: A Review of a Multi-System Disease

    Science.gov (United States)

    Divatia, Mukul; Kim, Sun A

    2012-01-01

    Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recently defined emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. IgG4-RSD usually affects middle aged and elderly patients, with a male predominance. It is associated with an elevated serum titer of IgG4, which acts as a marker for this recently characterized entity. The prototype is IgG4-related sclerosing pancreatitis or autoimmune pancreatitis (AIP). Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, however practically any organ can be involved, including upper aerodigestive tract, lung, aorta, mediastinum, retroperitoneum, soft tissue, skin, central nervous system, breast, kidney, and prostate. Fever or constitutional symptoms usually do not comprise part of the clinical picture. Laboratory findings detected include raised serum globulin, IgG and IgG4. An association with autoantibody detection (such as antinuclear antibodies and rheumatoid factor) is seen in some cases. Steroid therapy comprises the mainstay of treatment. Disease progression with involvement of multiple organ-sites may be encountered in a subset of cases and may follow a relapsing-remitting course. The principal histopathologic findings in several extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, along with atrophy and destruction of tissues. Immunohistochemical staining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). IgG4-RSD may potentially be rarely associated with the development of lymphoma and carcinoma. However, the nature and pathogenesis of IgG4-RSD are yet to be fully elucidated and provide immense scope for further studies. PMID:22187229

  19. Primary Sclerosing Cholangitis Risk Estimate Tool (PREsTo) Predicts Outcomes in PSC: A Derivation & Validation Study Using Machine Learning.

    Science.gov (United States)

    Eaton, John E; Vesterhus, Mette; McCauley, Bryan M; Atkinson, Elizabeth J; Schlicht, Erik M; Juran, Brian D; Gossard, Andrea A; LaRusso, Nicholas F; Gores, Gregory J; Karlsen, Tom H; Lazaridis, Konstantinos N

    2018-05-09

    Improved methods are needed to risk stratify and predict outcomes in patients with primary sclerosing cholangitis (PSC). Therefore, we sought to derive and validate a new prediction model and compare its performance to existing surrogate markers. The model was derived using 509 subjects from a multicenter North American cohort and validated in an international multicenter cohort (n=278). Gradient boosting, a machine based learning technique, was used to create the model. The endpoint was hepatic decompensation (ascites, variceal hemorrhage or encephalopathy). Subjects with advanced PSC or cholangiocarcinoma at baseline were excluded. The PSC risk estimate tool (PREsTo) consists of 9 variables: bilirubin, albumin, serum alkaline phosphatase (SAP) times the upper limit of normal (ULN), platelets, AST, hemoglobin, sodium, patient age and the number of years since PSC was diagnosed. Validation in an independent cohort confirms PREsTo accurately predicts decompensation (C statistic 0.90, 95% confidence interval (CI) 0.84-0.95) and performed well compared to MELD score (C statistic 0.72, 95% CI 0.57-0.84), Mayo PSC risk score (C statistic 0.85, 95% CI 0.77-0.92) and SAP statistic 0.65, 95% CI 0.55-0.73). PREsTo continued to be accurate among individuals with a bilirubin statistic 0.90, 95% CI 0.82-0.96) and when the score was re-applied at a later course in the disease (C statistic 0.82, 95% CI 0.64-0.95). PREsTo accurately predicts hepatic decompensation in PSC and exceeds the performance among other widely available, noninvasive prognostic scoring systems. This article is protected by copyright. All rights reserved. © 2018 by the American Association for the Study of Liver Diseases.

  20. Derivation of transplantable 7,12-dimethylbenz[a]anthracene-induced chicken fibrosarcoma lines: differences in metastasizing properties and organ specificity

    International Nuclear Information System (INIS)

    Galton, J.E.; Xue, B.; Hochwald, G.M.; Thorbecke, G.J.

    1982-01-01

    Transplantable 7,12-dimethylbenz[a]anthracene-induced SC chicken fibrosarcoma (CHCT-NYU) lines were studied for their ability to grow in internal organs after iv injection (artificial metastases) into 1- to 3-week-old chickens. Some tumor lines were recently derived, whereas others were studied after many serial subcutaneous transplantations. Artificial metastases were seen in the stomach, pancreas, lungs, heart, and muscle, and occasionally in the kidneys and liver. Agammaglobulinemic recipients showed more extensive organ involvement than normal recipients of the same age. Whole-body ν-irradiation enhanced the incidence of artificial metastases, particularly in lungs. Antibody from the serum of a primary tumor-bearing host reduced the growth of the corresponding tumor in many organs. The metastatic pattern of line CHCT-NYU4 was a relatively stable property. However, intravenous transplantation of tumor cells from line CHCT-NYU4 taken from the liver, lungs, and pancreas of a single recipient established sublines with changes in organ specificity. After a few such serial transplants of liver-derived tumor, a line was derived that grew virtually in the liver alone. A subline with preference for growth in lungs was also obtained, but its ability to grow in the pancreas persisted. A pancreas-derived tumor line also grew in the liver and lungs. Subcutaneous transplants of tissue fragments of the lung-derived tumor line caused the appearance of spontaneous metastases in lungs. The incidence of spontaneous metastases with the lung-derived line was much greater than that with the liver-derived line or with the original CHCT-NYU4 line

  1. Cellular mesoblastic nephroma (infantile renal fibrosarcoma): institutional review of the clinical, diagnostic imaging, and pathologic features of a distinctive neoplasm of infancy

    International Nuclear Information System (INIS)

    Bayindir, Petek; Guillerman, Robert Paul; Hicks, M.J.; Chintagumpala, M.M.

    2009-01-01

    Cellular mesoblastic nephroma has been associated with a more aggressive course than classic mesoblastic nephroma, including local recurrences and metastases. To define the clinicopathologic and imaging features distinguishing cellular from classic mesoblastic nephroma. Retrospective review of clinical charts and imaging studies of ten children with mesoblastic nephroma from 1996 to 2007 at a large children's hospital. In six children the mesoblastic nephroma was pure cellular, in two mixed, and in two classic. The mean ages at diagnosis were 107 days for those with the cellular form, and 32 days for those with the classic form. Hypoechoic or low-attenuation regions representing necrosis or hemorrhage were found in all children with the cellular form and in none of those with the classic form. Hypertension was present in 70% and hypercalcemia in 20% of the children and resolved following nephrectomy. Two cellular tumors encased major abdominal vessels. Local recurrence and metastases occurred within 6 months of tumor resection in two children with the cellular form. Intraspinal extension and intratumoral pseudoaneurysm were seen in one child with the cellular form. The cellular tumors shared histopathologic features with infantile fibrosarcoma (IFS), and RT-PCR testing in two children with the cellular form revealed the t(12;15) ETV6-NTRK3 gene fusion common to IFS. Distinct from the classic form, cellular mesoblastic nephroma is more heterogeneous in appearance on imaging, tends to be larger and present later in infancy, and can exhibit aggressive behavior including vascular encasement and metastasis. Intraspinal extension and intratumoral pseudoaneurysm are previously unreported findings encountered in our cellular mesoblastic nephroma series. The shared histopathology and translocation gene fusion support the concept of cellular mesoblastic nephroma as the renal form of IFS. (orig.)

  2. Comparative analysis of cytokeratin 15, TDAG51, cytokeratin 20 and androgen receptor in sclerosing adnexal neoplasms and variants of basal cell carcinoma.

    Science.gov (United States)

    Evangelista, Mara Therese P; North, Jeffrey P

    2015-11-01

    Desmoplastic trichoepithelioma (DTE), morpheaform basal cell carcinoma (BCC) and microcystic adnexal carcinoma (MAC) are sclerosing adnexal neoplasms with overlapping histopathologic features. We compared cytokeratin 15, (CK15), T-cell death-associated gene 51 (TDAG51), cytokeratin 20 (CK20) and androgen receptor (AR) in differentiating these tumors and assessed their expression in BCC subtypes. Fifteen DTE, 15 infundibulocystic BCC, 18 micronodular BCC, 18 morpheaform BCC and 6 MAC were assessed for CK15, TDAG51, CK20 and AR expression. Quantitative CK15 staining was higher in DTE compared with BCC (p < 0.0001) and MAC (p = 0.02). Quantitative TDAG51 staining was higher in DTE than BCC (p < 0.0001). The CK20+AR- immunophenotype was 100% sensitive and specific in diagnosing DTE. The CK20-AR+ immunophenotype was 95.24% specific and 83.33% sensitive for BCC. The CK20-AR- immunophenotype was 83.33% sensitive and 90.91% specific for MAC. CK15, CK20 and AR were positive in 87, 53 and 67% of infundibulocystic BCC cases, respectively. Combination of CK20 and AR best differentiated these sclerosing adnexal neoplasms. Greater positivity for CK15 and TDAG51 generally favors benign lesions. Infundibulocystic BCC has higher CK20 and lower AR immunopositivity than other BCC variants and a high degree of CK15 and TDAG51 positivity. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  3. Efficacy and safety of vedolizumab as a treatment option for moderate to severe refractory ulcerative colitis in two patients after liver transplant due to primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    Raúl Vicente Olmedo-Martín

    Full Text Available Vedolizumab is a humanized IgG1 monoclonal antibody that selectively blocks the lymphocyte integrin α4β7 and prevents its interaction with endothelial adhesion molecules and subsequent transmigration to the gastrointestinal tract. The drug was approved in 2014 for the induction and maintenance treatment of ulcerative colitis and moderate to severe Crohn's disease that is refractory or intolerant to conventional treatment with corticoids and immunosuppressants and/or anti-TNFα drugs. However, inflammatory bowel disease has a variable behavior following liver transplant. One third of patients with ulcerative colitis associated with primary sclerosing cholangitis are expected to deteriorate despite receiving immunosuppression to prevent rejection. There is limited experience with anti-TNFα agents in patients with inflammatory bowel disease in the setting of liver transplantation and the studies to date involve a limited number of cases. The efficacy and safety data of vedolizumab in this situation are unreliable and very preliminary. We present two cases with the aim to present the efficacy and safety of vedolizumab after one year of treatment in two patients who underwent a transplant due to primary sclerosing cholangitis. One case had de novo post-transplant ulcerative colitis refractory to two anti-TNFα drugs (golimumab and infliximab. The other patient had a colostomy due to fulminant colitis and developed severe ulcerative proctitis refractory to infliximab after reconstruction with an ileorectal anastomosis.

  4. Comparison of the multidetector-row computed tomography findings of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma

    International Nuclear Information System (INIS)

    Yata, M.; Suzuki, K.; Furuhashi, N.; Kawakami, K.; Kawai, Y.; Naganawa, S.

    2016-01-01

    Aim: To compare the multidetector-row computed tomography (MDCT) findings of IgG4-related sclerosing cholangitis (IgG4-SC) and extrahepatic cholangiocarcinoma (EH-CCA). Materials and methods: Two radiologists who had no knowledge of the patients' clinical information retrospectively evaluated the CT findings of patients with IgG4-SC (n=33) and EH-CCA (n=39) on a consensus basis. Another radiologist measured the biliary lesions. IgG4-SC was diagnosed using the Japan Biliary Association criteria (2012) or the Mayo Clinic's HISORt criteria. EH-CCA was diagnosed based on surgical findings. Results: Compared with EH-CCA, IgG4-SC exhibited the following findings significantly more frequently: (a) wall thickening alone, (b) concentric wall thickening, (c) smooth inner margins, (d) homogeneous attenuation in the arterial phase, (e) a lesion involving the intrapancreatic bile duct, (f) smooth outer margins, (g) fully visible lumen, (h) a funnel-shaped proximal bile duct, (i) skip lesions, and (j) abnormal pancreatic findings. Conversely, (k) dual-layered attenuation in all phases was significantly more common in EH-CCA. The specificity values of parameters (e–k) were >80%. Regarding dimensions, (l) the biliary lesions were longer in IgG4-SC than in EH-CCA. (m) The diameters of the dilated proximal common bile duct and (n) the dilated proximal intrahepatic bile duct were smaller in IgG4-SC than in EH-CCA. Conclusion: A number of CT findings are useful for differentiating between IgG4-SC and EH-CCA. CT findings (e–k) are particularly useful for this purpose. - Highlights: • Some CT findings are useful for differentiating between IgG4-SC and EH-CCA. • Homogeneous attenuation in all phases was more common in IgG4-SC than in EH-CCA. • Abnormal pancreatic findings showed high sensitivity and specificity values. • Dual-layered attenuation in all phases was more common in EH-CCA than in IgG4-SC.

  5. Comparative clinical characteristics and natural history of three variants of sclerosing cholangitis: IgG4-related SC, PSC/AIH and PSC alone.

    Science.gov (United States)

    Lian, Min; Li, Bo; Xiao, Xiao; Yang, Yue; Jiang, Pan; Yan, Li; Sun, Chunyan; Zhang, Jun; Wei, Yiran; Li, Yanmei; Chen, Weihua; Jiang, Xiang; Miao, Qi; Chen, Xiaoyu; Qiu, Dekai; Sheng, Li; Hua, Jing; Tang, Ruqi; Wang, Qixia; Eric Gershwin, M; Ma, Xiong

    2017-08-01

    There is increased interest and recognition of the clinical variants of Sclerosing Cholangitis (SC) namely IgG4-SC, PSC/AIH overlap and PSC. For most Centers, the characteristic of IgG4-SC has not been thoroughly clinically compared with other sclerosing cholangitis variants. Further there are relatively few PSC/AIH overlap patients and the clinical outcome is not well characterized, especially for the PSC/AIH overlap syndrome. Our objective herein is to clarify the differences and similarities of the natural history of IgG4-SC, the PSC/AIH overlap and PSC alone. We also place in perspective the diagnostic value of serum IgG4 for IgG4-SC and investigate biomarkers for predicting the prognosis of sclerosing cholangitis. In this study, we took advantage of our large and well-defined patient cohort to perform a retrospective cohort study including 57 IgG4-SC, 36 PSC/AIH overlap patients, and 55 PSC patients. Firstly, as expected, we noted significant differences among immunoglobulin profiles and all patients exhibited similar cholestatic profiles at presentation. Cirrhotic events were found in 20 of total 57 IgG4-SC, 15 of 36 PSC/AIH overlap, and 18 of 55 PSC patients. Serum IgG4 was elevated in 92.65% of IgG4-SC patients with an 86% sensitivity and 98% specificity for diagnosis. IgG4-SC patients had a better treatment response at 6-month and 1-year than PSC/AIH patients, while the latter responded better with steroids than PSC patients. Importantly the adverse outcome-free survival of IgG4-SC patients was reduced, unlike earlier reports, and therefore similar to the PSC/AIH overlap syndrome. Serum IgG and total bilirubin were useful to predict long-term survival of IgG4-SC and PSC/AIH, respectively. In conclusion, serum IgG4≧1.25 ULN shows an excellent predictability to distinguish IgG4-SC among SC patients. IgG4-SC appears to be immune-mediated inflammatory process, while PSC/AIH overlap more tends to be cholestatic disease. Copyright © 2017 Elsevier B.V. All

  6. High expression of CD109 antigen regulates the phenotype of cancer stem-like cells/cancer-initiating cells in the novel epithelioid sarcoma cell line ESX and is related to poor prognosis of soft tissue sarcoma.

    Directory of Open Access Journals (Sweden)

    Makoto Emori

    Full Text Available Epithelioid sarcoma (ES is a relatively rare, highly malignant soft tissue sarcoma. The mainstay of treatment is resection or amputation. Currently other therapeutic options available for this disease are limited. Therefore, a novel therapeutic option needs to be developed. In the present study, we established a new human ES cell line (ESX and analyzed the characteristics of its cancer stem-like cells/cancer-initiating cells (CSCs/CICs based on ALDH1 activity. We demonstrated that a subpopulation of ESX cells with high ALDH1 activity (ALDH(high cells correlated with enhanced clonogenic ability, sphere-formation ability, and invasiveness in vitro and showed higher tumorigenicity in vivo. Next, using gene expression profiling, we identified CD109, a GPI-anchored protein upregulated in the ALDH(high cells. CD109 mRNA was highly expressed in various sarcoma cell lines, but weakly expressed in normal adult tissues. CD109-positive cells in ESX predominantly formed spheres in culture, whereas siCD109 reduced ALDH1 expression and inhibited the cell proliferation in vitro. Subsequently, we evaluated the expression of CD109 protein in 80 clinical specimens of soft tissue sarcoma. We found a strong correlation between CD109 protein expression and the prognosis (P = 0.009. In conclusion, CD109 might be a CSC/CIC marker in epithelioid sarcoma. Moreover, CD109 is a promising prognostic biomarker and a molecular target of cancer therapy for sarcomas including ES.

  7. 18F-FDG PET and MRS of the early stages of subacute sclerosing panencephalitis in a child with a normal initial MRI

    International Nuclear Information System (INIS)

    Seo, Yeong-Seon; Jung, Da-Eun; Kim, Ho-Sung

    2010-01-01

    In subacute sclerosing panencephalitis (SSPE), conventional MRI findings have been reported. However, in the early clinical stages, imaging studies can appear normal. Moreover, with no history of infant measles infection, the diagnosis of SSPE can only be arrived at after extensive investigation that must eliminate a number of neurodegenerative diseases. We report here on 18 F-fluorodeoxyglucose positron emission tomography ( 18 F-FDG PET) and magnetic resonance spectroscopy (MRS) findings in a 14-year-old girl with a normal initial MRI who had not contracted measles. Although 18 F-FDG PET and MRS are not specific or diagnostic for SSPE, these techniques can demonstrate substantial metabolic impairments when MRI findings show no obvious abnormalities, as is often the case in the early stages of this disease. (orig.)

  8. Laparoscopic Treatment of Sclerosing Stromal Tumor of the Ovary in a Woman With Gorlin-Goltz Syndrome: A Case Report and Review of the Literature.

    Science.gov (United States)

    Grechi, Gianluca; Clemente, Nicolò; Tozzi, Alessandra; Ciavattini, Andrea

    2015-01-01

    Gorlin-Goltz syndrome is a rare hereditary multisystemic disease. Multiple basal cell carcinomas, odontogenic keratocysts, and skeletal abnormalities are the main clinical manifestations of the syndrome, but several organs can be involved. Moreover, this condition is associated with the development of various benign and malignant tumors, even in the genital tract. This report describes a rare association between Gorlin-Goltz syndrome and the sclerosing stromal tumor of the ovary. Because the ultrasound and magnetic resonance imaging patterns of this tumor can be similar to those of a malignant neoplasm, prompt surgical intervention and histological confirmation of diagnosis is mandatory; however, this is a benign lesion and thus can be approached with a laparoscopic fertility-sparing surgery. Gynecologists should be aware of this possible association to provide appropriate counseling for these women, and to take a fertility-sparing laparoscopic approach whenever possible. Copyright © 2015 AAGL. Published by Elsevier Inc. All rights reserved.

  9. {sup 18}F-FDG PET and MRS of the early stages of subacute sclerosing panencephalitis in a child with a normal initial MRI

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Yeong-Seon; Jung, Da-Eun [Ajou University School of Medicine, Department of Pediatrics, Suwon, Kyungki-do (Korea, Republic of); Kim, Ho-Sung [Ajou University School of Medicine, Department of Radiology, Suwon, Kyungki-do (Korea, Republic of)

    2010-11-15

    In subacute sclerosing panencephalitis (SSPE), conventional MRI findings have been reported. However, in the early clinical stages, imaging studies can appear normal. Moreover, with no history of infant measles infection, the diagnosis of SSPE can only be arrived at after extensive investigation that must eliminate a number of neurodegenerative diseases. We report here on {sup 18} F-fluorodeoxyglucose positron emission tomography ({sup 18}F-FDG PET) and magnetic resonance spectroscopy (MRS) findings in a 14-year-old girl with a normal initial MRI who had not contracted measles. Although {sup 18} F-FDG PET and MRS are not specific or diagnostic for SSPE, these techniques can demonstrate substantial metabolic impairments when MRI findings show no obvious abnormalities, as is often the case in the early stages of this disease. (orig.)

  10. Hemangioendotelioma epitelióide do pulmão: Raridade, dificuldades diagnósticas e terapêutica Pulmonary epithelioid hemangioendothelioma: Rarity, diagnosis and treatment difficulties

    Directory of Open Access Journals (Sweden)

    Telma Lopes

    2009-11-01

    Full Text Available Os autores apresentam um caso de hemangioendotelioma epitelioide (HEE primário do pulmão num doente de 51 anos, sexo masculino, que iniciou queixas de tosse seca, seguida de sintomas constitucionais e dispneia. Apôs realização de alguns exames complementares de diagnostico, que incluiram biopsia cirúrgica, foi considerada a hipótese de tuberculose pulmonar e iniciou antibacilares, que manteve durante três semanas. Por agravamento clínico e imagiologico, foi feita revisão do caso e estudo imunoistoquimico dos tecidos pulmonares biopsados e concluiu-se estar perante um HEE de alto grau/angiossarcoma do pulmão. Iniciou quimioterapia com carboplatina, etoposido e bevacizumab, com ligeira melhoria. Faleceu sete meses após o aparecimento dos primeiros sintomas e sete semanas apos o diagnostico definitivo. Os autores pretendem realçar a raridade desta neoplasia pulmonar, a importância da suspeição clínica e as dificuldades no diagnostico e tratamento, e salientar os potenciais benefícios da utilização de um fármaco antiangiogenico.The authors report a case of a primary pulmonary epithelioid haemangioendothelioma (EHE in a 51 year-old man, a mechanic, who complained of a dry cough followed by constitutional symptoms and dyspnoea. Patient underwent a series of diagnostic exams including surgical biopsy and pulmonary tuberculosis was diagnosed. He was prescribed tuberculosis drugs for three weeks. Following clinical and imagiology deterioration, the case was reviewed by pathologists who concluded the pulmonary biopsy revealed an intermediate/high grade pulmonary EHE/angiosarcoma. The patient underwent three cycles of chemotherapy with carboplatin, etoposide and bevacizumab with no complications. He died seven months after onset of symptoms and seven weeks after definitive diagnosis. The authors wish to highlight the rarity of this pulmonary neoplasm and the importance of clinical suspicion, and the diagnosis and treatment difficulties

  11. Congenital infantile fibrosarcoma mimicking sacrococcygeal ...

    African Journals Online (AJOL)

    most parts of Africa may mitigate this good fortune.[2,6]. We report the ... measuring 12 × 9 × 7 cm covered by an eclipse of skin with cut section showing ... assessment of the sacrococcygeal mass showed a hypercellular tumour composed of ...

  12. Primary Sclerosing Cholangitis (PSC)

    Science.gov (United States)

    ... Liver Function Tests Clinical Trials Liver Transplant FAQs Medical Terminology Diseases of the Liver Alagille Syndrome Alcohol-Related ... the Liver The Progression of Liver Disease FAQs Medical Terminology HOW YOU CAN HELP Sponsorship Ways to Give ...

  13. Subacute Sclerosing Panencephalitis

    Science.gov (United States)

    ... high in developing countries such as India and Eastern Europe. There is a higher incidence among males ... high in developing countries such as India and Eastern Europe. There is a higher incidence among males ...

  14. [Study of androgen receptor and phosphoglycerate kinase gene polymorphism in major cellular components of the so-called pulmonary sclerosing hemangioma].

    Science.gov (United States)

    Qi, Feng-jie; Zhang, Xiu-wei; Zhang, Yong-xing; Dai, Shun-dong; Wang, En-hua

    2006-05-01

    To study the clonality of polygonal cells and surface cuboidal cells in the so-called pulmonary sclerosing hemangioma (PSH). 17 female surgically resected PSH were found. The polygonal cells and surface cuboidal cells of the 17 PSH cases were microdissected from routine hematoxylin and eosin-stained sections. Genomic DNA was extracted, pretreated through incubation with methylation-sensitive restrictive endonuclease HhaI or HpaII, and amplified by nested polymerase chain reaction for X chromosome-linked androgen receptor (AR) and phosphoglycerate kinase (PGK) genes. The length polymorphism of AR gene was demonstrated by denaturing polyacrylamide gel electrophoresis and silver staining. The PGK gene products were treated with Bst XI and resolved on agarose gel. Amongst the 17 female cases of PSH, 15 samples were successfully amplified for AR and PGK genes. The rates of polymorphism were 53% (8/15) and 27% (4/15) for AR and PGK genes respectively. Polygonal cells and surface cuboidal cells of 10 cases which were suitable for clonality study, showed the same loss of alleles (clonality ratio = 0) or unbalanced methylation pattern (clonality ratio < 0.25). The polygonal cells and surface cuboidal cells in PSH demonstrate patterns of monoclonal proliferation, indicating that both represent true neoplastic cells.

  15. Structural defect linked to nonrandom mutations in the matrix gene of Biden strain subacute sclerosing panencephalitis virus defined by cDNA cloning and expression of chimeric genes

    International Nuclear Information System (INIS)

    Ayata, M.; Hirano, A.; Wong, T.C.

    1989-01-01

    Biken strain, a nonproductive measles viruslike agent isolated from a subacute sclerosing panencephalitis (SSPE) patient, contains a posttranscriptional defect affecting matrix (M) protein. A putative M protein was translated in vitro with RNA from Biken strain-infected cells. A similar protein was detected in vivo by an antiserum against a peptide synthesized from the cloned M gene of Edmonston strain measles virus. By using a novel method, full-length cDNAs of the Biken M gene were selectively cloned. The cloned Biken M gene contained an open reading frame which encoded 8 extra carboxy-terminal amino acid residues and 20 amino acid substitutions predicted to affect both the hydrophobicity and secondary structure of the gene product. The cloned gene was expressed in vitro and in vivo into a 37,500 M r protein electrophoretically and antigenically distinct from the M protein of Edmonston strain but identical to the M protein in Biken strain-infected cells. Chimeric M proteins synthesized in vitro and in vivo showed that the mutations in the carboxy-proximal region altered the local antigenicity and those in the amino region affected the overall protein conformation. The protein expressed from the Biken M gene was unstable in vivo. Instability was attributed to multiple mutations. These results offer insights into the basis of the defect in Biken strain and pose intriguing questions about the evolutionary origins of SSPE viruses in general

  16. Differential diagnosis of sclerosing cholangitis with autoimmune pancreatitis and periductal infiltrating cancer in the common bile duct at dynamic CT, endoscopic retrograde cholangiography and MR cholangiography

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jin Hee; Byun, Jae Ho; Lee, So Jung; Park, Seong Ho; Kim, Hyoung Jung; Lee, Seung Soo; Lee, Moon-Gyu [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Asanbyeongwon-gil 86, Songpa-Gu, Seoul (Korea, Republic of); Kim, Myung-Hwan [University of Ulsan College of Medicine, Asan Medical Center, Department of Internal Medicine, Asanbyeongwon-gil 86, Songpa-Gu, Seoul (Korea, Republic of); Kim, Jihun [University of Ulsan College of Medicine, Asan Medical Center, Department of Diagnostic Pathology, Asanbyeongwon-gil 86, Songpa-Gu, Seoul (Korea, Republic of)

    2012-11-15

    To compare findings at dynamic computed tomography (CT), endoscopic retrograde cholangiography (ERC) and magnetic resonance cholangiography (MRC) in patients with sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) and periductal infiltrating cancer in the common bile duct (CBD), and to evaluate the diagnostic performance of ERC and MRC in differentiating between the two diseases. Bile duct changes at dynamic CT, ERC and MRC were compared in 58 patients with SC-AIP and CBD involvement and 93 patients with periductal infiltrating CBD cancer. Two radiologists rated their confidence in differentiating between the two diseases and the diagnostic performances of ERC and MRC were compared. At CT, SC-AIP was more frequently associated with intrapancreatic CBD involvement, thinner CBD walls, concentric wall thickening, smooth outer margins, and lower degrees of upstream ductal dilatation and contrast enhancement (P {<=} 0.05) than CBD cancer. At ERC and MRC, SC-AIP was more frequently associated with smooth margins, gradual and symmetric narrowing, multifocal involvement and hourglass appearance (P {<=} 0.027) than CBD cancer. MRC showed good diagnostic performance comparable to ERC. Dynamic CT, ERC and MRC can be helpful in distinguishing SC-AIP from periductal infiltrating CBD cancer. MRC may be a useful diagnostic alternative to ERC in differentiating between the two diseases. (orig.)

  17. Differential diagnosis of sclerosing cholangitis with autoimmune pancreatitis and periductal infiltrating cancer in the common bile duct at dynamic CT, endoscopic retrograde cholangiography and MR cholangiography

    International Nuclear Information System (INIS)

    Kim, Jin Hee; Byun, Jae Ho; Lee, So Jung; Park, Seong Ho; Kim, Hyoung Jung; Lee, Seung Soo; Lee, Moon-Gyu; Kim, Myung-Hwan; Kim, Jihun

    2012-01-01

    To compare findings at dynamic computed tomography (CT), endoscopic retrograde cholangiography (ERC) and magnetic resonance cholangiography (MRC) in patients with sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) and periductal infiltrating cancer in the common bile duct (CBD), and to evaluate the diagnostic performance of ERC and MRC in differentiating between the two diseases. Bile duct changes at dynamic CT, ERC and MRC were compared in 58 patients with SC-AIP and CBD involvement and 93 patients with periductal infiltrating CBD cancer. Two radiologists rated their confidence in differentiating between the two diseases and the diagnostic performances of ERC and MRC were compared. At CT, SC-AIP was more frequently associated with intrapancreatic CBD involvement, thinner CBD walls, concentric wall thickening, smooth outer margins, and lower degrees of upstream ductal dilatation and contrast enhancement (P ≤ 0.05) than CBD cancer. At ERC and MRC, SC-AIP was more frequently associated with smooth margins, gradual and symmetric narrowing, multifocal involvement and hourglass appearance (P ≤ 0.027) than CBD cancer. MRC showed good diagnostic performance comparable to ERC. Dynamic CT, ERC and MRC can be helpful in distinguishing SC-AIP from periductal infiltrating CBD cancer. MRC may be a useful diagnostic alternative to ERC in differentiating between the two diseases. (orig.)

  18. High-Dose Ursodeoxycholic Acid Is Associated With the Development of Colorectal Neoplasia in Patients With Ulcerative Colitis and Primary Sclerosing Cholangitis

    Science.gov (United States)

    Eaton, John E.; Silveira, Marina G.; Pardi, Darrell S.; Sinakos, Emmanouil; Kowdley, Kris V.; Luketic, Velimir A.C.; Harrison, M. Edwyn; McCashland, Timothy; Befeler, Alex S.; Harnois, Denise; Jorgensen, Roberta; Petz, Jan; Lindor, Keith D.

    2011-01-01

    OBJECTIVES Some studies have suggested that ursodeoxycholic acid (UDCA) may have a chemopreventive effect on the development of colorectal neoplasia in patients with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC). We examined the effects of high-dose (28–30 mg/kg/day) UDCA on the development of colorectal neoplasia in patients with UC and PSC. METHODS Patients with UC and PSC enrolled in a prior, multicenter randomized placebo-controlled trial of high-dose UDCA were evaluated for the development of colorectal neoplasia. Patients with UC and PSC who received UDCA were compared with those who received placebo. We reviewed the pathology and colonoscopy reports for the development of low-grade or high-grade dysplasia or colorectal cancer. RESULTS Fifty-six subjects were followed for a total of 235 patient years. Baseline characteristics (including duration of PSC and UC, medications, patient age, family history of colorectal cancer, and smoking status) were similar for both the groups. Patients who received high-dose UDCA had a significantly higher risk of developing colorectal neoplasia (dysplasia and cancer) during the study compared with those who received placebo (hazard ratio: 4.44, 95% confidence interval: 1.30–20.10, P=0.02). CONCLUSIONS Long-term use of high-dose UDCA is associated with an increased risk of colorectal neoplasia in patients with UC and PSC. PMID:21556038

  19. Analysis of U2 small nuclear RNA fragments in the bile differentiates cholangiocarcinoma from primary sclerosing cholangitis and other benign biliary disorders.

    Science.gov (United States)

    Baraniskin, Alexander; Nöpel-Dünnebacke, Stefanie; Schumacher, Brigitte; Gerges, Christian; Bracht, Thilo; Sitek, Barbara; Meyer, Helmut E; Gerken, Guido; Dechene, Alexander; Schlaak, Jörg F; Schroers, Roland; Pox, Christian; Schmiegel, Wolff; Hahn, Stephan A

    2014-07-01

    Up to now the diagnosis of early stage cholangiocarcinoma (CC) has remained difficult, with low sensitivities reported for current diagnostic methods. Based on recent promising findings about circulating U2 small nuclear RNA fragments (RNU2-1f) as novel blood-based biomarkers for pancreatic and colorectal adenocarcinoma, we studied the utility of RNU2-1f as a diagnostic marker of CC in bile fluid. Bile fluid was collected from patients with CC (n = 12), controls (patients with choledocholithiasis) (n = 11) and with primary sclerosing cholangitis (PSC; n = 11). RNU2-1f levels were measured by real-time polymerase chain reaction normalized to cel-54. Measurement of RNU2-1f levels in bile fluids enabled the differentiation of patients with CC from controls in all cases. Furthermore, RNU2-1f levels in bile fluids of patients with CC were significantly higher than in patients with PSC, resulting in a receiver-operating characteristic curve area of 0.856, with sensitivity of 67 % and specificity of 91 %. Our data suggest that the measurement of RNU2-1 fragments detected in the bile fluid can be used as a diagnostic marker for CC and should be included in future prospective diagnostic studies for this disease entity.

  20. Increased cholestatic enzymes in two patients with long-term history of ulcerative colitis: consider primary biliary cholangitis not always primary sclerosing cholangitis.

    Science.gov (United States)

    Polychronopoulou, Erietta; Lygoura, Vasiliki; Gatselis, Nikolaos K; Dalekos, George N

    2017-09-25

    Several hepatobiliary disorders have been reported in ulcerative colitis (UC) patients with primary sclerosing cholangitis (PSC) being the most specific. Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, rarely occurs in UC. We present two PBC cases of 67 and 71 years who suffered from long-standing UC. Both patients were asymptomatic but they had increased cholestatic enzymes and high titres of antimitochondrial antibodies (AMA)-the laboratory hallmark of PBC. After careful exclusion of other causes of cholestasis by MRI/magnetic resonance cholangiopancreatography (MRCP), virological and microbiological investigations, a diagnosis of PBC associated with UC was established. The patients started ursodeoxycholic acid (13 mg/kg/day) with complete response. During follow-up, both patients remained asymptomatic with normal blood biochemistry. Although PSC is the most common hepatobiliary manifestation among patients with UC, physicians must keep also PBC in mind in those with unexplained cholestasis and repeatedly normal MRCP. In these cases, a reliable AMA testing can help for an accurate diagnosis. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  1. Pulmonary sclerosing hemangioma in a 21-year-old male with metastatic hereditary non-polyposis colorectal cancer: Report of a case

    Directory of Open Access Journals (Sweden)

    Angele Martin K

    2011-06-01

    Full Text Available Abstract Background Pulmonary sclerosing hemangioma (SH is a rare tumor of the lung predominantly affecting Asian women in their fifth decade of life. SH is thought to evolve from primitive respiratory epithelium and mostly shows benign biological behavior; however, cases of lymph node metastases, local recurrence and multiple lesions have been described. Case Presentation We report the case of a 21-year-old Caucasian male with a history of locally advanced and metastatic rectal carcinoma (UICC IV; pT4, pN1, M1(hep that was eventually identified as having hereditary non-polyposis colorectal cancer (HNPCC, Lynch syndrome. After neoadjuvant chemotherapy followed by low anterior resection, adjuvant chemotherapy and metachronous partial hepatectomy, he was admitted for treatment of newly diagnosed bilateral pulmonary metastases. Thoracic computed tomography showed a homogenous, sharply marked nodule in the left lower lobe. We decided in favor of atypical resection followed by systematic lymphadenectomy. Histopathological analysis revealed the diagnosis of SH. Conclusions Cases have been published with familial adenomatous polyposis (FAP and simultaneous SH. FAP, Gardner syndrome and Li-Fraumeni syndrome, however, had been ruled out in the present case. To the best of our knowledge, this is the first report describing SH associated with Lynch syndrome.

  2. Seahorse-derived peptide suppresses invasive migration of HT1080 fibrosarcoma cells by competing with intracellular α-enolase for plasminogen binding and inhibiting uPA-mediated activation of plasminogen.

    Science.gov (United States)

    Kim, Yong-Tae; Kim, Se-kwon; Jeon, You-Jin; Park, Sun Joo

    2014-12-01

    α-Enolase is a glycolytic enzyme and a surface receptor for plasminogen. α-Enolase-bound plasminogen promotes tumor cell invasion and cancer metastasis by activating plasmin and consequently degrading the extracellular matrix degradation. Therefore, α-enolase and plasminogen are novel targets for cancer therapy. We found that the amino acid sequence of a peptide purified from enzymatic hydrolysates of seahorse has striking similarities to that of α-enolase. In this study, we report that this peptide competes with cellular α-enolase for plasminogen binding and suppresses urokinase plasminogen activator (uPA)-mediated activation of plasminogen, which results in decreased invasive migration of HT1080 fibrosarcoma cells. In addition, the peptide treatment decreased the expression levels of uPA compared to that of untreated controls. These results provide new insight into the mechanism by which the seahorse-derived peptide suppresses invasive properties of human cancer cells. Our findings suggest that this peptide could emerge as a potential therapeutic agent for cancer.

  3. Celiac disease (CD), ulcerative colitis (UC), and primary sclerosing cholangitis (PSC) in one patient: a family study Enfermedad celiaca (EC), colitis ulcerosa (CU) y colangitis esclerosante primaria (CEP) asociadas en el mismo paciente: estudio familiar

    OpenAIRE

    V. Cadahía; L. Rodrigo; D. Fuentes; S. Riestra; R. de Francisco; M. Fernández

    2005-01-01

    We discuss the case of a 17-year-old male who at the age of 7 was diagnosed with celiac disease (CD) together with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC). The patient was treated with gluten-free diet and immunosuppressive drugs (azathioprine), and currently remains asymptomatic. The patient's younger, 12-year-old sister was diagnosed with CD when she was 1.5 years old, and at 7 years she developed type-I diabetes mellitus, which was difficult to control. A family st...

  4. Angiossarcoma epitelióide ósseo: relato de um caso, com estudo imuno-histoquímico Epithelioid angiosarcoma of bone: report of a case with immunohistochemical study

    Directory of Open Access Journals (Sweden)

    Cláudia Regina G. C. M. de Oliveira

    2001-03-01

    Full Text Available Angiossarcoma primário do osso é muito raro, representando menos de 1% de todos os angiossarcomas(4. Neoplasias vasculares também são incomuns, ocorrendo em 14% dos tumores malignos primários do osso. Os autores relatam caso em coluna lombar que merece atenção porque inicialmente tratava-se de um hemangioendotelioma, um tumor de malignidade intermediária, definido pelos critérios estabelecidos por Stout(11. Foi tratado com embolização local e radioterapia. Após dez anos, recidivou, com maior grau de malignidade, fenômeno interpretado como progressão tumoral, com características de Angiossarcoma epitelióide. O estudo imuno-histoquímico revelou positividade para marcadores vasculares. (Fator VIII, CD31 e CD34. O paciente evoluiu com metástases pulmonares. Em revisão bibliográfica, não foi encontado nenhum relato de caso semelhante, tendo em vista a longa evolução e progressão tumoral após dez anos de seguimento e expressão do fenotipo epitelióide.Primary angiosarcoma of bone is rare and accounts for less than 1% of all angiosarcomas(4. Vascular bone neoplasms represent 1,4% of primary malignant bone tumors. The authors describe an unusual case of bone angiosarcoma located in the spine. The diagnosis was initially hemangioendothelioma, a borderline endothelial vascular tumor, as stated by Stout(11. The lesion was treated by local embolization and radiation therapy. The tumor recurred locally ten years later as a high-grade angiosarcoma, showing epithelioid features, which indicates tumor progression. Immunohistochemical studies were positive for vascular markers such as factor VIII, CD31 and CD 34. The patient developed lung metastasis. I was not observed in the literature any other case with ten-year follow-up and tumor progression with epithelioid features.

  5. Combination Therapy of All-Trans Retinoic Acid With Ursodeoxycholic Acid in Patients With Primary Sclerosing Cholangitis: A Human Pilot Study.

    Science.gov (United States)

    Assis, David N; Abdelghany, Osama; Cai, Shi-Ying; Gossard, Andrea A; Eaton, John E; Keach, Jill C; Deng, Yanhong; Setchell, Kenneth D R; Ciarleglio, Maria; Lindor, Keith D; Boyer, James L

    2017-02-01

    To perform an exploratory pilot study of all-trans retinoic acid (ATRA) combined with ursodeoxycholic acid (UDCA) in patients with primary sclerosing cholangitis (PSC). PSC is a progressive disorder for which there is no accepted therapy. Studies in human hepatocyte cultures and in animal models of cholestasis indicate that ATRA might have beneficial effects in cholestatic disorders. ATRA (45 mg/m/d, divided and given twice daily) was combined with moderate-dose UDCA in patients with PSC who had incomplete response to UDCA monotherapy. The combination was administered for 12 weeks, followed by a 12-week washout in which patients returned to UDCA monotherapy. We measured alkaline phosphatase (ALP), alanine aminotransferase (ALT), bilirubin, cholesterol, bile acids, and the bile acid intermediate 7α-hydroxy-4-cholesten-3-one (C4) at baseline, week 12, and after washout. Fifteen patients completed 12 weeks of therapy. The addition of ATRA to UDCA reduced the median serum ALP levels (277±211 to 243±225 U/L, P=0.09) although this, the primary endpoint, did not reach significance. In contrast, median serum ALT (76±55 to 46±32 U/L, P=0.001) and C4 (9.8±19 to 7.9±11 ng/mL, P=0.03) levels significantly decreased. After washout, ALP and C4 levels nonsignificantly increased, whereas ALT levels significantly increased (46±32 to 74±74, P=0.0006), returning to baseline. In this human pilot study, the combination of ATRA and UDCA did not achieve the primary endpoint (ALP); however, it significantly reduced ALT and the bile acid intermediate C4. ATRA appears to inhibit bile acid synthesis and reduce markers of inflammation, making it a potential candidate for further study in PSC (NCT 01456468).

  6. Type 1 Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis Is Associated With Extrapancreatic Organ Failure, Malignancy, and Mortality in a Prospective UK Cohort

    Science.gov (United States)

    Kumar, M.; Hurst, J.M.; Rodriguez-Justo, M.; Chapman, M.H.; Johnson, G.J.; Pereira, S.P.; Chapman, R.W.

    2015-01-01

    OBJECTIVES Type I autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-related SC) are now recognized as components of a multisystem IgG4-related disease (IgG4-RD). We aimed to define the clinical course and long-term outcomes in patients with AIP/IgG4-SC recruited from two large UK tertiary referral centers. METHODS Data were collected from 115 patients identified between 2004 and 2013, and all were followed up prospectively from diagnosis for a median of 33 months (range 1–107), and evaluated for response to therapy, the development of multiorgan involvement, and malignancy. Comparisons were made with national UK statistics. RESULTS Although there was an initial response to steroids in 97%, relapse occurred in 50% of patients. IgG4-SC was an important predictor of relapse (P IgG4-RD, including three hepatopancreaticobiliary cancers. The risk of any cancer at diagnosis or during follow-up when compared with matched national statistics was increased (odds ratio = 2.25, CI = 1.12–3.94, P = 0.02). Organ dysfunction occurred within the pancreas, liver, kidney, lung, and brain. Mortality occurred in 10% of patients during follow-up. The risk of death was increased compared with matched national statistics (odds ratio = 2.07, CI = 1.07–3.55, P = 0.02). CONCLUSIONS Our findings suggest that AIP and IgG4-SC are associated with significant morbidity and mortality owing to extrapancreatic organ failure and malignancy. Detailed clinical evaluation for evidence of organ dysfunction and associated malignancy is required both at first presentation and during long-term follow-up. PMID:25155229

  7. Subacute sclerosing panencephalitis: clinical aspects and prognosis. The Brazilian Registry Panencefalite esclerosante subaguda, aspectos clínicos e prognóstico: Registro Brasileiro

    Directory of Open Access Journals (Sweden)

    MAGDA LAHORGUE NUNES

    1999-06-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is an inflammatory neurodegenerative disease related to the persistence of measles virus. Although its frequency is declining because of measles eradication, we still have some cases being diagnosed. With the aim to describe epidemiological aspects of SSPE in Brazil, we sent a protocol to Child Neurologists around the country, 48 patients were registered, 27 (56 % were from the southeast region, 34 (71% were male and 35 (73% white, 27 (56% had measles, 9 (19% had measles and were also immunized, 7 (14% received only immunization, 1 patient had a probable neonatal form. Mean time between first symptoms and diagnosis was 12 months (22 started with myoclonus or tonic-clonic seizures, 7 (14% with behavioral disturbances; 36 patients (75% had EEG with pseudoperiodic complexes. Follow up performed in 28 (58 % patients showed: 12 died, 2 had complete remission and the others had variable neurological disability Our data shows endemic regions in the country, a high incidence of post-immunization SSPE and a delay between first symptom and diagnosis.A panencefalite esclerosante subaguda é doença neurodegenerativa inflamatória relacionada à persistência do vírus do sarampo no organismo. Sua incidência vem diminuindo significativamente com a erradicação do sarampo, mas eventualmente alguns casos ainda têm sido diagnosticados. Com o objetivo de descrever aspectos epidemiológicos da panencefalite no Brasil contactamos Neurologistas Infantis de todo país. Foram registrados 48 pacientes, 27% da região sudeste, 34 (70% do sexo masculino, 35 (73% brancos, 9 (19% apresentaram sarampo e receberam imunização, 7 (14% somente imunizados, um paciente apresentou provável forma neonatal. Intervalo médio entre primeiro sintoma e diagnóstico de 12 meses, 22 pacientes (45% iniciaram o quadro com mioclonus ou convulsões tônico-clônicas, 7 (14% com distúrbios comportamentais; 36 (75% apresentaram EEG com

  8. Newly Diagnosed Colonic Adenocarcinoma: The Presenting Sign in a Young Woman with Undiagnosed Crohn’s Disease in the Absence of Primary Sclerosing Cholangitis and a Normal Microsatellite Instability Profile

    Directory of Open Access Journals (Sweden)

    Brett Matthew Lowenthal

    2017-01-01

    Full Text Available Ulcerative colitis has long been linked with an increased risk for colonic adenocarcinoma, whereas Crohn’s disease (CD has recently been reported to pose a similar increased risk. We report a 33-year-old healthy female with no family history who presented with abdominal pain and a colon mass. Histopathology revealed a moderately differentiated adenocarcinoma extending through the muscularis propria with metastatic lymph nodes and intact mismatch repair proteins by immunohistochemical expression and gene sequencing. The nonneoplastic grossly uninvolved background mucosa showed marked crypt distortion, crypt abscesses, CD-like lymphoid hyperplasia, transmural inflammation, and reactive epithelial atypia. Additional patient questioning revealed frequent loose stools since she was a teenager leading to diagnosis of a previously undiagnosed CD without primary sclerosing cholangitis (PSC. The adenocarcinoma is suspected to be related to the underlying CD. Newly diagnosed adenocarcinoma in a young female as the presenting sign for CD in the absence of PSC is extremely rare.

  9. Effect of mebendazole on fibrosarcoma in hamsters

    African Journals Online (AJOL)

    Purpose: To investigate the effect of mebendazole on an in vivo solid tumor ... The tumors were excised, their weight and diameters measured, and ... regions, is effective against cancer cell ..... No conflict of interest associated with this work.

  10. Epithelioid Haemangioendothelioma (EHE) of the Liver.

    Science.gov (United States)

    Khan, Abidullah; Humayun, Mohammad; Haider, Iqbal; Khan, Wazir Muhammad; Ajmal, Fahad; Ayub, Maimoona

    2016-06-01

    A45-year previously known hypertensive lady presented with 2-year history of upper abdominal pain, heaviness, and weight loss. Her radiological assessment suggested the possibility of hepatoma or liver metastases. Considering her age, overall good health and absence of cirrhosis, a liver biopsy was taken which showed hepatic epithelioidhaemangioendothelioma (HEHE), a rare and unusual intermediate grade vascular tumor which can easily be confused with hepatoma or metastatic liver disease. To the best of their knowledge, the authors are most probably reporting the first ever case of HEHE from Pakistan with special emphasis on its clinical presentations, clinico-radiological diagnostic clues, and the management options in the light of the limited retrospective studies.

  11. Celiac disease (CD, ulcerative colitis (UC, and primary sclerosing cholangitis (PSC in one patient: a family study Enfermedad celiaca (EC, colitis ulcerosa (CU y colangitis esclerosante primaria (CEP asociadas en el mismo paciente: estudio familiar

    Directory of Open Access Journals (Sweden)

    V. Cadahía

    2005-12-01

    Full Text Available We discuss the case of a 17-year-old male who at the age of 7 was diagnosed with celiac disease (CD together with ulcerative colitis (UC and primary sclerosing cholangitis (PSC. The patient was treated with gluten-free diet and immunosuppressive drugs (azathioprine, and currently remains asymptomatic. The patient's younger, 12-year-old sister was diagnosed with CD when she was 1.5 years old, and at 7 years she developed type-I diabetes mellitus, which was difficult to control. A family study was made, and both parents were found to be affected with silent CD. All were DQ2 (+. In relation to the case and family study, we provide a series of comments related to CD and its complications.Presentamos el caso de un varón de 17 años, que a la edad de 7 años fue diagnosticado de enfermedad celiaca (EC junto con una colitis ulcerosa (CU y una colangitis esclerosante primaria (CEP asociadas. Fue tratado con DSG e inmuno-supresores tipo azatioprina y se encuentra asintomático en la actualidad. Su hermana menor de 12 años, fue diagnosticada de EC cuando tenía 1,5 años y a los 7 años desarrolló una DM tipo 1 de difícil control. Se realizó un estudio familiar y ambos padres están afectos de una EC silente. Todos ellos son DQ2 (+. A propósito del caso y estudio familiar, se hacen una serie de consideraciones sobre la enfermedad celiaca y el desarrollo de complicaciones.

  12. Vascular adhesion protein-1 is elevated in primary sclerosing cholangitis, is predictive of clinical outcome and facilitates recruitment of gut-tropic lymphocytes to liver in a substrate-dependent manner.

    Science.gov (United States)

    Trivedi, Palak J; Tickle, Joseph; Vesterhus, Mette Nåmdal; Eddowes, Peter J; Bruns, Tony; Vainio, Jani; Parker, Richard; Smith, David; Liaskou, Evaggelia; Thorbjørnsen, Liv Wenche; Hirschfield, Gideon M; Auvinen, Kaisa; Hubscher, Stefan G; Salmi, Marko; Adams, David H; Weston, Chris J

    2018-06-01

    Primary sclerosing cholangitis (PSC) is the classical hepatobiliary manifestation of IBD. This clinical association is linked pathologically to the recruitment of mucosal T cells to the liver, via vascular adhesion protein (VAP)-1-dependent enzyme activity. Our aim was to examine the expression, function and enzymatic activation of the ectoenzyme VAP-1 in patients with PSC. We examined VAP-1 expression in patients with PSC, correlated levels with clinical characteristics and determined the functional consequences of enzyme activation by specific enzyme substrates on hepatic endothelium. The intrahepatic enzyme activity of VAP-1 was elevated in PSC versus immune-mediated disease controls and non-diseased liver (pgut-tropic α4β7 + lymphocytes to hepatic endothelial cells in vitro under flow was attenuated by 50% following administration of the VAP-1 inhibitor semicarbazide (pgut bacteria-was the most efficient (yielded the highest enzymatic rate) and efficacious in its ability to induce expression of functional mucosal addressin cell adhesion molecule-1 on hepatic endothelium. In a prospectively evaluated patient cohort with PSC, elevated serum soluble (s)VAP-1 levels predicted poorer transplant-free survival for patients, independently (HR: 3.85, p=0.003) and additively (HR: 2.02, p=0.012) of the presence of liver cirrhosis. VAP-1 expression is increased in PSC, facilitates adhesion of gut-tropic lymphocytes to liver endothelium in a substrate-dependent manner, and elevated levels of its circulating form predict clinical outcome in patients. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  13. Contrast enhanced liver MRI in patients with primary sclerosing cholangitis: inverse appearance of focal confluent fibrosis on delayed phase MR images with hepatocyte specific versus extracellular gadolinium based contrast agents.

    Science.gov (United States)

    Husarik, Daniela B; Gupta, Rajan T; Ringe, Kristina I; Boll, Daniel T; Merkle, Elmar M

    2011-12-01

    To assess the enhancement pattern of focal confluent fibrosis (FCF) on contrast-enhanced hepatic magnetic resonance imaging (MRI) using hepatocyte-specific (Gd-EOB-DTPA) and extracellular (ECA) gadolinium-based contrast agents in patients with primary sclerosing cholangitis (PSC). After institutional review board approval, 10 patients with PSC (6 male, 4 female; 33-61 years) with 13 FCF were included in this retrospective study. All patients had a Gd-EOB-DTPA-enhanced liver MRI exam, and a comparison ECA-enhanced MRI. On each T1-weighted dynamic dataset, the signal intensity (SI) of FCF and the surrounding liver as well as the paraspinal muscle (M) were measured. In the Gd-EOB-DTPA group, hepatocyte phase images were also included. SI FCF/SI M, SI liver/SI M, and [(SI liver - SI FCF)/SI liver] were compared between the different contrast agents for each dynamic phase using the paired Student's t-test. There was no significant difference in SI FCF/SI M in all imaging phases. SI liver/SI M was significantly higher for the Gd-EOB-DTPA group in the delayed phase (P DTPA group, mean [(SI liver - SI FCF)/SI liver] were as follows (values for ECA group in parentheses): unenhanced phase: 0.26 (0.26); arterial phase: 0.01 (-0.31); portal venous phase (PVP): -0.05 (-0.26); delayed phase (DP): 0.14 (-0.54); and hepatocyte phase: 0.26. Differences were significant for the DP (P DTPA-enhanced images. Copyright © 2011 AUR. Published by Elsevier Inc. All rights reserved.

  14. 3-(3-Hydroxy-4-methoxyphenyl)-4-(3,4,5-trimethoxyphenyl)-1,2,5-selenadiazole (G-1103), a novel combretastatin A-4 analog, induces G2/M arrest and apoptosis by disrupting tubulin polymerization in human cervical HeLa cells and fibrosarcoma HT-1080 cells.

    Science.gov (United States)

    Zuo, Daiying; Guo, Dandan; Jiang, Xuewei; Guan, Qi; Qi, Huan; Xu, Jingwen; Li, Zengqiang; Yang, Fushan; Zhang, Weige; Wu, Yingliang

    2015-02-05

    Microtubule is a popular target for anticancer drugs. In this study, we describe the effect 3-(3-hydroxy-4-methoxyphenyl)-4-(3,4,5-trimethoxyphenyl)-1,2,5-selenadiazole (G-1103), a newly synthesized analog of combretastatin A-4 (CA-4), showing a strong time- and dose-dependent anti-proliferative effect on human cervical cancer HeLa cells and human fibrosarcoma HT-1080 cells. We demonstrated that the growth inhibitory effects of G-1103 in HeLa and HT-1080 cells were associated with microtubule depolymerization and proved that G-1103 acted as microtubule destabilizing agent. Furthermore, cell cycle analysis revealed that G-1103 treatment resulted in cell cycle arrest at the G2/M phase in a time-dependent manner with subsequent apoptosis induction. Western blot analysis revealed that down-regulation of cdc25c and up-regulation of cyclin B1 was related with G2/M arrest in HeLa and HT-1080 cells treatment with G-1103. In addition, G-1103 induced HeLa cell apoptosis by up-regulating cleaved caspase-3, Fas, cleaved caspase-8 expression, which indicated that G-1103 induced HeLa cell apoptosis was mainly associated with death receptor pathway. However, G-1103 induced HT-1080 cell apoptosis by up-regulating cleaved caspase-3, Fas, cleaved caspase-8, Bax and cleaved caspase-9 expression and down-regulating anti-apoptotic protein Bcl-2 expression, which indicated that G-1103 induced HT-1080 cell apoptosis was associated with both mitochondrial and death receptor pathway. Taken together, all the data demonstrated that G-1103 exhibited its antitumor activity through disrupting the microtubule assembly, causing cell cycle arrest and consequently inducing apoptosis in HeLa and HT-1080 cells. Therefore, the novel compound G-1103 is a promising microtubule inhibitor that has great potentials for therapeutic treatment of various malignancies. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  15. Magnetic resonance in multiple sclerose twins

    International Nuclear Information System (INIS)

    Polman, C.H.; UitdeHaag, B.M.J.; Koetsier, C.J.; Valk, J.; Lucas, C.J.

    1989-01-01

    Magnetic resonance imaging (MR) examinations were performed in a series of 7 twin sets (4 monozygotic and 3 dizygotic) and one triplet set who were clinically discordant for multiple sclerosis (MS). MRI abnormalities were detected in a number of the unaffected members of the nonzygotic twin pairs. The authors discuss the possible implications of their findings for the present view on the aetiology of MS. (author). 3 refs.; 1 fig.; 1 tab

  16. Primary sclerosing lipogranuloma: an unusual scrotal mass

    African Journals Online (AJOL)

    2010-10-04

    Oct 4, 2010 ... Serology test for tuberculosis, fungal, cytomegalovirus, and filariasis from blood ... follicles of various sizes with prominent germinal centre and tangible ... As there are very little data on the use of other drugs in its treatment ...

  17. Sclerosing Stromal Tumor of the Ovary

    Directory of Open Access Journals (Sweden)

    Tayfun Güngör

    2011-04-01

    CONCLUSION: SSTs should be considered in young women with menstrual irregularity who have hypervascular solid and cystic adnexal masses. Though the tumor appears malignant, since it occurs in young women, care should be taken before embarking on radical surgery.

  18. Genetic Epidemiological Studies of Multiple Sclerose

    NARCIS (Netherlands)

    I.A. Hoppenbrouwers (Ilse)

    2011-01-01

    textabstractThe objective of this thesis was to find new risk alleles for MS. This may finally result in a better understanding of the pathogenesis of MS. Knowledge of MS disease pathways can direct strategies for prevention, diagnosis and therapy. In our study, we included MS patients from a

  19. Multiple sclerose : epidemiologie en kwaliteit van leven

    NARCIS (Netherlands)

    Zwanikken, Cornelis Petrus

    1997-01-01

    Multiple Sclerosis (MS) is a chronic disease of the brain and the spinal cord. The cause of MS is unknown. MS usually starts in young adulthood. In the course of the disease progression of neurological handicap and increase of impairments in daily life is common. In this study there were two aims.

  20. Effect of mebendazole on fibrosarcoma in hamsters | Popović ...

    African Journals Online (AJOL)

    Tropical Journal of Pharmaceutical Research. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 16, No 10 (2017) >. Log in or Register to get access to full text downloads.

  1. lessons from a hybrid epithelioid trophoblastic tumor and chorio

    African Journals Online (AJOL)

    (36,900 mIU/ml) and examination showed a bleeding cervical mass. An initial ... characterized by abnormal proliferation of placental trophoblasts and include .... MRI is invaluable to assess extra uterine disease spread and complications.

  2. Synchronous Epithelioid Stromal Tumour and Lipoma in the Stomach

    Directory of Open Access Journals (Sweden)

    Nabeel Al-Brahim

    2003-01-01

    Full Text Available An 82-year-old man presented with upper gastrointestinal bleeding. A polypoid lesion of the distal stomach with focal ulceration was seen at endoscopy. This was treated by a partial gastrectomy. The resected stomach contained two separate tumours near the pylorus: a gastrointestinal stromal tumour (GIST and an adjacent lipoma. The literature includes case reports of synchronously occurring GIST and adenocarcinoma, GIST and mucosa-associated lymphoid tissue lymphoma and GIST and carcinoid tumour. Herein is the first case report of two distinct mesenchymal tumors coexisting in the stomach.

  3. Perivascular epithelioid cell tumors of the uterine cervix.

    Science.gov (United States)

    Kudela, E; Biringer, K; Kasajova, P; Nachajova, M; Adamkov, M

    2016-08-01

    The World Health Organization (WHO) defines PEComas as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular cells. Uterus is the most common site of a subgroup of PEComas not otherwise specified(NOS). PEComas of the uterine cervix are extremely rare, and only thirteen cases have been described in the English literature to date. In this review, we summarize the available data concerning diagnostics, immunohistochemical analysis, genetics and treatment of cervical PEComas. Radical hysterectomy with bilateral salpingooophorectomy is the best surgical approach available. Adjuvant therapy in its present form is not efficient. Therefore, further studies are needed to evaluate the newest treatment strategies. Copyright © 2016 Elsevier GmbH. All rights reserved.

  4. Renal epithelioid angiomyolipoma presenting clinically as renal cell ...

    African Journals Online (AJOL)

    M.S. Johnson

    a Detroit Medical Center, Michigan State University School of Osteopathic Medicine, Detroit, MI, USA .... Immunohistochemically, the tumor cells stained strongly positive .... [10] Cao Q, Liu F, Xiao P, Tian X, Li B, Li Z. Coexistence of renal.

  5. Cicatriz radial/lesão esclerosante complexa: aspectos radiológicos com correlação clínica, ultra-sonográfica e anatomopatológica Radial scar/complex sclerosing lesion: radiologic features with clinical, ultrasonographic and pathologic correlation

    Directory of Open Access Journals (Sweden)

    Cristina Caetano Stefenon

    2003-03-01

    Full Text Available OBJETIVO: Estudar, retrospectivamente, as várias formas de apresentação da cicatriz radial/lesão esclerosante complexa (CR/LEC na mamografia, correlacionando-as com o exame clínico e os achados ultra-sonográficos. Os achados histopatológicos e a associação da CR/LEC com hiperplasia atípica e carcinoma são discutidos. MATERIAIS E MÉTODOS: Foi realizado estudo retrospectivo de 926 lesões impalpáveis em 901 pacientes submetidas a biópsia excisional após localização pré-cirúrgica, do arquivo do Centro de Diagnóstico por Imagem e do Hospital Santa Rita, Vitória, ES, no período de outubro de 1993 a dezembro de 2001, nas quais 57 pacientes tiveram diagnóstico histopatológico de CR/LEC. RESULTADOS: A idade variou de 31 a 84 anos (média de 49 anos. As lesões foram palpáveis em dez casos. Na mamografia, 48 casos se apresentaram como distorção arquitetural, e com a mesma freqüência o nódulo espiculado e a densidade assimétrica, quatro casos cada. As microcalcificações foram detectadas na mamografia em 14 casos e em 20 quando o espécime cirúrgico foi avaliado. A ultra-sonografia foi realizada em 51 casos, tendo expressão em 17 como área hipoecóica irregular com atenuação acústica posterior. Houve 42 casos de CR/LEC sem ou com proliferação típica, nove casos com proliferação epitelial atípica e seis casos com carcinoma infiltrativo associado. CONCLUSÃO: Não é possível fazer diagnóstico diferencial de CR/LEC com câncer pelos métodos de imagem e a biópsia excisional deve ser realizada.OBJECTIVE: To review the different types of radial scar/complex sclerosing lesion (RS/CSL seen on clinical, mammography, and ultrasound examinations. The histopathology findings and the association of RS/CSL with atypical hyperplasia and malignancy are discussed. MATERIALS AND METHODS: We performed a retrospective study of patients from the files of the "Centro de Diagnóstico por Imagem and Hospital Santa Rita" - Vit

  6. La Sclerose en Plaques en Afrique Noire (Francais) | Grunitzky ...

    African Journals Online (AJOL)

    \\'absence d\\'enquêtes de populations et de données épidémiologiques pertinentes font classer cette région du monde dans une zone ou la maladie reste probable mais exceptionnelle. Objectif Le but de ce travail est de montrer la fréquence ...

  7. Difficult preoperative diagnosis of a patient with sclerosing splenic hemangioma

    International Nuclear Information System (INIS)

    Edoute, Y.; Ben-Haim, S.A.; Ben-Arie, Y.; Fishman, A.; Barzilai, D.

    1989-01-01

    We present a young asymptomatic woman with splenomegaly and a large isolated splenic mass demonstrated by ultrasonography, 99m Tc sulfur colloid, and gallium scintigraphy studies. Computerized tomography (CT) and three-phase 99mTc-labeled red blood cell imaging suggested a malignant lesion. Repeated sonographically guided fine needle aspiration (FNA) obtained only blood, suggesting the possible vascular nature of the tumor. Splenectomy established the diagnosis of splenic hemangioma (SH) with marked sclerotic changes. We conclude from this case that (1) the sclerotic and cystic changes in the SH and the abdominal lymphadenopathy could explain why the three-phase red blood cell and CT scanning, respectively, suggested that the lesion was malignant rather than benign; (2) guided FNA of a splenic mass suspected to be hemangioma may be an additional safe and useful diagnostic procedure. Multiple aspirations yielding blood alone suggest hemangioma and may prevent an unnecessary operation. To the best of our knowledge, this is the first reported case in the literature of FNA of splenic hemangioma

  8. Sclerose laterale amyotrophique a en milieu tropical : description ...

    African Journals Online (AJOL)

    Les signes cliniques sont marqués par le syndrome pyramidal et le syndrome neurogène périphérique. L'imagerie cérébrale et médullaire (scanner, IRM) a montré une atrophie du bulbe et de la moelle cervicale. L'EMG a montré des signes d'activités spontanées et de dénervation et rénervation. Une atrophie neurogène ...

  9. Genetics Home Reference: SOST-related sclerosing bone dysplasia

    Science.gov (United States)

    ... areas. Abnormal bone growth can pinch (compress) the cranial nerves , which emerge from the brain and extend to ... of the head and neck. Compression of the cranial nerves can lead to paralyzed facial muscles (facial nerve ...

  10. The main directions of pharmacological correction of radioinduced scleroses

    International Nuclear Information System (INIS)

    Dubrovskaya, V.F.

    1991-01-01

    Results of clinical and experimental research on pharmacological correction of radiationinduced sclerosis were summarized. Efficiency of prophylaxis main trends and drug therapy was analyzed. Application of specific pharmaceuticals (preparations directly affecting certain chains of collagen metabolism) and nonspecific pharmaceuticals (preparations of indirect affect on collagen metabolism) was given. Further research of specific pharmaceuticals was shown to be expedient. Analysis of nonspecific pharmaceuticals used in complex therapy revealed problems in evaluating their efficiency at various stages of sclerosis development

  11. Enhanced photodynamic destruction of a transplantable fibrosarcoma using photochemical internalisation of gelonin

    Science.gov (United States)

    Dietze, A; Peng, Q; Selbo, P K; Kaalhus, O; Müller, C; Bown, S; Berg, K

    2005-01-01

    Photochemical internalisation (PCI) is a technique for releasing biologically active macromolecules from endocytic vesicles by light activation of a photosensitiser localised in the same vesicles of targeted cells. This study investigated the PCI of the toxin gelonin as a way of enhancing the effect of photodynamic therapy (PDT) on a human malignant fibrous histiocytoma transplanted into nude mice using the photosensitiser disulphonated aluminium phthalocyanine (AlPcS2a). Pharmacokinetic studies after intraperitoneal administration showed that the serum level of AlPcS2a fitted a biexponential model (half-lives of 1.8 and 26.7 h). The tumour concentration was roughly constant up to 48 h, although fluorescence microscopy showed that the drug location was initially mainly vascular, but became intracellular by 48 h. To compare PDT with PCI, 48 h after intraperitoneal injection of 10 mg kg−1 AlPcS2a, and 6 h after direct intratumour injection of 50 μg gelonin (PCI) or a similar volume of phosphate-buffered saline (PDT controls), tumour-bearing animals were exposed to red light (150 J cm−2). Complete response was observed for more than 100 days in 50% of the PCI tumours but only 10% of the PDT tumours (P<0.01). In tumours examined histologically 4 days after light delivery, the depth of necrosis was 3–4 mm after PDT, but 7 mm after PCI. The deeper effect after PCI demonstrates that the light fluence needed to kill tumour is less than with PDT. We conclude that PCI with gelonin can markedly enhance the effect of PDT on this type of tumour and may have a role clinically as an adjunct to surgery to control localised disease. PMID:15886704

  12. Unusual case of congenital/infantile fibrosarcoma in a new born ...

    African Journals Online (AJOL)

    We report an unusual case of CIFS occurring in new–born mimicking an hemangioma and causing hemorrhage in the neonatal period. The tumor is located in the left arm and axilla and associated with a hand malformation. A shoulder amputation is performed after chemotherapy failure. The infant is now two-years old with ...

  13. Epithelioid Myofibroblastoma in an Old-Male Breast: A Case Report with MRI Findings

    Directory of Open Access Journals (Sweden)

    Seyma Yildiz

    2015-01-01

    Full Text Available Myofibroblastoma of the breast (MFB is a very rare benign stromal tumor. In recent years, increase in mammographic screenings has resulted in increased diagnosis of MFB. Most cases are old males and postmenopausal women. MFB may be confused as malignant, clinically, morphologically, or by imaging. Immunohistochemistry is essential for final diagnosis in these cases. We report a case of a pathologically diagnosed MFB in an 80-year-old male patient who had coexisting prostate cancer and describe its imaging characteristics, especially magnetic resonance imaging (MRI. In this paper, histopathological and MRI findings of the MFB were discussed.

  14. Fusion events lead to truncation of FOS in epithelioid hemangioma of bone

    DEFF Research Database (Denmark)

    van IJzendoorn, David G P; de Jong, Danielle; Romagosa, Cleofe

    2015-01-01

    in exon 4 of the FOS gene and the fusion event led to the introduction of a stop codon. In all instances, the truncation of the FOS gene would result in the loss of the transactivation domain (TAD). Using FISH probes we found a break in the FOS gene in two additional cases, in none of these cases...... differential diagnosis of vascular tumors of bone. Our data suggest that the translocation causes truncation of the FOS protein, with loss of the TAD, which is thereby a novel mechanism involved in tumorigenesis....

  15. Prevalence of hepatobiliary dysfunction in a regional group of patients with chronic inflammatory bowel disease

    DEFF Research Database (Denmark)

    Wewer, V; Gluud, C; Schlichting, P

    1991-01-01

    A regional group of outpatients with chronic inflammatory bowel disease (ulcerative colitis, n = 396, and Crohn's disease, n = 125) was biochemically screened to estimate the prevalence of hepatobiliary dysfunction. Among the 396 patients with ulcerative colitis, 69 (17%; 95% confidence limits, 14...... primary sclerosing cholangitis, of whom two were primarily diagnosed; one patient had cholangiocarcinoma also primarily diagnosed; and two patients were found to have alcoholic hepatic damage. Among the 125 patients with Crohn's disease, 38 (30%; 95% confidence limits, 23-38%) had at least 1 abnormal...... the criteria for further evaluation as described above. One patient appeared to have epithelioid granuloma in the liver and one patient had alcoholic liver disease, whereas one patient refused further examination.(ABSTRACT TRUNCATED AT 250 WORDS)...

  16. Dendritic cell-based immunotherapy induces transient clinical response in advanced rat fibrosarcoma - comparison with preventive anti-tumour vaccination

    Czech Academy of Sciences Publication Activity Database

    Kučera, A.; Pýcha, K.; Pajer, Petr; Špíšek, R.; Škába, R.

    2009-01-01

    Roč. 55, č. 4 (2009), s. 119-125 ISSN 0015-5500 Institutional research plan: CEZ:AV0Z50520514 Keywords : dendritic cells * immunotherapy * cancer immunotherapy * chemotherapy Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 0.924, year: 2009

  17. The effect of hyperglycemia with or without hyperthermia on the radiation response of a spontaneous mouse fibrosarcoma

    International Nuclear Information System (INIS)

    Urano, M.; Todoroki, T.; Kahn, J.

    1985-01-01

    Hyperglycemia facilitates glycolysis with a resultant decrease in the tissue, particularly tumor pH. Another effect of hyperglycemia is to increase osmotic pressure in the extracellular fluid. The affect of hyperglycemia on the radiation response of our spontaneous tumor, FSa-II was examined. Further experiment includes the effect of hyperglycemia given 1 hour before local hyperthermia which was given in a 43.5 0 C water bath. Animals were C/sub 3/Hf/Sed mice from our defined flora colony. Tumor cell suspension was transplanted into the animal foot and the treatment was given when tumors reached an average diameter of 7 mm. Tumors were irradiated under clamped hypoxia, in air or under HPO (30 psi). Hyperglycemia, 5 mg/g given 1 hour before radiation, increased hypoxic cell fraction of the tumor without altering the slope fo the dose response curve. Hyperthermia enhanced the tumor response and increased hypoxic cell fraction. Further increase in the hypoxic cell fraction was noted following combined hyperglycemia and hyperthermia. Hyperthermia given 24 hours before radiation with or without glucose also increased hypoxic cell fraction, but decrease chronically hypoxic cell fraction (fraction not oxygenated under HPO)

  18. Umbelliprenin-coated Fe3O4 magnetite nanoparticles: Antiproliferation evaluation on human Fibrosarcoma cell line (HT-1080)

    International Nuclear Information System (INIS)

    Khorramizadeh, M.R.; Esmail-Nazari, Z.; Zarei-Ghaane, Z.; Shakibaie, M.; Mollazadeh-Moghaddam, K.; Iranshahi, M.; Shahverdi, A.R.

    2010-01-01

    The potential applications of Fe 3 O 4 magnetite nanoparticles (MNPs) in nanomedicine as drug delivery systems are well known. In this study we prepared umbelliprenin-coated Fe 3 O 4 MNPs and evaluated the antiproliferative effect of combination in vitro. After synthesis of Fe 3 O 4 MNPs, particles were characterized by transmission electron microscopy, energy-dispersive spectroscopy, and X-ray diffraction spectroscopy techniques. The natural candidate compound - umbelliprenin- was isolated and identified and umbelliprenin-coated Fe 3 O 4 MNPs were prepared, using precipitation method. The surface chemistry of umbelliprenin-coated Fe 3 O 4 MNPs as well as their thermal decomposition characteristics was examined using Fourier transform infrared spectroscopy and Thermogravimetric Analyzer equipment, respectively. HT-1080 cells were cultured until the logarithmic phase of growth, and MTT assay was successfully carried out to evaluate the possible cytotoxic effects of umbelliprenin-coated Fe 3 O 4 MNPs in viable cells in vitro. The results demonstrated that umbelliprenin has moderate antiproliferative effects with IC 50 value of 50 μg/mL. However, the combination of umbelliprenin and Fe 3 O 4 MNPs showed the IC 50 value of 9 μg/mL. In other words, cell proliferation decreased to the remarkably-low proportion of 45% after treating cells with umbelliprenin-coated Fe 3 O 4 MNPs. This suggests that with the aid of nanoparticles as carriers, natural products may have even broader range of medical applications in future.

  19. L\\'evaluation des troubles vesico-sphincteriens dans la sclerose en ...

    African Journals Online (AJOL)

    Our objective in this study is to examine the relations between the Urinary disorders (UD), the functional statue, the gravity of the neurological disease and the quality of life during the Multiple sclerosis (MS). Patients and methods 25 consecutive MS patients with mean age of 39 +-7,75 years were evaluated. The evaluation ...

  20. Abdominal Cocoon Syndrome (Idiopathic Sclerosing Encapsulating Peritonitis: How Easy Is Its Diagnosis Preoperatively? A Case Report

    Directory of Open Access Journals (Sweden)

    Julius A. A. Awe

    2013-01-01

    Full Text Available The abdominal cocoon syndrome (or idiopathic encapsulating peritonitis is a rare cause of intestinal obstruction. It has been reported predominantly in adolescent girls living in tropical/subtropical region in which diagnosis is only made at laparotomy in most cases. The cause and pathogenesis of the condition have not been elucidated. Prolonged administration of practalol, meconium peritonitis, and tuberculous infection of the female genital tract have been incriminated as possible causes. The author reports a case of a female patient with recurrent intestinal obstruction treated for years but failed to settle down on conservative treatment during her last hospital admission and had to undergo surgery. Preoperative diagnosis of this syndrome as the cause of her intestinal obstruction was not made until at laparotomy, when a thick fibrotic peritoneal wrapping of the bowel in a concertina-like fashion with some adhesions was found. Excision of this membrane and adhesiolysis were carried out without any need for bowel resection, and this led to relief of the obstruction and patient’s complete recovery. Awareness of this benign condition in the differential diagnosis of intestinal obstruction will result in early diagnosis and correct management and prevent unnecessary bowel resections and bad outcomes.

  1. BK virus encephalopathy and sclerosing vasculopathy in a patient with hypohidrotic ectodermal dysplasia and immunodeficiency.

    Science.gov (United States)

    Darbinyan, Armine; Major, Eugene O; Morgello, Susan; Holland, Steven; Ryschkewitsch, Caroline; Monaco, Maria Chiara; Naidich, Thomas P; Bederson, Joshua; Malaczynska, Joanna; Ye, Fei; Gordon, Ronald; Cunningham-Rundles, Charlotte; Fowkes, Mary; Tsankova, Nadejda M

    2016-07-13

    Human BK polyomavirus (BKV) is reactivated under conditions of immunosuppression leading most commonly to nephropathy or cystitis; its tropism for the brain is rare and poorly understood. We present a unique case of BKV-associated encephalopathy in a man with hypohidrotic ectodermal dysplasia and immunodeficiency (HED-ID) due to IKK-gamma (NEMO) mutation, who developed progressive neurological symptoms. Brain biopsy demonstrated polyomavirus infection of gray and white matter, with predominant involvement of cortex and distinct neuronal tropism, in addition to limited demyelination and oligodendroglial inclusions. Immunohistochemistry demonstrated polyoma T-antigen in neurons and glia, but expression of VP1 capsid protein only in glia. PCR analysis on both brain biopsy tissue and cerebrospinal fluid detected high levels of BKV DNA. Sequencing studies further identified novel BKV variant and disclosed unique rearrangements in the noncoding control region of the viral DNA (BKVN NCCR). Neuropathological analysis also demonstrated an unusual form of obliterative fibrosing vasculopathy in the subcortical white matter with abnormal lysosomal accumulations, possibly related to the patient's underlying ectodermal dysplasia. Our report provides the first neuropathological description of HED-ID due to NEMO mutation, and expands the diversity of neurological presentations of BKV infection in brain, underscoring the importance of its consideration in immunodeficient patients with unexplained encephalopathy. We also document novel BKVN NCCR rearrangements that may be associated with the unique neuronal tropism in this patient.

  2. Risk factors and prognosis for recurrent primary sclerosing cholangitis after liver transplantation

    DEFF Research Database (Denmark)

    Lindström, Lina; Jørgensen, Kristin K; Boberg, Kirsten M

    2018-01-01

    PSC for prognosis. MATERIALS AND METHODS: All liver transplanted PSC patients in the Nordic countries between 1984 and 2007 (n = 440), identified by the Nordic Liver Transplant Registry, were studied. Data were retrieved from patients' chart reviews. Multivariable Cox regression models were used to calculate risk...

  3. Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    de Vries, Elisabeth M G; de Krijger, Manon; Färkkilä, Martti

    2017-01-01

    across a multicenter PSC cohort. Liver biopsies from PSC patients were collected from seven European institutions. Histologic scoring was performed using the Nakanuma, Ishak, and Ludwig scoring systems. Biopsies were independently scored by six liver pathologists for interobserver agreement.......19-5.80] for endpoint 2 and HR, 2.06 [95% CI, 1.09-3.89] for endpoint 3). Only the Nakanuma staging system was independently associated with endpoint 1: HR, 2.14 (95% CI, 1.22-3.77). Interobserver agreement was moderate for Nakanuma stage (κ = 0.56) and substantial for Nakanuma component fibrosis (κ = 0.67), Ishak...

  4. OK-432 as a sclerosing agent to treat wound-healing impairment.

    Science.gov (United States)

    Fasching, G; Sinzig, M

    2007-12-01

    We report on the application of OK-432 (picibanil) in a patient with prolonged wound healing impairment. A 13-year-old girl had suffered a polytrauma with a displaced fracture of the sacrum which required neurosurgical decompression of the sacral plexus. Postoperatively, a seroma with recurrent fistulation was seen. Excision of the wound, prolonged suction drainage and the instillation of hypertonic glucose solution did not have any effect over a period of four months postoperatively. Relying on our personal experience of the treatment of lymphangiomas using OK-432 we instilled OK-432 into the wound. Leakage stopped immediately, there was a regression of fluid accumulation and four weeks later the ultrasound examination was normal. The patient is still asymptomatic four years after treatment. OK-432 can be used effectively for the treatment of chronic wound healing impairment.

  5. Remitting - Relapsing Subacute Sclerosing Panencephalitis (SSPE : Case Description And Mini Review

    Directory of Open Access Journals (Sweden)

    Kamath V

    2005-01-01

    Full Text Available Spontaneous remission for a substantial period is a rare feature of SSPE. We report a 21 year old boy who was diagnosed to have SSPE at the age of 11 years, based on clinical, CSF and electroencephalographic features. Over a period of one year, he gradually deteriorated to a bedridden state, being dependent for all activities of daily living. He showed a substantial spontaneous remission in clinical and electroencephalographic features and maintained this till 18 years of age. He however slowly deteriorated after prolonged remission over three years. A combination of clinical improvement and disappearance of periodic complexes with normalization of the background activity in the EEG may indicate long-term survival.

  6. Case report 446: Multicentric, metachronous, low-grade, sclerosing osteogenic sarcoma

    International Nuclear Information System (INIS)

    McCarthy, E.F.; Tolo, V.T.; Dorfman, H.D.

    1987-01-01

    This case of multicentric osteosarcoma has overlying features of the clinical subgroups described by Amstutz and Mahoney. This 38-year-old woman has survived 6 years with metachronous multifocal osteosarcomas. She presently has at least seven sites of involvement. The lesions are all densely sclerotic and are confined to the axial skeleton. She has had no treatment except for release of compression syndromes. She has no evidence of pulmonary or other visceral metastases. The low grade nature of this type of multifocal osteosarcoma is confirmed by a six year survival in the absence of ablative surgery, radiotherapy or chemotherapy. (orig.)

  7. Expression of hepatic transporters OATP-C and MRP2 in primary sclerosing cholangitis

    NARCIS (Netherlands)

    Oswald, M.; Kullak-Ublick, G. A.; Paumgartner, G.; Beuers, U.

    2001-01-01

    In chronic cholestatic liver diseases, biliary excretion of organic anions from blood into bile is impaired. The aim of this study was to identify the underlying mechanism. Expression of the basolateral organic anion transporting polypeptide OATP-C (SLC21A6) and the canalicular multidrug resistance

  8. Characterization of breast precancerous lesions and myoepithelial hyperplasia in sclerosing adenosis with apocrine metaplasia

    DEFF Research Database (Denmark)

    Celis, J.E.; Gromova, I.; Cabezón, T.

    2007-01-01

    ) with apocrine metaplasia. The latter is a benign proliferative lesion of the breast that exhibits an increase in the size of the TDLUs and characterized by retained two-cell lining, and myoepithelial (ME) and stromal hyperplasia. SA with apocrine metaplasia, i.e. apocrine adenosis (AA), presents with a higher...

  9. Distinct gut microbiota profiles in patients with primary sclerosing cholangitis and ulcerative colitis

    Czech Academy of Sciences Publication Activity Database

    Bajer, L.; Kverka, Miloslav; Kostovčík, Martin; Macinga, P.; Dvořák, Jiří; Stehlíková, Zuzana; Březina, J.; Wohl, P.; Špičák, J.; Drastich, P.

    2017-01-01

    Roč. 23, č. 25 (2017), s. 4548-4558 ISSN 1007-9327 R&D Projects: GA MZd(CZ) NV15-28064A Institutional support: RVO:61388971 Keywords : Dysbiosis * Inflammatory bowel disease * Ulcerative colitis Subject RIV: EE - Microbiology, Virology OBOR OECD: Microbiology Impact factor: 3.365, year: 2016

  10. Distinct gut microbiota profiles in patients with primary sclerosing cholangitis and ulcerative colitis

    Czech Academy of Sciences Publication Activity Database

    Bajer, L.; Kverka, Miloslav; Kostovčík, M.; Macinga, P.; Dvořák, J.; Stehlíková, Z.; Březina, J.; Wohl, P.; Špičák, J.; Drastich, P.

    2017-01-01

    Roč. 23, č. 25 (2017), s. 4548-4558 ISSN 1007-9327 R&D Projects: GA MZd(CZ) NV15-28064A Institutional support: RVO:68378041 Keywords : dysbiosis * inflammatory bowel disease * ulcerative colitis Subject RIV: FP - Other Medical Disciplines OBOR OECD: Gastroenterology and hepatology Impact factor: 3.365, year: 2016

  11. Epithelioid malignant mesothelioma of tunica vaginalis with deciduoid features: An unusual malignancy clinically masquerading an inguinal hernia

    Directory of Open Access Journals (Sweden)

    Sharique Ahmed

    2012-01-01

    Full Text Available Paratesticular/scrotal and inguinal canal mass lesions in elderly patients may pose a diagnostic challenge to both the surgeon as well as the pathologist. In most cases, these represent hernial sacs with their contents, and true neoplasms like lipomas, rhabdomyosarcomas, and fibrous pseudotumors are infrequent. Malignant mesotheliomas arising from the tunica layers are rare cause of inguinal and paratesticular tumors. Herein, we report a case of an elderly patient who presented with an inguinal hernia which pathologically had features of deciduoid malignant mesothelioma.

  12. The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification.

    Science.gov (United States)

    Travis, William D; Brambilla, Elisabeth; Nicholson, Andrew G; Yatabe, Yasushi; Austin, John H M; Beasley, Mary Beth; Chirieac, Lucian R; Dacic, Sanja; Duhig, Edwina; Flieder, Douglas B; Geisinger, Kim; Hirsch, Fred R; Ishikawa, Yuichi; Kerr, Keith M; Noguchi, Masayuki; Pelosi, Giuseppe; Powell, Charles A; Tsao, Ming Sound; Wistuba, Ignacio

    2015-09-01

    The 2015 World Health Organization (WHO) Classification of Tumors of the Lung, Pleura, Thymus and Heart has just been published with numerous important changes from the 2004 WHO classification. The most significant changes in this edition involve (1) use of immunohistochemistry throughout the classification, (2) a new emphasis on genetic studies, in particular, integration of molecular testing to help personalize treatment strategies for advanced lung cancer patients, (3) a new classification for small biopsies and cytology similar to that proposed in the 2011 Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification, (4) a completely different approach to lung adenocarcinoma as proposed by the 2011 Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification, (5) restricting the diagnosis of large cell carcinoma only to resected tumors that lack any clear morphologic or immunohistochemical differentiation with reclassification of the remaining former large cell carcinoma subtypes into different categories, (6) reclassifying squamous cell carcinomas into keratinizing, nonkeratinizing, and basaloid subtypes with the nonkeratinizing tumors requiring immunohistochemistry proof of squamous differentiation, (7) grouping of neuroendocrine tumors together in one category, (8) adding NUT carcinoma, (9) changing the term sclerosing hemangioma to sclerosing pneumocytoma, (10) changing the name hamartoma to "pulmonary hamartoma," (11) creating a group of PEComatous tumors that include (a) lymphangioleiomyomatosis, (b) PEComa, benign (with clear cell tumor as a variant) and (c) PEComa, malignant, (12) introducing the entity pulmonary myxoid sarcoma with an EWSR1-CREB1 translocation, (13) adding the entities myoepithelioma and myoepithelial carcinomas, which can show EWSR1 gene rearrangements, (14) recognition of usefulness of WWTR1-CAMTA1 fusions in diagnosis of epithelioid

  13. Usefulness of [18F]FDG-PET in diagnosis of bone and soft tissue tumors. Study with multi-center survey by questionnaire

    International Nuclear Information System (INIS)

    Kato, Katsuhiko; Hosono, Makoto; Okada, Masahiro; Komeya, Yoshihiro; Im, Sung-Woon; Tsuchiya, Norio; Ito, Kengo; Torizuka, Kanji

    2008-01-01

    In the aspect of future additional approval of [ 18 F]fluorodeoxyglucose positron emission tomography (FDG-PET) diagnosis of the title tumors in the health insurance, its usefulness was studied by questionnaire to 18 facilities, where PET had been conducted for those tumors in the period July, 2005-February, 2006. Major questions concerned the purpose and finding of PET, findings by other imaging and by tumor markers, and judgment of PET effectiveness compared with other imaging (more useful, equally or less, and its reason). Subjects were 75 cases (42 males, 33 females; 3-82 years old) in 20 diseases, which involved 21 cases of osteosarcoma, 7 of leiomyosarcoma, 8 of Ewing sarcoma, 6 of liposarcoma, 5 of hemangiosarcoma, 4 of synovial sarcoma, each 3 of rhabdomyosarcoma, giant cell tumor, Schwannoma, malignant fibrous histiocytoma, each 2 of chondrosarcoma, alveolar soft part sarcoma, each one of epithelioid sarcoma, endometrial storomal sarcoma, hibernoma, fibrosarcoma, multiple osteochondroma, sacral chondroma, Langerhans cell histiocytosis and neurofibromatosis. Obtained were the judgments of highly useful in 5 diseases, fairly useful in 4, useful in 3, and useful/inconclusive due to the only one case in 8. FDG-PET was thus found useful in all diseases examined. (R.T.)

  14. Immunohistochemical Examination of a Resected Advanced Hilar Cholangiocarcinoma Arising in a 29-Year-Old Male without Primary Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    Taketoshi Suehiro

    2010-05-01

    Full Text Available A 29-year-old man with advanced hilar cholangiocarcinoma was successfully treated with an extended right lobectomy. The carbohydrate antigen 19-9 (CA19-9 level was elevated to 939 IU/l, and the pathological findings revealed moderately differentiated tubular adenocarcinoma which involved almost the entire thickness of the hepatic duct and the adjacent liver tissue (T3 and which was associated with lymph node metastasis (N1. It was a stage IIB (T3N1M0 tubular adenocarcinoma according to UICC pathological staging. Immunohistochemical examination revealed that Ki-67, cyclin D1, and MMP-7 were positive, and 14-3-3σ and p27 were negative. The pathological and immunohistochemical findings indicated high malignant potential indicating poor prognosis. We administrated the postoperative adjunct gemcitabine combined with S-1 chemotherapy. The patient is alive without recurrence and doing well two years after surgery. We also review other reports of cholangiocarcinoma patients aged less than 30 years.

  15. Correlations between neurological signs and brain MR images of patients in consecutive stages of subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Kulczycki, J.; Kryst-Widzgowska, T.; Sobczyk, W.; Bochynska, A.; Pilkowska, E.; Milewska, D. [Pracownia Rezonansu Magnetycznego, Inst. Psychiatrii i Neurologii, Warsaw (Poland)

    1994-12-31

    Correlations between neurological symptoms and brain MRI changes during the course of SSPE in 10 patients were studied. Visual agnosia and mental regression - very frequent symptoms at the early stages of the disease were caused by nearly symmetrical, focal involvement of the occipital and frontal white matter in all cases. Pyramidal and extrapyramidal disturbances reflected rather diffuse lesions of both cerebral hemispheres in late second stage of the illness. In cases with clinical improvement the brain changes disclosed an entire stability, but not diminishing intensity. (author) 9 refs, 8 figs, 3 tabs

  16. Biomarkers in premalignant conditions of the gastrointestinal tract: Studies on Barrett’s esophagus and primary sclerosing cholangitis

    NARCIS (Netherlands)

    Timmer, M.R.

    2016-01-01

    In this thesis we have described our research on genetic abnormalities in (pre)malignant conditions of the gastrointestinal tract. The wide variation in biological behavior of cancerous and precancerous conditions may be largely explained by differences in genetic abnormalities. They are a source of

  17. Mapping chronic liver disease questionnaire scores onto SF-6D utility values in patients with primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Kalaitzakis, Evangelos; Benito de Valle, Maria; Rahman, Monira

    2016-01-01

    regression analyses were employed to devise a mapping function predicting utilities. This was validated in three random subsamples of the cohort and in a separate sample of PSC patients following liver transplantation. Adjusted R2 and root-mean-square error (RMSE) as well as Pearson’s r coefficients and mean...

  18. Irradiation of salivary glands in the amyotrophic lateral sclerosis; Irradiation des glandes salivaires dans la sclerose laterale amyotrophique

    Energy Technology Data Exchange (ETDEWEB)

    Bourry, N.; Lapeyre, M.; Tortochaux, J.; Gilliot, O.; Achard, J.L.; Verrelle, P. [Centre Jean-Perrin, Dept. de Radiotherapie 63 - Clermont-Ferrand (France); Clavelou, P.; Rouvet, S. [CHU Gabriel-Montpied, Service de Neurologie, 63 - Clermont-Ferrand (France)

    2006-11-15

    The irradiation of salivary glands in the amyotrophic lateral sclerosis is efficient. A dose about 20 Gy in five seances delivered by electrons seems a correct compromise between efficiency and toxicity. (N.C.)

  19. Deposition of C3, the terminal complement complex and vitronectin in primary biliary cirrhosis and primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Garred, P; Lyon, H; Christoffersen, P

    1993-01-01

    -dependent cytotoxic mechanisms in the pathogenesis. Therefore, we investigated liver biopsy specimens from 21 patients with PBC, six patients with PSC and six controls for complement deposits by immunohistochemistry using polyclonal and monoclonal antibodies against C3d, the terminal complement complex (TCC......) and vitronectin (S-protein). We found C3d, TCC and vitronectin deposits only in the portal tracts. C3d and TCC were present in the walls of the hepatic arteries and in the connective tissue stroma but never around the bile ducts. We found vitronectin deposits throughout the connective tissue, often independent...... of the TCC deposits. When vitronectin and TCC were co-localized, the staining patterns were inverse; that is, intense staining for TCC accompanied weak staining for vitronectin and vice versa. Occasionally complete dissociation between TCC and vitronectin staining was observed. Deposits of TCC...

  20. Correlations between neurological signs and brain MR images of patients in consecutive stages of subacute sclerosing panencephalitis

    International Nuclear Information System (INIS)

    Kulczycki, J.; Kryst-Widzgowska, T.; Sobczyk, W.; Bochynska, A.; Pilkowska, E.; Milewska, D.

    1994-01-01

    Correlations between neurological symptoms and brain MRI changes during the course of SSPE in 10 patients were studied. Visual agnosia and mental regression - very frequent symptoms at the early stages of the disease were caused by nearly symmetrical, focal involvement of the occipital and frontal white matter in all cases. Pyramidal and extrapyramidal disturbances reflected rather diffuse lesions of both cerebral hemispheres in late second stage of the illness. In cases with clinical improvement the brain changes disclosed an entire stability, but not diminishing intensity. (author)

  1. Diagnostic Dilemma in a Patient with Jaundice: How to Differentiate between Autoimmune Pancreatitis, Primary Sclerosing Cholangitis and Pancreas Carcinoma

    Directory of Open Access Journals (Sweden)

    Matthias Buechter

    2012-04-01

    Full Text Available A 68-year-old male patient was referred to our institution in May 2011 for a suspected tumor in the pancreatic head with consecutive jaundice. Using magnetic resonance imaging, further differentiation between chronic inflammation and a malignant process was not possible with certainty. Apart from cholestasis, laboratory studies showed increased values for CA 19-9 to 532 U/ml (normal <37 U/ml and hypergammaglobulinemia (immunoglobulin G, IgG of 19.3% (normal 8.0–15.8% with an elevation of the IgG4 subtype to 2,350 mg/l (normal 52–1,250 mg/l. Endoscopic retrograde cholangiopancreatography revealed a prominent stenosis of the distal ductus hepaticus communis caused by pancreatic head swelling and also a bihilar stenosis of the main hepatic bile ducts. Cytology demonstrated inflammatory cells without evidence of malignancy. Under suspicion of autoimmune pancreatitis with IgG4-associated cholangitis, immunosuppressive therapy with steroids and azathioprine was started. Follow-up endoscopic retrograde cholangiopancreatography after 3 months displayed regressive development of the diverse stenoses. Jaundice had disappeared and blood values had returned to normal ranges. Moreover, no tumor of the pancreatic head was present in the magnetic resonance control images. Due to clinical and radiological similarities but a consecutive completely different prognosis and therapy, it is of fundamental importance to differentiate between pancreatic cancer and autoimmune pancreatitis. Especially, determination of serum IgG4 levels and associated bile duct lesions induced by inflammation should clarify the diagnosis of autoimmune pancreatitis and legitimate immunosuppressive therapy.

  2. Use of Molecular Imaging Markers of Glycolysis, Hypoxia and Proliferation (18F-FDG, 64Cu-ATSM and 18F-FLT) in a Dog with Fibrosarcoma

    DEFF Research Database (Denmark)

    Zornhagen, Kamilla; Clausen, Malene; Hansen, Anders Elias

    2015-01-01

    Glycolysis, hypoxia, and proliferation are important factors in the tumor microenvironment contributing to treatment-resistant aggressiveness. Imaging these factors using combined functional positron emission tomography and computed tomography can potentially guide diagnosis and management...

  3. Distribution of Glutathione-Stabilized Gold Nanoparticles in Feline Fibrosarcomas and Their Role as a Drug Delivery System for Doxorubicin—Preclinical Studies in a Murine Model

    Directory of Open Access Journals (Sweden)

    Katarzyna Zabielska-Koczywąs

    2018-03-01

    Full Text Available Feline injection site sarcomas (FISS are malignant skin tumors with high recurrence rates despite the primary treatment of radical surgical resections. Adjunctive radiotherapy or chemotherapy with doxorubicin is mostly ineffective. Cellular and molecular causes of multidrug resistance, specific physio-chemical properties of solid tumors impairing drug transport, and the tumor microenvironment have been indicated for causing standard chemotherapy failure. Gold nanoparticles are promising imaging tools, nanotherapeutics, and drug delivery systems (DDS for chemotherapeutics, improving drug transport within solid tumors. This study was conducted to assess the distribution of 4-nm glutathione-stabilized gold nanoparticles in FISS and their influence on kidney and liver parameters in nude mice. The role of gold nanoparticles as a doxorubicin DDS in FISS was examined to determine the potential reasons for failure to translate results from in vitro to in vivo studies. Grade III tumors characterized by a large area of necrosis at their core displayed positive immuneexpression of tumor-associated macrophages (TAM at both the periphery and within the tumor core near the area of necrosis. Gold nanoparticles did not cause necrosis at the injection site and had no negative effect on liver and kidney parameters in nude mice. Gold nanoparticles accumulated in the tumor core and at the periphery and co-internalized with TAM—an important observation and potential therapeutic target warranting further investigation. The large area of necrosis and high immunoexpression of TAM, indicating “pro-tumor macrophages”, may be responsible for FISS tumor progression and therapeutic failure. However, further studies are required to test this hypothesis.

  4. In Silico and In Vitro Analyses of LncRNAs as Potential Regulators in the Transition from the Epithelioid to Sarcomatoid Histotype of Malignant Pleural Mesothelioma (MPM).

    Science.gov (United States)

    Singh, Anand S; Heery, Richard; Gray, Steven G

    2018-04-26

    Malignant pleural mesothelioma (MPM) is a rare malignancy, with extremely poor survival rates. At present, treatment options are limited, with no second line chemotherapy for those who fail first line therapy. Extensive efforts are ongoing in a bid to characterise the underlying molecular mechanisms of mesothelioma. Recent research has determined that between 70⁻90% of our genome is transcribed. As only 2% of our genome is protein coding, the roles of the remaining proportion of non-coding RNA in biological processes has many applications, including roles in carcinogenesis and epithelial⁻mesenchymal transition (EMT), a process thought to play important roles in MPM pathogenesis. Non-coding RNAs can be separated loosely into two subtypes, short non-coding RNAs (200 nucleotides). A significant body of evidence has emerged for the roles of short non-coding RNAs in MPM. Less is known about the roles of long non-coding RNAs (lncRNAs) in this disease setting. LncRNAs have been shown to play diverse roles in EMT, and it has been suggested that EMT may play a role in the aggressiveness of MPM histological subsets. In this report, using both in vitro analyses on mesothelioma patient material and in silico analyses of existing RNA datasets, we posit that various lncRNAs may play important roles in EMT within MPM, and we review the current literature regarding these lncRNAs with respect to both EMT and MPM.

  5. In Silico and In Vitro Analyses of LncRNAs as Potential Regulators in the Transition from the Epithelioid to Sarcomatoid Histotype of Malignant Pleural Mesothelioma (MPM

    Directory of Open Access Journals (Sweden)

    Anand S. Singh

    2018-04-01

    Full Text Available Malignant pleural mesothelioma (MPM is a rare malignancy, with extremely poor survival rates. At present, treatment options are limited, with no second line chemotherapy for those who fail first line therapy. Extensive efforts are ongoing in a bid to characterise the underlying molecular mechanisms of mesothelioma. Recent research has determined that between 70–90% of our genome is transcribed. As only 2% of our genome is protein coding, the roles of the remaining proportion of non-coding RNA in biological processes has many applications, including roles in carcinogenesis and epithelial–mesenchymal transition (EMT, a process thought to play important roles in MPM pathogenesis. Non-coding RNAs can be separated loosely into two subtypes, short non-coding RNAs (<200 nucleotides or long (>200 nucleotides. A significant body of evidence has emerged for the roles of short non-coding RNAs in MPM. Less is known about the roles of long non-coding RNAs (lncRNAs in this disease setting. LncRNAs have been shown to play diverse roles in EMT, and it has been suggested that EMT may play a role in the aggressiveness of MPM histological subsets. In this report, using both in vitro analyses on mesothelioma patient material and in silico analyses of existing RNA datasets, we posit that various lncRNAs may play important roles in EMT within MPM, and we review the current literature regarding these lncRNAs with respect to both EMT and MPM.

  6. The role of syndecan-1 in cellular signaling and its effects on heparan sulfate biosynthesis in mesenchymal tumors

    Directory of Open Access Journals (Sweden)

    Tünde eSzatmári

    2013-12-01

    Full Text Available Proteoglycans and in particular the syndecans are involved in the differentiation process across the epithelial-mesenchymal axis, principally through their ability to bind growth factors and modulate their downstream signalling. Malignant tumors have individual proteoglycan profiles, which are closely associated with their differentiation and biological behavior, mesenchymal tumors showing a different profile from that of epithelial tumors. Syndecan-1 is the main syndecan of epithelial malignancies, whereas in sarcomas its expression level is generally low, in accordance with their mesenchymal phenotype and highly malignant behaviour. This proteoglycan is often overexpressed in adenocarcinoma cells, whereas mesothelioma and fibrosarcoma cells express syndecan-2 and syndecan-4 more abundantly. Increased expression of syndecan-1 in mesenchymal tumors changes the tumor cell morphology to an epithelioid direction whereas downregulation results in a change in shape from polygonal to spindle-like morphology. Although syndecan-1 plays major roles on the cell surface, there are also intracellular functions, which are not very well studied. On the functional level, syndecan-1 affects mesenchymal tumor cell proliferation, adhesion, migration and motility, and the effect varies with the different domains of the core protein. Syndecan-1 may exert stimulatory or inhibitory effects, depending on the concentration of various mitogens, enzymes and signalling molecules, the ratio between the shed and membrane-associated syndecan-1 and histological grade of the tumour. Growth factor signaling seems to be delicately controlled by regulatory loops involving the syndecan expression levels and their sulfation patterns. Overexpression of syndecan-1 modulates the biosynthesis and sulfation of heparan sulfate and it also affects the expression of other proteoglycans. On transcriptomic level, syndecan-1 modulation results in profound effects on genes involved in

  7. Ablation of tumor and inflammatory tissue with absolute ethanol

    Energy Technology Data Exchange (ETDEWEB)

    Uflacker, R.; Paolini, R.M.; Nobrega, M.

    Absolute ethanol was used to ablate tumors, inflammatory lesions, and end-stage nephrosclerotic kidneys in 38 patients. Thirty patients had various types of renal tumors, and 3 had chronic end-stage renal failure with malignant hypertension. One patient had a fibrosarcoma of the right leg and one had a metastatis in the humerus from a renal carcinoma. A large adrenal carcinoma was treated with absolute ethanol in a patient who had liver metastases that were ablated one year after the first procedure. An additional patient had metastatic liver disease from a non-functioning adrenal carcinoma. The remaining patient had an extensive hypervascular inflammatory lesion (tuberculosis and aspergilloma) of the right upper pulmonary lobe. In addition to ethanol, coils were introduced in one patient and Gelfoam in another. The amount of ethanol used ranged from 5 to 50 ml. Twenty-two patients suffered from considerable transient pain during ethanol injection, but sedation was necessary in only 3 of them. Skin necrosis appeared in 2 patients requiring plastic reconstruction in one of them. Two patients died within 5 days of the procedure unrelated to the ablation. Two patients presented upper gastrointestinal bleeding within 2 days of the ethanol injection and one of these died in acute renal failure. One patient suffered from left colonic infarction after left renal tumor ablation, but survived for several months. Absolute ethanol was a useful and efficient sclerosing agent causing extensive tumor destruction and marked reduction of the vascularity in tumor and inflammatory lesions, but caused an 18% complication rate.

  8. The Level of Testosterone, Vitamin D, and Irregular Menstruation More Important than Omega-3 in Non-Symptomatic Women Will Define the Fate of Multiple Scleroses in Future.

    Science.gov (United States)

    Tavakol, Shima; Shakibapour, Sahar; Bidgoli, Sepideh Arbabi

    2018-01-01

    Multiple sclerosis is one of the most salient degenerative disorders of CNS with dysregulated immune process that resulted in axonal damage and demyelination. In the present investigation, the serum level of testosterone was assessed in women who were struggling with multiple sclerosis (MS). Also, the level of omega-3, vitamin D, and the irregular menstruation in women 5 years before the onset MS symptoms were surveyed. Although the levels of omega-3 and vitamin D in women MS patients were non-significant and significantly less than the healthy ones, they were significantly less in the whole population of MS patients. However, the MS patients more experienced more irregular menstruation some years before the onset of MS with the low level of testosterone. Based on the presented findings, it might be said that the vitamin D intake has significant protective role in women and men MS patients unlike the omega-3 that had significant protective role just in men. However, vitamin D metabolism encoding genes of CYP27B1 and CYP24A1 and predicting MS risk gene of HLA-DRB1*15:01 define its fate as well. Besides, vitamin D intake, through the proliferation decrement of pro-inflammatory cells, decreases of pro-inflammatory markers (IL-6, TNF-α, INF-γ) and auto-immune pathways have potential role in recovery of irregular menstruation in women with the low level of testosterone as a red warning factor of MS development. The low level of testosterone and vitamin D consumption increase the neural damage and pro-inflammatory pathways in MS patients, and the difference among the investigations is related to the long-standing history of MS that influences severity of damage to the neural cells and biomolecules and complicate its recovery.

  9. Vascular plug-assisted retrograde transvenous obliteration for the management of gastric varices: Comparative effectiveness between gelatin sponge embolization and permanent sclerosant

    International Nuclear Information System (INIS)

    Lee, Ji Hyun; Jo, Jeong Hyun; Park, Jae Hyung; Park, Byeong Ho; Jung, Gyoo Sik

    2016-01-01

    To evaluate the short-term outcome of plug-assisted retrograde transvenous obliteration (PARTO) using vascular plugs and gelatin sponges in comparison with balloon-occluded retrograde transvenous obliteration (BRTO) for the management of gastric varices. From January 2005 to October 2014, 171 patients were referred for management of gastric varices, of which, 52 patients with hemodynamically stable gastric varices (48 recent bleeding; 4 primary prophylaxes) were evaluated. Of these, 38 received BRTO (men/women 23/15; mean age 61.3; Child-Pugh classes A/B/C = 11/25/2) and 14 underwent PARTO (men/women 11/3; mean age 63.4; Child-Pugh classes A/B/C = 9/4/1). The technical success rate, complications, variceal changes, liver function, and exacerbation of ascites/pleural effusion were compared between the 2 groups within 3 months after the procedure. The technical success rates were 92.1% in the BRTO and 100% in the PARTO group. Procedure-related early complications occurred in the BRTO group alone (8%, n = 3). Among patients with technical success, follow-up CT at 1 month was available for 98% (n = 48/49). Complete thrombosis of gastric varices was achieved in 97.1% in the BRTO and 100% in the PARTO group. Worsening of esophageal varices was observed in 24% of the BRTO group alone (n = 8). The albumin level increased significantly in both groups and aspartate aminotransferase/alanine aminotransferase level improved significantly in the PARTO group (p < 0.05). Exacerbation of ascites/pleural effusion was observed in both groups (35.2% vs. 21.4%, both p > 0.05). PARTO appears to be equivalent to BRTO for short-term management of gastric varices

  10. Angiosarcoma arising in the non-operated, sclerosing breast after primary irradiation, surviving 6 years post-resection: A case report and review of the Japanese literature

    Directory of Open Access Journals (Sweden)

    Takaaki Ito

    2016-01-01

    Conclusion: Angiosarcoma may occur in the non-operated breast, post irradiation. The potential difficulties of diagnosing angiosarcoma against background fibrosis should be kept in mind. Initial radical surgery currently represents the only effective treatment for improving survival in these patients.

  11. Vascular plug-assisted retrograde transvenous obliteration for the management of gastric varices: Comparative effectiveness between gelatin sponge embolization and permanent sclerosant

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ji Hyun; Jo, Jeong Hyun; Park, Jae Hyung; Park, Byeong Ho [Dept. of Radiology, Dong A University Hospital, Dong A University College of Medicine, Busan (Korea, Republic of); Jung, Gyoo Sik [Dept. of Radiology, Gospel Hospital, Kosin University College of Medicine, Busan (Korea, Republic of)

    2016-08-15

    To evaluate the short-term outcome of plug-assisted retrograde transvenous obliteration (PARTO) using vascular plugs and gelatin sponges in comparison with balloon-occluded retrograde transvenous obliteration (BRTO) for the management of gastric varices. From January 2005 to October 2014, 171 patients were referred for management of gastric varices, of which, 52 patients with hemodynamically stable gastric varices (48 recent bleeding; 4 primary prophylaxes) were evaluated. Of these, 38 received BRTO (men/women 23/15; mean age 61.3; Child-Pugh classes A/B/C = 11/25/2) and 14 underwent PARTO (men/women 11/3; mean age 63.4; Child-Pugh classes A/B/C = 9/4/1). The technical success rate, complications, variceal changes, liver function, and exacerbation of ascites/pleural effusion were compared between the 2 groups within 3 months after the procedure. The technical success rates were 92.1% in the BRTO and 100% in the PARTO group. Procedure-related early complications occurred in the BRTO group alone (8%, n = 3). Among patients with technical success, follow-up CT at 1 month was available for 98% (n = 48/49). Complete thrombosis of gastric varices was achieved in 97.1% in the BRTO and 100% in the PARTO group. Worsening of esophageal varices was observed in 24% of the BRTO group alone (n = 8). The albumin level increased significantly in both groups and aspartate aminotransferase/alanine aminotransferase level improved significantly in the PARTO group (p < 0.05). Exacerbation of ascites/pleural effusion was observed in both groups (35.2% vs. 21.4%, both p > 0.05). PARTO appears to be equivalent to BRTO for short-term management of gastric varices.

  12. Guidelines for cytopathologic diagnosis of epithelioid and mixed type malignant mesothelioma. Complementary statement from the International Mesothelioma Interest Group, also endorsed by the International Academy of Cytology and the Papanicolaou Society of Cytopathology

    Science.gov (United States)

    Hjerpe, Anders; Ascoli, Valeria; Bedrossian, Carlos; Boon, Mathilde; Creaney, Jenette; Davidson, Ben; Dejmek, Annika; Dobra, Katalin; Fassina, Ambrogio; Field, Andrew; Firat, Pinar; Kamei, Toshiaki; Kobayashi, Tadao; Michael, Claire W.; Önder, Sevgen; Segal, Amanda; Vielh, Philippe

    2015-01-01

    To provide practical guidelines for the cytopathologic diagnosis of malignant mesothelioma (MM). Cytopathologists involved in the International Mesothelioma Interest Group (IMIG) and the International Academy of Cytology (IAC), who have an interest in the field contributed to this update. Reference material includes peer-reviewed publications and textbooks. This article is the result of discussions during and after the IMIG 2012 conference in Boston, followed by thorough discussions during the 2013 IAC meeting in Paris. Additional contributions have been obtained from cytopathologists and scientists, who could not attend these meetings, with final discussions and input during the IMIG 2014 conference in cape town. During the previous IMIG biennial meetings, thorough discussions have resulted in published guidelines for the pathologic diagnosis of MM. However, previous recommendations have stated that the diagnosis of MM should be based on histological material only.[12] Accumulating evidence now indicates that the cytological diagnosis of MM supported by ancillary techniques is as reliable as that based on histopathology, although the sensitivity with cytology may be somewhat lower.[345] Recognizing that noninvasive diagnostic modalities benefit both the patient and the health system, future recommendations should include cytology as an accepted method for the diagnosis of this malignancy.[67] The article describes the consensus of opinions of the authors on how cytology together with ancillary testing can be used to establish a reliable diagnosis of MM. PMID:26681974

  13. RGDS- and TAT-conjugated upconversion of NaYF4:Yb3+/Er3+&SiO2 nanoparticles: in vitro human epithelioid cervix carcinoma cellular uptake, imaging, and targeting

    Czech Academy of Sciences Publication Activity Database

    Kostiv, Uliana; Kotelnikov, Ilya; Proks, Vladimír; Šlouf, Miroslav; Kučka, Jan; Engstová, Hana; Ježek, Petr; Horák, Daniel

    2016-01-01

    Roč. 8, č. 31 (2016), s. 20422-20431 ISSN 1944-8244 R&D Projects: GA ČR(CZ) GA15-01897S; GA ČR(CZ) GA16-02702S Institutional support: RVO:61389013 ; RVO:67985823 Keywords : upconversion, nanoparticles * RGDS peptide * TAT peptide Subject RIV: CD - Macromolecular Chemistry; BO - Biophysics (FGU-C) Impact factor: 7.504, year: 2016

  14. Significado prognóstico dos graus histológicos do linfoma de Hodgkin do tipo esclerose nodular Prognostic significance of histopathological grading of nodular sclerosing Hodgkin’s lymphoma

    Directory of Open Access Journals (Sweden)

    Luís Fernando Pracchia

    2005-10-01

    Full Text Available INTRODUÇÃO: A esclerose nodular (EN, do tipo histológico freqüente do linfoma de Hodgkin (LH, apresenta grande variabilidade em sua composição celular. Na década de 80, pesquisadores do British National Lymphoma Investigation (BNLI propuseram uma subclassificação histológica do LH EN. Eles identificaram dois graus histológicos - o LH EN grau I (LH EN I e o LH EN grau II (LH EN II - e demonstraram que os portadores de LH EN II apresentavam menor sobrevida em comparação aos portadores de LH EN I. Outros estudos, entretanto, não reproduziram esses achados. OBJETIVO: Avaliar o significado prognóstico da graduação histológica proposta pelo BNLI. MATERIAIS E MÉTODOS: Estudo retrospectivo que incluiu 69 casos de LH EN. Mais de 90% dos casos foram tratados com terapia combinada ou quimioterapia exclusiva, não havendo diferença no tipo de tratamento oferecido a depender do grau histológico. RESULTADOS: Trinta e cinco casos (51% foram classificados com EN I e 34 (49% como EN II. Não observamos diferenças na distribuição de outros fatores prognósticos entre os portadores dos dois graus. Remissão completa após o tratamento inicial foi obtida em 85,7% dos casos de EN I e em 82,4% dos casos de EN II (p = 0,75. A probabilidade estimada de sobrevida global em cinco anos foi de 67% para EN I e de 83,5% para EN II (p = 0,13 e a taxa de sobrevida livre de doença em cinco anos foi de 85,2% versus 87%, respectivamente (p = 0,72. CONCLUSÃO: Nesta população de pacientes uniformemente tratados a graduação histológica BNLI não esteve associada com o prognóstico do LH EN.BACKGROUND: Nodular sclerosis (NS, a frequent histological subtype of Hodgkin’s lymphoma (HL, presents great variability in its cellular composition. In the 80's, researchers of the British National Lymphoma Investigation (BNLI proposed a histological-based subclassification for NS HL. They identified two histological grades - NS HL grade I (NS HL I and NS HL grade II (NS HL II - and reported that NS II patients had a lower survival rate in comparison with NS I patients. Others studies, however, did not reproduce these findings. OBJECTIVE: Evaluate the prognostic significance of the BNLI grading system. MATERIAL AND METHOD: We retrospectively studied 69 NS HL patients. More than 90% of the included cases were treated with combined modality therapy or exclusive chemotherapy, the treatment type was not different for NS I and NS II patients. RESULTS: Thirty-five cases (51% were classified as NS I and 34 (49% as NS II. We did not observe significant differences in the distribution of other prognostic factors between the two NS grades. The complete remission rate after initial therapy was 85.7% in the NS I group and 82.4% in the NS II group (p = 0.75. The predicted 5-year overall survival rate was 67% in the NS I patients and 83.5% in the NS II patients (p = 0.13. The predicted 5-year disease free survival rate for NSI and NSII patients was 85.2% and 87%, respectively (p = 0.72. CONCLUSION: The histological BNLI grading system was not associated with the prognosis of this uniformly treated NS LH population.

  15. Extraintestinal manifestations of inflammatory bowel disease: epidemiology, diagnosis, and management

    DEFF Research Database (Denmark)

    Larsen, Signe; Bendtzen, Klaus; Nielsen, Ole Haagen

    2010-01-01

    ', 'bronchiectasis', 'bronchitis', 'cutaneous manifestations', 'erythema nodosum', 'extraintestinal manifestations', 'hyperhomocysteinemia', 'infliximab', 'iridocyclitis', 'lung disease', 'ocular manifestations', 'osteomalacia', 'pancreatitis', 'primary sclerosing cholangitis', 'renal stones', 'sulfasalazine...

  16. Een kind met primaire scleroserende cholangitis

    NARCIS (Netherlands)

    Rademaker, A. A. E. M.; Benninga, M. A.; Beuers, U. H. W.; Plötz, F. B.

    2015-01-01

    Primary sclerosing cholangitis is a rare liver disease which is mainly diagnosed in adults. This chronic progressive disease, characterised by inflammation, fibrosis and strictures of the intra- and extrahepatic bile ducts, leads to cirrhosis. There is a strong association between primary sclerosing

  17. Upper motor and extra-motor neuron involvement in recent-onset motor neuron disease

    NARCIS (Netherlands)

    van der Graaff, M.M.

    2012-01-01

    Amyotrofische laterale sclerose (ALS), progressieve spinale spieratrofie en primaire laterale sclerose zijn ziekten in de motorische voorhoorncel. De aandoeningen worden vooral op klinische gronden van elkaar onderscheiden. Maaike van der Graaff bekeek of motorische en niet-motorische neuronen in de

  18. Case report 363

    International Nuclear Information System (INIS)

    Langer, R.; Kaufmann, H.J.

    1986-01-01

    In summary, the case is presented of a 10-year-old girl with a fibrosarcoma of the femoral shaft, which showed the radiological and histological features of a malignant bone tumor, but with no findings radiologically pathognomonic of fibrosarcoma. Malignant fibrous histiocytoma, fibrogenic osteosarcoma and even Ewing tumor could present with the same radiological features. Although permeative infiltration of bone (described in this case) is observed in primary fibrosarcoma, it is much more commonly associated with another malignant bone tumor of childhood - Ewing tumor. (orig./SHA)

  19. Study of [18F]FLT and [123I]IaraU for cellular imaging in HSV1 tk-transfected murine fibrosarcoma cells: evaluation of the tracer uptake using 5-fluoro, 5-iodo and 5-iodovinyl arabinosyl uridines as competitive probes

    International Nuclear Information System (INIS)

    Huang, Ho-Lien; Chiang, Li-Wu; Chen, Jia-Rong; Yang, Wen K.; Jeng, Kee-Ching; Chen, Jenn-Tzong; Duh, Ting-Shien; Lin, Wuu-Jyh; Farn, Shiou-Shiow; Chiang, Chi-Shiun; Huang, Chia-Wen; Lin, Kun-I; Yu, Chung-Shan

    2012-01-01

    As one of the most intensively studied probes for imaging of the cellular proliferation, [ 18 F]FLT was investigated whether the targeting specificity of thymidine kinase 1 (TK1) dependency could be enhanced through a synergistic effect mediated by herpes simplex type 1 virus (HSV1) tk gene in terms of the TK1 or TK2 expression. 5-[ 123 I]Iodo arabinosyl uridine ([ 123 I]IaraU) was prepared in a radiochemical yield of 8% and specific activity of 21 GBq/μmol, respectively. Inhibition of the cellular uptake of these two tracers was compared by using the arabinosyl uridine analogs such as 5-iodo, 5-fluoro and 5-(E)-iodovinyl arabinosyl uridine along with 2′-fluoro-5-iodo arabinosyl uridine (FIAU). Due to potential instability of the iodo group, accumulation index of 1.6 for [ 123 I]IaraU by HSV1-TK vs. control cells could virtually be achieved at 1.5 h, but dropped to 0.2 compared to 2.0 for [ 18 F]FLT at 5 h. The results from competitive inhibition by these nucleosides against the accumulation of [ 18 F]FLT implied that FLT exerted a mixed TK1- and TK2-dependent inhibition with HSV1-tk gene transfection because of the shifting of thymidine kinase status. Taken together, the combination of [ 18 F]FLT and HSV1-TK provides a synergistic imaging potency.

  20. Study of [18F]FLT and [123I]IaraU for cellular imaging in HSV1 tk-transfected murine fibrosarcoma cells: evaluation of the tracer uptake using 5-fluoro, 5-iodo and 5-iodovinyl arabinosyl uridines as competitive probes.

    Science.gov (United States)

    Huang, Ho-Lien; Chiang, Li-Wu; Chen, Jia-Rong; Yang, Wen K; Jeng, Kee-Ching; Chen, Jenn-Tzong; Duh, Ting-Shien; Lin, Wuu-Jyh; Farn, Shiou-Shiow; Chiang, Chi-Shiun; Huang, Chia-Wen; Lin, Kun-I; Yu, Chung-Shan

    2012-04-01

    As one of the most intensively studied probes for imaging of the cellular proliferation, [(18)F]FLT was investigated whether the targeting specificity of thymidine kinase 1 (TK1) dependency could be enhanced through a synergistic effect mediated by herpes simplex type 1 virus (HSV1) tk gene in terms of the TK1 or TK2 expression. 5-[(123)I]Iodo arabinosyl uridine ([(123)I]IaraU) was prepared in a radiochemical yield of 8% and specific activity of 21 GBq/μmol, respectively. Inhibition of the cellular uptake of these two tracers was compared by using the arabinosyl uridine analogs such as 5-iodo, 5-fluoro and 5-(E)-iodovinyl arabinosyl uridine along with 2'-fluoro-5-iodo arabinosyl uridine (FIAU). Due to potential instability of the iodo group, accumulation index of 1.6 for [(123)I]IaraU by HSV1-TK vs. control cells could virtually be achieved at 1.5 h, but dropped to 0.2 compared to 2.0 for [(18)F]FLT at 5 h. The results from competitive inhibition by these nucleosides against the accumulation of [(18)F]FLT implied that FLT exerted a mixed TK1- and TK2-dependent inhibition with HSV1-tk gene transfection because of the shifting of thymidine kinase status. Taken together, the combination of [(18)F]FLT and HSV1-TK provides a synergistic imaging potency. Copyright © 2012 Elsevier Inc. All rights reserved.