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Sample records for duodenal gangliocytic paraganglioma

  1. Paraganglioma gangliocítico duodenal Duodenal gangliocytic paraganglioma

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    C. Prieto

    2005-04-01

    Full Text Available Se presenta el caso de un varón de 85 años que ingresó por dolor abdominal e ictericia, por lo cual se le realizaron diversas exploraciones, apreciandose en 2ª porción duodenal una masa cuyo estudio histológico demostró que se trataba de un paraganglioma gangliocítico duodenal. Se describen las características clínicas de este infrecuente tumor y revisamos su diagnóstico y tratamiento.We present the case of an 85 year old male who was admitted to hospital with abdominal pain and jaundice. Different explorations were performed for this reason, with a mass observed in his 2nd duodenal portion. Histiological study showed that it was a duodenal gangliocytic paraganlioma. The clinical characteristics of this infrequent tumour are described and we review the diagnosis and treatment.

  2. Duodenal gangliocytic paraganglioma: A case report and literature review

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    Tyler J. Loftus

    2015-01-01

    Conclusion: Localized duodenal gangliocytic paragangliomas are best managed by resection with negative margins. In cases in which the tumor is resected with negative margins, it appears to be safe to embark on a course of surveillance and forego adjuvant therapy.

  3. Surgical management of endoscopically unresectable duodenal gangliocytic paraganglioma in a patient with partial upper gastrointestinal obstruction

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    David J Bertsch

    2010-11-01

    Full Text Available Context: Gangliocytic paragangliomas are unusual and often misunderstood tumors that occur almost exclusively in the second portion of the duodenum, although they have been described in other sites such as the urinary bladder, spermatic cord, prostate, urethra, uterus and scalp. We describe our experience with the surgical management of an endoscopically unresectable gangliocytic paraganglioma located in the third part of the duodenum causing a partial upper gastrointestinal obstruction. Case Report: A fifty-two-year-old male presented to the Geisinger clinic with a four-year history of postprandial projectile vomiting associated with epigastric discomfort. Computed tomography scan revealed an oval-shaped filling defect in the third part of the duodenum. Endoscopic ultrasonography showed a 22 x 16 x 35 mm submucosal mass that was not amenable to an endoscopic resection. Exploratory laparotomy revealed an absence of extraduodenal involvement. A long-stalked tumor was successfully excised and extruded through a longitudinal duodenotomy. The pathology report showed a gangliocytic paraganglioma with negative lymph nodes. Conclusions: In patients presenting with prolonged recurrent attacks of vomiting, diagnostic workup to exclude anatomic causes is mandatory. Gangliocytic paraganglioma must be considered in the differential diagnosis of an intraduodenal tumor.

  4. Duodenal gangliocytic paraganglioma with lymph node metastases: A case report and comparative review of 31 cases.

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    Cathcart, Sahara J; Sasson, Aaron R; Kozel, Jessica A; Oliveto, Jennifer M; Ly, Quan P

    2017-06-16

    Gangliocytic paraganglioma (GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindle-like/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using PubMed, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled (30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain (55%) and gastrointestinal bleeding or sequelae (42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure (except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin (SYN) stained all epithelioid endocrine cells (18/18). Neuron specific enolase (NSE) and SYN stained most ganglion-like cells (7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells (21/21). Our literature review of published

  5. Obstructing Gangliocytic Paraganglioma in the Third Portion of the Duodenum

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    Carlos M. Nuño-Guzmán

    2012-07-01

    Full Text Available Gangliocytic paragangliomas are infrequent tumors almost exclusively found in the second portion of the duodenum. An unusual case of a gangliocytic paraganglioma in the third portion of the duodenum with obstructive symptoms is herein reported. A 16-year-old male patient presented with epigastric pain, postprandial plenitude and reflux. A barium swallow failed to demonstrate abnormalities. Endoscopy showed a pedunculated submucosal tumor, originating at the third duodenal portion and causing partial obstruction. Biopsy was not performed due to the risk of bleeding. CT scan demonstrated a polypoid lesion. Through a transmesocolic approach and an anterior duodenotomy, resection of the tumor was performed. No lymph node or other organ affection was found. Histologic examination revealed a gangliocytic paraganglioma. Immunohistochemical examination was performed. Gangliocytic paragangliomas originating in the third or fourth portion of the duodenum, as in the present case, are extremely rare. Characteristic histologic features including epithelioid cells, spindle-shaped cells and ganglion-like cells were met. The majority of cases manifest with a similar benign behavior. Local resection of the tumor is recommended for these cases. An infrequent case of a gangliocytic paraganglioma located in the third portion of the duodenum, with a less common clinical presentation, is herein reported.

  6. Safe endoscopic resection of gangliocytic paraganglioma of the major duodenal papilla Ampulectomía endoscópica eficaz de un paraganglioma gangliocítico localizado en la papila de Vater

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    P. Sánchez-Pobre

    2004-09-01

    Full Text Available A case of gangliocytic paraganglioma of the papilla of Vater in a 76-year-old man with a history of recurrent obstructive jaundice is presented. This is the first case of gangliocytic paraganglioma of the major papilla succesfully resected by endoscopic ampullectomy.Se describe un caso de paraganglioma gangliocítico de la papila de Vater, en un varón de 76 años, manifestado con cuadros de ictericia obstructiva recidivante. Este es el primer caso descrito de paraganglioma de la papila mayor resuelto eficazmente mediante ampulectomía endoscópica.

  7. Large gangliocytic paraganglioma of the duodenum:A rare entity

    Institute of Scientific and Technical Information of China (English)

    Alejandra; Gordillo; Hernández; Eduardo; Dominguez-Adame; Lanuza; Auxiliadora; Cano; Matias; Rosario; Perez; Huertas; Katherine; Maria; Gallardo; Rodriguez; Purificacion; Gallinato; Perez; Fernando; Oliva; Mompean

    2015-01-01

    Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.

  8. A Unique Case of an Aggressive Gangliocytic Paraganglioma of the Filum Terminale.

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    Akbik, Omar S; Floruta, Crina; Chohan, Muhammad O; SantaCruz, Karen S; Carlson, Andrew P

    2016-01-01

    Paragangliomas are rare neuroendocrine tumors that are mostly found in the head and neck. Even less common are gangliocytic variant paragangliomas of the spine for which there are only 7 other documented cases in the literature. We report a case of gangliocytic paraganglioma of the sacral spine in a 68-year-old man. The growth pattern is documented over three years, which to our knowledge has not previously been reported in the literature and is different from the natural history. Clinical, radiological, and pathological characteristics of the tumor are discussed in light of available reports of this rare tumor.

  9. Posterior mediastinal paragangliomas: a report of three patients with peculiar tumours.

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    Ayadi-Kaddour, Aïda; Braham, Emna; Ismail, Olfa; Smati, Bellahssan; Djilani, Habiba; El Mezni, Faouzi

    2009-04-01

    Paragangliomas of the mediastinum are rare neoplasms, representing less than 0.3% of mediastinal tumours and less than 2% of all paragangliomas. To date, no definitive morphologic criteria exist that correlate with the clinical outcome of these tumours. This report describes three patients with peculiar primary paragangliomas of the posterior mediastinum: gangliocytic, dumbbell and functional paraganglioma. The clinicopathological characteristics of these patients are presented and the literature reviewed.

  10. Paraganglioma Anatomy

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    ... e.g. -historical Searches are case-insensitive Paraganglioma Anatomy Add to My Pictures View /Download : Small: 648x576 ... View Download Large: 2700x2400 View Download Title: Paraganglioma Anatomy Description: Paraganglioma of the head and neck; drawing ...

  11. Malignant Vagal Paraganglioma.

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    Hamersley, Erin R S; Barrows, Amy; Perez, Angel; Schroeder, Ashley; Castle, James T

    2016-06-01

    Paragangliomas are rare, typically benign neuroendocrine tumors that represent a small portion of head and neck tumors. A small percentage of these are known to have malignant potential. They arise from the carotid body, jugular bulb or vagus nerves. There is limited literature discussing the management of malignant vagal paragangliomas. We present a case of a 25 year old female with a left malignant vagal paraganglioma. The following case presentation will describe the presentation, classic radiologic findings, and management of a malignant vagal paraganglioma along with a review of the literature.

  12. Paraganglioma of urinary bladder

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    Vinod Priyadarshi

    2015-01-01

    Full Text Available Paraganglioma of the urinary bladder are tumors of chromaffin tissue originating from the sympathetic innervations of the urinary bladder wall and are extremely rare. Being functional, in most of the cases they are recognized by their characteristic presentation of hypertensive crisis and postmicturition syncope. A silent presentation of a bladder paraganglioma is very unusual but quite dangerous as they are easily misdiagnosed and adequate peri-operative attention is not provided. Here, we are presenting one such silent paraganglioma in adult women who presented with only a single episode of hematuria and severe hypertensive crisis occur during its trans-urethral resection.

  13. Familial Pheochromocytomas and Paragangliomas

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    King, Kathryn S.; Pacak, Karel

    2013-01-01

    Pheochromocytomas and paragangliomas are neural crest cell tumors of the adrenal medulla and parasympathetic/sympathetic ganglia, respectively, that are often associated with catecholamine production. Genetic research over the years has led to our current understanding of the association 13 susceptibility genes with the development of these tumors. Most of the susceptibility genes are now associated with specific clinical presentations, biochemical makeup, tumor location, and associated neoplasms. Recent scientific advances have highlighted the role of somatic mutations in the development of pheochromocytoma/paraganglioma as well as the usefulness of immunohistochemistry in triaging genetic testing. We can now approach genetic testing in pheochromocytoma/paraganglioma patients in a very organized scientific way allowing for the reduction of both the financial and emotional burden on the patient. The discovery of genetic predispositions to the development of pheochromocytoma/paraganglioma not only facilitates better understanding of these tumors but will also lead to improved diagnosis and treatment of this disease. PMID:23933153

  14. Genetics Home Reference: nonsyndromic paraganglioma

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    ... torso. However, a type of paraganglioma known as pheochromocytoma develops in the adrenal glands . Adrenal glands are ... their lifetime. Some people develop a paraganglioma or pheochromocytoma as part of a hereditary syndrome that may ...

  15. Hypoxia-Inducible Factor 2α Mutation-Related Paragangliomas Classify as Discrete Pseudohypoxic Subcluster

    NARCIS (Netherlands)

    S.M.J. Fliedner (Stephanie); U. Shankavaram (Uma); Marzouca, G. (Geena); Elkahloun, A. (Abdel); Jochmanova, I. (Ivana); Daerr, R. (Roland); Linehan, W.M. (W. Marston); H.J. Timmers (Henri); A.S. Tischler (Arthur); K. Papaspyrou (Konstantinos); Brieger, J. (Jürgen); R.R. de Krijger (Ronald); Breza, J. (Jan); Eisenhofer, G. (Graeme); Zhuang, Z. (Zhengping); Lehnert, H. (Hendrik); K. Pacak (Karel)

    2016-01-01

    textabstractRecently, activating mutations of the hypoxia-inducible factor 2α gene (HIF2A/EPAS1) have been recognized to predispose to multiple paragangliomas (PGLs) and duodenal somatostatinomas associated with polycythemia, and ocular abnormalities. Previously, mutations in the SDHA/B/C/D, SDHAF2,

  16. Hypoxia-Inducible Factor 2alpha Mutation-Related Paragangliomas Classify as Discrete Pseudohypoxic Subcluster

    NARCIS (Netherlands)

    Fliedner, S.M.; Shankavaram, U.; Marzouca, G.; Elkahloun, A.; Jochmanova, I.; Daerr, R.; Linehan, W.M.; Timmers, H.J.; Tischler, A.S.; Papaspyrou, K.; Brieger, J.; Krijger, R. de; Breza, J.; Eisenhofer, G.; Zhuang, Z.; Lehnert, H.; Pacak, K.

    2016-01-01

    Recently, activating mutations of the hypoxia-inducible factor 2alpha gene (HIF2A/EPAS1) have been recognized to predispose to multiple paragangliomas (PGLs) and duodenal somatostatinomas associated with polycythemia, and ocular abnormalities. Previously, mutations in the SDHA/B/C/D, SDHAF2, VHL,

  17. Malignant vagal paraganglioma

    DEFF Research Database (Denmark)

    Carlsen, Camilla S; Godballe, Christian; Krogdahl, Annelise S

    2003-01-01

    Approximately 20 cases of malignant vagal paragangliomas (MVP)have been reported in English literature. Malignancy is based on the presence of metastases. A careful preoperative evaluation is necessary to detect multicentricity and/or significant production of catecholamines. A new case of MVP tr...

  18. Paraganglioma: a potentially challenging tumor.

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    Trombetta, Mark; Silverman, Jan; Colonias, Athanasios; Lee, Vincent; Mohanty, Alok; Parda, David

    2008-03-01

    Paragangliomas are usually low-grade neoplasms with a benign natural history. While the treatment of paraganglioma has historically been controversial, surgery and radiotherapy have become standardized as therapies of choice for primary therapy. More recently, stereotactic radiosurgery has been used effectively against this rare tumor. The development of metastatic disease in patients with paraganglioma is an unusual and challenging event. This case report and review describes the specific features of this disease and the multiple therapeutic options.

  19. [Intraabdominal paraganglioma--case report].

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    Ordeanu, C; Mărginean, A

    2011-01-01

    Paraganglioma is a rare neuroendocrine neoplasm that may develop in the head, neck, torax or abdomen, with a not specified symptoms and the accurate diagnose is established histopathological. The authors present a case of one intraabdominal paraganglioma, incidentally found during ultrasonographic evaluation and diagnose with histopathological examination of excised pieces.

  20. Pheochromocytomas and secreting paragangliomas

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    Gimenez-Roqueplo Anne-Paule

    2006-12-01

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

  1. Stages of Pheochromocytoma and Paraganglioma

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    ... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... up tests will be needed. There are different types of treatment for patients with pheochromocytoma or paraganglioma. ...

  2. Duodenal lipoma associated with ectopic duodenal glands

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    Dianbo Cao

    2011-12-01

    Full Text Available Duodenal lipomas are relatively uncommon and are rarely responsible for clinical symptoms. Occasionally, searching for aetiology of gastrointestinal bleeding leads to the final diagnosis of duodenal lipomas. Here, we present the case of a 68-year-old woman who suffered with repeated melena and weight loss. Endoscopy, abdominal computed tomography (CT and histopathological outcomes are described in this case of duodenal lipoma with ectopic duodenal glands.

  3. Contemporary management of jugular paragangliomas.

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    Wanna, George B; Sweeney, Alex D; Haynes, David S; Carlson, Matthew L

    2015-04-01

    Jugular paragangliomas are generally benign slow-growing tumors that can cause pulsatile tinnitus, hearing loss, and cranial nerves neuropathy. Progressive growth can also lead to intracranial extension. Historically, the treatment of choice for these lesions has been gross total resection. However, over the last 15 years, many groups have adopted less invasive management strategies including stereotactic radiation therapy, subtotal resection, and primary observation in order to reduce treatment-associated morbidity. The focus of this article is to review the modern management of jugular paraganglioma, highlighting the evolving treatment paradigm at the Otology Group of Vanderbilt.

  4. Recurrent paraganglioma of Meckel's cave: Case report and a review of anatomic origin of paragangliomas

    OpenAIRE

    Prajsnar, Anna; Balak, Naci; Walter, Gerhard F.; Stan, Alexandru C; Deinsberger, Wolfgang; Tapul, Leyla; Bayindir, Cicek; Amirjamshidi, Abbas

    2011-01-01

    Background: Paragangliomas are rare, usually benign tumors of neural crest origin. They account for only 0.6% of all head and neck tumors. In the craniocervical area, they are more common in the carotid body and tympanico-jugular regions. To the authors’ knowledge, a case of paraganglioma in Meckel's cave has not yet been reported in the medical literature. The pathogenesis and natural history of paragangliomas are still not well understood. We present a case of recurrent paraganglioma in Mec...

  5. Paragangliomas/Pheochromocytomas: Clinically Oriented Genetic Testing

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    Rute Martins

    2014-01-01

    Full Text Available Paragangliomas are rare neuroendocrine tumors that arise in the sympathetic or parasympathetic nervous system. Sympathetic paragangliomas are mainly found in the adrenal medulla (designated pheochromocytomas but may also have a thoracic, abdominal, or pelvic localization. Parasympathetic paragangliomas are generally located at the head or neck. Knowledge concerning the familial forms of paragangliomas has greatly improved in recent years. Additionally to the genes involved in the classical syndromic forms: VHL gene (von Hippel-Lindau, RET gene (Multiple Endocrine Neoplasia type 2, and NF1 gene (Neurofibromatosis type 1, 10 novel genes have so far been implicated in the occurrence of paragangliomas/pheochromocytomas: SDHA, SDHB, SDHC, SDHD, SDHAF2, TMEM127, MAX, EGLN1, HIF2A, and KIF1B. It is currently accepted that about 35% of the paragangliomas cases are due to germline mutations in one of these genes. Furthermore, somatic mutations of RET, VHL, NF1, MAX, HIF2A, and H-RAS can also be detected. The identification of the mutation responsible for the paraganglioma/pheochromocytoma phenotype in a patient may be crucial in determining the treatment and allowing specific follow-up guidelines, ultimately leading to a better prognosis. Herein, we summarize the most relevant aspects regarding the genetics and clinical aspects of the syndromic and nonsyndromic forms of pheochromocytoma/paraganglioma aiming to provide an algorithm for genetic testing.

  6. Supraglottic paraganglioma originated from superior laryngeal nerve

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    Ali Akbar Beigi

    2014-01-01

    Full Text Available Neurogenic tumors, especially paraganglioma of larynx, are rare. In this article, we present a 64-year-old woman who complained of intermittent dysphagia to solid foods. Further evaluation revealed a supraglottic paraganglioma and she was treated successfully by total excision of tumor.

  7. A Rare Case: Sporadic Bladder Paraganglioma

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    Hakan Ercil

    2013-08-01

    Full Text Available Paraganglioma is a rare tumor which originates from paraganglia tissue from neural crest. Bladder paraganglioma is suggested to be from crommaffin cells by the remains of the embryological cells. In this report, we aimed to discuss the literature by a case of bladder paraganglioma. A 39 year old male applied to our clinic with gross hematuria. A 2x2 cm mass was revealed in urinary ultrasound. Paraganglioma was found in the resection specimen and partial cystectomy was performed to the patient. A six month follow up revealed no recurrences. Even though bladder paraganglioma is a rare disease, it should be kept in mind for differential diagnosis. [Cukurova Med J 2013; 38(4.000: 794-799

  8. Management of duodenal trauma

    Institute of Scientific and Technical Information of China (English)

    CHEN Guo-qing; YANG Hua

    2011-01-01

    Duodenal trauma is uncommon but nowadays seen more and more frequently due to the increased automobile accidents and violent events. The management of duodenal trauma can be complicated, especially when massive injury to the pancreatic-duodenal-biliary complex occurs simultaneously. Even the patients receive surgeries in time, multiple postoperative complications and high mortality are common. To know and manage duodenal trauma better, we searched the recent related literature in PubMed by the keywords of duodenal trauma, therapy, diagnosis and abdomen. It shows that because the diagnosis and management are complicated and the mortality is high,duodenal trauma should be treated in time and tactfully.And application of new technology can help improve the management. In this review, we discussed the incidence,diagnosis, management, and complications as well as mortality of duodenal trauma.

  9. Malignant paraganglioma (multiple, multicentric and metastatic) in a female patient with family history of paraganglioma.

    Science.gov (United States)

    Cobos González, Ernesto; Aragón López, José Arturo; Soria Céspedes, Danny Rolando; de la Rosa Abaroa, Marco Antonio; Martínez de la Vega Celorio, Andrea; Granados Gracia, Martín; Bargalló Rocha, Enrique

    2015-11-01

    Malignant paragangliomas are rare, but may occur especially in patients with familial forms of the disease. We present the case of a 23 year old woman diagnosed with bilateral carotid paraganglioma with distant and local metastases, associated to a family history of paraganglioma and we present a literature review. Copyright © 2014 AEC. Publicado por Elsevier España, S.L.U. All rights reserved.

  10. A Case of Right Atrioventricular Groove Paraganglioma

    Institute of Scientific and Technical Information of China (English)

    Yi ZHANG; Tie-cheng PAN

    2009-01-01

    @@ Cardiac paraganglioma is an extremely rare clinical en-tities of the heart. The usual investigations for pheochro-mocytoma are necessary, however the most useful, spe-cific to primary cardiac tumors, are coronary angiogra-phy and echocardiography. While complete surgical ex-cision is the standard treatment of mainstay. We report a case of a 55-year-old woman with cardiac paraganglioma located in fight atriaoventricular groove of the heart. As we know, this is the first case that cardiac paraganglioma located in fight atriaoventricular groove of the heart in English literature.

  11. Paraganglioma funcional extra-adrenal

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    Laura Arroyo-Martínez

    2006-03-01

    Full Text Available Los paragangliomas funcionales son tumores raros, se originan del tejido cromafín extraadrenal productor de catecolaminas, con frecuencia son malignos y tienen alta incidencia de enfermedad persistente o recurrente¹. Se les conoce como: glomus, quemodectomas, paragangliomas cromafines y glomerulocitomas. La localización es diversa y refleja la distribución paraganglionar en el cuerpo, desde la base del cráneo hasta el piso pélvico. Los paragangliomas se encuentran en donde hay ganglios del sistema autónomo, sin embargo, aproximadamente el 90% de estos tumores aparecen en las glándulas suprarrenales (y constituyen los feocromocitomas y el 10% restante tienen una ubicación extraadrenal, mas se ha dicho que su incidencia puede ser subestimada, variando del 18% al 22% en adultos, y en niños hasta un 30%. Los extra-adrenales se originan con mayor frecuencia en el abdomen (85%, otros en el tórax (12% y más raramente en la cabeza y el cuello (3% ². Los estudios de imágenes y la medición de la producción no fisiológica de catecolaminas pueden ayudar en el diagnóstico de esta entidad. La cirugía es el tratamiento de elección. Presentamos aquí el caso de una paciente de 32 años, primigesta con HTAIE que requirió cesárea, quien tuvo un postparto tórpido y pese a múltiples tratamientos antihipertensivos su patología fue de difícil manejo, con complicaciones oftálmicas. Tiempo después la paciente se estudia por hiperhidrosis, se solicitan exámenes de laboratorio e imágenes y se le documenta incidentalmente, una tumoración retroperitoneal izquierda, se le amplían los estudios, y se llega al diagnóstico correcto. La tumoración requirió resección quirúrgica. Tuvo un postoperatorio satisfactorio y la paciente egresó con control en la Consulta Externa.Functioning paragangliomas are rare tumors that produce catecholamines. They originate from extra-adrenal chromaffin cells. They are frequentIy malignant and are associated

  12. Nuclear Imaging of a Cardiac Paraganglioma.

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    Almenieir, Nada; Karls, Shawn; Derbekyan, Vilma; Lisbona, Robert

    2017-09-01

    We report a case of a cardiac paraganglioma in the right atrioventricular groove in which the use of different nuclear medicine studies aided in the diagnosis. © 2017 by the Society of Nuclear Medicine and Molecular Imaging.

  13. General Information about Pheochromocytoma and Paraganglioma

    Science.gov (United States)

    ... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... up tests will be needed. There are different types of treatment for patients with pheochromocytoma or paraganglioma. ...

  14. Treatment Option Overview (Pheochromocytoma and Paraganglioma)

    Science.gov (United States)

    ... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... up tests will be needed. There are different types of treatment for patients with pheochromocytoma or paraganglioma. ...

  15. MRI of paraganglioma of the filum terminale

    Energy Technology Data Exchange (ETDEWEB)

    Boncoeur-Martel, M.P.; Lesort, A.; Moreau, J.J.; Labrousse, F.; Roche, I.; Bouillet, P.; Pascaud, J.L.; Dupuy, J.P. [Cabinet of Rheumatology, Limoges (France)

    1996-01-01

    Paragangliomas are benign tumors arising from paraganglionic cells of the sympathetic nervous system. They are of neuroectodermal derivation, with the majority occurring in the adrenal medulla. Extraadrenal locations are usually nonfunctioning and mostly observed (90%) in the head and neck region (carotid body or glomus jugulare tumors). Rare reported locations include the mediastinum, retroperitoneum, lungs, duodenum, and bladder. Within the CNS, paragangliomas have been observed in the pineal region, petrous ridge, sella turcica, and spinal canal. Paraganglioma of the cauda equina was first reported, but not recognized as such, in 1970. We know of 10 reports that describe MRI appearance. We present a new case and review the imaging findings of a paraganglioma of the filum terminale. 14 refs., 3 figs.

  16. Paraganglioma funcional extra-adrenal

    Directory of Open Access Journals (Sweden)

    Laura Arroyo-Martínez

    2006-03-01

    Full Text Available Los paragangliomas funcionales son tumores raros, se originan del tejido cromafín extraadrenal productor de catecolaminas, con frecuencia son malignos y tienen alta incidencia de enfermedad persistente o recurrente¹. Se les conoce como: glomus, quemodectomas, paragangliomas cromafines y glomerulocitomas. La localización es diversa y refleja la distribución paraganglionar en el cuerpo, desde la base del cráneo hasta el piso pélvico. Los paragangliomas se encuentran en donde hay ganglios del sistema autónomo, sin embargo, aproximadamente el 90% de estos tumores aparecen en las glándulas suprarrenales (y constituyen los feocromocitomas y el 10% restante tienen una ubicación extraadrenal, mas se ha dicho que su incidencia puede ser subestimada, variando del 18% al 22% en adultos, y en niños hasta un 30%. Los extra-adrenales se originan con mayor frecuencia en el abdomen (85%, otros en el tórax (12% y más raramente en la cabeza y el cuello (3% ². Los estudios de imágenes y la medición de la producción no fisiológica de catecolaminas pueden ayudar en el diagnóstico de esta entidad. La cirugía es el tratamiento de elección. Presentamos aquí el caso de una paciente de 32 años, primigesta con HTAIE que requirió cesárea, quien tuvo un postparto tórpido y pese a múltiples tratamientos antihipertensivos su patología fue de difícil manejo, con complicaciones oftálmicas. Tiempo después la paciente se estudia por hiperhidrosis, se solicitan exámenes de laboratorio e imágenes y se le documenta incidentalmente, una tumoración retroperitoneal izquierda, se le amplían los estudios, y se llega al diagnóstico correcto. La tumoración requirió resección quirúrgica. Tuvo un postoperatorio satisfactorio y la paciente egresó con control en la Consulta Externa.

  17. Paragangliomas of mediastinum: A real case. Paraganglioma de mediastino: a proposito de un caso

    Energy Technology Data Exchange (ETDEWEB)

    Fernandez de Alarcon, M.; Escribano Fernandez, M.; Azuara Muslera, M.V.; Delgado Mije, ED. (Hospital ' ' Infanta Cristina' ' . Servicio de Radiodiagnostico. Badajoz (Spain))

    1993-05-01

    Paragangliomas are tumors derived from the paraganglia, structures associated with the sympathetic nervous system that are formed by paraganglionic cells that derive embryologically from the neural crest (neuroectoderm). These tumors can secrete catecholamines (chromaffin paragangliomas or extraadrenal pheochromocytomas) or chemoreceptors (chemodectoma). The diagnosis of these tumors is based on clinical features and laboratory tests and they are localized by means of simple radiology and with the aid of CT, MR and ''131I-MIBG scintigraphy. We present a case of chromaffin paraganglioma located atypically in posterior mediastinum. (Author)

  18. Paraganglioma of the filum terminale: case report

    Directory of Open Access Journals (Sweden)

    Antonelli Manila

    2009-12-01

    Full Text Available Abstract Background Paragangliomas affecting the filum terminale are extremely rare, benign tumors. The literature yielded thirty-two cases of paraganglioma in this site. Case presentation A 49 year-old-man, whose presenting symptoms were low back pain and left leg weakness, was diagnosed as having a paraganglioma of the filum terminale. The clinical, histological and radiological characteristics of this case, that brings the total number of cases described to 33, are discussed in the light of published data. Conclusions This extremely rare pathology can usually be successfully treated by total surgical resection, which represents the gold standard. In the event of incomplete removal, assiduous long-term follow-up is mandatory.

  19. Demonstration of S-100 protein in sustentacular cells of phaeochromocytomas and paragangliomas

    DEFF Research Database (Denmark)

    Schroder, H D; Johannsen, L

    1986-01-01

    Eighteen phaeochromocytomas, including both sporadic and familial cases, four cervical paragangliomas, two jugular paragangliomas, and one abdominal paraganglioma were examined immunohistochemically for the presence of S-100 protein. Positive staining in cells morphologically similar to the suste...

  20. Descolamento de retina seroso em paraganglioma: relato de caso Serous retinal detachment in paraganglioma: case report

    Directory of Open Access Journals (Sweden)

    Oscar Villas Boas

    2008-02-01

    Full Text Available Os autores descrevem um caso de uma paciente gestante com hipertensão arterial resistente ao tratamento e descolamento seroso bilateral de retina. Confirmou-se, pelo exame anátomo-patológico, ser um paraganglioma.The authors describe a case of a pregnant patient with arterial hypertension that resists to the treatment and retinal bilateral serous detachment. It was confirmed to be a paraganglioma by anatomicopathological examination.

  1. Duodenal stump leak following a duodenal switch: A case report

    Directory of Open Access Journals (Sweden)

    Lars Nelson

    2015-01-01

    Conclusion: Duodenal stump leaks must be diagnosed as early as possible, and treated appropriately with operative intervention. Regardless of the operative technique the key to appropriate treatment is stabilize the patient, repair the duodenal stump, and adequate drainage.

  2. Malignant paraganglioma in a cougar (Puma concolor).

    Science.gov (United States)

    Duhamelle, Alexis; Langlois, Isabelle; Pey, Pascaline; Tremblay, Josée; Ruel, Hélène; Parent, Joane; Lussier, Joanie; Doré, Monique

    2014-12-01

    A 7½-yr-old male cougar (Puma concolor) was presented with a 2-wk history of progressive hindlimb abnormalities. An abdominal mass was palpated on physical examination. Computed tomography of the abdomen showed a mass surrounding the left ureter. A postmortem diagnosis of paraganglioma was established.

  3. Dopamine-Secreting Paraganglioma in the Retroperitoneum.

    Science.gov (United States)

    Matsuda, Yusuke; Kimura, Noriko; Yoshimoto, Takanobu; Sekiguchi, Yoshihiro; Tomoishi, Junzo; Kasahara, Ichiro; Hara, Yoshihito; Ogawa, Yoshihiro

    2017-03-01

    Pheochromocytomas and paragangliomas, which exclusively produce dopamine, are very rare. Herein, we report for the first time a Japanese case of an exclusively dopamine-producing paraganglioma accompanied by detailed immunohistochemical analyses. A 70-year-old Japanese woman was referred to our hospital for functional examination of her left retroperitoneal mass. Her adrenal functions were normal, except for excessive dopamine secretion. After the tumorectomy, her dopamine level normalized. The histopathological diagnosis of the tumor was paraganglioma; this was confirmed by positive immunostaining of chromogranin A (CgA), tyrosine hydroxylase (TH), dopamine β-hydroxylase (DBH), and succinate dehydrogenase gene subunit B (SDHB). However, the immunostaining of CgA in the tumor cells showed peculiar dot-like staining located corresponding to Golgi complex in the perinuclear area, rather than the diffuse cytoplasmic staining usually observed in epinephrine- or norepinephrine-producing functional pheochromocytomas and paragangliomas. The immunohistochemical results suggested that the tumor cells had sparse neuroendocrine granules in the cytoplasm, resulting in inhibition of catecholamine synthesis from dopamine to norepinephrine in neurosecretory granules. This may be the mechanism responsible for exclusive dopamine secretion in the present case.

  4. Radiographic evaluation and assessment of paragangliomas.

    Science.gov (United States)

    Lustrin, E S; Palestro, C; Vaheesan, K

    2001-10-01

    Radiographic imaging plays an important role in the diagnosis and treatment of paragangliomas. Diagnosis and treatment should be performed as a team effort, with all the involved disciplines working together to provide the best possible individualized work-up and treatment plan for the patient.

  5. Comprehensive Molecular Characterization of Pheochromocytoma and Paraganglioma

    NARCIS (Netherlands)

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

    2017-01-01

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

  6. Cutaneous paraganglioma of the vertex in a child.

    Science.gov (United States)

    Kim; Lee, Il Jae; Park, Myong Chul; Kim, Joo Hyoung; Lim, Hyoseob

    2012-07-01

    Paraganglioma is a neuroendocrine neoplasm that may develop at various body sites, including the head, neck, thorax, and abdomen. Approximately 85% of paragangliomas develop on the abdomen, 12% develop on the chest, and only 3% develop on the head and neck. These tumors are found in locations that parallel the sympathetic chain ganglion in the thoracolumbar regions and parasympathetic nervous system in craniosacral regions, and all head and neck paragangliomas arise from the parasympathetic nervous system. Although the skin has a rich neural network, it is devoid of ganglia. There has been only 1 report of a paraganglioma on the scalp of a child. We describe a 3-year-old child with a primary cutaneous paraganglioma of the vertex scalp and review the literature on paragangliomas.

  7. Breast Metastasis from Malignant Paraganglioma: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Nam, Sang Yu; Han, Boo Kyung [Dept. of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2013-06-15

    We report a case of metastatic breast cancer from paraganglioma and describe the radiologic findings in a 32-year-old woman who had a history of excision for carotid body paraganglioma. Breast metastasis from malignant paraganglioma showed a well-defined mass with hypoechogenicity, posterior acoustic enhancement and iNcreased vascularity on ultrasonography, and strong enhancements on contrast-enhanced MRI and diffusion restriction on diffusion weighted image.

  8. Cervical Paraganglioma Mimicking Thyroid Nodule: A Rare Clinical Case

    Directory of Open Access Journals (Sweden)

    Berna İmge Aydoğan

    2016-01-01

    Full Text Available Objective. Paraganglioma is a rare neuroendocrine tumor. When it is located in the neck, it is commonly misdiagnosed as other thyroid neoplasms. Case Report. We report a case of cervical paraganglioma in a 55-year-old female. Patient was admitted to our clinic with goiter and neck pain. Thyroid ultrasonography revealed a 20 mm solitary, heterogeneous nodule located in the upper pole of left thyroid lobe. Fine needle aspiration cytology was nondiagnostic. She underwent left lobectomy and histopathology showed paraganglioma. Discussion. Cervical paragangliomas should be considered in the differential diagnosis of thyroid nodules.

  9. Synchronous malignant vagal paraganglioma with contralateral carotid body paraganglioma treated by radiation therapy

    Directory of Open Access Journals (Sweden)

    Devlina Chakarvarty

    2010-06-01

    Full Text Available Paragangliomas are rare tumors and very few cases of malignant vagal paraganglioma with synchronous carotid body paraganglioma have been reported. We report a case of a 20-year old male who presented with slow growing bilateral neck masses of eight years duration. He had symptoms of dysphagia to solids, occasional mouth breathing and hoarseness of voice. Fine needle aspiration cytology (FNAC performed where he lived showed a sinus histiocytosis and he was administered anti-tubercular treatment for six months without any improvement in his symptoms. His physical examination revealed pulsatile, soft to firm, non-tender swellings over the anterolateral neck confined to the upper-mid jugulo-diagastric region on both sides. Direct laryngoscopy examination revealed a bulge on the posterior pharyngeal wall and another over the right lateral pharyngeal wall. Magnetic resonance imaging (MRI, 99mTc-labeled octreotide scan and angiography diagnosed the swellings as carotid body paraganglioma, stage III on the right side with left-sided vagal malignant paraganglioma. Surgery was ruled out as a high morbidity with additional risk to life was expected due to the highly vascular nature of the tumor. The patient was treated with radiation therapy by image guided radiation to a dose of 5040cGy in 28 fractions. At a follow-up at 16 months, the tumors have regressed bilaterally and the patient can take solids with ease.

  10. Neurovascular complications following carotid body paraganglioma resection.

    Science.gov (United States)

    Lamblin, E; Atallah, I; Reyt, E; Schmerber, S; Magne, J-L; Righini, C A

    2016-11-01

    The present consecutive case series reports our experience in the management of carotid body paraganglioma and aims to assess whether the Shamblin classification or tumor size are predictive of early and late postoperative neurovascular complications. A retrospective study included 54 carotid body tumor resections in 49 patients, between 1980 and 2011. Data comprised early (3.2cm emerged as predictive factors for early postoperative peripheral neurological complications. Eight patients (17%) showed no cranial nerve deficit recovery, even after 18 months' follow-up; no predictive factors could be identified for this. Surgical resection remains the only curative treatment in carotid body paraganglioma, with low vascular morbidity. However, early postoperative nerve deficit remains frequent (56%), although mostly temporary, with 17% definitive sequelae at 18 months. Tumor size and Shamblin classification are predictive of early neurovascular complications. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  11. Nuclear medicine therapy of pheochromocytoma and paraganglioma

    Energy Technology Data Exchange (ETDEWEB)

    Troncone, L.; Rufini, V. [Rome Catholic University of the Sacred Heart, Rome (Italy). Dept. of Nuclear Medicine

    1999-12-01

    Pheochromocytomas and paragangliomas are rare catecholamine-producing tumors which arise from chromaffin tissue. When a pheochromocytoma/paraganglioma is suspected, biochemical confirmation is based on 24-hour urinary excretion rates of catecholamines and their metabolites (metanephrines, Vma, etc.). Following biochemical confirmation non invasive imaging techniques such as CT and/or MR of the abdomen and {sup 1}23{sup I}-Mibg scintigraphy are performed to localize the tumor. {sup 1}11{sup I}n-octreotide may also be applied, mainly to localize head and neck chemodectomas. Malignant paragangliomas of either adrenal or extra-adrenal origin show a variable natural history: from a locally invasive indolent tumor to a highly aggressive malignancy. Surgery with complete resection or debulking of the primary tumor is the standard treatment. External radiotherapy and chemotherapy are usually scarcely effective. An alternative treatment is {sup 1}31{sup I}-Mibg therapy which is performed with high specific activity {sup 1}31{sup I}-Mibg. Usually a standardized dose ranging from 3.7 to 9.1 GBq of {sup 1}31{sup I}-Mibg is administered by slow i.v. infusion. In advanced stage cases {sup 1}31{sup I}-Mibg therapy aims at symptom palliation and tumor function reduction as well as tumor arrest or tumor regression. In these cases Mibg therapy allows prolonged survival and good quality of life. In less advanced cases the purpose of Mibg therapy is to complement surgery and to achieve the total eradication of the tumor. Non functioning malignant paraganglioma can some time also concentrate Mibg and can be treated with high doses of the tracer. {sup 1}31{sup I}-Mibg therapy is a safe treatment and is usually well tolerated by the patient (with rather low myelotoxicity).

  12. Paraganglioma pré-aórtico gigante Giant preaortic paraganglioma

    Directory of Open Access Journals (Sweden)

    Sergio Renato Pais Costa

    2008-12-01

    Full Text Available INTRODUÇÃO: O paraganglioma é um tumor neuroendócrino raro que ocorre mais frequentemente em adultos jovens. Geralmente produz catecolaminas acarretando síndrome adrenérgica. No entanto, muito raramente, quando não-funcionante seus sintomas são mais frequentemente associados à massa abdominal ou mesmo dor. Nessas circunstâncias costumam representar diagnóstico difícil sendo confundidos com os sarcomas de retroperitônio. RELATO DO CASO: Paraganglioma pré-aórtico gigante não-funcionante em paciente com níveis baixos de catecolaminas (sérico e urinário. O diagnóstico pré-operatório foi dado por tomografia computadorizada, onde foi observada massa sólida, hipervascular com calcificações e área cística central. O paciente foi submetido à ressecção cirúrgica da massa com boa evolução pós-operatória. Um ano após a operação, o doente encontra-se vivo sem recidiva tumoral. CONCLUSÃO: Em que pese a raridade o paraganglioma pré-aortico não-funcionante deve ser lembrado com diagnóstico diferencial com os sarcomas de retroperitônio. Seu tratamento é cirúrgico e apresenta bom prognóstico.RACIONAL: Paraganglioma is a rare neuroendocrine tumor which often diagnosed in the young adult. Generally, paraganglioma produces catecholamines causing adrenergic syndrome. However, more rarely when tumor is nonfunctioning, their symptoms are more associated with an abdominal mass or even pain. In these circumstances, paraganglioma present a difficult diagnosis confounding with retroperitoneal sarcomas. CASE REPORT: The authors present a case of nonfunctioning giant preaortic paraganglioma. This patient had low levels of catecholamines (both seric and urinary. The preoperative diagnosis was done by means computed tomography. This lesion presented as well-vascularized tumor with calcifications and necrotic central area. The patient underwent a surgical resection with good postoperative outcome. To date, one year after surgical

  13. Functional Paraganglioma: A Rare Conus‑cauda Lesion

    African Journals Online (AJOL)

    of symptoms like low back pain, saddle sensory changes, sciatica ... only five case reports of functional spinal paragangliomas are available to the ... paraganglioma postoperatively after the patient died because of ... During surgery these tumors should be handled ... During surgery, blood pressure increased considerably.

  14. Unusual Cause of Acute Abdomen—Ruptured Retroperitoneal Paraganglioma

    Directory of Open Access Journals (Sweden)

    Kwok-Kay Yau

    2008-01-01

    Full Text Available Ruptured retroperitoneal paraganglioma is a rare cause of acute abdomen. Its clinical presentation and laparoscopic features have seldom been reported in the literature. Herein, we report a case of ruptured retroperitoneal paraganglioma that presented as acute abdomen, and its subsequent management.

  15. A rare presentation of clinically intractable hypertension: Pancreatic paraganglioma

    Directory of Open Access Journals (Sweden)

    Chao-Ming Tseng

    2016-09-01

    Full Text Available Paraganglioma is a rare extra-adrenal pheochromocytoma which originates from chromaffin cells within the ganglia of the sympathetic trunk and of the celiac, renal, suprarenal, and hypogastric plexuses. Pancreatic paragangliomas are rarer still. And even then, paragangliomas are mostly reported to be nonfunctional. We report a case of a 64-year-old woman with underlying disease of hypertension who presented with biliary colic. Contrast-enhanced computer tomography showed an enhancing mass in the uncinate process of the pancreas. Pylorus-sparing Whipple procedure was performed for complete tumor excision. Hypertensive crisis developed after Whipple, which improved after continuous intravenous nicardipine infusion. Pathology revealed a paraganglioma. A 24-h catecholamine urine test showed increased norepinephrine and vanillylmandelic acid level. Functional paraganglioma was diagnosed.

  16. Roxatidine in duodenal ulcer.

    OpenAIRE

    1995-01-01

    Roxatidine acetate is a new H2-receptor antagonist. A randomized double-blind clinical trial in fifty-three patients with endoscopically proven duodenal ulcers > 5 mm in diameter was undertaken to compare safety and efficacy of roxatidine with that of ranitidine. Twenty-six patients received roxatidine (75 mg bid) while 27 patients received ranitidine (150 mg bid) for 4 weeks. One patient in each group did not come for follow up. Roxatidine and ranitidine had comparable ulcer healing rates...

  17. Histological Value of Duodenal Biopsies

    Directory of Open Access Journals (Sweden)

    Limci Gupta

    2005-01-01

    Full Text Available This study was performed to see the value of histopathological diagnosis in management of patients with duodenal biopsies; to look for correlation of histology and serology in suspected cases of coeliac disease; the reasons for taking duodenal biopsies and whether proper adequate histories are provided on the forms sent with request for histopathological view on duodenal biopsies. Here are the observations of the study followed by the discussion.

  18. Paraganglioma de mediastino com metástases pulmonares Pulmonary metastasis of mediastinal paraganglioma

    Directory of Open Access Journals (Sweden)

    Manoel Ximenes Netto

    2005-02-01

    Full Text Available Descrevemos uma paciente de 27 anos que se apresentou com paraganglioma de mediastino anterior e médio e nódulos pulmonares bilaterais. O tratamento consistiu na ressecção das lesões pulmonares através de toracotomia anterior bilateral transesternal e retirada do paraganglioma com auxílio de circulação extracorpórea. Como tratamento neoadjuvante foram usadas radioterapia e quimioterapia. A evolução pós-operatória foi satisfatória, e catorze meses depois a paciente encontrava-se assintomática.Herein, we describe the case of a 27-year-old female presenting with paraganglioma of the anterior and middle mediastinum and bilateral pulmonary nodules. Treatment consisted of pulmonary resection by anterior bilateral thoracotomy and transverse sternotomy, in which the paraganglioma was excised with the aid of extracorporeal circulation. As neoadjuvant treatments, radiotherapy and chemotherapy were applied. Postoperative evolution was uneventful, and the patient was classified as asymptomatic after 14 months.

  19. Pathology of Human Pheochromocytoma and Paraganglioma Xenografts in NSG Mice

    Science.gov (United States)

    Powers, James F.; Pacak, Karel; Tischler, Arthur S.

    2016-01-01

    A major impediment to the development of effective treatments for metastatic or unresectable pheochromocytomas and paragangliomas has been the absence of valid models for pre-clinical testing. Attempts to establish cell lines or xenografts from human pheochromocytomas and paragangliomas have previously been unsuccessful. NOD-scid gamma (NSG) mice are a recently developed strain lacking functional B-cells, T-cells and NK cells. We report here that xenografts of primary human paragangliomas will take in NSG mice while maintaining their architectural and immunophenotypic characteristics as expressed in the patients. In contrast to grafts of cell lines and of most common types of primary tumors, the growth rate of grafted paragangliomas is very slow, accurately representing the growth rate of most pheochromocytomas and paragangliomas even in metastases in humans. Although the model is therefore technically challenging, primary patient derived xenografts of paragangliomas in NSG mice provide a potentially valuable new tool that could prove especially valuable for testing treatments aimed at eradicating the small tumor deposits that are often numerous in patients with metastatic paraganglioma. PMID:27709415

  20. Retroperitoneal Paraganglioma Presenting as a Chest Pain: A Case Report

    Directory of Open Access Journals (Sweden)

    Parag Brahmbhatt

    2013-01-01

    Full Text Available Paragangliomas are very rare tumors derived from neuroendocrine cells of autonomic nervous system. Extra-adrenal paragangliomas account for only 10 to 15% of all paragangliomas and may present incidentally as a mass. Typical triad of fluctuating hypertension, headache, and sweating is not always present which makes the diagnosis difficult sometimes. Definitive diagnosis is usually made with histologic findings and surgery is the treatment of choice. We report a case of a 53-year-old male who presented with chest pain and vomiting.

  1. Anterior mediastinal paraganglioma: A case for preoperative embolization

    Directory of Open Access Journals (Sweden)

    Shakir Murtaza

    2012-07-01

    Full Text Available Abstract Background Paraganglioma is a rare but highly vascular tumor of the anterior mediastinum. Surgical resection is a challenge owing to the close proximity to vital structures including the heart, trachea and great vessels. Preoperative embolization has been reported once to facilitate surgical treatment. Case presentation We report a case of anterior mediastinal paraganglioma that was embolized preoperatively, and was resected without the need for cardiopulmonary bypass and without major bleeding complications. Conclusion We make a case to further the role of preoperative embolization in the treatment of mediastinal paragangliomas.

  2. Robot assisted laparoscopic excision of a paraganglioma: new therapeutic approach

    Directory of Open Access Journals (Sweden)

    G. Cochetti

    2014-04-01

    Full Text Available The Paraganglioma is the most common extra-adrenal pheochromocytoma arising from neural crest (1 (It will better to write: The paraganglioma is an extra-adrenal pheocromocytoma arising from the neural crest. 10% of pheocromocytomas are extra-adrenal and can arise form chromaffin tissue derived from primitive neuroectoderm. Minimally invasive techniques allow surgeons to perform the procedure without wide exposure and mobilization of intra abdominal organs. To our knowledge we present the third case of robotic excision of a retroperitoneal paraganglioma (2,3.

  3. Paraganglioma of the urinary bladder with pelvic metastasis

    Directory of Open Access Journals (Sweden)

    Jiun-Hung Geng

    2014-09-01

    Full Text Available A 52-year-old male, diagnosed with paraganglioma of the urinary bladder, underwent transurethral resection of the bladder tumor 10 years ago. He was lost to follow-up after the operation but was recently admitted to our hospital for the treatment of nasopharyngeal cancer. However, refractory hypertension with palpitation was noted and a computed tomography scan revealed a round, well-defined mass at the right pelvic region. Retroperitoneal tumor excision surgery was performed and a subsequent pathological analysis revealed paraganglioma. The diagnosis of paraganglioma of the urinary bladder with pelvic metastasis was confirmed and his blood pressure returned to normal level without medication after the operation.

  4. MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma

    NARCIS (Netherlands)

    Burnichon, N.; Cascon, A.; Schiavi, F.; Morales, N.P.; Comino-Mendez, I.; Abermil, N.; Inglada-Perez, L.; Cubas, A.A. de; Amar, L.; Barontini, M.; Quiros, S.B. de; Bertherat, J.; Bignon, Y.J.; Blok, M.J.; Bobisse, S.; Borrego, S.; Castellano, M.; Chanson, P.; Chiara, M.D.; Corssmit, E.P.; Giacche, M.; Krijger, R.R. de; Ercolino, T.; Girerd, X.; Gomez-Garcia, E.B.; Gomez-Grana, A.; Guilhem, I.; Hes, F.J.; Honrado, E.; Korpershoek, E.; Lenders, J.W.; Leton, R.; Mensenkamp, A.R.; Merlo, A.; Mori, L.; Murat, A.; Pierre, P.; Plouin, P.F.; Prodanov, T.; Quesada-Charneco, M.; Qin, N.; Rapizzi, E.; Raymond, V.; Reisch, N.; Roncador, G.; Ruiz-Ferrer, M.; Schillo, F.; Stegmann, A.P.; Suarez, C.; Taschin, E.; Timmers, H.J.L.M.; Tops, C.M.; Urioste, M.; Beuschlein, F.; Pacak, K.; Mannelli, M.; Dahia, P.L.; Opocher, G.; Eisenhofer, G.; Gimenez-Roqueplo, A.P.; Robledo, M.

    2012-01-01

    PURPOSE: Pheochromocytomas (PCC) and paragangliomas (PGL) are genetically heterogeneous neural crest-derived neoplasms. Recently we identified germline mutations in a new tumor suppressor susceptibility gene, MAX (MYC-associated factor X), which predisposes carriers to PCC. How MAX mutations

  5. EANM 2012 guidelines for radionuclide imaging of phaeochromocytoma and paraganglioma

    NARCIS (Netherlands)

    Taieb, D.; Timmers, H.J.L.M.; Hindie, E.; Guillet, B.A.; Neumann, H.P.; Walz, M.K.; Opocher, G.; Herder, W.W. de; Boedeker, C.C.; Krijger, R.R. de; Chiti, A.; Al-Nahhas, A.; Pacak, K.; Rubello, D.

    2012-01-01

    PURPOSE: Radionuclide imaging of phaeochromocytomas (PCCs) and paragangliomas (PGLs) involves various functional imaging techniques and approaches for accurate diagnosis, staging and tumour characterization. The purpose of the present guidelines is to assist nuclear medicine practitioners in perform

  6. Paraganglioma presenting as cholesterol granuloma of the petrous apex.

    Science.gov (United States)

    Heman-Ackah, Selena E; Huang, Tina C

    2013-09-01

    We report the unique finding of a petrous apex cholesterol granuloma associated with a paraganglioma, also known as a glomus jugulare tumor, in a 52-year-old woman who presented to our department with pulsatile tinnitus, hearing loss, aural fullness, and disequilibrium. She had been treated for a petrous apex cholesterol granuloma 20 years earlier, at which time she had undergone drainage of the granuloma via subtotal petrous apicectomy. When she came to our facility approximately 20 years later, she had signs and symptoms consistent with a jugular paraganglioma, which was likely to have been present at the time of her initial presentation for the cholesterol granuloma. In fact, microscopic bleeding from the paraganglioma might have led to the formation of the cholesterol granuloma. The metachronous presentation of these two entities, which to our knowledge has not been reported previously in the literature, indicates the potential association of paragangliomas with the formation of cholesterol granulomas of the petrous apex.

  7. AN UNUSUAL BILATERAL VAGAL PARAGANGLIOMAS: ONE CASE STUDY

    Institute of Scientific and Technical Information of China (English)

    Hai-hong Chen; Liang Chai; Wei Pan

    2006-01-01

    @@ VAGAL paraganglioma (VP) is an uncommon neoplasm originating from neural crest paraganglion cells located along the vagus nerve, representing less than 5% of all paragangliomas of the head and neck.1 Despite improvement in microsurgical techniques,management of such a neoplasm still presents great challenge to most clinicians, surgical excision of it can be accompanied by serious morbidity and occasional mortality,especially bilateral or multiple VPs.

  8. Hybrid resection of duodenal tumors.

    Science.gov (United States)

    Poultsides, George A; Pappou, Emmanouil P; Bloom, George Peter; Orlando, Rocco

    2011-09-01

    The aim of this study was to review our experience with the hand-assisted laparoscopic management of duodenal tumors with no or low malignant potential and to compare this approach with published case reports of purely laparoscopic local duodenal resection. Eight cases of hand-assisted laparoscopic local duodenal resection performed from 2000 to 2008 were retrospectively reviewed. Hand-assistance was utilized for complete duodenal mobilization, and local duodenal resection was accomplished extracorporeally through the hand-access incision. Patient and tumor characteristics, operative time, length of stay, and complications were compared with 18 cases of totally laparoscopic local excision of duodenal tumors published since 1997. Patients with ampullary tumors were excluded. Compared with the purely laparoscopic approach, the hand-assisted technique was associated with shorter operative time (179 versus 131 minutes, P=.03) and was more commonly used for lesions located in the third portion of the duodenum (0% versus 37.5%, P=.02). Tumor size (2.9 cm versus 3.2 cm, P=.61) and length of hospital stay (5.9 versus 5.9 days, P=.96) were similar between the two groups. The rate of complications was also comparable (0% versus 12.5%, P=.31); 1 of 8 patients in the hand-assisted group developed an incisional hernia at the hand-access site. Hand-assisted laparoscopic local duodenal resection is a feasible, safe, and effective alternative to the totally laparoscopic approach. In addition to being associated with comparable length of hospital stay, hand-assistance can shorten operative time by facilitating duodenal mobilization as well as extracorporeal duodenal resection and closure.

  9. Roxatidine in duodenal ulcer.

    Directory of Open Access Journals (Sweden)

    Habibullah C

    1995-07-01

    Full Text Available Roxatidine acetate is a new H2-receptor antagonist. A randomized double-blind clinical trial in fifty-three patients with endoscopically proven duodenal ulcers > 5 mm in diameter was undertaken to compare safety and efficacy of roxatidine with that of ranitidine. Twenty-six patients received roxatidine (75 mg bid while 27 patients received ranitidine (150 mg bid for 4 weeks. One patient in each group did not come for follow up. Roxatidine and ranitidine had comparable ulcer healing rates (22/25 vs 22/26; roxatidine, however, resulted in greater reduction in the number and severity of night time pain episodes (p < 0.05. No adverse event was reported during 4 weeks of treatment with roxatidine. Thus roxatidine achieves the primary therapeutic goal of relief of pain better than ranitidine.

  10. Roxatidine in duodenal ulcer.

    Science.gov (United States)

    Habibullah, C M; Habeeb, M A; Singh, S P

    1995-01-01

    Roxatidine acetate is a new H2-receptor antagonist. A randomized double-blind clinical trial in fifty-three patients with endoscopically proven duodenal ulcers > 5 mm in diameter was undertaken to compare safety and efficacy of roxatidine with that of ranitidine. Twenty-six patients received roxatidine (75 mg bid) while 27 patients received ranitidine (150 mg bid) for 4 weeks. One patient in each group did not come for follow up. Roxatidine and ranitidine had comparable ulcer healing rates (22/25 vs 22/26); roxatidine, however, resulted in greater reduction in the number and severity of night time pain episodes (p roxatidine. Thus roxatidine achieves the primary therapeutic goal of relief of pain better than ranitidine.

  11. Simultaneous adrenal pheochromocytoma and carotid body paraganglioma in a woman

    Energy Technology Data Exchange (ETDEWEB)

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

    2012-03-15

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

  12. Retroperitoneal Non-functioning Paraganglioma: A Case Report

    Directory of Open Access Journals (Sweden)

    Faten Limaiem

    2013-06-01

    Full Text Available Retroperitoneal paragangliomas are relatively rare endocrine tumours derived from the extra-adrenal paraganglia of the autonomic nervous system. They can be functional when they secrete catecholamines or non-functional. The diagnosis of a paraganglioma is infrequently made preoperatively unless the tumour is functional. In this paper, the authors report a new case of non-functional paraganglioma. A 54-year-old woman with a past medical history of viral hepatitis, presented with vague abdominal pain of five years' duration. On examination, no abdominal mass was palpable and her blood pressure was 100/60 mm Hg. Abdominal computed tomography scan demonstrated a well-defined hypervascular retroperitoneal mass of heterogeneous density in the left para-aortic region measuring 3cm in diameter. As paraganglioma was suspected preoperatively, serum and urine cathecholamine levels were performed and were within normal range. Complete surgical excision of this mass was performed. Histological examination of the surgical specimen coupled with immunohistochemical study was consistent with the diagnosis of pararganglioma. Retroperitoneal paragangliomas are most commonly benign with good prognosis, but can be locally invasive and metastasize as well. The possibility for malignant transformation of paragangliomas makes surgical excision the treatment of choice. [J Interdiscipl Histopathol 2013; 1(3.000: 168-171

  13. Imaging and management of head and neck paragangliomas

    Energy Technology Data Exchange (ETDEWEB)

    Berg, Rene van den [Leiden University Medical Center, Department of Radiology, Leiden (Netherlands)

    2005-07-01

    Paragangliomas of the head and neck are highly vascular lesions originating from paraganglionic tissue located at the carotid bifurcation (carotid body tumors), along the vagus nerve (vagal paragangliomas), and in the jugular fossa and tympanic cavity (jugulotympanic paragangliomas). Diagnostic imaging can be considered in two clinical situations: (1) patients who present with clinical symptoms suggestive of a paraganglioma, and (2) individuals from families with hereditary paragangliomas. It is not only necessary to detect and characterize the lesion, but also to study the presence of multiplicity. For these purposes, MR imaging, and especially 3D TOF MRA, is the modality of choice. CT scanning is especially useful to show destruction of the temporal bone. Angiography in combination with embolization will mainly be used prior to surgical resection, but can also be used for diagnostic purposes when the diagnosis is not yet clear. Many parameters play a role in the decision to treat of which multifocality and impairment of cranial nerves are the most important. The primary therapeutic option for paragangliomas is complete excision of tumor with preservation of vital neurovascular structures. Resection however, should be balanced against a more conservative ''wait and scan'' policy or palliative treatments such as radiotherapy. (orig.)

  14. Identification of eight novel SDHB, SDHC, SDHD germline variants in Danish pheochromocytoma/paraganglioma patients

    DEFF Research Database (Denmark)

    Bennedbæk, Marc; Rossing, Maria; Rasmussen, Åse K

    2016-01-01

    BACKGROUND: Germline mutations in the succinate dehydrogenase complex genes SDHB, SDHC, and SDHD predispose to pheochromocytomas and paragangliomas. Here, we examine the SDHB, SDHC, and SDHD mutation spectrum in the Danish population by screening of 143 Danish pheochromocytoma and paraganglioma...

  15. Comprehensive Molecular Characterization of Pheochromocytoma and Paraganglioma.

    Science.gov (United States)

    Fishbein, Lauren; Leshchiner, Ignaty; Walter, Vonn; Danilova, Ludmila; Robertson, A Gordon; Johnson, Amy R; Lichtenberg, Tara M; Murray, Bradley A; Ghayee, Hans K; Else, Tobias; Ling, Shiyun; Jefferys, Stuart R; de Cubas, Aguirre A; Wenz, Brandon; Korpershoek, Esther; Amelio, Antonio L; Makowski, Liza; Rathmell, W Kimryn; Gimenez-Roqueplo, Anne-Paule; Giordano, Thomas J; Asa, Sylvia L; Tischler, Arthur S; Pacak, Karel; Nathanson, Katherine L; Wilkerson, Matthew D

    2017-02-13

    We report a comprehensive molecular characterization of pheochromocytomas and paragangliomas (PCCs/PGLs), a rare tumor type. Multi-platform integration revealed that PCCs/PGLs are driven by diverse alterations affecting multiple genes and pathways. Pathogenic germline mutations occurred in eight PCC/PGL susceptibility genes. We identified CSDE1 as a somatically mutated driver gene, complementing four known drivers (HRAS, RET, EPAS1, and NF1). We also discovered fusion genes in PCCs/PGLs, involving MAML3, BRAF, NGFR, and NF1. Integrated analysis classified PCCs/PGLs into four molecularly defined groups: a kinase signaling subtype, a pseudohypoxia subtype, a Wnt-altered subtype, driven by MAML3 and CSDE1, and a cortical admixture subtype. Correlates of metastatic PCCs/PGLs included the MAML3 fusion gene. This integrated molecular characterization provides a comprehensive foundation for developing PCC/PGL precision medicine.

  16. [The jugulotympanic paragangliomas: 41 cases report].

    Science.gov (United States)

    Nguyen, D Q; Boulat, E; Troussier, J; Reyt, E I; Lavieille, J P; Schmerber, S I

    2005-01-01

    The jugulotympanic paragangliomas (JTP) represents the most frequent tumour of the middle ear but also of the temporal bone, after the acoustic neurinoma. The management of these vascular tumours remains uncleared. The purpose of this study was to report our experience about JTP in the CHU of Grenoble. Retrospective study of 41 patients, between 1973 and 1996. Six stages A, 8 stages B and 27 stages C are reported in whom 20 cases (49%) presented an intracranial extension (classification of Fisch). There were 2 familial cases with multiple localisations, in particular carotid. All the patients were divided in 3 groups: surgery or radiation therapy in first intention, surgery followed by radiation therapy. A total tumor removal without recurrence was achieved by surgery in more than 95% of the cases with 6 years follow-up but was associated with significant morbidity (major cranial nerve injury). We noticed one death by laryngospasme (C2Di2 tumour operated by infratemporal A approach). A stabilization of the tumour was obtained with radiotherapy in first intention in 75% of the cases (5 years follow-up) but with a risk of radionecrosis. A revision surgery was necessary in 3 cases. The comparaison of our different therapeutic management, surgery (23), radiation therapy (16) or combined (2), encourage us to perform a radical surgery whenever possible. Because of the slow rate of growth, the radiotherapy is indicated for older patients, at risk for surgery or extensive tumors. The objectives of the radiation therapy are to obtain a tumoral stabilization with improvement of the symptoms and low morbidity. The management of this rare pathology must be multidisciplinary. The recent discoveries on genes encoding three succinate dehydrogenase subunits (SDHD, SDHB et SDHC) will allow a genetic detection of asymptomatic case and will define the procedures for their management, coordinated by a national network PGL.NET. A retrospective study could also study the real incidence

  17. Pheochromocytoma and Paraganglioma: Current Functional and Future Molecular Imaging

    Directory of Open Access Journals (Sweden)

    Elise M Blanchet

    2012-01-01

    Full Text Available Paragangliomas are neural crest-derived tumors, arising either from chromaffin sympathetic tissue (in adrenal, abdominal, intra-pelvic or thoracic paraganglia or from parasympathetic tissue (in head and neck paraganglia. They have a specific cellular metabolism, with the ability to synthesize, store and secrete catecholamines (although most head and neck paragangliomas do not secrete any catecholamines. This disease is rare and also very heterogeneous, with various presentations (e.g., in regards to localization, multifocality, potential to metastasize, biochemical phenotype, and genetic background. With growing knowledge, notably about the pathophysiology and genetic background, guidelines are evolving rapidly. In this context, functional imaging is a challenge for the management of paragangliomas.Nuclear imaging has been used for exploring paragangliomas for the last three decades, with MIBG historically as the first-line exam. Tracers used in paragangliomas can be grouped in three different categories. Agents that specifically target catecholamine synthesis, storage, and secretion pathways include: 123 and 131I-metaiodobenzylguanidine (123/131I-MIBG, 18F-fluorodopamine (18F-FDA, and 18F-fluorodihydroxyphenylalanine (18F-FDOPA. Agents that bind somatostatin receptors include 111In-pentetreotide and 68Ga-labelled somatostatin analog peptides. The non-specific agent most commonly used in paragangliomas is 18F-fluorodeoxyglucose (18F-FDG. This review will first describe conventional scintigraphic exams that are used for imaging paragangliomas. In the second part we will emphasize the interest in new PET approaches (specific and non-specific, considering the growing knowledge about genetic background and pathophysiology, with the aim of understanding how tumors behave, and optimally adjusting imaging technique for each tumor type.

  18. Paragangliomas timpânicos: relato de casos Tympanic paragangliomas: case reports

    Directory of Open Access Journals (Sweden)

    Maria Eugênia L. R. B de V. Neto

    2005-02-01

    Full Text Available Os tumores glômicos, também chamados de paragangliomas, são formados por células não cromafins. O tumor é tipicamente vascular formado por vasos capilares e pré-capilares, interposto por células epiteliais. Para a sua abordagem inicial, deve-se ressaltar que os sintomas mais comumentes encontrados são o zumbido pulsátil e hipoacusia. A investigação através de imagem (tomografia computadorizada e ressonância magnética se faz necessária. Apresentaremos neste trabalho 5 pacientes portadores de paragangliomas timpânicos atendidos no Hospital (de 1995 a 2001. O sexo predominante foi o feminino, a idade variou de 48 a 60 anos, com média de 50 anos. A queixa predominante foi o zumbido pulsátil e a hipoacusia. A conduta foi cirúrgica em todos os casos.Glomus tumors, also called paragangliomas, originate from nonchromaffin cells. The tumor is typically vascular and grows from capillary and pre-capillary vessels in-between epithelial cells. It is worth mentioning that the most common symptoms are pulsating tinnitus and hearing loss. Imaging studies (CT and MRI are necessary for diagnosis. This paper shows five patients seen at the Hospital between 1995 and 2001 presenting glomus tympanicum. Women were most commonly affected, and the age ranged from 48 to 60 years (mean age of 50 years. The most common complaints were pulsating tinnitus and hearing loss. All patients were treated surgically.

  19. Paraganglioma na coluna vertebral: relato de caso Paraganglioma in the spine: case report

    Directory of Open Access Journals (Sweden)

    Maurício Santos Gusmão

    2012-04-01

    Full Text Available Os paragangliomas são tumores neuroendócrinos raros com origem em células especializadas derivadas da crista neural, acometendo a região da carótida ou jugulotimpânica. São raros dentro do canal vertebral, e quando ocorrem são normalmente encontrados no compartimento intradural e extramedular da região lombossacra. Este relato apresenta o caso de um paciente de 26 anos, sexo masculino, com diagnóstico de fratura patológica da coluna torácica (T10, secundária a paraganglioma, causando lesão medular completa. Foi realizado tratamento cirúrgico no Hospital Geral do Estado da Bahia, que apresentou sangramento excessivo como complicação que indicou necessidade de embolização prévia. Com isso, os autores recomendam cautela ao avaliar e tratar esta rara lesão causadora de déficit neurológico, cujo planejamento cirúrgico deve ser criterioso a fim de evitar complicações catastróficas.Paragangliomas are rare neuroendocrine tumors that originate in specialized cells derived from neural crest cells, affecting the region of the carotid or jugular-tympanic artery. They are rare within the spinal canal and, when they occur, they are normally found in the extramedullary intradural compartment of the lumbosacral region. This report presents the case of a 26-year-old male patient who was diagnosed with pathological fracture of the thoracic spine (T10, secondary to paraganglioma, thus causing complete spinal cord injury. Surgical treatment was performed at the General Hospital of the State of Bahia and the complication of excessive bleeding occurred, which indicated that there had been a need for prior embolization. Because of this, the authors recommend caution when evaluating and treating this rare lesion that causes neurological deficits, with careful surgical planning in order to catastrophic complications.

  20. Cardiac Paraganglioma Arising From the Right Atrioventricular Groove in a Paraganglioma-Pheochromocytoma Family Syndrome With Evidence of SDHB Gene Mutation: An Unusual Presentation.

    Science.gov (United States)

    Del Forno, Benedetto; Zingaro, Carlo; Di Palma, Enza; Capestro, Filippo; Rescigno, Giuseppe; Torracca, Lucia

    2016-09-01

    Primary cardiac paragangliomas are extremely rare. Recently this neoplasm has been associated with a familiar syndrome as a result of mutation of genes that encode proteins in the mitochondrial complex II. We report a case of a 46-year-old woman having cases of vertebral paraganglioma in her family showing an unusual anatomic and clinical presentation of cardiac paraganglioma and expressing a genetic mutation never associated before with cardiac localization of this neoplasm.

  1. Paraganglioma of the Urinary Bladder: A Case Report

    Directory of Open Access Journals (Sweden)

    Wan-Yi Kang

    2003-03-01

    Full Text Available Paragangliomas of the urinary bladder are very rare. To date, there are no reliable methods for predicting their clinical behavior, so long-term follow-up is required. We describe a paraganglioma of the urinary bladder in a 32-year-old male who presented with painless gross hematuria. Abdominal ultrasonography revealed a protruding mass with hematoma over the right lateral wall of the urinary bladder. The tumor was not completely resected by transurethral resection of bladder tumor (TURBT due to intermingling with the bladder wall. Follow-up I131-metaiodobenzylguanidine was performed 3 weeks after surgery and suspected incomplete resection lesions were noted. Histologic examination of the tumor indicated paraganglioma of the urinary bladder. We also provide a brief review of the literature for comparison.

  2. Retroperitoneal extra-adrenal paraganglioma: a rare but important diagnosis.

    LENUS (Irish Health Repository)

    Ahmad, S

    2012-01-31

    BACKGROUND: Extra-adrenal paragangliomas of the retroperitonium are infrequently diagnosed. Their malignant behaviour cannot be predicted on initial clinical and histological assessment. These tumours have higher propensity for subsequent metastasis compared with pargangliomas at other sites. AIM: We aim to describe a case report of an incidental finding of left retroperitoneal paraganglioma in a young man who presented with right flank pain. We also aim to emphasize the importance of diagnosis and the malignant potential of these tumours. METHOD: Patient\\'s clinical notes, operative findings, imaging studies and laboratory investigations including histology results were reviewed. A literature search was done to look into the incidence, presentation, follow-up plan and treatment options for these tumours. CONCLUSION: Surgical resection is the only available curative option for extra-adrenal paragangliomas. Metastasis is observed years after surgery, hence long-term follow-up is required.

  3. CT of gastro-duodenal obstruction.

    Science.gov (United States)

    Millet, I; Doyon, F Curros; Pages, E; Faget, C; Zins, M; Taourel, P

    2015-10-01

    Gastro-duodenal obstruction encompasses a spectrum of benign and malignant disease. Historically, chronic peptic ulcer disease was the main cause of gastro-duodenal obstruction, whereas now malignant cause with gastric carcinomas for gastric obstruction and pancreatic tumors for duodenal obstruction predominate. This paper reviews the role of CT in diagnosing gastro-duodenal obstruction, its level, its cause by identifying intraluminal, parietal, or extrinsic process, and the presence of complication.

  4. Paraganglioma of the thyroid gland: A case report

    Directory of Open Access Journals (Sweden)

    Filipović Aleksandar

    2014-01-01

    Full Text Available Introduction. Thyroid paraganglioma is a very rare malignant neuroendocrine tumor. Immunohistochemical features of thyroid paraganglioma are helpful for the diagnosis. Case report. A 69-year-old female came to hospital with the presence of a growing thyroid nodule of the left lobe. Ultrasonic neck examination showed 5 cm hypoechoic nodule in the left thyroid lobe. Thyroid scintigraphy showed a big cold nodule in the left lobe. Computed tomography (CT scan showed left lobe thyroid tumor with tracheal deviation on the right site. Extended total thyroidectomy was done. Intraoperative consultation with the pathologist confirmed thyroid cancer. The pathologist diagnosed thyroid paraganglioma on the base of immuohistochemical investigation. This thyroid paraganglioma was positive for neuron-specific enolase, chomogranin A, synaptophysin, and S-100 protein highlighted the sustentacular cells. Tumor cells were nega-tive for thyroglobulin, epithelial membrane antigen, cytokeratin, calcitonin, and carcinoembryonic. After the surgery the patient was treated with chemotherapy, peptide receptor radionuclide therapy, and permanent TSH suppressive therapy. The patient was followed with measurements of thyroid hormone and serum neuron-specific enolase, chromogranin A level, every 6 months. Gastroscopy, colonoscopy, chest and abdomen CT scan as well as further tests (chest x-ray, ultrasound of the neck, and whole body octreotide scintigraphy were done. No primary neuroendocrine tumor in digestive sistem or in the chest was found. After more than 3 years the patient has no evidence of the recurrent disease. Conclusion. Radical resection of thyroid paraganglioma, followed by chemotherapy and peptide receptor radionuclide therapy, should be considered the treatment of choice in patients with thyroid gland paraganglioma.

  5. Duodenal duplication cyst identified with MRCP

    Energy Technology Data Exchange (ETDEWEB)

    Carbognin, G.; Guarise, A.; Biasiutti, C.; Pagnotta, N.; Procacci, C. [Department of Radiology, University Hospital ' G.B. Rossi' , Verona (Italy)

    2000-08-01

    We report a case of a stalked cystic duodenal duplication. The lesion, hyperintense on T2-weighted GRE images, maintained the signal intensity after oral administration of a negative contrast agent (Lumirem, Guerbet, Aulnay-Sous-Bois, France), confirming its independence from the duodenal lumen. To our knowledge, this is the first demonstration of duodenal duplication by means of MR cholangiopancreatography. (orig.)

  6. Multiple giant duodenal ulcers associated with duodenal gastrinoma.

    Science.gov (United States)

    Fujihara, Shintaro; Mori, Hirohito; Nishiyama, Noriko; Kobayashi, Mitsuyoshi; Kobara, Hideki; Masaki, Tsutomu

    2012-02-01

    A 59-year-old Japanese man with a history of chronic hepatitis C and cirrhosis was admitted to hospital because of severe abdominal pain and diarrhea. His discomfort had begun 2 months earlier and was localized to the upper abdomen. Upper gastrointestinal endoscopy showed multiple ulcerative lesions from the duodenal bulb to the descending part of the duodenum, one of which was a giant ulcer that filled half of the intestinal lumen. Despite continuous intravenous lansoprazole therapy, his abdominal symptoms did not improve. Upper gastrointestinal endoscopy was again performed to detect the tumor, but it was difficult to observe the tumor with a conventional endoscope. We then inverted a transnasal endoscope into the duodenum, and this enabled us to detect a 15-mm submucosal tumor in the upper wall of the duodenal bulb. Examination of specimens from endoscopic ultrasonography fine-needle aspiration biopsy of the tumor revealed gastrinoma in the duodenal bulb. We decided to perform an operative resection. The patient's symptoms resolved after surgery, and he remained asymptomatic at follow-up 18 months later. Therefore, when it is difficult to detect the tumor directly by conventional endoscopy, we recommend that attempts be made to detect the tumor by inverting a transnasal endoscope into the duodenal bulb.

  7. A legacy of tinnitus: multiple head and neck paragangliomas

    Directory of Open Access Journals (Sweden)

    Jeremy J. Turner

    2009-12-01

    Full Text Available We describe the case of a patient who presented with a right-sided glomus jugulare tumor and bilateral glomus vagale tumors. These proved to be nonmalignant paragangliomas on histopathological analysis. Genetic analysis revealed a germline heterozygous missense mutation (Pro81Leu in the succinate dehydrogenase subunit D (SDHD gene. We discuss the clinical presentations of the familial paraganglioma syndrome type 1, which is caused by mutations in SDHD, and the implications for the clinical diagnosis and care of such patients.

  8. Pheochromocytoma-paraganglioma: Biochemical and genetic diagnosis.

    Science.gov (United States)

    Cano Megías, Marta; Rodriguez Puyol, Diego; Fernández Rodríguez, Loreto; Sención Martinez, Gloria Lisette; Martínez Miguel, Patricia

    Pheochromocytomas and paragangliomas are tumours derived from neural crest cells, which can be diagnosed by biochemical measurement of metanephrine and methoxytyramine. Advances in genetic research have identified many genes involved in the pathogenesis of these tumours, suggesting that up to 35-45% may have an underlying germline mutation. These genes have a singular transcriptional signature and can be grouped into 2 clusters (or groups): cluster 1 (VHL and SHDx), involved in angiogenesis and hypoxia pathways; and cluster 2 (MEN2 and NF1), linked to the kinase signalling pathway. In turn, these genes are associated with a characteristic biochemical phenotype (noradrenergic and adrenergic), and clinical features (location, biological behaviour, age of presentation, etc.) in a large number of cases. Early diagnosis of these tumours, accompanied by a correct genetic diagnosis, should eventually become a priority to enable better treatment, early detection of complications, proper screening of family members and related tumours, as well as an improvement in the overall prognosis of these patients.

  9. Pheochromocytoma-paraganglioma: Biochemical and genetic diagnosis

    Directory of Open Access Journals (Sweden)

    Marta Cano Megías

    2016-09-01

    Full Text Available Pheochromocytomas and paragangliomas are tumours derived from neural crest cells, which can be diagnosed by biochemical measurement of metanephrine and methoxytyramine. Advances in genetic research have identified many genes involved in the pathogenesis of these tumours, suggesting that up to 35–45% may have an underlying germline mutation. These genes have a singular transcriptional signature and can be grouped into 2 clusters (or groups: cluster 1 (VHL and SHDx, involved in angiogenesis and hypoxia pathways; and cluster 2 (MEN2 and NF1, linked to the kinase signalling pathway. In turn, these genes are associated with a characteristic biochemical phenotype (noradrenergic and adrenergic, and clinical features (location, biological behaviour, age of presentation, etc. in a large number of cases. Early diagnosis of these tumours, accompanied by a correct genetic diagnosis, should eventually become a priority to enable better treatment, early detection of complications, proper screening of family members and related tumours, as well as an improvement in the overall prognosis of these patients.

  10. CLINICAL STUDY OF DUODENAL PERFORATION

    Directory of Open Access Journals (Sweden)

    Sambasiva Rao

    2016-04-01

    Full Text Available BACKGROUND The duodenal injury can pose a formidable challenge to the surgeon and failure to manage it properly may have devastating results. Over the centuries, there was little to offer the patient of acute abdomen beyond cupping, purgation and enemas, all of which did more harm than good. It was not until 1884 that Mikulicz made an attempt to repair a perforation. Recent statistics indicate roughly 10% of population develop gastric or duodenal ulcer in life time. Roughly 1-3% of population above the age of 20 years have some degree of peptic ulcer activity during any annual period. A detailed history with regards to the signs and symptoms of the patient, a meticulous examination, radiological and biochemical investigations help to arrive at a correct preoperative diagnosis. In this study, a sincere effort has been put to understand the demographic patterns, to understand the underlying aetiology and to understand the effectiveness of the standard methods of investigation and treatment in use today. METHODS This is a 24 months prospective study i.e., from September 2011 to September 2013 carried out at Dr. Pinnamaneni Siddhartha Institute of Medical Sciences & Research Foundation. The study included the patients presenting to Dr. Pinnamaneni Siddhartha Institute of Medical Sciences & Research Foundation to emergency ward with signs and symptoms of hollow viscus perforation. The sample size included 30 cases of duodenal perforation. RESULTS Duodenal ulcer perforation commonly occurs in the age group of 30-60 years, but it can occur in any age group. Majority of the patients were male. Smoking and alcohol consumption were risk factors in most cases (53.3% for the causation of duodenal ulcer perforation. Sudden onset of abdominal pain, situated at epigastrium and right hypochondrium was a constant symptom (100%. Vomiting, constipation and fever were not so common. CONCLUSION The emergency surgical management for perforated duodenal ulcer is by

  11. Head and neck paragangliomas : genetics, heredity and clinical characteristics

    NARCIS (Netherlands)

    Hensen, Erik Frans

    2012-01-01

    Paragangliomas are rare neoplasms that occur predominantly in the head and neck region. They originate from the neural crest derived cells within paraganglions, and are closely related to the pheochromocytomas of the adrenal medulla. In contrast to other European countries, the majority of head and

  12. Head and neck paragangliomas : genetics, heredity and clinical characteristics

    NARCIS (Netherlands)

    Hensen, Erik Frans

    2012-01-01

    Paragangliomas are rare neoplasms that occur predominantly in the head and neck region. They originate from the neural crest derived cells within paraganglions, and are closely related to the pheochromocytomas of the adrenal medulla. In contrast to other European countries, the majority of head and

  13. MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma

    NARCIS (Netherlands)

    N. Burnichon (Nelly); A. Cascoń (Alberto); F. Schiavi (Francesca); N. Morales (NicolePaes); I. Comino-Méndez (Iñaki); N. Abermil (Nasséra); L. Inglada-Pérez (Lucía); A.A. de Cubas (Aguirre); L. Amar (Laurence); M. Barontini (Marta); S.B. de Quiroś (Sandra Bernaldo); J. Bertherat (Jerome); Y.-J. Bignon (Yves-Jean); M.J. Blok (Marinus); S. Bobisse (Sara); S. Borrego (Salud); M. Castellano (Maurizio); P. Chanson (Philippe); A. de Chiara; E.P. Corssmit (Eleonora); M. Giacchè (Mara); R.R. de Krijger (Ronald); T. Ercolino (Tonino); X. Girerd (Xavier); E.B. Gómez García (Encarna); Á. Gómez-Graña (Álvaro); I. Guilhem (Isabelle); F.J. Hes (Frederik); E. Honrado (Emiliano); E. Korpershoek (Esther); J.W. Lenders (Jacques); R. Letón (Rocío); A.R. Mensenkamp (Arjen); A. Merlo (Anna); L. Mori (Luigi); A. Murat (Arnaud); P. Pierre (Peggy); P.F. Plouin (Pierre-Franco̧is); T. Prodanov (Tamara); M. Quesada-Charneco (Miguel); N. Qin (Nan); E. Rapizzi (Elena); E. Raymond (Eric); N. Reisch (Nicole); G. Roncador (Giovanna); M. Ruiz-Ferrer (Macarena); F. Schillo (Frank); A.P.A. Stegmann (Sander); C. Suarez (Carlos); E. Taschin (Elisa); H.J.L.M. Timmers; C. Tops (Carli); M. Urioste (Miguel); F. Beuschlein (Felix); K. Pacak (Karel); M. Mannelli (Massimo); P.L. Dahia (Patricia); G. Opocher (Giuseppe); G. Eisenhofer (Graeme); A.P. Gimenez-Roqueplo; M. Robledo (Mercedes)

    2012-01-01

    textabstractPurpose: Pheochromocytomas (PCC) and paragangliomas (PGL) are genetically heterogeneous neural crest-derived neoplasms. Recently we identified germline mutations in a new tumor suppressor susceptibility gene, MAX (MYC-associated factor X), which predisposes carriers to PCC. How MAX

  14. Raised dopamine metabolites in a case of malignant paraganglioma.

    OpenAIRE

    Florkowski, C M; Fairlamb, D. J.; Freeth, M G; Taylor, S.A.; A. Taylor; Weinkove, C.; Jacobs, A. G.

    1990-01-01

    This paper describes the case of a malignant retroperitoneal paraganglioma with extensive metastases. The patient presented with a supraclavicular mass and an absence of hypertension. Exclusively raised dopamine metabolites were detected which may be a marker of a malignant process and account for the lack of hypertension.

  15. MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma

    NARCIS (Netherlands)

    N. Burnichon (Nelly); A. Cascoń (Alberto); F. Schiavi (Francesca); N. Morales (NicolePaes); I. Comino-Méndez (Iñaki); N. Abermil (Nasséra); L. Inglada-Pérez (Lucía); A.A. de Cubas (Aguirre); L. Amar (Laurence); M. Barontini (Marta); S.B. de Quiroś (Sandra Bernaldo); J. Bertherat (Jerome); Y.-J. Bignon (Yves-Jean); M.J. Blok (Marinus); S. Bobisse (Sara); S. Borrego (Salud); M. Castellano (Maurizio); P. Chanson (Philippe); A. de Chiara; E.P. Corssmit (Eleonora); M. Giacchè (Mara); R.R. de Krijger (Ronald); T. Ercolino (Tonino); X. Girerd (Xavier); E.B. Gómez García (Encarna); Á. Gómez-Graña (Álvaro); I. Guilhem (Isabelle); F.J. Hes (Frederik); E. Honrado (Emiliano); E. Korpershoek (Esther); J.W. Lenders (Jacques); R. Letón (Rocío); A.R. Mensenkamp (Arjen); A. Merlo (Anna); L. Mori (Luigi); A. Murat (Arnaud); P. Pierre (Peggy); P.F. Plouin (Pierre-Franco̧is); T. Prodanov (Tamara); M. Quesada-Charneco (Miguel); N. Qin (Nan); E. Rapizzi (Elena); E. Raymond (Eric); N. Reisch (Nicole); G. Roncador (Giovanna); M. Ruiz-Ferrer (Macarena); F. Schillo (Frank); A.P.A. Stegmann (Sander); C. Suarez (Carlos); E. Taschin (Elisa); H.J.L.M. Timmers; C. Tops (Carli); M. Urioste (Miguel); F. Beuschlein (Felix); K. Pacak (Karel); M. Mannelli (Massimo); P.L. Dahia (Patricia); G. Opocher (Giuseppe); G. Eisenhofer (Graeme); A.P. Gimenez-Roqueplo; M. Robledo (Mercedes)

    2012-01-01

    textabstractPurpose: Pheochromocytomas (PCC) and paragangliomas (PGL) are genetically heterogeneous neural crest-derived neoplasms. Recently we identified germline mutations in a new tumor suppressor susceptibility gene, MAX (MYC-associated factor X), which predisposes carriers to PCC. How MAX mutat

  16. Mutations associated with succinate dehydrogenase D-related malignant paragangliomas.

    NARCIS (Netherlands)

    Timmers, H.J.L.M.; Pacak, K.; Bertherat, J.; Lenders, J.W.M.; Duet, M.; Eisenhofer, G.; Stratakis, C.A.; Niccoli-Sire, P.; Tran, B.H.; Burnichon, N.; Gimenez-Roqueplo, A.P.

    2008-01-01

    OBJECTIVE: Hereditary paraganglioma (PGL) syndromes result from germline mutations in genes encoding subunits B, C and D of the mitochondrial enzyme succinate dehydrogenase (SDHB, SDHC and SDHD). SDHB-related PGLs are known in particular for their high malignant potential. Recently, however, maligna

  17. Clinical aspects of SDHx-related pheochromocytoma and paraganglioma.

    NARCIS (Netherlands)

    Timmers, H.J.L.M.; Gimenez-Roqueplo, A.P.; Mannelli, M.; Pacak, K.

    2009-01-01

    Paragangliomas (PGLs) derive from either sympathetic chromaffin tissue in adrenal and extra-adrenal abdominal or thoracic locations, or from parasympathetic tissue of the head and neck. Mutations of nuclear genes encoding subunits B, C, and D of the mitochondrial enzyme succinate dehydrogenase (SDHB

  18. SOLITARY PARAGANGLIOMA OF THE HYPOGLOSSAL NERVE: CASE REPORT.

    LENUS (Irish Health Repository)

    2012-02-01

    SOLITARY PARAGANGLIOMA OF THE HYPOGLOSSAL NERVE:: Case Report BACKGROUND AND IMPORTANCE:: We report the case history of solitary hypoglossal paraganglioma in a 64-year-old woman. The surgical difficulties encountered in the removal of this challenging tumour are discussed with literature review. CLINICAL PRESENTATION:: A 64-year-old woman presented with a short history of dysphonia, occasional dysphagia, tinnitus, altered taste, and unilateral left sided tongue wasting. On examination there was left lower motor hypoglossal paralysis. Imaging showed a discrete enhancing lobulated mass, measuring 2cm x 2cm, in the region of the hypoglossal nerve extending into the hypoglossal canal suggestive of hypoglossal paraganglioma. A left dorsolateral sub occipital craniotomy was carried out in the sitting position. The hypoglossal nerve appeared to be enlarged and the jugular foramen was normal. Complete surgical debulking of the tumour was not attempted due to its vascular nature. The nerve was decompressed and neuropathology confirmed a low grade paraganglioma arising from the hypoglossal nerve. The patient is scheduled to receive stereotactic radiation for further management. CONCLUSION:: When a case of solitary hypoglossal paraganglioma is encountered in clinical practice, the aim of management should be mainly focussed on achieving a diagnosis and preserving the hypoglossal nerve function. If there is evidence of vascularity in the lesion noted in the MRI scan, a pre-operative angiogram should be performed with a view for embolisation.We decompressed the hypoglossal canal and achieved a good improvement in the patient\\'s symptoms. We recommend stereotactic radiosurgery for remnant and small hypoglossal tumours and regular follow up with MRI scans.

  19. SOLITARY PARAGANGLIOMA OF THE HYPOGLOSSAL NERVE: CASE REPORT.

    LENUS (Irish Health Repository)

    Raza, Kazim

    2011-01-25

    SOLITARY PARAGANGLIOMA OF THE HYPOGLOSSAL NERVE:: Case Report BACKGROUND AND IMPORTANCE:: We report the case history of solitary hypoglossal paraganglioma in a 64-year-old woman. The surgical difficulties encountered in the removal of this challenging tumour are discussed with literature review. CLINICAL PRESENTATION:: A 64-year-old woman presented with a short history of dysphonia, occasional dysphagia, tinnitus, altered taste, and unilateral left sided tongue wasting. On examination there was left lower motor hypoglossal paralysis. Imaging showed a discrete enhancing lobulated mass, measuring 2cm x 2cm, in the region of the hypoglossal nerve extending into the hypoglossal canal suggestive of hypoglossal paraganglioma. A left dorsolateral sub occipital craniotomy was carried out in the sitting position. The hypoglossal nerve appeared to be enlarged and the jugular foramen was normal. Complete surgical debulking of the tumour was not attempted due to its vascular nature. The nerve was decompressed and neuropathology confirmed a low grade paraganglioma arising from the hypoglossal nerve. The patient is scheduled to receive stereotactic radiation for further management. CONCLUSION:: When a case of solitary hypoglossal paraganglioma is encountered in clinical practice, the aim of management should be mainly focussed on achieving a diagnosis and preserving the hypoglossal nerve function. If there is evidence of vascularity in the lesion noted in the MRI scan, a pre-operative angiogram should be performed with a view for embolisation.We decompressed the hypoglossal canal and achieved a good improvement in the patient\\'s symptoms. We recommend stereotactic radiosurgery for remnant and small hypoglossal tumours and regular follow up with MRI scans.

  20. Magnetic resonance imaging findings of a nonfunctional mediastinal paraganglioma with an unusual presentation

    Energy Technology Data Exchange (ETDEWEB)

    Sahin-Akyar, G. [Department of Radiology, University of Ankara, Faculty of Medicine, Ibn-i Sina Hospital, Samanpazari, TR-06100 Ankara (Turkey); Erden, I. [Department of Radiology, University of Ankara, Faculty of Medicine, Ibn-i Sina Hospital, Samanpazari, TR-06100 Ankara (Turkey); Yagci, C. [Department of Radiology, University of Ankara, Faculty of Medicine, Ibn-i Sina Hospital, Samanpazari, TR-06100 Ankara (Turkey); Akyar, S. [Department of Radiology, University of Ankara, Faculty of Medicine, Ibn-i Sina Hospital, Samanpazari, TR-06100 Ankara (Turkey); Erekul, S. [Department of Pathology, University of Ankara, Faculty of Medicine, Ibn-i Sina Hospital, Samanpazari, TR-06100 Ankara (Turkey)

    1997-09-01

    A case of histologically proven mediastinal paraganglioma presenting with metastatic spread to supraclavicular lymph nodes is presented. Mediastinal paragangliomas are extremely rare tumors and their CT and MR imaging features have not been well documented in the radiologic literature. The T 1-weighted and T 2-weighted spin-echo MR findings of a locally invasive mediastinal paraganglioma with metastasis to the supraclavicular lymph nodes are described and the literature is briefly reviewed. (orig.). With 4 figs.

  1. Demonstration of S-100 protein in sustentacular cells of phaeochromocytomas and paragangliomas

    DEFF Research Database (Denmark)

    Schroder, H D; Johannsen, L

    1986-01-01

    Eighteen phaeochromocytomas, including both sporadic and familial cases, four cervical paragangliomas, two jugular paragangliomas, and one abdominal paraganglioma were examined immunohistochemically for the presence of S-100 protein. Positive staining in cells morphologically similar...... protein further supporting their Schwann cell relationship. The number of S-100 positive cells varied considerably. They demonstrated a spindle celled or elongated configuration with long slender processes. The nature of the sustentacular cell proliferation, neoplastic versus reactive, is discussed....

  2. Abdominal compartment syndrome from bleeding duodenal diverticulum

    Directory of Open Access Journals (Sweden)

    Vakhtang Tchantchaleishvili

    2012-01-01

    Full Text Available Duodenal diverticuli are acquired false diverticuli of unknown etiology. Although mostly asymptomatic, they can occasionally cause upper gastrointestinal hemorrhage, rarely with massive bleeding. In this report, we present (to the best of our knowledge the first reported case of duodenal diverticular bleeding, causing abdominal compartment syndrome. Albeit a rare event, duodenal diverticular bleeding should be included in the differential diagnosis of upper gastrointestinal bleeding. As with our case, a multidisciplinary approach to managing such patients is crucial.

  3. Malignant paraganglioma with vertebral metastasis: case report Paraganglioma maligno com metástase vertebral: relato de caso

    Directory of Open Access Journals (Sweden)

    Bruno Lázaro

    2003-06-01

    Full Text Available A paraganglioma is a rare tumor, composed of chromaffin cells, groups of cells associated to the autonomous system. When the tumor occurs in the adrenal gland, it is called pheochromocitoma. The malignant paraganglioma is a very rare presentation; it is diagnosed by local recurrence after total resection of the primary mass, or findings of distant metastases. We present a case report of a 29-year-old woman with cervico-brachial pain. In 1995 she underwent a carotid body tumor resection. Magnetic resonance imaging (MRI, plain X-rays and computerized tomography scan revealed multiple lesions in C5, T5 and T12. She underwent a surgical procedure to correct the cervical lesion. The histological and immunohistochemical assays revealed a malignant paraganglioma. She received adjuvant radiotherapy, showing clinical improvement after treatment, presenting no symptoms after one year. The therapeutic approach is based on the total resection of the tumor. The treatment of distant metastases can be made with adjuvant measures such as conventional radiotherapy, I¹³¹-MIBG, or chemotherapy, especially in malignant pheochromocitomas.O paraganglioma é tumor raro, composto de células cromafins, associado ao sistema nervoso autônomo. Quando localizado na glândula supra-renal, o tumor é chamado feocromocitoma. Descreve-se um caso de paciente do sexo feminino, 29 anos, que se apresentou com cervicobraquialgia e que havia sido operada em 1995 para exérese de tumor glômico da carótida cervical. RM, RX e TC revelaram múltiplas lesões acometendo o corpo vertebral de C5, T5 e T12. Foi submetida à ressecção cirúrgica radical da lesão cervical, com substituição do corpo vertebral por prótese de titânio. A histopatologia e o estudo imunohistoquímico da lesão confirmaram o diagnóstico de paraganglioma maligno. As outras lesões foram tratadas com radioterapia. Um ano após os procedimentos, a paciente apresenta-se assintomática. O tratamento destas

  4. Retroperitoneal Paraganglioma – a rare cause of arterial hypertension

    Directory of Open Access Journals (Sweden)

    Isabel Silva

    2017-03-01

    Full Text Available Paragangliomas are rare neuroendocrine tumours, most commonly found in adults. These differ from pheochromocytomas in that their location is extra-adrenal, and they are responsible for about 1% of arterial hypertension aetiologies. We report the case of a 30-year-old female whose past medical history was unremarkable. However, her arterial hypertension led to further examination in search of secondary aetiologies, in which a retroperitoneal mass and an increase in levels of catecholamines were detected; findings that led to the final diagnosis of paraganglioma. A multidisciplinary team, whose approach was to use pharmacological alpha-adrenergic blocking agents and a surgical resection of the lesion, treated the patient. The patient is clinically well but will continue to be monitored as an outpatient, and genetic testing is being encouraged.

  5. Solitary paraganglioma of the hypoglossal nerve: case report.

    LENUS (Irish Health Repository)

    Raza, Kazim

    2011-04-01

    BACKGROUND AND IMPORTANCE: We report the case history of solitary hypoglossal paraganglioma in a 64-year-old woman. The surgical difficulties encountered in the removal of this challenging tumor are discussed and as a literature review provided. CLINICAL PRESENTATION: A 64-year-old woman presented with a short history of dysphonia, occasional dysphagia, tinnitus, altered taste, and unilateral left-sided tongue wasting. On examination, there was left lower motor hypoglossal paralysis. Imaging showed a discrete enhancing lobulated mass, measuring 2 × 2 cm, in the region of the hypoglossal nerve extending into the hypoglossal canal suggestive of hypoglossal paraganglioma. A left dorsolateral suboccipital craniotomy was performed with the patient in the sitting position. The hypoglossal nerve appeared to be enlarged, and the jugular foramen was normal. Complete surgical debulking of the tumor was not attempted because of its vascular nature. The nerve was decompressed, and neuropathology confirmed a low-grade paraganglioma arising from the hypoglossal nerve. The patient was scheduled to receive stereotactic radiation for further management. CONCLUSION: When a case of solitary hypoglossal paraganglioma is encountered in clinical practice, the aim of management should be mainly focused on achieving a diagnosis and preserving the hypoglossal nerve function. If there is evidence of vascularity in the lesion noted on magnetic resonance imaging, a preoperative angiogram should be obtained with a view for embolization. We decompressed the hypoglossal canal and achieved good improvement in the patient\\'s symptoms. We recommend stereotactic radiosurgery for remnant and small hypoglossal tumors and regular follow-up with magnetic resonance imaging scans.

  6. Solitary paraganglioma of the hypoglossal nerve: case report.

    LENUS (Irish Health Repository)

    Raza, Kazim

    2012-02-01

    BACKGROUND AND IMPORTANCE: We report the case history of solitary hypoglossal paraganglioma in a 64-year-old woman. The surgical difficulties encountered in the removal of this challenging tumor are discussed and as a literature review provided. CLINICAL PRESENTATION: A 64-year-old woman presented with a short history of dysphonia, occasional dysphagia, tinnitus, altered taste, and unilateral left-sided tongue wasting. On examination, there was left lower motor hypoglossal paralysis. Imaging showed a discrete enhancing lobulated mass, measuring 2 x 2 cm, in the region of the hypoglossal nerve extending into the hypoglossal canal suggestive of hypoglossal paraganglioma. A left dorsolateral suboccipital craniotomy was performed with the patient in the sitting position. The hypoglossal nerve appeared to be enlarged, and the jugular foramen was normal. Complete surgical debulking of the tumor was not attempted because of its vascular nature. The nerve was decompressed, and neuropathology confirmed a low-grade paraganglioma arising from the hypoglossal nerve. The patient was scheduled to receive stereotactic radiation for further management. CONCLUSION: When a case of solitary hypoglossal paraganglioma is encountered in clinical practice, the aim of management should be mainly focused on achieving a diagnosis and preserving the hypoglossal nerve function. If there is evidence of vascularity in the lesion noted on magnetic resonance imaging, a preoperative angiogram should be obtained with a view for embolization. We decompressed the hypoglossal canal and achieved good improvement in the patient\\'s symptoms. We recommend stereotactic radiosurgery for remnant and small hypoglossal tumors and regular follow-up with magnetic resonance imaging scans.

  7. [Carotid body paraganglioma in a teenager. Case report].

    Science.gov (United States)

    López-Vázquez, María Elisa; Llamas-Macías, Francisco Javier; Nuño-Escobar, César; González-Ojeda, Alejandro; Fuentes-Orozco, Clotilde; Macías-Amezcua, Michel Dassaejv

    2014-01-01

    Paragangliomas of the head and neck are neuroendocrine tumors. They have a low incidence (0.6%), are generally benign, have a poorly defined etiology, and multiple factors have been associated with their origin. Humans and other species living at high altitudes (>2000 m above sea level) are subjected to a relatively chronic hypoxia and there is a high prevalence of the development of carotid body hyperplasia and eventually paragangliomas. This disease is usually seen in patients in their 50s and in their 30s if there is a family history. We present the case of a 16 year-old female with acute pharyngitis and growing tumor located on the left side of the neck, without symptoms. A duplex Doppler ultrasound showed a solid nodular lesion on the left carotid bifurcation. A left lateral cervicotomy was performed, finding a highly vascularized tumor of 4 × 3 × 3 cm involving the common carotid from its middle third, the internal carotid up to the cranial base, and the external carotid to its upper third, and intimately related to the trachea, esophagus and cervical spine. The tumor was completely resected and the histopathological analysis corroborated the presence of paragangliomas. The publication of this case is relevant and of clinical interest due to the uncommon age of presentation and the fact that it should be considered as a diagnostic possibility.

  8. A case of familial paraganglioma syndrome type 4 caused by a mutation in the SDHB gene.

    Science.gov (United States)

    Drucker, Aaron M; Houlden, Robyn L

    2006-12-01

    A 40-year-old man was referred to our clinic with recurrent paragangliomas. He had undergone resection of a paraganglioma superior to the right adrenal gland at 19 years of age, resection of two para-aortic paragangliomas at 39 years of age, and resection of a paraganglioma in the interatrial septum at 40 years. The patient's mother had died at age 39 years of metastases from a carotid body tumor. MRI and CT scanning, 131I-labeled metaiodobenzylguanidine scanning, and genetic testing for a mutation in the succinate dehydrogenase complex, subunit B gene. Familial paraganglioma syndrome type 4 caused by a mutation in the succinate dehydrogenase complex, subunit B gene. The patient underwent two surgical procedures in our clinic. The first was to remove two para-aortic paragangliomas, and the second to remove a paraganglioma that involved both atria. The patient is at high risk for malignant disease and should undergo an annual monitoring program that consists of physical examination and measurement of his blood pressure and levels of urinary catecholamines and metanephrines. If these procedures suggest a recurrence of paraganglioma, 123I-labeled metaiodobenzylguanidine scanning should be performed. As he might develop nonfunctional tumors, however, he should also undergo CT scanning, MRI scanning, or both, of the neck, thorax, abdomen, and pelvis every 6-12 months. Genetic testing has been offered to family members.

  9. Melanotic paraganglioma arising in the temporal horn following Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Jeong Hyun [Baylor College of Medicine, Neuroradiology Department, Houston, TX (United States); Ewha Womans University, Radiology Department, School of Medicine, Seoul (Korea); Rivera, Andreana [Baylor College of Medicine, Pathology Department, Houston, TX (United States); Naeini, Ramin M.; Yedururi, Sireesha; Megahead, Hatem [Baylor College of Medicine, Radiology Department, Houston, TX (United States); Bayindir, Petek [Ege Universitesi, Radiology Department, Lojmanlari (Turkey); Fuller, Gregory N. [MD Anderson Cancer Center, Pathology Department, Houston, TX (United States); Suh, Jeong Soo [Ewha Womans University, Radiology Department, School of Medicine, Seoul (Korea); Adesina, Adekunle M. [Baylor College of Medicine, Pathology Department, Houston, TX (United States); Texas Children' s Cancer Center, Houston, TX (United States); Hunter, Jill V. [Texas Children' s Cancer Center, Houston, TX (United States); Baylor College of Medicine, Neuroradiology Department, Houston, TX (United States)

    2008-05-15

    Intracerebral paragangliomas are rare because of the lack of paraganglial cells in the cerebral tissue. We report a rare case of melanotic paraganglioma arising from the temporal horn of the lateral ventricle in a patient with prior Langerhans cell histiocytosis (LCH) treated with chemotherapy and radiation. (orig.)

  10. Imaging findings of a primary paraganglioma of the liver: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Ji, Seung Woo; Kang, Ung Rae; Park, Jae Bok [Daegu Catholic University Medical Center, Catholic University of Daegu College of Medicine, Daegu (Korea, Republic of)

    2016-11-15

    Primary hepatic paraganglioma is an extremely rare type of tumor originating from extra-adrenal chromaffin cells. We report a case of primary intrahepatic paraganglioma in a 52-year-old man, with pathologic confirmation through right hepatectomy. An imaging study indicated a predominately hemorrhagic septated cystic mass and peripheral marked enhancement of the solid portions, which showed persistent enhancement.

  11. Traumatic duodenal hematoma in the pediatric patient.

    Science.gov (United States)

    Winthrop, A L; Wesson, D E; Filler, R M

    1986-09-01

    Twenty children with duodenal hematomas secondary to blunt trauma were treated between 1953 and 1983. The duodenal injury was isolated in ten cases and associated with intra-abdominal injuries in the others. In ten, the duodenal injury was suspected on admission and the diagnosis was confirmed within 24 hours by radiographic contrast studies. All ten were managed successfully with nasogastric suction and intravenous fluids. Ten patients underwent laparotomy for increasing abdominal tenderness and guarding. An isolated duodenal hematoma was found in four and treated by evacuation and/or gastroenterostomy. In five of the remaining six surgical patients, all of whom had multiple intra-abdominal injuries, the duodenum was left untouched. Three of these patients had postoperative contrast studies that showed early resolution of the duodenal hematoma. No duodenal stricture or leak developed in any patient. The children with isolated duodenal hematomas who were treated conservatively had a mean hospital stay of six days, whereas those treated surgically had a mean stay of 17 days. The ten patients with multiple intra-abdominal injuries had a mean hospital stay of 32 days. In this group, eight required total parenteral nutrition or nasojejunal feeds for nutritional support. In these patients, an isolated duodenal hematoma resulted in minimal morbidity and nonoperative management was usually successful. The presence of associated intra-abdominal injuries was responsible for the prolonged hospitalization and delayed return of normal intestinal function in some patients.

  12. Duodenal Transection without Pancreatic Injury following Blunt ...

    African Journals Online (AJOL)

    Journal of Surgical Technique and Case Report | Jul-Dec 2014 | Vol-6 | Issue-2. 67. Duodenal Transection ... Early interventions in duodenal injuries have improved outcome. Here we report a ... developed abdominal pain along with single episode of ... challenge to the surgeon and failure to manage it properly can result in ...

  13. Duodenal polyposis secondary to portal hypertensive duodenopathy

    Institute of Scientific and Technical Information of China (English)

    Ananta; Gurung; Philip; E; Jaffe; Xuchen; Zhang

    2015-01-01

    Portal hypertensive duodenopathy(PHD) is a recognized, but uncommon finding of portal hypertension in cirrhotic patients. Lesions associated with PHD include erythema, erosions, ulcers, telangiectasia, exaggerated villous pattern and duodenal varices. However, duodenal polyposis as a manifestation of PHD is rare. We report a case of a 52-year-old man who underwent esophagogastroduodenoscopy and was found with multiple small duodenal polyps ranging in size from 1-8 mm. Biopsy of the representative polyps revealed polypoid fragments of duodenal mucosa with villiform hyperplasia lined by reactive duodenal/gastric foveolar epithelium and underlying lamina propria showed proliferating ectatic and congested capillaries. The features were diagnostic of polyps arising in the setting of PHD.

  14. Perforated duodenal ulcer: Emerging pattern

    Directory of Open Access Journals (Sweden)

    Murtaza Ali Asger Calcuttawala

    2014-01-01

    Full Text Available Background: A total of 27 patients of perforated duodenal ulcer admitted in our institution between December 2010 and November 2012 were treated and studied. Materials and Methods: All patients were diagnosed on the basis of clinical and radiological findings, exploratory laparotomy was performed and simple closure of perforation with placement of Graham′s omental patch was carried out. This was followed by triple regimen for Helicobacter pylori eradication. Results: All patients were male, maximum incidence (61.54% was noted in the age group of 21-30, ′O′ +ve blood group was most commonly observed in our patients. Eight patients had history suggestive of acute acid peptic disease. Mean time interval between the start of symptoms and surgery was 43 h. No morbidity except minimal pleural effusion was seen in one case. There was no mortality in our series. Conclusion: We conclude that although a number of definitive surgeries have been described for acid peptic disease, but the requirement of such procedures has come down due to increasing use of H. pylori eradication therapy and proton pump inhibitors. However, surgery for complications especially for duodenal ulcer perforation has not reduced concomitantly. Incidence is greater in young males.

  15. Adenocarcinoma of the Minor Duodenal Papilla: Report of a Case

    Directory of Open Access Journals (Sweden)

    Kazuhiro Takami

    2011-04-01

    Full Text Available An 81-year-old male was found to have a duodenal tumor by screening upper gastrointestinal endoscopy. The tumor was located in the minor duodenal papilla. Pathological examination of the biopsy specimen revealed adenocarcinoma, and endoscopic ultrasound showed an elevated hypoechoic mass in the minor duodenal papilla. The preoperative diagnosis was therefore considered to be either adenocarcinoma of the minor duodenal papilla or duodenal cancer. We performed a subtotal stomach-preserving pancreaticoduodenectomy. Histopathological examination of the resected specimen showed the tumor cells to be primarily located in the submucosa of the minor duodenal papilla, with slight invasion into the pancreatic parenchyma through the accessory pancreatic duct. We therefore diagnosed a primary adenocarcima of the minor duodenal papilla. Adenocarcinoma of the minor duodenal papilla is considered to be a rare disease, but it may be underestimated because of the difficulty in distinguishing advanced adenocarcinoma of the minor duodenal papilla from primary duodenal cancer and cancer of the pancreatic head.

  16. Acute Necrotizing Esophagitis Followed by Duodenal Necrosis.

    Science.gov (United States)

    Del Hierro, Piedad Magdalena

    2011-12-01

    Acute Necrotizing Esophagitis is an uncommon pathology, characterized by endoscopic finding of diffuse black coloration in esophageal mucosa and histological presence of necrosis in patients with upper gastrointestinal bleeding. The first case of acute necrotizing esophagitis followed by duodenal necrosis, in 81 years old woman with a positive history of Type 2 Diabetes Mellitus, Hypertension, and usual intake of Nonsteroidal Anti-inflammatory drugs, is reported. Although its etiology remains unknown, the duodenal necrosis suggests that ischemia could be the main cause given that the branches off the celiac axis provide common blood supply to the distal esophageal and duodenal tissue. The massive gastroesophagic reflux and NSAID intake could be involved.

  17. [ASSOCIATION OF DUODENAL DIVERTICULUM PRESENCE WITH CHOLEDOCHOLITHIASIS

    Science.gov (United States)

    Machado Mayurí, Alberto; Gómez, Aldo; Yriberry, Simón; Valdiviezo, Alfredo; Lozano, Adelina; Villena, Ovidio

    1998-01-01

    OBJECTIVES: The aim of this study was to investigate in aretrospective way the frequency of duodenal diverticulum and its association with choledocholithiasis. Methods:This study included 2728 consecutive patients who underwent ERCP during the period 1993-1997. Duodenal diverticulum was discovered in 122 cases (4.5%). In 22 patients cannulation was not achieved since it was not technically possible. Results: In 100 patients with successful cannulation, 54 cases with juxtapapillary diverticulum were encountered associated with choledocholithiasis. CONCLUSION: These data suggestthat duodenal diverticulum is associated with choledocholithiasis.

  18. Duodenal Perforation Precipitated by Scrub Typhus.

    Science.gov (United States)

    Rajat, Raghunath; Deepu, David; Jonathan, Arul Jeevan; Prabhakar, Abhilash Kundavaram Paul

    2015-01-01

    Scrub typhus is an acute febrile illness usually presenting with fever, myalgia, headache, and a pathognomonic eschar. Severe infection may lead to multiple organ failure and death. Gastrointestinal tract involvement in the form of gastric mucosal erosions and ulcerations owing to vasculitis resulting in gastrointestinal bleeding is common. This process may worsen a pre-existent asymptomatic peptic ulcer, causing duodenal perforation, and present as an acute abdomen requiring surgical exploration. We report the case of a patient with no previous symptoms or risk factors for a duodenal ulcer, who presented with an acute duodenal perforation, probably precipitated by scrub typhus infection.

  19. Duodenal perforation precipitated by scrub typhus

    Directory of Open Access Journals (Sweden)

    Raghunath Rajat

    2015-01-01

    Full Text Available Scrub typhus is an acute febrile illness usually presenting with fever, myalgia, headache, and a pathognomonic eschar. Severe infection may lead to multiple organ failure and death. Gastrointestinal tract involvement in the form of gastric mucosal erosions and ulcerations owing to vasculitis resulting in gastrointestinal bleeding is common. This process may worsen a pre-existent asymptomatic peptic ulcer, causing duodenal perforation, and present as an acute abdomen requiring surgical exploration. We report the case of a patient with no previous symptoms or risk factors for a duodenal ulcer, who presented with an acute duodenal perforation, probably precipitated by scrub typhus infection.

  20. Ampullary duodenal diverticulum and cholangitis

    Directory of Open Access Journals (Sweden)

    Joaquim Mendes Castilho Netto

    Full Text Available CONTEXT: Ampullary duodenal diverticulum complicated by cholangitis is little known in clinical practice, especially when there are no gallstones in the common bile duct or there is no biliary tree ectasia or hyperamylasemia. A case of this association is presented, in which the surgical treatment was a biliary-enteric bypass. CASE REPORT: A 74-year-old diabetic white woman was admitted to the Taubaté University Hospital, complaining of pain in the right upper quadrant, jaundice and fever with chills (Charcot's triad. She had had cholecystectomy 30 years earlier. She underwent clinical treatment with parenteral hydration, insulin, antibiotics and symptomatic drugs. Imaging examinations were provided for diagnosis: ultrasound, computed tomography and endoscopic retrograde cholangiopancreatography. The surgical treatment consisted of choledochojejunostomy utilizing a Roux-en-y loop. The postoperative period progressed without incidents, and a DISIDA scan demonstrated the presence of dynamic biliary excretion. The patient remained asymptomatic when seen at outpatient follow-up.

  1. Endoscopic duodenal biopsy in children

    Directory of Open Access Journals (Sweden)

    El Mouzan Mohammad

    2006-01-01

    Full Text Available Background: Biopsy of the small bowel is frequently obtained by endoscopic forceps instead of the classical suction capsule, yet reports from developing countries are scarce. Aim of the study: to report our experience on the diagnostic value of this procedure in our community. Patients and methods: A retrospective analysis of all endoscopic duodenal biopsies (EDB, performed on all patients below 18 years of age. Data retrieved from the records included age, gender, nationality, indication for biopsy, the endoscopic findings, and the results of histopathology. Results: From 1993 to 2002, 241 endoscopic biopsies were performed on 241 consecutive children. Most of the children (96% were Saudi nationals, the age range between six weeks to 18 years, and male to female ratio was 0.7: 1. All of the biopsy material was adequate for routine histopathology. The commonest indications for biopsy were short stature and chronic diarrhea in 116/241 (48% and 102/241 (43% of the children respectively. Refractory rickets accounted for 11/241 (5% of the indications. The prevalence of villous atrophy was highest in children presenting with chronic diarrhea (40%, compared to short stature (22%. Other less common, but important findings were villous atrophy in three unusual conditions (one refractory rickets, one unexplained anemia, and one polyendocrinopathy, two cases of intestinal Giardia lamblia infestation, three cases of intestinal lymphangiectasis and one case of Mycobacterium avium intracellulare. Unexpected endoscopic findings were documented in 34/241 (14% of the children. Conclusions: Endoscopic duodenal biopsy is adequate not only for the diagnosis of villous atrophy, but also for the detection of other gastroenteropathies. Accordingly, when expertise and equipments are available, EDB should be the procedure of choice not only in industrialized but also in developing countries.

  2. Duodenal diverticulitis. computed tomography findings; Diverticulities duodenal. Hallazgos en la TC

    Energy Technology Data Exchange (ETDEWEB)

    Sanchez, E.; Martin, S.; Garcia, J.; Dominguez, A. [Hospital Ramon y Cajal. Madrid (Spain)

    2001-07-01

    Duodenal diverticular occur very frequently among the general public. However, duodenal diverticulitis is a very uncommon clinical entity, the diagnosis of which requires radiological studies since the clinical signs cam mimic a great number of disease processes with different treatments. We present a case of duodenal diverticulitis in which the diagnosis according to ultrasound and computed tomography (CT) studies was confirmed intraoperatively. We also review the few cases of this entity reported in the literature. The CT findings are highly suggestive of duodenal diverticulitis given their similarity to those associated with diverticulitis at other sites. (Author) 5 refs,.

  3. Malignant nonfunctioning paraganglioma of the retroperitoneum producing renovascular hypertension.

    Science.gov (United States)

    Mundis, R J; Bisel, H F; Sheps, S G; Sheedy, P F; Gaffey, T A; Sterioff, S

    1982-10-01

    Malignant nonchromaffin paraganglioma (chemodectoma) is an unusual tumor arising from chemoreceptor cells derived from the neural crest. Although these tumors generally occur in the head and neck, where the term "carotid body tumor" applies, more than 20 cases of retroperitoneal origin have been described. This case report presents the clinical, radiographic, and pathologic features of a chemodectoma arising in the retroperitoneal area that produced severe hypertension by unilateral real vascular compression. The patient underwent exploratory laparotomy, and a left nephrectomy was done. Follow-up examination 1 year after surgery disclosed no clinical evidence of disease, and he had normal blood pressures without medication.

  4. Malignant pheochromocytomas and paragangliomas - the importance of a multidisciplinary approach

    DEFF Research Database (Denmark)

    Andersen, Kim Francis; Altaf, Rahim; Krarup-Hansen, Anders;

    2011-01-01

    Approximately 10% of the pheochromocytomas and 20% of the paragangliomas are malignant with poor survival. As the biological behaviour of these tumours cannot be predicted with certainty from pathology the diagnosis of malignancy is difficult. Genetic testing is gaining impact as mutations...... in the tumour suppressor gene Von Hippel-Lindau and the mitochondrial succinate dehydrogenase enzyme complex subunit B (SDHB) are associated with malignancy. Excess release of catecholamines is characteristic for pheochromocytomas. High levels of chromogranin A, that is co-stored and co...

  5. The Genetic Basis of Pheochromocytoma and Paraganglioma: Implications for Management

    Science.gov (United States)

    Shuch, Brian; Ricketts, Christopher J.; Pacak, Karol; Linehan, W. Marston

    2015-01-01

    Chromaffin cells are catecholamine-producing cells derived from neural crest tissue. Chromaffin tumors (ChT) are rare tumors arising from these cells and are divided into pheochromocytoma (PCC) arising from adrenal tissue and paraganglioma (PGL) arising from extra-adrenal ganglia. Previously, ∼10% were believed to be hereditary, but advances in genome sequencing has shown roughly 35% of apparently sporadic tumors have a hereditary component. In this review we describe both classic and newly discovered hereditary ChT syndromes and provide recommendations for genetic testing. In many cases the genes associated with these conditions are linked to common kidney cancer pathways familiar to urologic oncologists. PMID:24642075

  6. A Case of Renal Cell Carcinoma Associated with Paraganglioma

    OpenAIRE

    住吉, 崇幸; 清水, 洋祐; 井上, 貴博; 大久保, 和俊; 渡部, 淳; 神波, 大己; 吉村, 耕治; 兼松, 明弘; 中村, 英二郎; 西山, 博之; 賀本, 敏行; 住吉, 真治; 小川, 修

    2011-01-01

    A 64-year-old man was referred to our hospital for the treatment of left renal cell carcinoma associated with a tumor located on the back of the inferior vena cava. At first the tumor located on the back of the inferior vena cava was suspected to be lymphnode metastasis of renal cell carcinoma. A more detailed examination at our hospital revealed elevation of vanillylmandelic acid in urine and 131Imetaiodobenzylguanidine uptake in the tumor. We diagnosed the tumor as paraganglioma and operate...

  7. Hypertension secondary to paraganglioma: case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Rubio-Marín, Andrea Constanza

    2016-04-01

    Full Text Available We report the case of a 25 year-old man with a history of transient stroke during adolescence, and longstanding uncontrolled hypertension who presented with palpitations, diaphoresis and headache. He was diagnosed with a paraaortic paraganglioma associated with catecholamine hypersecretion, confirmed by histopathology. There was complete resolution of the symptoms after removal of the tumor. Paraganglioma, a rare extra-adrenal tumor, derived from chromaffin cells, should be considered in the diagnosis of secondary hypertension. We performed a literature review of paraganglioma cases with catecholamine hypersecretion, and associated hypertension.

  8. Extra adrenal retroperitoneal paraganglioma associated with duplication of inferior vena cava

    Directory of Open Access Journals (Sweden)

    parangama chatterjee

    2008-09-01

    Full Text Available

    Retroperitoneal paragangliomas arise from specialized neural crest cells symmetrically distributed along the aorta in association with the sympathetic chain. If this tissue aggregates in the adrenal medulla pheochromocytoma may arise. When it remains in the paraaortic sites it could develop into extra-adrenal, retroperitoneal paraganglioma. We report a case of extra-adrenal paraganglioma in the renal hilum intimately related to the left side of a double inferior vena cava. To the best of our knowledge such an association has never been described before. The clinical significance of this venous anomaly is reviewed.

  9. [Persistent duodenal septum in an adult].

    Science.gov (United States)

    Helwing, E; Echtermeyer, V; Otten, G

    1977-02-01

    A case of duodenal obstruction by a congenital duodenal web in a 34-year-old woman is presented. A mucosal diaphragm obstructed the duodenum. It showed an excentric opening of 0.8 cm diameter, but the dilated diaphragm caused a total stop during the last months. Despite a typical history, exact X-ray, and endoscopic examination, the correct preoperative diagnosis was not found, because nobody thought it possible, that a mucosal diapharm of the duodenum could persist for 34 years.

  10. Multiple early gastric cancer with duodenal invasion

    Directory of Open Access Journals (Sweden)

    Okino Tetsuya

    2007-10-01

    Full Text Available Abstract Background Early gastric cancers with duodenal invasion are rare, and no previous case of multiple early gastric cancer, one invading the duodenal bulb, has been reported. Case presentation A 79-year-old woman was investigated for upper abdominal discomfort. Endoscopic examination revealed an irregular nodulated lesion in the antrum area, and a reddish aggregated-type semi-circumferential nodulated lesion extending from the prepyloric area to the duodenal bulb through the normal mucosa with the antrum lesion. Biopsy revealed a tubular adenoma for the antrum lesion and a well-differentiated tubular adenocarcinoma for the prepyloric lesion. Distal gastrectomy with sufficient duodenal resection was performed. Microscopically, the antrum lesion appeared as a papillary adenocarcinoma, and the prepyloric lesion as a mainly papillary adenocarcinoma which partially invaded the submucosa without any sequential elongation for endoscopic findings. The lesion extended into the duodenal bulb, and was 12 mm in length from the oral end of Brunner's gland's area and limited within the duodenal mucosa. Conclusion Here, we present an unusual case of multiple early gastric cancer, one of which invaded the duodenum with relative wide mucosal spreading. This case illustrates that even early stage cancers located in the gastric antrum, particularly in the prepyloric area can invade the duodenum directly.

  11. Rare vertebral metastasis in a case of Hereditary Paraganglioma

    Directory of Open Access Journals (Sweden)

    da Silva Manuel Eduardo

    2012-09-01

    Full Text Available Abstract Paragangliomas are rare tumours with a prevalence of 1/10000 to 1/30000. Tumors arising from the paraganglia are characteristically of low malignant potential. Vertebral metastases are exceedingly rare, and only isolated case reports have described them. The authors present the clinical course of a 47 years-old female patient with a familial paraganglioma [PGL] with vertebral metastastization, who underwent an intralesional tumor excision and corpectomy. Genetic screening demonstrated a new germinal frameshift mutation of the SDHB exon 6 [c.587-591DelC]. After surgery there was normalization of the analytical parameters and imagiologic screening. One year later she presented a new image in the the pedicle of T11 on the contralateral side of the surgical incision. She performed 2 treatments with MIBG and 1 cicle of radiotherapy that made the new lesion regress. Currently the patient does not present any clinical or analytical evidence of new metastasis. This case outlines the clinical course of a patient with a PGL syndrome for whom a rare vertebral metastasis was diagnosed. It highlights the importance of identifying patients with germline SDHB mutations, as these patients are at a high risk of developing malignant disease.

  12. Current and future anatomical and functional imaging approaches to pheochromocytoma and paraganglioma.

    NARCIS (Netherlands)

    Timmers, H.J.L.M.; Taieb, D.; Pacak, K.

    2012-01-01

    After establishing a biochemical diagnosis, pheochromocytomas and extra-adrenal paragangliomas (PPGLs) can be localized using different anatomical and functional imaging modalities. These include computed tomography, magnetic resonance imaging, single-photon emission computed tomography (SPECT) usin

  13. Ultrastructural changes in non-specific duodenitis

    Institute of Scientific and Technical Information of China (English)

    Cheng-Xin Wang; Li-Jiang Liu; Jing Guan; Xiao-Ling Zhao

    2005-01-01

    AIM: To investigate the ultrastructural and morphological changes of non-specific duodenitis (NSD) in an attempt to grade them according to the extent of the lesions.METHODS: Biopsies were taken from the mucosa of duodenal bulb of 44 patients selected from the patients undergoing upper gastrointestinal endoscopy for epigastric discomforts. From each patient, two pinch biopsies on the same area were obtained from duodenal bulb. One was for scanning electron microscopy and the other was stained with hematoxylin-eosin, Warthin-Starry silver and both were then examined under light microscope. A total of 12 specimens (three from each degree of the normal and Ⅰ-Ⅲ of NSD diagnosed and graded by histology) selected from the 44patients were dehydrated, critical point dried, coated with gold palladium and examined under a JEOL JSM-30 scanning electron microscope (SEM) at 20 kV.RESULTS: According to the ultrastructural morphologic changes, non-specific duodenitis was divided into normal (as control group), mild, moderate and severe degrees according to results of SEM. The normal villi of duodenal bulb were less than 0.2 mm. There were inflammation cells,occasionally red blood cells and macrophages on the mucosal epithelial surface. Erosion and desquamation of epithelium could be seen. Three cases (25%, 3/12) had gastric metaplasia and Helicobacter pylori(H pylori) infection could be found in 5 cases (41.67%, 5/12) in duodenal bulb mucosa. The most distinctive feature was the ulcer-like defect on the surface of epithelial cells.CONCLUSION: Non-specific duodenitis is a separate entity disease caused by different factors. SEM is of value as an aid in the diagnosis of mucosal diseases of duodenum.

  14. Integrative genetic, epigenetic and pathological analysis of paraganglioma reveals complex dysregulation of NOTCH signaling

    OpenAIRE

    2013-01-01

    Head and neck paragangliomas, rare neoplasms of the paraganglia composed of nests of neurosecretory and glial cells embedded in vascular stroma, provide a remarkable example of organoid tumor architecture. To identify genes and pathways commonly deregulated in head and neck paraganglioma, we integrated high-density genome-wide copy number variation (CNV) analysis with microRNA and immunomorphological studies. Gene-centric CNV analysis of 24 cases identified a list of 104 genes most significan...

  15. Prevalence, clinical, endoscopic and pathological features of duodenitis in children

    Science.gov (United States)

    Alper, Arik; Hardee, Steven; Rojas-velasquez, Danilo; Escalera, Sandra; Morotti, Raffaella A; Pashankar, Dinesh S.

    2015-01-01

    Objectives While gastritis and esophagitis are well studied in children, there is very limited literature on duodenitis in children. We aimed to assess the prevalence, etiology, clinical, endoscopic, and pathological features in a large cohort of unselected children with duodenitis. Methods We reviewed the pathology reports of all upper endoscopies performed at our institution over 5 years to identify children with duodenitis. Biopsy sections were reviewed to confirm the diagnosis of duodenitis. Demographic, clinical, endoscopic data and presence of associated gastritis and esophagitis were noted in all children with duodenitis. Etiology of duodenitis was correlated with the patient's clinical diagnosis. Results Out of 2772 children who had endoscopy, 352 had duodenitis with the prevalence rate of 12.7%. Gastritis was seen in 64% of children with duodenitis compared with 46% of children without duodenitis (P < 0.001). Common indications for endoscopy in children with duodenitis were abdominal pain, positive celiac serology and diarrhea. The most common etiology was celiac disease (32%), followed by Crohn's disease (13%), ulcerative colitis (3%), and Helicobacter pylori infection (6%). In 63% of cases, endoscopic appearance of duodenum was normal. Cryptitis, villous changes and cellular infiltration were noted on histology. Conclusions Prevalence of duodenitis is 12.7% in children undergoing endoscopy. Celiac disease and inflammatory bowel disease are common causes of duodenitis. Associated gastritis is common in children with duodenitis and correlation of endoscopic appearance with histology is poor. PMID:26252915

  16. An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis

    NARCIS (Netherlands)

    F.H. van Nederveen (Francien); J. Gaal (José); J. Favier (Judith); E. Korpershoek (Esther); R.A. Oldenburg (Rogier); E.M. de Bruyn (Elly); H.F. Sleddens (Hein); P. Derkx (Pieter); J. Rivière (Julie); H. Dannenberg (Hilde); B.J. Petri; P. Komminoth (Paul); K. Pacak (Karel); W.C.J. Hop (Wim); P.J. Pollard (Patrick); M. Mannelli (Massimo); J.P. Bayley; A. Perren (Aurel); S. Niemann (Stephan); A.A. Verhofstad (Albert); A.P. de Bruïne (Adriaan); E.R. Maher (Eamonn); F. Tissier (Frédérique); T. Méatchi (Tchao); C. Badoual (Cécile); J. Bertherat (Jerome); L. Amar (Laurence); D. Alataki (Despoina); E.A. van Marck (Eric); F. Ferrau (Francesco); J.F. François (Joseph); W.W. de Herder (Wouter); M.P.F.V. Peeters; A. van Linge (Anne); J.W. Lenders (Jacques); A.P. Gimenez-Roqueplo; R.R. de Krijger (Ronald); W.N.M. Dinjens (Winand)

    2009-01-01

    textabstractBackground: Phaeochromocytomas and paragangliomas are neuro-endocrine tumours that occur sporadically and in several hereditary tumour syndromes, including the phaeochromocytoma-paraganglioma syndrome. This syndrome is caused by germline mutations in succinate dehydrogenase B (SDHB), C

  17. Factors associated with gastro-duodenal disease in patients ...

    African Journals Online (AJOL)

    Factors associated with gastro-duodenal disease in patients undergoing ... recruit patients referred with upper gastro-intestinal symptoms for endoscopy. ... 64 had duodenal ulcer, 66 gastric ulcer, 27gastric cancer and 64 non-ulcer dyspepsia.

  18. Westernblottinginthediagnosisof duodenal-biliary and pancreaticobiliary relfuxesinbiliarydiseases

    Institute of Scientific and Technical Information of China (English)

    Guo-Zhe Xian; Shuo-Dong Wu; Chun-Chih Chen; Yang Su

    2009-01-01

    BACKGROUND: Currently adopted diagnostic methods for duodenal-biliary and pancreaticobiliary relfuxes carry many lfaws, so the incidence of the two relfuxes demands further larger sample size studies. This study aimed to evaluate Western blotting for the diagnosis of relfuxes in biliary diseases. METHODS: An oral radionuclide 99mTc-DTPA test (radio-nuclide, RN) was conducted for the observation of duodenal-biliary relfux prior to measuring bile radioactivity and Western blotting for detecting bile enterokinase (EK). Pancreaticobiliary relfux was assessed by biochemical and Western blotting tests for biliary amylase activity and trypsin-1, respectively. In accordance with bile sample origin, our samples were classiifed into ductal bile and gall bile groups;based on each individual biliary disease, we further classiifed the ductal bile group into ifve sub-groups, and the gall bile group into four sub-groups. Western blotting was conducted to assess the two relfuxes in biliary diseases. RESULTS: Consistencies were noted between EK and RN tests when diagnosing duodenal-biliary relfux (P0.05); in the common bile duct cyst group, the EK positive rate was signiifcantly lower than the trypsin-1 positive rate (P CONCLUSIONS: Western blotting can accurately relfect duodenal-biliary and pancreaticobiliary relfuxes. EK has greater sensitivity than RN for duodenal-biliary relfux. The majority of biliary amylase and lipase comes from the pancreas in all biliary diseases;pancreaticobiliary relfux is the predominant source in the common bile duct cyst group and duodenal-biliary relfux is responsible for the ductal pigment stone group.

  19. EANM 2012 guidelines for radionuclide imaging of phaeochromocytoma and paraganglioma

    Science.gov (United States)

    Timmers, Henri J.; Hindié, Elif; Guillet, Benjamin A.; Neumann, Hartmut P.; Walz, Martin K.; Opocher, Giuseppe; de Herder, Wouter W.; Boedeker, Carsten C.; de Krijger, Ronald R.; Chiti, Arturo; Al-Nahhas, Adil; Pacak, Karel

    2016-01-01

    Purpose Radionuclide imaging of phaeochromocytomas (PCCs) and paragangliomas (PGLs) involves various functional imaging techniques and approaches for accurate diagnosis, staging and tumour characterization. The purpose of the present guidelines is to assist nuclear medicine practitioners in performing, interpreting and reporting the results of the currently available SPECT and PET imaging approaches. These guidelines are intended to present information specifically adapted to European practice. Methods Guidelines from related fields, issued by the European Association of Nuclear Medicine and the Society of Nuclear Medicine, were taken into consideration and are partially integrated within this text. The same was applied to the relevant literature, and the final result was discussed with leading experts involved in the management of patients with PCC/PGL. The information provided should be viewed in the context of local conditions, laws and regulations. Conclusion Although several radionuclide imaging modalities are considered herein, considerable focus is given to PET imaging which offers high sensitivity targeted molecular imaging approaches. PMID:22926712

  20. Malignant pheochromocytoma and paraganglioma: future considerations for therapy.

    Science.gov (United States)

    Buzzoni, R; Pusceddu, S; Damato, A; Meroni, E; Aktolun, C; Milione, M; Mazzaferro, V; De Braud, F; Spreafico, C; Maccauro, M; Zaffaroni, N; Castellani, M R

    2013-06-01

    Pheochromocytoma and paraganglioma are rare neuroendocrine tumors. Knowledge about such neoplasms ameliorated in the last 10-15 years with the discovery of increasing number of germ line mutations even in apparently sporadic cases. Seemingly, genetic tests are going to be an integral part of diagnostic procedures. Standard therapies (advanced surgery, radiometabolic therapy, chemotherapy and radiotherapy) have revealed suboptimal results in tumor size reduction and survival. Currently, there is no standard therapeutic protocol and thus some patients end up with overtreatment while others are undertreated. An effective molecular target therapy aiming at permanent control of these highly complex neoplasms should be the aim of future efforts. In clinical setting investigatory trials with multiple drug therapies targeting a variety of different parallel pathways should be available. Successful management requires a multidisciplinary teamwork.

  1. Radiotherapy for jugulo-tympanic paragangliomas (Glomus jugulare tumours)

    Energy Technology Data Exchange (ETDEWEB)

    Sharma, P.D.; Johnson, A.P.; Whitton, A.C.

    1984-06-01

    Parasympathetic paraganglia are found in the region of the jugular bulb, in association with the tympanic branch of the glossopharyngeal nerve and the auricular branch of the vagus. The name commonly applied to these structures is 'glomus jugulare'. Tumours arising from these paraganglia (paragangliomas or glomus jugulare tumours) are usually histologically benign but locally destructive. They may involve the middle ear, the temporal bone, or the jugular foramen, and may extend into the neck or cranium. Very occasionally they are malignant and metastasise (Taylor et al., 1965). The clinical features of these tumours and the techniques for their diagnosis are well established, but treatment remains controversial. Radiotherapy has been the preferred treatment at St. Bartholomew's Hospital, London, and sixty cases seen at this hospital between 1942 and 1982 are reviewed here.

  2. Lubiprostone Stimulates Duodenal Bicarbonate Secretion in Rats

    OpenAIRE

    Mizumori, Misa; Akiba, Yasutada; Kaunitz, Jonathan D.

    2009-01-01

    Background Lubiprostone, a bicyclic fatty acid, is used for the treatment of chronic constipation. No published study has addressed the effect of lubiprostone on intestinal ion secretion in vivo. Aim The aim of this study was to test the hypothesis that lubiprostone augments duodenal HCO3 − secretion (DBS). Methods Rat proximal duodenal loops were perfused with pH 7.0 Krebs, control vehicle (medium-chain triglycerides), or lubiprostone (0.1–10 μM). We measured DBS with flow-through pH and CO2...

  3. Is duodenal biopsy appropriate in areas endemic for Helicobacter pylori?

    Science.gov (United States)

    Sahin, Abdurrahman; Cihangiroglu, Gulcin; Bilgic, Yilmaz; Calhan, Turan; Cengiz, Mustafa

    2017-01-01

    The primary reason for obtaining duodenal biopsy sample is to diagnose celiac disease. Helicobacter pylori (H. pylori) and drug injury are common causes of duodenitis. The aim of this retrospective study was to explore effects of H. pylori and drugs on duodenal mucosa. Duodenal biopsy samples of patients who underwent upper gastrointestinal endoscopy (UGIE) between February 2014 and December 2014 were retrospectively examined. Clinical symptoms, referral indications, endoscopic findings, H. pylori status, and drug history were recorded. Duodenal biopsy findings were compared based on presence of H. pylori and drug history. Of 2389 patients who underwent UGIE, 206 had duodenal biopsy. Eight patients (3.9%) were diagnosed with celiac disease. After excluding cases with celiac disease, 76 patients of remaining 198 patients (36.9%) had duodenal histopathological abnormality. H. pylori was found in 95 (47.9%) patients. Drug usage was less common (42%). Of patients who had histopathological duodenitis, 59% were H. pylori-infected. Rate of duodenitis was higher in H. pylori (+) group than in H. pylori (-) group (45% vs 27.1%; odds ratio, 2.4; 95% confidence interval, 1.3-4.4; p=0.005). There was no difference between groups regarding drug use in terms of histopathological duodenitis. H. pylori is the major contributor to duodenitis in high prevalence regions. Serological testing may be more appropriate before performing duodenal biopsy in patients with suspected celiac disease.

  4. Osseous Metastase of Occult Paraganglioma: A Diagnostic Medical Error

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    Ghasemi TA

    2013-10-01

    Full Text Available Introduction: Diagnostic errors have a natural complexity. Medical diagnoses make up a large proportion of all medical errors and cause much suffering and harm. Compared to other types of error, diagnostic errors receive little attention-a major factor in continuity of unacceptable rates of diagnostic error. Case: A 55-year-old woman presented to the emergency department (ED complaining of bone pain which has been started a month ago and increased gradually in the upper right thigh. Following the emergency evaluation she was sent home with pain medication. On the second visit, a femur neck fracture was seen in the x-ray. She underwent hemiarthroplasty and was discharged. Over several weeks she was reevaluated by many Physicians, because of her worsening pain .In the third visit after the surgery, her x-ray showed bone destruction and following bone biopsy, malignant paraganglioma was diagnosed. Discussion and solution: In all cases in which patient comes to us with skeletal pain, getting a comprehensive history and a full physical examination are prior to lab tests and x-rays. Bone metastasis which can develop severe pain and pathological fractures, is common in patients with malignant paraganglioma. Effective steps for diagnostic error prevention are: Considering the diagnostic error in the normal range of quality assurance surveillance and review, identifying the elements leading to diagnostic errors and getting feedback on the diagnoses Physicians make, in order to improve their skills. Conclusion: It is an every health system priority to identify, analyze, and prevent diagnostic errors in order to improve patient safety

  5. Clinical value of somatostatin receptor imaging in patients with suspected head and neck paragangliomas

    Energy Technology Data Exchange (ETDEWEB)

    Schmidt, Matthias; Dietlein, Markus; Weber, Kerstin; Moka, Detlef; Schicha, Harald [Klinik und Poliklinik fuer Nuklearmedizin, Universitaet zu Koeln, Joseph-Stelzmann-Strasse 9, 50924 Koeln (Germany); Fischer, Eva; Michel, Olaf; Stennert, Eberhard [Klinik und Poliklinik fuer Hals-, Nasen- und Ohrenheilkunde, Universitaet zu Koeln, Koeln (Germany)

    2002-12-01

    Paragangliomas or glomus tumours of the head and neck region are rare somatostatin receptor-expressing neuroendocrine tumours. Precise preoperative diagnosis is of special importance in order to adequately weigh the potential benefit of the operation against the inherent risks of the procedure. In this study, the clinical value of somatostatin receptor imaging was assessed in 19 patients who underwent somatostatin receptor scintigraphy because of known or suspected paraganglioma of the head and neck region. The results were compared with the results of computed tomography and/or magnetic resonance imaging, histology and clinical follow-up. [{sup 111}In-DTPA-D-Phe{sup 1}]-octreotide scintigraphy was performed 4-6 and 24 h after i.v. injection of 140-220 MBq {sup 111}In-octreotide. Whole-body and planar images as well as single-photon emission tomography images were acquired and lesions were graded according to qualitative tracer uptake. Somatostatin receptor imaging was positive in nine patients, identifying paragangliomas for the first time in three patients and recurrent disease in six patients. In one patient, a second, previously unknown paraganglioma site was identified. Negative results were obtained in ten patients. These patients included one suffering from chronic hyperplastic otitis externa, one with granuloma tissue and an organised haematoma, one with an acoustic neuroma, one with an asymmetric internal carotid artery, two with ectasia of the bulbus venae jugularis and one with a jugular vein thrombosis. In two patients with a strong family history of paraganglioma, individual involvement could be excluded. In only one patient did somatostatin receptor imaging and magnetic resonance imaging yield false negative results in respect of recurrent paraganglioma tissue. It is concluded that somatostatin receptor scintigraphy provides important information in patients with suspected paragangliomas of the head and neck region and has a strong impact on further

  6. Cytomegalovirus duodenitis associated with life-threatening duodenal hemorrhage in an immunocompetent patient: A case report

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    Lucy Shen

    2017-01-01

    Conclusion: Presented is a rare case of life-threatening GI hemorrhage caused by CMV duodenitis in an immunocompetent patient. The patient failed endoscopic and interventional-radiology treatment options, and ultimately stabilized after surgical intervention.

  7. Duodenal obstruction following acute pancreatitis caused by a large duodenal diverticular bezoar

    Institute of Scientific and Technical Information of China (English)

    Ji Hun Kim; Jae Hyuck Chang; Sung Min Nam; Mi Jeong Lee; Il Ho Maeng; Jin Young Park; Yun Sun Im

    2012-01-01

    Bezoars are concretions of indigestible materials in the gastrointestinal tract.It generally develops in patients with previous gastric surgery or patients with delayed gastric emptying.Cases of periampullary duodenal divericular bezoar are rare.Clinical manifestations by a bezoar vary from no symptom to acute abdominal syndrome depending on the location of the bezoar.Biliary obstruction or acute pancreatitis caused by a bezoar has been rarely reported.Small bowel obstruction by a bezoar is also rare,but it is a complication that requires surgery.This is a case of acute pancreatitis and subsequent duodenal obstruction caused by a large duodenal bezoar migrating from a periampullary diverticulum to the duodenal lumen,which mimicked pancreatic abscess or microperforation on abdominal computerized tomography.The patient underwent surgical removal of the bezoar and recovered completely.

  8. The complicated duodenal diverticulum: retrospective analysis of 11 cases.

    Science.gov (United States)

    de Perrot, Thomas; Poletti, Pierre-Alexandre; Becker, Christoph D; Platon, Alexandra

    2012-01-01

    A series of rare complicated duodenal diverticula were reported with emphasis on causes for misdiagnosis. Patients with a discharge diagnosis of complicated duodenal diverticulum were retrospectively obtained. Computed tomographic (CT) reports and findings were reviewed. Complications consisted of diverticulitis (n=2), perforation (n=7), or obstructive cholangitis (n=2). CT imaging demonstrated a duodenal diverticular structure with findings due to the kind of complications. At the time of CT interpretation, a complicated duodenal diverticulum was suspected in 5 out of 11 patients. Awareness of the duodenal diverticulum and complications may improve the diagnostic value of CT in this setting. Copyright © 2012 Elsevier Inc. All rights reserved.

  9. Duodenal organ injury severity (OIS) and outcome.

    Science.gov (United States)

    Kline, G; Lucas, C E; Ledgerwood, A M; Saxe, J M

    1994-07-01

    The effect of organ injury severity on outcome was assessed in 101 patients treated for duodenal trauma. Most patients were men (89%) and victims of penetrating wounds (93%). Grade I is minor hematoma or incomplete perforation; Grade II is major hematoma or small complete perforation; Grade III is large perforation excluding ampulla; Grade IV is large perforation at ampulla; Grade V is duodenopancreatic crunch. The injuries were as follows: Grade I (5 patients), Grade II (31), Grade III (40), Grade IV (12), and Grade V (13). Fourteen patients exsanguinated from associated vessel injury; each had Grade IV or Grade V injury. All 36 patients with Grade I and Grade II injury had primary repair; the single death was due to liver necrosis. Most (31 patients) Grade III injuries and three Grade IV injuries were treated by primary repair alone; the three deaths were unrelated to the duodenal injury. Other major injuries were treated by duodenal exclusion (4 patients), duodenal diverticulization (6), or resection (4); the single death was unrelated to the duodenum. Primary closure is favored for minor injuries and most Grade III injuries. Severe injuries may require exclusion, diverticulization, or resection.

  10. Duodenal Intracellular Bicarbonate and the 'CF Paradox'

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    Kaunitz JD

    2001-07-01

    Full Text Available HCO(3(- secretion, which is believed to neutralize acid within the mucus gel, is the most studied duodenal defense mechanism. In general, HCO(3(- secretion rate and mucosal injury susceptibility correlate closely. Recent studies suggest that luminal acid can lower intracellular pH (pH(i of duodenal epithelial cells and that HCO(3(- secretion is unchanged during acid stress. Furthermore, peptic ulcers are rare in cystic fibrosis (CF, although, with impaired HCO(3(- secretion, increased ulcer prevalence is predicted, giving rise to the 'CF Paradox'. We thus tested the hypothesis that duodenal epithelial cell protection occurs as the result of pH(i regulation rather than by neutralization of acid by HCO(3(- in the pre-epithelial mucus. Cellular acidification during luminal acid perfusion, and unchanged HCO(3(- secretion during acid stress are inconsistent with pre-epithelial acid neutralization by secreted HCO(3(-. Furthermore, inhibition of HCO(3(- secretion by 5-nitro-2-(3-phenylpropylamino benzoic acid (NPPB despite preservation of pH(i and protection from acid-induced injury further question the pre-epithelial acid neutralization hypothesis. This decoupling of HCO(3(- secretion and injury susceptibility by NPPB (and possibly by CF further suggest that cellular buffering, rather than HCO(3(- exit into the mucus, is of primary importance for duodenal mucosal protection, and may account for the lack of peptic ulceration in CF patients.

  11. Duodenal Amyloidosis Masquerading as Iron Deficiency Anemia

    Science.gov (United States)

    Hurairah, Abu

    2016-01-01

    The present study is a unique illustration of duodenal amyloidosis initially manifesting with iron deficiency anemia. It underscores the importance of clinical suspicion of amyloidosis while performing upper gastrointestinal endoscopy with a biopsy to establish the definite diagnosis in patients with unexplained iron deficiency anemia. PMID:27625911

  12. Endoscopic biopsy: Duodenal ulcer penetrating into liver

    Institute of Scientific and Technical Information of China (English)

    Baybora Kircali; Tülay Saricam; Aysegul Ozakyol; Eser Vardareli

    2005-01-01

    @@ TO THE EDITOR We have read with interest the recent report by E Kayacetin and S Kayacetin of Gastric ulcer penetrating to liver diagnosed by endoscopic biopsy[1] since we diagnosed the duodenal ulcer which penetrated into liver similarly. This is a rather unusual case because of the fifth case in the literature and responding to medical therapy.

  13. Adult duodenal intussusception associated with congenital malrotation

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Enteroenteric intussusception is a condition in which full-thickness bowel wall becomes telescoped into the lumen of distal bowel. In adults, there is usually an abnormality acting as a lead point, usually a Meckels' diverticulum, a hamartoma or a tumour. Duodenoduodenal intussusception is exceptionally rare because the retroperitoneal situation fixes the duodenal wall.The aim of this report is to describe the first published case of this condition. A patient with duodeno-duodenal intussusception secondary to an ampullary lesion is reported. A 66 year-old lady presented with intermittent abdominal pain, weight loss and anaemia. Ultrasound scanning showed dilated bile and pancreatic ducts.CT scanning revealed intussusception involving the full-thickness duodenal wall. The lead point was an ampullary villous adenoma. Congenital partial (type Ⅱ)malrotation was found at operation and this abnormality permitted excessive mobility of the duodenal wall such that intussusception was possible. This condition can be diagnosed using enhanced CT. Intussusception can be complicated by bowel obstruction, ischaemia or bleeding,and therefore the underlying cause should be treated as soon as possible.

  14. Mutation analysis of SDHB and SDHC : novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma

    NARCIS (Netherlands)

    Bayley, JP; van Minderhout, [No Value; Weiss, MM; Jansen, JC; Menko, FH; Pasini, B; Ferrando, B; Wong, N; Alpert, LC; Blair, E; Devilee, P; Taschner, PEM; Williams, O.

    2006-01-01

    Background: Germline mutations of the SDHD, SDHB and SDHC genes, encoding three of the four subunits of succinate dehydrogenase, are a major cause of hereditary paraganglioma and pheochromocytoma, and demonstrate that these genes are classic tumor suppressors. Succinate dehydrogenase is a heterotetr

  15. Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma

    NARCIS (Netherlands)

    Bayley, J.P.; van Minderhout, I.; Weiss, M.M.; Jansen, J.C.; Oomen, P.H.N.; Menko, F.H.; Pasini, B.; Ferrando, B.; Wong, N.; Alpert, L.C.; Williams, R; Blair, E.; Devilee, P.; Taschner, P.E.M.

    2006-01-01

    Background: Germline mutations of the SDHD, SDHB and SDHC genes, encoding three of the four subunits of succinate dehydrogenase, are a major cause of hereditary paraganglioma and pheochromocytoma, and demonstrate that these genes are classic tumor suppressors. Succinate dehydrogenase is a heterotetr

  16. Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma

    Directory of Open Access Journals (Sweden)

    Wong Nora

    2006-01-01

    Full Text Available Abstract Background Germline mutations of the SDHD, SDHB and SDHC genes, encoding three of the four subunits of succinate dehydrogenase, are a major cause of hereditary paraganglioma and pheochromocytoma, and demonstrate that these genes are classic tumor suppressors. Succinate dehydrogenase is a heterotetrameric protein complex and a component of both the Krebs cycle and the mitochondrial respiratory chain (succinate:ubiquinone oxidoreductase or complex II. Methods Using conformation sensitive gel electrophoresis (CSGE and direct DNA sequencing to analyse genomic DNA from peripheral blood lymphocytes, here we describe the mutation analysis of the SDHB and SDHC genes in 37 patients with sporadic (i.e. no known family history head and neck paraganglioma and five pheochromocytoma and/or paraganglioma families. Results Two sporadic patients were found to have a SDHB splice site mutation in intron 4, c.423+1G>A, which produces a mis-spliced transcript with a 54 nucleotide deletion, resulting in an 18 amino acid in-frame deletion. A third patient was found to carry the c.214C>T (p.Arg72Cys missense mutation in exon 4 of SDHC, which is situated in a highly conserved protein motif that constitutes the quinone-binding site of the succinate: ubiquinone oxidoreductase (SQR complex in E. coli. Together with our previous results, we found 27 germline mutations of SDH genes in 95 cases (28% of sporadic head and neck paraganglioma. In addition all index patients of five families showing hereditary pheochromocytoma-paraganglioma were found to carry germline mutations of SDHB: four of which were novel, c.343C>T (p.Arg115X, c.141G>A (p.Trp47X, c.281G>A (p.Arg94Lys, and c.653G>C (p.Trp218Ser, and one reported previously, c.136C>T, p.Arg46X. Conclusion In conclusion, these data indicate that germline mutations of SDHB and SDHC play a minor role in sporadic head and neck paraganglioma and further underline the importance of germline SDHB mutations in cases of

  17. Complete Resection of Ampullary Paragangliomas Confined to the Submucosa on Endoscopic Ultrasound May Be Best Achieved by Radical Surgical Resection

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    Kevin A. Ghassemi

    2009-06-01

    Full Text Available Paragangliomas of the gastrointestinal tract generally are benign tumors usually found in the second portion of the duodenum. We present a case of paraganglioma of the ampulla of Vater confined to the submucosa on endoscopic ultrasound examination. This was initially treated by endoscopic resection, followed by pancreaticoduodenectomy after local resection margins were positive. Histopathology showed a well-differentiated ampullary paraganglioma confined to the submucosa, but with involvement of one regional lymph node. Only 25 prior cases of paraganglioma at the ampulla of Vater have been reported, and nine of these have demonstrated local or distant metastases. Because of their malignant potential, ampullary paragangliomas should be treated with radical resection if the goal is to achieve complete resection, even if preoperative imaging shows local confinement.

  18. Neonatal Duodenal Obstruction: A 15-Year Experience

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    Kamal Nain Rattan

    2016-04-01

    Full Text Available Background: Congenital duodenal obstruction is one of the commonest causes of neonatal intestinal obstruction. We are presenting our 15-year experience by analyzing clinical spectrum and outcome in neonates with duodenal obstruction admitted at our center. Material and Methods: The hospital records of all neonates admitted with duodenal obstruction from June 2000 to June 2015 were reviewed. The patient records were analyzed for antenatal diagnosis, age, sex, clinical presentation, diagnosis, associated anomalies, surgical procedures performed; postoperative morbidity and mortality. We excluded from our study malrotation of gut associated with congenital diaphragmatic hernia and abdominal wall defects. Results: A total of 81 patients were admitted, out of which 56 were males and 25 were females. Polyhydramnios was detected in 24 (30% pregnancies. Average birth weight was 2.1±1.0Kg and average gestational age was 38 (SD±1 weeks with 17 (21% preterm neonates. Presenting features were vomiting in 81(100% which was bilious in 81% and non bilious in 19%, epigastric fullness in 56 (69% and dehydration in 18 (22% and failure to thrive in 16 (19%. Most common cause of obstruction was duodenal atresia in 38 (46.9%, followed by malrotation of gut in 33 (40.7%, and annular pancreas in 4 cases. Depending upon site of location, infra-ampullary obstruction was the most common in 64 (79%, supra-ampullary in 9 (7.4% and ampullary 8 neonates. Both duodenal atresia and malrotation of gut was present in 4 cases. X-ray abdomen was most commonly used investigation to confirm the diagnosis. All cases were managed surgically by open laparotomy. Eleven (13.5% patients died due to sepsis and associated congenital anomalies. Conclusion: Congenital duodenal obstruction most commonly presents in early neonatal period with features of upper GIT obstruction like vomiting and epigastrium fullness as in our series. Early antenatal diagnosis and surgical interventions hold the

  19. Treatment of Head and Neck Paragangliomas With External Beam Radiation Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Dupin, Charles, E-mail: c.dupin@bordeaux.unicancer.fr [Department of Radiotherapy, Comprehensive Cancer Center, Institut Bergonié, Bordeaux (France); Lang, Philippe [Department of Radiotherapy, Pitié Salpétrière, Paris (France); Dessard-Diana, Bernadette [Department of Radiotherapy, Hopital Européen Georges Pompidou, Paris (France); Simon, Jean-Marc; Cuenca, Xavier; Mazeron, Jean-Jacques; Feuvret, Loïc [Department of Radiotherapy, Pitié Salpétrière, Paris (France)

    2014-06-01

    Purpose: To retrospectively assess the outcomes of radiation therapy in patients with head and neck paragangliomas. Methods and Materials: From 1990 to 2009, 66 patients with 81 head and neck paragangliomas were treated by conventional external beam radiation therapy in 25 fractions at a median dose of 45 Gy (range, 41.4-68 Gy). One case was malignant. The median gross target volume and planning target volume were 30 cm{sup 3} (range, 0.9-243 cm{sup 3}) and 116 cm{sup 3} (range, 24-731 cm{sup 3}), respectively. Median age was 57.4 years (range, 15-84 years). Eleven patients had multicentric lesions, and 8 had family histories of paraganglioma. Paragangliomas were located in the temporal bone, the carotid body, and the glomus vagal in 51, 18, and 10 patients, respectively. Forty-six patients had exclusive radiation therapy, and 20 had salvage radiation therapy. The median follow-up was 4.1 years (range, 0.1-21.2 years). Results: One patient had a recurrence of temporal bone paraganglioma 8 years after treatment. The actuarial local control rates were 100% at 5 years and 98.7% at 10 years. Patients with multifocal tumors and family histories were significantly younger (42 years vs 58 years [P=.002] and 37 years vs 58 years [P=.0003], respectively). The association between family predisposition and multifocality was significant (P<.001). Two patients had cause-specific death within the 6 months after irradiation. During radiation therapy, 9 patients required hospitalization for weight loss, nausea, mucositis, or ophthalmic zoster. Two late vascular complications occurred (middle cerebral artery and carotid stenosis), and 2 late radiation-related meningiomas appeared 15 and 18 years after treatment. Conclusion: Conventional external beam radiation therapy is an effective and safe treatment option that achieves excellent local control; it should be considered as a first-line treatment of choice for head and neck paragangliomas.

  20. Mosaicism in HIF2A-related polycythemia-paraganglioma syndrome.

    Science.gov (United States)

    Buffet, Alexandre; Smati, Sarra; Mansuy, Ludovic; Ménara, Mélanie; Lebras, Maëlle; Heymann, Marie-Françoise; Simian, Christophe; Favier, Judith; Murat, Arnaud; Cariou, Bertrand; Gimenez-Roqueplo, Anne-Paule

    2014-02-01

    HIF2A germline mutations were known to cause congenital polycythemia. Recently, HIF2A somatic mutations were found in several patients with polycythemia and paraganglioma, pheochromocytoma, or somatostatinoma, suggesting the occurrence of a de novo postzygotic HIF2A mutation that has not been demonstrated clearly. Patient 1 is a woman suffering from polycythemia diagnosed at the age of 16 years. She was operated on for a pheochromocytoma at 45 years and for two abdominal paragangliomas at 59 years. She was also diagnosed with somatostatinoma. Patient 2 is a young boy who suffered from polycythemia since infancy. He underwent surgery for a nonfunctional adrenal paraganglioma at the age of 9 years. We sequenced by Sanger and next-generation sequencing the HIF2A gene in DNA extracted from tumors, leukocytes, and buccal cells. In patient 1, we identified a somatic HIF2A mutation (c.1586T>C; p.Leu529Pro) in DNA extracted from both paragangliomas. The mutation was detected as a somatic mosaic in DNA extracted from somatostatinoma and was absent from germline DNA. In patient 2, we found an HIF2A heterozygous mutation (c.1625T>C; p.Leu542Pro) in the paraganglioma, but the mutation was also present as a mosaic in leukocyte DNA and in DNA extracted from buccal cells (3.3 and 8.96% of sequencing reads, respectively). Both mutations disrupt the hydroxylation domain of the HIF2α protein. Our study shows that HIF2A-related tumors are caused by postzygotic mutations occurring in early developmental stages. Potential germline mosaicism should be considered during the familial genetic counseling when an individual has been diagnosed with HIF2A-related polycythemia-paraganglioma syndrome.

  1. A clinicopathological study of pheochromocytoma and paraganglioma with review of literature

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    D Manimaran

    2016-01-01

    Full Text Available Background and Aim: Pheochromocytoma and paraganglioma (PPGL are rare chromaffin cell tumors with variable clinical presentation which are derived from neural crest cells. Pheochromocytomas arising from the adrenal glands are sympathetic, and the paragangliomas arising from extra-adrenal sites are either sympathetic or parasympathetic in origin. These tumors usually come to clinical attention due to the catecholamine secretion or maybe detected as incidentalomas while imaging for unrelated symptoms. Our objective is to study the histopathological and histochemical features of PPGL with clinical correlation. Materials and Methods: PPGLs arising from both sympathetic and parasympathetic nervous systems were retrospectively selected from the records for 5 years, and clinical details were obtained. Routine hematoxylin and eosin (H and E stain and argentaffin and argyrophilic reactions were employed. Immunohistochemistry for chromogranin was done for cases with doubtful histomorphology in H and E sections. Results: Our study material consisted of 10 cases out of which five cases were adrenal pheochromocytomas; 4, carotid body tumors; and 1, paraganglioma of the organ of Zuckerkandl. Three pheochromocytomas out of five and the paraganglioma of organ of Zuckerkandl were functional and clinically presented with palpitation and hypertension. All the carotid body tumors and two of the pheochromocytomas were nonfunctional. The 24 h urinary levels of vanillylmandelic acid was increased in two cases, and urinary fractionated metanephrine levels was increased in one case of pheochromocytoma. Urinary normetanephrine alone increased in paraganglioma of organ of Zuckerkandl. All pheochromocytomas except one were positive for argentaffin reaction, and all paragangliomas at other sites were positive for argyrophilic reaction. Chromogranin immunohistochemistry stain was done for organ of Zuckerkandl lesion which came out to be positive. Conclusion: Proper clinical

  2. Paraganglioma of the heart. The value of magnetic resonance imaging in the preoperative evaluation.

    Science.gov (United States)

    Conti, V R; Saydjari, R; Amparo, E G

    1986-10-01

    Although the 131I-metaiodobenzylguanidine scan has proven reliable in identifying mediastinal paragangliomas, further localization has usually required dynamic computerized tomographic scanning which requires rapid bolus injection of contrast material. In the case presented herein, magnetic resonance imaging provided accurate preoperative localization and added important anatomic detail that was not appreciated with dynamic computerized tomograms or with other studies. Magnetic resonance imaging can accurately localize cardiac paragangliomas without injection of contrast material and may provide more detailed information for better guidance for surgical excision.

  3. Paraganglioma with unusual presentation in parotid gland: A diagnostic dilemma in fine needle aspiration

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    Anagh A Vora

    2012-01-01

    Full Text Available Paragangliomas (PGLs are uncommon tumors. Although PGLs are known to occur in the head and neck region, especially the carotid body, middle ear, and larynx, involvement of the parotid glands has not been reported. In this article, we report the fine needle aspiration features of tumor in an unusual location, presenting as a parotid gland mass, submitted to pathology for initial diagnosis. The clinical presentation, cytomorphology, and the immunohistochemical features for the diagnosis are described. To our knowledge, this is the first case of paraganglioma of the parotid gland reported in the literature.

  4. Endoscopic treatment of duodenal varices with cyanoacrylate

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    Ana Mora-Soler

    2013-12-01

    Full Text Available Background: the duodenum is the most common location for ectopic varices. Bleeding is rare, but when it appears, it is massive and difficult to control. Material and methods: retrospective description of five clinical cases of digestive bleeding secondary to duodenal varices that we observed between the years 2011 and 2012, together with their clinical characteristics, endoscopic diagnosis, endoscopic treatment with cyanoacrylate injection and the posterior follow-up and assessment of new bleeding. Results: all five patients were treated with an endoscopic cyanoacrylate injection and two of the patients experienced a digestive rebleeding. Three of the patients died during the follow-up period, only one due to cause digestive bleeding. Conclusion: in conclusion we can state that endoscopic treatment of duodenal varices with cyanoacrylate is technically possible, and it permits us to control the first bleeding before doing other definitive treatments, if the patient condition allows it.

  5. Pancreatic-induced Intramural Duodenal Haematoma

    Directory of Open Access Journals (Sweden)

    Julius K. Ma

    2008-04-01

    Full Text Available Spontaneous intramural duodenal haematoma (IDH is an uncommon pathology and it is usually related to anticoagulant therapy. Other causes include various pancreatic diseases, connective tissue disease, peptic ulcer disease and pancreaticoduodenal aneurysm. IDH of pancreatic origin has been infrequently reported. The disease course can be life-threatening and serious complications may occur, including gastric outlet obstruction, duodenal perforation and septicaemia. A case of pancreatic-induced IDH is presented, for which pancreaticoduodenectomy was performed as definitive treatment. In general, medical treatment with continuous nasogastric aspiration and total parenteral nutrition is recommended as initial management strategy. Surgical interventions (evacuation of blood clot or surgical resection are reserved for patients in whom medical treatment fails or complications occur.

  6. Pheochromocytoma and paraganglioma: understanding the complexities of the genetic background.

    Science.gov (United States)

    Fishbein, Lauren; Nathanson, Katherine L

    2012-01-01

    Pheochromocytomas and paragangliomas (PCC/PGL) are tumors derived from the adrenal medulla or extra-adrenal ganglia, respectively. They are rare and often benign tumors that are associated with high morbidity and mortality due to mass effect and high circulating catecholamines. Although most PCCs and PGLs are thought to be sporadic, over one third are associated with 10 known susceptibility genes. Mutations in three genes causing well characterized tumor syndromes are associated with an increased risk of developing PCCs and PGLs, including VHL (von Hippel-Lindau disease), NF1 (Neurofibromatosis Type 1), and RET (Multiple Endocrine Neoplasia Type 2). Mutations in any of the succinate dehydrogenase (SDH) complex subunit genes (SDHA, SDHB, SDHC, SDHD) can lead to PCCs and PGLs with variable penetrance, as can mutations in the subunit cofactor, SDHAF2. Recently, two additional genes have been identified, TMEM127 and MAX. Although these tumors are rare in the general population, occurring in two to eight per million people, they are more commonly associated with an inherited mutation than any other cancer type. This review summarizes the known germline and somatic mutations leading to the development of PCC and PGL, as well as biochemical profiling for PCCs/PGLs and screening of mutation carriers.

  7. Pheochromocytoma and paraganglioma: molecular testing and personalized medicine.

    Science.gov (United States)

    Burnichon, Nelly; Buffet, Alexandre; Gimenez-Roqueplo, Anne-Paule

    2016-01-01

    Pheochromocytomas and paragangliomas (PPGLs) are rare tumours, strongly associated with inherited susceptibility gene mutations, and presenting limited therapeutic options for patients with metastatic disease. This review discusses the recent developments in the characterization of PPGL genetic heterogeneity and associated tumorigenesis pathways, together with their potential clinical relevance. The mutational landscape of PPGL is now well defined, especially with the contribution of next-generation sequencing. Up to 70% of these tumours harbour a germline or a somatic mutation in one of the numerous predisposing genes. In parallel, 'omics' analyses have identified mutation-linked subsets of tumours substantially associated with molecular signatures suggesting new therapeutic targets for patients with a malignant transformation of the disease. In the near future, extended molecular testing of PPGL could be used to determine therapeutic approaches and assess diagnosis and prognosis biomarkers. Considering the current development of next-generation sequencing-based genetic screening, this technology appears as a good option to improve both PPGL molecular diagnosis and patient management.

  8. Nuclear molecular imaging of paragangliomas; Imagerie moleculaire nucleaire des paragangliomes

    Energy Technology Data Exchange (ETDEWEB)

    Taieb, D.; Tessonnier, L.; Mundler, O. [Service central de biophysique et de medecine nucleaire, CHU de la Timone, 13 - Marseille (France)

    2010-08-15

    Paragangliomas (PGL) are relatively rare neural crest tumors originating in the adrenal medulla (usually called pheochromocytoma), chemoreceptors (i.e., carotid and aortic bodies) or autonomic ganglia. These tumors are highly vascular, usually benign and slow-growing. PGL may occur as sporadic or familial entities, the latter mostly in association with germline mutations of the succinate dehydrogenase (SDH) B, SDHC, SDHD, SDH5, von Hippel-Lindau (VHL), ret proto-oncogene (RET), neurofibromatosis 1 (NF1) (von Recklinghausen's disease), prolyl hydroxylase domain protein 2 (PHD2) genes and TMEM127. Molecular nuclear imaging has a central role in characterization of PGL and include: somatostatin receptor imaging ({sup 111}In, {sup 68}Ga), MIBG scintigraphy ({sup 131}I, {sup 123}I), {sup 18}F-dihydroxy-phenylalanine ({sup 18}F-DOPA) positron emission tomography (PET), and {sup 18}F-deoxyglucose ({sup 18}F-FDG) PET. The choice of the tracer is not yet fully established but the work-up of familial forms often require the combination of multiple approaches. (authors)

  9. Duodenal damage complicating percutaneous access to kidney

    OpenAIRE

    Antonio Corrêa Lopes Neto; Marcos Tobias-Machado; Roberto Vaz Juliano; Marco Aurélio Lipay; Milton Borrelli; Eric Roger Wroclawski

    2000-01-01

    CONTEXT: Since the first percutaneous nephrostomy performed by Goodwin in 1954, technical advances in accessing the kidneys via percutaneous puncture have increased the use of this procedure and thus the complications too. Among these complications, digestive tract damage is not common. DESIGN: Case report. CASE REPORT: We report a duodenal lesion that was corrected using surgical exploration and we touch on the therapeutic options, which may be conservative or interventionist. We chose conse...

  10. Duodenal Perforation Precipitated by Scrub Typhus

    OpenAIRE

    Raghunath Rajat; David Deepu; Arul Jeevan Jonathan; Abhilash Kundavaram Paul Prabhakar

    2015-01-01

    Scrub typhus is an acute febrile illness usually presenting with fever, myalgia, headache, and a pathognomonic eschar. Severe infection may lead to multiple organ failure and death. Gastrointestinal tract involvement in the form of gastric mucosal erosions and ulcerations owing to vasculitis resulting in gastrointestinal bleeding is common. This process may worsen a pre-existent asymptomatic peptic ulcer, causing duodenal perforation, and present as an acute abdomen requiring surgical explora...

  11. Portal hypertensive duodenal polyp: A case report

    Institute of Scientific and Technical Information of China (English)

    Jean-David Zeitoun; Ariane Chryssostalis; Benoit Terris; Frederic Prat; Marianne Gaudric; Stanislas Chaussade

    2007-01-01

    Abnormalities of gastric mucosa in patients with portal hypertension are well documented. Manifestations of portal hypertension in small bowel and colon are less common. Colonic polypoid lesions microscopically consisting of a normal mucosa, with dilatation of submucosal vessels, have been described. We here report the first case of portal hypertensive duodenal polyp, responsible for gastro-intestinal bleeding.Endoscopic treatment turned out to be successful.

  12. [Perforated duodenal diverticulum: report of a case].

    Science.gov (United States)

    Gulotta, G; Agosta, G; Romano, G

    2001-01-01

    After the colon, the duodenum is the most common site of diverticula. Duodenal diverticula can be divided into two types: intraluminal or extraluminal. The latter are more frequent, with a prevalence ranging from 0.6 to 27% in relation to the diagnostic methods utilized. Females are more often affected than males. About 70-75% of extraluminal duodenal diverticula are located in a circular area centred around the ampulla of Vater within a radius of 2-3 cm; these are defined as periampullary or juxtapapillary. Perforation is the rarest type of complication and can simulate different clinical conditions. CT plays a fundamental role in diagnosis also in relation to the different diverticular topography. Perforation is an indication for emergency surgery. The authors describe the clinical case of a duodenal diverticulum containing the outlet of the papilla, complicated by perforation; CT showed retroduodenal fluid and free air. Emergency surgery with an external biliary drainage, naso-biliary probe, and a diverticulo-jejunostomy on a Roux-en-Y defunctionalised loop, resolved the condition.

  13. Radioiodinated MIBG in paraganglioma and pheochromocytoma: previous results and early experiences using no-carrier-added MIBG

    Energy Technology Data Exchange (ETDEWEB)

    James, Olga [Duke University Medical Center, Box 3949, Durham, NC 27710 (United States)], E-mail: olga.g.james@duke.edu; Coleman, R. Edward [Duke University Medical Center, Box 3949, Durham, NC 27710 (United States)], E-mail: colem010@mc.duke.edu

    2008-08-15

    The majority of pheochromocytomas and paragangliomas are benign, with malignancy occurring in approximately 10% of pheochromocytoma patients. The malignancy rate among paragangliomas is 15-35% or higher if associated with succinate dehydrogenase B gene mutations. The 5-year mortality rate in malignant pheochromocytoma and paraganglioma is nearly 50%. Malignancy of both pheochromocytoma and paraganglioma is determined by the existence of metastasis or local invasion and not by the cellular characteristics. There are no known clinical, biochemical or histopathological differences between pheochromocytoma and paraganglioma. Metaiodobenzylguanidine (MIBG) radiolabeled with either {sup 123}I or {sup 131}I has been used to diagnose neuroendocrine tumors such as paraganglioma and pheochromocytoma, and {sup 131}I-MIBG has been used to treat these tumors. The role of radioiodinated MIBG in treating neuroendocrine tumors is still being evaluated. More recently, no-carrier-added (nca) MIBG has become available, and the advantages of nca MIBG over ca MIBG are being demonstrated. This article reviews the biology of paragangliomas and pheochromocytomas, the role of MIBG imaging in the diagnosis of these tumors and the role of both ca and nca {sup 131}I-MIBG in the treatment of these tumors. New data on nca {sup 131}I-MIBG in the therapy of these tumors are included.

  14. Russell body duodenitis with immunoglobulin kappa lightchain restriction

    Institute of Scientific and Technical Information of China (English)

    William R Munday; Lucy Harn Kapur; Mina Xu; Xuchen Zhang

    2015-01-01

    Russell bodies are eosinophilic intracytoplasmicglobules which are likely the result of disturbedsecretion of immunoglobulins that accumulate withinthe plasma cell. Russell body collections have beenidentified within the stomach, known as Russellbody gastritis. Similar lesions within the duodenumare referred to as Russell body duodenitis, whichis rare. Several Russell body gastritis case reportsare associated with Helicobacter pylori . However,the etiology of Russell body duodenitis remainsunclear. Here we report the first case of Russell bodyduodenitis with immunoglobulin light chain restrictionin a background of peptic duodenitis.

  15. Perforated duodenal diverticulum caused by Bezoar: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Eun Jung; Moon, Sung Kyoung; Park, Seong Jin; Lim, Joo Won; Lee, Dong Ho; Ko, Young Tae [Dept. of Radiology, College of Medicine, Kyung Hee University, Seoul (Korea, Republic of)

    2013-11-15

    Duodenal diverticulum is common, but its perforation is a rare complication. Duodenal diverticulum perforation requires prompt treatments because of its high mortality rate. However, an accurate diagnosis is difficult to make due to nonspecific symptoms and signs. It can be misdiagnosed as pancreatitis, cholecystitis, or peptic ulcer. Herein, we report a case of perforated duodenal diverticulum caused by bezoar in a 33-year-old woman whom was diagnosed by abdomen computed tomography and ultrasonography.

  16. Pheochromocytoma/Paraganglioma: Review of perioperative management of blood pressure and update on genetic mutations associated with pheochromocytoma.

    Science.gov (United States)

    Fishbein, Lauren; Orlowski, Robert; Cohen, Debbie

    2013-06-01

    Pheochromocytomas and paragangliomas are rare tumors with high morbidity rates caused by excessive catecholamine secretion, even though the majority of tumors are benign. The use of perioperative blockade regimens, together with improved surgical techniques, has greatly impacted the perioperative morbidity associated with these tumors. The old dogma of the "tumor of tens" no longer holds true. For example, at least one third of all pheochromocytomas and paragangliomas are hereditary, with mutations in 1 of 10 well-characterized susceptibility genes, and one quarter of all tumors are malignant. This review focuses on the perioperative management of pheochromocytoma and paragangliomas and the clinical implications of the associated genetic mutations.

  17. Pheochromocytoma/Paraganglioma: Review of perioperative management of blood pressure and update on genetic mutations associated with pheochromocytoma

    Science.gov (United States)

    Fishbein, Lauren; Orlowski, Robert; Cohen, Debbie

    2015-01-01

    Pheochromocytomas and paragangliomas are rare tumors with high morbidity, due to excessive catecholamine secretion, even though the majority of tumors are benign. The use of perioperative blockade regimens, together with improved surgical techniques, has greatly impacted the perioperative morbidity associated with these tumors. The old dogma of the “tumor of tens” no longer holds true. For example, at least one-third of all pheochromocytomas and paragangliomas are hereditary with mutations in one of ten well characterized susceptibility genes, and one-quarter of all tumors are malignant. This review will focus on the perioperative management of pheochromocytoma and paragangliomas and the clinical implications of the associated genetic mutations. PMID:23730992

  18. Diagnosis and treatment of 34 cases of blunt duodenal injuries

    Institute of Scientific and Technical Information of China (English)

    BAO Shi-ting; WANG San-ming

    2007-01-01

    Objective: To summarize the experience on diagnosis and treatment of blunt duodenal injuries, and thus to improve the therapeutic skills. Methods: Clinical data of 34 cases of blunt duodenal injuries admitted to our hospital from 1990 to 2006 were retrospectively analyzed. Results: Among them, 28 cases were cured, 20 cases presented with complications, and 6 cases died. The causes of death were: duodenal or pancreatic fistula in 2 cases, intra- and retroperitoneal infection complicating septicopyemia in 2, disseminated intravascular coagulation in 1 and multiple organ failure in 1. Conclusions: Misdiagnosis and missed diagnosis predispose to happen for blunt duodenal injuries. Early recognition and surgical intervention are critical to a successful rescue.

  19. Russell body duodenitis with immunoglobulin kappa light chain restriction.

    Science.gov (United States)

    Munday, William R; Kapur, Lucy Harn; Xu, Mina; Zhang, Xuchen

    2015-01-16

    Russell bodies are eosinophilic intracytoplasmic globules which are likely the result of disturbed secretion of immunoglobulins that accumulate within the plasma cell. Russell body collections have been identified within the stomach, known as Russell body gastritis. Similar lesions within the duodenum are referred to as Russell body duodenitis, which is rare. Several Russell body gastritis case reports are associated with Helicobacter pylori. However, the etiology of Russell body duodenitis remains unclear. Here we report the first case of Russell body duodenitis with immunoglobulin light chain restriction in a background of peptic duodenitis.

  20. Urinary 5-HIAA excretion is not increased in patients with head and neck paragangliomas.

    NARCIS (Netherlands)

    Hulsteijn, L.T. van; Duinen, N. van; Romijn, J.A.; Smit, J.W.A.; Corssmit, E.P.

    2012-01-01

    Background: Case reports have documented carcinoid-like features in head and neck paragangliomas (HNPGLs), which, in addition to catecholamine storing granules, may also contain granules with serotonin. Serotonin is metabolized to 5-hydroxyindoleacetic acid (5-HIAA). Aim: To assess the urinary

  1. First experiences with genetic counselling based on predictive DNA diagnosis in hereditary glomus tumours (paragangliomas)

    NARCIS (Netherlands)

    Oosterwijk, JC; Jansen, JC; vanSchothorst, EM; Oosterhof, AW; Devilee, P; Bakker, E; Zoeteweij, MW; vanderMey, AGL

    1996-01-01

    Hereditary glomus tumour (MIM 168000) or paraganglioma (PGL) is a slowly progressive disorder causing benign tumour growth predominantly in the head and neck region. Though benign in nature the tumours can lead to severe morbidity. Inheritance of PGL is autosomal dominant and is strongly modified by

  2. Immunohistochemical expression of stem cell markers in pheochromocytomas/paragangliomas is associated with SDHx mutations

    NARCIS (Netherlands)

    Oudijk, L.; Neuhofer, C. M.; Lichtenauer, U. D.; Papathomas, T. G.; Korpershoek, E.; Stoop, H.; Oosterhuis, J. W.; Smid, M.; Restuccia, D. F.; Robledo, M.; De Cubas, A. A.; Mannelli, M.; Gimenez-Roqueplo, A. P.; Dinjens, W. N M; Beuschlein, F.; De Krijger, R. R.

    2015-01-01

    Objective: Pheochromocytomas (PCCs) are neuroendocrine tumors that occur in the adrenal medulla, whereas paragangliomas (PGLs) arise from paraganglia in the head, neck, thorax, or abdomen. In a variety of tumors, cancer cells with stem cell-like properties seem to form the basis of tumor initiation

  3. Protocol for the examination of specimens from patients with pheochromocytomas and extra-adrenal paragangliomas

    NARCIS (Netherlands)

    O. Mete (Ozgur); A.S. Tischler (Arthur); R.R. de Krijger (Ronald); A.M. McNicol (Anne Marie); G. Eisenhofer (Graeme); K. Pacak (Karel); E. Ezzat (Shereen); S.L. Asa (Sylvia)

    2014-01-01

    textabstractDuring the last decade there have been revolutionary breakthroughs in understanding the biology of pheochromocytomas and extra-adrenal paragangliomas. Discoveries of new susceptibility genes and genotype-phenotype correlations have led to the realization that appropriate patient care req

  4. Dopamine concentration in blood platelets is elevated in patients with head and neck paragangliomas

    NARCIS (Netherlands)

    Osinga, Thamara E.; van der Horst-Schrivers, Anouk N A; van Faassen, Martijn; Kerstens, Michiel N; Dullaart, Robin P F; Peters, Marloes A M; van der Laan, Bernard F A M; de Bock, Geertruida H; Links, Thera P; Kema, Ido P

    2015-01-01

    BACKGROUND: Plasma 3-methoxytyramine (3-MT), a metabolite of dopamine, is elevated in up to 28% of patients with head and neck paragangliomas (HNPGLs). As free dopamine is incorporated in circulating platelets, we determined dopamine concentration in platelets in patients with a HNPGL. METHODS: A si

  5. First experiences with genetic counselling based on predictive DNA diagnosis in hereditary glomus tumours (paragangliomas)

    NARCIS (Netherlands)

    Oosterwijk, JC; Jansen, JC; vanSchothorst, EM; Oosterhof, AW; Devilee, P; Bakker, E; Zoeteweij, MW; vanderMey, AGL

    1996-01-01

    Hereditary glomus tumour (MIM 168000) or paraganglioma (PGL) is a slowly progressive disorder causing benign tumour growth predominantly in the head and neck region. Though benign in nature the tumours can lead to severe morbidity. Inheritance of PGL is autosomal dominant and is strongly modified by

  6. Paraganglioma of the vagina: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Cai T

    2014-06-01

    Full Text Available Tao Cai,1 Yucong Li,2 Qingming Jiang,3 Dong Wang,2 Yu Huang2 1Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, 2Department of Gynecologic Oncology, 3Department of Pathology, Chongqing Cancer Research Institute, Chongqing, People’s Republic of China Abstract: Vaginal paraganglioma is a rare and unusual tumor occurring in the vaginal wall. It is a solitary primary paraganglioma, especially in atypical sites. Herein, we report an unusual case of a 17-year-old woman who had not experienced vomiting, or hypertension. She was found to have an immobile solid mass in the right side of her vaginal wall. Positron emission tomography/computed tomography scans revealed a well-defined solid ovoid mass adjacent to the bladder and pelvic floor. Tumor markers were within the normal range. A transient blood pressure increase occurred during the biopsy. After oral administration of antihypertensive drugs, surgery was performed to completely remove the mass. Histopathological examination indicated that it was a paraganglioma of the vagina. Repeat computed tomography examination did not reveal any local recurrence or distant metastasis during the 12-month follow-up period. Keywords: vaginal paraganglioma, neuroendocrine tumor, clinical features, treatment

  7. First experiences with genetic counselling based on predictive DNA diagnosis in hereditary glomus tumours (paragangliomas)

    NARCIS (Netherlands)

    Oosterwijk, JC; Jansen, JC; vanSchothorst, EM; Oosterhof, AW; Devilee, P; Bakker, E; Zoeteweij, MW; vanderMey, AGL

    Hereditary glomus tumour (MIM 168000) or paraganglioma (PGL) is a slowly progressive disorder causing benign tumour growth predominantly in the head and neck region. Though benign in nature the tumours can lead to severe morbidity. Inheritance of PGL is autosomal dominant and is strongly modified by

  8. A cryptic BAP1 splice mutation in a family with uveal and cutaneous melanoma, and paraganglioma

    DEFF Research Database (Denmark)

    Wadt, K.; Choi, J.; Chung, J.Y.;

    2012-01-01

    as paraganglioma, breast cancer, and suspected mesothelioma cases in the family. Bioinformatic analysis and splicing assays demonstrated that this mutation creates a strong cryptic splice donor, resulting in aberrant splicing and a truncating frameshift of the BAP1 transcript. Somatic loss of the wild-type allele...

  9. Genotype and tumor locus determine expression profile of pseudohypoxic pheochromocytomas and paragangliomas

    NARCIS (Netherlands)

    Shankavaram, U.; Fliedner, S.M.; Elkahloun, A.G.; Barb, J.J.; Munson, P.J.; Huynh, T.T.; Matro, J.C.; Turkova, H.; Linehan, W.M.; Timmers, H.J.L.M.; Tischler, A.S.; Powers, J.F.; Krijger, R. de; Baysal, B.E.; Takacova, M.; Pastorekova, S.; Gius, D.; Lehnert, H.; Camphausen, K.; Pacak, K.

    2013-01-01

    Pheochromocytomas (PHEOs) and paragangliomas (PGLs) related to mutations in the mitochondrial succinate dehydrogenase (SDH) subunits A, B, C, and D, SDH complex assembly factor 2, and the von Hippel-Lindau (VHL) genes share a pseudohypoxic expression profile. However, genotype-specific differences

  10. Biochemically silent abdominal paragangliomas in patients with mutations in the succinate dehydrogenase subunit B gene.

    NARCIS (Netherlands)

    Timmers, H.J.L.M.; Pacak, K.; Huynh, T.T.; Abu-Asab, M.; Tsokos, M.; Merino, M.J.; Baysal, B.E.; Adams, K.T.; Eisenhofer, G.

    2008-01-01

    CONTEXT: Patients with adrenal and extra-adrenal abdominal paraganglioma (PGL) almost invariably have increased plasma and urine concentrations of metanephrines, the O-methylated metabolites of catecholamines. We report four cases of biochemically silent abdominal PGL, in which metanephrines were no

  11. Retroperitoneal paraganglioma presenting with pancytopenia: A rare case with rare manifestation

    Directory of Open Access Journals (Sweden)

    Mutlu Ünver

    2015-01-01

    Discussion: Complete surgical excision is the treatment of choice for extra-adrenal paragangliomas as well as for recurrent or metastatic neoplasms. Reactive thrombocytosis is a common cause of thrombocytosis. Splenectomy was found to be one of the main causes of extreme reactive thrombocytosis. Reactive thrombocytosis is a predictable finding after splenectomy and management of the thrombocytosis and prevention of complications should be initiated.

  12. Pancreatic paraganglioma: An extremely rare entity and crucial role of immunohistochemistry for diagnosis

    Directory of Open Access Journals (Sweden)

    Mondita Borgohain

    2013-01-01

    Full Text Available Paragangliomas are rare neuroendocrine neoplasms arising in extra-adrenal chromaffin cells of autonomic nervous system and histologically akin to chemodectomas. They are rare, affecting about 1 in 2,000,000 population. It is a generic term applied to tumors of paraganglia regardless of the location. In rare instances, paragangliomas present around and involve the pancreas, thereby mimicking any one of the more common primary pancreatic lesions. Pancreatic paraganglioma is an extremely rare tumor. It grows slowly, so radical resection is recommended to achieve curability with good prognosis. These neoplasms present considerable diagnostic difficulty not only for the clinician and radiologist but also for the pathologist. Here, we report a case of a 55-year-old woman who presented with a left-sided abdominal swelling for 3 months duration, initially having clinical suspicion of an ovarian tumor. The radiological imaging revealed a lesion in the tail of pancreas with a differential diagnosis of pancreatic carcinoma and metastatic tumor. Only after exploratory laparotomy, the diagnosis was made as a rare case of pancreatic paraganglioma on the basis of histological examination and immunohistochemistry.

  13. Pancreatic paraganglioma: An extremely rare entity and crucial role of immunohistochemistry for diagnosis

    Science.gov (United States)

    Borgohain, Mondita; Gogoi, Gayatri; Das, Dipak; Biswas, Manjusha

    2013-01-01

    Paragangliomas are rare neuroendocrine neoplasms arising in extra-adrenal chromaffin cells of autonomic nervous system and histologically akin to chemodectomas. They are rare, affecting about 1 in 2,000,000 population. It is a generic term applied to tumors of paraganglia regardless of the location. In rare instances, paragangliomas present around and involve the pancreas, thereby mimicking any one of the more common primary pancreatic lesions. Pancreatic paraganglioma is an extremely rare tumor. It grows slowly, so radical resection is recommended to achieve curability with good prognosis. These neoplasms present considerable diagnostic difficulty not only for the clinician and radiologist but also for the pathologist. Here, we report a case of a 55-year-old woman who presented with a left-sided abdominal swelling for 3 months duration, initially having clinical suspicion of an ovarian tumor. The radiological imaging revealed a lesion in the tail of pancreas with a differential diagnosis of pancreatic carcinoma and metastatic tumor. Only after exploratory laparotomy, the diagnosis was made as a rare case of pancreatic paraganglioma on the basis of histological examination and immunohistochemistry. PMID:24083178

  14. Paragangliomas of the head and neck: clinical, morphological and immunohistochemical aspects

    Directory of Open Access Journals (Sweden)

    Pedro de Alcântara de Andrade Filho

    2001-05-01

    Full Text Available CONTEXT: Protein marker positivity can assist in the definition of the therapeutic approach towards head and neck paragangliomas. The establishment of the therapeutic approach should incorporate the results of such an investigation. OBJECTIVE: To establish criteria for benignancy and malignancy of vagal and jugular-tympanic paragangliomas, via the study of the relationships of sex, age, tumor size, duration of complaints, site, family history, presence of metastases, treatment, histological architecture and cell type with the immunohistochemical reactions to S100 protein, chromogranin and AgKi67. DESIGN: A retrospective study of histological and clinical records. SETTING: The Heliópolis and Oswaldo Cruz tertiary general hospitals, São Paulo. SAMPLE: 8 cases of head and neck paragangliomas. MAIN MEASUREMENTS: Determination of degree of positivity to paragangliomas via immunohistochemical reactions. RESULTS: 1. The protein markers for the principal cells (AgKi67 and chromogranin were sensitive in 100% of the tumors when used together. 2. S100 protein was well identified in the cytoplasm and nucleus of sustentacular cells and underwent reduction in the neoplasias. CONCLUSIONS: Chromogranin was proven to be a generic marker for neuroendocrine tumors; S100 protein was positive in all 8 cases and the AgKi67 had low positivity in all cases.

  15. The management of head-and-neck paragangliomas.

    Science.gov (United States)

    Capatina, Cristina; Ntali, Georgia; Karavitaki, Niki; Grossman, Ashley B

    2013-10-01

    Paragangliomas (PGLs) are tumours originating from neural crest-derived cells situated in the region of the autonomic nervous system ganglia. Head-and-neck PGLs (HNPGLs) originate from the sympathetic and parasympathetic paraganglia, most frequently from the carotid bodies and jugular, tympanic and vagal paraganglia, and are usually non-catecholamine secreting. Familial PGLs are considered to be rare, but recently genetic syndromes including multiple PGLs and/or phaeochromocytomas have been more thoroughly characterised. Nowadays, genetic screening for the genes frequently implicated in both familial and sporadic cases is routinely being recommended. HNPGLs are mostly benign, generally slow-growing tumours. Continuous growth leads to the involvement of adjacent neurovascular structures with increased morbidity rates and treatment-related complications. Optimal management mostly depends on tumour location, local involvement of neurovascular structures, estimated malignancy risk, patient age and general health. Surgery is the only treatment option offering the chance of cure but with significant morbidity rates, so a more conservative approach is usually considered, especially in the more difficult cases. Radiotherapy (fractionated or stereotactic radiosurgery) leads to tumour growth arrest and symptomatic improvement in the short term in many cases, but the long-term consequences are unclear. Early detection is essential in order to increase the chance of cure with a lower morbidity rate. The constant improvement in diagnostic imaging, surgical and radiation techniques has led to a safer management of these tumours, but there are still many therapeutic challenges, and no treatment algorithm has been agreed upon until now. The management of HNPGLs requires a multidisciplinary effort addressing the genetic, surgical, radiotherapeutic, oncological, neurological and endocrinological implications. Further progress in the understanding of their pathogenesis will lead to

  16. [Duodenal complications of rheumatoid purpura. Endoscopic aspects].

    Science.gov (United States)

    Chapoy, P; Guidon, M J; Louchet, E

    1984-01-01

    The aim of this work was to describe the endoscopic features and clinical outcome of the duodenal complications in anaphylactoid purpura. Over a 3-year period, 20 patients were hospitalized in our unit because of purpura rheumatica. Duodenal complications occurred in 5 cases warranting endoscopic assessment. All patients had bilious vomiting and epigastric pain, constantly associated with low-grade purpuric rash. Plasma factor XIII concentrations were always decreased. The duodenal complication was suspected radiologically in 2 cases when "thumbprint" impressions were seen. Petechiae, oedema and intramural hematoma with superficial erosions were present endoscopically in 3 cases. The lesions were severe and extensive, involving the entire duodenum in 3 cases and the jejunum in one case. In one patient, there was a stricture of the upper part of the second duodenum. Treatment consisted of parenteral nutrition (using a central catheter: 3 cases, or a peripheral vein: 2 cases) and cimetidine (30 mg/kg.bw). The clinical outcome was favorable in 4 patients; the symptoms vanished and the endoscopic lesions were reversible (including the stricture) with restitutio ad integrum after 10 days. The last patient died the 8th day of treatment, 3 days after digestive improvement; the cause of death was probably iatrogenic and related to accidental migration of the central catheter. These results suggest that endoscopic examination should be performed in all patients with anaphylactoid purpura presenting with bilious vomiting. Endoscopy seems to be of great value in deciding if parenteral nutrition is indicated--or not--and perhaps in order to contraindicate the use of steroid therapy in the case of ulcerated hematomas.

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

    NARCIS (Netherlands)

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

    2014-01-01

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

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

    NARCIS (Netherlands)

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

    2014-01-01

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

  19. Unusual duodenal perforation following endoscopic retrograde cholangiopancreatography

    Directory of Open Access Journals (Sweden)

    Martin Kobborg

    2011-02-01

    Full Text Available Perforation is a known but rare complication to Endoscopic retrograde cholangiopancreatography (ERCP with endoscopic sphincterotomy (ES. Most of the perforations are located in the periampullary area due to ES. This report presents an unusual perforation in the third part of the duodenum following ES. The patient an eigthy-sixt-year-old man underwent ERCP with ES. The patient had Magnetic Resonance Cholangio-pancreatography (MRCP and Computerized Tomography (CT verified cholelithiasis and intra- and extrahepatic cholestasis. The perforation was not found under the ERCP procedure but was clinically revealed when the patient developed pneumoscrotum after the procedure. A CT-scan with oral contrast later confirmed the duodenal perforation.

  20. Duodenal Metastasis of Malignant Pleural Mesothelioma

    Directory of Open Access Journals (Sweden)

    Huang-Chi Chen

    2008-12-01

    Full Text Available Metastatic malignant mesothelioma of the pleura is uncommon at the time of initial diagnosis. The gastrointestinal lumen is rarely found at autopsy in patients with widespread disease. Here, we describe an extremely rare case of isolated duodenal metastasis of sarcomatoid mesothelioma of the pleura in a 73-year-old man, without memory of any direct exposure to asbestos. The possibility of gastrointestinal tract metastasis should be considered in the presence of anemia or positive occult blood test in patients with malignant pleural mesothelioma.

  1. Duodenal adenocarcinoma in a 10-year-old boy

    Directory of Open Access Journals (Sweden)

    Zouari Mohamed

    2014-01-01

    Full Text Available Gastrointestinal malignancies are extremely rare in the paediatric population and duodenal cancers represent an even more unusual entity. It represents 0.3-1% of all gastrointestinal tumours. A case report of a 10-year-old boy with duodenal adenocarcinoma is reported and the difficulties of diagnosing and treating this rare tumour are discussed.

  2. Duodenal adenocarcinoma in a 10-year-old boy.

    Science.gov (United States)

    Mohamed, Zouari; Habib, Bouthour; Rabia, Ben Abdallah; Youssef, Hlel; Riath, Ben Malek; Youssef, Gharbi; Nejib, Kaabar

    2014-01-01

    Gastrointestinal malignancies are extremely rare in the paediatric population and duodenal cancers represent an even more unusual entity. It represents 0.3-1% of all gastrointestinal tumours. A case report of a 10-year-old boy with duodenal adenocarcinoma is reported and the difficulties of diagnosing and treating this rare tumour are discussed.

  3. Duodenal pseudolymphoma: A case report and review of literature

    Institute of Scientific and Technical Information of China (English)

    Yong-Heng Huang; Tian-Zhu Long; Zhi-Yu Xiao; Hua Ye; Yun-Le Wan; Jie Wang

    2011-01-01

    We report a rare case of duodenal pseudolymphoma without any symptoms. The lesion located in front of the head of the pancreas was found accidentally during a medical examination. The findings of computed tomography and positron emission tomography-computed tomography suggested a stromal tumor or malignant lymphoma. Surgical resection was performed. The lesions were pathologically diagnosed as duodenal pseudolymphoma.

  4. Retrospective analysis of duodenal injuries: A comprehensive overview

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    Sanjay Pandey

    2011-01-01

    Full Text Available Background/Aim: Duodenal injury is an uncommon finding, accounting for about about 3 - 5% of abdominal trauma, mainly resulting from both penetrating and blunt trauma, and is associated with significant mortality (6 - 25% and morbidity (30 - 60%. Patients and Methods : Retrospective analysis was performed in terms of presentation, management, morbidity and mortality on 14 patients of duodenal injuries out of a total of 172 patients of abdominal trauma attending Subharti Medical College. Results: Epigastric pain (100% along with vomiting (100% is the usual presentation of duodenal injuries in blunt abdominal trauma, especially to the upper abdomen. Computed tomography (CT was diagnostic in all cases. Isolated duodenal injury is a rare finding and the second part is mostly affected. Conclusion: Duodenal injury should always be suspected in blunt upper abdominal trauma, especially in those presenting with epigastric pain and vomiting. Investigation by CT and early surgical intervention in these patients are valuable tools to reduce the morbidity and mortality.

  5. Presentation and Surgical Management of Duodenal Duplication in Adults

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    Caroline C. Jadlowiec

    2015-01-01

    Full Text Available Duodenal duplications in adults are exceedingly rare and their diagnosis remains difficult as symptoms are largely nonspecific. Clinical presentations include pancreatitis, biliary obstruction, gastrointestinal bleeding from ectopic gastric mucosa, and malignancy. A case of duodenal duplication in a 59-year-old female is presented, and her treatment course is reviewed with description of combined surgical and endoscopic approach to repair, along with a review of historic and current recommendations for management. Traditionally, gastrointestinal duplications have been treated with surgical resection; however, for duodenal duplications, the anatomic proximity to the biliopancreatic ampulla makes surgical management challenging. Recently, advances in endoscopy have improved the clinical success of cystic intraluminal duodenal duplications. Despite these advances, surgical resection is still recommended for extraluminal tubular duplications although combined techniques may be necessary for long tubular duplications. For duodenal duplications, a combined approach of partial excision combined with mucosal stripping may offer advantage.

  6. Pheochromocytoma and paraganglioma in cyanotic congenital heart disease.

    Science.gov (United States)

    Opotowsky, Alexander R; Moko, Lilamarie E; Ginns, Jonathan; Rosenbaum, Marlon; Greutmann, Matthias; Aboulhosn, Jamil; Hageman, Abbie; Kim, Yuli; Deng, Lisa X; Grewal, Jasmine; Zaidi, Ali N; Almansoori, Ghadeera; Oechslin, Erwin; Earing, Michael; Landzberg, Michael J; Singh, Michael N; Wu, Fred; Vaidya, Anand

    2015-04-01

    Aberrant cellular oxygen sensing is a leading theory for development of pheochromocytoma (PHEO) and paraganglioma (PGL). The objective of the study was to test the hypothesis that chronic hypoxia in patients with cyanotic congenital heart disease (CCHD) increases the risk for PHEO-PGL. We investigated the association between CCHD and PHEO-PGL with two complementary studies: study 1) an international consortium was established to identify congenital heart disease (CHD) patients with a PHEO-PGL diagnosis confirmed by pathology or biochemistry and imaging; study 2) the 2000-2009 Nationwide Inpatient Survey, a nationally representative discharge database, was used to determine population-based cross-sectional PHEO-PGL frequency in hospitalized CCHD patients compared with noncyanotic CHD and those without CHD using multivariable logistic regression adjusted for age, sex, and genetic PHEO-PGL syndromes. In study 1, we identified 20 PHEO-PGL cases, of which 18 had CCHD. Most presented with cardiovascular or psychiatric symptoms. Median cyanosis duration for the CCHD PHEO-PGL cases was 20 years (range 1-57 y). Cases were young at diagnosis (median 31.5 y, range 15-57 y) and 7 of 18 had multiple tumors (two bilateral PHEO; six multifocal or recurrent PGL), whereas 11 had single tumors (seven PHEO; four PGL). PGLs were abdominal (13 of 17) or head/neck (4 of 17). Cases displayed a noradrenergic biochemical phenotype similar to reported hypoxia-related PHEO-PGL genetic syndromes but without clinical signs of such syndromes. In study 2, hospitalized CCHD patients had an increased likelihood of PHEO-PGL (adjusted odds ratio 6.0, 95% confidence interval 2.6-13.7, P < .0001) compared with those without CHD; patients with noncyanotic CHD had no increased risk (odds ratio 0.9, P = .48). There is a strong link between CCHD and PHEO-PGL. Whether these rare diseases coassociate due to hypoxic stress, common genetic or developmental factors, or some combination requires further

  7. Duodenal obstruction due to a preduodenal portal vein

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    MNC Vilakazi

    2014-01-01

    Full Text Available An infant presented with clinical signs and symptoms suggestive of a pyloric stenosis. On abdominal ultrasound, pyloric stenosis was excluded, and other causes for proximal duodenal obstruction, such as a duodenal web or annular pancreas, were suspected. At surgery, the cause was found to be due to an anterior portal vein or preduodenal portal vein, compressing the duodenum. There were no associated findings such as midgut malrotation, duodenal web and congenital anomalies. The treatment was a diamond-shaped duodeno-duodenostomy anterior to the portal vein. The patient improved after surgery.

  8. Duodenal Bleeding from Metastatic Renal Cell Carcinoma

    Science.gov (United States)

    Rustagi, Tarun; Rangasamy, Priya; Versland, Mark

    2011-01-01

    Massive upper gastrointestinal bleeding due to malignancy is relatively uncommon and the duodenum is the least frequently involved site. Duodenal metastasis is rare in renal cell carcinoma (RCC) and early detection, especially in case of a solitary mass, helps in planning further therapy. We report a case of intractable upper gastrointestinal bleeding from metastatic RCC to the duodenum. The patient presented with melena and anemia, 13 years after nephrectomy for RCC. On esophagogastroduodenoscopy, a submucosal mass was noted in the duodenum, biopsies of which revealed metastatic RCC. In conclusion, metastasis from RCC should be considered in nephrectomized patients presenting with gastrointestinal symptoms and a complete evaluation, especially endoscopic examination followed by biopsy, is suggested. PMID:21577373

  9. Duodenal Bleeding from Metastatic Renal Cell Carcinoma

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    Tarun Rustagi

    2011-04-01

    Full Text Available Massive upper gastrointestinal bleeding due to malignancy is relatively uncommon and the duodenum is the least frequently involved site. Duodenal metastasis is rare in renal cell carcinoma (RCC and early detection, especially in case of a solitary mass, helps in planning further therapy. We report a case of intractable upper gastrointestinal bleeding from metastatic RCC to the duodenum. The patient presented with melena and anemia, 13 years after nephrectomy for RCC. On esophagogastroduodenoscopy, a submucosal mass was noted in the duodenum, biopsies of which revealed metastatic RCC. In conclusion, metastasis from RCC should be considered in nephrectomized patients presenting with gastrointestinal symptoms and a complete evaluation, especially endoscopic examination followed by biopsy, is suggested.

  10. Duodenal bleeding from metastatic renal cell carcinoma.

    Science.gov (United States)

    Rustagi, Tarun; Rangasamy, Priya; Versland, Mark

    2011-04-20

    Massive upper gastrointestinal bleeding due to malignancy is relatively uncommon and the duodenum is the least frequently involved site. Duodenal metastasis is rare in renal cell carcinoma (RCC) and early detection, especially in case of a solitary mass, helps in planning further therapy. We report a case of intractable upper gastrointestinal bleeding from metastatic RCC to the duodenum. The patient presented with melena and anemia, 13 years after nephrectomy for RCC. On esophagogastroduodenoscopy, a submucosal mass was noted in the duodenum, biopsies of which revealed metastatic RCC. In conclusion, metastasis from RCC should be considered in nephrectomized patients presenting with gastrointestinal symptoms and a complete evaluation, especially endoscopic examination followed by biopsy, is suggested.

  11. Thyroid Storm Precipitated by Duodenal Ulcer Perforation

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    Shoko Natsuda

    2015-01-01

    Full Text Available Thyroid storm is a rare and life-threatening complication of thyrotoxicosis that requires prompt treatment. Thyroid storm is also known to be associated with precipitating events. The simultaneous treatment of thyroid storm and its precipitant, when they are recognized, in a patient is recommended; otherwise such disorders, including thyroid storm, can exacerbate each other. Here we report the case of a thyroid storm patient (a 55-year-old Japanese male complicated with a perforated duodenal ulcer. The patient was successfully treated with intensive treatment for thyroid storm and a prompt operation. Although it is believed that peptic ulcer rarely coexists with hyperthyroidism, among patients with thyroid storm, perforation of a peptic ulcer has been reported as one of the causes of fatal outcome. We determined that surgical intervention was required in this patient, reported despite ongoing severe thyrotoxicosis, and reported herein a successful outcome.

  12. Ganglioneuromatous paraganglioma of the cauda equina--a pathological case study.

    Science.gov (United States)

    Pytel, Peter; Krausz, Thomas; Wollmann, Robert; Utset, Manuel F

    2005-04-01

    This study presents a rare case of compound paraganglioma/ganglioneuroma with comprehensive immunohistochemical studies that reveal strong cytokeratin expression in all components. A 74-year-old woman presented with a mass lesion of the cauda equina. The 1.8-cm tumor showed 3 histomorphologically and immunohistochemically distinct components: typical paragangliomatous neuroendocrine areas, mature ganglion cell-like neuronal areas, and a "neuromatous" proliferation of Schwann cells with admixed axons. As often seen in cauda equina paragangliomas, the neuroendocrine cells were cytokeratin-positive. In addition, immunoreactivity for cytokeratins was also observed in the neurons and axons. This tumor illustrates the broad spectrum of divergent differentiation that can be seen in cells of sympathoadrenal lineage.

  13. Cardiomiopatía de Takotsubo en un paciente con paraganglioma torácico funcional

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    Melisa Arias-Quirós

    2015-06-01

    Full Text Available La cardiomiopatía de Takotsubo sigue siendo una entidad nosológica subdiagnosticada a causa de su variada presentación clínica. Esta patología tiene predilección por el sexo femenino; se manifiesta como una disfunción del ventrículo izquierdo desencadenada por un evento agudo de estrés; tiene la particularidad de ser un proceso reversible. La incidencia de paraganglioma es de menos del 1% de los tumores intratorácicos, y su relación con la génesis de cardiomiopatía de Takotsubo está más que demostrada, aunque es una causa poco frecuente de esta. Se reporta caso clínico de una paciente femenina de 43 años con cardiomiopatía de Takotsubo secundaria a un paraganglioma intratorácico funcional.

  14. Paraganglioma gangliocítico en la tercera porción del duodeno

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    Humberto Álvarez-Pertuz

    2015-03-01

    Full Text Available El paraganglioma gangliocítico es un tumor neuroendocrino no epitelial, originado de la cresta neural, que afecta en la gran mayoría de los casos la segunda porción del duodeno, sin embargo puede presentarse en cualquiera de sus porciones e inclusive originarse en el yeyuno proximal, en el apéndice y en otros sitios menos frecuentes. Por su localización y la tendencia a ulcerarse, estas lesiones pueden manifestarse por sangrado digestivo, ictericia, obstrucción o anemia como en el caso que describiremos más adelante, y muchas veces son encontradas de forma incidental durante procedimientos endoscópicos o autopsias. El paraganglioma gangliocítico es un tumor benigno de pronóstico favorable, aunque se han reportado algunos casos de recurrencia, invasión linfática o metástasis a distancia. Histológicamente el paraganglioma gangliocítico está compuesto por varios patrones celulares, los cuales pueden ser corroborados mediante inmunohistoquímica y su tratamiento debe ir dirigido a la resección local o radical en algunos casos.

  15. Genetics of Apparently Sporadic Pheochromocytoma and Paraganglioma in a Chinese Population.

    Science.gov (United States)

    Lee, C H; Cheung, C Y Y; Chow, W S; Woo, Y C; Yeung, C Y; Lang, B H H; Fong, C H Y; Kwok, K H M; Chen, S P L; Mak, C M; Tan, K C B; Lam, K S L

    2015-10-01

    Identification of germline mutation in patients with apparently sporadic pheochromocytomas and paragangliomas is crucial. Clinical indicators, which include young age, bilateral or multifocal, extra-adrenal, malignant, or recurrent tumors, predict the likelihood of harboring germline mutation in Caucasian subjects. However, data on the prevalence of germline mutation, as well as the applicability of these clinical indicators in Chinese, are lacking. We conducted a cross-sectional study at a single endocrine tertiary referral center in Hong Kong. Subjects with pheochromocytomas and paragangliomas were evaluated for the presence of germline mutations involving 10 susceptibility genes, which included NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, TMEM 127, MAX, and FH genes. Clinical indicators were assessed for their association with the presence of germline mutations. Germline mutations, 2 being novel, were found in 24.4% of the 41 Chinese subjects recruited and 11.4% among those with apparently sporadic presentation. The increasing number of the afore-mentioned clinical indicators significantly correlated with the likelihood of harboring germline mutation in one of the 10 susceptibility genes. (r=0.757, p=0.026). The presence of 2 or more clinical indicators should prompt genetic testing for germline mutations in Chinese subjects. In conclusion, our study confirmed that a significant proportion of Chinese subjects with apparently sporadic pheochromocytoma and paraganglioma harbored germline mutations and these clinical indicators identified from Caucasians series were also applicable in Chinese subjects. This information will be of clinical relevance in the design of appropriate genetic screening strategies in Chinese populations.

  16. HuR in pheochromocytomas and paragangliomas - overexpression in verified malignant tumors.

    Science.gov (United States)

    Leijon, Helena; Salmenkivi, Kaisa; Heiskanen, Ilkka; Hagström, Jaana; Louhimo, Johanna; Heikkilä, Päivi; Ristimäki, Ari; Paavonen, Timo; Metso, Saara; Mäenpää, Hanna; Haglund, Caj; Arola, Johanna

    2016-09-01

    Pheochromocytomas and paragangliomas are rare, neural crest-originating, neuroendocrine tumors. HuR is an mRNA-binding protein of the ELAV/Hu-protein family, which participates in posttranscriptional regulation of many cancer-associated genes. HuR expression has been connected with aggressive behavior of several malignancies. Cyclooxygenase-2 (COX-2) is also expressed in several malignant tumors, and its expression is regulated by HuR. Tissue microarray of 153 primary pheochromocytomas and paragangliomas was investigated for the expression of HuR and COX-2 proteins by immunohistochemistry using two different HuR antibodies (HuR19F12 and HuR3A). In these tumors, the expression of both intranuclear and cytoplasmic HuR was detectable. Increased cytoplasmic HuR expression was significantly associated with metastatic tumors. Increased COX-2 and MIB-1 expression also was associated with metastatic potential, and moreover, HuR and COX-2 expression correlated with each other. Our data suggest that increased expression of HuR protein is associated with metastatic potential of paragangliomas and pheochromocytomas, and COX-2 seems to be a target of HuR.

  17. Updated and New Perspectives on Diagnosis, Prognosis, and Therapy of Malignant Pheochromocytoma/Paraganglioma

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    Gabriele Parenti

    2012-01-01

    Full Text Available Malignant pheochromocytomas/paragangliomas are rare tumors with a poor prognosis. Malignancy is diagnosed by the development of metastases as evidenced by recurrences in sites normally devoid of chromaffin tissue. Histopathological, biochemical, molecular and genetic markers offer only information on potential risk of metastatic spread. Large size, extraadrenal location, dopamine secretion, SDHB mutations, a PASS score higher than 6, a high Ki-67 index are indexes for potential malignancy. Metastases can be present at first diagnosis or occur years after primary surgery. Measurement of plasma and/or urinary metanephrine, normetanephrine and metoxytyramine are recommended for biochemical diagnosis. Anatomical and functional imaging using different radionuclides are necessary for localization of tumor and metastases. Metastatic pheochromocytomas/paragangliomas is incurable. When possible, surgical debulking of primary tumor is recommended as well as surgical or radiosurgical removal of metastases. I-131-MIBG radiotherapy is the treatment of choice although results are limited. Chemotherapy is reserved to more advanced disease stages. Recent genetic studies have highlighted the main pathways involved in pheochromocytomas/paragangliomas pathogenesis thus suggesting the use of targeted therapy which, nevertheless, has still to be validated. Large cooperative studies on tissue specimens and clinical trials in large cohorts of patients are necessary to achieve better therapeutic tools and improve patient prognosis.

  18. Post-operative duodenal fistula: percutaneous treatment and review

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    D.Huerta

    2015-04-01

    Full Text Available Duodenal fistula is a complex condition, relatively frequent presentation, being in most cases of postoperative origin. Among the latter, 6% to 11% are secondary to surgical treatment of perforated duodenal ulcer, and more unusual, as a complication of cholecystectomy. Two cases treated percutaneous at the Polyclinic Bank city of Buenos Aires are presented. The first, a female patient with a duodenal fistula as a postoperative complication of a perforated duodenal ulcer and the second one patient male with the same pathology but as a complication of cholecystectomy. Percutaneous treatment of this disease has been reported sporadically without having proven its usefulness. Once diagnosed the same Fistulography and obliteration were performed percutaneously achieving complete remission of the disease.

  19. Duodenal injuries due to trauma: Review of the literature.

    Science.gov (United States)

    García Santos, Esther; Soto Sánchez, Ana; Verde, Juan M; Marini, Corrado P; Asensio, Juan A; Petrone, Patrizio

    2015-02-01

    Duodenal injuries constitute a challenge to the Trauma Surgeon, mainly due to their retroperitoneal location. When identified, they present associated with other abdominal injuries. Consequently, they have an increased morbidity and mortality. At best estimates, duodenal lesions occur in 4.3% of all patients with abdominal injuries, ranging from 3.7% to 5%, and because of their anatomical proximity to other organs, they are rarely an isolated injury. The aim of this paper is to present a concise description of the anatomy, diagnosis, surgical management and treatment of complications of duodenal trauma, and an analysis of complications and mortality rates of duodenal injuries based on a 46-year review of the literature. Copyright © 2014 AEC. Publicado por Elsevier España, S.L.U. All rights reserved.

  20. Duodenal metastases of renal cell carcinoma: a case report

    Institute of Scientific and Technical Information of China (English)

    HE Xiang-hui; LU Ning; ZHANG Rui; ZHU Li-wei

    2010-01-01

    @@ Malignancy accounts for about 5% of upper gastrointestinal hemorrhage and needs to be treated by surgical intervention. Duodenal metastases are a rare and peculiar cause of upper gastrointestinal bleeding.

  1. Helicobacter pylori in duodenal ulcer disease and its eradication

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    Sengupta S

    2002-01-01

    Full Text Available Antral biopsy specimens were processed for Helicobacter pylori by Gram staining, rapid urease test (RUT and culture from 25 patients with symptoms of duodenal ulcer, amongst whom the positivity rate was 84%. Follow up of 16 patients after appropriate therapy showed complete regression of the disease in 87.5% of cases whereas in 12.5% of cases a decrease in the extent of duodenal ulceration was noted.

  2. Isolated duodenal rupture due to blunt abdominal trauma

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    Celik Atilla

    2006-01-01

    Full Text Available Duodenal rupture following blunt abdominal trauma is rare and it usually seen with other abdominal organ injuries. It represents approximately 2% to 20% of patients with blunt abdominal injury and often occurs after blows to the upper abdomen, or abdominal compression from high-riding seat belts. Two cases of blunt duodenal rupture successfully treated surgically, are presented with their preoperative diagnosis and final out comes.

  3. Therapeutic angiography for giant bleeding gastro-duodenal artery pseudoaneurysm

    Institute of Scientific and Technical Information of China (English)

    Ram; Elazary; Mahmoud; Abu-Gazala; Avraham; Schlager; Noam; Shussman; Avraham; I; Rivkind; Allan; I; Bloom

    2010-01-01

    We present the case of an 18-year-old female transferred to our center from an outside hospital due to persistent gastrointestinal bleeding. Two weeks prior to her transfer she underwent duodenal omentopexy for a perforated duodenal peptic ulcer. The patient underwent a computed tomography angiogram which identified the source of bleeding as a giant gastroduodenal artery (GDA) pseudoaneurysm. The patient was taken to interventional radiology where successful microcoil embolization was performed. We present ...

  4. Malignant transformation of ectopic pancreatic cells in the duodenal wall

    Institute of Scientific and Technical Information of China (English)

    Roberto; Bini; Paolo; Voghera; Alberto; Tapparo; Raffaele; Nunziata; Andrea; Demarchi; Matteo; Capocefalo; Renzo; Leli

    2010-01-01

    Ectopic pancreas (EP) is the relatively uncommon presence of pancreatic tissue outside the normal location of the pancreas. This condition is usually asymptomatic and rarely complicated by pancreatitis and malignant transformation. A few cases of neoplastic phenomena that developed from EP into the duodenal wall are described in the literature. Herein we report a case of gastric outlet obstruction due to adenocarcinoma arising from EP of the duodenal wall. The patient underwent a Whipple's procedure and had...

  5. Omeprazole promotes proximal duodenal mucosal bicarbonate secretion in humans.

    OpenAIRE

    Mertz-Nielsen, A; Hillingsø, J; Bukhave, K; Rask-Madsen, J

    1996-01-01

    The proton pump inhibitor, omeprazole, surprisingly resulted in higher rates of proximal duodenal mucosal bicarbonate secretion than previously reported using an H2 receptor antagonist for gastric acid inhibition. Gastroduodenal perfusions were performed in healthy volunteers to evaluate whether this incidental finding is explained by more potent gastric acid inhibition by omeprazole or might be caused by the different mode of drug action. Basal and stimulated gastric and duodenal bicarbonate...

  6. [Perforated duodenal diverticula. Case report and treatment options].

    Science.gov (United States)

    Guardado-Bermúdez, Fernando; Ardisson-Zamora, Fernando Josafat; Rojas-González, Juan Daniel; Medina-Benítez, Alberto; Corona-Suárez, Fernando

    2013-01-01

    the presence of duodenal diverticula was first described in 1710 by Chromel. Duodenal diverticulum is the second most common site of diverticula in the digestive tract. Anatomically duodenal diverticula are located in 10 to 67% in the second portion of duodenum, and its finding in most cases incidental. About 90% of patients appear asymptomatic, manifesting symptoms mostly once established complications such as: gastrointestinal bleeding and perforation. 78-years-old woman who attended our Emergency department with dyspnea, moderate epigastralgia, abdominal bloating, constipation and difficulty to pass gas; Laparotomy was performed to identify duodenal diverticulum in the third portion of the duodenum with a perforation of 5 mm in its cupula. It proceeds with diverticulectomy. The diagnosis of duodenal diverticulum as a cause of acute abdomen must be considered in our differential diagnosis in acute abdomen supported by imaging and endoscopy. The surgical management of duodenal diverticulum, in particular the resection of the diverticulum, remains as the recommendation for treatment with less morbidity and a good recovery.

  7. Duodenal White Spots Mimicking Intestinal Candidiasis: Report of Case

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    Ozgur Turk

    2015-12-01

    Full Text Available Duodenal white spots are mentioned in these nonspecific lesions until recently. Although there is not enough studies about duedonal white spots yet; these lesions described in a separate syndrome. Here now we reported a case that we diagnosed multiple Duodenal white spots mimicking intestinal candidiasis. Clinical manifestation and endoscopic appearance of lesions gave rise to thought intestinal candidiasis histopathological examination revealed us several duodenitis. There was no evidence of fungal infection in PAS staining. Early after endoscopy patient took treatment of Lansoprozole at the 30 mg dose and bismuth salicylate. Patients compliant declined and control endoscopy exposed white duodenal spots cleared away three months later. Duodenal white spots are becoming to be designated as a separate disease even a syndrome. Etiology of duodenal white spots must be determined carefully during endoscopy. Sometimes it is difficult to make the correct diagnosis by appearance of lesion; in such cases histopathological examination can be useful both differential diagnosis of disease and determination of etiological factor. [J Contemp Med 2015; 5(4.000: 249-252

  8. Helicobacter pylori and gastric or duodenal ulcer.

    Science.gov (United States)

    2016-01-01

    In patients with gastric or duodenal ulcer associated with Helicobacter pylori, treatment of the infection improves healing and prevents complications and recurrences. The drug regimen generally consists of a high-dose proton-pump inhibitor (PPI) such as omeprazole plus antibiotics. Using the standard Prescrire methodology, we conducted a review of the literature in order to determine the standard empirical antibiotic regimen for H. pylori infection in adults with gastric or duodenal ulcer in France. In 2015, due to an increase in H. pylori resistance to clarithromycin, a 7-day course of the PPI + clarithromycin + amoxicillin combination is effective in only about 70% of cases. A Cochrane systematic review and meta-analysis of trials involving thousands of patients suggests that prolonging treatment with a PPI + amoxicillin + clarithromycin or a PPI + amoxicillin + metronidazole to 10 or 14 days improves the rate of H. pylori eradication by 5% to 10%. A metanalysis of seven trials including a total of about 1000 patients showed that combination therapy with a PPI + amoxicillin + clarithromycin + metronidazole for 5 days eradicates H. pylori in about 90% of cases, compared to about 80% of cases with a PPI + amoxicillin + clarithromycin given for 7 days. Sequential treatment with amoxicillin for 5 days, followed by clarithromycin + metronidazole for 5 days, has also been tested in thousands of patients. Efficacy and adverse effects were similar to those observed when the same antibiotics were taken simultaneously for 5 days. In randomised trials, replacing clarithromycin or amoxicillin with a fluoroquinolone yielded conflicting results. In 2009, nearly 20% of H. pylori isolates were resistant to levofloxacin in France. Tetracycline has only been evaluated in combination with bismuth. The few available data on doxycycline suggest that its efficacy is similar to that of tetracycline. A fixed-dose combination of bismuth subcitrate potassium + metronidazole

  9. Paraganglioma de cuerpo carotídeo: reporte de un caso clínico con correlación familiar Carotid body paraganglioma: clinical case report with family correlation

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    José Martín Toranzo Fernández

    2011-06-01

    Full Text Available Los paragangliomas de cabeza y cuello son neoplasias infrecuentes que se originan de un tejido paraganglionar del grupo braquiomérco que está ligado al desarrollo de los arcos branquiales. No son cromafines, ya que no secretan catecolaminas¹. De estos paragangliomas, el que se presenta con más frecuencia es el del cuerpo carotídeo, que se origina a partir de los paraganglios localizados en la adventicia de la bifurcación del cuerpo carotídeo².The head and neck paragangliomas are infrequent neoplases that originate from a paraganglionar tissue of the brachiomeric group that are linked to the development of the branchial arches; they are not cromafins since they do not secrete catecholamines¹. The most frequent one appears in the carotid body that originates from the paraganglions located in the adventitia of the carotid body's bifurcation².

  10. The terminal web of the duodenal enterocyte.

    Science.gov (United States)

    Leeson, T S

    1982-06-01

    The terminal web-brush border complex of rodent duodenal enterocytes has been studied by electron microscopy to investigate its structure in relation to currently accepted models of motility in this region. The main adherens zone is composed chiefly of a fine feltwork of 5 to 7 nm filaments, some of which originate in zonulae adherentes. In some cells, this is not a complete layer or sheet. Passing into it from its deep aspect are 10 nm tonofilaments, which also form the basal zone. The filament density in the basal zone is less than that of the adherens zone, and many of the tonofilaments are associated with spot desmosomes. The apical zone contains a loose meshwork of 5 to 7 nm filaments with more filaments lying adjacent to plasmalemmae of the zonula occludens. The core of each microvillus contains a bundle of 17 to 48 microfilaments, 5-7 nm in diameter, apparently attached to the apical plasmalemma and with some slender cross filaments between core filaments and the plasmalemma. In the main, these core bundles of microfilaments pass deeply into and often through the adherens zone of the terminal web where they terminate abruptly. Filaments of the terminal web appear to interconnect microfilaments of adjacent core bundles but without positive evidence of 'splaying' of microfilaments of a core bundle within the adherens zone. These findings are discussed in relation to movement of microvilli.

  11. Perforated duodenal ulcer in Asir central hospital

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    Jastaniah Suleiman

    1997-01-01

    Full Text Available In a study of 27 cases of perforated duodenal ulcer seen at Asir Central Hospital over a period of seven years, two patients were females and 25 males. The highest incidence was in the fourth decade and the average age was 36.3 years. The Saudi-foreigner ratio was 1:2.9 in an area where the Saudi-foreigner population ratio was 1:3. Nine patients (33.3% were previously diagnosed as having peptic ulcer and had received treatment at one time or the other before perforation. Eighteen patients (66.7% were first diagnosed to peptic ulcer after the perforation. All the identified perforations were located anteriorly and anterosuperiorly. Only one case occurred in the second part of duodenum. The rest occurred in the first part. Two patients were treated successfully conservatively. The month perforations occurred most was the month of Shaaban. The fasting period during the month of Ramadhan did not show any increase in the prevalence of perforation in this hospital.

  12. Vitamin D-enhanced duodenal calcium transport.

    Science.gov (United States)

    Wongdee, Kannikar; Charoenphandhu, Narattaphol

    2015-01-01

    For humans and rodents, duodenum is a very important site of calcium absorption since it is exposed to ionized calcium released from dietary complexes by gastric acid. Calcium traverses the duodenal epithelium via both transcellular and paracellular pathways in a vitamin D-dependent manner. After binding to the nuclear vitamin D receptor, 1,25-dihydroxyvitamin D3 [1,25(OH)2D3] upregulates the expression of several calcium transporter genes, e.g., TRPV5/6, calbindin-D9k, plasma membrane Ca(2+)-ATPase1b, and NCX1, thereby enhancing the transcellular calcium transport. This action has been reported to be under the regulation of parathyroid-kidney-intestinal and bone-kidney-intestinal axes, in which the plasma calcium and fibroblast growth factor-23 act as negative feedback regulators, respectively. 1,25(OH)2D3 also modulates the expression of tight junction-related genes and convective water flow, presumably to increase the paracellular calcium permeability and solvent drag-induced calcium transport. However, vitamin D-independent calcium absorption does exist and plays an important role in calcium homeostasis under certain conditions, particularly in neonatal period, pregnancy, and lactation as well as in naturally vitamin D-impoverished subterranean mammals.

  13. [Frequency, diagnosis and dependance of duodenitis upon the morphological status of the gastric mucosa (author's transl)].

    Science.gov (United States)

    Konstantinidis, T; Wagner, P K

    1978-08-01

    In a attempt to prove the dependence of duodenitis upon the morphological status of the gastric mucosa, a biopsy of the corpus, antrum, and duodenal canal had been made of 354 patients. In 56 cases, a superficial duodenitis was histologically verified; however, this infection had not contaminated the Brunner' glands. A duodenitis was found to be more often associated with surface gastritis of the corpus and antrum than it was with atropic gastritis. A duodenitis was discovered by a biopsy in 16 patients possessing a normal antrum mucosa and in 25 patients having a normal corpus mucosa, thus proving the possibility of isolated duodenitis.

  14. Prevalence and Clinical, Endoscopic, and Pathological Features of Duodenitis in Children.

    Science.gov (United States)

    Alper, Arik; Hardee, Steven; Rojas-Velasquez, Danilo; Escalera, Sandra; Morotti, Raffaella A; Pashankar, Dinesh S

    2016-02-01

    Although gastritis and esophagitis are well studied in children, there is very limited literature on duodenitis in children. We aimed to assess the prevalence, etiology, clinical, endoscopic, and pathological features in a large cohort of unselected children with duodenitis. We reviewed the pathology reports of all the upper endoscopies performed at our institution during 5 years to identify children with duodenitis. Biopsy sections were reviewed to confirm the diagnosis of duodenitis. Demographic, clinical, endoscopic data, and the presence of associated gastritis and esophagitis were noted in all of the children with duodenitis. The etiology of duodenitis was correlated with the patients' clinical diagnosis. Out of 2772 children who had endoscopy, 352 had duodenitis with the prevalence rate of 12.7%. Gastritis was seen in 64% of children with duodenitis compared with 46% of children without duodenitis (P < 0.001). Common indications for endoscopy in children with duodenitis were abdominal pain, positive celiac serology, and diarrhea. The most common etiology was celiac disease (32%), followed by Crohn disease (13%), ulcerative colitis (3%), and Helicobacter pylori infection (6%). In 63% of cases, the endoscopic appearance of duodenum was normal. Cryptitis, villous changes, and cellular infiltration were noted on histology. Prevalence of duodenitis is 12.7% in children undergoing endoscopy. Celiac disease and inflammatory bowel disease are common causes of duodenitis. Associated gastritis is common in children with duodenitis, and the correlation of endoscopic appearance with histology is poor.

  15. Primary duodenal neoplasms: A retrospective clinico-pathological analysis

    Science.gov (United States)

    Bal, Amanjit; Joshi, Kusum; Vaiphei, Kim; Wig, JD

    2007-01-01

    AIM: To analyze the clinico-pathological spectrum of primary duodenal neoplasms. METHODS: A total of 55 primary duodenal neoplasms reported in the last 10 years after excluding ampullary and periampullary tumors were included in the study. Clinical details were noted and routine hematoxylin and eosin stained paraffin sections were studied for histological subtyping of the tumors. RESULTS: On histopathological examination primary duodenal neoplasms were categorized as: epithelial tumor in 27 cases (49.0%) including 10 cases of adenoma, 15 cases of adenocarcinoma, and 2 cases of Brunner gland adenoma; mesenchymal tumor in 9 cases (16.3%) consisting of 4 cases of gastrointestinal stromal tumor, 4 cases of smooth muscle tumor and I case of neurofibroma; lymphoproliferative tumor in 12 cases (21.8%), and neuroendocrine tumor in 7 cases (12.7%). CONCLUSION: Although non-ampullary/periampullary duodenal adenocarcinomas are rare, they constitute the largest group. Histopathological examination of primary duodenal tumors is important for correct histological subtyping. PMID:17373748

  16. Primary duodenal neoplasms:A retrospective clinico-pathological analysis

    Institute of Scientific and Technical Information of China (English)

    Amanjit Bal; Kusum Joshi; Kim Vaiphei; JD Wig

    2007-01-01

    AIM:To analyze the clinico-pathological spectrum of primary duodenal neoplasms.METHODS:A total of 55 primary duodenal neoplasms reported in the last 10 years after excluding ampullary and periampullary tumors were included in the study.Clinical details were noted and routine hematoxylin and eosin stained paraffin sections were studied for histological subtyping of the tumors.RESULTS:On histopathological examination primary duodenal neoplasms were categorized as:epithelial tumor in 27 cases(49.0%)including 10 cases of adenoma,15 cases of adenocarcinoma,and 2 cases of Brunner gland adenoma;mesenchymal tumor in 9 cases (16.3%)consisting of 4 cases of gastrointestinal stromal tumor,4 cases of smooth muscle tumor and I case of neurofibroma;lymphoproliferative tumor in 12 cases (21.8%),and neuroendocrine tumor in 7 cases(12.7%).CONCLUSION:Although non-ampullary/periampullary duodenal adenocarcinomas are rare,they constitute the largest group.Histopathological examination of primary duodenal tumors is important for correct histological subtyping.

  17. Omeprazole promotes proximal duodenal mucosal bicarbonate secretion in humans.

    Science.gov (United States)

    Mertz-Nielsen, A; Hillingsø, J; Bukhave, K; Rask-Madsen, J

    1996-01-01

    The proton pump inhibitor, omeprazole, surprisingly resulted in higher rates of proximal duodenal mucosal bicarbonate secretion than previously reported using an H2 receptor antagonist for gastric acid inhibition. Gastroduodenal perfusions were performed in healthy volunteers to evaluate whether this incidental finding is explained by more potent gastric acid inhibition by omeprazole or might be caused by the different mode of drug action. Basal and stimulated gastric and duodenal bicarbonate secretion rates were measured in the same subjects in control experiments (n = 17) and after pretreatment with high dose omeprazole (n = 17) and ranitidine (n = 9), respectively, by use of a technique permitting simultaneous measurements. Concentrations of bicarbonate were measured in the respective effluents by the method of back titration. Both omeprazole and ranitidine completely inhibited gastric acid secretion (pH 6.9 v 6.8; p > 0.05). Omeprazole caused higher rates of basal (mean (SEM)) (597 (48) v 351 (39) mumol/h; p 0.05) duodenal bicarbonate secretion compared with control experiments. Also the combination of omeprazole and ranitidine increased (p = 0.05) duodenal bicarbonate secretion, while ranitidine alone caused no change in either basal or stimulated secretion. In the stomach basal as well as vagally stimulated bicarbonate secretion was independent of the means of acid inhibition. These results show that the proton pump inhibitor, omeprazole, promotes proximal duodenal mucosal bicarbonate secretion apparently independent of its gastric acid inhibitory effect. The mechanism of action remains speculative.

  18. Does Ramadan Fasting Increase duodenal ulcer perforation?

    Directory of Open Access Journals (Sweden)

    Abdoulhossein Davoodabadi

    2016-03-01

    Full Text Available Introduction: In Ramadan, healthy adult Muslims are obliged to fast. Prolonged fasting increase gastric acid and pepsin levels, which promote the risk of duodenal ulcer perforation (DUP. Effects of Ramadan fasting on DUP have not been thoroughly studied yet, and the limited number of studies investigating the impact of Ramadan fasting on DUP yielded discrepant results. This study aimed to evaluate DUP frequency during Ramadan 2011-2015 and compare it with other months. Methods: This cross-sectional study was performed in 82 patients undergoing surgery due to DUP during July 2011-September 2015. The demographics, history of addiction, use of nonsteroidal and antiinflammatory drugs, previous history of acid peptic disease, as well as complications and outcomes of treatment were recorded and analyzed, and the obtained results were compared between Ramadan and other lunar months. Results: The majority of patients were male (86.6%, 71 patients, with a mean age of 43.9±16.5 years (age range: 20-75 years. Male to female ratio was 6:1. Cases with less than 30 years of age were less frequent (22%, 18 patients. DUP was more frequent during Rajab with nine cases (11%, while during Ramadan, six cases were reported, the difference between Ramadan and other months regarding the incidence of DUP was not significant (P=0.7. Risk factors such as smoking (60% and addiction (44%; especially to crystal and crack were noted. Consumption of nonsteroidal antiinflammatory drugs in 20 (24% patients, and use of antacids in 17 (25% patients. Distribution of DUP in different blood types was as follows: O+=41%, A+=28%, B+=23%, AB=5%, and O-=3%; moreover, post-operative Helicobacter pylori antibody was present in 67% of the patients. Conclusion: Ramadan fasting did not escalate DUP incidence, and those with DUP risk factors can fast with the use of antacids.

  19. An unusual case of duodenal perforation caused by a blister pack: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Si-Yuan Yao

    2015-01-01

    Conclusion: Unnoticed ingestion of blister packs can cause duodenal perforation. Although prompt management is necessary, duodenal perforation, especially in the third portion, may be potentially fatal.

  20. Sdhd and SDHD/H19 knockout mice do not develop paraganglioma or pheochromocytoma.

    Directory of Open Access Journals (Sweden)

    Jean-Pierre Bayley

    Full Text Available BACKGROUND: Mitochondrial succinate dehydrogenase (SDH is a component of both the tricarboxylic acid cycle and the electron transport chain. Mutations of SDHD, the first protein of intermediary metabolism shown to be involved in tumorigenesis, lead to the human tumors paraganglioma (PGL and pheochromocytoma (PC. SDHD is remarkable in showing an 'imprinted' tumor suppressor phenotype. Mutations of SDHD show a very high penetrance in man and we postulated that knockout of Sdhd would lead to the development of PGL/PC, probably in aged mice. METHODOLOGY/PRINCIPAL FINDINGS: We generated a conventional knockout of Sdhd in the mouse, removing the entire third exon. We also crossed this mouse with a knockout of H19, a postulated imprinted modifier gene of Sdhd tumorigenesis, to evaluate if loss of these genes together would lead to the initiation or enhancement of tumor development. Homozygous knockout of Sdhd results in embryonic lethality. No paraganglioma or other tumor development was seen in Sdhd KO mice followed for their entire lifespan, in sharp contrast to the highly penetrant phenotype in humans. Heterozygous Sdhd KO mice did not show hyperplasia of paraganglioma-related tissues such as the carotid body or of the adrenal medulla, or any genotype-related pathology, with similar body and organ weights to wildtype mice. A cohort of Sdhd/H19 KO mice developed several cases of profound cardiac hypertrophy, but showed no evidence of PGL/PC. CONCLUSIONS: Knockout of Sdhd in the mouse does not result in a disease phenotype. H19 may not be an initiator of PGL/PC tumorigenesis.

  1. Significance of CT findings and catecholamine determination in peripheral blood of asymptomatic pheochromocytoma and paraganglioma

    Energy Technology Data Exchange (ETDEWEB)

    Saginoya, Toshiyuki; Miyake, Hidetoshi; Kiyosue, Hiro; Okahara, Mika; Hori, Yuko; Hata, Hiroyuki; Mori, Hiromu [Oita Medical Univ., Hasama (Japan)

    2001-01-01

    The purpose of this study was to assess the CT findings and significance of hormone determination in the peripheral blood of asymptomatic patients with pheochromocytoma and paraganglioma. CT findings in 29 patients with surgically proven pheochromocytoma (n=19) and paraganglioma (n=10) were reviewed. Nine patients (31%) were symptomatic and 20 (69%) were asymptomatic. Tumor size ranged from 39 mm to 114 mm (mean: 60 mm) in symptomatic patients and 11 mm to 100 mm (mean: 50 mm) in asymptomatic ones. Of the 9 symptomatic patients and 18 asymptomatic patients, a homogeneous solid pattern was seen in 4 and 4, mixed pattern in 2 and 6, and massive necrotic pattern in 3 and 8 patients, respectively, on CT scans. The CT attenuation values in symptomatic cases ranged from 30 HU to 50 HU (mean: 41 HU) on precontrast CT scans and 60 HU to 111 HU (mean: 77 HU) on postcontrast CT scans, while those in asymptomatic cases ranged from 15 HU to 48 HU (mean: 33 HU) on precontrast CT scans and 66 HU to 133 HU (mean: 95 HU) on postcontrast CT scans. There were no statistically significant differences in tumor size, homogeneity, or CT attenuation values between symptomatic and asymptomatic patients. All symptomatic patients and 17 (89%) of 19 asymptomatic cases showed elevated levels of catecholamine (epinephrine) or norepinephrine in the peripheral blood. Our study showed that the CT findings in asymptomatic patients were similar to those in symptomatic patients, and 89% of asymptomatic patients showed elevation of catecholamine in the peripheral blood. Determination of catecholamine level in the peripheral blood is recommended for preoperative diagnosis in patients suspected of having asymptomatic phenochromocytoma or paraganglioma on CT scans. (author)

  2. The identification of new familial pheochromocytoma/paraganglioma genes using whole exome sequencing

    OpenAIRE

    2015-01-01

    Tesis doctoral inédita leída en la Universidad Autónoma de Madrid, Facultad de Medicina, Departamento de Bioquímica. Fecha de lectura: 30-04-2015 Los feocromocitomas (PCC) son tumores neuroendocrinos, desarrollados a partir del tejido cromafín de la médula adrenal, que suelen causar hipertensión arterial por sobre-secreción de catecolaminas. Los paragangliomas (PGL) son PCCs, en su mayoría secretores y con un gran riesgo de malignizar, que se desarrollan a partir de paraganglios l...

  3. Paraganglioma del filum terminal como causa de síndrome de cauda equina

    Directory of Open Access Journals (Sweden)

    J. Undabeitia-Huertas

    2013-08-01

    Full Text Available Los paragangliomas son tumores neuroendocrinos originados a partir de células que migran de la cresta neural. Su localización es diversa, siendo frecuentes en cabeza, cuello, mediastino o retroperitoneo. Su crecimiento en la región del filum terminal es muy poco frecuente. Presentamos el caso de una paciente que debuta con un cuadro agudo de cauda equina. Describimos en detalle el proceso diagnóstico, las características radiológicas, el tratamiento y las propiedades macro y microscópicas de este tumor.

  4. Paraganglioma of the carotid body: Report of 26 patients and review of the literature

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    Zainine Rim

    2015-03-01

    Results: The present study included 6 men and 20 women. Bilateral involvement of the carotid glomus was noted in 2 cases and tympano-jugular location was associated in 2 other cases. Ultrasound of the neck, computed tomography (CT and magnetic resonance (MR tomography were performed in 13, 17 and 10 cases, respectively. 2 patients have had preoperative embolization and 22 patients, in total, were operated. External radiation was an exclusive therapeutic option in 2 patients and adjuvant to incomplete surgery in one patient. Only one case of malignant paraganglioma was noted that evolution was, in fact, rapidly fatal.

  5. Hypertensive crisis during wide excision of gastrointestinal stromal cell tumor (GIST): Undiagnosed paraganglioma -A case report-.

    Science.gov (United States)

    Shinn, Helen Ki; Jung, Jong Kwon; Park, Jay Kim; Kim, Jong Hoon; Jung, In Young; Lee, Hong Sik

    2012-03-01

    Although paraganglioma (PGL), an extra-adrenal retroperitoneal pheochromocytoma (PHEO), is a rare catecholamine-secreting neuroendocrine tumor, it can cause severe hypertensive crisis during anesthesia or surgery if undiagnosed preoperatively. Extraluminal perigastric masses may be presumed to be gastrointestinal stromal tumors (GISTs) or soft tissue sarcomas even when histologic confirmation is not possible. Therefore, without a histologic diagnosis or symptoms of excessive catecholamine secretion, PGL may be mistaken for GIST. We report a case of preoperatively undiagnosed PGL which caused hypertensive crisis during anesthesia for retroperitoneal mass excision.

  6. Treatment of metastatic para-aortic paraganglioma by surgery, radiotherapy and I-131 mIBG

    Energy Technology Data Exchange (ETDEWEB)

    Ball, A.B.S.; Tait, D.M.; Fisher, C.; Sinnett, H.D.; Harmer, C.L. (Royal Marsden Hospital, London (United Kingdom))

    1991-10-01

    A patient with a malignant, functioning, aortico-sympathetic paraganglioma and a solitary bone metastasis causing paraplegia was treated by spinal decompression, irradiation of the metastasis, surgical excision of the primary tumour and systemic I-131 meta-iodobenzyl-guanidine (mIBG). Sixteen months after treatment there was no clinical, radiological or biochemical evidence of residual disease and neurological function was restored. The case supports the use of combined treatment incorporating mIBG in patients with metastatic neuroendocrine tumours which demonstrate mIBG uptake. (author).

  7. Duodenal adenocarcinoma: Advances in diagnosis and surgical management

    Institute of Scientific and Technical Information of China (English)

    Jordan M Cloyd; Elizabeth George; Brendan C Visser

    2016-01-01

    Duodenal adenocarcinoma is a rare but aggressive malignancy. Given its rarity, previous studies have traditionally combined duodenal adenocarcinoma(DA) with either other periampullary cancers or small bowel adenocarcinomas, limiting the available data to guide treatment decisions. Nevertheless, management primarily involves complete surgical resection when technically feasible. Surgery may require pancreaticoduodenectomy or segmental duodenal resection; either are acceptable options as long as negative margins are achievable and an adequate lymphadenectomy can be performed. Adjuvant chemotherapy and radiation are important components of multi-modality treatment for patients at high risk of recurrence. Further research would benefit from multiinstitutional trials that do not combine DA with other periampullary or small bowel malignancies. The purpose of this article is to perform a comprehensive review of DA with special focus on the surgical management and principles.

  8. Acyclovir in the prevention of duodenal ulcer recurrence

    DEFF Research Database (Denmark)

    Rune, S J; Linde, J; Bonnevie, O;

    1990-01-01

    This study tests the hypothesis that reactivation of a latent herpes simplex virus infection may be a cause of recurrent duodenal ulceration. Patients with recently healed duodenal ulcer were entered into a double blind, randomised study of maintenance treatment with the antiviral drug acyclovir...... (400 mg bid) versus placebo, to determine if suppression of herpes virus infection would influence the natural history of the ulcer disease. One hundred and fifteen patients entered the trial and 76 patients completed it according to the protocol. Endoscopy was performed when ulcer symptoms recurred...... and at the end of the 25 week trial period. In the acyclovir group the cumulated relapse rate was 63% compared with 56% in the placebo group (NS). This result suggests that reactivation of herpes simplex virus is not a cause of recurrent duodenal ulcer....

  9. Tratamiento laparoscópico de la membrana duodenal

    Directory of Open Access Journals (Sweden)

    Luis Ricardo García-Vázquez

    Full Text Available La membrana duodenal congénita se presenta con mayor frecuencia en la región ampular, y se expresa clínicamente como obstrucción intestinal durante el periodo neonatal. En algunos escenarios el diagnóstico de esta condición clínica pasa inadvertida cuando dicha membrana es incompleta, como en los casos de membrana duodenal fenestrada. En consecuencia, se modifica la forma de presentación y se dificulta su diagnóstico temprano. Se cuenta con distintos accesos terapéuticos para esta enfermedad, desde los abiertos, hasta los endoscópicos y laparoscópicos. Se presenta el caso de una lactante con diagnóstico tardío de membrana duodenal fenestrada, que se trató quirúrgicamente mediante acceso laparoscópico.

  10. A newborn with duodenal atresia and a gastric perforation

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    Akcora Bulent

    2010-01-01

    Full Text Available Congenital duodenal atresia complicated by gastric perforation (GP is a very rare and a very mortal condition. Only three newborns could be cured in the reported 13 cases. We report a successfully treated newborn with this complicated disease. A 2-day-old male was hospitalized with prediagnosis of duodenal obstruction. Twelve hours later, significant abdominal distention occurred promptly. At laparotomy, GP and preampullary duodenal atresia were detected. Gastrorrhaphy and duodenoduodenostomy were performed in the same operation. The patient was discharged on the 15th postoperative day. This complicated disease can be treated by early diagnosis and surgical intervention. We choose one-stage operation because of the clean peritoneal cavity. However, generalized peritonitis may require two-stage operation in delayed cases.

  11. Laparoscopic gastric partitioning gastrojejunostomy for an unresectable duodenal malignant tumor

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    Matsumoto Toshifumi

    2005-01-01

    Full Text Available As a palliative bypass for unresectable gastric or periampullary cancer, gastrojejunostomy (GJ is sometimes associated with postoperative delayed gastric emptying. We report the successful laparoscopic application of this procedure in a 78-year-old man with duodenal obstruction. Computed tomography revealed a mass in the duodenum along with multiple masses in the liver. A radiological image showed an ulcerative tumour in the third portion of the duodenum occluding the lumen. He was diagnosed as having an unresectable duodenal cancer with multiple liver metastases. He needed palliative bypass surgery. Laparoscopically, the stomach was partially divided using an endoscopic autosuture device, and end-to-side GJ was performed successfully. He was given a normal diet on the fourth postoperative day, and there was no delayed gastric emptying. Laparoscopic gastric partitioning GJ is a feasible and safe procedure to prevent postoperative delayed gastric emptying in case of malignant duodenal obstruction.

  12. A Rare Cause of Acute Pancreatitis: Intramural Duodenal Hematoma

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    Hemant Goyal

    2012-01-01

    Full Text Available We describe an interesting case of intramural duodenal hematoma in an otherwise healthy male who presented to emergency room with gradually progressive abdominal pain, nausea, and vomiting. This condition was missed on initial evaluation and patient was discharged from emergency room with diagnosis of acute gastritis. After 3 days, patient came back to emergency room and abdominal imaging studies were conducted which showed that patient had intramural duodenal hematoma associated with gastric outlet obstruction and pancreatitis. Hematoma was the cause of acute pancreatitis as pancreatic enzymes levels were normal at the time of first presentation, but later as the hematoma grew in size, it caused compression of pancreas and subsequent elevation of pancreatic enzymes. We experienced a case of pancreatitis which was caused by intramural duodenal hematoma. This case was missed on initial evaluation. We suggest that physicians should be more vigilant about this condition.

  13. Upper gastrointestinal barium evaluation of duodenal pathology: A pictorial review

    Institute of Scientific and Technical Information of China (English)

    Pankaj; Gupta; Uma; Debi; Saroj; Kant; Sinha; Kaushal; Kishor

    2014-01-01

    Like other parts of the gastrointestinal tract(GIT), duodenum is subject to a variety of lesions both congenital and acquired. However, unlike other parts of the GIT viz. esophagus, rest of the small intestine and large intestine, barium evaluation of duodenal lesions is technically more challenging and hence not frequently reported. With significant advances in computed tomography technology, a thorough evaluation including intraluminal, mural and extramural is feasible in a single non-invasive examination. Notwithstanding, barium evaluation still remains the initial and sometimes the only imaging study in several parts of the world. Hence,a thorough acquaintance with the morphology of various duodenal lesions on upper gastrointestinal barium examination is essential in guiding further evaluation. We reviewed our experience with various common and uncommon barium findings in duodenal abnormalities.

  14. A RETROSPECTIVE STUDY ON DUODENAL ULCER PERFORATION AND OUTCOME

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    Parameshwara Chaldiganahalli

    2016-02-01

    Full Text Available INTRODUCTION Duodenal ulcer disease which was once so common 3-4 decades ago has drastically decreased in its incidence due to invent of PPIs and anti H. Pylori therapy. But percentage of patients with complications of duodenal ulcer has not shown a similar decline. In spite of understanding the disease effective resuscitation and prompt surgery there is still High incidence of morbidity and mortality. Hence in this study an attempt is made to analyse the various factors which effect the morbidity, mortality of patients with duodenal ulcer perforation and management of the same. AIMS The objective is to study, 1. The factors responsible for duodenal ulcer perforation. 2. The factors that affect the post-operative outcome. 3. Morbidity, mortality after surgery. MATERIALS AND METHODS Fifty patient’s case sheets were selected retrospectively who were diagnosed as duodenal ulcer perforation, admitted in MIMS Hospital, Mandya. Between 2012 to 2014 patients underwent Graham's omentoplasty. All the data related to the objectives of the study were collected. RESULTS Majority of patients belong to the. Age group of 30-50 years and commonly males Most of the perforations occur in first part of duodenum low socio-economic group, O+ve blood group with maximum seasonal incidence in October-January All cases were managed by Graham's omentoplasty. Four per cent of mortality noted. CONCLUSION Duodenal ulcer perforation is one of the common acute abdominal emergencies. The peak incidence between 30 and 50 years, majority cases males, common in lower socio–economic group, unskilled workers, maximum incidence period October-January, increased morbidity and mortality when perforation time period >24 hours, maximum in patient with blood group o+, early diagnosis and septicaemia management necessary for patients better prognosis, emergency procedure is Graham’s omentoplasty (perforations<2cm with H. pylori eradication treatment. Mortality noticed in longer

  15. Delayed duodenal hematoma and pancreatitis from a seatbelt injury.

    Science.gov (United States)

    Deambrosis, Katherine; Subramanya, Manjunath S; Memon, Breda; Memon, Muhammed A

    2011-02-01

    Traumatic duodenal hematoma is a rare condition that is encountered in the paediatric age group following blunt abdominal trauma. It poses both a diagnostic and therapeutic challenge. The main concern is increased morbidity secondary to delayed diagnosis and associated occult injuries to the adjacent structures. Most of these hematomas resolve spontaneously with conservative management, and the prognosis is good. We present a case of a 15-year-old boy who had a delayed presentation of duodenal hematoma and acute pancreatitis, which was treated conservatively with complete resolution.

  16. Delayed Duodenal Hematoma and Pancreatitis from a Seatbelt Injury

    Directory of Open Access Journals (Sweden)

    DeAmbrosis, Katherine

    2011-02-01

    Full Text Available Traumatic duodenal hematoma is a rare condition that is encountered in the paediatric age group following blunt abdominal trauma. It poses both a diagnostic and therapeutic challenge. The main concern is increased morbidity secondary to delayed diagnosis and associated occult injuries to the adjacent structures. Most of these hematomas resolve spontaneously with conservative management, and the prognosis is good. We present a case of a 15-year-old boy who had a delayed presentation of duodenal hematoma and acute pancreatitis, which was treated conservatively with complete resolution. [West J Emerg Med. 2011;12(1:128-130.

  17. A Lethal Complication of Endoscopic Therapy: Duodenal Intramural Hematoma

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    Turan Calhan

    2015-01-01

    Full Text Available Duodenal intramural hematoma (DIH usually occurs in childhood and young adults following blunt abdominal trauma. It may also develop in the presence of coagulation disorders and may rarely be an iatrogenic outcome of endoscopic procedures. Management of DIH is usually a conservative approach. A case of intramural duodenal hematoma that developed following endoscopic epinephrine sclerotherapy and/or argon plasma coagulation and that was nonresponsive to conservative therapy in a patient with chronic renal failure who died from sepsis is being discussed in this report. Clinicians should be aware of such possible complications after endoscopic hemostasis in patients with coagulation disorders.

  18. Gastric outlet obstruction from duodenal lipoma in an adult

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    Promise N Wichendu

    2013-01-01

    Full Text Available The duodenum is a rare site for gastrointestinal lipoma with less than 230 cases reported in the literature. Although, peptic ulcer disease remains the most common benign cause of gastric outlet obstruction (GOO, duodenal lipomas remain a rare, but possible cause of GOO and could pose a diagnostic challenge, especially in countries where access to endoscopy and modern imaging techniques poses a challenge. The authors present a case of GOO in a 40-year-old male, secondary to a duodenal lipoma. It was successfully treated by a transduodenal resection through a midline laparotomy. The histology report confirmed it was a submucosal lipoma.

  19. Isolated perforation of a duodenal diverticulum following blunt abdominal trauma

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    Metcalfe Matthew

    2010-01-01

    Full Text Available Only 10% of duodenal diverticula are symptomatic. We present the case of a man who fell from a height of 6 ft, landing on his abdomen and presenting 4 h later with severe back pain and a rigid abdomen. At laparotomy, a perforated retroperitoneal duodenal diverticulum was found and repaired with an omental patch. No other injury was noted. Not only is this perforation unusual, but the absence of other injuries sustained during this minor blunt trauma makes this case unique. This case highlights the need for a high index of suspicion when managing patients with back or abdominal pain following minor trauma.

  20. MRI findings of intrinsic and extrinsic duodenal abnormalities and variations

    Energy Technology Data Exchange (ETDEWEB)

    Atman, Ebru Dusunceli; Erden, Ayse; Ustuner, Evren; Uzun, Caglar; Bektas, Mehmet [Ankara University School of Medicine, Ankara (Turkmenistan)

    2015-12-15

    This pictorial review aims to illustrate the magnetic resonance imaging (MRI) findings and presentation patterns of anatomical variations and various benign and malignant pathologies of the duodenum, including sphincter contraction, major papilla variation, prominent papilla, diverticulum, annular pancreas, duplication cysts, choledochocele, duodenal wall thickening secondary to acute pancreatitis, postbulbar stenosis, celiac disease, fistula, choledochoduodenostomy, external compression, polyps, Peutz-Jeghers syndrome, ampullary carcinoma and adenocarcinoma. MRI is a useful imaging tool for demonstrating duodenal pathology and its anatomic relationships with adjacent organs, which is critical for establishing correct diagnosis and planning appropriate treatment, especially for surgery.

  1. The incidence and prognosis of true duodenal carcinomas

    DEFF Research Database (Denmark)

    Buchbjerg, Thomas; Fristrup, C.; Mortensen, M. B.

    2015-01-01

    Introduction Primary duodenal carcinoma (PDC) is a rare gastrointestinal tumor. The difficult distinction between PDC and other types of carcinoma (e.g. within the periampullary region) is reflected in the scarce literature on true duodenal carcinomas. However, this distinction may be important...... the criteria for true PDC. The incidence was 5.4 per 1,000,000, and the pathological classification was: Adenocarcinoma 87%, mucinous adenocarcinoma 7%, carcinoma 4% and signet ring cell carcinoma 1%. Intended curative resection was performed in 28 patients (39%) (22 Whipple procedures and 6 local resections...

  2. Duodenal intussusception secondary to web presenting as recurrent pancreatitis in a 7-year-old girl

    Energy Technology Data Exchange (ETDEWEB)

    Tu, Long H. [University of Connecticut School of Medicine, Farmington, CT (United States); Kaweah Delta Healthcare District, Graduate Medical Education, Visalia, CA (United States); Villalona, Gustavo A. [Yale School of Medicine, Department of Surgery, Section of Pediatric Surgery, New Haven, CT (United States); SSM Health Cardinal Glennon Children' s Hospital, Department of Pediatric Surgery, St. Louis, MO (United States); Cowles, Robert A. [Yale School of Medicine, Department of Surgery, Section of Pediatric Surgery, New Haven, CT (United States); Silva, Cicero T. [Yale School of Medicine, Department of Diagnostic Imaging, Section of Pediatric Radiology, 333 Cedar St., P.O. Box 208042, New Haven, CT (United States)

    2016-03-15

    Duodenal intussusception is a rare entity in children, with 32 cases reported in the English literature to our knowledge. Most reported cases are associated with endoluminal tubes or polyps, and the presenting symptoms are chronic and nonspecific. We report a case of duodenal intussusception in a 7-year-old girl secondary to a duodenal web and review the imaging findings. (orig.)

  3. Staging and functional characterization of pheochromocytoma and paraganglioma by 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography.

    NARCIS (Netherlands)

    Timmers, H.J.L.M.; Chen, C.C.; Carrasquillo, J.A.; Whatley, M.; Ling, A.; Eisenhofer, G.; King, K.S.; Rao, J.U.; Wesley, R.A.; Adams, K.T.; Pacak, K.

    2012-01-01

    BACKGROUND: Pheochromocytomas and paragangliomas (PPGLs) are rare tumors of the adrenal medulla and extra-adrenal sympathetic chromaffin tissues; their anatomical and functional imaging are critical to guiding treatment decisions. This study aimed to compare the sensitivity and specificity of (18)F-

  4. Frequent germ-line succinate dehydrogenase subunit D gene mutations in patients with apparently sporadic parasympathetic paraganglioma

    NARCIS (Netherlands)

    H. Dannenberg (Hilde); W.N.M. Dinjens (Winand); M. Abbou; H. van Urk (Hero); B.K. Pauw; D. Mouwen; W.J. Mooi (Wolter); R.R. de Krijger (Ronald)

    2002-01-01

    textabstractPURPOSE: Recently, familial paraganglioma (PGL) was shown to be caused bymutations in the gene encoding succinate dehydrogenase subunit D (SDHD). However, the prevalence of SDHD mutations in apparently sporadic PGL is unknown. We studied the frequency and spectrum of ge

  5. Plasma chromogranin A levels are increased in a small portion of patients with hereditary head and neck paragangliomas

    NARCIS (Netherlands)

    van Duinen, Nicolette; Kema, Ido P.; Romijn, Johannes A.; Corssmit, Eleonora P. M.

    2011-01-01

    P>Context The majority of patients with head and neck paragangliomas (HNPGL) have biochemically silent tumours. Chromogranin A (CgA) is a tumour marker for neuroendocrine tumours. Objective To assess the role of CgA as a tumour marker in patients with hereditary HNPGL. Patients and Methods We includ

  6. From Bad to Worse: Paraganglioma Diagnosis during Induction of Labor for Coexisting Preeclampsia

    Directory of Open Access Journals (Sweden)

    Sasima Dusitkasem

    2017-01-01

    Full Text Available Pheochromocytomas and extra-adrenal paragangliomas are catecholamine-secreting tumors that rarely occur in pregnancy. The diagnosis of these tumors in pregnancy can be challenging given that many of the signs and symptoms are commonly attributed to preeclampsia or other more common diagnoses. Early diagnosis and appropriate management are essential in optimizing maternal and fetal outcomes. We report a rare case of a catecholamine-secreting tumor in which diagnosis occurring at the time labor was being induced for concomitant preeclampsia with severe features. Her initial presentation in hypertensive crisis with other symptoms led to diagnostic workup for secondary causes of hypertension and led to eventual diagnosis of paraganglioma. Obtaining this diagnosis prior to delivery was essential, as this led to prompt multidisciplinary care, changed the course of her clinical management, and ultimately enabled good maternal and fetal outcomes. This case highlights the importance of maintaining a high index of suspicion for secondary causes of hypertension and in obstetric patients and providing timely multidisciplinary care.

  7. An incidentally discovered asymptomatic para-aortic paraganglioma with Peutz-Jeghers syndrome

    Directory of Open Access Journals (Sweden)

    Nazish Butt

    2012-01-01

    Full Text Available Peutz-Jeghers syndrome (PJS is an autosomal dominant inherited disorder characterized by mucocutaneous melanin pigmentation and gastrointestinal (GI tract hamartomatous polyps and an increased risk of malignancy. In addition to polyposis, previous studies have reported increased risk of GI and extraGI malignancies in PJS patients, compared with that of the general population. The most common extraintestinal malignancies reported in previous studies are pancreatic, breast, ovarian and testicular cancers.We report the case of a 17-year-old boy who presented with generalized weakness, recurrent sharp abdominal pain and melena, had exploratory laparotomy and ileal resection for ileo-ileal intussusception. Pigmentation of the buccal mucosa was noted. An abdominal computed tomography scan (CT revealed multiple polyps in small bowel loops. Gastroscopy revealed multiple dimunitive polyps in stomach and pedunculated polyp in duodenum. Colonoscopy revealed multiple colonic polyps. Pathological examination of the polyps confirmed hamartomas with smooth muscle arborization, compatible with Peutz-Jeghers polyps. CT scan guided left para-aortic lymph node biopsy revealed the characteristic features of extra-adrenal para-aortic paraganglioma. Although cases of various GI and extra GI malignancies in PJS patients has been reported, the present case appears to be the first in literature in which the PJS syndrome was associated with asymptomatic extraadrenal para-aortic paraganglioma. Patients with PJS should be treated by endoscopic or surgical resection and need whole-body screening.

  8. From Bad to Worse: Paraganglioma Diagnosis during Induction of Labor for Coexisting Preeclampsia

    Science.gov (United States)

    Herndon, Blair H.; Paluzzi, Dalton; Kuhn, Joseph

    2017-01-01

    Pheochromocytomas and extra-adrenal paragangliomas are catecholamine-secreting tumors that rarely occur in pregnancy. The diagnosis of these tumors in pregnancy can be challenging given that many of the signs and symptoms are commonly attributed to preeclampsia or other more common diagnoses. Early diagnosis and appropriate management are essential in optimizing maternal and fetal outcomes. We report a rare case of a catecholamine-secreting tumor in which diagnosis occurring at the time labor was being induced for concomitant preeclampsia with severe features. Her initial presentation in hypertensive crisis with other symptoms led to diagnostic workup for secondary causes of hypertension and led to eventual diagnosis of paraganglioma. Obtaining this diagnosis prior to delivery was essential, as this led to prompt multidisciplinary care, changed the course of her clinical management, and ultimately enabled good maternal and fetal outcomes. This case highlights the importance of maintaining a high index of suspicion for secondary causes of hypertension and in obstetric patients and providing timely multidisciplinary care. PMID:28197344

  9. Novel deployment of a covered duodenal stent in open surgery to facilitate closure of a malignant duodenal perforation

    Directory of Open Access Journals (Sweden)

    Psaila Josephine

    2009-10-01

    Full Text Available Abstract Background Its a dilemma to attempt a palliative procedure to debulk the tumour and/or prevent future obstructive complications in a locally advanced intra abdominal malignancy. Case presentation A 38 year old Vietnamese man presented with a carcinoma of the colon which had invaded the gallbladder and duodenum with a sealed perforation of the second part of the duodenum. Following surgical exploration, it was evident that primary closure of the perforated duodenum was not possible due to the presence of unresectable residual tumour. Conclusion We describe a novel technique using a covered duodenal stent deployed at open surgery to aid closure of a malignant duodenal perforation.

  10. Effect of oral epidermal growth factor on mucosal healing in rats with duodenal ulcer

    Institute of Scientific and Technical Information of China (English)

    Jane CJ Chao; Kuo-Yu Liu; Sheng-Hsuan Chen; Chia-Lang Fang; Chih-Wei Tsao

    2003-01-01

    AIM: To investigate the effect of epidermal growth factor (EGF) on mucosal healing in rats with duodenal ulcer.METHODS: Male Sprague-Dawley rats were randomly divided into sham operation without EGF, sham operation with EGF, duodenal ulcer without EGF, or duodenal ulcer with EGF groups. Additionally, normal rats without operation served as the control group. Duodenal ulcer was induced in rats by 300 mL/L acetic acid. Rats with EGF were orally administered at a dose of 60 μg/kg/day in drinking water on the next day of operation (day 1). Healing of duodenal ulcer was detected by haematoxylin and eosin staining. Cell growth of damaged mucosa was determined by the contents of nucleic acids and proteins. The level of EGF in duodenal mucosa was measured by ELISA.RESULTS: The pathological results showed that duodenal ulcer rats with EGF improved mucosal healing compared with those without EGF after day 5. Duodenal ulcer rats with EGF significantly increased duodenal DNA content compared with those without EGF on day 15 (6.44±0.54mg/g VS 1.45±0.52 mg/g mucosa, P<0.05). Duodenal RNA and protein contents did not differ between duodenal ulcer rats with and without EGF during the experimental period.Sham operation and duodenal ulcer rats with EGF significantly increased duodenal mucosal EGF content compared with those without EGF on day 5 (76.0±13.7 ng/g VS 35.7±12.9ng/g mucosa in sham operation rats, and 68.3±10.9 ng/gVS 28.3±9.2 ng/g mucosa in duodenal ulcer rats, P<0.05).CONCLUSION: Oral EGF can promote mucosal healing of the rats with duodenal ulcer by stimulating mucosal proliferation accompanied by an increase in mucosal EGF content.

  11. Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status.

    NARCIS (Netherlands)

    Eisenhofer, G.; Lenders, J.W.M.; Siegert, G.; Bornstein, S.R.; Friberg, P.; Milosevic, D.; Mannelli, M.; Linehan, W.M.; Adams, K.; Timmers, H.J.L.M.; Pacak, K.

    2012-01-01

    BACKGROUND: There are currently no reliable biomarkers for malignant pheochromocytomas and paragangliomas (PPGLs). This study examined whether measurements of catecholamines and their metabolites might offer utility for this purpose. METHODS: Subjects included 365 patients with PPGLs, including 105

  12. Duodenal atresia in association with situs inversus abdominus

    Directory of Open Access Journals (Sweden)

    Raghu Shankar

    2012-01-01

    Full Text Available We report a rare association of duodenal atresia with situs inversus abdominus in a newborn. The infantogram revealed "reverse double-bubble sign" without dextrocardia. The sonography and echocardiography confirmed the diagnosis of situs inversus abdominus with multiple cardiac anomalies. Laparotomy and a duodenoduodenostomy were carried out.

  13. Gastric pneumatosis associated with duodenal stenosis and malrotation

    Energy Technology Data Exchange (ETDEWEB)

    Kawano, Shoji [Dept. of Paediatrics, Kawasaki Medical School, Okayama (Japan); Dept. of Paediatrics, Shimonoseki Municipal Hospital, Yamaguchi (Japan); Tanaka, Hiroyuki; Daimon, Yusuke; Niizuma, Takahiro; Terada, Kihei; Kataoka, Naoki [Dept. of Paediatrics, Kawasaki Medical School, Okayama (Japan); Iwamura, Yoshinobu; Aoyama, Kohji [Department of Paediatric Surgery, Kawasaki Medical School, Okayama (Japan)

    2001-09-01

    Pneumatosis intestinalis, which is linear or cystic gas within the bowel wall, is usually found in premature babies in association with necrotizing enterocolitis. Gastric pneumatosis defined as intramural gas of the stomach is a rare sign during infancy. We report an infant with Down's syndrome and a duodenal web with obvious gastric pneumatosis. (orig.)

  14. Spontaneous oesophageal rupture with duodenal atresia in a newborn infant.

    OpenAIRE

    1981-01-01

    A newborn infant with spontaneous oesophageal rupture associated with duodenal atresia is described. The diagnosis was established by the pattern of hydropneumothorax on the chest x-ray film and by the aspiration of gastric juice by thoracocentesis. Necropsy showed a tear, which extended from the oesophagogastric junction to the upper portion of the stomach.

  15. Duodenal Toxicity After Fractionated Chemoradiation for Unresectable Pancreatic Cancer

    Energy Technology Data Exchange (ETDEWEB)

    Kelly, Patrick; Das, Prajnan; Pinnix, Chelsea C.; Beddar, Sam; Briere, Tina; Pham, Mary; Krishnan, Sunil; Delclos, Marc E. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Crane, Christopher H., E-mail: ccrane@mdanderson.org [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States)

    2013-03-01

    Purpose: Improving local control is critical to improving survival and quality of life for patients with locally advanced unresectable pancreatic cancer (LAPC). However, previous attempts at radiation dose escalation have been limited by duodenal toxicity. In order to guide future studies, we analyzed the clinical and dosimetric factors associated with duodenal toxicity in patients undergoing fractionated chemoradiation for LAPC. Methods and Materials: Medical records and treatment plans of 106 patients with LAPC who were treated with chemoradiation between July 2005 and June 2010 at our institution were reviewed. All patients received neoadjuvant and concurrent chemotherapy. Seventy-eight patients were treated with conventional radiation to 50.4 Gy in 28 fractions; 28 patients received dose-escalated radiation therapy (range, 57.5-75.4 Gy in 28-39 fractions). Treatment-related toxicity was graded according to Common Terminology Criteria for Adverse Events, version 4.0. Univariate and multivariate analyses were performed to assess prognostic influence of clinical, pathologic, and treatment-related factors by using Kaplan-Meier and Cox regression methods. Results: Twenty patients had treatment-related duodenal toxicity events, such as duodenal inflammation, ulceration, and bleeding. Four patients had grade 1 events, 8 had grade 2, 6 had grade 3, 1 had grade 4, and 1 had grade 5. On univariate analysis, a toxicity grade ≥2 was associated with tumor location, low platelet count, an absolute volume (cm{sup 3}) receiving a dose of at least 55 Gy (V{sub 55} {sub Gy} > 1 cm{sup 3}), and a maximum point dose >60 Gy. Of these factors, only V{sub 55} {sub Gy} ≥1 cm{sup 3} was associated with duodenal toxicity on multivariate analysis (hazard ratio, 6.7; range, 2.0-18.8; P=.002). Conclusions: This study demonstrates that a duodenal V{sub 55} {sub Gy} >1 cm{sup 3} is an important dosimetric predictor of grade 2 or greater duodenal toxicity and establishes it as a

  16. Abnormal duodenal loop demonstrated by X-ray. Correlation to symptoms and prognosis of dyspepsia

    Energy Technology Data Exchange (ETDEWEB)

    Thommesen, P.; Funch-Jensen, P.

    1986-01-01

    The occurrence of dyspeptic symptoms has previously been correlated with the shape of the duodenal loop in patients with X-ray-negative dyspepsia. An abnormal duodenal loop was associated with a significantly higher incidence of symtoms provoked by meals, vomiting, regurgitations, heartburn, and the irritable bowel syndrome. 89% of these patients (26 patients with a normal duodenal loop and 39 patients with abnormal duodenal loop) were available for a 5-year follow-up study of symptomatic outcome. The incidence of symptoms provoked by meals was still significantly higher in patients with an abnormal duodenal loop, and there was also a significant difference concerning symptomatic outcome. Approximately 75% of the patients with a normal duodenal loop had improved, and 25% had unchanged clinical conditions. Approximately 50% of the patients with an abnormal duodenal loop had improved, and 50% had an unchanged or even deteriorated clinical condition.

  17. Endoscopic treatment of sporadic small duodenal and ampullary neuroendocrine tumors.

    Science.gov (United States)

    Gincul, Rodica; Ponchon, Thierry; Napoleon, Bertrand; Scoazec, Jean-Yves; Guillaud, Olivier; Saurin, Jean-Christophe; Ciocirlan, Mihai; Lepilliez, Vincent; Pioche, Mathieu; Lefort, Christine; Adham, Mustapha; Pialat, Jean; Chayvialle, Jean-Alain; Walter, Thomas

    2016-11-01

    Background and study aim: As duodenal neuroendocrine tumors (NETs) are rare, their optimal management has not been clearly established. The aim of this study was to evaluate the feasibility and outcome of endoscopic treatment of duodenal NETs. Patients and methods: We reviewed the files of all patients who underwent endoscopic resection of a sporadic duodenal or ampullary NET between 1996 and 2014 at two centers. Results: A total of 29 patients with 32 uT1N0M0 NETs < 20 mm were included. Treatment consisted of endoscopic mucosal resection in 19 cases, and cap aspiration in 13 cases. Prior submucosal saline injection was used in 15 cases. Mortality was 3 % (one severe bleeding). Morbidity was 38 % (11/29). At post-resection analysis, mean tumor size was 8.9 mm (range 3 - 17 mm), 29 lesions were stage pT1, one was pT2, and 2 were pTx because of piecemeal resection. All NETs were well differentiated. A total of 27 lesions were classified as grade 1 and 5 were grade 2. The resection was R0, R1, and Rx for 16, 14, and 2 lesions, respectively. Three R1 patients underwent additional surgical treatment, with no residual tumor on the surgical specimen but with positive metastatic lymph nodes in two cases. One patient was lost to follow-up. Finally, 24 patients were included in the follow-up analysis. The median follow-up period was 56 months (range 6 - 175 months). Two patients presented a tumor recurrence during the follow-up period. Conclusions: Endoscopic treatment of small duodenal NETs was associated with significant morbidity, a difficulty in obtaining an R0 specimen, and the risk of lymph node metastasis. Nevertheless, it represents an interesting alternative in small grade 1 duodenal lesions and in patients at high surgical risk.

  18. Loss of intestinal O-glycans promotes spontaneous duodenal tumors.

    Science.gov (United States)

    Gao, Nan; Bergstrom, Kirk; Fu, Jianxin; Xie, Biao; Chen, Weichang; Xia, Lijun

    2016-07-01

    Mucin-type O-glycans, primarily core 1- and core 3-derived O-glycans, are the major mucus barrier components throughout the gastrointestinal tract. Previous reports identified the biological role of O-glycans in the stomach and colon. However, the biological function of O-glycans in the small intestine remains unknown. Using mice lacking intestinal core 1- and core 3-derived O-glycans [intestinal epithelial cell C1galt1(-/-);C3GnT(-/-) or double knockout (DKO)], we found that loss of O-glycans predisposes DKO mice to spontaneous duodenal tumorigenesis by ∼1 yr of age. Tumor incidence did not increase with age; however, tumors advanced in aggressiveness by 20 mo. O-glycan deficiency was associated with reduced luminal mucus in DKO mice before tumor development. Altered intestinal epithelial homeostasis with enhanced baseline crypt proliferation characterizes these phenotypes as assayed by Ki67 staining. In addition, fluorescence in situ hybridization analysis reveals a significantly lower bacterial burden in the duodenum compared with the large intestine. This phenotype is not reduced with antibiotic treatment, implying O-glycosylation defects, rather than bacterial-induced inflammation, which causes spontaneous duodenal tumorigenesis. Moreover, inflammatory responses in DKO duodenal mucosa are mild as assayed with histology, quantitative PCR for inflammation-associated cytokines, and immunostaining for immune cells. Importantly, inducible deletion of intestinal O-glycans in adult mice leads to analogous spontaneous duodenal tumors, although with higher incidence and heightened severity compared with mice with O-glycans constitutive deletion. In conclusion, these studies reveal O-glycans within the small intestine are critical determinants of duodenal cancer risk. Future studies will provide insights into the pathogenesis in the general population and those at risk for this rare but deadly cancer.

  19. High degree of duodenal inflammation in Nigerians with functional dyspepsia

    Directory of Open Access Journals (Sweden)

    Nwokediuko SC

    2013-12-01

    Full Text Available Sylvester Chuks Nwokediuko,1 Uchenna N Ijoma,1 Olive Obienu,1 Gideon E Anigbo,1 Okechukwu Okafor21Department of Medicine, 2Department of Morbid Anatomy, University of Nigeria Teaching Hospital, Ituku/Ozalla Enugu, NigeriaBackground: Functional dyspepsia (FD is a heterogeneous disorder associated with diverse pathophysiological mechanisms, including immune activation and low-grade mucosal inflammation. Genetic factors, physiological functions, and environmental factors may determine the relative importance of various pathophysiological mechanisms. This study was designed to determine the histological alterations in the duodenal mucosa of Nigerian patients with FD.Methods: Consecutive patients with dyspepsia seen over a 27-month period in two gastrointestinal endoscopy facilities in Enugu, South-East Nigeria were further evaluated with upper gastrointestinal endoscopy and duodenal mucosal biopsies if no lesion was found in the upper gastrointestinal tract. Patients with heartburn and/or regurgitation who did not have any dyspeptic symptoms and did not have any lesion in the upper gastrointestinal tract on endoscopy were presumed to have non-erosive reflux disease (NERD and they served as controls. The control subjects also had duodenal biopsies. The histopathological findings in the cases and controls were compared.Results: There were 68 patients with FD and 52 patients with NERD. The total inflammatory score was 242 in FD and 66 in NERD (Mann-Whitney U =1168, P=0.0011. Similarly, the scores for chronic inflammation, gastric metaplasia, neutrophilic activity, eosinophilic infiltration, and Helicobacter pylori were significantly higher in FD than NERD.Conclusion: Functional dyspepsia is associated with a high degree of inflammation in the duodenal mucosa. This may reflect the high prevalence of gastrointestinal infections in a tropical environment such as Nigeria. These findings may have therapeutic potential that further studies might elucidate

  20. Gastric paraganglioma: a case report and a review of the literature.

    Science.gov (United States)

    Pruiti, Vincenzo; Mazzeo, Francesca; Rossitto, Maurizio; Foti, Agata; Macrì, Antonio; Cucinotta, Eugenio

    2014-12-29

    I paragangliomi sono tumori neuroendocrini derivati dalla cresta neurale, provenienti dai paragangli, organi neuroendocrini dispersi e caratterizzati da cellule producenti catecolamine e peptidi. Presentano un’incidenza annuale stimata in 1 / 100.000, rappresentando il 10% dei tumori secernenti catecolamine. Riportiamo il caso di un uomo di 76 anni giunto alla nostra osservazione per la presenza di una lesione polipoide di 4 cm, vegetativaulcerata, tra il corpo e dell’antro gastrico rilevata durante una gastroscopia per la comparsa di anemia. Il paziente è stato sottoposto a gastrectomia subtotale con omentectomia e anastomosi gastrodigiunale. L’esame istologico ha rivelato un carcinoma gastrico polipoide ulcerato con cellule scarsamente aderenti con infiltrazione della parete gastrica muscolare e incidentalmente è stata identificata una lesione della parete gastrica posteriore incidentale che si rilevava essre un paraganglioma con la positività alle indagini di immunocitochimica per NSE e negatività per CD117, S100, CD34 e SMA. Con il termine di feocromocitoma si indica esclusivamente i tumori derivanti dalla midollare del surrene, mentre il paraganglioma extra-surrenalico suggerisce tumori delle cellule cromaffini con altre posizioni. La localizzazione gastrica o perigastrica, come nel nostro caso, è molto rara per queste neoplasie, e in letteratura vi sono solo pochi case report. La predisposizione genetica si osserva nel 30% di questi tumori e può essere responsabile della malattia ereditaria caratterizzata da differenze nella distribuzione del tumore, nella produzione di catecolamine, nel rischio di metastasi, e nell’associazione con altri tipi di tumori. In conclusione, nei pazienti con sintomi di tumore secernente cromaffini o in pazienti con una storia familiare di feocromocitoma-paraganglioma, che portano mutazioni e sottoposti a screening per il tumore asintomatico, la prima indagine diagnostica è determinare i livelli di metanefrina

  1. Mitochondrial complex II and genomic imprinting in inheritance of paraganglioma tumors.

    Science.gov (United States)

    Baysal, Bora E

    2013-05-01

    Germ line heterozygous mutations in the structural subunit genes of mitochondrial complex II (succinate dehydrogenase; SDH) and the regulatory gene SDHAF2 predispose to paraganglioma tumors which show constitutive activation of hypoxia inducible pathways. Mutations in SDHD and SDHAF2 cause highly penetrant multifocal tumor development after a paternal transmission, whereas maternal transmission rarely, if ever, leads to tumor development. This transmission pattern is consistent with genomic imprinting. Recent molecular evidence supports a model for tissue-specific imprinted regulation of the SDHD gene by a long range epigenetic mechanism. In addition, there is evidence of SDHB mRNA editing in peripheral blood mononuclear cells and long-term balancing selection operating on the SDHA gene. Regulation of SDH subunit expression by diverse epigenetic mechanisms implicates a crucial dosage-dependent role for SDH in oxygen homeostasis. This article is part of a Special Issue entitled: Respiratory complex II: Role in cellular physiology and disease.

  2. Whole-exome sequencing identifies somatic ATRX mutations in pheochromocytomas and paragangliomas.

    Science.gov (United States)

    Fishbein, Lauren; Khare, Sanika; Wubbenhorst, Bradley; DeSloover, Daniel; D'Andrea, Kurt; Merrill, Shana; Cho, Nam Woo; Greenberg, Roger A; Else, Tobias; Montone, Kathleen; LiVolsi, Virginia; Fraker, Douglas; Daber, Robert; Cohen, Debbie L; Nathanson, Katherine L

    2015-01-21

    Pheochromocytomas and paragangliomas (PCC/PGL) are the solid tumour type most commonly associated with an inherited susceptibility syndrome. However, very little is known about the somatic genetic changes leading to tumorigenesis or malignant transformation. Here we perform whole-exome sequencing on a discovery set of 21 PCC/PGL and identify somatic ATRX mutations in two SDHB-associated tumours. Targeted sequencing of a separate validation set of 103 PCC/PGL identifies somatic ATRX mutations in 12.6% of PCC/PGL. PCC/PGL with somatic ATRX mutations are associated with alternative lengthening of telomeres and clinically aggressive behaviour. This finding suggests that loss of ATRX, an SWI/SNF chromatin remodelling protein, is important in the development of clinically aggressive PCC/PGL.

  3. [Possibilities of surgical management of paraganglioma of the neck extending to the base of the skull].

    Science.gov (United States)

    Zotikov, A E; Aleksanian, V M; Badretdinov, I A; Karmazanovskiĭ, G G; Il'in, S A; Burtseva, E A; Timina, I E; Volkov, S K

    2012-01-01

    Presented in the article is a rare clinical case report concerning successful treatment of a female patient with vaginal paraganglioma of the neck - a tumour extending from the level of the trunk of the common carotid artery in cranial direction to the base of the skull. Mentioned are difficulties of carrying out an operational intervention requiring the use of certain surgical techniques. The tumour was removed as a single block with the involvement into the process of the major vessels of the neck and nervous formations. The reconstructive operation performed was common carotid - internal carotid prosthetic repair with a 6.3-4.5 mm cone-shape prosthesis 'Gore-tex' on the right.

  4. Profiling of Somatic Mutations in Phaeochromocytoma and Paraganglioma by Targeted Next Generation Sequencing Analysis

    Directory of Open Access Journals (Sweden)

    Andrea Luchetti

    2015-01-01

    Full Text Available At least 12 genes (FH, HIF2A, MAX, NF1, RET, SDHA, SDHB, SDHC, SDHD, SDHAF2, TMEM127, and VHL have been implicated in inherited predisposition to phaeochromocytoma (PCC, paraganglioma (PGL, or head and neck paraganglioma (HNPGL and a germline mutation may be detected in more than 30% of cases. Knowledge of somatic mutations contributing to PCC/PGL/HNPGL pathogenesis has received less attention though mutations in HRAS, HIF2A, NF1, RET, and VHL have been reported. To further elucidate the role of somatic mutation in PCC/PGL/HNPGL tumourigenesis, we employed a next generation sequencing strategy to analyse “mutation hotspots” in 50 human cancer genes. Mutations were identified for HRAS (c.37G>C; p.G13R and c.182A>G; p.Q61R in 7.1% (6/85; for BRAF (c.1799T>A; p.V600E in 1.2% (1/85 of tumours; and for TP53 (c.1010G>A; p.R337H in 2.35% (2/85 of cases. Twenty-one tumours harboured mutations in inherited PCC/PGL/HNPGL genes and no HRAS, BRAF, or TP53 mutations occurred in this group. Combining our data with previous reports of HRAS mutations in PCC/PGL we find that the mean frequency of HRAS/BRAF mutations in sporadic PCC/PGL is 8.9% (24/269 and in PCC/PGL with an inherited gene mutation 0% (0/148 suggesting that HRAS/BRAF mutations and inherited PCC/PGL genes mutations might be mutually exclusive. We report the first evidence for BRAF mutations in the pathogenesis of PCC/PGL/HNPGL.

  5. Successful chemotherapy of hepatic metastases in a case of succinate dehydrogenase subunit B-related paraganglioma.

    Science.gov (United States)

    He, J; Makey, D; Fojo, T; Adams, K T; Havekes, B; Eisenhofer, G; Sullivan, P; Lai, E W; Pacak, K

    2009-10-01

    Compared to other familial pheochromocytoma/paragangliomas (PHEO/PGLs), the succinate dehydrogenase subunit B (SDHB)-related PHEO/PGLs often present with aggressive and rapidly growing metastatic lesions. Currently, there is no proven effective treatment for malignant PHEO/PGLs. Here, we present a 35-year-old white man with primary malignant abdominal extra-adrenal 11 cm paraganglioma underwent surgical successful resection. But 6 months later, he developed extensive bone, liver, and lymph nodes metastasis, which were demonstrated by computed tomography scan and the (18)F-fluorodeoxyglucose positron emission tomography. However, his (123)I-metaiodobenzylguanidine scintigraphy was negative; therefore, the cyclophosphamide, vincristine, and dacarbazine (CVD) combination chemotherapy was initiated. The combination chemotherapy was very effective showing 80% overall reduction in the liver lesions and 75% overall reduction in the retroperitoneal mass and adenopathy, and normalization of plasma catecholamine and metanephrine levels. However, plasma levels of dopamine (DA) and methoxytyramine (MTY) were only partially affected and remained consistently elevated throughout the remaining period of follow-up evaluation. Genetic testing revealed an SDHB gene mutation. Here, we present an SDHB-related PHEO/PGL patient with extensive tumor burden, numerous organ lesions, and rapidly growing tumors, which responded extremely well to CVD therapy. We conclude patients with SDHB-related PHEO/PGLs can be particularly sensitive to CVD chemotherapy and may have an excellent outcome if this therapy is used and continued on periodic basis. The data in this patient also illustrate the importance of measuring plasma levels of DA and MTY to provide a more complete and accurate assessment of the biochemical response to therapy than provided by measurements restricted to other catecholamines and O-methylated metabolites.

  6. Early Rupture of an Ultralow Duodenal Stump after Extended Surgery for Gastric Cancer with Duodenal Invasion Managed by Tube Duodenostomy and Cholangiostomy

    Directory of Open Access Journals (Sweden)

    Konstantinos Blouhos

    2013-01-01

    Full Text Available When dealing with gastric cancer with duodenal invasion, gastrectomy with distal resection of the duodenum is necessary to achieve negative distal margin. However, rupture of an ultralow duodenal stump necessitates advanced surgical skills and close postoperative observation. The present study reports a case of an early duodenal stump rupture after subtotal gastrectomy with resection of the whole first part of the duodenum, complete omentectomy, bursectomy, and D2+ lymphadenectomy performed for a pT3pN2pM1 (+ number 13 lymph nodes adenocarcinoma of the antrum. Duodenal stump rupture was managed successfully by end tube duodenostomy, without omental patching, and tube cholangiostomy. Close assessment of clinical, physical, and radiological signs, output volume, and enzyme concentration of the tube duodenostomy, T-tube, and closed suction drain, which was placed near the tube duodenostomy site to drain the leak around the catheter, dictated postoperative management of the external duodenal fistula.

  7. Symptomatic duodenal perforation by inferior vena cava filter.

    Science.gov (United States)

    Baptista Sincos, Anna Pw; Sincos, Igor R; Labropoulos, Nicos; Donegá, Bruno C; Klepacz, Andrea; Aun, Ricardo

    2017-01-01

    Objectives Duodenal perforation by an inferior vena cava filter is rare and life threatening. Our objective is to find out number of occurrences and compare diagnosis and treatments. Method The reference list of Malgor's review in 2012 was considered as well as all new articles with eligible features. Search was conducted on specific databases: MEDLINE, Web of Sciences, and Literatura Latino-Americana e do Caribe em Ciências da Saúde. Results Most of the patients presented with upper abdominal pain and the use of radiologic studies was crucial for diagnosis. The most common treatment was laparotomy with filter or strut removal plus duodenum repair. However, clinical conditions of patients must be considered and the endovascular technique with endograft deployment into inferior vena cava may be an alternative. Conclusion Duodenal perforation by an inferior vena cava filter is uncommon and in high-risk surgical patients endovascular repair must be considered.

  8. Epidermal growth factor inhibits cysteamine-induced duodenal ulcers

    DEFF Research Database (Denmark)

    Poulsen, Steen Seier

    1983-01-01

    The effect of the duodenal ulcerogen cysteamine on secretion of epidermal growth factor from Brunner's gland pouches was studied in the rat. Total output of immunoreactive epidermal growth factor was reduced to approximately 55%, compared with controls, 5 h after administration of cysteamine (300...... mg/kg, s.c.). Furthermore, measurements on tissue extracts of the pouches revealed that 5 h after cysteamine treatment, Brunner's glands were depleted of epidermal growth factor. The effect on ulcer development of intraduodenally applied exogenous epidermal growth factor (1 micrograms/kg . h) also...... factor used, when tested on chronic fistula rats, had no effect on acid secretion and did not influence bicarbonate secretion from Brunner's gland pouches. These results demonstrate that epidermal growth factor has a cytoprotective effect on the duodenal mucosa, and it is suggested that inhibition...

  9. Total Robotic Biliopancreatic Diversion with Duodenal Switch Technique.

    Science.gov (United States)

    Antanavicius, Gintaras; Mohammed, Ricardo; Van Houtte, Olivier

    2016-12-31

    The use of robotic bariatric surgery has increased significantly since its advent in 1999. Its utility and safety has been demonstrated in the literature for all bariatric procedures, including most recently the biliopancreatic diversion with duodenal switch (BPDDS). The robotic-assisted biliopancreatic diversion with duodenal switch (RBPDDS) was first described by Sudan et al. in 2007 with the use of the robot primarily for the duodeno-ileal anastomosis, then in 2015, the same authors described 59 cases of totally RBPDDS using the robot for the most of the operation, but still utilizing laparoscopic staplers. Robotic staplers were introduced recently and it became possible to utilize robot throughout the entire procedure without relying on stapling performed by an assistant. Described here is the technique of a total RBPDDS utilizing robotic staplers.

  10. An Unusual Cause of Duodenal Obstruction: Persimmon Phytobezoar.

    Science.gov (United States)

    Fan, Shengxian; Wang, Jing; Li, Yousheng

    2016-12-01

    Duodenal phytobezoar, an unusual cause of acute duodenal obstruction, is rarely seen. The most common cause of this type of bezoar is persimmon. It frequently arises from underlying gastrointestinal tract pathologies (gastric surgery, etc.). Here, we report the case of a 66-year-old man who had undergone distal gastrectomy with Billroth I reconstruction for gastric cancer and experienced severe epigastric discomfort, abdominal pain, and vomiting for a few days. The abdominal computed tomography scan showed a large-sized mass in the horizontal portion of the duodenum. On following endoscopic examination, a large phytobezoar was revealed in the duodenum. He was treated with endoscopic fragmentation combined with nasogastric Coca-Cola. The patient tolerated the procedure well and resumed a normal oral diet 3 days later.

  11. Lipase assay in duodenal juice using a conductimetric method.

    Science.gov (United States)

    Ballot, C; Favre-Bonvin, G; Wallach, J M

    1984-11-15

    Lipase activity in duodenal juice is known to undergo important variations in pathologic states, especially in cases of chronic pancreatitis. Almost all of the current assay methods are based on the measurement of hydrolysis of olive oil or triolein, mainly by potentiometry. As we have developed a conductimetric method for enzyme activity measurements, we have applied it to lipase assay. A higher experimental conductimetric sensitivity is obtained when liberated acids have a short chain (higher limiting equivalent conductivity). We have therefore used triacetin as a substrate and compared out method with potentiometry (pH-stat) and spectrophotometry. The correlation coefficients of both methods with conductimetry were 0.94 and 0.97, respectively, indicating that the conductimetric method may be used for lipase assay in duodenal juice, using triacetin as a substrate.

  12. Unusual Presentation of Bladder Paraganglioma: Comparison of (131)I MIBG SPECT/CT and (68)Ga DOTANOC PET/CT.

    Science.gov (United States)

    Jain, Tarun Kumar; Basher, Rajender Kumar; Gupta, Nitin; Shukla, Jaya; Singh, Shrawan Kumar; Mittal, Bhagwant Rai

    2016-01-01

    Extraadrenal chromaffin cell-related tumors or paragangliomas are rare, especially in the bladder, accounting for less than 1% of cases. We report a 16-year-old boy who presented with hematuria and paroxysmal headache and was found to have a prostatic growth infiltrating the urinary bladder on anatomical imaging. Iodine-131 ((131)I) metaiodobenzylguanidine (MIBG) whole-body scanning and subsequently gallium-68 ((68)Ga) DOTANOC positron emission tomography/computed tomography (PET/CT) were performed. The MIBG scan revealed a non-tracer-avid soft-tissue mass, while DOTANOC PET/CT revealed a tracer-avid primary soft-tissue mass involving the urinary bladder and prostate with metastasis to the iliac lymph nodes. He underwent surgical management; histopathology of the surgical specimen revealed a bladder paraganglioma, whereas the prostate was found to be free of tumor.

  13. A significant discrepancy of uptake between I-131 MIBG and F-18 FDG in a patient with malignant paraganglioma

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jong Su; Kim, Hyun Keun; Choi, Kyu Young; Park, Hyung Ki; Kim, Eun Sil; Kim, Yun Kwon; Kim, So Yon [National Police Hospital, Seoul (Korea, Republic of)

    2007-06-15

    A 38-year-old man who was diagnosed with malignant paraganglioma underwent computed tomography (CT) and I-131 metaiodobenzylguanidine (MIBG) scan. CT showed extensive lymph node enlargement in right iliac area and retroperitoneum with severe hydronephrosis and mass on posterior bladder wall. However, I-131 MIBG scan didn't showed abnormal uptake. He also underwent F-18 fluorodeoxyglucose (FDG) positron emission tomography/CT for localizing accurate tumor site. F-18 FDG PET/CT showed multiple metastases of left supraclavicular, hilar, mediastinal para-aortic, inguinal, right iliac lymph nodes, lung, vertebrae, and pelvis. There are a few reports showing that the F-18 FDG PET/CT is helpful for staging and localizing tumor site of patients who are diagnosed with negative on the MIBG scans. Thus, we report a case with paraganglioma which showed negative I-131 MIBG scan, but revealed multiple intense hypermetabolic foci in F-18 FDG PET/CT.

  14. Duodenal gossypiboma: a case report and literature review.

    Science.gov (United States)

    Velasco-Mata, Stephany; Díaz-Gómez, Marialy; Cova-Bianco, Tamarys; Hopp-Mora, Evelyn; Rodriguez-Rojas, Roselin; Chirinos-Malave, Yeirama; Carreiro-Rodriguez, Manuel

    2015-09-01

    Gossypiboma is a retained surgical cotton matrix material in the body after a surgical procedure. Cases are rarely reported due to medico-legal concerns. Although infrequent, it causes serious morbidity and even mortality if left undiagnosed. We present the case of a trans-mural migration of gossypiboma and a review of the literature. Gossypiboma's trans-duodenal migration is a rare complication of retained gauzes. Cases reported in the literature were easy to diagnose based on clinical grounds and endoscopic studies.

  15. An uncommon cause of gastro-duodenal ulceration

    Institute of Scientific and Technical Information of China (English)

    Sebastian Mallach; Uwe Ramp; Andreas Erhardt; Marcus Schmitt; Dieter H(a)ussinger

    2008-01-01

    Gastrointestinal ulcers occur frequently and are mainly caused by H pylori infection.In this report, we present a rare case of gastro-duodenal ulcer following selective internal radiation therapy (SIRT).SIRT is a palliative treatment for unresectable liver tumours.During SIRT,90Y-microspheres are infused into the hepatic artery.Pretreatment evaluation for the presence of arterial shunts to neighbouring organs should be determined in order to avoid complications of SIRT.

  16. Gallstone ileus of duodenum with huge duodenal stone

    OpenAIRE

    Mohammad Ehsan; Mohammad Reza Ahangaran

    2006-01-01

    Gastrointestinal obstruction by a gallstone is an uncommon but important complication of biliary stone disease which mostly affects the elderly. The classic triad of radiological features includes pneumobilia, ectopic gallstone and evidence of intestinal obstruction. Terminal ileum is the most common site of obstruction, followed by jejunum and gastric outlet. We present a case of duodenal gallstone ileus of a large, fluid-density mixed biliary stone with a peripheral rim of hyperdensity (ver...

  17. Peptic Ulcer Disease Different Pathogenesis of Duodenal and Gastric Ulcer

    OpenAIRE

    Hendra Koncoro; I Dewa Nyoman Wibawa

    2015-01-01

    Despite decrease frequency of Helicobacter pylori (H. pylori) due to eradication therapy, peptic ulcer disease as a manifestation of this infection is still remain a health burden. Understanding the physiology of gastric acid secretion and its alteration by H. pylori induced inflammation will aid physician in differentiating peptic ulcer disease based on its location. Duodenal ulcer and gastric ulcer disease are two common condition that usually found in peptic ulcer. Recognition of symptoms ...

  18. An Unusual Cause of Gastrointestinal Bleeding: Duodenal Lipoma

    Directory of Open Access Journals (Sweden)

    R. Kadaba

    2011-04-01

    Full Text Available Common causes of chronic upper gastrointestinal bleeding include oesophageal varices, gastroduodenal ulcers and malignancy, and patients mostly present with iron deficiency type anaemia. We present the case of a 60-year-old lady who presented with iron deficiency anaemia and on investigation was found to have a large duodenal polyp requiring surgical excision. On histological examination, the polyp was revealed to be a lipoma. We review the recent literature and formulate a management plan for this rare entity.

  19. Perforated duodenal ulcer: a rare complication of deferasirox in children.

    Science.gov (United States)

    Yadav, Sunil Kumar; Gupta, Vipul; El Kohly, Ashraf; Al Fadhli, Wasmi

    2013-01-01

    Duodenal ulcer perforation in pediatric age group is an uncommon entity; hence, it is not usually considered in the differential diagnosis of acute abdomen in these patients. It is important for the emergency physician to consider perforated peptic ulcer in the differential diagnosis of children presenting with acute abdominal pain, gastrointestinal bleeding, or shock. We report a 6½-year-old male child with thalassemia major who presented to emergency room with an acute abdomen and shock, who was subsequently found to have a perforated duodenal ulcer, probably related to use of oral chelating agent, deferasirox. Although, gastrointestinal symptoms like nausea, vomiting, and abdominal pain has been mentioned as infrequent adverse event in the scientific product information of deferasirox, in our current knowledge this is the first case report of perforated duodenal ulcer after oral deferasirox. The severity of this event justifies the reporting of this case. This patient had an atypical presentation in that there were no signs or symptoms of peptic ulcer disease before perforation and shock he was successfully managed with open surgery after initial resuscitation and stabilization of his general condition.

  20. Childhood chronic gastritis and duodenitis: Role of altered sensory neuromediators

    Science.gov (United States)

    Islek, Ali; Yilmaz, Aygen; Elpek, Gulsum Ozlem; Erin, Nuray

    2016-01-01

    AIM To investigate the roles of the neuropeptides vasoactive intestinal peptide (VIP), substance P (SP), and calcitonin gene-related peptide (CGRP) in chronic gastritis and duodenitis in children. METHODS Biopsy samples from the gastric and duodenal mucosa of 52 patients and 30 control subjects were obtained. Samples were taken for pathological examination, immunohistochemical staining, enzyme activity measurements and quantitative measurements of tissue peptide levels. RESULTS We observed differential effects of the disease on peptide levels, which were somewhat different from previously reported changes in chronic gastritis in adults. Specifically, SP was increased and CGRP and VIP were decreased in patients with gastritis. The changes were more prominent at sites where gastritis was severe, but significant changes were also observed in neighboring areas where gastritis was less severe. Furthermore, the degree of changes was correlated with the pathological grade of the disease. The expression of CD10, the enzyme primarily involved in SP hydrolysis, was also decreased in patients with duodenitis. CONCLUSION Based on these findings, we propose that decreased levels of VIP and CGRP and increased levels of SP contribute to pathological changes in gastric mucosa. Hence, new treatments targeting these molecules may have therapeutic and preventive effects. PMID:27729741

  1. War, traffic and iatrogenic injuries of D3 duodenal segment

    Directory of Open Access Journals (Sweden)

    Ignjatović Dragan

    2005-01-01

    Full Text Available Background. Injuries of the duodenum at the level of aortomesenteric clamp (segment D3 are with a high incidence of death due to the development of fistula and peritonitis. In three successfully managed cases, we applied the biliary surgery method. Case reports. All three cases were with the injuries of D3 duodenal segment. The first patient suffered from the blast perforation of duodenum at the level of the aortomesenteric clamp which occurred at the 7th day after the injury. The second patient suffered from the duodenal injury caused in a traffic accident. The third patient suffered from an iatrogenic injury at the beginning of D3 duodenal segment inflicted during ureterolithotomy. The described surgical procedure included basically the suture to narrow the site of the injury, then lateroterminal anastomosis with the Roux-en-Y jejunal flexure and, finally, the placement of a silicone prosthesis starting from the duodenum through the site of injury and the Roux-en-Y out. Octreotide and the total parenteral nutrition were administered to the patients postoperatively. Conclusion. The use of the releasing silicone prosthesis in all three patients provided the repair of the site of the injury with anastomosed Reux-en-Y jejunum.

  2. Childhood chronic gastritis and duodenitis: Role of altered sensory neuromediators.

    Science.gov (United States)

    Islek, Ali; Yilmaz, Aygen; Elpek, Gulsum Ozlem; Erin, Nuray

    2016-10-07

    To investigate the roles of the neuropeptides vasoactive intestinal peptide (VIP), substance P (SP), and calcitonin gene-related peptide (CGRP) in chronic gastritis and duodenitis in children. Biopsy samples from the gastric and duodenal mucosa of 52 patients and 30 control subjects were obtained. Samples were taken for pathological examination, immunohistochemical staining, enzyme activity measurements and quantitative measurements of tissue peptide levels. We observed differential effects of the disease on peptide levels, which were somewhat different from previously reported changes in chronic gastritis in adults. Specifically, SP was increased and CGRP and VIP were decreased in patients with gastritis. The changes were more prominent at sites where gastritis was severe, but significant changes were also observed in neighboring areas where gastritis was less severe. Furthermore, the degree of changes was correlated with the pathological grade of the disease. The expression of CD10, the enzyme primarily involved in SP hydrolysis, was also decreased in patients with duodenitis. Based on these findings, we propose that decreased levels of VIP and CGRP and increased levels of SP contribute to pathological changes in gastric mucosa. Hence, new treatments targeting these molecules may have therapeutic and preventive effects.

  3. Treatment of duodenal ulcer with pirenzepine and cimetidine.

    Science.gov (United States)

    Brunner, H; Dittrich, H; Kratochvil, P; Brandstätter, G; Hentschel, E; Schütze, K; Tragl, K H; Kern, H; Löffelmann, K; Zeiler, H

    1984-01-01

    The purpose of this single blind controlled multicentre trial was to compare the relative effectiveness of pirenzepine and cimetidine in healing endoscopically proven duodenal ulcers. One hundred and twenty six patients with duodenal ulcer were treated with a daily dose of 100 mg pirenzepine (50 mg each before breakfast and before the evening meal), and 128 patients were treated with 1000 mg cimetidine (200 mg with breakfast, lunch, and evening meal and 400 mg at bedtime). Endoscopy was repeated after four weeks by an endoscopist who had not been informed about the treatment. Pirenzepine showed a healing rate of 64.3%, cimetidine one of 73.4%. This difference is not statistically significant (one-sided test: chi 1(2) = 2.48). After four weeks a higher proportion of first ulcers than of recurrent lesions was healed. Pain relief was rapidly achieved with both drugs. A significant trend in favour of cimetidine may, however, not be clinically relevant considering the small difference in the absolute numbers of pain free days and nights. Adverse effects were rare and reversible. We conclude that the efficacy of pirenzepine is similar to that of cimetidine in healing duodenal ulcers. PMID:6363220

  4. Perforated duodenal ulcer: An unusual manifestation of allergic eosinophilic gastroenteritis.

    Science.gov (United States)

    Riggle, Kevin M; Wahbeh, Ghassan; Williams, Elizabeth M; Riehle, Kimberly J

    2015-11-28

    Spontaneous perforation of a duodenal ulcer secondary to allergic eosinophilic gastroenteritis (EGE) has not been previously reported. We present such a case in a teenager who presented with peritonitis. After exploration and operative repair of his ulcer, he continued to experience intermittent abdominal pain, and further evaluation revealed eosinophilic gastroenteritis in the setting of multiple food allergies. His EGE resolved after adhering to a restrictive diet. Both duodenal ulcers and EGE are very rarely seen in pediatric patients. EGE has a variable presentation depending on the layer(s) of bowel wall affected and the segment of the gastrointestinal tract that is involved. Once diagnosed, it may respond to dietary changes in patients with recognized food allergies, or to steroids in patients in whom an underlying cause is not identified. Our case highlights the need to keep EGE in the differential diagnosis when treating pediatric patients with duodenal ulcers. The epidemiology, pathophysiology, and treatment of EGE are also discussed, along with a review of the current literature.

  5. Superoxide dismutase phenotypes in duodenal ulcers: A genetic marker?

    Directory of Open Access Journals (Sweden)

    Sulekha S

    2006-01-01

    Full Text Available Background:Cu-Zn superoxide dismutases are antioxidative defensive enzymes that catalyze the reduction of superoxide anions to hydrogen peroxide. Aim:The study focuses on the association of electromorph of superoxide dismutase with duodenal ulcers, which result due to an imbalance between aggressive and defensive factors. Materials and Methods:Endoscopically confirmed 210 duodenal ulcer patients and 185 healthy individuals for comparative analysis were considered for the present study. Phenotyping of superoxide dismutase was carried out by subjecting the RBC membranes to polyacrylamide gel electrophoresis, using appropriate staining protocols. Results:Statistical analysis of SOD phenotypes revealed a significant increase of SOD AFNx012 allele and Superoxide dismutases (SOD 2-2 phenotype in duodenal ulcer group. Among these individuals, a predominance of Helicobacter pylori infection was observed. The increased preponderance of homozygotes can be explained on the basis of reduced and altered enzyme activity, which may lead to disturbance in homeostasis of antioxidant/oxidant culminating in high lipid peroxidative gastric mucosal tissue damage and ulceration. No variation in the distribution of SOD phenotypes with respect to Helicobacter pylori indicates the role of Mn-SOD rather than Cu-Zn SOD in the Helicobacter pylori infected cases as reported earlier. Conclusions:Superoxide dismutase as a genetic marker / gene modifier, encoding for an antioxidant enzyme in maintaining tissue homeostasis of the gastric mucosa is discussed.

  6. Situs inversus totalis with perforated duodenal ulcer: a case report

    Directory of Open Access Journals (Sweden)

    Khan Faiz

    2011-07-01

    Full Text Available Abstract Introduction Situs inversus is an uncommon anomaly. Situs inversus viscerum can be either total or partial. Total situs inversus, also termed as mirror image dextrocardia, is characterized by a heart on the right side of the midline while the liver and the gall bladder are on the left side. Patients are usually asymptomatic and have a normal lifespan. The exact etiology is unknown but an autosomal recessive mode of inheritance has been speculated. The first case of perforated duodenal ulcer with situs inversus was reported in 1986; here, we report the second case of this nature in the medical literature. Case presentation A 22-year-old Pakistani man presented with severe epigastric and left hypochondrial pain. Examination and investigations (chest X-ray and ultrasonography confirm peritonitis in a case of situs inversus totalis. On exploratory laparotomy, a diagnosis of situs inversus totalis with perforated duodenal ulcer was confirmed. Graham's patch closure of the duodenal ulcer was performed with absorbable sutures, and a thorough peritoneal lavage was also performed; an incidental appendectomy was also performed to avoid further diagnostic problems. Our patient had an uneventful recovery. Conclusions A diagnostic dilemma arises whenever abdominal pathology occurs in patients with situs inversus. Although an uncommon anomaly, to choose a proper surgical incision site for abdominal exploration pre-operative recognition of the condition is important.

  7. Perforated duodenal ulcer: A rare complication of deferasirox in children

    Science.gov (United States)

    Yadav, Sunil Kumar; Gupta, Vipul; El Kohly, Ashraf; Al Fadhli, Wasmi

    2013-01-01

    Duodenal ulcer perforation in pediatric age group is an uncommon entity; hence, it is not usually considered in the differential diagnosis of acute abdomen in these patients. It is important for the emergency physician to consider perforated peptic ulcer in the differential diagnosis of children presenting with acute abdominal pain, gastrointestinal bleeding, or shock. We report a 6½-year-old male child with thalassemia major who presented to emergency room with an acute abdomen and shock, who was subsequently found to have a perforated duodenal ulcer, probably related to use of oral chelating agent, deferasirox. Although, gastrointestinal symptoms like nausea, vomiting, and abdominal pain has been mentioned as infrequent adverse event in the scientific product information of deferasirox, in our current knowledge this is the first case report of perforated duodenal ulcer after oral deferasirox. The severity of this event justifies the reporting of this case. This patient had an atypical presentation in that there were no signs or symptoms of peptic ulcer disease before perforation and shock he was successfully managed with open surgery after initial resuscitation and stabilization of his general condition. PMID:23833377

  8. Perforated duodenal ulcer: A rare complication of deferasirox in children

    Directory of Open Access Journals (Sweden)

    Sunil Kumar Yadav

    2013-01-01

    Full Text Available Duodenal ulcer perforation in pediatric age group is an uncommon entity; hence, it is not usually considered in the differential diagnosis of acute abdomen in these patients. It is important for the emergency physician to consider perforated peptic ulcer in the differential diagnosis of children presenting with acute abdominal pain, gastrointestinal bleeding, or shock. We report a 6½-year-old male child with thalassemia major who presented to emergency room with an acute abdomen and shock, who was subsequently found to have a perforated duodenal ulcer, probably related to use of oral chelating agent, deferasirox. Although, gastrointestinal symptoms like nausea, vomiting, and abdominal pain has been mentioned as infrequent adverse event in the scientific product information of deferasirox, in our current knowledge this is the first case report of perforated duodenal ulcer after oral deferasirox. The severity of this event justifies the reporting of this case. This patient had an atypical presentation in that there were no signs or symptoms of peptic ulcer disease before perforation and shock he was successfully managed with open surgery after initial resuscitation and stabilization of his general condition.

  9. Novel use of F-DOPA PET/CT imaging in a child with paraganglioma/pheochromocytoma syndrome.

    Science.gov (United States)

    Levine, Daniel S; Metzger, Daniel L; Nadel, Helen R; Oviedo, Angelica; Adam, Michael J; Skarsgard, Erik

    2011-10-01

    We report the use of F-DOPA PET/CT imaging in the evaluation of a teenager with marked hypertension and right pararenal, left adrenal and left para-aortic mass lesions. The use of the modality for this clinical application has not been described previously within the pediatric imaging literature. The value of this technique relative to conventional imaging modalities is discussed and warrants consideration of its use, if available, for evaluating children with suspected paragangliomas/pheochromocytomas.

  10. Novel use of F-DOPA PET/CT imaging in a child with paraganglioma/pheochromocytoma syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Levine, Daniel S.; Nadel, Helen R. [University of British Columbia, Department of Radiology and Nuclear Medicine, British Columbia Children' s Hospital, Vancouver (Canada); Metzger, Daniel L. [University of British Columbia, Department of Pediatrics, Division of Endocrinology, British Columbia Children' s Hospital, Vancouver (Canada); Oviedo, Angelica [University of British Columbia, Department of Pathology, British Columbia Children' s Hospital, Vancouver (Canada); Adam, Michael J. [University of British Columbia, Department of PET Chemistry, Tri-University Meson Facility (TRIUMF), Vancouver (Canada); Skarsgard, Erik [University of British Columbia, Department of Surgery, British Columbia Children' s Hospital, Vancouver (Canada)

    2011-10-15

    We report the use of F-DOPA PET/CT imaging in the evaluation of a teenager with marked hypertension and right pararenal, left adrenal and left para-aortic mass lesions. The use of the modality for this clinical application has not been described previously within the pediatric imaging literature. The value of this technique relative to conventional imaging modalities is discussed and warrants consideration of its use, if available, for evaluating children with suspected paragangliomas/pheochromocytomas. (orig.)

  11. A novel mutation in the succinate dehydrogenase subunit D gene in siblings with the hereditary paraganglioma-pheochromocytoma syndrome.

    Science.gov (United States)

    Prasad, Chaithra; Oakley, Gerard J; Yip, Linwah; Coyne, Christopher; Rangaswamy, Balasubramanya; Dixit, Sanjay B

    2014-01-01

    Germline mutations in the succinate dehydrogenase complex subunit D gene are now known to be associated with hereditary paraganglioma-pheochromocytoma syndromes. Since the initial succinate dehydrogenase complex subunit D gene mutation was identified about a decade ago, more than 131 unique variants have been reported. We report the case of two siblings presenting with multiple paragangliomas and pheochromocytomas; they were both found to carry a mutation in the succinate dehydrogenase complex subunit D gene involving a substitution of thymine to guanine at nucleotide 236 in exon 3. This particular mutation of the succinate dehydrogenase complex subunit D gene has only been reported in one previous patient in Japan; this is, therefore, the first report of this pathogenic mutation in siblings and the first report of this mutation in North America. With continued screening of more individuals, we will be able to create a robust mutation database that can help us understand disease patterns associated with particular variants and may be a starting point in the development of new therapies for familial paraganglioma syndromes.

  12. Association of Duodenal Atresia, Malrotation, and Atrial Septal Defect in a Down-Syndrome Patient

    Directory of Open Access Journals (Sweden)

    R Angotti

    2016-04-01

    Full Text Available Duodenal atresia is the frequent cause of neonatal intestinal obstruction. The association between duodenal atresia, intestinal malrotation, cardiac anomalies and Down syndrome is infrequently reported. We present a prenatally suspected case of duodenal atresia which was associated with malrotation and atrial septal defect in a patient of Down syndrome. Duodenotomy and resection of web was performed in addition to Ladd’s procedure. Postoperative course remained uneventful.

  13. Ectopic Opening of the Common Bile Duct into the Duodenal Bulb: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Hwang, Seong Su; Park, Soo Youn [Catholic University St. Vincent' s Hospital, Suwon (Korea, Republic of)

    2009-08-15

    An ectopic opening of the common bile duct into the duodenal bulb is a very rare congenital malformation of the bile duct, which may cause a recurrent duodenal ulcer or biliary diseases including choledocholithiasis or cholangitis. ERCP plays major role in the diagnosis of this biliary malformation. We report a case of an ectopic opening of the common bile duct into the duodenal bulb, which was detected on the upper gastrointestinal series.

  14. Management of biliary and duodenal complications of chronic pancreatitis.

    Science.gov (United States)

    Vijungco, Joseph D; Prinz, Richard A

    2003-11-01

    Biliary stricture and duodenal obstruction have been increasingly recognized as complications of chronic pancreatitis. The anatomical relationship of the distal common bile duct and the duodenum with the head of the pancreas is the main factor for their involvement in chronic pancreatitis. In hospitalized patients with pancreatitis, the incidence of biliary stricture and duodenal obstruction is reported to be about 6% and 1.2%, respectively. For patients requiring an operation for chronic pancreatitis the incidence increases to 35% for biliary stricture and 12% for duodenal obstruction. Fibrosis around the distal common bile duct can cause stenosis with obstruction of bile flow. Clinically, the presentation of these patients ranges from being asymptomatic with elevated alkaline phosphatase or bilirubin, or both, to being septic with cholangitis. Jaundice, cholangitis, hyperbilirubinemia, and persistent elevation of serum alkaline phosphatase occur more frequently in patients with pancreatitis with a biliary stricture. A twofold elevation of alkaline phosphatase is a marker of possible common duct stenosis in patients with chronic pancreatitis. The incidence of both biliary cirrhosis and cholangitis in these patients is about 10%. ERCP reveals a characteristic long, smoothly tapered stricture of the intrapancreatic common bile duct. In duodenal obstruction, the factors that convert self-limiting edema to chronic fibrosis and stricture formation are unknown, but ischemia superimposed on inflammation may be the major cause. These patients present with a prolonged history of nausea and vomiting. Barium studies typically show a long constricting lesion of the duodenum, and endoscopy reveals reactive inflammatory changes in a narrowed duodenum. Operation is indicated in patients with common bile duct strictures secondary to chronic pancreatitis when there is evidence of cholangitis, biliary cirrhosis, common duct stones, progression of stricture, elevation of alkaline

  15. A CLINICAL STUDY ON PATIENTS WITH DUODENAL ULCER PERFORATION

    Directory of Open Access Journals (Sweden)

    Kishore Babu

    2016-03-01

    Full Text Available INTRODUCTION Perforated duodenal ulcer, the most catastrophic complication was Associated with high mortality in the past due to late presentation of the patients, delay in surgery and lack of antibiotics. Various authors state that the incidence of peptic ulcer disease and perforation has been declining for the past 3 decades. Because of advances in the medical therapy of peptic ulcer with a wide range of drugs the management of peptic ulcer disease has been changing and the role of surgery has been declining. Perforation is usually seen in 3rd and 4th decades with a male preponderance and the epidemiological trend is not the same worldwide. Incidence is slightly declining in western countries. The present study has been done during the period between 2013 and 2014 in S. V. R. R. Government general hospital Tirupati. AIMS AND OBJECTIVES The aim of the present study is to analyze the probable factors for increase in incidence of duodenal ulcer perforation, with particular emphasis on assessment of impact of H2 receptor antagonists and Proton Pump inhibitors on the incidence of perforation. STUDY SETTING S. V. Medical College, Department of General Surgery, Tirupati. STUDY PERIOD Patients attending S. V. Medical College, Department of General Surgery with perforation during the period from November 2013 to October 2014. INCLUSION CRITERIA Patients between age group of more than 14 years presenting with pain abdomen and who are diagnosed to have peritonitis due to duodenal ulcer perforation. EXCLUSION CRITERIA Patients with peritonitis due causes other than duodenal ulcer. STUDY METHOD Prospective Observational study among the selected patients. Total numbers of peptic ulcer cases that were admitted in this hospital and treated either medically or surgically were noted. The details of their clinical history and findings, investigation reports, operative findings, post-operative complications were recorded. Simple closure was performed for all the

  16. Clinical diagnosis and surgical treatment of pancreatic and/or duodenal injuries

    Institute of Scientific and Technical Information of China (English)

    Zekuan Xu; Leyao Lian; Yi Miao; Xunliang Liu

    2005-01-01

    Objective: To investigate the points of the clinical diagnosis and surgical treatment for pancreatic and/or duodenal injuries. Methods: Clinical data of 30 patients who suffered from pancreatic and/or duodenal injuries were reviewed. Results: There were 29 cases who received surgical management. Of the 30 cases, 22 cases were cured, seven cases died, and postoperative complications occurred in 16 cases. The cure rate was 73.3%. Conclusion: Pancreatic and/or duodenal injuries are severe abdominal injuries and difficult to treat. The mortality and complication rate are high. The keys to successful treatments for pancreatic and/or duodenal injuries are early diagnosis, careful exploration and proper operational management.

  17. Duodenal intraepithelial T lymphocytes in patients with functional dyspepsia

    Institute of Scientific and Technical Information of China (English)

    Gilles Gargala; Stéphana Lecleire; Arnaud Frangois; Serge Jacquot; Pierre Déchelotte; Jean Jacques Ballet; Loic Favennec; Philippe Ducrotté

    2007-01-01

    AIM:To quantify the intraepithelial lymphocytes (IELs) and to document the membrane expression of CD4,CD8,TCRγδ and adhesion and/or activation-associated molecules (CD103,CD28,CD44,CD69,HLA-DR,CD95/Fas) in the duodenal mucosa of patients with functional dyspepsia (FD) in order to provide arguments for an immunological process in FD.METHODS:Twenty-six FD patients according to Rome Ⅱ criteria (20 were H pylori negative) were studied and compared to 12 healthy adults.IELs were isolated from five duodenal biopsy samples,then quantified by microscopy and flow cytometry while the membrane phenotypes were determined by cytofluorometry.RESULTS:Duodenal histological examination was normal.In H pylori negative patients,the number of IELs was not different from that in healthy controls.Median percentage expression of CD4,CD8,or TCRγδ and CD103,CD44,CD28,CD69 on CD3+ IELs,among the adhesion/activation associated molecules tested,was not different from that in healthy controls.In contrast,the median percentage expression of CD95/Fas [22(9-65) vs 45(19-88),P=0.03] and HLADR expressing CD3+ IELs [4(0-30) vs 13(4-42),P=0.041 was signifcantly lower in the H pylori negative FD group than in healthy controls,respectively.The number of IELs was significantly greater in H pylori positive FD patients than in healthy controls [median ratio for 100 enterocytes 27.5 (6.7-62.5) vs 10.8 (3-33.3), P = 0.02] due to a higher number of CD8+ CD3+ IELs.CONCLUSION: In H pylori negative FD patients, the phenotypic characterization of IELs suggests that we cannot exclude a role of IELs in FD.

  18. Microcirculatory remodeling in marginal zone of duodenal ulcer after bleeding

    Directory of Open Access Journals (Sweden)

    Sulayeva О.N.

    2009-01-01

    Full Text Available To estimate objectively vessels network remodeling in duodenal mucosa after ulcer bleeding the morphometric analysis of marginal ulcer zone biopsies was performed in 32 patients. It was shown that reparation is accompanied with chronic inflammation and acute alteration of microcirculation. Injection hemostasis led to enhancement of microcirculation, development of edema and ischemic alteration of mucosal tissues. Acute neutrophilic infiltration during 1 day was changed on 3 day with granular tissue development and angiogenesis stimulation. Intensification and prolongation of angiogenesis paral-leled with lymphocytes infiltration after 7 days resulted to villi dysmorphogenesis and changes in cellular content of intestinal epithelium.

  19. Intraoperative methods to stage and localize pancreatic and duodenal tumors.

    Science.gov (United States)

    Norton, J A

    1999-01-01

    Intraoperative methods to stage and localize tumors have dramatically improved. Advances include less invasive methods to obtain comparable results and precise localization of previously occult tumors. The use of new technology including laparoscopy and ultrasound has provided some of these advances, while improved operative techniques have provided others. Laparoscopy with ultrasound has allowed for improved staging of patients with pancreatic cancer and exclusion of patients who are not resectable for cure. We performed laparoscopy with ultrasound on 50 consecutive patients with adenocarcinoma of the pancreas or liver who appeared to have resectable tumors based on preoperative computed tomography. 22 patients (44%) were found to be unresectable because of tumor nodules on the liver and/or peritoneal surfaces or unsuspected distant nodal or liver metastases. The site of disease making the patient unresectable was confirmed by biopsy in each case. Of the 28 remaining patients in whom laparoscopic ultrasound predicted to be resectable for cure, 26 (93%) had all tumor removed. Thus laparoscopy with ultrasound was the best method to select patients for curative surgery. Intraoperative ultrasound (IOUS) has been a critical method to identify insulinomas that are not palpable. Nonpalpable tumors are most commonly in the pancreatic head. Because the pancreatic head is thick and insulinomas are small, of 9 pancreatic head insulinomas only 3 (33%) were palpable. However, IOUS precisely identified each (100%). Others have recommended blind distal pancreatectomy for individuals with insulinoma in whom no tumor can be identified. However, our data suggest that this procedure is contraindicated as these occult tumors are usually within the pancreatic head. Recent series suggest that previously missed gastrinomas are commonly in the duodenum. IOUS is not able to identify these tumors, but other methods can. Of 27 patients with 31 duodenal gastrinomas, palpation identified 19

  20. Peptic Ulcer Disease Different Pathogenesis of Duodenal and Gastric Ulcer

    Directory of Open Access Journals (Sweden)

    Hendra Koncoro

    2015-12-01

    Full Text Available Despite decrease frequency of Helicobacter pylori (H. pylori due to eradication therapy, peptic ulcer disease as a manifestation of this infection is still remain a health burden. Understanding the physiology of gastric acid secretion and its alteration by H. pylori induced inflammation will aid physician in differentiating peptic ulcer disease based on its location. Duodenal ulcer and gastric ulcer disease are two common condition that usually found in peptic ulcer. Recognition of symptoms and its pathogenesis may lead physician to understand the fate of each condition in the future. This article reviews concept of peptic ulcer pathogenesis according to ulcer etiology.

  1. Gallstone ileus of duodenum with huge duodenal stone

    Directory of Open Access Journals (Sweden)

    Mohammad Ehsan

    2006-12-01

    Full Text Available Gastrointestinal obstruction by a gallstone is an uncommon but important complication of biliary stone disease which mostly affects the elderly. The classic triad of radiological features includes pneumobilia, ectopic gallstone and evidence of intestinal obstruction. Terminal ileum is the most common site of obstruction, followed by jejunum and gastric outlet. We present a case of duodenal gallstone ileus of a large, fluid-density mixed biliary stone with a peripheral rim of hyperdensity (very fine calcification in CT scan. KEY WORDS: Gallstone ileus, duodenum, intestinal obstruction.

  2. SUPERIOR MESENTERIC ARTERY SYNDROME - AN UNUSUAL CAUSE OF DUODENAL OBSTRUCTION

    OpenAIRE

    Sahu SK, , , , , , ,; Singh PK; Ray J; Uniyal M; Sharma C; Sekhar C; Kapruwan H; Sachan PK

    2012-01-01

    Superior mesenteric artery (SMA) arising from aorta at the level of first lumbar vertebra usually takes an angular downward course from ventral surface of aorta. It is through this vascular angle that the 3rd part of duodenum passes at the level of 4th lumbar vertebra. Fat and lymphatics around SMA maintains the angle at 25° to 60° with a mean of 450 and provide protection against duodenal compression. In Superior Mesenteric Artery Syndrome, the SMA-aorta angle in narrowed down to 7° to 22° w...

  3. MULTIPLE ASSOCIATED ANOMALIES IN PATIENTS OF DUODENAL ATRESIA: A CASE SERIES

    Directory of Open Access Journals (Sweden)

    Bilal Mirza

    2012-04-01

    Full Text Available Duodenal atresia has been reported in association with various malformations and syndromes common being Down syndrome, malrotation, and annular pancreas. Its association with multiple anomalies is rare and scarcely reported in literature. Herein 3 cases of duodenal atresia associated with multiple congenital anomalies are being reported.

  4. Upper gastrointestinal bleeding from duodenal vascular ectasia in a patient with cirrhosis

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    We report a cirrhotic patient with duodenal vascular ectasia and spontaneous bleeding. The bleeding was successfully controlled with argon plasma coagulation.Duodenal vascular ectasia may be a cause of upper gastrointestinal bleeding in patients with cirrhosis, and argon plasma coagulation may be effective and safe to achieve hemostasis of this lesion.

  5. Chromosomal and methylation alterations in sporadic and familial adenomatous polyposis-related duodenal carcinomas.

    NARCIS (Netherlands)

    Berkhout, M.; Nagtegaal, I.D.; Cornelissen, S.J.B.; Dekkers, M.M.G.; Molengraft, F.J. van de; Peters, W.H.M.; Nagengast, F.M.; Krieken, J.H.J.M. van; Jeuken, J.W.M.

    2007-01-01

    Primary carcinomas of the small intestine are rare and the mechanism of their pathogenesis is poorly understood. Patients with familial adenomatous polyposis (FAP) have a high risk of developing duodenal carcinomas. The aim of this study is to gain more insight into the development of duodenal carci

  6. [The prognostic value of gastric metaplasia in the duodenal mucosa in patients with Helicobacter pylori positive duodenal bulb ulcer].

    Science.gov (United States)

    Marshalko, O V; Konorev, M R

    2008-01-01

    The predictive value of gastric metaplasia in the duodenal mucosa in patients Helicobacter pylori-positive patients with duodenal bulb ulcer (DBU) was investigated. One hundred and twenty four randomly selected patients with DBU were included in this prospective study. The detection of Helicobacter pylori (HP) in the stomach and duodenum was carried out with Giemsa (using standard visual analogue scale), rapid urease test (standard Jatrox-HP test, Rohm Pharma, Germany), and polymerase chain reaction (PCR) to detect the specific fragment of ureC HP gene (Helicopol II, Lytech, Russia). Regions of gastric metaplasia of the duodenum were confirmed by periodic acid-Schiff and alcian blue (Serva) staining (pH 1.0; 2.5) Duodenal ulcer (DU) complications were registered within 8 to 10 years. Estimation of the predictive factor (gastric metaplasia in the duodenum) was carried out in patients with non-complicated DU (Group 1; n = 73), and with such complications as bleeding, perforation, penetration, pyloroduodenal stenosis (Group 2; n = 51) which were revealed within the 8 to 10 years of observation. Gastric metaplasia in the duodenum was found in 64 or 87.7% of the 73 patients with non-complicated DU and in 5 or 9.8% of the 51 patients with complicated DU within 8 to 10 years of observation. The following facts about the predictive factor for the prognosis of DU complication were found: the sensitivity of 83.6%, the specificity of 92.8%, the predictive accuracy of 88.7%, the relative risk of the predicted outcome of 7.5, the relative risk of a different outcome of 0.11, the odds ration of 65.4. The study revealed a high and significant (p gastric metaplasia in the duodenum as a marker of non-complicated clinical course of DU in HP-positive patients within an 8 to 10-year period.

  7. Paraganglioma, neuroblastoma, and a SDHB mutation: Resolution of a 30-year-old mystery.

    Science.gov (United States)

    Schimke, R Neil; Collins, Debra L; Stolle, Catherine A

    2010-06-01

    Familial paraganglioma/pheochromocytoma (PGL/PCC) is genetically heterogenous with mutations in three of the four subunits of the heterotetrameric mitochondrial complex II enzyme succinate dehydrogenase (SDH) being causally responsible for the majority of cases. In addition to PGL/PCC an array of non-paraganglial tumors have been described in affected individuals. We present a 30-year follow-up on the family of a deceased patient who synchronously developed malignant neuroblastoma (NBL), PCC, and renal cell carcinoma (RCC). Other family members with late onset disease have come to our attention, and molecular study revealed a mutation in the SDHB gene. Despite the embryologic relationship, NBL has been seen in only two previous patients with familial PGL/PCC, both with deletions of the SDHB gene. Review of the literature suggests the lack of a reported association between NBL and familial PGL/PCC may be an ascertainment bias. We further suggest that study of the SDH genes in NBL survivors who develop secondary solid tumors, particularly RCC, may correct this bias, and provide for more effective and comprehensive tumor screening in this patient population.

  8. Molecular and therapeutic advances in the diagnosis and management of malignant pheochromocytomas and paragangliomas.

    LENUS (Irish Health Repository)

    Lowery, Aoife J

    2013-01-01

    Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare catecholamine-secreting tumors derived from chromaffin cells originating in the neural crest. These tumors represent a significant diagnostic and therapeutic challenge because the diagnosis of malignancy is frequently made in retrospect by the development of metastatic or recurrent disease. Complete surgical resection offers the only potential for cure; however, recurrence can occur even after apparently successful resection of the primary tumor. The prognosis for malignant disease is poor because traditional treatment modalities have been limited. The last decade has witnessed exciting discoveries in the study of PCCs and PGLs; advances in molecular genetics have uncovered hereditary and germline mutations of at least 10 genes that contribute to the development of these tumors, and increasing knowledge of genotype-phenotype interactions has facilitated more accurate determination of malignant potential. Elucidating the molecular mechanisms responsible for malignant transformation in these tumors has opened avenues of investigation into targeted therapeutics that show promising results. There have also been significant advances in functional and radiological imaging and in the surgical approach to adrenalectomy, which remains the mainstay of treatment for PCC. In this review, we discuss the currently available diagnostic and therapeutic options for patients with malignant PCCs and PGLs and detail the molecular rationale and clinical evidence for novel and emerging diagnostic and therapeutic strategies.

  9. Succinate dehydrogenase gene mutations are strongly associated with paraganglioma of the organ of Zuckerkandl.

    Science.gov (United States)

    Lodish, Maya B; Adams, Karen T; Huynh, Thanh T; Prodanov, Tamara; Ling, Alex; Chen, Clara; Shusterman, Suzanne; Jimenez, Camilo; Merino, Maria; Hughes, Marybeth; Cradic, Kendall W; Milosevic, Dragana; Singh, Ravinder J; Stratakis, Constantine A; Pacak, Karel

    2010-09-01

    Organ of Zuckerkandl paragangliomas (PGLs) are rare neuroendocrine tumors that are derived from chromaffin cells located around the origin of the inferior mesenteric artery extending to the level of the aortic bifurcation. Mutations in the genes encoding succinate dehydrogenase subunits (SDH) B, C, and D (SDHx) have been associated with PGLs, but their contribution to PGLs of the organ of Zuckerkandl PGLs is not known. We aimed to describe the clinical presentation of patients with PGLs of the organ of Zuckerkandl and investigate the prevalence of SDHx mutations and other genetic defects among them. The clinical characteristics of 14 patients with PGL of the organ of Zuckerkandl were analyzed retrospectively; their DNA was tested for SDHx mutations and deletions. Eleven out of 14 (79%) patients with PGLs of the organ of Zuckerkandl were found to have mutations in the SDHB (9) or SDHD (2) genes; one patient was found to have the Carney-Stratakis syndrome (CSS), and his PGL was discovered during surgery for gastrointestinal stromal tumor. Our results show that SDHx mutations are prevalent in pediatric and adult PGLs of the organ of Zuckerkandl. Patients with PGLs of the organ of Zuckerkandl should be screened for SDHx mutations and the CSS; in addition, asymptomatic carriers of an SDHx mutation among the relatives of affected patients may benefit from tumor screening for early PGL detection.

  10. Coexistence of paraganglioma/pheochromocytoma and papillary thyroid carcinoma: a four-case series analysis.

    Science.gov (United States)

    Bugalho, Maria João; Silva, Ana Luísa; Domingues, Rita

    2015-12-01

    The paraganglioma (PGL)/pheochromocytoma (PHEO)-papillary thyroid carcinoma (PTC) dyad has been reported rarely. Whether the association is coincidental or results from an underlying genetic predisposition is difficult to ascertain. We analyzed clinical and molecular data on four unrelated patients identified and treated by one of us (MJB) at a tertiary center. Patients were screened for germline variants in a panel of candidate genes: RET, VHL, SDHB, SDHC, SDHD, SDHAF2, TMEM127, MAX, PTEN, CDKN1B. All patients were female; median age at diagnosis of PGL/PHEO was 45 years and at diagnosis of PTC was 49.5 years. Only one patient had family history of thyroid cancer. PTC was multifocal in 2 cases, of the classical type in 2 cases and of the follicular type in 2 cases. Two patients harbored heterozygous germline variants of uncertain significance in the SDHB gene: Ser163Pro and Ala3Gly. The -79T>C polymorphism in the CDKN1B gene was present in all patients (3 in homozygous and 1 in heterozygous state). Results deriving from a comprehensive analysis of a panel of genes suggest that there is no single explanation for the association PGL/PHEO-PTC. It may occur through different mechanisms such as the combinatorial effect of different genetic variants, be a coincidental association or, alternatively, result from genetic variants in genes still awaiting identification.

  11. Additional value of hybrid SPECT/CT systems in neuroendocrine tumors, adrenal tumors, pheochromocytomas and paragangliomas.

    Science.gov (United States)

    Wong, K K; Chondrogiannis, S; Fuster, D; Ruiz, C; Marzola, M C; Giammarile, F; Colletti, P M; Rubello, D

    The aim of this review was to evaluate the potential advantages of SPECT/CT hybrid imaging in the management of neuroendocrine tumors, adrenal tumors, pheochromocytomas and paragangliomas. From the collected data, the superiority of fused images was observed as providing both functional/molecular and morphological imaging compared to planar imaging. This provided an improvement in diagnostic imaging, with significant advantages as regards: (1) precise locating of the lesions; (2) an improvement in characterization of the findings, resulting higher specificity, improved sensitivity, and overall greater accuracy, (3) additional anatomical information derived from the CT component; (4) CT-based attenuation correction and potential for volumetric dosimetry calculations, and (5) improvement on the impact on patient management (e.g. in better defining treatment plans, in shortening surgical operating times). It can be concluded that SPECT/CT hybrid imaging provides the nuclear medicine physician with a powerful imaging modality in comparison to planar imaging, providing essential information about the location of lesions, and high quality homogeneous images. Copyright © 2016 Elsevier España, S.L.U. y SEMNIM. All rights reserved.

  12. High-throughput screening for growth inhibitors using a yeast model of familial paraganglioma.

    Science.gov (United States)

    Bancos, Irina; Bida, John Paul; Tian, Defeng; Bundrick, Mary; John, Kristen; Holte, Molly Nelson; Her, Yeng F; Evans, Debra; Saenz, Dyana T; Poeschla, Eric M; Hook, Derek; Georg, Gunda; Maher, L James

    2013-01-01

    Classical tumor suppressor genes block neoplasia by regulating cell growth and death. A remarkable puzzle is therefore presented by familial paraganglioma (PGL), a neuroendocrine cancer where the tumor suppressor genes encode subunits of succinate dehydrogenase (SDH), an enzyme of the tricarboxylic acid (TCA) cycle of central metabolism. Loss of SDH initiates PGL through mechanisms that remain unclear. Could this metabolic defect provide a novel opportunity for chemotherapy of PGL? We report the results of high throughput screening to identify compounds differentially toxic to SDH mutant cells using a powerful S. cerevisiae (yeast) model of PGL. Screening more than 200,000 compounds identifies 12 compounds that are differentially toxic to SDH-mutant yeast. Interestingly, two of the agents, dequalinium and tetraethylthiuram disulfide (disulfiram), are anti-malarials with the latter reported to be a glycolysis inhibitor. We show that four of the additional hits are potent inhibitors of yeast alcohol dehydrogenase. Because alcohol dehydrogenase regenerates NAD(+) in glycolytic cells that lack TCA cycle function, this result raises the possibility that lactate dehydrogenase, which plays the equivalent role in human cells, might be a target of interest for PGL therapy. We confirm that human cells deficient in SDH are differentially sensitive to a lactate dehydrogenase inhibitor.

  13. Molecular and Therapeutic Advances in the Diagnosis and Management of Malignant Pheochromocytomas and Paragangliomas

    Science.gov (United States)

    Lowery, Aoife J.; Walsh, Siun; McDermott, Enda W.

    2013-01-01

    Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare catecholamine-secreting tumors derived from chromaffin cells originating in the neural crest. These tumors represent a significant diagnostic and therapeutic challenge because the diagnosis of malignancy is frequently made in retrospect by the development of metastatic or recurrent disease. Complete surgical resection offers the only potential for cure; however, recurrence can occur even after apparently successful resection of the primary tumor. The prognosis for malignant disease is poor because traditional treatment modalities have been limited. The last decade has witnessed exciting discoveries in the study of PCCs and PGLs; advances in molecular genetics have uncovered hereditary and germline mutations of at least 10 genes that contribute to the development of these tumors, and increasing knowledge of genotype-phenotype interactions has facilitated more accurate determination of malignant potential. Elucidating the molecular mechanisms responsible for malignant transformation in these tumors has opened avenues of investigation into targeted therapeutics that show promising results. There have also been significant advances in functional and radiological imaging and in the surgical approach to adrenalectomy, which remains the mainstay of treatment for PCC. In this review, we discuss the currently available diagnostic and therapeutic options for patients with malignant PCCs and PGLs and detail the molecular rationale and clinical evidence for novel and emerging diagnostic and therapeutic strategies. PMID:23576482

  14. Endoscopic Management of a Primary Duodenal Carcinoid Tumor

    Directory of Open Access Journals (Sweden)

    Albin Abraham

    2012-03-01

    Full Text Available Carcinoids are rare, slow-growing tumors originating from a variety of different neuroendocrine cell types. They are identified histologically by their affinity for silver salts and by positive reactions to neuroendocrine markers such as neuron-specific enolase, synaptophysin and chromogranin. They can present with various clinical symptoms and are difficult to diagnose. We present the case of a 43-year-old woman who was referred for evaluation of anemia. Upper endoscopy showed a duodenal bulb mass around 1 cm in size. Histopathological and immunohistochemistry staining were consistent with the diagnosis of a carcinoid tumor. Further imaging and endoscopic studies showed no other synchronous carcinoid lesions. Endoscopic ultrasound (EUS revealed a 1 cm lesion confined to the mucosa and no local lymphadenopathy. Successful endoscopic mucosal resection of the mass was performed. Follow-up surveillance 6 months later with EUS and Octreoscan revealed no new lesions suggestive of recurrence. No consensus guidelines exist for the endoscopic management of duodenal carcinoid tumors. However, endoscopic resection is safe and preferred for tumors measuring 1 cm or less with no evidence of invasion of the muscularis layer.

  15. Further experience with epigastric pain reproduction test in duodenal ulceration.

    Science.gov (United States)

    Earlam, R J

    1972-06-17

    Further evidence is presented that the epigastric pain of duodenal ulceration, situated between the rib margins and just below the xiphisternum, arises from the lower oesophagus.One-hundred patients with duodenal ulceration were divided into those with epigastric pain (61) and those with pain in the upper abdomen but not in the epigastrium (39). Perfusion of 0.1 N HCl into the lower oesophagus reproduced epigastric pain in 53 of the 61 with epigastric pain (mean 37 ml) but in none of the 39 without (mean 125 ml). All those who had been woken by epigastric pain at night in the previous four weeks had a positive test.In five the test remained positive even though the acid was neutralized by a continuous perfusion of alkali just below the gastro-oesophageal junction. In another five 200 ml 0.1 N HCl instilled into the stomach for 21 minutes did not reproduce epigastric pain, even though 30 ml perfused for three minutes into the lower oesophagus did.

  16. Duodenal pseudomelanosis (pseudomelanosis duodeni: a rare endoscopic finding

    Directory of Open Access Journals (Sweden)

    Aloísio Felipe-Silva

    2011-12-01

    Full Text Available Duodenal pseudomelanosis (or pseudomelanosis duodeni is a rare benigncondition characterized by black-brown speckled pigmentation of the duodenalmucosa. Collections of pigment−laden macrophages are found in the tips ofduodenal villi. The pigment is thought to be mostly composed of ferrous sulfide.Histochemichal stains for iron (Perl’s prussian blue or melanin (Masson-Fontana may be positive, but are usually negative or unpredictable. Duodenalpseudomelanosis occurs predominantly in middle-aged to old adults andmore commonly in females. It is associated with chronic renal failure, arterialhypertension, diabetes mellitus and gastrointestinal bleeding. Medications suchas ferrous sulfate, hydralazine, propranolol, hydrochlorothiazide and furosemideare thought to play a role as well. We report a case of a 86-year-old femalewho presented with a history of watery diarrhea and melena. The patient had ahistory of high blood pressure and ischemic stroke episodes. She was on multiplemedication including hidralazine, captopril, hydrochlorthiazide and aspirin. She wasdehydrated, her blood pressure was 96 × 60 mmHg and neurologic examinationshowed complete left hemiplegia with central VII nerve palsy. Laboratory testsshowed normal serum electrolytes and renal function. Hemoglobin level was10.7 g%. An upper endoscopy showed multiple diminutive black spots throughoutthe distal duodenal bulb and second portion. Histology showed multiple foci ofa brown-black granular pigment inside macrophages within the tips of the villi(pseudomelanosis. Stains for iron and melanin were negative. She was treatedwith omeprazol, parenteral fluid replacement with saline and partial fasting. Aftercomplete recovery she was discharged for ambulatory follow up.

  17. Roxatidine acetate in the long term maintenance of duodenal ulcers.

    Science.gov (United States)

    Brunner, G

    1988-01-01

    A non-comparative multicentre study of 105 patients with healed duodenal ulcers was conducted to determine the effect on ulcer recurrence of 6 months' maintenance treatment with roxatidine acetate 75 mg daily. All patients had previously received roxatidine acetate treatment. 31 patients out of 89 had 32 relapsed ulcers after 6 months of treatment, which represents an overall relapse rate of around 30%; the relapse rate in smokers was double that of non-smokers. The overall incidence of epigastric pain did not increase significantly over the period of the trial, although some patients complained of mild pain when they entered the study despite having endoscopically confirmed healed ulcers. At the end of the study continuous poor appetite and pyrosis were reported by 17% and 6% of patients, respectively. Side effects, which included constipation and diarrhoea, were reported by 4 patients, 1 of whom withdrew from therapy. There were no clinically significant changes in laboratory values. Thus, maintenance treatment with roxatidine acetate 75 mg daily proved a safe and effective method of preventing symptomatic duodenal ulcer relapse.

  18. Silent genetic alterations identified by targeted next-generation sequencing in pheochromocytoma/paraganglioma: A clinicopathological correlations.

    Science.gov (United States)

    Pillai, Suja; Gopalan, Vinod; Lo, Chung Y; Liew, Victor; Smith, Robert A; Lam, Alfred King Y

    2017-02-01

    The goal of this pilot study was to develop a customized, cost-effective amplicon panel (Ampliseq) for target sequencing in a cohort of patients with sporadic phaeochromocytoma/paraganglioma. Phaeochromocytoma/paragangliomas from 25 patients were analysed by targeted next-generation sequencing approach using an Ion Torrent PGM instrument. Primers for 15 target genes (NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2, TMEM127, MAX, MEN1, KIF1Bβ, EPAS1, CDKN2 & PHD2) were designed using ion ampliseq designer. Ion Reporter software and Ingenuity® Variant Analysis™ software (www.ingenuity.com/variants) from Ingenuity Systems were used to analysis these results. Overall, 713 variants were identified. The variants identified from the Ion Reporter ranged from 64 to 161 per patient. Single nucleotide variants (SNV) were the most common. Further annotation with the help of Ingenuity variant analysis revealed 29 of these 713variants were deletions. Of these, six variants were non-pathogenic and four were likely to be pathogenic. The remaining 19 variants were of uncertain significance. The most frequently altered gene in the cohort was KIF1B followed by NF1. Novel KIF1B pathogenic variant c.3375+1G>A was identified. The mutation was noted in a patient with clinically confirmed neurofibromatosis. Chromosome 1 showed the presence of maximum number of variants. Use of targeted next-generation sequencing is a sensitive method for the detecting genetic changes in patients with phaeochromocytoma/paraganglioma. The precise detection of these genetic changes helps in understanding the pathogenesis of these tumours. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. Targeted Exome Sequencing of Krebs Cycle Genes Reveals Candidate Cancer-Predisposing Mutations in Pheochromocytomas and Paragangliomas.

    Science.gov (United States)

    Remacha, Laura; Comino-Méndez, Iñaki; Richter, Susan; Contreras, Laura; Currás-Freixes, María; Pita, Guillermo; Letón, Rocío; Galarreta, Antonio; Torres-Pérez, Rafael; Honrado, Emiliano; Jiménez, Scherezade; Maestre, Lorena; Moran, Sebastian; Esteller, Manel; Satrústegui, Jorgina; Eisenhofer, Graeme; Robledo, Mercedes; Cascón, Alberto

    2017-07-18

    Purpose: Mutations in Krebs cycle genes are frequently found in patients with pheochromocytomas/paragangliomas. Disruption of SDH, FH or MDH2 enzymatic activities lead to accumulation of specific metabolites, which give rise to epigenetic changes in the genome that cause a characteristic hypermethylated phenotype. Tumors showing this phenotype, but no alterations in the known predisposing genes, could harbor mutations in other Krebs cycle genes.Experimental Design: We used downregulation and methylation of RBP1, as a marker of a hypermethylation phenotype, to select eleven pheochromocytomas and paragangliomas for targeted exome sequencing of a panel of Krebs cycle-related genes. Methylation profiling, metabolite assessment and additional analyses were also performed in selected cases.Results: One of the 11 tumors was found to carry a known cancer-predisposing somatic mutation in IDH1. A variant in GOT2, c.357A>T, found in a patient with multiple tumors, was associated with higher tumor mRNA and protein expression levels, increased GOT2 enzymatic activity in lymphoblastic cells, and altered metabolite ratios both in tumors and in GOT2 knockdown HeLa cells transfected with the variant. Array methylation-based analysis uncovered a somatic epigenetic mutation in SDHC in a patient with multiple pheochromocytomas and a gastrointestinal stromal tumor. Finally, a truncating germline IDH3B mutation was found in a patient with a single paraganglioma showing an altered α-ketoglutarate/isocitrate ratio.Conclusions: This study further attests to the relevance of the Krebs cycle in the development of PCC and PGL, and points to a potential role of other metabolic enzymes involved in metabolite exchange between mitochondria and cytosol. Clin Cancer Res; 1-10. ©2017 AACR. ©2017 American Association for Cancer Research.

  20. [Hypertension, catecholamine hypersecretion and potential for metastasis: recent progress in the pathophysiology and genetics of pheochromocytoma and paraganglioma].

    Science.gov (United States)

    Plouin, Pierre-François; Amar, Laurence; Gimenez-Roqueplo, Anne-paule

    2015-01-01

    Pheochromocytomas and paragangliomas are catecholamine-secreting tumors usually associated with arterial hypertension. They can contribute to acute cardiovascular events. Ten to 15 percent of tumors are metastatic. Autosomal dominant gene alterations are present in more than a third of cases. The secretory phenotype and the risk of malignancy are driven by the presence of gene mutations, specifically in the subunits of succinate dehydrogenase. Recent advances in genomics have clinical implications for family screening, biological follow-up, prediction of the risk of recurrence, and therapeutic options in cases with malignant recurrence.

  1. Giant Brunner’s Gland Adenoma of the Duodenal Bulb Presenting with Ampullary and Duodenal Obstruction Mimicking Pancreatic Malignancy

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    Vishal Gupta

    2011-07-01

    Full Text Available Context Brunner’s gland adenoma is a rare benign duodenal neoplasm. It usually presents with luminal obstruction or gastrointestinal bleeding. In rare cases, it may mimic a pancreatic malignancy and may present with obstructive jaundice. Case report A 65-year-old female presented with a two-month history of abdominal pain, early satiety and retrosternal burning pain. Liver function tests showed elevated enzymes with normal bilirubin. Imaging studies revealed a large mass in relation to the uncinate process of the pancreas and the distal duodenum along with the dilated common bile duct and the main pancreatic duct. Initial endoscopic evaluation failed to make a correct diagnosis. Repeat endoscopic evaluation combined with endoscopic sonography, however, revealed a large polypoid mass arising from the duodenal bulb. The patient underwent a transduodenal polypectomy. A diagnosis of Brunner’s gland adenoma was made on histopathological examination. Conclusion Giant Brunner’s adenoma may have unusual presentations. It may present with the features of ampullary obstruction mimicking periampullary or pancreatic malignancies. Extensive preoperative evaluation is required to reach a correct diagnosis in order to avoid more extensive surgery.

  2. Patients with Helicobacter pylori positive and negative duodenal ulcers have distinct clinical characteristics

    Institute of Scientific and Technical Information of China (English)

    Kent-Man Chu; Ka-Fai Kwok; Simon Law; Kam-Ho Wong

    2005-01-01

    AIM: To assess the clinical characteristics of Helicobacterpylori(H pylori) negative duodenal ulcer.METHODS: Patients with an endoscopic diagnosis of duodenal ulcer between 1996 and 2002 were included in the present study. Patients were considered to be negative for Hpylori, if both histological examination and rapid urease test of biopsy specimens were negative. A comparison was made between patients with H pyloripositive and negative duodenal ulcers.RESULTS: A total of 1 343 patients were studied. Their mean age was 54.7±0.5 years. There was a male preponderance (M:F = 2.5:1). Three hundred and ninetyeight patients (29.6%) did not have H pylori infection. The annual proportion of patients with H pylori negative duodenal ulcers increased progressively from 1996 to2002. On multivariate analysis, patients with H pylorinegative duodenal ulcer were more likely to be older, have concomitant medical problem, pre-existing malignancy, recent surgery, underlying sepsis, or taken non-steroidal anti-inflammatory drugs. In terms of clinical presentations, patients with H pylori negative duodenal ulcer were more likely to present with bleeding, multiple ulcers and larger ulcers.CONCLUSION: The proportion of patients with H pylori negative duodenal ulcers is on the rise because of a continued drop in incidence of H pylori positive duodenalulcers in recent years. Such patients have distinct clinical characteristics and it is important to ascertain the H pylori status before starting eradication therapy.

  3. Regulation of duodenal bicarbonate secretion during stress by corticotropin-releasing factor and beta-endorphin.

    Science.gov (United States)

    Lenz, H J

    1989-02-01

    Proximal duodenal mucosal bicarbonate secretion is an important factor in the pathogenesis of duodenal ulcer disease. To examine the central nervous system regulation of duodenal bicarbonate secretion, an animal model was developed that allowed cerebroventricular and intravenous injections as well as collection of duodenal perfusates in awake, freely moving rats. The hypothalamic peptide corticotropin-releasing factor (CRF) and stress (physical restraint) significantly stimulated duodenal bicarbonate secretion. These responses were abolished by pretreatment of the animals with the CRF receptor antagonist alpha-helical CRF-(9-41), hypophysectomy, and naloxone. In contrast, blockade of autonomic efferents by surgical and pharmacological means did not prevent the stimulatory effects of stress and CRF. Intravenous, but not cerebroventricular, administration of beta-endorphin that produced plasma concentrations of beta-endorphin that were similar to those produced by exogenous CRF and stress significantly stimulated duodenal bicarbonate secretion. These results indicate that endogenous CRF released during stress and exogenously administered CRF stimulate duodenal bicarbonate secretion by release of beta-endorphin from the pituitary, thus, demonstrating a functional hypothalamus-pituitary-gut axis.

  4. Effects of omeprazole and eradication of Helicobacter pylori on gastric and duodenal mucosal enzyme activities and DNA in duodenal ulcer patients.

    Science.gov (United States)

    Vetvik, K; Schrumpf, E; Mowinckel, P; Aase, S; Andersen, K J

    1994-11-01

    Duodenal and gastric content of mucosal enzymes in duodenal ulcer (DU) patients differs from that of controls. The purpose of this study has been to examine the effect of omeprazole and eradication of Helicobacter pylori on mucosal enzymes in DU patients. The enzyme activities of seven gastric and duodenal mucosal marker enzymes from the brush border, lysosomes, and mitochondria have been studied. In study I the measurements were made in 29 patients with an active DU before and after 14 days of omeprazole treatment. In study II 22 duodenal ulcer patients were given bismuth subnitrate, oxytetracycline, and metronidazole (triple therapy) for 2 weeks to eradicate H. pylori. Biopsy specimens were taken from the duodenum and the stomach for enzyme measurements and histologic assessment. In study II additional specimens were obtained from the prepyloric region for urease tests and culture of H. pylori. The ulcer healing rates were more than 90% after both omeprazole and triple therapy. H. pylori was eradicated in 86% after triple therapy. The activities of the brush-border enzymes lactase, neutral-alpha-glucosidase, alkaline phosphatase, leucyl-beta-naphthylamidase, and gamma-glutamyltransferase (gamma-GT) increased significantly in the duodenal bulb and the descending duodenum during treatment with omeprazole. No changes in duodenal enzyme activity were detected after triple therapy, whereas a significant fall in gamma-GT and acid phosphatase activities was seen in the stomach. The mucosal DNA in the gastric antrum decreased both after treatment with omeprazole and after triple therapy. A similar decrease in mucosal DNA of the gastric antrum was demonstrated after both omeprazole and triple therapy with bismuth subnitrate, oxytetracycline, and metronidazole. Omeprazole also affects the content of duodenal mucosal enzymes, whereas triple therapy particularly affects the gastric mucosal enzyme activity.

  5. Prevalence of non- Helicobacter pylori duodenal ulcer in Karachi,Pakistan

    Institute of Scientific and Technical Information of China (English)

    Javed Yakoob; Wasim Jafri; Nadim Jafri; Muhammad Islam; Shahab Abid; Saeed Hamid; Hasnain AliShah; Hizbullah Shaikh

    2005-01-01

    AIM: To determine the prevalence of non-Helicobacter pylori (H pylori)-related duodenal ulcer in patients with acid-peptic diseases.METHODS: Medical records of patients who attended the Gastroenterology Department at Aga Khan University Hospital from 1999 to 2001 and had endoscopic diagnosis of duodenal ulcers were reviewed. Duodenal ulcer associated with H pylori was diagnosed on the basis of endoscopy,rapid urease test and histopathology whereas histories of aspirin or other non-steroidal anti-inflammatory drugs (NSAIDs) related duodenal ulcers. Non-H pylori, non-NSAID duodenal ulcers were those without H pylori infection and history of NSAID intake. Co-morbid conditions associated were noted.RESULTS: Of 2 260 patients, 10% (217/2 260) had duodenal ulcer. Duodenal ulcer related to H pylori infectionaccounted for 53% (116/217), NSAID-related 10% (22/217),non-H pylori non-NSAID 29% (62/217), and 8% (17/217) had both Hpyloriinfection and histories of NSAID intake. Fifteen percent (18/116)_patients had past histories of peptic ulcer disease in Hpyloriinfection, while 8% (5/62) in non-Hpylorinon-NSAID ulcer. Co-morbid conditions in H pylori infection were seen in 23% (27/116) and 34%(21/62) in non-H pylori non-NSAID ulcer.CONCLUSION: Incidence of H pylori infection related with duodenal ulcer is common. In the presence of co-morbids, non-Hpyloriand non-NSAID duodenal ulcer is likely to be present.

  6. Clinical presentations, biochemical phenotypes, and genotype-phenotype correlations in patients with succinate dehydrogenase subunit B-associated pheochromocytomas and paragangliomas.

    NARCIS (Netherlands)

    Timmers, H.J.L.M.; Kozupa, A.; Eisenhofer, G.; Raygada, M.; Adams, K.T.; Solis, D.; Lenders, J.W.M.; Pacak, K.

    2007-01-01

    CONTEXT: Mutations of the gene encoding succinate dehydrogenase subunit B (SDHB) predispose to malignant paraganglioma (PGL). Recognition of the SDHB phenotype in apparently sporadic PGL directs appropriate treatment and family screening. OBJECTIVE: The objective of the study was to assess mutation-

  7. About two cases of cervical paraganglioma treated by exclusive irradiation; a propos de deux cas de paragangliome cervical traites par irradiation exclusive

    Energy Technology Data Exchange (ETDEWEB)

    Chekrine, T.; Eddekkaoui, H.; Bouchbika, Z.; Benchakroun, N.; Jouhadi, H.; Tawfiq, N.; Sahraoui, S.; Benider, A. [CHU Ibn-Rochd, Casablanca (Morocco)

    2011-10-15

    The authors briefly describe some characteristics of two inoperable cervical paraganglioma which have been treated by radiotherapy. Local control was good. In the first case, there was no noticeable toxicity after a ten year follow-up. In the second case, local control has been maintained after two years. Short communication

  8. Bladder paraganglioma with renal agenesis: A possible new association and its implications in the light of REarranged in transfection gene genetics

    Directory of Open Access Journals (Sweden)

    Rohan Satish Valsangkar

    2015-01-01

    Full Text Available Pheochromocytoma/paraganglioma and renal agenesis are commonly reported conditions. Their coexistence, however, is rare, with few cases reported. We report the case of a 21-year-old male who presented with painless hematuria. He was found to have congenital absent right kidney along with bladder mass on imaging. Examination including blood pressure was normal. He underwent cystoscopy that showed a solid looking tumor on the anterior wall. Paraganglioma was suspected due to intraoperative rise in blood pressure during resection and was confirmed on histopathology. Subsequently after work up and preoperative alpha blockade, patient underwent partial cystectomy and excision of the paravesical mass. Histopathology showed paraganglioma confined to bladder wall with surgical margins free and a paravesical mass that was seminal vesicle cyst. On follow-up, patient is normotensive and asymptomatic. This coexistence of paraganglioma and renal agenesis may have a common genetic mechanism in the form of REarranged in Transfection (RET gene mutation. This is a well-characterized gene, mutations of which are known to be associated with both conditions. Current knowledge of the role of RET gene in both conditions is reviewed to put forth RET mutation as the possible common underlying genetic mechanism along with possible clinical implications of the combination.

  9. Congenital duodenal obstruction with situs inversus totalis: Report of a rare association and discussion

    Directory of Open Access Journals (Sweden)

    Sharma Satendra

    2008-01-01

    Full Text Available This report is to present and discuss an extremely rare association of situs inversus with duodenal atresia in an 11-day-old male neonate born full term and weighing 1.9 kg. The baby presented with recurrent bilious vomiting. Babygram revealed situs inversus and duodenal obstruction. Echocardiography showed dextrocardia with a small ASD. Exploration confirmed a duodenal diaphragm with a central perforation between the third and fourth part of the duodenum and situs inversus. The literature search revealed 20 cases reported so far.

  10. First case reported of Bouveret's syndrome associated to duodenal and biliary perforation to retroperitoneum

    Directory of Open Access Journals (Sweden)

    María Victoria Vieiro-Medina

    Full Text Available We present the case of a 69 year old woman with a history of cholecystitis, who consulted for severe abdominal pain, nausea and vomiting. Abdominal CT showed duodenal obstruction caused by a gallstone, cholecystoduodenal fistula and pneumobilia, what is known as Bouveret's syndrome, a rare form of gallstone ileus. Additionally, she presented free duodenal and vesicular perforation to retroperitoneum at the same level of the cholecystoduodenal transit point. The patient underwent a difficult cholecystectomy, enterolithotomy, repair of the duodenal defect, extensive washing and drainage of the retroperitoneum. The postoperative course was uneventful except for a laparotomy infection.

  11. Upper gastrointestinal hemorrhage due to duodenal stromal tumor

    Directory of Open Access Journals (Sweden)

    Parreira José Gustavo

    2003-01-01

    Full Text Available BACKGROUND: Gastrointestinal stromal tumor represents a rare neoplasm that originates in the muscular wall of the hollow viscera. AIM: To report gastrointestinal stromal tumor as a source of upper gastrointestinal bleeding, which required urgent surgical control. PATIENT/METHOD: A man with 61 years old was admitted to the emergency service sustaining hematemesis and melena. Endoscopy showed active bleeding from a tumor in the second portion of the duodenum, which was controlled by heater probe cauterization. Surgery was performed through a median laparotomy. A local resection of a 4 cm tumor in the second portion of the duodenum was carried out, together with a primary end-to-end anastomosis and a duodenal diverticulization. No complications happened during the post-operative period. Morphologic examination showed gastrointestinal stromal tumor with no atypical mitosis and a preserved capsule. CONCLUSION: Albeit not being common, gastrointestinal stromal tumors can represent a source of substantial gastrointestinal hemorrhage.

  12. Functional characterization of serotonin receptor subtypes in human duodenal secretion

    DEFF Research Database (Denmark)

    Engelmann, Bodil Elisabeth; Bindslev, Niels; Poulsen, Steen Seier;

    2006-01-01

    of dyspeptic patients with or without Helicobacter pylori infection, and to determine the 5-HT receptor subtypes functionally involved. Biopsies from the second part of duodenum were obtained from 43 dyspeptic patients during routine endoscopy. Biopsies were mounted in modified Ussing chambers with air suction......: ketanserin, ondansetron, or SB-204070 (1-butyl-4 piperidinmethyl-8-amino-7-chloro-2,3-dihydro-1,4-benzodioxin-5-carboxylate HCl). Histological examination was performed on duodenal biopsies. Helicobacter urease testing and histological examination determined Helicobacter pylori infection. 5-HT induced a dose......-dependent and bumetanide-sensitive short-circuit current, which was independent of the presence of Helicobacter pylori infection. All the three 5-HT receptor antagonists failed to significantly effect basal and 5-HT-induced short-circuit current. Our results indicate that in human duodenum 1) 5-HT is a potent stimulator...

  13. Duodenal perforation as result of blunt abdominal trauma in childhood.

    Science.gov (United States)

    Hartholt, Klaas Albert; Dekker, Jan Willem T

    2015-12-23

    Blunt abdominal trauma may cause severe intra-abdominal injuries, while clinical findings could be mild or absent directly after the trauma. The absence of clinical findings could mislead physicians into underestimating the severity of the injury at the primary survey, and inevitably leads to a delay in the diagnosis. The Blunt Abdominal Trauma in Children (BATiC) score may help to identify children who are at a high risk for intra-abdominal injuries in an early stage and requires additional tests directly. A case of a 10-year-old girl with a duodenal perforation after a blunt abdominal trauma is presented. A delay in diagnosis may lead to an increased morbidity and mortality rate. A low admission threshold for children with abdominal pain after a blunt trauma is recommended.

  14. Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention.

    Science.gov (United States)

    Bausch, Birke; Schiavi, Francesca; Ni, Ying; Welander, Jenny; Patocs, Attila; Ngeow, Joanne; Wellner, Ulrich; Malinoc, Angelica; Taschin, Elisa; Barbon, Giovanni; Lanza, Virginia; Söderkvist, Peter; Stenman, Adam; Larsson, Catharina; Svahn, Fredrika; Chen, Jin-Lian; Marquard, Jessica; Fraenkel, Merav; Walter, Martin A; Peczkowska, Mariola; Prejbisz, Aleksander; Jarzab, Barbara; Hasse-Lazar, Kornelia; Petersenn, Stephan; Moeller, Lars C; Meyer, Almuth; Reisch, Nicole; Trupka, Arnold; Brase, Christoph; Galiano, Matthias; Preuss, Simon F; Kwok, Pingling; Lendvai, Nikoletta; Berisha, Gani; Makay, Özer; Boedeker, Carsten C; Weryha, Georges; Racz, Karoly; Januszewicz, Andrzej; Walz, Martin K; Gimm, Oliver; Opocher, Giuseppe; Eng, Charis; Neumann, Hartmut P H

    2017-09-01

    Effective cancer prevention is based on accurate molecular diagnosis and results of genetic family screening, genotype-informed risk assessment, and tailored strategies for early diagnosis. The expanding etiology for hereditary pheochromocytomas and paragangliomas has recently included SDHA, TMEM127, MAX, and SDHAF2 as susceptibility genes. Clinical management guidelines for patients with germline mutations in these 4 newly included genes are lacking. To study the clinical spectra and age-related penetrance of individuals with mutations in the SDHA, TMEM127, MAX, and SDHAF2 genes. This study analyzed the prospective, longitudinally followed up European-American-Asian Pheochromocytoma-Paraganglioma Registry for prevalence of SDHA, TMEM127, MAX, and SDHAF2 germline mutation carriers from 1993 to 2016. Genetic predictive testing and clinical investigation by imaging from neck to pelvis was offered to mutation-positive registrants and their relatives to clinically characterize the pheochromocytoma/paraganglioma diseases associated with mutations of the 4 new genes. Prevalence and spectra of germline mutations in the SDHA, TMEM127, MAX, and SDHAF2 genes were assessed. The clinical features of SDHA, TMEM127, MAX, and SDHAF2 disease were characterized. Of 972 unrelated registrants without mutations in the classic pheochromocytoma- and paraganglioma-associated genes (632 female [65.0%] and 340 male [35.0%]; age range, 8-80; mean [SD] age, 41.0 [13.3] years), 58 (6.0%) carried germline mutations of interest, including 29 SDHA, 20 TMEM127, 8 MAX, and 1 SDHAF2. Fifty-three of 58 patients (91%) had familial, multiple, extra-adrenal, and/or malignant tumors and/or were younger than 40 years. Newly uncovered are 7 of 63 (11%) malignant pheochromocytomas and paragangliomas in SDHA and TMEM127 disease. SDHA disease occurred as early as 8 years of age. Extra-adrenal tumors occurred in 28 mutation carriers (48%) and in 23 of 29 SDHA mutation carriers (79%), particularly with head

  15. Dosimetry for {sup 131}I-MIBG therapies in metastatic neuroblastoma, phaeochromocytoma and paraganglioma

    Energy Technology Data Exchange (ETDEWEB)

    Sudbrock, Ferdinand [University of Cologne, Department of Nuclear Medicine, Cologne (Germany); University Hospital of Cologne, Department of Nuclear Medicine, Cologne (Germany); Schmidt, Matthias; Eschner, Wolfgang; Schicha, Harald [University of Cologne, Department of Nuclear Medicine, Cologne (Germany); Simon, Thorsten; Berthold, Frank [University of Cologne, Children' s Hospital, Cologne (Germany)

    2010-07-15

    Radiation dosimetry is a basic requirement for targeted radionuclide therapies (TRT) which have become of increasing interest in nuclear medicine. Despite the significant role of the radiopharmaceutical {sup 131}I-metaiodobenzylguanidine (MIBG) for the treatment of metastatic neuroblastoma, phaeochromocytoma and paraganglioma details for a reliable dosimetry are still sparse. This work presents our procedures, the dosimetric data and experiences with TRT using {sup 131}I-MIBG. A total of 21 patients were treated with {sup 131}I-MIBG between 2004 and 2008 according to a clearly defined protocol. Whole-body absorbed doses were determined by a series of scintillation probe readings for all 21 cases. Tumour absorbed doses were calculated on the basis of quantitative imaging for an entity of 25 lesions investigated individually using the region of interest (ROI) technique based on five scans each. Typical whole-body absorbed doses are found in the region of 2 Gy (range: 1.0-2.9 Gy) whereas tumour absorbed doses in turn cover a span between 10 and 60 Gy. Nonetheless this variation of tumour absorbed doses is comparatively low. The trial protocol in use is a substantial advancement in terms of reliable dosimetry. A clearly defined modus operandi for MIBG therapies should involve precisely described dosimetric procedures, e.g. a minimum of 20 whole-body measurements using a calibrated counter and at least four gamma camera scans over the whole period of the inpatient stay should be carried out. Calculation of tumour volumes is accomplished best via evaluation of SPECT and CT images. (orig.)

  16. Clinical Significance of Quantitative 123I-MIBG SPECT/CT Analysis of Pheochromocytoma and Paraganglioma.

    Science.gov (United States)

    Nakamoto, Ryusuke; Nakamoto, Yuji; Ishimori, Takayoshi; Togashi, Kaori

    2016-11-01

    This retrospective study compared the diagnostic performances of quantitative versus visual analyses of I-MIBG scintigraphy in patients with suspected pheochromocytoma and paraganglioma (PPGL). SPECT images were obtained 6 and/or 24 h after MIBG injection from 68 patients with clinically suspected PPGL, with attenuation correction by low-dose unenhanced CT. Planar images were also obtained at each time point. SUVs of retroperitoneal tumors, including PPGLs, and physiological uptake by normal organs were measured using the SPECT images. The diagnostic performance of the quantitative assessment in differentiating PPGLs from other lesions or normal adrenal glands was assessed using receiver operating characteristic analysis. The planar scans and 6-h and 24-h SPECT/CT images were also assessed visually. PPGLs showed a significantly higher SUVmax (mean ± SD = 9.97 ± 3.86) than other retroperitoneal lesions (3.85 ± 1.51) or normal adrenal glands (3.91 ± 1.20). At an optimal cut-off of 6.57, the sensitivity, specificity, and accuracy of the quantitative assessment for 6-h SPECT/CT in differentiating PPGLs was 78.6%, 96.3%, and 92.6%, respectively; the area under the curve was 0.878. The diagnostic performance did not significantly differ between the quantitative and visual analyses, but the specificity of the former tended to be higher at 6 h (96.3% vs. 90.7%) and at 24 h (91.2% vs. 82.4%). The specificity, but not the sensitivity, of the quantitative approach was higher than that of visual assessment in differentiating PPGLs from other retroperitoneal pathologies and from physiological uptake in the normal adrenal gland.

  17. Pituitary Adenoma With Paraganglioma/Pheochromocytoma (3PAs) and Succinate Dehydrogenase Defects in Humans and Mice

    Science.gov (United States)

    Xekouki, Paraskevi; Szarek, Eva; Bullova, Petra; Giubellino, Alessio; Quezado, Martha; Mastroyannis, Spyridon A.; Mastorakos, Panagiotis; Wassif, Christopher A.; Raygada, Margarita; Rentia, Nadia; Dye, Louis; Cougnoux, Antony; Koziol, Deloris; Sierra, Maria de La Luz; Lyssikatos, Charalampos; Belyavskaya, Elena; Malchoff, Carl; Moline, Jessica; Eng, Charis; Maher, Louis James; Pacak, Karel; Lodish, Maya

    2015-01-01

    Context: Germline mutations in genes coding succinate dehydrogenase (SDH) subunits A, B, C, and D have been identified in familial paragangliomas (PGLs)/pheochromocytomas (PHEOs) and other tumors. We described a GH-secreting pituitary adenoma (PA) caused by SDHD mutation in a patient with familial PGLs. Additional patients with PAs and SDHx defects have since been reported. Design: We studied 168 patients with unselected sporadic PA and with the association of PAs, PGLs, and/or pheochromocytomas, a condition we named the 3P association (3PAs) for SDHx germline mutations. We also studied the pituitary gland and hormonal profile of Sdhb+/− mice and their wild-type littermates at different ages. Results: No SDHx mutations were detected among sporadic PA, whereas three of four familial cases were positive for a mutation (75%). Most of the SDHx-deficient PAs were either prolactinomas or somatotropinomas. Pituitaries of Sdhb+/− mice older than 12 months had an increased number mainly of prolactin-secreting cells and several ultrastructural abnormalities such as intranuclear inclusions, altered chromatin nuclear pattern, and abnormal mitochondria. Igf-1 levels of mutant mice tended to be higher across age groups, whereas Prl and Gh levels varied according to age and sex. Conclusion: The present study confirms the existence of a new association that we termed 3PAs. It is due mostly to germline SDHx defects, although sporadic cases of 3PAs without SDHx defects also exist. Using Sdhb+/− mice, we provide evidence that pituitary hyperplasia in SDHx-deficient cells may be the initial abnormality in the cascade of events leading to PA formation. PMID:25695889

  18. Clinical and biochemical responses after Gamma Knife surgery for a dopamine-secreting paraganglioma: case report.

    Science.gov (United States)

    Tuleasca, Constantin; Jaquet, Yves; Schweizer, Valerie; Negretti, Laura; Magaddino, Vera; Maeder, Philippe; Abid, Karim-Alexandre; Lhermitte, Benoit; Grouzmann, Eric; Levivier, Marc

    2016-01-01

    The efficacy of Gamma Knife surgery (GKS) in local tumor control of non-secreting paragangliomas (PGLs) has been fully described by previous studies. However, with regard to secreting PGL, only one previous case report exists advocating its efficacy at a biological level. The aims of this study were: 1) to evaluate the safety/efficacy of GKS in a dopamine-secreting PGL; 2) to investigate whether the biological concentrations of free methoxytyramine could be used as a marker of treatment efficacy during the follow-up. We describe the case of a 62-year-old man diagnosed with left PGL. He initially underwent complete surgical excision. Thirty months after, he developed recurrent biological and neuroradiological disease; the most sensitive biomarker for monitoring the disease, concentration of plasma free methoxytyramine, started to increase. GKS was performed at a maximal marginal dose of 16 Gy. During the following 30 months, concentration of free methoxytyramine gradually decreased from 0.14 nmol/l (2*URL) before GKS to 0.09 nmol/l, 6 months after GKS and 0.07 nmol/l at the last follow-up after GKS (1.1*URL), confirming the efficacy of the treatment. Additionally, at 30 months there was approximately 36.6% shrinkage from the initial target volume. The GKS treatment was safe and effective, this being confirmed clinically, neuroradiologically and biologically. The case illustrates the importance of laboratory tests taking into account methoxytyramine when analyzing biological samples to assess the biochemical activity of a PGL. In addition, the identification of methoxytyramine as a unique positive biomarker could designate it for the monitoring of tumor relapse after treatments, including Gamma Knife surgery.

  19. Anti-hypertensive treatment in pheochromocytoma and paraganglioma: current management and therapeutic features.

    Science.gov (United States)

    Mazza, Alberto; Armigliato, Michela; Marzola, Maria Cristina; Schiavon, Laura; Montemurro, Domenico; Vescovo, Giorgio; Zuin, Marco; Chondrogiannis, Sotirios; Ravenni, Roberta; Opocher, Giuseppe; Colletti, Patrick M; Rubello, Domenico

    2014-04-01

    Pheochromocytoma (PH) and paraganglioma (PG) are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and the sympathetic ganglia, respectively. Although are unusual cause of hypertension (HT) accounting for at most 0.1-0.2 % of cases, they may lead to severe and potentially lethal hypertensive crisis due to the effects of the released catecholamines. However, both PH and PG may be asymptomatic as ~30 % of subjects are normotensive or have orthostatic hypotension and in these cases the 24 h ambulatory blood pressure (BP) monitoring is an important toll to diagnose and treat HT. HT treatment may be difficult when PH or PG occurs in pregnancy or in the elderly subjects and in these cases a multidisciplinary team is required. When surgical excision is mandatory the perioperative management requires the administration of selective α1-adrenergic blocking agents (i.e., doxazosin, prazosin or terazosin) followed by a β-adrenergic blockade (i.e., propranolol, atenolol). This latter should never be started first because blockade of vasodilatory peripheral β-adrenergic receptors with unopposed α-adrenergic receptor stimulation can lead to a further elevation of BP. Although labetalol is traditionally considered the ideal agent due to its α- and β-adrenergic antagonism, experimental studies do not support its use in this clinical setting. As second regimen, the administration of vasodilators as calcium channel blockers (i.e., nicardipine, nifedipine) may be required to control BP. Oral and sublingual short-acting nifedipine are potentially dangerous in patients with hypertensive emergencies and are not recommend. The latest evidences into the diagnosis and treatment of hypertensive crisis due to PH and PG are reviewed here.

  20. The first Dutch SDHB founder deletion in paraganglioma – pheochromocytoma patients

    Directory of Open Access Journals (Sweden)

    Devilee Peter

    2009-04-01

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

  1. [Effects of halothane and isoflurane on the canine duodenal paraneurons].

    Science.gov (United States)

    Sato, K; Noguchi, R; Taga, K; Shimoji, K; Fujita, T

    1988-05-01

    Administration of amino acid solution (50 mM tryptophane and phenylalanine in saline) into the canine duodenum is known to cause an increase in pancreatic secretion. This response is mediated by the excitation of duodenal endocrine cells, paraneurons, which release cholecystokinin (CCK) into the systemic circulation in response to intraluminal amino acid stimuli. Pancreatic secretory cells are then evoked by the CCK in the blood to secrete the juice into the duodenum. The authors investigated the effects of two general anesthetics, halothane and isoflurane, on this response. Nine mongrel dogs were subjected to this study. Each dog underwent laparotomy under nitrous oxide (75%)-oxygen (25%) anesthesia with pancuronium (GO-Pb). The duodenal loop was exposed and two polyethylene cannulae (18Fr) were introduced into the loop. Proximal cannula was for the administration of the amino acid solution into the loop, and distal one was for drainage of the solution. The pancreatic duct was inserted with a polyethylene catheter, through which pancreatic juice was collected and measured for the volume and protein output by spectrophotometry. After these surgical procedures, the pancreatic secretory response to intraluminal amino acid stimuli was examined under GO-Pb (Control). Then halothane (1.0%) (Group 1, four dogs) or isoflurane (2.0%) (Group 2, five dogs) was administered for 30 min and the same response was tested. The pancreatic secretory response to intraluminal amino acid stimulus was suppressed by the surgical concentrations of both halothane (1.0%) and isoflurane (2.0%). Neither halothane nor isoflurane suppressed the pancreatic secretory response evoked by intravenous CCK infusion (10 Ivy Dog Units.(ABSTRACT TRUNCATED AT 250 WORDS)

  2. Radiological and pathological findings of a metastatic composite paraganglioma with neuroblastoma in a man: a case report

    Directory of Open Access Journals (Sweden)

    Koch Sonja

    2010-11-01

    Full Text Available Abstract Introduction Composite tumors of the adrenal medulla or paraganglia are extremely rare and present a diagnostic dilemma. These tumors consist of a neuroendocrine component mixed with a neural component. We describe the imaging characteristics together with the corresponding pathological findings of a composite tumor. Apart from any component-specific imaging findings, the hallmark of this entity is the presence of histologically distinguishable components. Case presentation A 61-year-old Caucasian man was referred to our hospital due to a suspect lesion found on chest computed tomography carried out for unclear thoracic pain. An abdominal computed tomography scan and ultrasound examination detected a retroperitoneal tumor comprising two different tumor components. Twenty-four-hour urine revealed high levels of normetanephrine, characteristic of a neuroendocrine tumor. An octreoscan prior to surgical procedures revealed multiple osseous and intra-hepatic metastases. The final histopathological workup revealed a composite paraganglioma with neuroblastoma. Our patient died ten months after the initial diagnosis from tumor-associated complications. Conclusions Composite paragangliomas with neuroblastoma are rare tumors of the retroperitoneum. Such tumors should be considered in the differential diagnosis of retroperitoneal masses.

  3. First clinical experience in applying XperGuide in embolization of jugular paragangliomas by direct intratumoral puncture

    Energy Technology Data Exchange (ETDEWEB)

    Spelle, Laurent; Moret, Jacques [Fondation Rothschild Hospital, Interventional Neuroradiology Department, Paris (France); Ruijters, Daniel; Babic, Drazenko; Homan, Robert; Mielekamp, Peter; Guillermic, Jeremy [Philips Healthcare, Cardio/Vascular Innovation, Best (Netherlands)

    2009-11-15

    The purpose of this study is to introduce a novel image-guided technique utilized in the embolization of jugular paraganglioma tumors, using preoperative diagnostic scans and planning together with perioperative X-ray fluoroscopy in a combined image. A lesion center and a skin entry point on the patient are selected and connected with a straight line, which resembles the most ideal lesion access trajectory to be followed during the needle insertion. The skin entry point and the corresponding line location are selected such that it avoids the impenetrable bones and vital anatomical structures. Two viewing incidence angles are defined to guide the cranial needle insertion: the entry view tangent to the planned trajectory, and the progression view perpendicular to the path. The proposed method was applied in two patients with jugular paragangliomas in order to navigate needles to the lesion location and subsequently embolize the tumors. The perioperative registration took less than 8 s. Using this method, it was possible to guide the needle within 5 mm of the planned path. The fluoroscopic needle navigation, overlaid on the corresponding soft tissue of the underlying anatomy, combined with a planned path, has been shown to be an accurate and efficient tool for needle guidance. The patient pose varied between the preoperative data and the fluoroscopy guided intervention, but this did not hinder the procedure. (orig.)

  4. [Analysis of laboratory data of 155 patients with pheochromocytoma-paraganglioma syndrome diagnosed during the past 20 years].

    Science.gov (United States)

    Balog, Beatrice; Tőke, Judit; Róna, Kálmán; Szücs, Nikolette; Igaz, Péter; Pusztai, Péter; Sármán, Beatrix; Gláz, Edit; Kiss, Róbert; Patócs, Attila; Rácz, Károly; Tóth, Miklós

    2015-04-19

    Laboratory diagnosis of pheochromocytoma-paraganglioma syndrome has been markedly improved during the past two decades. Retrospective assessment of diagnostic utility of urinary catecholamines and their metabolites as well as serum chromogranin A in 155 patients diagnosed at the 2nd Department of Medicine, Semmelweis University. Urinary catecholamines and metabolites were measured using high-performance liquid chromatography with electrochemical detection in 155 patients with pheochromocytoma-paraganglioma (of whom 28.4% had hereditary background) and in 170 non-pheochromocytoma patients used as controls. Serum chromogranin A was measured by immunoradiometry. Sensitivity (93.2%) and specificity (87.0%) of urinary fractionated metanephrines were higher than those of urinary catecholamines (90.9% vs. 85.7%, respectively) and serum chromogranin A (88.7% and 77.5%, respectively). Urinary normetanephrine and serum chromogranin A correlated positively with tumor size (r = 0.552, p<0.0001 and r = 0.618, p<0.0001, respectively). These data confirm the diagnostic utility of urinary catecholamines and their metabolites. Urinary normetanephrine and serum chromogranin A may help to estimate tumour mass and probably tumour progression.

  5. Management of Facial Nerve in Surgical Treatment of Previously Untreated Fisch Class C Tympanojugular Paragangliomas: Long-Term Results

    Science.gov (United States)

    Bacciu, Andrea; Ait Mimoune, Hassan; D'Orazio, Flavia; Vitullo, Francesca; Russo, Alessandra; Sanna, Mario

    2013-01-01

    The aim of this study was to evaluate the long-term facial nerve outcome according to management of the facial nerve in patients undergoing surgery for Fisch class C tympanojugular paragangliomas. The study population consisted of 122 patients. The infratemporal type A approach was the most common surgical procedure. The facial nerve was left in place in 2 (1.6%) of the 122 patients, anteriorly rerouted in 97 (79.5%), anteriorly rerouted with segmental resection of the epineurium in 7 (5.7%), and sacrificed and reconstructed in 15 (12.3%). One patient underwent cross-face nerve grafting. At last follow-up, House-Brackmann grade I to II was achieved in 51.5% of patients who underwent anterior rerouting and in 28.5% of those who underwent anterior rerouting with resection of the epineurium. A House-Brackmann grade III was achieved in 73.3% of patients who underwent cable nerve graft interposition. The two patients in whom the facial nerve was left in place experienced grade I and grade III, respectively. The patient who underwent cross-face nerve grafting had grade III. Gross total resection was achieved in 105 cases (86%). Management of the facial nerve in tympanojugular paraganglioma surgery can be expected to ensure satisfactory facial function long-term outcome. PMID:24498582

  6. Normal villous architecture with increased intraepithelial lymphocytes: a duodenal manifestation of Crohn disease.

    Science.gov (United States)

    Patterson, Emily R; Shmidt, Eugenia; Oxentenko, Amy S; Enders, Felicity T; Smyrk, Thomas C

    2015-03-01

    To assess a possible association between inflammatory bowel disease (IBD) and the histologic finding in duodenal biopsy specimens of increased intraepithelial lymphocytes (IELs) with normal villous architecture. We identified all patients with duodenal biopsy specimens obtained between 2000 and 2010 showing increased IELs and normal architecture. Among the 74 such patients who also had IBD, we characterized the clinical features of IBD and reviewed all available upper gastrointestinal biopsy specimens. Fifty-eight patients had Crohn disease, 13 had ulcerative colitis, and three had IBD, type unclassified. No duodenal sample with increased IELs had other histologic features of IBD. Among gastric biopsy specimens from 34 patients with Crohn disease, nearly half (16) had focal gastritis. We propose that Crohn disease be included in the differential diagnosis for increased IELs with normal villous architecture in duodenal biopsy specimens, particularly when gastric biopsy specimens show focal gastritis. Copyright© by the American Society for Clinical Pathology.

  7. Computer simulation of flow and mixing at the duodenal stump after gastric resection

    Institute of Scientific and Technical Information of China (English)

    Nenad Filipovic; Aleksandar Cvetkovic; Velibor Isailovic; Zoran Matovic; Mirko Rosic; Milos Kojic

    2009-01-01

    AIM: To investigate the flow and mixing at the duodenal stump after gastric resection, a computersimulation was implemented.METHODS: Using the finite element method, two different Billroth ? procedure cases (A and B) were modeled. Case A was defined with a shorter and almost straight duodenal section, while case B has a much longer and curved duodenal section. Velocity,pressure and food concentration distribution were determined and the numerical results were compared with experimental observations.RESULTS: The pressure distribution obtained by numerical simulation was in the range of the recorded experimental results. Case A had a more favorable pressure distribution in comparison with case B.However, case B had better performance in terms of food transport because of more continual fooddistribution,as well as better emptying of the duodenal section.

  8. Increased tissue concentration of neuropeptide Y in the duodenal mucosa in coeliac disease

    Energy Technology Data Exchange (ETDEWEB)

    Sjoelund, K.; Ekman, R. (Lund Univ. (Sweden))

    1989-01-01

    Neuropeptide Y (NPY) is localized to intestinal nerve fibres, of which there are few in normal duodenal mucosa. In the duodenal mucosa of 10 patients with coeliac disease and in a control group of 21 patients with other gastrointestinal symptoms, but with normal function of the small intestine, we studied the frequency of such fibres by immunohistochemistry and the tissue concentration of NPY by radioimmunoassay. Patients with coeliac disease had an increased number of NPY nerve fibres and significantly elevated tissue concentrations compared with the control group. The eluted fractions obtained by high-pressure liquid chromatography of duodenal extracts showed the same immunoreactive components in the two groups. This study therefore suggested proliferation of the peptide-containing nerve system in coeliac disease. The increased NPY levels in the duodenal mucosa may be of functional significance for the disease symptoms. 24 refs.

  9. Chronic diarrhea due to duodenal candidiasis in a patient with a history of kidney transplantation.

    Science.gov (United States)

    Nouri-Majalan, Nader; Moghaddasi, Sarasadat; Qane, Mohammad Davud; Shefaie, Farzane; Masoumi Dehshiri, Roghayyeh; Amirbaigy, Mohammad Kassem; Baghbanian, Mahmoud

    2014-11-01

    Candida infection in the small intestine is uncommon. We report an unusual case of duodenal candidiasis that presented as chronic diarrhea in a patient who had previously undergone kidney transplantation. A 60-year-old man presented with profuse watery diarrhea that had lasted 6 months 13 years after kidney transplantation. Upper gastrointestinal endoscopy results indicated candidiasis within the esophagus and duodenum. Biopsy results revealed active duodenitis with hyphal and yeast forms of Candida overlying the duodenal epithelium in periodic acid Schiff staining. The patient was successfully treated with fluconazole. After 6 months of follow-up, the patient had no complaint of diarrhea. Duodenal candidiasis may be the result of chronic diarrhea in patients with a history of kidney transplantation.

  10. Massive duodenal variceal bleed; complication of extra hepatic portal hypertension: Endoscopic management and literature review

    Institute of Scientific and Technical Information of China (English)

    Christopher; Steevens; Maisa; Abdalla; Truptesh; H; Kothari; Vivek; Kaul; Shivangi; Kothari

    2015-01-01

    Bleeding from duodenal varices is reported to be a catastrophic and often fatal event. Most of the cases in the literature involve patients with underlying cirrhosis. However, approximately one quarter of duodenal variceal bleeds is caused by extrahepatic portal hypertension and they represent a unique population given their lack of liver dysfunction. The authors present a case where a 61-year-old male with history of remote crush injury presented with bright red blood per rectum and was found to have bleeding from massive duodenal varices. Injection sclerotherapy with ethanolamine was performed and the patient experienced a favorable outcome with near resolution of his varices on endoscopic follow-up. The authors conclude that sclerotherapy is a reasonable first line therapy and review the literature surrounding the treatment of duodenal varices secondary to extrahepatic portal hypertension.

  11. Modeling data for pancreatitis in presence of a duodenal diverticula using logistic regression

    Science.gov (United States)

    Dineva, S.; Prodanova, K.; Mlachkova, D.

    2013-12-01

    The presence of a periampullary duodenal diverticulum (PDD) is often observed during upper digestive tract barium meal studies and endoscopic retrograde cholangiopancreatography (ERCP). A few papers reported that the diverticulum had something to do with the incidence of pancreatitis. The aim of this study is to investigate if the presence of duodenal diverticula predisposes to the development of a pancreatic disease. A total 3966 patients who had undergone ERCP were studied retrospectively. They were divided into 2 groups-with and without PDD. Patients with a duodenal diverticula had a higher rate of acute pancreatitis. The duodenal diverticula is a risk factor for acute idiopathic pancreatitis. A multiple logistic regression to obtain adjusted estimate of odds and to identify if a PDD is a predictor of acute or chronic pancreatitis was performed. The software package STATISTICA 10.0 was used for analyzing the real data.

  12. [Functional state of the gastro-duodenal area in gastro-esophageal reflux disease].

    Science.gov (United States)

    Vakhrushev, Ia M; Potapova, L O

    2007-01-01

    The complex study of stomach and duodenal function was performed in 150 patients with GERD. It was revealed that 84,3% of patients had increased stomach acid-production. In GERD exacerbation we found the disturbance of gastric mucosa decreasing protective properties of esophageal, stomach and duodenal mucosa. The patients had increased intragastral and intraduodenal pressure leading to decreasing of closing function of pylorus. Due to dynamic gastroscintigraphy the slowing of stomach evacuation was revealed in 69,2% patients, the acceleration - in 7,7% patients. The prevalence of bradyperistalsis was found in elecrogastromyography. The role of hormones (gastrin, insulin, cortisol, thyrotrophin, thyroxin) in disorders of gastro-duodenal complex function was shown. The complex investigation of gastro-duodenal complex function opens the pathophysiologic base of GERD and these data may be used in the choice of adequate therapy.

  13. [A case of pancreatic and duodenal fistula after total gastrectomy successfully treated with coagulation factor XIII].

    Science.gov (United States)

    Nishino, Hitoe; Kojima, Kazuhiro; Oshima, Hirokazu; Nakagawa, Koji; Fumura, Masao; Kikuchi, Norio

    2013-11-01

    Pancreatic fistula( PF) is a challenging postoperative complication. We report a case of PF following gastrectomy successfully treated using intravenous coagulation factor XIII( FXIII).A 78-year-old man with early gastric cancer underwent total gastrectomy with Roux-en-Y reconstruction. PF developed postoperatively, following which, leakage from the duodenal stump was observed. Percutaneous drainage and re-operative surgery were performed. A somatostatin analogue, antibiotic drugs, and gabexate mesilate were administrated along with nutritional support. The pancreatic and duodenal fistula had been producing duodenal juice for over 30 days since the re-operative surgery. As suspected, reduced FXIII activity was confirmed in the patient. After administering FXIII for 5 days, the amount of duodenal juice from the fistula markedly reduced, and the fistula closed immediately afterwards. The results of our study suggest that administration of FXIII could be a reasonable and effective treatment for patients with pancreatic or/and enterocutaneous fistula who are resistant to standard treatments.

  14. Inflammatory Duodenal Polyposis Associated with Primary Immunodeficiency Disease: A Novel Case Report

    Science.gov (United States)

    Shera, Irfan Ali; Khurshid, Sheikh Mudassir

    2017-01-01

    Agammaglobulinemia is a rare form of B-cell primary immunodeficiency disease characterized by reduced levels of IgG, IgA, or IgM and recurrent bacterial infections. Agammaglobulinemia is most commonly associated with diffuse nodular lymphoid hyperplasia. Duodenal polyps are a rare entity; however, due to wide use of esophagogastroduodenoscopy, incidental diagnosis of duodenal polyps appears to be increasing. Although inflammatory duodenal polyposis has been reported in the literature, its association with common variable immunodeficiency has not been reported till date to the best of our knowledge. We report a case of a 59-year-old male with chronic symptoms of agammaglobulinemia associated with inflammatory duodenal polyposis. PMID:28163721

  15. Effect of cysteamine on cytosolic somatostatin binding sites in rabbit duodenal mucosa.

    Science.gov (United States)

    Gonzalez-Guijarro, L; Lopez-Ruiz, M P; Bodegas, G; Prieto, J C; Arilla, E

    1987-04-01

    Administration of cysteamine in rabbits elicited a rapid depletion of both duodenal mucosa and plasma somatostatin. A significant reduction was observed within 5 min, returning toward control values by 150 min. The depletion of somatostatin was associated with an increase in the binding capacity and a decrease in the affinity of both high- and low-affinity binding sites present in cytosol of duodenal mucosa. Incubation of cytosolic fraction from control rabbits with 1 mM cysteamine did not modify somatostatin binding. Furthermore, addition of cysteamine at the time of binding assay did not affect the integrity of 125I-Tyr11-somatostatin. It is concluded that in vivo administration of cysteamine to rabbits depletes both duodenal mucosa and plasma somatostatin and leads to up-regulation of duodenal somatostatin binding sites.

  16. Effects of Ginkgo biloba extract on cytoprotective factors in rats with duodenal ulcer

    Institute of Scientific and Technical Information of China (English)

    Jane C.-J. Chao; Huei-Chen Hung; Sheng-Hsuan Chen; Chia-Lang Fang

    2004-01-01

    AIM: To investigate the effects of Ginkgo biloba extract on cytoprotective factors in rats with duodenal ulcer.METHODS: Sprague-Dawley rats were randomly divided into four groups: sham operation without ginkgo, sham operation with ginkgo, duodenal ulcer without ginkgo, and duodenal ulcer with ginkgo. Rats with duodenal ulcer were induced by 500 mL/L acetic acid. Rats with ginkgo were intravenously injected with Ginkgo biloba extract from the tail at a dose of 0.5 mg/(kg.d) for 7 and 14 days.RESULTS: Pathological result showed that duodenal ulcer rats with ginkgo improved mucosal healing and inflammation compared with those without ginkgo after 7 d treatment. After 14 d treatment, duodenal ulcer rats with ginkgo significantly increased weight gain (34.0±4.5 g versus 24.5±9.5 g,P<0.05) compared with those without ginkgo. Duodenal ulcer rats significantly increased cell proliferation (27.4l±4.0and 27.8±2.3 BrdU-labeled cells in duodenal ulcer rats with and without ginkgo versus 22.4±3.5 and 20.8±0.5 BrdUlabeled cells in sham operation rats with and without ginkgo,P<0.05) compared with sham operation rats. Mucosal prostaglandin E2 concentration significantly increased by 129% (P<0.05) in duodenal ulcer rats with ginkgo compared with that in those without ginkgo. Duodenal ulcer rats without ginkgo significantly decreased superoxide dismutase activity in the duodenal mucosa and erythrocytes (19.4±6.7 U/mg protein versus 38.1±18.9 U/mg protein in the duodenal mucosa,and 4.87±1.49 U/mg protein versus 7.78±2.16 U/mg protein in erythrocytes, P<0.05) compared with sham operation rats without ginkgo. However, duodenal ulcer rats with ginkgo significantly increased erythrocyte superoxide dismutase activity (8.22±1.92 U/mg protein versus 4.87±1.49 U/mg protein,P<0.05) compared with those without ginkgo. Duodenal ulcer rats without ginkgo significantly increased plasma lipid peroxides (4.18±1. 12 μmol/mL versus 1.60±1.10 μmol/mL and 1.80±0.73

  17. Endoscopic Injection of a Ruptured Duodenal Varix with N-butyl-2-cyanoacrylate

    Directory of Open Access Journals (Sweden)

    Paulo Salgueiro

    2014-04-01

    Conclusions: The presented case supports endoscopic injection sclerotherapy with N-butyl-2-cyanoacrylate as a treatment option for ruptured duodenal varices that, despite being a rare event, when it occurs, is often fatal.

  18. Inflammatory Duodenal Polyposis Associated with Primary Immunodeficiency Disease: A Novel Case Report

    Directory of Open Access Journals (Sweden)

    Irfan Ali Shera

    2017-01-01

    Full Text Available Agammaglobulinemia is a rare form of B-cell primary immunodeficiency disease characterized by reduced levels of IgG, IgA, or IgM and recurrent bacterial infections. Agammaglobulinemia is most commonly associated with diffuse nodular lymphoid hyperplasia. Duodenal polyps are a rare entity; however, due to wide use of esophagogastroduodenoscopy, incidental diagnosis of duodenal polyps appears to be increasing. Although inflammatory duodenal polyposis has been reported in the literature, its association with common variable immunodeficiency has not been reported till date to the best of our knowledge. We report a case of a 59-year-old male with chronic symptoms of agammaglobulinemia associated with inflammatory duodenal polyposis.

  19. Perforated duodenal ulcer a rare cause of acute abdomen in infancy: A report of two cases

    Directory of Open Access Journals (Sweden)

    Kadian Yogender

    2008-01-01

    Full Text Available Duodenal ulcer perforation is very uncommon in infants and children, that is why it is not usually considered in the differential diagnosis of acute abdomen in this age group. Moreover, the diagnosis of this condition is usually overlooked because of vague and variable symptoms and low index of suspicion on the part of the treating physicians. In this brief report, we are reporting two cases of successfully managed perforated duodenal ulcer in infancy.

  20. Gastric Duplication Cyst in Association with Duodenal Atresia in a Neonate

    Science.gov (United States)

    Mirshemirani, Alireza; Roshanzamir, Fatollah; Razavi, Sajad; Sarafi, Mehdi

    2016-01-01

    Concurrence of duodenal atresia and gastric duplication cyst is extremely rare entity. We report a 6-day-old female neonate who presented with neonatal intestinal obstruction. X-ray abdomen showed double bubble sign. At laparotomy, a huge cystic structure attached to greater curvature of the stomach along with duodenal atresia of second part of duodenum was found. The cystic structure was excised and duodeno-duodenostomy performed. Histopathology report confirmed it gastric duplication cyst. PMID:26816679

  1. Helicobacter pylori: the primary cause of duodenal ulceration or a secondary infection?

    Institute of Scientific and Technical Information of China (English)

    M Hobsley; Fl Tovey

    2001-01-01

    @@INTRODUCTION It is generally accepted that Helicobacter pylori ( H.pylori) infection has a role in duodenal ulceration .Eradicaton of H .pylori accelerates healing compared with placebo in the absence of control of gastric secretion and reduces ulcer recurrence .There is increasing evidence ,however ,that is may not be the primary cause of duodenal ulceration ,but that is may be a secondary factor in a nnmber of cases .This possibility is supported by four sets of observations : 1 Geographical distribution:

  2. Helicobacter pylori virulence factors in duodenal ulceration: A primary cause or a secondary infection causing chronicity

    Institute of Scientific and Technical Information of China (English)

    Frank I Tovey; Michael Hobsley; John Holton

    2006-01-01

    Reports from countries with a high prevalence of Helicobacter pylori (H pylori) infection do not show a proportionately high prevalence of duodenal ulceration,suggesting the possibility that H pylori cannot be a primary cause of duodenal ulceration. It has been mooted that this discrepancy might be explained by variations in the prevalence of virulence factors in different populations. The aim of this paper is to determine whether the published literature gives support to this possibility. The relevant literature was reviewed and analyzed separately for countries with a high and low prevalence of H pylori infection and virulence factors. Although virulent strains of H pylori were significantly more often present in patients with duodenal ulcer than without the disease in countries with a low prevalence of H pyloriinfection in the population, there was no difference in the prevalence of virulence factors between duodenal ulcer, non- ulcer dyspepsia or normal subjects in many countries, where the prevalence of both H pylori infection and of virulence factors was high.In these countries, the presence of virulence factors was not predictive the clinical outcome. To explain the association between virulence factors and duodenal ulcer in countries where H pylori prevalence is low,only two papers were found that give little support to the usual model proposed, namely that organisms with the virulence factors are more likely than those without them to initiate a duodenal ulcer. We offer an alternative hypothesis that suggests virulence factors are more likely to interfere with the healing of a previously produced ulcer. The presence of virulence factors only correlates with the prevalence of duodenal ulcer in countries where the prevalence of H pylori is low. There is very little evidence that virulence factors initiate duodenal ulceration, but they may be related to failure of the ulcer to heal.

  3. Dopamine enhances duodenal epithelial permeability via the dopamine D5 receptor in rodent.

    Science.gov (United States)

    Feng, X-Y; Zhang, D-N; Wang, Y-A; Fan, R-F; Hong, F; Zhang, Y; Li, Y; Zhu, J-X

    2017-05-01

    The intestinal barrier is made up of epithelial cells and intercellular junctional complexes to regulate epithelial ion transport and permeability. Dopamine (DA) is able to promote duodenal epithelial ion transport through D1-like receptors, which includes subtypes of D1 (D1 R) and D5 (D5 R), but whether D1-like receptors influence the duodenal permeability is unclear. FITC-dextran permeability, short-circuit current (ISC ), Western blot, immunohistochemistry and ELISA were used in human D5 R transgenic mice and hyperendogenous enteric DA (HEnD) rats in this study. Dopamine induced a downward deflection in ISC and an increase in FITC-dextran permeability of control rat duodenum, which were inhibited by the D1-like receptor antagonist, SCH-23390. However, DA decreased duodenal transepithelial resistance (TER), an effect also reversed by SCH-23390. A strong immunofluorescence signal for D5 R, but not D1 R, was observed in the duodenum of control rat. In human D5 R knock-in transgenic mice, duodenal mucosa displayed an increased basal ISC with high FITC-dextran permeability and decreased TER with a lowered expression of tight junction proteins, suggesting attenuated duodenal barrier function in these transgenic mice. D5 R knock-down transgenic mice manifested a decreased basal ISC with lowered FITC-dextran permeability. Moreover, an increased FITC-dextran permeability combined with decreased TER and tight junction protein expression in duodenal mucosa were also observed in HEnD rats. This study demonstrates, for the first time, that DA enhances duodenal permeability of control rat via D5 R, which provides new experimental and theoretical evidence for the influence of DA on duodenal epithelial barrier function. © 2016 Scandinavian Physiological Society. Published by John Wiley & Sons Ltd.

  4. Duodenal ileus caused by a calf feeding nipple in a cow

    OpenAIRE

    2011-01-01

    Abstract Background The aim of this report was to describe duodenal obstruction caused by a rubber foreign body in a cow. Case Presentation The clinical, biochemical and ultrasonographic findings in a five-year-old Swiss Braunvieh cow with duodenal ileus caused by a calf feeding nipple are described. The main clinical signs were anorexia, ruminal tympany, decreased faecal output and abomasal reflux syndrome. Ultrasonographic examination revealed reticular hyperactivity and a dilated duodenum....

  5. Divertículo duodenal perforado como causa de abdomen agudo quirúrgico Perforated duodenal diverticulum as cause of surgical acute abdomen

    Directory of Open Access Journals (Sweden)

    Nizahel Estévez Álvarez

    2011-12-01

    Full Text Available La enfermedad diverticular duodenal es considerada en el ámbito de la cirugía de vías digestivas como inusual. Por su parte, la complicación menos frecuente referida en la literatura médica lo constituye la perforación aguda, y cuando esta ocurre siempre se practicará tratamiento quirúrgico de urgencia. Resulta polémico llegar a un consenso general que dirija la metodología diagnóstica, debido a su atípica forma de presentación. La tomografía abdominal ha resultado de gran utilidad en el diagnóstico de esta entidad. Se reportan tasas de mortalidad altas (25-30 % originadas, generalmente, por la dificultad diagnóstica frente a esta enfermedad. Se presenta un nuevo caso de divertículo duodenal perforado en una paciente, del sexo femenino, de 60 años de edad. La técnica quirúrgica empleada en esta oportunidad consistió en la exclusión duodenal y el drenaje del retroperitoneo.The duodenal diverticular disease is considered within surgery of digestive tracts as unusual. The less frequent complication referred in medical literature is the acute perforation and when it occurs always will be carried out the emergency surgical treatment. It is polemic to arrive to a general consensus directing the diagnostic methodology due to its atypical way of presentation. The abdominal tomography has been very useful in diagnosis of this entity. Authors report high mortality rates (25-30 % in general originated by the diagnostic difficulty in face of this disease. A new case of perforated duodenal diverticulum of a female sex patient aged 60. The surgical technique used in this opportunity was the duodenal exclusion and the retroperitoneal drainage.

  6. Duodenal Wedge Resection for Large Gastrointestinal Stromal Tumour Presenting with Life-Threatening Haemorrhage

    Directory of Open Access Journals (Sweden)

    Alexander Shaw

    2013-01-01

    Full Text Available Background. Duodenal gastrointestinal stromal tumours (GISTs are an uncommon malignancy of the gastrointestinal (GI tract. We present a case of life-threatening haemorrhage caused by a large ulcerating duodenal GIST arising from the third part of the duodenum managed by a limited duodenal wedge resection. Case Presentation. A 61-year-old patient presented with acute life-threatening gastrointestinal bleeding. After oesophagogastroduodenoscopy failed to demonstrate the source of bleeding, a 5 cm ulcerating exophytic mass originating from the third part of the duodenum was identified at laparotomy. A successful limited wedge resection of the tumour mass was performed. Histopathology subsequently confirmed a duodenal GIST. The patient remained well at 12-month followup with no evidence of local recurrence or metastatic spread. Conclusion. Duodenal GISTs can present with life-threatening upper GI haemorrhage. In the context of acute haemorrhage, even relatively large duodenal GISTs can be treated by limited wedge resection. This is a preferable alternative to duodenopancreatectomy with lower morbidity and mortality but comparable oncological outcome.

  7. A brief analysis of patients suffering from stomach or duodenal ulcers in Almaty hospital №1

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    Aryzbekova Aliya

    2015-12-01

    Full Text Available Peptic ulcers are a serious problem worldwide, and affect about 4 million people each year. Their etiology is connected with the presence of Helicobacter pylori, the act of smoking, drinking alcohol, being stress, and taking excessively nonsteroidal anti-inflammatory drugs, as well as steroids. The most common symptoms are abdominal pain, nausea, chest pain and fatigue, while less frequent symptoms include vomiting and weight loss. Helicobacter pylori is responsible for about 80% of gastric and 90% of duodenal ulcer cases. In this work, an analysis is made of a correlation between stomach or duodenal ulcer and gender, residence and number of patients hospitalized in the Almaty hospital №1, from 2009-2012, in order to learn about trends in the incidence of these diseases in Kazakhstan. A total number of 950 patients with stomach and duodenal ulcers, in 2009-2012, were questioned. The patient’s residence, gender and stomach or duodenal ulcer problem were taken into account in the study. The result of this work reveals that the largest amount of hospitalized patients suffering from stomach or duodenal ulcers came from urban areas. Moreover, more women than men suffered from peptic ulcers. Furthermore, the number of patients admitted to the hospital due to duodenal ulcers did not show any variation throughout the study. However, the least number of patients suffering from gastric ulcers was noticed in December 2009, and the greatest was in October and November 2011. The obtained data show that ulcers are a serious problem in Kazakhstan.

  8. Successful mucosal incision-assisted biopsy for the histological diagnosis of duodenal lymphoma: A case report

    Science.gov (United States)

    MORISHITA, ASAHIRO; MORI, HIROHITO; KOBARA, HIDEKI; NISHIYAMA, NORIKO; FUJIHARA, SHINTARO; YACHIDA, TATSUO; AYAKI, MAKI; MATSUNAGA, TAE; SAKAMOTO, TEPPEI; MAEDA, EMIKO; NOMURA, TAKAKO; TANI, JOJI; MIYOSHI, HISAAKI; YONEYAMA, HIROHITO; HIMOTO, TAKASHI; KAGAWA, SEIKO; HABA, REIJI; MASAKI, TSUTOMU

    2016-01-01

    Tissue sampling of primary duodenal lymphoma is essential for its histological diagnosis. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), which is frequently used for submucosal tumor (SMT)-like duodenal tumors, is adequate for cytological diagnosis, but not for histological diagnosis. Therefore, in the present study, a mucosal incision-assisted biopsy (MIAB) was performed in an 81-year-old woman for the diagnosis of an SMT-like duodenal mass, as tissue sampling for histological analysis using a regular endoscopic biopsy had failed to establish a definite diagnosis of malignant lymphoma. EUS-FNA had also led to poor tissue sampling due to the difficult location of the duodenal tumor. The pathological examination of biopsy samples using MIAB revealed the presence of a diffuse proliferation of atypical lymphocytes, and the expression of cluster of differentiation (CD)20 and CD79a, but no expression of CD3 in the tumor specimens. The patient was diagnosed with diffuse large B-cell lymphoma. To the best of knowledge, this is first report of a case using MIAB as a sampling method for the histological diagnosis of SMT-like primary duodenal lymphoma. This case suggests that MIAB may be an essential method for obtaining tissue samples from SMT-like duodenal tumors. PMID:26870243

  9. Duodenal stenting for malignant gastric outlet obstruction:Prospective study

    Institute of Scientific and Technical Information of China (English)

    Eduardo Guimar(a)es Hourneaux Moura; Flávio Coelho Ferreira; Spencer Cheng; Diogo Turiani Hourneaux Moura; Paulo Sakai; Bruno Zilberstain

    2012-01-01

    AIM:To evaluate the results of duodenal stenting for palliation of gastroduodenal malignant obstruction by using a gastric outlet obstruction score (GOOS).METHODS:A prospective,non-randomized study was performed at a tertiary center between August 2005 and April 2010.Patients were eligible if they had malignant gastric outlet obstruction (GOO) and were not candidates for surgical treatment.Medical history and patient demographics were collected at baseline.Scheduled interviews were made on the day of the procedure and 15,30,90 and 180 d later or unscheduled as necessary.RESULTS:Fifteen patients (6 male,9 female; median age 61 years) with GOO who had undergone duodenal stenting were evaluated.Ten patients had metastasis at baseline (66.6%) and 14 were unable to accept oral intake (93.33%),including 7 patients who were using a feeding tube.Laboratory data showed biliary obstruction in eight cases (53.33%); all were submitted to biliary drainage.Two patients developed obstructive symptoms due to tumor ingrowth after 30 d and another due to tumor overgrowth after 180 d.Two cases of stent migration occurred.A good response to treatment was observed,with a mean time of approximately 1 d (19 h) until toleration of a liquid diet and slightly more than 2 d for both soft solids (51 h) and a solid food/normal diet (55 h).The mean time to first failure to maintain liquid intake (GOOS ≥ 1) was 93 d.During follow-up,the mean time to first failure to maintain the previously achieved GOOS of 2-3 (solid/semi-solid food),considered technical failure,was 71 d.On the basis of oral intake a GOOS is defined:0 for no oral intake; 1 for liquids only; 2 for soft solids only; 3 for lowresidue or full diet.CONCLUSION:Enteral stenting to alleviate gastroduodenal malignant obstruction improves quality of life in patients with limited life expectancy,which can be evaluated by using a GOO scoring system.

  10. Endoscopic and biopsy diagnoses of superficial, nonampullary, duodenal adenocarcinomas

    Science.gov (United States)

    Kakushima, Naomi; Kanemoto, Hideyuki; Sasaki, Keiko; Kawata, Noboru; Tanaka, Masaki; Takizawa, Kohei; Imai, Kenichiro; Hotta, Kinichi; Matsubayashi, Hiroyuki; Ono, Hiroyuki

    2015-01-01

    AIM: To investigate the accuracy of endoscopic or biopsy diagnoses of superficial nonampullary duodenal epithelial tumors (NADETs). METHODS: Clinicopathological data were reviewed for 84 superficial NADETs from 74 patients who underwent surgery or endoscopic resection between September 2002 and August 2014 at a single prefectural cancer center. Superficial NADETs were defined as lesions confined to the mucosa or submucosa. Demographic and clinicopathological data were retrieved from charts, endoscopic and pathologic reports. Endoscopic reports included endoscopic diagnosis, location, gross type, diameter, color, and presence or absence of biopsy. Endoscopic diagnoses were made by an endoscopist in charge of the examination before biopsy specimens were obtained. Endoscopic images were obtained using routine, front-view, high-resolution video endoscopy, and chromoendoscopy with indigocarmine was performed for all lesions. Endoscopic images were reviewed by at least two endoscopists to assess endoscopic findings indicative of carcinoma. Preoperative diagnoses based on endoscopy and biopsy findings were compared with histological diagnoses of resected specimens. Sensitivity, specificity, and accuracy were assessed for endoscopic diagnosis and biopsy diagnosis. RESULTS: The majority (81%) of the lesions were located in the second portion of the duodenum. The median lesion diameter was 14.5 mm according to final histology. Surgery was performed for 49 lesions from 39 patients, and 35 lesions from 35 patients were endoscopically resected. Final histology confirmed 65 carcinomas, 15 adenomas, and 3 hyperplasias. A final diagnosis of duodenal carcinoma was made for 91% (52/57) of the lesions diagnosed as carcinoma by endoscopy and 93% (42/45) of the lesions diagnosed as carcinoma by biopsy. The sensitivity, specificity, and accuracy of endoscopic diagnoses were 80%, 72%, and 78%, respectively, whereas those of biopsy diagnoses were 72%, 80%, and 74%, respectively

  11. Rare extra-adrenal paraganglioma mimicking a painful Schwannoma: case report.

    Science.gov (United States)

    Picchetto, Andrea; M Paganini, Alessandro; Balla, Andrea; Quaresima, Silvia; Cantisani, Vito; D'Ambrosio, Giancarlo; Lezoche, Emanuele

    2014-09-26

    I paragangliomi hanno un’incidenza annuale di circa 1/100000; essi originano dai tessuti cromaffini dei corpi di Zurckerkandl. In questo case report riportiamo il caso di un uomo di 53 anni, già trattato e seguito per un precedente limfoma di Hodkin che è stato sottoposta a RMN dell’addome per una lombalgia cronica che durava da due anni e che non riusciva ad alleviare con i FANS. La RMN ha rivelato un’incidentaloma: una massa in regione para-aortica, a livello della loggia surrenalica sinistra con le caratteristiche di una massa nodulare solida (29*25mm), che non sembrava originare direttamente dal surrene. Il pz ha inoltre eseguito un TC dell’addome che ha mostrato una massa localizzata di fronte al surrene di sinistra; il collega radiologo ha definito questa massa come un tumore maligno delle guaine nervose periferiche (Schwannoma) o come un linfonodo colliquato (essendo nota la patologia linfomatosa già descritta in anamnesi). Il paziente non riferiva cefalea, palpitazioni, picchi ipertensivi ne’ altri sintomi sistemici. Durante il suo ricovero, sono stati dosati i marker tumorali cromogranina e l’enolasi neurono-specifica che sono risultati essere rispettivamente 187.00 ng/mL and 7.7 ng/mL. La lombalgia è stata trattata inizialmente con Ketorolac, senza beneficio alcuno, quindi con Ketorolac più Tramadolo in pompa elastomerica, nuovamente senza beneficio per il paziente, infine con infusione continua di Morfina. Dato che l’esatta natura della neoformazione non era nota e che l’esecuzione di una biopsia TC guidata non era tecnicamente possibile, è stata eseguita un’escissione per via laparoscopica della massa, con approccio anteriore submesocolico trans peritoneale, così come gli Autori sono soliti eseguire per la surrenalectomia sinistra. Sin dai primi giorni postoperatori il paziente non ha più necessitato della morfina. Il decorso postoperatorio è stato del tutto regolare. L’esame istologico è risultato essere paraganglioma. I

  12. Symptomatic duodenal duplication cyst in an adult demonstrated by endoscopic retrograde cholangiopancreatography. Case report and literature review.

    Science.gov (United States)

    Luckmann, K F; Welch, R W; Schwesinger, W; Oswalt, C; Bannayan, G

    1979-08-01

    A patient with a symptomatic duodenal duplication cyst demonstrated by endoscopic retrograde cholangiopancreatography (ERCP) is presented. This lesion usually does not communicate with duodenal lumen but should be added to the list of cystic duodenal lesions demonstrable by ERCP. ERCP preoperatively is helpful to the surgeon in isolating adjacent duct structures but cyst wall histology is mandatory for correct anatomic diagnosis as this lesion has often been confused with Type III choledochal cysts.

  13. Effect of sucralfate on gastric emptying in duodenal ulcer patients

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    Petersen, J.M.; Caride, V.J.; Prokop, E.K.; Troncale, F.; McCallum, R.W.

    1985-05-01

    Duodenal ulcer (DU) patients may have accelerated gastric emptying (GE) suggesting that there is an increase in unbuffered gastric acid reaching the duodenum contributing to DU disease. Aluminum-containing antacids were shown to delay GE. The authors' aim was to investigate whether another aluminum-containing compound, Sucralfate, affects GE in normal and DU patients. Nine normal volunteers and 10 patients with documented DU disease were studied. For each test the subject ingested a meal composed of chicken liver Tc-99m-S-C mixed with beef stew and eaten with 4 oz. of water labelled with 100..mu..Ci of III-in-DTPA. On two separate days, subjects received 1 gram of Sucralfate (190 mg of aluminum per gram) or placebo in a randomized double-blind fashion one hour prior to the test meal. GE of liquids and solids in normal subjects was not significantly changed by Sucralfate. Sucralfate in the DU patients significantly slowed liquid emptying in the initial 40 min and solid food throughout the study compared to placebo (p<0.05). This paper summarizes that; GE of solids but not liquids is accelerated in DU patients compared to normal subjects; and sucralfate delays GE of both liquid and solid components of a meal in DU patients but has no effect on GE in normals. The authors conclude that a slowing of gastric emptying possibly mediated by aluminum ions, may be one mechanism by which Sucralfate enhances healing and decreases recurrence of DU.

  14. Formulation and evaluation of omeprazole tablets for duodenal ulcer

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    Choudhury A

    2010-01-01

    Full Text Available Omeprazole pellets containing mucoadhesive tablets were developed by direct punch method. Three mucoadhesive polymers namely hydroxypropylemethylcellulose K4M, sodium carboxy methylcellulose, carbopol-934P and ethyl cellulose were used for preparation of tablets which intended for prolong action may be due to the attachment with intestinal mucosa for relief from active duodenal ulcer. Mucoadhesive tablets were coated with respective polymer and coated with Eudragit L100 to fabricate enteric coated tablets. The prepared tablets were evaluated for different physical parameters and dissolution study were performed in three dissolution mediums like 0.1N hydrochloric acid for 2h, pH 6.5 and pH 7.8 phosphate buffer solution for 12hr. Sodium carboxymethylcellulose showed above 95% release within 10 h where as carbopol-934P showed slow release about 88% to 92% over a period of 12 h. having excellent mucoadhesive strength but ethyl cellulose containing tablets showed less than 65% release. The release mechanism of all formulation was diffusion controlled confirmed from Higuchi′s plot. Thus, the present study concluded that, carbopol-934P containing mucoadhesive tablets of omeprazole pellets can be used for local action in the ulcer disease as well as for oral controlled release drug delivery.

  15. Duodenal metastasis of pulmonary pleomorphic carcinoma: A case report

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    Jeong, Sun Hye; Paik, Sang Hyun; Koh, Eun Suk; Shin, Hwa Kyoon; Cha, Jang Gyu; Park, Jai Soung [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of); Lee, Nam Seok [Purun Radiologic Clinic, Yesan (Korea, Republic of)

    2016-02-15

    Pulmonary pleomorphic carcinoma is an uncommon malignant lesion of the lung. A chest radiograph of 53-year-old man who was suffering from a cough revealed a well-defined mass-like opacity with a broad base on the pleura at the apico-posterior segment of the right upper lobe of the lung. The subsequent chest computed tomography (CT) scan demonstrated an inhomogeneous enhancing mass with central low-attenuation in the right upper lobe. A lobectomy was performed and the mass was determined to be a pleomorphic carcinoma with visceral pleura invasion. Forty days after the operation, the patient complained of melena and an abdominal CT revealed an intraluminal and extraluminal protruding mass around the prepyloric antrum and duodenal bulb. The mass was removed by en-block surgery and diagnosed as metastatic pleomorphic carcinoma from the lung. Previous articles reported a median survival time of 3–10 months for pleomorphic carcinoma, but in this case, the patient has continued to survive, 11 years after surgery. Chest and abdominal CTs have revealed no evidence of tumor recurrence or metastasis.

  16. Primary duodenal adenocarcinoma: case report of an infrequent tumor

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    Óscar Moreno-Loaíza

    2013-10-01

    Full Text Available Introduction. Primary duodenal adenocarcinoma is an infrequent tumor both in our environment and in the world. There is no conclusive evidence on its epidemiology, diagnostic criteria, treatment or prognosis. Clinical case. We report a 77 year-old female patient, of mixed racial origin, native of Cusco (Peru who consulted for abdominal pain, weight loss, nausea, postprandial vomiting and bloating of three months course. At the time of examination she had second to third degree protein malnutrition with a BMI of 16.88 kg/m2, signs of moderate to severe chronic anemia and an 8 cm abdominal tumor in the epigastrium and right hypochondrium. The multislice spiral abdominal CT and ultrasonography revealed the presence of a solid tumor in the second portion of the duodenum. The patient was submitted to a gastroenterostomy without tumor resection. Biopsy confirmed tubular adenocarcinoma. Furthermore, no other primary tumors were found in the stomach, pancreas, biliary tree and colon. The patient was stabilized and was treated with 5-fluorouracil, irinotecan and leucovorin. Literature review. The article includes a brief review on the diagnosis, treatment and prognosis of this condition. Discussion. Management is not straightforward. There is little literature on the subject leaving decisions up to the attending physician’s criteria. We believe that all cases of rare diseases should be studied in depth, give rise to a thorough review of literature and, above all, be brought to the attention of the medical community.

  17. A STUDY OF DUODENAL ULCER PERFORATION: RISK FACTORS AND PROGNOSTIC DETERMINANTS IN BTGH, GULBARGA

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    Rajshekhar

    2015-11-01

    Full Text Available : BACKGROUND AND OBJECTIVES: Duodenal ulcer perforation is one of the acute abdominal emergencies in the surgical field. Duodenal ulcers are often caused due to imbalance between mucosal defences and acid /peptic injury. the cases of duodenal ulcer perforation in surgically treated patients were thoroughly studied with respect to trends in age, distribution of occurance, risk factors, seasonal variation, outcome of operative and non-operative modalities of treatment and factors influencing the prognosis of the disease. The current study summarizes epidemiology, risk factors, Pathophysiology, pathogenesis, clinical features, investigations, modalities of treatment and prognostic determinents of duodenal ulcer perforation in BTGH, Gulbarga. MATERIAL AND METHODS: The study was conducted in the Department of Surgery, Basaveshwara Teaching and General Hospital, Gulbarga Karnataka during the period of Nov. 2011 - Sep.2013. The diagnosis of duodenal ulcer perforation was that established by the admitting surgeon, based on clinical features and supposed by radiological evidence and confirm at operation. Surgery was defined as urgent less as 4 hours between admission and surgery, same day (4-24 hours and delayed at a later time during the same admission. This study comprises of 60 cases of duodenal ulcer perforation admitted in the Department of Surgery, Basaveshwar Teaching & General Hospital. Operative details included the site and nature of operation performed. Mortality was defined as death following surgical procedure. Post-operative morbidity was defined in terms of duration of hospital stay and associated complications following surgery. INCLUSION CRITERIA: All patients in whom a diagnosis of duodenal ulcer perforation was established on admission and confirmed by investigations between November 2011-September 2013 are included in this study. EXCLUSION CRITERIA: 1. Cases of gastric antral perforation. 2. Cases of traumatic duodenal perforation

  18. Repeated Radionuclide therapy in metastatic paraganglioma leading to the highest reported cumulative activity of 131I-MIBG

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    Ezziddin Samer

    2012-01-01

    Full Text Available Abstract 131I-MIBG therapy for neuroendocrine tumours may be dose limited. The common range of applied cumulative activities is 10-40 GBq. We report the uneventful cumulative administration of 111 GBq (= 3 Ci 131I-MIBG in a patient with metastatic paraganglioma. Ten courses of 131I-MIBG therapy were given within six years, accomplishing symptomatic, hormonal and tumour responses with no serious adverse effects. Chemotherapy with cisplatin/vinblastine/dacarbazine was the final treatment modality with temporary control of disease, but eventually the patient died of progression. The observed cumulative activity of 131I-MIBG represents the highest value reported to our knowledge, and even though 12.6 GBq of 90Y-DOTATOC were added intermediately, no associated relevant bone marrow, hepatic or other toxicity were observed. In an individual attempt to palliate metastatic disease high cumulative activity alone should not preclude the patient from repeat treatment.

  19. Targeting VEGF-VEGFR Pathway by Sunitinib in Peripheral Primitive Neuroectodermal Tumor, Paraganglioma and Epithelioid Hemangioendothelioma: Three Case Reports

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    Tiziana Prochilo

    2013-02-01

    Full Text Available Sunitinib malate (Sutent™; Pfizer Inc., New York, N.Y., USA is a small molecule kinase inhibitor with activity against a number of tyrosine kinase receptors, including vascular endothelial growth factor receptors, stem-cell factor receptor, and platelet-derived growth factor receptors alpha and beta. Sunitinib, registered for the treatment of renal cell carcinoma and gastrointestinal stromal tumors, has recently been approved for the treatment of patients with advanced pancreatic neuroendocrine tumors. Peripheral primitive neuroectodermal tumor (pPNET, paraganglioma (PGL and epithelioid hemangioendothelioma (EHE are rare tumors in which there is an overexpression of pro-angiogenic factors and in which a high intratumoral microvessel density is a significant poor prognostic factor. On the basis of this preclinical rationale and the lack of effective treatments in pre-treated advanced stages of these rare diseases, we report our interesting experience of pPNET, PGL and EHE treatment with sunitinib.

  20. A case report of a duodenal adenocarcinoma: a complication with Crohn's disease.

    Science.gov (United States)

    Sakakibara, Yuko; Yamada, Takuya; Kimura, Keiichi; Iwasaki, Ryuichirou; Iwasaki, Tetsuya; Ishihara, Akio; Nakazuru, Shoichi; Ishida, Hisashi; Kodama, Yoshinori; Mita, Eiji

    2016-03-01

    The prevalence of Crohn's disease (CD) in Japan is increasing, and so is the incidence of colorectal and small bowel cancers associated with CD. However, few reports have described the malignant transformation of duodenal lesions; moreover, such a diagnosis is rarely possible preoperatively. We present a case of malignant degeneration in the duodenal mucosa associated with CD. A 54-year-old man had been receiving treatment for CD for more than 20 years. Seven years ago, he was diagnosed with duodenal stenosis related to CD. He was asymptomatic, and biopsy results from the proximal stricture showed inflammatory changes without malignant transformation. The lesion was then monitored during follow-up. In 2013, he underwent an endoscopy, which revealed an ulcerated, nodular mucosa, immediately proximal to a high-grade obstruction of the descending duodenum. A biopsy of the ulcer lesion confirmed a diagnosis of adenocarcinoma. The patient then underwent duodenopancreatectomy. Histopathological results from the resected duodenum confirmed a poorly differentiated adenocarcinoma that had invaded the subserosa. The patient recovered, and no recurrence has been observed. Although the duodenum can be accessed without difficulty during endoscopy, it is challenging to preoperatively diagnose malignant transformation. There are only four reported cases of duodenal cancer stemming from CD-associated stricture, and only one of them received a preoperative diagnosis of malignancy based on endoscopic biopsy results. Progressive duodenal narrowing and ulceration in patients with CD should indicate a need for careful endoscopic evaluation and biopsy in order to exclude malignant degeneration of Crohn's duodenitis. Early diagnosis of cases of CD-associated cancers is necessary. We report the features of a rare and illustrative case of duodenal adenocarcinoma in a patient with CD.

  1. Congenital duodenal obstruction in neonates:a decade's experience from one center

    Institute of Scientific and Technical Information of China (English)

    Qing-Jiang Chen; Zhi-Gang Gao; Jin-Fa Tou; Yun-Zhong Qian; Min-Ju Li; Qi-Xing Xiong; Qiang Shu

    2014-01-01

    Background: Congenital duodenal obstruction (CDO) is one of the most common anomalies in newborns, and accounting for nearly half of all cases of neonatal intestinal obstruction. This study aimed to review our single-center experience in managing congenital duodenal obstruction while evaluate the outcomes. Methods: We conducted a retrospective analysis of the records of all neonates dianogsed with congenital duodenal obstruction admitted to our center between January 2003 and December 2012. We analyzed demographic criteria, clinical manifestations, associated anomalies, radiologic findings, surgical methods, postoperative complications, and fi nal outcomes. Results: The study comprised 287 newborns (193 boys and 94 girls). Birth weight ranged from 950 g to 4850 g. Fifty-three patients were born prematurely between 28 and 36 weeks' gestation. Malrotation was diagnosed in 174 patients, annular pancreas in 66, duodenal web in 55, duodenal atresia or stenosis in 9, preduodenal portal vein in 2, and congenital band compression in 1. Twenty patients had various combinations of these conditions. Presenting symptoms included bilious vomiting, dehydration, and weight loss. X-rays of the upper abdomen demonstrated the presence of a typical doublebubble sign or air-fl uid levels in 68.64% of patients, and confi rmatory upper and/or lower gastrointestinal contrast studies were obtained in 64.11%. Multiple associated abnormalities were observed in 50.52% of the patients. Various surgical approaches were used, including Ladd's procedure, duodenoplasty, duodenoduodenostomy, duodenojejunostomy, or a combination of these. Seventeen patients died postoperatively and 14 required re-operation. Conclusions: Congenital duodenal obstruction is a complex entity with various etiologies and often includes multiple concomitant disorders. Timely diagnosis and aggressive surgery are key to improving prognosis. Care should be taken to address all of the causes of duodenal obstruction and

  2. Long-term risk of gastrointestinal cancers in persons with gastric or duodenal ulcers.

    Science.gov (United States)

    Søgaard, Kirstine K; Farkas, Dóra K; Pedersen, Lars; Lund, Jennifer L; Thomsen, Reimar W; Sørensen, Henrik T

    2016-06-01

    Peptic ulcer predicts gastric cancer. It is controversial if peptic ulcers predict other gastrointestinal cancers, potentially related to Helicobacter pylori or shared lifestyle factors. We hypothesized that gastric and duodenal ulcers may have different impact on the risk of gastrointestinal cancers. In a nationwide cohort study using Danish medical databases 1994-2013, we quantified the risk of gastric and other gastrointestinal cancers among patients with duodenal ulcers (dominantly H. pylori-related) and gastric ulcers (dominantly lifestyle-related) compared with the general population. We started follow-up 1-year after ulcer diagnosis to avoid detection bias and calculated absolute risks of cancer and standardized incidence ratios (SIRs). We identified 54,565 patients with gastric ulcers and 38,576 patients with duodenal ulcers. Patient characteristics were similar in the two cohorts. The 1-5-year risk of any gastrointestinal cancer was slightly higher for gastric ulcers patients (2.1%) than for duodenal ulcers patients (2.0%), and SIRs were 1.38 (95% CI: 1.31-1.44) and 1.30 (95% CI: 1.23-1.37), respectively. The SIR of gastric cancer was higher among patients with gastric ulcer than duodenal ulcer (1.92 vs. 1.38), while the SIRs for other gastrointestinal cancers were similar (1.33 vs. 1.29). Compared with gastric ulcer patients, duodenal ulcer patients were at lower risk of smoking- and alcohol-related gastrointestinal cancers. The risk of nongastric gastrointestinal cancers is increased both for patients with gastric ulcers and with duodenal ulcers, but absolute risks are low. H. pylori may be less important for the development of nongastric gastrointestinal cancer than hypothesized.

  3. Observation and partial targeted surgery in the management of tympano-jugular paraganglioma: a contribution to the multioptional treatment.

    Science.gov (United States)

    Mazzoni, Antonio; Zanoletti, Elisabetta

    2016-03-01

    The objective of this study was to assess the value of a limited tumor excision in tympano-jugular paragangliomas treated with observation with the goal of preventing, or repairing, a neural (VII cranial nerve) or brain damage and continue an otherwise correct observation. This is a retrospective case review. Each patient was submitted to a complete neuro-radiological work-up for diagnosis including CT, MRI, and angiography if needed of tympano-jugular paraganglioma class C. All the cases were submitted to observation and in 16 cases partial targeted surgery was performed. In 10 cases, the procedure involved a targeted removal of the tumor growing in contact with, or invading, the Fallopius. In four cases, the procedure was a petro-occipital trans-sigmoid approach to remove the intradural portion of tumor producing the picture of brain stem compression. In two cases, there was the excision of the bleeding tumor surfaces in the outer ear canal. Follow-up ranged from 4 to 20 years. Success was considered the maintenance of observation without the change of class of tumor extension. In 13 over 16 cases, the observation could be continued, as it still was the more favorable option between natural and surgical morbidity. In the other three cases, a newly installed paralysis of the 9-10 cranial nerves due to tumor growth involved a correction of the strategy to a radical procedure. Partial targeted surgery was directed to cases submitted to observation. It allowed to prevent, or repair, an impending, or actual damage to the facial nerve or the brain stem and to continue the abstentional treatment by keeping the balance between natural and therapy morbidity in favor of observation.

  4. [Hypoplasia of internal carotid artery associated with carotid paraganglioma. A case report and review of the literature].

    Science.gov (United States)

    Ruiz-Mercado, Héctor; Maldonado-Hernández, Héctor; Cruz-González, Juan Antonio

    2014-01-01

    Antecedentes: la hipoplasia de la arteria carótida interna es una rara malformación congénita. Se le ha relacionado principalmente con aneurismas y otros padecimientos, pero no con paraganglioma. Su incidencia es menor de 0.01% de todas las anomalías de los vasos carotídeos. Aunque su origen exacto no se conoce, se cree que es una secuela de una lesión provocada por causas mecánicas o por estrés hemodinámico; sin embargo, también pudieran estar implicados aspectos de biología molecular del desarrollo embrionario. Caso clínico: se describe un caso raro de una mujer de 37 años de edad con paraganglioma concomitante con hipoplasia de la arteria carótida interna. En estudios angiográficos y análisis rutinarios previos no se había detectado alteración de la arteria carótida interna. En la intervención se extirpó el tumor y se ligó la arteria carótida externa dado que estaba involucrada en la lesión; se dejó intacta la arteria carótida interna hipoplásica. Conclusión: es importante realizar estudios angiográficos de la base del cráneo, así como análisis hemodinámicos para no pasar por alto estas anomalías. La evolución fue satisfactoria y sin secuelas.

  5. Pre-operative ⁶⁸Ga-DOTANOC somatostatin receptor PET/CT imaging demonstrating multiple synchronous lesions in a patient with head and neck paraganglioma.

    Science.gov (United States)

    Naswa, N; Karunanithi, S; Sharma, P; Soundararajan, R; Bal, C; Kumar, R

    2014-01-01

    Paragangliomas, or glomus tumors, are neoplasms arising from extra-adrenal chromaffin tissue. They frequently cause symptoms by over-production of catecholamines with known predilection to multicentricity. We describe the case of a patient with bilateral carotid body tumor who underwent a preoperative ⁶⁸Gallium labeled [1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid]-1-NaI3-Octreotide (⁶⁸Ga-DOTANOC) positron emission tomography/computed tomography (PET/CT) imaging for staging. This is a unique case in which multiple paraganglioma and pheochromocytoma were demonstrated in a single patient using ⁶⁸Ga-DOTANOC PET/CT. Copyright © 2013 Elsevier España, S.L.U. and SEMNIM. All rights reserved.

  6. Iodine-131 metaiodobenzylguanidine (I-131 MIBG) diagnosis and therapy of pheochromocytoma and paraganglioma: current problems, critical issues and presentation of a sample case.

    Science.gov (United States)

    Castellani, M R; Aktolun, C; Buzzoni, R; Seregni, E; Chiesa, C; Maccauro, M; Aliberti, G L; Vellani, C; Lorenzoni, A; Bombardieri, E

    2013-06-01

    Iodine-131 metaiodobenzylguanidine (I-131 MIBG) has been used for the diagnosis and treatment of malignant pheochromocytomas (PHEO) and paragangliomas (PGL) since 1980's. Despite increasing amount of experience with iodine-131 (I-131) MIBG therapy, many important questions still exist. In this article, we will discuss the current problems learned from clinical experience in diagnosis and therapy of PHEO/PGL with I-131 MIBG, and present a sample case to emphasize the critical aspects for an optimal treatment strategy.

  7. Robotic-assisted repair of a duodenal diaphragm in a child

    Directory of Open Access Journals (Sweden)

    Jennifer H. Myszewski

    2015-08-01

    Full Text Available The retroperitoneal location of the duodenum and the small volume of the peritoneal cavity in pediatric patients make the laparoscopic repair of congenital duodenal anomalies challenging. As a result, robotic-assisted repair of duodenal atresia in a pediatric patient has been reported only once in the literature. This report describes the robotic-assisted laparoscopic repair of a congenital duodenal diaphragm in a 2-year-old, 8 kg patient who presented with vomiting and failure to thrive. An upper gastrointestinal series revealed partial obstruction at the second part of the duodenum with proximal dilatation. These findings are consistent with a duodenal diaphragm. Traditional laparoscopy was utilized to kocherize the first and second parts of the duodenum and to identify a loop of proximal jejunum for the proposed anastomosis. A duodeno-jejunal anastomosis was then performed using intra-corporeal suturing with a daVinci SI robotic system. The patient had a quick and uneventful post-operative course. At 6 month follow-up, she was asymptomatic and the surgical incisions had healed with excellent cosmetic appearance. A combination of laparoscopic and robotic techniques offers a promising alternative to open or purely laparoscopic repair of congenital duodenal anomalies.

  8. Intramural Duodenal Haematoma after Endoscopic Biopsy: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Claudia Grasshof

    2012-01-01

    Full Text Available The development of intramural duodenal haematoma (IDH after small bowel biopsy is an unusual lesion and has only been reported in 18 children. Coagulopathy, thrombocytopenia and some special features of duodenal anatomy, e.g. relatively fixed position in the retroperitoneum and numerous submucosal blood vessels, have been suggested as a cause for IDH. The typical clinical presentation of IDH is severe abdominal pain and vomiting due to duodenal obstruction. In addition, it is often associated with pancreatitis and cholestasis. Diagnosis is confirmed using imaging techniques such as ultrasound, magnetic resonance imaging or computed tomography and upper intestinal series. Once diagnosis is confirmed and intestinal perforation excluded, conservative treatment with nasogastric tube and parenteral nutrition is sufficient. We present a case of massive IDH following endoscopic grasp forceps biopsy in a 5-year-old girl without bleeding disorder or other risk for IDH, which caused duodenal obstruction and mild pancreatitis and resolved within 2 weeks of conservative management. Since duodenal biopsies have become the common way to evaluate children or adults for suspected enteropathy, the occurrence of this complication is likely to increase. In conclusion, the review of the literature points out the risk for IDH especially in children with a history of bone marrow transplantation or leukaemia.

  9. Endoscopic resection of duodenal bulb neuroendocrine tumor larger than 10 mm in diameter

    Directory of Open Access Journals (Sweden)

    Naka Teiji

    2011-06-01

    Full Text Available Abstract Background Endoscopic treatment for duodenal bulb neuroendocrine tumor larger than 10 mm is still controversial. This report presents four cases successfully treated with endosonography (EUS-assisted endoscopic mucosal resection (EMR procedure for duodenal bulb neuroendocrine tumor larger than 10 mm in diameter. Methods The case series of four patients diagnosed with neuroendocrine tumor from 2003 to 2008 were reviewed. EUS demonstrated well-defined hypoechoic tumors confined to the submucosal hyperechoic layer and the underlying hypoechoic muscularis propria was intact in all four patients. EMR were planned and performed for the duodenal bulb neuroendocrine tumors larger than 10 mm. Results En bloc resections with tumor free lateral and basal margins were accomplished using an endoscopic diathermic snare with forward-viewing instruments without any complications. Neither residual duodenal neuroendocrine tumors nor metastatic lesions were detected during the observation period ranging 19 to 78 months Conclusion Duodenal bulb neuroendocrine, larger than 10 mm in diameter, can be treated by endoscopic procedure, after confirming that the tumor confined to the submucosal layer in EUS examination, and no lymph node involvement by abdominal CT and US.

  10. Endoscopic Division of Duodenal Web Causing Near Obstruction in 2-Year-Old with Trisomy 21.

    Science.gov (United States)

    Wood, Lauren S Y; Kastenberg, Zachary; Sinclair, Tiffany; Chao, Stephanie; Wall, James K

    2016-05-01

    Surgical intervention for duodenal atresia most commonly entails duodenoduodenostomy in the neonatal period. Occasionally, type I duodenal atresia with incomplete obstruction may go undiagnosed until later in life. Endoscopic approach to dividing intestinal webs has been reported as successful in patients as young as 7 days of age, and can be a useful modality particularly in patients with comorbidities who may not tolerate open or laparoscopic surgery. A 2-year-old female with a history of trisomy 21 and tetralogy of Fallot underwent laparoscopic and endoscopic exploration of intestinal obstruction as seen on upper gastrointestinal series for symptoms of recurrent emesis and weight loss. After laparoscopy confirmed a duodenal web as the cause of intestinal obstruction, endoscopic division of the membrane was carried out with a triangle tip electrocautery knife and 15 mm radially dilating balloon. The patient tolerated the procedure well, and also tolerated full age-appropriate diet by time of discharge on postoperative day 2. She remains asymptomatic as of 6 months postoperatively. This report describes a successful endoscopic approach for definitive treatment of a duodenal web in a 2-year-old girl with trisomy 21, and laparoscopy confirmed no intraabdominal obstructive process or complication from endoscopy. Endoscopy enables minimal recovery time and suggests an improved method of duodenal web division over pure surgical intervention.

  11. A Newly Designed Stent for Management of Malignant Distal Duodenal Stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Zhou, Wei-Zhong, E-mail: xmjbq007@163.com; Yang, Zheng-Qiang, E-mail: ntdoctoryang@hotmail.com; Liu, Sheng, E-mail: liusheng1137@sina.com; Zhou, Chun-Gao, E-mail: zhouchungao@aliyun.com; Xia, Jin-Guo, E-mail: docjinguoxia@hotmail.com; Zhao, Lin-Bo, E-mail: linberzhao@hotmail.com; Shi, Hai-Bin, E-mail: shihb@njmu.edu.cn [The First Affiliated Hospital of Nanjing Medical University, Department of Interventional Radiology (China)

    2015-02-15

    PurposeTo evaluate the clinical effectiveness of a newly designed stent for the treatment of malignant distal duodenal stenosis.MethodsFrom March 2011 to May 2013, six patients with malignant duodenal stenosis underwent fluoroscopically guided placement of the new duodenal stent consisting of braided, nested stent wires, and a delivery system with a metallic mesh inner layer. Primary diseases were pancreatic cancer in three patients, gastric cancer in two patients, and endometrial stromal sarcoma in one patient. Duodenal obstructions were located in the horizontal part in two patients, the ascending part in two patients, and the duodenojejunal flexure in two patients. Technical success, defined as the successful stent deployment, clinical symptoms before and after the procedure, and complications were evaluated.ResultsTechnical success was achieved in all patients. No major complications were observed. Before treatment, two patients could not take any food and the gastric outlet obstruction scoring system (GOOSS) score was 0; the other four patients could take only liquids orally (GOOSS score = 1). After treatment, five patients could take soft food (GOOSS score = 2) and one patient could take a full diet (GOOSS score = 3). The mean duration of primary stent patency was 115.7 days.ConclusionsThe newly designed stent is associated with a high degree of technical success and good clinical outcome and may be clinically effective in the management of malignant distal duodenal obstruction.

  12. Duodenal ileus caused by a calf feeding nipple in a cow

    Directory of Open Access Journals (Sweden)

    Gerspach Christian

    2011-01-01

    Full Text Available Abstract Background The aim of this report was to describe duodenal obstruction caused by a rubber foreign body in a cow. Case Presentation The clinical, biochemical and ultrasonographic findings in a five-year-old Swiss Braunvieh cow with duodenal ileus caused by a calf feeding nipple are described. The main clinical signs were anorexia, ruminal tympany, decreased faecal output and abomasal reflux syndrome. Ultrasonographic examination revealed reticular hyperactivity and a dilated duodenum. A diagnosis of duodenal ileus was made and the cow underwent right-flank laparotomy, which revealed a dilation of the cranial part of the duodenum because of obstruction by a pliable foreign body. This was identified via enterotomy as a calf feeding nipple. The cow was healthy at the time of discharge four days after surgery and went on to complete a successful lactation. Conclusions To our knowledge, this is the first description of duodenal obstruction by a calf feeding nipple. This is an interesting case, which broadens the spectrum of the causes of duodenal ileus, which is usually caused by obstruction of the duodenum by a phytobezoar.

  13. Pancreas duodenal homeobox-1 expression and significance in pancreatic cancer

    Science.gov (United States)

    Liu, Tao; Gou, Shan-Miao; Wang, Chun-You; Wu, He-Shui; Xiong, Jiong-Xin; Zhou, Feng

    2007-01-01

    AIM: To study the correlations of Pancreas duodenal homeobox-1 with pancreatic cancer characteristics, including pathological grading, TNM grading, tumor metastasis and tumor cell proliferation. METHODS: Reverse transcriptase-polymerase chain reaction (RT-PCR) was used to detect PDX-1 mRNA expression in pancreatic cancer tissue and normal pancreatic tissue. The expression of PDX-1 protein was measured by Western blot and immunohistochemistry. Immunohistochemistry was also used to detect proliferative cell nuclear antigen (PCNA). Correlations of PDX-1 with pancreatic cancer characteristics, including pathological grading, TNM grading, tumor metastasis and tumor cell proliferation, were analyzed by using χ2 test. RESULTS: Immunohistochemistry showed that 41.1% of pancreatic cancers were positive for PDX-1 expression, but normal pancreatic tissue except islets showed no staining for PDX-1. In consistent with the result of imunohistochemistry, Western blot showed that 37.5% of pancreatic cancers were positive for PDX-1. RT-PCR showed that PDX-1 expression was significantly higher in pancreatic cancer tissues than normal pancreatic tissues (2-3.56 ± 0.35 vs 2-8.76 ± 0.14, P < 0.01). Lymph node metastasis (P < 0.01), TNM grading (P < 0.05), pathological grading (P < 0.05) and tumor cell proliferation (P < 0.01) were significantly correlated with PDX-1 expression levels. CONCLUSION: PDX-1 is re-expressed in pancreatic cancer, and PDX-1-positive pancreatic cancer cells show more malignant potential compared to PDX-1-negative cells. Therefore, PDX-1-positive cells may be tumor stem cells and PDX-1 may act as alternate surface marker of pancreatic cancer stem cells. PMID:17552012

  14. Laparoscopic duodenoduodenostomy with parallel anastomosis for duodenal atresia.

    Science.gov (United States)

    Oh, Chaeyoun; Lee, Sanghoon; Lee, Suk-Koo; Seo, Jeong-Meen

    2017-06-01

    Currently, a diamond-shaped anastomosis is preferred for the surgical repair of duodenal atresia (DA) in both open and laparoscopic surgery. We report the results of laparoscopic duodenoduodenostomy with parallel anastomosis (LDPA) in DA. We retrospectively reviewed 22 patients who underwent laparoscopic duodenoduodenostomy from February 2005 to May 2015 in Samsung Medical Center. All patients underwent operation within the first month after birth. Patients who were transversely anastomosed after duodenotomy and patients who underwent simultaneous operation on combined anomalies were excluded. Parallel anastomosis was used in all surgeries. Four trocars were used in laparoscopic repair. After mobilization of both proximal and distal ends, the proximal end was incised transversely and the distal end was incised longitudinally. Duodenoduodenostomy with parallel anastomosis using a 5-0 glyconate monofilament was performed with interrupted sutures. Eleven patients (50 %) were male. Median gestational age was 36 + 6 weeks (32 + 7-40 + 6). Median age at the time of operation and median body weight were 3 days (1-12) and 2.53 kg (1.63-3.18), respectively. All patients were diagnosed prenatally and 16 patients (72.7 %) had associated anomalies. Median operation time was 142 min (96-290) and median postoperative day to start oral feeding was 5 days (3-9) and median postoperative day of reaching full feeding was 11 days (6-19). Median postoperative day was 13 days (10-60). There was no anastomotic leakage or stenosis. Median follow up was 3.5 months (1-21). Currently, there is no late complication. LDPA can be performed easily to patients who have DA in neonatal period. It is anatomically natural and the risk of leakage or stenosis does not seem significant. Therefore, parallel anastomosis should be considered as a safe procedural option for laparoscopic duodenoduodenostomy in DA.

  15. Pancreas duodenal homeobox-1 expression and significance in pancreatic cancer

    Institute of Scientific and Technical Information of China (English)

    Tao Liu; Shan-Miao Gou; Chun-You Wang; He-Shui Wu; Jiong-Xin Xiong; Feng Zhou

    2007-01-01

    AIM: To study the correlations of Pancreas duodenal homeobox-1 with pancreatic cancer characteristics,incluling pathological grading, TNM grading, tumor metastasis and tumor cell proliferation.METHODS: Reverse transcriptase-polymerase chain reaction (RT-PCR) was used to detect PDX-1 mRNA expression in pancreatic cancer tissue and normal pancreatic tissue. The expression of PDX-1 protein was measured by Western blot and immunohistochemistry.Immunohistochemistry was also used to detect proliferative cell nuclear antigen (PCNA). Correlations of PDX-1 with pancreatic cancer characteristics, including pathological grading, TNM grading, tumor metastasis and tumor cell proliferation, were analyzed by using χ2 test.RESULTS: Immunohistochemistry showed that 41.1% of pancreatic cancers were positive for PDX-1 expression,but normal pancreatic tissue except islets showed no staining for PDX-1. In consistent with the result of imunohistochemistry, Western blot showed that 37.5% of pancreatic cancers were positive for PDX-1. RT-PCR showed that PDX-1 expression was significantly higher in pancreatic cancer tissues than normal pancreatic tissues (2-3.56 ± 0.35 vs 2-8.76 ± 0.14, P< 0.01). Lymph node metastasis (P < 0.01), TNM grading (P < 0.05), pathological grading (P < 0.05) and tumor cell proliferation (P < 0.01) were significantly correlated with PDX-1 expression levels.CONCLUSION: PDX-1 is re-expressed in pancreatic cancer, and PDX-1-positive pancreatic cancer cells show more malignant potential compared to PDX-1-negative cells. Therefore, PDX-1-positive cells may be tumor stem cells and PDX-1 may act as alternate surface marker of pancreatic cancer stem cells.

  16. Precise role of H pylori in duodenal ulceration

    Institute of Scientific and Technical Information of China (English)

    Michael Hobsley; Frank I Tovey; John Holton

    2006-01-01

    The facts that H pylori infection is commoner in duodenal ulcer (DU) patients than in the normal population, and that eradication results in most cases being cured,have led to the belief that it causes DU. However, early cases of DU are less likely than established ones to be infected. H pylori-negative cases are usually ascribed to specific associated factors such as non-steroidal anti-inflammatory drugs (NSAIDs), Crohn's disease,and hypergastrinaemia, but even after excluding these, several H pylori-negative cases remain and are particularly common in areas of low prevalence of H pylori infection. Moreover, this incidence of H pylori negative DU is not associated with a fall in overall DU prevalence when compared with countries with a higher H pylori prevalence. In countries with a high H pylori prevalence there are regional differences in DU prevalence, but no evidence of an overall higher prevalence of DU than in countries with a low H pylori prevalence. There is no evidence that virulence factors are predictive of clinical outcome. After healing following eradication of H pylori infection DU can still recur.Medical or surgical measures to reduce acid output can lead to long-term healing despite persistence of H pylori infection. Up to half of cases of acute DU perforation are H pylori negative. These findings lead to the conclusion that H pylori infection does not itself cause DU, but leads to resistance to healing, i.e., chronicity. This conclusion is shown not to be incompatible with the universally high prevalence of DU compared with controls.

  17. Multifocal Head and Neck Paraganglioma Evaluated with Different PET Tracers: Comparison Between Fluorine-18-Fluorodeoxyglucose Between Fluorine-18-Fluorodeoxyglucose and Gallium-68-Somatostatin Receptor PET/CT

    Energy Technology Data Exchange (ETDEWEB)

    Castaldi, Paola; Rufini, Vittoria [Catholic Univ. of the Sacred Heart, Rome (Italy); Treglia, G. [Oncology Institute of Southern Switzerland, Bellinzona (Switzerland)

    2013-09-15

    We report the case of a 46-year-old woman with a succinate dehydrogenase subtype D (SDHD) gene mutation and multifocal head and neck paraganglioma evaluated with fluorine-18-fluorodeoxyglucose and gallium-68-somatostatin receptor positron emission tomography/computed tomography (PET/CT). Gallium-68-somatostatin receptor PET/CT correctly assessed the extent of the disease in this patient, detecting additional lesions compared with fluorine-18-fluorodeoxyglucose PET/CT and influencing the patient management. A 46-year-old woman was referred to our centre for surveillance of a gastrointestinal stromal tumour (GIST). The patient underwent fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (F-18-FDG-PET/CT), which demonstrated a focal area of increased radiopharmaceutical uptake corresponding to a lesion located between the right carotid vessels (yellow arrow; Fig. 1a). This F-18-FDG-PET/CT finding was suspicious for a paraganglioma of the neck. The patient underwent further examinations, including biochemical and genetic tests and a somatostatin receptor PET/CT using somatostatin analogues labelled with gallium-68 (Ga-68-DOTANOC-PET/CT). Laboratory data were suspicious for a non-functioning neuroendocrine tumour. Increased serum chromogranin A value and normal values of plasmatic and urinary catecholamines and their metabolites were found. The patient had no symptoms of a functioning tumour. Genetic tests demonstrated the presence of a succinate dehydrogenase subtype D (SDHD) gene mutation, which is associated with head and neck paragangliomas. Surprisingly, Ga-68-DOTANOC-PET/CT (Fig. 1b) showed multiple bilateral areas of increased radiopharmaceutical uptake in the head and neck region, corresponding to bilateral neuroendocrine lesions and suggesting the presence of bilateral paragangliomas (yellow arrows) with small cervical lymph nodal metastases with short axis less than 1 cm of diameter (red arrows). Physiological radiopharmaceutical

  18. The challenges of treating paraganglioma patients with {sup 177}Lu-DOTATATE PRRT: Catecholamine crises, tumor lysis syndrome and the need for modification of treatment protocols

    Energy Technology Data Exchange (ETDEWEB)

    Makis, William; Mccann, Karey; Mcewan, Alexander J. B. [Dept. of Diagnostic Imaging, Cross Cancer Institute, Alberta (China)

    2015-09-15

    A high percentage of paragangliomas express somatostatin receptors that can be utilized for targeted radioisotope therapy. The aim of this study was to describe and discuss the challenges of treating these tumors with {sup 177}Lu-[DOTA0,Tyr3]octreotate (DOTATATE) radioisotope therapy using established protocols. Three paraganglioma patients were treated with 4–5 cycles of {sup 177}Lu-DOTATATE and were evaluated for side effects and response to therapy. Two of the three patients developed severe adverse reactions following their first {sup 177}Lu-DOTATATE treatment. One patient developed a catecholamine crisis and tumor lysis syndrome within hours of treatment, requiring intensive care unit (ICU) support, and another developed a catecholamine crisis 3 days after treatment, requiring hospitalization. The treatment protocols at our institution were subsequently modified by increasing the radioisotope infusion time from 15 to 30 min, as recommended in the literature, to 2–4 h and by reducing the administered dose of {sup 177}Lu-DOTATATE. Subsequent {sup 177}Lu-DOTATATE treatments utilizing the modified protocols were well tolerated, and response to therapy was achieved in all three patients, resulting in significantly improved quality of life. {sup 177}Lu-DOTATATE is an exciting new therapeutic option in the management of paragangliomas; however, current treatment protocols described in the literature may need to be modified by lengthening the infusion time and/or lowering the initial treatment dose to prevent or reduce the severity of adverse reactions.

  19. Treatment and follow-up of a case of bleeding duodenal varix

    Directory of Open Access Journals (Sweden)

    Viveksandeep Thoguluva Chandrasekar

    2013-01-01

    Full Text Available Duodenal varices (DV are rare in patients with portal hypertension secondary to liver disease. Their tendency to bleed is less common than in gastroesophageal varices, but can sometimes produce a life-threatening bleed. They are often difficult to diagnose and treat. We present a case of a 35-year-old man with parenchymal liver disease admitted with complaints of hematemesis and melena. Upper gastrointestinal endoscopy was performed and a duodenal varix, with stigmata of a recent bleed, was noted in the second part of the duodenum. Five milliliters of N-butyl-2-cyanoacrylate glue was injected into the varix leading to obliteration. A follow-up study with an endoscopic ultrasound and repeat endoscopy showed near total obturation of the varix and success of the therapy. This report concludes that glue injection can effectively be used as a first-line treatment for bleeding duodenal varices.

  20. The Helicobacter pylori theory and duodenal ulcer disease. A case study of the research process

    DEFF Research Database (Denmark)

    Christensen, A H; Gjørup, T

    1995-01-01

    OBJECTIVES: To describe the medical research process from the time of the generation of a new theory to its implementation in clinical practice. The Helicobacter pylori (H. pylori) theory, i.e. the theory that H. pylori plays a significant causal role in duodenal ulcer disease was chosen as a case....... MATERIAL: Abstracts from 1984 to 1993, identified in the CD-Rom, Medline system, ("Silverplatter"), using the search terms Campylobacter pylori and Helicobacter pylori, and reviews and editorials about H. pylori in some of the most widespread clinical journals. RESULTS: 2204 papers on H. pylori were....... pylori in duodenal ulcer disease had been published in some of the most widespread clinical journals. In half of the papers the authors were convinced of the causal role of H. pylori in duodenal ulcer disease, while in the remainder they were sceptical. In seven cases the authors stated which patients...

  1. A Rare Case of Solitary Peutz Jeghers Type Hamartomatous Duodenal Polyp with Dysplasia!

    Science.gov (United States)

    Rathi, Chetan Devendra; Solanke, Dattatray Balasaheb; Kabra, Nikita Lalitkumar; Ingle, Meghraj Ananda; Sawant, Prabha Dilip

    2016-07-01

    Solitary Peutz Jeghers (SPJ) type hamartomatous polyp is a rare and separate entity from classic Peutz Jeghers syndrome (PJS). A hamartomatous polyp without associated mucocutaneous pigmentations, any other gastrointestinal polyp or a family history of PJS is diagnosed as a SPJ type polyp. We described a case of 22-year-old young man in whom solitary duodenal polyp was incidentally detected and resected. Histopathological examination revealed PJ type hamartomatous polyp with foci of adenomatous and moderate dysplastic change. Very few cases of solitary duodenal PJ type hamartomatous polyps with malignant transformation have been reported. They can be treated with endoscopic or surgical resection. Endoscopic ultrasound can give an idea about the depth of involvement. This is a rare case of duodenal solitary PJ polyp with dysplasia in a young patient.

  2. Gardner’s syndrome presenting as duodenal carcinoma in a young male

    Directory of Open Access Journals (Sweden)

    Sarma YS

    2015-10-01

    Full Text Available Gardners syndrome (GS is a variant of familial adenomatous polyposis (FAP and presents with both colonic and extra colonic manifestations. It is an autosomal dominant disorder and results from mutations in adenomatous polyposis coli (APC gene. Patients with GS if not treated early will invariably develop colonic cancers at a much younger age than those with sporadic colonic carcinoma. These patients also develop other malignant tumours like duodenal cancers, gastric cancer, hepatoblastoma, papillary carcinoma of the thyroid and multifocal cholangiocarcinomas. With early diagnosis and treatment of colonic polyposis, adenocarcinoma of the duodenum has become the leading cause of death in FAP patients. The mean age at which duodenal carcinoma is diagnosed in FAP is 45-52 years. We report the rare occurrence of duodenal carcinoma as the presenting feature of Gardner’s syndrome in a young 25-year-old male with no obvious malignant changes in the colonic adenomas.

  3. [THE REMOTE RESULTS OF SIMULTANEOUS LAPAROSCOPIC CORRECTION OF CHRONIC DUODENAL OBSTRICTION AND CHOLECYSTECTOMY IN CHOLELITHIASIS].

    Science.gov (United States)

    Isayev, H; Hachverdiyev, B

    2016-05-01

    The aim of the research was to investigate the remote results of surgical treatment of 75 patients with cholelithiasis combined with chronic duodenal obstruction. Control group was composed of 40 patients who underwent laparoscopic cholecystectomy. Compensated stage of cholelithiasis with chronic duodenal obstruction was detected in 16 (21.3%) patients, subcompensated in 37 (49.3%) and decompensated stage in 17 (22.7%) patients. In 14 patients (18.7%) with cholelithiasis combined with chronic duodenal obstruction laparoscopic cholecystectomy was conducted due to the positive results of preoperative conservative treatment. In the long-term quality of life after surgery in the main group of patients were average 35.4% higher than in the control group; in the main group postcholecystectomical syndrome was diagnosed in one case (2,1%) and in 13 (32,2%) cases in the control group.

  4. Perforated duodenal ulcer in high risk patients: Is percutaneous drainage justified?

    Directory of Open Access Journals (Sweden)

    Aly Saber

    2012-01-01

    Full Text Available Background: Conservative treatment was recommended as the treatment of choice in perforated acute peptic ulcer. Here, we adjunct percutaneous peritoneal drainage with nonoperative conservative treatment in high risk elderly patients with perforated duodenal ulcer. Aim: The work was to study the efficacy of percutaneous peritoneal drainage under local anesthesia supported by conservative measures in high risk elderly patients, according to the American Society of Anesthesiologists grading, with perforated duodenal ulcer. Patients and Methods: Twenty four high risk patients with age >65 years having associated medical illness with evidence of perforated duodenal ulcer. Results: The overall morbidity and mortality were comparable with those treated by conservative measures alone. Conclusion: In high risk patients with perforated peptic ulcer and established peritonitis, percutaneous peritoneal drainage under local anesthesia seems to be effective with least operative trauma and mortality rate.

  5. Oral administration of synthetic human urogastrone promotes healing of chronic duodenal ulcers in rats

    DEFF Research Database (Denmark)

    Poulsen, Steen Seier; Nexø, Ebba

    1986-01-01

    effective than cimetidine alone. These results show that a combination of an agent inhibiting gastric acid secretion and the cytoprotective and growth-stimulating peptide EGF/URO seems to be more effective with regard to duodenal ulcer healing than individual administration of the two substances. Synthetic......The effect of oral administration of synthetic human epidermal growth factor/urogastrone (EGF/URO) on healing of chronic duodenal ulcers induced by cysteamine in rats was investigated and compared with that of cimetidine, a H2-receptor antagonist. After 25 and 50 days of treatment, synthetic human...... EGF/URO significantly increased healing of chronic duodenal ulcers to the same extent as cimetidine. Combined treatment with synthetic human EGF/URO and cimetidine for 25 days was more effective than synthetic human EGF/URO given alone, whereas combined treatment for 50 days was significantly more...

  6. Acidic duodenal pH alters gene expression in the cystic fibrosis mouse pancreas.

    Science.gov (United States)

    Kaur, Simran; Norkina, Oxana; Ziemer, Donna; Samuelson, Linda C; De Lisle, Robert C

    2004-08-01

    The duodenum is abnormally acidic in cystic fibrosis (CF) due to decreased bicarbonate ion secretion that is dependent on the CF gene product CFTR. In the CFTR null mouse, the acidic duodenum results in increased signaling from the intestine to the exocrine pancreas in an attempt to stimulate pancreatic bicarbonate ion secretion. Excess stimulation is proposed to add to the stress/inflammation of the pancreas in CF. DNA microarray analysis of the CF mouse revealed altered pancreatic gene expression characteristic of stress/inflammation. When the duodenal pH was corrected genetically (crossing CFTR null with gastrin null mice) or pharmacologically (use of the proton pump inhibitor omeprazole), expression levels of genes measured by quantitative RT-PCR were significantly normalized. It is concluded that the acidic duodenal pH in CF contributes to the stress on the exocrine pancreas and that normalizing duodenal pH reduces this stress.

  7. Predicting Chemically Induced Duodenal Ulcer and Adrenal Necrosis with Classification Trees

    Science.gov (United States)

    Giampaolo, Casimiro; Gray, Andrew T.; Olshen, Richard A.; Szabo, Sandor

    1991-07-01

    Binary tree-structured statistical classification algorithms and properties of 56 model alkyl nucleophiles were brought to bear on two problems of experimental pharmacology and toxicology. Each rat of a learning sample of 745 was administered one compound and autopsied to determine the presence of duodenal ulcer or adrenal hemorrhagic necrosis. The cited statistical classification schemes were then applied to these outcomes and 67 features of the compounds to ascertain those characteristics that are associated with biologic activity. For predicting duodenal ulceration, dipole moment, melting point, and solubility in octanol are particularly important, while for predicting adrenal necrosis, important features include the number of sulfhydryl groups and double bonds. These methods may constitute inexpensive but powerful ways to screen untested compounds for possible organ-specific toxicity. Mechanisms for the etiology and pathogenesis of the duodenal and adrenal lesions are suggested, as are additional avenues for drug design.

  8. Duodenal Duplication Cyst: A Rare Differential Diagnosis in a Neonate with Bilious Vomiting

    Science.gov (United States)

    Župančić, Božidar; Gliha, Andro; Fuenzalida, Jose Varas; Višnjić, Stjepan

    2015-01-01

    Bilious vomiting is a relevant sign in neonates that requires immediate evaluation and diagnosis. A duplication of the intestinal tract is a possible cause of obstruction if located distally to the major duodenal papilla of Vater and most of them involve the jejunum, stomach, or colon. Duodenal duplications are very rare and can have an endoscopic or surgical treatment after diagnosis. We present a case of a 16-day-old term newborn that consulted because of bilious vomiting and after evaluation with imaging and upper endoscopy, a duodenal duplication cyst was found at the level of the third portion causing compression of the intestinal lumen that required surgical resolution with duodenocystostomy. PMID:26788454

  9. [Morphometric characteristics of fundal glands of the stomach in duodenal ulcer and pre-ulcer condition].

    Science.gov (United States)

    Uspenskiĭ, V M

    1978-01-01

    Biopsy material was used for morphometric study of the fundal glands in 114 patients with duodenal ulcers, 177 patients with various forms of gastroduodenal pathology but with typical signs of duodenal ulcer, and in 21 normal subjects. Three variants of the increase in the amount of secretory elements of the fundal glands were found: owing to an increase in the number of main cells (first variant), main and parietal cells (second variant), and parietal cells (third variant). These changes in the fundal glands are not specific for ulcer and pre-ulcer conditions, they are observed in normal subjects with constitutional hypersecretion of hydrochloric acid and pepsin. The patients with duodenal ulcer and pre-ulcer conditions are characterized by "hyper-pepsinogenic shift" in the epithelial formula of the fundal gland (45% and 31-38%, respectively) owing to an increase in the number of main cells.

  10. Usefulness of biopsying the major duodenal papilla to diagnose autoimmune pancreatitis: A prospective study using TgG4-immunostaining

    Institute of Scientific and Technical Information of China (English)

    Terumi Kamisawa; Yuyang Tu; Hitoshi Nakajima; Naoto Egawa; Kouji Tsuruta; Atsutake Okamoto

    2006-01-01

    AIM: To examine the histological and immunohistochemical findings of biopsy specimens taken from the major duodenal papilla of autoirnrnune pancreatitis (AIP)patients.METHODS: The major duodenal papilla in the resected pancreas of 3 patients with AIP and of 5 control patients [pancreatic carcinoma (n = 3) and chronic alcoholic pancreatitis (n = 2)] was irnrnunostained using anti-CD4-T cell, CD8-T cell and IgG4 antibodies. Forceps biopsy specimens taken from the major duodenal papilla of 2patients with AIP and 5 control patients with suspected papillitis were prospectively taken during duodenoscopy and immunohistochernically examined.RESULTS: Moderate or severe lyrnphoplasrnacytic infiltration including many CD4-positive or CD8-positive T lymphocytes and IgG4-positive plasma cells (≥10/HPF),was observed in the major duodenal papilla of all 3 patients with AIP. The same findings were also detected in the biopsy specimens taken from the major duodenal papilla of 2 patients with AIP, but in controls, there were only a few (≤3/HPF) IgG4-positive plasma cells infiltrating the major duodenal papilla.CONCLUSIONS: An abundant infiltration of IgG4-positive plasma cells is specifically detected in the major duodenal papilla of patients with AIP. Although this is a preliminary study, IgG4-irnmunostaining of biopsy specimens taken from the major duodenal papilla may support the diagnosis of AIP.

  11. Lower Bifidobacteria counts in both duodenal mucosa-associated and fecal microbiota in irritable bowel syndrome patients

    NARCIS (Netherlands)

    Kerckhoffs, Angele P. M.; Samsom, Melvin; van der Rest, Michel E.; de Vogel, Joris; Knol, Jan; Ben-Amor, Kaouther; Akkermans, Louis M. A.

    2009-01-01

    AIM: To determine the composition of both fecal and duodenal mucosa-associated microbiota in irritable bowel syndrome (IBS) patients and healthy subjects using molecular-based techniques. METHODS: Fecal and duodenal mucosa brush samples were obtained from 41 IBS patients and 26 healthy subjects. Fec

  12. Ursodeoxycholic acid counteracts celecoxib in reduction of duodenal polyps in patients with familial adenomatous polyposis : a multicentre, randomized controlled trial

    NARCIS (Netherlands)

    van Heumen, Bjorn W. H.; Roelofs, Hennie M. J.; Vink-Borger, M. Elisa; Dekker, Evelien; Mathus-Vliegen, Elisabeth M. H.; Dees, Jan; Koornstra, Jan J.; Langers, Alexandra M. J.; Nagtegaal, Iris D.; Kampman, Ellen; Peters, Wilbert H. M.; Nagengast, Fokko M.

    2013-01-01

    Background: Due to prophylactic colectomy, mortality in patients with familial adenomatous polyposis (FAP) has changed, with duodenal cancer currently being the main cause of death. Although celecoxib reduces duodenal polyp density in patients with FAP, its long-term use may increase the risk of car

  13. Ursodeoxycholic acid counteracts celecoxib in reduction of duodenal polyps in patients with familial adenomatous polyposis: a multicentre, randomized controlled trial

    NARCIS (Netherlands)

    Heumen, van B.W.; Roelofs, H.M.J.; Vink-Börger, M.E.; Dekker, E.; Mathus-Vliegen, E.M.; Dees, J.; Koornstra, J.J.; Langers, A.M.; Nagtegaal, I.D.; Kampman, E.; Peters, W.H.; Nagengast, F.M.

    2013-01-01

    Background Due to prophylactic colectomy, mortality in patients with familial adenomatous polyposis (FAP) has changed, with duodenal cancer currently being the main cause of death. Although celecoxib reduces duodenal polyp density in patients with FAP, its long-term use may increase the risk of card

  14. Acute duodenal Crohn's disease successfully managed with low-speed elemental diet infusion via nasogastric tube: A case report

    Institute of Scientific and Technical Information of China (English)

    Takayuki Yamamoto; Maki Nakahigashi; Satoru Umegae; Tatsushi Kitagawa; Koichi Matsumoto

    2006-01-01

    Duodenal Crohn's disease is rare, and patients without obstruction are treated medically. We herein report one case whose duodenal Crohn's disease was successfully managed with low-speed elemental diet infusion through a nasogastric tube. A 28-year-old female developed acute duodenal Crohn's disease. Upper GI radiologic and endoscopic examinations showed a stricture in the duodenal bulb. Using the duodenal biopsy specimens,mucosal cytokine levels were measured; interleukin (IL)-1β, IL-6, IL-8, and tumor necrosis factor-α levels were remarkably elevated. For initial 2 wk, powdered mesalazine was orally given but it was not effective. For the next 2 wk, she was treated with low-speed elemental diet therapy using a commercially available ElentalTM,which was infused continuously through a nasogastric tube using an infusion pump. The tip of the nasogastric tube was placed at an immediate oral side of the pylorus.The infusion speed was 10 mL/h (usual speed, 100 mL/h).After the 2-wk treatment, her symptoms were very much improved, and endoscopically, the duodenal stricture and inflammation improved. The duodenal mucosal cytokine levels remarkably decreased compared with those before the treatment. Although our experience was limited, lowspeed elemental diet infusion through a nasogastric tube may be a useful treatment for acute duodenal Crohn's disease.

  15. Autonomic nervous activity before and after eradication of Helicobacter pylori in patients with chronic duodenal ulcer.

    Science.gov (United States)

    Katoh, K; Nomura, M; Nakaya, Y; Iga, A; Nada, T; Hiasa, A; Ochi, Y; Kawaguchi, R; Uemura, N; Honda, H; Shimizu, I; Ito, S

    2002-04-01

    Helicobacter pylori infection is involved in the formation of chronic peptic ulcer. However, a previously reported hypothesis concerning the involvement of central autonomic nervous disorder in this condition cannot be ruled out. To use spectrum analysis of heart rate viability to examine autonomic nervous activity before and after H. pylori eradication. Twenty patients with chronic duodenal ulcer (duodenal ulcer group) and 20 age-matched normal adults (N group). In both groups, 24-h Holter electrocardiograms (ECGs) were recorded and spectrum analysis of heartrate variability was performed. In the duodenal ulcer group, Holter ECG was recorded before and after H. pylori eradication. In the N group, analysis of heart rate variability showed that high frequency (HF) power, an index of parasympathetic activity, was high at night, while the low frequency (LF)/HF ratio, an index of sympathetic function, was high during the daytime. In the duodenal ulcer group, HF power was higher at night than during the daytime, showing a similar pattern to the N group, but the power value was higher than in the N group (P < 0.05). In the duodenal ulcer group, LF/HF at night was significantly higher than that of the N group. In addition, in the duodenal ulcer group, autonomic activity after H. pylori eradication did not differ significantly from that before H. pylori eradication. In patients with chronic peptic ulcer, both sympatheticotonia and parasympatheticotonia may occur at night, and this abnormality in autonomic nervous activity may cause increased gastric acid secretion and gastric mucosal vasoconstriction. Abnormalities in autonomic activity persist even after H. pylori eradication, suggesting that they may be an independent risk factor in the formation of chronic peptic ulcer in addition to H. pylori infection.

  16. Duodenal Bulb Mucosa with Hypertrophic Gastric Oxyntic Heterotopia in Patients with Zollinger Ellison Syndrome

    Directory of Open Access Journals (Sweden)

    Emil Kohan

    2009-01-01

    Full Text Available Objectives. Zollinger-Ellison Syndrome (ZES results in hypersecretion of gastric acid (via gastrinoma leading to peptic ulcers, diarrhea, and abdominal pain. We describe the novel discovery of hypertrophic, heterotopic gastric mucosa in the proximal duodenal bulb in patients with ZES, which we hypothesize results in an increased incidence of postbulbar ulcers in patients with ZES (a mechanism previously unreported. We determined the incidence of the novel finding of duodenal gastric oxyntic hypertrophic heterotopia (GOH in patients with ZES. Methods. Seven patients with ZES were enrolled. The diagnosis of ZES was established by hypergastrinemia, gastric acid hypersecretion, and a positive secretin test or based on biopsy specimens (evaluated via tissue staining. Basal acid output (BAO and baseline gastrin secretion were determined by established methods. Endoscopic examinations with methylene blue staining and biopsy of the gastric and duodenal mucosa were conducted in all patients every 3–6 months for an average of 5 years. Results. The duodenal mucosa demonstrated hypertrophic GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. Biopsies from the bowel mucosa demonstrated patchy replacement of surface epithelium by gastric-type epithelium with hypertrophic oxyntic glands in the lamina propria in 5 patients. Two of the patients had no evidence of GOH in the duodenal bulb. Patients with GOH had an average serum gastrin level of 1245 pg/mL and BAO of 2.92 mEq/hr versus 724 pg/mL and 0.8 mEq/hr in patients without GOH. Conclusions. This study demonstrated the presence of duodenal mucosa with GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. The presence of hypertrophic and heterotopic gastric mucosa is proposed to result from increased gastrin levels and may contribute to the increased incidence of postbulbar ulcers in these patients.

  17. Ectopic opening of the common bile duct and duodenal stenosis: an overlooked association

    Directory of Open Access Journals (Sweden)

    Şaşmaz Nurgül

    2010-12-01

    Full Text Available Abstract Background Ectopic opening of the common bile duct into the duodenal bulb (EO-CBD-DB is a rare disease that may be complicated by duodenal ulcer, deformity, stenosis and biliary stones. The aim of this study is to report clinical presentations, endoscopic diagnosis and treatment of this entity as well as to investigate its association with duodenal stenosis. Methods Gastroduodenoscopic findings and radiological imaging were evaluated for ectopic papilla and duodenal stenosis. Diagnostic methods, endoscopic procedures and long-term outcomes of the endoscopic treatment were presented. Results EO-CBD-DB was found in 74 (77.1% of the 96 patients with duodenal deformity/stenosis (79 male, 17 female, mean age: 58.5, range: 30-87 years. The papilla with normal appearance was retracted to the bulb in 11 while it was at its usual location in the remaining 11. The history of biliodigestive surgery was more common in patients with EO-CBD-DB who were frequently presented with the common bile duct stone-related symptoms than the other patients. Thirteen (17.6% of the patients with EO-CBD-DB were referred to surgery. Endoscopic treatment was completed in 60 (81.1% patients after an average of 1.7 (range: 1-6 procedures. These patients were on follow-up for 24.8 (range: 2-46 months. Endoscopic intervention was required in 12 (20% of them because of recurrent biliary problems. Treatment of the patient who had stricture due to biliary injury during laparoscopic cholecystectomy is still continued. Conclusions The presence of EO-CBD-DB should be considered particularly in middle-aged male patients who have duodenal deformity/stenosis. Endoscopic treatment is feasible in these patients. The long-term outcomes of endoscopic therapy need to be compared with surgical treatment.

  18. Small duodenal carcinoids: a case series comparing endoscopic resection and autoamputation with band ligation.

    Science.gov (United States)

    Scherer, John R; Holinga, Julie; Sanders, Michael; Chennat, Jennifer; Khalid, Asif; Fasanella, Kenneth; Singhi, Aatur D; McGrath, Kevin

    2015-04-01

    We sought to compare the efficacy and safety of endoscopic ultrasound-guided endoscopic resection (ER) and endoscopic band ligation (EBL) for autoamputation of small duodenal carcinoids. The ideal management of small duodenal carcinoid tumors remains unclear. A retrospective review of duodenal carcinoids over a 10-year period (2002 to 2012) was performed at our tertiary-care teaching hospital. All patients with duodenal carcinoids ≤10 mm in size treated with either ER or EBL were included. The main outcome measurements were the efficacy and safety of endotherapy. A total of 37 patients with 39 subcentimeter duodenal carcinoids were identified. In the EBL group, the mean (SD) tumor size was 6.7±2.1 mm compared with 6.7±1.7 mm in the ER group (P=0.943). The mean Ki-67 index was ≤2% in specimens available for histologic analysis in both groups (16/23 EBL and 15/16 ER). The positive deep margin rate in the ER group was 68.8%. Residual carcinoid tumor cells were detected on follow-up biopsies in 1 patient after EBL, and 2 patients after ER. All underwent subsequent successful endotherapy. No adverse events occurred in the EBL group compared with an 18.8% adverse event rate in the ER group (P=0.066). Endoscopic ultrasound-guided EBL is a safe, effective method for removal of small superficial duodenal carcinoids and seems to be a lower risk alternative to conventional ER with cautery.

  19. Transarterial embolotherapy in patients with duodenal hemorrhage using microcoils and gelfoam particles

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Tae Beom [School of Medicine, Donga Univ., Busan (Korea, Republic of); Kim, Young Hwan; Seong, Chang Kyu [School of Medicine, Kyeongpook National Univ., Daegu (Korea, Republic of)] [and others

    2004-07-01

    To assess the efficacy and safety of arterial embolotherapy in patients with massive duodenal hemorrhage. Between January 1999 and June 2002, 25 patients (age: 34-81, mean 58, male: 19, female: 6) underwent arterial embolization for duodenal hemorrhage after failed endoscopic therapy. The hemorrhage originated from duodenal ulcer in sixteen patients, from cancer with duodenal invasion in five patients, from endoscopic sphincterectomy in two patients, and from pseudoaneurysm complicating acute pancreatitis in two patients. Hemorrhage was detected at endoscopy and an attempt was made to treat it endoscopically in all patients, but failed in each case. At angiography, direct bleeding signs such as contrast extravasation or pseudoaneurysm were demonstrated in nineteen patients. In the six patients without angiographic evidence of bleeding, blind embolization of the gastroduodenal artery was performed based on the endoscopic examination. Microcoil and gelfoam particles were used as embolic agents. Hemostasis was achieved immediately after embolotherapy in 21 patients (84%). Bleeding recurred in 4 patients (16%), and of these cases, one was successfully treated purely by endoscopic means, a second was reembolized three times due to bleeding from the collateral vessels of the tumor and the two others were treated by surgery. After the procedure, six patients died (24%). The causes of death were disseminated intravascular coagulopathy, multiorgan failure, sepsis and acute renal failure. The underlying diseases of the deceased patients were cancers with duodenal invasion (n=4) and abdominal aortic aneurysm with ischemic colitis (n=1). Transarterial embolotherapy in the case of massive duodenal hemorrhage is a safe and effective procedure. Even in the absence of angiographic evidence of bleeding, blind embolization of the gastroduodenal artery is effective for patients in the surgically high risk group.

  20. Association of transjugular intrahepatic portosystemic shunt with embolization in the treatment of bleeding duodenal varix refractory to sclerotherapy.

    Science.gov (United States)

    Illuminati, G; Smail, A; Azoulay, D; Castaing, D; Bismuth, H

    2000-01-01

    Bleeding from duodenal varices are often severe (mortality as high as 40%), and more difficult to sclerose than esophageal varices. We report a patient with a bleeding duodenal varix, refractory to sclerotherapy, successfully treated by the association of portosystemic shunt placement and varix embolization, via the same transjugular intrahepatic route. A 40-year-old Black male underwent emergency TIPS and duodenal varix embolization after failure of endoscopic sclerotherapy. The portosystemic pressure gradient droped from 16 to 9 mm Hg following TIPS. At 5 months from TIPS, the patient is well, with a patent shunt at Doppler ultrasound. The present report of successful control of duodenal varix, actively bleeding and refractory to sclerotherapy, by means of combined TIPS and embolization, supports the role of TIPS and suggests that its association to embolization can be valuably considered in the difficult setting of portal hypertension with bleeding duodenal varices. Copyright 2000 S. Karger AG, Basel

  1. MR Cholangiography and Dynamic Examination of Duodenal Fluid in the Differential Diagnosis between Extrahepatic Biliary Atresia and Infantile Hepatitis Syndrome

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    In order to evaluate the value of magnetic resonance cholangiography (MRC) and dynamic examination of duodenal fluid in the differential diagnosis between extrahepatic biliary atresia (EHBA) and infantile hepatitis syndrome (IHS), 52 pa tients with infantile cholestatic jaundice were examined by MRC and duodenal fluid examination. Original interpretations were compared with clinical outcome. Calculated sensitivity of duodenal fluid examination in diagnosis of EHBA was 100%, and specificity was 91.1%. Sensitivity of MRC in the diagnosis of EHBA was 94.4 % and specificity 88.24 %. The sensitivity of MRC and examination of duodenal fluid combined in diagnosis of EHBA was 94.4 % and specificity 97.06 %. We are led to conclude that MRC and dynamic examination of duodenal fluid are useful in the differential diagnosis between IHS and EHBA and the combined use of the two techniques yield better resutls.

  2. Duodenal variceal bleed: an unusual cause of upper gastrointestinal bleed and a difficult diagnosis to make.

    Science.gov (United States)

    Bhagani, Shradha; Winters, Conchubhair; Moreea, Sulleman

    2017-02-27

    We present a case of recurrent upper gastrointestinal (GI) bleeding in a man aged 57 years with primary biliary cholangitis who was ultimately diagnosed with an isolated duodenal variceal bleed, which was successfully treated with histoacryl glue injection. Duodenal varices are an uncommon presentation of portal hypertension and can result in significant GI bleeding with a high mortality. Diagnosis can be difficult and therapeutic options limited. Endoscopic variceal sclerotherapy with histoacryl glue provides an effective treatment, though endoscopists need to remain aware of and vigilant for the serious complications of this treatment option. 2017 BMJ Publishing Group Ltd.

  3. Conservative management of perforated duodenal diverticulum: A case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    David Martínez-Cecilia; Sebástian Rufián-Pe(n)a; Alvaro Arjona-Sánchez; Manuel Gómez-(A)lvarez; Eva Torres-Tordera; Antonio Luque-Molina; Victor Valentí-Azcárate; Javier Brice(n)o-Delgado; Francisco-Javier Padillo; Pedro López-Cillero

    2008-01-01

    Duodenal diverticula are a relatively common condition.They are asymptomatic, unless they become complicated, with perforation being the rarest but most severe complication. Surgical treatment is the most frequently performed approach. We report the case of a patient with a perforated duodenal diverticulum, which was diagnosed early and treated conservatively with antibiotics and percutaneous drainage of secondary retroperitoneal abscesses. We suggest this method could be an acceptable option for the management of similar cases, provided that the patient is in good general condition and without septic signs.

  4. Aneurysm of the Superior Posterior Pancreatic-Duodenal Artery Presenting with Recurrent Syncopes

    Directory of Open Access Journals (Sweden)

    I.A.J. van Doesburg

    2009-08-01

    Full Text Available We present a 61-year-old woman with hypovolemic shock due to a ruptured aneurysm of the superior posterior pancreatic-duodenal artery in whom recurrent syncopes were the first presenting sign of pancreatic-duodenal artery aneurysm (PDAA. PDAA is a rare but life-threatening condition. The widely varying symptomatology may lead to a delay in diagnosis and treatment. Patients with atypical symptoms, such as vague abdominal pain, recurrent dizziness or syncope, may actually suffer from a sentinel bleeding of the vascular malformation. Radiological imaging, especially selective angiography, may provide a diagnostic as well as a therapeutic tool in these patients.

  5. Treatment of a duodenal perforation secondary to an endoscopic sphincterotomy with clips

    Institute of Scientific and Technical Information of China (English)

    Panagiotis Katsinelos; George Paroutoglou; Basilios Papaziogas; Athanasios Beltsis; Stavros Dimiropoulos; Konstantinos Atmatzidis

    2005-01-01

    Perforation is one of the most serious complications of endoscopic sphincterotomy (ES) necessitating immediate surgical intervention. We present a case of successful management of such a complication with endoclipping. A85-year-old woman developed duodenal perforation after ES. The perforation was identified early and its closure was achieved using three metallic clips in a single session.There was no procedure-related morbidity or complications and our patient was discharged from hospital 10 d later.Endoclipping of duodenal perforation induced by ES is a safe, effective and alternative to surgery treatment.

  6. A Child with Severe Malaria Presenting with Acute Surgical Abdomen (Duodenal Perforation

    Directory of Open Access Journals (Sweden)

    Tika Ram Bhandari

    2016-01-01

    Full Text Available Plasmodium falciparum, the commonest cause of severe malaria in children, is an important cause of mortality in developing nations like Nepal. Duodenal perforation in a case of complicated malaria, although a rare entity, can occur in children. Early diagnosis, proper medical treatment, and early surgical repair can be a lifesaving measure in such cases. Here, we report a case of a 5-year-old male child with falciparum malaria complicated by a duodenal perforation that was successively managed with appropriate antimalarial drugs and early surgical repair.

  7. A Child with Severe Malaria Presenting with Acute Surgical Abdomen (Duodenal Perforation).

    Science.gov (United States)

    Bhandari, Tika Ram; Shahi, Sudha; Poudel, Rajesh; Chaudhary, Nagendra

    2016-01-01

    Plasmodium falciparum, the commonest cause of severe malaria in children, is an important cause of mortality in developing nations like Nepal. Duodenal perforation in a case of complicated malaria, although a rare entity, can occur in children. Early diagnosis, proper medical treatment, and early surgical repair can be a lifesaving measure in such cases. Here, we report a case of a 5-year-old male child with falciparum malaria complicated by a duodenal perforation that was successively managed with appropriate antimalarial drugs and early surgical repair.

  8. Duodenal application of Li+ in a submaximal therapeutic dose inhibits exocrine pancreatic secretion and modulates gastro-duodenal myoelectrical activity in a conscious pig model

    DEFF Research Database (Denmark)

    Naughton, Violetta; Hedemann, Mette Skou; Naughton, Patrick Joseph

    2013-01-01

    for electromyography of smooth muscles, and with a pancreatic duct catheter and a duodenal T-cannula for collection and re-entrant flow of pancreatic juice. After the recovery period, on alternative days, each animal was tested once with an intraduodenal infusion of Li+ (100 mmol·L–1 C3H5LiO3, 10 mL·kg−1·h−1) for 1 h......This study tested whether duodenal application of lithium inhibits gastroduodenal motility, and whether it suppresses secretion from the exocrine pancreas. Five suckling pigs, 16–18 days old, were surgically fitted with 3 serosal electrodes on the wall of the gastric antrum and the duodenum...

  9. Obstrução duodenal congênita do tipo veia porta pré-duodenal em recém-nascido

    Directory of Open Access Journals (Sweden)

    Willy Marcus França

    2016-10-01

    Full Text Available Introdução: A obstrução duodenal congênita manifesta-se tipicamente no período intrauterino e neonatal. Todavia, uma apresentação mais tardia e diversa pode ocorrer nos raros casos de veia porta pré-duodenal (VPPD. A identificação de achados radiológicos, como o sinal de dupla bolha, é determinante para a hipótese diagnóstica de oclusão ou sub-oclusão duodenal e a utilização de ultrassom com Doppler pode auxiliar na suspeita de obstrução duodenal tipo veia porta. Entretanto, o diagnóstico final da causa do quadro obstrutivo só pode ser confirmado mediante ao procedimento de laparotomia exploratória; Objetivos: Destacar o tratamento realizado, a evolução e o prognóstico do caso reportado de VPPD em recém-nascido; Metodologia: Acompanhamento clínico e cirúrgico, baseado na análise de prontuário do recém-nascido, incluindo registro fotográfico dos métodos diagnósticos e procedimentos cirúrgicos, com suporte de uma ampla revisão da literatura; Relato de caso: RN do sexo feminino, de gestação e parto sem complicações, apresentou, ao nascer, 55 ml de conteúdo gástrico após aspiração e exame físico sem alterações. Paciente evoluiu com vômitos após mamadas e significativa perda de peso, sendo prescrita nutrição parenteral, jejum e sonda orogástrica aberta. Foi realizado RX de trânsito intestinal contrastado, apresentando o sinal de dupla bolha, sendo indicada laparotomia exploratória, com achados de VPPD. Paciente evoluiu bem no pós- operatório, iniciando dieta enteral e posterior evacuação. Conclusões: O caso relatado traz à luz a discussão da terapêutica de VPPD associada à doença sub- oclusiva duodenal, e evidencia que é possível obter resultados satisfatórios e duradouros no que diz respeito ao alívio sintomático e melhoria da qualidade de vida.

  10. Fluctuating functions related to quality of life in advanced Parkinson disease: effects of duodenal levodopa infusion

    DEFF Research Database (Denmark)

    Isacson, D; Bingefors, K; Sønbø Kristiansen, Ivar

    2008-01-01

    OBJECTIVE: To assess fluctuations in quality of life (QoL) and motor performance in patients with advanced Parkinson disease (PD) treated with continuous daytime duodenal levodopa/carbidopa infusion or conventional therapy. METHODS: Of 18 patients completing a 6-week trial (DIREQT), 12 were...

  11. Peripheral neuropathy in Parkinson's disease : Levodopa exposure and implications for duodenal delivery [Universally Available

    NARCIS (Netherlands)

    Mueller, Thomas; van Laar, Teus; Cornblath, David R.; Odin, Per; Klostermann, Fabian; Grandas, Francisco J.; Ebersbach, Georg; Urban, Peter P.; Valldeoriola, Francesc; Antonini, Angelo

    2013-01-01

    In advanced Parkinson's disease (PD) patients, continuous intra-duodenal infusion of levodopa/carbidopa intestinal gel (LCIG) is an established approach in the management of motor complications that cannot be further improved by conventional oral therapy. In general, tolerability of LCIG has resembl

  12. Endoscopic ultrasound guided emergency coil and glue for actively bleeding duodenal varix after failed endoscopy

    Directory of Open Access Journals (Sweden)

    S Choudhary Narendra

    2015-01-01

    Full Text Available Management of ectopic variceal bleed may be difficult at times due to anatomical location or presence of collaterals. We present a case of an elderly cirrhotic male with acute upper gastrointestinal bleed due to a large duodenal varix successfully managed by endoscopic ultrasound guided coil placement along with glue (N-butyl-2-cyanoacrylate injection.

  13. Duodenal stenosis resulting from a preduodenal portal vein and an operation for scoliosis

    Institute of Scientific and Technical Information of China (English)

    Kouji Masumoto; Risa Teshiba; Genshiro Esumi; Kouji Nagata; Takanori Nakatsuji; Yuko Nishimoto; Sadako Yamaguchi; Kenzo Sumitomo; Tomoaki Taguchi

    2009-01-01

    A preduodenal portal vein (PDPV) is known to be a rare cause of duodenal stenosis. We treated a 22-yearold male patient with malnutrition as a result of PDPV and a previously performed operation for scoliosis,who showed an improvement in quality of life after being treated with a combination of nutritional support and surgery. The patient with PDPV had been admitted to our department with duodenal stenosis, ranging from the first to third portions. He had suffered from vomiting since 1 year of age, and he developed malnutrition during the last 6-mo period after orthopedic surgery for scoliosis. The stenosis was related to both the PDPV and the previously performed operation for scoliosis. After receiving nutritional support for 6 mo, a gastrojejunostomy with Braun's anastomosis for the first portion and a duodenojejunostomy for the second and third portions were performed. The postoperative course was almost uneventful. Three months later, he was discharged and able to attend university. In patients with widespread duodenal stenosis, there may be a complicated cause,such as PDPV and duodenal stretching induced by previous spinal surgery.

  14. [Periodontal status in children with various morphological forms of chronic gastritis and duodenitis].

    Science.gov (United States)

    Romanenko, E G

    2014-01-01

    The purpose of the study was to determine the condition of the periodontal tissues in 80 children with various morphological forms of gastritis and duodenitis. The examination included determination of oral hygiene and periodontal status by the simplified Green-Vermillion index, Silness-Low index, papillary-marginal-alveolar index, gingival Muhlemann-Cowell bleeding index, Schiller-Pisarev iodine index. In children with chronic gastritis and duodenitis high incidence of chronic catarrhal gingivitis (85.0%) was revealed. At the same time it was pointed out that inflammation in the periodontal tissues correlated with changes in the gastroduodenal mucosa. In 65.4% of children with superficial gastritis and duodenitis, chronic catarrhal gingivitis (localized in 38.5% of cases, generalized in 26.9%) was observed. In 94.4% of all children with diffuse and erosive gastritis and duodenitis, chronic generalized catarrhal gingivitis was observed, and low oral hygiene level was revealed. The severity and duration of the underlying disease aggravated clinical manifestations of chronic catarrhal gingivitis in children with lesions of the upper gastrointestinal tract.

  15. Migration of a biliary stent causing duodenal perforation and biliary peritonitis.

    Science.gov (United States)

    Issa, Hussain; Nahawi, Mamdouh; Bseiso, Bahaa; Al-Salem, Ahmed

    2013-10-16

    Migration of endoscopically placed biliary stents is a well-recognized complication of endoscopic retrograde cholangiopancreatography. Less than 1% of migrated stents however cause intestinal perforation. We present a case of a migrated biliary stent that resulted in duodenal perforation and biliary peritonitis.

  16. Migration of a biliary stent causing duodenal perforation and biliary peritonitis

    OpenAIRE

    Issa, Hussain; Nahawi, Mamdouh; Bseiso, Bahaa; Al-Salem, Ahmed

    2013-01-01

    Migration of endoscopically placed biliary stents is a well-recognized complication of endoscopic retrograde cholangiopancreatography. Less than 1% of migrated stents however cause intestinal perforation. We present a case of a migrated biliary stent that resulted in duodenal perforation and biliary peritonitis.

  17. Duodenal-jejunal bypass sleeve - a potential alternative to bariatric surgery?

    DEFF Research Database (Denmark)

    Rohde, Ulrich; Gylvin, Silas; Vilmann, Peter

    2014-01-01

    Overweight and obesity are risk factors for several co-morbidities reducing life expectancy. Conservative treatment of obesity is generally ineffective in the long-term. Bariatric surgery has proven effective, but is associated with potential complications. Duodenal-jejunal bypass sleeve is a novel...

  18. A review of the management of perforated duodenal ulcers at a ...

    African Journals Online (AJOL)

    EB

    complication includes upper gastrointestinal tract. (GIT) bleeding; perforation of the stomach, ... duodenal ulcer perforation and the outcome, at the. Obafemi Awolowo ... endoscopic findings (if previously done prior to ... states with six of them having features of systemic ... after discharge from the hospital ranged from 1 week.

  19. Mortality in perforated duodenal ulcer depends upon pre-operative risk: a retrospective 10-year study.

    LENUS (Irish Health Repository)

    Larkin, J O

    2012-01-31

    INTRODUCTION: Most patients presenting with acutely perforated duodenal ulcer undergo operation, but conservative treatment may be indicated when an ulcer has spontaneously sealed with minimal\\/localised peritoneal irritation or when the patient\\'s premorbid performance status is poor. We retrospectively reviewed our experience with operative and conservative management of perforated duodenal ulcers over a 10-year period and analysed outcome according to American Society of Anesthesiologists (ASA) score. METHODS: The records of all patients presenting with perforated duodenal ulcer to the Department of Surgery, Mayo General Hospital, between January 1998 and December 2007 were reviewed. Age, gender, co-morbidity, ASA-score, clinical presentation, mode of management, operative procedures, morbidity and mortality were considered. RESULTS: Of 76 patients included, 48 (44 operative, 4 conservative) were ASA I-III, with no mortality irrespective of treatment. Amongst 28 patients with ASA-score IV\\/V, mortality was 54.5% (6\\/11) following operative management and 52.9% (9\\/17) with conservative management. CONCLUSION: In patients with a perforated duodenal ulcer and ASA-score I-III, postoperative outcome is uniformly favourable. We recommend these patients have repair with peritoneal lavage performed, routinely followed postoperatively by empirical triple therapy. Given that mortality is equivalent between ASA IV\\/V patients whether managed operatively or conservatively, we suggest that both management options are equally justifiable.

  20. The use of pyloric exclusion for treating duodenal trauma: case series

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    Gustavo Pereira Fraga

    Full Text Available CONTEXT AND OBJECTIVES: Significant controversy exists regarding the best surgical treatment for complex duodenal injuries. The aims of this study were to report on a series of eight cases of duodenal repairs using pyloric exclusion and to describe reported complications or improvements in clinical outcomes among patients with complex duodenal trauma. DESIGN AND SETTING: Cross-sectional study followed by a case series in a university hospital. METHODS: Data on eight patients with duodenal trauma who underwent pyloric exclusion over a 17.5 year period were collected and analyzed. RESULTS: The causes of the injuries included penetrating gunshot wounds (GSW in five patients and motor vehicle accidents (blunt trauma in three patients. The time elapsed until surgery was longer in the blunt trauma group, while in one patient, the gunshot injury was initially missed and thus the procedure was carried out 36 hours after the original injury. The injuries were grade III (50% or IV (50% and the morbidity rate was 87.5%. Four patients (50% died during the postoperative period from complications, including hypovolemic shock (one patient, sepsis (peritonitis following the missed injury and pancreatitis with an anastomotic fistula (two patients. CONCLUSIONS: Pyloric exclusion was associated with multiple complications and a high mortality rate. This surgical technique is indicated for rare cases of complex injury to the duodenum and the surgeon should be aware that treatment with a minimalistic approach, with only primary repair, may be ideal.

  1. High-resolution analysis of the duodenal interdigestive phase III in humans.

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    Castedal, M; Abrahamsson, H

    2001-10-01

    To study the spatial organization of the propagating pressure waves of duodenal phase III, we performed fasting antroduodenal high-resolution manometry with a 16-channel catheter in 12 healthy subjects. The phase III pressure waves diverged in an anterograde and retrograde direction from the start site of each pressure wave. The pressure waves maintained this configuration as the activity front moved distally in the duodenum. The start site of the pressure waves moved gradually to a point approximately 12 cm (median) distal to the pylorus and remained at this point for about 40% of the phase III time before moving further distally. The length of retrograde pressure wave propagation increased to 6 cm (median) as the pressure wave origin moved aborally to a point 10-14 cm distal to the pylorus, and then decreased when the origin of pressure waves reached the distal end of the duodenum. Bidirectional pressure waves dominated in both retrograde and anterograde activity fronts. Three pressure-wave mechanisms behind the duodenal phase IV were observed. Isolated pyloric pressure waves were absent during late duodenal phase III retroperistalsis. Thus, a number of new features of the duodenal phase III-related motility were observed using high-temporospatial resolution recordings.

  2. A Metagenomic Investigation of the Duodenal Microbiota Reveals Links with Obesity.

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    Emmanouil Angelakis

    Full Text Available Few studies have tested the small intestine microbiota in humans, where most nutrient digestion and absorption occur. Here, our objective was to examine the duodenal microbiota between obese and normal volunteers using metagenomic techniques.We tested duodenal samples from five obese and five normal volunteers using 16S rDNA V6 pyrosequencing and Illumina MiSeq deep sequencing. The predominant phyla of the duodenal microbiota were Firmicutes and Actinobacteria, whereas Bacteroidetes were absent. Obese individuals had a significant increase in anaerobic genera (p < 0.001 and a higher abundance of genes encoding Acyl-CoA dehydrogenase (p = 0.0018 compared to the control group. Obese individuals also had a reduced abundance of genes encoding sucrose phosphorylase (p = 0.015 and 1,4-alpha-glucan branching enzyme (p = 0.05. Normal weight people had significantly increased FabK (p = 0.027, and the glycerophospholipid metabolism pathway revealed the presence of phospholipase A1 only in the control group (p = 0.05.The duodenal microbiota of obese individuals exhibit alterations in the fatty acid and sucrose breakdown pathways, probably induced by diet imbalance.

  3. Acute pancreatitis as an adverse event in patients with the duodenal-jejunal bypass liner

    NARCIS (Netherlands)

    Betzel, B; Homan, J.; Aarts, E.; Janssen, I.; Spanier, M.; Wahab, P.J.; Groenen, M.; Berends, F.

    2015-01-01

    Placement of the duodenal-jejunal bypass liner (DJBL) is a minimally invasive technique for the management of patients with type 2 diabetes mellitus and obesity. Acute pancreatitis was seen in 5 of 167 patients (3 %) in our series. It is suggested that acute pancreatitis in patients with the DJBL re

  4. Primary Duodenal Adenocarcinoma without Stenosis: A Case Report with a Brief Literature Review

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    Daisuke Usuda

    2014-07-01

    Full Text Available This article focuses on the symptomatic and diagnostic problems of primary duodenal adenocarcinoma (PDA by way of two case reports and a literature review. An 85-year-old woman with an adenocarcinoma in the 1st duodenal portion was offered palliative care. A 90-year-old woman with an adenocarcinoma in the 3rd duodenal portion was also offered palliative care. A unique finding in the two cases reported herein is that PDA did not cause stenosis and occlusion of the lumen. As no reports of PDA without stenosis have been published so far, these cases may add to our knowledge of PDA. The diagnosis of PDA is often delayed because its symptoms may be absent until the tumor has progressed, thus leading to a delay of several months. Patients typically present with a long history of variable and vague symptoms, and many are diagnosed with advanced disease. As regards clinical manifestations, abdominal pain is the most frequent symptom. The majority of these tumors are found to have infiltrated the duodenal wall at presentation, with many being unresectable due to local and distal invasion. Esophagogastroduodenoscopy and gastrointestinal barium radiography are the main diagnostic tests for PDA, detecting 88.6 and 83.3% of tumors, respectively. In some cases, ultrasonography or computed tomography are useful for detecting PDA and determining vascular invasion.

  5. Duodenal myotomy blocks reduction of meal size and prolongation of intermeal interval by cholecystokinin.

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    Lateef, Dalya M; Washington, Martha C; Raboin, Shannon J; Roberson, Allison E; Mansour, Mahmoud M; Williams, Carol S; Sayegh, Ayman I

    2012-02-01

    We have shown that vagotomy (VGX) attenuates the reduction of meal size (MS) produced by cholecystokinin (CCK) -8 and -33 and that celiaco-mesenteric ganglionectomy (CMGX) attenuates the prolongation of the intermeal interval (IMI) produced by CCK-33. Here, we report the following novel data. First, by determining the distribution of CCK(1) receptor messenger RNA, which mediates reduction of MS and prolongation of IMI by CCK, in seven regions of the gastrointestinal tract in the adult rat we found that the duodenum contains the highest concentration of this receptor in the gut. Second, based on the previous finding we performed a unique surgical technique known as duodenal myotomy (MYO), which severs all the nerves of the gut wall in the duodenum including vagus, splanchnic and enteric nerves. Third, we determined MS and IMI in duodenal MYO rats in responses to endogenous CCK-58 released by the non-nutrient, trypsin inhibitor, camostat and CCK-8 to test the possibility that the duodenum is the site of action for reduction of MS and prolongation of IMI. We found that, similar to the previous work reported by using CCK-8 and MS, duodenal MYO also blocked reduction of MS by camostat. Forth, duodenal MYO blocked prolongation of IMI by camostat. As such, our current results suggest that the duodenum is the gut site that communicates both feeding signals of endogenous CCK, MS and IMI, with the brain through vagal and splanchnic afferents.

  6. Cystic dystrophy of the duodenal wall is not always associated with chronic pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Raffaele Pezzilli; Roberto Corinaldesi; Donatella Santini; Lucia Calculli; Riccardo Casadei; Antonio Maria Morselli-Labate; Andrea Imbrogno; Dario Fabbri; Giovanni Taffurelli; Claudio Ricci

    2011-01-01

    Cystic dystrophy of the duodenal wall is a rare form of the disease which was described in 1970 by French authors who reported the presence of focal pancreatic disease localized in an area comprising the C-loop of the duodenum and the head of the pancreas. German authors have defined this area as a "groove". We report our recent experience on cystic dystrophy of the paraduodenal space and systematically review the data in the literature regarding the alterations of this space. A MEDLINE search of papers published between 1966 and 2010 was carried out and 59 papers were considered for the present study; there were 19 cohort studies and 40 case reports. The majority of patients having groove pancreatitis were middle aged. Mean age was significantly higher in patients having groove carcinoma. The diagnosis of cystic dystrophy of the duodenal wall can now be assessed by multidetector computer tomography, magnetic resonance imaging and endoscopic ultrasonography. These latter two techniques may also add more information on the involvement of the remaining pancreatic gland not involved by the duodenal malformation and they may help in differentiating "groove pancreatitis" from "groove adenocarcinoma". In conclusion, chronic pancreatitis involving the entire pancreatic gland was present in half of the patients with cystic dystrophy of the duodenal wall and, in the majority of them, the pancreatitis had calcifications.

  7. Morphological, immunophenotypic and clinical characteristics of dogs with lymphocytic - plasmacytic duodenitis

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    Đorđević Milena

    2012-01-01

    Full Text Available The most important morphological feature of the immune response in lymphocytic-plasmacytic duodenitis (LPD in dogs is the presence of a mononuclear infiltrate in the duodenal mucosa. The ethiopatogenesis of this disease is still unknown, nor are known all the immunophenotypic characteristics of the infiltrate cells, which would be of help in the elucidation of the pathogenesis of LPD. The study involved 60 adult dogs of different breeds and both sexes: 54 dogs with symptoms of diarrhea and vomiting that lasted longer than three weeks and six clinically healthy dogs that served as controls. Hematological and biochemical analysis of blood, radiology, ultrasound and endoscopic diagnosis were carried out. Samples of duodenal mucosal biopta were examined by histopathological and immunohistochemical methods. During endoscopic examination, duodenal wall thickening, mucosal edema and hyperemia were observed in 46 dogs. Microscopic studies showed on the epithelial cells of the duodenum degenerative changes, and sometimes desquamation. Most of the intestinal crypts were dilated, irregularly shaped and filled with detritus. On immunohistochemical examination of samples of the duodenum in a number of dogs with LPD a predominant expression of CD3 + T lymphocytes was observed. [Projekat Ministarstva nauke Republike Srbije, br. III 46002

  8. Traumatic duodenal lesions in children due to two-point seat-belt: the seat-belt syndrome.

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    Guanà, R; Sangiorgio, L; Tessiatore, P; Seymandi, P

    2012-02-01

    Two consecutive children, motor vehicle accident victims, were treated between December 2006 and January 2007. They reported duodenal perforations due to lap-belt action. In the first case (a 9-year-old boy), symptoms became immediately evident after the trauma. Early computerized tomography (CT) scan demonstrated biliaryhemoperitonitis and free sub-diaphragmatic air. At the laparotomy a complete duodenal transection, at the level of duodenal bulb, was found. In the second patient, early X-ray and CT scan revealed no free abdominal air; only the second CT scan, repeated after 48 hours, when general conditions of the girl become critical, demonstrated biliary peritonitis and aerial extraluminal gas image in the right peri-kidney space. At the surgical exploration a duodenal laceration was noted, at the passage between the second and the third duodenal portion, in the postero-lateral wall, with an extension of 6 cm. In both children direct suture of duodenal injury without stoma construction was performed. Not early or late postoperative complications were registered (follow-up: 18 months). In the traumatic duodenal lesions an early diagnosis is not always possible because of the paucity of the clinical signs. Tightened clinical and radiological monitoring of the patient is fundamental in the successive hours to the trauma. Early diagnosis and high level of suspicious rest crucial for better prognosis in this group of patients.

  9. Expression of macrophage migration-inhibitory factor in duodenal ulcer and its relation to Helicobacter pylori infection.

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    Yu, X H; Zhang, Q; Yang, X P; Yang, W; Dai, F; Qian, Z; Wang, Z L; Wu, C F; Zhao, H Z; Wang, G H

    2015-10-30

    The aim of this study was to examine the expression of macrophage migration-inhibitory factor (MIF) in duodenal ulcer epithelial cells and its relation to Helicobacter pylori (Hp) infection, and to discuss the pathogenic roles of MIF expression and Hp infection in duodenal ulcer. MIF protein and mRNA expression was examined in samples from patients with duodenal ulcer with and without Hp infection (N = 40 each, experimental group), and in normal duodenal bulb mucosal tissue (N = 40, control group) using immunohistochemistry and in situ hybridization. Patients without Hp infection received routine treatment, and treatment was provided to the patients positive for Hp to eradicate Hp infection. Hp and MIF expression levels before treatment and after the ulcer had been cured were compared. The positive rates of MIF protein and mRNA in patients with Hp infection before treatment were 67.5 and 65%, respectively, and were 18.9 and 21.6% in the 37 patients from whom Hp was eliminated. These were statistically different both before and after treatment compared with controls (P 0.05). The results of this study suggested that MIF is related to the development of duodenal ulcer, and that the presence of Hp is closely related with the expression of MIF in the duodenal mucosa and the development of duodenal ulcer.

  10. Hemorrhagic gastric and duodenal ulcers after the Great East Japan Earthquake Disaster.

    Science.gov (United States)

    Yamanaka, Kenichi; Miyatani, Hiroyuki; Yoshida, Yukio; Asabe, Shinichi; Yoshida, Toru; Nakano, Misaki; Obara, Shin; Endo, Hidehiko

    2013-11-14

    To elucidate the characteristics of hemorrhagic gastric/duodenal ulcers in a post-earthquake period within one medical district. Hemorrhagic gastric/duodenal ulcers in the Iwate Prefectural Kamaishi Hospital during the 6-mo period after the Great East Japan Earthquake Disaster were reviewed retrospectively. The subjects were 27 patients who visited our hospital with a chief complaint of hematemesis or hemorrhagic stool and were diagnosed as having hemorrhagic gastric/duodenal ulcers by upper gastrointestinal endoscopy during a 6-mo period starting on March 11, 2011. This period was divided into two phases: the acute stress phase, comprising the first month after the earthquake disaster, and the chronic stress phase, from the second through the sixth month. The following items were analyzed according to these phases: age, sex, sites and number of ulcers, peptic ulcer history, status of Helicobacter pylori (H. pylori) infection, intake of non-steroidal anti-inflammatory drugs, and degree of impact of the earthquake disaster. In the acute stress phase from 10 d to 1 mo after the disaster, the number of patients increased rapidly, with a nearly equal male-to-female ratio, and the rate of multiple ulcers was significantly higher than in the previous year (88.9% vs 25%, P earthquake disaster, the number of patients decreased to a level similar to that of the previous year. There were more male patients during this period, and many patients tended to have a solitary ulcer. All patients with duodenal ulcers found in the acute stress phase were negative for serum H. pylori antibodies, and this was significantly different from the previous year's positive rate of 75% (P earthquake disaster may have affected the characteristics of hemorrhagic gastric/duodenal ulcers.

  11. History of Helicobacter pylori, duodenal ulcer, gastric ulcer and gastric cancer.

    Science.gov (United States)

    Graham, David Y

    2014-05-14

    Helicobacter pylori (H. pylori) infection underlies gastric ulcer disease, gastric cancer and duodenal ulcer disease. The disease expression reflects the pattern and extent of gastritis/gastric atrophy (i.e., duodenal ulcer with non-atrophic and gastric ulcer and gastric cancer with atrophic gastritis). Gastric and duodenal ulcers and gastric cancer have been known for thousands of years. Ulcers are generally non-fatal and until the 20th century were difficult to diagnose. However, the presence and pattern of gastritis in past civilizations can be deduced based on the diseases present. It has been suggested that gastric ulcer and duodenal ulcer both arose or became more frequent in Europe in the 19th century. Here, we show that gastric cancer and gastric ulcer were present throughout the 17th to 19th centuries consistent with atrophic gastritis being the predominant pattern, as it proved to be when it could be examined directly in the late 19th century. The environment before the 20th century favored acquisition of H. pylori infection and atrophic gastritis (e.g., poor sanitation and standards of living, seasonal diets poor in fresh fruits and vegetables, especially in winter, vitamin deficiencies, and frequent febrile infections in childhood). The latter part of the 19th century saw improvements in standards of living, sanitation, and diets with a corresponding decrease in rate of development of atrophic gastritis allowing duodenal ulcers to become more prominent. In the early 20th century physician's believed they could diagnose ulcers clinically and that the diagnosis required hospitalization for "surgical disease" or for "Sippy" diets. We show that while H. pylori remained common and virulent in Europe and the United States, environmental changes resulted in changes of the pattern of gastritis producing a change in the manifestations of H. pylori infections and subsequently to a rapid decline in transmission and a rapid decline in all H. pylori-related diseases.

  12. A novel dynamic scintigraphic technique for assessing duodenal contractions during gastric emptying in humans: a feasibility study.

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    Kubo, Tadeu T A; Moraes, Eder R; Secaf, Marie; Troncon, Luiz E A

    2015-01-01

    Duodenal contractions are thought to play a role in the control of gastric emptying. Although noninvasive techniques, such as ultrasonography and MRI, have been proposed for studying duodenal contractile activity in humans, there are no reports on the use of scintigraphy for this purpose. This work aimed to describe a novel scintigraphic technique for assessing duodenal contractility during gastric emptying in humans, and to present preliminary data on the frequency and amplitude of contractions detected in three different duodenal segments. Fasted young healthy volunteers (N=12) were given either a liquid or a solid test meal of similar calorie content (400 kcal) labeled with 99mTc-phytate. Static images were collected to determine gastric emptying. Dynamic images of the anterior aspect of the abdomen (1 frame/s) were also acquired periodically in a standard position for 256 s at 15-30 min intervals. 'Activity versus time' curves were generated for regions of interest corresponding to the proximal, middle, and distal duodenal segments. Curves were digitally filtered and processed to estimate both dominant frequency (fast Fourier transform) and amplitude (mean ejection fraction) of postprandial duodenal contractions. There were no significant differences regarding dominant frequency among proximal, middle, and distal duodenal regions of interest. In addition, there were no significant differences between the liquid and the solid meal in terms of either frequency or amplitude of duodenal contractions. Characterization of duodenal contractions in humans using scintigraphy is feasible and yields consistent data for both the frequency and the amplitude of postprandial contractions, which seems to be rather independent of meal consistency.

  13. Cat scratch disease, a rare cause of hypodense liver lesions, lymphadenopathy and a protruding duodenal lesion, caused by Bartonella henselae.

    Science.gov (United States)

    van Ierland-van Leeuwen, Marloes; Peringa, Jan; Blaauwgeers, Hans; van Dam, Alje

    2014-10-29

    A 46-year-old woman presented with right upper abdominal pain and fever. At imaging, enlarged peripancreatic and hilar lymph nodes, as well as hypodense liver lesions, were detected, suggestive of malignant disease. At endoscopy, the mass adjacent to the duodenum was seen as a protruding lesion through the duodenal wall. A biopsy of this lesion, taken through the duodenal wall, showed a histiocytic granulomatous inflammation with necrosis. Serology for Bartonella henselae IgM was highly elevated a few weeks after presentation, consistent with the diagnosis of cat scratch disease. Clinical symptoms subsided spontaneously and, after treatment with azithromycin, the lymphatic masses, liver lesions and duodenal ulceration disappeared completely.

  14. External Beam Radiation Therapy (EBRT) for Patients with Malignant Pheochromocytoma and Non-Head and Neck Paraganglioma: Combination with 131I-MIBG

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    Fishbein, Lauren; Bonner, Lara; Torigian, Drew A.; Nathanson, Katherine L.; Cohen, Debbie L.; Pryma, Daniel; Cengel, Keith

    2015-01-01

    In patients with malignant pheochromocytoma and paraganglioma, 131I-MIBG radiotherapy can achieve an objective response rate of 30–50% with the dose limiting toxicity being hematologic. Patients with disseminated disease, who also have a few index bulky or symptomatic lesions, may benefit from the addition of targeted external beam radiotherapy alone or in combination with systemic 131I-MIBG. The records of patients with malignant paraganglioma who were treated with external beam radiotherapy at the University of Pennsylvania from February 1973 to February 2011 were reviewed in an institutional review board approved retrospective study. Of the 17 patients with tumors in the thorax, abdomen, or pelvis, 76% had local control or clinically significant symptomatic relief for at least one year or until death. As expected, the predominant toxicity was due to irradiation of tumor-adjacent normal tissues without clinically significant hematologic toxicity. Due to widespread systemic metastases with areas of bulky, symptomatic tumor, five of the 17 patients were treated with sequential 131I-MIBG (2 mCi/kg per treatment) and external beam radiotherapy to nine sites. In these patients, all areas that were irradiated with external beam radiotherapy showed durable objective response despite all patients eventually experiencing out-of-field systemic progression requiring other treatment. Four of these patients remain alive with excellent performance status 16, 18, 23, and 24 months after external beam radiotherapy. External beam radiotherapy can be highly effective in local management of malignant paraganglioma and can be used in conjunction with 131I-MIBG due to non-overlapping toxicities with excellent control of locally bulky tumors. PMID:22566196

  15. The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency

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    Devilee Peter

    2005-11-01

    Full Text Available Abstract Background The SDHA, SDHB, SDHC and SDHD genes encode the subunits of succinate dehydrogenase (succinate: ubiquinone oxidoreductase, a component of both the Krebs cycle and the mitochondrial respiratory chain. SDHA, a flavoprotein and SDHB, an iron-sulfur protein together constitute the catalytic domain, while SDHC and SDHD encode membrane anchors that allow the complex to participate in the respiratory chain as complex II. Germline mutations of SDHD and SDHB are a major cause of the hereditary forms of the tumors paraganglioma and pheochromocytoma. The largest subunit, SDHA, is mutated in patients with Leigh syndrome and late-onset optic atrophy, but has not as yet been identified as a factor in hereditary cancer. Description The SDH mutation database is based on the recently described Leiden Open (source Variation Database (LOVD system. The variants currently described in the database were extracted from the published literature and in some cases annotated to conform to current mutation nomenclature. Researchers can also directly submit new sequence variants online. Since the identification of SDHD, SDHC, and SDHB as classic tumor suppressor genes in 2000 and 2001, studies from research groups around the world have identified a total of 120 variants. Here we introduce all reported paraganglioma and pheochromocytoma related sequence variations in these genes, in addition to all reported mutations of SDHA. The database is now accessible online. Conclusion The SDH mutation database offers a valuable tool and resource for clinicians involved in the treatment of patients with paraganglioma-pheochromocytoma, clinical geneticists needing an overview of current knowledge, and geneticists and other researchers needing a solid foundation for further exploration of both these tumor syndromes and SDHA-related phenotypes.

  16. Accuracy of recommended sampling and assay methods for the determination of plasma-free and urinary fractionated metanephrines in the diagnosis of pheochromocytoma and paraganglioma: a systematic review.

    Science.gov (United States)

    Därr, Roland; Kuhn, Matthias; Bode, Christoph; Bornstein, Stefan R; Pacak, Karel; Lenders, Jacques W M; Eisenhofer, Graeme

    2017-06-01

    To determine the accuracy of biochemical tests for the diagnosis of pheochromocytoma and paraganglioma. A search of the PubMed database was conducted for English-language articles published between October 1958 and December 2016 on the biochemical diagnosis of pheochromocytoma and paraganglioma using immunoassay methods or high-performance liquid chromatography with coulometric/electrochemical or tandem mass spectrometric detection for measurement of fractionated metanephrines in 24-h urine collections or plasma-free metanephrines obtained under seated or supine blood sampling conditions. Application of the Standards for Reporting of Diagnostic Studies Accuracy Group criteria yielded 23 suitable articles. Summary receiver operating characteristic analysis revealed sensitivities/specificities of 94/93% and 91/93% for measurement of plasma-free metanephrines and urinary fractionated metanephrines using high-performance liquid chromatography or immunoassay methods, respectively. Partial areas under the curve were 0.947 vs. 0.911. Irrespective of the analytical method, sensitivity was significantly higher for supine compared with seated sampling, 95 vs. 89% (p sampling compared with 24-h urine, 95 vs. 90% (p sampling, seated sampling, and urine. Test accuracy increased linearly from 90 to 93% for 24-h urine at prevalence rates of 0.0-1.0, decreased linearly from 94 to 89% for seated sampling and was constant at 95% for supine conditions. Current tests for the biochemical diagnosis of pheochromocytoma and paraganglioma show excellent diagnostic accuracy. Supine sampling conditions and measurement of plasma-free metanephrines using high-performance liquid chromatography with coulometric/electrochemical or tandem mass spectrometric detection provides the highest accuracy at all prevalence rates.

  17. Perioperative outcomes of syndromic paraganglioma and pheochromocytoma resection in patients with von Hippel-Lindau disease, multiple endocrine neoplasia type 2, or neurofibromatosis type 1.

    Science.gov (United States)

    Butz, James J; Yan, Qi; McKenzie, Travis J; Weingarten, Toby N; Cavalcante, Alexandre N; Bancos, Irina; Young, William F; Schroeder, Darrell R; Martin, David P; Sprung, Juraj

    2017-09-14

    Pheochromocytoma and/or paraganglioma associated with neurofibromatosis type 1, multiple endocrine neoplasia type 2A, and von Hippel-Lindau disease have different catecholamine biochemical phenotypes. We examined perioperative outcomes of pheochromocytoma/paraganglioma resection in 3 syndromic forms. Retrospective review of patients undergoing resection of syndromic pheochromocytoma/paraganglioma from 2000 through 2016. Eighty-one patients underwent pheochromocytoma/paraganglioma resection (multiple endocrine neoplasia type 2A, n = 36; neurofibromatosis type 1, n = 26; von Hippel-Lindau disease, n = 19). Tumor size differed across groups; patients with neurofibromatosis type 1 and von Hippel-Lindau disease had the largest tumors (P = .017). Larger tumor volumes correlated with higher urine 24-hour total metanephrine (r = 0.94, P < .001; r = 0.67, P = .033; and r = 0.89, P < .001 for multiple endocrine neoplasia type 2A, von Hippel-Lindau disease, and neurofibromatosis type 1, respectively). High adrenergic secretion (24-hour urine metanepinephrine) was found in neurofibromatosis type 1 (median, 861 μg/24 h), similar to that found in multiple endocrine neoplasia type 2A (median, 809 μg/24 h). The highest noradrenergic secretion (24-hour urine normetanephrine) occurred with von Hippel-Lindau disease (median, 4,598 μg/24 h), followed by neurofibromatosis type 1 and multiple endocrine neoplasia type 2A (median, 1,607 and 923 μg/24 h, respectively). The highest graded complications occurred among patients with neurofibromatosis type 1 (P = .036). However, when comparing postoperative outcomes across 3 groups in those who had laparoscopic resection, there was no significant difference (P = .955). Patients with neurofibromatosis type 1 had the most volatile intraoperative hemodynamic course and more severe postoperative complications. These complications are related to large tumors associated with abundant catecholamine secretion and the fact that a high

  18. Giant duodenal ulcer perforation: a case of innovative repair with an antrum gastric patch Perforación de ulcus gigante duodenal: reparación innovadora mediante una plastia del antro gástrico

    Directory of Open Access Journals (Sweden)

    Javier A. Cienfuegos

    2012-08-01

    Full Text Available Backgrounds: the treatment of a perforated giant duodenal ulcer (GUDs represents a formidable surgical challenge regarding the duodenal wall defect repair in severe peritonitis setting. A high incidence of dehiscence and hospital mortality (15-40%- has been reported with the majority of the techniques. We report a case of GUDs perforation successfully treated with a subtotal gastrectomy and a gastric patch with the remnant antrum, for repairing the duodenal defect. Case report: a 63-years-old man with antecedents of peptic ulcer disease presents a large duodenal ulcer perforation with 48 hrs delay and associated with severe peritonitis and a retroperitoneal collection. A subtotal gastrectomy with Billroth II reconstruction and reconstruction of the duodenal defect with a patch of the remnant antrum was carried out. The patient was discharged at 17th postoperative day with good tolerance. Discussion: the duodenal defect repair with a patch of the remant antrum, represents a valid alternative in similar circumstances. To our knowledge, it appears to be the first clinical description of this technique.Antecedentes: el tratamiento de un ulcus duodenal gigante (UDG; > 2 cm perforado entraña una gran dificultad técnica, por la reparación del gran defecto duodenal; y por la peritonitis sobreañadida. Todas las técnicas descritas se asocian con un índice elevado de dehiscencias y una mortalidad del 15-40%. Describimos por primera vez el caso de un UDG perforado, tratado mediante una gastrectomía subtotal y con una plastia del antro gástrico remanente. Caso clínico: varón de 63 años que se interviene de un UDG perforado en la 2ª porción duodenal asociado con peritonitis severa y disección de la gotiera parieto-cólica derecha retroperitoneo. Se realiza gastrectomía tipo Bilroth II y reparación del defecto duodenal mediante una plastia con la pared del antro gástrico remanente. El paciente fue dado de alta a los 17 días. Discusión: la

  19. Anomalous opening of the common bile duct into the duodenal bulb: endoscopic treatment

    Directory of Open Access Journals (Sweden)

    Oguz Dilek

    2007-07-01

    Full Text Available Abstract Background Anomalous biliary opening especially the presence of the ampulla of Vater in the duodenal bulb is a very rare phenomenon. We report clinical implications, laboratory and ERCP findings and also therapeutic approaches in 53 cases. Methods The data were collected from the records of 12.158 ERCP. The diagnosis was established as an anomalous opening of the common bile duct (CBD into the duodenal bulb when there is an orifice observed in the bulb with the absence of a papillary structure at its normal localization and when the CBD is visualized by cholangiography through this orifice without evidence of any other opening. Results A total of 53 cases were recruited. There was an obvious male preponderance (M/F: 49/4. Demographic data and ERCP findings were available for all, but clinical characteristics and laboratory findings could be obtained from 39 patients with full records. Thirty – seven of 39 cases had abdominal pain (95% and 23 of them (59% had cholangitis as well. Elevated AP and GGT were found in 97.4% (52/53. History of cholecystectomy was present in 64% of the cases, recurrent cholangitis in 26% and duodenal ulcer in 45%. Normal papilla was not observed in any of the patients and a cleft-like opening was evident instead. The CBD was hook shaped at the distal part that opens to the duodenal bulb. Pancreatic duct (PD was opening separately into the bulb in all the cases when it was possible to visualize. Dilated CBD in ERCP was evident in 94% and the CBD stone was demonstrated in 51%. PD was dilated in four of 12 (33% cases. None of them has a history of pancreatitis. Endoscopically, Papillary Balloon Dilatation instead of Sphincterotomy carried out in 19 of 27 patients (70% with choledocholithiazis. Remaining eight patients had undergone surgery (30%. Clinical symptoms were resolved with medical treatment in 16(32% patients with dilated CBD but no stone. Perforation and bleeding were occurred only in two patients

  20. Intraluminal duodenal diverticulum causing recurrent pancreatitis: diagnostic role of computed tomography; Diverticulo duodenal intraluminal como causa de pancreatitis recidivante. Diagnostico mediante TC

    Energy Technology Data Exchange (ETDEWEB)

    Sanchis-Querol, E.; Abreu, J. A.; Canto, J.; Elizalde, A. [Hospital General d' Igualada. Barcelona (Spain)

    2000-07-01

    Intraluminal duodenal diverticulum is among the infrequent causes of pancreatitis. We present the case of a 23-year-old patient in which this congenital morphological anomaly was diagnosed as the cause of recurrent pancreatitis by means of computed tomography and upper gastrointestinal series. The radiological findings included a contrast-filled, sac-like structure partially occupying the second portion of the duodenum, from which it was separated by a band of radio lucent tissue (halo sign). We review the literature, comparing the findings of other authors with those observed in our patient. (Author) 8 refs.

  1. [Utility of the WallFlexTM duodenal stent for unresectable advanced gastric cancer related to gastric outlet obstruction].

    Science.gov (United States)

    Aoki, Taro; Hyuga, Satoshi; Kato, Aya; Chono, Teruhiro; Watanabe, Risa; Komori, Takamichi; Matsumoto, Takashi; Takachi, Kou; Nishioka, Kiyonori; Uemura, Yoshio; Kobayashi, Kenji

    2012-11-01

    Duodenal stenting for malignant disease related to gastric outlet obstruction(GOO) has been covered by health insurance in Japan since April 2010. We inserted WallFlexTM duodenal stents(WDS) in 4 patients with GOO caused by unresectable gastric cancer. WDS insertion was successful in all 4 cases. Duodenal perforation occurred in 1 case. One case each of stent obstruction and stent migration occurred. All patients could eat a soft-food diet for 3-6 months (median, 5.3 months). Survival time ranged between 5 and 14 months (median, 6 months). Three patients underwent S-1 combination chemotherapy. Duodenal stenting is expected to be effective for advanced gastric cancer related to GOO.

  2. A Study of Changes in Stomach Wall at Sites Other Than the Ulcer in Chronic Duodenal Ulcer Patients

    National Research Council Canada - National Science Library

    Mishra, Jagmohan; Panigrahi, Souvagya

    2011-01-01

    ...). To study the different changes if any in stomach wall at sites other than the ulcer in chronic duodenal ulcer patients by upper Gastro-Intenstinal Endoscopy followed by histopathological examination...

  3. Treatment of duodenal ulceration with Furazolidine in China preceded the discovery of its association with H pylori

    Institute of Scientific and Technical Information of China (English)

    Frank Ivor Tovev

    2007-01-01

    @@ It is not generally known that patients with duodenal ulceration were being treated with an antibiotic, Furazolidone, in China five or more years before Marshall and Warren published their seminal paper in 1984 about the association between duodenal ulceration and Campylobacter like organisms in the stomach, later named Hpylori. Marshall and Warren won the 2005 Nobel Prize in physiology or medicine for their work on how a bacterium can relate to gastric inflammation or peptic ulceration.

  4. Role of Melatonin, Neuropeptide S and Short Chain Fatty Acids in Regulation of Duodenal Mucosal Barrier Function and Motility

    OpenAIRE

    Wan Saudi, Wan Salman

    2015-01-01

    The duodenal epithelium is regularly exposed to HCl, digestive enzymes, bacteria and toxins, and sometimes also to ethanol and drugs. The imbalance of aggressive factors in the intestinal lumen and mucosal barrier function increases the risk of tissue injury and inflammation. The key components of the duodenal barrier function include mucosal permeability, bicarbonate transport and the secretion or absorption of fluids. This thesis aims to elucidate the role of melatonin, neuropeptide S (NPS)...

  5. EUS mini probes in diagnosis of cystic dystrophy of duodenal wall in heterotopic pancreas:A case report

    Institute of Scientific and Technical Information of China (English)

    Ivan Jovanovic; Srbislav Knezevic; Marjan Micev; Miodrag Krstic

    2004-01-01

    Cystic dystrophy of the duodenal wall is a rare condition characterized by the development of cysts in heterotopic pancreatic tissue localized in the duodenal wall. A 38-yearold man was admitted to the hospital for abdominal pain and vomiting after food intake. lhe diagnosis of acute pancreatitis was initially suspected. Abdominal ultrasound examination revealed thickening of the second portion of duodenal wall within which, small cysts (diameter, less than 1 cm) were present in the vicinity of pancreatic head.The head of pancreas appeared enlarged (63 mm×42 mm)and hypoechoic. Upper endoscopy and barium X-ray series were performed revealing a severe circumferential deformation, as well as 4 cm long stenosis of the second portion of the duodenum. CT examination revealed multiple cysts located in an enlarged, thickened duodenal wall with moderate to strong post-contrast enhancement. We suspected that patient had cystic dystrophy of duodenal wail developed in the heterotopic pancreas and diagnosis was confirmed by endoscopic ultrasound (EUS). Endoscopic ultrasound (EUS) revealed circular stenosis from the duodenal bulb onwards. A twenty megaHertz mini-probe examination further showed diffuse (intramural) infiltration of duodenal wall limited to the submucosa and muscularis propria of the second portion of duodenum with multiple microoysts within the thickened mucosa and submucosa. Patient was successfully surgically treated and pancreatoduodenectomy was performed. The pathological examination confirmed a diagnosis of cystic dystrophy of a heterotopic pancreas.Endoscopic ultrasonography features allow preoperative diagnosis of cystic dystrophy of a heterotopic pancreas in duodenal wall, with intraluminal 20 MHz mini probe sonography being more efficient in cases of luminal stenosis.

  6. Role of positron emission tomography and bone scintigraphy in the evaluation of bone involvement in metastatic pheochromocytoma and paraganglioma: specific implications for succinate dehydrogenase enzyme subunit B gene mutations.

    NARCIS (Netherlands)

    Zelinka, T.; Timmers, H.J.L.M.; Kozupa, A.; Chen, C.C.; Carrasquillo, J.A.; Reynolds, J.C.; Ling, A.; Eisenhofer, G.; Lazurova, I.; Adams, K.T.; Whatley, M.A.; Widimsky, J.Jr.; Pacak, K.

    2008-01-01

    We performed a retrospective analysis of 71 subjects with metastatic pheochromocytoma and paraganglioma (30 subjects with mutation of succinate dehydrogenase enzyme subunit B (SDHB) gene and 41 subjects without SDHB mutation). Sixty-nine percent presented with bone metastases (SDHB +/-: 77% vs 63%),

  7. Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma.

    NARCIS (Netherlands)

    Timmers, H.J.L.M.; Chen, C.C.; Carrasquillo, J.A.; Whatley, M.; Ling, A.; Havekes, B.; Eisenhofer, G.; Martiniova, L.; Adams, K.T.; Pacak, K.

    2009-01-01

    CONTEXT: Besides (123)I-metaiodobenzylguanidine (MIBG), positron emission tomography (PET) agents are available for the localization of paraganglioma (PGL), including (18)F-3,4-dihydroxyphenylalanine (DOPA), (18)F-fluoro-2-deoxy-D-glucose ((18)F-FDG), and (18)F-fluorodopamine ((18)F-FDA). OBJECTIVE:

  8. Is Helicobacter pylori Infection the Primary Cause of Duodenal Ulceration or a Secondary Factor? A Review of the Evidence

    Directory of Open Access Journals (Sweden)

    Vikram Kate

    2013-01-01

    Full Text Available Helicobacter pylori (H. pylori has a role in the multifactorial etiology of peptic ulcer disease. A link between H. pylori infection and duodenal ulcer disease is now established. Other contributing factors and their interaction with the organism may initiate the ulcerative process. The fact that eradication of H. pylori infection leads to a long-term cure in the majority of duodenal ulcer patients and the fact that the prevalence of infection is higher in ulcer patients than in the normal population are cogent arguments in favor of it being the primary cause of the ulceration. Against this concept there are issues that need explanation such as the reason why only a minority of infected persons develop duodenal ulceration when infection with H. pylori is widespread. There is evidence that H. pylori infection has been prevalent for several centuries, yet duodenal ulceration became common at the beginning of the twentieth century. The prevalence of duodenal ulceration is not higher in countries with a high prevalence of H. pylori infection. This paper debate puts forth the point of view of two groups of workers in this field whether H. pylori infection is the primary cause of duodenal ulcer disease or a secondary factor.

  9. Evaluation of endoscopically obtained duodenal biopsy samples from cats and dogs in an adapter-modified Ussing chamber

    Science.gov (United States)

    DeBiasio, John V.; Suchodolski, Jan S.; Newman, Shelley; Musch, Mark W.; Steiner, Jörg M.

    2014-01-01

    This study was conducted to evaluate an adapter-modified Ussing chamber for assessment of transport physiology in endoscopically obtained duodenal biopsies from healthy cats and dogs, as well as dogs with chronic enteropathies. 17 duodenal biopsies from five cats and 51 duodenal biopsies from 13 dogs were obtained. Samples were transferred into an adapter-modified Ussing chamber and sequentially exposed to various absorbagogues and secretagogues. Overall, 78.6% of duodenal samples obtained from cats responded to at least one compound. In duodenal biopsies obtained from dogs, the rate of overall response ranged from 87.5% (healthy individuals; n = 8), to 63.6% (animals exhibiting clinical signs of gastrointestinal disease and histopathological unremarkable duodenum; n = 15), and 32.1% (animals exhibiting clinical signs of gastrointestinal diseases and moderate to severe histopathological lesions; n = 28). Detailed information regarding the magnitude and duration of the response are provided. The adapter-modified Ussing chamber enables investigation of the absorptive and secretory capacity of endoscopically obtained duodenal biopsies from cats and dogs and has the potential to become a valuable research tool. The response of samples was correlated with histopathological findings. PMID:24378587

  10. The personality pattern of duodenal ulcer patients in relation to spontaneous ulcer healing and relapse

    DEFF Research Database (Denmark)

    Jess, P; von der Lieth, L; Matzen, Peter

    1989-01-01

    compared with 30% of the controls (P less than 0.0001). Neuroticism was connected with a high frequency of relapse (P less than 0.05) whereas failure of spontaneous ulcer healing had no certain relation to personality disorders. Patients with non-neurotic personality disorders had more frequently suffered......One hundred consecutive out-patients with duodenal ulceration from a hospital and a gastroenterological clinic were tested with the Minnesota Multiphasic Personality Inventory (MMPI). This was carried out in order to investigate whether neuroticism or other personality disorders were...... characteristics of duodenal ulcer patients, and whether the presence of such possible personality disorders might influence the prognosis of the disease. Neuroticism occurred in 53% of the patients, but only in 5% of controls (P less than 0.0001). Overall, personality disorders were present in 69% of the patients...

  11. Duodenal Lipomatosis as a Curious Cause of Upper Gastrointestinal Bleed: A Report with Review of Literature.

    Science.gov (United States)

    Zirpe, Dinesh; Wani, Majid; Tiwari, Priyanka; Ramaswamy, Palaniswamy Kalipatti; Kumar, Reddy Prasanna

    2016-05-01

    Lipomas of the gastrointestinal tract are rare. Duodenal lipomas are incidental and mostly asymptomatic. Tumours may produce symptoms of abdominal pain and discomfort or cause bleeding due to ulceration or intestinal obstruction due to intussusception. We describe a 45-year-old man presenting in emergency with 3 days history of melena with normal gastroduodenoscopy and contrast enhanced computed tomography revealing multiple polypoid lesion in duodenum and proximal jejunum suggestive of lipoma. Due to ongoing bleed, he underwent laparotomy with duodenectomy and uneventful postoperative recovery. Our review of cases published in last 67 years indicate that duodenal lipomas are rare to occur but commonly found in second part, they may be seen in third and fourth part of duodenum which may be missed on endoscopy. They can be multiple and may present as severe UGI bleeding which could be managed surgically. Though CT is diagnostic, histopathology confirms the diagnosis which shows lipomatous lesion composed of mature adipose arranged in lobules.

  12. Fatal upper gastrointestinal bleed arising from duodenal varices secondary to undiagnosed portal hypertension.

    Science.gov (United States)

    Larson, John V; Steensma, Elizabeth A; Burke, Leandra H; Bartholomew, David M

    2013-08-16

    Duodenal varices are an unexpected source of upper gastrointestinal haemorrhage associated with high mortality. The prevalence of ectopic variceal bleeding accounts for 2-5% of all variceal bleeding; of this, only 17% occurs in the duodenum. Diagnosis is difficult, and insufficient evidence exists to demonstrate the best treatment option when haemorrhage occurs. We report the case of a 69-year-old man with a history of chronic alcoholism who presented to the emergency department (ED) with nausea, vomiting and several episodes of haematochezia. Diagnostic workup in the ED included CT with multiplanar reconstruction, which revealed a network of large tortuous blood vessels running near the second portion of the duodenum between the inferior vena cava and portal vein. The patient was emergently treated with endoscopic therapy and clipping of the vessel. This failed, and he was subsequently taken to the operating room for suture ligation of the bleeding duodenal varices.

  13. The р53 expression in cells of marginal zone of duodenal ulcers during acute bleeding

    Directory of Open Access Journals (Sweden)

    Sulayeva O.N.

    2009-01-01

    Full Text Available The aim of the work was to estimate the intensity and tissue distribution of proapoptogen p53 expression in duodenal mucosa in patients with acute bleeding caused by peptic ulcers. In 31 patients (1st group the healing of ulcer was detected after effective endoscopic hemostasis and in 15 patients rebleeding took place (2nd group. Performed immunocytochemical investigation allow to determine that acute ulcer bleeding was associated with activation of marginal zone cells apoptosis which was maximal in covering epithelium, vascular endothelium and regions infiltrated by lymphocytes. In patients with rebleeding the higher values of р53 expression were detected in crypts epithelium and in endothelium of dilatating vessels accompanied with intensive edema and lymphocytes infiltration of lamina propria and muscularis mucosae with perifocal activation of cells death. Additionally to apoptosis in 2nd group duodenum the necrosis of cells and epithelium desquamation were found, reflecting the tissue disintegration of duodenal mucosa.

  14. Clinical and histological features of duodenal ulcer in children and adolescents

    OpenAIRE

    Elisabete Kawakami; Machado, Rodrigo S. [UNIFESP; Fonseca,Jacqueline A.; Francy R. S. PATRÍCIO

    2004-01-01

    OBJETIVO: Avaliar as características clínicas e histológicas de crian��as e adolescentes com úlcera duodenal. MÉTODOS: Foram avaliadas prospectiva e consecutivamente 43 pacientes com úlcera duodenal ao longo de 6 anos (7,2 pacientes por ano). A avaliação consistiu de questionário clínico de sintomas dispépticos, exame físico e endoscopia digestiva com biópsias gástricas para exame histológico e detecção do Helicobacter pylori. RESULTADOS: A idade no diagnóstico variou de 4 anos e 8 meses a 17...

  15. The role of colloidal bismuth subcitrate in the short-term treatment of duodenal ulcer.

    Science.gov (United States)

    Barbara, L; Corinaldesi, R; Rea, E; Paternicò, A; Stanghellini, V

    1986-01-01

    Colloidal bismuth subcitrate (CBS) is a drug used in the treatment of duodenal ulcer; it acts mainly by increasing mucosal resistance against endoluminal aggressive agents, without inhibiting gastric secretion. In previous clinical trials, CBS solution induced healing rates significantly higher than placebo and similar to those observed with cimetidine. In spite of these promising results, the drug has never been widely employed, mainly because of its unpleasant taste, which greatly reduced patient compliance. For this reason, chewing tablets have been introduced. CBS tablets have been reported to induce healing rates significantly higher than placebo and similar to those obtained with CBS solution, cimetidine, and ranitidine. CBS may therefore represent an important alternative to antisecretory drugs in the therapy of duodenal ulcer patients.

  16. Cysteamine-induced duodenal ulcer and acid secretion in the rat

    DEFF Research Database (Denmark)

    Poulsen, Steen Seier

    1980-01-01

    Duodenal ulcers can be produced in rats within 24 h by a single subcutaneous administration of cysteamine. To determine the role of gastric acid secretion in the pathogenesis of these ulcers, secretory and pathoanatomic studies were performed in chronic fistula rats ater an ulcerogenic dose...... of cysteamine. A prolonged increase of acid secretion was seen after cysteamine, reaching fourfold the basal level after 5 h. The acid response lasted for 10 to 11 h. After vagotomy cysteamine-induced acid secretion was markedly reduced. Ulcer formation was prevented by vagotomy and by drainage of the gastric...... for ulcer formation, the hypersecretion of acid induced by cysteamine is not the only factor responsible for the development of duodenal ulcer....

  17. Novel oesophago-gastro-duodenal stenting for gastric leaks after laparoscopic sleeve gastrectomy.

    Science.gov (United States)

    Liu, Shirley Yuk-Wah; Wong, Simon Kin-Hung; Ng, Enders Kwok-Wai

    2015-01-01

    The management of gastric leak after laparoscopic sleeve gastrectomy (LSG) can be complex and challenging. Whilst operative interventions are mostly complicated and reserved for unstable or refractory cases, endoscopic self-expandable metal stenting (SEMS) is increasingly preferred as a safer treatment option. Yet, SEMS carries the problems of frequent stent migration and inconsistent healing as ordinary SEMS is designed mainly for stenotic disease. We hereby present two cases of early and chronic post-LSG leakage that were respectively failed to be treated by surgery and ordinary SEMS but were successfully managed by a dedicated extra-long oesophago-gastro-duodenal stent. In oesophago-gastro-duodenal stenting, the characteristics of extra-long stent length allow total gastric exclusion between the mid-oesophagus and the first part of duodenum to prevent stent migration and to equalise high pressure gradient within the gastric sleeve to promote fistula healing.

  18. Biodistribution of the radiopharmaceutical sodium pertechnetate after biliopancreatic bypass with a duodenal switch

    Energy Technology Data Exchange (ETDEWEB)

    Araujo-Filho, Irami; Rego, Amalia Cinthia Meneses; Brandao-Neto, Jose; Villarim-Neto, Arthur; Egito, Eryvaldo Socrates Tabosa; Azevedo, Italo Medeiros; Medeiros, Aldo Cunha [Universidade Federal do Rio Grande do Norte, Natal, RN (Brazil). Programa de Pos-graduacao em Ciencias da Saude]. E-mail: aldo@ufrnet.br

    2007-09-15

    Study with the purpose to examine the effects of duodenal switch (DS), regularly performed in morbidly obese patients, on biodistribution of sodium pertechnetate in several organs of rats. There was no early or late mortality in either rats groups. The values of percent radioactivity per gram of tissue (%ATI/g), showed no significant difference in liver, stomach, small bowel, duodenum, kidney, heart, bladder, bone and brain, when compared the DS rats with sham and controls rats. A postoperative significant increase (p<0.05) in mean %ATI/g levels was observed in spleen, pancreas and muscle in group DS rats, as compared to group S and C rats. In the lung there was an increase and in thyroid a decrease in mean %ATI/g of DS rats, when compared to sham rats (p<0.05). In conclusion, the biliopancreatic diversion with duodenal switch in rats modified the biodistribution of sodium pertechnetate in thyroid, lung, pancreas, spleen and muscle. (author)

  19. Down syndrome: Molecular mapping of the congenital heart disease and duodenal stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Korenburg, J.R. (University of California, Los Angeles (United States)); Bradley, C.; Disteche, C.M. (University of Washington, Seattle (United States))

    1992-02-01

    Down syndrome (DS) is a major cause of congenital heart and gut disease and mental retardation. DS individuals also have characteristic facies, hands, and dermatoglyphics, in addition to abnormalities of the immune system, and increased risk of leukemia, and an Alzheimer-like dementia. Although their molecular basis is unknown, recent work on patients with DS and partial duplications of chromosome 21 has suggested small chromosomal regions located in band q22 that are likely to contain the genes for some of these features. The authors now extend these analyses to define molecular markers for the congenital heart disease, the duodenal stenosis, and an 'overlap' region for the facial and some of the skeletal features. They report the clinical, cytogenetic, and molecular analysis of two patients. These studies provide the molecular basis for the construction of a DS phenotypic map and focus the search for genes responsible for the physical features, congenital heart disease, and duodenal stenosis of DS.

  20. [Prospective study of 420 biopsies realised in patients with duodenal ulcer with positive Helicobacter pylori].

    Science.gov (United States)

    Khayat, Olfa; Kilani, Afef; Chedly-Debbiche, Achraf; Zeddini, Abdelfattah; Gargouri, Dalila; Kharrat, Jamel; Souissi, Adnene; Ghorbel, Abdel Jabbar; Ben Ayed, Mohamed; Ben Khelifa, Habib

    2006-06-01

    It's a prospective study leaded between September 1997 and july 1999 (23 months ) in 75 patients with duodenal ulcer and positif for Helicobacter pylori. All patients had a first endoscopy with antral, fundic and duodenal biopsies, followed one month later by a second control fibroscopy with biopsies of the same sites. A total of 420 biopsies was realised. Chronic gastritis was evaluated according to sydney system. Patients was divided by randomisation in 4 groups. Every group was received a different therapeutic association. The results was conform to liberation concering activity 80%, intestinal metaplasia 12%. inflammation 100%. Atrophy was observed in 56% of cases, this percentage is variable in literature; chronic gastritis was predominant in antre relatively to fundus (pomeprazol, amoxcillin, metronidazol by comparison with the others 3 groups (p<0.005).