463 results match your criteria Somatostatinomas


Coexistence of neurofibromatosis type 1 with multiple malignant neoplasia.

Neuro Endocrinol Lett 2018 Sep;39(3):149-155

Clinical Department of Endocrinology, Jagiellonian University in Krakow, Poland.

Neurofibromatosis type 1 (NF1, von Recklinghausen disease) is inherited in autosomal dominant way genetic disorder, with an incidence at birth 1:3000. It is one of the most common congenital disorders. It is characterized by café-au-lait spots, neurofibromas, and less common MPTST and gliomas of the optic nerve. Read More

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September 2018
3 Reads

Prognostic relevance of proliferation-related miRNAs in pancreatic neuroendocrine neoplasms

Eur J Endocrinol 2018 10 1;179(4):219-228. Epub 2018 Oct 1.

‘Lendület’ Hereditary Endocrine Tumours Research Group, Hungarian Academy of Sciences – Semmelweis University, Budapest, Hungary

Objective: Pancreatic neuroendocrine neoplasms (PanNENs) are rare tumors arising from the endocrine pancreas; however, their prognosis differs significantly upon their proliferative state, which is characterized by histopathological grading. MiRNAs are small, noncoding RNAs posttranscriptionally regulating gene expression. Our aim was to identify miRNAs with altered expression upon proliferation which can be used as prognostic biomarkers in PanNENs. Read More

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October 2018
8 Reads

Neoplasms of the Neuroendocrine Pancreas: An Update in the Classification, Definition, and Molecular Genetic Advances.

Adv Anat Pathol 2019 Jan;26(1):13-30

Departments of Pathology, Massachusetts General Hospital, Boston, MA.

This review focuses on discussing the main modifications of the recently published 2017 WHO Classification of Neoplasms of the Neuroendocrine Pancreas (panNEN). Recent updates separate pancreatic neuroendocrine tumors into 2 broad categories: well-differentiated pancreatic neuroendocrine tumors (panNET) and poorly differentiated pancreatic neuroendocrine carcinoma (panNEC), and incorporates a new subcategory of "well-differentiated high-grade NET (G3)" to the well-differentiated NET category. This new classification algorithm aims to improve the prediction of clinical outcomes and survival and help clinicians select better therapeutic strategies for patient care and management. Read More

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January 2019
18 Reads

Management of functional neuroendocrine tumors of the pancreas.

Authors:
Kjell Öberg

Gland Surg 2018 Feb;7(1):20-27

Department of Medical Sciences, Endocrine Oncology, Uppsala University Hospital, Uppsala, Sweden.

Pancreatic neuroendocrine tumors (pNETs) constitute a heterogenous group of malignancies with varying clinical presentation, tumor biology and prognosis. The incidence of pNETs has steadily increased during the last decades with an estimated incidence 2012 of 4.8/100,000. Read More

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February 2018
8 Reads

Somatostatinoma Masquerading as Chronic Pancreatitis.

Pancreas 2018 Apr;47(4):e19-e20

Department of Gastrointestinal Surgery and Liver Transplantation AIIMS New Delhi, India Department of Pathology AIIMS New Delhi, India Department of Radiodiagnosis AIIMS New Delhi, India Department of Gastrointestinal Surgery and Liver Transplantation AIIMS New Delhi, India.

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April 2018
4 Reads
2.960 Impact Factor

HIF-2alpha: Achilles' heel of pseudohypoxic subtype paraganglioma and other related conditions.

Eur J Cancer 2017 11 22;86:1-4. Epub 2017 Sep 22.

Section on Medical Neuroendocrinology, Eunice Kennedy Shriver NICHD, NIH, Building 10, CRC, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, MD 20892-1109, USA. Electronic address:

Paragangliomas (PGLs) belong to the most hereditary endocrine tumours. The existence of mutated HIF2A in these tumours, the role of oncometabolites on HIFs stabilisation and a recent concept proposing how hereditary PGLs converge on the hypoxia-signalling pathway, brought solid evidence of the existence of PGL hypoxiom. Hypoxia-inducible factor 2alpha (HIF-2α) antagonists -PT2385, and PT2399 have been shown to have promising results in the management of clear cell renal cell carcinoma by targeting the HIF-2α pathway in recent and ongoing clinical trials (PT2799). Read More

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November 2017
10 Reads

Mixed mucinous adenocarcinoma and somatostatinoma of the ampulla of Vater associated with neurofibromatosis type 1.

Pathology 2017 Aug 8;49(5):553-555. Epub 2017 Jul 8.

Department of Anatomical Pathology, St George Hospital, Sydney, NSW, Australia.

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August 2017
3 Reads

[Diabetes mellitus secondary to an endocrine pathology : when to think about it ?]

Rev Med Suisse 2017 May;13(565):1158-1162

Service d'endocrinologie, diabétologie, hypertension et nutrition, HUG, 1211 Genève 14.

An endocrine disease can be associated with glucose intolerance or diabetes mellitus, and the latter can falsely be considered as type 2 diabetes. Glycemic imbalance can be a direct or indirect consequence of excessive hormone production. Endocrine diseases such as acromegaly, Cushing's syndrome and pheochromocytoma can increase glucose production and cause insulin resistance. Read More

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May 2017
14 Reads

Course of Aggressive Somatotroph, Corticotroph and Mammotroph Tumors under Temozolomide; Report of Three Cases and Review of the Literature.

Turk Neurosurg 2017 May 7. Epub 2017 May 7.

Ankara University Faculty of Medicine, Department of Endocrinology and Metabolism.

Treatment of aggressive pituitary tumors may be challenging. Temozolomide (TMZ) is a promising agent when conventional treatment methods fail. We present three patients with aggressive pituitary tumors with atypical morphology, who were resistant to conventional treatments and treated with TMZ. Read More

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May 2017
12 Reads

Functional Imaging Signature of Patients Presenting with Polycythemia/Paraganglioma Syndromes.

J Nucl Med 2017 08 23;58(8):1236-1242. Epub 2017 Mar 23.

Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland

Pheochromocytoma/paraganglioma (PPGL) syndromes associated with polycythemia have previously been described in association with mutations in the von Hippel-Lindau gene. Recently, mutations in the prolyl hydroxylase gene () 1 and 2 and in the hypoxia-inducible factor 2 α () were also found to be associated with multiple and recurrent PPGL. Such patients also presented with PPGL and polycythemia, and later on, some presented with duodenal somatostatinoma. Read More

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August 2017
15 Reads

Novel insights into the polycythemia-paraganglioma-somatostatinoma syndrome.

Endocr Relat Cancer 2016 12 27;23(12):899-908. Epub 2016 Sep 27.

Section on Medical NeuroendocrinologyEunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

Worldwide, the syndromes of paraganglioma (PGL), somatostatinoma (SOM) and early childhood polycythemia are described in only a few patients with somatic mutations in the hypoxia-inducible factor 2 alpha (HIF2A). This study provides detailed information about the clinical aspects and course of 7 patients with this syndrome and brings into perspective these experiences with the pertinent literature. Six females and one male presented at a median age of 28 years (range 11-46). Read More

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December 2016
26 Reads

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

Neoplasia 2016 09;18(9):567-76

Section of Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA. Electronic address:

Recently, 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, VHL, FH, PHD1, and PHD2 genes have been associated with HIF activation and the development of pseudohypoxic (cluster-1) PGLs. These tumors overlap in terms of tumor location, syndromic presentation, and noradrenergic phenotype to a certain extent. Read More

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September 2016
28 Reads

Correlative Light- and Electron Microscopy Using Quantum Dot Nanoparticles.

J Vis Exp 2016 08 7(114). Epub 2016 Aug 7.

Correlative Microscopy Group, Ingham Institute for Applied Medical Research; Electron Microscopy Laboratory, Department of Anatomical Pathology, Sydney South West Pathology Service, New South Wales Health Pathology; School of Medical Sciences, Faculty of Medicine, University of New South Wales Australia.

A method is described whereby quantum dot (QD) nanoparticles can be used for correlative immunocytochemical studies of human pathology tissue using widefield fluorescence light microscopy and transmission electron microscopy (TEM). To demonstrate the protocol we have immunolabeled ultrathin epoxy sections of human somatostatinoma tumor using a primary antibody to somatostatin, followed by a biotinylated secondary antibody and visualization with streptavidin conjugated 585 nm cadmium-selenium (CdSe) quantum dots (QDs). The sections are mounted on a TEM specimen grid then placed on a glass slide for observation by widefield fluorescence light microscopy. Read More

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August 2016
6 Reads

Combined presence of multiple gastrointestinal stromal tumors along with duodenal submucosal somatostatinoma in a patient with neurofibromatosis type 1.

Indian J Pathol Microbiol 2016 Jul-Sep;59(3):359-61

Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.

Neurofibromatosis type-1 (NF-1) is an autosomal dominant disorder, with increased risk of developing benign and malignant tumors of the gastrointestinal tract (GIT). However, the synchronous presence of multiple GIT stromal tumors and duodenal submucosal somatostatinoma, like in this 50-year-old female NF-1 patient, is very rare. She presented with hematemesis, malena, along with multiple neurofibromas all over the body. Read More

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March 2017
14 Reads

Pancreatic neuroendocrine tumors: contemporary diagnosis and management.

Hosp Pract (1995) 2016 Aug 18;44(3):109-19. Epub 2016 Jul 18.

a Department of Internal Medicine, Division of Gastroenterology and Hepatology , University of Utah School of Medicine, Huntsman Cancer Center , Salt Lake City , Utah , USA.

Pancreatic neuroendocrine tumors (PNETs) are neoplasms that arise from the hormone producing cells of the islets of Langerhans, also known as pancreatic islet cells. PNETs are considered a subgroup of neuroendocrine tumors, and have unique biology, natural history and clinical management. These tumors are classified as 'functional' or 'non-functional' depending on whether they release peptide hormones that produce specific hormone- related symptoms, usually in established patterns based on tumor subtype. Read More

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August 2016
13 Reads

Somatostatinoma of the minor duodenal papilla associated with pancreas divisum treated by endoscopic papillectomy.

Endoscopy 2016 14;48 Suppl 1:E135-7. Epub 2016 Apr 14.

Departments of Surgery and Anatomy, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Ribeirão Preto, São Paulo, Brazil.

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January 2017
10 Reads

Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors.

Surg Oncol Clin N Am 2016 Apr 18;25(2):363-74. Epub 2016 Feb 18.

Department of Surgery, Helen F. Graham Cancer Center, 4701 Ogletown-Stanton Road, S-4000, Newark, DE 19713, USA.

Pancreatic neuroendocrine tumors are a rare group of neoplasms that arise from multipotent stem cells in the pancreatic ductal epithelium. Although they comprise only 1% to 2% of pancreatic neoplasms, their incidence is increasing. Most pancreatic neuroendocrine tumors are nonfunctioning, but they can secrete various hormones resulting in unique clinical syndromes. Read More

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April 2016
9 Reads

The Coexistence of Somatostatinoma and Gastrointestinal Stromal Tumor in the Duodenum of a Patient with Von Recklinghausen's Disease.

Intern Med 2016 15;55(6):617-22. Epub 2016 Mar 15.

Department of Gastroenterology and Hepatology, Saitama Medical Center, Saitama Medical University, Japan.

We herein describe a case of somatostatinoma coexisting with a gastrointestinal stromal tumor (GIST) in the duodenum of an 81-year-old woman with Von Recklinghausen's disease (VRD) and common bile duct stone who presented with diarrhea of three months in duration. Gastroduodenoscopy revealed an ulcer on the second part of the duodenum. A 2. Read More

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September 2016
11 Reads

A short history of neuroendocrine tumours and their peptide hormones.

Best Pract Res Clin Endocrinol Metab 2016 Jan 23;30(1):3-17. Epub 2015 Oct 23.

Yale University School of Medicine, New Haven, CT, USA.

The discovery of neuroendocrine tumours of the gastrointestinal tract and pancreas started in 1870, when Rudolf Heidenhain discovered the neuroendocrine cells, which can lead to the development of these tumours. Siegfried Oberndorfer was the first to introduce the term carcinoid in 1907. The pancreatic islet cells were first described in 1869 by Paul Langerhans. Read More

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January 2016
8 Reads

Somatostatinoma of the Ampulla: An Incidental Postoperative Finding Following Colorectal Cancer Resection.

ACG Case Rep J 2016 Jan 20;3(2):109-11. Epub 2016 Jan 20.

Department of Medicine, Division of Gastroenterology, Wake Forest Baptist Hospital, Winston-Salem, NC.

Somatostatinoma of the gastrointestinal tract is a rare finding, especially arising from the ampulla of Vater. We present a man recently diagnosed with rectal adenocarcinoma who was found on imaging to have an ampullary mass. Histology and immunohistochemical staining of the biopsied tissue were consistent with a nonfunctioning somatostatinoma. Read More

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January 2016
3 Reads

Somatostatin-Immunoreactive Pancreaticoduodenal Neuroendocrine Neoplasms: Twenty-Three Cases Evaluated according to the WHO 2010 Classification.

Neuroendocrinology 2016 28;103(5):567-77. Epub 2015 Oct 28.

Background/objective: Neuroendocrine neoplasms of the pancreas and duodenum with predominant or exclusive immunoreactivity for somatostatin (pdSOMs) are rare, and knowledge about tumour biology, treatment, survival and prognostic factors is limited. This study aims to describe clinical, pathological and biochemical features as well as treatment and prognosis of pdSOMs.

Design: Twenty-three patients with pdSOM (9 duodenal, 12 pancreatic and 2 unknown primary tumours) were identified from our prospective neuroendocrine tumour database, and data according to the study aims were recorded. Read More

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May 2017
4 Reads

Neuroendocrine tumour in a patient with neurofibromatosis type 1 and HIV.

South Afr J HIV Med 2015 26;16(1):323. Epub 2015 Jun 26.

Department of Internal Medicine, Tshepong Hospital, University of the Witwatersrand, South Africa.

We report the case of an HIV-positive female patient with neurofibromatosis type 1 who was treated for recurrent peptic ulcer disease and later developed diabetes mellitus and chronic diarrhoea. A metastasising somatostatinoma was histologically proven and evidence of a concomitant gastrin-producing neuroendocrine tumour was found. Neuroendocrine tumours (NETs) are very rare neoplasms originating from a wide variety of endocrine and nervous system tissue with the ability to produce different hormones. Read More

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June 2015
2 Reads

Clinical features of pancreatic neuroendocrine tumors.

J Hepatobiliary Pancreat Sci 2015 Aug 17;22(8):578-85. Epub 2015 Feb 17.

Neuroendocrine Tumor Unit, Endocrinology and Metabolism Service, Department of Medicine, Hadassah-Hebrew University Medical Center, P.O.B. 12000, Jerusalem, 91120, Israel.

Pancreatic neuroendocrine tumors (PNETs), also known as islet cell tumors, are rare neoplasms that arise in the endocrine tissues of the pancreas. Most of pancreatic NETs (50-75%) are nonfunctioning (not associated with a hormonal clinical syndrome); however, in up to one third they can secrete a variety of peptide hormones, including insulin, gastrin, glucagon, vasoactive intestinal peptide, somatostatin etc., resulting in rare but unique clinical syndromes. Read More

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August 2015
7 Reads
3 Citations
2.310 Impact Factor

Somatostatinoma of the minor papilla treated by local excision in a patient with neurofibromatosis type 1.

JOP 2015 Jan 31;16(1):81-4. Epub 2015 Jan 31.

Department of Surgery, University of Melbourne, Austin Health. Heidelberg, Melbourne, Victoria, Australia.

Context: Somatostatinoma arising from the minor papilla in a patient with neurofibromatosis type 1 (NF1) is a known but very rare condition, which may cause non-specific symptoms and can present because of its mass effect.

Case Report: A fifty-year-old female presenting with ongoing non-specific abdominal pain for a few months duration was found to have a mass involving the minor papilla. She had a history of NF1 but was otherwise well. Read More

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January 2015
7 Reads

Somatic mosaicism of EPAS1 mutations in the syndrome of paraganglioma and somatostatinoma associated with polycythemia.

Hum Genome Var 2015 10;2:15053. Epub 2015 Dec 10.

Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health , Bethesda, MD, USA.

We recently described a novel, non-inherited syndrome of tumor-specific mutations of hypoxia-inducible factor 2α, encoded by EPAS1, leading to formation of multiple paragangliomas and somatostatinomas in the setting of congenital polycythemia. Although we had suspected that somatic mosaicism of EPAS1 mutations was the underlying cause of tumorigenesis, we could not validate this theory in our initial findings. In this report, we developed a sensitive, peptide nucleic acid sequencing assay to uncover the presence of EPAS1 mutations in blood and other somatic tissues of the two patients who were described in the initial characterization of this syndrome. Read More

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April 2016
3 Reads

Endoscopic ultrasound-guided fine-needle aspiration diagnosis of mixed endocrine somatostatinoma of the minor papilla in von Recklinghausen disease.

Endoscopy 2014 19;46 Suppl 1 UCTN:E664-5. Epub 2014 Dec 19.

Endoscopy Service, Department of Diagnostic and Therapeutic Services, Mediterranean Institute for Transplantation and Advanced Specialized Therapies (ISMETT), Palermo, Italy.

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September 2015
5 Reads

Mixed periampullary adenocarcinoma and somatostatinoma with small bowel gastrointestinal stromal tumour in neurofibromatosis type 1.

JOP 2014 Nov 28;15(6):600-3. Epub 2014 Nov 28.

Department of Gastrointestinal Surgery, Nottingham Digestive Diseases Centre National Institute of Health Research Biomedical Research Unit, Nottingham University Hospitals NHS Trust, Queen's Medical Centre. Nottingham, UK.

Context: Gastrointestinal (GI) involvement is present in about one quarter of cases of neurofibromatosis type 1 (NF1). Adenocarcinomas have been reported in several organs. Gastrointestinal stromal tumors are the most common GI lesion seen in NFI. Read More

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November 2014
3 Reads

Ocular manifestations of hypoxia-inducible factor-2α paraganglioma-somatostatinoma-polycythemia syndrome.

Ophthalmology 2014 Nov 8;121(11):2291-3. Epub 2014 Aug 8.

Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.

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November 2014
41 Reads

HIF signaling pathway in pheochromocytoma and other neuroendocrine tumors.

Physiol Res 2014 ;63 Suppl 2:S251-62

Department of Internal Medicine, Faculty of Medicine, P. J. Šafárik University, Košice, Slovakia; Section on Medical Neuroendocrinology, Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver NICHD, NIH, Bethesda, MD, USA.

Hypoxia-inducible factors (HIFs) are transcription factors controlling energy, iron metabolism, erythropoiesis, and development. Dysregulation of these proteins contributes to tumorigenesis and cancer progression. Recent findings revealed the important role of HIFs in the pathogenesis of neuroendocrine tumors, especially pheochromocytoma (PHEO) and paraganglioma (PGL). Read More

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January 2015
1 Read

Polycythemia and paraganglioma with a novel somatic HIF2A mutation in a male.

Pediatrics 2014 Jun 12;133(6):e1787-91. Epub 2014 May 12.

Departments of Pediatrics.

Recently, a new syndrome of paraganglioma, somatostatinoma, and polycythemia has been discovered (known as Pacak-Zhuang syndrome). This new syndrome, with somatic HIF2A gain-of-function mutations, has never been reported in male patients. We describe a male patient with Pacak-Zhuang syndrome who carries a newly discovered HIF2A mutation. Read More

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June 2014
11 Reads

A new twist in neuroendocrine tumor research: Pacak-Zhuang syndrome, HIF-2α as the major player in its pathogenesis and future therapeutic options.

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2014 Jun 29;158(2):175-80. Epub 2014 Apr 29.

st Department of Internal Medicine, Medical Faculty, P.J. Safarik University, Trieda SNP 1, 04011, Kosice, Slovak Republic.

Unlabelled: Backround. There is increasing evidence of the role of hypoxia or pseudohypoxia in tumorigenesis, including pheochromocytoma (PHEO) and paraganglioma (PGL). (Pseudo)hypoxia leads to activation of hypoxia-inducible transcription factors (HIFs) and thus, promotes the transcription of hypoxia-responsive genes which are involved in tumorigenesis. Read More

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June 2014
7 Reads

Education and imaging. Gastrointestinal: neurofibromatosis type 1, duodenal somatostatinoma and gastrointestinal stromal tumors; a triad worth remembering.

Authors:
B Njei H Sanchez

J Gastroenterol Hepatol 2014 Apr;29(4):663

Department of Medicine, University of Connecticut, Farmington, USA.

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April 2014
5 Reads

Cotargeting the PI3K and RAS pathways for the treatment of neuroendocrine tumors.

Clin Cancer Res 2014 Mar 17;20(5):1212-22. Epub 2014 Jan 17.

Authors' Affiliations: Departments of Surgery, Internal Medicine, and Biostatistics; Markey Cancer Center, University of Kentucky, Lexington, Kentucky; and Department of Surgery, University of Texas Medical Branch, Galveston, Texas.

Background: The precise involvement of the PI3K/mTOR and RAS/MEK pathways in carcinoid tumors is not well defined. Therefore, the purpose of our study was to evaluate the role these pathways play in carcinoid cell proliferation, apoptosis, and secretion and to determine the effects of combined treatment on carcinoid tumor inhibition.

Methods: The human neuroendocrine cell lines BON (pancreatic carcinoid), NCI-H727 (lung carcinoid), and QGP-1 (somatostatinoma) were treated with either the pan-PI3K inhibitor, BKM120, or the dual PI3K-mTOR inhibitor, BEZ235, alone or in combination with the MEK inhibitor, PD0325901; proliferation, apoptosis, and protein expression were assessed. Read More

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March 2014
9 Reads
19 Citations
8.720 Impact Factor

Pancreatic somatostatinoma diagnosed preoperatively: report of a case.

JOP 2014 Jan 10;15(1):66-71. Epub 2014 Jan 10.

Department of Surgery, School of Medicine, University of Occupational and Environmental Health. Kitakyushu, Japan.

Context: Somatostatinoma is a rare neoplasm of the pancreas. Preoperative diagnosis is often difficult.

Case Report: We report a 72-year-old woman with a pancreatic head tumor measuring 37 mm in diameter, and enlargement of the lymph nodes on the anterior surface of the pancreatic head and the posterior surface of the horizontal part of the duodenum. Read More

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January 2014
9 Reads

Mosaicism in HIF2A-related polycythemia-paraganglioma syndrome.

J Clin Endocrinol Metab 2014 Feb 25;99(2):E369-73. Epub 2013 Nov 25.

Assistance Publique-Hôpitaux de Paris (A.B., C.S., A-P.G.-R.), Hôpital Européen Georges Pompidou (HEGP), Service de Génétique, 75908 Paris, France; Clinique d'Endocrinologie (S.S., M.L., A.M., B.C.), l'Institut du Thorax, Centre Hospitalier Universitaire (CHU) de Nantes, 44007 Nantes, France; Département d'Oncologie et d'Hématologie Pédiatriques et de Thérapie Cellulaire (L.M.), CHU de Nancy, Hôpital d'Enfants, 54500 Vandœuvre-lès-Nancy, France; Inserm (M.M., J.F., A.-P.G.-R.), Unité Mixte de Recherche 970, Centre de recherche cardiovasculaire de l'HEGP, 75015 Paris, France; Service d'Anatomopathologie (M.-F.H.), CHU de Nantes, 44007 Nantes, France; INSERM (M.-F.H.), UMR957, Faculté de Médecine, 44035 Nantes, France; and Université Paris Descartes (J.F., A.-P.G.-R.), Faculté de Médecine, 75006 Paris Cité Sorbonne, France.

Context: 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.

Patients: Patient 1 is a woman suffering from polycythemia diagnosed at the age of 16 years. Read More

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February 2014
7 Reads

Gastric somatostatinoma: an extremely rare cause of upper gastrointestinal bleeding.

Clin Endosc 2013 Sep 30;46(5):582-5. Epub 2013 Sep 30.

Division of Gastroenterology, Department of Internal Medicine, Siriraj Hospital, Mahidol University Faculty of Medicine, Bangkok, Thailand.

A 49-year-old woman presented with chronic abdominal discomfort, significant weight loss, and chronic intermittent diarrhea. She suddenly developed massive upper gastrointestinal bleeding and was referred for further treatment. Endoscopy indicated a large mass in the upper gastric body with antral and duodenal bulb involvement. Read More

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September 2013
3 Reads

[New considerations in the surgical treatment of pancreatic neuroendocrine tumours].

Authors:
Gyula Farkas

Orv Hetil 2013 Sep;154(39):1565-8

Szegedi Tudományegyetem, Általános Orvostudományi Kar Sebészeti Klinika Szeged Szőkefalvi-Nagy B. u. 6. 6720.

Except insulinomas, pancreatic neuroendocrine tumours are malignant in more than 50% of cases. Treatment of these tumours should be directed against the tumours and the excess of hormones produced by the tumours. Surgical treatment may reduce the mortality and morbidity of patients and, in case of localized lesions, may result in a definitive cure. Read More

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September 2013
7 Reads

PGP 9.5 immunocytochemical staining for pancreatic endocrine tumors.

Authors:
Tatsuo Tomita

Islets 2013 May-Jun;5(3):122-8. Epub 2013 Jun 27.

Departments of Integrative Biosciences and Pathology; Oregon National Primate Center; Oregon Health and Science University; Portland, OR USA.

Aims/hypothesis: Protein gene product 9.5 (PGP 9.5) is a marker for neuroendocrine cells but has not been used for pancreatic islet cells and pancreatic endocrine tumors (PETs). Read More

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May 2014
3 Reads

Duodenal somatostatinoma: a case report and review of the literature.

J Med Case Rep 2013 Apr 25;7:115. Epub 2013 Apr 25.

General Surgery Department, Istanbul Education and Research Hospital, Org, Abdurrahman Nafiz Gurman Street, Fatih Istanbul, 34096, Turkey.

Introduction: About 70% of well-differentiated endocrine tumors arise from the gastrointestinal tract. Duodenal well-differentiated endocrine tumors account for only 2.6% of all neuroendocrine tumors. Read More

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April 2013
4 Reads

Patient with adult-onset type II citrullinemia beginning 2 years after operation for duodenal malignant somatostatinoma: Indication for liver transplantation.

Hepatol Res 2013 May;43(5):563-8

Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto.

We report a 51-year-old female patient with adult-onset type II citrullinemia (CTLN2) who had a history of pancreatoduodenectomy for duodenal somatostatinoma with metastases to regional lymph nodes at age 49 years, paying special attention to indications for liver transplantation. At age 50 years, she developed hepatic encephalopathy with elevation of plasma ammonia and citrulline levels. A diagnosis of CTLN2 was made by DNA analysis of the SLC25A13 gene and treatment with conservative therapies was begun, including a low-carbohydrate diet and supplementation with arginine and sodium pyruvate. Read More

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May 2013
10 Reads

Biochemical markers for gastroenteropancreatic neuroendocrine tumours (GEP-NETs).

Best Pract Res Clin Gastroenterol 2012 Dec;26(6):791-802

University of Athens Medical School, Athens, Greece.

Biochemical markers are applied in gastroenteropancreatic neuroendocrine tumours (GEP-NETs) for diagnostic, prognostic or predictive purposes. Chromogranin A is the most important general marker and it is recommended to be measured in every patient with a suspected NET, whereas Neuron Specific Enolase is elevated mainly in poorly differentiated NETs. Pancreatic Polypeptide is used in the diagnosis of pancreatic non-functioning NETs, whereas Chorionic Gonadotrophin has an adjunctive role. Read More

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December 2012
4 Reads

Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances.

Best Pract Res Clin Gastroenterol 2012 Dec;26(6):737-53

Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.

Pancreatic neuroendocrine tumors (pNETs) comprise with gastrointestinal carcinoids, the main groups of gastrointestinal neuroendocrine tumors (GI-NETs). Although these two groups of GI-NETs share many features including histological aspects; over-/ectopic expression of somatostatin receptors; the ability to ectopically secrete hormones/peptides/amines which can result in distinct functional syndromes; similar approaches used for tumor localization and some aspects of treatment, it is now generally agreed they should be considered separate. They differ in their pathogenesis, hormonal syndromes produced, many aspects of biological behaviour and most important, in their response to certain anti-tumour treatment (chemotherapy, molecular targeted therapies). Read More

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December 2012
4 Reads

Case report: recurrent acute pancreatitis secondary to papillary somatostatinoma--a new association.

Pancreatology 2013 Mar-Apr;13(2):186-8. Epub 2013 Feb 9.

Department of Gastroenterology, Royal Berkshire Hospital, London Road, Reading RG1 5AN, United Kingdom.

Acute pancreatitis may rarely be caused by papillary mass lesions such as adenocarcinomas and neuroendocrine tumours. Occasionally these papillary lesions may cause recurrent episodes of acute pancreatitis and patients presenting in this way require further pancreatic investigation. We believe this to be the first reported case of a duodenal papillary somatostatinoma causing recurrent acute pancreatitis. Read More

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October 2013
4 Reads

Incidence, hormonal distribution and postoperative follow up of atypical pituitary adenomas.

Turk Neurosurg 2013 ;23(2):226-31

Ankara Numune Research and Education Hospital, Department of Neurosurgery, Ankara, Turkey.

Aim: To assess the incidence, hormonal activity and postoperative follow up of the cases that are histopathologically diagnosed as atypical pituitary adenoma (APA) in our series.

Material And Methods: In this study, 13 atypical pituitary adenoma cases, by the WHO 2004 criteria, among the 146 pituitary adenoma patients operated on in our clinic between January 2009 and May 2012 by endoscopic endonasal transsphenoidal approach were included.

Results: In histological studies, 133 cases were diagnosed as typical pituitary adenoma (91. Read More

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September 2013
4 Reads

First report of bilateral pheochromocytoma in the clinical spectrum of HIF2A-related polycythemia-paraganglioma syndrome.

J Clin Endocrinol Metab 2013 May 28;98(5):E908-13. Epub 2013 Mar 28.

Department of Nuclear Medicine, La Timone University Hospital, Centre Européen de Recherche en Imagerie Médicale, Aix-Marseille University, 13005 Marseille, France.

Context: Molecular genetic research has so far resulted in the identification of 10 well-characterized susceptibility genes for hereditary pheochromocytoma (PHEO) or paraganglioma (PGL). Recently, a new syndrome characterized by multiple PGLs and somatostatinomas associated with congenital polycythemia due to somatic mutations in HIF2A has been reported.

Objective: The aim of the study was to define the genetic defect in a new case of bilateral PHEO and multiple PGLs associated with congenital polycythemia. Read More

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May 2013
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New syndrome of paraganglioma and somatostatinoma associated with polycythemia.

J Clin Oncol 2013 May 18;31(13):1690-8. Epub 2013 Mar 18.

Section on Medical Neuroendocrinology, Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver NICHD, NIH, Bethesda, MD 20892-1109, USA.

Purpose: The occurrence of ≥ two distinct types of tumors, one of them paraganglioma (PGL), is unusual in an individual patient, except in hereditary cancer syndromes.

Patients And Methods: Four unrelated patients were investigated, with thorough clinical evaluation. Plasma and tissue catecholamines and metanephrines were measured by high-performance liquid chromatography. Read More

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May 2013
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