Neoplasms of the Neuroendocrine Pancreas: An Update in the Classification, Definition, and Molecular Genetic Advances
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 be...
Source: Advances in Anatomic Pathology - December 7, 2018 Category: Pathology Tags: Review Articles Source Type: research
Perioperative Hypotensive Crisis in an Adolescent with a Pancreatic VIPoma and MEN1-Gene Variant
Conclusion: This case highlights the importance of screening for a VIPoma in patients with high-volume secretory diarrhoea and preventing cardiovascular complications with perioperative VIP suppression. Furthermore, careful interpretation of genetic results within the clinical context is required.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - October 16, 2018 Category: Endocrinology Source Type: research
Results of Duodenopancreatic Reoperations in Multiple Endocrine Neoplasia Type 1
ConclusionReoperations are frequently necessary for dpNEN in MEN1 patients, but are not associated with an increased perioperative morbidity in specialized centers. Organ-sparing resections should be preferred as initial duodenopancreatic procedures to maintain pancreatic function and avoid completion pancreatectomy. (Source: World Journal of Surgery)
Source: World Journal of Surgery - October 4, 2018 Category: Surgery Source Type: research
[Surgical strategies for small sporadic neuroendocrine pancreatic tumors].
Abstract
Small (<2 cm) sporadic neuroendocrine pancreatic neoplasms (pNENs) are a heterogeneous group of neoplasms, which can be separated into functionally active or non-functional tumors. Functionally active pNENs release various hormones, such as insulin, gastrin, glucagon and vasoactive intestinal hormones and therefore lead to severe symptoms independent of their size. The main symptoms include hypoglycemia, coma (insulinoma), diarrhea (gastrinoma, VIPoma), intestinal ulcers (gastrinoma) and hyperglycemia (glucagonoma). Asymptomatic inactive pNENs do not cause a hormonal syndrome even though in ...
Source: Der Chirurg - April 10, 2018 Category: Surgery Authors: Holzer K Tags: Chirurg Source Type: research
Verner-Morrison syndrome. Literature review.
In conclusion, the physicians should be aware that there are some rare tumoral causes of chronic diarrhea, often under-diagnosed. If the diagnosis is not considered, extensive gastrointestinal investigations will be undertaken, delaying the diagnosis and avoidable morbidity will occur.
PMID: 28730220 [PubMed - in process] (Source: Romanian Journal of Morphology and Embryology)
Source: Romanian Journal of Morphology and Embryology - July 23, 2017 Category: General Medicine Tags: Rom J Morphol Embryol Source Type: research
Percutaneous Irreversible Electroporation for Pancreatic VIPoma: A Case Report
The purpose of this study was to investigate the safety and efficacy of irreversible electroporation (IRE) for the management of unresectable pancreatic vasoactive intestinal peptide tumor (VIPoma) in a 34-year-old male patient. The initial symptom was watery diarrhea, which could not be stopped by fasting. Laboratory tests revealed high vasoactive intestinal peptide (VIP) hormone levels, hypokalemia, and metabolic acidosis. Computed tomography examination showed a 6.0 × 5.0-cm, contrast-enhanced lesion in the neck and body of the pancreas and obliteration of the portal vein. Pathological and immunohistochemical findings ...
Source: Pancreas - December 16, 2016 Category: Gastroenterology Tags: Case Report Source Type: research
Pancreatic neuroendocrine tumor secreting vasoactive intestinal peptide and dopamine with pulmonary emboli: a case report.
CONCLUSIONS: VIPoma is a rare subtype of pancreatic neuroendocrine tumor that can secrete dopamine and can be associated with thromboembolism.
PMID: 27583474 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)
Source: The Journal of Clinical Endocrinology and Metabolism - August 31, 2016 Category: Endocrinology Authors: Nilubol N, Freedman EM, Quezado MM, Patel D, Kebebew E Tags: J Clin Endocrinol Metab Source Type: research
Emergency therapy for liver metastases from advanced VIPoma: surgery or transarterial chemoembolization?
VIPoma is a rare neuroendocrine tumor (NET) with a high potential to develop hepatic metastases and poor prognosis. The primitive tumor is nonsymptomatic and usually localized within the pancreas. Liver metastasis drives the prognosis and induces profuse watery diarrhea or renal failure. We herein present severe renal failure or diarrhea in two patients hospitalized in intensive care justifying emergency treatment of liver metastasis. The two patients experienced severe diarrhea due to a hypersecretion of vasoactive intestinal peptide (VIP) from liver metastasis released into the blood circulation. Therapeutic management w...
Source: Therapeutic Advances in Medical Oncology - August 7, 2016 Category: Cancer & Oncology Authors: Dreanic, J., Lepere, C., El Hajjam, M., Gouya, H., Rougier, P., Coriat, R. Tags: Reviews Source Type: research
VIPomas: Report of three cases with pancreatic/small gut primary NET
Introduction: VIPoma is a rare neuroendocrine tumor (NET) with un incidence extimated of 1/10.000.000/year. Most cases arise within the pancreas, with others reported in the lungs, colon, liver, adrenals and neuroganglia. (Source: Pancreatology)
Source: Pancreatology - May 31, 2016 Category: Gastroenterology Authors: Valbona Lico, Anna Caterina Milanetto, Eugenio De Carlo, Rita Alaggio, Sergio Pedrazzoli, Claudio Pasquali Source Type: research
Ventricular fibrillation resulting from electrolyte imbalance reveals vipoma in MEN1 syndrome
Abstract
Sporadic VIPoma is an exceedingly rare tumor with an annual incidence of 1:10 million people worldwide, yet it is described in approximately 5 % of MEN1 patients. The majority of VIPomas are malignant and radical surgery is the best therapeutic option. A 58-year-old man presented with cardiocirculatory arrest due to ventricular fibrillation. The patient had a 3-month history of epigastric pain with diarrhea. After reanimation, laboratory data revealed severe hypokalemia and hypercalcemia. Further investigations showed hyperparathyroidism, left adrenal adenoma and pituitary microprolactinoma and ...
Source: Familial Cancer - April 11, 2016 Category: Cancer & Oncology Source Type: research
Surgical resection of vasoactive intestinal peptideoma with hepatic metastasis aids symptom palliation: A case report.
Authors: Zhang X, Zhou L, Liu Y, Li W, Gao H, Wang Y, Yao B, Jiang D, Hu P
Abstract
Vasoactive intestinal peptideoma (VIPoma) is a rare pancreatic endocrine tumor associated with a well-defined clinical syndrome characterized by watery diarrhea, hypokalemia and metabolic acidosis. In adults, VIPoma is most commonly found in the pancreas, with 80% of the tumors occurring in the body and tail and 20% occurring in the pancreatic head. VIPomas can represent a significant diagnostic challenge due to their nonspecific clinical presentation, which can result in the misdiagnosis of a VIPoma as another condition, s...
Source: Experimental and Therapeutic Medicine - March 23, 2016 Category: Journals (General) Tags: Exp Ther Med Source Type: research
Epidemiological features of gastroenteropancreatic neuroendocrine tumors in Chengdu city with a population of 14 million based on data from a single institution
ConclusionsThere is a distinct epidemiologic profile between West China and western countries based on a single institution data. The delayed diagnosis reflects inadequate disease awareness of GEP‐NETs and paucity of research funding. (Source: Asia-Pacific Journal of Clinical Oncology)
Source: Asia-Pacific Journal of Clinical Oncology - February 29, 2016 Category: Cancer & Oncology Authors: Lin‐Jie GUO, Chun‐Hui WANG, Cheng‐Wei TANG Tags: ORIGINAL ARTICLE Source Type: research
When Symptomatic Treatment Becomes Antitumor Treatment for Vipoma: Opportunity for Frail Elderly Adults
(Source: Journal of the American Geriatrics Society)
Source: Journal of the American Geriatrics Society - February 18, 2016 Category: Geriatrics Authors: Claire Godard‐Sebillotte, Moustapha Dramé, Cédric Fagour, Tatiana Basileu, Lidvine Godaert Tags: Letters to the Editor Source Type: research
[Comment] Universal everolimus for malignant neuroendocrine tumours?
Neuroendocrine neoplasms are a heterogeneous group of malignant tumours which vary in localisation, clinical presentation, tumour biology, and prognosis. The tumours derive from the diffuse neuroendocrine cell system, which is spread all over the body. The main tissues affected are thymus, lungs, gastrointestinal tract, and pancreas. Some tumours present clinical syndromes related to peptide and amine production with clinical syndromes like gastrinoma, insulinoma, VIPoma, and glucagonoma syndromes, and the carcinoid syndrome related to serotonin and tachykinin production from small intestinal neuroendocrine tumours (carcin...
Source: LANCET - December 15, 2015 Category: Journals (General) Authors: Kjell Öberg Tags: Comment Source Type: research
