A Variant Pancreatic Insulinoma in MEN1 Syndrome Characterized by Normoglycemia/Normoinsulinemia but Abnormal C-Peptide and Abnormal Proinsulin Levels Abstract #179

Introduction: Insulinoma associated w/ hyperinsulinemia is frequent in functioning pancreatic NET in MEN 1. We report on familial variant insulinoma w/ normoglycemia/norminsulinemia, but abnormal C-peptide and proinsulin.
Aim(s): To draw attention to variant pancreatic insulinoma in MEN 1 syndrome, characterized by normoglycemia/normoinsulinemia but abnormal C-peptide & proinsulin.
Materials and methods: A 45-year-old pt. (index Pt.) and his 18-year-old daughter with hyperparthyroidism and prolactinoma underwent determination of gastrin (normal), VIP (normal), CgA, CT pancreas and extended fast monitoring glucose, insulin, C-peptide (CP) and proinsulin (PI). Genetic studies were also done. Brother of index pt. and three of his children also have MEN 1.
Conference: 8th Annual ENETS Conference (2011)
Category: Clinical
Presenting Author: Dr. Mohammed NMI Ahmed

To read results and conclusion, please login ...

Further abstracts you may be interested in

#40 Clinical and prognostic implications of the genetic diagnosis of hereditary NET syndromes in asymptomatic patients
Introduction: Neuroendocrine tumors (NETs) are rare neoplasms, heterogeneous, and with variable biological behavior. NETs can be sporadic or they can arise in complex endocrine hereditary syndromes such as Multiple Endocrine Neoplasias (MEN), Familial Paragangliomatosis (FPGL), Neurofibromatosis type 1 (NF1), von Hippel-Lindau Disease (VHL), Tuberous Sclerosis (TSC) and Carney Complex (CC). By performing genetic screening, patients with hereditary NETs can be identified before the development of tumors.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Antongiulio Faggiano
#91 Endoscopic ultrasound of 66 insulinomas
Introduction: Ultrasound is the virtual representation of the morphology of organs and tissues. Endoscopic ultrasound can be used in detailed scanning of pancreas and preoperative topical diagnostics of insulinomas. Insulinoma requires surgical treatment in all cases. This fact allows control of preoperative diagnostic methods.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: MD Tatiana L. Silina
#22 A Case Illustrative of Phenotypic Heterogeneity and Challenges in the Management of Paraganglioma
Introduction: Paragangliomas (PGLs) are extra-adrenal, usually benign, highly vascularized tumors that originate from neural-crest-derived chromaffin cells. These tumors are subdivided as either sympathetic or parasympathetic, depending on their location and catecholamine production. Sympathetic PGLs are situated along the abdominal sympathetic trunk and usually produce catecholamines, whereas parasympathetic PGLs are located in the head and neck, and these usually do not produce catecholamines. PGLs may present as sporadic or inherited tumor syndrome, including MEN 2, with RET germline mutations, von Hippel-Lindau (VHL) disease due to germline mutations in VHL gene, and pheochromocytoma-PGL syndrome. The latter is frequently a hereditary condition and is caused by germline mutations in the SDHB, SDHC, or SDHC genes. Patients with familial PGLs may present at a younger age, often as multifocal tumors, with an increased risk of recurrence and a higher frequency of malignancy in those with SDHB mutations. SDH mutations induce angiogenesis and tumorogenesis through the inhibition of hypoxia-inducible factors (HIF)-propyl hyroxylase. A younger age at onset, malignancy, and a positive family history are clinical parameters of high specificity, but low sensitivity for diagnosis. Genetic analysis for mutations in SDH genes for the patient and family members, and surveillance for the affected patient and family members, are necessary where there are no clear clinical or family indicators for the syndrome. We present a case of a large abdominal malignant PGL in a 20-year-old pt. that went on without clinical detection for at least three years.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Dr Mohammed Ahmed
#58 Surgical approaches in 84 patients with insulinomas in multiple endocrine neoplasia type 1 (MEN 1)
Introduction: Management of insulinomas in the setting of Multiple Endocrine Neoplasia type 1 (MEN1) remains controversial.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Dr Delphine Vezzosi
#50 Clinical-morphology correlation in diagnostics and evaluating surgery results of pancreatic insulinoma and nesidiobastosis
Introduction: Organic hyperinsulinism syndrome (OHS) is revealed in 85% cases of pancreas insulinoma. Nesidioblastosis (NB) induces OHS in 15% of the cases. Surgical cure provides all symptoms of insulinoma disappearing, while cytoreductive operation results in considerable improvement of life quality. There is no intraoperative opportunity to reveal insulinoma grade, even if an urgent biopsy is performed. Thus, it is essential to determine the cause of OHS and to apply the surgical cure method during the preoperational period, thereby improving the long-term prognosis.

Conference: 7th Annual ENETS Conference (2010)
Category: Basic
Presenting Author: Dr. Evelina Askenderova
Close
Notice
Important Notice:

In preparation of the upcoming ENETS Barcelona 2018 Annual Conference, we have discovered in the world wide web at least one professional entity suggestive of possessing an ENETS mandate for conference registrations. Therefore, we must inform you that 100% of all conference participants are registered through ENETS official website www.enets.org and http://enetsconference.org/. There are no further options to validly register for Barcelona 2018 (or for any other ENETS event).

Please stay away from fraudulent scams abusing the ENETS acronym to register – and charge you above official ENETS conference fees! Such entities, against whom ENETS presses criminal charges, are neither authorized, nor commissioned nor instructed by ENETS to make such representations.