Abstract Library

#883 Primary Hepatic Neuroendocrine Tumors: Four Familial Case Series with Review of Literature

Introduction: Non-multiple endocrine neoplasia (MEN) familial neuroendocrine tumors (NET) are very rare with only six families being described to date. Primary hepatic neuroendocrine tumors (PHNETs) are rare tumors with a particular sporadic diagnosis. Herein, we report a series of four members of one Lebanese family, diagnosed with primary hepatic neuroendocrine tumors.

Conference: 11th Annual ENETSConcerence (2014)

Presenting Author: Assi R

Authors: Assi R, Haidar A, Khalife M, Temraz S, Mukherji D,

Keywords: familial primary hepatic neurendocrine tumors, genetic studies ,

#465 Management of an Advanced Pancreatic Neuroendocrine Tumor

Introduction: Whereas an array of treatment modalities have evolved for management of pancreatic NETs, there has been a lack of information regarding a clear algorithm of optimal treatment or an appropriate sequence in which different treatment modalities can be applied to a given patient for optimal results.

Conference: 9th Annual ENETSConcerence (2012)

Presenting Author:

Authors: Ahmed M, Al-Hindi H,

Keywords: pancreatic NETs, Everolimus ,

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

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.

Conference: 8th Annual ENETSConcerence (2011)

Presenting Author:

Authors: Ahmed M, Al Qaraawi A, Al Faifi J,

Keywords: MEN 1 syndrome, insulinoma, genetic analysis,

#177 Dissociation Between Iodine-131 meta-iodobenzylguanidine (MIBG) Scintigraphy and Radiolabeled Octreotide in the Localization and Management of Sporadic Malignant Pheochromocytoma: An Impact on Management

Introduction: The Rx of malignant pheochromocytomas w/negative MIBG scan remains a challanging problem. Octreoscan is helpful in localization and Rx planning in such cases. Rx using SST analog & PRRT is a desirable goal.

Conference: 8th Annual ENETSConcerence (2011)

Presenting Author:

Authors: Ahmed M,

Keywords: malignant pheochromocytoma, octreotide scintigraphy, [(131)I]MIBG, SST analogs ,

#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 ENETSConcerence (2010)

Presenting Author: Ahmed D

Authors: Ahmed D, Amin D, Al Faraj D, Al Qahtani D,

Keywords: paraganglioma, succinate dehydrogenase B mutation, abdominal tumor, malignant/metastatic paraganglioma, serum urinary, catecholamines,