Abstract Library

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#261 Recognizing Carcinoid Heart Disease and Timing of Cardiac and Abdominal Procedures

Introduction: Recognition of right heart failure in Carcinoid Heart Disease enables a decision to be made concerning the proper timing of Cardiac and abdominal tumor-reductive surgery.

Conference: 8th Annual ENETSConcerence (2011)

Presenting Author:

Authors: Zacks J, Shafir M, Castillo J, Choi J, Warner R,

Keywords: carcinoid heart disease, valve surgery, right heart failure, abdominal carcinoid, neuroendocrine ,

#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,