World Journal of Endocrine Surgery

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VOLUME 14 , ISSUE 1 ( January-April, 2022 ) > List of Articles

REVIEW ARTICLE

Retroperitoneal Paraganglioma often Atypical: Short Case Series and Review of the Literature

Kah Heng Alexander Lim, Daniel Spernat, Christine Su Li Lai, David CA Walsh

Keywords : Extra-adrenal pheochromocytoma, Paragangliomas, Pheochromocytoma, Retroperitoneal neoplasm

Citation Information : Lim KH, Spernat D, Lai CS, Walsh DC. Retroperitoneal Paraganglioma often Atypical: Short Case Series and Review of the Literature. World J Endoc Surg 2022; 14 (1):15-20.

DOI: 10.5005/jp-journals-10002-1418

License: CC BY-NC 4.0

Published Online: 16-07-2022

Copyright Statement:  Copyright © 2022; The Author(s).


Abstract

Retroperitoneal paragangliomas (rPGL) are rare chromaffin cell tumors traditionally associated with symptoms of catecholamine excess; however, this notion is currently in question. Three cases of rPGL that were presented consecutively to our center over a period of 6 months are discussed. Each presented under completely different circumstances; the first was detected during diagnostic workup for weight loss; the second was found synchronously during workup for gynecological malignancy; and the third was diagnosed postoperatively following resection of an incidentally detected, asymptomatic retroperitoneal tumor. In all cases, typical symptoms were absent from the history. Recent advancements and ongoing gaps in understanding of this rare entity are reviewed including theories of tumor phenotype, the outdated notion of classical symptomatology, and the emerging importance of succinate dehydrogenase (SDH) gene mutations for apparent sporadic rPGL as well as disease prognostication. Pitfalls in diagnosis and management of this frequently silent but potentially catastrophic and malignant tumor are discussed. rPGL is a complex clinical entity that may not present with typical symptoms of catecholamine excess; a high index of suspicion of rPGL is necessary for all clinicians dealing with retroperitoneal tumors.


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