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Surgical Management of the Succinate Dehydrogenase–Associated Familial Paraganglioma Syndromes

Surgical Management of the Succinate Dehydrogenase–Associated Familial Paraganglioma Syndromes RESIDENT’S FORUM SECTION EDITORS: RICHARD D. SCHULICK, MD, MBA; PAMELA A. LIPSETT, MD, MPHE Surgical Management of the Succinate Dehydrogenase–Associated Familial Paraganglioma Syndromes Andrei Cocieru, MD; Pierre F. Saldinger, MD aragangliomas are rare neuroendocrine tumors arising from the neural crest cells in the extra-adrenal location. Paragangliomas can be sporadic or associated with a range of endocrine and genetic syndromes in 25% to 30% of all cases. Specifically, succinate de- P hydrogenase gene mutations are involved in the development of paraganglioma syn- dromes type 1 through type 4. In this article, we will describe 2 cases of succinate dehydrogenase– associated familial paraganglioma syndrome and provide a review of the existing literature on the condition’s etiologic factors, diagnosis, and management. Arch Surg. 2012;147(1):89-91 Paragangliomas (PGLs) are rare neuroen- a well-encapsulated tumor with argyro- docrine tumors arising from the neural philic tumor cells positive on immuno- crest cells in the extra-adrenal location. chemical analysis for chromogranin and Paragangliomas can be sporadic or asso- negative for S-100 protein, consistent ciated with the other hereditary tumor with the diagnosis of PGL. The patient syndromes. had complete resolution of the arterial In this article, we report on 2 cases of hypertension with normalization of uri- http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Surgery American Medical Association

Surgical Management of the Succinate Dehydrogenase–Associated Familial Paraganglioma Syndromes

JAMA Surgery , Volume 147 (1) – Jan 1, 2012

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Publisher
American Medical Association
Copyright
Copyright 2012 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
ISSN
2168-6254
eISSN
2168-6262
DOI
10.1001/archsurg.2011.552
pmid
22250122
Publisher site
See Article on Publisher Site

Abstract

RESIDENT’S FORUM SECTION EDITORS: RICHARD D. SCHULICK, MD, MBA; PAMELA A. LIPSETT, MD, MPHE Surgical Management of the Succinate Dehydrogenase–Associated Familial Paraganglioma Syndromes Andrei Cocieru, MD; Pierre F. Saldinger, MD aragangliomas are rare neuroendocrine tumors arising from the neural crest cells in the extra-adrenal location. Paragangliomas can be sporadic or associated with a range of endocrine and genetic syndromes in 25% to 30% of all cases. Specifically, succinate de- P hydrogenase gene mutations are involved in the development of paraganglioma syn- dromes type 1 through type 4. In this article, we will describe 2 cases of succinate dehydrogenase– associated familial paraganglioma syndrome and provide a review of the existing literature on the condition’s etiologic factors, diagnosis, and management. Arch Surg. 2012;147(1):89-91 Paragangliomas (PGLs) are rare neuroen- a well-encapsulated tumor with argyro- docrine tumors arising from the neural philic tumor cells positive on immuno- crest cells in the extra-adrenal location. chemical analysis for chromogranin and Paragangliomas can be sporadic or asso- negative for S-100 protein, consistent ciated with the other hereditary tumor with the diagnosis of PGL. The patient syndromes. had complete resolution of the arterial In this article, we report on 2 cases of hypertension with normalization of uri-

Journal

JAMA SurgeryAmerican Medical Association

Published: Jan 1, 2012

References

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