Document Detail

Long-term outcomes of surgical treatment for hereditary pheochromocytoma.
MedLine Citation:
PMID:  23317575     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: The ideal surgical management of hereditary pheochromocytomas includes planning for a potential metachronous bilateral presentation and the possibility of lifelong steroid dependence if bilateral adrenalectomy is needed. An intact and viable cortical remnant after bilateral pheochromocytoma resection can eliminate the necessity for steroid dependency, but can increase the risk of pheochromocytoma recurrence.
STUDY DESIGN: We retrospectively reviewed outcomes of all patients with a diagnosis of hereditary pheochromocytomas treated at our tertiary cancer institution from 1962-2011, with subset analysis of patients undergoing a cortical-sparing procedure in the setting of bilateral adrenalectomy.
RESULTS: Of the ninety-six patients who underwent adrenalectomy for hereditary pheochromocytomas, 47 presented with bilateral disease. In 15 of the 49 patients (30%) who originally underwent unilateral adrenalectomy, pheochromocytoma developed in the contralateral gland at a median of 8.2 years (range 1 to 20 years) after the initial diagnosis. There were 4 recurrences in 55 cortical-sparing remnants (7%) and 3 recurrences in the adrenal bed after 101 intended total adrenal resections (3%) (p = 0.24). Total bilateral adrenalectomy was performed in 25 patients and acute adrenal insufficiency developed in 5 (20%) of those patients. An intended cortical-sparing adrenalectomy was performed in 39 patients and acute adrenal insufficiency developed in 1 (3%). Of these patients with adequate follow-up, 21 of 27 (78%) were steroid independent at 3-year follow-up. Sex, median age, adrenal vein preservation, metachronous adrenal resection, and bilateral cortical-sparing procedures did not predict steroid independence at 3 years.
CONCLUSIONS: Cortical-sparing adrenalectomy avoids long-term corticosteroid dependence in the majority of patients with hereditary pheochromocytoma with minimal risk of acute adrenal insufficiency. Recurrence occurs in approximately 7% of adrenal remnants.
Elizabeth G Grubbs; Thereasa A Rich; Chaan Ng; Priya R Bhosale; Camilo Jimenez; Douglas B Evans; Jeffrey E Lee; Nancy D Perrier
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of the American College of Surgeons     Volume:  216     ISSN:  1879-1190     ISO Abbreviation:  J. Am. Coll. Surg.     Publication Date:  2013 Feb 
Date Detail:
Created Date:  2013-01-15     Completed Date:  2013-03-12     Revised Date:  2014-09-03    
Medline Journal Info:
Nlm Unique ID:  9431305     Medline TA:  J Am Coll Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  280-9     Citation Subset:  AIM; IM    
Copyright Information:
Copyright © 2013 American College of Surgeons. Published by Elsevier Inc. All rights reserved.
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MeSH Terms
Adrenal Gland Neoplasms / genetics,  surgery*
Adrenalectomy / methods*
Aged, 80 and over
Child, Preschool
Genetic Testing
Middle Aged
Neoplasm Recurrence, Local
Pheochromocytoma / genetics,  surgery*
Postoperative Complications
Retrospective Studies
Treatment Outcome
Grant Support

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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