Abstract | 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:
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Authors | Elizabeth G Grubbs, Thereasa A Rich, Chaan Ng, Priya R Bhosale, Camilo Jimenez, Douglas B Evans, Jeffrey E Lee, Nancy D Perrier |
Journal | Journal of the American College of Surgeons
(J Am Coll Surg)
Vol. 216
Issue 2
Pg. 280-9
(Feb 2013)
ISSN: 1879-1190 [Electronic] United States |
PMID | 23317575
(Publication Type: Journal Article)
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Copyright | Copyright © 2013 American College of Surgeons. Published by Elsevier Inc. All rights reserved. |
Topics |
- Adolescent
- Adrenal Gland Neoplasms
(genetics, surgery)
- Adrenalectomy
(methods)
- Adult
- Aged
- Aged, 80 and over
- Child
- Child, Preschool
- Female
- Genetic Testing
- Humans
- Infant
- Male
- Middle Aged
- Neoplasm Recurrence, Local
- Pheochromocytoma
(genetics, surgery)
- Postoperative Complications
- Retrospective Studies
- Treatment Outcome
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