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Cortical-sparing adrenalectomy for patients with bilateral pheochromocytoma. Surgery 1996 Dec;120(6):1064-70; discussion 1070-1

Date

12/01/1996

Pubmed ID

8957496

DOI

10.1016/s0039-6060(96)80056-0

Scopus ID

2-s2.0-0029906124 (requires institutional sign-in at Scopus site)   178 Citations

Abstract

BACKGROUND: Bilateral pheochromocytomas are common in patients with multiple endocrine neoplasia type 2 (MEN 2) and von Hippel-Lindau disease (VHL). In an effort to avoid long-term steroid dependence and Addisonian crisis, we have performed cortical-sparing adrenalectomy in this patient population.

METHODS: Retrospective chart review was completed for patients with MEN 2- or VHL-related pheochromocytomas who underwent laparotomy at our institution for intended cortical-sparing adrenalectomy between June 1965 and March 1995.

RESULTS: Fifteen patients (MEN 2A [10], MEN 2B [2], VHL [3]) underwent laparotomy for cortical-sparing adrenalectomy. None of the tumors were malignant. Cortical-sparing adrenalectomy was possible in 14 (93%). Thirteen of these 14 patients (93%) had normal postoperative plasma cortisol measurements and did not require steroid hormone supplementation. At a median follow-up of 138 months, two patients had died of metastatic medullary thyroid cancer, no patient had suffered Addisonian crisis, and three patients (21%) had recurrent pheochromocytomas (at 118, 176, and 324 months after operation). The remaining nine patients were alive without pheochromocytomas.

CONCLUSIONS: Cortical-sparing adrenalectomy can be performed successfully in MEN 2 or VHL patients with bilateral pheochromocytomas, avoiding chronic steroid hormone replacement and the risk of Addisonian crisis in most patients. Long-term follow-up is necessary because recurrence may develop many years after operation.

Author List

Lee JE, Curley SA, Gagel RF, Evans DB, Hickey RC

Author

Douglas B. Evans MD Chair, Professor in the Surgery department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Adrenal Cortex
Adrenal Gland Neoplasms
Adrenalectomy
Adult
Drosophila Proteins
Female
Humans
Male
Middle Aged
Morbidity
Mortality
Multiple Endocrine Neoplasia Type 2a
Mutation
Neoplasm Recurrence, Local
Pheochromocytoma
Postoperative Period
Proto-Oncogene Proteins
Proto-Oncogene Proteins c-ret
Receptor Protein-Tyrosine Kinases
Retrospective Studies
von Hippel-Lindau Disease