Estimated risk of pheochromocytoma recurrence after adrenal-sparing surgery in patients with multiple endocrine neoplasia type 2A.
OBJECTIVE
Adrenal-sparing adrenalectomy is considered the treatment of choice for hereditary bilateral pheochromocytoma in patients with multiple endocrine neoplasia type 2A (MEN 2A).


METHODS
Retrospective analysis of prospectively documented data with a mean +/- SD follow-up of 81.5 +/- 85.3 months. The PubMed database was searched for articles published between 1975 and 2004 to identify published series and/or case reports.


METHODS
University hospital referral center.


METHODS
In 17 (22%) of 77 patients with various mutations of the RET proto-oncogene, unilateral (n = 12) or bilateral (n = 5) pheochromocytomas were documented at the time of diagnosis or during the course of MEN 2A. Adrenal-sparing surgery was performed in 13 patients (group 1), synchronous bilateral total adrenalectomy in 4 patients (group 2A), and metachronous bilateral total adrenalectomy in 5 patients after adrenal-sparing adrenalectomy (group 2B).


METHODS
Measurement of 24-hour urinary catecholamine levels (noradrenaline, adrenaline, and dopamine) and, in case of high catecholamine levels, imaging studies to localize the tumors in 1 or both adrenal glands to determine the size and exclude extra-adrenal tumors and distant metastasis.


RESULTS
The mean+/-SD estimated 5- and 10-year cumulative risk of developing recurrence in both groups was 38.5% +/- 15.7%. Five (38%) of 13 patients in group 1 developed recurrence in the contralateral gland. Two (22%) of 9 patients in groups 2A and 2B developed several episodes of an addisonian crisis, 1 of whom died.


CONCLUSIONS
Substantial morbidity and mortality are associated with addisonian crisis after bilateral adrenalectomy. Adrenal-sparing adrenalectomy and close monitoring of the remnant may be the treatment of choice for hereditary bilateral pheochromocytoma in MEN 2A, since overall recurrence is low.
Asari
Reza
R
Division of General Surgery, Section of Endocrine Surgery, and Department of Surgery, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria. reza.asari@meduniwien.ac.at
Scheuba
Christian
C
Kaczirek
Klaus
K
Niederle
Bruno
B
D000310:Adrenal Gland Neoplasms / Q000453:epidemiology* / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018813:Multiple Endocrine Neoplasia Type 2a; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology*; D009378:Neoplasms, Multiple Primary; D010673:Pheochromocytoma / Q000453:epidemiology* / Q000601:surgery*; D000090063:Proto-Oncogene Mas; D012189:Retrospective Studies; D018570:Risk Assessment