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Reza Asari, MD; Christian Scheuba, MD; Klaus Kaczirek, MD; Bruno Niederle, MD

Arch Surg. 2006;141:1199-1205.

Hypothesis  Adrenal-sparing adrenalectomy is considered the treatment of choice for hereditary bilateral pheochromocytoma in patients with multiple endocrine neoplasia type 2A (MEN 2A).

Design  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.

Setting  University hospital referral center.

Patients  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).

Main Outcome Measures  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.

Author Affiliations: Division of General Surgery, Section of Endocrine Surgery (Drs Asari, Scheuba, and Kaczirek), and Department of Surgery (Dr Niederle), Medical University of Vienna, Vienna, Austria.

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