Abstract

The surgical management of familial childhood pheochromocytoma is based on data derived from clinical series that have focused primarily on the multiple endocrine neoplasia (MEN)-associated cases. Prophylactic bilateral total adrenalectomy has been advocated for this group due to a high incidence of bilateral adrenal involvement and the propensity for tumor recurrence after partial adrenal resections. We retrospectively reviewed our experience with a cohort of children with nonsyndromatic familial pheochromocytoma to see if their surgical management should parallel that advocated for patients with the MEN syndrome. From 1977 through 1991, 17 children from 9 families with a strong family history of pheochromocytoma were treated at the Children's Hospital of Pittsburgh. None of the patients or their families displayed an association with the MEN syndrome. There were 13 males and 4 females with a mean age of 10.1 years. Follow-up was obtained on all patients and averaged 6 years. All tumors were located within the abdominal cavity. Seven patients presented with unilateral adrenal tumors while four patients had synchronous bilateral tumors. Eight patients had tumors in extra-adrenal locations; 2 with concomitant adrenal tumors. Ten patients underwent total unilateral adrenalectomy with or without resection of extra-adrenal tumor(s). All patients with synchronous bilateral adrenal tumors underwent unilateral adrenalectomy in combination with a partial contralateral adrenal resection without a recurrence after an average of 7.3 years. Three patients developed metachronous adrenal tumors an average of 4.2 years after their initial surgery. All required complete removal of the affected adrenal, resulting in two patients with bilateral adrenalectomies.(ABSTRACT TRUNCATED AT 250 WORDS)

View details for Web of Science ID A1993MD71600006

View details for PubMedID 8263682