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February 1985

Familial Extra-adrenal Pheochromocytoma: A New Syndrome

Author Affiliations

From the Division of Nuclear Medicine, Department of Internal Medicine (Drs Glowniak, Shapiro, Sisson, and Beierwaltes), and the Departments of Surgery (Dr Thompson), Pediatric Surgery (Dr Coran), Pathology (Dr Lloyd), and Pediatrics (Dr Kelsch), University of Michigan, Ann Arbor.

Arch Intern Med. 1985;145(2):257-261. doi:10.1001/archinte.1985.00360020081015

• Pheochromocytomas in the same anatomic site, the right renal hilum, occurred in a family over three successive generations. For two patients in the latter two generations, scintigraphy with iodine 131—tagged metaiodobenzylguanidine (MIBG) showed tumors only in the region of the right renal hilum, thus indicating that they were primary lesions. At surgery, except for lymph node metastases noted microscopically in one patient, tumors were found only near the right renal hilum. The adrenal glands seemed normal on inspection, palpation, and computed tomography. In another family, a mother and son had primary pheochromocytomas arising from the urinary bladder. We suggest that primary extra-adrenal pheochromocytoma is a syndrome in which specific genetic abnormalities determine sites of tumor development.

(Arch Intern Med 1985;145:257-261)