Familial extra-adrenal pheochromocytoma. A new syndrome

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Abstract
Pheochromocytomas in the same anatomic site, the right renal hilum, occurred in a family over 3 successive generations. For 2 patients in the latter 2 generations, scintigraphy with 131I-tagged metalodobenzylguanidine (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 1 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. Primary extra-adrenal pheochromocytoma is a syndrome in which specific genetic abnormalities determine sites of tumor development.