家族性両側性褐色細胞腫の1例

Abstract

The sixth kindred of familial pheochromocytoma in Japan, consisting of a younger brother and a elder brother, was reported. The elder brother was operated for a pheochromocytoma, arising from the left adrenal gland, at his age 39 at another hospital. Bilateral pheochromocytoma, arising from the bilateral adrenal glands, were found in the younger brother at the age of 34 in our clinic, and bilateral adrenalectomy was performed. Analysis of the 44 reported kindred (138 cases) in the world literature revealed that the mean age of male cases was 31 years and that of female was 29 years, that familial pheochromocytoma occured especially in the bilateral adrenal glands with high incidence (56.7%), and that associated diseases of familial pheochromocytoma, such as medullary thyroid carcinoma (27.5 %) or neurocutaneous syndrome (5.8%), were found relatively frequent.