両側副腎褐色細胞腫摘除術26年後に局所再発したvon Hippel-Lindau病随伴褐色細胞腫の1例

Abstract

A 60-year-old man who had undergone left adrenalectomy and right partial adrenalectomy for bilateral pheochromocytoma 26 years ago was found to have an elevated serum noradrenaline (NA) and dopamine (DA) during a long-term follow-up. At the time of right partial adrenalectomy, the normal part of the right adrenal gland was preserved. His cousin and second cousin had pheochromocytoma associated with von Hippel-Lindau (VHL) disease. His eldest daughter had cerebellar hemangioblastoma. Computed tomography and magnetic resonance imaging revealed a tumor which was 17 mm in diameter with contrast enhancement in the vicinity of the S6 region in the liver. 123 I-metaiodobenzylguanidine (MIBG) scintigraphy showed an abnormal accumulation in the same area. The tumor was surgically removed under the diagnosis of recurrence of pheochromocytoma. Histopathological examination revealed findings consistent with recurrent pheochromocytoma. After operation, serum NA and DA returned to normal range and the abnormal up-take on 123I-MIBG scintigraphy disappeared. Genetic testing revealed that the patient and his daughter had VHL disease. An extensive genetic examination and long-term follow-up should be considered for the present family.