von Hippel-Lindau病に合併した両側副腎褐色細胞腫の1例  [in Japanese] BILATERAL PHEOCHROMOCYTOMAS WITH VON HIPPEL-LINDAU DISEASE : A CASE REPORT  [in Japanese]

患者は32歳男性.家族歴は妹がvon Hippel-Lindau(VHL)病.高血圧にて近医を受診していたが,精査目的で撮影したCTにて両側副腎腫瘍を指摘され,平成18年1月に当科入院.血中及び尿中のカテコラミンの上昇,^<131>I-MIBGシンチで両側副腎に著明な集積,MRIにて小脳血管芽腫・脊髄血管芽腫が認められた.VHL病に合併した両側褐色細胞腫と診断し,2月22日に経腹的に両側副腎摘出術を施行した.また,左腎静脈内に達する腫瘍血栓が術中に認められた為,左腎摘出術も同時に施行した.病理診断も褐色細胞腫であり,術後に血圧や炎症所見などが正常化し,術後8カ月の現在経過良好である.VHL病に合併した両側褐色細胞腫は自験例が本邦報告11例目である.

A case of bilateral pheochromocytomas with von Hippel Lindau disease (VHL) is reported. A 32-year-old man visited Kumamoto Red Cross Hospital for further examination of hypertension. Computed tomography revealed bilateral adrenal tumors and noradrenalin levels in serum and urine were elevated. Suspecting bilateral pheochromocytoma, he was reffered to our hospital for further examination and treatment. ^<131>I-MIBG scintigraphy showed accumulation in bilateral adrenal glands. Moreover, he had cerebellar and spinal hemangioblastomas. Bilateral adrenalectomies and left nephrectomy were performed because tumor thrombus extended into the left renal vein, and pathological diagnosis was pheochromocytoma. His sister had been diagnosed as VHL disease. We diagnosed the patient as VHL disease because of the existence of cerebellar and spinal hemangioblastomas, bilateral pheochromocytomas, missense mutation and his family history. This is the eleventh case of bilateral pheochromocytomas with VHL disease reported in Japanese literatures.