von Recklinghausen病に合併した膀胱癌の1例

Abstract

53歳男.幼少時より皮膚腫瘤が認められ, 5歳の時von Recklinghausen病(R病)を指摘された.肉眼的血尿, 頻尿, 排尿時痛を主訴とし入院.体表に大小様々のcafe-au-lait spotを認め, 全身に多数の神経線維腫を認めた.下腹部やや左側よりに鶏卵大の腫瘤を触知, 膀胱尿道造影で膀胱内に長径10cmの陰影欠損を認め, 膀胱鏡にて尿道内に突出する白色の非乳頭状腫瘤を認め生検で移行上皮癌と診断した.術前バルーン阻血下動注療法(シスプラチン, アドリアマイシン)を施行, 膀胱腫瘍は92.8%の縮小率を認めたが, 膀胱全摘除術とハウトマン法による回腸膀胱造設術を施行した.術後8週より化学療法を3コース行った

A 53-year-old man was admitted to our hospital with urinary frequency and miction pain. He had von Recklinghausen's disease with multiple cafe-au-lait spots and neurofibromatosis. Computed tomography scan and magnetic resonance imaging revealed an invasive bladder tumor 10 cm in diameter, and not metastasis. He was diagnosed as having a bladder tumor (T3a N0 M0 with von Recklinghausen's disease. After balloon occluded arterial infusion (BOAI) chemotherapy, total cystectomy was performed. Pathological diagnosis was transitional cell carcinoma, G3, pT3aN0M0. We reviewed and discussed 97 cases of carcinoma associated with von Recklinghausen's disease reported in the Japanese literature. Only 5 cases of bladder cancer have been reported, including the present case.