骨盤腔内に限局し腫瘤形成がみられた特発性後腹膜線維症の1例

Abstract

腹部超音波検査で右水腎症が認められた54歳女で, 点滴腎盂造影及びCTにより右水腎水尿管症及び右下部尿管周囲腫瘤が明らかにされた.腫瘤はMRIのT1及びT2強調像で低信号を示し, 線維性成分が示唆された.各種腫瘍マーカーは正常範囲内にあった.術中新鮮凍結切片により悪性がない事を確認した後, 下部尿管と共に腫瘍を摘出し, 尿管膀胱新吻合術を行った.組織学的に腫瘍は特発性後腹膜線維症と診断された

We report a case of idiopathic retroperitoneal fibrosis localized in the pelvic cavity. A 54-year-old female was found to have a right hydronephrosis by abdominal ultrasonogram and was admitted to our department. Drip infusion pyelography (DIP) and computed tomography (CT) revealed a right hydroureteronephrosis and a right lower periureteral mass. The mass was confirmed to show low intensity on both T1 and T2 weighted image in magnetic resonance imaging (MRI), which suggested fibrous component. Various tumor marker values were within normal range. Tumor extirpation including lower ureter and ureterovesiconeostomy were performed after confirming pathologically the absence of malignancy by fresh frozen section during surgery. The tumor was diagnosed histologically as idiopathic retroperitoneal fibrosis. We briefly reviewed 223 cases of idiopathic retroperitoneal fibrosis in the Japanese literature.