家族性大腸腺腫症の結腸全摘1年3ヵ月後に巨大な後腹膜線維肉腫の認められた1例

Giant Retroperitoneal Fibrosarcoma Associated with Familial Polyposis Coli, Report of a Case

要旨　家族性大腸腺腫症の結腸全摘後約1年に左下腹部腫瘤がみられ来院．その腫瘤が急速に増大し，呼吸困難が出現したため入院．腹部超音波検査にて腫瘤は左腹部全体を占居していた．腹部血管造影検査にてその腫瘤は腰動脈と上腸間膜動脈によって栄養されていた．更に左腎は排出機能の低下を示していた．手術は回腸部分切除，左腎摘出術，左総腸骨動脈再建，姑息的腫瘤切除術を施行した．切除標本にて，被膜に覆われた腫瘤の大きさは35.5×27.0×14.Ocmで，その重量は7.8kgであった．組織学的には線維肉腫であった．家族性大腸腺腫症の結腸全摘1年3ヵ月後に巨大な後腹膜線維肉腫の認められた1例を経験したので報告した．

　Familial polyposis coli with giant retroperitoneal fibrosarcoma is rare. A 31 year-old woman had gone through total colectomy with ileostomy for familial polyposis coli with an advanced cancer. Surgical specimen of the colon had 2,969 polyps and an advanced cancer of the rectum (Fig. 2). Nine polyps out of them showed, histologically, cancer in adenoma. A large tumor localized in the left lower quadrant of the abdomen was noticed about eleven months after the operation, and she was readmitted to the hospital.

　Ultrasonography showed a giant abdominal tumor which occupied the entire left abdominal cavity (Fig. 4). Angiography also showed a giant retroperitoneal tumor fed by the lumbar and superior mesenteric arteries (Fig. 5). Tumor resection with left nephrectomy and partial ileoectomy and common iliac artery reconstruction were conservatively performed on October, 1982. Macroscopic findings of the resected specimen showed a smooth-surfaced tumor measuring about 35.5×27.0×14.0 cm in dimensions and weighing 7.8 kg (Fig. 6). The tumor was fibrosarcoma histologically (Fig. 7).