腎悪性末梢神経鞘腫瘍の1例

Abstract

50歳男.主訴は左腰背部痛.CT・MRI検査で左後腹膜腔に辺縁石灰化を伴う嚢胞性の巨大腫瘤を認め, また右腎は腎盂尿管移行部狭窄による水腎症を認めた.左後腹膜腫瘍の診断で, 後腹膜腫瘍摘除術および下行結腸合併切除術を施行した.病理組織学的診断は水腎症が長期存在していた左腎実質から発生した腎悪性末梢神経鞘腫瘍(MPNST)であった.術後3ヵ月, 左下肺野多発性転移のため死亡した

We report a case of malignant peripheral nerve sheath tumor (MPNST) of the kidney. A 50-year-old man was referred to our hospital because of left back pain. Computed tomographic scan and magnetic resonance imaging revealed a huge left retroperitoneal tumor (30 x 13 x 11 cm), which consisted of a solid tumor with calcification surrounding the cystic component. He underwent resection of the retroperitoneal tumor and additional descending colectomy. Histopathological examination revealed MPNST arising from severely atrophic renal parenchyma of giant hydronephrosis. He died of tumor metastasis to the lungs 3 months after the operation. To our knowledge, this is the fourth case of MPNST of the kidney, moreover the third case arising from the renal parenchyma to be reported in Japan.