急速に進行した嚢胞内出血とリンパ節病変を伴った後腹膜血管筋脂肪腫の1例

Abstract

34歳女.下腹部痛を主訴とした.腹部造影CTで腹部左半側に後腹膜巨大嚢胞と嚢胞内への出血を認めた.巨大嚢胞により腹腔内臓器は右半側に, 左腎は前方に圧排されていた.2度の動脈塞栓術を行ったが嚢胞内への出血が続くため, 経腹的手術を試みた.嚢胞との強固な癒着により左腎は温存不可能と考え, 左腎と嚢胞合併摘除術を施行した.術中, 出血部位と原発巣は同定できなかったが, 傍大動脈部に腫大したリンパ節を認め摘除した.病理組織診断は, リンパ節病変を伴った後腹膜血管筋脂肪腫であった

We report a very rare case of retroperitoneal angiomyolipoma with rapidly progressing intracytic hemorrhage and lymph node involvement in a 34-year-old female. She was admitted to our hospital complaining of a severe pain in the lower abdominal region, followed by oligemic shock. Abdominal enhanced computed tomography (CT) demonstrated a retroperitoneal giant cyst with intracystic hematoma, which displaced the left kidney anteriorly. Because transarterial embolization was unsuccessful transabdominal left nephrectomy combined with excision of the giant cyst was performed to keep the continuing hemorrhage under control. Simultaneously, a swollen paraaortic lymph node was also resected. The histologic findings of resected tissue and lymph node were retroperitoneal angiomyolipoma with lymph node involvement. The patient is being followed up at our hospital without recurrence.