神経線維腫症I型(von Recklinghausen病)に合併した後腹膜悪性末梢神経鞘腫瘍(MPNST)の1例

Abstract

55歳女.神経線維腫症l型(von Recklinghausen病)と診断されている.腹部膨隆を主訴とした.左腹部に児頭大の膨隆と全身に多発する皮下腫瘤, カフェオレ斑, 小紫斑および側彎を認めた.CT・MRIで左後腹膜腔内に9×9cmの腫瘤が認められ, 第2～3胸椎左側と右臀部股関節背側にそれぞれ2.5×3.5cmと6×3cmの腫瘤を認めた.皮下腫瘤生検術および経腹膜的後腹膜腫瘍切除術を施行した.切除標本は線維性被膜に覆われ, 割面は黄色充実性, 重量1120gであった.組織学的検査で後腹膜悪性末梢神経鞘腫瘍(MPNST)と診断された.皮下腫瘤の生検はすべて神経線維腫であった.以上より, 神経線維腫症1型に合併したMPNSTと診断した

A retroperitoneal malignant peripheral nerve sheath tumor (MPNST) in a patient with von Recklinghausen's disease is reported. A 55-year-old woman was admitted with a left side abdominal mass. Physical examination showed numerous cafe-au-lait-spots, subcutaneous masses, scoliosis, and a baby's head-sized fixed mass in the left abdomen. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 9 x 9 cm retroperitoneal mass. Two other tumors were also found. One on the left side of the T2-T3 thoracic spine, and the other posterior to the right hip joint. The retroperitoneal tumor was resected en bloc. The tumor was a solid yellow mass. Macroscopically it has a pseudocapsule of fibrous tissue, weighed 1, 120 g and measured 9 x 9 x 15 cm. The histopathological diagnosis was malignant peripheral nerve sheath tumor (MPNST). Since the responsiveness of these tumors to chemotherapy and radiation therapy is poor, we did not administer adjuvant therapy. The patient is alive with no evidence of recurrence more than 6 months after surgey.