化学療法で部分寛解した悪性神経鞘腫

RESPONDED TO COMBINATION CHEMOTHERAPY OF ADRIAMYCIN AND CISPLATINUM

　レックリングハウゼン病の28歳の男性に，巨大な後腹膜腫瘍と骨破壊を伴う仙骨部脊髄腫瘍を認め，後腹膜腫瘍の切除術にて悪性神経鞘腫の病理診断を得た。残存した後腹膜腫瘍の急速な増大を見たが，化学療法にて部分寛解を得た。術後8ヵ月目に死亡し，剖検にて脊髄腫瘍の完全消失を認めた。レックリングハウゼン病の悪性化の報告は多いが，化学療法が有効であつた例は稀であり，今後の検討を期待して報告した。

A 28-year-old man was referred to our hospital because of a rapidly-growing abdominal mass and lumbago. He had cutaneous characteristics of von Recklinghausen's disease (VRD). A CT scan revealed an adult-head-sized retroperitoneal tumor and a spinal cord tumor in the sacral region. Because the symptoms arising from the retroperitoneal tumor were very strong, tumor resection was tried first. Most part of the tumor was resected, and pathological examination showed a malignant peripheral nerve sheath tumor. But the residual tumor grew rapidly, returning to the preoperative size within 1 month.