多発性神経鞘腫の1例

A Case of Multiple Schwannomas

　はじめに

　多発性神経鞘腫は末梢および中枢神経系に神経鞘腫が多発する疾患で，家族性を示す例は少ない^1）。現在，常染色体優性遺伝を示す神経線維腫症（neurofibromatosis：以下，NFと略）とは別の疾患群であると考えられている^2）。

　今回われわれは，家族歴を認めない右顎下部，右肩，右足背部に神経鞘腫を生じた多発性神経鞘腫の1例を経験したので，若干の文献的考察を加えて報告する。

A 15-year-old woman presented with multiple schwannomas, which arised from the right subman-dibular region, right shoulder and right foot. Cafe-au-lait spots and family history were not observed. Preoperative CT examination of the submandibular region revealed a 30 × 20 mm round tumor in thelower portion of submandibular gland. Surgical extirpations of the mass in the submandibular gland shoulder and foot were done. The pathological diagnosis was schwannoma.

Schwannomas are benign neurogenic tuomrs and usually occur in the head and neck region. Usually schwannoma is a solitary. slow growing tumor and involves peripheral nerve. In 1984, Shishiba et al described 4 cases with multiple cutaneous neurilem-monas as “neurilemmomatosis” which is a systemic disease distinct from neurofibromatosis. The pres-ent case was considered to be “neurilemmomatosis”.