眼瞼腫脹で発見された眼窩・副鼻腔の横紋筋肉腫の1症例

Lid swelling as the initial manifestation in a case of rhabdomyosarcoma in the orbit and paranasal sinus

　左下眼瞼腫脹が4週間前から生じている18歳男性が紹介受診した。前医では麦粒腫と診断されていた。左眼の下眼瞼に弾性硬の腫瘤が触知され，MRIで眼窩と上顎洞に腫瘍陰影が発見された。可能な範囲で腫瘍を摘出し，眼球は保存した。腫瘍の病理診断は，胎児型横紋筋肉腫であった。4剤併用による化学療法で残存腫瘍は縮小したが，2か月後に局所に再発した。このとき，化学療法による骨髄抑制が強く，以後播種型血管内凝固症候群が併発し，初診から9か月後に死の転帰をとった。胎児型横紋筋肉腫には全身的な化学療法が有効であるが，副作用が問題となった症例である。

An 18-year-old male presented with swelling of the left lower eyelid of four weeks' duration. He had been treated in vain for suspected hordeolum elsewhere. Magnetic resonance imaging, MRI, showed a low-density mass in the left orbit and maxillary sinus suggesting malignant tumor. The tumor was surgically removed as much as possible preserving the eyeball. It was histologically diagnosed as embryonal rhab-domyosarcoma. Postoperative chemotherapy with four cytostatic agents induced regression of remnant tumor mass but had to be discontinued for bone marrow suppression. Focal recurrence occurred two months after surgery. The patient died 9 months after the initial clinical manifestation due to bone marrow suppres-sion and disseminated intravascular coagulation, DIC. This case illustrates the efficacy of chemotherapy for embryonal rhabdomyosarcoma and its limitation because of systemic side effects.