眼窩および頭蓋内に進展した後部副鼻腔原発の胎児型横紋筋肉腫の1症例

Intracranial and Intraorbital Extension of Embryonal Rhabdomyosarcoma from Ethmo-sphenoidal Sinuses:Report of a Case

　緒言

　横紋筋肉腫は中胚葉系腫瘍の中でも極めて悪性度が高く，予後不良の疾患である。頭頸部はその好発部位であるが，特に鼻副鼻腔領域は比較的頻度が高く，頭蓋内に近接する解剖学的な理由により，その診断と治療に困難を呈する場合が多い。最近われわれは，副鼻腔より中頭蓋内に進展し，化学療法のみで寛解に至った若年者の横紋筋肉腫症例を経験したので，若干の文献的考察を加えて報告する。

A 16-year-old male presented with right nasal obstruction and recurrent nasal bleeding. Reduction of right visual acuity and exophthalmos gradually developed. Enhanced CT revealed in-traorbital and intracranial extension of soft tissue density from right ethmosphenoidal lesion. Combi-nation chemotherapy with vincristine, actinomycin D, cyclophsphamide, and epirubicin hydrochloride was performed and complete remission was obtai-ned. Side effect was minimal except for bone marrow suppression. Six months later he is still well with no sign of recurrence. For the treatment of rahbdomyosrcoma, multidisciplinary method with surgery, irradiation, and combination chemo-therapy is recommended.