포상 연부 육종(Alveolar soft part sarcoma)은 매우 희귀한 질환으로 흔히 사지의 근육에서 발생하며 매우 드물게 안와에서도 발생한다.국내에서는 성인에서 발생한 안와 포상 연부 육종은 아직 보고된 예가 없다. 저자들은 최근 좌안의 안구 돌출을 주소로 안과를 방문한 29세 남자환자에서 수술전 시행한 안초음파,자기공명영상(MRI)및 혈관종 단일광자방출전산화단층촬영(heman-giomaSPECT)결과 안와 해면양 혈관종으로 의심하여 크뢴라인안와 절개(Kronlein orbitotomy)방법으로 종양을 제거한 후 조직병리검사상 안와 포상 연부 육종으로 확진하고 혈관종과의 감별진단이 필요하다고 생각되어 문헌고찰과 함께 보고하는 바이다.(한안지 40:2299~2303,1999).

Alveolar soft part sarcoma is a very rare malignant neoplasm which originates from the soft tissue.The common site is lower extremity and it is especially rare to occur primarily in the orbit.It is characterized that tumor shows organoid arrangement of lobules separated by numerous delicate vascular septa and individual tumor cell contained PAS positive crystalline granules in the cytoplasm. Moreover, because of hypervascular tumor,it is easy to misdiagnosed as a cavernous hemangioma which commonly occurs in the orbit. We experienced a 29-year-old male who had suffered from unilateral proptosis.Before operation, ultrasonography,MRI and single photon emission computed tomography(SPECT) were performed and we suspected that the retro-orbital mass was a cavernous hemangioma. Using Kronlein method,lateral orbitotomy was performed and large well encapsulated tumor mass(2.5 ×2.5 ×2.0 ㎝)was excised.On histopathological examination the tumor was confirmed as an alveolar soft part sarcoma. Therefore,we report this interesting case with review of the literature. (J Korean Ophthalmol Soc 40:2299~2303,1999).