안와에서 발생한 원발성 활액막 육종 1예
A Case of Primary Orbital Synovial Sarcoma
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목적: 안와에서 드물게 발생하는 원발성 활액막 육종 1예를 보고하고자 한다. 증례요약: 55세 남자 환자가 2달 전부터 시작된 좌안 시야감소를 주소로 내원하였다. 좌안 최대 교정시력은 1.0이었으며 2.5 mm의안구돌출이 관찰되었다. 좌안에서 상방 및 우측 주시 시 경미한 안구 운동장애가 있었고 시야 검사상 좌안 하이측 부위에 시야 결손이관찰되었다. 안저검사상 좌안의 시신경 유두부종 소견이 보였고 안와 자기공명영상의 T1, T2 강조영상에서 중간 정도의 신호강도를보이는 약 2.7×2.0×2.2 cm 크기의 경계가 명확한 종괴가 좌안 안구 뒤 하이측에서 관찰되었다. 좌안 가쪽눈구석절개 및 가쪽눈구석인대 절개를 한 뒤 결막을 통해 종괴에 접근하였고 종괴가 시신경 주위를 둘러싸고 있어 종괴 감량술을 하여 종괴 일부만 제거하였다. 조직병리학적 검사와 면역조직화학적 염색 검사를 통해 활액막 육종으로 진단되었으며 추가적으로 방사선 치료를 시행하였다. 결론: 시신경을 둘러싸고 있는 안와 종괴에서 흔하지 않은 활액막 육종도 감별해야 하며 방사선치료는 완전한 수술적 제거가 어려운원발성 활액막 육종 치료에 유용하다.
Abstract
Purpose: To report a rare case of primary orbital synovial sarcoma. Case summary: A 55-year-old male visited our clinic with a 2-month history of decreased visual field in the left eye. The best corrected visual acuity of the left eye was 1.0, and 2.5 mm of proptosis was observed. There was slight limitations in motility and a mild visual field defect in the inferotemporal quadrant of the left eye. Funduscopic examination of the left eye revealed papilledema. Orbital magnetic resonance imaging (MRI) demonstrated the presence of an orbital mass that extended inferotemporally into the left orbit. On magnetic resonance imaging, a well-demarcated and 2.7 × 2.0 × 2.2 cm sized tumor was identified with intermediate signal intensities on T1/T2-weighted scans with slight homogenous enhancement. We approached the orbital mass using a lateral conjunctival incision with left canthotomy and cantholysis and performed partial surgical resection of the mass because the mass encircled the optic nerve. The mass was diagnosed as synovial sarcoma based on the findings of both histopathology and immunohistochemical staining. Postoperatively, the patient underwent radiation therapy to the rest of the tumor site. Conclusions: Synovial sarcoma should be considered in the differential diagnosis of an orbital mass lesion encircling the optic nerve. Radiation therapy is useful to treat completely unresectable primary synovial sarcoma.
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