안와에 생긴 악성 고립성 섬유종
Malignant Solitary Fibrous Tumor of the Orbit
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목적: 고립성 섬유종은 주로 중장년층 이상에서 흉막, 가슴세로칸(mediastinum), 심장막 등에 발생하는 종양이며, 안와에 발생하는 경우는 드문 것으로 알려졌다. 특히 안와에 악성 고립성 섬유종이 발생하는 경우는 매우 드물다. 저자들은 아직 국내에서는 보고된 바없는 안와 내에 발생한 악성 고립성 섬유종 1예를 경험하였기에 이를 보고하는 바이다. 증례요약: 67세 남자가 4개월 전부터 발생한 우안 안구돌출을 주소로 내원하였다. 안과 검사에서 우안에 9 mm의 안구돌출이 관찰되었고, 우안의 전방향 운동장애가 있었다. 전산화단층촬영 및 자기공명영상에서 안와 외상방에 조영 증강이 잘되면서 외직근에 연해 있는분엽성 종괴가 관찰되어 절제 생검을 시행하였다. 조직검사에서 방추세포와 교원질이 불규칙하게 배열되어 있었고, 높은 세포밀도, 세포의 다형태성, 출혈, 괴사 소견과 더불어 유사분열이 활발하였으며, 면역조직화학검사상 CD34, CD99, Ki-67, p53에 양성을 보여악성 고립성 섬유종으로 진단하였다. 결론: 안와에 악성 고립성 섬유종이 발생하는 경우는 매우 드물지만, 빠르게 진행하는 편측성 안구 돌출의 감별진단에 포함되어야 한다.
Abstract
Purpose: Solitary fibrous tumor (SFT) is a rare tumor of the pleura, mediastinum, pericardium and other organs affecting predominantly middle-aged patients. SFT arising in the orbit is extremely rare, and its malignant form is even rarer. The authors herein describe a case of malignant solitary fibrous tumor of the orbit. Case summary: A 67-year-old male presented with a 4-month history of right proptosis. On ophthalmologic examination, 9-mm proptosis was observed in the right eye, and extraocular movements were limited in all directions of gaze. CT scan and MR imaging showed a lobulated, well-enhancing mass adjacent to the lateral rectus muscle in the superolateral retrobulbar space. Excisional biopsy through a lateral orbitotomy was performed. Histopathological examination showed proliferation of spindle cells with a fascicular pattern interspersed with bands of collagen, increased cellularity, cellular pleomorphism, hemorrhage, necrosis and high mitotic activity. Immunohistochemical staining revealed diffuse positivity for CD34, CD99, Ki67 and p53, and malignant SFT was diagnosed. Conclusions: Malignant SFT should be considered in the differential diagnosis of acutely progressing unilateral proptosis.
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