目的:眼窩之神經鞘細胞瘤是非常罕見之眼窩腫瘤,本文之目的是報告病例,並加以討論其臨床表現、診斷方法、組織病理、治療及預後。 方法:自1989年7月至2004年1月之回溯性病歷研究,共搜集了四位接受病理診斷之病例,並回顧了近年來發表之文章,來探討眼窩之神經鞘細胞瘤。 結果:64歲女性,主訴緩慢進行性之凸眼及眼瞼下垂達五年之久,並無疼痛或其他伴隨之症狀。電腦斷層發現在上眼窩處有兩腫塊。病人接受了經外眼窩腫瘤移除,病理報告證實為神經鞘細胞瘤,其預後良好無復發。 結論:眼窩之神經鞘細胞瘤沒有特殊的臨床表徵及影像診斷,因此在病理診斷前不易與其它軟組織眼窩腫瘤區分。但由於腫瘤型態完整,一旦腫瘤被切除之後病人預後良好,若是能早期診斷更為上策。
Purpose: Neurilemoma (Schwannoma) is a very rare orbital tumor that arises from the Schwann's cell sheath of peripheral nerves, accounting for about 1% of orbital tumors. The clinical features, diagnostic tools, pathology, treatment and prognosis of orbital neurilemoma will be discussed. Methods: A retrospective study with a chart review was performed from July 1989 to January 2004. A total of 4 patients (2 males, 2 females) with pathologic diagnosis of orbital neurilemoma at National Taiwan University hospital were collected. The clinical features including pre- and post-operative best-corrected visual acuity and Hertel exophthalmometry, imaging studies, diagnosis, treatment, and prognosis will be presented. Case presentation: A 64-year-old female developed slow progressive left eye proptosis. A palpable mass on her left upper eyelid was also noticed for 5 years without tenderness and other accompanying symptoms. Orbital CT scan demonstrated two mass lesions over the left upper orbit. The encapsulated, elongated and lobular tumor was completely removed by lateral orbitotomy. Histopathologic examination showed a neurilemoma (schwannoma) measuring 3×1.4×0.7cm^3. The patient had excellent vision and no motility disturbance both before and after surgery. Conclusions: Orbital neurilemoma has no highly distinctive features on clinical or imaging studies, so that it can be difficult to differentiate from other soft-tissue orbital tumor before the pathology proven. Since it's well encapsulated, the prognosis is good after complete tumor excision, especially in cases of early diagnosis.