안와주위에 발생한 결절근막염 3예
Three Cases of Periorbital Nodular Fasciitis
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목적: 안와주위에 발생한 종괴를 주소로 내원하여 결절근막염을 진단 받은 3예를 보고하고자 한다. 증례요약: 18세 남자 환자가 3개월 전에 발견한 왼쪽 눈썹 옆 관자놀이 부위의 종괴를 주소로 내원하였다. 이학적 검사상 20 mm 크기의 피부밑 종괴가 촉지되었다. 자기공명영상에서 T1에서 isointensity, T2에서 hyperintensity를 보이며 조영증강이 잘 되는 고형종괴로 나타났다. 36세 남자가 6개월 전부터 시작된 우측 위눈꺼풀 종괴를 주소로 내원하였다. 이학적 검사상 우측 눈썹 중앙부 아래로 15 mm 크기의 피부밑 종괴가 촉지되었다. 안와 전산화 단층촬영에서 균일하고 주변조직과 구별이 잘 되는 종괴로 나타났으며, 중등도의 조영증강을 보였다. 3세 남자 환자가 4개월 전 시작된 우측 위눈꺼풀의 종괴로 내원하였다. 안과 검사상 위안와연 내측에 10 mm 크기의 종괴가 촉지되었다. 자기공명영상에서 조영증강이 잘 되는 부정형의 종괴를 나타내었다. 세 증례 모두 절제 생검을 시행하였고 병리조직검사 상에서 특징적인 방추형 세포의 증식과 함께, 면역조직화학검사에서 smooth muscle actin 양성, S-100 음성, CD34 음성 등의 특징을 보여 결절근막염으로 확진하였다. 각각 수술 후 4개월, 3개월, 48개월까지 경과관찰하였을 때 재발은 관찰되지 않았다. 결론: 결절근막염은 드물지만 안와 주위에 발생할 수 있으며, 특히 소아 및 청장년층에서 비교적 이환 기간이 짧은 피부밑 종괴의 감별진단으로 염두에 두어야 한다.
Abstract
Purpose: Herein, we report 3 cases of nodular fasciitis presenting with periorbital mass. Case summary: An 18-year-old male presented with a mass in his left lateral temple area 3 months in duration. On physical examination, a subcutaneous mass approximately 20 mm in size was palpable with upper eyelid swelling. Orbital magnetic resonance imaging (MRI) showed T1 isointensity and T2 hyper intensity and a well-circumscribed enhancing lesion. A 36-year-old male presented with a 6-month history of a bump under his right upper eyelid. External examination revealed a 15 mm-sized subcutaneous mass in the right central sub-brow area. Orbital computed tomography showed a homogenous, well-circumscribed mass with moderate enhancement. A 3-year-old boy presented with a mass in his right upper eyelid 4 months in duration. A subcutaneous mass approximately 10 mm in size was palpated at the medial superior orbital rim. Orbital MRI revealed an enhanced mass of irregular shape. Surgical excision was performed for all cases. Histopathological examination showed pathognomonic proliferation of spindle cells and immunohistochemical stains showed the spindle cells were positive for smooth muscle actin, negative for S-100 and negative for CD34, consistent with nodular fasciitis. Recurrence of the tumors after excision in the 3 cases was not observed after 4 months, 3 months and 48 months, respectively. Conclusions: Nodular fasciitis is rare but can occur at the periorbital region. In particular, nodular fasciitis should be considered as a differential diagnosis of a subcutaneous mass short in duration in children or young adults.
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