하사근을 침범한 안와의 Phakomatous Choristoma 1예
Phakomatous Choristoma of the Orbit with Inferior Oblique Muscle Involvement
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목적: 하사근을 침범한 안와의 phakomatous choristoma 1예를 보고하고자 한다. 증례요약: 2개월 남아가 출생 시부터 있었던 우측 안와 종괴를 주소로 내원하였다. 안와 자기공명영상에서 우측 안와의 하내측에 균일하게 조영증강되는 경계가 명확한 종양이 관찰되었고, 골미란 소견은 없었다. 이에 결막 경유 안와절개술을 시행하였고, 하사근을 침범한 안와종양을 확인한 후 절제하였다. 조직병리검사 결과 콜라겐 기질 내에 상피세포로 구성된 거짓샘 구조(pseudo-glandular structure)와 이를 둘러싸는 두꺼운 기저막, 기질 내의 일부 사종형 석회화(psammo-matous calcific foci)와 관내강의 호산성 물질이 관찰되었다. 면역조직화학검사상 S-100, 사이토 케라틴(cytokeratin) 양성 소견을 보여 phakomatous choristoma로 진단하였다. 결론: 본 증례는 하사근을 침범한 안와의 phakomatous choristoma를 확인한 국내 첫 보고이다. Phakomatous choristoma의 발생 빈도는 드물지만, 안와의 하내측에 발생한 종괴를 감별진단할 때 고려하여야 한다.
Abstract
Purpose: We report a case of phakomatous choristoma presenting as an orbital tumor with involvement of the inferior oblique muscle. Case summary: A 2-month-old male infant presented to our clinic with a right orbital mass that had been present since birth. Magnetic resonance imaging demonstrated a homogenous enhanced well-defined mass located in the inferomedial portion of the right orbit without bone erosion. By transconjunctival orbitotomy, the orbital tumor invading the inferior oblique muscle was identified and resected. Histopathology showed a thick basement membrane surrounding pseudoglandular structures embedded in a collagenous stroma, psammomatous calcific foci in the stroma, and eosinophilic material in the lumen. Immunohistochemical analysis showed positive staining for S-100 and cytokeratin. On histopathological evaluation, the tumor was diagnosed as phakomatous choristoma. Conclusions: To our knowledge, this is the first report in South Korea of phakomatous choristoma of the orbit with involvement of the inferior oblique muscle. Although rare, phakomatous choristoma should be included in the differential diagnosis of tumors occurring on the inferomedial side of the orbit.
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