성인의 눈꺼풀에 발생한 Muir-Torre 증후군 1예
A Case of Muir-Torre Syndrome
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목적: Muir-Torre 증후군은 피지선 종양과 내장의 악성종양이 동반되는 드문 증후군으로 저자들은 국내에서 최초로 Muir-Torre 증후 군 1례를 경험하였기에 이를 보고하고자 한다. 증례요약: 65세 여자 환자가 1년 전 발생한 우안 아래눈꺼풀 종괴를 주소로 내원하였다. 경계가 불규칙한 결절성의 종괴가 관찰되어 절제 생검 및 Tenzel 피판을 이용한 아래눈꺼풀 재건술을 시행하였다. 병리조직학 검사에서 아래눈꺼풀 종괴는 분화도가 좋은 피지샘 세포로 이루어져 있었으며, 피지샘종에 특징적인 소견을 보였다. 환자는 5년 전 자궁 내막암 및 결장암으로 복식전자궁적출술 및 저위 전방절제술을 시행하였고 3년 전 유두성 요로상피암종으로 신요관적출술을 시행하였다. 악성 종양 병력이 있는 환자에서 피지샘종을 확인하여 Muir-Torre 증후군으로 진단하였다. 결론: 성인에서 발생한 눈꺼풀 종괴가 피지샘종으로 진단된 경우 Muir-Torre 증후군을 감별진단에 포함시켜야 하며, 전신적인 검사를 통해 내장의 악성병소 종양 동반 여부를 확인하는 것이 중요하다.
Abstract
Purpose: To present a rare case of Muir-Torre syndrome characterized by the association of sebaceous skin tumors and systemic malignancies. Case summary: A 65-year-old female visited our clinic with an irregular nodular mass of the right lower eyelid, which developed 1 year earlier. An excisional biopsy and lower lid reconstruction with Tenzel’s semicircular rotational flap was performed under local anesthesia. Histopathologic examination showed well-differentiated sebaceous cells, consistent with sebaceous adenoma. The patient had undergone total abdominal hysterectomy and lower anterior resection due to endometrial cancer and sigmoid colon cancer 5 years before, and nephroureterectomy due to papillary urothelial carcinoma 3 years before. Based on the history of systemic malignancy and sebaceous skin cancer, a diagnosis of Muir-Torre syndrome was made. Conclusions: When a sebaceous gland tumor of the eyelids is detected, Muir-Torre syndrome should be included in the differential diagnosis, and systemic work-up for the internal malignancy must be performed.
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