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结膜黏液瘤:一种罕见眼部肿瘤的概述

Conjunctival myxoma: a synopsis of a rare ocular tumor.

作者信息

Xiong Meng-Jun, Dim Daniel C

机构信息

Department of Pathology, University of Missouri, Kansas City, School of Medicine, Kansas City.

出版信息

Arch Pathol Lab Med. 2015 May;139(5):693-7. doi: 10.5858/arpa.2013-0532-RS.

DOI:10.5858/arpa.2013-0532-RS
PMID:25927154
Abstract

Conjunctival myxoma is an exceptionally rare, slow-growing, benign neoplasm of primitive mesenchyme origin. Forty-one cases of conjunctival myxoma from a literature review, including the authors' case, are listed. The usual clinical history is a painless mass appearing during months to years. Grossly, the tumor is a well-circumscribed, cystlike, gelatinous, yellow-to-pink, translucent-to-solid mass. Microscopically, the hypocellular tumor contains stellate- and spindle-shaped cells in a mucoid stroma with abundant hyaluronic acid mucopolysaccharides. Vimentin and α-smooth muscle actin highlight the spindle and stellate cells. S100 protein and desmin are negative for the tumor cells. Treatment is complete surgical excision, with no recurrence reported in the follow-up period. Notably, conjunctival myxoma may be associated with Carney complex, an autosomal-dominant disorder associated with skin pigmentation, endocrine abnormalities, and myxoma of the heart and eye. Physicians should appreciate this unique ocular tumor because of its potential association with Carney complex.

摘要

结膜黏液瘤是一种极为罕见的、生长缓慢的、起源于原始间充质的良性肿瘤。本文通过文献回顾,列出了包括作者病例在内的41例结膜黏液瘤病例。其常见临床病史为在数月至数年期间出现无痛性肿块。大体上,肿瘤是边界清楚、囊肿样、胶冻状、黄至粉色、半透明至实性的肿块。显微镜下,细胞稀少的肿瘤在富含透明质酸黏多糖的黏液样基质中含有星状和梭形细胞。波形蛋白和α-平滑肌肌动蛋白可突出显示梭形和星状细胞。肿瘤细胞的S100蛋白和结蛋白呈阴性。治疗方法为完整手术切除,随访期间未见复发报告。值得注意的是,结膜黏液瘤可能与卡尼综合征有关,卡尼综合征是一种常染色体显性疾病,与皮肤色素沉着、内分泌异常以及心脏和眼部黏液瘤有关。由于结膜黏液瘤可能与卡尼综合征相关,医生应了解这种独特的眼部肿瘤。

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