A rare case of vulvar extraskeletal myxoid chondrosarcoma: mimics and diagnostic clues.
A rare case of vulvar extraskeletal myxoid chondrosarcoma: mimics and diagnostic clues.
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DOI：
10.4322/acr.2021.322
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发表时间：
2021
影响因子：
--
通讯作者：
Moatamed NA
中科院分区：
文献类型：
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作者：
Liou SS;Memarzadeh S;Dry SM;Graham RP;Moatamed NA
文献报道了外阴骨外黏液样软骨肉瘤（EMC） 14例。我们报告一例63岁女性外阴EMC，经EWSR1和NR4A3荧光原位杂交证实，后者是对该实体更特异性的探针。该肿瘤位置异常，粘液样形态突出，因此需要广泛的鉴别诊断，这需要彻底的组织学评估和免疫组织化学和细胞遗传学研究形式的确证性辅助检测。本文旨在回顾外阴骨外粘液样软骨肉瘤和各种诊断线索，以帮助区分其与组织学模拟。这是文献中确认NR4A3基因重排的第五例外阴EMC。
Only 14 cases of extraskeletal myxoid chondrosarcoma (EMC) of the vulva have been documented in the literature. We report a case of a 63-year-old woman with EMC of the vulva confirmed by both EWSR1 and NR4A3 fluorescence in situ hybridization, the latter of which is a more specific probe for this entity. The unusual location of this tumor of prominent myxoid morphology gave rise to a wide differential diagnosis, which necessitated thorough histologic evaluation and confirmatory ancillary testing in the form of immunohistochemistry and cytogenetic studies. This article aims to review extraskeletal myxoid chondrosarcoma of the vulva and various diagnostic clues to help differentiate it from its histologic mimics. This is the fifth case of vulvar EMC in the literature with confirmation of a NR4A3 gene rearrangement.