尤因肉瘤/外周原始神经外胚层肿瘤伴骨外黏液样软骨肉瘤样区域：一例报告
Ewing's sarcoma/peripheral primitive neuroectodermal tumor with extraskeletal myxoid chondrosarcoma-like areas: a case report.
作者信息
Wang Zhe, Dang Hongwei, Jiang Jinfang, Liu Xiuxia, Liang Weihua, Ren Yan
机构信息
Department of Pathology and Key Laboratory of Xinjiang Endemic and Ethnic Diseases (Ministry of Education), Shihezi University School of Medicine Shihezi 832002, Xinjiang, China.
Department of Obstetrics and Gynecology, The First Affiliated Hospital of Shihezi University Shihezi 832002, Xinjiang, China.
出版信息
Int J Clin Exp Pathol. 2019 Oct 1;12(10):3940-3943. eCollection 2019.
INTRODUCTION
Ewing's sarcoma (EWS)/peripheral primitive neuroectodermal tumor (pPNET) (EWS/pPNET) is a group of highly aggressive small round cell tumors of the bone or soft tissue with high metastatic potential and an aggressive course in children and young adults. EWS/pPNET microscopically does not often have a myxoid background.
CASE DESCRIPTION
We report an EWS/pPNET, which exhibited an unusual morphology with cells having an acidophilic cytoplasm set in a myxoid background, raising the possibility of extraskeletal myxoid chondrosarcoma (EMC). A reverse transcription-polymerase chain reaction analysis confirmed the presence of an EWS-FLI1 fusion transcript.
CONCLUSIONS
Morphology, immunohistochemistry, and molecular assays may be necessary to avoid a potential diagnostic pitfall as EWS/pPNET with a myxoid background may histologically resemble an EMC.
引言
尤因肉瘤（EWS）/外周原始神经外胚层肿瘤（pPNET）（EWS/pPNET）是一组发生于骨或软组织的侵袭性很强的小圆细胞肿瘤，具有高转移潜能，在儿童和年轻人中病程进展迅速。EWS/pPNET在显微镜下通常没有黏液样背景。
病例描述
我们报告1例EWS/pPNET，其具有不寻常的形态，细胞具有嗜酸性细胞质，位于黏液样背景中，增加了骨外黏液样软骨肉瘤（EMC）的可能性。逆转录-聚合酶链反应分析证实存在EWS-FLI1融合转录本。
结论
形态学、免疫组织化学和分子检测对于避免潜在的诊断陷阱可能是必要的，因为具有黏液样背景的EWS/pPNET在组织学上可能类似于EMC。
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