病例报告:宫颈原发性血管肉瘤罕见病例及文献综述

Yuelin Song, Ruizhe Li, Lifei Wang, Hongjing Wang
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摘要

摘要 原发性血管肉瘤是一种罕见的软组织肉瘤,起源于内皮细胞。这种肉瘤可发生在身体的任何部位,预后较差。不过,它们常见于老年白人男性的皮肤,尤其是头皮和头部区域。宫颈原发性血管肉瘤异常罕见。迄今为止,全世界仅报道过两例这种疾病。这种疾病在显微镜下很难诊断,需要通过免疫组化和基因检测来鉴别。我们报告了最近的一例病例,术前认为病变是高级别子宫内膜间质肉瘤。一名 35 岁的妇女出现阴道出血和宫颈糜烂。考虑为累及宫颈的高级别子宫内膜间质肉瘤,于是进行了改良根治性子宫切除术,同时进行了双侧输卵管切除术和前哨淋巴结切除术。通过荧光原位杂交对YWHAE易位融合进行基因诊断,结果为阴性,排除了YWHAE易位的高级别子宫内膜间质肉瘤。最终确诊为宫颈原发性血管肉瘤。宫颈原发性血管肉瘤非常罕见,妇科病理学家对其也不甚了解,因此很容易误诊。免疫组化和基因检测有助于确诊。
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Case Report: A Rare Case of Primary Angiosarcoma of the Cervix with a Literature Review
Abstract Primary angiosarcomas are a rare type of soft-tissue sarcomas that originate from endothelial cells. These sarcomas can develop in any part of the body and have a poor prognosis. However, they are commonly found in the skin of elderly white men, particularly on the scalp and head region. Primary angiosarcoma of the cervix is exceptionally rare. To date, only two cases of this disease have been reported worldwide. The diagnosis of the disease is difficult microscopically, requiring immunohistochemistry and genetic testing to distinguish. We report a recent case, in which the lesion was preoperatively considered a high-grade endometrial stromal sarcoma. A 35-year-old woman presented with vaginal bleeding and cervical erosions. A high-grade endometrial stromal sarcoma involving the cervix was considered and a modified radical hysterectomy was performed with bilateral salpingo-oophorectomy and sentinel lymph nodes resection. The gene diagnosis performed by fluorescence in situ hybridization for YWHAE translocation fusion was negative excluding a YWHAE-translocated high-grade endometrial stromal sarcoma. A primary angiosarcoma of the cervix was finally diagnosed. Primary angiosarcoma of the cervix is rare, and gynecologic pathologists do not know it well, so it is easy to be wrongly considered. Immunohistochemistry and genetic testing help confirm the diagnosis.
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