宫颈血管周上皮样细胞肿瘤一例

doi:10.12280/gjfckx.20230974

摘要/Abstract

摘要：

血管周上皮样细胞肿瘤（perivascular epithelioid cell tumor，PEComa）是一组临床上少见的间叶源性肿瘤，主要发生在围绝经期和绝经后的女性，原发肿瘤多位于子宫和腹膜后等部位，其中子宫PEComa多发生于子宫体浆膜下，少数患者发生于宫颈及黏膜下，恶性者罕见。报告1例因考虑腹盆腔占位及多发骨转移就诊的宫颈PEComa，该患者因左膝关节反复疼痛在当地医院行骶髂关节CT，发现腹盆腔占位性病变及双侧髂骨及腰骶尾骨多发高密度灶，2023年7月收治后结合影像学检查和盆腔肿物穿刺活检考虑子宫肌瘤恶变，排除手术禁忌证后，在全身麻醉下行腹式全子宫切除术+双侧附件切除术+盆腔淋巴结活检+盆腔粘连松解术。术后病理提示宫颈PEComa、淋巴管平滑肌瘤病，出院后定期门诊复诊，术后5个月未见复发。宫颈PEComa的临床表现、影像学表现缺乏特异性，临床上易误诊、漏诊，病理诊断是金标准，目前主要治疗手段为手术切除肿瘤组织。

关键词: 血管周上皮样细胞肿瘤, 间叶瘤, 宫颈肿瘤, 免疫组织化学, 病例报告

Abstract:

Perivascular epithelioid cell tumor (PEComa) is a group of clinically rare mesenchymal tumors, mainly occuring in perimenopausal and postmenopausal women. The primary tumors are mostly located in uterus, retroperitoneum and other sites. PEComa of uterus mostly occurs in the uterine body subserous membrane, and a few patients occur in cervix and submucosa, and malignancy is rare. A case of cervical PEComa was reported due to abdominal pelvic space occupying and multiple bone metastases. The patient underwent CT of the sacroiliac joint in a local hospital due to repeated pain of the left knee joint, and was found that abdominal pelvic space occupying lesions and multiple high density lesions of the iliac bone and lumbosacral coccygeal bone. After admission in July 2023, combined with imaging examination and pelvic mass biopsy, malignant myoma was considered, and abdominal total hysterectomy, double adnexectomy, pelvic lymph node biopsy and pelvic adhesion lysis were performed under general anesthesia after exclusion of surgical contraindications. Postoperative pathology indicated cervical PEComa and lymphangioleiomyomatosis. Regular outpatient follow-up after discharge, no recurrence was found 5 months after surgery. The clinical and imaging manifestations of cervical PEComa are lack of specificity, and it is easy to be misdiagnosed and missed clinically. Pathological diagnosis is the gold standard, and the main treatment method at present is surgical resection of tumor tissue.

Key words: Perivascular epithelioid cell neoplasms, Mesenchymoma, Uterine cervical neoplasms, Immunohistochemistry, Case reports

许佳蓉, 谢乐, 庞卓超. 宫颈血管周上皮样细胞肿瘤一例[J]. 国际妇产科学杂志, 2024, 51(2): 227-231.

XU Jia-rong, XIE Le, PANG Zhuo-chao. A Case Report of Cervical Perivascular Epithelioid Cell Tumor[J]. Journal of International Obstetrics and Gynecology, 2024, 51(2): 227-231.

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