高级别子宫内膜间质肉瘤1例及文献复习

High-Grade Endometrial Stromal Sarcoma: A Case Report and Review of Literature

摘要: 目的：探讨高级别子宫内膜间质肉瘤(HGESS)病理分型、病因、临床特征、诊断、治疗方案及预后。背景：子宫内膜间质肉瘤(ESS)是一种罕见的间质癌，高级别子宫内膜间质肉瘤(HGESS)是恶性程度高、发病率极低、临床表现非特异的恶性肿瘤。方法：报道一例高级别子宫内膜间质肉瘤的病例，患者术前月经量异常，行宫腔镜检查及取子宫内膜病理活检，术后病理确诊为高级别子宫内膜间质肉瘤，行二次手术(经腹全子宫切除术 + 双侧附件切除术 + 盆腔粘连松解术 + 盆腔淋巴结清扫术 + 腹主动脉旁淋巴结切除术 + 阑尾切除术 + 大网膜切除术)，术后给予化疗，于术后第3个月肿瘤复发，口服靶向药物抗肿瘤治疗。查阅文献对高级别子宫内膜间质肉瘤病理、病因、临床特征、诊断、治疗及预后进行探讨分析。结果：本例高级别子宫内膜间质肉瘤患者术后第3个月复发，后续口服靶向药物维持治疗。结论：高级别子宫内膜间质肉瘤恶性程度高，临床症状不典型，需依据病理确诊，目前明确诊断后可选择的处理手段包括：手术治疗、术后放化疗；分子靶向治疗或许是HGESS患者复发维持治疗的不错方式。

Abstract: Objective: To investigate the pathological classification, etiology, clinical features, diagnosis, treat-ment and prognosis of high-grade endometrial stromal sarcoma (HGESS). Background: Endometrial stromal sarcoma (ESS) is a rare stromal carcinoma. High-grade HGESS is a malignant tumor with high degree of malignancy, low incidence and non-specific clinical presentation. Methods: A case of high-grade endometrial stromal sarcoma was reported. The patient had abnormal menstrual vol-ume before surgery and underwent hysteroscopy and endometrial pathological biopsy. The patient was diagnosed as high-grade endometrial stromal sarcoma by postoperative pathology. A second operation (total abdominal hysterectomy + bilateral adnexectomy + pelvic adhesiolysis + pelvic lymph node dissection + para-aortic lymph node resection + appendectomy + greater omentum re-section) was performed, chemotherapy was given after surgery, and tumor recurrence occurred in the third month after surgery, and oral anti-tumor therapy with targeted drugs was performed. The pathology, etiology, clinical features, diagnosis, treatment and prognosis of high- grade endometrial stromal sarcoma were analyzed by literature review. Results: In this case, high- grade endometrial stromal sarcoma recurred 3 months after surgery, followed by oral maintenance therapy with tar-geted drugs. Conclusion: High-grade endometrial stromal sarcoma has a high degree of malignancy and atypical clinical symptoms, which needs to be confirmed according to pathological diagnosis. Molecular targeted therapy may be a good way to maintain the recurrence of HGESS patients.