原发性卵巢平滑肌肉瘤3例报告并文献复习

Primary ovarian leiomyosarcoma:arebort of three cases report and review of literature

目的分析原发性卵巢平滑肌肉瘤的病因、组织学特性、临床特点、治疗方法及预后。方法回顾性分析解放军总医院收治的3例原发性卵巢平滑肌肉瘤的诊疗经过,并结合近10年来国内外的文献资料,总结原发性卵巢平滑肌肉瘤的诊治经验。结果本组3例原发性卵巢平滑肌肉瘤均发生于>50岁的女性,发病原因不明确,临床表现无特异性,病情进展快。确诊主要依靠病理,同时结合免疫组化Desmin、Vimentin、SMA等诊断。手术范围及治疗方法目前仍存在争议,无确切有效的辅助放化疗方案。结论原发性卵巢平滑肌肉瘤早期诊断困难,恶性程度高,预后差。研究发病机制及最佳治疗方案是今后临床研究的重要课题。

Objective To analyze the origin,etiology,histological and clinical features,treatment and prognosis of primary ovarian leiomyosarcoma（POLMS）.Methods Based on the retrospective analysis of the clinical data of three patients with POLMS admitted into the General Hospital of PLA,follomed by a review of the pertinent literature published the last decade,the diagnostic and treatment experiences for POLMS were summarized.Results POLMS occurred when they were over 50 years old in all the three patients.The etiology was unclear with no speecific clinical features.The symptoms of disease developed rapidly.The final diagnosis was mainly founded on the pathological examination,combined with the immunohistochenmical staining of negative Desmin and positive Vimentin and SMA.The surgical excision range and therapeutic regimen of POLMS remained controversial.No suitable adjuvant chemotherapy and radiotherapy regimens were available nowadays.Conclusions The early diagnosis of POLMS,which is a highly malignant tumor with poor prognosis,is difficult.It is important hereafter to study the pathogenesis and the best treatment modality for POLMS.