腹膜播散性平滑肌瘤病7例临床病理分析  

A clinicopathological analysis of 7 cases of leiomyomatosis peritonealis disseminata

作　　者：刘冰莹[1] 朱连成[1] 丛建平 武艳霞[1] 刘娟娟[1] 齐跃[1] 林蓓[1] 

机构地区：[1]中国医科大学附属盛京医院妇产科,辽宁沈阳110004

出　　处：《现代肿瘤医学》2017年第24期4037-4041,共5页Journal of Modern Oncology

基　　金：盛京自由研究者计划(编号:201303)

摘　　要：目的:探讨腹膜播散性平滑肌瘤病(leiomyomatosis peritonealis disseminata/disseminated peritoneal leiyomyomatosis,LPD/DPL)的临床病理特征、治疗方法及预后。方法:对2000年1月至2017年1月17年间中国医科大学附属盛京医院7例腹膜播散性平滑肌瘤病的临床病理特征、诊断、治疗及预后进行了回顾性分析。结果:7例患者平均发病年龄为39.3岁(26~47岁)。均为女性患者,其中1例为本病术后复发。多以发现包块为主诉,症状无特殊性,术前诊断率低。主要依靠术中所见及病理确诊,大体可见散在分布于壁层及脏层腹膜的灰白或灰红小结节,边界清楚,表面光滑;镜下见瘤细胞呈梭型,编织状排列,核分裂象少见。7例全部行手术治疗,只有1例术中快速冰冻病理回报符合LPD,术后石蜡病理明确诊断。随访5例,中位随访时间为37个月,2例复发。结论:LPD是一种良性肿瘤,但具有播散种植行为,确诊依靠术后石蜡病理,治疗以手术为主,以病灶切除干净为原则,因考虑该病为雌激素依赖性疾病及大网膜为易种植部位,年龄偏大无生育要求者可以考虑全子宫双附件切除术+大网膜切除术,术后需定期复查,严密随访。Objective：To investigate the clinical and pathological features, therapy, prognosis of leiomyomatosis peritonealis disseminata. Methods：7 cases of leiomyomatosis peritonealis disseminata admitted between January 2000 and January 2017 in Affiliated Hospital of China Medical University were analyzed retrospectively, including detailed clinical data. Results： The mean age of onset of the involved 7 patients was 39.3 years old （ ranging from 26 - 47 years old）. All the patients were females, and one of them was the postoperative recurrence of the disease. Chief com- plaint was the appearance of mass by most of the patients. Symptoms of the disease were not specific with low preoper- ative diagnosis rate. The diagnosis was made on the basis of the intraoperative findings and pathology. Gray or small gray nodules scattered in the parietal and visceral peritoneum were seen in general,with clear border and smooth sur- face. Microscopically,the tumor cells were spindle shaped and arranged in a braided configuration, and mitotic figures were rare. All 7 cases underwent surgical treatment, only 1 case of intraoperative rapid frozen pathological result was consistent with LPD, and the pathological diagnosis of paraffin was confirmed postoperatively. A total of 5 cases were followed up, and the median follow - up time was 37 months ,2 of them experienced recurrence. Conclusion：LPD is a benign tumor characterized by a growth manner suggestive of disseminated neoplasm. The diagnosis was made with postoperative paraffin pathology as a reference, and operation is the main treatment of LPD according to the principle of complete resection of lesions. Considering the disease as an estrogen dependent disease, the greater omentum is the predominant site of dissemination, total hysterectomy and bilateral adnexectomy ＋ omentectomy may be considered in older patients with no fertility requirements, combined with regular review and strict follow - up after operation.

关 键 词：腹膜播散性平滑肌瘤病 临床病理特点 鉴别诊断 治疗 预后 

分 类 号：R735.5[医药卫生—肿瘤]