青年女性子宫内膜间质肉瘤:由一例特殊病例到21例病例的回顾性分析

Endometrial stromal sarcoma in young women: a case report and retrospective analysis of 21 cases

目的研究青年女性子宫内膜间质肉瘤(ESS)患者的临床特征、治疗方案及预后。方法回顾性分析1994年4月至2017年4月北京协和医院收治的ESS患者293例,其中年龄小于25岁的患者21例。收集这21例患者的临床资料,对其临床表现、治疗方法及随诊结局进行总结分析。结果293例ESS患者确诊年龄(43.5±12.2)岁(15~81岁),年龄小于25岁的青年患者21例(占7.5%)。这21例患者的临床表现和影像学表现无特异性,85.7%(18/21)表现为异常子宫出血(AUB),90.5%(19/21)影像学存在占位性病变,其中5例患者最初接受内分泌治疗效果不佳,随后接受手术评估。就病理类型而言,低度恶性ESS(LGESS)12例,高度恶性ESS(HGESS)7例,未分化子宫肉瘤(UUS)1例;19例长期随诊患者中,11例患者在中位时间22(5~91)个月出现复发,其中8例出现子宫外复发,1例HGESS复发患者死亡;比较有/无复发两组的肿瘤类型、初次手术方式、是否接受辅助治疗的情况,结果显示LGESS患者、保留卵巢的患者复发率显著增加(P<0.05);8例接受保留生育功能手术的患者中,5例(62.5%)在术后5~22个月复发,仅1例(12.5%)LGESS患者妊娠并足月阴道分娩。结论青年女性发生ESS的情况临床罕见,AUB为其突出表现,但绝大多数都有占位性病变,且对内分泌药物治疗反应不佳;应相对积极进行手术病理评估,以求早期诊断,规范治疗;ESS可尝试保留生育功能的治疗,但复发率高且复发部位不限于子宫局部;保留卵巢显著增加ESS复发风险,生存预后尚可,需要长期随诊。

Objective： To evaluate the clinical characters, treatment and prognosis in young patients with endometrial stromal sarcoma（ESS）. Methods： A total of 293 patients with ESS were treated in Peking Union Medical College Hospital from April 1994 to April 2017. Among them, 21 patients were younger than 25 years old. The medical records of the young patients were collected. The clinical character, treatment and prognostic information were evaluated retrospectively. Results： Of 293 patients,the mean age at initial diagnosis was 43.5 years,and only 21（7.5%）patients were younger than 25 year old. The clinical and imaging findings of the 21 patients were non specific, 85.7%（18/21）patients suffered from irregular uterus bleeding（AUB）and 90.5% （19/21）imaging features shown solid mass of uterus. Five younger patients accepted hormonotherapy as their initial treatment with poor response,and received surgery evaluation subsequently. Among them, 12 patients were diagnosed as low grade ESS（LGESS）, 7 high grade ESS（HGESS）, and 1 undifferentiated uterine sarcoma（UUS）. Of the 19 patients long term follow up, 11 patients had recurrence at a median time of 22（5 -91）months,8 had extrauterus relapse, 1 patient with HGESS recurrence died. Comparing the types of tumors, primary surgical procedures,and adjuvant treatment between the two groups patients with and without recurrence, the results showed that the recurrence rate in the LGESS patients and the patients with ovarian preservation increased significantly（P〈0.05）. Of 8 patients who accepted fertility sparing management for the initial treatment, 5 （62.5%） relapsed 5 to 22 months after surgery, and only 1 （12.5%） of LGESS patients had gestation and full term vaginal delivery. Conclusions： Endometrial stromal sarcoma is a rare kind of uterine malignancy, especially in young women. If young patients with AUB whose imaging examination indicated solid mass of uterus and had poorly response to ＂the endocrine ＂therapy, surgery evaluation should be ＂taken actively for early diagnosis and standard treatment. Although high recurrence rate and extrauterine recurrences for ESS,＇the favorable prognosis may make it a promising candidate for fertility sparing surgery. Ovary sparing surgery significantly increased ＂the risk of recurrence. A long ＂term follow up is necessary.