11例卵巢高钙血症型小细胞癌临床分析并文献复习

Clinical characteristics of 11 patients with small cell carcinoma of the ovary,hypercalcemic type and literature review

背景与目的:卵巢高钙血症型小细胞癌(small cell carcinoma of the ovary,hypercalcemic type,SCCOHT)是一种罕见的恶性程度极高的妇科肿瘤,好发于年轻女性。分析SCCOHT的临床特征及诊疗情况,并通过文献复习梳理其临床表现、治疗模式及预后因素等特征。方法:对复旦大学附属肿瘤医院2000年1月—2019年12月收治的11例SCCOHT患者的病例资料进行回顾性分析。结果:11例患者中位发病年龄为31岁(22~40岁),临床主要表现为腹痛(63.7%)及盆腔占位性病变(36.4%)。根据原发性卵巢癌2019年国际妇产科联合会(International Federation of Gynecology and Obstetrics,FIGO)分期,11例患者中Ⅰ期有4例,Ⅲ期1例,Ⅳ期6例。4例(36.4%)患者表现为血钙升高。11例患者均接受了手术治疗及术后辅助化疗,9例(81.8%)患者出现盆腔复发,在确诊后1年内死亡,中位生存时间为6个月,6个月生存率为45.5%,10个月生存率为13.6%。结论:SCCOHT的发病年龄较轻,恶性程度极高,预后较差。手术治疗联合辅助化疗是一线治疗手段。盆腔及腹腔是最常见的复发转移部位。靶向治疗及免疫治疗可能有一定的应用前景。

Background and purpose:Small cell carcinoma of the ovary,hypercalcemic type(SCCOHT)is a rare gynecological malignancy with poor prognosis,which mostly occurs in young women.This study analyzed clinical characteristics and treatment of 11 patients with SCCOHT,then clinical manifestations,treatment patterns and prognostic factors were summarized through literature review.Methods:A retrospective study was conducted on clinical data of 11 cases diagnosed with SCCOHT from Jan.2000 to Dec.2019 in Fudan University Shanghai Cancer Center.Results:The median age of 11 patients was 31 years(from 22 to 40 years).The main clinical presentations were abdominal pain(63.7%)and pelvic mass(36.4%).According to 2019 International Federation of Gynecology and Obstetrics(FIGO)staging system for ovarian cancer,stageⅠincluded 4 cases,stageⅢconsisted of 1 case and stageⅣhad 6 cases.Four(36.4%)patients had elevated serum calcium.All patients were administered with surgery followed by adjuvant chemotherapy.Nine(81.8%)cases died within 1 year from initial diagnosis,and the median survival time was 6 months.In this cohort,6-month survival rate was 45.5%,and 10-month survival rate was 13.6%.Conclusion:SCCOHT occurs in younger patients and is difficult to deal with due to its significantly aggressive behavior.Surgery combined with adjuvant chemotherapy is first-line treatment strategy.Pelvis and abdomen are the most common recurrence sites.Targeted therapy and immunotherapy are promising for SCCOHT.