卵巢高钙血症型小细胞癌7例临床分析

目的：总结并分析卵巢高钙血症型小细胞癌（small cell carcinoma of the ovary-hypercalcemic type，SCCOHT）的临床特点、诊断、治疗及预后。方法：对我院1994-2018年诊治的7例SCCOHT病例资料进行回顾性分析。结果：7 例SCCOHT患者的平均年龄为（23.4±9.9）岁，＜30岁5例。Ⅰ期4例，Ⅲ期2例，Ⅳ期1例。临床多以腹痛、腹胀及发现盆腔包块为主诉，其中6例患者右侧卵巢受累，1例发生于左侧，5例（71.4%）诊断时伴血钙升高，6例（85.7%）血清CA125水平升高。7例患者均接受了手术及以铂类为基础的联合化疗，1例患者术后还接受了放疗。随访时间2～120个月，其中2例患者生存时间超过3年，其余5例均在发病2年内死亡。结论：SCCOHT是一类罕见的卵巢高度恶性肿瘤，预后很差，易发生于年轻女性，临床表现缺乏特异性，免疫组化有助于诊断，治疗以手术及以铂类为基础的化疗为主要治疗手段，最佳化疗方案有待进一步研究。

Clinical Characteristics of 7 Cases with Small Cell Carcinoma of the Ovary-hypercalcemic Type

Objective：To analyze the clinical characteristics, diagnosis, treatment and outcomes of patients with SCCOHT. Methods：A retrospective analysis was performed on clinical and pathological data of 7 patients with pathologically confirmed SCCOHT between 1994 and 2018. Results：The average age of 7 cases was (23.4 ±9.9) years old, with 5 cases younger than 30 years old. There were 4 cases in stage I, 2 in stage III, and 1 in stage IV. Abdominal pain, bloating and pelvic mass were the most common clinical presentation. The affected ovary of 6 patients was on the right side, 1 patient was on the left. Five patients (71.4%) had elevated serum calcium and 6 (85.7%) had elevated serum CA125 level at the time of diagnosis. All 7 patients were treated with surgery and platinum-based combination chemotherapy. Only 1 patient received radiotherapy after surgery. During the follow-up period of 2-120 months, only 2 patients survived more than 3 years, and the remaining 5 patients all died within 2 years of onset. Conclusions：SCCOHT is an uncommon and highly malignant tumor that usually occurs in young females with a dismal prognosis. The clinical manifestations are nonspecific and immunohistochemistry is an efficient method for diagnosis. The traditional treatments for SCCOHT are surgery and adjuvant platinum-based chemotherapy. The optimal regimen needs further study.