Department of Obstetrics and Gynecology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Shuaifuyuan No. 1, Dongcheng District, 100730, Beijing, China.
J Ovarian Res. 2020 Dec 8;13(1):142. doi: 10.1186/s13048-020-00749-x.
Ovarian fibrosarcoma is an extremely rare and malignant sex cord-stromal tumor. Due to its low incidence and poor prognosis, until now, very few cases have been reported, and most of the reported cases have been sporadic. Therefore, the treatments and prognostic factors of ovarian fibrosarcoma are still debatable. Here, we report 5 cases of ovarian fibrosarcoma that presented at Peking Union Medical College Hospital over the past 20 years. The 5 patients were 41, 51, 54, 76, and 76 years of age when initial symptoms of pelvic mass or pain appeared. On ultrasound, this disease usually manifests as unilateral pelvic masses, within which uneven echo enhancement and some blood flow signals are observed. No significant increase was observed in the levels of preoperative tumor markers, such as serum CA125 and sex hormones. The final diagnosis depends on postoperative histopathological results since these tumors are easily misdiagnosed when intraoperative frozen sections are used for examination. Pathologic examinations showed that the tumor cells were spindle-shaped with moderate to severe atypia and high mitotic counts. The immunohistochemistry profile is not specific, but the positive rate of Ki-67 was consistent with the degree of malignancy and the prognosis of patients with this tumor. In addition, the tumor may also be positive for Vimentin, α-inhibin, SMA, estrogen receptor and progesterone receptor. Significant differences were observed in the surgical methods used, and no unified chemotherapy regimen has been established. The overall survival was > 15, >7, > 6, <1, and < 1 year for each patient. After reviewing the literature, evidence-based large-scale case studies were lacking. For treatments, complete cytoreductive surgery plus regimens typically used against malignant sex cord-stromal tumors, as described in the NCCN guidelines, are recommended. Due to its low incidence, both multicenter clinical studies and molecular studies are required to provide gynecologists with a better understanding and guidance for future management of patients with ovarian fibrosarcoma.
卵巢纤维肉瘤是一种极其罕见且恶性的性索-间质肿瘤。由于其发病率低、预后差，到目前为止，报道的病例很少，且大多数报道的病例都是散发性的。因此，卵巢纤维肉瘤的治疗方法和预后因素仍存在争议。在此，我们报告了过去 20 年来北京协和医院收治的 5 例卵巢纤维肉瘤患者。这 5 例患者初次出现盆腔肿块或疼痛时的年龄分别为 41 岁、51 岁、54 岁、76 岁和 76 岁。超声检查通常表现为单侧盆腔肿块，不均匀回声增强，可见部分血流信号。术前肿瘤标志物如血清 CA125 和性激素水平无明显升高。最终诊断依赖于术后组织病理学结果，因为这些肿瘤在术中冷冻切片检查时容易误诊。病理检查显示肿瘤细胞呈梭形，中度至重度异型性，有较高的有丝分裂计数。免疫组化谱不具有特异性，但 Ki-67 的阳性率与肿瘤的恶性程度和患者的预后一致。此外，肿瘤可能还会表达波形蛋白、α-抑制素、SMA、雌激素受体和孕激素受体。手术方法的使用存在显著差异，尚未建立统一的化疗方案。每位患者的总生存率分别为>15 年、>7 年、>6 年、<1 年和<1 年。通过文献复习，发现缺乏基于证据的大规模病例研究。对于治疗，建议采用完全细胞减灭术加 NCCN 指南中描述的针对恶性性索-间质肿瘤的常规方案。由于发病率低，需要进行多中心临床研究和分子研究，以便为妇科医生提供更好的理解，并为未来卵巢纤维肉瘤患者的管理提供指导。
