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卵巢原发原始神经外胚层肿瘤(PNETs)是向原始神经方向分化的小圆细胞恶性肿瘤,临床罕见,侵袭性强、且死亡率高,病理是诊断的金标准。目前无明确诊治指南,文献多为个案报道,其治疗方案常借鉴尤文氏肉瘤家族肿瘤,多采用根治性切除手术联合全身性化疗和(或)放疗的综合方案。卵巢原发PNETs在首次就诊时常表现出转移,临床预后极差,生存期从10.8个月到3年不等。
Ovarian primitive primary neuroectodermal tumors (PNETs) is a small round cell malignant tumor that differentiates to the original nerve direction. It is rare in clinic, has strong invasiveness, high mortality and pathological diagnosis. There is no clear diagnosis and treatment guidelines, the literature is mostly case reports, the treatment programs often learn from Ewing’s sarcoma family tumors, and more use of radical resection combined with systemic chemotherapy and / or radiotherapy comprehensive program. Primary ovarian PNETs often show metastases at first visit, with poor prognosis and survival from 10.8 months to 3 years.