卵巢原发原始神经外胚层肿瘤4例临床分析并文献复习

原始神经外胚层肿瘤（primitive neuroectodermal tumor，PNET）分为中枢型和外周型，外周型PNET属于尤文肉瘤家族肿瘤，其发生是由于基因调控失常，原始干细胞向神经上皮各不同阶段分化，成为形态上类似于儿童中枢神经系统的肿瘤，其组织学及临床生物学行为类似于骨外的尤文肉瘤。卵巢原发PNET为外周型PNET，属于卵巢单胚层畸胎瘤，极为罕见，疾病早期即可发生转移、容易复发，预后极差。其临床表现缺乏特异性，患者常以腹痛、腹胀、盆腔包块就诊，病理是诊断的金标准。目前无明确诊治指南，国内外相关文献多为病例报道，1975-2017年英文文献报道的原发于卵巢的PNET仅17例。本文报道4例我院诊治的卵巢原发PNET，同时复习相关文献，描述此种罕见疾病的临床表现、治疗方法及预后情况。

Ovary Primary Primitive Neuroectodermal Tumor: Clinical Analysis of 4 Cases and Literature Review

Primitive neuroectodermal tumor (PNET) is divided into central and peripheral types. Peripheral PNET belongs to the family of Ewing′s sarcoma. Its occurrence is due to abnormal gene regulation, thus the primitive stem cells differentiate into different stages of neural epithelialcells which is similar to pediatric central nervous system tumors morphologically. The histological and clinical biological behavior of the peripheral PNET are similar to extra-osteous Ewing′s sarcoma. Ovarian primary PNET is peripheral PNET, belonging to the ovarian monoderm teratoma, which is extremely rare and metastasizes early, relapses easily with poor prognosis. The clinical manifestation is lack of specificity, and patients always show with chief complaints with abdominal pain, abdominal distention and pelvic mass. The pathology is the gold standard for diagnosis. There is still no exact guideline for treatment, and most articles regarding this neoplasm have been case reports because of its rarity. Only seventeen cases of ovarian primary PNET had been reported in the English language literature between 1975 and 2017. And we reported four additional cases treated at the Peking Union Medical College Hospital to depict the clinical behavior, treatment strategies and prognosis of the ovarian primary PNET.