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Objective to investigate the diagnosis, treatment and prognosis of a rare disease, ovarian yolk sac tumor (OYST).Methods The clinical data of 9 patients with ovarian yolk sac tumor admitted into Oct.2004 to Oct.2014 from Tianjin Central Hospital of Gynecology and Obstetrics were collected, and a retrospective analysis was made and a conclusion was drawn.Results 1.The 9 patients were aged from 17 to 60 Y and the median age was 21 Y.2.5 patients had the symptom of abdominal pain, 4 patients had abdominal distending, and patients with early manifestations occupied 66.7%;The preoperative AFP level of 9 patients increased, the range was 3568 to 94705 ng/ml, and the mean was 38579.1 ng/ml.3.All 9 patient underwent surgical treatment,the reproductive function of 8 patients who had reproductive requirements was reserved, the tumor of 2 patients was not controlled after operation and underwent radical tumor cytoreductive surgery, and 1 menopausal patient directly underwent radical surgery;9 patients received postoperative auxiliary BEP (Bleomycin, Etoposide and Platinol) regimen chemotherapy, and for the 2 patients who underwent tumor cytoreductive surgery again one patient shifted to IEP (Ifosfamide, Etoposide and Platinol) due to the dissatisfying results of a drop in AFP, and the other patient shifted to the Velplang regimen chemotherapy (Vincristine, Ifosfamide and Bleomycin).4.The median follow-up time was 95 months (20-118 months), 1 ⅢA phase patient died, the survival time of 2 ⅢC phase patients was >96 months and > 32 months respectively, the survival time of 6 IC phase patients was > 94 months, >54 months, > 48 months, > 114 months, > 118 months and > 20 months respectively, and 2 of the 6 patients with reproductive functions reserved had given birth to infants.Conclusions ovarian yolk sac tumor is an ovarian malignant tumor originating from genital cells, it is relatively rare and commonly occurs to young women.Serum AFP is a kind of specific tumor marker secreted by OYST, and delivers a significant meaning in the diagnosis of OYST.OYST is very sensitive to chemotherapy.For patients with reproductive requirements, the surgery that reserved the reproductive functions was adopted for the patients with reproductive requirements, and after operation the effective BEP regimen auxiliary chemotherapy was adopted.