ObjectiveTo analyze the management and prognosis of primary choriocarcinoma (PCC) in male patients.MethodsThe clinical records of males with PCC who were treated at Peking Union Medical College Hospital between 1990 and 2012 were analyzed retrospectively. The literature regarding this clinical condition was also reviewed.ResultsThe median survival interval of the 13 patients treated at Peking Union Medical College Hospital was 54 months (range, 6–115 months), and the 1- and 3-year survival rates were 53.8% and 43.1%, respectively. All patients were treated with surgery; 12 were treated with combined chemotherapy. After including 100 cases found in the literature, for a total of 113 patients, the median survival interval was 10 months (range, 6.4–13.6 months). The testis was the most common primary site (36.2%). Most patients (70.9%) had metastatic lesions at diagnosis. Univariate and multivariate analyses revealed that longer median overall survival was significantly associated with patient age <34 years old (48 months vs 10 months, odds ratio [OR] =0.47, P=0.029), the presence of other histological components (54 months vs 11 months, OR =0.54, P=0.011), and combined chemotherapy and surgical treatments (14 months vs 2.5 months, OR =0.18, P=0.002).ConclusionPCC is an extremely rare disease among men, and its prognosis is much worse than that of gestational choriocarcinoma. The complete resection of the primary site and metastases followed by chemotherapy seems to provide patients with the best chance at survival. Furthermore, additional chemotherapy cycles might facilitate better progress.