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In males it is rare and often associated with other non-seminomatous germ cell tumours of the testis. Choriocarcinoma often presents with metastatic disease and elevated \u03b2HCG levels. Usually, patients' symptoms are associated with the different metastatic sites and they can be severe or even life-threatening. Moreover, choriocarcinoma is chemosensitive and the administration of chemotherapy with curative intent may lead to tumour-lysis syndrome and the more specific choriocarcinoma syndrome (CS). Therefore, the treatment of metastatic choriocarcinoma is complex, involving both oncological therapy and the management of acute complications. This review explores choriocarcinoma in males, focusing on its clinical presentation, pathogenetic mechanisms, and treatment options, including investigational therapies. 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