Lee M W, Stephens R L
Cancer. 1987 Sep 1;60(5):1053-5. doi: 10.1002/1097-0142(19870901)60:5<1053::aid-cncr2820600520>3.0.co;2-1.
A 25-year-old man presented with diffuse metastatic pure choriocarcinoma, thyrotoxicosis, and cardiac tamponade. No discernable testicular primary tumor was found. The patient's peripheral blood karyotype was 47, XXY and phenotypic features of Klinefelter's syndrome were present. The patient was treated with aggressive combination chemotherapy followed by salvage surgery and remains in complete remission 3 years after diagnosis. Pure choriocarcinoma, although rare as a primary testicular neoplasm, accounts for 15% of extragonadal germ cell tumors in general and 30% of germ cell tumors in patients with Klinefelter's syndrome. Historically, the diagnosis of pure choriocarcinoma has been thought to convey a very poor prognosis. The occurrence of hyperthyroidism is unique to tumors containing choriocarcinomatous elements and the management of this disorder is discussed. Treatment of extragonadal germ cell tumors is also discussed with special reference to the roles of combination chemotherapy and salvage surgery.
一名25岁男性患者，患有弥漫性转移性纯绒毛膜癌、甲状腺毒症和心脏压塞。未发现明显的睾丸原发性肿瘤。患者外周血核型为47, XXY，具有克氏综合征的表型特征。患者接受了积极的联合化疗，随后进行了挽救性手术，诊断后3年仍处于完全缓解状态。纯绒毛膜癌作为原发性睾丸肿瘤虽然罕见，但一般占性腺外生殖细胞肿瘤的15%，在克氏综合征患者的生殖细胞肿瘤中占30%。从历史上看，纯绒毛膜癌的诊断一直被认为预后很差。甲状腺功能亢进症的发生是含有绒毛膜癌成分肿瘤所特有的，本文讨论了该疾病的管理。本文还特别参考联合化疗和挽救性手术的作用，讨论了性腺外生殖细胞肿瘤的治疗。
