Gerl A, Clemm C, Kohl P, Niedermaier J, Schalhorn A
Medizinische Klinik III, Klinikum Grosshadern, Universität München.
Med Klin (Munich). 1994 May 15;89(5):240-4.
Primary extragonadal germ cell tumors are a rare malignant disease in young males. They account for only 1 to 4% of all germ cell tumors.
In this paper we describe three selected cases of primary extragonadal germ cell tumors. The literature is reviewed with regard to clinical features, differential diagnosis and treatment.
Tumor markers alpha-fetoprotein and human chorionic gonadotropin are of considerable diagnostic value if disease distribution is considered. With cisplatin-based combination chemotherapy similar disease-free survival rates are achieved as for testicular tumors with poor-prognosis metastatic disease. Surgical procedures play a role as adjunctive modality.
If young males present with a mass in the retroperitoneum or in the anterosuperior mediastinum, a primary extragonadal germ cell tumor, should be taken into consideration. Tumors of both localisations have distinct clinical features but carry a similar prognosis. Patients benefit from the cumulative experience of a specialist unit.
原发性性腺外生殖细胞肿瘤是年轻男性中一种罕见的恶性疾病。它们仅占所有生殖细胞肿瘤的1%至4%。
本文描述了3例原发性性腺外生殖细胞肿瘤的精选病例。对有关临床特征、鉴别诊断和治疗的文献进行了综述。
如果考虑疾病分布，肿瘤标志物甲胎蛋白和人绒毛膜促性腺激素具有相当大的诊断价值。采用以顺铂为基础的联合化疗，可获得与预后不良的转移性睾丸肿瘤相似的无病生存率。手术作为辅助手段发挥作用。
如果年轻男性出现腹膜后或前上纵隔肿块，应考虑原发性性腺外生殖细胞肿瘤。这两个部位的肿瘤具有不同的临床特征，但预后相似。患者受益于专科单位的累积经验。
