Ekstragonadal germ cell tumors (EGCT) are rare and unique neoplasms of which both diagnosis and management need specialization. This review aims to discuss current EGCT classification, diagnosis, prognosis and treatment.

They are mostly located on the midline structures from pineal gland to sacrococygeal area. There is no gonadal primary. In adults, EGCT is most frequently located in the mediastinum. Retroperitoneum is the second most frequent location. More than 90% of adult age EGCTs are diagnosed between 20 to 35 years. Isochromosome 12p is the common chromosomal anomaly observed in both EGCT and gonadal GCTs. The histological subtype of the tumor plays an important role in management and prognosis. Treatment modalities may not reach the success seen in gonadal GCTs (for example: survival in mediastinal non-seminomatous EGCTs is 40-50%). Cisplatinum-based chemotherapy, surgery for residual masses and radiotherapy for intracranial masses are the main management strategies. High dose chemotherapy and salvage surgery is preferred fort the treatment of relapses.

Keywords: extragonadal germ cell tumors; dysgerminoma, primary mediastinal non-seminomatous germ cell tumors, retroperitoneal germ cell tumors, teratoma