Extragonadal germ cell tumors: clinical presentation and management

Purpose of review The extragonadal germ cell tumors (EGCTs) represent a unique entity, and as such require specialized management. This review article will discuss the diagnosis, prognosis and treatment modalities for EGCTs. Recent findings The anterior mediastinal germ cell tumors (GCTs) are the most common EGCT. These tumors originate in the anterior mediastinum without any testis primary. Mediastinal nonseminomatous GCTs carry a poor prognosis with 40-50% overall survival and should be treated with cisplatin-based chemotherapy followed by surgical resection of the residual tumor. At Indiana University, we recommend etoposide (VP-16), ifosfamide and cisplatin (VIPx4) instead of bleomycin, etoposide and platinum (BEPx4) to prevent pulmonary complications, as these patients require extensive thoracic surgical resection. Patients who relapse have a dismal outcome with only 10% long-term survival. Our preferred treatment option is surgery for localized relapse; if surgery is not feasible, then high-dose chemotherapy with stem cell transplant in an experienced center is a reasonable approach. Retroperitoneal GCT should be treated in a similar fashion to primary testis cancer. Summary The utilization of cisplatin-based chemotherapy is associated with the best chance of cure for EGCTs. This should be followed by surgical resection of residual tumor in nonseminomatous GCT.