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.