Malignant germ cell tumors in childhood

Malignant germ cell tumors are a very heterogeneous group of neoplasms that have historically carried a poor prognosis. A review of the cooperative studies on malignant extracranial germ cell tumors in children was performed to update the progress in treatment and outcome for this rare group of tumors. The advent of platinum-based chemotherapy in the 1980s dramatically improved the prognosis for these tumors and allowed redefinition of risk groups. Chemotherapy regimens have been sequentially modified to reduce toxicity while maintaining survival. Surgical management has evolved from an aggressive en bloc resection at diagnosis to a more tailored approach, with resection and meticulous staging for low stage tumors at diagnosis and biopsy with neoadjuvant chemotherapy for advanced stage tumors. Improved surgical margins and prognosis are seen in post chemotherapy resections for high stage tumors. Large persisting masses after chemotherapy are often due to coexisting elements of benign teratoma, and aggressive attempts at resection are warranted in these patients with expectation of successful outcome.