Extracranial malignant germ cell tumors represent approximately 3% of cancers in children and adolescents. Their age distribution is bimodal, with a first peak in early childhood and a second after puberty, extending into adulthood. Non-seminomatous germ cell tumors (NSGCTs), characterized by tumor markers secretion, predominate. Treatment strategies have evolved over successive clinical protocols.

Subsequent studies, particularly the Franco-Belgian TGM95 and TGM13-NS protocols, have demonstrated an excellent overall prognosis for NSGCTs. Management is primarily based on a surgical approach aiming to achieve oncologic control while preserving organ function whenever feasible, coupled, when indicated, with platinum-based chemotherapy tailored to the risk group. To minimize treatment-related toxicities, chemotherapy regimens and the number of cycles have been progressively optimized.

We present the current national therapeutic recommendations for NSGCTs. Tumor stage and site, initial tumor marker levels, and patient age allow risk stratification and guide treatment decision. Adolescents should benefit from coordinated care between pediatric and adult oncology teams and be included in clinical trials whenever possible.

Following completion of the TGM13-NS protocol, the Germ Cell Tumor Group of the French Society of Childhood Cancer developed updated management guidelines and is simultaneously involved in international collaborations to establish larger-scale studies, justified by the rarity of these cancers.