[Pediatric germ cell tumours]

Germ cell tumours include a group of highly heterogeneous tumours regarding to their clinical and histological appearance. Altogether, they represent 3% of cancers diagnosed in children and adolescent younger than 15 years. A bimodal age distribution is observed with a small peak during infancy and a larger peak after puberty. Non-seminomateous germ cell tumours are largely predominant as compared to seminomateous tumours, rarely seen before puberty. During infancy, sacrococcygeal locations predominate with either teratomas in neonates or yolk sac tumours in infants above three months. In adolescents, mixed germ cell tumours predominate with either gonadal, mediastinal or intracranial tumour. Surgical resection of the tumour is fundamental and must be carcinologic and conservative at the same time. Neoadjuvant chemotherapy may help to decrease the volume of the tumour making possible a delayed sparing surgery. Indeed, except for teratomas, these tumours are highly sensitive to chemotherapy, in particular to platinum salts. Prognosis is good even in metastatic diseases. This raises the question of a therapeutic de-escalation in an attempt to decrease long-term toxicity, in particular audiologic and renal impairment. On the opposite, recurrent or refractory diseases after chemotherapy carry a dismal prognosis and therapeutic strategies remain to be defined in such situations.