Germ cell tumours in children and adolescents

Germ cell tumours (GCTs) are a heterogeneous group of predominantly midline tumours uniquely occurring from birth to late adulthood. All are believed to arise from the totipotent primordial germ cell. In childhood, ∼50% are gonadal and ∼50% extragonadal (∼20% intracranial and ∼30% extracranial) and clinical presentation depends on tumour site. Teratomas account for ∼50% of all paediatric GCTs and are considered benign in childhood. Malignant GCTs often secrete the tumour markers alpha-fetoprotein (AFP) and human chorionic gonadotrophin (ß-HCG), which may help in diagnosis and follow-up. Management involves complete surgical resection for teratomas and non-metastatic gonadal tumours. In the UK, chemotherapy is reserved for stage 2–4 extracranial malignant GCTs, resulting in >90% five-year overall survival (OS). Radiotherapy is rarely indicated for extracranial disease. Intracranial tumours typically occur in the pineal or suprasellar region. Intracranial germinomas are cured in >90% cases with radiotherapy or combined chemo-radiotherapy. About two-thirds of non-germinomatous intracranial tumours are cured with combined chemo-radiotherapy. Current issues relating to the management of teenagers and young adults (TYAs) with GCTs and implications of tumour biology are highlighted in this review.