Background
Diagnosis of intracranial germ cell tumours (IC-GCTs) occurs
predominantly in children with peak incidence during the second decade
of life 1-3.
The two most frequent brain locations are the pineal gland and the
suprasellar region or both sites simultaneously (“bifocal disease”).
IC-GCTs can also arise in the basal ganglia, thalamus, cerebral
hemisphere, and cerebellum 4,5.
According to the WHO classification system, IC-GCTs are divided into
germinomas (GTs) and non-germinomatous (NGGCTs) 6.
Germinomas comprise 60-65% of all paediatric IC-GCTs. IC-GCTs can also
be divided into ”secreting” and ”non-secreting” tumours based on the
presence of tumour markers measured in the cerebral spinal fluid (CSF)
and/or serum: alpha-fetoprotein (AFP) and beta-human chorionic
gonadotropin (beta-HCG) 7.
Different CT and RT treatment regimens have been developed in Western
Countries 8-11 and in Japan12according to histology and prognosis. Intracranial germinomas have an
excellent overall survival (OS) >90%, whereas the
prognosis for patients with recurrent IC-NGGCTs is poor13,14.