Patients aged under 16 years presenting to the Royal Children's Hospital be
tween 1967 and 1997 with pineal region tumours were retrospectively reviewe
d. Thirty-seven patients were identified, with 13 germinomas, 7 nongerminom
atous germ cell tumours, 6 pineoblastomas, 2 pineocytomas, and 3 astrocytom
as, while in 6 patients no histopathological diagnosis was obtained. The mo
st common presentation was with symptoms of raised intracranial pressure du
e to hydrocephalus. Thirty-two of the 37 patients required a shunt. Thirtee
n had a biopsy as a separate procedure, 3 of which were stereotactic. Tumou
r excision was performed in 21 patients and was complete in 4 and subtotal
in 17. There were 2 perioperative deaths and 6 patients who were neurologic
ally worse after surgery. Twenty-six patients had radiotherapy and 16 chemo
therapy, with significant complications of radiotherapy in half of the pati
ents who received it. The 5-year survival of patients with benign tumours w
as 75%, 5-year survival with germinomas 62% and with other malignant tumour
s 14%. This series demonstrates significant improvements in management of p
ineal region tumours in the last 30 years and highlights some of the curren
t controversies. A collaborative research approach is necessary to determin
e optimal management of the varied tumour types occurring in the pineal reg
ion in childhood.