Pineal region tumours in childhood - A 30-year experience

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.