title: Pineal region tumours in childhood. A 30-year experience.
abstract: Patients aged under 16 years presenting to the Royal Children's Hospital between 1967 and 1997 with pineal region tumours were retrospectively reviewed. Thirty-seven patients were identified, with 13 germinomas, 7 nongerminomatous germ cell tumours, 6 pineoblastomas, 2 pineocytomas, and 3 astrocytomas, while in 6 patients no histopathological diagnosis was obtained. The most common presentation was with symptoms of raised intracranial pressure due to hydrocephalus. Thirty-two of the 37 patients required a shunt. Thirteen had a biopsy as a separate procedure, 3 of which were stereotactic. Tumour 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 neurologically worse after surgery. Twenty-six patients had radiotherapy and 16 chemotherapy, with significant complications of radiotherapy in half of the patients who received it. The 5-year survival of patients with benign tumours was 75%, 5-year survival with germinomas 62% and with other malignant tumours 14%. This series demonstrates significant improvements in management of pineal region tumours in the last 30 years and highlights some of the current controversies. A collaborative research approach is necessary to determine optimal management of the varied tumour types occurring in the pineal region in childhood.
authors.affiliation: Department of Surgery, The Royal Melbourne Hospital, Parkville, Victoria, Australia. kate.drummond@nwhcn.org.au
authors.forename: K J
authors.initials: KJ
authors.lastname: Drummond
authors.forename: J V
authors.initials: JV
authors.lastname: Rosenfeld
mesh_terms: D000293:Adolescent; D001706:Biopsy; D001932:Brain Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D002557:Cerebrospinal Fluid Shunts; D002648:Child; D002675:Child, Preschool; D004358:Drug Therapy; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000150:complications / Q000601:surgery; D007223:Infant; D019586:Intracranial Hypertension / Q000209:etiology; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D010870:Pineal Gland / Q000473:pathology*; D011612:Psychosurgery / Q000379:methods; D011878:Radiotherapy; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome