Pineal region tumours in childhood. A 30-year experience

Childs Nerv Syst. 1999 Mar;15(2-3):119-26; discussion 127. doi: 10.1007/s003810050347.


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.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Biopsy
  • Brain Neoplasms / complications
  • Brain Neoplasms / diagnosis*
  • Brain Neoplasms / mortality
  • Brain Neoplasms / therapy*
  • Cerebrospinal Fluid Shunts
  • Child
  • Child, Preschool
  • Drug Therapy
  • Female
  • Humans
  • Hydrocephalus / complications
  • Hydrocephalus / surgery
  • Infant
  • Intracranial Hypertension / etiology
  • Male
  • Neoplasms, Germ Cell and Embryonal / diagnosis*
  • Neoplasms, Germ Cell and Embryonal / mortality
  • Neoplasms, Germ Cell and Embryonal / therapy*
  • Pineal Gland / pathology*
  • Psychosurgery / methods
  • Radiotherapy
  • Retrospective Studies
  • Survival Rate
  • Treatment Outcome