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Published on: January 17, 2018
Pineal region tumours in childhood. A 30-year experience
1Department of Surgery, The Royal Melbourne Hospital, Parkville, Victoria, Australia. kate.drummond@nwhcn.org.au
Insights
Management of pediatric pineal region tumors has improved, with better survival rates for germinomas and benign types. Further research is needed for optimal treatment of diverse childhood pineal tumors.
Area of Science:
- Pediatric Oncology
- Neurosurgery
- Neuropathology
Background:
- Pineal region tumors are rare in children.
- Diagnosis and treatment have evolved over three decades.
Purpose of the Study:
- To review the management and outcomes of pediatric pineal region tumors.
- To identify trends and challenges in treatment.
Main Methods:
- Retrospective review of 37 patients under 16 years old.
- Analysis of tumor types, surgical interventions, and adjuvant therapies.
Main Results:
- Germinomas were the most common malignant type.
- Hydrocephalus was the most frequent presentation, requiring shunts in most patients.
- Survival rates varied by tumor type, with benign tumors faring best.
Conclusions:
- Significant improvements in pediatric pineal region tumor management observed.
- Radiotherapy complications were notable.
- Collaborative research is essential for optimizing treatment strategies.
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.
