Pediatric low grade focal brainstem glioma: outcomes of different treatment strategies and prognostic factors
Abstract
Background: This study explores the prognostic factors and outcomes of different treatment modalities in focal brain stem glioma (FBSG). Materials & methods: Pediatric FBSG patients diagnosed during 2010–2017 were retrospectively reviewed for clinical and therapeutic data. Results: A total of 71 cases were identified and the median age was 6.4 years. The 5-year overall- and progression-free survival were 74.5 and 70.6%, respectively. Radiotherapy was the main line of treatment (66.2%) and there were no survival differences between radiotherapy, chemotherapy and surveillance groups. Two independent poor prognostic factors were identified on multivariate analysis: age <8 years and cervicomedullary tumor site (p = 0.02 for both). Conclusion: Surveillance, radiotherapy and chemotherapy have comparable clinical outcomes in pediatric FBSG.
Papers of special note have been highlighted as: • of interest
References
- 1. . Global incidence of malignant brain and other central nervous system tumors by histology, 2003–2007. Neuro Oncol. 19(11), 1553–1564 (2017).
- 2. . Pediatric brainstem gliomas: new understanding leads to potential new treatments for two very different tumors. Curr. Oncol. Rep. 17(3), 12 (2015).
- 3. A clinicopathologic reappraisal of brain stem tumor classification: identification of pilocytic astrocytoma and fibrillary astrocytoma as distinct entities. Cancer 89(7), 1569–1576 (2000).
- 4. . Brainstem tumors. In: Pediatric Neurosurgery. Di Rocco CHockley AChoux M (Eds). Churchill Livingstone, NY, USA, 471–491 (2000).
- 5. Surgical approaches for brainstem tumors in pediatric patients. Childs Nerv. Syst. 31(10), 1815–1840 (2015).
- 6. . Brainstem glioma: a review. Curr. Neurol. Neurosci. Rep. 13(5), 346 (2013).
- 7. . Comparison of electronic portal imaging and cone beam computed tomography for position verification in children. Clin. Oncol. 22(10), 850–861 (2010).
- 8. US Department of Health and Human Services. Common Terminology Criteria for Adverse Events (CTCAE) (2009). https://ctep.cancer.gov/protocolDevelopment/electronic_applications/ctc.htm#ctc_40
- 9. Management and outcome of focal low-grade brainstem tumors in pediatric patients: the St. Jude experience. J. Neurosurg. Pediatr. 11(3), 274–281 (2013). • A valuable experience from Saint Jude analyzing the prognostic variables and the outcomes of 52 children with low grade focal brain stem glioma.
- 10. Favorable outcome with conservative treatment for children with low grade brainstem tumors. Pediatr. Blood Cancer 58(4), 556–560 (2012). • The analysis of 96 cases of low grade FBSG cases in the Sick Children Hospital encourages the initial observation and delay of chemotherapy or radiotherapy until progression.
- 11. Diffuse and focal brain stem tumors in childhood: prognostic factors and surgical outcome. Childs Nerv. Syst. 22(9), 1127–1135 (2006).
- 12. . Brain stem tumors in children and adolescents: single institutional experience. Childs Nerv. Syst. 29(8), 1321–1331 (2013). • From the few articles who added the tumor site within the brain stem and their types, either intrinsic or exophytic, in the prognostic variable analysis.
- 13. Brainstem low-grade gliomas in children – excellent outcomes with multimodality therapy. J. Child Neurol. 32(2), 194–203 (2017).
- 14. Randomized study of two chemotherapy regimens for treatment of low-grade glioma in young children: a report from the Children's Oncology Group. J. Clin. Oncol. 30(21), 2641 (2012).
- 15. Vincristine and carboplatin chemotherapy for unresectable and/or recurrent low-grade astrocytoma of the brainstem. Pediatr. Blood Cancer 55(3), 471–477 (2010).
- 16. . New strategies in pediatric gliomas: molecular advances in pediatric low-grade gliomas as a model. Clin. Cancer Res. 19(17), 4553–4558 (2013).
- 17. Long-term follow-up of the multicenter, multidisciplinary treatment study HIT-LGG-1996 for low-grade glioma in children and adolescents of the German Speaking Society of Pediatric Oncology and Hematology. Neuro Oncol. 14(10), 1265–1284 (2012). • A large multicenteric trial discussing the management of pediatric low grade glioma.
- 18. SIOP PODC Adapted treatment guidelines for low grade gliomas in low and middle income settings. Pediatr Blood Cancer. 64(Suppl. 5), (2017).
- 19. Long-term outcomes among adult survivors of childhood central nervous system malignancies in the Childhood Cancer Survivor Study. J Natl Cancer Inst. 101(13), 946–958 (2009).
- 20. . Focal brain-stem astrocytomas causing symptoms of involvement of the facial nerve nucleus: long-term survival in six pediatric cases. J. Neurosurg. 80(1), 20–25 (1994).
- 21. . Long term survivors of childhood brain stem gliomas treated with hyperfractionated radiotherapy: clinical characteristics and treatment related toxicities. Cancer 77(3), 555–562 (1996).
- 22. . Phase II trial of conformal radiation therapy for pediatric low-grade glioma. J. Clin. Oncol. 27(22), 3598 (2009).
- 23. Cognitive outcomes among survivors of focal low-grade brainstem tumors diagnosed in childhood. J. Neurooncol. 129(2), 311–317 (2016).
- 24. Conformal radiation therapy for pediatric patients with low-grade glioma: results from the Children's Oncology Group Phase II Study ACNS0221. Int. J. Radiat. Oncol. Biol. Phys. 103(4), 861–868 (2019). • A recent Children’s Oncology Group trial discussing the clinical target volume delineation in low-grade glioma. It concluded that 5mm clinical target volume is acceptable and does not lead to a high rate of marginal relapse.
- 25. A multivariate analysis of factors determining tumor progression in childhood low-grade glioma: a population-based cohort study (CCLG CNS9702). Neuro Oncol. 12(12), 1257–1268 (2010).
- 26. Outcome analysis of childhood low-grade astrocytomas. Pediatr. Blood Cancer 51(2), 245–250 (2008).
- 27. Cervicomedullary tumors in children. J. Neurosurg. Pediatr. 16(4), 357–366 (2015). • A unique retrospective review of 31 case of cevrvicomedullary tumor, discussing their management and outcome.
- 28. . Long-term disease and neurological outcomes in patients with pediatric intramedullary spinal cord tumors. J. Neurosurg. Pediatr. 13(6), 600–612 (2014).