More than 70% of children with tumors of the central nervous system (CNS) will survive at least 5 years from diagnosis (Smith, 2010; Howlader, 2011).
However... the dismal survival of select tumor subtypes, such as diffuse intrinsic pontine gliomas (DIPG), is not reflected in this number (Warren, 2012). Here we go again with the creative math. What kind of accounting is that?
The median survival for children with DIPG is less than 1 year from diagnosis (Mandell, 1999; Cohen, 2011), and no improvement in survival has been realized in more than three decades (Warren, 2012). The reason for this stagnancy has, at least in part, been attributed to our lack of understanding of the biology of this disease. What is the other part, no financial incentive perhaps?
MIND YOU: Diffuse intrinsic pontine gliomas are the most common brainstem tumors in children, representing 75–80% of pediatric brainstem tumors.
In the past few years, considerable coordinated and collaborative efforts have been made to address this. Notably, more has been published on the biology and pathophysiology of DIPG in the past 5 years than in all prior years combined, generating a groundswell of excitement and cautious enthusiasm. This is truly great.
How to best apply this data to the treatment of children with DIPG remains to be seen, but improved outcome for these patients is anticipated as we move beyond empiric therapy and attempt to bridge the gap between bench and bedside (Warren, 2012). Let’s not make it another several decades, please.
Smith, M., Seibel, N., Altekruse, S., Ries, L., Melbert, D., O’Leary, M., et al. (2010). Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J. Clin. Oncol. 28, 2625–2634.
Howlader, N., Noone, A., Krapcho, M., Neyman, N., Aminou, R., Waldron, W., et al. (2011). SEER Cancer Statistics Review, 1975–2008. Bethesda, MD: National Cancer Institute.
Warren, K. E. (2012). Diffuse intrinsic pontine glioma: poised for progress. Frontiers in Oncology, 2, 205.
Mandell, L., Kadota, R., Freeman, C., Douglass, E., Fontanesi, J., Cohen, M., et al. (1999). There is no role for hyperfractionated radiotherapy in the management of children with newly diagnosed diffuse intrinsic brainstem tumors: results of a Pediatric Oncology Group phase III trial comparing conventional vs. hyperfractionated radiotherapy. Int. J. Radiat. Oncol. Biol. Phys. 43, 959–964
Cohen, K., Heideman, R., Zhou, T., Holmes, E., Lavey, R., Bouffet, E., et al. (2011). Temozolomide in the treatment of children with newly diagnosed diffuse intrinsic pontine gliomas: a report from the Children’s Oncology Group. Neuro Oncol. 13, 410–416
