Young age may predict a better outcome for children with diffuse pontine glioma

Alberto Broniscer, Fred H. Laningham, Robert P. Sanders, Larry E. Kun, David W. Ellison, Amar Gajjar

Research output: Contribution to journalArticle

38 Citations (Scopus)

Abstract

BACKGROUND. Because diffuse pontine glioma (DPG) is rare among young children, the outcome of affected patients is unknown. METHODS. The authors reviewed clinical and radiologic characteristics of all children aged <3 years with DPG who were evaluated at their institution. Inclusion followed standard magnetic resonance imaging criteria for the diagnosis of DPG. RESULTS. The median age at diagnosis in 10 patients was 2.2 years (range, 0.8-2.7 years). The median interval between the onset of symptoms and diagnosis was 2.5 months. All patients presented with cranial nerve palsy with (n = 7) or without (n = 3) other neurologic deficits attributable to brainstem involvement. All patients had pons-based tumors involving >50% of this brainstem segment. Histologic confirmation was attempted in 2 patients who had atypical radiologic features at diagnosis. Four patients initially were observed only. All patients received therapy, which consisted of radiation therapy (RT) (n = 2), RT and chemotherapy (n = 6), or chemotherapy only (n = 2). Four patients died of tumor progression after a median of 0.7 years (range, 0.5-3.7 years). Six patients have survived for a median of 2.3 years (range, 0.9-8 years). The 3-year progression-free and overall survival rates were 45% ± 19% and 69% ± 19%, respectively. CONCLUSIONS. Children aged <3 years with DPG potentially may fare better than older patients with the same diagnosis despite the use of similar therapy. The current results suggested that DPG in younger children may be distinct biologically from similar tumors in older age groups.

Original languageEnglish (US)
Pages (from-to)566-572
Number of pages7
JournalCancer
Volume113
Issue number3
DOIs
StatePublished - Aug 1 2008

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Glioma
Radiotherapy
Drug Therapy
Brain Stem
Disease-Free Survival
Neoplasms
Survival Rate
Age Groups
Therapeutics

Keywords

  • Brainstem
  • Children
  • Pons
  • Radiation therapy
  • Treatment

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Broniscer, A., Laningham, F. H., Sanders, R. P., Kun, L. E., Ellison, D. W., & Gajjar, A. (2008). Young age may predict a better outcome for children with diffuse pontine glioma. Cancer, 113(3), 566-572. https://doi.org/10.1002/cncr.23584

Young age may predict a better outcome for children with diffuse pontine glioma. / Broniscer, Alberto; Laningham, Fred H.; Sanders, Robert P.; Kun, Larry E.; Ellison, David W.; Gajjar, Amar.

In: Cancer, Vol. 113, No. 3, 01.08.2008, p. 566-572.

Research output: Contribution to journalArticle

Broniscer, A, Laningham, FH, Sanders, RP, Kun, LE, Ellison, DW & Gajjar, A 2008, 'Young age may predict a better outcome for children with diffuse pontine glioma', Cancer, vol. 113, no. 3, pp. 566-572. https://doi.org/10.1002/cncr.23584
Broniscer A, Laningham FH, Sanders RP, Kun LE, Ellison DW, Gajjar A. Young age may predict a better outcome for children with diffuse pontine glioma. Cancer. 2008 Aug 1;113(3):566-572. https://doi.org/10.1002/cncr.23584
Broniscer, Alberto ; Laningham, Fred H. ; Sanders, Robert P. ; Kun, Larry E. ; Ellison, David W. ; Gajjar, Amar. / Young age may predict a better outcome for children with diffuse pontine glioma. In: Cancer. 2008 ; Vol. 113, No. 3. pp. 566-572.
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AB - BACKGROUND. Because diffuse pontine glioma (DPG) is rare among young children, the outcome of affected patients is unknown. METHODS. The authors reviewed clinical and radiologic characteristics of all children aged <3 years with DPG who were evaluated at their institution. Inclusion followed standard magnetic resonance imaging criteria for the diagnosis of DPG. RESULTS. The median age at diagnosis in 10 patients was 2.2 years (range, 0.8-2.7 years). The median interval between the onset of symptoms and diagnosis was 2.5 months. All patients presented with cranial nerve palsy with (n = 7) or without (n = 3) other neurologic deficits attributable to brainstem involvement. All patients had pons-based tumors involving >50% of this brainstem segment. Histologic confirmation was attempted in 2 patients who had atypical radiologic features at diagnosis. Four patients initially were observed only. All patients received therapy, which consisted of radiation therapy (RT) (n = 2), RT and chemotherapy (n = 6), or chemotherapy only (n = 2). Four patients died of tumor progression after a median of 0.7 years (range, 0.5-3.7 years). Six patients have survived for a median of 2.3 years (range, 0.9-8 years). The 3-year progression-free and overall survival rates were 45% ± 19% and 69% ± 19%, respectively. CONCLUSIONS. Children aged <3 years with DPG potentially may fare better than older patients with the same diagnosis despite the use of similar therapy. The current results suggested that DPG in younger children may be distinct biologically from similar tumors in older age groups.

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