Management and outcome of focal low-grade brainstem tumors in pediatric patients

The St. Jude experience

Paul Klimo, Atmaram S.Pai Panandiker, Clinton J. Thompson, Frederick Boop, Ibrahim Qaddoumi, Amar Gajjar, Gregory Armstrong, David W. Ellison, Larry E. Kun, Robert J. Ogg, Robert A. Sanford

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Object. Whereas diffuse intrinsic pontine gliomas generally have a short symptom duration and more cranial nerve involvement, focal brainstem gliomas are commonly low grade, with fewer cranial neuropathies. Although these phenotypic distinctions are not absolute predictors of outcome, they do demonstrate correlation in most cases. Because there is a limited literature on focal brainstem gliomas in pediatric patients, the objective of this paper was to report the management and outcome of these tumors. Methods. The authors reviewed the records of all children diagnosed with radiographically confirmed low-grade focal brainstem gliomas from 1986 to 2010. Each patient underwent biopsy or resection for tissue diagnosis. Eventfree survival (EFS) and overall survival were evaluated. Univariate analysis was conducted to identify demographic and treatment variables that may affect EFS. Results. Fifty-two patients (20 girls, 32 boys) with follow-up data were identified. Median follow-up was 10.0 years, and the median age at diagnosis was 6.5 years (range 1-17 years). The tumor locations were midbrain (n = 22, 42%), pons (n = 15, 29%), and medulla (n = 15, 29%). Surgical extirpation was the primary treatment in 25 patients (48%). The 5- and 10-year EFS and overall survival were 59%/98% and 52%/90%, respectively. An event or treatment failure occurred in 24 patients (46%), including 5 deaths. Median time to treatment failure was 3.4 years. Disease progression in the other 19 patients transpired within 25.1 months of diagnosis. Thirteen of these patients received radiation, including 11 within 2 months of primary treatment failure. Although children with intrinsic tumors had slightly better EFS at 5 years compared with those with exophytic tumors (p = 0.054), this difference was not significant at 10 years (p = 0.147). No other variables were predictive of EFS. Conclusions. Surgery suffices in many children with low-grade focal brainstem gliomas. Radiation treatment is often reserved for disease progression but offers comparable disease control following biopsy. In the authors' experience, combining an assessment of clinical course, imaging, and tumor biopsy yields a reasonable model for managing children with focal brainstem tumors.

Original languageEnglish (US)
Pages (from-to)274-281
Number of pages8
JournalJournal of Neurosurgery: Pediatrics
Volume11
Issue number3
DOIs
StatePublished - Mar 1 2013

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Brain Stem Neoplasms
Pediatrics
Glioma
Survival
Brain Stem
Treatment Failure
Neoplasms
Biopsy
Disease Progression
Radiation
Cranial Nerve Diseases
Pons
Cranial Nerves
Mesencephalon
Therapeutics
Demography

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Management and outcome of focal low-grade brainstem tumors in pediatric patients : The St. Jude experience. / Klimo, Paul; Panandiker, Atmaram S.Pai; Thompson, Clinton J.; Boop, Frederick; Qaddoumi, Ibrahim; Gajjar, Amar; Armstrong, Gregory; Ellison, David W.; Kun, Larry E.; Ogg, Robert J.; Sanford, Robert A.

In: Journal of Neurosurgery: Pediatrics, Vol. 11, No. 3, 01.03.2013, p. 274-281.

Research output: Contribution to journalArticle

Klimo, P, Panandiker, ASP, Thompson, CJ, Boop, F, Qaddoumi, I, Gajjar, A, Armstrong, G, Ellison, DW, Kun, LE, Ogg, RJ & Sanford, RA 2013, 'Management and outcome of focal low-grade brainstem tumors in pediatric patients: The St. Jude experience', Journal of Neurosurgery: Pediatrics, vol. 11, no. 3, pp. 274-281. https://doi.org/10.3171/2012.11.PEDS12317
Klimo, Paul ; Panandiker, Atmaram S.Pai ; Thompson, Clinton J. ; Boop, Frederick ; Qaddoumi, Ibrahim ; Gajjar, Amar ; Armstrong, Gregory ; Ellison, David W. ; Kun, Larry E. ; Ogg, Robert J. ; Sanford, Robert A. / Management and outcome of focal low-grade brainstem tumors in pediatric patients : The St. Jude experience. In: Journal of Neurosurgery: Pediatrics. 2013 ; Vol. 11, No. 3. pp. 274-281.
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abstract = "Object. Whereas diffuse intrinsic pontine gliomas generally have a short symptom duration and more cranial nerve involvement, focal brainstem gliomas are commonly low grade, with fewer cranial neuropathies. Although these phenotypic distinctions are not absolute predictors of outcome, they do demonstrate correlation in most cases. Because there is a limited literature on focal brainstem gliomas in pediatric patients, the objective of this paper was to report the management and outcome of these tumors. Methods. The authors reviewed the records of all children diagnosed with radiographically confirmed low-grade focal brainstem gliomas from 1986 to 2010. Each patient underwent biopsy or resection for tissue diagnosis. Eventfree survival (EFS) and overall survival were evaluated. Univariate analysis was conducted to identify demographic and treatment variables that may affect EFS. Results. Fifty-two patients (20 girls, 32 boys) with follow-up data were identified. Median follow-up was 10.0 years, and the median age at diagnosis was 6.5 years (range 1-17 years). The tumor locations were midbrain (n = 22, 42{\%}), pons (n = 15, 29{\%}), and medulla (n = 15, 29{\%}). Surgical extirpation was the primary treatment in 25 patients (48{\%}). The 5- and 10-year EFS and overall survival were 59{\%}/98{\%} and 52{\%}/90{\%}, respectively. An event or treatment failure occurred in 24 patients (46{\%}), including 5 deaths. Median time to treatment failure was 3.4 years. Disease progression in the other 19 patients transpired within 25.1 months of diagnosis. Thirteen of these patients received radiation, including 11 within 2 months of primary treatment failure. Although children with intrinsic tumors had slightly better EFS at 5 years compared with those with exophytic tumors (p = 0.054), this difference was not significant at 10 years (p = 0.147). No other variables were predictive of EFS. Conclusions. Surgery suffices in many children with low-grade focal brainstem gliomas. Radiation treatment is often reserved for disease progression but offers comparable disease control following biopsy. In the authors' experience, combining an assessment of clinical course, imaging, and tumor biopsy yields a reasonable model for managing children with focal brainstem tumors.",
author = "Paul Klimo and Panandiker, {Atmaram S.Pai} and Thompson, {Clinton J.} and Frederick Boop and Ibrahim Qaddoumi and Amar Gajjar and Gregory Armstrong and Ellison, {David W.} and Kun, {Larry E.} and Ogg, {Robert J.} and Sanford, {Robert A.}",
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T1 - Management and outcome of focal low-grade brainstem tumors in pediatric patients

T2 - The St. Jude experience

AU - Klimo, Paul

AU - Panandiker, Atmaram S.Pai

AU - Thompson, Clinton J.

AU - Boop, Frederick

AU - Qaddoumi, Ibrahim

AU - Gajjar, Amar

AU - Armstrong, Gregory

AU - Ellison, David W.

AU - Kun, Larry E.

AU - Ogg, Robert J.

AU - Sanford, Robert A.

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N2 - Object. Whereas diffuse intrinsic pontine gliomas generally have a short symptom duration and more cranial nerve involvement, focal brainstem gliomas are commonly low grade, with fewer cranial neuropathies. Although these phenotypic distinctions are not absolute predictors of outcome, they do demonstrate correlation in most cases. Because there is a limited literature on focal brainstem gliomas in pediatric patients, the objective of this paper was to report the management and outcome of these tumors. Methods. The authors reviewed the records of all children diagnosed with radiographically confirmed low-grade focal brainstem gliomas from 1986 to 2010. Each patient underwent biopsy or resection for tissue diagnosis. Eventfree survival (EFS) and overall survival were evaluated. Univariate analysis was conducted to identify demographic and treatment variables that may affect EFS. Results. Fifty-two patients (20 girls, 32 boys) with follow-up data were identified. Median follow-up was 10.0 years, and the median age at diagnosis was 6.5 years (range 1-17 years). The tumor locations were midbrain (n = 22, 42%), pons (n = 15, 29%), and medulla (n = 15, 29%). Surgical extirpation was the primary treatment in 25 patients (48%). The 5- and 10-year EFS and overall survival were 59%/98% and 52%/90%, respectively. An event or treatment failure occurred in 24 patients (46%), including 5 deaths. Median time to treatment failure was 3.4 years. Disease progression in the other 19 patients transpired within 25.1 months of diagnosis. Thirteen of these patients received radiation, including 11 within 2 months of primary treatment failure. Although children with intrinsic tumors had slightly better EFS at 5 years compared with those with exophytic tumors (p = 0.054), this difference was not significant at 10 years (p = 0.147). No other variables were predictive of EFS. Conclusions. Surgery suffices in many children with low-grade focal brainstem gliomas. Radiation treatment is often reserved for disease progression but offers comparable disease control following biopsy. In the authors' experience, combining an assessment of clinical course, imaging, and tumor biopsy yields a reasonable model for managing children with focal brainstem tumors.

AB - Object. Whereas diffuse intrinsic pontine gliomas generally have a short symptom duration and more cranial nerve involvement, focal brainstem gliomas are commonly low grade, with fewer cranial neuropathies. Although these phenotypic distinctions are not absolute predictors of outcome, they do demonstrate correlation in most cases. Because there is a limited literature on focal brainstem gliomas in pediatric patients, the objective of this paper was to report the management and outcome of these tumors. Methods. The authors reviewed the records of all children diagnosed with radiographically confirmed low-grade focal brainstem gliomas from 1986 to 2010. Each patient underwent biopsy or resection for tissue diagnosis. Eventfree survival (EFS) and overall survival were evaluated. Univariate analysis was conducted to identify demographic and treatment variables that may affect EFS. Results. Fifty-two patients (20 girls, 32 boys) with follow-up data were identified. Median follow-up was 10.0 years, and the median age at diagnosis was 6.5 years (range 1-17 years). The tumor locations were midbrain (n = 22, 42%), pons (n = 15, 29%), and medulla (n = 15, 29%). Surgical extirpation was the primary treatment in 25 patients (48%). The 5- and 10-year EFS and overall survival were 59%/98% and 52%/90%, respectively. An event or treatment failure occurred in 24 patients (46%), including 5 deaths. Median time to treatment failure was 3.4 years. Disease progression in the other 19 patients transpired within 25.1 months of diagnosis. Thirteen of these patients received radiation, including 11 within 2 months of primary treatment failure. Although children with intrinsic tumors had slightly better EFS at 5 years compared with those with exophytic tumors (p = 0.054), this difference was not significant at 10 years (p = 0.147). No other variables were predictive of EFS. Conclusions. Surgery suffices in many children with low-grade focal brainstem gliomas. Radiation treatment is often reserved for disease progression but offers comparable disease control following biopsy. In the authors' experience, combining an assessment of clinical course, imaging, and tumor biopsy yields a reasonable model for managing children with focal brainstem tumors.

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