Management and outcome of focal low-grade brainstem tumors in pediatric patients: the St. Jude experience

Clinical article

View More View Less
  • 1 Semmes-Murphey Neurologic & Spine Institute;
  • | 2 Department of Neurosurgery, University of Tennessee Health Science Center;
  • | 3 Departments of Surgery,
  • | 4 Radiological Sciences,
  • | 5 Oncology,
  • | 6 Epidemiology and Cancer Control, and
  • | 7 Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee; and
  • | 8 School of Public Health and Health Services, The George Washington University, Washington, DC
Restricted access

Purchase Now

USD  $45.00

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $515.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $612.00
USD  $45.00
USD  $515.00
USD  $612.00
Print or Print + Online Sign in

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. Event-free 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.

Abbreviations used in this paper:

EFS = event-free survival; GTR = gross-total resection; NTR = near-total resection; STR = subtotal resection.

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $515.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $612.00
USD  $515.00
USD  $612.00
  • 1

    Barkovich AJ, , Krischer J, , Kun LE, , Packer R, , Zimmerman RA, & Freeman CR, et al.: Brain stem gliomas: a classification system based on magnetic resonance imaging. Pediatr Neurosurg 16:7383, 1991

    • Search Google Scholar
    • Export Citation
  • 2

    Boydston WR, , Sanford RA, , Muhlbauer MS, , Kun LE, , Kirk E, & Dohan FC Jr, et al.: Gliomas of the tectum and periaqueductal region of the mesencephalon. Pediatr Neurosurg 17:234238, 1992

    • Search Google Scholar
    • Export Citation
  • 3

    Bricolo A, , Turazzi S, , Cristofori L, & Talacchi A: Direct surgery for brainstem tumours. Acta Neurochir Suppl (Wien) 53:148158, 1991

  • 4

    Broadway SJ, , Ogg RJ, , Scoggins MA, , Sanford R, , Patay Z, & Boop FA: Surgical management of tumors producing the thalamopeduncular syndrome of childhood. Clinical article. J Neurosurg Pediatr 7:589595, 2011

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 5

    Choux M, , Lena G, & Do L, Brainstem tumors. Choux M, , Di Rocco C, & Hockley A, et al.: Pediatric Neurosurgery New York, Churchill Livingstone, 2000. 471491

    • Search Google Scholar
    • Export Citation
  • 6

    Dellaretti M, , Touzet G, , Reyns N, , Dubois F, , Gusmão S, & Pereira JL, et al.: Correlation among magnetic resonance imaging findings, prognostic factors for survival, and histological diagnosis of intrinsic brainstem lesions in children. Clinical article. J Neurosurg Pediatr 8:539543, 2011

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 7

    Donaldson SS, , Laningham F, & Fisher PG: Advances toward an understanding of brainstem gliomas. J Clin Oncol 24:12661272, 2006

  • 8

    Epstein F, & McCleary EL: Intrinsic brain-stem tumors of childhood: surgical indications. J Neurosurg 64:1115, 1986

  • 9

    Epstein F, & Wisoff JH: Intrinsic brainstem tumors in childhood: surgical indications. J Neurooncol 6:309317, 1988

  • 10

    Fangusaro J: Pediatric high-grade gliomas and diffuse intrinsic pontine gliomas. J Child Neurol 24:14091417, 2009

  • 11

    Farmer JP, , Montes JL, , Freeman CR, , Meagher-Villemure K, , Bond MC, & O'Gorman AM: Brainstem gliomas. A 10-year institutional review. Pediatr Neurosurg 34:206214, 2001

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 12

    Fisher PG, , Breiter SN, , Carson BS, , Wharam MD, , Williams JA, & Weingart JD, et al.: A clinicopathologic reappraisal of brain stem tumor classification. Identification of pilocystic astrocytoma and fibrillary astrocytoma as distinct entities. Cancer 89:15691576, 2000

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 13

    Fried I, , Hawkins C, , Scheinemann K, , Tsangaris E, , Hesselson L, & Bartels U, et al.: Favorable outcome with conservative treatment for children with low grade brainstem tumors. Pediatr Blood Cancer 58:556560, 2012

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 14

    Gonçalves-Ferreira AJ, , Herculano-Carvalho M, & Pimentel J: Stereotactic biopsies of focal brainstem lesions. Surg Neurol 60:311320, 2003

  • 15

    Jallo GI, , Biser-Rohrbaugh A, & Freed D: Brainstem gliomas. Childs Nerv Syst 20:143153, 2004

  • 16

    Kestle J, , Townsend JJ, , Brockmeyer DL, & Walker ML: Juvenile pilocytic astrocytoma of the brainstem in children. J Neurosurg 101:1 Suppl 16, 2004

    • Search Google Scholar
    • Export Citation
  • 17

    Kwon JW, , Kim IO, , Cheon JE, , Kim WS, , Moon SG, & Kim TJ, et al.: Paediatric brain-stem gliomas: MRI, FDG-PET and histological grading correlation. Pediatr Radiol 36:959964, 2006

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 18

    Lesniak MS, , Klem JM, , Weingart J, & Carson BS Sr: Surgical outcome following resection of contrast-enhanced pediatric brainstem gliomas. Pediatr Neurosurg 39:314322, 2003

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 19

    Liao CH, , Pan DH, , Yang HC, , Wu HM, , Ho DM, & Wong TT, et al.: Gamma Knife radiosurgery as a treatment modality for low-grade pediatric brainstem gliomas: report of two cases. Childs Nerv Syst 28:175178, 2012

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 20

    Mauffrey C: Paediatric brainstem gliomas: prognostic factors and management. J Clin Neurosci 13:431437, 2006

  • 21

    Mehta VS, , Chandra PS, , Singh PK, , Garg A, & Rath GK: Surgical considerations for ‘intrinsic’ brainstem gliomas: proposal of a modification in classification. Neurol India 57:274281, 2009

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 22

    Pérez-Gómez JL, , Rodríguez-Alvarez CA, , Marhx-Bracho A, & Rueda-Franco F: Stereotactic biopsy for brainstem tumors in pediatric patients. Childs Nerv Syst 26:2934, 2010

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 23

    Recinos PF, , Sciubba DM, & Jallo GI: Brainstem tumors: where are we today?. Pediatr Neurosurg 43:192201, 2007

  • 24

    Sandri A, , Sardi N, , Genitori L, , Giordano F, , Peretta P, & Basso ME, et al.: Diffuse and focal brain stem tumors in childhood: prognostic factors and surgical outcome. Experience in a single institution. Childs Nerv Syst 22:11271135, 2006

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 25

    Schild SE, , Stafford SL, , Brown PD, , Wood CP, , Scheithauer BW, & Schomberg PJ, et al.: The results of radiotherapy for brainstem tumors. J Neurooncol 40:171177, 1998

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 26

    Stroink AR, , Hoffman HJ, , Hendrick EB, & Humphreys RP: Diagnosis and management of pediatric brain-stem gliomas. J Neurosurg 65:745750, 1986

  • 27

    Teo C, & Siu TL: Radical resection of focal brainstem gliomas: is it worth doing?. Childs Nerv Syst 24:13071314, 2008

  • 28

    Ueoka DI, , Nogueira J, , Campos JC, , Maranhão Filho P, , Ferman S, & Lima MA: Brainstem gliomas—retrospective analysis of 86 patients. J Neurol Sci 281:2023, 2009

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 29

    Weiner HL, , Freed D, , Woo HH, , Rezai AR, , Kim R, & Epstein FJ: Intra-axial tumors of the cervicomedullary junction: surgical results and long-term outcome. Pediatr Neurosurg 27:1218, 1997

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 30

    Yen CP, , Sheehan J, , Steiner M, , Patterson G, & Steiner L: Gamma knife surgery for focal brainstem gliomas. J Neurosurg 106:817, 2007

Metrics

All Time Past Year Past 30 Days
Abstract Views 803 153 0
Full Text Views 204 33 10
PDF Downloads 229 36 5
EPUB Downloads 0 0 0