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Brain stem tumors in children and adolescents: single institutional experience

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Abstract

Purpose

Pediatric brain stem tumors (BsT) are a heterogeneous group of diseases. Our aim was to analyze our experience to find out prognostic factors.

Method

A retrospective study with BsT patients was performed. Imaging characteristics, extension of surgery, pathology, and adjuvant therapy were analyzed and correlated with overall survival (OS) and progression-free survival (PFS) as outcome measures.

Result

Since 1980 to 2010, we analyzed 65 BsT patients, 41 of them girls (63 %), median age of 8 years (range 13.9 months to 17.6 years). Twenty-two patients (33.8 %) had diffuse intrinsic pontine gliomas (DIPG) and 43 (66.2 %) presented with focal BsT. Histology was available in 42 patients; the most frequent is low-grade glioma in 24/42 patients (57 %). DIPG’s histology (obtained usually at necropsy) confirmed five high-grade gliomas. After median follow-up of 49.3 months (0.5–175 months), 20/22 DIPG patients have died (90.9 %), while 27/43 with focal tumors were alive (62.8 %). Variables related to outcome were histology (better for low-grade glioma (LGG) OS p < 0.001), surgery (better if operated OS p < 0.001), and adjuvant therapy (worse if given, PFS p = 0.001, OS p = 0.024). The outcome for DIPG was dismal, median OS/EFS of 14.2/9.4 months, significantly worse than focal BsT (p = 0.000), while OS/EFS was 122.8/87.2 months for focal intrinsic, 88.2/47.1 months for exophytic, and 124.4/54 months for cervico–medullary tumors: no differences were found among them, except the histology (OS p < 0.001 for low-grade vs high-grade tumors).

Conclusion

BsT in children comprised two different groups: diffuse (DIPG) and focal gliomas. The DIPGs continue having a dismal prognosis, needing new approaches, while focal tumors including LGG have better prognosis.

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Acknowledgments

We thank all patients and their families for their trust. We are grateful to our nursing and multidisciplinary staff for their support in patient care. We thank Raquel Iniesta for her support in Statistics (Fundació Sant Joan de Déu, working under financial support from the Instituto Carlos III, contract number CA08/00151) and Daniel Cuadras, Servei d’Assessoria en Estadística.

The authors also thank the institutional Biobank “Biobanco del Hospital Materno-Infantil de San Juan de Dios para la Investigación” as part of the “Red Temática de Biobancos Hospitalarios del ISCIII”.

Financial support

This study was partially supported by a grant from the Alicia Pueyo Fund (www.fondoaliciapueyo.org).

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Authors and Affiliations

  1. Department of Pediatric Neurosurgery, Hospital Sant Joan de Déu, University of Barcelona, Passeig St. Joan de Déu, 2, Esplugues de Llobregat, 08950, Barcelona, Spain

    Magda Garzón, Gemma García-Fructuoso & Antonio Guillén

  2. Department of Pathology, Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain

    Mariona Suñol

  3. Department of Oncology, Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain

    Jaume Mora & Ofelia Cruz

Authors
  1. Magda Garzón
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  2. Gemma García-Fructuoso
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  3. Antonio Guillén
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  4. Mariona Suñol
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  5. Jaume Mora
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  6. Ofelia Cruz
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Corresponding author

Correspondence to Magda Garzón.

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Garzón, M., García-Fructuoso, G., Guillén, A. et al. Brain stem tumors in children and adolescents: single institutional experience. Childs Nerv Syst 29, 1321–1331 (2013). https://doi.org/10.1007/s00381-013-2137-1

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  • DOI: https://doi.org/10.1007/s00381-013-2137-1

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