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. 2019 Dec;145(3):519-529.
doi: 10.1007/s11060-019-03319-4. Epub 2019 Oct 22.

Evaluating pediatric spinal low-grade gliomas: a 30-year retrospective analysis

Steven S Carey  1 , Zsila Sadighi  2 , Shengjie Wu  3 , Jason Chiang  4 , Giles W Robinson  5 , Yahya Ghazwani  5 , Anthony P Y Liu  5 , Sahaja Acharya  6 , Thomas E Merchant  6 , Frederick A Boop  7   8   9   10 , Amar Gajjar  5 , Ibrahim Qaddoumi  5
Affiliations

Evaluating pediatric spinal low-grade gliomas: a 30-year retrospective analysis

Steven S Carey et al. J Neurooncol. 2019 Dec.

Abstract

Purpose: Most pediatric spinal tumors are low-grade gliomas (LGGs). Characterization of these tumors has been difficult given their heterogeneity and rare incidence. The objective was to characterize such tumors diagnosed at our institution.

Methods: Spinal tumors diagnosed in our pediatric patients between 1984 and 2014 were reviewed retrospectively. Demographics, presentation, pathology, imaging, management, and sequelae were examined.

Results: Forty patients had spinal LGG tumors, 24 (62%) of which were pilocytic astrocytomas. The most common initial presentations were pain (n = 15), partial extremity paralysis (n = 13), and ataxia (n = 11), with the diagnosis frequently delayed by months (median = 5.9 months, range 4 days-6.2 years). Twenty-nine patients had some tumor resection, and 8 required adjuvant therapy with chemotherapy (n = 4) or radiation (n = 4) post-resection. Ten other patients received only biopsy for histologic diagnosis, who were treated with chemotherapy (n = 4) or radiation (n = 5) post biopsy. Tumor progression was noted in 16 patients (2 after gross-total resection; 10, partial resection; and 4, biopsy). During the evaluation period, 3 patients died secondary to tumor progression. BRAF status could have shortened progression-free survival: patients with BRAFV600E mutations (n = 3) all experienced progression within 10 months. Long-term sequelae of the disease/treatment were mostly residual neurologic deficits (paresthesia, paralysis), chemotherapy-induced hearing loss, and scoliosis.

Conclusions: Spinal LGG is a rare entity with significant long-term effects. Although surgery is the most common initial treatment option, more in-depth analysis of molecular biomarkers may improve stratification and prognostication.

Keywords: BRAF; Biomarkers; Low grade glioma; Pediatric; Spinal; Treatment.

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Conflict of interest statement

Compliance with Ethical Standards

Conflict of Interest:

All authors report no conflict of interest.

Figures

Fig.1
Fig.1
Survival rates in patient cohort, OS overall survival, PFS progression-free survival, RFS radiation-free survival
Fig.2
Fig.2
The effect of (a) BRAF mutation status and (b) gross-total resection on progression-free survival. V600E BRAF V600E mutation, GTR gross-total resection, PFS progression-free survival
See this image and copyright information in PMC

References

    1. Ostrom QT, Gittleman H, Xu J et al. (2016) CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2009–2013. Neuro Oncol 18 (suppl_5):v1–v75. doi: 10.1093/neuonc/now207 - DOI - PMC - PubMed
    1. Qaddoumi I, Sultan I, Gajjar A (2009) Outcome and prognostic features in pediatric gliomas: a review of 6212 cases from the Surveillance, Epidemiology, and End Results database. Cancer 115 (24):5761–5770. doi: 10.1002/cncr.24663 - DOI - PMC - PubMed
    1. Ahmed R, Menezes AH, Awe OO et al. (2014) Long-term incidence and risk factors for development of spinal deformity following resection of pediatric intramedullary spinal cord tumors. J Neurosurg Pediatr 13 (6):613–621. doi: 10.3171/2014.1.PEDS13317 - DOI - PubMed
    1. Schneider C, Hidalgo ET, Schmitt-Mechelke T et al. (2014) Quality of life after surgical treatment of primary intramedullary spinal cord tumors in children. J Neurosurg Pediatr 13 (2):170–177. doi: 10.3171/2013.11.PEDS13346 - DOI - PubMed
    1. Arnautovic A, Billups C, Broniscer A et al. (2015) Delayed diagnosis of childhood low-grade glioma: causes, consequences, and potential solutions. Childs Nerv Syst 31 (7):1067–1077. doi: 10.1007/s00381-015-2670-1 - DOI - PMC - PubMed