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

TitleEvaluating pediatric spinal low-grade gliomas: a 30-year retrospective analysis
Authors
KeywordsLow grade glioma
Pediatric
Spinal
Treatment
Biomarkers
Issue Date2019
PublisherSpringer New York LLC. The Journal's web site is located at https://www.springer.com/medicine/oncology/journal/11060
Citation
Journal of Neuro-Oncology, 2019, v. 145, p. 519-529 How to Cite?
AbstractPurpose: 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.
Descriptioneid_2-s2.0-85074016354
Persistent Identifierhttp://hdl.handle.net/10722/288133
ISSN
2019 Impact Factor: 3.267
2015 SCImago Journal Rankings: 1.274
PubMed Central ID
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorCarey, SS-
dc.contributor.authorSadighi, Z-
dc.contributor.authorWu, S-
dc.contributor.authorChiang, J-
dc.contributor.authorRobinson, GW-
dc.contributor.authorGhazwani, Y-
dc.contributor.authorLiu, APY-
dc.contributor.authorAcharya, S-
dc.contributor.authorMerchant, TE-
dc.contributor.authorBoop, FA-
dc.contributor.authorGajjar, A-
dc.contributor.authorQaddoumi, I-
dc.date.accessioned2020-10-05T12:08:21Z-
dc.date.available2020-10-05T12:08:21Z-
dc.date.issued2019-
dc.identifier.citationJournal of Neuro-Oncology, 2019, v. 145, p. 519-529-
dc.identifier.issn0167-594X-
dc.identifier.urihttp://hdl.handle.net/10722/288133-
dc.descriptioneid_2-s2.0-85074016354-
dc.description.abstractPurpose: 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. -
dc.languageeng-
dc.publisherSpringer New York LLC. The Journal's web site is located at https://www.springer.com/medicine/oncology/journal/11060-
dc.relation.ispartofJournal of Neuro-Oncology-
dc.rightsThis is a post-peer-review, pre-copyedit version of an article published in [insert journal title]. The final authenticated version is available online at: https://doi.org/[insert DOI]-
dc.subjectLow grade glioma-
dc.subjectPediatric-
dc.subjectSpinal-
dc.subjectTreatment-
dc.subjectBiomarkers-
dc.titleEvaluating pediatric spinal low-grade gliomas: a 30-year retrospective analysis-
dc.typeArticle-
dc.identifier.emailLiu, APY: apyliu@hku.hk-
dc.identifier.authorityLiu, APY=rp01357-
dc.description.naturelink_to_OA_fulltext-
dc.identifier.doi10.1007/s11060-019-03319-4-
dc.identifier.pmid31642023-
dc.identifier.pmcidPMC6913043-
dc.identifier.scopuseid_2-s2.0-85074016354-
dc.identifier.hkuros315668-
dc.identifier.volume145-
dc.identifier.spage519-
dc.identifier.epage529-
dc.identifier.isiWOS:000494209100002-
dc.publisher.placeUnited States-
dc.identifier.issnl0167-594X-

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