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

Autor: Carey SS; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA. steven.carey@stjude.org., Sadighi Z; Department of Neurology, St. Jude Children's Research Hospital, Memphis, TN, USA., Wu S; Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, TN, USA., Chiang J; Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA., Robinson GW; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA., Ghazwani Y; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA., Liu APY; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA., Acharya S; Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA., Merchant TE; Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA., Boop FA; Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA.; Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, TN, USA.; Le Bonheur Neuroscience Institute, Le Bonheur Children's Hospital, Memphis, TN, USA.; Semmes Murphey Clinic, Memphis, TN, USA., Gajjar A; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA., Qaddoumi I; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA.
Jazyk: angličtina
Zdroj: Journal of neuro-oncology [J Neurooncol] 2019 Dec; Vol. 145 (3), pp. 519-529. Date of Electronic Publication: 2019 Oct 22.
DOI: 10.1007/s11060-019-03319-4
Abstrakt: 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.
Databáze: MEDLINE
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