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

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.

SUBMITTER: Carey SS 

PROVIDER: S-EPMC6913043 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature