Intracranial Metastases Originating From Pediatric Primary Spinal Cord Glioblastoma Multiforme: A Case Report and Literature Review

Abstract

Primary spinal cord glioblastoma multiforme (scGBM) is an uncommon entity in pediatrics, and intracranial metastasis originating in spinal cord gliomas is very rare. A 7-year-old female presented with weakness in the limbs, paralysis of the lower limbs and incontinence. The initial MRI of the spinal cord revealed expansion and abnormal signals from T2 to T5. She was initially diagnosed with Neuromyelitis optica spectrum disorders and treated with high-dose glucocorticoid and gamma globulin. Four months later, her symptoms worsened and follow-up imaging showed multiple intracranial mass lesions. We performed a subtotal resection of the right thalamic basal ganglia tumor and gross total resection of the right frontal lobe tumor under microscopic examination. Histopathology revealed scGBM with intracranial metastasis and the molecular pathology diagnosis suggested H3K27M mutant diffuse midline glioma WHO grade IV, which had previously been misdiagnosed as a Neuromyelitis optica spectrum disorders. We review the literature of intracranial metastases originating from pediatric primary spinal cord glioblastoma multiforme and summarize possible methods of differentiation, including changes in muscle strength or tone, intramedullary heterogeneously enhancing solitary mass lesions and cord expansion in MRI. Finally, we emphasize that in unexpected radiological changes or disadvantageous response to the treatment, a biopsy to achieve a pathological diagnosis is necessary to discard other diseases, especially neoplasms.

Magnetic Resonance Imaging Characteristics of Molecular Subgroups in Pediatric H3 K27M Mutant Diffuse Midline Glioma

“…Few studies show single MRI cases of spinal H3 K27M mutant DMG in different age groups, not differentiating between H3.1 and H3.3 subgroups [24,31,[36][37][38][39]. With our study, we, for the first time, systematically analyzed MRI parameters of spinal DMG in the pediatric population with respect to H3 K27 mutation status but there was no H3.1 mutant spinal tumor in our cohort.…”

Pediatric spinal cord diffuse midline glioma, H3 K27-altered with intracranial and spinal leptomeningeal spread: A case report

“…Spinal cord DMG, H3 K27-altered is a very rare entity in children. 5,[7][8][9][10][11][12][13][14][15][16] In the past decades, there have been several changes in the structuring and nomenclature of (pediatric) high-grade gliomas, reflecting the incorporation of molecular features and its advances into integrated tumor diagnoses with combined histopathological and molecular characteristics. 6,18,19 Histologically GBMs are defined as malignant infiltrating astrocytomas with necrosis and/or microvascular proliferation.…”

“…22 However, since neuroimaging and symptoms may be non-specific, a biopsy or surgical sampling should be performed to provide the final diagnosis. 5 Pediatric spinal cord DMG, H3 K27-altered has an aggressive course with poor prognosis; median survival time is about 11 months. 15 To date, there is no consensus or standard of care treatment.…”

“…Glioblastoma (GBM) is a very rare entity in children. [1][2][3][4][5] Compared to adults, less than 3% occur in the pediatric population. 1 Although high-grade gliomas (CNS WHO grade 3 and 4) are the most common class of central nervous system malignancies in pediatric patients, high-grade gliomas histologically defined as GBM account for only up to 4.6% of all primary pediatric tumors of the central nervous system.…”

“…6 Due to its rarity, there is still a limited though growing corpus of literature dealing with pediatric spinal cord DMG, H3 K27altered. 5,[7][8][9][10][11][12][13][14][15][16] The aim of this case report is to present a 15year-old female with histopathologically confirmed spinal cord GBM with molecular findings of H3F3A K27 M mutation consistent with a DMG, H3 K27-altered.…”