Background: Primary intramedullary glioblastoma (scGBM), is exceedingly rare in children, accounting for

1.5% of pediatric spinal cord tumors. These highly aggressive neoplasms are associated with rapid neurological

decline and dismal prognosis.

Case presentation: We report a 12‑year‑old boy with progressive tetraparesis, headaches, and cervical pain.

MRI demonstrated a cervico-medullary intramedullary mass extending to C7 with heterogeneous enhancement

management.

Primary spinal cord glioblastoma (scGBM) in children represents a rare diagnostic and therapeutic challenge,

accounting for only ~1.5% of pediatric spinal cord tumors. Prognosis is poor, with median survival typically

below 14 months despite aggressive treatment. The introduction of the 2021 WHO classification and increasing

emphasis on molecular profiling have reshaped the understanding of these tumors, yet management remains

extrapolated from intracranial glioblastoma protocols or limited pediatric series. We present a fulminant pediatric

case and discuss diagnostic, therapeutic, and prognostic considerations in the context of recent literature.

A 12‑year‑old boy with no prior medical history presented with two months of headaches, cervical pain, and

was given. Postoperatively, the patient developed progressive respiratory and autonomic dysfunction and died

four months after diagnosis.

of surgical resection. Younger patients and those undergoing maximal safe resection tend to achieve superior

outcomes [3,4]. These findings emphasize the need for early recognition and aggressive, multimodal treatment

strategies.

MRI with gadolinium remains the diagnostic cornerstone, typically revealing T1 hypointensity, T2

hyperintensity, and heterogeneous enhancement with necrotic or hemorrhagic components [5]. Leptomeningeal

dissemination is present in up to 40% of cases, mandating neuraxis-wide MRI and, when feasible, cerebrospinal

fluid (CSF) cytology [5]. Our case lacked craniospinal staging, representing a limitation.

Histomolecular profiling is increasingly recognized as indispensable. Many pediatric scGBMs harbor H3 K27

alterations, categorizing them as diffuse midline gliomas (DMG) with uniformly poor prognosis [6]. Additional

function. However, true gross total resection is achieved in a minority of cases due to the infiltrative nature of

scGBM and eloquent tumor location. Intraoperative neurophysiological monitoring is recommended to minimize

morbidity [9,10].

Adjuvant radiotherapy represents the cornerstone of postoperative management and has been shown to

significantly prolong survival compared with surgery alone. Conformal techniques, including intensity-

modulated radiotherapy (IMRT) and proton beam therapy, allow delivery of adequate tumoricidal doses within

spinal cord tolerance [11]. Craniospinal irradiation is considered in cases with leptomeningeal dissemination

[12].

The Stupp regimen—radiotherapy with concomitant temozolomide followed by adjuvant temozolomide—

remains the most widely adopted systemic therapy, though pediatric-specific evidence is limited [13]. Recent

treated without adjuvant therapy, experienced rapid decline and represents the poorest-prognosis subgroup,

aligning with the literature.

In addition to disease-directed therapies, postoperative supportive management is critical—particularly for

cervicomedullary lesions with high risk of respiratory failure. Evidence supports proactive strategies including

close respiratory monitoring, early tracheostomy when indicated, intensive physiotherapy, nutritional support,

and prevention of infectious complications [5,10]. Such measures may improve short-term survival and quality

of life. The rapid respiratory deterioration observed in our case highlights the importance of early

multidisciplinary supportive interventions.

Recent systematic reviews and meta-analyses [1–3] reinforce several recurring themes: the extreme rarity and

aggressiveness of pediatric scGBM, survival advantage with radiotherapy and combined chemoradiation, strong

management emphasizes histomolecular diagnosis and multimodal therapy, though survival benefits remain

modest. Future progress depends on multicenter registries, prospective pediatric trials, and integration of targeted

and immunotherapeutic approaches.

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2. Guerra PLN, et al. Epidemiological and clinical characteristics of primary spinal cord glioblastomas: a

systematic review and meta-analysis. J Clin Neurosci. 2024; 130:110862.

3. Pfaff E, et al. Pediatric-type high-grade glioma of the spinal cord in the molecular era: institutional series and

updated systematic review. Neurooncol Adv. 2025;7(1): vdae185.

7. Vento-Tormo R, et al. Molecular and pathological features of pediatric high-grade glioma. Front Oncol.

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8. Wang Q, et al. Advances in targeted therapy for spinal GBM. Cancers (Basel). 2024;16(16):2781.

9. Reinhardt A, et al. Prognostic indicators in pediatric spinal glioblastoma. Childs Nerv Syst. 2023;39(5):1001–

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10. Kothbauer KF, et al. Management of intramedullary spinal cord tumors in children: diagnosis and outcomes.

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11. Narayan RK, et al. Role of radiotherapy in spinal cord gliomas. Pediatr Neurosurg. 2023; 59(1) :54–61.

12. Yang T, et al. Craniospinal irradiation for high-grade spinal cord tumors: patterns of failure and survival. J

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