Desmoplastic infantile astrocytoma with multiple intracranial and intraspinal localizations at presentation

Area of Science:

• Pediatric Oncology
• Neuro-oncology
• Pediatric Neurosurgery

Background:

• Desmoplastic infantile gliomas (DIG) are rare pediatric brain tumors diagnosed in infants 1-24 months old.
• While generally having a favorable prognosis after surgical resection, established treatment algorithms for multifocal intracranial and intraspinal presentations are lacking.

Observation:

• A case study of an 11-month-old girl with nystagmus presented with multiple contrast-enhancing lesions on MRI, including suprasellar, cerebellar vermis, and spinal axis locations.
• Histological examination of the resected cerebellar vermis tumor confirmed desmoplastic infantile astrocytoma (DIA) WHO grade I.
• A 'wait-and-see' approach was adopted postoperatively, with follow-up MRI revealing no posterior fossa tumor recurrence, mild suprasellar tumor progression, and significant spinal tumor regression.

Findings:

• Desmoplastic infantile astrocytoma (DIA) is a rare, typically benign, pediatric brain tumor.
• Histological confirmation is essential for the definitive diagnosis of DIA.
• Surgical resection is the primary treatment for symptomatic DIA, though management of multifocal disease remains challenging.

Implications:

• This case highlights the potential for favorable outcomes with surgical management and observation in select cases of multifocal DIA.
• Further research and clinical experience are necessary to establish evidence-based treatment guidelines for infants with multiple intracranial and intraspinal desmoplastic infantile astrocytomas.
• The findings underscore the importance of individualized treatment strategies for rare pediatric brain tumors.