Pediatric Fourth Ventricular Atypical Meningioma With a Whorling-Sclerosing Pattern: A Report of a Rare Tumor

Area of Science:

• Neuro-oncology
• Pediatric Neurosurgery
• Pathology

Background:

• Meningiomas are uncommon in children and exceptionally rare in the fourth ventricle.
• Pediatric fourth ventricle tumors often present with neurological deficits.

Purpose of the Study:

• To report a rare case of pediatric fourth ventricle meningioma.
• To describe the clinical presentation, imaging findings, and histopathological features of this rare tumor.

Main Methods:

• Case report of an 11-year-old boy with neurological symptoms.
• Magnetic Resonance Imaging (MRI) for tumor visualization.
• Surgical tumor excision and subsequent histopathological analysis.
• Immunohistochemical staining for tumor markers (EMA, PR, SSTR2A, MTAP, H3k27me3, MKI67).
• Genetic analysis for CDKN2A/B deletion and TERT promoter mutation.

Main Results:

• MRI revealed a fourth ventricle lesion initially suspected as medulloblastoma.
• Histopathology confirmed a brain invasive atypical meningioma with a unique whorling-sclerosing architecture.
• Immunohistochemistry showed positivity for EMA, PR, SSTR2A, with retained MTAP and H3k27me3 expression.
• MKI67 proliferation index was 8-10%.
• Absence of CDKN2A/B homozygous deletion and TERT promoter mutation was noted.

Conclusions:

• Atypical meningioma is a rare but possible diagnosis for pediatric fourth ventricle tumors.
• Surgical resection led to significant clinical improvement in this pediatric patient.
• This case highlights the importance of thorough histopathological evaluation for accurate diagnosis and treatment planning.