Pediatric Fourth Ventricular Atypical Meningioma With a Whorling-Sclerosing Pattern: A Report of a Rare Tumor.
Mihir Mohan Vaidya, Shruti Rao, Rashmi Chintan Parikh et al.
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Case in brief
An 11-year-old boy presented with frequent involuntary movements and gait imbalance. MRI showed a well-defined T2- and FLAIR-hyperintense fourth-ventricular lesion, initially suspected to be a medulloblastoma. After excision, histology identified a brain-invasive atypical meningioma with whorling-sclerosing architecture. Tumor cells expressed EMA, PR and SSTR2A, with retained MTAP and H3K27me3; the MKI67 index was 8% to 10%. Testing found no CDKN2A/B homozygous deletion or TERT promoter mutation. His symptoms improved immediately after surgery, and he was asymptomatic at discharge and follow-up.
What made this case unusual
The tumor combined an atypical meningioma diagnosis with a whorling-sclerosing pattern in the fourth ventricle of a child, where the MRI appearance instead suggested medulloblastoma.
Diagnostic / clinical pearl
A fourth-ventricular lesion suspected radiologically to be medulloblastoma may prove to be an atypical meningioma; histology and immunohistochemistry established the diagnosis in this case.