CNS Embryonal tumor NOS

Case contributed by Jeremy Jones
Diagnosis almost certain

Presentation

Unsteady on feet. Headaches.

Patient Data

Age: 4 years
Gender: Female
mri

Solitary left frontal aggressive tumor. Diffusion restriction and surrounding edema. Thick irregular enhancing peripheral rim and central non-enhancing necrotic center.

Case Discussion

This is a historical case originally diagnosed pathologically as sPNET. This has subsequently been removed from the WHO 2016 classification for brain tumors. Without additional pathological information, it is best classified as a CNS embryonal tumor, NOS.

Embryonal tumors include medulloblastoma, ATRT (in the very young) and the rarer embryonal tumors, e.g. embryonal tumors with multilayered rosettes (ETMR). The majority of tumors that were previously described as sPNETs are ETMRs.

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