Embryonal tumor with multilayered rosettes (ETMR)
First seizure with sunsetting. Increasing head circumference and delayed motor milestones.
Loading Stack -
0 images remaining
The obvious intracranial mass lesion is a mixed solid and cystic lesion that is centered around the midline in the anterior cranial fossa. The more solid elements enhance and demonstrate diffusion restriction.
Features here are of a high-grade, aggressive tumor which is highly vascular. The differential includes supratentorial PNET and supratentorial ependymoma. The MRS is not particularly helpful.
A biopsy of the central solid components was undertaken and revealed that the tumor was a supratentorial PNET. The biopsy of the lesion resulted in significant hemorrhage and 4 units of blood along with clotting factors were required.
Chemotherapy did not significantly alter the volume of MRI characteristics of the lesion and it continued to grow.
Note: The current (2016) WHO classification of CNS tumors has made substantial changes to tumors previously considered to be supratentorial PNET, now classified as embryonal tumors with multilayered rosettes (ETMR), along with a number of other entities, in recognition of characteristic amplification of the C19MC region on chromosome 19 (19q13.42).
2 article feature images from this case
22 public playlist includes this case
- GD: Paediatrics - CNS - Brain tumours
- Neuro - Clinical Conditions - Masses
- NeuroImaging 1 - Infection, Tumours
- GK - Paeds - Neuro
- Frcr 40 most common FRCRII neuro Cases
- GK - Neuro - Brain tumours
- WDHB - Paediatrics
- CYSTIC INTRACRANIAL TUMORS
- Paed - Clinical Conditions - Brain tumours
- Neuro (illustrative case)
- RANZCR 2018 practice set 7 - Neuro
- BRain tumours
- Pediatric / Phakomatosis
- Neurorad - Mixed Cases
- WDHB - Neuro, Head and Neck
- Tumors and a Tumor-like
- Paed ITU Neuro teaching