Presentation
Drowsiness, headache and vomiting for few days.
Patient Data
There is a large intra-axial mainly multiloculated cystic lesion showing two separate solid nodular components (one superiorly and the other inferomedial) in the right parietotemporal region with surrounding vasogenic edema extending to the frontal and occipital lobes and causing midline shift of about 2 cm with subfalcine herniation and compression of the lateral and 3rd ventricle. This causes dilatation of the contralateral ventricle with trans ependymal edema as well as mass-effect on the midbrain and posterior part of the corpus callosum.
The lesion shows peripheral enhancement of the cystic component as well as thick heterogeneous enhancement of the solid component which also shows restricted diffusion.
Impression:
Large intra-axial multiloculated cystic lesion with internal solid nodular components causing significant mass effect, herniation, and obstructive hydrocephalus.
The differential diagnosis includes embryonal tumor with multilayered rosettes (ETMR), atypical teratoid/rhabdoid tumor (AT/RT), desmoplastic infantile ganglioglioma/astrocytoma and supratentorial ependymoma.
The patient went on to have a resection confirming the diagnosis of an embryonal tumor with multilayered rosettes (ETMR).
Case Discussion
Embryonal tumors with multilayered rosettes (ETMR) are WHO grade 4 tumors and are considered small round blue cell tumors of the central nervous system.
They are relatively rare, occurring mostly in children aged 4 years and under, particularly in children under 2 years, and are more common in girls. They have a poor prognosis.
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