Presentation
Loss of consciousness and seizure.
Patient Data
There is a very large intra axial mass at right frontal lobe which is low signal on T1W with areas of hemorrhage and heterogenous signal on T2W. The mass has cystic (necrosis) areas and central neoangiogenesis.
Severe compression effect on right lateral ventricle body and frontal horn is visible by this mass leading to left sided midline shift. Left lateral ventricle dilation due to compression of mass on foramen of monro is seen.
Restriction diffusion of solid portion of mass is notable on DWI sequence.
After contrast administration, heterogenous avid enhancement of this mass is notable with areas of necrosis.
Based on pathology findings, high grade embryonal tumor in favor of atypical teratoid/rhabdoid tumor is the final diagnosis.
Case Discussion
Large solitary right frontal lobe intra-axial mass, favored to represent an aggressive primary CNS neoplasm.
Differentials include infantile embryonal tumor (possibly primitive neuroectodermal tumor or atypical teratoid/rhabdoid tumor), infantile anaplastic ependymoma or high-grade glioma.
The tumor was debulked, and pathological analysis confirmed the diagnosis of an embryonal atypical teratoid/rhabdoid tumor (AT/RT).
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