Axial Noncontrast Head CT and T2 MRI of a AT/RT in the cerebellopontine angle (CPA), which causes CSF outflow obstruction and obstructive hydrocephalus.
Atypical Teratoid/Rhabdoid Tumor (AT/RT)
Overview
- Malignant, high-grade (Grade 4), pediatric-onset embryonal tumor.
Clinical Presentation
- Pediatric tumor: onset typically < 3 y.o.
- Most common location:
- Cerebellopontine angle (Obstructive hydrocephalus) - (majority will have < 2 yr survival)
- Less common location:
- Cerebrum - (if localized and with favorable immunohistochemical characteristics will have ~ 5 yr survival)
- Suprasellar region (CN deficits)
- Pineal region
- Rare location:
- Spinal cord
- Leptomeningeal/CSF dissemination is common and a worrisome (often terminal) finding.
Pathology
- Hemorrhagic/necrotic tumors that comprise sheets of poorly differentiated rhabdoid cells.
- Cells have hyperchromatic nuclei, scant cytoplasm, and frequent mitoses.
- Contain components of divergent cellular differentiaion:
- Epithelial
- Mesenchymal (produces a firm-texture)
- Neuronal
- Glial
Immunohistochemistry
- Immunopositivity for the following:
- Epithelial membrane antigen (EMA)
- Vimentin
- +/- Smooth muscle actin (SMA)
Differential Diagnosis
- Medulloblastoma
- Choroid plexus carcinoma
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