Atypical teratoid rhabdoid tumor

Case contributed by Hani Makky Al Salam
Diagnosis certain

Presentation

Infant with a mass on CT (not shown) for further evaluation

Patient Data

Age: 4 months
Gender: Female

Left lateral intraventricular mass lesion.

The patient went on to have a craniotomy and debulking of the tumor. 

Histology

Frozen section diagnosis: Left lateral ventricular tumor: Malignant neoplasm, differential between choroid plexus carcinoma and atypical teratoid rhabdoid tumor and anaplastic ependymoma.

Immunohistochemistry: CTK+, EMA+, GFAP+, NFP-, VIMENTIN+, SYNAPTOPHYSIN+, CD56 focal, BAF-47 absent, SMA+

Electron Microscopy: EM07-513

FINAL DIAGNOSIS: Atypical teratoid / rhabdoid tumor (AT/RT)

Case Discussion

This very aggressive tumor with extension into the ventricles was shown to be an atypical teratoid / rhabdoid tumor (AT/RT), which more frequently occurs in the posterior fossa. A reasonable differential on imaging in this age group is of a choroid plexus carcinoma

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