Atypical teratoid rhabdoid tumour (AT/RT)

Case contributed by Dr Hanisalam

Presentation

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

Patient Data

Age: 4 months
Gender: Female
Modality: MRI

Left lateral intraventricular mass lesion.

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

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 tumour (AT/RT)

Case Discussion

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

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Case Information

rID: 10712
Case created: 11th Sep 2010
Last edited: 1st Jan 2016
Inclusion in quiz mode: Included

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