Dysembryoplastic neuroepithelial tumor (DNET) - cerebellum

Case contributed by Dr Angel Donato

Presentation

History of headache and difficulty walking. She notes a headache that is centered frontally, of episodic severe quality, associated with nausea and vomiting. She describes difficulty with balance and "swerving to the side".

Patient Data

Age: 20 years
Gender: Female

There is an oval-shaped low-density lesion in the superior posterior aspect of the cerebellar vermis immediately anterior to the falx cerebelli.  Surrounding cerebellum is of normal density and appearance. Fourth ventricle and aqueduct are normal.

There is a focal lesion within the left of midline superior aspect of the cerebellar vermis that measures 1.6 cm x 1.2 cm x 1.6 cm in AP, transverse and craniocaudal dimensions, hypointense on T1 and hyperintense on T2 with no appreciable enhancement noted. FLAIR images show mixed signal behavior with small cystic areas.

Resection confirmed the diagnosis of a dysembryoplastic neuroepithelial tumor (WHO GRADE I)

Case Discussion

Dysembryoplastic neuroepithelial tumors (DNET) are clinicopathologically unique benign neoplasms (WHO Grade I) typically located in the temporal lobes, frequently presenting with intractable seizures in children or young adults.

This case is an unusual presentation of an infratentorial DNET. 

Case contributed by Dr Angel Donato and Dr Ramon Figueroa. 

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Case information

rID: 51383
Published: 14th Feb 2017
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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