PEComa of the orbit

Case contributed by A.Prof Frank Gaillard

Presentation

Proptosis.

Patient Data

Age: 25 years
CT

A well-circumscribed mass within the right orbit with moderate homogenous enhancement. No bony destruction. 

MRI

The mass in the right orbit demonstrates intermediate T1 and T2 signal, with contrast and enhancement and quite low ADC values. 

Case Discussion

Exenteration of orbit with pathological confirmation of diagnosis as a Perivascular epithelioid cell tumor (PEComa) was performed at another institution.

PEComas are rare tumors, often in association with tuberous sclerosis (not present in this case). They are found in many locations, with the orbit being an uncommon location of this already rare tumor. 

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Case information

rID: 57712
Published: 26th Feb 2018
Last edited: 14th Aug 2019
System: Head & Neck
Inclusion in quiz mode: Included

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