Presentation
Proptosis.
Patient Data
Age: 25 years
From the case:
PEComa of the orbit
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A well-circumscribed mass within the right orbit with moderate homogenous enhancement. No bony destruction.
From the case:
PEComa of the orbit
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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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