Orbital lymphoproliferative lesions compromise a wide spectrum of diseases ranging from benign to malignant.
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Clinical presentation
Patients may present with a palpable mass or proptosis. Pain is an uncommon symptom unlike orbital pseudotumor, which manifests with acute pain.
Pathology
These lesions represent a spectrum of disorders including:
lymphoid hyperplasia of the orbit (benign): 10-40% 1
atypical lymphoid hyperplasia of the orbit
ocular adnexal non-Hodgkin lymphoma (malignant): most common, 60-90% 1
Lesions may be a manifestation of systemic lymphoma or arise primarily from the orbit.
Location
They can occur anywhere in the orbit with a predilection for anterior extraconal space (often in the superotemporal quadrant) and lacrimal gland (in 40% of cases) 2 and are bilateral in 25% of patients 1.
Radiographic features
Orbital lymphoproliferative lesions are typically seen as solid, homogeneously enhancing orbital tumors that mold to and encase orbital structures.
CT
iso-attenuating to slightly hyperattenuating (highly cellular)
diffuse homogeneous enhancement
bone destruction indicates aggressive histology
MRI
MRI is the modality of choice for evaluating the location and extent of disease 1.
T1: homogeneously mildly hyperintense to muscle
T2: mildly hyperintense to muscle
T1 C+ (Gd): moderate to marked homogeneous enhancement
Treatment and prognosis
Complications
The major long-term risk is the development of systemic lymphoma (33-50%) within 10 years after the initial diagnosis of an orbital lymphoproliferative disorder 1.
Differential diagnosis
On imaging, possible considerations include:
orbital Sjogren syndrome
See also
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