Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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January 13, 2014
Orbital Lymphoma with Perineural Spread
- Lymphoid tumors are the most common primary neoplasms of the orbit and usually are unilateral, of insidious onset, and cause painless proptosis.
- Perineural spread refers to neoplastic extension along a nerve, and its presence may significantly alter a patient’s treatment and diminish their prognosis.
- Key Diagnostic Features: Homogenously enhancing intraorbital mass may be intraconal or extraconal, most commonly in anterosuperior orbit. Diffusion-weighted imaging and ADC maps may demonstrate increased restriction of diffusion in orbital lymphoma compared with orbital inflammatory syndrome or cellulitis. However, inflammatory or necrotic lymphoma may render DWI/ADC inconclusive. Perineural spread is identified as enlarged enhancing nerve segments extending from the primary lesion and obliteration of foraminal fat planes.
- DDx: Adenoid cystic carcinoma, squamous cell carcinoma, meningioma, neurofibroma
- Rx: Radiotherapy mainstay with chemotherapy ± Rituximab (anti-CD20) for high-grade/malignant lymphomas.
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