Bilateral Large Orbital Lymphoma With Proptosis

Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoid malignancy in adults. It is an aggressive malignancy and requires a multidisciplinary approach with various modalities which include chemotherapy, radiotherapy as well as immunotherapy. A 63-year-old Malay male patient with underlying type 2 diabetes mellitus, hypertension, ischemic heart disease, and stage II chronic kidney disease presented with a one-month history of bilateral eye proptosis associated with lid swelling and red eye. He also complained of progressive right eye blurring of vision. Visual acuity was counting fingers on the right and 6/18 on the left. On examination, the relative afferent pupillary defect was negative. There was bilateral eye proptosis, conjunctival chemosis, and restricted extra-ocular movement in all gazes. There was also exposure keratopathy over the right eye, and intraocular pressure was raised. Bilateral cervical and axillary lymph nodes were palpable. A computerized tomography scan of the brain and orbit revealed bilateral orbital masses with no bony erosions. An incisional biopsy over the upper lid confirmed the diagnosis of diffuse large B-cell lymphoma with multiple myeloma-1 (MUM-1) positivity which defines the activated B-cell subtype (ABC). He was co-managed with a hematologist and was commenced on the rituximab-cyclophosphamide, doxorubicin, vincristine, prednisone (R-CHOP) chemotherapy regime. Bilateral eye proptosis, chemosis, and restriction of extra-ocular movement resolved after the completion of treatment. However, right eye vision remains poor as the patient developed central self-sealed corneal perforation with iris plugging which has healed with scarring. Diffuse large B-cell orbital lymphoma is a fast-growing and aggressive tumor, hence early diagnosis and prompt multi-disciplinary treatment are crucial for a good outcome.