Large B-Cell Lymphoma Arising from the Optic Apparatus in an Immunocompetent Patient: Case Report and Review of the Literature

A 28-year-old healthy male presented one-month after experiencing a rapid deterioration in the temporal visual field of the left eye, which was followed by a complete loss of vision in the right eye. Ophthalmologic examination revealed no light perception in the right eye, 20/70 visual acuity in the nasal field of the left eye, and a dense temporal field cut in the left eye. MRI of the brain demonstrated a 4 × 2.2 × 2 cm enhancing infiltrative process involving the bilateral optic nerves, optic chiasm, and right greater than left optic tracts. The differential included lymphoma, glioma, and germ cell tumors. Lumbar puncture was nondiagnostic. Laboratory work revealed normal pituitary function and serology was negative for HIV, HCV, and HBV. CT of the chest, abdomen, and pelvis was negative for additional lesions or lymphadenopathy. Testicular ultrasound was positive for testicular atrophy and multiple subcentimeter hyperechoic nodules, which urology deemed too small for biopsy. A bone marrow biopsy was negative for malignancy.

A right pterional craniotomy was performed and an enlarged right optic nerve was encountered. Proximal dissection revealed a diffusely enlarged chiasm and swollen left optic nerve. Sharp arachnoidal dissection between the carotid terminus perforators revealed the right optic tract and multiple biopsies were performed. The right optic tract was chosen for the site of biopsy to preserve the greatest possible volume of visual fields in light of the presenting exam.

Final pathology was consistent with high grade large B cell lymphoma, unclassifiable, with features intermediate between Burkitt and diffuse large B cell lymphomas. The K_i-67 proliferation index was 95%, consistent with an aggressive lesion. Postoperative PET scan was performed and was positive for a hypermetabolic soft tissue mass in the ascending colon, which was concerning for a neoplastic process. The patient was referred for colonoscopy, which has yet to be performed. Interestingly, no hypermetabolic activity was identified in the testicles.

Based on the pathology and lack of definitive evidence of systemic lymphoma, the patient was treated as having primary central nervous system lymphoma (PCNSL). Methotrexate and Cytarabine treatment, along with corticosteroids, were started as an inpatient. Visual acuity in the right eye improved to hand motion at two feet in the temporal field. Visual acuity in the left eye improved to 20/60 in the nasal field and the temporal field deficit persisted.

Lymphoma of the optic apparatus, including spread from systemic disease or PCNSL, is rare and confirming the diagnosis may be challenging. In cases of PCNSL, tissue diagnosis often requires open biopsy of the optic apparatus. As seen in our case and others within the literature, significant recovery of visual function may occur with treatment and the site of biopsy must be tailored to maximize visual outcomes. We review the literature as it pertains to the imaging, surgical management, treatment, and prognosis of lymphoma of the optic apparatus.