Primary Intraocular T-cell Lymphoma

PURPOSE: Intraocular lymphoma can be divided into primary and secondary usually involving B-cell lymphoma. Intraocular T-cell lymphoma is mostly secondary lymphoma while primary intraocular T-cell lymphoma is extremely rare. We report a case of primary T-cell lymphoma. CASE SUMMARY: A 62-year-old male without any systemic disease presented with a floater in the right eye. A fundus examination showed multiple whitish retinal infiltrations in the right eye. Intraocular lymphoma was suspected, and systemic examination was performed, but all results were normal. During steroid treatment, previous lesions were enlarged, new lesions developed, and a diagnosis of primary T-cell lymphoma was made by diagnostic vitrectomy. Consecutive intravitreal injections of methotrexate were performed. After eight injections, the vitreous and retinal lesions improved but we decided to terminate the injections due to corneal epitheliopathy. The corneal epitheliopathy was recovered and the patient is currently undergoing periodic follow-ups without progression of the lesion. CONCLUSIONS: Although intraocular T-cell lymphoma is a rare condition, this primary T-cell type should be considered when an intraocular lymphoma lesion is suspected.

Secondary Choroidal Lymphoma Associated with Systemic Choroidal Lymphoma

PURPOSE: To report a case of secondary choroidal lymphoma. CASE SUMMARY: A 57-year-old female presented at our clinic with decreased vision in her right eye 1 month in duration. Slit-lamp examination showed vitreous cells in the right eye. Fundus examination of the right eye revealed an elevated yellowish mass-like lesion at the temporal area and multifocal yellowish patches at the choroidal level. The clinical impression was intraocular lymphoma. We performed diagnostic vitrectomy, but the result was negative. A systemic evaluation revealed enlarged cervical lymph nodes and lymph node biopsy showed diffuse large B-cell lymphoma. The patient was diagnosed as secondary choroidal lymphoma associated with systemic lymphoma and was treated with systemic chemotherapy and 4 injections of intravitreal methotrexate. The patient is scheduled for regular follow-ups. CONCLUSIONS: Choroidal mass may be the initial presenting sign of systemic lymphoma, and an extensive systemic evaluation should be performed.

Intraocular lymphoma in Korea: the Consortium for Improving Survival of Lymphoma (CISL) study

BACKGROUND: Intraocular lymphoma (IOL) is a rare malignant lymphoma that most closely resembles a diffuse large B-cell lymphoma, and it is a subtype of primary central nervous system lymphoma (PCNSL). IOL is located inside the eye in the retina, uvea, and/or optic nerve. We retrospectively analyzed IOL patient data to identify treatment patterns and survival rates in Korea. METHODS: Cytological confirmation for a diagnosis of IOL was performed for all patients. The clinical data collected from medical records included Ann Arbor stage, International Prognostic Index, performance status, date of diagnosis, treatment modality and response, date of relapse, and date of last follow-up. RESULTS: Twenty patients who were diagnosed with IOL, between December 2007 and June 2014 at multiple centers in Korea, were included in the analysis. Four patients were diagnosed with IOL alone, not involving the CNS. Two patients with isolated IOL later developed PCNSL. Nine patients developed CNS lesions before the onset of ocular lymphoma. Five patients had simultaneous onset in the eye and CNS. Twelve patients were treated by intravitreal injection of methotrexate for IOL. The median progression-free survival (PFS) for patients was 19.7 months (95% CI, 8.7-30.7 mo). The estimated 3-year overall survival (OS) for all patients was 75.1%. CONCLUSION: Treatment for IOL patients included radiotherapy and intraocular chemotherapy. IOL patients showed favorable PFS and OS. These patients would require long-term follow-up to identify relapse and adverse effects of radiotherapy or intraocular chemotherapy.

A Case of Primary Central Nervous System Lymphoma with Ocular Involvement

PURPOSE: To report one case of primary central nervous system lymphoma with ocular involvement confirmed by pars plana vitrectomy. METHODS: A 52-year-old healthy man visited our hospital complaining of progressive dysarthria, memory impairment, and chronic headache. Brain CT scans and MRI scans revealed a brain tumor in the left basal ganglia and the frontal lobe. Stereotactic biopsy of the tumor showed a large diffuse B-cell type malignant lymphoma. Three months later, he presented with decreased vision and sensation of floaters in the right eye. Funduscopic examination of the right eye revealed a vitreous opacity peripherally. He underwent pars plana vitrectomy and vitreous biopsy to rule out ocular involvement of lymphoma. Smears from the cytospin of the specimen prepared from vitrectomy in the right eye showed a greater number of large, pleomorphic lymphoid cells. Flow cytometric immunophenotyping showed a clonal population of CD 20 which was conclusive for infiltration with B-cell lymphoma. RESULTS: The patient underwent treatment with radiotherapy to the brain and the right eye in combination with systemic and intrathecal chemotherapy. After five cycles of chemotherapy and radiotherapy, the brain mass disappeared, the neurological symptoms were improved, and the visual acuity of the right eye increased.