Primary oculo-cerebral lymphoma: A diagnostic challenge

Author: Mona Rekik (Tunisia)

Co-authors: Sonda Kammoun, Takwa Sammouda, Omar Hbaieb, Omar Ayadi, Imen Abid, Amira Trigui

Purpose

to report a case of panuveitis revealing oculo-cerebral lymphoma and demonstrate the supporting role of multimodal imaging in the diagnosis of primary vitreoretinal lymphoma.

Setting/Venue

Department of Ophthalmology, Habib Bourguiba University Hospital, Faculty of Medicine, University of Sfax, Tunisia

Methods

case report

Results

A 53-year-old man patient presented with complains of visual loss in both eyes for the past month. He had not previous medical history. Visual acuity was 2/10 in the right eye, light perceptions in the left eye. Biomicroscopy showed keratic precipitates with cells in the anterior chamber in both eyes. Fundus examination revealed vitritis in both eyes, papillary edema and creamy-yellow subretinal lesions in the right eye; retinal detachment in the left eye. Fluorescein angiography showed hyperfluorescent and hypofluorescent lesions in the right eye. On indocyanine green angiography, hypocianescent lesions were seen. Optical coherence tomography scans through the lesions revealed subretinal hyperreflective material with pigment epithelium detachments in the right eye. The patient underwent extensive blood work, neurological examination and brain MRI that resulted all negative. He received corticosteroids. A slight improvement was noted after one week of treatment. One month later, the patient developed neurological symptoms. A brain MRI was repeated and an expansive lesion in the internal capsule extending to the midbrain suggestive of lymphoma was detected. Lumbar puncture showed atypical cells and IL10 levels was elevated. The patient deteriorated rapidly, dying a week later despite an attempt of treatment.

Conlusions

Primary oculo-cerebral lymphoma is rare (1% of brain tumors) affecting people over 50 years old. It is a serious disease with highly varied clinical presentations. Ocular signs precede neurological manifestations in 80% of cases. The disease typically masquerades as chronic intermediate and/or posterior uveitis in aged patients with no history of uveitis, and it is unresponsive to corticosteroid therapy. In many cases the initial positive response to corticosteroid therapy for presumed uveitis, delay accurate diagnosis. Some characteristics on multimodal imaging, should raise suspicious for primary vitreoretinal lymphoma and lead to a diagnostic vitrectomy and/or retinal biopsy. The prognosis is related to early diagnosis. This case report stresses two points : first, a lymphoma must be one of the diagnosis to evocate if multimodal imaging shows infiltrative signs, second : it emphasizes the value of vitreous analysis searching for lymphoma during chronic uveitis in aged patients.

Financial Disclosure

Financial disclosure:None