[Orbital mantle cell lymphoma succesfully treated by Bcl-2 inhibitor: Report of a case].

We describe the case of a patient with an orbital recurrence of mantle cell lymphoma successfully treated with oral Bcl-2 inhibitor monotherapy. A 58-year-old man who was treated with R-CHOP 8 years ago for mantle cell lymphoma, in remission for 5 years, presented with progressive decreased visual acuity in the left eye, along with binocular diplopia. Clinical examination revealed a decrease in visual acuity in the left eye to 1/20 Parinaud 20 and a relative afferent pupillary defect on the left. External examination revealed a left cranial nerve VI palsy, 2mm of painless proptosis, and hypesthesia of the left V1 territory, leading to a diagnosis of left orbital apex syndrome. The disc and macular OCT were normal. The visual field showed enlargement of the left blind spot. An emergency CT scan and MRI revealed an apical extraconal tissue mass infiltrating the medial rectus muscle, extending to the superior orbital fissure, optic canal and left cavernous sinus, hyperintense on T2 weighted images and isointense on T1. The morphological appearance was strongly suggestive of an infiltrative lymphomatous process. An 18 FDG PET-scan identified the orbital lesion as well as enhancing lesions in the axilla and colon; given the clinical features and test results, the diagnosis of recurrent mantle cell lymphoma was made without biopsy. Treatment with Venetoclax (Bcl-2 inhibitor) was initiated. At one month of treatment, the orbital apex syndrome had entirely resolved, with visual acuit...
Source: Journal Francais d Ophtalmologie - Category: Opthalmology Tags: J Fr Ophtalmol Source Type: research