ABSTRACT
Lymphoma is the most common orbital malignancy in adults. Among the types of lymphoma, mantle cell lymphoma is a particularly aggressive form, often discovered through systemic involvement, with a dismal prognosis due to frequent recurrences. It is secondary to a t (11 ; 14) (q13; q32) chromosomal translocation resulting in an anti-apoptotic signal via overexpression of Bcl-2. Treatment is based on R-CHOP poly-chemotherapy. 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 acuity increased to 8/10 Parinaud 4 and a metabolic return to normal on PET scan. The PET scanner and clinical examination at 3 months were entirely normal. At the one-year follow-up visit, the patient was still on Venetoclax, the clinical examination was unchanged, and the PET-scan still showed a complete metabolic response.
Subject(s)
Exophthalmos , Lymphoma, Mantle-Cell , Adult , Exophthalmos/diagnosis , Exophthalmos/etiology , Humans , Lymphoma, Mantle-Cell/diagnosis , Lymphoma, Mantle-Cell/drug therapy , Magnetic Resonance Imaging , Male , Middle Aged , Neoplasm Recurrence, Local , OrbitABSTRACT
PURPOSE: To describe the anatomical landmarks of the retro-caruncular approach and its clinical applications based on a cadaveric study. PATIENTS AND METHOD: A dissection of 8 orbits providing from 4 fresh cadavers was carried out at the anatomical laboratory of the University Hospital of Nice, France between October 2018 and January 2019. RESULTS: Main anatomical relationships encountered are anteriorly the Duverney-Horner muscle and the lacrimal sac, posteriorly the anterior and posterior ethmoidal arteries, superiorly the pulley of the superior oblique muscle, inferiorly the lacrimonasal duct and the tendon of the inferior oblique muscle. The retro-caruncular approach allows a safe surgical access behind the lacrimal sac and Duverney-Horner muscle. Many oculoplastic surgical procedures can be performed through this approach: dacryocystorhinostomy, medial orbital fractures repair, "médial" orbital "décompression", biopsy of medial and extraconal tumours, medial periosteal fixation in third-nerve palsy. CONCLUSION: The retro-caruncular approach is a safe procedure avoiding skin incision. It allows a wide surgical space even if it is reduced compared to a more conventional skin route. It requires a great anatomical knowledge and a longer surgical learning curve.
