Exophthalmos and blindness revealing primary T-cell lymphoma of the nasal cavity and paranasal sinuses

Background : We report a case of non-Hodgkin's malignant lymphoma of the ce rvico facial region revealed by unilateral exophthalmos and blindness, an u nusual mode of expression. Case report:A 40-year-old man with a 4-month history of diabetes mellitus h ad suffered from exophthalmos and blindness of the right eye for 20 years. Physical examination showed a homolateral hemifacial tumefaction and ophtha lmoplegia. The right ocular fundus showed papillar edema and non-proliferat ive diabetic retinopathy. The left eye was normal. The otolaryngology explo rations revealed a voluminous tumor in the anterior nasal cavity and in the cavum. Two biopsies were performed. Histology reported non-Hodgkin's T-cel l lymphoma. Orbitocerebral and cervicofacial computed tomography visualized the aggressive ethmoidomaxillary extension with intraorbital and intracran ial involvement. Chemotherapy (CHOP) combined with radiotherapy led to tumo r regression and involution of the exophthalmos. Diagnostic difficulties, m anagement and prognosis are discussed.