Mantle cell lymphoma: how not to be misled

Abstract

AbstractPurposeTo report a case of a multifocal mantle cell lymphoma (MCL) affecting both nodal and extra nodal sites in the orbital and maxillofacial region.MethodsObservational report of a patient presenting with palpebral masses in both upper eyelids and salivary glands. The patient underwent an extensive multidisciplinary work‐up including MRI, F 18 FDG PET/CT and biopsy with morphological and immunohistochemical analysis.ResultsA 77‐year‐old Caucasian male presented with asymmetrical painless swelling in both upper eyelids for more than 6 months. He reported weight loss but no fever or night sweats. His medical history included a JAK 2 + myeloproliferative disorder. He had no decreased vision and ocular examination was normal. Palpation of the head and neck revealed a swelling of the parotid and submandibular glands as well as a cervical lymphadenopathy. MRI confirmed multifocal lesions in the orbital and maxillofacial region. AS PET/CT scan showed a pathological FDG uptake at both upper eyelids with sparing of the lacrimal glands, the presumed diagnosis of a stage IV lymphoma was withheld. Incisional biopsies of the upper eyelid and supraclavicular node were performed.Immunohistochemical analysis were positive for CD20, CD5, Cyclin D1, Bcl‐2 but negative for CD10 and Bcl‐6. High expression of the proliferation index by Ki‐67 (locally up to 50%) was observed.ConclusionsThe purpose of this case was to describe an uncommon presentation of a non‐Hodgkin lymphoma. The diagnosis of MCL was based on a biopsy with immunohistochemical analysis. Although MCL is relatively uncommon, ophthalmologists should be aware of this rare diagnosis.