Follicular large-cell lymphoma of the orbit: a clinicopathologic, immunohistochemical and molecular genetic description of one case

Background: Follicular large cell lymphoma of the orbit is a very rare and aggressive lymphoproliferative disease of the ocular adnexa. In this study we analyzed the clinicopathologic characteristics of one patient, including the immunoglobulin gene rearrangement assay by means of polymerase chain r eaction. Case report: A 71-year-old female underwent an incisional biopsy i n the superior nasal quadrant of the left orbit following the occurrence of bilateral eyelid edema and ptosis. Histology and immunohistochemistry reve aled a follicular large cell lymphoma of the orbit. Computerized tomography aimed at staging the disease revealed a supraclavicular lymphoadenopathy a pproximately 0.5 cm in diameter. Bone marrow biopsy was negative for lympho ma. The patient was classified as stage IV. She underwent a IO-week cycle o f polychemotherapy (VP16, adriamycin, cyclophosphamide, vincristine, predni sone, bleomycin). Ten months after therapy, disease recurred at, the cervic al lymph nodes and the patient underwent a radiotherapy cycle of 4320 cGy w ith disease remission. At the time of writing, the patient: presents a left axillary adenopathy on computerized tomography followup. Conclusion: Ophth almologists should be aware of possible rare occurrences of follicular larg e cell lymphomas of the orbit. In this case the clinical outcome is always more aggressive than the more common MALT-type lymphoma, which usually exhi bits benign behavior.