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 reaction.

Case Report: A 71-year-old female underwent an incisional biopsy in the superior nasal quadrant of the left orbit following the occurrence of bilateral eyelid edema and ptosis. Histology and immunohistochemistry revealed a follicular large cell lymphoma of the orbit. Computerized tomography aimed at staging the disease revealed a supraclavicular lymphoadenopathy approximately 0.5 cm in diameter. Bone marrow biopsy was negative for lymphoma. The patient was classified as stage IV. She underwent a 10-week cycle of polychemotherapy (VP16, adriamycin, cyclophosphamide, vincristine, prednisone, bleomycin). Ten months after therapy, disease recurred at the cervical lymph nodes and the patient underwent a radiotherapy cycle of 4320 cGy with disease remission. At the time of writing, the patient presents a left axillary adenopathy on computerized tomography followup.

Conclusion: Ophthalmologists should be aware of possible rare occurrences of follicular large cell lymphomas of the orbit. In this case the clinical outcome is always more aggressive than the more common MALT-type lymphoma, which usually exhibits benign behavior.