Primary ocular adnexal mantle cell lymphoma with distant spread and involvement of the contralateral eye one year later; a case report and literature review

TL;DR

A rare case of mantle cell lymphoma in the eye spreads to the other eye and becomes systemic within a year.

Contribution

Highlights the rare progression of primary ocular adnexal mantle cell lymphoma and its systemic spread.

Findings

Mantle cell lymphoma initially presented in one eye and spread to the contralateral eye within three months.

Systemic involvement was confirmed through PET CT and biopsy after initial localized treatment.

MCL constitutes only 5% of B-cell ocular adnexal lymphomas, emphasizing the need for early detection.

Abstract

We herein report a middle-aged gentleman who initially presented with ocular adnexal mantle cell lymphoma (MCL) on the right eyelid. The lesion was excised and the patient was treated with radiation therapy. During the initial presentation, a PET CT was performed and did not reveal disease involvement beyond the eyelid. The patient presented 3 months later with ocular adnexal MCL of the contralateral eye. Re-evaluation using PET CT revealed a slight increase in the uptake in several lymph nodes and the spleen, which, after biopsy, confirmed systemic MCL. The patient was started on six cycles of chemotherapy. The patient also underwent autologous hematopoietic stem cell transplant. Approximately 80% of primary ocular adnexal lymphomas are B-cell in origin, with MCL being the rarest subtype constituting only 5% of B-cell ocular adnexal lymphomas. Despite its rarity, it is crucial for clinicians to detect the entity early and ensure rapid initiation of appropriate therapy.

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