# Rare Orbital Involvement Originating from Extranodal Marginal Zone Lymphoma

## TL;DR

This paper reports a rare case of orbital lymphoma treated with targeted chemotherapy instead of radiotherapy, achieving long-term remission.

## Contribution

The study presents a successful treatment approach using R-CVP chemotherapy for extranodal marginal zone lymphoma of the ocular adnexa region.

## Key findings

- The patient achieved complete remission after six cycles of R-CVP chemotherapy.
- Radiotherapy was avoided due to potential side effects, and no relapse occurred.
- The case highlights an alternative treatment strategy for EMZL of the ocular adnexa region.

## Abstract

Ocular adnexa region (OAR) primary lymphomas are uncommon, accounting for 1–2% of non-Hodgkin lymphomas and 8% of extranodal lymphomas. Extranodal marginal zone lymphoma (EMZL) originates from several epithelial tissues, including the stomach, salivary gland, lung, small intestine, thyroid gland, and ocular adnexa region. Here, we report a 66-year-old female patient who was diagnosed with EMZL of OAR. In consideration of the possible side effect of radiotherapy, such as conjunctivitis, visual acuity impairment, and even retinal complications, she received six cycles of triweekly targeted chemotherapy with rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP) without radiotherapy. Then, she remained in complete remission up to the present day.