[Extranodal marginal zone lymphoma of the ocular adnexa]

Abstract

Introduction: Lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is among the most common malignant lymphomas of the ocular adnexa and it furthermore shows an increasing incidence. The disease is most often localized and indolent of nature; gentle treatment is therefore of essence to avoid complications, especially visual impairment. MALT lymphoma can be treated with radiotherapy (RT), but in case of relapse, alternatives are required. New treatment modalities such as anti-cluster of differentiation (CD)-20 monoclonal antibodies (rituximab) and local injection of interferon-alpha have shown promising results.

Material and methods: During the 2001-2006 period, nine patients with MALT lymphoma of the ocular adnexa were treated with RT at the Copenhagen University Hospital, Denmark. The visual acuity of six of the nine patients was evaluated before and after receiving RT.

Results: All nine patients achieved complete remission after a total RT dose of 26 Gy. One patient relapsed after 26 months and was then successfully treated with rituximab. Furthermore, one patient experienced a striking improvement of vision after RT: from 2/60 to 6/6 in the affected eye. The visual acuity of five patients was either constant or decreased a maximum of one line on Snellen's Chart.

Conclusion: RT is effective in treatment of MALT lymphomas of the ocular adnexa. Rituximab showed an excellent result in one patient with recurrent disease. However, latent infections such as hepatitis B virus (HBV) should be considered before rituximab is administered.