Marginal zone B-cell lymphoma of the conjunctiva

Lymphomas are the most common orbital malignancies, marginal zone B-cell lymphoma being the most common histotype. One quarter of these lymphomas exhibit conjunctival involvement, which is almost exclusively observed in indolent lymphomas (96% of conjunctival lymphomas). Primary conjunctival marginal zone B-cell lymphoma exhibits interesting and peculiar features, with some molecular, biological, clinical and prognostic differences from the rest of the ocular adnexal lymphomas. Recent studies demonstrate that chromosomal abnormalities, autoimmune disorders and infectious agents are variably associated with the development and behavior of this lymphoma. Conjunctival lymphomas are rarely lethal tumors and, in selected cases, may be managed without anti-tumor treatment. In other cases, conjunctival lymphomas cause symptoms that require immediate treatment. To date, no guidelines for the management of patients with conjunctival lymphoma exist. Surgical resection, radiotherapy and chemotherapy are the most common approaches to these malignancies, but the use of systemic immunotherapy, bacteria-eradicating antibiotic therapy and local treatments, such as intralesional injections of interferon or rituximab and eyedrops of cytostatics, is progressively growing.