Update of Ocular Adnexal Lymphomas

Sarah CouplandUnited Kingdom Speaker Uveal melanoma prognostication: beyond chromosome 3Prognosis for uveal melanoma (UM) is determined by a combination of clinical, histopathological, and molecular factors. Clinical markers include tumour size and location, while histopathological factors include cell type and mitotic activity. The Liverpool Ocular Oncology Centre has a long track record in UM prognostication, and has devised an algorithm (LUMPO3) for more precise risk stratification and identifying patients at high risk for metastasis. It is a multiparametric model, which also included genetic data, particularly chromosome 3 and 8. It has been demonstrated that BAP1 immunohistochemistry is a very close surrogate for the status of the BAP1 gene, which if mutated is associated with a poor prognosis in UM. Our recent work has investigated the inclusion of BAP1 immunohistochemistry in LUMPO3, in labs where chromosomal analysis is not available. Early detection of metastatic UM is critical, as the prognosis is poor once widespread metastases develop. If detected earlier, surgical and newer immunotherapy options have been demonstrated to prolong survival.Decoding intraocular masqueradeIntraocular lymphomas can be divided into 3 main subtypes: primary vitreoretinal lymphoma (VRL), primary choroidal lymphoma, and secondary intraocular lymphoma. VRL is a rare but aggressive form of non-Hodgkin lymphoma that affects the eye. The most common subtype is a Diffuse large cell B-cell lymphoma (DLBCL), and displays a similar morphology, immuno- and genetic profile to the primary CNS lymphomas. Indeed, VRL can relapse in the CNS, and similarly CNSL can involve the eye. VRL often "masquerades" as chronic uveitis, making early and accurate diagnosis challenging but crucial for effective treatment and improved prognosis. In contrast, primary choroidal lymphoma is an indolent non-Hodgkin lymphoma similar to the Marginal Zone B-cell lymphomas of the ocular adnexa, and do not spread to the CNS. This lecture will provide an update about the biology, diagnostics and treatment of VRL and choroidal lymphomas. It will also provide examples of differential diagnoses to consider in vitrectomy specimens.Update of Ocular Adnexal LymphomasOcular adnexal lymphomas are most commonly non-Hodgkin lymphomas (NHL) that develop in the conjunctiva, eyelid, lacrimal gland, and orbit. The most common subtype is an extranodal marginal zone lymphoma (EMZL), which often presents as a painless, salmon-coloured lesion on the conjunctiva or as a mass causing symptoms like proptosis, double vision, or swelling in the orbit. Other common NHL of the ocular adnexa include follicular lymphomas, diffuse large cell B-cell lymphomas and mantle cell lymphomas. Treatment varies as per lymphoma subtype and requires joined-up assessment with the haematologists and radiologists. Typically treatment is local with low-dose radiation therapy being a standard option for localised disease, while systemic treatment may be used for more advanced cases. This lecture will provide an update about the biology, diagnostics and treatment of ocular adnexal lymphomas.