Ocular and Adnexal T-Cell Lymphoma

Ocular and adnexal T-cell lymphoma represents a rare non-Hodgkin’s lymphoma and is mostly secondary to systemic malignancies. The known etiology is mainly due to genetic predisposition and/or viral infections in different subsets of T-cell lymphoma. Both primary and metastatic T-cell lymphoma can invade intraocular and adnexal tissues. Clinical presentations vary largely based on the different intraocular and adnexal sites that are affected. Pathology based on intraocular/adnexal biopsies is the gold standard for diagnosis. Other diagnostic techniques, including ocular/orbital imaging, immunogenotyping, and molecular diagnosis, are also useful adjuncts to pathological diagnosis and subclassification. Current treatment is based on localized radiotherapy and chemotherapy in patients with local disease and combined with systemic high-dose methotrexate chemotherapy and/or stem cell transplantation in patients with systemic malignancy.