Long term outcome of ocular adnexal lymphoma subtyped according to the REAL classification

Aim-To classify ocular adnexal lymphomas according to the Revised European and American Lymphoma (REAL) classification and to determine any correlatio n between clinical features or histomorphological variables with the patien ts' outcome. Methods-Conventional and immunohistology were performed on representative s ections of 53 specimens of 46 patients with ocular adnexal lymphoma. The an tibodies used were CD20, BCL-2, CD21, CD23, CD43, CD3, CD5, p53, cyclin D1, pan-cytokeratin, kappa, lambda, IgD, and IgM. The growth fraction of the t umours was determined using the MIB-1 antibody directed against the Ki-67 a ntigen. Clinical follow up data regarding the outcome were obtained from th e treating physicians and/or hospital files. The Student's t test and log r ank test were used for statistical analysis. Results-The patient collective consisted of 29 females and 17 males with an age range of 32-89.7 years (average 63 years). Almost all specimens repres ented B cell non-Hodgkin's lymphomas: extranodal marginal zone lymphoma (EM ZL) (n=38), diffuse large cell B cell lymphoma (n=8), lymphoplasmocytic lym phoma/immunocytoma (n=2), mantle cell lymphoma (n=2), follicle centre lymph oma (n=1), and plasmacytoma (n=1). One case of a secondary anaplastic large cell lymphoma of T cell type (T-ALCL) was diagnosed. The majority of the p atients had stage I disease. A variety of therapeutic regimens was administ ered, the main form of treatment being radiotherapy. The average follow up time was 85 months. Complete remission was achieved in 24 patients (10 afte r excision alone, eight after radiotherapy alone, three after combined exci sion and radiotherapy, one after chemotherapy alone, and two after combined radiotherapy and chemotherapy). 12 patients died of causes related to lymp homa; in one patient the cause of death was unknown. Six patients had persi stent tumour at final follow up and two patients were lost to follow up. Th e stage at presentation, as well as the lymphoma malignancy category, had a significant correlation with the final course of the disease (p=0.0001 and p=0.03, respectively). A significant correlation was also noted between th e final outcome (p<0.05) and tumour cell expression for Ki-67 antigen and p 53 protein. Conclusion-67% of patients with ocular adnexal lymphoma had EMZL. The stage at presentation had a significant influence on the final outcome. MIB-1 an d p53 expression by the tumour cells proved to be important immunohistochem ical markers concerning the prognosis. It is suggested that, following thor ough staging investigations, primary EMZL (stage I) (if accessible) should be treated with excisional biopsy and subsequent low dose radiotherapy. Pri mary diffuse large cell B cell lymphoma of the ocular adnexa requires at le ast similar therapeutic measures and regular intensive follow up.