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

Abstract

Aim-To classify ocular adnexal lymphomas according to the Revised European and American Lymphoma (REAL) classification and to determine any correlation between clinical features or histomorphological variables with the patients' outcome. Methods-Conventional and immunohistology were performed on representative sections of 53 specimens of 46 patients with ocular adnexal lymphoma. The antibodies 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 tumours was determined using the MIB-1 antibody directed against the Ki-67 antigen. Clinical follow up data regarding the outcome were obtained from the treating physicians and/or hospital files. The Student's t test and log rank 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 represented B cell non-Hodgkin's lymphomas: extranodal marginal zone lymphoma (EMZL) (n=38), diVuse large cell B cell lymphoma (n=8), lymphoplasmocytic lymphoma/immunocytoma (n=2), mantle cell lymphoma (n=2), follicle centre lymphoma (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 patients had stage I disease. A variety of therapeutic regimens was administered, the main form of treatment being radiotherapy. The average follow up time was 85 months. Complete remission was achieved in 24 patients (10 after excision alone, eight after radiotherapy alone, three after combined excision and radiotherapy, one after chemotherapy alone, and two after combined radiotherapy and chemotherapy). 12 patients died of causes related to lymphoma; in one patient the cause of death was unknown. Six patients had persistent tumour at final follow up and two patients were lost to follow up. The 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 the final outcome (p<0.05) and tumour cell expression for Ki-67 antigen and p53 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 and p53 expression by the tumour cells proved to be important immunohistochemical markers concerning the prognosis. It is suggested that, following thorough staging investigations, primary EMZL (stage I) (if accessible) should be treated with excisional biopsy and subsequent low dose radiotherapy. Primary diVuse large cell B cell lymphoma of the ocular adnexa requires at least similar therapeutic measures and regular intensive follow up. (Br J Ophthalmol 2001;85:63-69)

Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options

“…29,87,88 In all published series except that by Cho et al, 42 the median age was approximately 65 years. 11,13,18,23,27,28,30,36,37,39,41,[43][44][45][83][84][85] Few patients have B symptoms or a performance status (PS) greater than 1, though these findings are rarely reported in published series. 11,18,28,30,42,84 Lacrimal gland and intraorbital sites, observed in approximately 50% of patients, are the most frequent ophthalmologic sites.…”

“…11,13,18,22,23,27,28,[36][37][38][39][40][41][42][43][44][45][46][47] However, a large majority of reported OALs-between 95% and 100% of reported casescorrespond to the B-cell type, and 80% of B-cell lymphomas are low grade. A major characteristic of OAL is the high incidence of marginal zone B-cell lymphoma or mucosa-associated lymphoid tissue (MALT) lymphoma, reported in approximately 50% of patients.…”

“…11,18,28,30,42,84 Lacrimal gland and intraorbital sites, observed in approximately 50% of patients, are the most frequent ophthalmologic sites. 11,18,30,37,41,45,83,85 Conjunctival sites are observed in one third of patients and almost always consist of low-grade NHL (96% of patients). 89 Eyelid involvement is observed in 0% to 44% of patients in the various published series, with a mean of approximately 10%.…”

Systemic non-Hodgkin’s lymphoma involving the orbit and leptomeninges

“…The second most common type is either follicular, 12% to 23%, or DLBCL, 7% to 21%, depending on the study. [4][5][6][7][8][9] The frequency of ocular adnexal lymphoma with localization to the orbit has been cited as 54%. 5 It has been suggested that it may be more appropriate to analyze ocular 10 found that for secondary ocular adnexal lymphoma, follicular type is most common (33%-66%) followed by (in approximate decreasing frequency) multiple myeloma/plasmacytoma, lymphoplasmocytic lymphoma/immunocytoma, mantle cell lymphoma, DLBCL, Burkitt lymphoma, MALT lymphoma and chronic lymphocytic leukemia.…”

Lacrimal Gland Marginal Zone Lymphoma: Regression after Treatment of Chronic Hepatitis C Virus Infection: Case Report and Review of the Literature

“…The extranodal marginal zone B-cell lymphoma of mucosa-associated tissue (MALT lymphoma) represents the most common lymphoma subtype in the ocular adnexa (1,2). Ocular adnexal lymphomas, which arise in the conjunctiva, lacrimal gland, eyelid, and orbital connective tissue, account for 8-12% of all ocular adnexal tumors (3,4), and 8% of all extranodal non-Hodgkin lymphomas (NHL) (5).…”