Clinicopathologic Characteristics Associated with Prognosis in Ocular Extranodal Marginal Zone B Cell Lymphoma

Abstract

Background and Objectives: Extranodal marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT) type is the most common subtype of the ocular adnexal lymphoma. Despite its excellent prognosis, some patients experience partial remission or progressive disease. We aimed to evaluate clinicopathologic differences in the treatment responder group by comparing complete remission (CR) and non-complete remission (non-CR). Materials and Methods: This study retrospectively reviewed 48 patients who were diagnosed with ocular adnexal MALT lymphoma at Ulsan University Hospital between March 2002 and August 2018. Patients who were followed up for less than 6 months were excluded. Histologic and clinical features were analyzed. The patients were divided into two groups: CR and non-CR. Results: Among the 48 patients, 33 achieved CR and 15 achieved non-CR during the median follow-up period of 40.00 months (range, 7-109 months). In univariable analysis, more patients tend to undergo treatment in the CR group, and post-radiotherapy (post-RT) SUVmax, PET and serum lactate dehydrogenase (LDH) levels were higher in the non-CR group (p = 0.043, p = 0.016, and p = 0.042, respectively). In a multivariable analysis, only application of treatment, including radiotherapy or chemotherapy with immunotherapy, was related to CR (odd ratio 7.301, 95% confidence interval 1.273-41.862, p = 0.026). In subgroup analysis according to the site of involvement, none of the variables were significant except for the post-RT SUVmax of PET and level of serum LDH in the non-conjunctiva group (p = 0.026, and p = 0.037, respectively). Seven (14.6%) patients had a recurrence, and those with a recurring site other than the primary site had a higher Ki-67 labeling index, although it was not statistically significant (9.56% vs. 18.00%, p = 0.095). Conclusions: Although belonging to the early stages, the non-CR rate was high in patients with high serum LDH levels, and recurred patients had higher Ki-67. Thus, considering active treatment is recommended in this group of patients.

Keywords: MALT lymphoma; histology; ocular adnexa; prognostic factor.

Figure 1

Images show pathological characteristics. (a) Neoplastic cells are infiltrating in a confluent pattern. (H&E, ×100), (b) Neoplastic cells are infiltrating in a nonconfluent pattern. (H&E, ×200), (c,d) Tumor cells are differentiated into sheets of plasma cells. d represents the magnification view of the inlet box of c. (H&E, c; ×200, d; 400), (e) Multifocal sclerosis is intermixed with neoplastic lymphoid cells. (H&E, ×100), (f) Immunohistochemistry staining for CK reveals lymphoepithelial lesion. (CK, ×400) (g) Medium-sized lymphoid cells are intermixed with cells transformed into large cells. The large cells are indicated with red circles. (H&E, ×400), (h) High power field view shows lymphovascular invasion. (H&E, ×400). H&E, hematoxylin, and eosin; CK, cytokeratin.

Figure 2

Images of Ki-67 index in case6, and diffuse large B cell lymphoma transformed case7. (a,b) Immunohistochemistry staining for Ki-67 shows nuclear positivity in 5% of tumor cells in the initial slide but is increased to 30% in the recurred slide in eyelid. (Ki-67, a; ×100, b; ×100), (c,d) Transformed cells are scattered between small lymphoid cells and Ki-67 staining shows increment. (H&E, c; ×400, Ki-67, d; ×100).