Ocular adnexal diffuse large B-cell lymphoma: local disease correlates with better outcomes

Abstract

Purpose To describe the clinical, immunohistochemical and prognostic features, as well as outcomes of a large series of patients with orbital and periorbital diffuse large B-cell lymphoma (DLBCL). Design This study is a multicentre, retrospective non-comparative consecutive case series. Methods The setting for this study was institutional. A total of 37 consecutive patients identified from the institutions' databases with periorbital and orbital DLBCL were enrolled in the study. A retrospective chart review was used for observation. The main outcome measures were patient demographics, clinical features, imaging, immunohistochemical and histopathological data, treatments administered, and survival. Results A total of 20 out of 37 cases (54.1%) represented localised periorbital disease (group L), 11 of 37 (29.7%) had systemic disease at presentation with periorbital disease (group S1), and 6 of 37 (16.2%) had previous history of systemic lymphoma (group S2). In all, 28 out of 30 (93.3%) patients were CD20 þ , 5 of 25 (20%) were CD3 þ , and 11 of 11 (100%) were CD79a þ (varying denominators reflect the different numbers of patients tested). A total of 25 out of 32 patients (78.1%) received chemotherapy, 14 (43.8%) received rituxmab plus chemotherapy, and 19 (59.3%) received radiotherapy. Nine deaths occurred, one in group L (not lymphoma related), six in group S1, and two in group S2. Five-year Kaplan-Meier survival estimates were 55.9% for all cases, 90.9% for group L, 36.0% for group S1, and 0% for group S2. One-year progression-free survival estimates in groups S1 and S2 combined were 58.3% for patients treated with rituximab and 28.6% for those who were not. Conclusions To our knowledge, this report represents the largest series of patients with periorbital and orbital DLBCL in the literature. The difference in survival between groups L, S1 and S2 was striking, reflecting the grave prognosis of systemic DLBCL, but conversely the relatively optimistic outlook for patients with localised disease. Rituximab plus chemotherapy may be associated with increased survival.

Diffuse large B‐cell lymphoma and mantle cell lymphoma of the ocular adnexal region, and lymphoma of the lacrimal gland: An investigation of clinical and histopathological features

“…Previous studies found a similar clinical and biological profile in patients with DLBCL and MCL outside the ocular adnexal region (Looi et al 2005;Hon et al 2006;Madge et al 2010;Precupanu et al 2010). MCL patients were predominantly males (Looi et al 2005;Hon et al 2006), whereas the distribution was equal between males and females in DLBCL patients (Madge et al 2010;Precupanu Fig.…”

“…Information regarding the clinical and pathological characteristics of these two lymphoma subtypes involving the ocular adnexal region is sparse, because current investigations are limited to series of ocular adnexal lymphoma covering a wide range of subtypes (Coupland et al 2004;Ferry et al 2007;Sjo¨et al 2008) or are focused on centre-based populations (Looi et al 2005;Madge et al 2010;Precupanu et al 2010;Stacy et al 2012).…”

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

“…All stages are further divided on the basis of the absence (A) or presence (B) of systemic symptoms. The overall risk of lymphoma related death secondary to DLBCL has been reported as 38%, however a recent study by Madge et al 15 of ocular adnexal DLBCL found that in 11 patients with concurrent systemic disease at presentation, 5-year KaplanMeier survival estimates were 36%. 5 Our patient presented with simultaneous orbital and leptomeningeal involvement, perhaps a unique presentation.…”

Primary non hodgkin’s lymphoma of lacrimal sac presented as recurrent acute dacryocystitis: A case report

“…The study done by SN Madge et al [9] revealed various types of treatment for ocular adnexal DLBCL including chemotherapy, local radiotherapy or combination of both or with surgical debulking. Radiotherapy and chemotherapy had favourable prognosis of lacrimal sac lymphoma [10].…”

“…High survival rate of localized DLBCL relate with large proportion of patients receiving chemotherapy as treatment and used of Rituximab in addition to CHOP. Some patients received combination with local radiotherapy [9].…”