Clinical Features and Treatment Outcomes of Non-Hodgkin’s Lymphomas Involving Rare Extranodal Sites: A Single-Center Experience

Abstract

Background: The involvement of certain organs such as the adrenal gland and ovaries is rare in non-Hodgkin’s lymphoma (NHL). There are few studies comparing clinical features and prognosis based on the extranodal organ involved. Methods: We selected patients presenting with predominantly extranodal involvement among patients diagnosed with NHL from 1998 to 2009 at the Samsung Medical Center. Results: Forty-eight patients with NHL involving rare extranodal sites were analyzed. The extranodal sites were as follows: adrenal gland (n = 14), ovary (n = 13), pancreas (n = 11), uterus (n = 4), esophagus (n = 4) and prostate (n = 2). Diffuse large B-cell lymphoma (DLBCL) was the most common (n = 39), and the median overall survival (OS) was 16.63 months. There was no significant difference in OS according to the involved sites. Rituximab plus CHOP failed to provide an additive survival benefit over CHOP alone in 39 DLBCL patients. The OS of DLBCL in rare extranodal sites was worse than that in common sites when compared based on tumor stage. Conclusions: NHL involving rare extranodal sites had a poor prognosis, and the impact of rituximab on survival was negligible. Thus, more intensive therapeutic strategies should be considered for NHL involving rare extranodal sites.

Primary Extra Nodal Non Hodgkin Lymphoma: A 5 Year Retrospective Analysis

“…Worse therapeutic outcome was seen for patients with lymphoma involving rare sites (Yun et al, 2010), those with T-cell phenotype (Ko et al, 2009), and among pediatric age group (Temmim et al, 2004). In another study, age, performance status, stage of disease, and serum LDH level were independent prognostic variables, whereas nodal or extra nodal site did not have any prognostic significance (Lal et al, 2008).…”

“…), skin, central nervous system (CNS), bone, testis, thyroid, breast, orbit, and rarely adrenal, pancreas, and the genitourinary tract (Singh et al, 2003;Temmim et al, 2004;Al Shemmari et al, 2008;Aoki et al, 2008;Gross et al, 2008;Lal et al, 2008;Fujita et al, 2009;Chen et al, 2010;Yoon et al, 2010;Yun et al, 2010;Arora et al, 2011;Nagi et al, 2011;Yaqo et al, 2011;Yang et al, 2011).…”

Unusual Presentation of Ewing Sarcoma in the Adrenal Gland: A Secondary Malignancy from a Survivor of Burkitt Lymphoma

“…Although there were no constitutional symptoms such as B symptoms, our first impression was relapse of Burkitt lymphoma rather than another adrenal gland tumor including pheochromocytoma because the radiologic imaging findings strongly suggested the relapse of lymphoma. Indeed, the invasion of the adrenal gland by a lymphoma appears in up to 25% of NHLs and shows a poor prognosis (23,24). However, the first biopsy failed to show evidence of lymphoma with only a few cells exhibiting atypical morphology.…”

Incidentally Discovered Diffuse Large B-Cell Lymphoma Limited to the Endocervical Mucosa in a Young Female Patient

“…It is unknown whether chemotherapy alone or in combination with radiotherapy or surgical interventions such as hysterectomy, pelvic node dissection or salpingo-oophorectomy would be the optimal therapeutic approach to this group of diseases. Although R-CHOP could be considered the first-line regimen with promising results, a larger number of reported cases are needed to reach better-founded conclusions [1,4,7,8,9]. …”