Clinicopathological analysis of primary adrenal diffuse large B-cell lymphoma: effectiveness of rituximab-containing chemotherapy including central nervous system prophylaxis

Abstract

BackgroundPrimary adrenal lymphoma (PAL) is an extremely rare subtype of extranodal non-Hodgkin’s lymphoma. Some researchers have reported some of the characteristics of PAL and its association with poor prognosis; however, the clinicopathological features of PAL remain to be elucidated.MethodsFrom 2008 to 2011 we experienced seven cases of PAL in our institutions. We retrospectively analyzed the clinical and pathological features of these patients.ResultsThe patients ranged in age from 50 to 85 years, with a median of 71 years. The overall male:female ratio was 6:1. All seven patients were diagnosed with diffuse large B-cell lymphoma (DLBCL) pathologically. Bilateral adrenal involvement was confirmed in five patients. The median largest tumor diameter at diagnosis was 58 mm. The Ki-67 index was generally high (>70%). All patients were treated with rituximab-containing chemotherapy, and central nervous system (CNS) prophylaxis was conducted for three patients. One patient with CNS involvement at the time of the diagnosis also received whole-brain radiation. The overall survival rate at two years was 57% (median follow-up; 24.8 months). It is noteworthy that the three patients who received a full course of the rituximab-containing regimen and CNS prophylaxis are currently alive without disease relapse, and that none of the seven patients died due to progression of lymphoma.ConclusionsPrimary adrenal DLBCL can be a clinically aggressive disease entity. Rituximab-containing chemotherapy combined with CNS prophylaxis could be a reasonable option for the treatment of PAL; however, analyses of more PAL cases are needed for the establishment of this strategy.

Bilateral Adrenal Lymphoma Presenting as Primary Adrenal Insufficiency

“…A study conducted chemotherapy including the Rituximab and a central nervous system preventive therapy for diffuse large B cell lymphoma reported that the two-year survival rate was 57%, and the average survival time was 24.8 months (12). Additionally, in another multi-center study it was reported that when Rituximab-CHOP regimen was used, the two-year survival rate was 68.3%, and the disease free survival increased to 51.1% in patients diagnosed with diffuse large B cell lymphoma among primary adrenal lymphomas (13).…”

Bilateral primary adrenal lymphoma with adrenal insufficiency

“…The proposed underlying pathogenic mechanisms include immune system dysfunction, the proliferation of haematopoietic tissue in both adrenal glands after resting owing to the effects of the Epstein-Barr and JC polyoma viruses, and mutations in the p53 and c-KIT genes 11. PAL most commonly involves DLBCL (85% of cases), followed by mixed large-cell and small-cell lymphoma, T-cell lymphoma and undifferentiated lymphoma 2 12…”

Bilateral Adrenal Primary Diffuse Large B-Cell Lymphoma not Accompanied by Adrenal Insufficiency: A Report of Two Cases

“…Ana Mozos et al summarized 10 cases of PA-DLBCL patients, which 5 are affected bilaterally, during 4.5 months of random visitations, 7 cases died of lymphoma, 1 died of unrelated disease, 1 survived with the disease, 1 cured and survived [14]. Satoshi Ichikawa et al analyzed 7 cases of PA-DLBCL patients, 5 affected bilaterally, underwent R-CHOP chemotherapy, 2 year survival rate was 57% [15]. Yu Ri Kim et al analyzed 31 cases of PA-DLBCL, considered R-CHOP is an effective first line treatment, and they thought age, size of tumor, damage of adrenal gland function are relates to prognosis [16].…”