Adrenal involvement in non-Hodgkin's lymphoma: four cases and review of literature

Abstract

Primary adrenal non-Hodgkin's lymphoma is very rare. We have seen 2 such cases among 241 cases of non-Hodgkin's lymphoma (0.83%) over the past 4 years. We could detect 2 more cases who had adrenal involvement, in addition to other extra nodal disease sites. Review of English literature revealed 65 cases of primary adrenal non-Hodgkin's lymphoma, most being single case reports. Salient features included-median age of 68 years (mean age of 47.25 years in our series), bilateral adrenal involvement in 60%, and predominantly diffuse large cell histology with 'B' cell immunophenotype. Adrenal insufficiency was present in two-thirds of the patients at diagnosis. Chemotherapy with or without radiotherapy is the mainstay of treatment. About half the patients respond to treatment, with median survival of 4 months. The high index of suspicion for early diagnosis and prompt treatment may improve outcome.