Primary adrenal lymphoma.

Primary adrenal lymphoma is a rare extranodal lymphoma with characteristic clinical features including a high incidence of bilateral involvement, predominantly diffuse large B-cell histology, and a low incidence of extra-adrenal disease at diagnosis. Patients are most commonly older men presenting with fever, lumbar pain, and/or symptoms of adrenal insufficiency. Prolonged disease-free survival appears uncommon, which may reflect a publication bias and/or the presence of additional adverse prognostic factors at diagnosis in most patients. Given the rarity of this disease, no prospective chemotherapy studies have been reported. Unresolved therapeutic issues include the optimal chemotherapy regimen (with vs. without monoclonal antibody), the role of bilateral adrenalectomy and/or adjuvant radiation therapy, and the need for central nervous system prophylaxis, given recent reports raising the possibility of a high risk of parenchymal or meningeal relapse. Multicenter collaborative retrospective reviews and prospective trials are needed to address these issues.