Case Report

Annals of Hematology

, Volume 83, Issue 7, pp 460-463

First online:

Primary adrenal lymphoma presenting as Addison’s disease: case report and review of the literature

  • George MantziosAffiliated withHematology Department, Metropolitan Hospital
  • , Panagiotis TsirigotisAffiliated withHematology Department, Metropolitan Hospital Email author 
  • , Filio VeliouAffiliated withHematology Department, Metropolitan Hospital
  • , Iosif BoutsikakisAffiliated withInternal Medicine Department, Metropolitan Hospital
  • , Lillian PetrakiAffiliated withHistopathology Department, Metropolitan Hospital
  • , John KolovosAffiliated withRadiology Department, Metropolitan Hospital
  • , Sotirios PapageorgiouAffiliated withHematology Department, Metropolitan Hospital
  • , Yannis RobosAffiliated withHematology Department, Metropolitan Hospital

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Primary adrenal lymphoma (PAL) is an extremely rare entity and adrenal insufficiency is a common complication. Bilateral enlargement of adrenal glands should raise the suspicion of lymphoma, especially in patients with clinical or laboratory features of adrenal insufficiency. Most of these cases are highly aggressive tumors and should be treated with multiagent chemotherapy. The role of bilateral adrenalectomy and/or radiotherapy cannot be estimated. Our patient with PAL was an 80-year-old man who presented with clinical and laboratory features of adrenal insufficiency. Combination chemotherapy plus rituximab was unsuccessful and our patient died from progressive disease.


Adrenal lymphoma Addison’s disease Extranodal lymphoma