A 78-year-old male was admitted to our hospital complaining of nausea, general fatigue and anorexia in November, 1999. Clinical findings on admission were weight loss and dehydration but surface lymph nodes were not palpable. Masses in the bilateral adrenal glands were detected by ultrasonography, computed tomography and magnetic resonance imaging. Laboratory examinations revealed hyponatremia and hyperkalemia. Subsequent endocrine function tests showed normal serum cortisol and increased adrenocorticotropic hormone (ACTH) levels. Rapid ACTH test and cortico-hormone releasing hormone (CRH) test revealed insufficient secretion of cortisol. The histological diagnosis of the adrenal gland by laparotomy was diffuse large B-cell lymphoma. We diagnosed primary adrenal lymphoma with adrenal insufficiency. The patient underwent hormone supplementary therapy and chemotherapy, but he died two months later. We report on this rare primary adrenal lymphoma case and summarize the reports of this disease in the Japanese literature.

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