Sudden unexpected death with primary adrenal lymphoma.

An 82-year-old man was found dead on the road near his home with unwitnessed interval of 3 h from final witness. He had been diagnosed with hypertension and mild aortic stenosis (AS) 13 years before death, and was continuously followed up with medication. Although a recent medical check-up related to cardiac function was stable and consistent with moderate AS, he sometimes complained of general fatigue, anorexia associated with intermittent mild fever and rare vomiting in the weeks before death. At autopsy, no lethal injury or drug intoxication was found, but congenital bicuspid aortic valve (BAV) with central rache was found. Although calcification was found in a restricted area of one cusp, valvular structural deformity was clearly milder than in typical severe AS cases. Moderate left ventricular hypertrophy without coronary disease was found. A brownish-red, soft nodular lesion was found in both adrenal glands, but no other tumorous focus was evident in any other organs. Immunohistochemical examination showed that B-lymphocyte-derived markers (CD20, melanoma associated antigen (mutated) 1, and CD79a) were exclusively positive. Therefore, we diagnosed primary adrenal lymphoma (PAL), diffuse large B-cell lymphoma phenotype. We concluded that the cause of sudden unexpected death (SUD) was adrenal insufficiency associated with PAL, with a background of moderate AS related to BAV.