Angiotropic large cell lymphoma presenting as primary adrenal insufficiency

Angiotropic large cell lymphoma is reported in two patients who developed severe metabolic acidosis and unexplained hypotension before death. In addition to the usual multiorgan involvement of the disease, the adrenal glands of both patients were symmetrically enlarged. Histologically, the cortical vessels were engorged and filled with neoplastic lymphoid cells of B-cell lineage, whereas the parenchymal cells were compressed and atrophic. Angiotrophic large cell lymphoma seems to be a distinct and unrecognized cause of primary adrenal insufficiency, and should be included in the differential diagnosis of adrenal hormone deficiency.