Primary adrenal failure and central nervous system lesions: a rare case report of primary adrenal lymphoma

Primary adrenal lymphoma (PAL) is an extremely rare entity, usually presenting with bilateral adrenal masses and without extra-adrenal involvement, what can cause symptoms of adrenal insufficiency. We describe an unusual case of a patient presenting with neurologic and constitutional symptoms including vomiting, gait disturbance and confusion associated with fever and night sweats. Cranial computed tomography scan showed captive focal areas with a nodular configuration on the ependymal space. Adrenal insufficiency was diagnosed and a thoracoabdominal computed tomography (CT) scan revealed bilateral adrenal masses. The histological exam was consistent with diffuse large B cell lymphoma. The purpose of this case report is to emphasize the diagnostic approach with high degree of clinical suspicion needed, timely recognition and multidisciplinary treatment. The prognosis is usually poor and depends on involvement of other organs such as the central nervous system. The development of Addisonian crisis can contribute to the patient's morbidity and mortality and immediate glucocorticoid replacement therapy is recommended when adrenal insufficiency is suspected.