Introduction: Primary adrenal lymphoma is extremely rare. It accounts for <1% of extranodal lymphoma. It affects typically old males with bilateral adrenal gland involvement that leads to adrenal hypofunction. Prognosis is very poor due to the lack of optimum chemotherapeutic regimens.
Case Report: A 71 yearr old man with prior history of non insulin dependent diabetes, was admitted to the emergency department with symptoms of progressive weakness, fatigue, anorexia, and 15 kg weight loss in the previous 6 months. These symptoms were associated with hypotension, nausea, vomiting and unsteady gait. On physical examination the patient looked caquectic, chronically ill but alert. He had orthostatic hypotension. Skin hyperpigmentation was absent. Laboratory assays showed hyponatraemia (127 mEq/l) and a sligthly high potassium (5.6 mEq/l), normoglycemia and anemia. The patient initially responded to intravenous hydrocortisone in large doses with remission of symptoms. But orthostatic hypotension and ionogram alterations persisted so fludrocortisone was added with good results. Screening for malignancy was made with a hole body computed tomography scan (CT) that showed bilateral adrenal masses with more than 10 HU. Further assesment by adrenal MNR scan showed hypointense nodular images in T1 and T2 in both adrenal glands. With a nodule of 4 cm on the right gland and another of 4.5 cm on the left one. They appeared hyperintense in Out Phase, and had a post-gadolinium heterogeneous reinforcement. The ACTH test showed a complete failure of response (Basal Cortisol 0.3 ug/dl post-ACTH Cortisol 30 min: 0.4 ug/dl. Urine metanephrines and catecholamines were normal excluding phaeochromocytoma. Urgent needle biopsy of the left adrenal gland was performed wich revealed diffuse large B-cell lymphoma. Bone marrow biopsy was negative for lymphomatous involvement. After the diagnosis was made the patient deteriorated rapidly and passed away as a result of his condition.
Conclusion: Primary adrenal lymphoma is extremely rare. When it occurs, diffuse large B-cell lymphoma is the most common subtype involved. This case highlights the importance of considering diffuse large B-cell lymphoma (PAL) as an unusual cause of adrenal insufficiency. Specially in older patients who present with adrenal insufficiency associated with bilateral adrenal masses.
18 - 21 May 2019
European Society of Endocrinology