Primary Adrenal Lymphoma: When B Symptoms Appear Hand in Hand with Adrenal Masses

Primary adrenal lymphoma (PAL) is a very rare type of non-Hodgkin's lymphoma (NHL). Herein, we report a case of NHL of both adrenal glands in a 69-year-old man. The patient was admitted because of a 1-month history of B symptoms and symptomatic hypotension. Biochemical analysis showed normocytic normochromic anaemia, hyponatraemia, hyperkalaemia, and elevated lactate dehydrogenase, C-reactive protein and D-dimers. A computed tomography scan revealed bilateral enlargement of the adrenal glands. There was no evidence of endocrine adrenal dysfunction. The mass in the right adrenal gland was biopsied and histopathology identified a diffuse large B-cell lymphoma of the activated B-cell subtype. A positron emission tomography 18F-fluorodeoxyglucose scan showed intensive hypermetabolic lesions involving both adrenal glands, as well as other locations, with higher uptake in the adrenal glands. Taken together, these findings suggested the diagnosis of PAL. The patient responded favourably to debulking therapy and is currently undergoing chemotherapy.

Learning points: Primary adrenal lymphoma is a rare condition presenting with unspecific symptoms; diagnosis requires histopathological confirmation.Adrenal function must be evaluated to rule out insufficiency.Positron emission tomography may reveal hitherto unsuspected extension of disease and should be performed where available.