Primary adrenal lymphoma (PAL) is an extremely rare entity, with approximately 70 cases reported in the English literature and 120 cases worldwide. Here we report the cases of a 53-year-old and a 62-year-old male patient and a 60-year-old female patient affected by large B-cell non-Hodgkin lymphoma of the adrenal gland. We summarize the diagnostic approaches that confirmed the diagnosis of PAL and describe individual treatment outcomes after therapy. Based on these case reports and a review of the literature patients are usually in the 6th or 7th decade of life and present with B-symptoms or rapidly progressive adrenal insufficiency in case of bilateral involvement. The identification of bilateral adrenal masses often causes a severe diagnostic problem. An etiological approach with assessment of the hormonal profile and detailed diagnostic imaging should be aimed at. Furthermore, if PAL is suspected biopsy of the adrenal mass should be performed after biochemical exclusion of a pheochromocytoma. Once the diagnosis is established further treatment decisions should be made in a multi-disciplinary setting in specialized centers.
© J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New York.