Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. More than 90% is of B-cell origin. The condition is bilateral in up to 75% of cases, with adrenal insufficiency in two of three patients. We report two cases of adrenal insufficiency presenting at the age of 70 and 79 years, respectively. Both patients had negative 21-hydroxylase antibodies with bilateral adrenal lesions on CT. Biopsy showed B-cell lymphoma. One of the patients experienced intermittent disease regression on replacement dosage of glucocorticoids.
Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. Bilateral adrenal masses of unknown origin or in individuals with suspected extra-adrenal malignancy should be biopsied quickly when pheochromocytoma is excluded biochemically. Steroid treatment before biopsy may affect diagnosis. Adrenal insufficiency with negative 21-hydroxylase antibodies should be evaluated radiologically.
Endocrinology, diabetes & metabolism case reports. 2020 Mar 10 [Epub ahead of print]
Kaja Grønning, Archana Sharma, Maria Adele Mastroianni, Bo Daniel Karlsson, Eystein S Husebye, Kristian Løvås, Ingrid Nermoen
Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway., Department of Haematology, Akershus University Hospital, Lorenskog, Norway., Department of Radiology, Akershus University Hospital, Lorenskog, Norway., Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway.