Primary adrenal lymphoma as a cause of adrenal insufficiency, a report of two cases

Kaja Grønning; Archana Sharma; Maria Adele Mastroianni; Bo Daniel Karlsson; Eystein S Husebye; Kristian Løvås; Ingrid Nermoen

doi:10.1530/EDM-19-0131

Primary adrenal lymphoma as a cause of adrenal insufficiency, a report of two cases

Endocrinol Diabetes Metab Case Rep. 2020 Mar 10:2020:19-0131. doi: 10.1530/EDM-19-0131. Online ahead of print.

Authors

Kaja Grønning¹, Archana Sharma¹, Maria Adele Mastroianni², Bo Daniel Karlsson³, Eystein S Husebye^{4

5}, Kristian Løvås^{4

5}, Ingrid Nermoen^{1

6}

Affiliations

¹ Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway.
² Department of Haematology, Akershus University Hospital, Lorenskog, Norway.
³ Department of Radiology, Akershus University Hospital, Lorenskog, Norway.
⁴ Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway.
⁵ Department of Medicine, Haukeland University Hospital, Bergen, Norway.
⁶ Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway.

Abstract

Summary: 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.

Learning points: 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.

Keywords: 2020; ACTH; ACTH stimulation; Abdominal discomfort; Adrenal; Adrenal antibodies; Adrenal insufficiency; Adrenal lymphoma*; Anorexia; Arthralgia; Biopsy; Bowel movements - bleeding; CT scan; Cortisol; Cortisone acetate; Creatinine (serum); Cyclophosphamide*; Dizziness; Doxorubicin; Fatigue; Fludrocortisone; Fluid repletion; Geriatric; Glucocorticoids; Hydrocortisone; Hyponatraemia; Hypotension; MRI; Male; March; Mineralocorticoids; Nausea; Norway; Oncology; Potassium; Prednisolone; Pyrexia; Radiology/Rheumatology; Radiotherapy; Renal failure; Rituximab; Sodium; Sodium chloride; Tachycardia; Unique/unexpected symptoms or presentations of a disease; Urine osmolality; Urology; Vincristine*; Vomiting; Weight loss; White.