Primary adrenal lymphoma as a cause of adrenal insufficiency, a report of two cases. (10th March 2020)

• Record Type: Journal Article
• Authors: Grønning, Kaja; Sharma, Archana; Mastroianni, Maria Adele; Karlsson, Bo Daniel; Husebye, Eystein S; Løvås, Kristian; Nermoen, Ingrid
• Is Part Of: Endocrinology, diabetes & metabolism case reports. (2020)
• Publication Date: 2020-03-10
• Subjects: Geriatric -- Male -- White -- Norway; Adrenal -- Adrenal -- ACTH -- Cortisol -- Adrenal insufficiency -- Adrenal lymphoma*; Dizziness -- Anorexia -- Nausea -- Arthralgia -- Weight loss -- Renal failure -- Fatigue -- Hyponatraemia -- Vomiting -- Pyrexia -- Tachycardia -- Hypotension -- Abdominal discomfort -- Bowel movements - bleeding -- Adrenal antibodies -- CT scan -- Biopsy -- ACTH -- Creatinine (serum) -- Potassium -- Sodium -- Cortisol -- ACTH stimulation -- Urine osmolality -- MRI -- Radiotherapy -- Fluid repletion -- Glucocorticoids -- Sodium chloride -- Fludrocortisone -- Mineralocorticoids -- Cortisone acetate -- Doxorubicin -- Prednisolone -- Vincristine* -- Cyclophosphamide* -- Hydrocortisone -- Rituximab; Oncology -- Radiology/Rheumatology -- Urology; Unique/unexpected symptoms or presentations of a disease -- March -- 2020; Endocrinology -- Periodicals; Diabetes -- Periodicals; Diabetes Mellitus; Endocrinology; Diabetes; Endocrinology; Case Reports; Periodicals; Periodicals; 616.4
• DOI: 10.1530/EDM-19-0131
• Languages: English
• ISSNs: 2052-0573

Abstract

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.