Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Mar 10:2020:19-0131.
doi: 10.1530/EDM-19-0131. Online ahead of print.

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

Kaja Grønning  1 , Archana Sharma  1 , Maria Adele Mastroianni  2 , Bo Daniel Karlsson  3 , Eystein S Husebye  4   5 , Kristian Løvås  4   5 , Ingrid Nermoen  1   6
Affiliations

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

Kaja Grønning et al. Endocrinol Diabetes Metab Case Rep. .

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.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Contrast-enhanced computed tomography (CT) of the adrenals in patient 2 showing bilateral masses (arrows). Right-sided lesion consisting of two parts with ventral diameter measuring 1.8 cm and dorsal diameter measuring 1.6 cm. The left lesion measures 4.2 × 2.1 cm.
Figure 2
Figure 2
Adrenal CT 2 months later. Almost total regress of the right dorsal lesion and the left lesion. The right ventral lesion is still present (adenoma according to wash-out analyses).
Figure 3
Figure 3
Adrenal CT 7 months after spontaneous regress. Heterogenous tumor of the right adrenal measuring 6 × 7 × 12 cm (A). Atropic left adrenal without mass (B).
See this image and copyright information in PMC

References

    1. Sævik ÅB, Akerman AK, Grønning K, Nermoen I, Valland SF, Finnes TE, Isaksson M, Dahlqvist P, Bergthorsdottir R, Ekwall O, et al. Clues for early detection of autoimmune Addison's disease - myths and realities. Journal of Internal Medicine 2018. 283 190–199. ( 10.1111/joim.12699) - DOI - PubMed
    1. Erichsen MM, Lovas K, Skinningsrud B, Wolff AB, Undlien DE, Svartberg J, Fougner KJ, Berg TJ, Bollerslev J, Mella B, et al. Clinical, immunological, and genetic features of autoimmune primary adrenal insufficiency: observations from a Norwegian Registry. Journal of Clinical Endocrinology and Metabolism 2009. 94 4882–4890. ( 10.1210/jc.2009-1368) - DOI - PubMed
    1. Rashidi A, Fisher SI. Primary adrenal lymphoma: a systematic review. Annals of Hematology 2013. 92 1583–1593. ( 10.1007/s00277-013-1812-3) - DOI - PubMed
    1. Tallis PH, Rushworth RL, Torpy DJ, Falhammar H. Adrenal insufficiency due to bilateral adrenal metastases - a systematic review and meta-analysis. Heliyon 2019. 5 e01783 ( 10.1016/j.heliyon.2019.e01783) - DOI - PMC - PubMed
    1. McKay PaJAC LI. Corticosteroids in the Treatment of Neoplasms. Holland-Frei Cancer Medicine. 6th ed. Hamilton, ON: BC Decker, 2003. (available at: https://www.ncbi.nlm.nih.gov/books/NBK13383).

LinkOut - more resources