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Diagnosis and Treatment > Signs and Symptoms

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Kaja Grønning Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

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Archana Sharma Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

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Maria Adele Mastroianni Department of Haematology, Akershus University Hospital, Lorenskog, Norway

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Bo Daniel Karlsson Department of Radiology, Akershus University Hospital, Lorenskog, Norway

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Eystein S Husebye Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway

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Kristian Løvås Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway

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Ingrid Nermoen Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway
Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway

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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.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Topic, Adrenal, Hormone, ACTH, Cortisol, Condition/ Syndrome, Adrenal insufficiency, Patient Demographics, Age, Geriatric, Gender, Male, Ethnicity, White, Country of Treatment, Norway, Diagnosis and Treatment, Signs and Symptoms, Abdominal discomfort, Anorexia, Arthralgia, Bowel movements - bleeding, Dizziness, Fatigue, Hyponatraemia, Hypotension, Nausea, Pyrexia, Renal failure, Tachycardia, Vomiting, Weight loss, Investigation, ACTH, ACTH stimulation, Adrenal antibodies, Biopsy, Cortisol, Creatinine (serum), CT scan, MRI, Potassium, Sodium, Urine osmolality, Intervention, Fluid repletion, Radiotherapy, Medication, Cortisone acetate, Doxorubicin, Fludrocortisone, Glucocorticoids, Hydrocortisone, Mineralocorticoids, Prednisolone, Rituximab, Sodium chloride, Related Disciplines, Oncology, Radiology/Rheumatology, Urology, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-19-0131
Volume/Issue:
Volume 2020: Issue 1
Open access