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

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.

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

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

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).