The Vanishing Adrenal Glands: A Transient Regression of Adrenal Lymphoma After a Single Dose of 1 mg Dexamethasone

Abstract

Objective: Dexamethasone is a known treatment for lymphoma, but the potency and rapidity of its effect have not been recognized. We present a case of bilateral adrenal lymphoma that significantly reduced in size after a single dose of dexamethasone.

Methods: We present the clinical course and investigations, including adrenocorticotropic hormone, cortisol, short synacthen test, computed tomography (CT), and adrenal biopsy results.

Results: A 52-year-old man had a fall and was incidentally found to have bilateral adrenal masses (left, 6 cm; right, 5 cm) on CT. His adrenal function tests included plasma metanephrines (normetanephrine, 830 pmol/L [normal, <1180]; metanephrine, <100 pmol/L [<510]; 3-methoxytyramine, <100 pmol/L [<180]); aldosterone, 270 pmol/L( 90-700); and random cortisol, 230 nmol/L (160-550). An overnight dexamethasone suppression test with 1 mg of dexamethasone showed cortisol of <28 nmol/L (0-50). A repeat CT scan 8 days thereafter showed adrenal masses of 4.5 and 3.5 cm on the left and right, respectively. He had a follow-up CT scan 3 months later that showed adrenal lesions measuring 8 cm (left) and 9 cm (right). He subsequently presented with fatigue and dizziness. Morning cortisol of 201 nmol/L (160-550) with adrenocorticotropic hormone of 216 ng/L (10-30) indicated primary adrenal insufficiency. Mineralocorticoid and glucocorticoid replacement therapy commenced. An adrenal biopsy showed abnormal enlarged B cells, consistent with a diagnosis of diffuse large B-cell lymphoma.

Conclusion: A diagnosis of lymphoma should be considered when adrenal lesions shrink following even a single low dose of dexamethasone administered as a part of a diagnostic test.

Keywords: ACTH, adrenocorticotropic hormone; CT, computed tomography; DLBCL, diffuse large B-cell lymphoma; ONDST, overnight dexamethasone suppression test; PAI, primary adrenal insufficiency; PET, positron emission tomography; SST, short synacthen test; adrenal; adrenal cancer; adrenal insufficiency.

Fig. 1

CT images showing the varying sizes of the adrenal lesions during the course of the disease. A, Bilateral adrenal lesions (arrows) replacing the entirety of both adrenal glands (July 2018). B, Reduction in the size of the adrenal lesions (arrows) following an overnight dexamethasone suppression test but with residual adrenal nodularity (October 2018). C, Re-enlargement of adrenal masses (arrows) 2 months later (January 2019), which were now larger than the presentation CT image (January 2019). D, Normal-sized adrenal glands following completion of 2 cycles of chemotherapy (September 2019). CT = computed tomography.

Fig. 2

Histology. A, Hematoxylin and eosin (magnification: x10) and B, hematoxylin and eosin (magnification: x40) both show the lymphoma, which is composed of a diffuse infiltrate of atypical large lymphoid cells with pleomorphic nuclei. C, CD20 staining (magnification: x20) confirms that these are neoplastic B cells.