PRIMARY BILATERAL ADRENAL LYMPHOMA PRESENTING WITH IMPENDING ADRENAL CRISIS

Abstract

Primary bilateral adrenal lymphoma is a very rare cause of adrenal insufficiency. We report the case of a 63-year-old woman who presented with signs and symptoms of impending adrenal crisis when referred for evaluation of large bilateral adrenal masses diagnosed on a computed tomography scan two weeks prior. Based on a high clinical suspicion of adrenal insufficiency, patient was initiated on glucocorticoid and mineralocorticoid therapy prior to laboratory confirmation of adrenal insufficiency. After stabilizing the patient and excluding pheochromocytoma, we proceeded with adrenal biopsy that revealed a nongerminal center-type diffuse large B-cell lymphoma. Our patient was treated with R-CHOP chemotherapy, with good response after 3 cycles but eventually died after the fifth cycle from neurologic complications. This case highlights the notion that primary adrenal insufficiency should be considered in patients presenting with bilateral adrenal masses. Although primary adrenal lymphoma is a very rare adrenal malignancy it should be considered in patients presenting with bilateral rapidly growing adrenal tumors and primary adrenal insufficiency.

Keywords: adrenal biopsy; adrenal crisis; adrenal lymphoma.

Figure 1

Baseline coronal (Panel A) and axial (Panel B) computed tomography (CT) demonstrates bilateral adrenal masses, 98 ×126 mm on left, unenhanced Hounsfield units (HU) of 23-40, and 86 × 118 mm on right, unenhanced HU of 35-51. A follow-up contrast-enhanced CT scan after 3 cycles of chemotherapy demonstrates a decrease in bilateral adrenal masses, now measuring 55 ×36 mm on the left and 47 × 38 mm on the right (Panel C).

Figure 2

Biopsy of one of the adrenal masses demonstrated a diffuse large B-cell lymphoma (hematoxylin and eosin-stained x 600), Panel A, with CD 20 positivity, 400 x magnification, Panel B, and a positive Ki-67 immunohistochemistry, 400 x magnification, in ~90% of cells (Panel C).