Primary Adrenal Lymphoma Presenting With Severe Hyponatremia

Document Type

Conference Proceeding

Publication Date

1-2018

Publication Title

American Journal of Clinical Pathology

Abstract

Primary adrenal lymphoma (PAL) is a rare entity that usually presents with massive enlargement of both adrenals, and is associated with clinical and laboratory evidence of adrenal dysfunction. Few cases of PAL have been reported, most of which illustrate evidence of Addison disease. When 90% of the adrenal cortex is destroyed, serum cortisol dramatically decreases. However, we describe a case of PAL in a patient with refractory hyponatremia and adequate serum cortisol. A 63-year-old man presented to our institution with a six-month history of weakness, weight loss, and losses of consciousness; he was hospitalized multiple times and diagnosed with the syndrome of inappropriate antidiuretic hormone secretion (SIADH). His serum sodium and osmolality, along with urine sodium and osmolality, fulfilled the diagnostic criteria for SIADH. Potassium and morning serum cortisol (20.3 ug/dL) were normal. The patient quickly deteriorated after admission and expired. A CT scan revealed mass-like enlargement of the adrenals with extensive disease involving the kidneys, liver, and spleen. A liver biopsy displayed diffuse infiltration by large, atypical cells with high nuclear pleomorphism. The cells were cohesive and were seen in clusters and single file. Immunohistochemical stains for CD79A, PAX5, MUM1, and BCL6 were positive in neoplastic cells, consistent with diffuse large B-cell lymphoma (DLBCL). Interestingly, the cells did not express CD20. PAL should be considered in the differential diagnosis when nodular lesions involve both adrenals. The most common subtype is DLBCL. PAL has a predilection for men averaging 65 years of age and a propensity to involve multiple extranodal regions, including the liver and spleen. Despite difficulty identifying the adrenal cortex grossly and microscopically, and although a Cortrosyn stimulation test was not performed, the overall picture argues against adrenal insufficiency as the cause of hyponatremia. The cause of SIADH remains unclear.

Volume

149

Issue

Suppl 1

First Page

S10

Comments

American Society for Clinical Pathology (ASCP) Annual Meeting, November 2-6, 2017, Kissimmee, FL

Last Page

S11

DOI

10.1093/ajcp/aqx115.024

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