Endocrine Abstracts

Primary adrenal lymphoma is rare and accounts for <1% of extra-nodal non-Hodgkin’s lymphoma cases. It presents with B symptoms, bilateral adrenal masses and hypoadrenalism; and thought to have a poor prognosis. We describe a patient that was diagnosed with and successfully treated for primary adrenal diffuse large B cell lymphoma (DLBCL).

Case: A 62-year-old gentleman with longstanding hypertension presented with a 5-month history of weight loss, fatigue, sweats, and a 1-day history of vomiting and abdominal pain. He was tachycardic, normotensive, and had right flank tenderness but no palpable lymphadenopathy or organomegaly. Admission bloods showed acute kidney injury with eGFR 40 ml/min, hyponatremia (127 mmol/l), hyperkalemia (5.6 mmol/l), and CRP 428 mg/dl. I.v. antibiotics were started for presumed pyelonephritis. Subsequent CT abdomen revealed bilateral necrotic adrenal masses measuring 10.8 and 9.4 cm in their maximal diameter with retroperitoneal lymphadenopathy. The largest nodes were a 35×20 mm aortocaval node and 40×32 mm para-aortic node. 24-h urinary catecholamines and 24-h urinary free cortisol were within normal limits but short Synacthen test confirmed adrenal insufficiency with a baseline cortisol of 230 nmol/l rising to 242 nmol/l. He was commenced on hydrocortisone. Biopsy of the right adrenal mass was performed which showed diffuse lymphoid infiltrates, large pleomorphic cells, and prominent nucleoli positive for CD20, BCL2, BCL6, and MUM1. He was diagnosed with stage 2 BCL2-positive DLBCL. He underwent six cycles of R-CHOP chemotherapy with remission of symptoms and resolution of the masses and lymphadenopathy on CT. Four years later, he remains disease-free but still requires glucocorticoid replacement.

Conclusion: Primary adrenal lymphoma is rare but should be considered in patients presenting with bilateral adrenal masses and B-symptoms. Poor outcomes are reported but our patient has shown an excellent medium-term response to R-CHOP chemotherapy.