Bilateral Primary Adrenal B-Cell Lymphoma Leading to Adrenal Insufficieny

Presentation Number: SUN 376
Date of Presentation: April 2nd, 2017

Muhammad Talha*1, Rim Hasan2 and Peter Anthony Goulden3
1Central Arkansas Veterans Healthcare System/University of Arkansas for Medical Sciences, Little Rock, AR, 2Central Arkansas Veterans Health Care System/University of Arkansas for Medical sciences, Little Rock, AR, Little Rock, AR, 3University of Arkansas for Medical Sciences/ Central Arkansas Veterans Health Care System, Little Rock, AR

Abstract

Background: Bilateral adrenal masses constitute 10-15% of adrenal incidentalomas with varying differentials including metastatic cancers. Metastatic lymphomas to adrenals are found in 25% of autopsies, but primary adrenal lymphomas are extremely rare. They account for <1% of all cases of non-Hodgkin’s lymphoma cases. We are presenting a rare case of bilateral primary adrenal B-cell lymphoma leading to adrenal insufficiency.

Clinical case: A 79-year-old Caucasian male with a history of type 2 diabetes mellitus, hypertension and upper gastrointestinal bleed underwent evaluation for thrombocytopenia. Work up showed platelets 70-110 K/uL (150-450 K/uL), normal liver function, US abdomen: hepatic steatosis and mild splenomegaly, bone marrow biopsy showed slight hypercellular bone marrow 40%, mild increased megakaryocytes and normal flow cytometry and FISH analysis.

Abdominal CT showed mild splenomegaly, nodular liver, mild ascites and evidence for portal hypertension consistent with cirrhosis but most strikingly bilateral adrenal masses measuring 7.7 cm on the right and 12.4 cm on the left adrenal. No enlarged lymph nodes or suspicious osseous lesions.

CT guided left adrenal mass FNA showed diffuse large B-cell lymphoma, BCL-2-positive and MYC-negative. Flow cytometry - Monotypic B-cell population identified. PET/CT Scan confirmed the large bilateral adrenal masses and were intensely hypermetabolic but no adenopathy noted. Ascitic fluid was negative for malignancy. He was initiated on chemotherapy with rituxan + bendamustine and prednisone.

Plasma and 24 hour urinary metanephrines were unremarkable. Aldosterone 3.4 ng/dL (< 31.0 ng/dL) and renin 6.8 ng/ml/hr. (Supine 0.2-1.6 Upright 0.5-4.0 ng/mL/hr.). Random cortisol was 4.1 ug/dL (AM: 5 - 23, PM: 3 - 16 ug/dL) and he failed cosyntropin stimulation test with baseline ACTH 61 pg/ml (7 - 69 pg/mL) cortisol 8.7 ug/dL, 11.1 ug/dL at 30 minutes and 10.8 ug/dL at 60 minutes. He was started on Hydrocortisone replacement for adrenal insufficiency.

Conclusion: In patients with bilateral adrenal masses, primary adrenal lymphoma although rare should be considered within a differential to obtain a diagnosis and ensure prompt treatment. These patients should also be evaluated for adrenal insufficiency as most adrenal lymphomas are bilateral and adrenal insufficiency may often present insidiously.

 

Disclosure: PAG: Coinvestigator, Novo Nordisk. Nothing to Disclose: MT, RH