Endocrine Abstracts

Background: Primary adrenal lymphoma (PAL) is an extremely rare condition, typically involving bilateral adrenal glands. It has a male predominance and generally presents in elderly. Adrenal insufficiency, abdominal pain, and systemic symptoms such as weight loss and fatigue are common presentations.

Case presentation: We present a case of a 69-year-old male admitted with feature of adrenal failure including tanned skin, 20 kg weight loss over 5 months.

Investigation: Initial investigation showed normocytic anaemia (Hb 91 g/l), hyponatremia (Na 132 mmol/l), and hypercalcemia (adjusted Ca 3.32 mmol/l). The unusual presentation of hypercalcaemia and significant weight loss led to further investigation. A short Synacthen test confirmed adrenal insufficiency with a serum cortisol peak of 60 nmol/l, elevated ACTH >1250 ng/l. CT Abdomen and Pelvis revealed bilateral adrenal hyperplasia and 75mm left adrenal mass. Adrenal androgens and plasma metanephrines were normal. PET CT Scan confirmed bilaterally avid adrenal glands. Endoscopic ultrasound-guided biopsy left adrenal gland was conducted and revealed high-grade diffuse large B-cell lymphoma with a non-germinal centre double expresser (BCL2+/c-Myc+) immunophenotype.

Management: Replacement hydrocortisone therapy was commenced. Calcium level normalized after 3 days of intravenous saline. Patient received eight cycles of chemotherapy (POLA-R-CHP). A repeat PET CT after the third cycle showed complete metabolic resolution of adrenal uptake. However, he continued to required steroid replacement (9am cortisol was low at 51nmol/l). The recovery of his adrenal function post-lymphoma treatment remains uncertain.

Conclusions: Primary adrenal lymphoma should be one of the differential diagnoses of adrenal insufficiency, particularly in the elderly in the setting of negative 21-hydroxylase antibody and/or those presented with hypercalcemia. While caution not to biopsy a potential primary adrenocortical carcinoma, bilateral adrenal masses of unknown origin or in individuals with suspected extra-adrenal malignancy should be biopsied quickly when pheochromocytoma is excluded biochemically.