Endocrine Abstracts

Primary adrenal lymphoma is a rare phenomenon with a male preponderance. There is bilateral gland involvement in two thirds of cases. Histology usually reveals an aggressive diffuse large B-cell subtype. Adrenal insufficiency is a recognised complication. We present a 62 year old man with a 4 month history of weight loss, night sweats and malaise. Clinical examination revealed right renal angle tenderness with no palpable lymphadenopathy. Inflammatory markers were raised with a normocytic anaemia. He was hyponatraemic at 127 mmol/L with potassium of 5.4 mmol/L. Abdominal ultrasound raised the possibility of a right renal mass. Subsequent Computer Tomography (CT) identified bilateral necrotic adrenal masses, measuring 10.8 cm on the right and 9.4 cm on the left, with retroperitoneal lymphadenopathy. Microbiological screens for recurrent pyrexia were negative. There was no biochemical evidence of phaeochromocytoma, and screening for hyperaldosteronism and cortisol excess was negative. Short synacthen test demonstrated a blunted response to ACTH with basal cortisol of 230 nmol/L rising to 242 nmol/L at 30 minutes. CT-guided adrenal biopsy revealed lymphoid infiltrate with large pleomorphic cells consistent with diffuse large B cell lymphoma. Immunophenotyping was positive for BCL-2. There was no evidence of marrow infiltration on trephine biopsy. He was commenced on supplemental hydrocortisone and fludrocortisone for adrenal insuffuciency. Following 6 cycles of R-CHOP chemotherapy (Rituximab with Cyclophosphamide, Doxorubicin, Vincristine and Prednisolone), there had been a 50% reduction in adrenal mass size with diminution in lymphadenopathy on follow up imaging. Whilst rare, primary adrenal lymphoma is aggressive and requires rapid diagnosis and prompt initiation of treatment. Long term remission is rarely achieved. The diagnosis should be considered in cases of isolated adrenal enlargement.

Declaration of interest: There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.

Funding: No specific grant from any funding agency in the public, commercial or not-for-profit sector.

Figure 1. Computer Tomogrophy shows diffusely enlarged and necrotic adrenal glands.