Endocrine Abstracts

Introduction: Bilateral adrenal masses may appear in the context of metastatic disease, adrenal congenital hyperplasia, primary tumors or infections, among others. Primary adrenal lymphoma is rare, accounting for approximately 1% of cases of non-Hodgkin lymphoma.

Clinical case: 47-year-old woman, with a history of erythema nodosum and left hemithyroidectomy in the context of benign nodular disease. She presented with persistent fever and tiredness. An abdominal ultrasound showed bilateral adrenal nodular lesions. An abdominal CT confirmed the presence of bilateral adrenal masses, the one on the right with 129x63mm and the one the left with 113x63mm, with relative and absolute washouts of 16% and 40%, respectively. Suspicious retroperitoneal adenopathies were also present. The patient presented no signs or symptoms suggestive of pheochromocytoma, hypercortisolism or hyperandrogenism and physical examination showed cutaneous hyperpigmentation. Bloodwork revealed: ACTH 1964 (7.2-63.3) pg/ml, plasma cortisol 6.03 (5.0-25.0) ug/dl, immeasurable levels of testosterone and estradiol, DHEA-S 3.16 (35-256) ug/dl, and normal urinary metanephrines and 17-OH progesterone. She started therapy with 20mg of hydrocortisone daily. In order to study the possibility of adrenal metastasis of a primary occult tumor, an 18F-FDP-PET/CT was performed, that showed bilateral malignant involvement of the adrenal glands as well as pelvic and axillary lymph node metastasis. Given the absence of a primary occult tumor and hormonal hypersecretion, and taking into account the rarity of bilateral adrenal carcinomas, primary adrenal lymphoma presented as a possible diagnosis. Additional bloodwork showed elevated β2-microglobulin [6.03 (<3) ug/dl], LDH [421 (67-248) U/l] and ferritin [293 (10-120) ug/l]. A left adrenal biopsy confirmed the diagnosis of large cell diffuse B lymphoma. The patient was started on chemotherapy, with significant improvement.

Conclusions: Although rare, adrenal lymphoma, whether primary or secondary, must be thought of as a possible diagnosis in the presence of bilateral adrenal masses with suspicious characteristics on imaging studies. Clinical and analytical evaluation is essential.