Endocrine Abstracts

A 44-year old male patient with HIV-1 stage C2 presented with intermittent fever and weight loss (10 kg in 3 months). He was being treated with tenofovir/emtricitabine and atazanavir, and had a CD4 levels of 705/mm³ and HIV viral load of 1400 copies/ml. Upon admission, the complete blood panel showed anaemia and thrombocytopenia (haemoglobin: 75 g/l, platelets: 98 G/l), normal white blood cell count, normal hepatic tests and renal function, normal electrolytes and an elevated CRP (85 mg/l) and erythrocyte sedimentation rate (990 mm). Abdominal CT-scan showed bilateral adrenal tumours, 10×9×15 cm on the right and 11×8×6 cm on the left, without invasion of adjacent structures. PET-CT scan showed intense hypermetabolism of the adrenal masses. Bone morrow biopsy and flow cytometry were negative for lympho-proliferative disease. Plasma fractionated free metanephrines were normal, excluding pheochromocytoma. The patient had no physical signs of Cushing disease and morning plasma cortisol was 291 nmol/l. An adrenal biopsy was performed which showed primary effusion lymphoma, solid variant. The patient received chemotherapy treatment (associated with high dose glucocorticoids) which led to clinical improvement and shrinkage of the adrenal masses (4×4 cm on the right and 3×2 cm on the left) at 4 months follow-up. Nevertheless, PET hypermetamolism of the adrenals persisted and right adrenalectomy was decided in order to rule out persistent disease. At that time, the patient had primary adrenal insufficiency, with low plasma cortisol levels (18 nmol/l in the morning and 211 nmol/l 1-h after injection of ACTH 250 mg) and high morning ACTH (75 ng/l). Histology confirmed disease remission. Differential diagnosis of bilateral adrenal masses includes bilateral pheochromocytoma, adrenal metastasis, bilateral adrenal carcinoma, haemorrhage, infection, congenital adrenal hyperplasia etc. Primary adrenal lymphoma is a rare cause, but is associated with adrenal insufficiency in more than 50% of cases. This is a rare case of extracavitary primary effusion lymphoma, solid variant, often associated with HHV8 infection, which was also found in our patient.