Endocrine Abstracts

A 41 year old female presented to the haematologists with a coincidental finding of polycythaemia: haemoglobin 198 g/L, white blood cell count 8×10⁹/L, platelets 236×10⁹/L, haematocrit 0.57 L/L and red blood cell count 6.16×10¹²/L. Six years earlier haemoglobin 151 g/L, white blood cell count 7.7×10⁹/L, platelets 297×10⁹/L, haematocrit 0.4 L/L and red blood cell count 4.53×10¹²/L. She was a non-smoker taking no medications with no history of cardiovascular or respiratory diseases. Investigations: exon 12 of the JAK 2 gene and exon 9 of the CALR gene analyses were normal. Erythropoietin 15 mU/ml (5–25) was inappropriately normal for prevailing haemoglobin level. Ultrasound of abdomen suggested the presence of a right adrenal mass. CT imaging confirmed a hypodense 5.8×5.7×5.2 adrenal mass with peripheral heterogeneous enhancement. Endocrine investigations: 09:00 hours cortisol 241 nmol/L and ACTH 14 ng/L,16:00 hours cortisol 88 nmol/L, ACTH 6.3 ng/L, PRA 0.5 nmol/L/hr and aldosterone 56 pmol/L. Urinary metadrenalines×3 were normal as were plasma metadrenaline levels, haemoglobin 207 g/L, haematocrit 0.6 L/L, red blood cell count 6.43×10¹²/L and erythropoietin 19 mU/ml. MIBG whole body scan was negative. Right laparoscopic adrenalectomy resulted in a fall in erythropoietin, haemoglobin and red blood cell count to initial sub-normal levels with rapid normalisation. Histopathology examination suggested the adrenal lesion to be an unusual benign adreno cortical adenoma. Adrenal adenomas, carcinomas and phaeochromocytomas are commonly listed as erythropoietin secreting tumours. A literature search, whilst identifying numerous cases of polycythaemia associated with adrenal adenoma secreting cortisol and testosterone, failed to identify a single case secreting solely erythropoietin.