Endocrine Abstracts

Background: Adrenocortical carcinoma (AC) is a rare malignancy (0.7–2 cases per million in Western countries) of the adrenal gland. AC can be associated with various syndromes and conditions, one of the rarest being polycythemia. Erythrocytosis refers to an increase in the number of circulating red blood cells. Secondary erythrocytosis, which is characterised by increased levels of Erythropoietin (EPO), can occur due to several causes, among which - hyperandrogenism and EPO-secreting tumors – both have been previously linked to AC. Although the association of AC and polycythemia is known, literature data concerning this comorbidity is lacking.

Case presentation: We present a 41-year-old male, assessed for new-onset hypertension, mild weight gain and abdominal fullness. On physical examination, he was hypertensive with central obesity. Initial laboratory tests revealed erythrocytosis (HgB 206 g/l, RBC 6,90×10¹²). Imagining of the adrenals showed a large 11.5×10.5 cm left adrenal mass, consistent with adrenocortical carcinoma. Further investigation revealed elevated late night serum cortisol, non-suppressible early morning cortisol after 1 mg dexamethasone, low adrenocorticotropic hormone (ACTH) and increased dehydroepiandrosterone (DHEA). Patient underwent erythropheresis, followed by adrenalectomy. A histopathological diagnosis of left adrenocortical carcinoma with vascular invasion was made. After surgery RBC and HgB returned to normal and hypercortisolism disappeared. The patient was later referred for subsequent management by oncologist.

Conclusions: This clinical case illustrates a rare presentation of AC. Awareness of this presentation may aid early assessment and timely management.

Keywords: Adrenal carcinoma; Erythrocytosis; Cushing disease.