Papillary Endothelial Hyperplasia of the Adrenal Gland: Report of a Case and Review of the Literature

Abstract

Papillary endothelial hyperplasia (PEH) is a benign vascular proliferative process most frequently seen in the skin and integument, but may involve any of the visceral organs. It is a rare entity, with less than 30 cases of visceral PEH described in the literature. Adrenal papillary endothelial hyperplasia is an exceedingly rare process and is the basis of this review. A 66-year-old female was referred for evaluation of an asymptomatic 6 cm right adrenal mass. Computed tomography indicated that the lesion was solid and hypervascular. After appropriate workup, the patient underwent laparoscopic adrenalectomy. The pathologic analysis was consistent with adrenal PEH. The patient recovered without incident and is doing well at 1 year follow-up. A review of the world's literature on papillary endothelial hyperplasia (PEH), and in particular adrenal PEH, yields five previous reports of this entity, and no comprehensive review. A compilation of the now six patients with adrenal PEH reveals several common features: five of six patients were female and mean age was 64 years. The disease radiologically mimics adrenal cortical carcinoma mandating a surgical oncological technique. Pathologic differentiation from angiosarcoma can be a difficult task requiring evaluation by an experienced pathologist.