Advances in management of pheochromocytoma – a short review

Abstract

Pheochromocytoma is a rare neuroendocrine neoplasm. It is characterized by overproduction of catecholamines, which causes clinical symptoms associated with elevated blood pressure values, and can even lead to life-threatening complications. The tumor can be associated with genetic syndromes such as multiple endocrine neoplasia type 2 (MEN-2) or von Hippel-Lindau syndrome (VHL), and currently available and constantly evolving genetic testing makes it possible to detect the inherited form and plan appropriate therapy. Management of pheochromocytoma is based on initial laboratory diagnosis, confirmation by imaging studies, determination of hormonal activity and resulting therapy. Surgical resection by laparoscopic approach is the most recommended. For unresectable tumors or advanced disease with distant metastases, systemic therapies under development currently allow the cure or inhibition of tumor progression. In this paper, we will review advances in management of pheochromocytoma over the past decade and potential directions for future research.