The cause of pheochromocytoma is unknown. However, about a third of cases occur in people who have family members with the disease.
Pheochromocytoma tumors are more likely to occur in people who have inherited conditions or disorders from their parents. The most common disorders that can increase the risk of developing pheochromocytoma tumors are: multiple endocrine neoplasia type II, Von Hippel-Lindau disease, neurofibromatosis 1, and hereditary paraganglioma syndrome. These potentially inherited cases are more commonly cancerous.