Mutations of VHL tumor suppressor gene are responsible for the development of a variety of tumors including clear cell renal cell carcinoma (RCC), pheochromocytomas, endolymphatic sac tumors, and pancreatic cysts [13], even though only loss of VHL tumor suppressor gene is not enough to induce RCC [14]. The gene discussed is VHL; the disease is pheochromocytoma.