Basic information for topics, such as "What is it?" and "How many people are affected?" is available in the Condition Information section. In addition, Frequently Asked Questions (FAQs) that are specific to a certain topic are answered in this section.
The prognosis for patients with a completely removed tumor is excellent.1 The 5-year survival rate for patients with benign pheochromocytomas is between 86% and 92%.2
In patients with malignant pheochromocytomas, the 5-year survival rate may be less than 50%, although prospective studies are missing.3Life expectancy varies for patients with malignant pheochromocytoma depending on where the tumor has spread. Survival is generally less than 5 years when tumors have spread to the liver and lung and somewhat longer when tumors have spread to the bones.4
Pheochromocytoma can be inherited as part of another syndrome or may coexist with other diseases. Some patients with pheochromocytoma have the inheritable disease von Hippel-Lindau (VHL) syndrome or neurofibromatosis type 1 (NF1).5 Both VHL and NF1 are cancer syndromes in which patients have tumors at multiple sites.
Another disease in which pheochromocytoma is often present is multiple endocrine neoplasia type 2 (MEN2), which produces tumors in the endocrine glands, including the parathyroid gland.5 Approximately 50% of patients with MEN2 have pheochromocytoma.5
Pheochromocytomas are equally common in males and females.6
Pheochromocytomas are diagnosed in people of all races but are less common in African Americans.5
Approximately 30% of pheochromocytoma cases are inheritable and the remaining cases occur sporadically.5 Children are at risk of developing pheochromocytoma/paraganglioma if they inherit one of the known disease-causing mutated genes from their parents. The penetrance of the disease, or the percentage of individuals with a known genetic mutation who develop the disease, varies with different mutations.
Pregnancy may be complicated by the clinical signs of pheochromocytoma. In pregnant women, high blood pressure, cardiovascular problems, and seizures, which occur in patients with pheochromocytoma,6 could affect the health of the unborn child.
Yes. Genetic testing is available for pheochromocytoma. Genetic testing is especially recommended for some groups of patients who5:
Genetic testing may also be recommended for other patients depending upon family history, the patient's age, and the location of the patient's tumor.7
Disease screening should be offered to presymptomatic relatives who have a diagnosed mutation, especially because familial syndromes are also associated with other types of tumors and early diagnosis improves the prognosis of these patients. Patients with known genetic mutations should undergo regular periodic screening with biochemical testing and anatomic imaging (CT or MRI scans) to rule out the presence of disease.
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