What is a pheochromocytoma?
A pheochromocytoma is a tumor on the adrenal gland that secretes epinephrine and norepinephrine hormones.
- Epinephrine and norepinphrine are responsible for regulating heart rate and blood pressure, among other functions.
- Pheochromocytomas occur most frequently in young to middle-aged adults.
- Ten percent of people affected are children, who usually develop symptoms between the ages of 6 and 14.
At Boston Children's Hospital, pheochromocytomas are treated through the Division of Endocrinology — a multidisciplinary program that provides comprehensive diagnosis, treatment, and management for patients with disorders of the adrenal gland and other parts of the body associated with the endocrine system.
Pheochromocytomas | Symptoms & Causes
What are the symptoms of pheochromocytomas?
The most common symptom seen with a pheochromocytoma is high blood pressure, which is sometimes extreme. While each child may experience symptoms differently, some of the most common include:
- rapid pulse
- heart palpitations
- poor weight gain despite good appetite
- growth failure
- abdominal pain
- pale skin
- clammy skin
What causes pheochromocytomas?
In most cases, both genetic and environmental factors play a role. The condition can be seen on its own or in combination with other disorders. The most common disorders associated with pheochromocytomas are:
- Von Hippel-Lindau syndrome
- multiple endocrine neoplasia (MEN)
- tuberous sclerosis
- Sturge-Weber syndrome
Pheochromocytomas | Diagnosis & Treatments
How do doctors diagnose a pheochromocytoma?
In addition to a complete medical history and physical examination, some of the diagnostic procedures your child’s doctor may request include:
- blood and urine tests (to measure hormone levels)
- computerized tomography scan (also called a CT or CAT scan)
- radioisotope scan: uses radioactive substances introduced into the body to create an image of the functioning adrenal gland
How are pheochromocytomas treated?
Treatment for a pheochromocytoma usually includes removing the tumor.
- Before removing the tumor, your child's physician may prescribe medications to control high blood pressure.
- Your child may have multiple tumors, and in order to be thorough, we will conduct an extensive evaluation to locate them before surgery.
Continuous medical follow-up may be required to monitor the development of future tumors.