Pheochromocytoma in Children

Pheochromocytoma is a tumor that grows on the adrenal glands, which lie on top of the kidneys. The tumor's cells secrete hormones such as epinephrine and norepinephrine. When present in normal amounts, these hormones help to regulate heart rate and blood pressure. In excessive amounts, these hormones cause: 

• Very high blood pressure
• Rapid heartbeat or palpitations
• Excessive sweating
• Severe headaches

10 percent of those affected by this disease are children. Symptoms usually appear between the ages of 6 and 14.

Risk Factors 

Factors that may increase your child’s chance of pheochromocytoma include:

• Family history of the disease
• Tumors in other glands of the body
• Hormonal disorders

 Genetic diseases associated with pheochromocytoma include:

• Von Hippel-Lindau disease
• Multiple endocrine neoplasia, type 2
• Neurofibromatosis type 1
• Paraganglioma syndromes 

Symptoms of Pheochromocytoma

Your child may not have any symptoms. In children, pheochromocytoma can cause:

• High blood pressure
• Severe headaches
• Excessive sweating
• Warmth, flushing
• Fast heart rate and pounding heartbeat
• Sensation of a panic attack
• Blurred vision
• Nausea, vomiting, constipation, or diarrhea
• Chest pain
• Involuntary trembling
• Pain in the lower chest or upper abdomen
• Increased appetite
• Weight loss
• Excessive thirst and urination
• Insomnia
• Tingling, burning, or numbness in the legs and feet
• Shortness of breath
• Muscle weakness
• Anxiety
• Trouble coping with high temperature

Diagnosis & Treatment at UVA Health Children's

Your child’s doctor will need to test urine and blood for metanephrines (a type of hormone). If necessary, your child's doctor will order a clonidine suppression test — a blood test to test certain hormone levels after an oral dose of clonidine hydrochloride— if these tests do not confirm the diagnosis. 

Your child might need further tests to see if the tumor has spread, including:

• CT scan
• Ultrasound
• MRI scan
• MIBG scintiscan, or adrenal medullary imaging
• PET scan

Treatments for Pheochromocytoma

Laparoscopic adrenalectomy

This procedure uses small incisions and special instruments to remove the tumor and, depending on the size of the tumor, the adrenal glands as well.

Chemotherapy and radiation therapy    

If the tumor is cancerous and has spread outside the adrenal glands, your child's doctor may recommend chemotherapy and radiation therapy.