Unusual Cancers of Childhood (PDQ®): Treatment - Patient Information [NCI] - Other Rare Unusual Cancers of Childhood
There are several types of MEN syndrome, and each type may need different treatment:
- Patients with the MEN1 syndrome are treated for parathyroid, pancreatic and pituitary tumors.
- Patients with the MEN2A syndrome usually have surgery to remove the thyroid by age 5 or earlier if genetic tests show certain gene changes. The surgery is done to diagnose cancer or to prevent cancer from forming or spreading.
- Infants with the MEN2B syndrome may have the thyroid removed to prevent cancer.
- Patients with Hirschsprung disease and certain gene changes may have the thyroid removed to prevent cancer.
- A clinical trial of targeted therapy with a tyrosine kinase inhibitor for medullary thyroid cancer.
Pheochromocytoma and Paraganglioma
Pheochromocytoma and paraganglioma are rare tumors that come from the same type of nerve tissue.
- Pheochromocytoma forms in the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla. Pheochromocytoma is a tumor of the adrenal medulla. The adrenal glands make important hormones called catecholamines. Adrenaline (epinephrine) and noradrenaline (norepinephrine) are two types of catecholamines that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Some pheochromocytomas release extra adrenaline and noradrenaline into the blood and cause symptoms of disease.
- Paraganglioma forms outside the adrenal glands near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body. Some paragangliomas make extra catecholamines called adrenaline and noradrenaline. The release of extra adrenaline and noradrenaline into the blood may cause symptoms of disease.
Risk Factors, Symptoms, and Diagnostic and Staging Tests
Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor doesn't mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk.
The risk of pheochromocytoma or paraganglioma is increased by having any of the following inherited syndromes or gene changes:
- Multiple endocrine neoplasia 1 syndrome.
- Multiple endocrine neoplasia 2 syndrome (MEN2A and MEN2B).
- von Hippel-Lindau disease (VHL).
- Neurofibromatosis type 1 (NF1).
- Carney-Stratakis dyad (paraganglioma and gastrointestinal stromal tumor [GIST]).
- Carney triad (paraganglioma, GIST, and pulmonary chondroma).
- Changes in certain genes including SDHD, SDHB, SDHA, and TMEM127.
More than half of the children and adolescents diagnosed with pheochromocytoma or paraganglioma have an inherited syndrome or gene change that increased the risk of cancer. Genetic counseling (a discussion with a trained professional about inherited diseases) and testing is an important part of the treatment plan.