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Adrenocortical Tumors (ACT) Disease Information


IPACTR

Adrenocortical tumors (ACT) are rare tumors (growths) that start in the adrenal glands. These glands sit on top of each kidney. Healthy adrenal glands produce many hormones that are important for body functions, such as:

If a person has ACT, the tumor cells can make too much of some adrenal hormones. The extra hormones can change how the body works. If your child has ACT, she might have a variety of body changes caused by too much of one or more adrenal hormones. Often, the changes caused by too much adrenal hormone are the first sign that a child has ACT.

Drawing of adrenocortical tumor on the adrenal glands/kidney

ACT can be different sizes depending on when the tumor is found. Large tumors are harder to treat. Tumors can be carcinomas (cancer, which are more aggressive tumors) or adenomas (not cancer, which are less aggressive). Carcinomas, but not adenomas, can spread to organs near the adrenal glands, such as the kidneys or liver. They can also spread to organs far away, such as the lungs and bones. Scientists do not know what causes ACT. They do know that these tumors can happen in children with or without a family member with cancer. About 2 out of every 3 children with ACT has a mutation (change) in a gene called TP53. This mutation is sometimes passed down (inherited) from one parent.

How Common is ACT?

Signs and Symptoms

Diagnosis and Treatment

Current Research