Disease Information

Solid Tumor: Adrenocortical Carcinoma

Alternate Names: None

Definition

Adrenocortical carcinoma is a very rare tumor that grows from a gland that is located on top of the kidney – the adrenal gland. This gland normally produces hormones that help to keep our body in balance. When a tumor grows from this gland, it may also produce these hormones, but at an increased rate. For this reason children with this tumor may present with a variety of signs due to excess of hormone production. Some of these signs may simulate puberty – axillaries and pubic hair, increase in the size of the genitals, changing in the voice, etc. It is also common that the child's appetite will increase his/her appetite and his/her weight.

To learn more about adrenocortical carcinoma, see the International Pediatric Adrenocortical Tumor Registry (IPACTR).

Incidence

• Adrenocortical carcinoma is a very rare disease occurring in less then 0.2 percent of all childhood cancers.
• Tumors arising from the adrenal gland can also grow in adults, but those tumors are considered different diseases with different behaviors.

Influencing Factors

• The cause of adrenocortical carcinoma is unknown.
• Sometimes it may appear in families.

Treatment Strategies

• Patients with suspected adrenocortical carcinoma should be referred to a specialized center.
• Complete removal of the tumor by surgery is the best chance of cure.
• It may be necessary to complete treatment with chemotherapy and/or radiation therapy.

Current Research

• St. Jude is currently participating in an international study in which researchers are investigating a new surgery technique associated with adjuvant chemotherapy treatment.
• Researchers are growing tumor cells in cultures to study new drugs for treatment.
• St. Jude participated in the discovery of a new gene mutation in some patients. An epidemiological genetic study is being held to understand more about this gene mutation and its role to the incidence, early diagnosis and treatment of disease.

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