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.
The cells in the adrenal cortex make important hormones that help your body work properly. When cells in the adrenal cortex become cancerous, they may make too much of one or more hormones, which can cause symptoms such as high blood pressure, weakening of the bones, or diabetes. If male or female hormones are affected, the body may go through changes such as a deepening of the voice, growing hair on the face, swelling of the sex organs, or swelling of the breasts. Cancers that make hormones are called functioning tumors. Many cancers of the adrenal cortex do not make extra hormones and are called nonfunctioning tumors.
There are three primary treatment options for patients with cancer of the adrenal cortex. Local therapy to remove the tumor. Tissues around the tumor and nearby lymph nodes may also be removed during the operation.
When treating cancer of the adrenal cortex, a doctor may take out the adrenal gland in an operation called an adrenalectomy. Tissues around the adrenal glands that contain cancer may also be removed. Lymph nodes in the area may be removed as well (lymph node dissection).
Treatment with drugs that kill cancer cells. Most anticancer drugs are injected into a vein or muscle; some are given by mouth. Chemotherapy is a systemic treatment, meaning that the drugs flow through the bloodstream to nearly every part of the body to kill cancerous cells. It is generally given in cycles; a treatment period is followed by a recovery period, then another treatment period, and so on.
Treatment with high-energy rays that damage cancer cells and stop them from growing and dividing. It is a local therapy that only affects cancer cells in the treated area. Radiation may come from a machine (external radiation) or from an implant placed directly into or near a tumor (internal radiation). External radiation is typically the method used for cancer of the adrenal cortex. Besides treatment for the cancer itself, a patient with cancer of the adrenal cortex may also receive therapy to prevent or treat symptoms caused by the extra hormones that are made by the cancer.