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Adrenocortical carcinoma



Overview

Adrenal glands and Kidney

There are two adrenal glands located one above each kidney; your kidneys are found deep inside the upper part of your abdomen. The adrenal cortex makes important hormones that:

  • Balance the water and salt in the body.
  • Help keep blood pressure normal.
  • Help manage the body's use of protein, fat, and carbohydrates.
  • Cause the body to have masculine or feminine characteristics.

There are 2 main types of adrenal cortex tumors:

  • Benign adenoma -- these tumors are not cancer
  • Adrenal cortical carcinoma -- these tumors are cancer
Adrenal glands

In most cases, adrenal cortical cancers are much larger than adenomas. If a tumor is larger than about 2 to 2-1/2 inches (5 or 6 centimeters), it is most likely cancer. The cause is unknown. Adrenal cortical carcinoma may be associated with an inherited cancer syndrome. Both men and women develop this tumor, but in women it is more often functional (secreting hormones). Adrenocortical carcinoma is typically an aggressive cancer and is usually found because excess hormone production causes symptoms which prompt patients to seek medical attention. Many patients will present with pain in the abdomen and flank (nearly all that don't present with symptoms of hormone excess will seek medical attention because of pain). Spread to distant organs (metastasis) occurs most commonly to the abdominal cavity, lungs, liver, and bone.
Although potentially curable at early stages, only 30% of these malignancies are confined to the adrenal gland at the time of diagnosis. Because these tumors tend to be found years after they began growing, they have the opportunity to invade nearby organs, spread to distant organs (metastasize) and cause numerous changes in the body because of the excess hormones they produce.
Adrenal cortical carcinomas can produce the hormones cortisol or aldosterone. The average age of those found with adrenal cancer is around 45 to 50, but it can happen at any age, even in children. It seems to occur more often in women.


Review Date: 3/28/2011

Reviewed By: The UT Health Science Center Surgical Oncology Faculty



Signs/Symptoms

  • Flushed rounded face with pudgy cheeks (moon face)
  • Obesity
  • Buffalo hump (rounded fatty hump high on the back just below the neck)
  • Short stature (impaired linear growth, may be less than 5th percentile)
  • Virilization: increased body hair (hypertrichosis), especially on face, pubic hair, acne, deepening of voice, enlarged clitoris (girls)
  • Findings suggestive of increased aldosterone production are symptoms of low potassium (weakness, muscle cramps, increased thirst, and urination).
  • High blood pressure

Some of the hormone problems have specific syndrome/condition names:

  • hypercortisolism (Cushing's syndrome) (excess cortisol produced)
  • adrenogenital syndrome (excess sex steroids produced)
  • virilization (acquisition of male traits in a female because of excess testosterone production)
  • feminization (acquisition of female traits in a male because of excess estrogen production)
  • precocious puberty (puberty occurring too early because of excess sex steroids produced)
  • hyperaldosteronism (Conn's syndrome) (excess aldosterone leading to hypertension and low potassium)
Diagnosis

  • Physical exam
  • 24-hour urine test
  • Adrenal angiography
  • Adrenal venography
  • PET scan
  • Blood work to test for high serum cortisol levels, low serum ACTH, low serum potassium, and high serum aldosterone
  • CT scan of the abdomen (may demonstrate a mass in the adrenal gland)
  • Abdominal X-ray
  • Abdominal MRI (may demonstrate a mass in the adrenal gland)

Please click here for complete National Cancer Institute (NCI) information on these diagnostic tests

Treatment

Adrenal glands and Kidney
  • Surgical treatment — Surgical treatment will be dictated by the patient's individual situation. Most small benign tumors can be removed using minimally invasive surgical techniques but larger tumors (larger than 10 centimeters), and particularly tumors known to be malignant, are rarely removed laparoscopically. A more formal open surgical exploration is generally required for a malignant adrenal tumor.

The preferred surgical technique is the thoracoabdominal (see image right). This approach allows the greatest exposure to the adrenal gland and all the surrounding structures and entails making a large incision which traverses both the abdominal and thoracic cavities ... including some the diaphragm which separates them. This large operation is reserved for only the largest and most malignant tumors of the adrenal gland in hopes of providing adequate exposure of the tumor and its blood supply to allow curative resection.

  • Chemotherapy — Adrenocortical carcinoma may not respond well to chemotherapy, but it is sometimes used to stop hormone production in the adrenal cortex and to relieve symptoms caused by the hormones. Other medications may be given to reduce production of cortisol which is responsible for many of the symptoms.
  • Radiation therapy — Use of radiation therapy depends on the size and stage of the tumors.
  • Biologics — Biologic therapy may be used. Biologics use the patient's own immune system to fight the cancer, boosting or even restoring the body's natural defenses to fight the disease.
  • Clinical trials — Patients might want to consider participating in a clinical trial. A reputable & responsible resource for current ongoing clinical trials is the National Cancer Institute's clinical trials website:
    Click here to go to the NCI clinical trials homepage.
Prevention

Unfortunately, there is no known way to prevent this form of cancer. Scientists have found a few risk factors that make a person more likely to get adrenal cancer, but even if a person does have one or more risk factors for adrenal gland tumors, we can't know for sure how large a part they played in causing the cancer. The cell mutations that occur inside the body cannot be predicted or halted. A parent who had or has adrenal cancer may make a child slightly more likely to develop adrenal cancer from inherited DNA that causes adrenal cancer cell division, but even a genetic predisposition to adrenal cancer has not been definitively proven.

Outlook

The outcome is dependent on how early the diagnosis is made and whether the tumor has spread (metastasized). It is hard to find adrenal cancer early. In most cases the tumor has grown quite large before it is found. It is often found earlier in children than in adults because children react more to the hormones these tumors make. In adults, these tumors may be found early by accident, for example when a CT scan is done for some other health problem. These tumors are often fatal within a few years.