DefinitionAdrenocortical carcinoma is a cancer of the adrenal glands.
Alternative NamesTumor - adrenal
Causes, incidence, and risk factorsAdrenocortical carcinoma is most common in children younger than 5 and adults in their 30s and 40s.
Adrenocortical carcinoma may be linked to a cancer syndrome that is passed down through families (inherited). Both men and women can develop this tumor. Adrenocortical carcinoma can produce the hormones cortisol, estrogen, or aldosterone. In women the tumor often releases the hormones, which can lead to male characteristics.
The cause is unknown. About 2 people per million develop this type of tumor.
SymptomsSymptoms that suggest increased cortisol production:
- Fatty, rounded hump high on the back just below the neck (buffalo hump)
- Flushed rounded face with pudgy cheeks (moon face )
- Obesity
- Stunted growth in height (short stature)
- Virilization - the appearance of male characteristics, including increased body hair especially on face, pubic hair, acne, deepening of voice, and enlarged clitoris (girls)
Symptoms that suggest increased aldosterone production are the same as symptoms of low potassium and include weakness, muscle cramps, increased thirst, and urination.
Signs and testsA physical exam reveals high blood pressure. Blood work will be done to check hormone levels:
Imaging tests may include:
TreatmentPrimary treatment is surgery to remove the tumor. Adrenocortical carcinoma may not improve with chemotherapy. Medications may be given to reduce production of cortisol, which causes many of the symptoms.
Expectations (prognosis)The outcome depends on how early the diagnosis is made and whether the tumor has spread (metastasized). Tumors that have spread often lead to death within 1 to 3 years.
ComplicationsThe tumor can spread to the liver, bone, lung, or other areas.
Calling your health care providerCall your health care provider if you or your child has symptoms of adrenocortical carcinoma, Cushing syndrome, or failure to grow.
ReferencesLal G, O'Dorisio T, McDougall R, Weigel RJ. Cancer of the endocrine system. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKena WG, eds. Clinical Oncology. 4th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 75.