Patient Education - Endocrine Encyclopedia
Endocrine Surgery Encyclopedia
Definition:
Adrenocortical carcinoma is a malignant tumor of the adrenal glands.
Alternative Names: Tumor - adrenal
Causes, incidence, and risk factors:
Adrenocortical carcinomas can produce the hormones cortisol or aldosterone. The disease
is most common in two age groups: children younger than 5, and adults in their 30s
and 40s.
Adrenocortical carcinoma may be associated with an inherited cancer syndrome.
Both men and women develop this tumor. In women it is often a functional tumor (one
that secretes hormones).
The cause is unknown. About 2 people per million develop this type of tumor.
Symptoms:
Findings suggestive of increased cortisol production:
- 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).
Signs and tests:
- High blood pressure
- High serum cortisol levels
- Low serum ACTH
- Low serum potassium
- 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)
Treatment:
Primary treatment consists of surgical removal of the tumor. Adrenocortical carcinoma
may not respond well to chemotherapy. Medications may be given to reduce production
of cortisol which is responsible for many of the symptoms.
Support Groups:
Expectations (prognosis):
The outcome is dependent on how early the diagnosis is made and whether the tumor
has spread (metastasized). Metastatic tumors are often fatal within a few years.
Complications:
A complication is metastasis (frequently to the liver, bone, and lung).
Calling your health care provider:
Call your health care provider if you or your child has symptoms of adrenocortical
carcinoma, Cushing's syndrome, or failure to grow.
Prevention:
Review Date: 4/15/2005
Reviewed By: Rita Nanda, MD, Department of Medicine, Section of Hematology/Oncology,
University of Chicago Medical Center, Chicago, IL. Review provided by VeriMed Healthcare
Network.
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