What Is the Survival Rate of Adrenal Cancer?

Three quarters of people diagnosed with early-stage adrenal cancer are still alive five years after diagnosis. For people in whom the cancer has spread to nearby lymph nodes, that five-year survival rate drops to a little more than half. Distant metastases cut the rate even further to 37%.

Three quarters of people diagnosed with early-stage adrenal cancer are still alive five years after diagnosis. For people in whom the cancer has spread to nearby lymph nodes, that five-year survival rate drops to a little more than half. Distant metastases cut the rate even further to 37%.

Adrenal cancer occurs when cells in the adrenal glands, small glands that sit above each of the kidneys, start to grow out of control. 

There are two main types of adrenal cortex tumors:

• Adenomas (benign or non-cancerous tumors): most tumors are adenomas 
• Carcinomas (malignant or cancerous tumors): rare

Symptoms of adrenal tumors vary depending on whether they are caused by the hormones produced by the tumor, or by the tumor itself growing so large it pushes against other nearby organs. About half the symptoms are caused by hormones and the other half are caused by tumor growth. 

Symptoms of adrenal tumors caused by androgen or estrogen production include: 

• Symptoms of excess sex hormones are more pronounced in children who have not yet gone through puberty
  • Androgens (male hormones) cause: 
    • Excessive growth of facial and body hair (such as in the pubic and underarm areas) in children
    • Enlarged penis in boys or enlarged clitoris in girls
  • Estrogens (female hormones) cause: 
    • Early puberty in girls
      • Breast development (can also occur in boys)
      • Early menstrual periods
• Symptoms of excess sex hormones in adults are easier to notice if the tumor is making the hormone usually found in the opposite sex
  • Symptoms in men with tumors that produce estrogen include
    • Breast enlargement and tenderness 
    • Sexual problems such as erectile dysfunction (impotence) and loss of sex drive
  • Symptoms in women with tumors that produce androgens (male hormones) include
    • Excessive facial and body hair growth
    • Receding hairline
    • Irregular menstrual periods
    • Deepening of their voice

Symptoms of adrenal tumors caused by cortisol production include Cushing’s syndrome:

• Weight gain, usually most above the collar bone, in the cheek area (moon face), and around the abdomen
• Fat deposits behind the neck and shoulders (fatty hump or buffalo hump)
• Purple stretch marks on the abdomen
• Excessive hair growth on the face, chest, and back in women
• Menstrual irregularities
• Weakness and loss of muscle mass in the legs
• Easy bruising
• Depression and/or mood swings
• Weakened bones (osteoporosis), which can lead to fractures
• High blood sugar levels (hyperglycemia), that can lead to diabetes
• High blood pressure (hypertension)

Symptoms of adrenal tumors caused by aldosterone production include:

• High blood pressure
• Weakness
• Muscle cramps
• Low blood potassium levels

Symptoms caused by a large adrenal cancer pressing on nearby organs:

• Pain near the tumor
• Feeling of fullness in the abdomen
• Trouble eating due to feeling of being full

The exact cause of adrenal cancer is unknown, though it is believed to be due to genetic changes. The cause of these changes is not understood. 

Risk factors for developing adrenal cancer include: 

• Genetic syndromes
  • Li-Fraumeni syndrome
  • Beckwith-Wiedemann syndrome
  • Multiple endocrine neoplasia (MEN1)
  • Familial adenomatous polyposis (FAP)
  • Lynch syndrome or hereditary nonpolyposis colorectal cancer (HNPCC)
• Lifestyle and environmental factors (these are also risk factors for many types of cancer)
  • Being overweight
  • Smoking
  • Sedentary lifestyle
  • Exposure to cancer-causing substances in the environment

Adrenal cancer is diagnosed with a patient history and physical examination. Tests used to help diagnose adrenal cancer include: 

• Imaging tests
  • Chest x-ray
  • Ultrasound
  • Computed tomography (CT)
  • Magnetic resonance imaging (MRI)
  • Positron emission tomography (PET)
• Laparoscopy
• Biopsy
• Blood and urine tests for adrenal hormones
  • Tests for high cortisol levels
  • Tests for high aldosterone levels
  • Tests for high androgen or estrogen levels

• Surgery 
  • Removal of the adrenal gland (adrenalectomy)
• Radiation therapy 
  • External beam radiation therapy 
  • Brachytherapy (internal radiation therapy) 
• Chemotherapy 
  • Mitotane (Lysondren)
  • Combination of cisplatin, doxorubicin (Adriamycin), and etoposide (VP-16) plus mitotane
  • Streptozocin plus mitotane
  • Chemo drugs used less often:
  • Paclitaxel (Taxol)
  • 5-fluorouracil (5-FU)
  • Vincristine (Oncovin)
• Other drugs
  • Ketoconazole and metyrapone to reduce adrenal steroid hormone production and help relieve symptoms caused by these hormones, but they do not shrink the cancer
  • Drugs that block the effects of the hormones made by the tumor include:
    • Spironolactone (Aldactone): decreases effects of aldosterone
    • Mifepristone (Korlym): decreases cortisol effects
    • Tamoxifen, toremifene (Fareston), and fulvestrant (Faslodex): block the effects of estrogen

Life expectancy for adrenal cancer is often expressed in five-year survival rates, that is, how many people will be alive five years after diagnosis. 

Adrenal cancer five-year survival rates:

• Localized (no sign the cancer has spread outside the adrenal gland): 74% 
• Regional (cancer has spread outside the adrenal gland to nearby structures or lymph nodes): 56% 
• Distant (cancer has spread to distant parts of the body such as the liver or lungs): 37%