Adrenocortical Carcinoma

Adrenocortical Carcinoma


Adrenocortical carcinoma or adrenal cancer is a rare type of cancer that develops in one or both of the adrenal glands. The adrenal glands are responsible for hormone production for all parts of the body. Adrenocortical carcinoma most commonly affects young children or adults in their mid-forties or mid-fifties. Adrenocortical carcinoma is curable if identified early.

The adrenal gland

The adrenal gland is a paired organ located above each one of our kidneys. It produces various hormones, such as adrenaline, cortisol, and aldosterone. Each adrenal gland has a cortex and a medulla, responsible for producing different types of hormones. Some of them are the basis for the production of the male and female hormones. Therefore, adrenal cancer might present with a heterogeneous clinical picture, depending on the type of hormone produced in excess. Sometimes, the symptoms and signs of adrenal cancer may overlap with other conditions of the adrenal glands or the human body in general.

How common is adrenocortical carcinoma?

Adrenocortical carcinoma is rare, and it affects around 200 people per year in the US. The five-year survival rate depends on the tumor’s SEER stage. If the cancer is localized, the five-year survival rate is about 74%. The all-SEER-stages-combined five-year survival rate is 51%. Regional adrenocortical carcinomas have a five-year survival rate of 56%, and for distant ones, it drops to 37%.

What is the prognosis of adrenocortical carcinoma?

The prognosis of adrenocortical carcinoma depends on its stage. In general, adrenal cancer spreads quickly to nearby blood vessels and the rest of the body. When the tumor is functional, the diagnosis might be early due to symptoms and signs. However, non-functional tumors might not be identifiable until they spread to other tissues. Distant metastasis of adrenocortical carcinoma means a poor prognosis. However, adrenal cancer is curable if identified early.

What is the cause of adrenocortical carcinoma?

Scientists are not sure what causes adrenocortical carcinoma. They believe that some mutations or changes in the DNA of some cells might be responsible for their cancerous transformation. Adrenocortical carcinoma can generally run in the families. Individuals with a family history of adrenal cancer should do regular screenings to identify early a potentially malignant tumor. Some genetic conditions might significantly raise your chances of developing adrenocortical carcinoma. The following are some of them:

  • Beckwith-Wiedemann syndrome
  • Familial adenomatous polyposis (FAP)
  • Hereditary nonpolyposis colorectal cancer (HNPC)
  • Li-Fraumeni syndrome
  • Multiple endocrine neoplasias (MEN1)

Risk factors for adrenocortical carcinoma

As risk factors for adrenocortical carcinoma, doctors suggest that some genetic syndromes increase your risk for developing adrenal cancer. The following are some of the most well-established ones:

  • Li-Fraumeni syndrome
  • Beckwith-Wiedemann syndrome
  • Von Hippel-Lindau disease
  • Multiple endocrine neoplasias (types 1 and 2)
  • Familial adenomatous polyposis (FAP)
  • Lynch syndrome (hereditary nonpolyposis colorectal cancer)
  • Carney complex

Most people with one of these genetic syndromes do not develop cancer, but they most commonly present with an adrenal tumor.

Symptoms and signs of adrenocortical carcinoma

Adrenocortical carcinoma may present with some of the following symptoms and signs:

  • Weight gain or loss
  • Feeling your muscles weak
  • Newly formed pink or purple stretch marks on your skin
  • Excess facial hair in women
  • Hair loss on the head in women
  • Irregular periods
  • Enlarged breasts in men
  • Shrinking testicles
  • Nausea and vomiting
  • Abdominal bloating and gas
  • Back pain
  • Fever
  • Loss of appetite

Some adrenal tumors are functional, meaning they produce excessive levels of a hormone. For example, too much aldosterone might lead to high blood pressure, frequent urination, and muscle weakness. Excessive cortisol production might lead to central gain weight, high levels of blood sugar, easy bruising, high blood pressure, pink or purple stretch marks on the skin, extra fat on the back of the neck, and round and red face. Excessive levels of estrogen in men might lead to breast growth and erectile dysfunction. Women might experience irregular periods or vaginal bleeding. Finally, too much testosterone might lead to hair loss on the head or facial hair in women, along with a deeper voice. Men might not experience any changes.

Types of adrenal tumors

Adrenal tumors may be benign or malignant. The following are some of the main types of adrenal tumors:

  • Adenomas
  • Adrenocortical carcinomas
  • Neuroblastomas
  • Pheochromocytoma

Diagnosis of adrenocortical carcinoma

To diagnose adrenal carcinoma, your doctor will take your history and perform a physical examination. The tests that she or he will perform to you will help to exclude other causes and establish a differential diagnosis. To check your hormone levels and see if there are any abnormalities, you will have to undergo a blood exam and a urine test. Your doctor might suggest some imaging tests, such as CT, positron emission tomography (PET), or MRI scan, to see if there is any metastasis, and to stage the tumor. Laboratory analysis of your adrenal gland might be necessary if your doctor suspects you have adrenocortical carcinoma. To do that, the removal of your adrenal gland is essential.

What is the treatment for adrenocortical carcinoma?

Surgery is the best treatment option for adrenocortical carcinoma. Other therapies are also beneficial to prevent the recurrence of the tumor. Some patients may have contraindications regarding operations and might need an alternative treatment. However, surgery is the only method that can treat adrenocortical carcinoma. Other therapeutic interventions that might be necessary are the following:

  • Pharmacological treatment with Mitotane to reduce the risk of recurrence after surgery
  • Radiation therapy to kill any remaining cancer cells
  • Chemotherapy, orally or intravenously, to chemically kill any remaining cancerous cells


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