Adrenal cancer is a rare cancer that originates in the adrenal glands. The prognosis varies based on the cancer’s stage at diagnosis.

Adrenocortical carcinoma (ACC) starts in the adrenal cortex and causes symptoms related to excessive hormone production or the tumor’s size. Diagnosis typically occurs when someone experiences symptoms or when imaging, for other purposes, finds the tumor.

Survival rates vary based on the cancer’s aggressiveness and stage at diagnosis and can change as diagnosis treatment strategies develop. The main treatment at most stages is surgical removal. Doctors may also use chemotherapy drugs and radiation. While there is a potential for cure, there is also a chance that the cancer recurs or becomes metastatic.

This article will discuss adrenal cancer’s prognosis, definition, symptoms, and diagnosis, as well as review current treatment options.

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Cancer’s aggressiveness, as well as when a person receives a diagnosis, will affect someone’s adrenal cancer prognosis.

Statistics

ACC, or adrenal cancer, can occur at any age, but peaks before age 5 and between 30–40 years of age. It is a rare cancer, with experts estimating an annual, worldwide incidence rate of 0.5 to 2 individuals per million population.

It is important to know that prognosis statistics can change as diagnosis and treatment develop.

Adrenal cancer diagnosis usually occurs in advanced stages, which can result in a poor prognosis. The median survival is about 4 years.

Survival rates depend on the cancer’s stage at diagnosis, and estimates can vary. The American Cancer Society (ACS) offers the following 5-year survival rates based on people with a diagnosis between 2012 and 2018:

  • localized (limited to the adrenal glands): 73%
  • regionalized (advanced to nearby areas): 53%
  • distant (advanced to distant areas): 38%

There is a chance for a cure, but 50–80% of people, who have their tumor surgically removed, develop metastatic or recurrent disease.

A note about sex and gender

Sex and gender exist on spectrums. This article will use the terms “male,” “female,” or both to refer to sex assigned at birth. Click here to learn more.

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Adrenal cancer is a rare form of cancer that starts in the adrenal glands. While adrenal tumors are present in 3–10% of the general population, these tumors are usually benign, or noncancerous, adenomas. Cancerous tumors found in the adrenal glands commonly come from another cancer that has spread.

ACC, also called adrenal cancer, is a cancer that originates in the outer part of the adrenal glands, called the adrenal cortex.

Pheochromocytomas, which are typically benign tumors, and neuroblastomas can also start in the adrenal glands.

Learn more about adrenal cancer.

Adrenal cancer can cause symptoms due to hormone overproduction or tumor size.

Symptoms due to hormones

Adrenal tumors can secrete male-type hormones, called androgens, or female-type hormones, called estrogens. Hormone-related symptoms are more noticeable in children than adults.

A child’s symptoms may include:

  • excessive facial or body hair
  • an enlarged penis or clitoris
  • enlarged breasts
  • early puberty

Symptoms for adults may include erectile dysfunction and a decreased sex drive. Males may experience tender or enlarged breasts, and females may experience excessive hair growth, a deepening voice, a receding hairline or balding, and irregular menstruation.

Adrenal cancer can also cause Cushing’s syndrome or excessive cortisol levels. However, cancer is not the only cause of Cushing’s syndrome.

Symptoms of Cushing’s syndrome include:

  • weight gain, especially seen in the upper body
  • extra fat on the upper back and above the collarbone and a rounded face
  • high blood sugar
  • high blood pressure
  • weakened or thin bones
  • muscle weakness and loss of muscle mass
  • easily bruising skin
  • purple stretch marks, typically on the abdomen or under the arms
  • depression and difficulty thinking clearly
  • excess facial hair in females

On rare occasions, cancerous adrenal tumors will produce aldosterone, which may cause weakness, muscle cramps, high blood sugar, or low potassium levels.

Learn more about Cushing’s syndrome.

Symptoms due to tumor size

If a tumor is large, it can put pressure on surrounding organs. This can result in abdominal pain, a feeling of fullness, or difficulty eating until full.

Diagnosis usually occurs at advanced stages when a person approaches a doctor with symptoms or when imaging for other medical issues finds an adrenal tumor.

A doctor usually begins by reviewing a person’s medical history and completing a physical exam. An exam involves looking for a tumor and conducting tests to measure hormone levels and other indicators.

A doctor will order imaging if they suspect a tumor is present. Imaging allows a doctor to determine factors, such as tumor location, size, and potential spread. Several imaging options include:

The next step is usually surgery, in order to perform a biopsy on the suspected tumor. A biopsy allows doctors to determine if a tumor is cancerous and involves examining a piece of the removed tissue.

Biopsies typically occur after surgical removal, because taking a tissue sample without removing the tumor may allow any cancer that is present to spread.

Current treatment options include surgery, chemotherapy, and radiation. Some doctors may combine multiple treatment methods.

Surgical removal is the foundational treatment for earlier stages. A doctor will try to remove all of the cancer, including the affected adrenal gland and sometimes the lymph nodes. In late stages, when the cancer has spread, a doctor may try to remove parts of the cancer. Chemotherapy or radiation therapy may follow surgery.

Chemotherapy

Chemotherapy is another treatment option. Mitotane is the most common chemotherapy drug for adrenal cancer. While mitotane can kill cancer cells, it has a response rate of around 30% and can cause toxicity and adverse effects.

Radiation therapy

Doctors may also use radiation therapy to treat adrenal cancer. Radiation aims to kill cancer cells with high-energy radiation. It is typically not a primary treatment, and a doctor may use radiation to inhibit cancer growth or lessen the severity of symptoms.

A doctor may use other drugs or treatment options, such as radiofrequency ablation (RFA) and peptide receptor radionuclide therapy (PRRT).

Adrenocortical carcinoma, or adrenal cancer, is a rare cancer that forms in the adrenal glands. Diagnosis generally occurs in advanced stages, which affects prognosis. Surgical removal can lead to a cure, but recurrent or metastatic disease is also possible. The 5-year survival estimates for localized, regionalized, and distant adrenal cancer are 73%, 53%, and 38%, respectively.

Symptoms are usually due to a tumor’s hormone production or its size. Excessive hormone secretion can cause excessive facial or body hair, early puberty, tender or enlarged breasts in males, and voice deepening, balding, and irregular menstruation in females. Someone may also experience Cushing’s syndrome symptoms. When the tumor is large, it can press on surrounding tissues and cause pain, a feeling of fullness, and difficulty eating until full.

Diagnosis usually happens when someone approaches their doctor with symptoms or when imaging tests for another condition find the tumor. Doctors will typically perform a physical examination and run tests, use imaging scans, and surgically remove the mass to test for cancer via a biopsy.

Treatment options include surgical removal, chemotherapy drugs, radiation, and other drugs or therapies.