Adrenocortical Carcinoma: What You Need to Know

April 14, 2023

Introduction to Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare type of cancer that develops in the outer layer of the adrenal gland, which is located on top of each kidney. The adrenal gland is responsible for producing hormones that regulate various bodily functions, including blood pressure, heart rate, and metabolism.

Symptoms
The symptoms of ACC can vary depending on the size and location of the tumor, as well as the amount of hormones produced by the tumor. Common symptoms include:

Diagnosis
Diagnosis of ACC is challenging, as symptoms may be non-specific and the tumor may not be visible on imaging studies. The following tests may be used to diagnose ACC:

  • CT scan or MRI to visualize the tumor
  • Blood and urine tests to measure hormone levels
  • Biopsy to confirm the diagnosis

Treatment
Treatment options for ACC include surgery, radiation therapy, and chemotherapy. The most common treatment is surgical removal of the tumor, which is usually the only chance for cure. If the tumor is inoperable, radiation therapy and chemotherapy may be used to shrink the tumor and alleviate symptoms.

Prognosis
The prognosis for ACC is generally poor, with a five-year survival rate of less than 10%. This is due to the tendency of the tumor to invade surrounding tissue and spread to distant organs, as well as the lack of effective chemotherapy options.

Conclusion
Adrenocortical carcinoma is a rare and aggressive cancer that can be difficult to diagnose and treat. It is essential for individuals to be aware of the symptoms and risk factors and seek medical attention if they suspect they may have ACC. Early detection and treatment are crucial for improving the prognosis of this disease.

Causes and Risk Factors of Adrenocortical Carcinoma

The exact cause of adrenocortical carcinoma (ACC) is not well understood. However, there are several risk factors that have been associated with an increased risk of developing this cancer.

Inherited Genetic Syndromes

Several inherited genetic syndromes are associated with an increased risk of ACC. These include:

  • Li-Fraumeni syndrome
  • Beckwith-Wiedemann syndrome
  • Carney complex
  • Lynch syndrome

Other Risk Factors

  • Gender: ACC is more common in women than in men
  • Age: ACC is most commonly diagnosed in people between the ages of 30 and 60
  • Exposure to certain chemicals: Long-term exposure to certain chemicals, such as pesticides and herbicides, may increase the risk of ACC.

Hormonal Imbalances

  • High levels of androgens (male hormones) or estrogen in the body may increase the risk of ACC.
  • Cushing’s syndrome, a condition in which the body produces too much of the hormone cortisol, is associated with an increased risk of ACC.

While the exact cause of ACC is not known, certain inherited genetic syndromes and hormonal imbalances, as well as environmental exposure, have been linked to an increased risk of this cancer. It is important for individuals who have a family history of ACC or who have risk factors to be aware of the signs and symptoms of this disease and to seek medical attention if they suspect they may have ACC.

Symptoms and Diagnosis of Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare cancer that develops in the outer layer of the adrenal gland, which is located on top of each kidney. The symptoms of ACC can be non-specific and may not appear until the tumor has grown large enough to cause problems.

Symptoms

Symptoms of ACC can vary depending on the size and location of the tumor, as well as the amount of hormones produced by the tumor. Common symptoms include:

  • Abdominal pain or swelling
  • Weight gain
  • Back pain
  • Fatigue
  • High blood pressure
  • Purple or red stretch marks on the skin (striae)
  • Bruising easily
  • Darkening of the skin (hyperpigmentation)

Diagnosis

Diagnosis of ACC can be challenging, as symptoms may be non-specific and the tumor may not be visible on imaging studies. The following tests may be used to diagnose ACC:

Imaging studies

  • CT scan or MRI to visualize the tumor
  • PET/CT scan to detect any spread of the cancer

Blood and urine tests

  • Blood and urine tests to measure hormone levels
  • Elevated levels of hormones such as cortisol, aldosterone, and androgens can indicate the presence of an ACC.

Biopsy

  • A biopsy is the most definitive way to diagnose ACC. A sample of tissue is removed and examined under a microscope to confirm the diagnosis.

ACC can be difficult to diagnose due to its non-specific symptoms. If a person has risk factors or symptoms that suggest ACC, they should seek medical attention. Early detection and diagnosis are crucial for improving the prognosis of this disease.

Treatment Options for Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare and aggressive cancer that can be difficult to treat. The treatment options for ACC will depend on the stage and location of the cancer, as well as the patient’s overall health and preferences.

Surgery

Surgery is the most common treatment for ACC and is often the only chance for a cure. The goal of surgery is to remove the entire tumor, as well as some of the surrounding healthy tissue. The type of surgery will depend on the location and size of the tumor, as well as the patient’s overall health.

Radiation therapy

Radiation therapy uses high-energy radiation to kill cancer cells. It may be used to shrink the tumor before surgery or to alleviate symptoms if the tumor is inoperable.

Chemotherapy

Chemotherapy uses drugs to kill cancer cells. It may be used in conjunction with surgery or radiation therapy to shrink the tumor, or as a palliative treatment to alleviate symptoms.

Targeted therapy

Targeted therapy is a newer type of cancer treatment that uses drugs to target specific molecules that are involved in the growth and spread of cancer cells. Targeted therapy may be used in conjunction with surgery, radiation therapy, or chemotherapy.

Hormonal therapy

Hormonal therapy may be used to block the production of hormones that promote the growth of ACC.

The treatment options for ACC are varied and will depend on the stage and location of the cancer, as well as the patient’s overall health and preferences. Surgery is usually the primary treatment, but other options such as radiation therapy, chemotherapy, targeted therapy, and hormonal therapy may be used as well. It is important for patients to discuss all treatment options with their healthcare provider to determine the best course of treatment for their individual case.

Prognosis and Supportive Care for Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare and aggressive cancer that can be difficult to treat. The prognosis for ACC is generally poor, with a five-year survival rate of less than 10%. This is due to the tendency of the tumor to invade surrounding tissue and spread to distant organs, as well as the lack of effective chemotherapy options. However, with early detection and treatment, the prognosis can be improved.

Prognosis

The prognosis for ACC depends on the stage of the cancer at the time of diagnosis. The five-year survival rate for people with localized ACC (cancer that has not spread beyond the adrenal gland) is about 50%. The five-year survival rate for people with advanced ACC (cancer that has spread to other parts of the body) is less than 10%.

Supportive Care

Supportive care is an essential part of the treatment for ACC. It is intended to alleviate symptoms, improve quality of life, and provide emotional and psychological support for the patient and their family.

Pain management

Patients may experience pain due to the tumor or treatment-related side effects. Pain management strategies such as medication, nerve blocks, and physical therapy may be used to manage pain.

Symptom management

Symptoms such as fatigue, nausea, and vomiting may occur as a result of the cancer or treatment. Symptom management strategies such as medication and lifestyle changes may be used to alleviate these symptoms.

Emotional and psychological support

Living with ACC can be difficult, both for the patient and their loved ones. Emotional and psychological support can be provided through counseling, support groups, and other resources.

The prognosis for ACC is generally poor, but with early detection and treatment the prognosis can be improved. Supportive care is an essential part of treatment and is intended to alleviate symptoms, improve quality of life, and provide emotional and psychological support for the patient and their family. It is important for patients to have a comprehensive treatment plan that includes not just medical treatment but also emotional and psychological support.

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