How Adrenocortical Carcinoma Is Treated

Adrenocortical carcinoma is cancer of the adrenal cortex. The adrenal glands are hormone-secreting glands located on top of the kidneys. Their outer layer is called the adrenal cortex.  Doctors often diagnose this rare form of cancer in its later stages, after it has spread (metastasized), because initial symptoms are nonexistent, vague, or hard to spot.

First-line treatment for adrenocortical carcinoma may include surgery, radiation therapy, or chemotherapy. Doctors may use a combination of these therapies to treat this type of cancer. Additionally, new treatments are emerging for cancer of the adrenal cortex, including biologics and targeted therapy.

Surgeries and Specialist-Driven Procedures

The standard treatment for adrenocortical carcinoma typically involves a combination of surgery, chemotherapy, and radiation therapy. 

Surgery

Doctors may decide to remove the adrenal gland in a procedure called adrenalectomy. Surgery is often the first treatment option used when cancer of the adrenal cortex is caught early and is still considered stage 1 or stage 2. In these stages, the tumor has not yet spread.

The surgery usually involves removing the entire affected adrenal gland. In people with this type of cancer, rarely are both adrenal glands affected. Therefore, removing one gland shouldn’t cause side effects since the other adrenal gland will continue to function.

If the surgeon notices that nearby lymph nodes are enlarged, the doctor may remove them and send them to pathology to check for cancer cells. Lymph nodes are small structures of the lymphatic system, along which travel fluids and immune cells.

When the cancer is in the later stages, after it has spread, surgery will involve also removing nearby affected lymph nodes and tissues. When the cancer has spread to other parts of the body, doctors will use surgery combined with radiation and chemotherapy to provide symptom relief. Surgery alone can’t treat stage 4 adrenocortical carcinoma.

Depending on the spread of the cancer, surgery may be laparoscopic (minimally invasive procedure using small incisions to insert a camera and instruments) or involve an abdominal or back incision.

Radiation Therapy

This type of treatment involves high-energy radiation that kills cancer cells. Doctors may use this treatment combined with surgery and chemotherapy as a palliative care measure to help provide symptom relief and improve a person’s quality of life. 

While there are two types of radiation therapy, doctors use external radiation therapy to treat adrenocortical carcinoma. This type of therapy involves a machine that targets radiation at the cancer.

Radiation therapy lasts for several weeks and involves multiple sessions throughout the week. You shouldn’t experience any pain during radiation treatment itself.

However, side effects may include:

• Nausea
• Vomiting
• Diarrhea 
• Hair loss in the area receiving radiation therapy
• Skin changes in the area receiving radiation therapy
• Fatigue
  

Doctors might use radiation therapy to treat early-stage 1 or 2 adrenocortical carcinoma if the surgery didn’t remove all the cancer.

Treatment after surgery is called adjuvant therapy. It aims to kill any remaining cancer cells that may have been left behind and are too small to be visibly detected. Adjuvant therapy minimizes the risk of cancer recurring.

Chemotherapy

Chemotherapy drugs prevent cancer cells from growing, or it kills them outright. Chemotherapy is often given intravenously, while some chemotherapy drugs can be taken by mouth.

In some cases, chemotherapy is delivered directly into the body. This is called regional chemotherapy. With combination chemotherapy, you receive several cancer-killing drugs at once. 

Chemotherapy administration and drug type depend on what cancer you have and the stage of the cancer. Doctors may use chemotherapy in combination with radiation therapy and surgery to relieve symptoms of adrenocortical carcinoma and improve a person’s quality of life.

In people with stages 1 or 2 adrenocortical carcinoma, doctors may also recommend chemotherapy after surgery if the cancer is not entirely removed or there’s a risk of cancer returning.

Doctors most commonly prescribe a chemotherapy drug called Lysodren (mitotane) to treat adrenal gland cancers. It’s taken in pill form multiple times a day. Because Lysodren blocks hormone production in noncancerous adrenal gland tissue, it can cause side effects such as:

• Fatigue
• Weakness 
• Vomiting 
• Diarrhea
• Nausea
• Confusion
• Rashes
  

You may need to take hormone pills to make up for reduced hormone production. However, in some people, Lysodren can actually help reverse the effects of hormone overproduction. 

Chemotherapy may also cause the following side effects even if hormone production isn’t affected:

• Nausea
• Vomiting 
• Hair loss
• Appetite loss
• Rashes
• Sores in the mouth
• Diarrhea 
• Weakened immune system
• Anemia
• Fatigue
• Shortness of breath

Doctors may also prescribe other chemotherapy drugs in combination with mitotane, such as:

• Platinol (cisplatin)
• Adriamycin (doxorubicin)
• VP-16 (etoposide)
• Zanosar (streptozocin)
• Taxol (paclitaxel)
• Carac (5-fluorouracil)
• Oncovin (vincristine)
  

An ongoing clinical trial is studying the effects of Cabometyx (cabozantinib-s-malate), a chemotherapy drug. The researchers hope to find an effective option for treating late-stage adrenocortical carcinoma that produces fewer side effects than Lysodren. The trial has an estimated completion date of June 2022.

Biologics and Targeted Therapies

These are new therapies currently being studied in clinical trials. They show promise for the treatment of rare cancers, including adrenocortical carcinoma. Biologic therapy uses drugs derived from living organisms. Immunotherapy is one type of biologic therapy. It uses the body’s immune system to combat cancer.

The results of a small clinical trial involving 16 people with adrenal cortex cancer found that immunotherapy with pembrolizumab was moderately effective in preventing the progression of the cancer. It also produced minimal adverse effects.

Targeted therapy involves drugs that target and attack specific cancer cells. Unlike traditional treatments like chemotherapy and radiation, targeted therapy doesn’t damage noncancerous cells. Multiple trials looking into the efficacy and tolerability of targeted therapy drugs are currently ongoing.

Over-the-Counter (OTC) and Alternative Therapies

There is no OTC treatment or home remedy for cancer. It’s your choice if you wish to take supplements or try alternative therapies. However, you should always talk to your doctor about taking supplements or herbal remedies. 

Additionally, while complementary and alternative therapies (CAM) shouldn’t replace traditional treatments for cancer, you may find them helpful for managing cancer symptoms or cancer treatment side effects.

Summary

Adrenocortical carcinoma is a rare type of cancer of the adrenal glands. If found in early stages, it is treated with surgery. Follow-up treatment may be with radiation therapy, chemotherapy, or biologic therapy. For later-stage cancer, these treatments may be used as well.

A Word From Verywell

Despite being a rare cancer, treatments are available for adrenocortical carcinoma. After receiving a diagnosis of adrenocortical carcinoma, you may feel overwhelmed and wonder about the treatment options available. Treatment will depend primarily on the stage of the cancer.

Talk to your oncologist about treatment options. Don’t be afraid to ask your doctor about potential side effects and the best ways to manage them.