Are you experiencing stomach pain or sudden weight gain? Sometimes these can be caused by a condition that we don't talk about much, but is important to know about. It's a condition called Adrenocortical Carcinoma . Don't worry, the name may sound a bit complicated, but let's keep it simple.
What is Adrenocortical Carcinoma?
Simply put, adrenocortical carcinoma is a cancer that develops in the outer layer of your adrenal glands , the adrenal cortex . Think of these glands as two little hats on top of your kidneys. They are very important because they produce hormones that control many things in our body, including our metabolism , blood pressure, and how we respond to stress.
Now, in this case of adrenocortical carcinoma, cancerous tumors develop in these adrenal glands. Sometimes these tumors can produce an abnormal amount of hormones. Then the hormones in the body increase too much, which can affect the functioning of our body. Some tumors can grow very quickly and can also put pressure on other organs around them. Although doctors can sometimes treat this condition, sometimes this disease can recur.
Although this condition is most common in adults, it can sometimes develop in young children. However, in this article, we will focus primarily on adrenocortical carcinoma, which affects adults.
Are there types of adrenocortical carcinoma?
Yes, there are two main types. The symptoms of these two types are also different from each other.
1. Functioning tumors: This is the most common type. These tumors produce more hormones like aldosterone , cortisol , estrogen, and testosterone than they should. So, the symptoms depend on the type of hormone that is being produced in excess.
2. Non-functioning tumors: These tumors do not affect hormone production. However, they can grow large and press on surrounding organs and tissues, causing discomfort.
How common is this condition?
In fact, this is a very rare cancer. Although it is the most common type of cancer that develops in the adrenal glands, it is very rare overall. Only about one in a million people are diagnosed with this disease each year. So don't be alarmed by the name.
What are the symptoms of adrenocortical carcinoma?
Surprisingly, some people with this cancer do not experience any symptoms . Studies have shown that between 20% and 30% of these patients are diagnosed incidentally during imaging tests for another condition.
However, when symptoms appear, you may see things like:
- Abdominal pain or discomfort.
- Abnormal hair growth on the face or body of women (hirsutism). It can feel like a man's beard is growing.
- Enlarged breasts in men (Gynecomastia).
- High blood pressure.
- High blood sugar.
- Weight gain, especially in the face, neck, and trunk. It's like someone suddenly got bigger.
How aggressive is adrenocortical carcinoma?
This is a very aggressive cancer. This means that the tumor can grow very quickly and spread (metastasize) outside the adrenal gland to other parts of the body, such as the lungs and bones. Once it spreads, it becomes even more difficult to treat.
What are the causes of adrenocortical carcinoma?
Researchers still haven't found the exact cause . Some people develop the condition because of certain genetic conditions that are passed down from generation to generation. This means that if someone in the family has the condition, others are at a higher risk of developing it too.
In other cases, certain genetic mutations appear to increase the risk. For example, research has found that changes in the tumor suppressor genes (TP53) and (IGF2) can lead to this cancer. Simply put, tumor suppressor genes are genes that control the growth of our cells. If there is a change in these genes, cells can divide and multiply uncontrollably and become cancerous.
What hereditary conditions increase my risk?
You are at higher risk of developing adrenocortical carcinoma if you have any of the following hereditary conditions:
- Beckwith-Wiedemann syndrome
- Carney complex
- Familial adenomatous polyposis (FAP)
- Li-Fraumeni syndrome
- Lynch syndrome
- Multiple endocrine neoplasia type 1 (MEN1)
- Neurofibromatosis Type 1 (NF1)
- Von Hippel-Lindau syndrome (VHL syndrome)
If anyone in your family has these conditions, it's a good idea to talk to a doctor and learn about genetic testing.
What are the possible complications of adrenocortical carcinoma?
This cancer can spread (metastasize) very quickly. Once it spreads to other parts of the adrenal gland, it is very difficult to treat. Also, some hormone-producing tumors can cause conditions like:
- Cushing's syndrome: This is caused by the adrenal glands producing too much of the hormone cortisol. Symptoms include a puffy face and a swollen body.
- Conn's syndrome: This condition is caused by excessive production of the hormone aldosterone. It can cause things like high blood pressure.
How is adrenocortical carcinoma diagnosed? (Diagnosis)
If you are diagnosed with an adrenal tumor during testing for another condition, or if you have the symptoms mentioned above, your doctor will perform tests such as:
- Imaging tests: Tests such as an MRI scan, CT scan, or PET scan to see if there are tumors.
- Blood tests and urinalysis: Check your hormone levels.
- Biopsy: This test is done to confirm whether the tumor is cancerous or not and to obtain tissue samples.
What are the stages of adrenocortical carcinoma?
Doctors use cancer staging to plan treatment and determine what to expect after treatment (prognosis). The stage of adrenocortical carcinoma is determined by the size of the tumor, its location, and whether it has spread to nearby lymph nodes or distant organs.
Here are the main stages:
- Stage I: The tumor is 5 centimeters (about 2 inches) or smaller. It has not spread outside the adrenal gland.
- Stage II: The tumor is larger than 5 centimeters, but has not spread beyond the adrenal gland.
- Stage III: The tumor has spread to nearby lymph nodes.
- Stage IV: The tumor has spread to nearby lymph nodes, nearby organs, or distant organs.
Information about the stage of cancer can sometimes seem confusing. So never hesitate to ask your doctor for clarification. Also, ask how this affects your condition.
What are the treatments for adrenocortical carcinoma?
The most common treatment is an adrenalectomy , which involves surgically removing one or both adrenal glands. In addition, doctors often prescribe medications such as:
- Mitotane (Lysodren®): This medication works by suppressing the adrenal glands. This can reduce the risk of the cancer coming back. It is used after one adrenal gland has been removed or when surgery is not possible. Because this medication stops hormone production, people taking it also need to take hormone pills.
- Metyrapone (Metopirone®): This type of medication may be prescribed to reduce symptoms caused by excess hormone production by the ovaries.
However, in some cases, the tumor may be too large to be safely removed surgically when the disease is diagnosed. In such cases, doctors may use chemotherapy . Although this treatment cannot completely eliminate the cancerous tumor, it can reduce symptoms and slow down the growth of the tumor.
You may also want to consider palliative care as part of your treatment. Palliative care is a type of treatment that helps manage symptoms and side effects of treatment. Palliative care specialists can also provide you with psychological and emotional support.
What are the complications or side effects of treatment?
Surgery and medications can cause various complications or side effects. For example, surgery to remove an adrenal gland can cause things like:
- Effects on hormone production: Usually, the remaining adrenal gland takes over the function of the removed gland and produces enough hormones. However, if this does not happen, you may need to take additional hormone pills until the remaining gland produces hormones properly. This condition is also called adrenal insufficiency .
- Infection: Infections can occur in the abdomen or at the surgical site.
- Excessive bleeding: This can happen during or after surgery.
What are the complications or side effects of the medication?
Your doctor may recommend that you take mitotane for two to five years after surgery. Side effects of mitotane may include:
- Nausea and vomiting
- Diarrhea
- Skin rashes
- Confusion
What are the survival rates for adrenocortical carcinoma?
Overall, 50% of people with adrenocortical carcinoma are alive five years after diagnosis. However, this survival rate depends on many factors. For example:
- The stage of the cancer at the time of diagnosis.
- Whether the tumor is active (producing hormones) or inactive.
- your age.
- Your overall health.
The five-year survival rate by stage is as follows:
- Phase I: 81%
- Phase II: 61%
- Phase III: 50%
- Stage IV: 13%
These statistics may make you feel scared and anxious. That's normal. But remember, these statistics are based on past experience. Experts measure these survival rates every five years. A lot can change in five years, and those changes can affect the results. Also, keep in mind that these survival rates are not an estimate of how long you will live.
If you have specific questions about cancer survival rates, talk to your doctor. He or she is your best resource, as he or she knows your situation best.
Can adrenocortical carcinoma be prevented?
No, it's not really preventable. Researchers know that about half of adrenocortical carcinomas are caused by mutations in certain genes that cause cancer cells to multiply and form tumors. However, they don't yet know what causes these mutations. Therefore, they can't recommend ways to prevent them from occurring.
However, some hereditary conditions increase the risk of developing this disease. If you have a family history of these conditions, it is a good idea to talk to a doctor and learn about genetic testing that can identify the genetic mutations that cause these conditions.
How do I take care of myself? (How do I take care of myself?)
Adrenocortical carcinoma is a rare, often recurring cancer. These two challenges can make you feel scared, lonely, and anxious. Fortunately, there are some steps you can take to help:
- Find support: Ask your medical team about support groups for people with adrenocortical carcinoma. Spending time and talking with people who are going through the same things you are going through can be helpful.
- Consider cancer survivorship programs: Many people with adrenocortical carcinoma are afraid that the cancer will come back. Cancer survivorship programs are one resource to help deal with that anxiety.
- Continue palliative care: If you have chemotherapy after surgery, you may need help managing the side effects of the treatment.
When should I see my doctor?
Adrenocortical carcinoma often recurs, especially within the first two years after surgery. Your doctor will schedule follow-up appointments to monitor for signs of the disease coming back. For example:
- Imaging tests: Every three months for two years, then every three to six months for another three years.
- Blood tests: Your doctor will do regular blood tests to check hormone levels.
What questions should I ask my doctor?
Adrenocortical carcinoma is a rare disease. You may have many questions about what to expect. Here are some questions that we think you may find helpful:
- What does it mean when a tumor is active or inactive?
- Has the tumor spread outside my adrenal gland? If so, how far?
- What treatment options do I have?
- What are the side effects of the treatment?
- Should I get genetic testing for gene mutations that cause adrenocortical carcinoma in my family?
- If the treatment is successful, what are the chances of the tumor coming back?
Adrenocortical carcinoma is a rare, aggressive cancer that affects your adrenal gland. It may not cause any symptoms, and many people are diagnosed during tests for a completely different condition. You may be worried and scared about your diagnosis and the news that your adrenocortical carcinoma may come back after treatment.
If you are feeling overwhelmed by this condition, take the time you need to understand what is happening. Never hesitate to ask questions about the treatments and how they will affect you. Your doctors will understand why you are having certain reactions. They will be there to answer your questions, from diagnosis, through treatment, and years of follow-up.
Take-Home Message
Although adrenocortical carcinoma is a serious condition, it is important to be aware of it, seek proper medical advice, and seek support.
- This is a rare cancer: so don't be discouraged by the name.
- Be aware of symptoms: Watch out for things like stomach upset, unusual weight gain, and hormonal changes.
- Seek immediate medical advice: If in any doubt, see a doctor and get checked out.
- There are treatments: surgery, medications, chemotherapy, etc. Your doctor will decide which treatment is best for you.
- Psychological support is important: You are not alone in this journey. Talk to family, friends, and support groups.
Remember, every patient is different. Your condition, your treatment, and your recovery journey are unique. Never give up hope. Work closely with your medical team and approach this challenge with a positive attitude.
Adrenocortical carcinoma, adrenal cancer, adrenal gland tumor, hormone producing tumor, cortisol, aldosterone, adrenal surgery, mitotane
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