Patient Guide by Let’s Cure ACC

Adrenocortical carcinoma (ACC) is a rare and aggressive cancer, affecting overall 1 person in a million, and the information you will found about it may feel overwhelming with words like rare, aggressive, and poor prognosis, but you are not a statistic, and we, as patients, are here to tell you that there is hope!

Let’s Cure ACC was created by patients for patients. This is your space to connect, share, and equip yourself with the knowledge and information to advocate for your health. While we don’t replace medical professionals, we provide resources, guidance, and a supportive network to help you ask the right questions and navigate your journey with confidence, and much more. Our association is recognized by valuable medical organizations and partners..

If you want to support us in our overall action, please consider becoming a member of our organization as numbers matter, and all together reunited, we can make a real difference towards authorities worldwide!

Any donation would also be greatly appreciated as our organization receives no funding to provide you with all these services, and rely only on generosity.

Welcome to a community that stands with you. Together, we are stronger.

ABOUT THIS SITE

This webpage is available in (language) to provide you with key information around this rare disease in your language. Detailed and additional information is available in the following languages: English, French, German, Spanish, Italian, Portuguese, Chinese and Japanese. You’re familiar with one of these languages? Go on and explore…

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What is ACC?

Adrenocortical Carcinoma (ACC) is a rare disease in which malignant (cancer) cells form in the outer layer of the adrenal gland (called the adrenal cortex). There are two adrenal glands in the body. The adrenal glands are small and shaped like a triangle. One adrenal gland sits on top of each kidney. Adrenocortical carcinoma can occur in both adults and children. Treatment for children, however, is different from treatment for adults.

Adrenal glands play an important role in the body since they produce hormones (like cortisol, DHEA, aldosterone…) that help regulate your metabolism, immune system, blood pressure, response to stress and other essential functions.

Because of its rarity, ACC is most likely to be discovered during another routine exam like a scanner or physical exam.

Symptoms: 

Adrenal cancer acts in one of two ways, depending on the tumor characteristics: 

  1. it can cause symptoms linked to the tumor size and compression of other organs, or
  2. It can cause symptoms related to hormone production of tumors (Cushing syndrome)

The most common symptom reported by patients with ACC is pain in the back or side (the flank). This pain is common and doesn’t directly suggest disease of the adrenal cortex. You also may not be experiencing pain at all. This symptom of back/side pain is usually from the tumor compressing organs, nerves, and other structures around the adrenal gland. Some people also experience feeling full with no appetite because of pressure on the stomach and other abdominal organs.

ACC is often a functional tumor (meaning it secretes hormones), and in about 40-60% of cases, it produces excess cortisol (or other adrenal gland hormones), leading to Cushing syndrome. Cushing syndrome is often the first sign something is wrong with your adrenal glands (when the role of the pituitary gland has been excluded). 

Symptoms of Cushing syndrome may include (but not limited to):

  • Obesity
  • Flushed, rounded face with pudgy cheeks (moon face)
  • Fatty, rounded hump high on the back just below the neck (buffalo hump)
  • Virilization – the appearance of male sex characteristics, including increased body hair (especially on the face), pubic hair, acne, deepening of the voice, and enlarged clitoris in females
  • Excess sweating
  • Purple stretch marks especially on the abdomen, thighs, breasts, and arms
  • Thinning, fragile skin that bruises easily
  • Irregular or absent menstrual periods for those who have a menstrual cycle
  • Mood swings
  • Stunted growth (short stature)

Symptoms of increased aldosterone are the same as symptoms of low potassium and include:

  • Muscle cramps
  • Weakness
  • Abdominal pain

Comprehensive blood tests will be done to check your hormone levels. Patients with ACC-induced Cushing syndrome often show abnormal blood levels of multiple adrenal hormones, not just cortisol. The key hormone changes mostly include:

HormoneExpected Blood Level in ACCReason
Cortisol↑ HighOverproduction by ACC tumor
ACTH↓ LowDue to negative feedback on the pituitary gland
DHEA-S (Dehydroepiandro- sterone sulfate)↑ High (sometimes)ACC can secrete adrenal androgens
AldosteroneVariableACC can be non-functional or produce excess aldosterone in some cases
Testosterone (in women)↑ HighSome ACC tumors produce androgens
Estradiol (in men)↑ HighACC may produce estrogen, leading to feminization in males
Potassium (K⁺)↓ Low (if aldosterone is produced)Hyperaldosteronism leads to potassium loss

Your Medical team

ACC is a rare, orphan disease, so you will need a team of doctors – including mainly an endocrinologist, an oncologist, a surgeon, maybe a nutritionist, radiologist – who will be able to communicate together, as one unanimous voice, about your medical results in the future. You are in the driver seat and you need to feel clearly comfortable with them. A second opinion can always help, whether it is in person or remotely. Your medical team could ask for it by themselves.

Let’s Cure ACC is in contact with more than a hundred of ACC doctors in more than 40 countries to help you find help in your country or in your language. If you need our help to be connected with an expert, do not hesitate to contact us.

Supporting You Through ACC

If you’re here, you might feel like you’re the only one dealing with this cancer—maybe even the only case in your country. That sense of isolation can be overwhelming, but you are not alone. This international network exists so you can connect with others like you who truly understand what you’re going through. Here, you’ll find people from across the world with the same questions, facing the same challenges, the same need for support.

Being diagnosed with ACC or any other rare cancer/illness can cause a multitude of emotions including anger, sadness, fear, etc.  All of your feelings are valid.

Here are a few tips to help you:

  • Don’t be afraid to ask for help. Giving friends/family specific tasks is a great way to get help and to allow people who care about you to feel like they are supporting you.
  • Seek out appropriate online resources for the diagnosis.  While we are not doctors, our website provides you with trusted relevant information and resources. Always consult a qualified medical professional for advice about your condition.  
  • Give yourself grace. Your house is a mess because you’re so distracted with the news/ the treatments? That’s ok!
  • Prioritize rest if/when it is needed. Pushing yourself to the point of exhaustion will only worsen your mental health. 
  • Explore meditation/mindfulness. 
  • If you are working during treatment, explore what accommodations your employer can provide you.
  • Engage in activities that you enjoy and create moments of happiness with your loved ones, by doing the things you love to do.
  • If you are able to exercise, even a quick walk outdoors can be beneficial to your mental health. 
  • If you have concerns about your emotions or mental health, share them with your loved ones and your medical team!
  • Contact us if you ever need to talk to patients on any topic. YOU ARE NO LONGER ALONE !

TREATMENT OPTIONS

There are a few different options available for treatment. This is dependent on the medical care available in your country and the status of your diagnosis. This is a brief list:

1. Surgery: Surgery is the best option and the most recommended one,  whenever possible, since it plays a major role in the prognosis and can be an improving factor. This is true when it comes to the removal of the primary tumor (and its metastases if any occur) as well as for local or distant recurrences. It should be handled by an expert surgeon. 

Based on the finding of the pathologist, after the study of your removed tumor, you will be informed with the treatment most adapted to your case (see ACC prognosis).  

2. Oral chemotherapy / Adjuvant  therapy: This means taking the only drug available on the market called Lysodren/Mitotane. This is called an adjuvant therapy since the aim is to decrease the chance of recurrence. It is recommended for adrenal cancer with medium to high recurrence risk as well as in advanced disease. In low recurrence risk the decision is made individually. You will need to take as much as you can tolerate, with the aim of reaching the therapeutic level, between 14 mg/L and 20 mg/L, as the drug is most effective between those numbers. As Mitotane builds up in your body over time, it might take several months to achieve this goal. Some people will never reach the therapeutic range, but this doesn’t mean that the drug won’t be effective, as shown by recent studies. The side effects can vary from person to person: patients often share their tips: feel free to contact us!

3. Chemotherapy: It is used to kill cancer cells. It can take many forms but in the case of ACC, the product is injected directly in the body (intravenously). It is the recommended treatment for advanced stages of ACC or recurrence, and the regimen of EDP (etoposide, doxorubicin, cisplatin) often combined with Mitotane gives the best response rate and is now the “standard of care”.Based on trials, a combined regimen of gemcitabine/capecitabine is now offered as second-line therapy, especially in Dutch and German expert centers

4. Radiotherapy: A more localized approach where only a specific zone is targeted. Radiation is used to eradicate cancer cells. Like chemotherapy, radiation therapy affects not only cancer cells but also healthy cells. After incomplete surgical resection, radiotherapy in combination with Mitotane can be recommended. 

5. Immunotherapy: This is one of the newest forms of therapy available to patients with ACC. The goal of immunotherapy is to empower your own immune system to be more capable of killing cancer cells in your body. One of these immunotherapy drugs is called pembrolizumab.

6. Targeted therapy: Many research are ongoing to offer new treatments in the future. Several trials are available and your medical team might help you to enter one if your data fits the research. 

What to Know About ACC Prognosis

Warning : this part might be hard to read, so if you don’t feel ready please skip it. As difficult as it might be: facts are facts and we could not avoid talking about them even if our primary focus is on the solution. We will purposely give a general overview based on our research, but as always please consult your physician or other qualified health provider regarding your health. Knowledge is power, but only when you feel prepared to absorb it.

ACC is an aggressive cancer and medical papers will mention a generally poor prognosis. But you are not a statistic and your state of mind, your combativity are important too. While the 5-year survival rate is statistically low, outcomes vary widely based on multiple individual factors. Each ACC case is unique, and your doctor is the best source for personalized information. Be sure to ask your physician about these 4 key factors to better understand your specific situation and treatment options:

1. Resection score: “Resection” contains 4 statuses: R0, R1, R2, and Rx. The “R” stands for residual and your score refers to the degree of resection (cutting out tumor/tissue). 

  • R0: resection is complete (whole tumor has been successfully removed) with clear margins
  • R1: resection is incomplete, as some microscopic tumoral tissues cannot be removed
  • R2: resection is incomplete with macroscopic tumoral tissues left (this means the tissue can be seen by the naked eye)
  • Rx: it is impossible to determine the resection status and is unknown

2. Weiss Score: This score is used to classify adrenocortical tumors as either benign or malignant. There are 9 criteria and the presence of at least 3 of them indicates malignant potential. This is complex data that can be calculated once the tumor is removed, but this data is not easy to obtain. There is a lack of objectivity in this score, as pointed out by doctors and researchers, but it is an important way to know the gravity of your cancer and it should be included in your follow-up medical report (whenever possible). 

3. Ki67/ grade of the tumor: Ki67, expressed in percentage (%), measures how fast cancer cells in a tumor are dividing. It is one of the most powerful tools to predict the risk of recurrence. A high Ki-67 proliferation index means many cells are dividing quickly and that the cancer is likely to grow and spread. Its number will also define the grade of the tumor:

– grade 1 tumors with Ki67 <10%
– grade 2 with Ki67  between 10–19%
– grade 3 tumors with Ki67 ≥20%

4. Stage of your cancer: 

  • Stage I: Tumor < 5 cm, contained in adrenal
  • Stage II: Tumor > 5 cm, contained in adrenal
  • Stage III: Cancer has spread to areas near adrenal (vena cava, renal vein)
  • Stage IV: Metastatic disease (the cancer has spread in the body)

ACC in Kids & Teens

Infants, children and adolescents are not just small adults! The special needs of this group of patients are addressed by pediatric physicians. There are fundamental differences in the development and genetic characteristics between tumors in children and adults. Therefore, the findings regarding adrenal cancer cannot be uncritically transferred from adults to children. Since abnormal growth of tissue (neoplasms) that arise from the adrenal cortex tends to be faster in younger patients, it is essential that pediatricians work closely with physicians who treat adult tumors to ensure comprehensive care. It is also critical to treat any abnormal tissue growth as early as possible. The medical term adrenocortical tumor (ACT) is used more broadly to refer to benign, malignant and borderline/ premalignant adrenal neoplasms. This term also encompasses the cancerous form known as adrenocortical carcinoma (ACC).  

Especially in childhood, ACC is often associated with so-called cancer predisposition syndromes, meaning a genetically inherited, familial occurrence of these tumors (e.g., Li-Fraumeni or Beckwith-Wiedemann syndromes). Genetic testing is generally recommended for all children with ACC. If a genetic predisposition is identified, it is advisable for family members to undergo genetic testing for the associated syndrome. This may help reduce cancer risk through close monitoring.

In contrast to ACC tumors in adults, almost all tumors in children produce hormones (80% of cases are hormonally active). Primarily, the stress hormone cortisol and male hormone precursors are secreted. However, about 60 percent of those affected actually suffer from hormonal disorders and therefore seek medical help. The main conditions include Cushing’s syndrome (with symptoms such as weight gain, high blood pressure, skin changes, etc.), early puberty development, or abdominal pain.

Generally, the following factors are favorable for the prognosis in children with ACC: 

  • Age under four years
  • Androgen hormone production
  • Complete tumor removal (R0 resection)
  • Low tumor stage
  • Low Ki67 level
  • Absence of distant metastasis

Nevertheless, each case must be considered and assessed individually. No two children are alike. 

Since pediatric ACC is very rare, experts worldwide collaborate closely together, both clinically and scientifically. For pediatric tumors, the international working group “KIDS” of ENS@T-PACT has been established. These experts are working closely together with the Brazilian/American working group IC-PACT. Only in this way can children worldwide benefit from medical advances, and the disease be made curable for everyone in the long term. 

Contact us to be put in contact with one of these groups for a second opinion for cases of ACT in children and adolescents up to 21 years of age. 

SOURCES

If you want to support us in our overall action, please consider becoming a member of our organization as numbers matter, and all together reunited, we can make a real difference towards authorities worldwide!

Any donation would also be greatly appreciated as our organization receives no funding to provide you with all these services, and rely only on generosity.

 

Other resources in your language:

Let’s Cure ACC works closely with national and local medical organizations/ patients associations. 

You can contact us to reach out to our Let’s Cure ACC ambassador speaking your language.

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But these resources in your language can also help you: