Adrenal cortex cancer is a rare cancer caused by malignant tumours. They often secrete hormones, which form the characteristic symptoms of the disease. It is found in one person in a million. The difficulty in treating this disease lies in the correct diagnosis and determination of the stage, but in the early stages, adrenal cancer is well-curable. You can read about what criteria are used to diagnose adrenal cancer and the current protocols for its treatment in this article.
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What is adrenal cancer?
Adrenal cortical cancer, or adrenocortical carcinoma, is a rare oncology arising from the outer layer of adrenal tissue and capable of forming metastases. Thanks to a quality programme of medical examinations and check-ups abroad, the disease is often detected at 1-2 stages.
The disease occurs in adults and children. Most patients are between the ages of 43-49 years. Paediatric adrenocortical carcinoma is usually diagnosed at the age of four years, and less often – in adolescents.
At the time of diagnosis, it is important to distinguish adrenal cancer from benign tumours. The latter occur in 1-32% of people and are not life-threatening.
Consult with a foreign oncologist
Leading specialists from Germany, Israel, Turkey and other countries consult patients from all over the world on the treatment of endocrine tumours. They hold face-to-face and online meetings. This is an opportunity to clarify the diagnosis and receive a more personalised treatment programme. Write to the MediGlobus coordinating physicians and we will advise you on whom to contact.
What are the symptoms of adrenal cancer?
In 40-60% of patients, the first sign of adrenal cancer is elevated hormone levels. It can manifest through:
Cushing’s syndrome – fat hump between the shoulders, rounded face, pink or purple stretch marks on the skin, high blood pressure, loss of bone mass, and in some cases, type 2 diabetes. Caused by excessive amounts of cortisol.
Hair loss on the face, neck, chest, abdomen, lower back, buttocks or thighs.
Virilisation – accelerated growth, enlargement of the penis or clitoris and acne.
Feminisation in men – enlarged mammary glands, fatigue, decreased libido.
Premature puberty in children.
Hyperaldosteronism – weakness and muscle cramps caused by low potassium levels.
In one-third of patients, the main reason for seeking medical attention is non-specific symptoms such as abdominal or flank pain, fullness in the abdomen or early satiety. In a small number of patients, the tumour is detected incidentally during a routine examination. This is usually how cancer is detected at stage 1 when it is easily treatable.
The symptoms of an adrenal tumour in women depend on which hormones the tumour secretes. When there is an excess of androgens – male hormones – patients may experience lowered voice and facial hair growth. Adrenal tumours that cause increased levels of estrogen are harder to diagnose in women. They can manifest through the disturbed menstrual cycle, swelling, mood swings and headaches, cystic fibrosis in the breasts, etc.
Adrenal tumour symptoms in men also differ depending on which hormone is elevated. Tumours causing increased levels of female hormones can lead to breast enlargement or breast soreness. Androgen-secreting tumours are detected in men in advanced stages when the tumour begins to press on nearby organs. In boys, the symptoms may be more obvious – increased body hair growth, penile growth, uncharacteristic for their age.
How is adrenal cancer diagnosed?
When there are signs of an adrenal tumour, doctors prescribe a set of diagnostic procedures:
Hormone tests
Since up to 80% of adrenal cortex cancer tumours secrete hormones, testing their levels in blood and urine is a mandatory diagnostic procedure. Subsequently, these tests can be used as cancer markers – a decrease in hormone levels will indicate the shrinkage of the tumour and the effectiveness of oncotherapy for adrenal cancer.
During diagnosis, they measure:
Cortisol;
Mineralocorticoids;
Androgens;
Estrogens.
Visualisation
Computed tomography (CT) or magnetic resonance imaging (MRI) is usually performed to detect tumours. Adrenal tumours are well detected using these methods. Based on the results of the tests, the stage of the disease can usually be determined. In addition, doctors routinely order a chest CT scan to check for lung metastases.
Other oncomarkers that are tested for adrenal cancer include MCM2, SOAT1, CXCR 4, CXCR 7, BIRC7, VAV2, and others.
20-30% of patients with adrenal cortex cancer are initially misdiagnosed. Contact an experienced international oncologist to be confident in your diagnosis and treatment. To book a consultation, leave a request on our website by clicking the button below.
Stages of adrenal cancer
The stage of adrenocortical carcinoma is determined depending on the size of the tumour, depth of tissue penetration, and presence of metastases in lymph nodes and distant organs.
Stage 1 | Tumour less than 5 cm; located in the adrenal gland |
Stage 2 | Tumour bigger than 5 cm; located in the adrenal gland |
Stage 3 | Tumour of any size; spread to lymph nodes near the adrenal gland |
Stage 4 | Tumour of any size; spread to the lung, bone, liver and/or abdominal wall |
Prognosis of patients with adrenocortical carcinoma
Survival in adrenal cancer depends on various factors, including the extent of the disease at the time of diagnosis, the age of the individual and whether the tumour produces hormones. Patients who have tumours of low-grade malignancy with no evidence of invasion into local tissues or lymph node involvement have a better prognosis.
The five-year survival rate for patients with stage 1-2 adrenocortical carcinoma is 75%. If the cancer has spread to surrounding tissues or lymph nodes, the rate drops to 56%. If the cancer has spread to a distant part of the body, 37% of patients survive the five-year threshold.
Among children with adrenal cancer, 80% survive if the tumour can be completely removed by surgery.
CANCER TREATMENT ABROAD
MediGlobus coordinating doctors will advise you on the best clinics for cancer treatment abroad. We can help you choose a doctor and clinic according to your budget and help you organise your trip. For a free consultation with our coordinators, please contact us by clicking the button below.
How to treat adrenal cancer in adults?
At stages 1 and 2, adrenal cancer is treated by complete removal of the tumour. In most cases, the surgery is performed laparoscopically – the tumour is removed through 4-5 small incisions in the abdomen through which surgical instruments are inserted. Compared to open surgery, this procedure is associated with less blood loss and faster rehabilitation.
At stage 3 of the disease, patients also have their lymph nodes removed. Depending on the results of examinations and oncomarker indicators, the attending physician may recommend additional drug treatment.
At stage 4 of adrenal cancer, a comprehensive approach is used: surgery, chemotherapy and radiation therapy.
How are adrenal cortical tumours treated in children?
Unlike adults, it is more difficult to distinguish benign adrenal tumours from malignant tumours in children. A more careful approach and the expertise of the medical team is required in making the diagnosis. The symptoms of adrenal tumours in children are the same as in adults; however, they are easier to detect before puberty. Doctors usually make a correct diagnosis 5-8 months after the initial onset of symptoms. In most cases, neoplasms in children are not life-threatening.
In foreign clinics in 2\3 children adrenal cancer can be detected in the first stages, when the survival rate is the highest.
The main methods of adrenal cancer treatment:
Surgery | International medical organisations recommend an aggressive surgical approach in the treatment of adrenal cancer and its metastases. If an adrenocortical tumour is suspected, laparotomy – open surgery – is recommended to avoid the risk of tumour rupture. |
Chemotherapy | The same drugs are used in the treatment of adrenal tumours in children and adults. The effectiveness of chemo in patients of different ages is at the same level. |
Targeted therapyя | Clinical trials of targeted therapies show about 50% efficacy in paediatric adrenocortical carcinoma in advanced stages or recurrence. |
Radiation therapy is rarely used in the treatment of paediatric adrenal cortical cancer. These tumours often have a mutation in the TP53 gene, making radiotherapy ineffective in their treatment.
What to do in case of metastases to the adrenal gland?
Most often, adrenal cancer metastasises to the lungs, liver and peritoneum. Less frequently, tumours are found in bones and large veins. If possible, such tumours are surgically removed. If complete resection is not possible, alternative treatment options include a combination of chemotherapy, radiation therapy and anti-hormonal drugs.
Radical open surgical excision remains the gold standard for the treatment of patients with metastatic adrenal malignancies. Its overall 5-year survival rate is approximately 38% to 46%.
Where adrenocortical carcinoma is treated abroad
Summary
Adrenal cortical cancer is a rare oncology. The characteristic symptom of the disease is the excessive secretion of hormones which, depending on their function, can cause various symptoms: feminisation or masculinisation, Cushing’s syndrome, metabolic disorders, etc.
Adrenal cortex cancer is extremely rare, but when diagnosed in time, it can be successfully treated. The diagnostic procedures that are prescribed for this disease include a hormone panel and imaging studies of the body.
The main method of treatment for adrenal cortex cancer is surgery. If the tumour can be completely excised by surgery, 75-80% of patients have a positive prognosis. Other patients are treated with chemotherapy, hormone therapy, and targeted therapy. Radiation therapy may also be prescribed for adult patients.
Some of the leading medical centres for adrenal cancer treatment include Liv, Koç and Medicana clinics in Turkey, Uniclinica Navarra in Spain, University Hospital of Cologne in Germany, Ichilov Hospital in Israel and SoonChunHyang Hospital in South Korea.
To make an appointment for adrenal cancer treatment abroad, click on the button below and fill out the form. Our coordinators will call you back within 30 minutes. They will provide you with all the information you need and help you with your travel arrangements.
Sources:
- 1. National Cancer Institute about Adenocortical Carcinoma
- 2. University of California
- 3. Nature Reviews Endocrinology: Adrenocortical carcinoma markers
- 4. Biomedicines: The Role of Biomarkers in Adrenocortical Carcinoma
- 5.American Society of Clinical Oncology: Adrenal Gland Tumors
