Cancer can be treated with medication and other therapies. The course and prognosis depend crucially on the stage at which the tumor is detected and treated (ENSAT stage I has a relatively good prognosis, ENSAT stages II-IV have an increasingly poor prognosis, see also classification into stages). Even at an advanced stage, there are various treatment options available to patients at our specialist center. Each patient case is discussed individually in our interdisciplinary neuroendocrine tumor board.
What is adrenocortical carcinoma?
Adrenocortical carcinoma is a particularly rare, very malignant tumor. It can originate from any of the three layers of the adrenal cortex. This type of cancer is also known as adrenocortical carcinoma, NN carcinoma or NN-Ca.
Adrenocortical carcinoma can be hormonally active and release hormones into the bloodstream. The most common of these messenger substances are glucocorticoids(cortisol). This results in what is known as Cushing’s syndrome, which is associated, for example, with considerable weight gain, a typical distribution of fat on the chest and abdomen and a reduction in muscle mass. Some adrenocortical carcinomas, on the other hand, release more male sex hormones(androgens). Symptoms of masculinization then develop, for example a deep voice, increased body hair or acne.
Adrenocortical carcinoma can also be hormonally inactive and produce no messenger substances. Then it often remains undetected for a long time. Only when the malignant tumor grows and displaces neighboring tissue does it cause symptoms. Doctors often only diagnose cancer in the adrenal cortex at a late stage, when it is well advanced. It has then left the adrenal cortex, invaded neighboring tissue or has already spread to other organs, i.e. formed cancer metastases or distant metastases.
Treatment involves surgery, the drug Mitotane (which specifically attacks the adrenal cortex cells), radiotherapy and chemotherapy.
Adrenocortical carcinoma – frequency and age
Adrenocortical carcinoma is a very rare type of cancer. Experts estimate that only around one to two people per million inhabitants develop this malignant tumor. In principle, it can occur at any age. But there are two age peaks: in childhood and between the fourth and fifth decade of life. Women fall ill slightly more often than men (about one and a half to two times as often).
Adrenocortical carcinoma: causes are largely unknown
The causes of adrenal carcinoma are still unclear. However, experts suspect that adrenogenital syndrome – a congenital disorder of hormone production in the adrenal cortex – could play a role. Researchers have also observed mutations of a specific gene – the p53 oncogene – in a high percentage of tumors.
The adrenal gland with the adrenal cortex and the adrenal medulla is one of the body’s endocrine glands that produce and release hormones. The adrenal cortex produces steroid hormones, primarily glucocorticoids, mineralocorticoids and male sex hormones (androgens). The adrenal medulla, on the other hand, is responsible for the stress hormones adrenaline and noradrenaline.
The cancer can originate from all three layers of the adrenal cortex. Every cancer begins when a benign cell transforms into a malignant cell. The cells then divide unchecked and multiply.
Symptoms: Adrenocortical carcinoma causes various symptoms
The symptoms of renal cortical carcinoma depend on whether the tumor produces hormones and how large it is. As a rule, malignant tumors in the adrenal cortex are hormonally active, but not always.
Hormone-active tumors that secrete cortisol often develop the symptoms of Cushing’s syndrome. Carcinomas in the zona fasciculata in particular produce cortisol. Possible signs are
- Fat gain, especially on the trunk of the body such as the stomach and chest (trunk obesity), in the neck (“bull neck”) and on the face (hamster cheeks, full moon face)
- Thin arms and legs due to muscle loss
- Reddish stretch marks on the stomach, hips and armpits – similar to stretch marks during pregnancy
- Bruising, poor wound healing
- Skin changes: Acne, thin parchment skin
- Masculinization, such as increased hair on the body and face
- Absence of menstruation
- Psychological changes: Anxiety, depressive thoughts,
- Mood swings
- High blood pressure (hypertension)
- Diabetes mellitus
- Bone loss (osteoporosis) and therefore more frequent bone fractures
If the tumor produces the hormone aldosterone, symptoms of Conn’s syndrome (hyperaldosteronism) may occur. A cancer of the adrenal cortex that develops in the zona glomerulosa mainly produces aldosterone.
Signs can be:
A renal cortex cancer in the zona reticularis predominantly produces androgens, i.e. male sex hormones. Signs of masculinization (“virilization”) in women include a deeper voice than usual, hair loss, baldness, acne and increased body hair. In men, the opposite effect can occur – feminization. Estrogen-producing tumors cause the breasts to grow more (gynecomastia).
Hormonally inactive renal cortical carcinomas, on the other hand, initially cause no symptoms and go unnoticed. Only when they grow and become larger can they cause discomfort. This is because the tumor can grow into adjacent structures, displace tissue or spread to other organs and form metastases. Many adrenocortical carcinomas are already more than eight centimeters in size at the time of diagnosis. And in around 30 percent of those affected, metastases are already detectable.
The following, usually very unspecific symptoms can occur:
- Feeling of pressure and pain in the upper abdomen
- Tiredness, fatigue
- Decrease in physical performance
- Unintentional weight loss
- Nausea, nausea
Adrenocortical carcinoma: diagnosis with us
The diagnosis of adrenocortical carcinoma always begins with taking your medical history (anamnesis). The following questions play a special role here:
- What symptoms do you have and since when?
- How pronounced are they?
- Have you noticed any visual changes?
- Have you gained a lot of weight in the past?
- Do you have any known underlying diseases?
- Are you taking any medications? If yes: Which and since when?
We will then examine you physically. For example, we palpate the abdomen and check the size and position of organs. We also examine the physique, fat distribution, muscles and skin. We also look out for any signs of masculinization. A blood pressure measurement shows how good your blood pressure values are. Further investigations then follow:
- Hormone diagnostics: Determination of various hormones in the blood and in the 24-hour urine collection. This examination can show an excess of cortisol, androgens (male hormones) or aldosterone.
- Computer tomography (CT) with contrast medium
- Magnetic resonance imaging (MRT = magnetic resonance tomography)
- Positron emission tomography (PET) combined with CT: nuclear medicine specialists use low-level radioactive substances (tracers); FDG (F-18 deoxyglucose) – a sugar molecule labeled with radioactive fluorine – is often used in the diagnosis of adrenocortical carcinoma. Hormone-producing tumors can thus be easily detected.
- Biopsy
The size of the tumor can also indicate whether it is benign or malignant. Smaller tumors under four centimeters are more often benign. However, the likelihood of adrenocortical carcinoma is increased if it is larger than eight centimeters. Many carcinomas of the adrenal cortex are already larger than eight centimeters at the time of diagnosis. And in around 30 percent of those affected, metastases are already detectable.
Adrenocortical carcinoma: classification into stages
Doctors divide tumors into different stages depending on their extent. The treatment and prognosis also depend on this. For adrenocortical carcinoma, they use the classification of the European Network for the Study of Adrenal Tumors (ENSAT) for staging.
- Stage I: The tumor is still limited to the adrenal cortex and measures less than five centimetres.
- Stage II: The tumor is larger than five centimeters, but still limited to the adrenal cortex.
- Stage III: The tumor has left the capsule of the organ, there are lymph node metastases, the tumor has grown into the surrounding area or has caused blood clots in certain vessels (vena cava).
- Stage IV: Distant metastases can be detected in other organs, usually in the lungs or liver.
Adrenocortical carcinoma: prevention, early detection, prognosis
You cannot prevent adrenocortical carcinoma because the exact causes are not known. Doctors are also not aware of any risk factors that would promote a malignant tumor in the adrenal cortex. There are also no special measures for early detection. Always see your doctor immediately if you notice any unusual symptoms, such as increased body weight, changes in fat distribution or signs of masculinization.
Course and prognosis of adrenocortical carcinoma
The course and prognosis of adrenocortical carcinoma depend on the stage of the tumor. Many tumors are already larger than 5 cm when they are discovered and the cancer is more advanced (ENSAT stage II-IV). We find metastases in around 30 percent of patients when they are first diagnosed. Then – as with most types of cancer – the prognosis statistically worsens.
As the disease progresses, the tumor can still spread and form cancer metastases – even if it appears to have been completely removed during an operation. The neighboring lymph nodes, liver, lungs and, more rarely, the bones can be affected. For this reason, even after complete removal of the tumor, close follow-up care is very important in order to be able to detect and treat a recurrence of the tumor or metastases at an early stage. This follow-up care consists of clinical, laboratory and imaging checks every three months for two years, then every six months for three years and then further follow-up examinations individually determined by the neuroendocrine tumor board.
However, the individual course and prognosis can vary considerably despite the same tumor stage – also depending on the treatment and aftercare.
All patients with adrenal carcinoma should therefore be referred to a specialized center like ours, where each patient case is discussed on an interdisciplinary basis in the neuroendocrine tumour board in order to determine the optimal diagnostic, treatment and follow-up strategies on an individual basis.
Adrenocortical carcinoma: treatment with several strategies
Doctors from several disciplines are always involved in the treatment of adrenocortical carcinoma (oncology, endocrinology (hormone specialists), radio-oncology, nuclear medicine, diagnostic and interventional radiology, surgery, gastroenterology, pathology, psycho-oncology and palliative medicine). All these disciplines work together in our neuroendocrine tumor board to decide on the best possible treatment strategies for each patient. There are several treatment options.