Adrenal cancer

Adrenal cancer is a rare disease that affects only 0.5-2% of people per million population per year. The most dangerous malignant tumor is adrenocortical carcinoma, which develops from cells of the adrenal cortex. At an early stage, the disease can be cured with aggressive surgery. At the same time, treatment options for metastatic tumors are limited and often do not help control disease progression. Therefore, there is currently an active search for new therapeutic approaches, and immunotherapy is one of the most promising areas in this area.

Adrenal cancer – what is it and why is it dangerous?

Cancerous tumors can develop in both the cortex and the adrenal medulla. Adrenal neoplasms have a variety of clinical manifestations, including symptoms associated with an excess of hormones, as well as nonspecific signs that arise as a result of pressure of the growing tumor on surrounding tissues. Very often, adrenal cancer develops asymptomatically and is discovered incidentally during an imaging study for other reasons.

Detection of tumors at an early clinical stage is critical for the success of radical resection (surgical removal of the tumor). Unfortunately, the danger of this type of cancer is that initially it does not manifest itself in any way. Therefore, in many patients the disease is discovered only after the tumor has spread. Most often, adrenocortical cancer metastasizes to the lymph nodes, lungs, liver and bones.

Early detection and qualified treatment of adrenal tumors are the key to successful treatment. Write or call us. The MedTour coordinator will select the best clinic and doctor specializing in the diagnosis and treatment of endocrine cancer for free.

Causes of adrenal cancer

In most cases, the causes of cancer remain unknown. Among possible risk factors, scientists identify the following genetic diseases:

• Li-Fraumeni syndrome (a rare hereditary disease manifested by the development of single or multiple malignant tumors);
• Hippel-Lindau disease (hereditary tumor syndrome);
• syndrome of multiple endocrine neoplasia types 1 and 2 (a group of genetic diseases characterized by multiple tumor lesions of the glands of the endocrine system);
• Beckwith-Wiedemann syndrome (a congenital developmental disorder associated with a predisposition to the development of tumors of embryonic origin).

Conn’s and Cushing’s syndromes can also cause adrenal tumors, but most of them are nonfunctional adenomas.

Adrenal cancer affects people of all ages, but is most often diagnosed in middle age. Some tumors, such as neuroblastoma, primarily occur in children.

Symptoms and signs of adrenal cancer

The specific manifestations of the disease are associated with the type of hormone that the tumor secretes. The first signs, as a rule, are not specific, which makes it difficult to diagnose cancer at an early stage. Elevated hormone levels can be detected through urine and blood tests. However, more often neoplasms are discovered during an ultrasound, CT or MRI for another reason.

The most common symptoms of adrenal cancer are:

• hypertension caused by excessive release of norepinephrine;
• uncontrolled weight gain due to increased cortisol production;
• muscle weakness associated with excess cortisol levels;
• a feeling of fullness in the stomach, pain in the abdomen and lower back caused by compression of surrounding tissues and organs by the tumor.

Changes in hormonal levels can also lead to excess hair growth and menstrual irregularities in women. Men sometimes experience gynecomastia (enlarged breast tissue) and atrophy (shrinkage) of the testicles.

Types of adrenal cancer

Oncologists classify malignant neoplasms depending on the location of the tumor and the type of cells of which it consists.

Adrenocortical carcinoma

It is a rare and aggressive type of cancer that develops in the adrenal cortex. The tumor can produce different types of hormones, including cortisol, aldosterone and sex hormones, leading to a variety of symptoms such as hypertension, obesity, fatigue and menstrual irregularities. Due to its aggressive nature and ability to metastasize early, adrenocortical carcinoma is often diagnosed at an advanced stage, which complicates treatment.

Pheochromocytoma

A rare neuroendocrine tumor that develops in the chromaffin cells of the adrenal glands or paraganglia. It can secrete excess amounts of adrenaline and norepinephrine, which causes characteristic symptoms such as severe hypertension (increased blood pressure), palpitations, sweating, headache and body tremors. Symptoms of pheochromocytoma can be episodic and mimic other conditions, making diagnosis difficult.

Neuroblastoma

A rare and highly aggressive type of cancer that is most often diagnosed in infants and children under 5 years of age, but can also occur in older age groups. Adrenal neuroblastomas can present with a variety of symptoms, including abdominal pain, abdominal swelling, and weight loss. In addition, there are symptoms associated with excess adrenaline and norepinephrine, such as high blood pressure, palpitations, and muscle weakness. Due to the aggressive nature of the tumor and its ability to metastasize quickly, early detection and treatment are critical.

Stages of adrenal cancer

The tumor staging process is based on characteristics such as size, extent of spread to surrounding tissue, and presence of distant metastases.

• Stage 1 – the tumor is less than 5 cm in diameter and has not spread beyond the adrenal gland.
• Stage 2 – the tumor is more than 5 cm in diameter, still localized within the adrenal gland.
• Stage 3 – the neoplasm of any size, the malignant process has spread to nearby tissues or lymph nodes.
• Stage 4 – the tumor of any size, distant metastases are present.

Accurate determination of the stage of cancer is of great importance when choosing a therapeutic strategy and predicting the outcome of the disease.

Diagnosis of adrenal cancer

To detect a tumor and determine its characteristics, doctors usually use diagnostic imaging tests such as ultrasound, CT, MRI, and PET. Sometimes laparoscopic surgery is performed. Its purpose is to assess the extent of spread of the malignant process and the possibility of complete removal of the tumor through surgery.

Doctors additionally prescribe laboratory tests to measure the level of cortisol, aldosterone, and androgens or estrogens. Carrying out a biopsy for adrenal cancer is not advisable, since there is a high risk of tumor metastasis if its capsule is damaged.

Because malignant adrenal tumors are so rare, it is important that the diagnosis be carried out by a doctor with sufficient experience in this field. Call the phone number listed on the website or fill out the feedback form. The MedTour coordinator will select an available, highly qualified specialist for you.

How is an adrenal tumor removed?

Surgery is advisable at any stage of cancer. At an early stage, resection of the tumor with a small amount of surrounding tissue is possible; at later stages, the entire adrenal gland and, in some cases, regional lymph nodes into which cancer cells could have spread are removed.

Most often, the following types of operations are used for surgical treatment of cancer:

• Posterior retroperitoneal adrenalectomy. The adrenal gland is removed through an incision in the back. This approach has several advantages over transabdominal adrenalectomy. It can significantly reduce the operation time, reduce its trauma and pain, and also reduce the risk of blood loss. Surgeons typically remove small, localized tumors using this method.
• Transabdominal adrenalectomy. Access to the surgical field is through an incision in the abdominal cavity. This approach allows the surgeon to better visualize the abdominal organs and assess the extent of the spread of the malignant process.
• Thoracoabdominal adrenalectomy. It is used for resection of large tumors. To gain access to the adrenal gland, the surgeon makes a large incision from the chest to the abdomen. Along with the adrenal gland, the lymph nodes and nearby cancer-affected tissues are removed.

The choice of surgical technique depends on the size of the adrenal cancer and its stage. Whenever possible, surgeons prefer the least traumatic techniques.

Removal of adrenal adenoma

Oncologists make the decision to remove the adenoma if the tumor grows or causes symptoms. Sometimes surgery is performed to exclude the possibility of a malignant neoplasm. The following surgical techniques are used to remove an adrenal adenoma:

• Open surgery. It is performed through one large incision. It is performed to remove large tumors.
• Laparoscopic intervention. It is the most common technique. To gain access to the adrenal gland, the surgeon makes several punctures in the abdominal wall, into which a laparoscope (a special instrument equipped with a light source and a micro-video camera) and miniature instruments are inserted. This is a minimally invasive technique, the main advantages of which are minimizing the risk of infection and bleeding, the absence of noticeable post-operative scars and the rapid recovery of the patient.
• Robotic surgery. The operation is performed laparoscopically. Its main feature is that all surgical procedures are performed by a robotic unit, controlled by an experienced surgeon. The main advantage of this technique is the highest accuracy of the operation.

After surgery, patients are usually prescribed hormone replacement therapy.

Adrenal cancer treatment

For localized primary tumors, radical surgical resection is the treatment of choice and offers the best chance of long-term disease-free survival. However, patients with recurrent or metastatic disease are rarely cured by surgery alone. In the case of adrenal cancer, recurrences occur even in the absence of objective or biochemical evidence of residual tumor after the initial surgery.

Chemotherapy and radiation therapy are given as adjuvant (post-surgery) treatment for patients at high risk of recurrence and as palliative therapy for patients with metastatic cancer.

Adrenal cancer vaccine

Immuno-oncology is a rapidly expanding field of research focused on the interaction between the human immune system and malignancies. Research in this area has led to the development of vaccine therapies aimed at tricking a patient’s immune system into recognizing and destroying cancer cells.

Vaccine therapy has demonstrated clinical benefit in a wide range of solid cancers. Because of its proven activity against many types of cancer, scientists are actively studying its effectiveness in the treatment of rare malignancies such as adrenocortical carcinoma.

There are different types of cancer vaccines. But they are all similar in principle of operation. The main goal of vaccine therapy is to overcome the immune system’s tolerance to cancer cells. Simply put, vaccine components “train” immune cells to recognize and attack a tumor.

Cancer autovaccines are manufactured individually for each patient. This requires a small amount of blood and a sample of a previously removed tumor. There are also xenovaccines. They are based on proteins of animal origin, which are similar in structure to tumor antigens.

Vaccine therapy is used as part of the complex treatment of adrenal cancer. It helps improve the results of standard therapy and reduce the risk of disease relapse.

If you want to learn more about an innovative cancer treatment method, call the numbers listed on the website or fill out the feedback form. The MedTour coordinator will tell you whether you are a candidate for vaccine therapy, answer all your questions and help organize treatment.

Adrenal cancer: prognosis

Survival from adrenal cancer primarily depends on the extent of the spread of the malignant process.

• Localized tumor. If the cancer has not spread beyond the adrenal glands, the 5-year survival rate is 73%.
• Regional metastasis. If the tumor has spread to nearby tissue or lymph nodes, the 5-year survival rate is 53%.
• Distant metastasis. In the presence of metastases to distant tissues or organs, the 5-year survival rate is 38%.

According to the American Cancer Society, the overall 5-year survival rate for adrenal cancer is 50%. It should be borne in mind that this indicator depends on many factors, such as the patient’s age and his general health, so the prognosis is determined individually after a comprehensive diagnostic examination.

Is it possible to live without adrenal glands?

The adrenal glands secrete hormones and biologically active substances that directly affect the development and functioning of vital organs. Therefore, it is impossible to live without hormone replacement therapy. However, the life expectancy of those patients who take hormones after removal of the adrenal glands, regularly visit the doctor to monitor their levels, and also lead a healthy lifestyle may be the same as that of healthy people.

The best clinics for the treatment of adrenal cancer

MedTour company cooperates with the best oncology centers specializing in the treatment of adrenal tumors. Write or call us for a free consultation. The coordinating doctor will select a clinic in accordance with your individual needs and wishes, answer all your questions, and also help organize treatment.

The best doctors for the treatment of adrenal cancer

Adrenocortical carcinoma is a rare form of cancer. Therefore, the diagnosis and treatment of this disease should be carried out by an experienced specialist. The MedTour platform contains information about the best oncologists from leading clinics in the world. Call us by phone or fill out the feedback form. The MedTour coordinator will select an experienced and qualified specialist for you free of charge.

Cancer (tumor) of the adrenal glands – symptoms, causes, diagnosis and treatment in the best clinics

Modern methods of treating adrenal cancer. Use and effectiveness of vaccines against adrenal cancer. Find out more on the MedTour website!

Published: 27.09.2023

Updated: 11.11.2023

Medical author, Medical editor:

Olena Kursabaieva

Medical author, Medical editor:

Natalia Segen