Adrenal Cortical Carcinoma

General Illness Information

Medical Term: Adrenal Cortical Carcinoma

Common Name: ACC, adrenal cortical carcinoma, adrenal cortical cancer, adrenal cortex cancer

Description: Adrenal cortical carcinoma is a malignant tumor that develops from the adrenal cortex cells. The disease is considered extremely rare.

Causes: No one knows the exact causes of cancer development, although research work in this direction is very active. It is known that some genes (sections of our DNA) contain instructions for managing cell division and growth. Some of the genes that contribute to cell division are called oncogenes. Other genes that slow down the division of cancer cells or cause their death, are called tumor suppressor genes or tumor suppressors. It is also known that cancer can be caused by DNA mutations, as a result of which “oncogenes” are switched on or “on-off” of oncopypressors. Some mutations are hereditary, but most of them are acquired during the life cycle for reasons unknown to science.

The inherited genetic syndromes that can affect the risk of developing adrenal cortex cancer include:

  • Lee-Fraumeni syndrome;
  • Beckwith-Wiedemann syndrome;
  • multiple endocrine neoplasias;
  • family adenomatous polyposis.

Non-hereditary risk factors for adrenal cortical cancer include:

  • high-fat diet;
  • smoking;
  • passive lifestyle;
  • exposure to environmental carcinogens.

Prevention:

Since the causes of adrenal tumors are not fully established, prevention is reduced to preventing relapses of distant tumors and possible complications. After adrenalectomy, control examinations of patients by an endocrinologist are necessary every 6 months with the subsequent correction of therapy depending on the state of health and the results of the studies.

Signs & Symptoms:

Approximately half of patients with adrenal cancer suffer from the symptoms of the disease with the hormones produced by the tumor. The other half of the patients suffer from symptoms caused by squeezing the overgrown tumor of nearby organs.

In childhood, the symptoms of adrenal cortical cancer are caused, as a rule, by androgens, produced by the tumor. The most characteristic signs are excessive hair growth on the face and body, an increase in the penis (in boys) and the clitoris (in girls). If the tumor produces estrogens, in girls this can cause an early onset of pubertal, manifested by the growth of the mammary glands and early menstrual periods (boys can also experience an increase in the mammary glands). Adults with adrenal cancer symptoms associated with high levels of sex hormones are not so noticeable.

These symptoms are easier to detect if there is a hyperproduction of the hormone opposite to the patient’s sex: estrogens in men and androgens in women. For example, in men this can be manifested in the loss of libido and impotence, in women – in excessive hair growth, irregular menstruation.

Elevated levels of cortisol, which also occurs in adrenal cancer, manifests itself in the so-called Cushing’s syndrome, which includes the following symptoms:

  • overweight, especially in the abdomen, clavicles, neck, shoulders (“fat cushions”);
  • purple stripes on the abdomen (“stretch marks”);
  • excess hair growth on the face, back and chest in women;
  • weakness and loss of muscle mass of the lower limbs;
  • irregular menstruation;
  • tendency to form bruises;
  • depression;
  • weak bone density, osteoporosis, fractures;
  • high blood pressure.

The hypersecretion of aldosterone causes increased pressure, weakness, muscle cramps and a decrease in the level of potassium in the blood. Excess aldosterone is more often associated with adrenal glands than with malignant tumors.

As the cancerous tumor grows, it begins to squeeze the adjacent organs and tissues. This can cause pain in the area of tumor localization, a feeling of a full stomach and related problems with food absorption.

Diagnosis:

Oncological patients with suspected adrenal cancer should conduct a thorough diagnosis, which includes:

  • Chest X-ray, which allows to detect metastasis in lung tissue or other organic structures;
  • Positron emission tomography, which allows to determine the scale of the tumor process, to clarify the stage of a tumor development and to assess the possibility of performing surgical removal;
  • Magnetic resonance imaging – especially recommended in case of suspected metastasis in the structures of the brain or spinal cord, etc .;
  • Ultrasound diagnosis, which helps to identify the tumor and hepatic metastases;
  • Computed tomography – visualizes the formation, shows the presence of lesions in lymph nodes and other organic structures, helps to decide on the method of surgical intervention and the scope of the surgery;
  • Laboratory studies of biomaterials such as urine and blood, allowing to determine the hormonal level, to clarify the localization of the primary focus, etc.

Treatment:

The main method of adrenal tumor treatment is surgery.

At the first stage of tumor development, when the formation does not exceed 5 cm and there is no metastasis, laparoscopic removal is performed.

If the tumor has reached the second stage of development and has grown more than 5 cm, then the method of removal is determined by doctors based on MRI or CT.

At stage 3 of adrenal cancer, when the tumor affects the lymph nodes, a cavitary surgery is indicated. During removal, the surgeon examines nearby organs and tissues for the presence of metastases.

Adrenal cancer of the 4th stage is usually accompanied by metastatic sprouting in the tissue of the kidneys, liver, etc. Such tumors are not always operable. The possibility of prompt removal is determined by the doctor individually.

As additional treatment, other methods are used. Radiotherapy is actively used in pheochromocytomas.

A patient can get a radioactive isotope injection, which kills cancer cells, reducing the size of the tumor and destroying metastases. With the same purpose antitumor drugs from the group of cytostatics can be used, i.e., chemotherapy.

The chemotherapeutic effect is of low effectiveness, therefore it is used only in case of extensive metastasis.

Sometimes, in order to reduce the hormonal productivity of the adrenal cortex, a patient receives drugs such as Chloditane, Mitotana, etc. The course duration is about 2 months. The drugs have side reactions like nausea and vomiting, lack of appetite, frequent headaches and a sense of intoxication.

After treatment, a constant medical supervision and periodic follow-up examinations are necessary. Such an approach will allow a timely relapse.

Prognosis:

Timely removal of benign tumors of the adrenal glands is accompanied by a favorable prognosis. However, after the removal of androsteroma, patients often have short stature. Half of the patients undergoing surgery for pheochromocytoma have moderate tachycardia, hypertension (constant or transitory). With the removal of aldosteroma, blood pressure comes to normal in 70% of patients, in 30% of cases, moderate hypertension remains, responding well to antihypertensive therapy. Malignant adrenal tumors and their metastasis are extremely unfavorable.

Other:

Patients after adrenalectomy for adrenal tumors are contraindicated physical and mental stress, the use of sleeping pills and alcohol.

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