Cancer of the adrenal gland is a rare malignant neoplasm originating in the cortical part of this gland. This disease is rare - on average, 1-2 cases per million inhabitants per year, it accounts for 0.05-0.2% of all malignant neoplasms. Find out what causes adrenal cancer and how to treat it.

Contents:

  1. Adrenal gland cancer - causes
  2. Adrenal gland cancer - symptoms
  3. Adrenal carcinoma - diagnosis
  4. Adrenal cancer - treatment
  5. Adrenal carcinoma - prognosis

Adrenal canceraffects women more often, while the peak incidence is in the 4-5th decade of life, and in children under 5 years of age. Adrenal neoplasm is characterized by high malignancy, rapid progression of symptoms and poor prognosis.

Primary adrenal tumors in most cases are hormonally active tumors, which means that such a tumor secretes large amounts of hormones in an uncontrolled manner, regardless of the needs and state of the body, which causes symptoms of hyperadrenocorticism.

The most common adrenal gland tumor secretes cortisol, less often aldosterone, and there are sporadic tumors secreting androgens. There are also hormonally inactive tumors, the so-called The "mute" symptoms they cause depend on the size of the tumor and the degree of invasion of adjacent tissues.

Adrenal gland cancer - causes

The causes of adrenal cortex cancer are not fully understood. Patients with:

  • mutation of the TP53 gene
  • Beckwith-Wiedemann syndrome
  • Li Fraumeni band
  • Carney's syndrome

People with MEN1 syndrome are also at risk of developing an adrenal gland tumor, where an adrenal tumor coexists with a pituitary tumor and primary hyperparathyroidism.

Renal neoplasms, lung cancers and melanomas tend to metastasize within the adrenal cortex, therefore it is important to monitor cancer patients for the presence of a secondary adrenal tumor.

Environmental risk factors for adrenal gland tumors have also not been established, it is assumed that men are at increased risk of developing adrenal gland cancer with smoking, and for women a risk factor is taking oral hormonal contraceptives.

Adrenal carcinoma -symptoms

The symptoms of adrenal cancer depend on whether the tumor is hormonally active or not.

Symptoms of hormone-secreting tumors also depend on the type and amount of hormone the tumor produces.

In the case of a "silent" tumor, i.e. a tumor that does not secrete hormones, the symptoms are non-specific and result from infiltration or pressure on the surrounding tissues and organs by the tumor.

The most common symptoms are swelling of the lower limbs resulting from the pressure of the tumor on the blood vessels. Ascites, weight loss and cachexia may also occur.

These symptoms, however, appear when the tumor reaches a significant size. Previously, silent tumors did not give any symptoms, which is why most often an adrenal tumor is diagnosed accidentally while performing an ultrasound examination of the abdomen for a different reason.

In more than half of patients, the adrenal gland tumor is hormonally active, most often it secretes cortisol and androgens, much less often androgens and aldosterone.

A hormonally active tumor most often presents with clinical symptoms of hyperfunction of the adrenal cortex, with particularly intense virilization, i.e. the appearance of male features in women. This mainly applies to hirsutism, lowered vocal range and menstrual disorders.

Symptoms accompanying a functioning adrenal tumor include:

  • high blood pressure (often not responding to antihypertensive treatment, so-called "resistant hypertension")
  • excessive hair, voice deepening, menstrual disorders (in women)
  • enlargement of the mammary glands, decreased libido, erectile dysfunction (in men)
  • excessive accumulation of fat on the stomach and neck
  • changing the shape of the face to a more rounded one
  • thinning of the skin, which is easily damaged and difficult to heal
  • changes on the skin like stretch marks
  • constant fatigue
  • muscle weakness and muscle spasms
  • a significant decrease or increase in body weight
  • diabetes
  • sleep disturbance
  • heart rhythm disturbance
  • pallor
  • sweating
  • headaches

These symptoms are characteristic of an overactive adrenal cortex and together form the clinical picture of Cushing's syndrome with concomitant symptoms of androgenization.

The presence of such symptoms does not necessarily indicate the presence of an adrenal cortex tumor, it may also be related to hormonal disorders.

There are also tumors that are characterized by subclinical hormonal activity. This means that they secrete hormones in such a small amountamounts that do not cause symptoms in everyday life, but in the case of severe stress, an uncontrolled release of hormones may occur, which may be life-threatening.

In the case of non-functional tumors, patients seek medical advice in connection with non-specific symptoms such as edema, dyspnea, ascites or varicose veins.

In such cases, the tumor is usually already large and infiltrates the surrounding tissues, and also metastasizes to distant organs. According to research, as many as 70% of patients visiting a doctor already have distant metastases.

They are most often located in the lungs, lymph nodes, liver and bones, showing symptoms of failure of these organs.

Adrenal carcinoma - diagnosis

During an interview and physical examination, a doctor may suspect a tumor of the adrenal gland. Imaging tests are then performed, which are important both for diagnosis and planning the surgery.

Tests performed when an adrenal tumor is suspected include:

  • USG
  • computed tomography
  • magnetic resonance imaging
  • PET is also more and more often performed with a marker that selectively only shows the cortex of the adrenal glands, thanks to which it is possible to accurately assess this organ

Non-contrast high-resolution computed tomography also plays an important role in imaging examinations, which allows to distinguish a malignant adrenal tumor from an adenoma by measuring the density of lesions in the adrenal glands.

It is important that if an adrenal tumor is found, imaging examinations of the chest should also be performed in order to exclude the presence of metastases, as well as to assess the position of the adrenal tumor in relation to the kidney, blood vessels and intestines, which allows for careful planning of the surgical operation.

The next stage of diagnostics is the assessment of the hormonal activity of the tumor. For this purpose, laboratory tests of blood and urine are performed to assess the level of cortisol and androgens in the blood.

The studies assess the level of cortisol in the blood, its metabolites in the urine, and the level of androgens - DHEA and DHEA-S.

The so-called functional test involving the administration of dexamethasone and the assessment of cortisol concentration after its administration.

Physiologically, after administration of this agent, the secretion of cortisol should be inhibited.

In the case of cortisol-secreting tumors, no inhibition of cortisol secretion is observed in the dexamethasone test.

Adrenal cancer - treatment

Cancer treatmentadrenal glands primarily involves surgery, followed by chemotherapy and hormone replacement.

The operation consists in the complete removal of the tumor along with the surrounding tissues, sometimes it is necessary to remove the adjacent organ or its part, in which an infiltration or nodular lesion has been found.

If single distant metastases are found, it is advisable to remove them.

In the case of large tumors infiltrating vital organs, surgery is not possible, then the use of chemotherapy is recommended.

Chemotherapy can be used both before surgery to reduce the tumor mass and after surgery as an adjuvant treatment. In adrenal tumor chemotherapy, mitotane and doxorubicin are used, the duration of therapy depends on the patient's condition.

In patients after adrenal tumor surgery, it is necessary to administer the hormones secreted by the adrenal cortex orally for the rest of their lives.

Adrenal carcinoma - prognosis

Adrenal cancer is a very malignant tumor with poor prognosis.

If cancer is detected at an early stage, the 5-year survival rate is approx. 50%.

In advanced cases, 5-year survival is estimated at 10%. The average survival time of patients with adrenal cancer is approximately 28 months from the time of diagnosis.

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About the authorBow. Agnieszka MichalakA graduate of the First Faculty of Medicine at the Medical University of Lublin. Currently a doctor during postgraduate internship. In the future, she plans to start a specialization in pediatric hematooncology. She is particularly interested in paediatrics, hematology and oncology.

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Category:

Oncology