What is the prognosis for medullary thyroid cancer? All about medullary thyroid cancer Medullary thyroid cancer who is.

Medullary thyroid cancer is one of the most aggressive malignant neoplasms of this localization. It is quite rare, accounting for no more than 5% of cases of thyroid cancer.

Medullary forms of cancer are characterized by a large number of malignant cells within the tumor. The thyroid gland consists of cells that differ radically from each other in morphological characteristics and functions.

Different types of thyroid cancer are distinguished by histopathological properties - the type of cells that undergo malignant transformation. There are 4 types of oncological diseases of this localization: papillary, anaplastic, follicular and medullary.

Forms 2 and 3 are diagnosed most often; they are characterized by a low degree of oncogenicity and a favorable prognosis. Medullary carcinoma is the most aggressive type of cancer, leaving virtually no chance of recovery. It is formed from parafollicular cells, which are responsible for the production of the hormone calcitonin and are part of the diffuse endocrine system.

The hormone is involved in the processes of calcium absorption. Cancer cells also produce and release this substance into the blood, moreover, they do this at an accelerated pace, so a patient’s blood test shows a multiple increase in calcitonin content.

Causes of the disease

It is believed that medullary thyroid cancer can form under the influence of several provoking factors. The main reason for its development is considered to be genetic predisposition.

The mechanism of carcinoma formation is based on damage to the RET oncogene located on chromosome 10q12.2. It is involved in the activity of neuroendocrine cells, which include the parafollicular elements of the thyroid gland. Various gene damages contribute to the occurrence of different syndromes, including MTC.

Important! Exposure to ionizing radiation is an equally common cause of thyroid cancer. According to statistics, the number of cases increased sharply after the Chernobyl accident.

Some types of cancer began to be detected 10 times more often, and the incidence of medullary carcinoma increased significantly. Irradiation of the brain and larynx can cause damage to healthy cells and their further malignant degeneration.

Medullary thyroid cancer can occur even in childhood, but people over 40 years of age are more susceptible to it. This is due to the appearance of a large number of failures in the processes of cell division. Familial cases of the disease have also been reported, suggesting that it can be inherited.

If cancer is detected in one of the family members, all immediate relatives must be tested for the presence of the damaged gene. If it is detected, the probability of developing MTC is close to 100%. Thyroidectomy helps prevent its development.

A person’s lifestyle also plays an important role in the formation of malignant neoplasms. Thus, many types of cancer are often found in people who work with heavy metals, are exposed to frequent stress and have bad habits.

There are also diseases against which medullary thyroid cancer can develop:

malignant breast tumors;
malignant tumors of the larynx;
endocrine neoplasia;
diffuse goiter;
polyposis of the uterus and rectum.

Clinical picture of the disease

Asymptomatic progression is the main danger of medullary carcinoma. The first sign of the disease is enlargement of the cervical lymph nodes, which appears only at stage 3. Few people attach importance to the appearance of small lumps in the thyroid gland. In the early stages, pain syndrome is usually absent. As they develop, the nodes increase in size.

In advanced stages of thyroid cancer, symptoms will include neck pain, trouble swallowing, dry cough and shortness of breath. The clinical picture of the disease also depends on the cause of the formation of the malignant tumor. So, in hereditary forms of cancer, it includes signs of damage to other glands.

At stage 4, the thyroid gland increases significantly in size, secondary lesions are detected in the liver, lungs, and brain.

The functions of these organs are disrupted, causing corresponding symptoms to appear. Depending on the mechanism of development, medullary thyroid cancer is divided into hereditary and sporadic. Most cases of the disease belong to the second type.

Forms of medullary carcinoma

The international classification distinguishes 4 types of MTC: sporadic, MEN II-A, MEN II-B and congenital. The last 3 forms are classified as hereditary pathologies. MEN II-A is considered a syndrome of multiple neoplasia affecting several parts of the endocrine system.

This type accounts for the majority of cases of hereditary cancer.

It is considered congenital and may present as hyperparathyroidism, pheochromocytoma, or medullary carcinoma.

MEN II-B belongs to the same category and is characterized by the following pathological processes: malignant tumors of the adrenal cortex and medullary carcinoma of the thyroid gland. A typical symptom of these syndromes is the formation of tumors in the oral cavity.

The disease is inherited in a dominant manner and rarely occurs by chance. The congenital form of cancer is not accompanied by damage to other organs. It is the least aggressive malignant neoplasm that can be treated.

Diagnosis and treatment of thyroid cancer

Ultrasound is often used to detect malignant tumors. This is a safe and quite effective method of examination. It allows you to assess the condition of the thyroid gland, determine the location and size of tumors. However, it is impossible to identify the cause of their appearance using this method.

Differential diagnosis of medullary carcinoma is quite difficult. A biopsy followed by histological examination is the most informative diagnostic procedure. It is carried out using a thin needle under ultrasound control. The study of the obtained samples allows us to study the characteristics of the cells that make up the tumor.

Oncogenetics is a modern diagnostic method that allows one to detect genetic forms of cancer. The patient donates blood, after which the RET gene in lymphocytes is examined. Other laboratory methods are also used, such as calcitonin levels. In case of MTC, it exceeds the norm several times.

To make a final diagnosis, it is necessary to conduct a comprehensive examination, since genetic analysis alone may be uninformative. An increase in the number of hormones and tumor markers can also be observed in some other pathologies.

Treatment of medullary thyroid cancer is carried out in several ways, the most effective of which is surgery. In the presence of small malignant neoplasms, it is possible to remove part of the gland, but this method does not prevent the regrowth of the tumor.

Most often, the oncologist decides on the need for a total thyroidectomy. If regional lymph nodes are affected, they are also removed.

The operation does not lead to disability of a person; after a few months he can return to his usual way of life, and it does not affect reproductive functions. Its main disadvantage is the need for lifelong replacement therapy.

In the first weeks after surgery, you are treated with radioactive iodine, which destroys any remaining cancer cells. The therapeutic course includes taking drugs that suppress the activity of thyroid-stimulating hormones.

Radiation for medullary cancer is ineffective, so it is practically not used. Chemotherapy is used in the presence of distant metastases.

Medullary carcinoma is an aggressive malignant neoplasm characterized by early active metastasis. The prognosis depends on the stage at which the disease was detected. The average 5-year survival rate for grade 1-2 MTC is 80-90%; about half of the patients live more than 10 years from the date of diagnosis.

Medullary thyroid cancer is the most severe cancer of this organ. The pathology is characterized by the fact that there are several times more cancer cells in the tumor than connective tissue cells. Medullary thyroid cancer is a fairly rare disease. Of all thyroid cancers, medullary thyroid cancer is observed in only 5-6% of patients.

The thyroid gland is located at the front of a person's throat. In shape it resembles a butterfly with its wings spread. In the human body, the thyroid gland performs the functions of producing a variety of hormones. Any thyroid disease leads to hormonal imbalance in the body and, as a consequence, to various pathologies.

The structure of the thyroid gland resembles the well-known polystyrene foam - it also consists of very large cells, closely interconnected. There are many types of cells in the structure of the thyroid gland, and each of them produces its own hormone.

Medullary thyroid cancer affects those cells in the thyroid gland that produce calcitonin, they are called “C-cells.” This hormone is responsible for the absorption of calcium in the body. Cancer carcinomas that affect “C-cells” do not stop the production of calcitonin, but on the contrary, they increase its synthesis and this has an extremely negative effect on the functioning of the body. The study of medullary thyroid cancer began in 1959. Before this, thyroid cancer did not differ by type. It was only in 1989 that all types of thyroid carcinomas were finally established; anaplastic, medullary, papillary and follicular.

Causes of medullary thyroid cancer

Medullary thyroid cancer is currently still under study. In this regard, no clear reason for its occurrence has been identified. There are several reasonable and consistent hypotheses; one of the most likely is genetic inheritance. The very gene that is responsible for the occurrence of pathology has already become known - 10q12.2. That is, if this gene undergoes a mutation, then it is inherited from parent to child. But it is not a fact that the disease will manifest itself without fail; cancer can appear within a generation.

Alternative causes of the development of modular cancer are also considered;

Hard gamma radiation. This reason began to be considered after the accident at the Chernobyl nuclear power plant. The fact is that after the disaster, the number of patients with thyroid cancer increased sharply, approximately 15 times. The scientific community could not ignore this fact, and scientific research began in this direction.
Radiation therapy. Modular cancer can occur as a consequence of radiation therapy, especially to the head or neck. Of course, it does not occur immediately after the procedure; the cells begin to mature only after 5 or even 10 years, after exposure to hard radiation on the bottom.
Advanced age of a person. Metastases of modular cancer can occur at any age. But statistics show that people over 50 years of age are affected by this disease more often than younger people. This is explained by the fact that cell division and replacement of old ones with new ones becomes slower over the years, and failures in this process often occur. Human life expectancy has increased significantly over the past 100 years, thanks to rapidly developing medicine. But the possibility of surviving cancer in people has become much more likely.
The likelihood of developing medullary cancer increases significantly if a person spends his entire life working in hazardous work and is forced to breathe air with a high content of heavy metals.
A person's bad habits, such as smoking and drinking alcohol, significantly increases the risk of developing thyroid cancer of any type.
Oncology in the thyroid gland can develop as a consequence of certain diseases; the presence of polyps in the rectum, endocrine neoplasia, tumors in the female reproductive system, tumors in the thyroid gland itself.
A psychological state can also cause the development of cancer. Thus, prolonged depression, a state of chronic stress or nervous tension weakens the body so much that an oncological tumor can begin to develop in it.

Symptoms of medullary cancer

Symptoms of medullary cancer appear already at the stage when the tumor begins to metastasize to the lymph nodes, liver and other organs. Until this moment, a small tumor in the thyroid gland is not noticeable and does not cause any discomfort to the person. In addition, the first symptoms are in no way associated with cancer and often go unnoticed. Here is an approximate list of physiological abnormalities accompanying medullary thyroid cancer;

The appearance of certain lumps and nodes in the structure of the thyroid gland. This fact is revealed by palpation of the thyroid gland.
The lymph nodes in the neck become visually enlarged.
The patient's voice changes, it becomes hoarse and weak.
Outwardly unmotivated coughing attacks occur.
Swallowing nakedly causes pain or does not occur at all. That is, a person tries to swallow, but he fails.
The patient has breathing problems, feels suffocated, and cannot take a deep breath.
There is severe pain in the throat.
Symptoms of cancer include lack of appetite and nausea.
The patient's sweating increases.
The general muscle tone weakens, the person quickly gets tired.
The patient's entire body decreases sharply.

The younger the patient, the less symptoms appear. In older and elderly patients. Metastases cause noticeable tumors in the throat area, goiter.

Diagnosis of medullary cancer

Diagnosis of medullary thyroid cancer is carried out in a situation where symptoms have just begun to appear. If cancer can be diagnosed in the early stages, before metastases appear in the lymph nodes and internal organs of a person, then the prognosis for treatment will be positive.

The very first and simplest way to diagnose the presence of a tumor in the thyroid gland is ultrasound. The equipment for this study is simple and available to almost any medical institution. The examination is carried out quickly and painlessly. An ultrasound machine can detect a tumor several millimeters in size, that is, at an early stage of its development.

But in order for the treatment of medullary thyroid cancer to be adequate, it is necessary to accurately recognize it. An ultrasound cannot show the type of cancer. For this, the patient undergoes a biopsy. This procedure allows you to take a sample of tumor tissue and determine what it consists of. The biopsy is performed using the same ultrasound; the scanner allows you to very accurately insert a needle into the thyroid gland to collect a sample. The procedure is somewhat painful, but quite simple.

In laboratory conditions, samples of the patient’s blood are studied, the amount of calcitonin in the body is determined from them, and if there is an order of magnitude more of it than necessary, a conclusion is made about the presence of a cancerous tumor in the body.

The most informative study of thyroid cells is magnetic resonance imaging. This method allows you to determine the size of the tumor from a few tenths of a millimeter, which has a positive effect on the prognosis.

Treatment of medullary thyroid cancer

Medullary thyroid cancer has only one treatment - surgical removal of the tumor. No other effective treatment has yet been found. If the tumor is small and the metastases have not yet reached the lymph nodes, then only part of the thyroid gland is removed, the one in which the tumors are located. But such an operation does not guarantee the appearance of new tumors, but in a different part of the thyroid gland.

If the tumor is large and metastasizes to internal organs, the thyroid gland is completely removed, and the patient undergoes chemotherapy. However, in this case the forecast will be disappointing.

Medullary thyroid cancer is a rare type of cancer. Most often it develops in women after 50 years of age. This type of cancer occurs unnoticed and produces the following symptoms: hoarseness, difficulty swallowing, and enlarged lymph nodes in the neck.

Soreness appears in the neck and the thyroid gland itself to the touch. Treatment for medullary carcinoma involves removing the thyroid gland. The prognosis depends on age and tumor type.

Morbidity

Medullary thyroid cancer (MTC) is a rare form of thyroid tumor that forms from a special type of cell. These are called C cells and represent only 1/1000 of the mass of the entire thyroid gland.

Although they are few in number, they are responsible for the bulk of hormone production. In addition, they can release other substances, for example, vasoactive intestinal peptide, adrenocorticotropic hormone, carcinoembryonic antigen, serotonin, prostaglandins and much more. These substances are part of a very complex neuroendocrine system, all of whose functions are not yet fully understood. With this disease, excessive release of each of the listed substances may occur.

There are two types of MTC:

Sporadic, that is, occurring without discernible causes (70-80% of cases).
Consequently mutations in the ret gene, which can lead to the occurrence of isolated (when no other tumor diseases are noted) or multiple endocrine neoplasia type 2, the so-called men 2a or men 2b (involvement of other glands, for example, adrenal glands, parathyroid glands).

Medullary carcinoma is a high-grade disease that accounts for about 25% of all cases. Its development may be genetically determined (MEN2A syndrome is the result of an embryonic mutation of proto-oncogenes in part of the RET gene, and MEN2B is associated with a mutation of the RET918 gene).

Causes

The disease is extremely rare - it accounts for about 1% of all malignant tumors. The incidence of MTC varies geographically.

Russia belongs to a region in which the incidence rate is relatively low. This tumor can occur at any age, but is most often diagnosed in people over 40 years of age.

Genetic factors

Genetic predisposition plays an important role in the development of thyroid cancer. If someone in your family has suffered from a medullary (including follicular and papillary) type of cancer, the risk of its development increases approximately 4 times.

Approximately 25% of MTC cases are hereditary tumors.

Ionizing radiation

Radiation therapy to the neck area in childhood (most often due to lymphoma) significantly increases the risk of developing one type of thyroid cancer 10-20 years later.

Iodine supply in the diet

It has been noted that follicular thyroid cancer is more common in areas with iodine deficiency, while in areas with a good iodine supply, the medullary-papillary type predominates.

Females get sick more often

Women have a greater risk of developing thyroid cancer (all types), at least 3-4 times more likely than men.

Symptoms

MTC develops, as a rule, latently, and the first symptom with which patients consult a doctor is a palpable, but painless, solitary tumor on the neck.

Of course, it could be a manifestation of another disease (for example, more common types of thyroid cancer or an enlarged lymph node or even a change in inflammatory disease). In any case, if such a symptom appears, you should immediately consult a doctor.

Other symptoms that increase the likelihood of diagnosing MTC include:

soreness thyroid gland to the touch;
difficulties with swallowing;
hoarseness;
diarrhea(caused by excess calcitonin);
syndrome Cushing(symptoms of excess adrenal hormones - thin limbs with abdominal obesity, a buffalo hump on the neck, stretch marks);
enlargement of cervical lymphatic nodes (a sign indicating the possibility of metastases).

Medullary carcinoma can metastasize, mainly to the liver, lungs and bones. Less commonly, the adrenal glands and pituitary gland are affected.

Diagnostics

The most important test in the diagnosis of medullary thyroid cancer is the determination of the concentration of calcitonin in the blood. The measurement can be taken directly (at any time) and after taking a drug that increases calcitonin secretion. This hormone is also prescribed after surgical treatment (to ensure that all secretory calcitonin cells have been removed), as well as if recurrent malignancy is suspected.

Calcitonin concentration under normal conditions should be less than 10 pg/ml. Importantly, it tends to be elevated in people with large, palpable tumors. But the indicator should not exceed the normal limits, especially if the change is small. Because of this, it is recommended to perform a test that stimulates the secretion of calcitonin (the so-called provocative test).

A provocative test involves rapid intravenous administration of one of three substances:

Pentagastrin(the most commonly used drug, side effects may occur after taking it - chest pain, hot flashes, nausea, vomiting, abdominal pain);
10% solution calcium;
Omeprazole.

After 2-5 minutes after taking one of the listed drugs, the result is determined. The doctor interprets it as follows - if there is a two or threefold excess of calcitonin concentration relative to the output, the result of the attempt is defined as negative.

Visual exploration

An important examination is an ultrasound of the neck. The examination is performed by an experienced doctor who looks for suspicious changes and evaluates the lymph nodes. When clinical signs show a long duration of the disease (for example, a very high concentration of calcitonin, the tumor is greatly enlarged), it is advisable to supplement the diagnosis with tomography or MRI of the chest.

Suspicion of metastases requires a scintigraphic examination of the thyroid gland using one of the three available radioisotopes.

Final confirmation of the disease is achieved by histopathological examination of material obtained by biopsy of the change. This method does not confirm the diagnosis in 50% of cases. The doctor must observe the full clinical picture (increased calcitonin concentration, enlarged lymph nodes and other significant signs).

Before performing the operation, the concentration of catecholamines or metanephrines in the urine should be additionally determined (with daily collection) - this will exclude the possible coexistence of the tumor and adrenal pheochromocytoma.

Nowadays, in an era of increasing access to modern imaging methods in nuclear medicine, positron emission tomography is possible for accurate diagnosis.

Treatment

In recent years, there has been great progress in the treatment of medullary thyroid cancer, but the main method is still surgery - removal of the thyroid gland and lymph nodes of the neck.

Depending on the severity of the disease and the risk of infection with cancer of the lymph nodes, a decision is often made to increase the scope of the operation. If there is a high risk of disease recurrence, the doctor may additionally prescribe radiation to selected areas of the neck.

In cases of more complex forms of the disease, lymph nodes on both sides of the neck may be removed. Local relapses and single distant metastases of medullary cancer are also treated surgically. After surgery to remove the thyroid gland, patients should receive L-thyroxine in replacement doses, that is, ensuring that TSH is maintained at the normal level for healthy people.

When it is not possible to perform surgery for MTC or the disease is too complex (for example, the occurrence of metastases in other organs), it is possible to use modern targeted drugs - monoclonal antibodies called Caprelsa or Cometriq.

Isotope therapy is considered for thyroid cancer as palliative treatment. In this case, the iodine isotope 131 is used on the meta-iodine-guanidine benzyl carrier (131I-mIBG). However, only 30-40% of patients have the right to use this method of treatment, that is, those in whom accumulation of the marker is indicated. A positive response to treatment is achieved in about half of these people.

Radiation therapy and chemotherapy are used only as adjuvant therapy, used mainly in advanced stages of the disease and only in selected cases. Radiation is sometimes prescribed to patients in whom, after surgery to remove metastases in the lymph nodes, the concentration of calcitonin is not normalized, and at the same time, the presence of distant metastases is not determined (irradiation is carried out in the neck and mediastinum).

Chemotherapy may be considered after other treatments have failed, but its effectiveness is usually low (response to clinical treatment is less than 20%).

Patients who have a combination of tumor processes and developed metastases in the liver, leading to severe diarrhea and severe abdominal pain, sometimes undergo hepatic artery embolization, the purpose of which is to reduce the mass of the tumor.

Somatostatin analogues are used to reduce symptoms such as diarrhea, redness, weight loss, and bone pain caused by hormone production in tumors. The effectiveness of these analogues is best described in cases of treatment of diarrhea accompanying consolidated RRT.

Currently, great hope in the treatment of medullary thyroid cancer rests on the use of tyrosine kinase inhibitors - they contradict the possibilities of pharmacological therapy. Tyrosine kinase inhibitors work by blocking the activity of enzymes (Tyrosine Kinases) at certain receptors (such as VEGF, MET and RET) on the surface of cells (including cancer cells).

They activate several processes, including cell division and the growth of new blood vessels. By blocking these receptors in cancer cells, the drug reduces their growth and spread.

Forecast

The prognosis of MTC will depend on the type and stage of the tumor at the time of diagnosis and the use of appropriate treatment.

With properly selected therapy, the prognosis is very good. The 10-year life extension with medullary-papillary cancer is more than 90%, with follicular cancer 85-90%. In the case of patients with cancer of low severity (small changes), a complete cure is possible.

In the absence of proper treatment, the disease is characterized by an aggressive course and a very unfavorable prognosis. MTC progresses at a rapid rate and is fatal, usually within a few years of diagnosis.

After treatment

All patients with thyroid cancer of any type after surgery and treatment should be under constant medical supervision and undergo regular examinations.

It is recommended to evaluate the effectiveness of treatment in the first 6-12 months after treatment with radioactive iodine. In this case, an ultrasound examination of the thyroid gland is performed, the concentration of thyroglobulin is determined, and scintigraphy of the whole body is performed under conditions of TSH stimulation (using alpha thyrotropin or discontinuation of levothyroxine). If the results of the study give a good result, subsequent checks (every 6 months) will include an ultrasound examination of the thyroid gland, determination of the concentration of TSH and thyroglobulin, and after 5 years - once a year.

In patients with MTC, calcitonin concentrations are also examined to assess the effectiveness of treatment and monitor remission of the disease after surgery.

For each patient with medullary carcinoma, genetic studies are carried out aimed at searching for mutations in genes characteristic of hereditary types of medullary cancer. If confirmed, genetic studies in this direction are performed on family members of the patient.

In most cases, when a thyroid tumor occurs, the cause of its occurrence cannot be determined. However, scientists can say with certainty that medullary thyroid cancer is provoked by three factors:

Hereditary predisposition. Thus, statistics say that if one of the parents has medullary thyroid carcinoma, children suffer from this disease in 20% of cases. It has also been proven that 60% of children whose parents were sick are carriers of the medullary thyroid cancer gene.
Frequent exposure of the head to gamma rays can also cause this disease.
Autoimmune or other diseases that provoke excessive production of thyroid hormones.

Important! The main cause of the development of medullary cancer is considered to be genetic pathology.

The largest number of cases have crossed the threshold of 40 years, but have not yet reached 60.

Women suffer from medullary cancer more often than men. For every 3 sick women, there are 2 men.

There are no particular differences in the incidence rate in iodine-deficient areas and in regions with normal iodine levels.

What happens in the body with medullary thyroid cancer?

The thyroid gland produces the thyroid hormones T3 and T4, as well as the hormone calcitonin, which is important for maintaining muscle tone and metabolism.

It is produced by C-cells; they are also found in the thyroid follicles. But at a certain period, C-cells begin to rapidly divide and produce calcitonin in excess quantities. A node forms in a place where cell growth is too rapid. It grows quickly and at first does not manifest itself in any way. The tumor does not have a capsule and penetrates the cervical lymph nodes very early, causing their enlargement and inflammation. Metastases form here; This occurs in the early stages of medullary thyroid cancer.

With great speed, metastases penetrate into all organs of the neck: trachea, larynx, bronchi. Through the blood, cancer cells enter the adrenal glands and liver, muscle tissue, and lungs.

Symptoms of the disease

Medullary carcinoma is one of the most aggressive types of cancer. For a long time it proceeds without external manifestations. The patient is not worried about anything, you can only notice a nodule on the front of the neck, in the area of the thyroid gland. The nodule grows quickly; Symptoms characteristic of tumor diseases of the thyroid gland appear:

A sore throat.
Dry cough.
Hoarseness of voice.
Weakness, fatigue.

Important! When medullary cancer is diagnosed early, before metastases appear in the lymph nodes, patient survival within 10 years is 75%.

In the future, medullary cancer is characterized by:

Rapid enlargement of the node, which can be observed visually (visually).
Indigestion, diarrhea.
Enlarged lymph nodes.
Difficulty breathing, attacks of suffocation when lying on your back.
Noticeable increase in goiter.
Difficulty swallowing, pain.

After metastases penetrate into distant organs, corresponding symptoms appear:

Choking cough.
Constant shortness of breath.
Diarrhea, diarrhea.
Intestinal colic.
Large goiter.
Fragility, brittle bones due to osteoporosis.

Important! If a tumor is detected at a stage with metastases in the lymph nodes, survival within 10 years is 40%.

Tumor differentiation

To establish a diagnosis of medullary thyroid carcinoma, a number of tests will be required.

To determine the presence of a tumor, an ultrasound scan of the thyroid gland and cervical lymph nodes is performed. It will detect the tumor itself and determine the presence of metastases in the lymph nodes.
To determine the nature of the tumor, a fine-needle aspiration biopsy is performed. Histology will give an answer about the presence of cancer cells in the thyroid gland.
To clarify the level of calcitonin in the blood, a biochemical blood test for hormones is prescribed.
To establish the hereditary form, a DNA test is performed.
MRI will help rule out the presence of metastases in other organs of the neck.
According to indications, chest radiography and scintigraphy are performed.

There are three forms of medullary carcinoma. 1 - sporadic (80%) occurs at the age of 40-60 years. 2 A - Sipple syndrome: multiple lesions (neoplasia) of the endocrine system. Has the worst prognosis. 2 B is a hereditary form that affects the age group of 30–40 years.

Features of treatment depending on the spread of metastases

Treatment for medullary thyroid cancer will depend on the presence of metastases in the lymph nodes and other organs.

Important! Medullary carcinoma is more aggressive compared to follicular and papillary cancer. Another difference is that medullary tumor cancer cells do not respond to radioactive iodine.

When treating medullary thyroid cancer, the following is used:

Irradiation. It is often prescribed first, before thyroidectomy, or after surgery. The neck area is irradiated, since this type of carcinoma spreads very quickly to the cervical lymph nodes. This allows you to avoid the spread of metastases and tumor relapses. The procedure is prescribed if the results of a biopsy confirm the presence of cancer cells outside the thyroid gland.

Surgical removal of the tumor. The appearance of metastases in the lymph nodes requires removal of not only the thyroid gland, but also the cervical lymph nodes. Operation statistics indicate that the gland often has to be completely removed, and lymph nodes are removed in two out of three cases.
Chemotherapy. It is carried out using protein inhibitors (protein kinases). Since this tumor does not respond to iodine 123, the cancer cells that remain in the blood after surgery must be removed with chemotherapy. This procedure causes a number of side effects: nausea, hypertension, diarrhea and bleeding.

The chart shows the five-year survival rate of patients: Stage I - 95%, stage II - 75%, stage III A (Sipple syndrome) - 25% and stage III B - 48%

Survival prognosis

At an early stage, before metastases penetrate the lymph nodes, medullary cancer has a relatively favorable prognosis, and 90–95% of patients survive the 5-year mark. If metastases penetrate the lymph nodes, and cancer is most often detected during this period, survival rate is significantly reduced and within 10 years does not exceed 40%.

Important. The prognosis will be difficult for cancer that has affected the organs of the neck; here the prognosis is disappointing. Death occurs from breathing problems when metastases grow into the organs of the neck (compression of the trachea occurs). From extensive bleeding - with metastases affecting blood vessels, liver, and brain.

Often, when carcinoma with metastases to distant organs is detected, endocrinologists predict the patient to live only a few months.

Medical statistics indicate an increase in the number of malignant changes in the endocrine system. Medullary thyroid cancer occupies a special place. It is rarely observed, at the initial stage there are no symptoms, so it is difficult to diagnose. Complete information about this pathology will help early detection and competent therapy.

Features of the neoplasm

What is medullary carcinoma and how does it develop? Watch the following video on the topic:

Carcinoma (or medullary thyroid carcinoma, MTC) is a rare but aggressive disease. It is distinguished by the rapid growth and distribution of metastases in the tissue of internal organs and neighboring lymph nodes. It often begins to develop after 40 years of age, and is rarely observed in children.

The thyroid gland is located on the front side of the throat and is responsible for producing hormones and maintaining hormonal balance. The structure of the gland is similar to foam, where different cells are connected to each other. Each type of such cell produces specific hormones.

Medullary cancer damages the cells that produce calcitonin. This hormone ensures the absorption of calcium. The disease does not interrupt the synthesis of the hormone, in some cases it even enhances its production, which negatively affects the functioning of internal organs.

Reasons for the development of pathology

Rapid uncontrolled division of thyroid cells occurs for many reasons. Experts say the main culprit is genetic predisposition. The gene responsible for the development of the disease has been identified. But the pathology does not necessarily have to manifest itself in the next generation. Mutation occurs when favorable factors coincide.

The main causes of medullary cancer:

immunodeficiency (congenital or acquired);
long-term work in hazardous work;
influence of ionizing radiation;
irradiation;
benign thyroid tumors;
unstable level of iodine in the body;
some diseases (neoplasms in the reproductive system in women, rectal polyps, endocrine neoplasia);
smoking, drugs, alcohol abuse;
old age;
nervous tension, frequent depression and prolonged stress.

Prolonged stay in a region with unfavorable environmental conditions can provoke the development of oncological degeneration of thyroid cells.

We will tell you in the picture what types of thyroid cancer are most common:

Symptoms of the disease

It is very difficult to detect medullary thyroid carcinoma at an early stage. The signs are not bright, visible only to a specialist, there is no pain. Often a doctor is consulted when lumps are detected in the thyroid gland. This happens even in the presence of metastases and a large tumor size.

For early detection of thyroid cancer, it is necessary to know the manifestations of MTC. This is especially important for people at risk for this disease.

Initial signs of medullary cancer:

the voice becomes hoarse, its strength disappears;
dry cough without cold;
difficulty, pain when swallowing;
thyroid pain;
the appearance of shortness of breath;
When palpating the thyroid gland, compactions and nodules are detected.

Symptoms of pathology depend on the cause of development. For example, during genetic transmission of cancer, damage to other glands is observed. During the formation of a malignant neoplasm, the thyroid gland continues to intensively produce calcitonin. This shows up in symptoms.

The digestive system suffers the most. Abdominal bloating begins, accumulation of intestinal gases, constipation and diarrhea replace each other, there is a decrease in appetite, and abdominal pain. Against this background, general weakness, constant fatigue, and heart rhythm disturbances appear. As cancer progresses, problems begin with the ligaments and muscles; they lose elasticity, firmness and strength. Bones become thin and fragile. The proportions of the human body change.

Patients often experience increased blood pressure, increased sweating, emotional instability, and nervous tension, which develops into prolonged stress. Without treatment, metastases will occur in lung tissue, bones, kidneys, liver, and brain structures. This will only complicate the condition of the sick person.

Diagnostic procedures

Modern equipment makes it possible to detect pathology in the early stages of development. This will have a positive effect on treatment. To make a diagnosis, the following types of studies are performed:

Oncogenetics. A modern way to determine the genetic type of cancer using blood.
Ultrasound of the thyroid gland. This is a quick and painless diagnosis that allows you to detect a tumor several millimeters in size and its location. But this test does not identify the type of cancer.
A blood test for calcitonin helps to identify the presence of the disease at the initial stage. A high level of the hormone indicates the appearance of pathological changes when there are no signs yet.
Biopsy. A tissue sample is examined to determine whether the tumor is malignant or benign. Tissue sampling occurs under ultrasound control using a thin needle. The procedure is painful, but is carried out as quickly as possible.

This is what the procedure for taking material for a biopsy looks like:

Magnetic resonance imaging helps to clarify the diagnosis and shows the area of spread of metastases.

If necessary, the doctor prescribes other diagnostic methods to detect oncology and the development of the disease. For example, this is an examination of individual internal organs to determine the extent of their damage by metastases.

Main directions of treatment

After the diagnosis is made, the patient is sent for treatment. Therapy depends on the degree of development of the tumor. To obtain positive results, specialists combine treatment methods.

The main directions of treatment of medullary cancer:

surgical removal of the thyroid gland;
use of radioactive iodine;
radiation therapy;
chemotherapy method;
taking hormones.

If pathology is detected at an early stage, treatment is more effective.

Surgical treatment

This is a radical method in which one lobe of the thyroid gland is removed (lobectomy) or the entire organ (thyroidectomy). Removal of one lobe is indicated for small tumors and detection at the initial stages of the disease. Removal of the entire thyroid gland occurs when the tumor is large. In this case, it is necessary to cut out the nearby lymph nodes. Often they contain metastases. After surgery, the patient must take radioactive iodine to destroy any remaining cancer cells.

Surgical removal can stop the development of cancer in the body. After it there is a risk of complications. This is an infection during surgery, a change in voice or its complete loss. This phenomenon may be temporary or permanent. There is also a possibility of damage to the parathyroid gland, the development of internal bleeding, and the formation of hematomas in the area of medical procedures.

Treatment with radioactive iodine

We'll tell you what radioactive iodine is in the picture:

All iodine is found in the thyroid gland. Radiation leads to the death of cancer and healthy cells. Nearby organs are not negatively affected.

This method is used after surgery to eliminate remaining malignant cells. It is also effective to use radioactive iodine in the fourth stage of cancer. In the early stages, surgical removal is preferable.

Complications after such treatment depend on the dose of radiation. The following changes are most often observed:

dry mouth;
dry eyes;
vomit;
swelling in the neck;
attacks of nausea;
swelling and hardening of the salivary glands.

The choice of treatment method remains with the specialists; they take into account many aspects of the patient’s health status.

Radiation therapy

Radiation therapy is used to reduce the risk of relapses after surgery or reduce the rate of spread of metastases. Duration is up to 5 weeks. There are about 5 sessions per day. First, the radiation dose is determined by measuring anatomical structures. The therapy does not cause pain or discomfort to the patient.

Radiation therapy can cause some side effects. After the sessions, the skin changes its color and dryness appears in the oral cavity.

Chemotherapy method

This method is used to block abnormal proteins that appear in the body after radiation therapy. Because of it, medullary carcinoma may appear again. Chemotherapy is used using protein kinase inhibitors. It causes nausea, high blood pressure, bleeding, and indigestion of food in the form of diarrhea.

Taking hormones

To ensure normal functioning of the thyroid gland and the entire body, hormonal medications are taken. They are also designed to stop the growth of remaining cancer cells. After removal of the gland, few hormones enter the blood; to compensate for their volume, substances are supplied in the form of tablets. The drugs are selected individually and will be taken for life.

Many experts believe that the best treatment for medullary cancer is removal of the entire organ. It is possible to live without a thyroid gland; surgery will not lead to disability. Removal of the gland has little effect on the usual lifestyle and does not change reproductive functions. You just need to take pills all your life that replace the hormones of this gland, but this is almost always one pill a day.

If you leave the thyroid gland with an aggressive form of cancer, there is a huge risk of developing cancer further throughout the body. All other methods are complementary to surgical removal of the main cause of the disease.