Embryonic rhabdomyosarcoma: treatment, prognosis

Rhabdomyosarcoma refers to one of the varieties of sarcoma - cancer of soft tissues, bones or connective tissue. The tumor most often appears in the muscles attached to the bones. Rhabdomyosarcomas are malignant neoplasms that originate in the skeletal muscle. They begin in one part of the body or in several places at once.

Microscopically rhabdomyosarcoma in adults has the appearance of oblong or rounded cells of a bizarre shape. This tumor is more typical for childhood.

Types of rhabdomyosarcomas

The rhabdomyosarcomas are divided into internal types according to the internal structure:

1. Embryonic rhabdomyosarcoma consists of round and spindle-shaped cells, the cytoplasmic examination of which shows transverse or longitudinal striation. This type of rhabdomyosarcom occurs most often. Appears embryonic rhabdomyosarcoma in children and adolescents. It is located in the neck or head, on the genitals, in the nasopharynx. The tumor is sensitive to radiation therapy, but is prone to rapid relapses.

2. Alveolar rhabdomyosarcoma has in its composition groups of cells of round and oval shape, which are surrounded by septa from connective tissue. For this type of tumor characterized by the appearance on the legs or arms, abdomen, chest, anal region or genitals. Often, it can be found in adolescents and adults on the limbs. The outlook is unfavorable.

3. The composition of pleomorphic rhabdomyosarcoma is polymorphous spindle-shaped, ribbon-shaped, stellate cells, tumors of which can appear on the legs or hands of people. As a rule, the growth of rhabdomyosarcoma occurs quickly and does not cause pain and impairment of functions. There is only an expansion of veins. They often sprout through the skin and become covered with ulcers, forming exophytic, bleeding tumors.

Risk factors

Among environmental factors, no one has been found that increases the likelihood of the appearance of this tumor. This distinguishes rhabdomyosarcoma from most of the cancerous tumors.

Due to the fact that there are no exact reasons for the development of the tumor, no advice can be given to avoid its formation. However, it should be borne in mind that with adequate treatment of rhabdomyosarcoma, patients can sometimes be completely cured.

The risk factors for the appearance of embryonic rhabdomyosarcoma are such genetically determined diseases:

1. Lee-Fraumeni syndrome, which is a rare disease, in which the trait is inherited when enough damage to the gene occurs in one parent, so that tumors of various types occur at a high frequency. By inheritance, given the pathology, the specificity of tissue reactions to the tumor is transmitted, when the probability of development of malignant tumors at a young age (up to 30 years) increases sharply.

2. Neurofibromatosis of the first type is the most common hereditary disease that causes predisposition to the appearance of malignant neoplasms.

3. Beckwith-Wiedemann syndrome is a pathology characterized by macrosomia, macroglossia and omphalocele. It is also manifested in children with defects in the anterior wall of the abdomen, hernia, neonatal hypoglycemia, divergence of the rectus muscles.

4. Costello syndrome is a rare disease characterized by congenital anomalies: stunted growth, rough facial features, skin changes.

5. Noonan's syndrome is a genetic disease characterized by stunting and abnormalities in somatic development. Can develop from the whole family or from its individual members.

Infants who had a large weight and height at birth have a greater risk of embryonic rhabdomyosarcoma than others. As a rule, the causes of embryonic rhabdomyosarcoma are unknown. A sign of the disease is the appearance of a tumor, which is becoming more and more.

Symptoms

Symptomatic disease is determined by the form of cancer. An urgent consultation with a doctor is required if the following signs of oncology are observed in children:

• Swelling or swelling, increasing in size or not passing, sometimes painful;
• Bulging eyes;
• Strong headache;
• Difficulties with bowel movements and urination;
• The appearance of blood in the urine;
• Haemorrhage in the rectum, throat, nose.

Diagnostics

To date, there has been no development of methods for determining rhabdomyosarcoma before the appearance of its first obvious symptoms.

The earliest signs of oncology are local swelling or densification, which do not initially cause problems or painful sensations. This symptom is characteristic of rhabdomyosarcoma arms, legs and trunk.

If the tumor is located in the peritoneum or pelvic region, there may be abdominal pain, vomiting, or constipation. This is an embryonic rhabdomyosarcoma of the retroperitoneal space. Rarely, developing in the biliary tract, rhabdomyosarcoma causes jaundice.

If there is a suspicion of this neoplasm, it is necessary to conduct a biopsy, which will help to clarify which form of rhabdomyosarcoma has arisen.

In most cases, rhabdomyosarcoma occurs in areas where it is easily detected, for example, in the nasal cavity or outside the eyeball. If the eye is bulging, or nasal discharge appears, you should immediately consult a doctor with a suspicion of a tumor, in order to carry out the diagnosis at an early stage of its development. In the case where rhabdomyosarcoma appears on the surface of the body, it is easily detected without a thorough examination. This is the so-called embryonic rhabdomyosarcoma of soft tissues.

In 30% of patients, embryonic rhabdomyosarcoma is found in an early stage, when it can be completely removed. But in the vast majority of patients a detailed examination reveals small metastases, the treatment in the presence of which requires chemotherapy.

Embryonic rhabdomyosarcomas located in the testicle region are most often formed in young children and are detected by the parents of the child during washing. If tumors occur in the urinary tract, they cause difficulty urinating or spotting - symptoms that can not be ignored.

Stages of tumor

1. The first stage. A tumor can be of any size, but it has not yet spread to the nodes of the lymphatic system and is located in one of the prognostically favorable areas of the body:

- eyes or okolaglaznoj areas;

- neck and head (excluding tissues in the zone of the brain and spinal cord);

- biliary tract and gallbladder;

- in the uterus or testicles.

Places that are not listed above are considered unfavorable areas.

2. The second stage. The tumor is located in one of the unfavorable zones (zones that are not on the list of favorable ones). The size of the tumor does not exceed 5 cm, and there was no spread to the lymph nodes.

3. The third stage. The tumor is located in an unfavorable area, and one of the following conditions is met:

- tumor size less than 5 cm, and spread to the nearest lymph nodes;

- the size of the tumor is more than 5 cm, and there is a risk that it will spread to the nearest lymph nodes.

4. The fourth stage. The size of the tumor can be any, and spread to the nearest lymph nodes. The cancer spread to remote areas of the body.

Risk groups

The risk group assigned to the patient determines the probability of recurrence of rhabdomyosarcoma. Any child receiving treatment for embryonic rhabdomyosarcoma must necessarily receive chemotherapy in addition to reducing the likelihood of a tumor recurrence. The type of antitumor drug, dosage and number of sessions - all this is determined by the risk group of the child - low, intermediate or high.

Treatment Options

Patients with embryonic rhabdomyosarcoma can be treated in many ways.
Treatment of malignant tumors involves certain techniques that are considered traditional. Other methods are at the stage of clinical trials. Clinical trials are used to improve existing treatment methods or increase the amount of data on the newest methods of treating patients with rhabdomyosarcoma. If, as a result of clinical trials, it turns out that new methods of treatment are superior to traditional ones, the new treatment method becomes traditional.

Since malignant neoplasms have the ability to appear in different areas of the body, a large number of treatments are used. Treatment of rhabdomyosarcoma in children is supervised by a child oncologist.

Among the methods of treatment are those that can cause side effects, even when a lot of time has passed since the treatment.

Surgery

The surgical method, or the removal of a tumor, is widely used in the treatment of rhabdomyosarcoma. This operation is called "wide local excision". This intervention consists in removing the tumor and part of the adjacent tissues together with lymph nodes, which also affects rhabdomyosarcoma. Treatment sometimes consists of performing several operations. The following factors influence the decision on the purpose of the operation and its type:

- the place of the initial localization of the tumor;

- what functions of the child's organism are affected by the tumor;

- tumor response to radiation therapy or chemotherapy, which can be applied as a matter of priority.

In most children, surgical removal of the entire tumor volume is impossible.

Embryonic rhabdomyosarcoma of soft tissues has the property to form in different places of the body, each of which requires its own operation. Surgical intervention is indicated in the case of rhabdomyosarcoma of the genitals or eyes, if the biopsy has confirmed the diagnosis. Before the operation, chemotherapy or radiation therapy can be prescribed to reduce large tumors that can not be operated on to simplify the task of removing the cancer.

Even if the doctor can remove the entire tumor, patients after the operation should be subjected to chemotherapy to suppress the cancer cells that survived. In addition to chemotherapy, radiotherapy is used. Treatment aimed at reducing the risk of recurrence of the disease is called adjuvant therapy.

Radiation therapy

In this type of treatment, x-ray or other types of radiation are used. Radiation therapy is aimed at killing tumor cells or stopping its growth. Its use in medicine found two methods of radiation therapy - external and internal.

External uses a radiation source outside the human body to irradiate the tumor area. Internal radiotherapy, or brachytherapy, irradiates a tumor with the placement of radioactive substances in the immediate vicinity of it inside the human body. It is used to treat cancer of the bladder, head, neck, prostate, vulva.

The type of radiation therapy and dose are determined by the age of the child and the type of tumor, its initial localization, as well as the presence of tumor remnants and the degree of swelling of the tumor of the nearest lymph nodes after the operation.

Chemotherapy

It is a type of tumor treatment using medical means to stop the growth of cancer cells. Chemotherapy drugs help kill these cells or avoid their division. When conducting a course of systemic chemotherapy drugs are taken orally or administered intravenously or intramuscularly. Entering the blood, drugs affect the tumor cells throughout the body. With regional chemotherapy drugs are injected directly into the organs and body cavities, as well as into the cerebrospinal fluid. Quite often, combined chemotherapy is used, which is a treatment in which more than one anticancer drug is used. The method of chemotherapy is chosen depending on the type and stage of development of the tumor.

Chemotherapy is indicated for each child who is treated for rhabdomyosarcoma. It helps to minimize the likelihood of recurrence of the disease. The choice of medication, its dose and the number of procedures are determined by the risk group of rhabdomyosarcoma.

New treatments

New treatments for rhabdomyosarcomas undergoing tests:

• Large doses of chemotherapy in conjunction with the transplantation of stem cells. The method allows you to organize the replacement of hematopoietic cells that are destroyed during the treatment of cancer. Immature blood cells before treatment are removed from the bone marrow or blood of the patient and canned. After the end of chemotherapy, the canned stem cells are re-activated and returned to the patient by the infusion method. Thus, the blood cells are restored.
• Immunotherapy. With this type of treatment, the patient's immune system is used to fight cancer. The drugs used serve to enhance or restore the body's natural defense against cancer. This type of treatment of tumors is called biotherapy.
• Target therapy. This type of therapy is directed against metastases. Target therapy is based on the use of special antibodies, which are bound by a cancer cell. These antibodies are similar to the traditional antibodies of the human body. At the same time they are high-tech medicines. Thanks to targeted therapy in the treatment of "chemistry" you can find and attack only cancer cells, without harming healthy cells. In rhabdomyosarcoma, angiogenesis inhibitors are used as targeted therapy. These drugs interfere with the formation of blood vessels in neoplasms. This leads to the fact that the tumor is starving and stops growing. Angiogenesis inhibitors and monoclonal antibodies are types of agents undergoing clinical trials for the control of embryonic rhabdomyosarcoma.