Rhabdomyosarcoma of soft tissues in children: embryonic, alveolar and pleomorphic, prognosis of stage 4

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Pathological education occurs in people of different ages. Can be localized in any part of the body.

Rhabdomyosarcoma quickly become noticeable. Its initial stage is well treatable. Therefore, do not delay with an appeal to a specialist.

What is rhabdomyosarcoma?

Malignant formation that occurs in the striated muscles. These tissues are called skeletal muscles.

A negative process can also capture connective tissues. Localized pathology also in smooth muscle formations, for example:

  • in the prostate,
  • in the bladder,
  • in the vagina,
  • in the spermatic cord.

Types of

Rhabdomyosarcoma has the following varieties:

  • Alveolar. One-fourth of patients who contract rhabdomyosarcoma suffer from a variant of it, which is called the alveolar. Locations:
    • small pelvic organs,
    • muscles of the trunk,
    • of the limb muscles.

    These types of tumors are affected in adolescence and in adulthood.

  • Embryonic rhabdomyosarcoma. Pathology occurs in children. The most common variety among the total number of diseases( about 60%).
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    A tumor of this type often occurs:
    • in the genitourinary system,
    • on the neck,
    • on the head.

    This variety of tumors has a subtype:

    • Botryoid. It occurs in children under the age of eight. It is localized in organs where there are mucous membranes. New growths are represented by groats.
  • Pleiomorphic rhabdomyosarcoma. Type of tumor formation that affects older people. Tumors occur in the region of the extremities or on the trunk.
  • Anaplastic. Aggressive form of the disease. They occur in the adult population.
  • Undifferentiated. Pathology occurs in infants until the year. Cells can not be defined. This type of tumor is located on the trunk and extremities.

Reasons for

To date, there are no well-established reasons for the formation of rhabdomyosarcoma, which can be said to directly cause the disease.

It is known that all sorts of mutations in living organisms are provoked:

  • by unfavorable ecology,
  • stay in the ionizing radiation zone,
  • by exposure to harmful chemicals,
  • is negatively affected by trauma.

It is noticed that people have a predisposition to rhabdomyosarcoma, who have a disease:

  • neurofibromatosis,
  • Lee-Fraumeni syndrome,
  • albinism.

Symptoms of

Signals from the body may be different, because they largely depend on the location of the tumor.

If you have such problems, as listed below, then you should contact a specialist as soon as possible.

  • There was an education that is noticeable, it can be probed. It is inactive, does not hurt and does not go away by itself. In time, pain in the pathology area may appear.
  • The tumor can bleed.
  • Enlargement of lymph nodes.
  • Bulging of eyes, vision problems. Education is in the eye area.
  • If the genitals are affected, it may be spotting from them and swelling of the scrotum.
  • Bleeding from the nose and a sense of its congestion is possible with a pathological process in this area.
  • If education in the abdominal cavity - pain in the abdomen, frustration, constipation.
  • Discharge mucosal bleeding from the ear canal, hearing impairment if the problem is localized in this area.
  • Urination is painful and difficult, the appearance in the urine of blood - with a tumor process in the bladder.
  • Decline in forces.
  • There is a loss of appetite and, correspondingly, body weight.

Stages of

Depending on the degree of tumor development, the subdivision of pathology is divided into four groups.

Sequence number of the group characteristic Percentage of the total number of the disease
1 Education is susceptible to complete surgical removal. 16
2 The tumor did not release metastases, but pathological cells already appeared in nearby tissues. At this stage, education can be removed. 20
3 There are no distant metastases. There is no way to remove the tumor completely. 48
4 Tumor development at the stage where distant metastases are detected. 16

Diagnostic Methods

  • A blood test is performed to see the status of the immune system and to test tumor markers.
  • An ultrasound examination will clarify the size and the extent of penetration into adjacent layers.
  • Biopsy of tissues exposed to pathological process. The fence of a small part of the tumor, and its careful study gives clarity about the nature and malignancy of the formation.
  • X-ray examination shows whether there are metastases in the lungs.
  • Scintigraphy clarifies whether bone structures are affected by metastases.
  • Magnetic resonance imaging is a method that provides exhaustive detailed information on the state of soft tissues.
  • Analysis of cerebrospinal fluid - procedure is performed if the pathological process proceeds next to the brain or spine.

On Photo shots CT diagnostics that shows rhabdomyosarcoma of the brain

  • Computer tomography will help you to know the size of the pathology, which organs are affected and the condition of the bone structures.

Treatment options

The way to help the patient is selected by specialists in each case, taking into account all the individual characteristics.

Matches:

  • degree of development of pathology,
  • the place where it was formed;
  • the presence of other diseases,
  • age.

Surgery

The procedure is performed under general anesthesia. To remove education, open operations are used.

Three cases of surgical intervention are typical:

  • Localization of pathology in places where it is impossible to immediately remove it without causing a dangerous injury or cosmetic defect. Usually, this is the area of ​​the neck, head. Begin treatment with radiotherapy or chemotherapy to reduce the area of ​​necessary surgical intervention.
  • In other areas, treatment begins with surgery. The operation is performed by removing as far as possible the tumor, affecting also healthy tissues.
    If some part fails to be removed, excised, then, that is possible. If the pathology is located on the limb, then there may be an amputation.
  • The most favorable case, when the formation is immediately amenable to complete removal.

Radiotherapy

The method is often used in addition to chemotherapy. It helps to kill the remaining cancer cells. Applied in the periods after the operation, there are cases when it is advisable and before surgery.

If malignant formation has occurred in places where its spread is possible on the skull bones, brain envelopes, central nervous system, it may be decided to immediately perform radiation therapy.

Chemotherapy

It is carried out in any case. If the patient is not operable, then with the help of chemotherapy try to improve the situation and increase life expectancy.

Drugs that are used for the procedure alone or in combination:

  • cyclophosphamide,
  • vincristine,
  • platinum preparations,
  • dactinomycin,
  • doxorubicin,
  • etoposide,
  • and ifosfamide,
  • topotecan,
  • rhinotecan.

The bone marrow transplant

The procedure is done in difficult cases to support the body at high doses of chemotherapy.

Because not only the pathological cells are destroyed, but the impact is also on healthy cells. Immunity falls, side effects occur.

Before the chemotherapy, bone marrow sampling is done. After the course of chemotherapy, the body is strengthened by the introduction of a prepared portion of the bone marrow. The same positive effect is produced by the introduction of stem cells taken before chemotherapy.

Immunotherapy

This is a new development in the treatment of tumors. By injection, monoclonal antibodies are introduced. This reinforces the natural defense against pathological cells.

Prognosis in children and adults

The result of treatment depends on the stage at which the disease was diagnosed at the time of diagnosis. Of course, the localization and characteristics of the patient are important: other diseases and age.

In the case of pediatric rhabdomyosarcoma, three risk groups are distinguished:

  • Low. Finding the formation of any size in an area that is considered favorable:
    • in the gallbladder,
    • in the eye area,
    • in the uterus or on the testicles,
    • in the neck or head region( if distal to the brain of the dorsum or head).
  • Medium. Alveolar or fetal formation left after surgery. It is possible to affect the pathological process and the lymph nodes that are near.
  • High. The same types of formations as in the case of medium risk, but which have gained considerable distribution in the body.

In children under the age of nine, the prognosis of rhabdomyosarcoma is most positive, especially in a low risk group. Up to 80% of patients are cured.

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