Among tumor diseases, there are those that are not malignant, but can threaten no less serious consequences. These include the disease described below.
The concept of
Benign tumor , formed by striated muscular tissue - rhabdomyoma, can have different localization.
Usually, this pathology is detected near large joints - knee, hip, but there are also cardiac( in children), tongue and throat( in adults) and genital organs( only in women).
A preliminary diagnosis can be made by a specialist when examining a solid tumor up to 15 cm in size that is not welded to surrounding tissues, since it is encapsulated.
The section shows that it occupies the area of subcutaneous adipose tissue and muscle and has a dark brown color.
Specialists note that this tumor can be independent or located inside other formations, for example, teratomas, and also often consists of several nodules.
In adults and adolescents the neoplasm is more often detected in the behind-the-ear zone, on the abdominal wall, the tissues of the legs, on the chest wall.
The average age of diagnosis among adults is 36 years.Types of the disease
There are several classifications of pathology, the main one includes a subdivision at the location:
- Cardiac ( cardiac) tumors are in the heart muscle.
- Extracardial - have a different localization.
As for the types of tumors by their histological examination, the following can be designated:
- Fetal. They can be polypoid, cellular, myxoid, differing from one another in the type of fiber arrangement and type of growth. Appear in a person from birth.
- Adults. Their structure is similar to that in physiologically normal striated tissue. A separate line is a tumor of the female genitalia.
All types of rhabdomyomas do not tend to invasive( damaging surrounding tissues) growth, therefore are considered benign. Some of them may recur after treatment, then this phenomenon is considered very rare.
Up to 10% of tumors can be transformed into malignant rhabdomyosarcomas.
Reasons for
The exact reasons are still a debatable issue. How normal muscle cells degenerate into a tumor is not exactly clear, but it is assumed that this happens under the influence of some provoking factors.
These factors lead to a malfunction in the production of myocytes( muscle cells) or cells that form the Purkinje fibers in the heart. But other researchers hold the view that such cells break their structure already at the time of aging.
It is possible that both processes take place in the pathogenesis of the tumor, depending on which the fetal or adult formation appears.
What can influence the wrong formation and development of myocytes in the tissues of the body? Presumably, such factors are:
- burdened heredity;
- bad ecology;
- work in hazardous industries;
- poisoning, intoxication;
- hormonal failures;
- disease metabolism, especially, carbohydrate metabolism;
- mutation of genes during conception.
Rhabdomyoma of the heart in newborns
Despite the fact that not always this disease is detected immediately after birth( sometimes the diagnosis is made after 10-15 years), the prerequisites for its appearance are directly from birth.
Often, pathology is detected during fetal ECHO-CG, which is performed in a planned manner or to find the reasons for the delay in growth and the prerequisites of fetal hypoxia.
Benign heart tumor of this type is the most frequent localization of rhabdomyoma in children. Most often it is located in the left or right ventricle or on a septum between them, but sometimes it appears in other departments.
This benign tumor is very dangerous, because many of them differ in intracavitary enlargement in size, so they can block the way of blood flow, than provoke the death of the child.
A favorable outcome is possible, as almost half of such formations tend to spontaneously decrease( involution) and disappear without treatment when the glycogen reserve in them is depleted. That is why indications for surgery for a child's heart tumor are not always present, but only in the development of complications and concomitant diseases.
In the presence of rhabdomyoma, parents should be sure to conduct a thorough examination of the heart in newborns. The fact is that there are family forms of heart damage, which are caused by the presence of defective genes.
Complications of heart formation in children can include heart failure, aortic stenosis, cardiomyopathy and other serious pathologies, so treatment, if necessary, should be timely.
Symptoms of
Later, the detection of the disease is often due to the fact that it does not give clinical signs, develops over the years, but is not visible in appearance. Only when the tumor reaches a significant size, it manifests itself as described below symptomatology.
Of course, disrupting the work of the heart will result in a decrease in cardiac output, various malfunctions in conductivity.
The main manifestations of pathology that occurs in the heart area of children can be as follows:
- arrhythmias( bradycardia, tachycardia, extrasystole, etc.);
- edema of the extremities and abdomen;
- poor gain in weight;
- impaired appetite;
- skin cyanosis;
- frequent crying, weakness, capriciousness of the child;
- various kinds of deviations in mental development.
If the tumor is located inside the ventricles, it most often causes the development of cardiac blockades. Serious conduction failures occur when it is located on the interventricular septum, and they are often accompanied by a loss of consciousness of a protracted nature.
If the tumor continues to grow in size, it can result in asystole - cardiac arrest, and when it closes the ventricular tract - acute heart failure with poor outcome.
Symptomatic complex of single abdominal rhabdomyomas - areas of the abdominal wall, genitalia, extremities, neck and head - is usually represented by visual or palpation their detection on their own or during a routine examination with a doctor.
Tumors do not cause pain, discomfort, and many are perceived as "adolescents."Precisely to diagnose can only the doctor after carrying out of the corresponding analyzes.
Diagnosis
A heart tumor can be diagnosed in utero or after the birth of a child, as well as at any other age.
The main research method continues to be ECHO-KG( ultrasound of the heart), but in recent years, a more accurate method of diagnosis is the MRI of the heart.
These techniques allow you to diagnose, assess the size, location of education, identify violations of hemodynamics in the heart, and analyze the growth of the tumor.
An accurate diagnosis can be indicated only after a biopsy, despite the fact that instrumental methods are of great importance for the preliminary evaluation of the scope of the operation( if necessary).A biopsy is taken by cardiac catheterization, but this procedure is fraught with various complications, therefore it is not always performed.
Tumors of other localization are diagnosed using the same methods - ultrasound and MRI, but with an external location of the tumor, its biopsy will not be so difficult.
Often, histological analysis is performed after complete removal of the tumor.
In case of erroneous diagnosis and subsequent detection of a malignant tumor, the patient is subsequently irradiated with the operated zone.
Treatment of rhabdomyoma
The only way to get rid of this disease is surgical intervention. The tumor is removed together with the capsule, after which the risk of its recurrence is minimized.
The neoplasms on the heart are excised as far as possible in full or in part, using open cardiosurgical access. In the future, a mandatory histological conclusion is required to confirm the benign nature of the pathology.
Observation tactics at the onset of rapid growth of the tumor should be replaced by an operation, and after it a person must be observed for a long time at the doctor for early detection of a relapse.