Soft tissue fibrosarcoma is a malignant tumor based on bone material. The tumor develops in the thickness of the muscles and can proceed for a very long time without certain symptoms. This disease is found in young people, and in addition, in children (this audience is about fifty percent of all cases of soft tissue tumors).
Rare among the elderly. Basically, such a tumor attacks the lower limbs. Arising in muscles, under a layer of fat and in ligaments, the formation can be invisible for a long time. Soft tissue fibrosarcoma, despite the fact that it is an oncological disease, is not considered a cancerous tumor, since it is formed from connective tissues, and cancer from epithelial ones. Let us further consider in more detail what fibrosarcoma is, find out for what reasons it is formed and how to treat it.
What do we know about fibrosarcomas?
Such a phenomenon as soft tissue fibrosarcoma refers to malignant pathological neoplasms. As a rule, the basis of such a tumor is formed by immature connective tissue. The bulk of patients with such neoplasia are young people and children. As for mature and old age, such a disease in this category of people is diagnosed extremely rarely. The highest incidence of all cases of fibrosarcoma is detected under the age of five years. During this period, the share of pathology accounts for almost half of all neoplasms of soft tissues.
Limb injury
Usually such a tumor in humans affects the limbs. It is worth emphasizing that the legs, as a rule, suffer most often. A tumor that occurs in the limbs is usually located in the thickness of the ligaments, muscles and fat layer, so it can go unnoticed for a very long time. Other localizations of soft tissue fibrosarcomas are also possible, in particular, in the region of the retroperitoneal space. This form of pathology is very dangerous, since there are certain difficulties in diagnosing, and directly surgical procedures can become very traumatic and even impossible due to the involvement of internal organs.
It is not uncommon for a soft tissue tumor to be called cancer, but that is not entirely correct. As is well known, cancer differs in epithelial origin, and fibrosarcomas in connective tissue, so it is wrong to call such a tumor "leg cancer", "muscle cancer" and the like.
It is worth noting that soft tissue fibrosarcomas are capable of spreading not only alonglymphatic vessels, but also through the blood vessels, although the hematogenous route is perhaps the predominant one. Neoplastic secondary nodes can be found in the lungs, liver, and bones. Tumor ingrowth into the surrounding tissue may be accompanied by its destruction, and in addition, damage to nerves and blood vessels and penetration into the bone. Next, find out what are the causes of this dangerous pathology.
Reasons for education
For what reasons soft tissue fibrosarcoma develops (pictured) is still unknown. There is a version that chromosomal mutations that fail in the womb are capable of provoking the occurrence of such a tumor. For these reasons, a tumor can appear in a child.
In an adult, fibrosarcoma appears as a result of repeated exposure to ionizing and X-rays (for example, in the treatment of another oncological disease). Moreover, the time interval from exposure to the appearance of fibrosarcoma can be fifteen years. Also, doctors do not exclude the version that injuries and severe bruises can affect the development of this disease. Or they start the growth processes of an existing connective tissue tumor.
Types of pathology
Photos of soft tissue fibrosarcoma abound.
Tumors are divided into two main types:
- Highly differentiated view.
- Low-differentiated view.
Highly differentiated fibrosarcomas are characterized by a low degreemalignancy, and besides, weaker growth in comparison with poorly differentiated ones. Its cells, which are surrounded by collagen fibers, are similar to he althy tissues. Such tumors do not have a special effect on the body, and at the same time they do not metastasize to the neighboring structure.
Poorly differentiated fibrosarcomas are more aggressive forms of the disease. The cells of this tumor can differ sharply from he althy ones, and at the same time they grow rapidly. That is why such a tumor rapidly increases in size and metastasizes to other tissues.
Below in the photo - soft tissue fibrosarcoma. Its cells are able to spread throughout the body through the lymphatic and blood vessels. Often there is a hematogenous route of spread. Predominantly metastases enter the liver, bone structure and lungs. Metastasizing to neighboring tissues and organs, fibrosarcomas lead to their destruction, and also grow into bones, damaging nerve fibers with blood vessels.
The following types are found:
- The appearance of fibromyxoid sarcoma can occur in people in adulthood. In this case, the bones of the shoulders, torso, thighs and lower leg can be affected. Soft tissue fibrosarcoma of the thigh, for example, has a low grade of malignancy.
- The occurrence of dermatofibrosarcoma is a rare disease. Such a formation can develop in the connective tissue and is located on the skin surface. It is also called Darier's tumor.
- The appearance of neurofibrosarcoma is a dangerous forma disease that develops around a nerve fiber. Fifty percent of cases can occur in people with a disease called neurofibromatosis.
- Myxoid fibromas are a rare form of disease that affects cartilage. The formation is considered benign, it makes up about one percent of all bone neoplasms.
- Infantile fibrosarcomas are malignancies that occur in infants and children under five years of age. It can be characterized by aggressive growth, and in addition, a very high malignancy. The pathology usually affects the extremities, but can also occur on the neck and head.
- Ovarian fibrosarcomas are considered a rare form of cancer, accounting for about four percent.
Photos of soft tissue fibrosarcoma at the initial stage are of interest to many.
Symptoms of disease
The manifestation of fibrosarcoma largely depends on its location, size, malignancy of the neoplasm. Highly differentiated tumors may not make themselves felt for a long time, they are detected randomly during a patient's examination for other diseases. The patient turns to the doctor when the formation already reaches a large size. Highly differentiated fibrosarcomas can be asymptomatic for up to fifteen years, and poorly differentiated ones appear already within the first twelve months from the moment of formation. Sometimes fibrosarcoma (pictured above) is detected earlier due to the pain syndrome resulting from compression of nerve endings and bone deformity.
The skin does not change above the surface of the tumor. Only against the background of a rapidly growing form of fibrosarcoma, thinning can be observed along with blue skin in the area of the neoplasm. The formation of a venous network is not excluded. Fibrosarcoma on palpation is confused with a benign formation, since it has the property of forming a round capsule with a smooth border.
Fibrosarcomas of small size are able to move during palpation, but when the neoplasm becomes larger in size, it is very difficult to move it due to ingrowth into bone tissue. Pain syndrome occurs against the background of compression of the nerve fiber and blood vessels. Against the background of the introduction of a tumor into the bone, the pain becomes excruciating and becomes chronic.
The symptoms of fibrosarcoma are very unpleasant. Increasing in size, it affects the general well-being of the patient. There may be a sharp decrease in body weight along with anemia and fever. Thus, the human body is depleted, losing strength due to the fact that the tumor absorbs nutrients and energy. The body is quickly poisoned by the products of the activity of tumor cells, a fever occurs, which is permanent. Patients feel unwell due to severe pain and limited mobility. This can lead to depression.
Fibrosarcomas at the terminal stage metastasize to other structures, such as the liver and lungs. Abdominal pain appears along with shortness of breath and cough with hemoptysis.
What is the prognosis for fibrosarcomasoft tissue, see below.
Diagnostics
Due to the fact that this pathology has been latent in patients for a long time, seventy percent of patients seek medical help at the last stage of the disease. If there is the slightest suspicion of the presence of fibrosarcoma, the patient is sent for an initial diagnosis for x-rays and ultrasound. This makes it possible to find out exactly where the tumor is located, what size it has.
In order to detect secondary metastases, X-ray of the sternum, ultrasound of the abdominal region and scintigraphy of the skeleton as a whole are performed. Biochemical analysis is carried out to assess the general condition of the patient and, looking at this, they decide whether it is possible to perform an operation aimed at removing the oncological disease. The method of final diagnosis is a biopsy. As part of this procedure, a part of the formation is taken for histological analysis.
There are two types of biopsy in total:
- Puncture biopsy is performed using an injection with a special needle, through which the punctate (that is, the contents of the neoplasm) is taken.
- Performing an open biopsy is carried out by surgery. This technique gives more accurate results, but is dangerous due to the possible activation of tumor growth.
Treatment of pathology
The method of treatment of fibrosarcoma is selected by the doctor depending on the area, size, stage of the disease andgeneral well-being of the patient. Modern medicine offers the following techniques:
- Performing surgical removal allows you to completely excise the neoplasm. This method of treatment is most effective in removing highly differentiated fibrosarcoma. In the event that the tumor has a high degree of malignancy, in addition to the operation, a course of radiation therapy or chemotherapy is prescribed.
- Radiation treatment is carried out in the format of brachytherapy or remote exposure through rays. A special component is introduced into the area of tumor localization, which enhances the effect of rays on fibrosarcoma. Through radiation therapy, it becomes possible to remove that part of the neoplasm that cannot be eliminated with the help of surgery (this is often practiced when removing a highly differentiated tumor). Irradiation is also used as an independent method of therapy in the event that the state of he alth of patients does not allow the removal of education using alternative methods.
- Carrying out treatment with medications, namely through chemotherapy. Doctors in this case use potent drugs in the form of "Cisplatin", "Cyclophosphan", "Doxorubicin" and "Vincristine". The drug is selected individually depending on the size, well-being of the patient and the structure of the tumor.
Neoadjuvant and adjuvant chemotherapy
Different between neoadjuvant and adjuvant chemotherapy. The first is used before surgery in order to reduce the size of the formation andelimination of metastases. This makes it possible to facilitate the removal of education during surgery. The second type of chemotherapy is used immediately after surgery to remove any remaining tumor particles.
At the end of therapy, the doctor continues to observe the patient for quite a long time. In the first three years, patients are required to visit an oncologist every three months, and then once a year or six months.
Fibrosarcoma stages
The following stages of the disease in question are distinguished:
- At the initial stage, the size of the tumor can vary within one to two centimeters. Depending on its position, the primary signs may differ. Fibrosarcoma of soft tissues at the initial stage (pictured) can be located in the mucous membranes or in the region of the submucosal layer in the form of a small node. In this case, the node, as a rule, does not go beyond the border of the fascial case. At this stage, the disease can proceed without any symptoms with a complete absence of metastases. Early diagnosis contributes to positive treatment outcomes.
- The main sign of the second stage is the damage to organs. As part of surgery, there is a need to extract tissue. The prognosis of this stage is already much worse, however, the frequency of relapses is extremely rare. At this stage, the tumor is already noticeable, it begins to penetrate through all layers, including the skin. Fibrosarcoma of soft tissues at this stage is characterized by the germination of the fascialneoplasms, the tumor in this case reaches three or even five centimeters.
- At the third stage of fibrosarcoma, neoplasms appear in the patient's body directly on neighboring organs. In addition to this, there may be the occurrence of regional metastases that become noticeable in the lymph nodes. Metastases accumulate in various lymph nodes. A tumor of the soft tissue, as a rule, disrupts the functions of organs, deforming them. The size of the neoplasm at this stage already reaches ten centimeters, and metastases actively enter the regional lymph nodes, which is accompanied by acute and intense pain. The prognosis for a patient whose pathology has moved to the third stage is very disappointing. Treatment in this case will be surgical intervention. The disease can recur.
- The tumor at the last (fourth stage) reaches a huge size. At this stage, as a rule, a tumor conglomerate is formed, which occurs due to compression and its penetration into neighboring organs. This can often result in severe bleeding. Metastases are diagnosed in regional lymph nodes, and signs of secondary cancer may also appear. Metastases may be found in the liver, lungs, and any other distant organs. The difference between this stage and the third stage is that the external manifestation of the pathology becomes more pronounced along with the presence of metastases in various organs.
Forecast: how long can you live?
Providedlocalization in the region of the extremities of highly differentiated fibrosarcoma, the prognosis is likely to be favorable. Of course, if it is detected at the initial stage. The situation is much worse if the tumor is detected too late, when it has already managed to metastasize to various organs. It is also worth noting that at advanced stages, regardless of the position of the pathological formation (whether it is fibrosarcoma of the soft tissues of the neck, thigh or abdominal space), the prognosis will be disappointing.
