Soft tissue fibrosarcoma is a malignant tumor based on bone material. The tumor develops in the thickness of the muscles and can last a very long time without certain symptoms. This disease is found in young people, and also in children (such an audience makes up about fifty percent of all soft tissue tumors).
Among the elderly is rare. Basically, such a tumor attacks the lower limbs. Arising in the muscles, under the layer of fat and in the ligaments, the formation can be invisible for a long time. Fibrosarcoma of soft tissues, 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. Let us consider in more detail what fibrosarcoma is, find out why 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 similar neoplasia are young people and children. As for adulthood and advanced age, such a disease is rarely diagnosed in this category of people. The highest frequency of all cases of fibrosarcoma is detected before the age of five. During this period, pathology accounts for almost half of all neoplasms of soft tissues.
Limb affection
Typically, 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 extremities 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 fibrosarcomas of soft tissues are possible, in particular, in the area of retroperitoneal space. This form of pathology is very dangerous, since there are certain difficulties in diagnosing, and directly surgical manipulations can become very traumatic and even impossible due to the involvement of internal organs.
Often a soft tissue tumor is called cancer, but this is not entirely correct. As is well known, cancer is epithelial in origin, and fibrosarcoma is connective tissue, so it is not legitimate 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 through the lymphatic vessels, but also through the blood vessels, although the hematogenous pathway is perhaps the predominant one. Neoplastic secondary nodes can be found in the lungs, in the liver, and in the bones. The growth of a tumor into the surrounding tissue may be accompanied by its destruction, and in addition, damage to nerves and blood vessels and implantation into the bone. Next, we find out what are the causes of this dangerous pathology.
Reasons for education
The reasons for the development of soft tissue fibrosarcoma (pictured) are still unknown. There is a version that chromosomal mutations that cause failure in the womb can provoke the appearance 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 cancer). 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 are abundant.
Tumors are divided into two main types:
- Highly differentiated look.
- Low grade view.
Highly differentiated fibrosarcomas are characterized by a low degree of malignancy, and in addition, weaker growth in comparison with low-grade. Her cells, which are surrounded by collagen fiber, look like healthy tissues. Such tumors do not have a special effect on the body, and at the same time do not give metastases to the neighboring structure.
Low-grade fibrosarcomas are more aggressive forms of the disease. The cells of this tumor can be very different from healthy ones, and at the same time 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 pathway. Mostly metastases enter the liver, bone structure and lungs. Metastasizing to adjacent tissue 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, trunk, hips and lower leg may be affected. Fibrosarcoma of the soft tissues of the thigh, for example, has a low degree of malignancy.
- The occurrence of dermatofibrosarcoma is a rare type of disease. Such a formation can develop in connective tissue and is located on the skin surface. It is also called a Daria tumor.
- The appearance of neurofibrosarcoma is a dangerous form of the disease that develops around the nerve fiber. Fifty percent of cases can occur in people with a disease called neurofibromatosis.
- Myxoid fibromas are a rare form of the disease that affects cartilage. Education is considered benign, it makes up about one percent of all bone tumors.
- Infantile fibrosarcoma is a malignant formation found in infants and children under five years of age. It can be characterized by aggressive growth, and in addition, very high malignancy. Pathology usually affects the limbs, but, in addition, can occur on the neck and head.
- Ovarian fibrosarcomas are considered a rare form of oncology, accounting for about four percent.
Photo of fibrosarcoma of soft tissues at the initial stage is of interest to many.
Symptoms of the disease
The manifestation of fibrosarcoma largely depends on the place of its localization, size, malignancy of the neoplasm. Highly differentiated tumors for a long time may not be felt, they are detected randomly during examination of the patient 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 low-grade fibrosarcomas appear within the first twelve months from the moment of formation. Sometimes fibrosarcoma (pictured above) is detected earlier due to pain resulting from compression of nerve endings and bone deformation.
Above the surface of the resulting tumor, the skin does not change. Only on the background of the rapidly growing form of fibrosarcoma, thinning can be observed along with bluish skin in the area of localization 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.
Small fibrosarcomas are able to shift 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 the tumor into the bone, the pain becomes painful and becomes chronic.
Symptoms of fibrosarcoma are very unpleasant. Increasing in size, it affects the general well-being of the patient. A sharp decrease in body weight can be observed 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, and a fever occurs that is permanent. Patients feel unwell due to severe pain and limited mobility. This can lead to depression.
At the terminal stage, fibrosarcomas metastasize to other structures, for example, to the liver and lungs. There is abdominal pain along with shortness of breath and coughing with hemoptysis.
What is the prognosis for soft tissue fibrosarcoma, we will describe below.
Diagnostics
Due to the fact that this pathology occurs in patients in a latent form 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 x-ray and ultrasound for initial diagnosis. This makes it possible to find out exactly where the tumor is located, what size it has.
In order to identify secondary metastases, a chest x-ray, ultrasound of the abdominal region, and skeleton scintigraphy as a whole are performed. Biochemical analysis is carried out to assess the general condition of the patient and, looking at this, decide on whether it is possible to carry out an operation aimed at removing the cancer. The final diagnosis method is a biopsy. As part of this procedure, part of the education is taken to perform histological analysis.
In total, two types of biopsy are distinguished:
- A puncture biopsy is performed by injection with a special needle, through which punctate is taken (i.e. the contents of the neoplasm).
- An open biopsy is performed in an operational way. This technique gives more accurate results, but is dangerous due to the possible activation of tumor growth.
Pathology treatment
The method of treatment of fibrosarcoma is selected by the doctor depending on the area of the position, size, stage of the disease and the general well-being of the patient. The following techniques are offered by modern medicine:
- Performing surgical removal allows you to completely excise the tumor. This treatment technique is most effective in removing highly differentiated fibrosarcoma. In the event that the tumor is highly malignant, in addition to surgery, a course of radiation therapy or chemotherapy is prescribed.
- Radiation treatment is carried out in the form of brachytherapy or remote exposure through rays. A special component is introduced into the tumor localization region, which enhances the effect of rays on fibrosarcoma. By means of radiation therapy, it becomes possible to remove that part of the neoplasm that cannot be eliminated with the help of surgical intervention (this is often practiced when removing a highly differentiated tumor). Irradiation is also used as an independent method of therapy if the state of health of patients does not allow to remove the formation using alternative methods.
- Conducting treatment with medication, namely through chemotherapy. Doctors in this case use potent drugs in the form of “Cisplatin”, “Cyclophosphamide”, “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
Neoadjuvant and adjuvant chemotherapy are distinguished. The first is used before surgery in order to reduce the size of the formation and eliminate 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 possibly remaining tumor particles.
Upon completion of therapy, the doctor continues to observe the patient for a sufficiently 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 symptoms may vary. 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. Diagnosing at an early stage contributes to positive treatment outcomes.
- The main sign of the second stage is organ damage. As part of surgery, there is a need for tissue extraction. The prognosis of this stage is already much worse, however, the recurrence rate is extremely rare. At this stage, the tumor is already noticeable, it begins to penetrate through all layers and skin, including. Soft tissue fibrosarcoma at this stage is characterized by the germination of a fascial neoplasm, the tumor in this case reaches three or even five centimeters.
- At the third stage of fibrosarcoma in the patient’s body, neoplasms arise directly on neighboring organs. In addition to this, the occurrence of regional metastases can be noted, which become noticeable in the lymph nodes. Metastases accumulate in various lymph nodes. A soft tissue tumor, 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 passed to the third stage is very disappointing. The treatment in this case will be surgical intervention. The disease can recur.
- The tumor at the last (fourth stage) reaches enormous size. At this stage, as a rule, a tumor conglomerate is formed, which occurs due to compression and its penetration into neighboring organs. Often, severe bleeding can be caused. Metastases are diagnosed in regional lymph nodes, and signs of secondary cancer may also appear. Metastases in the liver, lungs, and any other distant organs can be detected. 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 much can you live?
Given the localization of highly differentiated fibrosarcoma in the limb region, 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 give metastases to various organs. It is also worth noting that in advanced stages, regardless of the position of the pathological formation (be it fibrosarcoma of the soft tissues of the neck, thigh or abdominal space), the prognosis will be disappointing.