Fibrosarcoma: what is it, causes, treatment, prognosis

The fibrosarcoma relates to Magadana malignant tumors, and the basis formed by the immature connective tissue. The bulk of patients with this neoplasia are children and young people, adulthood and old age the disease is diagnosed only rarely. The highest frequency of all cases of cm detected at the age of five, where it accounts for almost half of all soft-tissue tumors.

Typically, the tumor affects the limbs, and the legs are affected more often and being located deep in the muscles, ligaments, fat layer for a long time unnoticed. Another possible localization of the cm, particularly the retroperitoneum. This form of pathology is very dangerous, as there are certain difficulties in diagnosis and surgical removal can be very traumatic and even not feasible due to involvement of internal organs.

Often soft tissue tumors called cancer, but this is not true. As you know, cancers are of epithelial origin sarcoma – connective tissue, a fibrosarcoma and therefore wrongly called «muscle cancer», «cancer leg», etc.

Causes and types of fibrosarcoma

On unraveling the causes of tumors scientists are fighting for many years, but until now it remains unclear the origin of a large number of diseases. Why develop a fibrosarcoma – the question of the number unresolved. There is evidence that the role of chromosomal anomalies and spontaneous mutation that occurred in utero under the influence of external causes, and then the tumors can appear in childhood.

Фибросаркома: что это, причины, лечение, прогноз

The risk of cm in adults is associated with ionizing radiation. For example, repeated exposure in connection with another cancer, unnecessary x-rays. It is important to note that the tumor may appear after 10-15 years after the fact of exposure, which may sick by the time even to forget.

Among the possible reasons are considered traumatic injuries and scars. Because the majority of scientists are inclined to spontaneous appearance of tumor node, injuries and scars are perceived more as a provoking factor that triggers the growth of already existent neoplasia, which, from time to time was in «sleep» state.

In any type of tumor it is important to determine its histological types and degree of differentiation of the cell component. Fibrosarcoma in two varieties:

  • Vysokomehanizirovannoe;
  • Poorly-differentiated neoplasia.

Vysokomehanizirovannoe fibrosarcoma has a lower potential of malignancy and grow slowly. It consists of verminophobia cells that is correctly oriented in the ground tissue and surrounded by collagen fibers.

Poorly-differentiated fibrosarcoma is very malignant, metastasizes early, grows quickly, depleting the patient in a short time. It consists mainly of cells that are supported by a small number of the fibrous component. The cells of such a tumor is very pleomorphic, very different from the normal, actively dividing, therefore, pathological mitoses – a characteristic feature of this variant of CIN.

Фибросаркома: что это, причины, лечение, прогноз

histological the cm

Fibrosarcoma is able to spread as lymphatic and blood vessels, but hematogenous route priority. Secondary neoplastic nodes found in lungs, bones and liver. Tumor ingrowth in surrounding tissues is accompanied by their destruction, damage to blood vessels and nerves, introduction to bones.

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Manifestations cm

Symptoms of fibrosarcoma depends on the place of growth, education, size, involvement of surrounding tissue, neuro-vascular trunks. The insidiousness of neoplasia is that for a long time she may not give any symptoms, and the patient will seek help when the tumor reaches a significant volume. Located in the deep soft tissues or muscles, it some time does not prevent movement and does not cause pain, so it goes unnoticed.

Фибросаркома: что это, причины, лечение, прогноз

Magadana sarcoma on the leg

The growth of cm, there is a configuration change of the limb – deformity, tumor formation and increase in size become visible to the naked eye. If the tumor invades the elements of the joint, disturbed mobility, the patient limps or is unable to perform movements with his fingers and hands.

When probing the supposed locations of the tumor may reveal a dense knot, the skin over which is either not changed, or becomes thinner and contains many dilated veins. Germination fibrous tissue of the lesion malignant transformation creates the impression of similarity of the capsule, and the tumor can be taken as quite benign, usually having clear boundaries.

Small cm can easily be displaced by palpation, but as their sizes increase, and lost the mobility of Neoplasia is embedded in tissue elements and to move it somewhere becomes difficult. The pain manifests in the involvement of nerves and blood vessels, and if the tumor has invaded the bone, the pain will be constant and very painful.

The growth of tumors and unavoidably violate General well-being. Taking away the nutrients and energy substrates, fibrosarcoma promotes the loss of body weight until exhaustion, anemia, severe weakness. Tumor metabolites poison the body, increases intoxication, fever becomes permanent. The increasingly deteriorating health, limitation of usual activity, pain will inevitably lead to depression, depression, reduced emotional background.

The fourth stage of the cm of the soft tissues is evident not only symptoms of intoxication and cachexia, but also signs of metastatic lesions of other organs. Most often affects the lungs and the liver, and the patient complains of cough, shortness of breath, abdominal pain or jaundice.

Diagnosis and treatment

Unfortunately, more than 70% of patients come to the doctor at an advanced stage. The culprit is a long latent period and a meager symptomatology, not pushing a patient on emergency visit to the oncologist.

If you suspect a fibrosarcoma patient first sent for x-rays, then perform ultrasound examination of the affected area. Clarifying methods consider CT and MRI. The survey plan must include laboratory studies of blood and urine, ultrasound examination of abdominal cavity, chest x-ray and scintigraphy of the skeleton to exclude metastases.

Фибросаркома: что это, причины, лечение, прогноз

left: CT scan, right: ultrasonography of the abdominal cavity

Treatment of cm depends on the stage, the localization of the formation, size and degree of involvement of the surrounding tissues. Oncologist is surgical method, which is considered a staple in the majority of cases, possible chemotherapy and radiation therapy. For each patient the physician selects the optimal treatment, but possibly a combination of both.

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Surgery allows you to more fully remove the tumor tissue, and the best results are achieved with highly differentiated fibrosarcoma. By increasing the degree of malignancy only one operation may not be enough, and then she is complemented by irradiation or chemotherapy. The combination of last two methods without surgery is possible when the patient and high operational risk, but in this case, the probability of complete removal of the tumor and, consequently, cure is very small.

The risk of recurrence of the neoplasm and the possibility of distant metastases are always malignant neoplasia, and the lower the degree of differentiation of cm, the more likely to have these complications. For their prevention in the postoperative period addressed the issue of additional antitumor conservative treatment, good results show neo — and adjuvant therapy.

Surgical treatment consists in excision of the neoplastic tissue. Since cm often located on the extremities, until recently, surgeons were mainly amputations. However, the analysis of treatment results showed that organ-sparing surgery are not inferior in their effectiveness, but allow you to save and limb and its function, minimizing disability patients.

Organ-sparing interventions consider all possible resections, but the extent of resection should be at least 5-6 cm Radical treatment – amputation of the limb. In the second stage of the disease there is a need radiation before and after surgery, and at the third stage is added to chemotherapy. Fourth, most severe stage often leaves no possibility to remove the tumor, so treatment includes symptomatic treatment and chemotherapy.

Фибросаркома: что это, причины, лечение, прогноз

fibrosarcoma prepared for surgical removal

If the volume of the tumor requires removal of large tissue fragments, the treatment is supplemented by various types of plastics and reconstructive surgery, the possible transplantation of patient’s own tissues, setting synthetic materials.

The indications for amputation can be:

  • A large volume of diseased tissue, when it is not possible to save the limb (the affected muscles, large vessels and nerve trunks involved joints, etc.);
  • The growth of cm on the hands or feet.
  • In these cases, issues of scale of intervention solved individually, taking into account the risks of metastasis and tumor recurrence. When fibrosarcoma of the hand or the foot amputation and disarticulation are considered to be quite effective.

    Radiation therapy is used in the form of remote irradiation and brachytherapy when the tumor growth area introduces features that enhance the local effect of the irradiation. When fibrosarcoma high-grade a maximum total dose of 70 Gy, divided in 30-35 sessions. In the case of highly differentiated fibrosarcoma irradiation helps to remove residual tumor tissue that cannot resect surgically, while it must be commenced not later than one month from the date of the transaction.

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    As an independent method of treatment of radiation is used for inoperable neoplasia, that is, localization, tumor size, the patient’s condition does not allow her to excise. Irradiation of such patients are assigned a maximum dose of 70 Gy in 35 sessions.

    Medical treatment with chemotherapy is quite effective in soft-tissue malignant tumors. Used tools – doxorubicin, vincristine, cyclophosphamide, cisplatin, and others. The scheme is chosen individually in accordance with the histological structure of the neoplasm and its sensitivity to a particular drug.

    Фибросаркома: что это, причины, лечение, прогнозIf the chemotherapy is carried out before the planned removal of the tumor, it is called neoadjuvant. The drug is prescribed in some courses with an interval of two to three weeks. The goal of neoadjuvant treatment is to destroy microscopic metastases and reduce the tumor size, which further facilitate the task of the surgeon and will allow you to save the maximum amount of tissue.

    Post-operative treatment (adjuvant chemotherapy) are directed against the possible «remains» of the tumor and micrometastases and distant foci of tumor growth. The drug is prescribed in some courses, but not less than three.

    In parallel with the main stages of treatment, symptomatic and supportive therapy. The patient required analgesics, antiemetics, drugs that reduce nausea (especially during chemotherapy), vitamins and minerals. In severe cases of infusion for the restoration of acid-base balance and electrolyte balance of the blood.

    After treatment it is necessary to provide monitoring and patient care that can be achieved by regular visits to the oncologist. The first three years it is done once in three to four months, in the fourth year – every 6 months, then annually.

    In addition to the examination, should undergo x-ray chest and ultrasound abdomen to exclude metastatic foci annually with fibrosarcoma differentiated and aggressive tumors every 6 months. To exclude relapse is assigned to CT and/or MRI of the affected region every year.

    The prognosis for fibrosarcoma can be successful only with a high degree of differentiation of malignancy early detection of the disease, provided patients of all stages of cancer treatment. If fibrosarcoma is discovered later, has a high degree of malignancy, has managed to have metastatic screenings, the probability of a successful outcome is dramatically reduced, and survives not more than half of patients.

    Video: the place of radiotherapy in treatment of soft tissue sarcomas