Fibrosarkoma – a malignant tumor of a soyedinitelnotkanny origin. Develops in the thickness of muscles, long time asymptomatically can proceed. Represents the painless, round, dense, melkobugristy knot which is well delimited from surrounding fabrics. Skin over a tumor is not changed. The high-differentiated fibrosarkoma, as a rule, proceed rather favorably, low-differentiated can give hematogenic and limfogenny metastasises. The diagnosis is exposed taking into account the clinical symptoms given to a X-ray analysis, KT, MPT, a biopsy and other researches. Treatment – operation, radiotheraphy, chemotherapy.
Fibrosarkoma – the malignant new growth developing from unripe fibrous connecting fabric. Enters into group of sarcomas. Usually strikes deeply located fabrics (muscles, sinews, fastion). At the previous traumatic damages and impact of the ionizing radiation can arise in hypodermic fatty cellulose. Until recently the fibrosarkoma was considered as a widespread oncological disease, however as a result of immunohistochemical researches it was succeeded to establish that a considerable part of the pathological centers which were considered earlier as fibrosarkoma, make fibromatoses and fibrous gistiotsitoma. Occurs at adults seldom, usually develops aged from 20 up to 50 years. At children of younger age comes to light rather often. In age group up to 5 years of a fibrosarkoma make about 50% of total of oncological diseases of soft fabrics. Usually strike proximal departments of extremities (the lower extremities suffer more often than top), but can come to light also in other areas of a human body. Treatment by a fibrosarok is performed by experts in the field of oncology and orthopedics.
The reasons of development of a fibrosarkoma are not found out yet. As the possible contributing factor at children consider the chromosomal anomalies which are aggravated at violations of an embryogenesis as a result of adverse external effects. At adults the risk of emergence of a fibrosarkoma increases at the ionizing radiation previous repeated (for example, when performing radiation therapy concerning other malignant new growth). At the same time the time interval between receiving a high dose of the ionizing radiation and emergence of the first symptoms of a fibrosarkoma can make from 4 to 10-15 years.
Among other adverse factors experts specify injuries and the cicatricial changes arising after some diseases of soft fabrics. At the same time assume that traumatic damages do not cause development of a fibrosarkoma, and stimulate growth of already existing tumoral rudiment. Malignization of good-quality new growths is considered improbable, however some researchers point to a possible ozlokachestvleniye of fibromas, fibromyomas and fibroadeny. The majority are considered by a fibrosarok as spontaneous.
Classification of a fibrosarkoma
Distinguish two main types a fibrosarok: high-differentiated and low-differentiated. Tumors consist of the round or spindle-shaped cages which are settling down between collagenic fibers. In fabric high-differentiated by a fibrosarok the correct arrangement of spindle-shaped cages and fibers of collagen comes to light. The weak vaskulyarization, insignificant quantity of mitoses and polymorphism of cages of various degree of expressiveness are noted. The centers of a necrosis are poorly expressed or are absent. The high-differentiated fibrosarkoma are inclined mainly to local destructively current, metastasis is uncharacteristic.
Distinctive feature low-differentiated by a fibrosarok is prevalence of cages over collagenic fibers. The intensive vaskulyarization, the expressed cellular polymorphism, kernels and a large number of mitoses are found. In fabric of a fibrosarkoma the large sites consisting of polygonal cages are looked through. When progressing oncological process in the field of these sites the necrosis centers which extend to the next zones develop and become the reason of disintegration of a tumor.
The low-differentiated fibrosarkoma differ in more aggressive growth, can affect lymph nodes and give hematogenic metastasises, mainly – in lungs, is more rare in bones and a liver. Both types a fibrosarok have high tendency to a retsidivirovaniye. Some experts note that the number of the patients addressing concerning a fibrosarkoma recurrence, exceeds the number of patients with for the first time the revealed new growth. Lack of the capsule, a possibility of existence of several rudiments of a tumor in one anatomic zone, tendency to aggressive growth and insufficiently radical surgery become the reasons of development of a recurrence. Apply traditional four-phasic classification and classification of TNM to assessment of prevalence of a fibrosarkoma.
Clinical manifestations depend on localization of a tumor and prevalence of process. At an arrangement in the depth of soft fabrics of a fibrosarkoma can proceed long time asymptomatically. New growths become a casual find when carrying out inspection in connection with other disease. Many patients for the first time see a doctor, only when the fibrosarkoma reaches the considerable sizes, causes deformation of the struck area or becomes the reason of development of a contracture of a nearby joint. Fibrosarkoma of proximal departments of extremities, can come to light because of development of the pain syndrome caused by a sdavleniye of nerves or involvement of a periost earlier.
Skin over a tumor is usually not changed. At the fast-growing large and superficially located fibrosarkoma thinning of integuments, a cyanotic shade of skin and expanded network of hypodermic veins in a new growth zone can be noted. At a palpation single oval or round opukholevidny formation of a dense consistence is found. Characteristic of all types of sarcomas, including – and a fibrosarok the impression of limitation of knot, "the false capsule" or the pseudo-capsule representing layers of fibrous fabric is. When progressing false borders of a tumor become less expressed.
Degree of mobility of a fibrosarkoma is defined by prevalence of process. Small local educations can be displaced (more often – in the cross direction). At germination of surrounding fabrics of a fibrosarkom becomes motionless. At a new growth arrangement in intermuscular space knots are well palpated during relaxation of muscles, lose contours and lose mobility at a muscular tension. At initial stages of a fibrosarkoma are usually painless. At a sdavleniye of nerves morbidity at a palpation is noted. At damage of a bone of pain become constants.
At late stages of a fibrosarkoma symptoms of the general intoxication come to light. The patient loses the weight and appetite. Temperature increase, anemia, the increasing weakness, emotional lability, a depression or a subdepression are noted. At emergence of the remote metastasises signs of defeat of appropriate authorities are observed. At metastatic damage of bones there is a persistent pain syndrome which is not eliminated with the anesthetizing medicines. At metastasises in lungs cough, short wind and a blood spitting are noted; at metastatic cancer of a liver jaundice and increase in body come to light.
Diagnostics of a fibrosarkoma
Timely diagnosis of this disease is one of urgent problems of oncology. The long asymptomatic current, imaginary inoffensiveness of a fibrosarkoma and lack of oncological vigilance among doctors of the general profile lead to the fact that the diagnosis in 70-80% of cases is exposed at the started disease stages. The partial solution of this problem consists in careful collecting the anamnesis and special attention to the myagkotkanny volume educations located in places, typical for a fibrosarkoma, – in the field of proximal departments of extremities.
At a stage of primary diagnostics of patients with suspicion on fibrosarky direct to a radiological research. According to a X-ray analysis the knot of an ovoidny form decides on uniform structure and indistinct borders. In the field of knot sites of calcification can come to light, at widespread processes the bone uzuration is noted. For specification of the diagnosis appoint the ultrasonography of soft fabrics allowing to estimate more precisely the size and structure of a fibrosarkoma, and also level of its vaskulyarization. During a research carry out a punktsionny or tolstoigolny biopsy.
For definition of a stage of a disease and obtaining fuller data on structure, the size and an arrangement of a fibrosarkoma if necessary appoint KT and MPT. Apply a X-ray analysis of a thorax, KT of a thorax, ultrasonography of abdominal organs and a stsintigrafiya of bones of a skeleton to identification of the remote metastasises. For assessment of the general condition of the patient and making decision on a possibility of surgery appoint the general blood tests and urine, biochemical blood test and other researches (depending on the revealed pathology).
Treatment and the forecast at a fibrosarkoma
Depending on prevalence of process treatment of a fibrosarkoma can be radical or palliative. The purpose of radical treatment is elimination of malignant cages, the prevention of a retsidivirovaniye and metastasis, and also restoration of function of the struck area. As main objectives of palliative treatment of a fibrosarkoma consider improvement of quality and increase in life expectancy of the patient.
In the course of radical treatment mainly organ-preserving operations – a local, radical or sectoral resection are used. The minimum volume of the excised visually not changed fabrics at a fibrosarkoma has to make not less than 5-6 cm. Operation can include plasticity of large vessels, replacement of the formed defects of bones and soft fabrics. And an exarticulation it are required for amputation no more than in 10% of cases and are applied at large widespread new growths in a disintegration stage. At II and subsequent stages of a fibrosarkoma in before - and the postoperative period carry out radiation therapy. Application of intraoperative radiation and brachytherapy is possible. At the III stage in addition include chemotherapy in the plan of treatment. Appoint himiopreparata and symptomatic funds to the IV stages.
At the high-differentiated fibrosarkoma and early stages of the low-differentiated tumor the forecast rather favorable. At the widespread low-differentiated fibrosarkoma five-year survival does not exceed 40-50%. The risk of development of a recurrence directly depends on prevalence of primary oncological process. All patients fibrosarkomy within 3 years after the end of treatment have to have quarterly the general examination, ultrasonography of the struck area, ultrasonography of abdominal organs and a X-ray analysis of a thorax. Within the next 2 years of inspection carry out time to half a year.