Desmoidny tumor – the tumor developing from musculo structures and which is intermediate between good-quality and malignant new growths. It is inclined to germination of surrounding fabrics, but does not give the remote metastasises. Can arise on any part of the body, it is more often localized in the field of a forward belly wall, a back and a humeral belt. Represents the dense opukholevidny education located in the thickness of muscles or connected with muscles. When progressing can sprout vessels, bones, joints and internals. The diagnosis is exposed on the basis of survey and these additional researches. Treatment – surgeries, radiation therapy, chemotherapy.
Desmoidny tumor (, desmoidny fibroma, musculo a fibromatosis) – the rare soyedinitelnotkanny tumor developing from fastion, muscles, sinews and aponeuroses. It is microscopically deprived of signs of a zlokachestvennost and never gives the remote metastasises, at the same time it is inclined to the local aggressive growth and a frequent retsidivirovaniye (quite often repeated) therefore oncologists consider as a conditional and good-quality new growth. Makes 0,03-0,16% of total of new growths. In 64-84% of cases women suffer.
At women the desmoidny tumor usually arises on the second or third decade of life, in 94% of cases comes to light at the giving birth patients, in 6% of cases – at not giving birth. For male patients it is more often diagnosed at children's or teenage age. In the postpubertatny period the number of cases at men sharply decreases. Slow progressing is usually observed. Diagnostics and treatment of desmoidny tumors experts in the field of oncology, dermatology carry out, surgeries, traumatology and orthopedics.
Reasons and patanatomiya of a desmoidny tumor
The reasons of development of a desmoidny tumor remain obscure so far. As one of the most probable factors experts consider traumatic injuries of muscles, sheaves and aponeuroses (including – in the course of childbirth at women). Besides, researchers point to possible communication of a desmoidny tumor with the level of sex hormones and some genetic disorders. Statistically, it is diagnosed for 20% of the patients having family adenomatosis – the hereditary disease caused by a genetic mutation.
The Desmoidny tumor represents the single knot dense, as a rule, having a fibrous structure. Color of a new growth on a section grayish-yellow. At a microscopic research of a desmoidny tumor the bunches of collagenic fibers located diversely and intertwining among themselves are visible. Mature fibrotsita and fibroblasta come to light. Mitoses arise very seldom. At a research of visually not changed surrounding fabrics excised together with a new growth microscopic elements of a tumor can be found.
Symptoms of a desmoidny tumor
The Desmoidny tumor can develop in any part of a body, however most often settles down on a forward surface of a belly wall. Enough widespread localizations are also among a back and area of a humeral belt. On a thorax, the top and lower extremities desmoidny tumors develop seldom, however, such new growths have special value as often settle down close to bones and joints, are intimately accustomed to drinking with nearby educations or sprout them, breaking mobility of joints, durability and an opornost of bones.
Desmoidny tumors on hands and legs are always localized on the sgibatelny surface of an extremity. Depending on features of defeat of fabrics distinguish four clinical types of ekstraabdominalny desmoidny tumors: single knot with defeat of a surrounding fastion, single knot with damage of fastsialny vaginas on an extent, multiple knots on various parts of the body and malignant regeneration of a desmoid – transformation of a new growth into desmoid-sarcoma.
Along with ekstraabdominalny allocate intraabdominalny and ekstrabryushny desmoidny tumors which can settle down in a bryzheyka of a small intestine, in zabryushinny space, area of a scrotum and a bladder. Similar new growths come to light less often than peripheral desmoid and desmoid of a forward belly wall. Desmoidny tumors in the field of a bryzheyka are quite often combined with family adenomatosis. Symptoms of a disease depend on localization and the size of a new growth, existence or lack of germination of nearby bodies and fabrics.
Slow growth and malosimptomny current is characteristic of a desmoidny tumor. At desmoidny tumors of the big size morbidity can be observed. At germination of joints contractures are possible, at germination of bones – pathological changes, at germination of internals – malfunction of these bodies. During external survey the dense inactive new growth of a rounded or oval shape with a smooth surface which is settling down in the thickness of muscles or connected with muscles and ligaments is found.
Diagnostics and treatment of a desmoidny tumor
The diagnosis is exposed on the basis of survey, the yielded tool researches and results of a biopsy. Patients with a desmoidny tumor are directed to ultrasonography, KT and MPT. The most informative method of a research allowing to establish borders of a tumor and degree of its invasion in surrounding fabrics is the magnetic and resonant tomography. If necessary appoint an angiography of vessels and other researches. At germination of vessels, nerves, internals and bone structures consultation of the vascular surgeon, neurologist, abdominal surgeon, thoracic surgeon, traumatologist, orthopedist and other experts can be required.
Because of high probability of a retsidivirovaniye of desmoidny tumors the combined treatment including surgeries and radiation therapy is preferable. In some cases apply chemotherapy and hormonal therapy. According to various data, desmoidny tumors recur in 70-90% of cases after the expeditious treatment used as a monotechnique. Performing the combined therapy allows to reduce the frequency of a recurrence significantly.
Operation whenever possible has to be radical. In the presence of single knot it is deleted together with the struck fastion and surrounding muscles. At spread of a desmoidny tumor on a fastsialny vagina excise a fastion on an extent. At germination of bone structures carry out removal of a kortikalny plate or rezetsirut a bone tissue. At damage of internals, a close arrangement of vessels and nerves tactics of operation is defined individually.
Excision of the big massif of fabrics at large desmoidny tumors leads to formation of defects. At an opportunity such defects eliminate right after removal of a tumor, carrying out local plasticity, using bone auto-and gomotransplantata etc. In some cases in the remote period perform plastic surgeries. At damage of a nearby joint of an extremity endoprosthesis replacement can be required. At multiple desmoidny tumors stage-by-stage surgical treatment with especially radical removal of surrounding fabrics as such new growths show the increased tendency to a retsidivirovaniye is shown.
To adult patients with a desmoidny tumor appoint preoperative and postoperative radiation therapy. A lack of a method are the expressed post-beam changes of fabrics. The trophic violations and large hems arising after performing radiation therapy can complicate repeated surgical treatment in case of a retsidivirovaniye. In the course of treatment of patients of children's age radiation therapy is not applied because of possible premature closing of rostkovy zones of a bone in the field of radiation. At desmoidny tumors at children perform surgeries against the background of preoperative and postoperative medicamentous therapy. To patients appoint low doses of tsitostatik and antiestrogenovy medicines. Duration of drug treatment of a desmoidny tumor can make up to 2 and more years.
The forecast at the combined treatment including surgical intervention and radiation therapy or surgical intervention and himio-hormonal therapy, rather favorable. A recurrence comes to light at 10-15% of patients, usually – within 3 years after removal of a desmoidny tumor. Most often recur the desmoida located in the field of foot and a shin. The remote metastasises are not formed. The lethal outcome is possible at germination of vitals, usually – at an arrangement of a desmoidny tumor in the head, a neck, a stomach and a thorax.