Rhabdomyosarcoma - a tumor disease with high malignancy that develops from certain elements of striated muscle.Rhabdomyosarcoma affects different age groups, but more often it affects the middle and older generation.
This tumor pathological character takes fourth position in the number of diagnosed cases and is almost 7% of all observations.Moreover, among males this anomaly can be found much more often than in the female half.Typically, such a tumor localized at the extremities, neck, head and pelvis.
Rhabdomyosarcoma can manifest itself in various forms, among which the polymorphocellular, embryonal, alveolar and botrioidnuyu.All of them at the macroscopic study is a limitation in the form of a smooth or bumpy capsule fraudulently with tenderloin and myagkovata consistency.
rhabdomyosarcoma have a sectional gray-pink hue with some areas of the yellow-brown color, which can sometimes crumble.Also in this pathology it is often possible to find pockets of necrotic and hemor
etiological factors of rhabdomyosarcoma is not well understood.
There are some suggestions in the development of pathological diseases of genetic factors that may influence cancer-causing substances.Because of this disturbed metabolism of cells striated muscle, their entire cycle, and the mechanism of formation.These new cells begin to divide rapidly and form tumors, which can then be visually seen.This is confirmed by pathology detected immediately after birth, as well as the appearance of their siblings have.In addition, associated with rhabdomyosarcoma Lee Fra Skills syndrome, which is caused by mutations of the gene suppressor.
There are also case reports of other pathology combination with rhabdomyosarcoma such as congenital retinoblastoma, neurofibromatosis, familial polyposis, a giant congenital melanocytic nevus, epidermal nevus syndrome, Gorlin-Goltz, albinism and multiple lentigines syndrome.
also not revealed until the end of the influence of exogenous and endogenous factors on the properties of the incidence of rhabdomyosarcoma.But the hereditary factor in this case can not be excluded.After all, there are assumptions and cases that malignancy of rhabdomyosarcoma is more common in families where there were already documented pathological anomalies.These include such as osteosarcoma, brain tumors, breast cancer, melanoma and others. Rhabdomyosarcoma in such cases is an early manifestation of primary shapes, unfavorable course and prognosis, as well as a high probability of re-formation.
Among the risk factors emit radiation exposure of the mother during pregnancy, genetic predisposition, toxins and trauma.
This form is considered a neoplasm tumor with high malignancy and accounts for nearly 20% of all rhabdomyosarcoma, which occur in childhood.Among patients with this form of pathology predominant amount observed in children up to ten years, but sometimes tumor affects people more and adulthood.
Usually, alveolar rhabdomyosarcoma is localized on the trunk, head (usually near the eye socket), neck, extremities (in most cases), the tissues in the pelvis.Alveolar rhabdomyosarcoma mostly developing very deep in the soft tissues of the structure.As for this tumor is characterized by extreme aggression, patients are turning to professionals to have lymph node metastases and penetration into various tissues and organs.It can be the lungs, central nervous system and bones.
Grossly rhabdomyosarcoma looks like a soft node whitish-yellow color in diameter from two to eight centimeters.A microscopic analysis fails to detect cancer cells that grow in the form of nests or very close to each other and separated by distinctive layers.
The most distinctive feature of alveolar rhabdomyosarcoma is the alveolar structure of the cells of the tumor.They are formed by a large number of fibrous septa and chaotic localized vessels.In central regions of most marked scattered location of tumor cells, and on the periphery - a syndrome bonding, which is expressed in these cells closely with septa of connective tissue or blood vessels, which combines them with papillary structures and glandular.Alveolar rhabdomyosarcoma
its structure is a later stage in the development of muscle fibers embriogeneznogo unlike embryonic rhabdomyosarcoma type.For alveolar rhabdomyosarcoma tumor cells characteristic of mitotic activity.Therefore, so far this form of cancer diagnosed as immunoblastic lymphoma, angiosarcoma, synovial sarcoma, neuroblastoma, and others.
The main features of alveolar rhabdomyosarcoma tumor nodules are in the form of single or multiple formations in deep muscle tissue.On palpation they are dense and flexible education, which is related to their location deep.This tumor has fuzzy borders, rapidly growing, painless, does not disturb the function at the location and is accompanied by veins located beneath the skin.Unlike other malignant pathologies, characterized in alveolar rhabdomyosarcoma early germination capacity and her skin ulceration in the formation of bleeding exophytic character growths.This type of rhabdomyosarcoma observed partial or absolute limit her mobility.
only after clinical and radiological examination fails to assume a malignant tumor in the structure of soft tissue.Definitive diagnosis made after the morphological study of tumors.
relapse when alveolar rhabdomyosarcoma occurs in 50% of patients.
embryonal rhabdomyosarcoma or rabdopoeticheskaya sarcoma is considered to be a rare form of malignant disease, and accounts for nearly 1/3 of all types of skin tumors.Histological examination of the pathological formation of a stage of embryonic development normal striated muscle.They are formed by a variety of cells that can be round, stellate, thin, elongated bipolar resembling bundles weave or sinovilnuyu mass.Embryonal rhabdomyosarcoma cells are mainly in myxomatous substance.This type of cancer is the mucous abnormalities, amorphous substance dispersed embryonic muscle cells that have otroschatuyu cytoplasm.Also revealed gigantic symplasts with more cores.
Embryonal rhabdomyosarcoma is localized predominantly in the neck and head, as a rule, in the tissues of the orbit.Also, this type of tumor can diagnose bladder, testicles, vagina and prostate.Embryonic rhabdomyosarcoma mostly diagnosed in infants and newborns.
Clinical picture consists of the appearance of seals in the abdominal area, and gastrointestinal disorders.All symptoms underlies the localization of tumor focus, and then its distribution lymphogenous and hematogenous route.
Treatment of rhabdomyosarcoma
As a rule, used to treat rhabdomyosarcoma complex therapy, which includes: a radical surgical removal of abnormal growths, chemotherapy, and radiation exposure.
Modern Chemotherapy involves the use of cytotoxic drugs such as cyclophosphamide and ifosfamide, which are alhiliruyuschimi agents;Vincristine (vinca alkaloids), doxorubicin (antranilinovy antibiotic), etoposide (topoizokerazy inhibitor), Actinomycin-D, carboplatin (platinum drug).
Surgical treatment of rhabdomyosarcoma should be carried out, applying the general principles of operative radicalism.To start with the inoperable tumors primarily conduct research on biopsy material.This helps to further prevent regrowth of disease macroscopic or microscopic residual nature, which is important when rhabdomyosarcoma locations in the head and neck region, especially in the soft tissues of the orbit.In some cases, even after the effective treatment of chemotherapeutic agents, a tumor may remain inoperable.For example, in its localization in the nasopharynx or parameningealno.In that case, surgery is replaced by conservative treatment beam irradiation and chemotherapy.
Rhabdomyosarcoma, localized in the tissues of extremities, can affect large surface and involve the neurovascular bundle in the pathological process.In these cases, even in the absence of signs of metastasis of malignant pathology in the bones of the skeleton, the only radical solution is amputation of the limb.Transactions in which the authorities keep movement possible only by reducing tumor lesions under the influence of chemotherapy, as well as initial, where there are small formations with clear boundaries, identified on ultrasound, MRI or CT.
With rhabdomyosarcoma of the bladder with isolated his defeat, after a positive response to combination chemotherapy may appoint resection of its walls.But mostly the tumor in the bladder or totally localized vesical triangle, which indicates the need for a hysterectomy body, with its subsequent reconstruction surgically to drain urine.
optimal one-stage surgical treatment in this case is considered to be holding ureterosigmostomy or ureterokutaneostomii.For the first type of surgery with chemotherapy increases the risk of an ascending urinary tract infection, which can lead to pyelonephritis.For the second surgery increases the risk of not rooting artificial reservoir for urine, which may be due to the actions of cytotoxic drugs that reduce the regenerative capacity of tissues.Therefore, initially conducted ureterokutaneostomiyu then chemoradiotherapy, and the next stage - form the artificial urinary reservoir.
Malignancies paratestikulyarnogo character is removed, spending orhofunikulektomiyu from the groin area.With the localization of rhabdomyosarcoma in the uterus or vagina shows their extirpation.After the spread of tumor metastasis to the lungs is also assigned the surgical removal if it is operable.
radiotherapy treatment is appointed, given the criteria selected treatment program.All doses of radiation exposure will depend on the spread of rhabdomyosarcoma and its localization.
to treat the most severe stages of rhabdomyosarcoma (fourth) used high-dose chemotherapy followed by bone marrow transplantation.
rhabdomyosarcoma can be attributed to malignant pathologies having a relatively favorable prognosis.For example, at the first stage, the survival rate is nearly 80%, while the second - 65%, the third stage is characterized by only 40%, but for the fourth peculiar to a fatal prognosis.Embryonic (70%) type rhabdomyosarcoma has a positive prognosis Unlike alveolar form (50%).Also, predicting the disease depends on the size of the tumor.If it is growing rapidly, and the prognosis is unfavorable.
to favorable localizations include rhabdomyosarcoma, such as orbit paratestikulyarnaya area of the neck and head, the vulva, uterus and vagina.Adverse localization - limbs, prostate, deep parts of the neck and head, torso, bladder, breast and abdominal space.In addition, the positive outcomes observed among children under the age of seven.All local recurrences fatal for patients.