Ewing's sarcoma - ranks second after osteogenic sarcoma malignant tumor of the bone skeleton, usually develops in childhood or adolescence and affects clavicle, spine, scapula, pelvis, ribs, lower divisionslong bones.Most often this tumor develops in the age range from ten to sixteen years.Very rarely there may be vnekostnaya Ewing's sarcoma, for which characterized by soft tissue.The most common sarcoma develops in the long bones (humerus, fibula, tibia, femur), then quite rapidly spreading to the surrounding soft tissue, including tendons and muscles.
been classified an entire family of aggressive tumors, similar to Ewing's sarcoma.In addition nekostnoy and bone Ewing's sarcoma, belong to the family of PNET (primitive neuroectodermal tumors of the peripheral), among which Askin tumor.Cells PNET and Ewing's sarcoma are not completely identical to each other, but have similar cytogenetic and molecular biological characteristics.Treatment options for these cancers are also simil
Stages of Ewing's sarcoma - localized and metastatic.When the localized stage of the sarcoma primary tumor can spread to nearby soft tissue, but distant metastases are not detected.When metastatic tumor spreads to other parts of the body - the lungs, bones, bone marrow, lymph system, liver, central nervous system, and so forth.
Ewing's sarcoma is considered one of the most aggressive cancers.Prior to the need systemic therapy in about 90% of the patients have already developed metastases.At the time of primary diagnosis of primary localization of metastases were usually bones, bone marrow and lungs.Lymphatic spread of metastases is very rare, but always associated with a very poor prognosis.Mediastinal and retroperitoneal spread of metastases is also quite rare
Ewing's sarcoma - Causes
risk of developing Ewing's sarcoma is slightly increased in the event of a patient specific abnormalities of the genitourinary system, and some benign bone tumors (enhodroma etc.).Ewing's sarcoma is not considered as genetically caused disease, but sometimes certain factors suggest a genetic predisposition - there are cases where PNET occurs in children of the same family.
There is a widespread belief that Ewing's sarcoma develops as a result of trauma, and the period between the moment of trauma, and tumor development can be so great that does not definitively prove this theory.It is possible, and that the trauma merely draws attention to a certain area of the body where the tumor is found as a result that existed there before.
Another theory is that the cause of Ewing sarcoma chromosomal changes are manifested as the translocation between chromosome 11 and the regions of chromosome 22 that leads to impaired function and location of specific genes.More than 90% of the cells of the sarcoma contain a specific protein, the diagnostic detection which helps determine Ewing
Ewing's sarcoma - symptoms
characteristic symptom of bone form of Ewing's sarcoma is a pain at the site of bone tumors, which in contrast to the pain associated with trauma, is notsubsides after fixing limb, and vice versa, eventually amplified.Often such pain mistaken for a symptom of the onset of inflammation, but even after taking antibiotics, they do not pass.
Most patients with a form of bone Ewing's sarcoma, and in all patients with vnekostnoy, the site of the tumor gradually developed swelling, which then usually spreads to the nearby joint, disrupting its normal functioning.Zone swelling with time rapidly increases in size, its local temperature increases (30% of cases), palpation it is usually painful.Skin lesion on a slightly reddish color, it show through the veins.
long bone tumors may result in pathological bone fracture.In the case of lesions of the spine and its surrounding tissues, various violations of the proper functioning of the pelvic organs and / or movement disorders.
As further spread of tumor in the body there is a general symptoms: fever, weight loss, fatigue, and weakness.In 50% of patients with symptoms of Kaposi celebrated for more than three months before the correct diagnosis.
Ewing's sarcoma most commonly diagnosed a few months after the first symptoms of the disease.This happens because the original swelling and pain is considered a sign of inflammation or injury.That is why in the case of long-lived bone pain should consult oncologist
diagnosis of Ewing's sarcoma
Perhaps the first studies to identify Ewing's sarcoma is a radiographic study.This tumor on radiographs has enough distinctive look that makes it easier initial diagnosis.To determine the nature of the soft-tissue component and estimate the size of tumors ultrasound performed the same area.
To clarify the diagnosis and to obtain more information on neoplastic held computer and magnetic resonance tomography.These methods can detect metastases to assess the state of the medullary canal, around the affected bone to reveal the degree of soft tissue, to clarify the size and location of sarcoma.
In addition, to clarify the nature of the observed formation of a biopsy of the affected area of bone or soft-tissue component.Displaying immunohistochemical and histological analysis aimed at identifying translocations and other chromosome abnormalities.
Because Ewing's sarcoma usually metastasizes to the lungs, to confirm their presence or absence in the body is carried out CT scans and chest X-ray.For the detection of bone metastases bone scan performed.In order to identify lesions of bone marrow or bone marrow biopsy performed puncture
Ewing's sarcoma - treated
Ewing's sarcoma is a very aggressive tumor, quickly giving micrometastases.That is why when it must be treated systemic effects of chemotherapy on the whole body, otherwise inevitable recurrence.Additionally it is shown radiotherapy and / or surgical treatment.Most often, Ewing's sarcoma, surgical removal of the tumor.This occurs in lesions of the upper / lower limbs, shoulder blades, ribs and so on. It can be removed as the soft tissue component of the tumor and the affected area of the bone, and the bone fragment removed replace the endoprosthesis.In some cases, it is imperative amputation of the affected limb, but modern treatments often allow to avoid it.After complete resection of the tumor to destroy the remaining cancer cells in the area where it is located, it is shown radiotherapy.
If for any reason it is not possible to conduct a full surgical removal of tumors, to control it is used radiotherapy (Ewing's sarcoma cells are very sensitive to radiation).However, in such cases, the risk of relapse is significantly increased.
chemotherapy in the treatment of Ewing's sarcoma is applied as after and before the operation.Removal of tumors examined for policy making subsequent chemotherapy.The most common treatment used during the following drugs: etoposide, ifosfamide, dactinomycin, cyclophosphamide, doxorubicin, vincristine.Typically, a course of chemotherapy for Ewing's sarcoma is from seven to ten months.When
which developed metastatic patients recommend the passage of high-dose chemotherapy with autologous stem cell or bone marrow.
Five-year survival for patients with localized Ewing's sarcoma form provided timely adequate combination treatment is about 70%.When metastatic disease, particularly in cases of bone and bone marrow, even the use of high-dose chemotherapy combined with bone marrow transplantation provides not more 25% five-year survival rate.In addition, a further prognosis depends on the sensitivity of tumors to the used of drugs and the place of its location.
for early detection of recurrence and monitor side effects after the treatment shows the passage of regular inspections.After a hip replacement to restore normal motor volume usually requires long-term rehabilitation.In the case of amputation prosthesis shown holding followed by a fairly lengthy period of rehabilitation.
after chemotherapy and radiation therapy, there may be long-term side effects such as: a variety of bone growth disorders, infertility, cardiomyopathy, and so on. In addition, a significantly increased risk of developing secondary cancers.However, despite all this, a lot of patients after treatment lead a full life.