Rhabdomyosarcoma is a cancer in childhood that arises from embryonic mesenchymal cells that still have the ability to differentiate into skeletal muscle cells. It can arise from almost any kind of muscle tissue on each part of the body and leads to very diverse clinical manifestations. The carcinomas are usually detected by means of computed tomography or magnetic resonance imaging and the diagnosis is confirmed by biopsy. Treatment includes surgical procedures, radiotherapy and chemotherapy.

Rhabdomyosarcoma is (after the neuroblastoma and Wilms tumor) is the third most common extracranial cancer in children. However, it accounts for only 3-4% of all children carcinomas. The rhabdomyosarcoma belongs to a group of soft tissue sarcoma known as tumors and is the most common cancer in this group.

Rhabdomyosarcoma is a cancer in childhood that arises from embryonic mesenchymal cells that still have the ability to differentiate into skeletal muscle cells. It can arise from almost any kind of muscle tissue on each part of the body and leads to very diverse clinical manifestations. The carcinomas are usually detected by means of computed tomography or magnetic resonance imaging and the diagnosis is confirmed by biopsy. Treatment includes surgical procedures, radiotherapy and chemotherapy. Rhabdomyosarcoma is (after the neuroblastoma and Wilms tumor) is the third most common extracranial cancer in children. However, it accounts for only 3-4% of all children carcinomas. The rhabdomyosarcoma belongs to a group of soft tissue sarcoma known as tumors and is the most common cancer in this group. The incidence of rhabdomyosarcoma in children is 4.3 / 1 million a year. Two-thirds of cancers are diagnosed in children under 7 years. The disease is more common than among black (mainly because of the lower incidence among black girls) in fair-skinned people and occurs slightly more often in boys than in girls. Histology There are 2 major histological subtypes: Embryonal: Characterized by loss of heterozygosity on chromosome 11p15.5 Alveolar: In connection with the translocation t (2; 13), which merges the PAX3 gene with the FOXO1 (FKHR) gene, and t (1; 13), which merges the PAX7 gene with the FOXO1 (FKHR) gene location Although rhabdomyosarcoma may occur anywhere in the body, the disease has a Prädilektionsorte. Head and neck area (about 35%), usually near or in the nasopharyngeal areas: Most common in school-age children urogenital tract (about 25%), usually in the bladder, prostate or vagina: usually in infants and young children extremities (20%): Most common in young people hull / other sites (20%) ca. 15 to 25% of children are metastasized at diagnosis. The lung is the most common site of metastasis; Bone, bone marrow and lymph nodes are other possible areas. Symptoms and discomfort children do not have typical systemic symptoms such as fever, night sweats or weight loss. In general, the children stand with a solid palpable tumor or organ disorders because of interference from the cancer before. The orbital and nasopharyngeal carcinoma cause tears, eye pain or proptosis. The nasopharyngeal carcinoma can cause a stuffy nose, voice changes or slimy purulent discharge. Urogenitalkarzinome are usually present in infants and young children. They cause abdominal pain, a palpable abdominal tumor, difficulty in urination and hematuria. The extremities carcinomas appear as solid tumor striking somewhere on the arms or legs. A regional distribution of lymph nodes occurs frequently and metastases in the lungs, bone marrow and lymph nodes may occur and usually cause no symptoms. Diagnostic computer tomography or biopsy or excision Magnetresonaztomographie The tumor masses are examined by computed, although, in head and neck tumors, the magnetic resonance imaging is more appropriate. The diagnosis of rhabdomyosarcoma is confirmed by biopsy or by removal of the tumors. The standard examination for metastasis includes a thoracic computed tomography, a bone scan and a bone marrow aspiration bilateral marrow aspiration and biopsy one. Forecast The forecast is based on the location of the cancer (eg. As the prognosis is better when not parameningealen head / neck and genitourinary tract carcinomas, not related to the bladder or prostate) completeness of resection presence of metastases age (prognosis is worse for children < 1 year old or> 10 years histology (embryonal histology is associated with a better result than the alveolar histology) a combination of all these prognostic factors, the children divided into one, low, medium and high risk category. a complex system of risk assessment exists based two staging systems (see the National Cancer Institute stage information for rhabdomyosarcoma). the treatment is intensified with each category, the overall survival rate in children in low risk Kate. gory at> 90%, and among children in the high risk category <50%. Treatment Surgery and chemotherapy radiotherapy for residual mass or microscopic disease. The treatment of rhabdomyosarcoma is to surgery, chemotherapy and possibly radiation. The complete excision of the primary cancer is recommended if it is risk-free. Since the Karzinomauf reacts radiation and chemotherapy, an aggressive resection is recommended, even if it leads to organ injury or organic disorder. The children are treated in all categories with chemotherapy. The most common drugs are vincristine, actinomycin D, cyclophosphamide, doxorubicin, ifosfamide, and etoposide. Topotecan and irinotecan are newer drugs that show activity against this cancer. The irradiation is generally performed in children with residual disease or microscopic residual tumors after surgery and reserved for children with a moderate and high risk. For more information information on the degree of rhabdomyosarcoma (National Cancer Institute)

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