RABDOMIOSARCOMA PDF

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. A malignant soft tissue tumor which develops from cells of striated muscle.

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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

A malignant soft tissue tumor which develops from cells of striated muscle. It is the most common form of tumor found in children and adolescents. The median age of diagnosis is 5 years. Rhabdomyosarcoma can develop anywhere in the body, including in sites where striated muscle does not normally occur. Rhabdomyosarcoma has a high risk of local relapse, a high risk of local extension to lymph nodes and a lower risk of metastases.

Clinically there is little difference between the two types. The alveolar form occurs more readily in the limbs, nearly never in the orbits and more frequently affects the lymph nodes. The cause of rhabdomyosarcoma is unknown, but a translocation t 2;13 is very often associated with the alveolar type. Diagnosis is based on positive muscular markers and immunohistochemistry.

The evidence of a translocation t 2;13 leads to diagnosis of alveolar forms. Differential diagnoses include small round cell tumors lymphoma, neuroblastoma, Ewing sarcoma; see these terms.

It is possible to distinguish between tumors using immunohistochemistry. Some rhabdomyosarcomas are associated with a genetic predisposition to cancer as part of Li-Fraumeni syndrome or neurofibromatosis type 1 see these terms. Treatment should be managed by a multidisciplinary team, expert in the treatment of childhood cancers.

Initial surgery should only be considered if it can be completed, and completed without significant consequences. Chemotherapy is indicated in all other cases from diagnosis. Local treatment is carried out by secondary surgery, often complemented by radiotherapy.

Alveolar forms require more intensive treatment than embryonic forms. The objective of current protocols is to avoid radiotherapy, if possible, in order to reduce long term sequelae. Survival depends on the location of the tumor. Other search option s Alphabetical list. Suggest an update. Summary and related texts. Related genes. Clinical signs. Check this box if you wish to receive a copy of your message. Disease definition A malignant soft tissue tumor which develops from cells of striated muscle.

Clinical description The median age of diagnosis is 5 years. Etiology The cause of rhabdomyosarcoma is unknown, but a translocation t 2;13 is very often associated with the alveolar type. Diagnostic methods Diagnosis is based on positive muscular markers and immunohistochemistry.

Differential diagnosis Differential diagnoses include small round cell tumors lymphoma, neuroblastoma, Ewing sarcoma; see these terms. Management and treatment Treatment should be managed by a multidisciplinary team, expert in the treatment of childhood cancers.

Prognosis Survival depends on the location of the tumor. Detailed information Article for general public English Professionals Review article English Clinical practice guidelines English Health care resources for this disease Expert centres Diagnostic tests 26 Patient organisations 69 Orphan designation s and orphan drug s Specialised Social Services Eurordis directory.

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

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Cancer starts when cells in the body begin to grow out of control. Cells in nearly any part of the body can become cancer, and can spread to other areas of the body. To learn more about how cancers start and spread, see What Is Cancer? For information about the differences between childhood cancers and adult cancers, see Cancer in Children. Sarcomas are cancers that develop from connective tissues in the body, such as muscles, fat, bones, the linings of joints, or blood vessels. There are many types of sarcomas.

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Continual improvements in survival have been achieved for children and adolescents with cancer. Childhood and adolescent cancer survivors require close monitoring because side effects of cancer and its therapy may persist or develop months or years after treatment. Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors. Childhood rhabdomyosarcoma is a soft tissue malignant tumor of mesenchymal origin. It accounts for approximately 3. Fifty percent of these cases are seen in the first decade of life. Incidence may depend on the histologic subtype of rhabdomyosarcoma, as follows:.

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