Rhabdomyosarcome

Rhabdomyosarcoma (RMS) is a highly aggressive form of cancer that develops from mesenchymal cells that have failed to fully differentiate into myocytes of skeletal muscle. Cells of the tumor are identified as rhabdomyoblasts. The four subtypes are embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma, pleomorphic rhabdomyosarcoma, and spindle-cell/sclerosing rhabdomyosarcoma. Embryonal and alveolar are the main groups, and these types are the most common soft tissue sarcomas of childhood and adolescence. The pleomorphic type is usually found in adults. It is generally considered to be a disease of childhood, as the vast majority of cases occur in those below the age of 18. It is commonly described as one of the small-blue-round-cell tumors of childhood due to its appearance on an H&E stain. Despite being relatively rare, it accounts for approximately 40% of all recorded soft-tissue sarcomas. RMS can occur in any soft-tissue site in the body, but is primarily found in the head, neck, orbit, genitourinary tract, genitals, and extremities. No clear risk factors have been identified, but the disease has been associated with some congenital abnormalities. Signs and symptoms vary according to tumor site, and prognosis is closely tied to the location of the primary tumor. Common sites of metastasis include the lungs, bone marrow, and bones. There are many classification systems for RMS and a variety of defined histological types. Embryonal rhabdomyosarcoma is the most common type and comprises about 60% of cases. Outcomes vary considerably, with five-year survival rates between 35 and 95%, depending on the type of RMS involved, so clear diagnosis is critical for effective treatment and management. Accurate and quick diagnosis is often difficult due to the heterogeneity of RMS tumors and a lack of strong genetic markers of the disease, although recent research by UVA Health researchers discovered "multiple lines of evidence supporting [the gene] AVIL is powerful driver for both major types of rhabdomyosarcoma," according to researcher Hui Li of the University of Virginia School of Medicine's Department of Pathology and UVA Cancer Center.

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Concepts associés (11)

Sarcome d'Ewing

Le sarcome d'Ewing est une forme de cancer des os qui touche principalement les enfants et les jeunes adultes (la moyenne d'âge est de 13 ans). Il concerne 10 % des tumeurs osseuses primaires. C'est une tumeur rare touchant trois personnes sur un million par an en France. Les sarcomes d'Ewing extra-osseux sont très rares. Cette tumeur osseuse de l'adolescent est plus fréquente chez les garçons et beaucoup plus fréquente chez les sujets blancs que chez les noirs.

Chemotherapy regimen

A chemotherapy regimen is a regimen for chemotherapy, defining the drugs to be used, their dosage, the frequency and duration of treatments, and other considerations. In modern oncology, many regimens combine several chemotherapy drugs in combination chemotherapy. The majority of drugs used in cancer chemotherapy are cytostatic, many via cytotoxicity. A fundamental philosophy of medical oncology, including combination chemotherapy, is that different drugs work through different mechanisms, and that the results of using multiple drugs will be synergistic to some extent.

Rhabdomyosarcome

Un rhabdomyosarcome est une tumeur maligne développée à partir du tissu musculaire strié squelettique. Il s'agit d'une tumeur d'origine mésenchymateuse. Les rhabdomyosarcomes sont des tumeurs plus fréquentes chez les enfants et les adolescents. Les rhabdomyosarcomes se développent électivement au niveau des cavités céphaliques (orbite, nez, sinus, oreille) ou de la sphère urogénitale (vessie, prostate, vagin, cordon spermatique). Plus rarement, ils sont musculaires au niveau des membres.