Pathology of Alveolar Soft Part Sarcoma
Alveolar soft part sarcoma (ASPS) is a rare orphan sarcoma of uncertain differentiation according to the latest WHO classifcation of soft tissue tumors with a somewhat indolent clinical course. The common histomorphological alveolar-type presentation is unique. It is usually not graded according to...
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| Formato: | Capítulo de libro |
| Lenguaje: | inglés |
| Publicado: |
IntechOpen
2022
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| Materias: | |
| Acceso en línea: | https://www.intechopen.com/chapters/80976 |
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| Sumario: | Alveolar soft part sarcoma (ASPS) is a rare orphan sarcoma of uncertain differentiation according to the latest WHO classifcation of soft tissue tumors with a somewhat indolent clinical course. The common histomorphological alveolar-type presentation is unique. It is usually not graded according to the French Federation of Cancer Centers grading system, but nonetheless defined as a high-grade sarcoma. The tumor adopts a clinical pattern with a distinctive natural history marked by local recurrences up to 50% of cases and a high prevalence of metastases in such diverse sites such as the lung, liver, brain, bone that can occur more than 10 years after the primary event. ASPS is driven by a specific recurrent nonreciprocal translocation der(17)t(X;17)(p11;q25). This chimeric gene fusion is also found (albeit in the balanced mode) in a subset of renal cell carcinomas in the young. Nevertheless, its high specificity and sensitivity in ASPS is a recognized feature and accurate diagnosis requires trained pathologists and molecular testing. Prognostication is based on age, size of tumor, primary site, and the presence or not of metastasis. |
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| ISBN: | 978-1-80355-723-6 |
| DOI: | 10.5772/intechopen.102954 |