Parotid Ewing's sarcoma: Extra-skeletal uncommon condition.
Ontology highlight
ABSTRACT: Introduction
The parotid gland is a very rare location of Ewing's sarcoma. Its clinical and radiological manifestations are atypical. Its management is based on a multidisciplinary approach to improve prognosis and survival.Discussion
The diagnosis of parotid Ewing's sarcoma is determined by a combination of genetic and histological criteria. Its local aggressiveness and high metastatic potential require prompt and appropriate management. Many prognostic features affect its management, the most important is the TNM staging.Conclusion
Limited cases of parotid Ewing's sarcoma have been published in the literature. This small number of reported cases is the only way to study its characteristics.
SUBMITTER: Sabr A
PROVIDER: S-EPMC8080453 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Refractory and/or recurrent Ewing's sarcoma (EWS) remains a clinical challenge because the disease's resistance to therapy makes it difficult to achieve durable results with standard treatments that include chemotherapy, radiation, and surgery. Recently, insulin-like-growth-factor-1-receptor (IGF1R) antibodies have been shown to have a modest single-agent activity in EWS. Patient selection using biomarkers and understanding response and resistance mechanisms in relation to IGF1R and mammalian target of rapamycin pathways are areas of active research. Since EWS has a unique tumor-specific EWS-FLI1 t(11;22) translocation and oncogenic fusion protein, inhibition of EWS-FLI1 transcription, translation, and/or protein function may be key to eradicating EWS at the stem-cell level. Recently, a small molecule that blocks the protein-protein interaction of EWS-FLI1 with RNA helicase A has been shown in preclinical models to inhibit EWS growth. The successful application of this first-in-class protein-protein inhibitor in the clinic could become a model system for translocation-associated cancers such as EWS.
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Project description:Affymetrx U95Av2 GeneChip hybridizations, RNA extracted from primary human pediatric alveolar (aRMS) and embryonal (eRMS) rhabdomyosarcoma, plus primary pediatric Ewing's sarcoma (EWS) Keywords = pediatric sarcoma Keywords = Ewing's sarcoma Keywords = rhabdomyosarcoma Keywords: other
2004-03-13 | GSE967 | GEO
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