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Review
. 2021 May:68 Suppl 2:e28279.
doi: 10.1002/pbc.28279.

Non-rhabdomyosarcoma soft-tissue sarcoma

Sarah A Milgrom  1 , Lynn Million  2 , Henry Mandeville  3 , Akmal Safwat  4 , Ralph P Ermoian  5 , Stephanie Terezakis  6
Affiliations
Review

Non-rhabdomyosarcoma soft-tissue sarcoma

Sarah A Milgrom et al. Pediatr Blood Cancer. 2021 May.

Abstract

Non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS) comprise 4% of childhood cancers and consist of numerous histologic subtypes. Prognostic factors associated with poor outcome include high histologic grade, large tumor size, presence of metastases, and unresectability. Complete surgical resection is critical for the best oncologic outcomes and is prioritized in treatment algorithms. The use of radiation therapy (RT) and chemotherapy is based upon factors such as resectability, histologic grade, tumor size, and stage. North American and European trials are defining a risk-based approach to NRSTS to limit treatment-related toxicity and to maximize therapeutic efficacy. In this paper, we summarize the current roles of surgery, RT, and chemotherapy in NRSTS and describe ongoing research that is advancing the care of NRSTS patients.

Keywords: COG; NRSTS; SIOP; chemotherapy; non-rhabdomyosarcoma soft-tissue sarcoma; pazopanib; radiation therapy; sarcoma; surgery.

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References

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