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Marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: A case report of an ETV6-NTRK3 positive infantile fibrosacroma of the distal tibia

The infantile fibrosarcoma (IFS) is a non-rhabdomyosarcoma soft tissue sarcoma with locally aggressive properties. State of the art therapy consists of neoadjuvant chemotherapy followed by wide resection according to the criteria of the musculoskeletal tumor society. DIAGNOSES: An ETV6-NTRK3 positiv...

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Detalles Bibliográficos
Autores principales: Weber, Markus, von Kunow, Frederik, Hillmann, Axel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10328687/
https://www.ncbi.nlm.nih.gov/pubmed/37417635
http://dx.doi.org/10.1097/MD.0000000000034194
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author Weber, Markus
von Kunow, Frederik
Hillmann, Axel
author_facet Weber, Markus
von Kunow, Frederik
Hillmann, Axel
author_sort Weber, Markus
collection PubMed
description The infantile fibrosarcoma (IFS) is a non-rhabdomyosarcoma soft tissue sarcoma with locally aggressive properties. State of the art therapy consists of neoadjuvant chemotherapy followed by wide resection according to the criteria of the musculoskeletal tumor society. DIAGNOSES: An ETV6-NTRK3 positive IFS of the distal tibia in a 21-months old child showed good response to chemotherapy. INTERVENTIONS: Due to refusal of amputation marginal resection completing the margins with a high speed drill and filling the space with bone cement was performed. OUTCOMES: At latest follow-up 10 years after surgery, no recurrence was observed. LESSONS: An individual therapy for surgical treatment of IIFS is recommended. This comprises marginal resection in instead of the golden standard “wide resection” in selected cases.
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spelling pubmed-103286872023-07-08 Marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: A case report of an ETV6-NTRK3 positive infantile fibrosacroma of the distal tibia Weber, Markus von Kunow, Frederik Hillmann, Axel Medicine (Baltimore) 7100 The infantile fibrosarcoma (IFS) is a non-rhabdomyosarcoma soft tissue sarcoma with locally aggressive properties. State of the art therapy consists of neoadjuvant chemotherapy followed by wide resection according to the criteria of the musculoskeletal tumor society. DIAGNOSES: An ETV6-NTRK3 positive IFS of the distal tibia in a 21-months old child showed good response to chemotherapy. INTERVENTIONS: Due to refusal of amputation marginal resection completing the margins with a high speed drill and filling the space with bone cement was performed. OUTCOMES: At latest follow-up 10 years after surgery, no recurrence was observed. LESSONS: An individual therapy for surgical treatment of IIFS is recommended. This comprises marginal resection in instead of the golden standard “wide resection” in selected cases. Lippincott Williams & Wilkins 2023-07-07 /pmc/articles/PMC10328687/ /pubmed/37417635 http://dx.doi.org/10.1097/MD.0000000000034194 Text en Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle 7100
Weber, Markus
von Kunow, Frederik
Hillmann, Axel
Marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: A case report of an ETV6-NTRK3 positive infantile fibrosacroma of the distal tibia
title Marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: A case report of an ETV6-NTRK3 positive infantile fibrosacroma of the distal tibia
title_full Marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: A case report of an ETV6-NTRK3 positive infantile fibrosacroma of the distal tibia
title_fullStr Marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: A case report of an ETV6-NTRK3 positive infantile fibrosacroma of the distal tibia
title_full_unstemmed Marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: A case report of an ETV6-NTRK3 positive infantile fibrosacroma of the distal tibia
title_short Marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: A case report of an ETV6-NTRK3 positive infantile fibrosacroma of the distal tibia
title_sort marginal resection as a potential curative treatment option of infantile fibrosarcoma with good response after chemotherapy: a case report of an etv6-ntrk3 positive infantile fibrosacroma of the distal tibia
topic 7100
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10328687/
https://www.ncbi.nlm.nih.gov/pubmed/37417635
http://dx.doi.org/10.1097/MD.0000000000034194
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