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Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review

Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males. Patients present with advanced disease and the overall survival is dismal. Multiple studies report relatively favorable outcomes with...

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Autores principales: Reijers, Sophie J. M., Siew, Caroline C. H., Kok, Niels F. M., Honoré, Charles, van Houdt, Winan J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10136577/
https://www.ncbi.nlm.nih.gov/pubmed/37185412
http://dx.doi.org/10.3390/curroncol30040299
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author Reijers, Sophie J. M.
Siew, Caroline C. H.
Kok, Niels F. M.
Honoré, Charles
van Houdt, Winan J.
author_facet Reijers, Sophie J. M.
Siew, Caroline C. H.
Kok, Niels F. M.
Honoré, Charles
van Houdt, Winan J.
author_sort Reijers, Sophie J. M.
collection PubMed
description Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males. Patients present with advanced disease and the overall survival is dismal. Multiple studies report relatively favorable outcomes with multimodal treatment consisting of chemotherapy, surgery and radiotherapy. If resection is feasible, complete cytoreductive surgery is the cornerstone of surgical treatment. The benefit of hyperthermic intraperitoneal chemotherapy in addition to cytoreductive surgery is unclear, and few studies have evaluated this option. We sought to identify the role of hyperthermic intraperitoneal chemotherapy in patients with intra-abdominal desmoplastic small round cell tumor. Our review of the available literature revealed no clear survival benefit in performing hyperthermic intraperitoneal chemotherapy after cytoreductive surgery.
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spelling pubmed-101365772023-04-28 Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review Reijers, Sophie J. M. Siew, Caroline C. H. Kok, Niels F. M. Honoré, Charles van Houdt, Winan J. Curr Oncol Review Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males. Patients present with advanced disease and the overall survival is dismal. Multiple studies report relatively favorable outcomes with multimodal treatment consisting of chemotherapy, surgery and radiotherapy. If resection is feasible, complete cytoreductive surgery is the cornerstone of surgical treatment. The benefit of hyperthermic intraperitoneal chemotherapy in addition to cytoreductive surgery is unclear, and few studies have evaluated this option. We sought to identify the role of hyperthermic intraperitoneal chemotherapy in patients with intra-abdominal desmoplastic small round cell tumor. Our review of the available literature revealed no clear survival benefit in performing hyperthermic intraperitoneal chemotherapy after cytoreductive surgery. MDPI 2023-03-31 /pmc/articles/PMC10136577/ /pubmed/37185412 http://dx.doi.org/10.3390/curroncol30040299 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Reijers, Sophie J. M.
Siew, Caroline C. H.
Kok, Niels F. M.
Honoré, Charles
van Houdt, Winan J.
Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
title Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
title_full Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
title_fullStr Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
title_full_unstemmed Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
title_short Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
title_sort intra-abdominal desmoplastic small round cell tumor (dsrct) and the role of hyperthermic intraperitoneal chemotherapy (hipec): a review
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10136577/
https://www.ncbi.nlm.nih.gov/pubmed/37185412
http://dx.doi.org/10.3390/curroncol30040299
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