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Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature

Dermatofibrosarcoma Protuberans (DFSP) is a rare skin tumor, characterized by frequent local recurrence but is seldom metastatic. It is histologically characterized by storiform arrangement of spindle cells. Cytogenetically, most tumors are characterized by translocation 17:22 leading to overexpress...

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Autores principales: Dhir, Mashaal, Crockett, David G, Stevens, Todd M, Silberstein, Peter T, Hunter, William J, Foster, Jason M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4179866/
https://www.ncbi.nlm.nih.gov/pubmed/25276339
http://dx.doi.org/10.1186/2045-3329-4-8
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author Dhir, Mashaal
Crockett, David G
Stevens, Todd M
Silberstein, Peter T
Hunter, William J
Foster, Jason M
author_facet Dhir, Mashaal
Crockett, David G
Stevens, Todd M
Silberstein, Peter T
Hunter, William J
Foster, Jason M
author_sort Dhir, Mashaal
collection PubMed
description Dermatofibrosarcoma Protuberans (DFSP) is a rare skin tumor, characterized by frequent local recurrence but is seldom metastatic. It is histologically characterized by storiform arrangement of spindle cells. Cytogenetically, most tumors are characterized by translocation 17:22 leading to overexpression of tyrosine kinase PDGFB which can be targeted with tyrosine kinase inhibitor, Imatinib. We describe the first case of unresectable pancreatic metastases from DFSP treated with neoadjuvant Imatinib and subsequently R0 metastectomy. Additionally, a comprehensive systematic review of DFSP pancreatic metastases and the current published data on the use of Imatinib in DFSP is summarized.
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spelling pubmed-41798662014-10-01 Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature Dhir, Mashaal Crockett, David G Stevens, Todd M Silberstein, Peter T Hunter, William J Foster, Jason M Clin Sarcoma Res Case Report Dermatofibrosarcoma Protuberans (DFSP) is a rare skin tumor, characterized by frequent local recurrence but is seldom metastatic. It is histologically characterized by storiform arrangement of spindle cells. Cytogenetically, most tumors are characterized by translocation 17:22 leading to overexpression of tyrosine kinase PDGFB which can be targeted with tyrosine kinase inhibitor, Imatinib. We describe the first case of unresectable pancreatic metastases from DFSP treated with neoadjuvant Imatinib and subsequently R0 metastectomy. Additionally, a comprehensive systematic review of DFSP pancreatic metastases and the current published data on the use of Imatinib in DFSP is summarized. BioMed Central 2014-08-06 /pmc/articles/PMC4179866/ /pubmed/25276339 http://dx.doi.org/10.1186/2045-3329-4-8 Text en Copyright © 2014 Dhir et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/4.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Dhir, Mashaal
Crockett, David G
Stevens, Todd M
Silberstein, Peter T
Hunter, William J
Foster, Jason M
Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature
title Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature
title_full Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature
title_fullStr Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature
title_full_unstemmed Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature
title_short Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature
title_sort neoadjuvant treatment of dermatofibrosarcoma protuberans of pancreas with imatinib: case report and systematic review of literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4179866/
https://www.ncbi.nlm.nih.gov/pubmed/25276339
http://dx.doi.org/10.1186/2045-3329-4-8
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