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The challenge of extraabdominal desmoid tumour management in patients with Gardner’s syndrome: radiofrequency ablation, a promising option

Desmoid tumours are benign, myofibroblastic stromal neoplasms common in Gardner’s syndrome, which is a subtype of familial adenomatous polyposis characterized by colonic polyps, osteomas, thyroid cancer, epidermoid cysts, fibromas and sebaceous cysts. The primary treatment is surgery, followed by ad...

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Autores principales: Cobianchi, Lorenzo, Ravetta, Valentina, Viera, Francesca Torello, Filisetti, Claudia, Siri, Barbara, Segalini, Edoardo, Maestri, Marcello, Dominioni, Tommaso, Alessiani, Mario, Rossi, Sandro, Dionigi, Paolo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4258061/
https://www.ncbi.nlm.nih.gov/pubmed/25429890
http://dx.doi.org/10.1186/1477-7819-12-361
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author Cobianchi, Lorenzo
Ravetta, Valentina
Viera, Francesca Torello
Filisetti, Claudia
Siri, Barbara
Segalini, Edoardo
Maestri, Marcello
Dominioni, Tommaso
Alessiani, Mario
Rossi, Sandro
Dionigi, Paolo
author_facet Cobianchi, Lorenzo
Ravetta, Valentina
Viera, Francesca Torello
Filisetti, Claudia
Siri, Barbara
Segalini, Edoardo
Maestri, Marcello
Dominioni, Tommaso
Alessiani, Mario
Rossi, Sandro
Dionigi, Paolo
author_sort Cobianchi, Lorenzo
collection PubMed
description Desmoid tumours are benign, myofibroblastic stromal neoplasms common in Gardner’s syndrome, which is a subtype of familial adenomatous polyposis characterized by colonic polyps, osteomas, thyroid cancer, epidermoid cysts, fibromas and sebaceous cysts. The primary treatment is surgery, followed by adjuvant radiotherapy, but the local recurrence rate is high, and wide resection can result in debilitating loss of function. We report the case of a 39-year-old man with Gardner’s syndrome who had already undergone a total prophylactic colectomy. He developed desmoid tumours localized in the mesenteric root, abdominal wall and dorsal region, which were treated from 2003 through 2013 with several surgical procedures and percutaneous radiofrequency ablation. In 2008 and 2013, RFA was applied under ultrasonographic guidance to two desmoid tumours localized in the dorsal thoracic wall. The outcomes were low-grade pain and one case of superficial skin necrosis, but so far there has been no recurrence of desmoid tumours in these locations. Surgical resection remains the first-line therapy for patients with desmoid tumours, but wide resection may lead to a poor quality of life. Radiofrequency ablation is less invasive and expensive and is a possible therapeutic option for desmoid tumours in patients with Gardner’s syndrome.
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spelling pubmed-42580612014-12-07 The challenge of extraabdominal desmoid tumour management in patients with Gardner’s syndrome: radiofrequency ablation, a promising option Cobianchi, Lorenzo Ravetta, Valentina Viera, Francesca Torello Filisetti, Claudia Siri, Barbara Segalini, Edoardo Maestri, Marcello Dominioni, Tommaso Alessiani, Mario Rossi, Sandro Dionigi, Paolo World J Surg Oncol Case Report Desmoid tumours are benign, myofibroblastic stromal neoplasms common in Gardner’s syndrome, which is a subtype of familial adenomatous polyposis characterized by colonic polyps, osteomas, thyroid cancer, epidermoid cysts, fibromas and sebaceous cysts. The primary treatment is surgery, followed by adjuvant radiotherapy, but the local recurrence rate is high, and wide resection can result in debilitating loss of function. We report the case of a 39-year-old man with Gardner’s syndrome who had already undergone a total prophylactic colectomy. He developed desmoid tumours localized in the mesenteric root, abdominal wall and dorsal region, which were treated from 2003 through 2013 with several surgical procedures and percutaneous radiofrequency ablation. In 2008 and 2013, RFA was applied under ultrasonographic guidance to two desmoid tumours localized in the dorsal thoracic wall. The outcomes were low-grade pain and one case of superficial skin necrosis, but so far there has been no recurrence of desmoid tumours in these locations. Surgical resection remains the first-line therapy for patients with desmoid tumours, but wide resection may lead to a poor quality of life. Radiofrequency ablation is less invasive and expensive and is a possible therapeutic option for desmoid tumours in patients with Gardner’s syndrome. BioMed Central 2014-11-27 /pmc/articles/PMC4258061/ /pubmed/25429890 http://dx.doi.org/10.1186/1477-7819-12-361 Text en © Cobianchi et al.; licensee BioMed Central Ltd. 2014 This article is published under license to BioMed Central Ltd. 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
Cobianchi, Lorenzo
Ravetta, Valentina
Viera, Francesca Torello
Filisetti, Claudia
Siri, Barbara
Segalini, Edoardo
Maestri, Marcello
Dominioni, Tommaso
Alessiani, Mario
Rossi, Sandro
Dionigi, Paolo
The challenge of extraabdominal desmoid tumour management in patients with Gardner’s syndrome: radiofrequency ablation, a promising option
title The challenge of extraabdominal desmoid tumour management in patients with Gardner’s syndrome: radiofrequency ablation, a promising option
title_full The challenge of extraabdominal desmoid tumour management in patients with Gardner’s syndrome: radiofrequency ablation, a promising option
title_fullStr The challenge of extraabdominal desmoid tumour management in patients with Gardner’s syndrome: radiofrequency ablation, a promising option
title_full_unstemmed The challenge of extraabdominal desmoid tumour management in patients with Gardner’s syndrome: radiofrequency ablation, a promising option
title_short The challenge of extraabdominal desmoid tumour management in patients with Gardner’s syndrome: radiofrequency ablation, a promising option
title_sort challenge of extraabdominal desmoid tumour management in patients with gardner’s syndrome: radiofrequency ablation, a promising option
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4258061/
https://www.ncbi.nlm.nih.gov/pubmed/25429890
http://dx.doi.org/10.1186/1477-7819-12-361
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