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Management of serious complications in intra‐abdominal desmoid‐type fibromatosis

BACKGROUND: Desmoid fibromatosis (DF) is a rare and locally infiltrative monoclonal fibroblastic proliferation arising from connective tissues, with lack of metastatic potential. About 10% of all DF cases are intra‐abdominally sited. Complications in this site, due to the locally infiltrative nature...

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Autores principales: Bini, Federica, Fiore, Marco, Provenzano, Salvatore, Bertulli, Rossella, Ottini, Arianna, Colombo, Chiara, Vitellaro, Marco, Greco, Gabriella, Morosi, Carlo, Gronchi, Alessandro, Casali, Paolo Giovanni, Palassini, Elena
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8714535/
https://www.ncbi.nlm.nih.gov/pubmed/34165246
http://dx.doi.org/10.1002/cnr2.1411
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author Bini, Federica
Fiore, Marco
Provenzano, Salvatore
Bertulli, Rossella
Ottini, Arianna
Colombo, Chiara
Vitellaro, Marco
Greco, Gabriella
Morosi, Carlo
Gronchi, Alessandro
Casali, Paolo Giovanni
Palassini, Elena
author_facet Bini, Federica
Fiore, Marco
Provenzano, Salvatore
Bertulli, Rossella
Ottini, Arianna
Colombo, Chiara
Vitellaro, Marco
Greco, Gabriella
Morosi, Carlo
Gronchi, Alessandro
Casali, Paolo Giovanni
Palassini, Elena
author_sort Bini, Federica
collection PubMed
description BACKGROUND: Desmoid fibromatosis (DF) is a rare and locally infiltrative monoclonal fibroblastic proliferation arising from connective tissues, with lack of metastatic potential. About 10% of all DF cases are intra‐abdominally sited. Complications in this site, due to the locally infiltrative nature of the disease, may be severe and potentially life threatening. However, data on incidence, management, and outcome of these complications are limited. AIM: Data of patients with sporadic or FAP‐related intra‐abdominal DF treated at Istituto Nazionale dei Tumori (INT) in Milano from 2005 to 2020 who developed a serious complication during the course of their disease were retrospectively collected and analyzed with a descriptive statistics. METHODS AND RESULTS: Out of 72 intra‐abdominal DF, 8 cases were identified (M/F: 5/3, median age: 35 years, FAP‐related/sporadic: 2/6): 3 with bowel obstruction, 5 with bowel perforation. In 4 cases the serious complication was the first evidence of disease; in the other 4 cases it occurred at a time interval from diagnosis in the range of 4–44 months (during an active surveillance program in one case and during chemotherapy in the other 3 cases). A surgical treatment was feasible and successful in 5 cases. In 3 surgically unmanageable patients, all progressing and experiencing acute complications while on chemotherapy, a non‐surgical approach with intensive supportive treatment and with a prompt change of chemotherapy regimen was implemented, being successful in two, the other patient dying due to a concomitant progressive lymphoma thereafter. CONCLUSION: In this series of intra‐abdominal DF, the incidence of serious complications was 11%. Most patients were successfully treated with surgery. When surgery was deemed to be unfeasible, a conservative management with intensive supportive care and a careful choice of chemotherapy was adopted, ensuring a favorable outcome in most.
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spelling pubmed-87145352022-01-05 Management of serious complications in intra‐abdominal desmoid‐type fibromatosis Bini, Federica Fiore, Marco Provenzano, Salvatore Bertulli, Rossella Ottini, Arianna Colombo, Chiara Vitellaro, Marco Greco, Gabriella Morosi, Carlo Gronchi, Alessandro Casali, Paolo Giovanni Palassini, Elena Cancer Rep (Hoboken) Original Articles BACKGROUND: Desmoid fibromatosis (DF) is a rare and locally infiltrative monoclonal fibroblastic proliferation arising from connective tissues, with lack of metastatic potential. About 10% of all DF cases are intra‐abdominally sited. Complications in this site, due to the locally infiltrative nature of the disease, may be severe and potentially life threatening. However, data on incidence, management, and outcome of these complications are limited. AIM: Data of patients with sporadic or FAP‐related intra‐abdominal DF treated at Istituto Nazionale dei Tumori (INT) in Milano from 2005 to 2020 who developed a serious complication during the course of their disease were retrospectively collected and analyzed with a descriptive statistics. METHODS AND RESULTS: Out of 72 intra‐abdominal DF, 8 cases were identified (M/F: 5/3, median age: 35 years, FAP‐related/sporadic: 2/6): 3 with bowel obstruction, 5 with bowel perforation. In 4 cases the serious complication was the first evidence of disease; in the other 4 cases it occurred at a time interval from diagnosis in the range of 4–44 months (during an active surveillance program in one case and during chemotherapy in the other 3 cases). A surgical treatment was feasible and successful in 5 cases. In 3 surgically unmanageable patients, all progressing and experiencing acute complications while on chemotherapy, a non‐surgical approach with intensive supportive treatment and with a prompt change of chemotherapy regimen was implemented, being successful in two, the other patient dying due to a concomitant progressive lymphoma thereafter. CONCLUSION: In this series of intra‐abdominal DF, the incidence of serious complications was 11%. Most patients were successfully treated with surgery. When surgery was deemed to be unfeasible, a conservative management with intensive supportive care and a careful choice of chemotherapy was adopted, ensuring a favorable outcome in most. John Wiley and Sons Inc. 2021-06-24 /pmc/articles/PMC8714535/ /pubmed/34165246 http://dx.doi.org/10.1002/cnr2.1411 Text en © 2021 The Authors. Cancer Reports published by Wiley Periodicals LLC. https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Articles
Bini, Federica
Fiore, Marco
Provenzano, Salvatore
Bertulli, Rossella
Ottini, Arianna
Colombo, Chiara
Vitellaro, Marco
Greco, Gabriella
Morosi, Carlo
Gronchi, Alessandro
Casali, Paolo Giovanni
Palassini, Elena
Management of serious complications in intra‐abdominal desmoid‐type fibromatosis
title Management of serious complications in intra‐abdominal desmoid‐type fibromatosis
title_full Management of serious complications in intra‐abdominal desmoid‐type fibromatosis
title_fullStr Management of serious complications in intra‐abdominal desmoid‐type fibromatosis
title_full_unstemmed Management of serious complications in intra‐abdominal desmoid‐type fibromatosis
title_short Management of serious complications in intra‐abdominal desmoid‐type fibromatosis
title_sort management of serious complications in intra‐abdominal desmoid‐type fibromatosis
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8714535/
https://www.ncbi.nlm.nih.gov/pubmed/34165246
http://dx.doi.org/10.1002/cnr2.1411
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