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Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study

BACKGROUND: Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate. QUESTIONS/PURPOSE: This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities...

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Autores principales: Cuomo, Pierluigi, Scoccianti, Guido, Schiavo, Alberto, Tortolini, Valentina, Wigley, Catrin, Muratori, Francesco, Matera, Davide, Kukushkina, Mariia, Funovics, Philipp Theodor, Lingitz, Marie-Theres, Windhager, Reinhard, Dijkstra, Sander, Jasper, Jorrit, Müller, Daniel A., Kaiser, Dominik, Perlaky, Tamás, Leithner, Andreas, Smolle, Maria Anna, Campanacci, Domenico Andrea
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8059004/
https://www.ncbi.nlm.nih.gov/pubmed/33879110
http://dx.doi.org/10.1186/s12885-021-08189-6
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author Cuomo, Pierluigi
Scoccianti, Guido
Schiavo, Alberto
Tortolini, Valentina
Wigley, Catrin
Muratori, Francesco
Matera, Davide
Kukushkina, Mariia
Funovics, Philipp Theodor
Lingitz, Marie-Theres
Windhager, Reinhard
Dijkstra, Sander
Jasper, Jorrit
Müller, Daniel A.
Kaiser, Dominik
Perlaky, Tamás
Leithner, Andreas
Smolle, Maria Anna
Campanacci, Domenico Andrea
author_facet Cuomo, Pierluigi
Scoccianti, Guido
Schiavo, Alberto
Tortolini, Valentina
Wigley, Catrin
Muratori, Francesco
Matera, Davide
Kukushkina, Mariia
Funovics, Philipp Theodor
Lingitz, Marie-Theres
Windhager, Reinhard
Dijkstra, Sander
Jasper, Jorrit
Müller, Daniel A.
Kaiser, Dominik
Perlaky, Tamás
Leithner, Andreas
Smolle, Maria Anna
Campanacci, Domenico Andrea
author_sort Cuomo, Pierluigi
collection PubMed
description BACKGROUND: Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate. QUESTIONS/PURPOSE: This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation. METHODS: This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF. RESULTS: Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3–85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group. CONCLUSION: Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.
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spelling pubmed-80590042021-04-21 Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study Cuomo, Pierluigi Scoccianti, Guido Schiavo, Alberto Tortolini, Valentina Wigley, Catrin Muratori, Francesco Matera, Davide Kukushkina, Mariia Funovics, Philipp Theodor Lingitz, Marie-Theres Windhager, Reinhard Dijkstra, Sander Jasper, Jorrit Müller, Daniel A. Kaiser, Dominik Perlaky, Tamás Leithner, Andreas Smolle, Maria Anna Campanacci, Domenico Andrea BMC Cancer Research BACKGROUND: Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate. QUESTIONS/PURPOSE: This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation. METHODS: This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF. RESULTS: Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3–85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group. CONCLUSION: Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle. BioMed Central 2021-04-20 /pmc/articles/PMC8059004/ /pubmed/33879110 http://dx.doi.org/10.1186/s12885-021-08189-6 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research
Cuomo, Pierluigi
Scoccianti, Guido
Schiavo, Alberto
Tortolini, Valentina
Wigley, Catrin
Muratori, Francesco
Matera, Davide
Kukushkina, Mariia
Funovics, Philipp Theodor
Lingitz, Marie-Theres
Windhager, Reinhard
Dijkstra, Sander
Jasper, Jorrit
Müller, Daniel A.
Kaiser, Dominik
Perlaky, Tamás
Leithner, Andreas
Smolle, Maria Anna
Campanacci, Domenico Andrea
Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study
title Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study
title_full Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study
title_fullStr Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study
title_full_unstemmed Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study
title_short Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study
title_sort extra-abdominal desmoid tumor fibromatosis: a multicenter emsos study
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8059004/
https://www.ncbi.nlm.nih.gov/pubmed/33879110
http://dx.doi.org/10.1186/s12885-021-08189-6
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