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01-12-2021 | Fibromatosis | Research

Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study

Authors: Pierluigi Cuomo, Guido Scoccianti, Alberto Schiavo, Valentina Tortolini, Catrin Wigley, Francesco Muratori, Davide Matera, Mariia Kukushkina, Philipp Theodor Funovics, Marie-Theres Lingitz, Reinhard Windhager, Sander Dijkstra, Jorrit Jasper, Daniel A. Müller, Dominik Kaiser, Tamás Perlaky, Andreas Leithner, Maria Anna Smolle, Domenico Andrea Campanacci

Published in: BMC Cancer | Issue 1/2021

Abstract

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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Title: Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study
Authors: Pierluigi Cuomo
Guido Scoccianti
Alberto Schiavo
Valentina Tortolini
Catrin Wigley
Francesco Muratori
Davide Matera
Mariia Kukushkina
Philipp Theodor Funovics
Marie-Theres Lingitz
Reinhard Windhager
Sander Dijkstra
Jorrit Jasper
Daniel A. Müller
Dominik Kaiser
Tamás Perlaky
Andreas Leithner
Maria Anna Smolle
Domenico Andrea Campanacci
Publication date: 01-12-2021
Publisher: BioMed Central
Keyword: Fibromatosis
Published in: BMC Cancer / Issue 1/2021
Electronic ISSN: 1471-2407
DOI: https://doi.org/10.1186/s12885-021-08189-6

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