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Multicenter Study
. 2021 Apr 20;21(1):437.
doi: 10.1186/s12885-021-08189-6.

Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study

Pierluigi Cuomo  1   2 , Guido Scoccianti  3 , Alberto Schiavo  3 , Valentina Tortolini  4 , Catrin Wigley  5 , Francesco Muratori  3 , Davide Matera  3 , Mariia Kukushkina  6 , Philipp Theodor Funovics  7 , Marie-Theres Lingitz  7 , Reinhard Windhager  7 , Sander Dijkstra  8 , Jorrit Jasper  8 , Daniel A Müller  9 , Dominik Kaiser  9 , Tamás Perlaky  10 , Andreas Leithner  11 , Maria Anna Smolle  11 , Domenico Andrea Campanacci  3
Affiliations
Multicenter Study

Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study

Pierluigi Cuomo et al. BMC Cancer. .

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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Conflict of interest statement

The Authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
remission likelihood after DTF surgical excision is significantly higher with increasing age (p = 0.02). Red line: remission chance. Shaded area: 95% confidence interval
See this image and copyright information in PMC

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