Pierluigi Cuomo1,2, Guido Scoccianti3, Alberto Schiavo3, Valentina Tortolini4, Catrin Wigley5, Francesco Muratori3, Davide Matera3, Mariia Kukushkina6, Philipp Theodor Funovics7, Marie-Theres Lingitz7, Reinhard Windhager7, Sander Dijkstra8, Jorrit Jasper8, Daniel A Müller9, Dominik Kaiser9, Tamás Perlaky10, Andreas Leithner11, Maria Anna Smolle11, Domenico Andrea Campanacci3. 1. Orthopaedic Oncology Department, Azienda Ospedaliero Universitaria Careggi, Florence, Italy. pierluigicuomo@gmail.com. 2. Sarcoma Unit, Royal National Orthopaedic Hospital, Stanmore, UK. pierluigicuomo@gmail.com. 3. Orthopaedic Oncology Department, Azienda Ospedaliero Universitaria Careggi, Florence, Italy. 4. CERM Foundation, University of Florence, Florence, Italy. 5. Sarcoma Unit, Royal National Orthopaedic Hospital, Stanmore, UK. 6. Department of Skin and Soft Tissue Tumors, National Cancer Institute, Kiev, Ukraine. 7. Department of Orthopaedics and Trauma Surgery, Medical University of Vienna, Vienna, Austria. 8. Department of Orthopaedic Surgery, University of Leiden, Leiden, The Netherlands. 9. Balgrist University Hospital, Zurich, Switzerland. 10. Department of Orthopaedics, Semmelweis University, Budapest, Hungary. 11. Department of Orthopaedics and Trauma, Medical University of Graz, Graz, Austria.
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.
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.
Authors: A Gronchi; P G Casali; L Mariani; S Lo Vullo; M Colecchia; L Lozza; R Bertulli; M Fiore; P Olmi; M Santinami; J Rosai Journal: J Clin Oncol Date: 2003-04-01 Impact factor: 44.544
Authors: A Gronchi; C Colombo; C Le Péchoux; A P Dei Tos; A Le Cesne; A Marrari; N Penel; G Grignani; J Y Blay; P G Casali; E Stoeckle; F Gherlinzoni; P Meeus; C Mussi; F Gouin; F Duffaud; M Fiore; S Bonvalot Journal: Ann Oncol Date: 2013-12-09 Impact factor: 32.976
Authors: Andreas Leithner; Markus Gapp; Katharina Leithner; Roman Radl; Peter Krippl; Alfred Beham; Reinhard Windhager Journal: J Surg Oncol Date: 2004-06-01 Impact factor: 3.454
Authors: Bimal Mayur Kumar Vora; Peter L Munk; Nagavalli Somasundaram; Hugue A Ouellette; Paul I Mallinson; Adnan Sheikh; Hanis Abdul Kadir; Tien Jin Tan; Yet Yen Yan Journal: PLoS One Date: 2021-12-23 Impact factor: 3.240