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The Desmoid Tumor Research Foundation

April 2021

Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study [Full Article]

Author Information

Cuomo P(1,2), Scoccianti G(1), Schiavo A(3), Tortolini V(3), Wigley C(2), Muratori F(1), Matera D(1), Kukushkina M(4), Funovics PT(5), Lingitz MT(5), Windhager R(5), Dijkstra S(6), Jasper J(6), Muller DA(7), Kaiser D(7), Perlaky T(8), Leithner A(9), Smolle MA(9), Campanacci DA(1).

(1)Orthopaedic Oncology Dept, Azienda Ospedaliero Universitaria Careggi, Florence, Italy. (2)Sarcoma Unit, Royal National Orthopaedic Hospital, Stanmore, UK. (3)CERM Foundation, University of Florence, Florence, Italy. (4)Dept of Skin and Soft Tissue Tumors, National Cancer Institute, Kiev, Ukraine. (5)Dept of Orthopaedics and Trauma Surgery, Medical University of Vienna, Vienna, Austria. (6)Dept of Orthopaedic Surgery, University of Leiden, Leiden, The Netherlands. (7)Balgrist University Hospital, Zurich, Switzerland. (8)Dept of Orthopaedics, Semmelweis University, Budapest, Hungary. (9)Dept of Orthopaedics and Trauma, Medical University of Graz, Graz, Austria.

Publication

BMC Cancer

Summary

This paper aimed to (1) to compare the outcomes of extra-abdominal desmoid tumor fibromatosis (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. This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). 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. 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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