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Published in: BMC Musculoskeletal Disorders 1/2021

Open Access 01-12-2021 | Fibromatosis | Research article

Upfront surgery is not advantageous compared to more conservative treatments such as observation or medical treatment for patients with desmoid tumors

Authors: Shinji Tsukamoto, Piergiuseppe Tanzi, Andreas F. Mavrogenis, Manabu Akahane, Akira Kido, Yasuhito Tanaka, Marilena Cesari, Davide Maria Donati, Alessandra Longhi, Costantino Errani

Published in: BMC Musculoskeletal Disorders | Issue 1/2021

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Abstract

Background

This study compared the clinical and functional outcomes of patients initially treated with observation or medical treatment with those of patients treated with local treatment (surgery alone or surgery with adjuvant radiotherapy) to confirm whether observation or medical treatment is an appropriate first-line management approach for patients with desmoid tumors.

Methods

We retrospectively reviewed the medical records of 99 patients with histologically confirmed primary desmoid tumors treated between 1978 and 2018. The median follow-up period was 57 months. We evaluated event-free survival, defined as the time interval from the date of initial diagnosis to the date of specific change in treatment strategy or recurrence or the last follow-up.

Results

An event (specific change in treatment strategy or recurrence) occurred in 28 patients (28.3%). No significant difference in event-free survival was found between the first-line observation/medical treatment and local treatment groups (p = 0.509). The median Musculoskeletal Tumor Society score of the patients treated with first-line local treatment was 29 (interquartile range [IQR], 23–30), whereas that of the patients managed with first-line observation or medical treatment was 21 (IQR, 19–29.5). First-line observation or medical treatment was more frequently chosen for larger tumors (p = 0.045). In the patients treated with local treatment, local recurrence was not related to the surgical margin (p = 0.976).

Conclusion

Upfront surgery is not advantageous compared to more conservative treatments such as observation or medical treatment for patients with desmoid tumors.
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Literature
1.
go back to reference Goldblum JR, Fletcher JA. Desmoid-type fibromatosis. In: Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F, editors. WHO classification of tumours of soft tissue and bone. Lyon: IARC; 2013. p. 72–3. Goldblum JR, Fletcher JA. Desmoid-type fibromatosis. In: Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F, editors. WHO classification of tumours of soft tissue and bone. Lyon: IARC; 2013. p. 72–3.
2.
go back to reference Kasper B, Baumgarten C, Bonvalot S, Haas R, Haller F, Hohenberger P, et al. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients’ and professionals’ expertise—a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/soft tissue and bone sarcoma group initiative. Eur J Cancer. 2015;51:127–36.CrossRef Kasper B, Baumgarten C, Bonvalot S, Haas R, Haller F, Hohenberger P, et al. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients’ and professionals’ expertise—a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/soft tissue and bone sarcoma group initiative. Eur J Cancer. 2015;51:127–36.CrossRef
3.
go back to reference Fiore M, Rimareix F, Mariani L, Domont J, Collini P, Le Péchoux C, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009;16:2587–93.CrossRef Fiore M, Rimareix F, Mariani L, Domont J, Collini P, Le Péchoux C, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009;16:2587–93.CrossRef
4.
go back to reference Kasper B, Baumgarten C, Garcia J, Bonvalot S, Haas R, Haller F, et al. An update on the management of sporadic desmoid-type fibromatosis: a European consensus initiative between sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/soft tissue and bone sarcoma group (STBSG). Ann Oncol. 2017;28:2399–408.CrossRef Kasper B, Baumgarten C, Garcia J, Bonvalot S, Haas R, Haller F, et al. An update on the management of sporadic desmoid-type fibromatosis: a European consensus initiative between sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/soft tissue and bone sarcoma group (STBSG). Ann Oncol. 2017;28:2399–408.CrossRef
5.
go back to reference Desmoid Tumor Working Group. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer. 2020;127:96–107.CrossRef Desmoid Tumor Working Group. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer. 2020;127:96–107.CrossRef
6.
go back to reference Newman ET, Lans J, Kim J, Ferrone M, Ready J, Schwab J, et al. PROMIS function scores are lower in patients who underwent more aggressive local treatment for desmoid tumors. Clin Orthop Relat Res. 2020;478:563–77.CrossRef Newman ET, Lans J, Kim J, Ferrone M, Ready J, Schwab J, et al. PROMIS function scores are lower in patients who underwent more aggressive local treatment for desmoid tumors. Clin Orthop Relat Res. 2020;478:563–77.CrossRef
7.
go back to reference Slowik V, Attard T, Dai H, Shah R, Septer S. Desmoid tumors complicating familial adenomatous polyposis: a meta-analysis mutation spectrum of affected individuals. BMC Gastroenterol. 2015;15:84.CrossRef Slowik V, Attard T, Dai H, Shah R, Septer S. Desmoid tumors complicating familial adenomatous polyposis: a meta-analysis mutation spectrum of affected individuals. BMC Gastroenterol. 2015;15:84.CrossRef
8.
go back to reference Bishop AJ, Landry JP, Roland CL, Ratan R, Feig BW, Moon BS, et al. Certain risk factors for patients with desmoid tumors warrant reconsideration of local therapy strategies. Cancer. 2020;126:3265–73.CrossRef Bishop AJ, Landry JP, Roland CL, Ratan R, Feig BW, Moon BS, et al. Certain risk factors for patients with desmoid tumors warrant reconsideration of local therapy strategies. Cancer. 2020;126:3265–73.CrossRef
9.
go back to reference Azzarelli A, Gronchi A, Bertulli R, Tesoro JD, Baratti D, Pennacchioli E, et al. Low-dose chemotherapy with methotrexate and vinblastine for patients with advanced aggressive fibromatosis. Cancer. 2001;92:1259–64.CrossRef Azzarelli A, Gronchi A, Bertulli R, Tesoro JD, Baratti D, Pennacchioli E, et al. Low-dose chemotherapy with methotrexate and vinblastine for patients with advanced aggressive fibromatosis. Cancer. 2001;92:1259–64.CrossRef
10.
go back to reference Eisenhauer EA, Therasse P, Bogaerts J, Schwartz LH, Sargent D, Ford R, et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer. 2009;45:228–47.CrossRef Eisenhauer EA, Therasse P, Bogaerts J, Schwartz LH, Sargent D, Ford R, et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer. 2009;45:228–47.CrossRef
11.
go back to reference Enneking WF, Dunham W, Gebhardt MC, Malawar M, Pritchard DJ. A system for the functional evaluation of reconstructive procedures after surgical treatment of tumors of the musculoskeletal system. Clin Orthop Relat Res. 1993;286:241–6. Enneking WF, Dunham W, Gebhardt MC, Malawar M, Pritchard DJ. A system for the functional evaluation of reconstructive procedures after surgical treatment of tumors of the musculoskeletal system. Clin Orthop Relat Res. 1993;286:241–6.
12.
go back to reference Bonvalot S, Eldweny H, Haddad V, Rimareix F, Missenard G, Oberlin O, et al. Extra-abdominal primary fibromatosis: aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol. 2008;34:462–8.CrossRef Bonvalot S, Eldweny H, Haddad V, Rimareix F, Missenard G, Oberlin O, et al. Extra-abdominal primary fibromatosis: aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol. 2008;34:462–8.CrossRef
13.
go back to reference Barbier O, Anract P, Pluot E, Larouserie F, Sailhan F, Babinet A, et al. Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only. Orthop Traumatol Surg Res. 2010;96:884–9.CrossRef Barbier O, Anract P, Pluot E, Larouserie F, Sailhan F, Babinet A, et al. Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only. Orthop Traumatol Surg Res. 2010;96:884–9.CrossRef
14.
go back to reference Salas S, Dufresne A, Bui B, Blay J-Y, Terrier P, Ranchere-Vince D, et al. Prognostic factors influencing progression-free survival determined from a series of sporadic desmoid tumors: a wait-and-see policy according to tumor presentation. J Clin Oncol. 2011;29:3553–8.CrossRef Salas S, Dufresne A, Bui B, Blay J-Y, Terrier P, Ranchere-Vince D, et al. Prognostic factors influencing progression-free survival determined from a series of sporadic desmoid tumors: a wait-and-see policy according to tumor presentation. J Clin Oncol. 2011;29:3553–8.CrossRef
15.
go back to reference Briand S, Barbier O, Biau D, Bertrand-Vasseur A, Larousserie F, Anract P, et al. Wait-and-see policy as a first-line management for extra-abdominal desmoid tumors. J Bone Joint Surg Am. 2014;96:631–8.CrossRef Briand S, Barbier O, Biau D, Bertrand-Vasseur A, Larousserie F, Anract P, et al. Wait-and-see policy as a first-line management for extra-abdominal desmoid tumors. J Bone Joint Surg Am. 2014;96:631–8.CrossRef
16.
go back to reference Huang K, Wang CM, Chen JG, Du CY, Zhou Y, Shi YQ, et al. Prognostic factors influencing event-free survival and treatments in desmoid-type fibromatosis: analysis from a large institution. Am J Surg. 2014;207:847–54.CrossRef Huang K, Wang CM, Chen JG, Du CY, Zhou Y, Shi YQ, et al. Prognostic factors influencing event-free survival and treatments in desmoid-type fibromatosis: analysis from a large institution. Am J Surg. 2014;207:847–54.CrossRef
17.
go back to reference Park JS, Nakache Y-P, Katz J, Boutin RD, Steffner RJ, Monjazeb AM, et al. Conservative management of desmoid tumors is safe and effective. J Surg Res. 2016;205:115–20.CrossRef Park JS, Nakache Y-P, Katz J, Boutin RD, Steffner RJ, Monjazeb AM, et al. Conservative management of desmoid tumors is safe and effective. J Surg Res. 2016;205:115–20.CrossRef
18.
go back to reference Burtenshaw SM, Cannell AJ, McAlister ED, Siddique S, Kandel R, Blackstein ME, et al. Toward observation as first-line management in abdominal desmoid tumors. Ann Surg Oncol. 2016;23:2212–9.CrossRef Burtenshaw SM, Cannell AJ, McAlister ED, Siddique S, Kandel R, Blackstein ME, et al. Toward observation as first-line management in abdominal desmoid tumors. Ann Surg Oncol. 2016;23:2212–9.CrossRef
19.
go back to reference Penel N, Le Cesne A, Bonvalot S, Giraud A, Bompas E, Rios M, et al. Surgical versus non-surgical approach in primary desmoid-type fibromatosis patients: a nationwide prospective cohort from the French sarcoma group. Eur J Cancer. 2017;83:125–31.CrossRef Penel N, Le Cesne A, Bonvalot S, Giraud A, Bompas E, Rios M, et al. Surgical versus non-surgical approach in primary desmoid-type fibromatosis patients: a nationwide prospective cohort from the French sarcoma group. Eur J Cancer. 2017;83:125–31.CrossRef
20.
go back to reference Orbach D, Brennan B, Bisogno G, Van Noesel M, Minard-Colin V, Daragjati J, et al. The EpSSG NRSTS 2005 treatment protocol for desmoid-type fibromatosis in children: an international prospective case series. Lancet Child Adolesc Health. 2017;1:284–92.CrossRef Orbach D, Brennan B, Bisogno G, Van Noesel M, Minard-Colin V, Daragjati J, et al. The EpSSG NRSTS 2005 treatment protocol for desmoid-type fibromatosis in children: an international prospective case series. Lancet Child Adolesc Health. 2017;1:284–92.CrossRef
21.
go back to reference Cassidy MR, Lefkowitz RA, Long N, Qin L-X, Kirane A, Sbaity E, et al. Association of MRI T2 signal intensity with desmoid tumor progression during active observation: a retrospective cohort study. Ann Surg. 2020;271:748–55.CrossRef Cassidy MR, Lefkowitz RA, Long N, Qin L-X, Kirane A, Sbaity E, et al. Association of MRI T2 signal intensity with desmoid tumor progression during active observation: a retrospective cohort study. Ann Surg. 2020;271:748–55.CrossRef
22.
go back to reference van Broekhoven DLM, Verschoor AJ, van Dalen T, Grünhagen DJ, den Bakker MA, Gelderblom H, et al. Outcome of nonsurgical management of extra-abdominal, trunk, and abdominal wall desmoid-type fibromatosis: a population-based study in the Netherlands. Sarcoma. 2018;2018:5982575.PubMedPubMedCentral van Broekhoven DLM, Verschoor AJ, van Dalen T, Grünhagen DJ, den Bakker MA, Gelderblom H, et al. Outcome of nonsurgical management of extra-abdominal, trunk, and abdominal wall desmoid-type fibromatosis: a population-based study in the Netherlands. Sarcoma. 2018;2018:5982575.PubMedPubMedCentral
23.
go back to reference van Houdt WJ, Husson O, Patel A, Jones RL, Smith MJF, Miah AB, et al. Outcome of primary desmoid tumors at all anatomic locations initially managed with active surveillance. Ann Surg Oncol. 2019;26:4699–706.CrossRef van Houdt WJ, Husson O, Patel A, Jones RL, Smith MJF, Miah AB, et al. Outcome of primary desmoid tumors at all anatomic locations initially managed with active surveillance. Ann Surg Oncol. 2019;26:4699–706.CrossRef
24.
go back to reference Turner B, Alghamdi M, Henning J-W, Kurien E, Morris D, Bouchard-Fortier A, et al. Surgical excision versus observation as initial management of desmoid tumors: a population-based study. Eur J Surg Oncol. 2019;45:699–703.CrossRef Turner B, Alghamdi M, Henning J-W, Kurien E, Morris D, Bouchard-Fortier A, et al. Surgical excision versus observation as initial management of desmoid tumors: a population-based study. Eur J Surg Oncol. 2019;45:699–703.CrossRef
25.
go back to reference de Bruyns A, Li H, MacNeil A, Simmons C, Clarkson P, Goddard K, et al. Evolving practice patterns over two decades (1993–2013) in the management of desmoid-type fibromatosis in British Columbia. Clin Oncol (R Coll Radiol). 2020;32:e102–10.CrossRef de Bruyns A, Li H, MacNeil A, Simmons C, Clarkson P, Goddard K, et al. Evolving practice patterns over two decades (1993–2013) in the management of desmoid-type fibromatosis in British Columbia. Clin Oncol (R Coll Radiol). 2020;32:e102–10.CrossRef
26.
go back to reference Kito M, Ogose A, Yoshida M, Nishida Y. Usefulness of surgical treatment for asymptomatic patients with extra-peritoneal desmoid-type fibromatosis: a systematic review and meta-analysis. Jpn J Clin Oncol. 2020;50:574–80.CrossRef Kito M, Ogose A, Yoshida M, Nishida Y. Usefulness of surgical treatment for asymptomatic patients with extra-peritoneal desmoid-type fibromatosis: a systematic review and meta-analysis. Jpn J Clin Oncol. 2020;50:574–80.CrossRef
27.
go back to reference Bonvalot S, Ternès N, Fiore M, Bitsakou G, Colombo C, Honoré C, et al. Spontaneous regression of primary abdominal wall desmoid tumors: more common than previously thought. Ann Surg Oncol. 2013;20:4096–102.CrossRef Bonvalot S, Ternès N, Fiore M, Bitsakou G, Colombo C, Honoré C, et al. Spontaneous regression of primary abdominal wall desmoid tumors: more common than previously thought. Ann Surg Oncol. 2013;20:4096–102.CrossRef
28.
go back to reference Timbergen MJM, Schut A-RW, Grünhagen DJ, Sleijfer S, Verhoef C. Active surveillance in desmoid-type fibromatosis: a systematic literature review. Eur J Cancer. 2020;137:18–29.CrossRef Timbergen MJM, Schut A-RW, Grünhagen DJ, Sleijfer S, Verhoef C. Active surveillance in desmoid-type fibromatosis: a systematic literature review. Eur J Cancer. 2020;137:18–29.CrossRef
30.
go back to reference Duhil de Bénazé G, Vigan M, Corradini N, Minard-Colin V, Marie-Cardine A, Verite C, et al. Functional analysis of young patients with desmoid-type fibromatosis: Initial surveillance does not jeopardize long term quality of life. Eur J Surg Oncol. 2020;46:1294–300.CrossRef Duhil de Bénazé G, Vigan M, Corradini N, Minard-Colin V, Marie-Cardine A, Verite C, et al. Functional analysis of young patients with desmoid-type fibromatosis: Initial surveillance does not jeopardize long term quality of life. Eur J Surg Oncol. 2020;46:1294–300.CrossRef
31.
go back to reference Gronchi A, Colombo C, Le Péchoux C, Dei Tos AP, Le Cesne A, Marrari A, et al. Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm—a position paper from the Italian and the French sarcoma group. Ann Oncol. 2014;25:578–83.CrossRef Gronchi A, Colombo C, Le Péchoux C, Dei Tos AP, Le Cesne A, Marrari A, et al. Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm—a position paper from the Italian and the French sarcoma group. Ann Oncol. 2014;25:578–83.CrossRef
32.
go back to reference Sparber-Sauer M, Seitz G, von Kalle T, Vokuhl C, Leuschner I, Scheer M, et al. Systemic therapy of aggressive fibromatosis in children and adolescents: report of the cooperative Weichteilsarkom Studiengruppe (CWS). Pediatr Blood Cancer. 2018;65:e26943.CrossRef Sparber-Sauer M, Seitz G, von Kalle T, Vokuhl C, Leuschner I, Scheer M, et al. Systemic therapy of aggressive fibromatosis in children and adolescents: report of the cooperative Weichteilsarkom Studiengruppe (CWS). Pediatr Blood Cancer. 2018;65:e26943.CrossRef
33.
go back to reference He XD, Zhang YB, Wang L, Tian ML, Liu W, Qu Q, et al. Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: analysis of 114 patients at a single institution. Eur J Surg Oncol. 2015;41:1013–9.CrossRef He XD, Zhang YB, Wang L, Tian ML, Liu W, Qu Q, et al. Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: analysis of 114 patients at a single institution. Eur J Surg Oncol. 2015;41:1013–9.CrossRef
34.
go back to reference Colombo C, Miceli R, Le Péchoux C, Palassini E, Honoré C, Stacchiotti S, et al. Sporadic extra abdominal wall desmoid-type fibromatosis: surgical resection can be safely limited to a minority of patients. Eur J Cancer. 2015;51:186–92.CrossRef Colombo C, Miceli R, Le Péchoux C, Palassini E, Honoré C, Stacchiotti S, et al. Sporadic extra abdominal wall desmoid-type fibromatosis: surgical resection can be safely limited to a minority of patients. Eur J Cancer. 2015;51:186–92.CrossRef
35.
go back to reference Crago AM, Denton B, Salas S, Dufresne A, Mezhir JJ, Hameed M, et al. A prognostic nomogram for prediction of recurrence in desmoid fibromatosis. Ann Surg. 2013;258:347–53.CrossRef Crago AM, Denton B, Salas S, Dufresne A, Mezhir JJ, Hameed M, et al. A prognostic nomogram for prediction of recurrence in desmoid fibromatosis. Ann Surg. 2013;258:347–53.CrossRef
36.
go back to reference Huang K, Fu H, Shi Y-Q, Zhou Y, Du C-Y. Prognostic factors for extra-abdominal and abdominal wall desmoids: a 20-year experience at a single institution. J Surg Oncol. 2009;100:563–9.CrossRef Huang K, Fu H, Shi Y-Q, Zhou Y, Du C-Y. Prognostic factors for extra-abdominal and abdominal wall desmoids: a 20-year experience at a single institution. J Surg Oncol. 2009;100:563–9.CrossRef
37.
go back to reference Hong H, Nadesan P, Poon R, Alman BA. Testosterone regulates cell proliferation in aggressive fibromatosis (desmoid tumour). Br J Cancer. 2011;104:1452–8.CrossRef Hong H, Nadesan P, Poon R, Alman BA. Testosterone regulates cell proliferation in aggressive fibromatosis (desmoid tumour). Br J Cancer. 2011;104:1452–8.CrossRef
38.
go back to reference Janssen ML, van Broekhoven DLM, Cates JMM, Bramer WM, Nuyttens JJ, Gronchi A, et al. Meta-analysis of the influence of surgical margin and adjuvant radiotherapy on local recurrence after resection of sporadic desmoid-type fibromatosis. Br J Surg. 2017;104:347–57.CrossRef Janssen ML, van Broekhoven DLM, Cates JMM, Bramer WM, Nuyttens JJ, Gronchi A, et al. Meta-analysis of the influence of surgical margin and adjuvant radiotherapy on local recurrence after resection of sporadic desmoid-type fibromatosis. Br J Surg. 2017;104:347–57.CrossRef
Metadata
Title
Upfront surgery is not advantageous compared to more conservative treatments such as observation or medical treatment for patients with desmoid tumors
Authors
Shinji Tsukamoto
Piergiuseppe Tanzi
Andreas F. Mavrogenis
Manabu Akahane
Akira Kido
Yasuhito Tanaka
Marilena Cesari
Davide Maria Donati
Alessandra Longhi
Costantino Errani
Publication date
01-12-2021
Publisher
BioMed Central
Keyword
Fibromatosis
Published in
BMC Musculoskeletal Disorders / Issue 1/2021
Electronic ISSN: 1471-2474
DOI
https://doi.org/10.1186/s12891-020-03897-9

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