Skip to main content
Top
Published in: Annals of Surgical Oncology 13/2012

01-12-2012 | Bone and Soft Tissue Sarcomas

Management and Recurrence Patterns of Desmoids Tumors: A Multi-institutional Analysis of 211 Patients

Authors: Peter D. Peng, MD, Omar Hyder, MD, Michael N. Mavros, MD, Ryan Turley, MD, Ryan Groeschl, MD, Amin Firoozmand, MD, Michael Lidsky, MD, Joseph M. Herman, MD, MSc, Michael Choti, MD, Nita Ahuja, MD, Robert Anders, MD, PhD, Daniel G. Blazer III, MD, T. Clark Gamblin, MD, Timothy M. Pawlik, MD, MPH, PhD

Published in: Annals of Surgical Oncology | Issue 13/2012

Login to get access

Abstract

Background

Desmoid tumors are rare soft-tissue neoplasms with limited data on their management. We sought to determine the rates of recurrence following surgery for desmoid tumors and identify factors predictive of disease-free survival.

Methods

Between January 1983 and December 2011, 211 patients with desmoid tumors were identified from three major surgical centers. Clinicopathologic and treatment characteristics were analyzed to identify predictors of recurrence.

Results

Median age was 36 years; patients were predominantly female (68 %). Desmoid tumors most commonly arose in extremities (32 %), abdominal cavity (23 %) or wall (21 %), and thorax (15 %); median size was 7.5 cm. Most patients had an R0 surgical margin (60 %). The 1- and 5-year recurrence-free survival was 81.3 and 52.8 %, respectively. Factors associated with worse recurrence-free survival were: younger age (for each 5-year increase in age, hazard ratio [HR] = 0.90, 95 % confidence interval [95 % CI] 0.82–0.98) and extra-abdominal tumor location (abdominal wall referent: extra-abdominal site, HR = 3.28, 95 % CI, 1.46–7.36) (both P < 0.05).

Conclusions

Recurrence remains a problem following resection of desmoid tumors with as many as 50 % of patients experiencing a recurrence within 5 years. Factors associated with recurrence included age, tumor location, and margin status. While surgical resection remains central to the management of patients with desmoid tumors, the high rate of recurrence highlights the need for more effective adjuvant therapies.
Literature
1.
go back to reference Latchford AR, Sturt NJ, Neale K, Rogers PA, Phillips RK. A 10-year review of surgery for desmoid disease associated with familial adenomatous polyposis. Br J Surg. 2006;93:1258–64.PubMedCrossRef Latchford AR, Sturt NJ, Neale K, Rogers PA, Phillips RK. A 10-year review of surgery for desmoid disease associated with familial adenomatous polyposis. Br J Surg. 2006;93:1258–64.PubMedCrossRef
2.
go back to reference Phillips SR, A’Hern R, Thomas JM. Aggressive fibromatosis of the abdominal wall, limbs and limb girdles. Br J Surg. 2004;91:1624–9.PubMedCrossRef Phillips SR, A’Hern R, Thomas JM. Aggressive fibromatosis of the abdominal wall, limbs and limb girdles. Br J Surg. 2004;91:1624–9.PubMedCrossRef
3.
go back to reference Reitamo JJ, Scheinin TM, Hayry P. The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg. 1986;151:230–7.PubMedCrossRef Reitamo JJ, Scheinin TM, Hayry P. The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg. 1986;151:230–7.PubMedCrossRef
4.
go back to reference Rock MG, Pritchard DJ, Reiman HM, Soule EH, Brewster RC. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984;66:1369–74.PubMed Rock MG, Pritchard DJ, Reiman HM, Soule EH, Brewster RC. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984;66:1369–74.PubMed
5.
go back to reference Müller J. Über den feineren Bau und die Formen der krankhaften Geschwülste. Erste Lieferung. Berlin: G. Reimer; 1838. Müller J. Über den feineren Bau und die Formen der krankhaften Geschwülste. Erste Lieferung. Berlin: G. Reimer; 1838.
6.
go back to reference Sakorafas GH, Nissotakis C, Peros G. Abdominal desmoid tumors. Surg Oncol. 16:131–42. Sakorafas GH, Nissotakis C, Peros G. Abdominal desmoid tumors. Surg Oncol. 16:131–42.
7.
go back to reference Huang K, Fu H, Shi YQ, Zhou Y, Du CY. Prognostic factors for extra-abdominal and abdominal wall desmoids: a 20-year experience at a single institution. J Surg Oncol. 2009;100:563–9.PubMedCrossRef Huang K, Fu H, Shi YQ, Zhou Y, Du CY. Prognostic factors for extra-abdominal and abdominal wall desmoids: a 20-year experience at a single institution. J Surg Oncol. 2009;100:563–9.PubMedCrossRef
8.
go back to reference Lev D, Kotilingam D, Wei C, Ballo MT, Zagars GK, Pisters PW, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25:1785–91.PubMedCrossRef Lev D, Kotilingam D, Wei C, Ballo MT, Zagars GK, Pisters PW, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25:1785–91.PubMedCrossRef
9.
go back to reference Salas S, Dufresne A, Bui B, Blay JY, 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.PubMedCrossRef Salas S, Dufresne A, Bui B, Blay JY, 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.PubMedCrossRef
10.
go back to reference Rodriguez-Bigas MA, Mahoney MC, Karakousis CP, Petrelli NJ. Desmoid tumors in patients with familial adenomatous polyposis. Cancer. 1994;74:1270–4.PubMedCrossRef Rodriguez-Bigas MA, Mahoney MC, Karakousis CP, Petrelli NJ. Desmoid tumors in patients with familial adenomatous polyposis. Cancer. 1994;74:1270–4.PubMedCrossRef
11.
go back to reference Kulaylat MN, Karakousis CP, Keaney CM, McCorvey D, Bem J, Ambrus Sr JL. Desmoid tumour: a pleomorphic lesion. Eur J Surg Oncol. 1999;25:487–97.PubMedCrossRef Kulaylat MN, Karakousis CP, Keaney CM, McCorvey D, Bem J, Ambrus Sr JL. Desmoid tumour: a pleomorphic lesion. Eur J Surg Oncol. 1999;25:487–97.PubMedCrossRef
12.
go back to reference Ballo MT, Zagars GK, Pollack A, Pisters PW, Pollack RA. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999;17:158–67.PubMed Ballo MT, Zagars GK, Pollack A, Pisters PW, Pollack RA. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999;17:158–67.PubMed
13.
go back to reference Gronchi A, Casali PG, Mariani L, Lo Vullo S, Colecchia M, Lozza L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol. 2003;21:1390–7.PubMedCrossRef Gronchi A, Casali PG, Mariani L, Lo Vullo S, Colecchia M, Lozza L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol. 2003;21:1390–7.PubMedCrossRef
14.
go back to reference Sprague S, Matta JM, Bhandari M, et al. Multicenter collaboration in observational research: improving generalizability and efficiency. J Bone Joint Surg Am. 2009;91 Suppl 3:80–6.PubMedCrossRef Sprague S, Matta JM, Bhandari M, et al. Multicenter collaboration in observational research: improving generalizability and efficiency. J Bone Joint Surg Am. 2009;91 Suppl 3:80–6.PubMedCrossRef
15.
go back to reference Dindo D, Demartines N, Clavien PA. Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg. 2004;240:205–13.PubMedCrossRef Dindo D, Demartines N, Clavien PA. Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg. 2004;240:205–13.PubMedCrossRef
16.
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.PubMedCrossRef 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.PubMedCrossRef
17.
go back to reference Stoeckle E, Coindre JM, Longy M, Binh MB, Kantor G, Kind M, et al. A critical analysis of treatment strategies in desmoid tumours: a review of a series of 106 cases. Eur J Surg Oncol. 2009;35:129–34.PubMedCrossRef Stoeckle E, Coindre JM, Longy M, Binh MB, Kantor G, Kind M, et al. A critical analysis of treatment strategies in desmoid tumours: a review of a series of 106 cases. Eur J Surg Oncol. 2009;35:129–34.PubMedCrossRef
18.
go back to reference Lazar AJ, Tuvin D, Hajibashi S, Habeeb S, Bolshakov S, Mayordomo-Aranda E, et al. Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am J Pathol. 2008;173:1518–27.PubMedCrossRef Lazar AJ, Tuvin D, Hajibashi S, Habeeb S, Bolshakov S, Mayordomo-Aranda E, et al. Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am J Pathol. 2008;173:1518–27.PubMedCrossRef
19.
go back to reference Sorensen A, Keller J, Nielsen OS, Jensen OM. Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1–27 years. Acta Orthop Scand. 2002;73:213–9.PubMedCrossRef Sorensen A, Keller J, Nielsen OS, Jensen OM. Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1–27 years. Acta Orthop Scand. 2002;73:213–9.PubMedCrossRef
20.
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.PubMedCrossRef 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.PubMedCrossRef
21.
go back to reference Salas S, Chibon F, Noguchi T, Terrier P, Ranchere-Vince D, Lagarde P, et al. Molecular characterization by array comparative genomic hybridization and DNA sequencing of 194 desmoid tumors. Genes Chromosomes Cancer. 2010;49:560–8.PubMed Salas S, Chibon F, Noguchi T, Terrier P, Ranchere-Vince D, Lagarde P, et al. Molecular characterization by array comparative genomic hybridization and DNA sequencing of 194 desmoid tumors. Genes Chromosomes Cancer. 2010;49:560–8.PubMed
22.
go back to reference Merchant NB, Lewis JJ, Woodruff JM, Leung DH, Brennan MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer. 1999;86:2045–52.PubMedCrossRef Merchant NB, Lewis JJ, Woodruff JM, Leung DH, Brennan MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer. 1999;86:2045–52.PubMedCrossRef
Metadata
Title
Management and Recurrence Patterns of Desmoids Tumors: A Multi-institutional Analysis of 211 Patients
Authors
Peter D. Peng, MD
Omar Hyder, MD
Michael N. Mavros, MD
Ryan Turley, MD
Ryan Groeschl, MD
Amin Firoozmand, MD
Michael Lidsky, MD
Joseph M. Herman, MD, MSc
Michael Choti, MD
Nita Ahuja, MD
Robert Anders, MD, PhD
Daniel G. Blazer III, MD
T. Clark Gamblin, MD
Timothy M. Pawlik, MD, MPH, PhD
Publication date
01-12-2012
Publisher
Springer-Verlag
Published in
Annals of Surgical Oncology / Issue 13/2012
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-012-2634-6

Other articles of this Issue 13/2012

Annals of Surgical Oncology 13/2012 Go to the issue