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Published in: Annals of Surgical Oncology 13/2012

01-12-2012 | Bone and Soft Tissue Sarcomas

Desmoid Tumor: Analysis of Prognostic Factors and Outcomes in a Surgical Series

Authors: John T. Mullen, MD, Thomas F. DeLaney, MD, Wendy K. Kobayashi, BA, Jackie Szymonifka, BA, Beow Y. Yeap, ScD, Yen-Lin Chen, MD, Andrew E. Rosenberg, MD, David C. Harmon, MD, Edwin Choy, MD, PhD, Sam S. Yoon, MD, Kevin A. Raskin, MD, G. Petur Nielsen, MD, Francis J. Hornicek, MD, PhD

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

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Abstract

Background

Desmoid tumors are rare and exhibit a highly unpredictable natural history. We sought to analyze prognostic factors associated with recurrence in a large single-institution study of patients with desmoid tumors.

Methods

We performed a retrospective review of 177 patients with desmoid tumor who underwent macroscopically complete surgical resection, with or without the addition of radiotherapy (RT) or systemic therapy, from 1970 to 2009. We examined patterns of presentation, all known risk factors for recurrence, and their association with recurrence-free survival (RFS).

Results

Twenty-two patients (12 %) had intra-abdominal desmoid tumors, and 155 (88 %) had extra-abdominal tumors. Patterns of presentation included primary (n = 133, 75 %) and locally recurrent (n = 44, 25 %) disease. Treatment was surgery alone in 125 patients (71 %), surgery and RT in 36 (20 %), and surgery and systemic therapy with or without RT in 20 (11 %). Median follow-up was 40 months. Overall, the local relapse rate was 29 %, and 10-year RFS was 60 %. R0 resection status was the only predictor of freedom from local recurrence on multivariate analysis (odds ratio 0.32; 95 % confidence interval 0.15–0.66; P = 0.002). The selective use of adjuvant RT appeared to improve local control in patients with positive margins.

Conclusions

For patients with desmoid tumors undergoing surgery, wide excision with negative margins should be the goal, but not at the expense of function, as fewer than half of patients with positive margins will experience recurrence.
Literature
1.
go back to reference Alman BA, Pajerski ME, Diaz-Cano S, et al. Aggressive fibromatosis (desmoid tumor) is a monoclonal disorder. Diagn Mol Pathol. 1997;6:98–101.PubMedCrossRef Alman BA, Pajerski ME, Diaz-Cano S, et al. Aggressive fibromatosis (desmoid tumor) is a monoclonal disorder. Diagn Mol Pathol. 1997;6:98–101.PubMedCrossRef
2.
go back to reference Li M, Cordon-Cardo C, Gerald WL, Rosai J. Desmoid fibromatosis is a clonal process. Hum Pathol. 1996;27:939–43.PubMedCrossRef Li M, Cordon-Cardo C, Gerald WL, Rosai J. Desmoid fibromatosis is a clonal process. Hum Pathol. 1996;27:939–43.PubMedCrossRef
3.
go back to reference Bonvalot S, Eldweny H, Haddad V, 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, 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
4.
go back to reference Salas S, Dufresne A, Bui B, 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, 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
5.
go back to reference Acker JC, Bossen EH, Halperin EC. The management of desmoid tumors. Int J Radiat Oncol Biol Phys. 1993;26:851–8.PubMedCrossRef Acker JC, Bossen EH, Halperin EC. The management of desmoid tumors. Int J Radiat Oncol Biol Phys. 1993;26:851–8.PubMedCrossRef
6.
go back to reference Karakousis CP, Mayordomo J, Zografos GC, Driscoll DL. Desmoid tumors of the trunk and extremity. Cancer. 1993;72:1637–41.PubMedCrossRef Karakousis CP, Mayordomo J, Zografos GC, Driscoll DL. Desmoid tumors of the trunk and extremity. Cancer. 1993;72:1637–41.PubMedCrossRef
7.
go back to reference Lopez R, Kemalyan N, Moseley HS, et al. Problems in diagnosis and management of desmoid tumors. Am J Surg. 1990;159:450–3.PubMedCrossRef Lopez R, Kemalyan N, Moseley HS, et al. Problems in diagnosis and management of desmoid tumors. Am J Surg. 1990;159:450–3.PubMedCrossRef
8.
go back to reference McKinnon JG, Neifeld JP, Kay S, et al. Management of desmoid tumors. Surg Gynecol Obstet. 1989;169:104–6.PubMed McKinnon JG, Neifeld JP, Kay S, et al. Management of desmoid tumors. Surg Gynecol Obstet. 1989;169:104–6.PubMed
9.
go back to reference Plukker JT, van Oort I, Vermey A, et al. Aggressive fibromatosis (non-familial desmoid tumour): therapeutic problems and the role of adjuvant radiotherapy. Br J Surg. 1995;82:510–4.PubMedCrossRef Plukker JT, van Oort I, Vermey A, et al. Aggressive fibromatosis (non-familial desmoid tumour): therapeutic problems and the role of adjuvant radiotherapy. Br J Surg. 1995;82:510–4.PubMedCrossRef
10.
go back to reference Rock MG, Pritchard DJ, Reiman HM, et al. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984;66:1369–74.PubMed Rock MG, Pritchard DJ, Reiman HM, et al. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984;66:1369–74.PubMed
11.
go back to reference Spear MA, Jennings LC, Mankin HJ, et al. Individualizing management of aggressive fibromatoses. Int J Radiat Oncol Biol Phys. 1998;40:637–45.PubMedCrossRef Spear MA, Jennings LC, Mankin HJ, et al. Individualizing management of aggressive fibromatoses. Int J Radiat Oncol Biol Phys. 1998;40:637–45.PubMedCrossRef
12.
go back to reference Posner MC, Shiu MH, Newsome JL, et al. The desmoid tumor. Not a benign disease. Arch Surg. 1989;124:191–6.CrossRef Posner MC, Shiu MH, Newsome JL, et al. The desmoid tumor. Not a benign disease. Arch Surg. 1989;124:191–6.CrossRef
13.
go back to reference Pritchard DJ, Nascimento AG, Petersen IA. Local control of extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1996;78:848–54.PubMed Pritchard DJ, Nascimento AG, Petersen IA. Local control of extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1996;78:848–54.PubMed
14.
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
15.
go back to reference Ballo MT, Zagars GK, Pollack A, et al. 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, et al. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999;17:158–67.PubMed
16.
go back to reference Huang K, Fu H, Shi YQ, et al. 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, et al. 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
17.
go back to reference Merchant NB, Lewis JJ, Woodruff JM, et al. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer. 1999;86:2045–52.PubMedCrossRef Merchant NB, Lewis JJ, Woodruff JM, et al. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer. 1999;86:2045–52.PubMedCrossRef
18.
go back to reference Gronchi A, Casali PG, Mariani 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, 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
19.
go back to reference Lev D, Kotilingam D, Wei C, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25:1785–91.PubMedCrossRef Lev D, Kotilingam D, Wei C, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25:1785–91.PubMedCrossRef
20.
go back to reference Goy BW, Lee SP, Eilber F, et al. The role of adjuvant radiotherapy in the treatment of resectable desmoid tumors. Int J Radiat Oncol Biol Phys. 1997;39:659–65.PubMedCrossRef Goy BW, Lee SP, Eilber F, et al. The role of adjuvant radiotherapy in the treatment of resectable desmoid tumors. Int J Radiat Oncol Biol Phys. 1997;39:659–65.PubMedCrossRef
21.
go back to reference Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT 3rd. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer. 2000;88:1517–23.PubMedCrossRef Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT 3rd. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer. 2000;88:1517–23.PubMedCrossRef
22.
go back to reference Ballo MT, Zagars GK, Pollack A. Radiation therapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys. 1998;42:1007–14.PubMedCrossRef Ballo MT, Zagars GK, Pollack A. Radiation therapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys. 1998;42:1007–14.PubMedCrossRef
23.
go back to reference Gluck I, Griffith KA, Biermann JS, et al. Role of radiotherapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys. 2011;80:787–92.PubMedCrossRef Gluck I, Griffith KA, Biermann JS, et al. Role of radiotherapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys. 2011;80:787–92.PubMedCrossRef
24.
go back to reference Micke O, Seegenschmiedt MH. Radiation therapy for aggressive fibromatosis (desmoid tumors): results of a national patterns of care study. Int J Radiat Oncol Biol Phys. 2005;61:882–91.PubMedCrossRef Micke O, Seegenschmiedt MH. Radiation therapy for aggressive fibromatosis (desmoid tumors): results of a national patterns of care study. Int J Radiat Oncol Biol Phys. 2005;61:882–91.PubMedCrossRef
25.
go back to reference Patel SR, Evans HL, Benjamin RS. Combination chemotherapy in adult desmoid tumors. Cancer. 1993;72:3244–7.PubMedCrossRef Patel SR, Evans HL, Benjamin RS. Combination chemotherapy in adult desmoid tumors. Cancer. 1993;72:3244–7.PubMedCrossRef
26.
go back to reference de Camargo VP, Keohan ML, D’Adamo DR, et al. Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer. 2010;116:2258–65.PubMed de Camargo VP, Keohan ML, D’Adamo DR, et al. Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer. 2010;116:2258–65.PubMed
27.
go back to reference Fiore M, Rimareix F, Mariani L, et al. Desmoid-type fibromatosis: a frontline conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009;16:2587–93.PubMedCrossRef Fiore M, Rimareix F, Mariani L, et al. Desmoid-type fibromatosis: a frontline conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009;16:2587–93.PubMedCrossRef
Metadata
Title
Desmoid Tumor: Analysis of Prognostic Factors and Outcomes in a Surgical Series
Authors
John T. Mullen, MD
Thomas F. DeLaney, MD
Wendy K. Kobayashi, BA
Jackie Szymonifka, BA
Beow Y. Yeap, ScD
Yen-Lin Chen, MD
Andrew E. Rosenberg, MD
David C. Harmon, MD
Edwin Choy, MD, PhD
Sam S. Yoon, MD
Kevin A. Raskin, MD
G. Petur Nielsen, MD
Francis J. Hornicek, MD, 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/s10434-012-2638-2

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