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Erschienen in: Annals of Surgical Oncology 13/2013

01.12.2013 | Bone and Soft Tissue Sarcomas

Spontaneous Regression of Primary Abdominal Wall Desmoid Tumors: More Common than Previously Thought

verfasst von: Sylvie Bonvalot, MD, PhD, Nils Ternès, MS, Marco Fiore, MD, Georgina Bitsakou, MD, Chiara Colombo, MD, Charles Honoré, MD, Andrea Marrari, MD, Axel Le Cesne, MD, Federica Perrone, MD, Ariane Dunant, MS, Alessandro Gronchi, MD

Erschienen in: Annals of Surgical Oncology | Ausgabe 13/2013

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Abstract

Purpose

The relevance of the initial observational approach for desmoid tumors (DTs) remains unclear. We investigated a new conservative management treatment for primary abdominal wall DTs.

Methods

Data were collected from 147 patients between 1993 and 2012. The initial therapeutic approaches were categorized as front-line surgery [surgery group (SG), n = 41, 28 %] and initial observation or medical treatment [nonsurgery group (NSG), n = 106, 72 %]. The cumulative incidence of the last strategy modification was estimated using competing risk methods with variable censoring times.

Results

Of the 147 patients, 143 were female (97 %). In the SG, 27 patients (66 %) required full-thickness abdominal wall mesh repair. In the NSG, 102 patients (96 %) underwent initial observation and four received medical treatment. In the NSG, the 1- and 3-year incidences of changing to medical treatment (no further changes during the follow-up) were 19 % [95 % confidence interval (CI) 11–28] and 25 % (95 % CI 17–35), respectively, and the 1- and 3-year incidences of a final switch to surgery were 14 % (95 % CI 8–22) and 16 % (95 % CI 9–24), respectively. An initial tumor size of >7 cm was associated with a higher strategy modification risk (p = 0.004). Of the 102 patients initially observed, 29 experienced spontaneous regression over a median follow-up period of 32 months. All second-intent resections were macroscopically completed, with R0 resections achieved in 82 % of patients.

Conclusions

This study supports an initial nonsurgical approach to abdominal wall DTs ≤7 cm, followed by surgery based on tumor growth in select cases.
Literatur
1.
Zurück zum Zitat Peng PD, Hyder O, Mavros MN, et al. Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients. Ann Surg Oncol. 2012;19:4036–42.PubMedCrossRef Peng PD, Hyder O, Mavros MN, et al. Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients. Ann Surg Oncol. 2012;19:4036–42.PubMedCrossRef
2.
Zurück zum Zitat Mullen JT, Delaney TF, Kobayashi WK, et al. Desmoid tumor: analysis of prognostic factors and outcomes in a surgical series. Ann Surg Oncol. 2012;19:4028–35.PubMedCrossRef Mullen JT, Delaney TF, Kobayashi WK, et al. Desmoid tumor: analysis of prognostic factors and outcomes in a surgical series. Ann Surg Oncol. 2012;19:4028–35.PubMedCrossRef
3.
Zurück zum Zitat von Mehren M, Benjamin RS, Bui MM, et al. Soft tissue sarcoma, version 2.2012: featured updates to the NCCN guidelines. J Natl Compr Canc Netw. 2012;10:951–60. von Mehren M, Benjamin RS, Bui MM, et al. Soft tissue sarcoma, version 2.2012: featured updates to the NCCN guidelines. J Natl Compr Canc Netw. 2012;10:951–60.
4.
Zurück zum Zitat ESMO/European Sarcoma Network Working Group. Soft tissue and visceral sarcomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 2012;23(Suppl 7):vii92–9. ESMO/European Sarcoma Network Working Group. Soft tissue and visceral sarcomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 2012;23(Suppl 7):vii92–9.
5.
Zurück zum Zitat 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
6.
Zurück zum Zitat Fiore M, Rimareix F, Mariani L, 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, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009;16:2587–93.PubMedCrossRef
7.
Zurück zum Zitat 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
8.
Zurück zum Zitat Gronchi A, Raut CP. Optimal approach to sporadic desmoid tumors: from radical surgery to observation. Time for a consensus? Ann Surg Oncol. 2012;19:3995–7.PubMedCrossRef Gronchi A, Raut CP. Optimal approach to sporadic desmoid tumors: from radical surgery to observation. Time for a consensus? Ann Surg Oncol. 2012;19:3995–7.PubMedCrossRef
9.
Zurück zum Zitat Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist. 2011;16:682–93.PubMedCrossRef Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist. 2011;16:682–93.PubMedCrossRef
10.
Zurück zum Zitat Holme H, Westbury CB, Morgenstern D, et al. An important role for initial observation in the management of paediatric desmoid fibromatosis. Clin Oncol (R Coll Radiol). 2013;25:e16.PubMedCrossRef Holme H, Westbury CB, Morgenstern D, et al. An important role for initial observation in the management of paediatric desmoid fibromatosis. Clin Oncol (R Coll Radiol). 2013;25:e16.PubMedCrossRef
11.
Zurück zum Zitat Honeyman JN, Theilen TM, Knowles MA, et al. Desmoid fibromatosis in children and adolescents: a conservative approach to management. J Pediatr Surg. 2013;48:62–6.PubMedCrossRef Honeyman JN, Theilen TM, Knowles MA, et al. Desmoid fibromatosis in children and adolescents: a conservative approach to management. J Pediatr Surg. 2013;48:62–6.PubMedCrossRef
12.
Zurück zum Zitat Nishida Y, Tsukushi S, Urakawa H, et al. Is it possible to identify clinically useful prognostic groups for patients with desmoid tumors? J Clin Oncol. 2012;30:1390.PubMedCrossRef Nishida Y, Tsukushi S, Urakawa H, et al. Is it possible to identify clinically useful prognostic groups for patients with desmoid tumors? J Clin Oncol. 2012;30:1390.PubMedCrossRef
13.
Zurück zum Zitat Reitamo JJ, Hayry P, Nykyri E, et al. The desmoid tumor. I. Incidence, sex-, age and anatomical distribution in the Finnish population. Am J Clin Pathol. 1982;77:665–73.PubMed Reitamo JJ, Hayry P, Nykyri E, et al. The desmoid tumor. I. Incidence, sex-, age and anatomical distribution in the Finnish population. Am J Clin Pathol. 1982;77:665–73.PubMed
14.
Zurück zum Zitat 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
15.
Zurück zum Zitat Stoeckle E, Coindre JM, Longy 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, 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
16.
Zurück zum Zitat Bonvalot S, Desai A, Coppola S, et al. The treatment of desmoid tumors: a stepwise clinical approach. Ann Oncol. 2012;23(Suppl 10):x158–66.PubMedCrossRef Bonvalot S, Desai A, Coppola S, et al. The treatment of desmoid tumors: a stepwise clinical approach. Ann Oncol. 2012;23(Suppl 10):x158–66.PubMedCrossRef
17.
Zurück zum Zitat Bertani E, Chiappa A, Testori A, et al. Desmoid tumors of the anterior abdominal wall: results from a monocentric surgical experience and review of the literature. Ann Surg Oncol. 2009;16:1642–9.PubMedCrossRef Bertani E, Chiappa A, Testori A, et al. Desmoid tumors of the anterior abdominal wall: results from a monocentric surgical experience and review of the literature. Ann Surg Oncol. 2009;16:1642–9.PubMedCrossRef
18.
Zurück zum Zitat Catania G, Ruggeri L, Iuppa G, et al. Abdominal wall reconstruction with intraperitoneal prosthesis in desmoid tumors surgery. Updates Surg. 2012;64:43–8.PubMedCrossRef Catania G, Ruggeri L, Iuppa G, et al. Abdominal wall reconstruction with intraperitoneal prosthesis in desmoid tumors surgery. Updates Surg. 2012;64:43–8.PubMedCrossRef
19.
Zurück zum Zitat Sutton RJ, Thomas JM. Desmoid tumours of the anterior abdominal wall. Eur J Surg Oncol. 1999;25:398–400.PubMedCrossRef Sutton RJ, Thomas JM. Desmoid tumours of the anterior abdominal wall. Eur J Surg Oncol. 1999;25:398–400.PubMedCrossRef
20.
Zurück zum Zitat UICC. Tumor of bone and soft tissues. R classification. In: Sobin LH, Wittekind CH, editors. TNM classification of malignant tumours. 6th ed. New York: Wiley; 2002. p. 110. UICC. Tumor of bone and soft tissues. R classification. In: Sobin LH, Wittekind CH, editors. TNM classification of malignant tumours. 6th ed. New York: Wiley; 2002. p. 110.
21.
Zurück zum Zitat van Elteren PH. On the combination of independent two-sample tests of Wilcoxon. Bull Int Stat Inst. 1960;37:351–61. van Elteren PH. On the combination of independent two-sample tests of Wilcoxon. Bull Int Stat Inst. 1960;37:351–61.
22.
23.
Zurück zum Zitat Francis WP, Zippel D, Mack LA, et al. Desmoids: a revelation in biology and treatment. Ann Surg Oncol. 2009;16:1650–4.PubMedCrossRef Francis WP, Zippel D, Mack LA, et al. Desmoids: a revelation in biology and treatment. Ann Surg Oncol. 2009;16:1650–4.PubMedCrossRef
24.
Zurück zum Zitat Pencavel T, Strauss DC, Thomas JM, et al. The surgical management of soft tissue tumours arising in the abdominal wall. Eur J Surg Oncol. 2010;36:489–95.PubMedCrossRef Pencavel T, Strauss DC, Thomas JM, et al. The surgical management of soft tissue tumours arising in the abdominal wall. Eur J Surg Oncol. 2010;36:489–95.PubMedCrossRef
25.
Zurück zum Zitat Yezhelyev MV, Deigni O, Losken A. Management of full-thickness abdominal wall defects following tumor resection. Ann Plast Surg. 2012;69:186–91.PubMedCrossRef Yezhelyev MV, Deigni O, Losken A. Management of full-thickness abdominal wall defects following tumor resection. Ann Plast Surg. 2012;69:186–91.PubMedCrossRef
26.
Zurück zum Zitat van Persijn van Meerten EL, Gelderblom H, Bloem JL. RECIST revised: implications for the radiologist. A review article on the modified RECIST guideline. Eur Radiol. 2010;20:1456–67. van Persijn van Meerten EL, Gelderblom H, Bloem JL. RECIST revised: implications for the radiologist. A review article on the modified RECIST guideline. Eur Radiol. 2010;20:1456–67.
27.
Zurück zum Zitat Hansmann A, Adolph C, Vogel T, et al. High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer. 2004;100:612–20.PubMedCrossRef Hansmann A, Adolph C, Vogel T, et al. High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer. 2004;100:612–20.PubMedCrossRef
28.
Zurück zum Zitat Benson JR, Mokbel K, Baum M. Management of desmoid tumours including a case report of toremifene. Ann Oncol. 1994;5:173–7. Benson JR, Mokbel K, Baum M. Management of desmoid tumours including a case report of toremifene. Ann Oncol. 1994;5:173–7.
29.
Zurück zum Zitat 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
30.
Zurück zum Zitat Garbay D, Le Cesne A, Penel N. Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG). Ann Oncol. 2012;23:182–6.PubMedCrossRef Garbay D, Le Cesne A, Penel N. Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG). Ann Oncol. 2012;23:182–6.PubMedCrossRef
31.
Zurück zum Zitat 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
32.
Zurück zum Zitat Nuyttens JJ, Rust PF, Thomas CR Jr, et al. 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, et al. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles Cancer. 2000;88:1517–23.PubMedCrossRef
33.
Zurück zum Zitat 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
34.
Zurück zum Zitat 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
35.
Zurück zum Zitat 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
37.
Zurück zum Zitat Matono H, Tamiya S, Yokoyama R, et al. Abnormalities of the Wnt/β-catenin signalling pathway induce tumour progression in sporadic desmoid tumours: correlation between β-catenin widespread nuclear expression and VEGF overexpression. Histopathology. 2011;59:368–75.PubMedCrossRef Matono H, Tamiya S, Yokoyama R, et al. Abnormalities of the Wnt/β-catenin signalling pathway induce tumour progression in sporadic desmoid tumours: correlation between β-catenin widespread nuclear expression and VEGF overexpression. Histopathology. 2011;59:368–75.PubMedCrossRef
38.
Zurück zum Zitat Lazar AJ, Tuvin D, Hajibashi S, 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, 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
Metadaten
Titel
Spontaneous Regression of Primary Abdominal Wall Desmoid Tumors: More Common than Previously Thought
verfasst von
Sylvie Bonvalot, MD, PhD
Nils Ternès, MS
Marco Fiore, MD
Georgina Bitsakou, MD
Chiara Colombo, MD
Charles Honoré, MD
Andrea Marrari, MD
Axel Le Cesne, MD
Federica Perrone, MD
Ariane Dunant, MS
Alessandro Gronchi, MD
Publikationsdatum
01.12.2013
Verlag
Springer US
Erschienen in
Annals of Surgical Oncology / Ausgabe 13/2013
Print ISSN: 1068-9265
Elektronische ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-013-3197-x

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