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.
Similar content being viewed by others
References
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.
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.
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.
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.
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.
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.
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.
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.
Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist. 2011;16:682–93.
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.
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.
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.
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.
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.
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.
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.
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.
Catania G, Ruggeri L, Iuppa G, et al. Abdominal wall reconstruction with intraperitoneal prosthesis in desmoid tumors surgery. Updates Surg. 2012;64:43–8.
Sutton RJ, Thomas JM. Desmoid tumours of the anterior abdominal wall. Eur J Surg Oncol. 1999;25:398–400.
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.
van Elteren PH. On the combination of independent two-sample tests of Wilcoxon. Bull Int Stat Inst. 1960;37:351–61.
Pintilie M. Analysing and interpreting competing risk data. Stat Med. 2007;26:1360–7.
Francis WP, Zippel D, Mack LA, et al. Desmoids: a revelation in biology and treatment. Ann Surg Oncol. 2009;16:1650–4.
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.
Yezhelyev MV, Deigni O, Losken A. Management of full-thickness abdominal wall defects following tumor resection. Ann Plast Surg. 2012;69:186–91.
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.
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.
Benson JR, Mokbel K, Baum M. Management of desmoid tumours including a case report of toremifene. Ann Oncol. 1994;5:173–7.
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.
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.
Lev D, Kotilingam D, Wei C, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25:1785–91.
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.
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.
Spear MA, Jennings LC, Mankin HJ, et al. Individualizing management of aggressive fibromatoses. Int J Radiat Oncol Biol Phys. 1998;40:637–45.
Rock MG, Pritchard DJ, Reiman HM, et al. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984;66:1369–74.
Fiore M, Coppola S, Cannel AJ. Desmoid-type fibromatosis and pregnancy. A multi-institutional analysis of recurrence and obstetric risk. Ann Surg. doi:10.1097/SLA.0000000000000224.
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.
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.
DISCLOSURE
The authors declare no conflict of interest.
Author information
Authors and Affiliations
Corresponding authors
Rights and permissions
About this article
Cite this article
Bonvalot, S., Ternès, N., Fiore, M. et al. Spontaneous Regression of Primary Abdominal Wall Desmoid Tumors: More Common than Previously Thought. Ann Surg Oncol 20, 4096–4102 (2013). https://doi.org/10.1245/s10434-013-3197-x
Received:
Published:
Issue Date:
DOI: https://doi.org/10.1245/s10434-013-3197-x