Annals of Surgical Oncology

, Volume 20, Issue 11, pp 3384–3390 | Cite as

Surgical Treatment of Pediatric Desmoid Tumors. A 12-Year, Single-Center Experience

  • Miguel A. Soto-Miranda
  • John A. Sandoval
  • Bhaskar Rao
  • Mike Neel
  • Matt Krasin
  • Sheri Spunt
  • Jesse J. Jenkins
  • Andrew M. Davidoff
  • Jon P. Ver Halen
Bone and Soft Tissue Sarcomas



Pediatric desmoid tumors (PDTs) represent a group of rare, distinct lesions. While sparse, available literature suggests that PDT are particularly aggressive and difficult to control when compared with their adult counterpart.


A retrospective review identified 39 patients who underwent treatment of PDT at St. Jude Children’s Research Hospital over a 12-year period. Clinicopathologic and treatment characteristics were analyzed to identify predictors of outcome.


A total of 39 patients were treated during the study period, with a total number of 67 resections. Median age was 12.2 years; 49 % of patients were male, and 51 % were female. Median tumor size was 9.8 cm. PDT most commonly arose in the extremities (40 %), thorax (23 %), head and neck (21 %), and trunk (16 %). Also, 18 % of resections had negative margins (R0), 48 % were microscopic positive (R1), and 30 % were macroscopic positive (R2). The 1- and 5-year recurrence-free survival (RFS) was 97.1 and 73.1 %, respectively. Factors associated with worse RFS were patient age >12 years (HR = 5.08, p = 0.038) and tumor size >5 cm (HR = 1.22, p = 0.0597). Margin status did not affect RFS. Selective use of radiation therapy appeared to improve RFS.


Our study suggests that margin status alone at the time of extirpation is not a predictor of ultimate cure or likelihood of recurrence. Many patients received adjuvant therapy, with benefits suggested after analysis. For patients with PDT, surgical extirpation should not come at the expense of functional preservation, as overall survival is excellent.


Sorafenib Familial Adenomatous Polyposis Margin Status Negative Margin Aggressive Fibromatosis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



None of the authors has any source of financial or other support, or any financial of professional relationship that may pose a competing interest.


  1. 1.
    Rao BN, Horowitz ME, Parham DM, Etcubanas EE, Fleming ID, Pratt CB, et al. Challenges in the treatment of childhood fibromatosis. Arch Surg. 1987;122:1296–8.PubMedCrossRefGoogle Scholar
  2. 2.
    Reitamo JJ, Scheinin TM, Häyry P. The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg. 1986;151:230–7.PubMedCrossRefGoogle Scholar
  3. 3.
    Meazza C, Bisogno G, Gronchi A, Fiore M, Cecchetto G, Alaggio R, et al. Aggressive fibromatosis in children and adolescents: the Italian experience. Cancer. 2010;116:233–40.PubMedGoogle Scholar
  4. 4.
    Sharma A, Ngan B-Y, Sándor GKB, Campisi P, Forte V. Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review. J Pediatr Surg. 2008;43:1596–604.PubMedCrossRefGoogle Scholar
  5. 5.
    Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist. 2011;16:682–93.PubMedCrossRefGoogle Scholar
  6. 6.
    Posner MC, Shiu MH, Newsome JL, Hajdu SI, Gaynor JJ, Brennan MF. The desmoid tumor. Not a benign disease. Arch Surg. 1989;124:191–6.PubMedCrossRefGoogle Scholar
  7. 7.
    Rutenberg MS, Indelicato DJ, Knapik JA, Lagmay JP, Morris C, Zlotecki RA, et al. External-beam radiotherapy for pediatric and young adult desmoid tumors. Pediatr Blood Cancer. 2011;57:435–42.PubMedCrossRefGoogle Scholar
  8. 8.
    Bertario L, Russo A, Sala P, Varesco L, Giarola M, Mondini P, et al. Multiple approach to the exploration of genotype–phenotype correlations in familial adenomatous polyposis. J Clin Oncol. 2003;21:1698–707.PubMedCrossRefGoogle Scholar
  9. 9.
    Clark SK, Phillips RK. Desmoids in familial adenomatous polyposis. Br J Surg. 1996;83:1494–504.PubMedCrossRefGoogle Scholar
  10. 10.
    Dahn I, Jonsson N, Lundh G. Desmoid tumours. A series of 33 cases. Acta Chir Scand. 1963;126:305–14.PubMedGoogle Scholar
  11. 11.
    Casali PG, Blay J-Y. Gastrointestinal stromal tumours: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2010;21(Suppl 5):v98–102.PubMedCrossRefGoogle Scholar
  12. 12.
    Lev D, Kotilingam D, Wei C, Ballo MT, Zagars GK, Pisters PWT, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25:1785–91.PubMedCrossRefGoogle Scholar
  13. 13.
    Lazar AJF, Hajibashi S, Lev D. Desmoid tumor: from surgical extirpation to molecular dissection. Curr Opin Oncol. 2009;21:352–9.PubMedCrossRefGoogle Scholar
  14. 14.
    Merchant TE, Nguyen D, Walter AW, Pappo AS, Kun LE, Rao BN. Long-term results with radiation therapy for pediatric desmoid tumors. Int J Radiat Oncol Biol Phys. 2000;47:1267–71.PubMedCrossRefGoogle Scholar
  15. 15.
    Faulkner LB, Hajdu SI, Kher U, La Quaglia M, Exelby PR, Heller G, et al. Pediatric desmoid tumor: retrospective analysis of 63 cases. J Clin Oncol. 1995;13:2813–8.PubMedGoogle Scholar
  16. 16.
    Oudot C, Orbach D, Minard-Colin V, Michon J, Mary P, Glorion C, et al. Desmoid fibromatosis in pediatric patients: management based on a retrospective analysis of 59 patients and a review of the literature. Sarcoma. 2012;2012:475202 (9 pages). doi: 10.1155/2012/475202.Google Scholar
  17. 17.
    Bonvalot S, Eldweny H, Haddad V, Rimareix F, Missenard G, Oberlin O, et al. Extra-abdominal fibromatosis: aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol. 2008;34:462–8.PubMedCrossRefGoogle Scholar
  18. 18.
    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.PubMedCrossRefGoogle Scholar
  19. 19.
    de Bree E, Keus R, Melissas J, Tsiftsis D, van Coevorden F. Desmoid tumors: need for an individualized approach. Expert Rev Anticancer Ther. 2009;9:525–35.PubMedCrossRefGoogle Scholar
  20. 20.
    Raney B, Evans A, Granowetter L, Schnaufer L, Uri A, Littman P. Nonsurgical management of children with recurrent or unresectable fibromatosis. Pediatrics. 1987;79:394–8.PubMedGoogle Scholar
  21. 21.
    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.PubMedGoogle Scholar
  22. 22.
    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 desmoids tumors. Am J Pathol. 2008;173:1518–27.PubMedCrossRefGoogle Scholar
  23. 23.
    Casali PG, Blay JY. Soft tissue sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2010;21:198–203.CrossRefGoogle Scholar
  24. 24.
    Mullen JT, DeLaney TF, Kobayashi WK, Szymonifka J, Yeap BY, Chen YL, et al. Desmoid tumor: analysis of prognostic factors and outcomes in a surgical series. Ann Surg Oncol. 2012;19:4028–35.PubMedCrossRefGoogle Scholar
  25. 25.
    Peng PD, Hyder O, Mavros MN, Turley R, Groeschl R, Firoozmand A, et al. Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients. Ann Surg Oncol. 2012;19:4036–42.PubMedCrossRefGoogle Scholar
  26. 26.
    Skapek SX, Hawk BJ, Hoffer FA, Dahl GV, Granowetter L, Gebhardt MC, et al. Combination chemotherapy using vinblastine and methotrexate for the treatment of progressive desmoid tumor in children. J Clin Oncol. 1998;16:3021–7.PubMedGoogle Scholar
  27. 27.
    Bertani E, Chiappa A, Testori A, Mazzarol G, Biffi R, Martella S, 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.PubMedCrossRefGoogle Scholar
  28. 28.
    Abbas AE, Deschamps C, Cassivi SD, Nichols III FC, Allen MS, Schleck CD, et al. Chest-wall desmoid tumors: results of surgical intervention. Ann Thorac Surg. 2004;78:1219–23.PubMedCrossRefGoogle Scholar
  29. 29.
    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.PubMedCrossRefGoogle Scholar
  30. 30.
    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.PubMedCrossRefGoogle Scholar
  31. 31.
    Spear MA, Jennings LC, Mankin HJ, Spiro IJ, Springfield DS, Gebhardt MC, et al. Individualizing management of aggressive fibromatoses. Int J Radiat Oncol Biol Phys. 1998;40;637–45.PubMedCrossRefGoogle Scholar
  32. 32.
    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.PubMedGoogle Scholar
  33. 33.
    Ballo MT, Zagars GK, Pollack A. Radiation therapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys. 1998;42:1007–14.PubMedCrossRefGoogle Scholar
  34. 34.
    Kamath SS, Parsons JT, Marcus RB, Zlotecki RA, Scarborough MT. Radiotherapy for local control of aggressive fibromatosis. Int J Radiat Oncol Biol Phys. 1996;36:325–8.PubMedCrossRefGoogle Scholar
  35. 35.
    Skapek SX, Ferguson WS, Granowetter L, Devidas M, Perez-Atayde AR, Dehner LP, et al. Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial. J Clin Oncol. 2007;25:501–6.PubMedCrossRefGoogle Scholar
  36. 36.
    Skapek SX, Anderson JR, Hill DA, Henry D, Spunt SL, Meyer W, et al. Safety and efficacy of high-dose tamoxifen and sulindac for desmoid tumor in children: results of a Children’s Oncology Group (COG) Phase II Study. Pediatr Blood Cancer. 2013;60:1108–12.PubMedCrossRefGoogle Scholar
  37. 37.
    Gounder MM, Lefkowitz RA, Keohan ML, D’Adamo DR, Hameed M, Antonescu CR, et al. Activity of sorafenib against desmoid tumor/deep fibromatosis. Clin Cancer Res. 2011;17:4082–90.PubMedCrossRefGoogle Scholar

Copyright information

© Society of Surgical Oncology 2013

Authors and Affiliations

  • Miguel A. Soto-Miranda
    • 1
  • John A. Sandoval
    • 2
  • Bhaskar Rao
    • 2
  • Mike Neel
    • 2
  • Matt Krasin
    • 2
  • Sheri Spunt
    • 2
  • Jesse J. Jenkins
    • 2
  • Andrew M. Davidoff
    • 2
  • Jon P. Ver Halen
    • 2
  1. 1.Department of Plastic SurgeryUniversity of TennesseeMemphisUSA
  2. 2.St. Jude Children’s Research HospitalMemphisUSA

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