Journal of the American Academy of Dermatology
Volume 61, Issue 6 , Pages 1014-1023 , December 2009

Surgical management of congenital dermatofibrosarcoma protuberans

  • W. Elliot Love, DO

      Affiliations

    • Department of Dermatology, University Hospitals Case Medical Center, Cleveland, Ohio
    • Case Western Reserve University School of Medicine, Cleveland, Ohio
  • ,
  • Susan A. Keiler, MD

      Affiliations

    • Department of Dermatology, University Hospitals Case Medical Center, Cleveland, Ohio
    • Case Western Reserve University School of Medicine, Cleveland, Ohio
  • ,
  • Joan E. Tamburro, DO

      Affiliations

    • Department of Dermatology, University Hospitals Case Medical Center, Cleveland, Ohio
    • Case Western Reserve University School of Medicine, Cleveland, Ohio
  • ,
  • Kord Honda, MD

      Affiliations

    • Department of Dermatology, University Hospitals Case Medical Center, Cleveland, Ohio
    • Case Western Reserve University School of Medicine, Cleveland, Ohio
  • ,
  • Arun K. Gosain, MD

      Affiliations

    • Department of Plastic Surgery, Rainbow Babies and Children's Hospital, Cleveland, Ohio
    • Case Western Reserve University School of Medicine, Cleveland, Ohio
  • ,
  • Jeremy S. Bordeaux, MD, MPH

      Affiliations

    • Department of Dermatology, University Hospitals Case Medical Center, Cleveland, Ohio
    • Case Western Reserve University School of Medicine, Cleveland, Ohio
    • Corresponding Author InformationReprint requests: Jeremy S. Bordeaux, MD, MPH, Department of Dermatology, University Hospitals Case Medical Center, 11100 Euclid Ave, Lakeside 3500, Cleveland, OH 44124.

References 

  1. Gloster HM. Dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;35:355–374
  2. Price VE, Fletcher JA, Zielenska M, Cole W, Viero S, Manson DE, et al. Imatinib mesylate: an attractive alternative in young children with large, surgically challenging dermatofibrosarcoma protuberans. Pediatr Blood Cancer. 2005;44:511–515
  3. Snow SN, Gordon EM, Larson PO, Bagheri MM, Bentz ML, Sable DB. Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature. Cancer. 2004;101:28–38
  4. Gloster HM, Harris KR, Roenigk RK. A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;35:82–87
  5. Thomas CJ, Wood GC, Marks VJ. Mohs micrographic surgery in the treatment of rare aggressive cutaneous tumors: the Geisinger experience. Dermatol Surg. 2007;33:333–339
  6. Nouri K, Lodha R, Jimenez G, Robins P. Mohs micrographic surgery for dermatofibrosarcoma protuberans: University of Miami and NYU experience. Dermatol Surg. 2002;28:1060–1064
  7. Ratner D, Thomas CO, Johnson TM, Sondak VK, Hamilton TA, Nelson BR, et al. Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans: results of a multiinstitutional series with an analysis of the extent of microscopic spread. J Am Acad Dermatol. 1997;37:600–613
  8. Borrie P. Dermatofibrosarcoma protuberans. Br J Surg. 1952;39:452–453
  9. Topar G, Hönlinger B, Eisendle K, Zelger B. An unusual congenital lesion in a 26-year-old woman. Clin Exp Dermatol. 2008;33:373–374
  10. Malerich P, Pride H, Marks V, Tyler W. Congenital dermatofibrosarcoma protuberans treated with Mohs micrographic surgery. J Am Acad Dermatol. 2007;56:AB157
  11. Lee MS, Kang MJ, Kim MY, Kim HO, Song KY, Park YM. Congenital Bednar tumor (pigmented dermatofibrosarcoma protuberans). J Eur Acad Dermatol Venereol. 2008;22:509–511
  12. Muniesa C, Curcó N, Pagerols X, García-Font M, Tarroch X, Vives P. Congenital dermatofibrosarcoma protuberans: a case report. Actas Dermo-Sif. 2007;98:617–620
  13. von Stebut E, Bräuninger W. Congenital dermatofibrosarcoma protuberans: 30 years of follow-up. J Dtsch Dermatol Ges. 2007;5:223–225
  14. Maire G, Fraitag S, Galmiche L, Keslair F, Ebran N, Terrier-Lacombe MJ, et al. A clinical, histologic, and molecular study of 9 cases of congenital dermatofibrosarcoma protuberans. Arch Dermatol. 2007;143:203–210
  15. Reddy C, Hayward P, Thompson P, Kan A. Dermatofibrosarcoma protuberans in children. J Plast Reconstr Aesthet Surg. 2009;62:819–823
  16. Fidalgo A, Feio AB, Bajanca R. Congenital dermatofibrosarcoma protuberans. J Eur Acad Dermatol Venereol. 2006;20:879–881
  17. Gu W, Ogose A, Kawashima H, Umezu H, Kudo N, Hotta T, et al. Congenital dermatofibrosarcoma protuberans with fibrosarcomatous and myxoid change. J Clin Pathol. 2005;58:984–986
  18. Thornton SL, Reid J, Papay FA, Vidimos AT. Childhood dermatofibrosarcoma protuberans: role of preoperative imaging. J Am Acad Dermatol. 2005;53:76–83
  19. Weinstein JM, Drolet BA, Esterly NB, Rogers M, Bauer BS, Wagner AM, et al. Congenital dermatofibrosarcoma protuberans: variability in presentation. Arch Dermatol. 2003;139:207–211
  20. Terrier-Lacombe MJ, Guillou L, Maire G, Terrier P, Vince DR, de Saint Aubain Somerhausen N, et al. Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data–a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol. 2003;27:27–39
  21. Marini M, Saponaro A, Magariños G, de Baldrich A, Lynch P, Remorino L. Congenital atrophic dermatofibrosarcoma protuberans. Int J Dermatol. 2001;40:448–450
  22. Checketts SR, Hamilton TK, Baughman RD. Congenital and childhood dermatofibrosarcoma protuberans: a case report and review of the literature. J Am Acad Dermatol. 2000;42:907–913
  23. Maeda T, Hirose T, Furuya K, Shirakawa K, Kobayashi K. Giant cell fibroblastoma associated with dermatofibrosarcoma protuberans: a case report. Mod Pathol. 1998;11:491–495
  24. Marcus JR, Few JW, Senger C, Reynolds M. Dermatofibrosarcoma protuberans and the Bednar tumor: treatment in the pediatric population. J Pediatr Surg. 1998;33:1811–1814
  25. Martin L, Combemale P, Dupin M, Chouvet B, Kanitakis J, Bouyssou-Gauthier ML, et al. The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases. Br J Dermatol. 1998;39:719–725
  26. Garcia C, Clark RE, Buchanan M. Dermatofibrosarcoma protuberans. Int J Dermatol. 1996;35:867–871
  27. Kahn TA, Liranzo MO, Vidimos AT, Papay FA, Bergfeld WF. Pathological case of the month: congenital dermatofibrosarcoma protuberans. Arch Pediatr Adolesc Med. 1996;150:549–550
  28. Jimenez FJ, Grichnik JM, Buchanan MD, Clark RE. Immunohistochemical margin control applied to Mohs micrographic surgical excision of dermatofibrosarcoma protuberans. J Dermatol Surg Oncol. 1994;20:687–689
  29. Annessi G, Cimitan A, Girolomoni G, Giannetti A. Congenital dermatofibrosarcoma protuberans. Pediatr Dermatol. 1993;10:40–42
  30. Gorczyca W, Woyke S, Uciński M. Storiform neurofibroma (Bednar tumor): a case report. Patol Pol. 1991;42:126–127
  31. McKee PH, Fletcher CD. Dermatofibrosarcoma protuberans presenting in infancy and childhood. J Cutan Pathol. 1991;18:241–246
  32. Weber PJ, Gretzula JC, Hevia O, Garland LB, Barquin P, Gould E. Dermatofibrosarcoma protuberans. J Dermatol Surg Oncol. 1988;14:555–558
  33. Chapman JE, Lynch RC, Weaver EN. Dermatofibrosarcoma protuberans: a case report. J La State Med Soc. 1977;129:235–238
  34. Schvarcz LW. Congenital dermatofibrosarcoma protuberans of the hand. Hand. 1977;9:182–186
  35. Burkhardt BR, Soule EH, Winkelmann RK, Ivins JC. Dermatofibrosarcoma protuberans: study of fifty-six cases. Am J Surg. 1966;111:638–644
  36. Taylor HB, Helwig EB. Dermatofibrosarcoma protuberans: a study of 115 cases. Cancer. 1962;15:717–725
  37. Pack GT, Tabah EJ. Dermatofibrosarcoma protuberans: a report of thirty-nine cases. Arch Surg. 1951;62:391–411
  38. Gerlini G, Mariotti G, Urso C, Brandani P, Reali UM, Borgognoni L. Dermatofibrosarcoma protuberans in childhood: two case reports and review of the literature. Pediatr Hematol Oncol. 2008;25:559–566
  39. Jafarian F, McCuaig C, Kokta V, Laberge L, Ben Nejma B. Dermatofibrosarcoma protuberans in childhood and adolescence: report of eight patients. Pediatr Dermatol. 2008;25:317–325
  40. Feramisco J, Larsen F, Weitzul S, Cockerell C, Ghali F. Congenital atrophic dermatofibrosarcoma protuberans in a 7-month-old boy treated with Mohs micrographic surgery. Pediatr Dermatol. 2008;25:455–459
  41. Mentzel T, Beham A, Katenkamp D. Dei Tos AP, Fletcher CD. Fibrosarcomatous (“high-grade”) dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance. Am J Surg Pathol. 1998;22:576–587
  42. Brenner W, Schaefler K, Chhabra H, Postel A. Dermatofibrosarcoma protuberans metastatic to a regional lymph node: report of a case and review. Cancer. 1975;36:1897–1902
  43. McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans: an analysis of 86 cases e five with metastasis. Ann Surg. 1967;166:803–816
  44. Maki RG, Awan RA, Dixon RH, Jhanwar S, Antonescu CR. Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans. Int J Cancer. 2002;100:623–626
  45. National Cancer Institute. Available from: URL: http://www.cancer.gov/search/ResultsClinicalTrials. Accessed April 21, 2009.
  46. Parker TL, Zitelli JA. Surgical margins for excision of dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1995;32:233–236
  47. Wright TI, Petersen JE. Treatment of recurrent dermatofibrosarcoma protuberans with imatinib mesylate, followed by Mohs micrographic surgery. Dermatol Surg. 2007;33:741–744
  48. Mikhail GR, Lynn BH. Dermatofibrosarcoma protuberans. J Dermatol Surg Oncol. 1978;4:81–84

 Funding sources: Dr Bordeaux is supported by the Dermatology Foundation Clinical Career Development Award in Dermatologic Surgery.

 Conflicts of interest: None declared.

PII: S0190-9622(09)00672-0

doi: 10.1016/j.jaad.2009.05.028

Journal of the American Academy of Dermatology
Volume 61, Issue 6 , Pages 1014-1023 , December 2009