eMedicine Specialties > Dermatology > Malignant Neoplasms

Dermatofibrosarcoma Protuberans: Follow-up

Author: Chih-Shan Jason Chen, MD, PhD, Associate Attending, Dermatology Service, Memorial Sloan-Kettering Cancer Center, New York; Director, Dermatologic Surgery and Mohs Micrographic Surgery Unit, MSK Skin Cancer Center at Hauppauge, Long Island; Chief, Dermatologic Surgery, Northport Veterans Affairs Medical Center, Northport, Long Island
Coauthor(s): Daniel Mark Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate
Contributor Information and Disclosures

Updated: Aug 17, 2009

Follow-up

Further Outpatient Care

Because of the high local recurrence rate of dermatofibrosarcoma protuberans (DFSP), patients require close follow-up care after treatment.  

  • Most recurrences occur within 3 years of the primary excision. Patients should be seen every 6 months during this period and annually thereafter.1,15
  • A literature review of DFSP case series treated with Mohs surgery shows that 50% of recurrences appear within the first 3 years after operation and 25% of local recurrences are detected after 5 years. A large case review from a series of 159 patients treated at Memorial Sloan-Kettering Cancer Center (New York) showed the medium time to the development of a local recurrence was 32 months. The indolent nature of DFSP requires lifelong surveillance for recurrence.19
  • In each follow-up visit, a complete history and a review of systems, as well as complete physical examinations, including skin examination and palpation of the excision site and regional lymph nodes, should be performed. An extensive workup is not warranted unless metastatic disease is suspected.

Prognosis

Dermatofibrosarcoma protuberans (DFSP) is characterized by its aggressive local invasion. The tumor invades local tissue by extending tentaclelike projections underneath healthy skin, rendering complete removal of the tumor very difficult. Incomplete removal of these neoplastic cells results in a high local recurrence rate.

Despite the local invasiveness, DFSP rarely metastasizes. For classic form of DFSP, the risk is assumed to be only 0.5%. According to the literature, the overall risk for development of metastatic disease is 5%, including 1% with regional lymph node metastasis and 4% with distant metastasis. Regional lymph node involvement represents a sign of poor prognosis; most patients die within 2 years. The lungs are the most common site of distant metastasis that occurs via hematogenous spread. Usually, metastatic disease is preceded by multiple local recurrences.15

The extent of surgical excision determines the prognosis for the patient. To reduce the local recurrence rate, a wide surgical excision with adequate margins or Mohs technique are used. The latter imparts a better outcome.

Histologic features of DFSP may also serve as prognostic indicators. A high number of mitotic figures, increased cellularity, DNA aneuploidy, TP53 gene overexpression, and the presence of fibrosarcomatous changes within the tumor are poor prognostic indicators. Of note, fibrosarcomatous variants of DFSP lacking a genetic marker of translocation between chromosomes 17 and 22 may not respond to imatinib. The loss of the t(17,22) cytogenetic marker in the fibrosarcomatous progression DFSP variant may represent progression of the malignancy.1,18,33

Age older than 50 years is also a risk factor associated with a poor clinical outcome.19

Patient Education

Patients are advised to seek for dermatologic evaluation if they have noticed a slow-growing skin lump or scarlike lesion on any part of their body. 

Miscellaneous

Medicolegal Pitfalls

Because of the rarity of dermatofibrosarcoma protuberans (DFSP), misdiagnosis and delayed diagnosis often occur. When encountering an atypical scarlike lesion, the clinician should consider skin biopsy.

 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.



More on Dermatofibrosarcoma Protuberans

Overview: Dermatofibrosarcoma Protuberans
Differential Diagnoses & Workup: Dermatofibrosarcoma Protuberans
Treatment & Medication: Dermatofibrosarcoma Protuberans
Follow-up: Dermatofibrosarcoma Protuberans
Multimedia: Dermatofibrosarcoma Protuberans
References
Further Reading
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References

  1. Lemm D, Mugge LO, Mentzel T, Hoffken K. Current treatment options in dermatofibrosarcoma protuberans. J Cancer Res Clin Oncol. May 2009;135(5):653-65. [Medline].

  2. Dimitropoulos VA. Dermatofibrosarcoma protuberans. Dermatol Ther. Nov-Dec 2008;21(6):428-32. [Medline].

  3. Gisselsson D, Hoglund M, O'Brien KP, Dumanski JP, Mertens F, Mandahl N. A case of dermatofibrosarcoma protuberans with a ring chromosome 5 and a rearranged chromosome 22 containing amplified COL1A1 and PDGFB sequences. Cancer Lett. Nov 27 1998;133(2):129-34. [Medline].

  4. Kikuchi K, Soma Y, Fujimoto M, et al. Dermatofibrosarcoma protuberans: increased growth response to platelet-derived growth factor BB in cell culture. Biochem Biophys Res Commun. Oct 15 1993;196(1):409-415. [Medline].

  5. McArthur G. Molecularly targeted treatment for dermatofibrosarcoma protuberans. Semin Oncol. Apr 2004;31(2 Suppl 6):30-6. [Medline].

  6. Naeem R, Lux ML, Huang SF, Naber SP, Corson JM, Fletcher JA. Ring chromosomes in dermatofibrosarcoma protuberans are composed of interspersed sequences from chromosomes 17 and 22. Am J Pathol. Dec 1995;147(6):1553-8. [Medline].

  7. Shimizu A, O'Brien KP, Sjoblom T, et al. The dermatofibrosarcoma protuberans-associated collagen type Ialpha1/platelet-derived growth factor (PDGF) B-chain fusion gene generates a transforming protein that is processed to functional PDGF-BB. Cancer Res. Aug 1 1999;59(15):3719-23. [Medline].

  8. Simon MP, Pedeutour F, Sirvent N, et al. Deregulation of the platelet-derived growth factor B-chain gene via fusion with collagen gene COL1A1 in dermatofibrosarcoma protuberans and giant-cell fibroblastoma. Nat Genet. Jan 1997;15(1):95-8. [Medline].

  9. Criscione VD, Weinstock MA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol. Jun 2007;56(6):968-73. [Medline].

  10. Monnier D, Vidal C, Martin L, et al. Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002. J Eur Acad Dermatol Venereol. Nov 2006;20(10):1237-42. [Medline].

  11. Abbott JJ, Oliveira AM, Nascimento AG. The prognostic significance of fibrosarcomatous transformation in dermatofibrosarcoma protuberans. Am J Surg Pathol. Apr 2006;30(4):436-43. [Medline].

  12. Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E. Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol. Jun 1992;18(3):241-8. [Medline].

  13. Gloster HM Jr, Harris KR, Roenigk RK. A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol. Jul 1996;35(1):82-7. [Medline].

  14. McLelland J, Chu T. Dermatofibrosarcoma protuberans arising in a BCG vaccination scar. Arch Dermatol. Apr 1988;124(4):496-7. [Medline].

  15. Ugurel S, Kortmann RD, Mohr P, Mentzel T, Garbe C, Breuninger H. Short German guidelines: dermatofibrosarcoma protuberans. J Dtsch Dermatol Ges. May 2008;6 Suppl 1:S17-8. [Medline].

  16. Thornton SL, Reid J, Papay FA, Vidimos AT. Childhood dermatofibrosarcoma protuberans: role of preoperative imaging. J Am Acad Dermatol. Jul 2005;53(1):76-83. [Medline].

  17. Torreggiani WC, Al-Ismail K, Munk PL, Nicolaou S, O'Connell JX, Knowling MA. Dermatofibrosarcoma protuberans: MR imaging features. AJR Am J Roentgenol. Apr 2002;178(4):989-93. [Medline].

  18. McArthur G. Dermatofibrosarcoma protuberans: recent clinical progress. Ann Surg Oncol. Oct 2007;14(10):2876-86. [Medline].

  19. Bowne WB, Antonescu CR, Leung DH, et al. Dermatofibrosarcoma protuberans: A clinicopathologic analysis of patients treated and followed at a single institution. Cancer. Jun 15 2000;88(12):2711-20. [Medline].

  20. Quigley EA, Marghoob AA, Busam KJ, Chen CS. A firm red-brown plaque on the arm. Dermatofibrosarcoma protuberans(DFSP), pigmented variant (Bednar tumor. Arch Dermatol. May 2009;145(5):589-94. [Medline].

  21. Abenoza P, Lillemoe T. CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. Am J Dermatopathol. Oct 1993;15(5):429-34. [Medline].

  22. Prieto VG, Reed JA, Shea CR. CD34 immunoreactivity distinguishes between scar tissue and residual tumor in re-excisional specimens of dermatofibrosarcoma protuberans. J Cutan Pathol. Aug 1994;21(4):324-9. [Medline].

  23. Dagan R, Morris CG, Zlotecki RA, Scarborough MT, Mendenhall WM. Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol. Dec 2005;28(6):537-9. [Medline].

  24. Hancox JG, Kelley B, Greenway HT Jr. Treatment of dermatofibroma sarcoma protuberans using modified Mohs micrographic surgery: no recurrences and smaller defects. Dermatol Surg. Jun 2008;34(6):780-4. [Medline].

  25. Nelson RA, Arlette JP. Mohs micrographic surgery and dermatofibrosarcoma protuberans: a multidisciplinary approach in 44 patients. Ann Plast Surg. Jun 2008;60(6):667-72. [Medline].

  26. Nouri K, Lodha R, Jimenez G, Robins P. Mohs micrographic surgery for dermatofibrosarcoma protuberans: University of Miami and NYU experience. Dermatol Surg. Nov 2002;28(11):1060-4; discussion 1064. [Medline].

  27. Paradisi A, Abeni D, Rusciani A, et al. Dermatofibrosarcoma protuberans: wide local excision vs. Mohs micrographic surgery. Cancer Treat Rev. Dec 2008;34(8):728-36. [Medline].

  28. Ratner D, Thomas CO, Johnson TM, 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. Oct 1997;37(4):600-13. [Medline].

  29. 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. Jul 1 2004;101(1):28-38. [Medline].

  30. Cecchi R, Rapicano V. Micrographic surgery (Tübingen technique) for the treatment of dermatofibrosarcoma protuberans: a single-centre experience. Eur J Dermatol. Nov-Dec 2007;17(6):543-4. [Medline].

  31. Hafner HM, Moehrle M, Eder S, Trilling B, Rocken M, Breuninger H. 3D-Histological evaluation of surgery in dermatofibrosarcoma protuberans and malignant fibrous histiocytoma: differences in growth patterns and outcome. Eur J Surg Oncol. Jun 2008;34(6):680-6. [Medline].

  32. Kimmel Z, Ratner D, Kim JY, Wayne JD, Rademaker AW, Alam M. Peripheral excision margins for dermatofibrosarcoma protuberans: a meta-analysis of spatial data. Ann Surg Oncol. Jul 2007;14(7):2113-20. [Medline].

  33. Sasaki M, Ishida T, Horiuchi H, MacHinami R. Dermatofibrosarcoma protuberans: an analysis of proliferative activity, DNA flow cytometry and p53 overexpression with emphasis on its progression. Pathol Int. Sep 1999;49(9):799-806. [Medline].

  34. Brown MD. Recognition and management of unusual cutaneous tumors. Dermatol Clin. Jul 2000;18(3):543-52. [Medline].

  35. Dawes KW, Hanke CW. Dermatofibrosarcoma protuberans treated with Mohs micrographic surgery: cure rates and surgical margins. Dermatol Surg. Jun 1996;22(6):530-4. [Medline].

  36. Fiore M, Miceli R, Mussi C, et al. Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol. Oct 20 2005;23(30):7669-75. [Medline].

  37. Gloster HM Jr. Dermatofibrosarcoma protuberans. J Am Acad Dermatol. Sep 1996;35(3 Pt 1):355-74; quiz 375-6. [Medline].

  38. Gutierrez G, Ospina JE, de Baez NE, de Escorcia EK, Gutierrez R. Dermatofibrosarcoma protuberans. Int J Dermatol. Jul-Aug 1984;23(6):396-401. [Medline].

  39. McArthur GA, Demetri GD, van Oosterom A, et al. Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol. Feb 1 2005;23(4):866-73. [Medline].

  40. Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibrosarcoma protuberans. Cancer. Dec 1 2004;101(11):2503-8. [Medline].

  41. Peters CW, Hanke CW, Pasarell HA, Bennett JE. Chemosurgical reports. Dermatofibrosarcoma protuberans of the face. J Dermatol Surg Oncol. Oct 1982;8(10):823-6. [Medline].

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Keywords

dermatofibrosarcoma protuberans, DFSP, sarcomatous tumors resembling keloid, hypertrophic morphea, progressive and recurring dermatofibroma, fibrosarcomatous tumors with attenuated dermal surfaces, fibrosarcoma of the skin

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Contributor Information and Disclosures

Author

Chih-Shan Jason Chen, MD, PhD, Associate Attending, Dermatology Service, Memorial Sloan-Kettering Cancer Center, New York; Director, Dermatologic Surgery and Mohs Micrographic Surgery Unit, MSK Skin Cancer Center at Hauppauge, Long Island; Chief, Dermatologic Surgery, Northport Veterans Affairs Medical Center, Northport, Long Island
Chih-Shan Jason Chen, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American Society for Dermatologic Surgery, Association of Professors of Dermatology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Daniel Mark Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate
Daniel Mark Siegel, MD, MS is a member of the following medical societies: American Academy of Dermatology, American Academy of Facial Plastic and Reconstructive Surgery, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physician Executives, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, American Society for MOHS Surgery, and International Society for Dermatologic Surgery
Disclosure: Nothing to disclose.

Medical Editor

Abdul-Ghani Kibbi, MD, Chairman and Professor, Department of Dermatology, American University of Beirut Medical Center, Lebanon
Disclosure: none None None

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

John G Albertini, MD, Consulting Staff, Dermatologic Surgery, The Skin Surgery Center
John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology and American College of Mohs Micrographic Surgery and Cutaneous Oncology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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