Dermatofibrosarcoma Protuberans Treatment & Management

  • Author: from Memorial Sloan-Kettering - Chih-Shan Jason Chen, MD, PhD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 23, 2010
 

Medical Care

Currently, conventional chemotherapy is rarely used in the treatment of dermatofibrosarcoma protuberans (DFSP). Limited case reports have not shown any significant value of conventional chemotherapy in the treatment of DFSP.[26]

Radiation therapy (RT) has had a limited role in the past, but, recently, it has been used as an adjunct to surgery. Radiation therapy may be recommended for patients if the margins of resection are positive or for situations in which adequate wide excision alone may result in major cosmetic or functional deficits. Postoperative adjuvant RT may reduce the risk of recurrence when clear surgical margins are not confident. The complete radiation therapy dose ranges from 50-70 Gy. Overall, the risk of severe complications from RT is low. Close follow-up care after radiation therapy is warranted because some DFSP tumors may become more aggressive.[1, 16, 27, 28]

Based on the knowledge that constitutively activated PDGFB-PDGFR-beta signaling pathway plays a central role in the proliferation of DFSP tumor cells, the development of molecularly targeted therapy holds promise as an additional treatment option.[29] Originally approved for the treatment of chronic myelogenic leukemia, imatinib mesylate has been found to have significant therapeutic value in the treatment of DFSP. Imatinib is a potent and specific inhibitor of several protein-tyrosine kinases, including the platelet-derived growth factor (PDGF) receptors.[5]

On October 19, 2006, the US Food and Drug Administration granted approval for imatinib mesylate (Gleevec) as a single agent for the treatment of DFSP. Imatinib mesylate is indicated for the treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP. The recommended oral dose is 800 mg/d.[20]

With limited clinical data to date, a response rate of approximately 65% has been achieved among DFSP patients treated with imatinib. A small subset of DFSP patients lacking the classic t(17,22) gene aberration seems to have no response to imatinib.[1]

Neoadjuvant imatinib therapy for DFSP has been proposed in recent studies.[21, 30] Using imatinib as a preoperative therapy agent in locally advanced or recurrent DFSP may decrease tumor load, promote tumor cell apoptosis, and subsequently reduce the extent of surgery. Caution should be used when applying such a therapeutic strategy, because the potential exists for creating a skip area wherein discontiguous tumor may obscure the accurate pathology assessment of surgical margins.

For a partial listing of active and recruiting clinical trials, see Further Reading.

Next

Surgical Care

Surgical excision remains the mainstay of treatment for dermatofibrosarcoma protuberans (DFSP). Despite controversy, Mohs micrographic surgery has been increasingly accepted as the treatment of choice, while others advocate wide local excision.[14, 28, 31] The fundamental difference of these 2 techniques is the pathology processing. Usually, the specimen from wide excision is sectioned in conventional bread-loaf fashion, while the Mohs specimen is freshly frozen and sectioned en face along the margins. Mohs surgery requires less tissue removal and allows complete margin assessment. However, large tumor can be a challenge for this very time-consuming procedure.

Because of its infiltrating growth pattern, DFSP commonly extends far beyond the clinical margins; this accounts in part for the high recurrence rate after standard surgical excision. Hence, a wide excision of 2-3 cm or more of the margins beyond clinically identifiable tumor border, down to and including the fascia, is recommended for the treatment of DFSP.[14, 31] Despite wide local excisions, an average recurrence rate of 15.7% is still observed among 1201 body cases and 51.8% is observed among 193 head and neck cases, as reported in the literature since 1951. A superior cure rate (an overall average recurrence rate of 1.3% among 463 cases reported) and tissue conservation are seen when Mohs micrographic surgery is used; thus, Mohs micrographic surgery is now considered the treatment of choice, particularly when a lesion is located in the head and neck region.[22, 31, 32, 33, 34, 35, 36, 37]

Although some Mohs surgeons consider it unnecessary, taking an extra layer of tissue around the surgical defect at the completion of Mohs surgery for permanent pathology section and/or CD34 immunostaining may potentially enhance the cure rate. Alternatively, some have adopted modified Mohs techniques, or so-called "slow Mohs," by using rush paraffin sections instead of a fresh tissue technique.[32, 35, 38, 39, 40] Mohs surgery may not be readily accessible in many parts of the world. The physician should exercise clinical judgment to offer the best treatment available for the patient and consider multidisciplinary collaboration. Studies have demonstrated a low recurrence rate after surgery for DFSP if a multidisciplinary approach and careful pathology margin assessment are used.[41, 42]

Previous
Next

Consultations

In dermatofibrosarcoma protuberans (DFSP), an informative consultation includes proper staging, prognostic evaluation, explanation of treatment options, and planning. All these depend on thorough history taking and physical examination. Imaging studies may facilitate the assessment of local invasion and distal metastasis. Multidisciplinary collaboration between a dermatologist, surgical oncologist, plastic surgeon, medical oncologist, radiation oncologist, and pathologist is necessary in locally advanced, recurrent, or metastatic cases of DFSP.

Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

from Memorial Sloan-Kettering - Chih-Shan Jason Chen, MD, PhD  Associate Attending, Dermatology Service, Memorial Sloan-Kettering Cancer Center; Director, Dermatologic Surgery and Mohs Micrographic Surgery Unit, MSK Skin Cancer Center; Chief, Dermatologic Surgery, Northport Veterans Affairs Medical Center

from Memorial Sloan-Kettering - 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 College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physician Executives, American Society for Dermatologic Surgery, American Society for MOHS Surgery, and International Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Abdul-Ghani Kibbi, MD  Professor and Chair, Department of Dermatology, American University of Beirut Medical Center, Lebanon

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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.

John G Albertini, MD  Consulting Staff, Dermatologic Surgery, The Skin Surgery Center; Program Director, ACGME Accredited Fellowship in Procedural Dermatology

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.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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.

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]. [Full Text].

  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. 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].

  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. Hussain SK, Sundquist J, Hemminki K. Incidence trends of squamous cell and rare skin cancers in the Swedish national cancer registry point to calendar year and age-dependent increases. J Invest Dermatol. May 2010;130(5):1323-8. [Medline].

  12. 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].

  13. 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].

  14. 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].

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

  16. 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].

  17. 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].

  18. 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].

  19. Riggs K, McGuigan KL, Morrison WB, Samie FH, Humphreys T. Role of magnetic resonance imaging in perioperative assessment of dermatofibrosarcoma protuberans. Dermatol Surg. Dec 2009;35(12):2036-41. [Medline].

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

  21. Han A, Chen EH, Niedt G, Sherman W, Ratner D. Neoadjuvant imatinib therapy for dermatofibrosarcoma protuberans. Arch Dermatol. Jul 2009;145(7):792-6. [Medline].

  22. 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].

  23. 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].

  24. 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].

  25. 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].

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

  27. 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].

  28. Heuvel ST, Suurmeijer A, Pras E, Van Ginkel RJ, Hoekstra HJ. Dermatofibrosarcoma protuberans: recurrence is related to the adequacy of surgical margins. Eur J Surg Oncol. Jan 2010;36(1):89-94. [Medline].

  29. 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].

  30. Kerob D, Porcher R, Verola O, et al. Imatinib mesylate as a preoperative therapy in dermatofibrosarcoma: results of a multicenter phase II study on 25 patients. Clin Cancer Res. Jun 15 2010;16(12):3288-95. [Medline].

  31. Meguerditchian AN, Wang J, Lema B, Kraybill WG, Zeitouni NC, Kane JM 3rd. Wide excision or Mohs micrographic surgery for the treatment of primary dermatofibrosarcoma protuberans. Am J Clin Oncol. Jun 2010;33(3):300-3. [Medline].

  32. 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].

  33. 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].

  34. 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].

  35. 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].

  36. 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].

  37. 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].

  38. 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].

  39. 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].

  40. 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].

  41. DuBay D, Cimmino V, Lowe L, Johnson TM, Sondak VK. Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution. Cancer. Mar 1 2004;100(5):1008-16. [Medline].

  42. Farma JM, Ammori JB, Zager JS, et al. Dermatofibrosarcoma protuberans: how wide should we resect?. Ann Surg Oncol. Aug 2010;17(8):2112-8. [Medline].

  43. 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].

 
Previous
Next
 
Dermatofibrosarcoma protuberans manifesting as an irregular red-to-violaceous plaque on the chest.
Closer view of dermatofibrosarcoma protuberans. It has an irregular surface and borders with palpable dermal and subcutaneous induration.
A large dermatofibrosarcoma protuberans is seen on the abdomen. It has an appearance of atrophic plaque, while foci of nodularity also can be seen.
Bednar tumor, a pigmented variant of dermatofibrosarcoma protuberans, contains melanin-rich dendritic cells scattered among neoplastic spindle-shaped cells.
A featureless reddish brown plaque on the arm of a white female. Another example of Bednar tumor.
Dermatofibrosarcoma protuberans (DFSP) tumor cells take over the dermis and subcutaneous adipose tissue and then approach the fascia plane. The tumor nodule manifests with high cellularity. Under histopathologic examination, these DFSP tumor cells are spindle shaped. They tend to grow in a storiform pattern in the center portion of the tumor. They may also grow in a diffuse infiltrative pattern at the periphery, forming a honeycomb pattern. Often, no defined border can be recognized between the tumor and normal tissue.
Higher-power view of dermatofibrosarcoma protuberans reveals subcutaneous adipocytes entrapped by densely infiltrative spindle-shaped tumor cells.
DFSP demonstrates strong CD34 staining with immunohistochemistry.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.