Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma Protuberans

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Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon soft tissue neoplasm of intermediate- to low-grade malignancy. Metastasis rarely occurs. DFSP is a locally aggressive tumor with a high recurrence rate.

Although DFSP may have been reported in the literature as early as 1890, Darier and Ferrand first described it in 1924 as a distinct cutaneous disease entity called progressive and recurring dermatofibroma. Hoffman officially coined the term dermatofibrosarcoma protuberans in 1925. [1] Note the images below.

Dermatofibrosarcoma protuberans (DFSP) is a cutaneous malignancy that arises from the dermis and invades deeper tissue (eg, fat, fascia, muscle, bone).

The cellular origin of DFSP is not clear. Evidence supports the cellular origin being fibroblastic, histiocytic, or neuroectodermal. DFSP manifests partial features of each. Therefore, many authorities suggest pluripotential progenitor cells, such as undifferentiated mesenchymal cells, may be the origin of DFSP, because they have the capacity to differentiate into all 3 cell types. [2]

Cultured DFSP tumor cells have increased growth in response to platelet-derived growth factor (PDGF)–beta. Cytogenetic studies reveal specific abnormalities in DFSP tumor cells, such as reciprocal translocations of chromosomes 17 and 22, t(17;22), and supernumerary ring chromosomes composed of interspersed sequences from bands 17(17q22) and 22(22q12). These rearrangements fuse the collagen type I alpha 1 (COL1A1) and the PDGF-beta chain (PDGFB, c-sis proto-oncogene) genes. The collagen promoter drives COL1A1 and PDGFB fusion protein production. The fusion protein is then processed into functional PDGF-B and subsequently interacts with the PDGF receptor on the cell surface of DFSP tumor cells. The activation of the PDGF receptor tyrosine kinase triggers the proliferation of DFSP tumor cells. [2, 3, 4, 5, 6, 7, 8]

The cause of dermatofibrosarcoma protuberans (DFSP) is unknown. Laboratory studies have shown that chromosomal aberrations may contribute to the pathogenesis of DFSP; however, no evidence of hereditary or familial predisposition exists. In 10-20% of patients with this tumor, trauma at the site seems to be incriminated. Surgical and burn scars and sites of vaccinations have been reported as sites of DFSP. [9]

United States

Dermatofibrosarcoma protuberans (DFSP) accounts for less than 0.1% of all malignant neoplasms and approximately 1% of all soft tissue sarcomas. DFSP is the most common type of cutaneous sarcoma. The incidence of DFSP has been estimated to be 0.8-5 case per million population per year in 2 separate studies. [2] In a another study based on data from 9 cancer registries from 1973-2002, the annual incidence of DFSP in the United States is 4.2 cases per million population per year. [8]


The annual incidence of DFSP is reported as 3 cases per million population from a population-based cancer registry from 1982-2002 in France. [10] A study of the population-based National Cancer Registry shows the incidence of DFSP is approximately 4 cases per million per year in Sweden from 1990-2005. [11]

Dermatofibrosarcoma protuberans (DFSP) has been reported in persons of all races, and no racial predilection seems to exist in previous reports. However, a study conducted by Criscione and Weinstock found the incidence among African Americans (6.5 cases per million population) was almost double the incidence among American whites (3.9 cases per million population).

An uncommon pigmented variant of DFSP, accounting for 1% of all DFSP cases, is called the Bednar tumor. Annual incidence of Bednar tumor among blacks is 7.5 times higher than that of white patients. [8] Note the images below.

Several studies of dermatofibrosarcoma protuberans (DFSP) reveal an almost equal sexual distribution or a slight male predominance. In a large study of 902 patients with DFSP conducted by Rutgers et al, 514 (57%) patients were male and 388 (43%) patients were female. [12] A study based on 405 DFSP cases from the Swedish National Cancer Registry between 1990 and 2005 shows a very small difference in annual incidence of male (4.4 cases per million) versus female (4.0 cases per million). [11] However, a larger cancer registry study of 2885 cases reveals females might have a slightly higher incidence of DFSP, 4.4 cases versus 4.2 cases per million population per year. [8]

Dermatofibrosarcoma protuberans (DFSP) usually occurs in adults aged 20-50 years. Rarely, DFSP has been reported in newborns and elderly individuals (80 y). [13]

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 the classic form of DFSP, the risk is assumed to be only 0.5%. According to the literature, the overall risk for the 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. [8] The lungs are the most common site of distant metastasis that occurs via hematogenous spread. Usually, metastatic disease is preceded by multiple local recurrences. [14]

A small subset of DFSP patients presents with fibrosarcomatous progression. This fibrosarcomatous progression DFSP variant is more aggressive in nature, and the clinical outcome usually is poor. [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, 16, 17]

Age older than 50 years is also a risk factor associated with a poor clinical outcome. [18]

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

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

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

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. 1998 Nov 27. 133(2):129-34. [Medline].

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. 1993 Oct 15. 196(1):409-415. [Medline].

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

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. 1995 Dec. 147(6):1553-8. [Medline]. [Full Text].

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. 1999 Aug 1. 59(15):3719-23. [Medline].

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. 1997 Jan. 15(1):95-8. [Medline].

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

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. 2006 Nov. 20(10):1237-42. [Medline].

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. 2010 May. 130(5):1323-8. [Medline].

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

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. 1996 Jul. 35(1):82-7. [Medline].

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

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

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

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. 1999 Sep. 49(9):799-806. [Medline].

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

Kumar D, Vallacha A. Dermatofibrosarcoma Protuberans of Breast. J Coll Physicians Surg Pak. 2018 Aug. 28 (8):645-647. [Medline].

Bernard J, Poulalhon N, Argenziano G, Debarbieux S, Dalle S, Thomas L. Dermoscopy of dermatofibrosarcoma protuberans: a study of 15 cases. Br J Dermatol. 2013 Jul. 169 (1):85-90. [Medline].

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

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

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

Basu S, Goliwale F. Aggressive Clinical Course of Dermatofibrosarcoma protuberans: 18F-FDG PET-CT predictive of Tumor Biology. J Nucl Med Technol. 2015 Sep 3. [Medline].

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

Hoesly PM, Lowe GC, Lohse CM, Brewer JD, Lehman JS. Prognostic impact of fibrosarcomatous transformation in dermatofibrosarcoma protuberans: a cohort study. J Am Acad Dermatol. 2015 Mar. 72 (3):419-25. [Medline].

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. 2009 May. 145(5):589-94. [Medline].

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

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. 1994 Aug. 21(4):324-9. [Medline].

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

Bonadies A, Elia F, Solivetti FM, Vidiri A, Muscardin L, Bucher S. Electrochemotherapy of a Multirecurrent Dermatofibrosarcoma Protuberans of the Orbital Margin: A Case Report. Anticancer Res. 2015 Nov. 35 (11):6121-6. [Medline].

Stivala A, Lombardo GA, Pompili G, Tarico MS, Fraggetta F, Perrotta RE. Dermatofibrosarcoma protuberans: Our experience of 59 cases. Oncol Lett. 2012 Nov. 4(5):1047-1055. [Medline]. [Full Text].

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

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. 2010 Jan. 36(1):89-94. [Medline].

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. 2005 Feb 1. 23(4):866-73. [Medline].

Rastogi S, Dhamija E, Barwad A, Aggarwal A, Sharma A, Panday R. Advanced Dermatofibrosarcoma Protuberans Treatment With Imatinib: Experience From a Dedicated Sarcoma Medical Oncology Clinic in India. J Glob Oncol. 2018 Jul. 1-7. [Medline].

Chen YT, Chen WT, Huang WT, Wu CC, Chai CY. Expression of MMP-2, MMP-9 and MMP-11 in dermatofibroma and dermatofibrosarcoma protuberans. Kaohsiung J Med Sci. 2012 Oct. 28(10):545-9. [Medline].

Stacchiotti S, Pantaleo MA, Negri T, Astolfi A, Tazzari M, Dagrada GP, et al. Efficacy and biological activity of imatinib in metastatic dermatifibrosarcoma protuberans (DFSP). Clin Cancer Res. 2015 Aug 10. [Medline].

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. 2010 Jun 15. 16(12):3288-95. [Medline].

Tan YG, Chia CS, Loh WL, Teo MC. Single-institution review of managing dermatofibrosarcoma protuberans. ANZ J Surg. 2015 Sep 2. [Medline].

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. 2010 Jun. 33(3):300-3. [Medline].

Foroozan M, Sei JF, Amini M, Beauchet A, Saiag P. Efficacy of Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans: systematic review. Arch Dermatol. 2012 Sep 1. 148(9):1055-63. [Medline].

Serra-Guillén C, Llombart B, Sanmartín O. Dermatofibrosarcoma Protuberans. Actas Dermosifiliogr. 2012 Oct 25. [Medline].

Lyu A, Wang Q. Dermatofibrosarcoma protuberans: A clinical analysis. Oncol Lett. 2018 Aug. 16 (2):1855-1862. [Medline].

Lee SH, Oh Y, Nam KA, Oh B, Roh, Chung KY. Mohs micrographic surgery for dermatofibrosarcoma protuberans: Comparison of frozen and paraffin techniques. J Eur Acad Dermatol Venereol. 2018 Aug 1. [Medline].

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

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

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

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

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. 1997 Oct. 37(4):600-13. [Medline].

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

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

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. 2008 Jun. 34(6):680-6. [Medline].

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. 2007 Jul. 14(7):2113-20. [Medline].

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. 2004 Mar 1. 100(5):1008-16. [Medline].

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

Raman K Madan, MD Resident Physician, Department of Dermatology, State University of New York Downstate Medical Center

Disclosure: Nothing to disclose.

Georgina Marie Ferzli, MD, MS Resident Physician, Department of Dermatology, State University of New York Downstate Medical Center

Disclosure: Nothing to disclose.

Stephanie M Gallitano, MD Resident Physician, Department of Dermatology, State University of New York Downstate Medical Center

Stephanie M Gallitano, MD is a member of the following medical societies: American Academy of Dermatology, Women’s Dermatologic Society

Disclosure: Nothing to disclose.

Chih-Shan Jason Chen, MD, PhD Attending Physician, 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; Associate Professor of Clinical Dermatology, Stony Brook University School of Medicine

Chih-Shan Jason Chen, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery, Association of Professors of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Daniel Mark Siegel, MD, MS Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate Medical Center

Daniel Mark Siegel, MD, MS is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery, American Association for Physician Leadership, American Society for Dermatologic Surgery, American Society for MOHS Surgery, International Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

John G Albertini, MD Private Practice, The Skin Surgery Center; Clinical Associate Professor (Volunteer), Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine; President-Elect, American College of Mohs Surgery

John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: QualDerm Partners; Novascan<br/>Have a 5% or greater equity interest in: QualDerm Partners – North Carolina.

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD.

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

Disclosure: Nothing to disclose.

Dermatofibrosarcoma Protuberans

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