eMedicine Specialties > Dermatology > Malignant Neoplasms

Dermatofibrosarcoma Protuberans

Author: Chih-Shan Jason Chen, MD, PhD, Assistant Professor, Department of Dermatology, State University of New York at Stony Brook; Director, Dermatologic Surgery and Mohs Micrographic Surgery Unit, MSK Skin Cancer Center, Memorial Sloan-Kettering Cancer Center; Chief, Dermatologic Surgery, Northport Veterans Affairs Medical Center
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: Jun 12, 2007

Introduction

Background

Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon soft tissue neoplasm with intermediate-to-low grade malignancy. Although 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.

Pathophysiology

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

The cellular origin of DFSP is not clear at this time. Evidence exists that supports the cellular origin being fibroblastic, histiocytic, or neuroectodermal.

Cultured DFSP tumor cells have increased growth response to platelet-derived growth factor (PDGF) – B. Cytogenetic studies may reveal specific lesions 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-B 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 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.

Frequency

United States

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

International

The annual incidence of DFSP is reported as 3 cases per million population from a population-based cancer registry from 1982-2002 in France.2

Mortality/Morbidity

DFSP is a locally aggressive tumor with a high recurrence rate. Although metastasis of DFSP is rare (only 1-4% reported), almost all metastatic cases have been associated with local recurrence and a poor prognosis. Most of the patients with metastatic DFSP have died within 2 years.

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.

Race

DFSP has been reported in persons of all races, and no racial predilection seems to exist in the previous reports. However, a recent study conducted by Criscione and Weinstock1 found the incidence among blacks (6.5 cases per million population) was almost double the incidence among whites (3.9 cases per million population). An uncommon pigmented variant of DFSP, called the Bednar tumor, is found predominantly in black patients.

Sex

Several studies reveal an almost equal sexual distribution or a slight male predominance. In a large study3 of 902 patients with DFSP conducted by Rutgers et al, 514 (57%) patients were male and 388 (43%) patients were female.

Age

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

Clinical

History

DFSP is a very slowly growing tumor. Because of the slow progression, the diagnosis is often delayed.

  • It may start as a small asymptomatic papule, which is likely ignored.
  • The tumor may gradually enlarge into a lumpy nodule, or it may evolve into an atrophic and/or sclerotic plaque.

Physical

  • DFSP usually presents as a large indurated plaque several centimeters in diameter. It is composed of firm, irregular nodules varying in color from flesh to reddish brown.
  • Sometimes, it may present as a morphealike, atrophic, sclerotic, violaceous plaque without nodularity that may ulcerate as it slowly increases in size.
  • DFSP most commonly occurs on the trunk, followed by the proximal extremities. It rarely occurs above the neck.

Causes

Currently, the cause of 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 old burn scars and sites of vaccinations have all been reported as sites of DFSP.

More on Dermatofibrosarcoma Protuberans

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

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Further Reading

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Keywords

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, Assistant Professor, Department of Dermatology, State University of New York at Stony Brook; Director, Dermatologic Surgery and Mohs Micrographic Surgery Unit, MSK Skin Cancer Center, Memorial Sloan-Kettering Cancer Center; Chief, Dermatologic Surgery, Northport Veterans Affairs Medical Center
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 College of Mohs Micrographic Surgery and Cutaneous Oncology, American Society for Dermatologic Surgery, 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: Nothing to disclose.

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

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

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