eMedicine Specialties > Dermatology > Malignant Neoplasms

Dermatofibrosarcoma Protuberans

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

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 1925.1


Dermatofibrosarcoma protuberans manifesting as an...

Dermatofibrosarcoma protuberans manifesting as an irregular red-to-violaceous plaque on the chest.

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Dermatofibrosarcoma protuberans manifesting as an...

Dermatofibrosarcoma protuberans manifesting as an irregular red-to-violaceous plaque on the chest.



Closer view of dermatofibrosarcoma protuberans. I...

Closer view of dermatofibrosarcoma protuberans. It has an irregular surface and borders with palpable dermal and subcutaneous induration.

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Closer view of dermatofibrosarcoma protuberans. I...

Closer view of dermatofibrosarcoma protuberans. It has an irregular surface and borders with palpable dermal and subcutaneous induration.

Pathophysiology

Dermatofibrosarcoma protuberans (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. Evidence exists that 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)–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.2,3,4,5,6,7,8

Frequency

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 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 cases per million population per year.9

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.10

Mortality/Morbidity

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumor with a high recurrence rate. DFSP is usually not the direct cause of death. The relative 5-year survival rate for DFSP is 99.2%. 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.9

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.11

Race

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.9


Bednar tumor, a pigmented variant of dermatofibro...

Bednar tumor, a pigmented variant of dermatofibrosarcoma protuberans, contains melanin-rich dendritic cells scattered among neoplastic spindle-shaped cells.

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Bednar tumor, a pigmented variant of dermatofibro...

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 featureless reddish brown plaque on the arm of a white female. Another example of Bednar tumor.

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A featureless reddish brown plaque on the arm of ...

A featureless reddish brown plaque on the arm of a white female. Another example of Bednar tumor.

Sex

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 However, a larger cancer registry study of 2885 cases reveals female might have slightly higher incidence of DFSP, 4.4 cases versus  4.2 cases per million population per year.9

Age

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

Clinical

History

Dermatofibrosarcoma protuberans (DFSP) is a very slowly growing tumor. Because of the slow progression, the diagnosis is often delayed for months to years.

  • DFSP may start as a small asymptomatic papule or nonindurated patch, which is likely ignored.
  • The tumor may gradually enlarge into a lumpy nodule, or it may evolve into an atrophic and/or sclerotic plaque.
  • Accelerated growth, ulceration, and hemorrhage may be observed when DFSP grows into certain size.

Physical

  • Dermatofibrosarcoma protuberans (DFSP) usually presents as a large, indurated plaque several centimeters in diameter. DFSP is composed of firm, irregular nodules varying in color from flesh to reddish brown. Telangiectasia may be apparent on the surface or at the periphery.
  • Sometimes, DFSP may present as a morphealike, atrophic, sclerotic, violaceous plaque without nodularity that may ulcerate as it slowly increases in size.


A large dermatofibrosarcoma protuberans is seen o...

A large dermatofibrosarcoma protuberans is seen on the abdomen. It has an appearance of atrophic plaque, while foci of nodularity also can be seen.

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A large dermatofibrosarcoma protuberans is seen o...

A large dermatofibrosarcoma protuberans is seen on the abdomen. It has an appearance of atrophic plaque, while foci of nodularity also can be seen.

  • Mostly the tumor is mobile upon palpation; however, fixation to deeper structures such as fascia, muscle, and bone may occur in the later stage of the tumor.
  • DFSP most commonly occurs on the trunk (42-72%), followed by the proximal extremities (16-30%). DFSP rarely occurs above the neck (10-16%).1

Causes

Currently, 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 old burn scars and sites of vaccinations have all been reported as sites of DFSP.14

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

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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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