Dermatofibrosarcoma Protuberans 

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

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] Note the images below.

Dermatofibrosarcoma protuberans manifesting as an Dermatofibrosarcoma protuberans manifesting as an irregular red-to-violaceous plaque on the chest. Closer view of dermatofibrosarcoma protuberans. ItCloser view of dermatofibrosarcoma protuberans. It has an irregular surface and borders with palpable dermal and subcutaneous induration.
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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 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 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-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]

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Epidemiology

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] 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]

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.[12]

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] Note the images below.

Bednar tumor, a pigmented variant of dermatofibrosBednar 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 aA 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.[13] 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.[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).[14]

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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.

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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.
 
 
 
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