eMedicine Specialties > Dermatology > Malignant Neoplasms

Dermatofibrosarcoma Protuberans

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
Daniel Mark Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate

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 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.

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 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.

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 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

Differential Diagnoses

Dermatofibroma
Epidermal Inclusion Cyst
Keloid and Hypertrophic Scar
Malignant Melanoma
Metastatic Carcinoma of the Skin
Morphea

Other Problems to Be Considered

Lymphoma
Fibrosarcoma

Workup

Laboratory Studies

No blood test is available to help detect or diagnose dermatofibrosarcoma protuberans (DFSP).

Imaging Studies

In most cases of dermatofibrosarcoma protuberans (DFSP), no imaging studies are used unless metastatic disease is suspected after a thorough history taking and a complete physical examination.

• Chest radiography may be ordered for baseline screening for pulmonary metastasis in high-risk cases, such as recurrence or suspicion for a fibrosarcoma variant of DFSP.¹⁵
• Computed tomography scanning is indicated if direct bone involvement or metastasis is suspected.¹
• Studies seem to support a role of MRI for preoperative assessment in larger or atypical lesions and recurrent disease. MRI may be helpful to define the approximate tumor border and depth of invasion.^15,16,17
• Ultrasonography may be helpful for monitoring local DFSP or regional lymph node metastasis.
• Reports suggest fluorodeoxyglucose (FDG)–positron emission tomography scanning may be helpful in monitoring metastatic disease.¹⁸

Procedures

A skin biopsy is essential for definitive diagnosis.

Histologic Findings

In the plaque type of dermatofibrosarcoma protuberans (DFSP), slender tumor cells with large, spindle-shaped nuclei are embedded fairly uniformly in the collagen stroma, parallel to the skin surface. Mitotic figures are sparse. The more characteristic findings are seen in the nodular type. These findings include the high cellularity and irregular, short, intersecting bands of tumor cells forming a storiform pattern. Also typical are cells radiating from a central hub of fibrous tissue forming a cartwheel pattern. The degree of cellular atypia is higher in nodular lesions than in plaque lesions. Occasionally, DFSP may show focal fibrosarcomatous changes with a characteristic herringbone pattern. The cellular atypia is then even more prominent with hyperchromatic nuclei and more mitotic figures.^2,19

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.

In the pigmented variant of DFSP, also known as Bednar tumor, the melanin-containing dendritic cells are scattered between the neoplastic spindle-shaped cells.²⁰

In the juvenile form (giant cell fibroblastoma), cleftlike pseudovascular spaces are lined by multinucleated cells. The intervening tumor may have loose hypocellular areas and areas that resemble mature DFSP.

Immunohistochemistry studies have shown moderate-to-strong staining of human progenitor cell antigen CD34 in tumor cells. CD34 is a useful marker that allows differentiation of DFSP tumor cells from normal stroma cells and dermatofibroma. In dermatofibroma, tumor cells are positive for factor XIIIa and are rarely positive for CD34. Additionally, immunostaining using CD34 as a marker is helpful in identifying tumor cells at the surgical margins, particularly when treating recurrent DFSP in which tumor cell fascicles are often interspersed with the scar tissue.^21,22

DFSP demonstrates strong CD34 staining with immunohistochemistry.

Staging

The American Joint Committee on Cancer has not developed a staging system for DFSP. Because of its very low risk of metastasis, DFSP can be viewed as mostly  a local disease. The following simple staging system published in Short German guidelines for DFSP may be helpful in clinical use¹⁵ :

• Stage I - Primary tumor, localized disease
• Stage II - Lymph node metastasis
• Stage III - Distal metastasis

Treatment

Medical Care

Currently, chemotherapy is rarely used in the treatment of dermatofibrosarcoma protuberans (DFSP). Radiation therapy 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. The complete radiation therapy dose ranges from 60-70 Gy. Close follow-up care after radiation therapy is warranted because some DFSP tumors may become more aggressive.^1,15,23

The development of molecularly targeted therapy holds promise as an additional treatment option. 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 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 dose is 800 mg/d.¹⁸

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 lacking the classic t(17,22) gene aberration seems to have no response to imatinib.¹  

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

Surgical Care

Surgical excision remains the mainstay of treatment for dermatofibrosarcoma protuberans (DFSP). Mohs micrographic surgery has been increasingly accepted as the treatment of choice (see Mohs Micrographic Surgery).¹³

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 3 cm or more of the margins, down to and including the fascia, is recommended for the treatment of DFSP. 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.^{19,24,25,26,27,28,29}

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.^{27,24,30,31,32}

Medication

Although medical therapy is not a first-line treatment for localized dermatofibrosarcoma protuberans (DFSP), the newly approved molecular targeted drug, imatinib mesylate, is an effective oral medication for unresectable, recurrent, and/or metastatic DFSP.

Molecular targeted therapy

Before starting imatinib therapy, cytogenetic studies to confirm PDGFB gene rearrangement may be necessary in predicting the clinical response. Chromosome translocation t(17,22) is detected in more than 90% of DFSP tumors.

Imatinib mesylate (Gleevec)

Inhibitor of receptor tyrosine kinase for PDGF. Inhibits PDGF-B receptor-mediated cellular events, the key pathogenetic pathway in DFSP.

Dosing

Adult

800 mg/d PO divided bid

Pediatric

Not established

Interactions

CYP3A4 inhibitors (ketoconazole increases distribution of imatinib); CYP3A4 substrates (simvastatin increases maximum concentration of imatinib by a 2- to 3.5-fold factor); CYP3A4 inducers (phenytoin decreases AUC by approximately one fifth of typical AUC); likely to increase blood levels of drugs that are substrates of CYP2C9, CYP2D6, and CYP3A4/5
Increase dose by at least 50% in patients receiving concomitant CYP3A4 inducers (eg, rifampin, phenytoin)

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Dose must be reduced or interrupted if edema or anemia occurs, transaminase or bilirubin levels become elevated, or grade 3-4 neutropenia or thrombocytopenia develops; pediatric patients commonly experience musculoskeletal pain
Adverse effects include congestive heart failure, increased risk of hypereosinophilic cardiac toxicity, grade 3-4 gastrointestinal and tumor site bleeding, pleural effusion, and gastrointestinal perforation (including fatalities); increased risk of edema, severe congestive heart failure, and left ventricular dysfunction reported in elderly persons (>65 y); fatigue, hypokalemia, and hypophosphatemia reported; monitor CBC counts qwk for first month, q2wk for second month, and periodically thereafter; perform liver function tests at baseline and then monthly or as clinically indicated
Initiation in post-thyroidectomy patients receiving levothyroxine has resulted in elevated levels of thyrotropin and symptoms of hypothyroidism

Follow-up

Further Outpatient Care

Because of the high local recurrence rate of dermatofibrosarcoma protuberans (DFSP), patients require close follow-up care after treatment.  

• Most recurrences occur within 3 years of the primary excision. Patients should be seen every 6 months during this period and annually thereafter.^1,15
• A literature review of DFSP case series treated with Mohs surgery shows that 50% of recurrences appear within the first 3 years after operation and 25% of local recurrences are detected after 5 years. A large case review from a series of 159 patients treated at Memorial Sloan-Kettering Cancer Center (New York) showed the medium time to the development of a local recurrence was 32 months. The indolent nature of DFSP requires lifelong surveillance for recurrence.¹⁹
• In each follow-up visit, a complete history and a review of systems, as well as complete physical examinations, including skin examination and palpation of the excision site and regional lymph nodes, should be performed. An extensive workup is not warranted unless metastatic disease is suspected.

Prognosis

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 classic form of DFSP, the risk is assumed to be only 0.5%. According to the literature, the overall risk for 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. The lungs are the most common site of distant metastasis that occurs via hematogenous spread. Usually, metastatic disease is preceded by multiple local recurrences.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.^1,18,33

Age older than 50 years is also a risk factor associated with a poor clinical outcome.¹⁹

Patient Education

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

Miscellaneous

Medicolegal Pitfalls

Because of the rarity of dermatofibrosarcoma protuberans (DFSP), misdiagnosis and delayed diagnosis often occur. When encountering an atypical scarlike lesion, the clinician should consider skin biopsy.

Multimedia

Media file 1: Dermatofibrosarcoma protuberans manifesting as an irregular red-to-violaceous plaque on the chest.

Media file 2: Closer view of dermatofibrosarcoma protuberans. It has an irregular surface and borders with palpable dermal and subcutaneous induration.

Media file 3: A large dermatofibrosarcoma protuberans is seen on the abdomen. It has an appearance of atrophic plaque, while foci of nodularity also can be seen.

Media file 4: Bednar tumor, a pigmented variant of dermatofibrosarcoma protuberans, contains melanin-rich dendritic cells scattered among neoplastic spindle-shaped cells.

Media file 5: A featureless reddish brown plaque on the arm of a white female. Another example of Bednar tumor.

Media file 6: 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.

Media file 7: Higher-power view of dermatofibrosarcoma protuberans reveals subcutaneous adipocytes entrapped by densely infiltrative spindle-shaped tumor cells.

Media file 8: DFSP demonstrates strong CD34 staining with immunohistochemistry.

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

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.

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.

Clinical trials

• Imatinib in Dermatofibrosarcoma Protuberans (DFSP) (active, not recruiting)
• A Short Course of Neoadjuvant Gleevec (Imatinib Mesylate) in Dermatofibrosarcoma Protuberans (recruiting)
• Imatinib Mesylate in Treating Patients With Locally Recurrent or Metastatic Dermatofibrosarcoma Protuberans (DFSP) or Transformed Fibrosarcomatous DFSP (active, not recruiting)
• Neoadjuvant Imatinib in Dermatofibrosarcoma Protuberans (recruiting)
• Clinical Outcome of Pediatric Dermatofibrosarcoma Protuberans (recruiting)

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