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. 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.

Physical Examination

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.

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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]and extremely rarely on the breast.^[19]

Differential Diagnoses

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Media Gallery

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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Author

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

Disclosure: Nothing to disclose.

Coauthor(s)

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.

Specialty Editor Board

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; Past President, 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.

Chief Editor

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<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

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

Disclosure: Nothing to disclose.

Sections Dermatofibrosarcoma Protuberans
Overview
Presentation
DDx
Workup
Treatment
Medication
Questions & Answers
Media Gallery
References

Dermatofibrosarcoma Protuberans Clinical Presentation

History

Physical Examination

References

Tables

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