eMedicine Specialties > Dermatology > Malignant Neoplasms

Dermatofibrosarcoma Protuberans: Differential Diagnoses & Workup

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

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 cell...

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.

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Dermatofibrosarcoma protuberans (DFSP) tumor cell...

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 protuber...

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

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Higher-power view of dermatofibrosarcoma protuber...

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 immun...

DFSP demonstrates strong CD34 staining with immunohistochemistry.

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DFSP demonstrates strong CD34 staining with immun...

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

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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