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Diseases

Genetic and Rare Diseases Information Center (GARD)

Dermatofibrosarcoma protuberans


Other Names for this Disease
  • DFSP
  • Giant cell fibroblastoma
  • Familial dermatofibrosarcoma protuberans (subtype)
  • Metastatic dermatofibrosarcoma protuberans (subtype)
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My husband had a mole on his leg and a biopsy confirmed that it is a dermatofibrosarcoma protuberans.  Is this very harmful like other cancers?  Is there any chance it could spread to other parts of the body?  What is the chance for recurrence?  What are the available treatments?  Are there precautions to take to avoid recurrence?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is dermatofibrosarcoma protuberans?

Dermatofibrosarcoma protuberans is an uncommon cancer in which tumors arise in the deeper layers of skin. The tumor usually starts as a small, firm patch of skin; it may be purplish, reddish, or flesh-colored. It is commonly found on the torso, usually in the shoulder and chest area. The tumor typically grows slowly but has a tendency to recur after being removed. It rarely spreads to other parts of the body. The cause of DFSP is unknown, but injury to the affected skin may be a predisposing factor. Treatment usually involves surgically removing the tumor. If the tumor is unable to be removed completely, additional therapy may be needed. Regular follow-up is important to monitor for recurrence.[1][2][3]
Last updated: 2/9/2016

What is the chance of dermatofibrosarcoma protuberans spreading to other parts of the body?

Dermatofibrosarcoma protuberans is a slow-growing tumor and does not usually spread to distant parts of the body (metastasize). The overall rate of distant metastasis is estimated to be around 4%, and the rate of metastasis to nearby lymph nodes is 1%.[4]
Last updated: 11/18/2010

What is the chance of recurrence of dermatofibrosarcoma protuberans?

Dermatofibrosarcoma protuberans has an average recurrence rate of 15.7% after being removed by standard surgery known as wide local excision (when the entire tumor and some of the surrounding normal tissue are removed).  Most local recurrences occur within 3 years.  Recent studies suggest that a newer surgical technique, Mohs’ micrographic surgery, may help reduce this recurrence rate to approximately 1.3%. [4]
Last updated: 11/18/2010

References
Other Names for this Disease
  • DFSP
  • Giant cell fibroblastoma
  • Familial dermatofibrosarcoma protuberans (subtype)
  • Metastatic dermatofibrosarcoma protuberans (subtype)
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.