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Genetic and Rare Diseases Information Center (GARD)

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Dermatofibrosarcoma protuberans


Other Names for this Disease

  • DFSP
  • Familial dermatofibrosarcoma protuberans (subtype)
  • Giant cell fibroblastoma
  • 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 tumor that arises in the skin. The tumor is firm and often flesh-colored although it can be reddish, bluish, or purplish. The tumor is often found on the chest or shoulders, but it can be found on other parts of the body.  Dermatofibrosarcoma protuberans may cause no symptoms, and the initial size of the tumor tends to be around 1 to 5 centimeters. This tumor has a low potential to spread to other tissues (metastasize). Treatment often involves surgery to remove the tumor, such as by Mohs’ micrographic surgery[1]
Last updated: 11/18/2010

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%.[2]
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%. [2]
Last updated: 11/18/2010

References
  • Wood GS. et al. Nonmelanoma Skin Cancers: Basal Cell and Squamous Cell Carcinomas. In: Abeloff et al. Clinical Oncology, 4th ed. Philadelphia, PA: Churchill Livingstone; 2008;
  • Chen CSJ and Siegel DM. Dermatofibrosarcoma Protuberans. eMedicine. July 23, 2010; http://emedicine.medscape.com/article/1100203-overview. Accessed 11/14/2010.
Other Names for this Disease
  • DFSP
  • Familial dermatofibrosarcoma protuberans (subtype)
  • Giant cell fibroblastoma
  • 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.