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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 mother had a tumor removed from her back. They diagnosed the tumor as a dermatofibrosarcoma protuberans. They don't think they got it all. How quickly will this tumor regrow. Will my mother have to have another surgery. She is in her 80's and does not want to undergo a second surgery.

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 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

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

Should my mother undergo another surgery for her dermatofibrosarcoma protuberans?

We recommend that you discuss your mother's concern about undergoing a second surgery with her doctors. They will be best able to help you anticipate the course of her condition and make decisions regarding her treatment options.
Last updated: 7/2/2009

Who can I contact to learn more about dermatofibrosarcoma protuberans?

Dermatofibrosarcoma protuberans is a type of soft tissue sarcoma. You can obtain comprehensive information on soft tissue sarcoma by calling the National Cancer Institute Cancer Information Service (CIS) toll-free at 1-800-4-CANCER (1-800-422-6237) or by visiting their Web site. You may want to ask if they can provide you with information specific to dermatofibrosarcoma protuberans as well. CIS provides the most current information on cancer for patients, health professionals, and the general public. Click on the link below to read the NCI's information page on soft tissue sarcomas.
http://www.cancer.gov/cancertopics/pdq/treatment/adult-soft-tissue-sarcoma/patient

NCI Public Inquiries Office
6116 Executive Blvd., Room 3036A
Bethesda, MD 20892-8322
Toll free:  800-422-6237
TTY: 800-332-8615
E-mail: cancergovstaff@mail.nih.gov
Web site:   http://cis.nci.nih.gov

You can also find a list of additional resources that provide information on this topic on the GARD dermatofibrosarcoma protuberan's resource page by clicking here

Last updated: 7/2/2009

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.