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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)
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Your Question

My husband had dermatofibrosarcoma protuberans on his thigh.  Will it spread to other parts of the body by the lymph?  If it spreads, what are the symptoms?  Can dermatofibrosarcoma affect heart, lungs, liver, kidney or other internal organs?  If so, what are the symptoms?  Can any symptoms be found during a complete body checkup before it spreads?

Our Answer

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

Can dermatofibrosarcoma protuberans spread through the lymph system?

There is up to a 1% chance that dermatofibrosarcoma protuberans could spread (metastasize) through the lymph system.[1]  When cancer cells spread by the lymph, they collect in lymph nodes near the original site of the tumor.  As these cells accumulate in a lymph node, the node swells to form a lump or bump that feels firm and is usually painless.  A growing mass is a sign that cancer cells may have spread by the lymph system.
Last updated: 12/20/2010

Can dermatofibrosarcoma protuberans affect internal organs?

Dermatofibrosarcoma protuberans can affect other organs if it spreads (metastasizes) beyond the original location of the tumor in the skin.  The most common site of metastasis for this tumor is the lungs.[2]  Shortness of breath can be a sign of lung metastasis.[3]
Last updated: 12/20/2010

What signs and symptoms suggest that dermatofibrosarcoma protuberans has spread to other parts of the body?

If dermatofibrosarcoma protuberans spread to other parts of the body, the signs and symptoms vary depending on the size of the metastatic tumor and which organ it affects.[3]  Regular physical examinations can help detect recurrence of the tumor in the original location or signs that it has spread to other parts of the body.[1]  The physical examination includes a skin examination to look for any changes in the skin's appearance and to feel for any differences in the tissues underneath the skin.  Special attention is given to the original site of the tumor and nearby lymph nodes.[4]  A sign of spread to the lymph nodes is a firm, movable mass near the original location of the tumor.
Last updated: 12/20/2010