Other Skin Cancers

Fibrohistiocytic Skin Cancers and Extramammary Paget's Disease
  • Thomas StaskoEmail author
  • Sarah Grummer

Core Messages

  • Dermatofibrosarcoma protuberans is a rare malignancy of intermediate aggressiveness which usually presents as an asymptomatic, slowly enlarging, indurated violaceous to flesh-colored plaque. Subclinical histologic extension consisting of tentacle-like projections between subcutaneous shelf-like tumor masses makes complete excision, even with wide margins, difficult. Mohs surgery appears to offer the best chance for complete excision. Inhibition of platelet-derived growth factor receptors with imatinib may reduce tumor size in metastatic or unresectable tumors.

  • Atypical fibroxanthoma is a spindle cell tumor most commonly found on actinically damaged skin, primarily the head and neck, of elderly Caucasians with a small potential for metastasis. It is characterized by spindle cells, bizarre multinucleated giant cells, xanthomatous cells and atypical mitoses. Complete excision, often with Mohs surgery, is the usual treatment.

  • Most tumors formerly diagnosed as malignant fibrous histiocytoma have now been reclassi-fied as non-sarcomatous malignancies. The remaining tumors, which tend to have a very aggressive course, are now best termed undif-ferentiated pleomorphic sarcomas.

  • Extramammary Paget's Disease is a rare cutaneous adenocarcinoma occurring on apocrine glandbearing skin in which the initial diagnosis if often delayed because of its clinical resemblance chronic conditions such as inter-trigo or contact dermatitis. Because of an association with internal malignancies, a through work-up for other cancers is necessary.


Malignant Fibrous Histiocytoma Wide Local Excision Seborrheic Dermatitis Spindle Cell Tumor Soft Tissue Malignancy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2010

Authors and Affiliations

  1. 1.Vanderbilt UniversityNashvilleUSA
  2. 2.The Vanderbilt ClinicNashvilleUSA

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