Dermatofibrosarcoma protuberans is a low-grade cutaneous neoplasm originating from the dermis with a high propensity for local recurrence. These malignancies are classified as soft tissue sarcoma although regional or distance metastatic spread is rare. Germ-line mutations of p53 have been described with a rare autosomal dominant syndrome in patients with soft tissue sarcoma and breast adenocarcinoma (Li Fraumeni's syndrome). The described case illustrates the rare occurrence of dermatofibrosarcoma protuberans (DFSP) and breast adenocarcinoma with an emphasis on the possibility of a molecular or genetic association.
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References
1.
JemalA, TomasA, MurrayT, . Cancer statistics 2002.Cancer2002;52:25.
2.
LiFP, FraumeniJF.Soft tissue sarcomas, breast cancers and other neoplasms.Ann Intern Med1969;71:747–52.
3.
DarierJ, FerrandM.Dermatofibromas Progressiffa et Recidivants, on fibrosarcomes de la peau.Ann Dermatol Syph, 1924; 5:545–70.
4.
FisherB, RedmondC, PoissonR, . Eight-year results of a randomized clinical trial comparing total mastectomy and lumpectomy with or without radiation in the treatment of breast cancer.N Engl J Med1989;320:822–8.
5.
BrennerW, SchaeferK, ChaabraJ, . Dermatofibrosarcoma protuberans with metastasis to a regional lymph node: Report of a case and review.Cancer, 1975;36:1897–1902.
6.
AdamsJT, SaltzsteinSL.Metastasizing dermatofibrosarcoma protuberans: Report of two cases.Am J Surg1963;29:878–86.
