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Dermatofibrosarcoma protuberans, version 1.2014
Christopher K Bichakjian1, Thomas Olencki1, Murad Alam1
1From University of Michigan Comprehensive Cancer Center; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute; Robert H. Lurie Comprehensive Cancer Center of Northwestern University; City of Hope Comprehensive Cancer Center; University of Washington/Seattle Cancer Care Alliance; Huntsman Cancer Institute at the University of Utah; Roswell Park Cancer Institute; UC San Diego Moores Cancer Center; Moffitt Cancer Center; UCSF Helen Diller Family Comprehensive Cancer Center; Memorial Sloan-Kettering Cancer Center; University of Colorado Cancer Center; Fred & Pamela Buffett Cancer Center at The Nebraska Medical Center; The University of Texas MD Anderson Cancer Center; Duke Cancer Institute; Fox Chase Cancer Center; Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine; St. Jude Children's Research Hospital/The University of Tennessee Health Science Center; University of Alabama at Birmingham Comprehensive Cancer Center; The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins; Dana-Farber/Brigham and Women's Cancer Center; Vanderbilt-Ingram Cancer Center; and National Comprehensive Cancer Network.
Abstract:
Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor characterized by a relatively high risk of local recurrence and low risk of metastasis. The NCCN Guidelines for DFSP provide multidisciplinary recommendations on the management of patients with this rare disease. These NCCN Guidelines Insights highlight the addition of the Principles of Pathology section, which provides recommendations on the pathologic assessment of DFSP. Because DFSP can mimic other lesions, immunohistochemical studies are often required to establish diagnosis. Cytogenetic testing for the characteristic translocation t(17;22)(q22;q13) can also be valuable in the differential diagnosis of DFSP with other histologically similar tumors.
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