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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.
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor with high recurrence risk. New NCCN guidelines emphasize pathology, including immunohistochemistry and cytogenetics, for accurate diagnosis and management of this challenging cancer.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue neoplasm.
- DFSP presents a high risk of local recurrence but a low risk of metastasis.
- Management of DFSP requires a multidisciplinary approach.
Framework:
- The NCCN Guidelines for DFSP offer updated recommendations for patient care.
- Key updates include the addition of a Principles of Pathology section.
- This section focuses on the pathologic assessment of DFSP.
Implementation:
- Accurate diagnosis of DFSP is crucial due to its potential to mimic other skin lesions.
- Immunohistochemical studies are frequently necessary for definitive diagnosis.
- Cytogenetic testing for the t(17;22)(q22;q13) translocation aids in differentiating DFSP from similar tumors.
Implications:
- Enhanced diagnostic accuracy improves patient outcomes for DFSP.
- Standardized pathologic assessment ensures consistent and reliable diagnoses.
- Genetic insights into DFSP support differential diagnosis and inform treatment strategies.
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