Jeremy Bordeaux, Rachel Blitzblau, Sumaira Z Aasi, Murad Alam, Arya Amini, Kristin Bibee, Diana Bolotin, Pei-Ling Chen, Carlo M Contreras, Dominick DiMaio, Jessica M Donigan, Jeffrey M Farma, Karthik Ghosh, Kelly Harms, Nicole LeBoeuf, John Nicholas Lukens, Susan Manber, Lawrence Mark, Theresa Medina, Kishwer S Nehal, Paul Nghiem, Kelly Olino, Gyorgy Paragh, Soo Park, Tejesh Patel, Jason Rich, Ashok R Shaha, Bhavina Sharma, Yemi Sokumbi, Divya Srivastava, Valencia Thomas, Courtney Tomblinson, Puja Venkat, Yaohui Gloria Xu, Siegrid Yu, Mehran Yusuf, Beth McCullough, Sara Espinosa
{"title":"Dermatofibrosarcoma Protuberans, Version 1.2025, NCCN Clinical Practice Guidelines In Oncology.","authors":"Jeremy Bordeaux, Rachel Blitzblau, Sumaira Z Aasi, Murad Alam, Arya Amini, Kristin Bibee, Diana Bolotin, Pei-Ling Chen, Carlo M Contreras, Dominick DiMaio, Jessica M Donigan, Jeffrey M Farma, Karthik Ghosh, Kelly Harms, Nicole LeBoeuf, John Nicholas Lukens, Susan Manber, Lawrence Mark, Theresa Medina, Kishwer S Nehal, Paul Nghiem, Kelly Olino, Gyorgy Paragh, Soo Park, Tejesh Patel, Jason Rich, Ashok R Shaha, Bhavina Sharma, Yemi Sokumbi, Divya Srivastava, Valencia Thomas, Courtney Tomblinson, Puja Venkat, Yaohui Gloria Xu, Siegrid Yu, Mehran Yusuf, Beth McCullough, Sara Espinosa","doi":"10.6004/jnccn.2025.0001","DOIUrl":null,"url":null,"abstract":"<p><p>Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. DFSP frequently exhibits extensive local infiltration. Initial treatment is through surgical excision, and care should be taken to ensure that negative margins are achieved to minimize recurrence. Although DFSP has a reported high rate of recurrence, metastasis is more uncommon. Fibrosarcomatous DFSP is an aggressive variant with an increased risk for local recurrence and metastasis. If achieving negative margins or resection is not feasible, radiation therapy or systemic treatment are options that may be considered by a multidisciplinary team. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) outline recommended treatment options available for DFSP.</p>","PeriodicalId":17483,"journal":{"name":"Journal of the National Comprehensive Cancer Network","volume":"23 1","pages":""},"PeriodicalIF":14.8000,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of the National Comprehensive Cancer Network","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.6004/jnccn.2025.0001","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"ONCOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. DFSP frequently exhibits extensive local infiltration. Initial treatment is through surgical excision, and care should be taken to ensure that negative margins are achieved to minimize recurrence. Although DFSP has a reported high rate of recurrence, metastasis is more uncommon. Fibrosarcomatous DFSP is an aggressive variant with an increased risk for local recurrence and metastasis. If achieving negative margins or resection is not feasible, radiation therapy or systemic treatment are options that may be considered by a multidisciplinary team. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) outline recommended treatment options available for DFSP.
期刊介绍：
JNCCN—Journal of the National Comprehensive Cancer Network is a peer-reviewed medical journal read by over 25,000 oncologists and cancer care professionals nationwide. This indexed publication delivers the latest insights into best clinical practices, oncology health services research, and translational medicine. Notably, JNCCN provides updates on the NCCN Clinical Practice Guidelines in Oncology® (NCCN Guidelines®), review articles elaborating on guideline recommendations, health services research, and case reports that spotlight molecular insights in patient care.
Guided by its vision, JNCCN seeks to advance the mission of NCCN by serving as the primary resource for information on NCCN Guidelines®, innovation in translational medicine, and scientific studies related to oncology health services research. This encompasses quality care and value, bioethics, comparative and cost effectiveness, public policy, and interventional research on supportive care and survivorship.
JNCCN boasts indexing by prominent databases such as MEDLINE/PubMed, Chemical Abstracts, Embase, EmCare, and Scopus, reinforcing its standing as a reputable source for comprehensive information in the field of oncology.
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