Dermatofibrosarcoma protuberans: from translocation to targeted therapy

查看全文 作　　者：Jonathan [1]Noujaim;Khin [1]Thway;Cyril [1]Fisher;Robin [1]L.Jones

机构地区：[1]Sarcoma Unit, Royal Marsden NHS Foundation Trust

出　　处：《Cancer Biology & Medicine》索引2015年第12卷第4期,共10页

基　　金：support from the NIHR Royal Marsden/ICR Biomedical Research Center 摘　　要：Dermatofibrosarcoma protuberans(DFSP), the most common dermal sarcoma, is a low-grade, slow growing fibroblastic malignant neoplasm that most frequently affects middle aged adults and is characterized by a high local recurrence rate and a low propensity for metastasis. Wide surgical resection or Mohs micrographic surgery(MMS) are the preferred approaches for localized disease, while radiation therapy is warranted for inoperable disease or for cases with positive margins where re-excision is not possible. DFSP is generally regarded as refractory to conventional chemotherapy. Treatment options for systemic disease were limited until the discovery of a unique translocation, t(17;22)(q22;q13)(COL1A1;PDGFB) found in a majority of cases. In recent years, imatinib, a PDGFβR, ABL and KIT inhibitor, has revolutionized systemic therapy in DFSP. In this review, we summarize the epidemiological, clinical, histological and genetic characteristics of DFSP and update the readers on its current management. 关键词：靶向治疗皮肤肿瘤纤维肉瘤全身性疾病转运显微外科手术成纤维细胞恶性肿瘤

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Dermatofibrosarcoma protuberans: from translocation to targeted therapy

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