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皮肤肉瘤的诊断与治疗指南:隆突性皮肤纤维肉瘤

Guidelines for Diagnosis and Treatment of Cutaneous Sarcomas: Dermatofibrosarcoma Protuberans.

作者信息

Llombart B, Serra C, Requena C, Alsina M, Morgado-Carrasco D, Través V, Sanmartín O

机构信息

Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.

Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.

出版信息

Actas Dermosifiliogr (Engl Ed). 2018 Dec;109(10):868-877. doi: 10.1016/j.ad.2018.05.006. Epub 2018 Jul 4.

DOI:10.1016/j.ad.2018.05.006
PMID:30539729
Abstract

Sarcomas comprise a broad group of tumors, many of whose biological behavior and aggressiveness differ from one type to another. The therapeutic approach is generally multidisciplinary and often complex. Developments in surgical and oncological dermatology during the last few decades have positioned dermatologists as specialists in the diagnosis and treatment of skin cancer. The aim of this article is to review the main soft tissue sarcomas that typically affect the skin. Dermatofibrosarcoma protuberans is a low-grade malignant sarcoma. It exhibits slow-growth, is locally invasive, and has low metastatic potential (<3%). Mohs micrographic surgery is the treatment of choice. The COL1A1-PDGFB translocation should be analyzed in cases of unclear diagnosis and when it is necessary to identify candidates for tyrosine kinase inhibitors. Imatinib is indicated for the treatment of locally advanced and metastatic dermatofibrosarcoma protuberans.

摘要

肉瘤是一大类肿瘤,其中许多肿瘤的生物学行为和侵袭性因类型而异。治疗方法通常是多学科的,而且往往很复杂。过去几十年里,外科和肿瘤皮肤科的发展使皮肤科医生成为皮肤癌诊断和治疗方面的专家。本文旨在综述通常影响皮肤的主要软组织肉瘤。隆突性皮肤纤维肉瘤是一种低级别恶性肉瘤。它生长缓慢,具有局部侵袭性,转移潜能低(<3%)。莫氏显微外科手术是首选治疗方法。在诊断不明确以及需要确定酪氨酸激酶抑制剂候选患者时,应分析COL1A1-PDGFB易位情况。伊马替尼适用于治疗局部晚期和转移性隆突性皮肤纤维肉瘤。

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