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肺泡软组织肉瘤：分子发病机制及对新型靶向治疗的意义

Alveolar soft part sarcomas: molecular pathogenesis and implications for novel targeted therapies.

作者信息

Mitton Bryan, Federman Noah

机构信息

Division of Pediatric Hematology/Oncology, Department of Pediatrics, Mattel Children's Hospital at UCLA, UCLA David Geffen School of Medicine, 10833 Le Conte Avenue, Los Angeles, CA 90095-175, USA.

出版信息

Sarcoma. 2012;2012:428789. doi: 10.1155/2012/428789. Epub 2012 Apr 8.

DOI:10.1155/2012/428789
PMID:22566752
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3337503/
Abstract

Alveolar soft part sarcoma (ASPS) is a very rare soft tissue sarcoma which arises primarily in children and young adults. Despite its unique histology and well-characterized genetic translocation, many questions remain regarding the pathogenesis and treatment of this tumor type. Though collective clinical experience with this tumor type spans more than 60 years, there has been little progress made in treating this uncommon but frequently fatal disease. This paper focuses on the available data regarding its molecular pathogenesis and insights into targeted therapeutics as well as the results of clinical trials performed to date to hopefully improve the outcome of patients with this rare malignancy.

摘要

肺泡软组织肉瘤（ASPS）是一种非常罕见的软组织肉瘤，主要发生于儿童和年轻人。尽管其具有独特的组织学特征和明确的基因易位，但关于这种肿瘤类型的发病机制和治疗仍存在许多问题。虽然针对这种肿瘤类型的临床经验已有60多年，但在治疗这种罕见却常致命的疾病方面进展甚微。本文重点关注其分子发病机制的现有数据、靶向治疗的见解以及迄今为止进行的临床试验结果，以期改善这种罕见恶性肿瘤患者的治疗效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c620/3337503/a43eb892803d/SRCM2012-428789.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c620/3337503/3c02c1e9efc1/SRCM2012-428789.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c620/3337503/a43eb892803d/SRCM2012-428789.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c620/3337503/3c02c1e9efc1/SRCM2012-428789.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c620/3337503/a43eb892803d/SRCM2012-428789.002.jpg

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