Rutkowski Piotr, Przybył Joanna, Świtaj Tomasz
Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, 5 Roentgen Street, Warsaw, Poland.
Department of Molecular and Translational Biology, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, 5 Roentgen Street, Warsaw, Poland.
Int J Biochem Cell Biol. 2014 Aug;53:466-74. doi: 10.1016/j.biocel.2014.03.024. Epub 2014 Apr 2.
Soft tissue and bone sarcomas comprise a heterogeneous group of mesenchymal tumors that include roughly 130 distinct diagnostic entities. Many of them are exceptionally rare, with only few cases diagnosed worldwide each year. Development of novel targeted treatment in this group of tumors is of special importance since many sarcoma subtypes are resistant to conventional chemotherapy and the effective therapeutic options are limited. In this review we aim to discuss the molecular implications for targeted therapy in selected rare soft tissue and bone sarcoma subtypes, including dermatofibrosarcoma protuberans (DFSP), alveolar soft part sarcoma (ASPS), clear cell sarcoma (CCS), giant cell tumor of bone (GCTB) and perivascular epithelioid cell neoplasms (PEComas). This article is part of a Directed Issue entitled: Rare cancers.
软组织和骨肉瘤是一组异质性的间充质肿瘤，包括约130种不同的诊断实体。其中许多极为罕见，每年全球仅诊断出少数病例。由于许多肉瘤亚型对传统化疗耐药且有效的治疗选择有限，因此在这类肿瘤中开发新型靶向治疗尤为重要。在本综述中，我们旨在讨论选定的罕见软组织和骨肉瘤亚型（包括隆突性皮肤纤维肉瘤（DFSP）、腺泡状软组织肉瘤（ASPS）、透明细胞肉瘤（CCS）、骨巨细胞瘤（GCTB）和血管周上皮样细胞瘤（PEComas））靶向治疗的分子意义。本文是名为“罕见癌症”的定向专题的一部分。
