Genetics of rare mesenchymal tumors: implications for targeted treatment in DFSP, ASPS, CCS, GCTB and PEComa.
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.
Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, 5 Roentgen Street, Warsaw, Poland. Electronic address: rutkowskip@coi.waw.pl.
Piotr
P
Rutkowski
Department of Molecular and Translational Biology, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, 5 Roentgen Street, Warsaw, Poland.
Joanna
J
Przybył
Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, 5 Roentgen Street, Warsaw, Poland.
Tomasz
T
Świtaj
D018223:Dermatofibrosarcoma / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D018212:Giant Cell Tumor of Bone / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D006801:Humans; D008648:Mesoderm / Q000473:pathology; D058990:Molecular Targeted Therapy; D054973:Perivascular Epithelioid Cell Neoplasms / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D018234:Sarcoma, Alveolar Soft Part / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D018227:Sarcoma, Clear Cell / Q000235:genetics / Q000473:pathology / Q000628:therapy*
Alveolar soft part sarcoma; Clear cell sarcoma; Dermatofibrosarcoma protuberans; Giant cell tumor of bone; Perivascular epithelioid cell neoplasms; Targeted treatment