Premium
Soft tissue sarcomas: From a morphological to a molecular biological approach
Author(s) -
Oda Yoshinao,
Yamamoto Hidetaka,
Kohashi Kenichi,
Yamada Yuichi,
Iura Kunio,
Ishii Takeaki,
Maekawa Akira,
Bekki Hirofumi
Publication year - 2017
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/pin.12565
Subject(s) - synovial sarcoma , malignant peripheral nerve sheath tumor , sarcoma , fusion gene , liposarcoma , pathology , biology , tfe3 , cancer research , myxoid liposarcoma , soft tissue , solitary fibrous tumor , comparative genomic hybridization , clear cell sarcoma , soft tissue sarcoma , immunohistochemistry , gene , medicine , gene expression , cd34 , stem cell , chromosome , microbiology and biotechnology , promoter , biochemistry
Recently developed molecular genetic techniques have led to the elucidation of tumor‐specific genomic alterations and thereby the reclassification of tumor entities of soft tissue sarcoma. A solitary fibrous tumor‐mimicking tumor with the AHRR‐NCOA2 gene has been isolated as angiofibroma of soft tissue. As for small round cell sarcomas, novel fusion genes such as CIC‐DUX4 and BCOR‐CCNB3 have been identified in these tumor groups. SMARCB1/INI1 deficient tumors with round cell morphology are also expected to be reclassified in three types, based on the combination of their morphology and genotype. The identification of the MDM2 gene amplification in pleomorphic sarcomas has extended the entity of dedifferentiated liposarcoma (DDLS). Our recent molecular investigations elucidated candidates for novel therapeutic strategies. Activation of the Akt‐mTOR pathway was correlated with poor prognosis or tumor grade in spindle cell sarcomas including malignant peripheral nerve sheath tumor. In vitro and in vivo studies of transcription factor Forkhead Box M1 (FOXM1) demonstrated the close correlation between aggressive biological behavior or chemosensitivity and FOXM1 expression in synovial sarcoma, so far. Finally, in regard to the investigation of cancer‐testis antigens, myxoid/round cell liposarcoma and synovial sarcoma showed frequent and high expression of PRAME and NY‐ESO‐1.