Soft tissue sarcomas: From a morphological to a molecular biological approach

Yoshinao Oda, Hidetaka Yamamoto, Kenichi Kouhashi, Yuichi Yamada, Kunio Iura, Takeaki Ishii, Akira Maekawa, Hirofumi Bekki

Research output: Contribution to journalReview article

10 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)435-446
Number of pages12
JournalPathology International
Volume67
Issue number9
DOIs
Publication statusPublished - Sep 1 2017

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Sarcoma
Synovial Sarcoma
Liposarcoma
Neoplasms
Small Cell Sarcoma
Solitary Fibrous Tumors
Angiofibroma
Forkhead Transcription Factors
Genetic Techniques
Gene Amplification
Gene Fusion
Neurilemmoma
Testicular Neoplasms
Molecular Biology
Genotype
Antigens
Genes

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine

Cite this

Soft tissue sarcomas : From a morphological to a molecular biological approach. / Oda, Yoshinao; Yamamoto, Hidetaka; Kouhashi, Kenichi; Yamada, Yuichi; Iura, Kunio; Ishii, Takeaki; Maekawa, Akira; Bekki, Hirofumi.

In: Pathology International, Vol. 67, No. 9, 01.09.2017, p. 435-446.

Research output: Contribution to journalReview article

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