TY - JOUR
T1 - Malignant solitary fibrous tumor with high-grade nuclear atypia
T2 - An alternate entity for the undetermined tumor group
AU - Yamada, Yuichi
AU - Kohashi, Kenichi
AU - Bekki, Hirofumi
AU - Ishii, Takeaki
AU - Iura, Kunio
AU - Maekawa, Akira
AU - Yamamoto, Hidetaka
AU - Iwamoto, Yukihide
AU - Oda, Yoshinao
N1 - Funding Information:
This study was supported by a Grant-in-Aid for Scientific Research (B) (no. 2139107 ) from the Japan Society for the Promotion of Science , the National Cancer Center Research and Development Fund ( 12-B-12 ), and a Scholarship from the Takeda Science Foundation .
Publisher Copyright:
© 2014 Elsevier GmbH.
PY - 2015/2/1
Y1 - 2015/2/1
N2 - Recently, a novel fusion transcript, NAB2-STAT6, and its variants have also been reported to be specific diagnostic markers for solitary fibrous tumors (SFTs). In this study, we validated the existence of the NAB2-STAT6 fusion gene in SFTs and examined its relation with the pathological features. Frozen samples from 9 tumors were assessed for fusion gene. The detected fusion genes exhibited large intron sequences and the insertion of unknown and previously unreported sequences. The fusion genes were not detected in the 2 malignant cases with high-grade nuclear atypia, nuclear pleomorphism and necrosis, that was confirmed by multiplex PCR method. In addition, 1 of the 2 NAB2-STAT6 fusion gene-negative tumors showed amplification of the MDM2 and CDK4 genes. It was suggested that a certain proportion of tumors previously diagnosed as malignant SFTs with high-grade nuclear atypia lacking NAB2-STAT6 should be categorized into a special subtype of SFT, which is genetically different from conventional SFTs, and which cannot be apparently distinguished from dedifferentiated liposarcoma or undifferentiated pleomorphic sarcoma.
AB - Recently, a novel fusion transcript, NAB2-STAT6, and its variants have also been reported to be specific diagnostic markers for solitary fibrous tumors (SFTs). In this study, we validated the existence of the NAB2-STAT6 fusion gene in SFTs and examined its relation with the pathological features. Frozen samples from 9 tumors were assessed for fusion gene. The detected fusion genes exhibited large intron sequences and the insertion of unknown and previously unreported sequences. The fusion genes were not detected in the 2 malignant cases with high-grade nuclear atypia, nuclear pleomorphism and necrosis, that was confirmed by multiplex PCR method. In addition, 1 of the 2 NAB2-STAT6 fusion gene-negative tumors showed amplification of the MDM2 and CDK4 genes. It was suggested that a certain proportion of tumors previously diagnosed as malignant SFTs with high-grade nuclear atypia lacking NAB2-STAT6 should be categorized into a special subtype of SFT, which is genetically different from conventional SFTs, and which cannot be apparently distinguished from dedifferentiated liposarcoma or undifferentiated pleomorphic sarcoma.
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U2 - 10.1016/j.prp.2014.12.002
DO - 10.1016/j.prp.2014.12.002
M3 - Article
C2 - 25554652
AN - SCOPUS:84977810354
SN - 0344-0338
VL - 211
SP - 117
EP - 124
JO - Pathology Research and Practice
JF - Pathology Research and Practice
IS - 2
ER -