Abstract:
:Nodular fasciitis (NF) is a self-limiting benign disease that is characterized by rapid proliferation of fibroblastic and myofibroblastic cells. The characteristic gene fusion containing the USP6 gene is a genetic hallmark of NF and MYH9-USP6 is the most frequent fusion, suggesting that NF is not a reactive condition but a neoplastic disease. Malignant transformation of NF has been reported rarely as a single case associated with the PPP6R3-USP6 fusion. Here we report a case of soft part tumor of which the histological feature was a typical NF but showed aggressive and non-regressing growth with local invasion. Targeted RNA sequencing and fluorescence in situ hybridization analysis identified PPP6R3-USP6 with gene amplification. These findings indicate that the present case is the second case of malignant NF, and we suggest potential malignant transformation in certain NF cases.
journal_name
Pathol Intjournal_title
Pathology internationalauthors
Teramura Y,Yamazaki Y,Tanaka M,Sugiura Y,Takazawa Y,Takeuchi K,Nakayama T,Kaneko T,Musha Y,Funauchi Y,Ae K,Matsumoto S,Nakamura Tdoi
10.1111/pin.12851subject
Has Abstractpub_date
2019-12-01 00:00:00pages
706-709issue
12eissn
1320-5463issn
1440-1827journal_volume
69pub_type
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