AI Article Synopsis
- * Although NF is typically non-cancerous, there's rare evidence of it turning malignant, with one documented case associated with the PPP6R3-USP6 fusion.
- * This report describes a new case of NF that exhibited aggressive growth and local invasion, with genetic analysis confirming the PPP6R3-USP6 fusion, marking it as a potential second instance of malignant NF.
Article 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.
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| http://dx.doi.org/10.1111/pin.12851 | DOI Listing |
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