Synopsis: Gli-1-Altered Tumors, an emerging diagnostic entity (Gli-1) (0:30:51)

Feature Summary of Gli-1-altered Tumors  
1.  A distinct group of mesenchymal neoplasms with low malignant behavior.  
2.  Found in a wide variety of organ sites. 
3.  Multinodular or plexiform growth patterns, and monomorphic round to spindle epithelioid tumor cells.  
4.  Positive for CD56 and S100, but highly variable immunohistochemical stain profile. 
5.  Gli-1 gene fusion involving ACTB, MALAT1, and PTCHI gene partners is characteristic. 
6.  Gli-1 gene amplification accompanied by co-amplification of other genes mapped to the chromosome 12q13 to q15 region.  
7.  Main differential diagnosis include solitary fibrous tumor, glomus tumor, clear cell sarcoma, and follicular dendritic cell sarcoma.

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