“ PRRX1 -rearranged mesenchymal tumors”: expanding the immunohistochemical profile and molecular spectrum of a recently described entity with the proposed revision of nomenclature
Virchows Archiv : an international journal of pathology(2023)
摘要
Since the publication of the 2020 World Health Organization classification of soft tissue and bone tumors, the classification of “fibroblastic” tumors has expanded to include a novel subset of tumors characterized by PRRX1 :: NCOA1/2 gene fusions. These tumors defy conventional classification and are morphologically distinct, characterized by a multi-nodular growth of bland spindle cells suspended in a myxo-collagenous stroma with mild cytologic atypia, “staghorn-like” vessels, and variable perivascular hyalinization. Mitotic activity is rare, and necrosis is not identified. Herein, we present six additional cases of PRRX1 -rearranged mesenchymal tumors, including five cases with PRRX1 :: NCOA1 fusion and one case with PRRX1 :: KMT2D fusion. Three cases (3/6, 50%) demonstrated focal co-expression of S100 protein and SOX10, thereby expanding the immunohistochemical profile of this emerging entity. Like prior reported cases, there was no evidence of malignant behavior on short-term follow-up. The novel fusion, PRRX1 :: KMT2D , further expands the molecular spectrum of this entity and leads to a proposed revision of the provisional nomenclature to “ PRRX1 -rearranged mesenchymal tumor” to both accommodate non- NCOA1/2 fusion partners and allow for the possibility of partial neural or neuroectodermal differentiation.
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关键词
Mesenchymal tumor,PRRX1,NCOA1,KMT2D,Spindle cell,Soft tissue
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