Ameloblastic fibrosarcoma: Clinicopathological and molecular analysis of 7 cases highlighting frequent BRAF and occasional NRAS mutations
Histopathology Jan 10, 2020
Agaimy A, Skalova A, Franchi A, et al. - Researchers reported 7 ameloblastic fibromas (AF) (aggressive odontogenic neoplasm featuring malignant mesenchymal stroma in addition to ameloblastic epithelial component) cases and examined them for the first time for mutations in the BRAF/NRAS pathway. Individuals who participated were four women and three men aged 23 to 57 years. Three tumors developed following one or multiple recurrences of AF (4 to 20 years after initial diagnosis), two exhibited transition from AF-like bland areas and two developed de novo. BRAF/NRAS mutations in AFS was studied for the first time in this work. In view of the activity of RAF and MEK inhibitors over various cancers harboring V600E mutations, researchers robustly propose to had all AFS genetically tested and to clinically examine targeted treatment strategies in this extremely rare sarcoma subtype.
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