Metastatic Rhabdoid Meningioma with BRAF V600E Mutation and Good Response to Personalized Therapy: Case Report and Review of the Literature. (3rd April 2015)

• Record Type: Journal Article
• Authors: Mordechai, Oz; Postovsky, Sergey; Vlodavsky, Eugene; Eran, Ayelet; Constantini, Shlomi; Dotan, Eynat; Cagnano, Emmanuela; Weyl-Ben-Arush, Myriam
• Is Part Of: Pediatric hematology and oncology. Volume 32:Number 3(2015:Sep.)
• Page Start: 207
• Page End: 211
• Publication Date: 2015-04-03
• Subjects: BRAF mutation -- MEK inhibitor -- meningioma -- targeted therapy; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Hematologic Diseases -- Child; Hematologic Diseases -- Infant; Neoplasms -- Child; 618.9215
• DOI: 10.3109/08880018.2014.936058
• Languages: English
• ISSNs: 0888-0018

Abstract

Abstract : Rhabdoid meningioma is an aggressive phenotype of meningioma, associated with a poor prognosis. We present a very rare case of high-grade meningioma with rhabdoid features that eventually expressed in a coma state. Comprehensive genomic profiling using a Next Generation Sequencing (NGS) assay revealed three genomic alterations: activating BRAF mutation (V600E), loss of CDKN2A/2B, and APC I1307K. After treatment with BRAF inhibitor (dabrafenib), the child's clinical condition improved progressively. After seven months, an MEK inhibitor was added (trametinib).