Arch Clin Med Case Rep 2020; 4 (4): 596-611 DOI: 10.26502/acmcr.96550235 Archives of Clinical and Medical Case Reports 596 Case Report Meningeal Melanocytoma - Focus on Molecular Aspects with 3 New Molecular Alterations: A Literature Review and Report of Two Cases Axel de BERNARDI 1 , Maureen BERNADACH 1,3 , Judith PASSILDAS-JAHANMOHAN 1-4* , Julian BIAU 1,2,6 , Jean-Louis KEMENY 7 , Xavier DURANDO 1-4 1 Centre Jean Perrin, 58 rue Montalembert F-63011 Clermont-Ferrand, France 2 Université Clermont Auvergne, Centre Jean Perrin, INSERM, U1240 Imagerie Moléculaire et Stratégies 3 Théranostiques, 58 rue Montalembert F-63000 Clermont-Ferrand, France 4 Division de Recherche Clinique, Délégation Recherche Clinique et Innovation, Centre Jean Perrin, 58 rue Montalembert F-63011 Clermont-Ferrand, France 5 Centre d'Investigation Clinique, UMR501, 63011, Clermont-Ferrand, France 6 Département de Radiothérapie, Centre Jean PERRIN, 63011, Clermont-Ferrand, France 7 CHU de Clermont-Ferrand, Service d’Anatomie et de cytologie pathologique, 58 rue Montalembert 63000 Clermont-Ferrand, France * Corresponding Author: Dr. Judith PASSILDAS-JAHANMOHAN, CLCC Centre Jean Perrin, Division de Recherche Clinique 58, rue Montalembert, BP 392, 63011 Clermont-Ferrand Cedex 1, France, Tel: +33463663337 (or) +33695841465; Fax: +33473278029; E-mail: Judith.PASSILDAS@clermont.unicancer.fr Received: 22 April 2020; Accepted: 04 May 2020; Published: 03 July 2020 Abstract Background: Meningeal melanocytoma (MM) is a locally aggressive, low-grade primary melanocytic tumor of the central nervous system (PMN-CNS). According to the literature, GNAQ and GNA11 mutations are relatively frequent. In contrast, BRAF and NRAS mutations are very rare. Other series have reported BAP1 mutation, monosomy 3 and gain of chromosome 6. In this paper, we discussed two cases of MM for whom a FoundationOne CDx TM assay was performed. Results: Case report 1: A 48 year old man was diagnosed with intermediate-grade MM based on histological assessment, which is consistent with the clinical evolution. The FoundationOne CDx TM assay detected a SF3B1