This report documents the first case of a conventional (bone-type) giant cell tumor of the larynx, in which the diagnosis was confirmed by molecular genetic analysis. A 50-year-old non-smoking man experienced progressive hoarseness lasting for 3 months. Imaging showed a 40-mm tumor arising from the right thyroid cartilage. The total laryngectomy was performed. Grossly, the tumor was solid and whitish, with areas of hemorrhage. Microscopically, the tumor consisted of a biphasic population with mononuclear cells with round to oval nuclei, small nucleoli, and pale eosinophilic cytoplasm admixed with evenly distributed dispersed osteoclast-like giant cells. Immunohistochemically, the neoplastic mononuclear cells expressed diffusely vimentin and p63 and focally SATB2. Admixed mononuclear histiocytes coexpressed CD68 and CD163, while the osteoclast-like giant cells showed only CD68 expression. Most importantly, all mononuclear tumor cells showed strong nuclear expression of anti-histone H3.3 G34W antibody. Subsequent next-generation sequencing confirmed the missense mutation of gene H3-3A: c.103G>T (p.Gly35Trp).

Comprehensive Cancer Center European Metropolitan Area Erlangen Nuremberg Friedrich Alexander University of Erlangen Nuremberg Erlangen Germany

Department of Otorhinolaryngology and Head and Neck Surgery Charles University Faculty of Medicine in Hradec Kralove Hradec Kralove Czech Republic

Department of Otorhinolaryngology and Head and Neck Surgery University Hospital Hradec Kralove Hradec Kralove Czech Republic

Department of Radiology University Hospital Hradec Kralove Hradec Kralove Czech Republic

Institute of Pathology Erlangen University Hospital Friedrich Alexander University of Erlangen Nuremberg Erlangen Germany

The Fingerland Department of Pathology Charles University Faculty of Medicine in Hradec Kralove Hradec Kralove Czech Republic

The Fingerland Department of Pathology University Hospital Hradec Kralove Sokolska 581 500 05 Hradec Kralove Czech Republic

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