Nasal chondromesenchymal hamartoma: Case report

We report the case of an 18-month-old boy with a large heterogeneous and lobulated soft-tissue mass, filling completely the right nasal cavity and anterior ethmoid, displacing the nasal septum, eroding the lamina papyracea, compressing the orbit and extending to the endocranium. There were multiple intralesional foci of calcification. The tumor was completely removed via an endonasal endoscopic approach. The definitive histopathologic diagnosis was that of a nasal chondromesenchymal hamartoma (NCMH). This is an exceedingly rare benign tumor of the upper respiratory tract that shares similarities to the mesenchymal hamartoma arising from the chest wall in neonates. Moreover as some NCMHs are associated with a “pleuropulmonary blastoma”, they can be part of the heredofamilial disease complex associated with PPB and linked to mutations of gene DICER1.