The 'rosette-forming glioneuronal tumor' of the fourth ventricle

Tumors containing both neuronal and glial components are a rare heterogeneous group with unique features that require further subclassification. The rosette-forming glioneuronal tumor of the fourth ventricle is one of a number of recently described glioneuronal tumors, which has been accorded official WHO nosologic status only in 2007. We describe the clinical and pathologic features of two patients with rare rosette-forming glioneuronal tumors of the fourth ventricle, one of which was associated with dysgenetic tricho-rhinopharyngeal type I syndrome.