ABSTRACT

Cerebellar liponeurocytoma is a rare cerebellar neoplasm of adults with neuronal/neurocytic differentiation, a low proliferative potential, and a favorable prognosis (Kleihues et al., 2000). Given its relatively benign clinical behavior the tumor was assigned a Grade I, II designation on the 2000 WHO’s four-tier biological behavior scale (Kleihues et al., 2000; George and Scheithauer, 2001). This relatively new entity has been included in the group of the mixed neuronal-glial tumors characterized by a variable extent of neuronal and glial differentiation (Burger et al., 2002). Cerebellar liponeurocytoma has been previously classified as lipomatous medulloblastoma, lipidized medulloblastoma (Bechtel et al., 1978; Daron et al., 1993; Soylemezoglu et al., 1996) or medullocytoma (Giangaspero et al., 1996), lipomatous glioneurocytoma (Alleyne et al., 1998), lipidized mature neuroectodermal tumor of the cerebellum with myoid differentiation (Gonzalez-Campora and Wellert, 1998), and neurolipocytoma (Ellison et al., 1993). The new nomenclature eliminates the word ‘medulloblastoma’ and reinforces its benign character, since the label medulloblastoma may lead to unnecessarily aggressive adjuvant therapy (Jackson et al., 2001). After the first 16 cases reported in the 2000 WHO classification, another 10 cases have been described in the last few years (Alkadhi et al., 2001; Jackson et al., 2001; Taddei et al. 2001; Burger et al., 2002; Montagna et al., 2002; Akhaddar et al., 2003; Fung et al., 2003). Some cases of medulloblastoma with lipidized cells have been reported, which cannot share the clinical and pathologic features of cerebellar liponeurocytoma (Giordana et al., 2000).