Dysembryoplastic neuroepithelial tumour of the cerebellum

Abstract

A case of dysembryoplastic neuroepithelial tumour of the cerebellum occurring in a 28-year-old woman is presented. The lesion extended from the cortex of the inferior vermis upwards into the white matter. Histologically, it exhibited areas of microcystic cerebellar astrocytoma and glial regions with hamartomatous blood vessels as well as areas with oligodendrocyte-like cells (OLC) with a delicate, fibrillary stroma lying in a mucinous, often microcystic matrix. The OLC showed prominent rosette formation and immunohistochemical features suggesting neuronal, i.e. granule cell, differentiation.

A rosette-forming glioneuronal tumor of the spinal cord: the first case of a rosette-forming glioneuronal tumor originating from the spinal cord

“…Initially, an RGNT of the fourth ventricle was reported to be a dysembryoplastic neuroepithelial tumor of the cerebellum [5]. Komori et al [2] analyzed 11 cases that had distinct clinicopathologic features and proposed an RGNT of the fourth ventricle to be an independent type of lesion.…”

Classification and pathogenesis of brain tumors

“…Most are located in the temporal lobe and frequently involve mesial structures (Daumas-Duport 1993). They have also been described in the caudate nucleus (Cervera-Pierot et al 1997), cerebellum (Daumas-Duport et al 1988b;Kuchelmeister et al 1995) and pons (Leung et al 1994). The characteristic histopathological feature is the glioneuronal element composed of small neuronal cells arranged in columns that are frequently oriented at right angles to the cortical surface.…”

“…Previously, all embryonal tumors of the CNS, irrespective of location were regarded as PNETs (Rorke 1983). The 2000 refinement of the classification arose from the recognition that medulloblastomas develop from the dysembryoplastic neuroepithelial tumor involving the cerebellum (Kuchelmeister et al 1995). A larger case series subsequently established the distinctive nature of this neoplasm (Komori et al 2002).…”

The Expanding Family of Glioneuronal Tumors

“…The RGNT of the fourth ventricle was established as a distinct entity in 2002, based on a series of 11 tumors reported to arise in the posterior fossa region by Komori et al [18][19][20] A tumor morphologically resembling this lesion had been previously reported in the cerebellum as a dysembryoplastic neuroepithelial tumor in 1995 by Kuchelmeister et al 21 Similar to the papillary glioneuronal tumor, the incidence of this lesion in the general population is not known because of the limited number of cases reported in the literature. Patients have ranged in age from 12 to 59 years, with a mean age of 31 years.…”