Rosette-forming glioneuronal tumor of the fourth ventricle: Report of two cases with a differential diagnostic overview

Vajtai, Istvan; Arnold, Marlène; Kappeler, Andreas; Jeless, Olivia; Lukes, Anton; Mariani, Luigi; Paulus, Werner (2007). Rosette-forming glioneuronal tumor of the fourth ventricle: Report of two cases with a differential diagnostic overview. Pathology, research and practice, 203(8), pp. 613-9. München: Elsevier 10.1016/j.prp.2007.04.009

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We report on clinicopathological findings in two cases of rosette-forming glioneuronal tumor of the fourth ventricle (RGNT) occurring in females aged 16 years (Case 1) and 30 years (Case 2). Symptoms included vertigo, nausea, cerebellar ataxia, as well as headaches, and had been present for 4-months and 1 week, respectively. Magnetic resonance imaging (MRI) indicated a cerebellar-based tumor of 1.8cm (Case 1) and 5cm (Case 2) diameter each, bulging into the fourth ventricle. Case 2 involved a cyst-mural-nodule configuration. In both instances, the solid component appeared isointense on T(1) sequences, hyperintense in the T(2) mode, and enhanced moderately. Gross total resection was achieved via suboccipital craniotomy. However, functional recovery was disappointing in Case 1. On microscopy, both tumors comprised an admixture of low-grade astrocytoma interspersed with circular aggregates of synaptophysin-expressing round cells harboring oligodendrocyte-like nuclei. The astrocytic moiety in Case 1 was nondescript, and overtly pilocytic in Case 2. The architecture of neuronal elements variously consisted of neurocytic rosettes, of pseudorosettes centered on a capillary core, as well as of concentric ribbons along irregular lumina. Gangliocytic maturation, especially "floating neurons", or a corresponding immunoreactivity for neurofilament protein was absent. Neither of these populations exhibited atypia, mitotic activity, or a significant labeling for MIB-1. Cerebellar parenchyma included in the surgical specimen did not reveal any preexisting malformative anomaly. Despite sharing some overlapping histologic traits with dysembryoplastic neuroepithelial tumor (DNT), the presentation of RGNT with respect to both patient age and location is consistent enough for this lesion to be singled out as an autonomous entity.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Service Sector > Institute of Pathology
04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurosurgery

UniBE Contributor:

Vajtai, Istvan; Arnold, Marlene; Kappeler, Andreas; Lukes, Anton and Mariani, Luigi

ISSN:

0344-0338

ISBN:

17651910

Publisher:

Elsevier

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:53

Last Modified:

04 May 2014 23:15

Publisher DOI:

10.1016/j.prp.2007.04.009

PubMed ID:

17651910

Web of Science ID:

000249092000008

URI:

https://boris.unibe.ch/id/eprint/22639 (FactScience: 35693)

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