An Individual Patient Data Meta-Analysis on Characteristics and Outcome of Patients with Papillary Glioneuronal Tumor, Rosette Glioneuronal Tumor with Neuropil-Like Islands and Rosette Forming Glioneuronal Tumor of the Fourth Ventricle

Abstract

Background and PurposeIn 2007, the WHO classification of brain tumors was extended by three new entities of glioneuronal tumors: papillary glioneuronal tumor (PGNT), rosette-forming glioneuronal tumor of the fourth ventricle (RGNT) and glioneuronal tumor with neuropil-like islands (GNTNI). Focusing on clinical characteristics and outcome, the authors performed a comprehensive individual patient data (IPD) meta-analysis of the cases reported in literature until December 2012.MethodsPubMed, Embase and Web of Science were searched for peer-reviewed articles reporting on PGNT, RGNT, and GNTNI using predefined keywords.Results95 publications reported on 182 patients (PGNT, 71; GNTNI, 26; RGNT, 85). Median age at diagnosis was 23 years (range 4–75) for PGNT, 27 years (range 6–79) for RGNT, and 40 years (range 2–65) for GNTNI. Ninety-seven percent of PGNT and 69% of GNTNI were located in the supratentorial region, 23% of GNTNI were in the spinal cord, and 80% of RGNT were localized in the posterior fossa. Complete resection was reported in 52 PGNT (73%), 36 RGNT (42%), and 7 GNTNI (27%) patients. Eight PGNT, 3 RGNT, and 12 GNTNI patients were treated with chemo- and/or radiotherapy as the primary postoperative treatment. Follow-up data were available for 132 cases. After a median follow-up time of 1.5 years (range 0.2–25) across all patients, 1.5-year progression-free survival rates were 52±12% for GNTNI, 86±5% for PGNT, and 100% for RGNT. The 1.5-year overall-survival were 95±5%, 98±2%, and 100%, respectively.ConclusionsThe clinical understanding of the three new entities of glioneuronal tumors, PGNT, RGNT and GNTNI, is currently emerging. The present meta-analysis will hopefully contribute to a delineation of their diagnostic, therapeutic, and prognostic profiles. However, the available data do not provide a solid basis to define the optimum treatment approach. Hence, a central register should be established.

Epilepsy-associated tumours: what epileptologists should know about neuropathology, terminology, and classification systems

“…Papillary glioneuronal tumour (PGNT), rosette‐forming glioneuronal tumour (RGNT), glioneuronal tumour with neuropil‐like islands (GNTNI), and angiocentric neuroepithelial tumour (ANET) have been recognised only very recently as distinct tumours with benign behaviour (Louis et al ., ), and represent even more rare entities (Williams et al ., ; Shakur et al ., ; Takada et al ., ; Agarwal et al ., ; Alexandru et al ., ; Demetriades et al ., ; Schlamann et al ., ; Ni et al ., ).…”

FGFR- gene family alterations in low-grade neuroepithelial tumors

“…While in recent studies FGFR1 hotspot mutations were invariably detected among RGNTs [66], their presence is not currently required for the diagnosis, (and as previously discussed, is certainly not unique to RGNT). Moreover, while RGNT corresponds histologically to WHO grade I and is generally considered benign, dissemination and progression have been reported in rare instances [1,2,62,68,74]. Of note, frequent comutation with PIK3CA as well as NF1 have been reported in RGNT [66].…”

Gliom Disi Intra-Aksiyal Beyin Tumorleri

“…Nadiren tümör içi kanama ve peritümöral ödem gösterebilen tümör, frontal ve temporal lobları sıklıkla tutarken, subependimal ve intraventriküler yerleşimde gösterebilir [10]. Tipik olarak kistik, solid veya kist içinde opaklaşan mural nodül şeklinde izlenen tümör (Resim 4), sıklıkla ganglioglioma olarak raporlanır ve ayırıcı tanısını sağlayan belirgin bir radyolojik ölçüt yoktur [10][11][12]. Cerrahi olarak çıkarılan tümörlerde progresyon ve rekürrens nadirdir [11].…”