All Title Author
Keywords Abstract

PLOS ONE  2014 

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

DOI: 10.1371/journal.pone.0101211

Full-Text   Cite this paper   Add to My Lib


Background and Purpose In 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. Methods PubMed, Embase and Web of Science were searched for peer-reviewed articles reporting on PGNT, RGNT, and GNTNI using predefined keywords. Results 95 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. Conclusions The 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.


[1]  Adachi J, Nishikawa R, Hirose T, Matsutani M (2005) Mixed neuronal-glial tumor of the fourth ventricle and successful treatment of postoperative mutism with bromocriptine: case report. Surg Neurol 63: 375–379. doi: 10.1016/j.surneu.2004.05.039
[2]  Agarwal S, Sharma MC, Singh G, Suri V, Sarkar C, et al. (2012) Papillary glioneuronal tumor - a rare entity: report of four cases and brief review of literature. Childs Nerv Syst 28: 1897–1904. doi: 10.1007/s00381-012-1860-3
[3]  Allende DS, Prayson RA (2009) The expanding family of glioneuronal tumors. Adv Anat Pathol 16: 33–39. doi: 10.1097/pap.0b013e3181915e3b
[4]  Amemiya S, Shibahara J, Aoki S, Takao H, Ohtomo K (2008) Recently established entities of central nervous system tumors: review of radiological findings. J Comput Assist Tomogr 32: 279–285. doi: 10.1097/rct.0b013e31814ce981
[5]  Anan M, Inoue R, Ishii K, Abe T, Fujiki M, et al. (2009) A rosette-forming glioneuronal tumor of the spinal cord: the first case of a rosette-forming glioneuronal tumor originating from the spinal cord. Hum Pathol 40: 898–901. doi: 10.1016/j.humpath.2008.11.010
[6]  Atri S, Sharma MC, Sarkar C, Garg A, Suri A (2007) Papillary glioneuronal tumour: a report of a rare case and review of literature. Childs Nerv Syst 23: 349–353. doi: 10.1007/s00381-006-0196-2
[7]  Barnes NP, Pollock JR, Harding B, Hayward RD (2002) Papillary glioneuronal tumour in a 4-year-old. Pediatr Neurosurg 36: 266–270. doi: 10.1159/000058431
[8]  Benesch M, Eder HG, Sovinz P, Raith J, Lackner H, et al. (2006) Residual or recurrent cerebellar low-grade glioma in children after tumor resection: is re-treatment needed? A single center experience from 1983 to 2003. Pediatr Neurosurg 42: 159–164. doi: 10.1159/000091859
[9]  Bisson EF, Pendlebury WW, Horgan MA (2005) Glioneuronal tumor with unique imaging and histologic features. J Neurooncol 72: 89–90. doi: 10.1007/s11060-004-2277-y
[10]  Bouvier-Labit C, Daniel L, Dufour H, Grisoli F, Figarella-Branger D (2000) Papillary glioneuronal tumour: clinicopathological and biochemical study of one case with 7-year follow up. Acta Neuropathol 99: 321–326. doi: 10.1007/pl00007445
[11]  Broholm H, Madsen FF, Wagner AA, Laursen H (2002) Papillary glioneuronal tumor - a new tumor entity. Clin Neuropathol 21: 1–4.
[12]  Buccoliero AM, Castiglione F, Degl’innocenti DR, Moncini D, Paglierani M, et al. (2012) Glioneuronal tumor with neuropil-like islands: clinical, morphologic, immunohistochemical, and molecular features of three pediatric cases. Ped Dev Pathol 15: 352–360. doi: 10.2350/12-01-1147-oa.1
[13]  Celli P, Caroli E, Giangaspero F, Ferrante L (2006) Papillary glioneuronal tumor. Case report and literature review. J Neurooncol 80: 185–189. doi: 10.1007/s11060-006-9170-9
[14]  Collett D (2003) Modelling survival data in medical research. 2nd edition ed. London: Chapman and Hall.
[15]  Ellezam B, Theeler BJ, Luthra R, Adesina AM, Aldape KD, et al. (2012) Recurrent PIK3CA mutations in rosette-forming glioneuronal tumor. Acta neuropathol 123: 285–287. doi: 10.1007/s00401-011-0886-z
[16]  Epelbaum S, Kujas M, Van Effenterre R, Poirier J (2006) Two cases of papillary glioneuronal tumours. Br J Neurosurg 20: 90–93. doi: 10.1080/02688690600682465
[17]  Fraum TJ, Barak S, Pack S, Lonser RR, Fine HA, et al. (2012) Spinal cord glioneuronal tumor with neuropil-like islands with 1p/19q deletion in an adult with low-grade cerebral oligodendroglioma. J Neurooncol 107: 421–426. doi: 10.1007/s11060-011-0760-9
[18]  Friedrich C, Müller K, von Hoff K, Kwiecien R, Rutkowski S, et al. (2014) Adults with CNS primitive neuroectodermal tumors/pineoblastomas: results of multimodal treatment according to the pediatric HIT 2000 protocol. J Neurooncol 116: 567–575. doi: 10.1007/s11060-013-1327-8
[19]  Frydenberg E, Laherty R, Rodriguez M, Ow-Yang M, Steel T (2010) A rosette-forming glioneuronal tumour of the pineal gland. J Clin Neurosci 17: 1326–1328. doi: 10.1016/j.jocn.2010.03.003
[20]  Gelpi E, Preusser M, Czech T, Slavc I, Prayer D, et al. (2007) Papillary glioneuronal tumor. Neuropathology 27: 468–473. doi: 10.1111/j.1440-1789.2007.00802.x
[21]  Harris BT, Horoupian DS (2000) Spinal cord glioneuronal tumor with “rosette” neuropil islands and meningeal dissemination: a case report. Acta neuropathol 100: 575–579. doi: 10.1007/s004010000217
[22]  Ishizawa T, Komori T, Shibahara J, Ishizawa K, Adachi J, et al. (2006) Papillary glioneuronal tumor with minigemistocytic components and increased proliferative activity. Hum Pathol 37: 627–630. doi: 10.1016/j.humpath.2005.12.014
[23]  Javahery RJ, Davidson L, Fangusaro J, Finlay JL, Gonzalez-Gomez I, et al. (2009) Aggressive variant of a papillary glioneuronal tumor. Report of 2 cases. J Neurosurg Pediatr 3: 46–52. doi: 10.3171/2008.10.peds08242
[24]  Komori T, Scheithauer BW, Anthony DC, Rosenblum MK, McLendon RE, et al. (1998) Papillary glioneuronal tumor: a new variant of mixed neuronal-glial neoplasm. Am J Surg Pathol 22: 1171–1183. doi: 10.1097/00000478-199810000-00002
[25]  Komori T, Scheithauer BW, Hirose T (2002) A rosette-forming glioneuronal tumor of the fourth ventricle: infratentorial form of dysembryoplastic neuroepithelial tumor? Am J Surg Pathol 26: 582–591. doi: 10.1097/00000478-200205000-00004
[26]  Kuchelmeister K, Demirel T, Schlorer E, Bergmann M, Gullotta F (1995) Dysembryoplastic neuroepithelial tumour of the cerebellum. Acta neuropathol 89: 385–390. doi: 10.1007/bf00309634
[27]  Lamszus K, Makrigeorgi-Butera M, Laas R, Westphal M, Stavrou D (2003) September 2002: 24-year-old female with a 6-month history of seizures. Brain Pathol 13: 115–117.
[28]  Lavrnic S, Macvanski M, Ristic-Balos D, Gavrilov M, Damjanovic D, et al. (2012) Papillary glioneuronal tumor: unexplored entity. J Neurol Surg A Cent Eur Neurosurg 73: 224–229. doi: 10.1055/s-0032-1313636
[29]  Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, et al. (2007) The 2007 WHO classification of tumours of the central nervous system. Acta neuropathol 114: 97–109. doi: 10.1007/s00401-007-0243-4
[30]  Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, et al. (2007) WHO Classification of tumours of the central nervous system. Lyon, IARC Press.
[31]  Lu JQ, Scheithauer BW, Sharma P, Scott JN, Parney IF, et al. (2009) Multifocal complex glioneuronal tumor in an elderly man: an autopsy study: case report. Neurosurgery 64: E1193–1195. doi: 10.1227/01.neu.0000345640.40566.48
[32]  Malats N, Bustos A, Nascimento CM, Fernandez F, Rivas M, et al. (2005) P53 as a prognostic marker for bladder cancer: a meta-analysis and review. Lancet Oncol 6: 678–686. doi: 10.1016/s1470-2045(05)70315-6
[33]  Mazloom A, Hodges JC, Teh BS, Chintagumpala M, Paulino AC (2012) Outcome of patients with pilocytic astrocytoma and leptomeningeal dissemination. Int J Radiat Oncol Biol Phys 84: 350–354. doi: 10.1016/j.ijrobp.2011.12.044
[34]  Moher D, Liberati A, Tetzlaff J, Altman DG (2009) Group P (2009) Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. J Clin Epidemiol 62: 1006–1012. doi: 10.1016/j.jclinepi.2009.06.005
[35]  Newton HB, Dalton J, Ray-Chaudhury A, Gahbauer R, McGregor J (2008) Aggressive papillary glioneuronal tumor: case report and literature review. Clin Neuropathol 27: 317–324. doi: 10.5414/npp27317
[36]  Peduzzi P, Concato J, Feinstein AR, Holford TR (1995) Importance of events per independent variable in proportional hazards regression analysis. II. Accuracy and precision of regression estimates. J Clin Epidemiol 48: 1503–1510. doi: 10.1016/0895-4356(95)00048-8
[37]  Poliani PL, Sperli D, Valentini S, Armentano A, Bercich L, et al. (2009) Spinal glioneuronal tumor with neuropil-like islands and meningeal dissemination: histopathological and radiological study of a pediatric case. Neuropathology 29: 574–578. doi: 10.1111/j.1440-1789.2008.00988.x
[38]  Prayson RA (2000) Papillary glioneuronal tumor. Arch Path Lab Med 124: 1820–1823.
[39]  Rainov NG, Wagner T, Heidecke V (2010) Rosette-forming glioneuronal tumor of the fourth ventricle. Cent Eur Neurosurg 71: 219–221. doi: 10.1055/s-0029-1242760
[40]  Rosenblum MK (2007) The 2007 WHO Classification of Nervous System Tumors: newly recognized members of the mixed glioneuronal group. Brain Pathol 17: 308–313. doi: 10.1111/j.1750-3639.2007.00079.x
[41]  Ruppert B, Welsh CT, Hannah J, Giglio P, Rumboldt Z, et al. (2011) Glioneuronal tumor with neuropil-like islands of the spinal cord with diffuse leptomeningeal neuraxis dissemination. J Neurooncol 104: 529–533. doi: 10.1007/s11060-010-0505-1
[42]  Scheithauer BW, Silva AI, Ketterling RP, Pula JH, Lininger JF, et al. (2009) Rosette-forming glioneuronal tumor: report of a chiasmal-optic nerve example in neurofibromatosis type 1: special pathology report. Neurosurgery 64: E771–772. doi: 10.1227/01.neu.0000340979.81362.f3
[43]  Shah MN, Leonard JR, Perry A (2010) Rosette-forming glioneuronal tumors of the posterior fossa. J Neurosurg Pediatr 5: 98–103. doi: 10.3171/2009.7.peds09113
[44]  Simmonds MC, Higgins JP, Stewart LA, Tierney JF, Clarke MJ, et al. (2005) Meta-analysis of individual patient data from randomized trials: a review of methods used in practice. Clin Trials 2: 209–217. doi: 10.1191/1740774505cn087oa
[45]  Solis OE, Mehta RI, Lai A, Mehta RI, Farchoukh LO, et al. (2011) Rosette-forming glioneuronal tumor: a pineal region case with IDH1 and IDH2 mutation analyses and literature review of 43 cases. J Neurooncol 102: 477–484. doi: 10.1007/s11060-010-0335-1
[46]  Teo JG, Gultekin SH, Bilsky M, Gutin P, Rosenblum MK (1999) A distinctive glioneuronal tumor of the adult cerebrum with neuropil-like (including “rosette”) islands: report of 4 cases. Am J Surg Pathol 23: 502–510. doi: 10.1097/00000478-199905000-00002
[47]  Vaquero J, Coca S (2007) Atypical papillary glioneuronal tumor. J Neurooncol 83: 319–323. doi: 10.1007/s11060-007-9333-3
[48]  Wang Y, Xiong J, Chu SG, Liu Y, Cheng HX, et al. (2009) Rosette-forming glioneuronal tumor: report of an unusual case with intraventricular dissemination. Acta neuropathol 118: 813–819. doi: 10.1007/s00401-009-0569-1
[49]  Williams SR, Joos BW, Parker JC, Parker JR (2008) Papillary glioneuronal tumor: a case report and review of the literature. Ann Clin Lab Sci 38: 287–292.
[50]  Xiao H, Ma L, Lou X, Gui Q (2011) Papillary glioneuronal tumor: radiological evidence of a newly established tumor entity. J Neuroimaging 21: 297–302. doi: 10.1111/j.1552-6569.2010.00478.x
[51]  Xiong J, Liu Y, Chu SG, Chen H, Chen HX, et al. (2012) Rosette-forming glioneuronal tumor of the septum pellucidum with extension to the supratentorial ventricles: rare case with genetic analysis. Neuropathology 32: 301–305. doi: 10.1111/j.1440-1789.2011.01261.x
[52]  Zhang J, Babu R, McLendon RE, Friedman AH, Adamson C (2013) A comprehensive analysis of 41 patients with rosette-forming glioneuronal tumors of the fourth ventricle. J Clin Neurosci 20: 335–341. doi: 10.1016/j.jocn.2012.09.003
[53]  Zwiener I, Blettner M, Hommel G (2011) Survival analysis: part 15 of a series on evaluation of scientific publications. Dtsch Arztebl Int 108: 163–169.


comments powered by Disqus