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Glioneuronal and Neuronal Tumors of the Central Nervous System

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Human Brain and Spinal Cord Tumors: From Bench to Bedside. Volume 2

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 1405))

Abstract

Glioneuronal and neuronal tumors (GNTs) are rare neoplasms composed of neural and glial elements frequently located in the temporal lobe. Epilepsy is the main symptom and diagnosis mostly occurs before adulthood. The great majority of GNTs are WHO grade I tumors, but anaplastic transformations and forms exist. Their common association with focal cortical dysplasia is well recognized and should be taken into consideration during neurophysiological presurgical and surgical planning since the aim of surgery should be the removal of the tumor and of the entire epileptogenic zone according to anatomo-electrophysiological findings. Surgery still remains the cornerstone of symptomatic GNT, while radiotherapy, chemotherapy, and new target therapies are generally reserved for anaplastic, unresectable, or evolving tumors. Furthermore, since many GNTs show overlapping clinical and neuroradiological features, the definition of specific histopathological, genetic, and molecular characteristics is crucial. Epileptological, oncological, neurosurgical, and pathological issues of these tumors make a multidisciplinary management mandatory.

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References

  • Adachi Y, Yagishita A (2008) Gangliogliomas: characteristic imaging findings and role in the temporal lobe epilepsy. Neuroradiology 50:829–834. https://doi.org/10.1007/s00234-008-0410-x

    Article  CAS  PubMed  Google Scholar 

  • Anan M, Inoue R, Ishii K, Abe T, Fujiki M, Kobayashi H, Goya T, Nakazato Y (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. https://doi.org/10.1016/j.humpath.2008.11.010

    Article  PubMed  Google Scholar 

  • Aronica E, Leenstra S, van Veelen CW, van Rijen PC, Hulsebos TJ, Tersmette AC, Yankaya B, Troost D (2001) Glioneuronal tumors and medically intractable epilepsy: a clinical study with long-term follow-up of seizure outcome after surgery. Epilepsy Res 43:179–191. https://doi.org/10.1016/S0920-1211(00)00208-4

    Article  CAS  PubMed  Google Scholar 

  • Babini M, Giulioni M, Galassi E, Marucci G, Martinoni M, Rubboli G, Volpi L, Zucchelli M, Nicolini F, Marliani AF, Michelucci R, Calbucci F (2013) Seizure outcome of surgical treatment of focal epilepsy associated with low-grade tumors in children: Clinical article. J Neurosurg Pediatr 11:214–223. https://doi.org/10.3171/2012.11.PEDS12137

    Article  PubMed  Google Scholar 

  • Baisden BL, Brat DJ, Melhem ER, Rosenblum MK, King AP, Burger PC (2001) Dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: a lesion often misdiagnosed as glioma: report of 10 cases. Am J Surg Pathol 25:494–499. https://doi.org/10.1097/00000478-200104000-00009

    Article  CAS  PubMed  Google Scholar 

  • Bautista F (2014) Vemurafenib in pediatric patients with BRAFV600E mutated high-grade gliomas. Pediatr Blood Cancer 61:1101–1103. https://doi.org/10.1002/pbc

    Article  CAS  PubMed  Google Scholar 

  • Becker AJ, Blümcke I, Urbach H, Hans V, Majores M (2006) Molecular neuropathology of epilepsy-associated glioneuronal malformations. J Neuropathol Exp Neurol 65:99–108

    Article  CAS  PubMed  Google Scholar 

  • Behling F, Schittenhelm J (2019) Oncogenic BRAF alterations and their role in brain tumors. Cancers 11:1–16. https://doi.org/10.3390/cancers11060794

    Article  CAS  Google Scholar 

  • Beland B, Tsang RY, Sutherland G (2018) Unprecedented response to combination BRAF and MEK inhibitors in adult anaplastic ganglioglioma. J Neurooncol 137:667–669. https://doi.org/10.1007/s11060-018-2760-5

    Article  CAS  PubMed  Google Scholar 

  • Blessing MM, Blackburn PR, Balcom JR, Krishnan C, Harrod VL, Zimmermann MT, Barr Fritcher EG, Zysk CD, Jackson RA, Nair AA, Jenkins RB, Halling KC, Kipp BR, Ida CM (2018) Novel BRAF alteration in desmoplastic infantile ganglioglioma with response to targeted therapy. Acta Neuropathol Commun 6:118. https://doi.org/10.1186/s40478-018-0622-1

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Blümcke I, Aronica E, Becker A, Capper D, Coras R, Honavar M, Jacques TS, Kobow K, Miyata H, Mühlebner A, Pimentel J, Söylemezoglu F, Thom M (2016) Low-grade epilepsy-associated neuroepithelial tumours-the 2016 WHO classification. Nat Rev Neurol 12:732–740. https://doi.org/10.1038/nrneurol.2016.173

    Article  PubMed  Google Scholar 

  • Blumcke I, Spreafico R, Haaker G, Coras R, Kobow K, Bien CG, Pfäfflin M, Elger C, Widman G, Schramm J, Becker A, Braun KP, Leijten F, Baayen JC, Aronica E, Chassoux F, Hamer H, Stefan H, Rössler K, Thom M, Walker MC, Sisodiya SM, Duncan JS, McEvoy AW, Pieper T, Holthausen H, Kudernatsch M, Meencke HJ, Kahane P, Schulze-Bonhage A, Zentner J, Heiland DH, Urbach H, Steinhoff BJ, Bast T, Tassi L, Lo Russo G, Özkara C, Oz B, Krsek P, Vogelgesang S, Runge U, Lerche H, Weber Y, Honavar M, Pimentel J, Arzimanoglou A, Ulate-Campos A, Noachtar S, Hartl E, Schijns O, Guerrini R, Barba C, Jacques TS, Cross JH, Feucht M, Mühlebner A, Grunwald T, Trinka E, Winkler PA, Gil-Nagel A, Toledano Delgado R, Mayer T, Lutz M, Zountsas B, Garganis K, Rosenow F, Hermsen A, von Oertzen TJ, Diepgen TL, Avanzini G, EEBB Consortium (2017) Histopathological findings in brain tissue obtained during epilepsy surgery. N Engl J Med 377:1648–1656. https://doi.org/10.1056/NEJMoa1703784

  • Blümcke I, Wiestler OD (2002) Gangliogliomas: an intriguing tumor entity associated with focal epilepsies. J Neuropathol Exp Neurol 61:575–584

    Article  PubMed  Google Scholar 

  • Brastianos PK, Shankar GM, Gill CM, Taylor-Weiner A, Nayyar N, Panka DJ, Sullivan RJ, Frederick DT, Abedalthagafi M, Jones PS, Dunn IF, Nahed B V., Romero JM, Louis DN, Getz G, Cahill DP, Santagata S, Curry WT, Barker FG (2016) Dramatic response of BRAF V600E mutant papillary craniopharyngioma to targeted therapy. J Natl Cancer Inst 108. https://doi.org/10.1093/jnci/djv310

  • Bonney PA, Glenn CA, Ebeling PA et al (2015) Literature review seizure freedom rates and prognostic indicators after resection of gangliogliomas: a review. World Neurosurg 84:1988–1996

    Article  PubMed  Google Scholar 

  • Campos AR, Clusmann H, Von Lehe M, Niehusmann P, Becker AJ, Schramm J, Urbach H (2009) Simple and complex dysembryoplastic neuroepithelial tumors (DNT) variants: clinical profile, MRI, and histopathology. Neuroradiology 51:433–443. https://doi.org/10.1007/s00234-009-0511-1

    Article  PubMed  Google Scholar 

  • Cataltepe O, Turanli G, Yalnizoglu D, Topçu M, Akalan N (2005) Surgical management of temporal lobe tumor-related epilepsy in children. J Neurosurg 102:280–287. https://doi.org/10.3171/ped.2005.102.3.0280

    Article  PubMed  Google Scholar 

  • Chamberlain MC (2013) Salvage therapy with BRAF inhibitors for recurrent pleomorphic xanthoastrocytoma: a retrospective case series. J Neurooncol 114:237–240. https://doi.org/10.1007/s11060-013-1176-5

    Article  CAS  PubMed  Google Scholar 

  • Chen W, Kong Z, Fu J, Zhao D, Wang R, Ma W, Wang Y (2020) Diffuse leptomeningeal glioneuronal tumour (DLGNT) with hydrocephalus as an initial symptom: a case-based update. Child’s Nerv Syst

    Google Scholar 

  • Clusmann H, Schramm J, Kral T, Helmstaedter C, Ostertun B, Fimmers R, Haun D, Elger CE (2002) Prognostic factors and outcome after different types of resection for temporal lobe epilepsy. J Neurosurg 97:1131–1141. https://doi.org/10.3171/jns.2002.97.5.1131

    Article  PubMed  Google Scholar 

  • Del Bufalo F, Carai A, Fig-Talamanca LS, Pettorini B, Mallucci C, Giangaspero F, Antonelli M, Badiali M, Moi L, Bianco G, Cacchione A, Locatelli F, Ferretti E, Mastronuzzi A (2014) Response of recurrent BRAFV600E mutated ganglioglioma to vemurafenib as single agent. J Transl Med 12:1–7. https://doi.org/10.1186/s12967-014-0356-1

    Article  CAS  Google Scholar 

  • Deng MY, Sill M, Chiang J, Schittenhelm J, Ebinger M, Schuhmann MU, Monoranu CM, Milde T, Wittmann A, Hartmann C, Sommer C, Paulus W, Gärtner J, Brück W, Rüdiger T, Leipold A, Jaunmuktane Z, Brandner S, Giangaspero F, Nozza P, Mora J, Morales la Madrid A, Cruz Martinez O, Hansford JR, Pietsch T, Tietze A, Hernáiz-Driever P, Stoler I, Capper D, Korshunov A, Ellison DW, von Deimling A, Pfister SM, Sahm F, Jones DTW (2018) Molecularly defined diffuse leptomeningeal glioneuronal tumor (DLGNT) comprises two subgroups with distinct clinical and genetic features. Acta Neuropathol 136:239–253. https://doi.org/10.1007/s00401-018-1865-4

    Article  CAS  PubMed  Google Scholar 

  • Devaux B, Chassoux F, Landré E, Turak B, Laurent A, Zanello M, Mellerio C, Varlet P (2017) Surgery for dysembryoplastic neuroepithelial tumors and gangliogliomas in eloquent areas. Functional results and seizure control. Neurochirurgie 63:227–234. https://doi.org/10.1016/j.neuchi.2016.10.009

    Article  CAS  PubMed  Google Scholar 

  • Dias-Santagata D, Lam Q, Vernovsky K, Vena N, Lennerz JK, Borger DR, Batchelor TT, Ligon KL, Iafrate AJ, Ligon AH, Louis DN, Santagata S (2011) Braf V600E mutations are common in pleomorphic xanthoastrocytoma: diagnostic and therapeutic implications. Plos One 6. https://doi.org/10.1371/journal.pone.0017948

  • Fallah A, Weil AG, Sur S et al (2015) Epilepsy surgery related to pediatric brain tumors: Miami Children’s Hospital experience. J Neurosurg Pediatr 16:675–680

    Article  PubMed  Google Scholar 

  • Flaherty K, Puzanov I, Kim K, Ribas A, McArthur G, Sosman J, O’Dwyer P, Lee R, Grippo J, Nolop K, Chapman P (2010) Inhibition of mutated, activated BRAF in metastatic melanoma. N Engl J Med 363:809–819. https://doi.org/10.1056/NEJMoa1002011.Inhibition

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Gembruch O, Junker A, Mönninghoff C, Ahmadipour Y, Darkwah Oppong M, Sure U, El Hindy N, Lemonas E (2018) Liponeurocytoma: systematic review of a rare entity. World Neurosurg 120:214–233. https://doi.org/10.1016/j.wneu.2018.09.001

    Article  PubMed  Google Scholar 

  • Giulioni M, Gardella E, Rubboli G, Roncaroli F, Zucchelli M, Bernardi B, Tassinari CA, Calbucci F (2006) Lesionectomy in epileptogenic gangliogliomas: seizure outcome and surgical results. J Clin Neurosci 13:529–535. https://doi.org/10.1016/j.jocn.2005.07.017

    Article  PubMed  Google Scholar 

  • Giulioni M, Marucci G, Pelliccia V, Gozzo F, Barba C, Didato G, Villani F, Di Gennaro G, Quarato PP, Esposito V, Consales A, Martinoni M, Vornetti G, Zenesini C, Efisio Marras C, Specchio N, De Palma L, Rocchi R, Giordano F, Tringali G, Nozza P, Colicchio G, Rubboli G, Lo Russo G, Guerrini R, Tinuper P, Cardinale F, Cossu M (2017) Epilepsy surgery of “low grade epilepsy associated neuroepithelial tumors”: a retrospective nationwide italian study. Epilepsia 58:1832–1841. https://doi.org/10.1111/epi.13866

    Article  PubMed  Google Scholar 

  • Giulioni M, Rubboli G, Marucci G, Martinoni M, Marliani AF, Bartiromo F, Calbucci F (2013) Focal epilepsies associated with glioneuronal tumors: review article. Panminerva Med 55:225–238

    CAS  PubMed  Google Scholar 

  • Giulioni M, Rubboli G, Marucci G, Martinoni M, Volpi L, Michelucci R, Marliani AF, Bisulli F, Tinuper P, Castana L, Sartori I, Calbucci F (2009) Seizure outcome of epilepsy surgery in focal epilepsies associated with temporomesial glioneuronal tumors: lesionectomy compared with tailored resection. J Neurosurg 111:1275–1282. https://doi.org/10.3171/2009.3.JNS081350

    Article  PubMed  Google Scholar 

  • Grever MR (2016) BRAF inhibitor: targeted therapy in hairy cell leukemia. Blood 127:2782–2784. https://doi.org/10.1182/blood-2016-04-710459

    Article  CAS  Google Scholar 

  • Hammond RR, Duggal N, Woulfe JMJ, Girvin JP (2000) Malignant transformation of a dysembryoplastic neuroepithelial tumor: case report. J Neurosurg 92:722–725. https://doi.org/10.3171/jns.2000.92.4.0722

    Article  CAS  PubMed  Google Scholar 

  • Harrison W, Elsamadicy AA, McMahon JT, Chagoya G, Sobel RA, McLendon RE, Adamson C (2019) Glioneuronal tumor with features of ganglioglioma and neurocytoma arising in the fourth ventricle: a report of 2 unusual cases and a review of infratentorial gangliogliomas. J Neuropathol Exp Neurol 78:780–787. https://doi.org/10.1093/jnen/nlz060

    Article  PubMed  Google Scholar 

  • Horstmann S, Perry A, Reifenberger G, Giangaspero F, Huang H, Hara A, Masuoka J, Rainov NG, Bergmann M, Heppner FL, Brandner S, Chimelli L, Montagna N, Jackson T, Davis DG, Markesbery WR, Ellison DW, Weller RO, Taddei GL, Conti R, Del Bigio MR, González-Cámpora R, Camporee RV, Söylemezoglu F, Uro-Coste E, Qian J, Kleihues P, Ohgaki H (2004) Genetic and expression profiles of cerebellar liponeurocytomas. Brain Pathol 14:281–289. https://doi.org/10.1111/j.1750-3639.2004.tb00065.x

    Article  CAS  PubMed  Google Scholar 

  • Huse JT, Edgar M, Halliday J, Mikolaenko I, Lavi E, Rosenblum MK (2013) Multinodular and vacuolating neuronal tumors of the cerebrum: 10 cases of a distinctive seizure-associated lesion. Brain Pathol 23:515–524. https://doi.org/10.1111/bpa.12035

    Article  PubMed  PubMed Central  Google Scholar 

  • Huse JT, Snuderl M, Jones DTW, Brathwaite CD, Altman N, Lavi E, Saffery R, Sexton-Oates A, Blumcke I, Capper D, Karajannis MA, Benayed R, Chavez L, Thomas C, Serrano J, Borsu L, Ladanyi M, Rosenblum MK (2017) Polymorphous low-grade neuroepithelial tumor of the young (PLNTY): an epileptogenic neoplasm with oligodendroglioma-like components, aberrant CD34 expression, and genetic alterations involving the MAP kinase pathway. Acta Neuropathol 133:417–429. https://doi.org/10.1007/s00401-016-1639-9

    Article  CAS  PubMed  Google Scholar 

  • Jaiswal S (2016) Role of immunohistochemistry in the diagnosis of central nervous system tumors. Neurol India 64:502–512. https://doi.org/10.4103/0028-3886.181547

    Article  PubMed  Google Scholar 

  • Johnson DR, Giannini C, Jenkins RB, Kim DK, Kaufmann TJ (2019) Plenty of calcification: imaging characterization of polymorphous low-grade neuroepithelial tumor of the young. Neuroradiology 61:1327–1332. https://doi.org/10.1007/s00234-019-02269-y

    Article  PubMed  Google Scholar 

  • Jones DTW, Kieran MW, Bouffet E, Alexandrescu S, Bandopadhayay P, Bornhorst M, Ellison D, Fangusaro J, Fisher MJ, Foreman N, Fouladi M, Hargrave D, Hawkins C, Jabado N, Massimino M, Mueller S, Perilongo G, Schouten van Meeteren AYN, Tabori U, Warren K, Waanders AJ, Walker D, Weiss W, Witt O, Wright K, Zhu Y, Bowers DC, Pfister SM, Packer RJ (2018) Pediatric low-grade gliomas: next biologically driven steps. Neuro Oncol 20:160–173. https://doi.org/10.1093/neuonc/nox141

    Article  CAS  PubMed  Google Scholar 

  • Kaley T, Touat M, Subbiah V, Hollebecque A, Rodon J, Lockhart AC, Keedy V, Bielle F, Hofheinz R-D, Joly F, Blay J-Y, Chau I, Puzanov I, Raje NS, Wolf J, DeAngelis LM, Makrutzki M, Riehl T, Pitcher B, Baselga J, Hyman DM (2018) BRAF inhibition in BRAFV600-mutant gliomas: results from the VE-BASKET study. J Clin Oncol 36:3477–3484. https://doi.org/10.1200/JCO.2018.78.9990

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Kane AJ, Sughrue ME, Rutkowski MJ, Aranda D, Mills SA, Lehil M, Fang S, Parsa AT (2012) Atypia predicting prognosis for intracranial extraventricular neurocytomas. J Neurosurg 116:349–354. https://doi.org/10.3171/2011.9.JNS10783

    Article  PubMed  Google Scholar 

  • Kitamura Y, Komori T, Shibuya M, Ohara K, Saito Y, Hayashi S, Sasaki A, Nakagawa E, Tomio R, Kakita A, Nakatsukasa M, Yoshida K, Sasaki H (2018) Comprehensive genetic characterization of rosette-forming glioneuronal tumors: independent component analysis by tissue microdissection. Brain Pathol 28:87–93. https://doi.org/10.1111/bpa.12468

    Article  CAS  PubMed  Google Scholar 

  • Koh HY, Kim SH, Jang J, Kim H, Han S, Lim JS, Son G, Choi J, Park BO, Do Heo W, Han J, Lee HJ, Lee D, Kang H-C, Shong M, Paik S-B, Kim DS, Lee JH (2018) BRAF somatic mutation contributes to intrinsic epileptogenicity in pediatric brain tumors. Nat Med 24:1662–1668. https://doi.org/10.1038/s41591-018-0172-x

    Article  CAS  PubMed  Google Scholar 

  • La Corte E, Younus I, Pivari F, Selimi A, Ottenhausen M, Forbes JA, Pisapia DJ, Dobri GA, Anand VK, Schwartz TH (2018) BRAF V600E mutant papillary craniopharyngiomas: a single-institutional case series. Pituitary 21:571–583. https://doi.org/10.1007/s11102-018-0909-z

    Article  PubMed  Google Scholar 

  • Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (2016) WHO classification of tumours of the central nervous system, 4th edn. International Agency for Research on Cancer

    Google Scholar 

  • Lucas CHG, Villanueva-Meyer JE, Whipple N, Oberheim Bush NA, Cooney T, Chang S, McDermott M, Berger M, Cham E, Sun PP, Putnam A, Zhou H, Bollo R, Cheshier S, Poppe MM, Fung KM, Sung S, Glenn C, Fan X, Bannykh S, Hu J, Danielpour M, Li R, Alva E, Johnston J, Van Ziffle J, Onodera C, Devine P, Grenert JP, Lee JC, Pekmezci M, Tihan T, Bollen AW, Perry A, Solomon DA (2019) Myxoid glioneuronal tumor, PDGFRA p.K385-mutant: clinical, radiologic, and histopathologic features. Brain Pathol. https://doi.org/10.1111/bpa.12797

  • Luyken C, Blümcke I, Fimmers R, Urbach H, Elger CE, Wiestler OD, Schramm J (2003) The spectrum of long-term epilepsy-associated tumors: long-term seizure and tumor outcome and neurosurgical aspects. Epilepsia 44:822–830. https://doi.org/10.1046/j.1528-1157.2003.56102.x

    Article  PubMed  Google Scholar 

  • MacConaill LE, Campbell CD, Kehoe SM, Bass AJ, Hatton C, Niu L, Davis M, Yao K, Hanna M, Mondal C, Luongo L, Emery CM, Baker AC, Philips J, Goff DJ, Fiorentino M, Rubin MA, Polyak K, Chan J, Wang Y, Fletcher JA, Santagata S, Corso G, Roviello F, Shivdasani R, Kieran MW, Ligon KL, Stiles CD, Hahn WC, Meyerson ML, Garraway LA (2009) Profiling critical cancer gene mutations in clinical tumor samples. PLoS ONE 4:1–7. https://doi.org/10.1371/journal.pone.0007887

    Article  CAS  Google Scholar 

  • Majores M, Von Lehe M, Fassunke J, Schramm J, Becker AJ, Simon M (2008) Tumor recurrence and malignant progression of gangliogliomas. Cancer 113:3355–3363. https://doi.org/10.1002/cncr.23965

    Article  PubMed  Google Scholar 

  • Mallick S, Benson R, Melgandi W, Giridhar P, Rath GK (2018) Impact of surgery, adjuvant treatment, and other prognostic factors in the management of anaplastic ganglioglioma. Child’s Nerv Syst 34:1207–1213. https://doi.org/10.1007/s00381-018-3780-3

    Article  Google Scholar 

  • Marks AM, Bindra RS, DiLuna ML, Huttner A, Jairam V, Kahle KT, Kieran MW (2018) Response to the BRAF/MEK inhibitors dabrafenib/trametinib in an adolescent with a BRAF V600E mutated anaplastic ganglioglioma intolerant to vemurafenib. Pediatr Blood Cancer 65:e26969. https://doi.org/10.1002/pbc.26969

    Article  CAS  PubMed  Google Scholar 

  • Marucci G, Martinoni M, Giulioni M (2013) Relationship between focal cortical dysplasia and epilepsy-associated low-grade tumors: an immunohistochemical study. APMIS 121:22–29. https://doi.org/10.1111/j.1600-0463.2012.02938.x

    Article  PubMed  Google Scholar 

  • Miller C, Guillaume D, Dusenbery K, Clark HB, Moertel C (2016) Report of effective trametinib therapy in 2 children with progressive hypothalamic optic pathway pilocytic astrocytoma: documentation of volumetric response. J Neurosurg Pediatr 19:1–6. https://doi.org/10.3171/2016.9.PEDS16328

    Article  Google Scholar 

  • Minkin K, Klein O, Mancini J, Lena G (2008) Surgical strategies and seizure control in pediatric patients with dysembryoplastic neuroepithelial tumors: a single-institution experience. J Neurosurg Pediatr 1:206–210. https://doi.org/10.3171/PED/2008/1/3/206

    Article  PubMed  Google Scholar 

  • Morris HH, Matkovic Z, Estes ML, Prayson YA, Comair YG, Turnbull J, Najm I, Kotagal P, Wyllie E (1998) Ganglioglioma and intractable epilepsy: clinical and neurophysiologic features and predictors of outcome after surgery. Epilepsia 39:307–313. https://doi.org/10.1111/j.1528-1157.1998.tb01378.x

    Article  CAS  PubMed  Google Scholar 

  • Ostrom QT, Gittleman H, Truitt G, Boscia A, Kruchko C, Barnholtz-Sloan JS (2018) CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2011–2015. Neuro Oncol 20:iv1–iv86. https://doi.org/10.1093/neuonc/noy131

  • Ozlen F, Gunduz A, Asan Z, Tanriverdi T, Ozkara C, Yeni N, Yalcinkaya C, Ozyurt E, Uzan M (2010) Dysembryoplastic neuroepithelial tumors and gangliogliomas: clinical results of 52 patients. Acta Neurochir 152:1661–1671. https://doi.org/10.1007/s00701-010-0696-4

    Article  PubMed  Google Scholar 

  • Pages M, Lacroix L, Tauziede-Espariat A, Castel D, Daudigeos-Dubus E, Ridola V, Gilles S, Fina F, Andreiuolo F, Polivka M, Lechapt-Zalcman E, Puget S, Boddaert N, Liu X-Q, Bridge JA, Grill J, Chretien F, Varlet P (2015) Papillary glioneuronal tumors: histological and molecular characteristics and diagnostic value of SLC44A1-PRKCA fusion. Acta Neuropathol Commun 3:85. https://doi.org/10.1186/s40478-015-0264-5

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Pekmezci M, Stevers M, Phillips JJ, Van Ziffle J, Bastian BC, Tsankova NM, Kleinschmidt-DeMasters BK, Rosenblum MK, Tihan T, Perry A, Solomon DA (2018a) Multinodular and vacuolating neuronal tumor of the cerebrum is a clonal neoplasm defined by genetic alterations that activate the MAP kinase signaling pathway. Acta Neuropathol 135:485–488. https://doi.org/10.1007/s00401-018-1820-4

    Article  PubMed  PubMed Central  Google Scholar 

  • Pekmezci M, Villanueva-Meyer JE, Goode B, Van Ziffle J, Onodera C, Grenert JP, Bastian BC, Chamyan G, Maher OM, Khatib Z, Kleinschmidt-DeMasters BK, Samuel D, Mueller S, Banerjee A, Clarke JL, Cooney T, Torkildson J, Gupta N, Theodosopoulos P, Chang EF, Berger M, Bollen AW, Perry A, Tihan T, Solomon DA (2018b) The genetic landscape of ganglioglioma. Acta Neuropathol Commun 6:47. https://doi.org/10.1186/s40478-018-0551-z

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Pelliccia V, Deleo F, Gozzo F, Sartori I, Mai R, Cossu M, Tassi L (2017) Early and late epilepsy surgery in focal epilepsies associated with long-term epilepsy-associated tumors. J Neurosurg 127:1147–1152. https://doi.org/10.3171/2016.9.JNS161176

    Article  PubMed  Google Scholar 

  • Peng X, Hou P, Chen Y, Dai Y, Ji Y, Shen Y, Su Y, Liu B, Wang Y, Sun D, Jiang Y, Zha C, Xie Z, Ding J, Geng M, Ai J (2019) Preclinical evaluation of 3D185, a novel potent inhibitor of FGFR1/2/3 and CSF-1R, in FGFR-dependent and macrophage-dominant cancer models. J Exp Clin Cancer Res 38:372. https://doi.org/10.1186/s13046-019-1357-y

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Prayson RA, Fong J, Najm I (2010) Coexistent pathology in chronic epilepsy patients with neoplasms. Mod Pathol 23:1097–1103. https://doi.org/10.1038/modpathol.2010.94

    Article  PubMed  Google Scholar 

  • Radhakrishnan A, Abraham M, Vilanilam G, Menon R, Menon D, Kumar H, Cherian A, Radhakrishnan N, Kesavadas C, Thomas B, Sarma SP, Thomas SV (2016) Surgery for “long-term epilepsy associated tumors (LEATs)”: Seizure outcome and its predictors. Clin Neurol Neurosurg 141:98–105. https://doi.org/10.1016/j.clineuro.2015.12.020

    Article  PubMed  Google Scholar 

  • Rhoton A (2003) Rhoton cranial anatomy and surgical approaches. Schaumburg, IL

    Google Scholar 

  • Riesberg G, Bathla G, Gupta S, Watal P, Moritani T (2018) Malignant transformation and leptomeningeal spread of recurrent ganglioglioma: case report and review of literature. Clin Imaging 48:7–11. https://doi.org/10.1016/j.clinimag.2017.09.011

    Article  PubMed  Google Scholar 

  • Rivera B, Gayden T, Carrot-Zhang J, Nadaf J, Boshari T, Faury D, Zeinieh M, Blanc R, Burk DL, Fahiminiya S, Bareke E, Schüller U, Monoranu CM, Sträter R, Kerl K, Niederstadt T, Kurlemann G, Ellezam B, Michalak Z, Thom M, Lockhart PJ, Leventer RJ, Ohm M, MacGregor D, Jones D, Karamchandani J, Greenwood CMTT, Berghuis AM, Bens S, Siebert R, Zakrzewska M, Liberski PP, Zakrzewski K, Sisodiya SM, Paulus W, Albrecht S, Hasselblatt M, Jabado N, Foulkes WD, Majewski J (2016) Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors. Acta Neuropathol 131:847–863. https://doi.org/10.1007/s00401-016-1549-x

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Rodriguez FJ, Schniederjan MJ, Nicolaides T, Tihan T, Burger PC, Perry A (2015) High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN). Acta Neuropathol 129:609–610. https://doi.org/10.1007/s00401-015-1400-9

    Article  PubMed  PubMed Central  Google Scholar 

  • Roessler K, Heynold E, Buchfelder M, Hermann S, Hamer HM (2019) Current value of intraoperative electrocorticography (iopECoG). Epilepsy Behav 91:20–24. https://doi.org/10.1016/j.yebeh.2018.06.053

    Article  PubMed  Google Scholar 

  • Schindler G, Capper D, Meyer J, Janzarik W, Omran H, Herold-Mende C, Schmieder K, Wesseling P, Mawrin C, Hasselblatt M, Louis DN, Korshunov A, Pfister S, Hartmann C, Paulus W, Reifenberger G, von Deimling A (2011) Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. Acta Neuropathol 121:397–405. https://doi.org/10.1007/s00401-011-0802-6

    Article  CAS  PubMed  Google Scholar 

  • Schreck KC, Grossman SA, Pratilas CA (2019) BRAF mutations and the utility of RAF and MEK inhibitors in primary brain tumors. Cancers 11:1–18. https://doi.org/10.3390/cancers11091262

    Article  CAS  Google Scholar 

  • Sievers P, Stichel D, Schrimpf D, Sahm F, Koelsche C, Reuss DE, Wefers AK, Reinhardt A, Huang K, Ebrahimi A, Hou Y, Pajtler KW, Pfister SM, Hasselblatt M, Stummer W, Schick U, Hartmann C, Hagel C, Staszewski O, Reifenberger G, Beschorner R, Coras R, Keyvani K, Kohlhof P, Diomedi-Camassei F, Herold-Mende C, Giangaspero F, Rushing E, Giannini C, Korshunov A, Jones DTW, von Deimling A (2018) FGFR1:TACC1 fusion is a frequent event in molecularly defined extraventricular neurocytoma. Acta Neuropathol 136:293–302. https://doi.org/10.1007/s00401-018-1882-3

    Article  CAS  PubMed  Google Scholar 

  • Sievert AJ, Lang SS, Boucher KL, Madsen PJ, Slaunwhite E, Choudhari N, Kellet M, Storm PB, Resnick AC (2013) Paradoxical activation and RAF inhibitor resistance of BRAF protein kinase fusions characterizing pediatric astrocytomas. Proc Natl Acad Sci USA 110:5957–5962. https://doi.org/10.1073/pnas.1219232110

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Silveira L, DeWitt J, Thomas A, Tranmer B (2019) Disseminated rosette-forming glioneuronal tumor with spinal drop metastasis, a uniquely aggressive presentation of rare tumor. World Neurosurg 132:7–11. https://doi.org/10.1016/j.wneu.2019.08.055

    Article  PubMed  Google Scholar 

  • Stone TJ, Keeley A, Virasami A, Harkness W, Tisdall M, Izquierdo Delgado E, Gutteridge A, Brooks T, Kristiansen M, Chalker J, Wilkhu L, Mifsud W, Apps J, Thom M, Hubank M, Forshew T, Cross JH, Hargrave D, Ham J, Jacques TS (2018a) Comprehensive molecular characterisation of epilepsy-associated glioneuronal tumours. Acta Neuropathol 135:115–129. https://doi.org/10.1007/s00401-017-1773-z

    Article  CAS  PubMed  Google Scholar 

  • Stone TJ, Rowell R, Jayasekera BAP, Cunningham MO, Jacques TS (2018b) Review: molecular characteristics of long-term epilepsy-associated tumours (LEATs) and mechanisms for tumour-related epilepsy (TRE). Neuropathol Appl Neurobiol 44:56–69. https://doi.org/10.1111/nan.12459

    Article  CAS  PubMed  Google Scholar 

  • Surrey LF, Jain P, Zhang B, Straka J, Zhao X, Harding BN, Resnick AC, Storm PB, Buccoliero AM, Genitori L, Li MM, Waanders AJ, Santi M (2019) Genomic analysis of dysembryoplastic neuroepithelial tumor spectrum reveals a diversity of molecular alterations dysregulating the MAPK and PI3K/mTOR pathways. J Neuropathol Exp Neurol. https://doi.org/10.1093/jnen/nlz101

    Article  PubMed  Google Scholar 

  • Terrier L-M, Bauchet L, Rigau V, Amelot A, Zouaoui S, Filipiak I, Caille A, Almairac F, Aubriot-Lorton M-H, Bergemer-Fouquet A-M, Bord E, Cornu P, Czorny A, Dam Hieu P, Debono B, Delisle M-B, Emery E, Farah W, Gauchotte G, Godfraind C, Guyotat J, Irthum B, Janot K, Le Reste P-J, Liguoro D, Loiseau H, Lot G, Lubrano V, Mandonnet E, Menei P, Metellus P, Milin S, Muckenstrum B, Roche P-H, Rousseau A, Uro-Coste E, Vital A, Voirin J, Wager M, Zanello M, François P, Velut S, Varlet P, Figarella-Branger D, Pallud J, Zemmoura I, Club de Neuro-Oncologie of the Société Française de Neurochirurgie C de N-O of the SF de (2017) Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French brain tumor database. Neuro Oncol 19:678–688. https://doi.org/10.1093/neuonc/now186

  • Touat M, Gratieux J, Auliac SC, Sejean K, Aldea S, Savatovsky J, Perkins G, Blons H, Ligon KL, Idbaih A, Hollebecque A, Gimenez-Roqueplo AP, Laurent-Puig P, Sanson M, Villa C, Di Stefano AL (2018) Vemurafenib and cobimetinib overcome resistance to vemurafenib in BRAF-mutant ganglioglioma. Neurology 91:523–525. https://doi.org/10.1212/WNL.0000000000006171

    Article  PubMed  PubMed Central  Google Scholar 

  • Tuleasca C, Al-Risi AS, David P, Adam C, Aghakhani N, Parker F (2019) Paragangliomas of the spine: a retrospective case series in a national reference French center. Acta Neurochir. https://doi.org/10.1007/s00701-019-04186-8

    Article  PubMed  Google Scholar 

  • van Tilburg CM, Selt F, Sahm F, Bächli H, Pfister SM, Witt O, Milde T (2018) Response in a child with a BRAF V600E mutated desmoplastic infantile astrocytoma upon retreatment with vemurafenib. Pediatr Blood Cancer 65. https://doi.org/10.1002/pbc.26893

  • Vasiljevic A, Champier J, Figarella-Branger D, Wierinckx A, Jouvet A, Fèvre-Montange M (2013) Molecular characterization of central neurocytomas: potential markers for tumor typing and progression. Neuropathology 33:149–161. https://doi.org/10.1111/j.1440-1789.2012.01338.x

    Article  CAS  PubMed  Google Scholar 

  • Yadav N, Rao S, Saini J, Prasad C, Mahadevan A, Sadashiva N (2017) Papillary glioneuronal tumors: a radiopathologic correlation. Eur J Radiol 97:44–52. https://doi.org/10.1016/j.ejrad.2017.10.004

    Article  PubMed  Google Scholar 

  • Yang C, Fang J, Li G, Li S, Ha T, Wang J, Yang B, Yang J, Xu Y (2017) Histopathological, molecular, clinical and radiological characterization of rosette-forming glioneuronal tumor in the central nervous system. Oncotarget 8:109175–109190. https://doi.org/10.18632/oncotarget.22646

  • Yang I, Chang EF, Han SJ, Barry JJ, Fang S, Tihan T, Barbaro NM, Parsa AT (2011) Early surgical intervention in adult patients with ganglioglioma is associated with improved clinical seizure outcomes. J Clin Neurosci 18:29–33. https://doi.org/10.1016/j.jocn.2010.05.002

    Article  PubMed  Google Scholar 

  • Yao Z, Torres NM, Tao A, Gao Y, Luo L, Li Q, de Stanchina E, Abdel-Wahab O, Solit DB, Poulikakos PI, Rosen N (2015) BRAF mutants evade ERK-dependent feedback by different mechanisms that determine their sensitivity to pharmacologic inhibition. Cancer Cell 28:370–383. https://doi.org/10.1016/j.ccell.2015.08.001

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Yaşargil MG, Krayenbühl N, Roth P, Hsu SPC, Yaşargil DCH (2010) The selective amygdalohippocampectomy for intractable temporal limbic seizures. J Neurosurg 112:168–185. https://doi.org/10.3171/2008.12.JNS081112

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Neurosurgery, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

    Matteo Martinoni, Emanuele La Corte & Francesco Toni

  2. Surgical Pathology Section, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

    Viscardo Paolo Fabbri & Sofia Asioli

  3. Department of Biomedical and Neuromotor Sciences (DIBINEM) – Alma Mater Studiorum – University of Bologna, Bologna, Italy

    Sofia Asioli

  4. Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA

    Caterina Giannini

  5. Division of Anatomic Pathology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN, 55905, USA

    Caterina Giannini

  6. Programma di neuroradiologia con tecniche ad elevata complessità, IRCCS Istituto delle Scienze Neurologiche di Bologna ETC, Bologna, Italy

    Francesco Toni

  7. Pediatric Neurosurgery, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

    Mino Zucchelli

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Correspondence to Matteo Martinoni .

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  1. Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran

    Nima Rezaei

  2. Tehran University of Medical Sciences, Tehran, Iran

    Sara Hanaei

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Martinoni, M. et al. (2023). Glioneuronal and Neuronal Tumors of the Central Nervous System. In: Rezaei, N., Hanaei, S. (eds) Human Brain and Spinal Cord Tumors: From Bench to Bedside. Volume 2. Advances in Experimental Medicine and Biology, vol 1405. Springer, Cham. https://doi.org/10.1007/978-3-031-23705-8_9

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