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Rosette-forming glioneuronal tumor originating in the hypothalamus

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Abstract

Rosette-forming glioneuronal tumors (RGNT) of the fourth ventricle are slow-growing tumors that primarily involve the fourth ventricular region. We here report the first patient, an 8-year-old girl, with an RGNT originating in the hypothalamus and manifesting with precocious puberty. After partial removal, the remaining tumor showed rapid enlargement, and the pathologic diagnosis at the second surgery revealed histopathologic features similar to those found in the initial samples, including biphasic patterns of neurocytic rosettes and GFAP-stained astrocytic components. These tumor cells had mildly atypical nuclei; however, mitotic figures and necrosis were absent. Eosinophilic granular bodies and a glomeruloid vasculature were found, but Rosenthal fibers were absent. The Ki-67 proliferative index was 3.5 % (vs 1.1 % at the initial surgery). No recurrence was recorded during the 3-year period after the proton radiotherapy.

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References

  1. 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

    Article  PubMed  Google Scholar 

  2. Anan M, Inoue R, Ishii K 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(6):898–901

    Article  PubMed  Google Scholar 

  3. Chakraborti S, Mahadevan A, Govindan A et al (2012) Rosette-forming glioneuronal tumor—evidence of stem cell origin with biphenotypic differentiation. Virchows Arch 461:581–588

    Article  CAS  PubMed  Google Scholar 

  4. Chiba K, Aihara Y, Eguchi S et al (2014) Rosette-forming glioneuronal tumor of the fourth ventricle with neurocytoma component. Childs Nerv Syst 30:351–356

    Article  PubMed  Google Scholar 

  5. Ellezam B, Theeler BJ, Luthra R et al (2012) Recurrent PIK3CA mutations in rosette-forming glioneuronal tumor. Acta Neuropathol 123:285–287

    Article  PubMed  Google Scholar 

  6. Frydenberg E, Laherty R, Rodriguez M et al (2010) A rosette-forming glioneuronal tumour of the pineal gland. J Clin Neurosci 17:1326–1328

    Article  CAS  PubMed  Google Scholar 

  7. Gessi M, Lambert SR, Lauriola L et al (2012) Absence of KIAA1549-BRAF fusion in rosette-forming glioneuronal tumors of the fourth ventricle (RGNT). J Neurooncol 110:21–25

    Article  CAS  PubMed  Google Scholar 

  8. Ghosal N, Furtado SV, Hegde AS (2010) Rosette forming glioneuronal tumor pineal gland and tectum: an intraoperative diagnosis on smear preparation. Diagn Cytopathol 38:590–593

    PubMed  Google Scholar 

  9. Kemp S, Achan A, Ng T et al (2012) Rosette-forming glioneuronal tumour of the lateral ventricle in a patient with neurofibromatosis 1. J Clin Neurosci 19:1180–1181

    Article  CAS  PubMed  Google Scholar 

  10. Lu JQ, Scheithauer BW, Sharma P et al (2009) Multifocal complex glioneuronal tumor in an elderly man: an autopsy study: case report. Neurosurgery 64:E1193–E1195

    Article  PubMed  Google Scholar 

  11. Luan S, Zhuang D, Sun L et al (2010) Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle: case report and review of literature. Clin Neurol Neurosurg 112:362–364

    Article  PubMed  Google Scholar 

  12. Marhold F, Preusser M, Dietrich W et al (2008) Clinicoradiological features of rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle: report of four cases and literature review. J Neurooncol 90:301–308

    Article  PubMed  Google Scholar 

  13. Rainov NG, Wagner T, Heidecke V (2010) Rosette-forming glioneuronal tumor of the fourth ventricle. Cent Eur Neurosurg 71:219–221

    Article  CAS  PubMed  Google Scholar 

  14. Scheithauer BW, Silva AI, Ketterling RP et al (2009) Rosette-forming glioneuronal tumor: report of a chiasmal-optic nerve example in neurofibromatosis type 1: special pathology report. Neurosurgery 64:E771–E772

    Article  PubMed  Google Scholar 

  15. Sharma P, Swain M, Padua MD et al (2011) Rosette-forming glioneuronal tumors: a report of two cases. Neurol India 59:276–280

    Article  PubMed  Google Scholar 

  16. Solis OE, Mehta RI, Lai A 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

    Article  PubMed Central  PubMed  Google Scholar 

  17. Wang Y, Xiong J, Chu SG et al (2009) Rosette-forming glioneuronal tumor: report of an unusual case with intraventricular dissemination. Acta Neuropathol 118:813–819

    Article  PubMed  Google Scholar 

  18. Xu J, Yang Y, Liu Y et al (2012) Rosette-forming glioneuronal tumor in the pineal gland and the third ventricle: a case with radiological and clinical implications. Quant Imaging Med Surg 2:227–231

    PubMed Central  PubMed  Google Scholar 

  19. Jacques TS, Eldridge C, Patel A et al (2006) Mixed glioneuronal tumour of the fourth ventricle with prominent rosette formation. Neuropathol Appl Neurobiol 32:217–220

    Article  CAS  PubMed  Google Scholar 

  20. Pimentel J, Resende M, Vaz A et al (2008) Rosette-forming glioneuronal tumor: pathology case report. Neurosurgery 62:E1162–E1163

    Article  PubMed  Google Scholar 

  21. Thurston B, Gunny R, Anderson G et al (2013) Fourth ventricle rosette-forming glioneuronal tumour in children: an unusual presentation in an 8-year-old patient, discussion and review of the literature. Childs Nerv Syst 29:839–847

    Article  PubMed  Google Scholar 

  22. Zhang J, Babu R, McLendon RE et al (2013) A comprehensive analysis of 41 patients with rosette-forming glioneuronal tumors of the fourth ventricle. J Clin Neurosci 20:335–341

    Article  PubMed  Google Scholar 

  23. Louis DN, Ohgaki H, Wiestler OD et al (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109

    Article  PubMed Central  PubMed  Google Scholar 

  24. Adachi J, Nishikawa R, Hirose T et al (2005) Mixed neuronal-glial tumor of the fourth ventricle and successful treatment of postoperative mutism with bromocriptine: case report. Surg Neurol 63:375–379

    Article  PubMed  Google Scholar 

  25. Albanese A, Mangiola A, Pompucci A et al (2005) Rosette-forming glioneuronal tumour of the fourth ventricle: report of a case with clinical and surgical implications. J Neurooncol 71:195–197

    Article  CAS  PubMed  Google Scholar 

  26. Alturkustani M, Ang LC (2012) Rosette-forming glioneuronal tumour of the 4th ventricle in a NF1 patient. Can J Neurol Sci 39:95–96

    Article  PubMed  Google Scholar 

  27. Arai A, Sasayama T, Tamaki M et al (2010) Rosette-forming glioneuronal tumor of the fourth ventricle–case report. Neurol Med Chir (Tokyo) 50:224–228

    Article  Google Scholar 

  28. Cabezas SG, Blanch RS, Sanchez-Sanchez R et al (2015) Rosette-forming glioneuronal tumour (RGNT) of the fourth ventricle: a highly aggressive case. Brain Tumor Pathol 32:124–130

    Article  Google Scholar 

  29. Fushimi Y, Miyasaki A, Taki H et al (2011) Rosette-forming glioneuronal tumor of the fourth ventricle with bilateral olivary degeneration. Jpn J Radiol 29:445–448

    Article  PubMed  Google Scholar 

  30. Gao C, Xie R, Cao XY et al (2009) Rosette-forming glioneuronal tumor of the fourth ventricle. A rare neoplasm of the central nervous system. Neurosciences (Riyadh) 14:292–293

    Google Scholar 

  31. Gessi M, Waha A, Setty P et al (2011) Analysis of KIAA1549-BRAF fusion status in a case of rosette-forming glioneuronal tumor of the fourth ventricle (RGNT). Neuropathology 31:654–657

    Article  PubMed  Google Scholar 

  32. Hsu C, Kwan G, Lau Q et al (2012) Rosette-forming glioneuronal tumour: imaging features, histopathological correlation and a comprehensive review of literature. Br J Neurosurg 26:668–673

    Article  PubMed  Google Scholar 

  33. Johnson M, Pace J, Burroughs JF (2006) Fourth ventricle rosette-forming glioneuronal tumor. Case report. J Neurosurg 105:129–131

    Article  PubMed  Google Scholar 

  34. Joseph V, Wells A, Kuo YH et al (2009) The ‘rosette-forming glioneuronal tumor’ of the fourth ventricle. Neuropathology 29:309–314

    Article  PubMed  Google Scholar 

  35. Karafin M, Jallo GI, Ayars M et al (2011) Rosette forming glioneuronal tumor in association with Noonan syndrome: pathobiological implications. Clin Neuropathol 30:297–300

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  36. Kinno M, Ishizawa K, Shimada S et al (2010) Cytology is a useful tool for the diagnosis of rosette-forming glioneuronal tumour of the fourth ventricle: a report of two cases. Cytopathology 21:194–197

    Article  CAS  PubMed  Google Scholar 

  37. Li YM, Li WQ, Pan Y et al (2009) Rosette-forming glioneuronal tumour of the fourth ventricle with previous intratumoural haemorrhage: case report and review of the literature. J Int Med Res 37:958–966

    Article  CAS  PubMed  Google Scholar 

  38. Matyja E, Grajkowska W, Nauman P et al (2011) Rosette-forming glioneuronal tumor of the fourth ventricle with advanced microvascular proliferation—a case report. Neuropathology 31:427–432

    Article  PubMed  Google Scholar 

  39. Nandeesh BN, Chabra MS, Babu MK et al (2012) A rare posterior cranial fossa tumor. J Cancer Res Ther 8:644–646

    Article  PubMed  Google Scholar 

  40. Podlesek D, Geiger K, Hendry DJ et al (2011) Rosette-forming glioneuronal tumor of the fourth ventricle in an elderly patient. J Neurooncol 103:727–731

    Article  CAS  PubMed  Google Scholar 

  41. Preusser M, Dietrich W, Czech T et al (2003) Rosette-forming glioneuronal tumor of the fourth ventricle. Acta Neuropathol 106:506–508

    Article  PubMed  Google Scholar 

  42. Shah MN, Leonard JR, Perry A (2010) Rosette-forming glioneuronal tumors of the posterior fossa. J Neurosurg Pediatr 5:98–103

    Article  PubMed  Google Scholar 

  43. Tan CC, Gonzales M, Veitch A (2008) Clinical implications of the infratentorial rosette-forming glioneuronal tumor: case report. Neurosurgery 63:E175–E176

    Article  PubMed  Google Scholar 

  44. Thommen F, Hewer E, Schäfer SC et al (2013) Rosette-forming glioneuronal tumor of the cerebellum in statu nascendi: an incidentally detected diminutive example indicates derivation from the internal granule cell layer. Clin Neuropathol 32:370–376

    Article  PubMed  Google Scholar 

  45. Vajtai I, Arnold M, Kappeler A et al (2007) Rosette-forming glioneuronal tumor of the fourth ventricle: report of two cases with a differential diagnostic overview. Pathol Res Pract 203:613–619

    Article  PubMed  Google Scholar 

  46. Xiong J, Liu Y, Chu SG 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

    Article  PubMed  Google Scholar 

  47. Xiong J, Ding L, Chen H et al (2013) Mixed glioneuronal tumor: a dysembryoplastic neuroepithelial tumor with rosette-forming glioneuronal tumor component. Neuropathology 33:431–435

    Article  CAS  PubMed  Google Scholar 

  48. Yin B, Liu L, Chen XR et al (2012) Rosette-forming glioneuronal tumor of the fourth ventricle. J Neuroradiol 39:129–130

    Article  PubMed  Google Scholar 

  49. Wendt S, Shelso J, Wright K et al (2014) Neoplastic causes of abnormal puberty. Pediatr Blood Cancer 61:664–671

    Article  PubMed  Google Scholar 

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Acknowledgments

We thank Flaminia Miyamasu (Editor in the Life Sciences [ELS], Medical English Communications Center, University of Tsukuba) for grammatical revision and Alexander Zaboronok (University of Tsukuba Hospital, Office for the Promotion of International Medical Affairs) for critical comments.

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Authors and Affiliations

  1. Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan

    Tetsuya Yamamoto, Teppei Matsubara, Masahide Matsuda, Ai Muroi, Eiichi Ishikawa, Hiroyoshi Akutsu, Kei Nakai & Akira Matsumura

  2. Department of Pathology, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan

    Kaishi Satomi & Noriaki Sakamoto

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  1. Tetsuya Yamamoto
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  2. Teppei Matsubara
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  5. Masahide Matsuda
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Correspondence to Tetsuya Yamamoto.

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Yamamoto, T., Matsubara, T., Satomi, K. et al. Rosette-forming glioneuronal tumor originating in the hypothalamus. Brain Tumor Pathol 32, 291–296 (2015). https://doi.org/10.1007/s10014-015-0226-4

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  • DOI: https://doi.org/10.1007/s10014-015-0226-4

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