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Clinical Course of Central Neurocytoma with Malignant Transformation—An Indication for Craniospinal Irradiation

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Pathology & Oncology Research

Abstract

Central neurocytoma is generally considered to be a benign tumor and the literature suggests that a cure may be attained by surgery ± adjuvant focal irradiation. However, there is a need for change in the therapeutic strategy for the subgroup of patients with aggressive central neurocytoma. An example case is presented and the literature on central neurocytoma cases with malignant features and dissemination via the cerebrospinal fluid is reviewed and the radiotherapeutic strategies available for central neurocytoma treatment is discussed. Nineteen cases including the present report with a malignant course and cerebrospinal fluid dissemination have been described to date, most of them involving an elevated MIB-1 labeling index. Our case exhibited atypical central neurocytoma with an initially elevated MIB-1 labeling index (25–30 %). The primary treatment included surgery and focal radiotherapy. Three years later the disease had disseminated throughout the craniospinal axis. A good tumor response and symptom relief were achieved with repeated radiation and temozolomide chemotherapy. Central neurocytoma with an initially high proliferation activity has a high tendency to spread via the cerebrospinal fluid. The chemo- and radiosensitivity of the tumor suggest a more aggressive adjuvant therapy approach. Cases with a potential for malignant transformation should be identified and treated appropriately, including irradiation of the entire neuroaxis and adjuvant chemotherapy may be considered.

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References

  1. Hassoun J, Gambareli D, Grisoli F, Pellet W, Salamon G, Pellisier JF, Toga M (1982) Central neurocytoma—an electron-microscopic study of 2 cases. Acta Neuropathol 56:151–156

    Article  PubMed  CAS  Google Scholar 

  2. Leenstra JL, Rodriguez FJ, Frechette CM, Giannini C, Stafford SL, Pollock BE, Schild SE, Scheithauer BW, Jenkins RB, Buckner JC, Brown PD (2007) Central neurocytoma: management recommendations based on a 35-year experience. Int J Radiat Oncol Biol Phys 67:1145–1154

    Article  PubMed  Google Scholar 

  3. Rades D, Fehlauer F, Lamszus K, Schild SE, Hagel C, Westphal M, Alberti W (2005) Well-differentiated neurocytoma: what is the best available treatment? Neuro-Oncol 7:77–83

    Article  PubMed Central  PubMed  Google Scholar 

  4. Schmidt MH, Gottfried ON, von Koch CS, Chang SM, McDermott MW (2004) Central neurocytoma: a review. J Neurooncol 66:377–384

    Article  PubMed  Google Scholar 

  5. Vasiljevic A, Francois P, Loundou A, Fevre-Montange M, Jouvet A, Roche PH, Figarella-Branger D (2012) Prognostic factors in central neurocytomas: a multicenter study of 71 cases. Am J Surg Pathol 36:220–227

    Article  PubMed  Google Scholar 

  6. Kane AJ, Sughrue ME, Rutkowski MJ, Tihan T, Parsa AT (2011) The molecular pathology of central neurocytomas. J Clin Neurosci 18:1–6

    Article  PubMed  CAS  Google Scholar 

  7. Kubota T, Hayashi M, Kawano H, Kabuto M, Sato K, Ishise J, Kawamoto K, Shirataki K, Iizuka H, Tsunoda S, Katsuyama J (1991) Central neurocytoma - immunohistochemical and ultrastructural-study. Acta Neuropathol 81:418–427

    Article  PubMed  CAS  Google Scholar 

  8. Von Deimling A, Janzer R, Kleihues P, Wiestler OD (1990) Patterns of differentiation in central neurocytoma—an immunohistochemical study of 11 biopsies. Acta Neuropathol 79:473–479

    Article  Google Scholar 

  9. Coca S, Moreno M, Martos JA, Rodriguez J, Barcena A, Vaquero J (1994) Neurocytoma of spinal-cord. Acta Neuropathol 87:537–540

    Article  PubMed  CAS  Google Scholar 

  10. Hassoun J, Soylemezoglu F, Gambarelli D, Figarella-Branger D, Von Ammon K, Kleihues P (1993) Central neurocytoma—a synopsis of clinical and histological features. Brain Pathol 3:297–306

    Article  PubMed  CAS  Google Scholar 

  11. Kim DG, Chi JG, Parks SH, Chang KH, Lee SH, Jung HW, Kim HJ, Cho BK, Choi KS, Han DH (1992) Intraventricular neurocytoma - clinicopathological analysis of 7 cases. J Neurosurg 76:759–765

    Article  PubMed  CAS  Google Scholar 

  12. Figarella-Branger D, Söylemezoglu F, Kleihues P, Hassoun J, Cavenee WK (2000) Central neurocytoma. In: Kleihues P (ed) World health classification of tumours. Tumours of the nervous system. Pathology and genetics. IARC, Lyon, pp 107–109

    Google Scholar 

  13. Sharma MC, Deb P, Sharma S, Sarkar C (2006) Neurocytoma: a comprehensive review. Neurosurg Rev 29:270–285

    Article  PubMed  Google Scholar 

  14. Giangaspero F, Cenacchi G, Losi L, Cerasoli S, Bisceglia M, Burger PC (1997) Extraventricular neoplasms with neurocytoma features—a clinicopathological study of 11 cases. Am J Surg Pathol 21:206–212

    Article  PubMed  CAS  Google Scholar 

  15. Ferreol E, Sawaya R, de Courten-Myers GM (1989) Primary cerebral neuro-blastoma (neurocytoma) in adults. J Neurooncol 7:121–128

    Article  PubMed  CAS  Google Scholar 

  16. Kulkarni V, Rajshekhar V, Haran RP, Chandi SM (2002) Long-term outcome in patients with central neurocytoma following stereotactic biopsy and radiation therapy. Br J Neurosurg 16:126–132

    Article  PubMed  CAS  Google Scholar 

  17. Sharma MC, Rathore A, Karak AK, Sarkar C (1998) A study of proliferative markers in central neurocytoma. Pathology 30:355–359

    Article  PubMed  CAS  Google Scholar 

  18. Sharma MC, Sarkar C, Karak AK, Gaikwad S, Mahapatra AK, Mehta VS (1999) Intraventricular neurocytoma: a clinicopathological study of 20 cases with review of the literature. J Clin Neurosci 6:319–323

    Article  PubMed  Google Scholar 

  19. Sharma S, Sarkar C, Gaikwad S, Suri A, Sharma MC (2005) Primary neurocytoma of the spinal cord: a case report and review of literature. J Neurooncol 74:47–52

    Article  PubMed  Google Scholar 

  20. Jaiswal S, Vij M, Rajput D, Mehrotra A, Jaiswal AK, Srivastava AK, Behari S, Krishnani N (2011) A clinicopathological, immunohistochemical and neuroradiological study of eight patients with central neurocytoma. J Clin Neurosci 18:334–339

    Article  PubMed  Google Scholar 

  21. Chen CM, Chen KH, Jung SM, Hsu HC, Wang CM (2008) Central neurocytoma: 9 case series and review. Surg Neurol 70:204–209

    Article  PubMed  Google Scholar 

  22. Chen H, Zhou R, Liu J, Tang J (2012) Central neurocytoma. J Clin Neurosci 19:849–853

    Article  PubMed  Google Scholar 

  23. Chen CL, Shen CC, Wang J, Lu CH, Lee HT (2008) Central neurocytoma: a clinical, radiological and pathological study of nine cases. Clin Neurol Neurosurg 110:129–136

    Article  PubMed Central  PubMed  Google Scholar 

  24. Ishiuchi S, Nakazato Y, Iino M, Ozawa S, Tamura M, Ohye C (1998) In vitro neuronal and glial production and differentiation of human central neurocytoma cells. J Neurosci Res 51:526–535

    Article  PubMed  CAS  Google Scholar 

  25. Ishiuchi S, Tamura M (1997) Central neurocytoma: an immunohistochemical, ultrastructural and cell culture study. Acta Neuropathol 94:425–435

    Article  PubMed  CAS  Google Scholar 

  26. Kawashima M, Suzuki SO, Doh-ura K, Iwaki T (2000) alpha-Synuclein is expressed in a variety of brain tumors showing neuronal differentiation. Acta Neuropathol 99:154–160

    Article  PubMed  CAS  Google Scholar 

  27. Nakagawa K, Aoki Y, Sakata K, Sasaki Y, Matsutani M, Akanuma A (1993) Radiation-therapy of well-differentiated neuroblastoma and central neurocytoma. Cancer 72:1350–1355

    Article  PubMed  CAS  Google Scholar 

  28. Namiki J, Nakatsukasa M, Murase I, Yamazaki K (1998) Central neurocytoma presenting with intratumoral hemorrhage 15 years after initial treatment by partial removal and irradiation—case report. Neurol Med 38:278–282

    Article  CAS  Google Scholar 

  29. Nishio S, Takeshita I, Kaneko Y, Fukui M (1992) Cerebral neurocytoma—a new subset of benign neuronal tumors of the cerebrum. Cancer 70:529–537

    Article  PubMed  CAS  Google Scholar 

  30. Kim DG, Kim JS, Chi JG, Park SH, Jung HW, Choi KS, Han DH (1996) Central neurocytoma: proliferative potential and biological behavior. J Neurosurg 84:742–747

    Article  PubMed  CAS  Google Scholar 

  31. Kim DG, Paek SH, Kim IH, Chi JG, Jung HW, Han DH, Choi KS, Cho BK (1997) Central neurocytoma—the role of radiation therapy and long term outcome. Cancer 79:1995–2002

    Article  PubMed  CAS  Google Scholar 

  32. Kim DH, Suh YL (1997) Pseudopapillary neurocytoma of temporal lobe with glial differentiation. Acta Neuropathol 94:187–191

    Article  PubMed  CAS  Google Scholar 

  33. Kim DG, Choe WJ, Chang KH, Song IC, Han MH, Jung HW, Cho BK (2000) In vivo proton magnetic resonance spectroscopy of central neurocytomas. Neurosurgery 46:329–333

    Article  PubMed  CAS  Google Scholar 

  34. Kim CY, Paek SH, Jeong SS, Chung HT, Han JH, Park CK, Jung HW, Kim DG (2007) Gamma knife radiosurgery for central neurocytoma—primary and secondary treatment. Cancer 110:2276–2284

    Article  PubMed  Google Scholar 

  35. Rades D, Schild SE (2006) Treatment recommendations for the various subgroups of neurocytomas. J Neurooncol 77:305–309

    Article  PubMed  Google Scholar 

  36. Soylemezoglu F, Scheithauer BW, Esteve J, Kleihues P (1997) Atypical central neurocytoma. J Neuropathol Exp Neurol 56:551–556

    Article  PubMed  CAS  Google Scholar 

  37. Mackenzie IRA (1999) Central neurocytoma—histologic atypia, proliferation potential, and clinical outcome. Cancer 85:1606–1610

    Article  PubMed  CAS  Google Scholar 

  38. Lenzi J, Salvati M, Raco A, Frati A, Piccirilli M, Delfini R (2006) Central neurocytoma: a novel appraisal of a polymorphic pathology—our experience and a review of the literature. Neurosurg Rev 29:286–292

    Article  PubMed  Google Scholar 

  39. Rades D, Schild SE, Fehlauer F (2004) Prognostic value of the MIB-1 labeling index for central neurocytomas. Neurology 62:987–989

    Article  PubMed  Google Scholar 

  40. Lee J, Chang SM, McDermott MW, Parsa AT (2003) Intraventricular neurocytomas. Neurosurg Clin N Am 14:483–508

    Article  PubMed  Google Scholar 

  41. Paek SH, Han JH, Kim JW, Park CK, Jung HW, Park SH, Kim IH, Kim DG (2008) Long-term outcome of conventional radiation therapy for central neurocytoma. J Neurooncol 90:25–30

    Article  PubMed  Google Scholar 

  42. Schild SE, Scheithauer BW, Haddock MG, Schiff D, Burger PC, Wong WW, Lyons MK (1997) Central neurocytomas. Cancer 79:790–795

    Article  PubMed  CAS  Google Scholar 

  43. Rades D, Fehlauer F, Schild SE (2004) Treatment of atypical neurocytomas. Cancer 100:814–817

    Article  PubMed  Google Scholar 

  44. Rades D, Schild SE, Ikezaki K, Fehlauer F (2003) Defining the optimal dose of radiation after incomplete resection of central neurocytomas. Int J Radiat Oncol Biol Phys 55:373–377

    Article  PubMed  Google Scholar 

  45. Rades D, Schild SE (2006) Value of postoperative stereotactic radiosurgery and conventional radiotherapy for incompletely resected typical neurocytomas. Cancer 106:1140–1143

    Article  PubMed  Google Scholar 

  46. Neto MC, Ramina R, de Meneses MS, Arruda WO, Milano JB (2003) Peritioneal dissemination from central neurocytoma—case report. Arq Neuropsiquiatr 61:1030–1034

    Article  Google Scholar 

  47. Stapleton CJ, Walcott BP, Kahle KT, Codd PJ, Nahed BV, Chen L, Robison NJ, Delalle I, Goumnerova LC, Jackson EM (2012) Diffuse central neurocytoma with craniospinal dissemination. J Clin Neurosci 19:163–166

    Article  PubMed  Google Scholar 

  48. Amini E, Roffidal T, Lee A, Fuller GN, Mahajan A, Ketonen L, Kobrinsky N, Cairo MS, Wells RJ, Wolff JEA (2008) Central neurocytoma responsive to topotecan, ifosfamide, carboplatin. Pediatr Blood Cancer 51:137–140

    Article  PubMed  Google Scholar 

  49. Elek G, Slowik F, Eross L, Tóth S, Szabó Z, Bálint K (1999) Central neurocytoma with malignant course: neuronal and glial differentiation and craniospinal dissemination. Pathol Oncol Res 5:155–159

    Article  PubMed  CAS  Google Scholar 

  50. Eng DY, DeMonte F, Ginsberg L, Fuller GN, Jaeckle K (1997) Craniospinal dissemination of central neurocytoma—report of two cases. J Neurosurg 86:547–552

    Article  PubMed  CAS  Google Scholar 

  51. Brandes AA, Amista P, Gardiman M, Volpin L, Danieli D, Guglielmi B, Carollo C, Pinna G, Turazzi S, Monfardini S (2000) Chemotherapy in patients with recurrent and progressive central neurocytoma. Cancer 88:169–174

    Article  PubMed  CAS  Google Scholar 

  52. Ando K, Ishikura R, Morikawa T, Nakao N, Ikeda J, Matsumoto T, Arita N (2002) Central neurocytoma with craniospinal dissemination. Magn Reson Med Sci 1:179–182

    Article  PubMed  Google Scholar 

  53. Tran H, Medina-Flores R, Cerilli LA, Phelps J, Lee FC, Wong G, Turner P (2010) Primary disseminated central neurocytoma: cytological and MRI evidence of tumor spread prior to surgery. J Neurooncol 100:291–298

    Article  PubMed  Google Scholar 

  54. Ogawa Y, Sugawara T, Seki H, Sakuma T (2006) Central neurocytomas with MIB-1 labeling index over 10% showing rapid tumor growth and dissemination. J Neurooncol 79:211–216

    Article  PubMed  Google Scholar 

  55. Takao H, Nakagawa K, Ohtomo K (2003) Central neurocytoma with craniospinal dissemination. J Neurooncol 61:255–259

    Article  PubMed  Google Scholar 

  56. Amagasa M, Yuda F, Sat S, Kojima H (2008) Central neurocytoma with remarkably large rosette formation and rapid malignant progression: a clinicopathological follow-up study with autopsy report. Clin Neuropathol 27:252–257

    Article  PubMed  CAS  Google Scholar 

  57. Tomura N, Hirano H, Watanabe O, Watarai J, Itoh Y, Mineura K, Kowada M (1997) Central neurocytoma with clinically malignant behavior. Am J Neuroradiol 18:1175–1178

    PubMed  CAS  Google Scholar 

  58. Cook DJ, Christie SD, Macaulay RJB, Rheaume DE, Holness RO (2004) Fourth ventricular neurocytoma: case report and review of the literature. Can J Neurol Sci 31:558–564

    PubMed  Google Scholar 

  59. Bertalanffy A, Roessler K, Koperek O, Gelpi E, Prayer D, Knosp E (2005) Recurrent central neurocytomas. Cancer 104:135–142

    Article  PubMed  Google Scholar 

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Acknowledgments

The research project was supported by the OTKA fund No. 75833. The OTKA is an official grant provided by the Hungarian government via the Hungarian Scientific Academy.

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Correspondence to Petra Mozes.

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Mozes, P., Szanto, E., Tiszlavicz, L. et al. Clinical Course of Central Neurocytoma with Malignant Transformation—An Indication for Craniospinal Irradiation. Pathol. Oncol. Res. 20, 319–325 (2014). https://doi.org/10.1007/s12253-013-9697-y

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  • DOI: https://doi.org/10.1007/s12253-013-9697-y

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