Skip to main content

Advertisement

Log in

Brain tumors of childhood: nosological and diagnostic problems

  • Original Papers
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Brain tumors containing undifferentiated cells were selected from a series of 504 childhood brain tumors; 117 were analyzed. Most tumors were medulloblastomas, followed by cerebral neuroblastomas, pineocytomas-blastomas, ependymoblastomas, and polar spongioblastomas. Of each oncotype, the main histological features were evaluated, including differentiation and the most important prognostic factors. The terminology and different tumor entities are discussed in light of the recent PNET system. The usefulness of its application is evaluated in relation to prognosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Abay EO, Edward RL, Grado GL, Bruckman JE, Forbes GS, Gomez MR, Scott M (1981) Pineal tumors in children and adolescents: treatment by CSF shunting and radiotherapy. J Neurosurg 55:889–895

    Google Scholar 

  2. Azzarelli B, Richards DE, Anton AH, Roessmann U (1977) Central neuroblastoma: electron microscopic observations and catecholamine determinations. J Neuropathol Exp Neurol 36:384–397

    Google Scholar 

  3. Bailey P, Cusching HA (1926) A classification of the tumors of the glioma group on a histogenetic basis with a correlated study of prognosis. Lippincott, Philadelphia

    Google Scholar 

  4. Barnard RO, Pambakian H (1980) Astrocytic differentiation in medulloblastoma. J Neurol Neurosurg Psychiatry 43:1041–1044

    Google Scholar 

  5. Becker LE, Hinton D (1983) Primitive neuroectodermal tumors of the central nervous system. Hum Pathol 14:538–550

    Google Scholar 

  6. Bennet JP, Rubinstein LJ (1984) The biological behavior of primary cerebral neuroblastoma: a reappraisal of the clinical course in a series of 70 cases. Ann Neurol 16:21–27

    Google Scholar 

  7. Berger MS, Edwards MS, Wara WM, Levin VA, Wilson CB (1983) Primary cerebral neuroblastoma. Long-term follow-up review and therapeutic guidelines. J Neurosurg 59:418–423

    Google Scholar 

  8. Boesel CP, Suhan JP, Bradel EJ (1978) Ultrastructure of primitive neuroectodermal neoplasms of the central nervous system. Cancer 42:194–201

    Google Scholar 

  9. Borit A, Blackwood W (1979) Pineocytomas with astrocytomatous differentiation. J Neuropathol Exp Neurol 38:253–258

    Google Scholar 

  10. Borit A, Blackwood W, Mair WGP (1980) The separation of pineocytoma from pineoblastoma. Cancer 45:1408–1418

    Google Scholar 

  11. Bruce DA, Allen JA (1985) Tumor staging for pineal region tumors of childhood. Cancer 56:1792–1794

    Google Scholar 

  12. Burger PC, Grahmann FC, Bliestle A, Kleihues P (1987) Differentiation in the medulloblastoma. A histological and immunohistochemical study. Acta Neuropathol (Berl) 73:115–123

    Google Scholar 

  13. Burger PC, Kleihues P (1988) Cytologic composition of untreated glioblatoma multiforme: a postmortem study of 18 cases. Cancer (in press)

  14. Camins MB, Cravioto HM, Epstein F, Ransohoff J (1980) Medulloblastoma: an ultrastructural study. Evidence for astrocytic and neuronal differentiation. Neurosurgery 6:398–411

    Google Scholar 

  15. Caputy AJ, McCullough DC, Manz HJ, Patterson K, Hammock MK (1987) A review of the factors influencing the prognosis of medulloblastoma. The importance of cell differentiation. J Neurosurg 66:80–87

    Google Scholar 

  16. Chatty EM, Earle KM (1971) Medulloblastoma: a report of 201 cases with emphasis on the relationship of histologic variance to survival. Cancer 28:977–983

    Google Scholar 

  17. Coffin CM, Mukai K, Dehner LP (1983) Glial differentiation in medulloblastomas. Am J Surg Pathol 7:555–565

    Google Scholar 

  18. Cruz-Sanchez FF, Haustein J, Rossi ML, Cervós-Navarro J, Hughes JT (1988) Ependymoblastoma: a histological, immunohistological and ultrastructural study of five cases. Histopathology 12:17–27

    Google Scholar 

  19. Dastur DK (1982) Cerebral ganglio-neuroblastoma: an unusual brain tumour of the neuron series. J Neurol Neurosurg Psychiatry 45:139–142

    Google Scholar 

  20. De Armond SJ, Eng LF, Rubinstein LJ (1980) The application of glial fibrillary acidic (GFA) protein immunohistochemistry in neurooncology. A progress report. Pathol Res Pract 168:374–394

    Google Scholar 

  21. Deck JHN, Eng LF, Bigbee J, Woodcock SM (1978) The role of glial fibrillary acidic protein in the diagnosis of central nervous system tumors. Acta Neuropathol (Berl) 42:183–190

    Google Scholar 

  22. De Girolami U, Zvaigzne O (1973) Modification of the Achucarro-Hortega pineal stain for paraffin-embedded formalin-fixed tissue. Stain Technol 48:48–50

    Google Scholar 

  23. Del Cerro MP, Snider RS (1972) Studies on the developing cerebellum. II. The ultrastructure of the external granular layer. J Comp Neurol 144:131–138

    Google Scholar 

  24. Delpech A, Delpech B, Girard N, Vidard MN (1978) Localisation immunohistologique des 3 antigènes associés au tissue nerveux (GFA, ANS, brain glycoprotein). Biol Cell 32:207–214

    Google Scholar 

  25. Donoso LA, Folberg R, Arbizo V (1985) Retinal S-antigen and retinoblastoma: a monoclonal antibody histopathologic study. Arch Ophthalmol 103:857–885

    Google Scholar 

  26. Duffner PK, Cohen ME, Heffner RR, Freeman AI (1981) Primitive neuroectodermal tumors of the cerebrum in childhood. J Neurosurg 55:376–381

    Google Scholar 

  27. Edwards MSB, Hudgins RJ, Wilson CB, Levin VA, Wara WM (1988) Pineal region tumors in children. J Neurosurg 68:689–697

    Google Scholar 

  28. Eng LF, Rubinstein LJ (1978) Contribution of immunohistochemistry to diagnostic problems of human cerebral tumors. J Histochem Cytochem 26:513–522

    Google Scholar 

  29. Ermel AE, Brucher JM (1974) Arguments ultrastructuraux en faveur de l'appartenence du medulloblastome à la ligne neuronale. Acta Neurol Belg 74:208–220

    Google Scholar 

  30. Feigin I, Epstein F, Mangiardi J (1983) Extensive advanced maturation of medulloblastoma to astrocytoma and ependymoma. J Neurooncol 1:95–108

    Google Scholar 

  31. Foerster O, Gagel O (1939) Das umschriebene Arachnoidalsarkom des Kleinhirns. Z Ges Neurol Psychiatr 164:565–580

    Google Scholar 

  32. Friedman HS, Burger PC, Bigner SH, Trojanowski JQ, Halperin EC, Bigner DD (1985) Establishment and characterization of the human medulloblastoma tumor cell line D283 MED. J Neuropathol Exp Neurol 44:592–605

    Google Scholar 

  33. Gaffney CC, Sloane JP, Bradley NJ, Bloom HJG (1985) Primitive neuroectodermal tumors of the cerebrum. Pathology and treatment. J Neurooncol 3:23–33

    Google Scholar 

  34. Gangemi M, Maiuri F, Fiorillo A, Migliorati R, Pettinati G, Del Giudice E (1987) Primary cerebral neuroblastomas. Neurochirurgia 30:48–52

    Google Scholar 

  35. Ghandour MS, Labourdette G, Vincendon G (1981) A biochemical and immunohistological study of S 100 protein in developing rat cerebellum. Dev Neurosci 4:98–103

    Google Scholar 

  36. Giordana MT, Mauro A, Migheli A, Schiffer D (1983) Contributions of immunohistochemistry to the problem of differentiation in medulloblastoma. Ital J Neurol Sci 4:411–415

    Google Scholar 

  37. Goldammer D, Goebel HH (1980) Dense core vesicles in the desmoplastic variant of cerebral neuroblastoma. Acta Neuropathol (Berl) 50:81–83

    Google Scholar 

  38. Gullotta F (1967) Das sogenannte Medulloblastom. Springer, Berlin Heidelberg New York

    Google Scholar 

  39. Gullotta F, Kuchelmeister K (1986) GFAP bei Hirntumoren. Verh Dtsch Ges Pathol 70:380–381

    Google Scholar 

  40. Haimoto H, Takahasi Y, Koshikawa T, Nagura H, Kato K (1985) Immunohistochemical localization of gamma-enolase in normal human tissues other than nervous and neuroendocrine tissues. Lab Invest 52:257–263

    Google Scholar 

  41. Hart MN, Earle KM (1973) Primitive neuroectodermal tumors of the brain in children. Cancer 32:890–897

    Google Scholar 

  42. Hassoun J, Gambarella D, Peragut JC, Toga M (1983) Specific ultrastructural markers of human pinealomas: a study of four cases. Acta Neuropathol (Berl) 62:31–40

    Google Scholar 

  43. Herman MM, Rubinstein LJ (1978) Divergent glial and neuronal differentiation in a human medulloblastoma maintained in an organ culture system. J Neuropathol Exp Neurol 37:627

    Google Scholar 

  44. Herman MM, Rubinstein LJ (1984) Divergent glial and neuronal differentiation in a cerebellar medulloblastoma in an organ culture system: in vitro occurrence of synaptic ribbons. Acta Neuropathol (Berl) 65:10–24

    Google Scholar 

  45. Herpers MJHM, Budka H (1985) Primitive neuroectodermal tumors including the medulloblastoma: glial differentiation signaled by immunoreactivity for GFAP is restricted to the pure desmoplastic medulloblastoma (“arachnoidal sarcoma of the cerebellum”). Clin Neuropathol 4:12–18

    Google Scholar 

  46. Herrick M, Rubinstein LJ (1979) The cytological differentiation potential of pineal parenchymal neoplasms (true pinealomas): a clinical study of 28 tumors. Brain 102:289–320

    Google Scholar 

  47. Hirano A, Shin W-Y (1979) Unattached presynaptic terminals in a cerebellar neuroblastoma in the human. Neuropathol Appl Neurobiol 5:63–70

    Google Scholar 

  48. Horten BC, Rubinstein LJ (1976) Primary cerebral neuroblastoma: a clinicopathological study of 35 cases. Brain 99:735–756

    Google Scholar 

  49. Kadin ME, Rubinstein LJ, Nelson JS (1970) Neonatal cerebellar medulloblastoma originating from the fetal external granular layer. J Neuropathol Exp Neurol 29:583–600

    Google Scholar 

  50. Katsetos CD, Herman MM, Frankfurter A, Collins VP, Walker CC, Barnard RO, Rubinstein LJ (1988) Dual neuronal and astroglial differentiation in cerebellar desmoplastic medulloblastomas: a further immunohistochemical characterization of the “pale islands.” 64th Annual Meeting, American Association of Neuropathologists, Charleston, South Carolina

  51. Kosnick EJ, Boesel CT, Bay J, Sayers MP (1978) Primitive neuroectodermal tumors of the central nervous system in children. J Neurosurg 48:741–746

    Google Scholar 

  52. Latchaw JP, Hahn JF, Moylan DJ, Humphries R, Mealey J (1985) Medulloblastoma: period of risk reviewed. Cancer 55:186–189

    Google Scholar 

  53. Langford LA, Camel MH (1987) Palisades pattern in cerebral neuroblastoma mimicking the primitive polar spongioblastoma: an ultrastructural study. Acta Neuropathol (Berl) 73:153–159

    Google Scholar 

  54. Lewis PD, Fülöp Z, Hajós F, Balázs R, Woodhams PL (1977) Neuroglia in the internal granular layer of the developing rat cerebellar cortex. Neuropathol Appl Neurobiol 3:183–190

    Google Scholar 

  55. Lorentzen M, Hägerstrand I (1980) Congenital ependymoblastoma. Acta Neuropathol (Berl) 49:71–74

    Google Scholar 

  56. Mannoji H, Becker LE (1988) Ependymal and choroid plexus tumors. Cytokeratin and GFAP expression. Cancer 61:1377–1385

    Google Scholar 

  57. Markesbery WR, Haugh RM, Young AB (1981) Ultrastructure of pineal parenchymal neoplasms. Acta Neuropathol (Berl) 55:143–149

    Google Scholar 

  58. McLendon RE, Burger PC (1987) The primitive neuroectodermal tumor: a cautionary view. J Pediatr Neurosci 3:1–8

    Google Scholar 

  59. Mørk SV, Rubinstein LJ (1985) Ependymoblastoma: a reappraisal of a rare embryonal tumor. Cancer 55:1356–1542

    Google Scholar 

  60. Müller W, Schaefer HE (1974) Beitrag zur morphologischen Onkotypie des Medulloblastoms. Acta Neuropathol (Berl) 30:51–61

    Google Scholar 

  61. Nielsen SL, Wilson CB (1975) Ultrastructure of a “pineocytoma.” J Neuropathol Exp Neurol 34:148–158

    Google Scholar 

  62. Olivera-Rabiela JE, Altamirano Dimas M, Cisneros Davila A (1971) Ependymoblastoma: informe de uno caso y revision de la literatura. Pathologia 9:27–33

    Google Scholar 

  63. Packer RJ, Sutton LN, Rorke BL, Littman PA, Sposto R, Rosenstock JG, Bruce DA, Shut L (1984) Prognostic importance of cellular differentiation in medulloblastoma of childhood. J Neurosurg 61:296–301

    Google Scholar 

  64. Palmer JO, Kasselberg AG, Netsky MG (1981) Differentiation of medulloblastoma: studies including immunohistochemical localization of glial fibrillary acidic protein. J Neurosurg 55:161–169

    Google Scholar 

  65. Park TS, Hoffman HJ, Hendrick EB (1983) Medulloblastoma: clinical presentation and management. Experience at the Hospital for Sick Children. J Neurosurg 58:543–552

    Google Scholar 

  66. Parker JC, Mortara RH, McCloskey JJ (1975) Biological behaviour of the primitive neuroectodermal tumors: significant supratentorial childhood gliomas. Surg Neurol 4:383–388

    Google Scholar 

  67. Pearl GS, Takei Y (1981) Cerebellar neuroblastoma: nosology as it relates to medulloblastoma. Cancer 47:772–779

    Google Scholar 

  68. Pearl GS, Mirra SS, Miles ML (1981) Intracerebral ganglioneuroblastoma with intracytoplasmatic microtubular aggregates. Case report and ultrastructural study. Ultrastruct Pathol 2:337–342

    Google Scholar 

  69. Pearson J, Milstoc M, Harris J, Budzilovich GN, Feigin I (1976) Anaplastic neuronal tumors of brain. Cancer 38:1424–1437

    Google Scholar 

  70. Perentes E, Rubinstein LJ, Herman MM, Donoso LA (1986) S-Antigen immunoreactivity in human pineal glands and pineal parenchymal tumors: a monoclonal antibody study. Acta Neuropathol (Berl) 71:224–227

    Google Scholar 

  71. Queiroz LS, Lopes de Faria J, Cruz Neto JN (1975) An ependymoblastoma of the pons. J Pathol 115:207–210

    Google Scholar 

  72. Rhodes RH, Davis RL, Kassel SH, Clague BH (1978) Primary cerebral neuroblastoma: a light- and electron-microscopic study. Acta Neuropathol (Berl) 41:124–199

    Google Scholar 

  73. Ringertz N, Nordenstam H, Flyger G (1954) Tumors of the pineal region. J Neuropathol Exp Neurol 13:540–561

    Google Scholar 

  74. Roessmann U, Velasco ME, Gambetti P, Autilio-Gambetti L (1983) Neuronal and astrocytic differentiation in human neuroepithelial neoplasms. An immunohistochemical study. J Neuropathol Exp Neurol 42:113–121

    Google Scholar 

  75. Rorke LB (1983) The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors. J Neuropathol Exp Neurol 42:1–15

    Google Scholar 

  76. Rorke LB, Gilles FH, Davis RL, Becker LE (1985) Revision of the World Health Organization Classification. Cancer 56:1869–1886

    Google Scholar 

  77. Rubinstein LJ (1970) The definition of ependymoblastoma. Arch Pathol 90:35–45

    Google Scholar 

  78. Rubinstein LJ (1985) Embryonal central neuroepithelial tumors and their differentiating potential. A cytogenetic view of a complex neuro-oncological problem. J Neurosurg 62:795–805

    Google Scholar 

  79. Rubinstein LJ (1987) The correlation of neoplastic vulnerability with central neuroepithelial cytogeny and glioma differentiation. J Neurooncol 5:11–27

    Google Scholar 

  80. Rubinstein LJ, Northfield DWC (1964) Medulloblastoma and so-called “arachnoidal cerebellar sarcoma.” A critical reexamination of a nosological problem. Brain 87:379–412

    Google Scholar 

  81. Rubinstein LJ, Okazaki H (1970) Gangliogliomatous differentiation in a pineocytoma. J Pathol 102:27–32

    Google Scholar 

  82. Rubinstein LJ, Herman MM, Hanbery JW (1974) The relationship between differentiating medulloblastoma and dedifferentiating diffuse cerebellar astrocytoma: light-electron microscope tissue and organ culture observation. Cancer 33:675–690

    Google Scholar 

  83. Russell DS (1955) Polar spongioblastomas: their place in the glioma series. II International Congress of Neuropathology, London. Exc Med Neurol Psychiatry 8:818

    Google Scholar 

  84. Russell DS, Cairns H (1947) Polar spongioblastomas. Arch Histol (Buenos Aires) 3:423–432

    Google Scholar 

  85. Russell DS, Rubinstein LJ (1989) Pathology of tumors of the nervous system, 5th edn. Arnold, London

    Google Scholar 

  86. Sakaki S, Mori Y, Motozaki T, Nakagawa K, Matsuoka K (1981) A cerebral neuroblastoma with extracranial metastases. Surg Neurol 16:53–59

    Google Scholar 

  87. Schiffer D, Giordana MT, Mauro A, Migheli A (1983) Glial fibrillary acid protein (GFAP) in human cerebral tumors. An immunohistochemical study. Tumori 69:95–104

    Google Scholar 

  88. Schiffer D, Giordana MT, Germano I, Mauro A (1986) Anaplasia and heterogeneity of GFAP expression in gliomas. Tumori 72:163–170

    Google Scholar 

  89. Schindler E, Gullotta F (1983) Glial fibrillary acidic protein in medulloblastomas and other embryonic CNS tumors of children. Virchows Arch [A] 395:263–275

    Google Scholar 

  90. Schmitt HP (1983) Rapid anaplastic transformation of gliomas in childhood. Neuropediatrics 14:137–143

    Google Scholar 

  91. Schin WY, Laufer H, Lee YC, Aftalion B, Hirano A, Zimmerman HM (1978) Fine structure of a cerebellar neuroblastoma. Acta Neuropathol (Berl) 50:139–142

    Google Scholar 

  92. Sobel RA, Trice JE, Nielsen SL, Ellis WG (1981) Pineoblastoma with ganglionic and glial differentiation. Acta Neuropathol (Berl) 55:243–246

    Google Scholar 

  93. Stefanko SZ, Manschot W (1979) Pineoblastoma with retinoblastomatous differentiation. Brain 102:321–332

    Google Scholar 

  94. Torres LF, Grant N, Harding BN, Scaravilli F (1985) Intracerebral neuroblastoma. Report of a case with neuronal maturation and long survival. Acta Neuropathol (Berl) 68:110–114

    Google Scholar 

  95. Tso MOM (1980) Clues to the cells of origin in retinoblastoma. Int Ophthalmol Clin 20:191–197

    Google Scholar 

  96. Velasco ME, Ghorbria MW, Ross ER (1985) Neuron-specific enolase and neurofilament protein as markers of differentiation in medulloblastoma. Surg Neurol 23:177–182

    Google Scholar 

  97. Vinores SA, Bonnin JM, Rubinstein LJ, Marangos PJ (1984) Immunohistochemical demonstration of neuron-specific enolase in neoplasms of the CNS and other tissues. Arch Pathol Lab Med 108:536–540

    Google Scholar 

  98. Yagashita S, Itah Y, Chiba Y, Yamashita T, Nakazima F, Kuwabara T (1980) Cerebellar neuroblastoma: a light and ultrastructural study. Acta Neuropathol (Berl) 50:139–142

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

Supported by grant no. 87.01446.44 from the Italian National Research Council (CNR), Special Project “Oncology”, Rome, and from the Italian Association for Cancer Research (AIRC), Milan

Rights and permissions

Reprints and permissions

About this article

Cite this article

Schiffer, D., Giordana, M.T. & Vigliani, M.C. Brain tumors of childhood: nosological and diagnostic problems. Child's Nerv Syst 5, 220–229 (1989). https://doi.org/10.1007/BF00271023

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00271023

Key words

Navigation