Acta Neuropathologica

, Volume 109, Issue 2, pp 231–234 | Cite as

Ependymoma with neuropil-like islands: a case report with diagnostic and histogenetic implications

  • Marco Gessi
  • Carla Marani
  • Jennian Geddes
  • Antonella Arcella
  • Giovanna Cenacchi
  • Felice Giangaspero
Case Report

Abstract

We describe a case of ependymoma with neuronal differentiation in form of neuropil-like islands. A 6-year-old boy presented at clinical examination for a short history of headaches and vomiting. Brain computed tomography showed a large, partially cystic, parieto-occipital lesion. The tumor was composed by glial fibrillary acidic protein-positive round cells with a perivascular arrangement and scattered neuropil-like islands, showing intense positivity for synaptophysin. Despite radiotherapy, the tumor recurred, showing frank features of anaplasia, but lacking the neuropil-like islands. The histological features of the tumor are discussed in the light of the concept that neuronal differentiation can occur occasionally in gliomas of different lineage without affecting the expected biological behavior.

Keywords

Ependymoma Neuropil-like islands Neurocytic differentiation 

References

  1. 1.
    Broholm H, Madsen FF, Wagner AA, Laursen H (2002) Papillary glioneuronal tumor: a new tumor entity. Clin Neuropathol 21:1–4PubMedGoogle Scholar
  2. 2.
    Burger PC, Scheithauer BW, Vogel FS (2002) Surgical pathology of the nervous system and its coverings, 4th edn. Churchill-Livingstone, London, pp 290–292Google Scholar
  3. 3.
    Cenacchi G, Giangaspero F (2004) Emerging tumor entities and variants of the CNS neoplasms. J Neuropathol Exp Neurol 63:185–192PubMedGoogle Scholar
  4. 4.
    Gyure KA, Prayson RA (1997) Subependymal giant cell astrocytoma: a clinicopathologic study with HMB-45 and Mib 1 immunohistochemical analysis. Mod Pathol 10:313–317PubMedGoogle Scholar
  5. 5.
    Harris BT, Horoupian DS (2000) Spinal cord glioneuronal tumor with “rosetted” neuropil islands and meningeal dissemination: a case report. Acta Neuropathol 100:575–579CrossRefPubMedGoogle Scholar
  6. 6.
    Hasselblatt M, Paulus W (2003) Sensitivity and specificity of epithelial membrane antigen staining patterns in ependymomas. Acta Neuropathol 106:385–388CrossRefPubMedGoogle Scholar
  7. 7.
    Keyvani K, Rickert CH, Wild K von, Paulus W (2001) Rosetted glioneuronal tumor: a case with proliferating neuronal nodules. Acta Neuropathol 101:525–528PubMedGoogle Scholar
  8. 8.
    Kleihues P, Cavenee WK (2000) Pathology and genetics of tumours of the nervous system, 3rd edn. WHO/IARC, Lyon, pp 96–114Google Scholar
  9. 9.
    Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, Cavenee WK (2002) The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol 61:215–225Google Scholar
  10. 10.
    Komori T, Scheitahuer BW, Anthony DC, Rosenblum MK, McLendon RE, Scott RM, Okazaki H, Kobayashi M (1998) Papillary glioneuronal tumor: a new variant of mixed neuronal-glial neoplasm. Am J Surg Pathol 22:1171–1183CrossRefPubMedGoogle Scholar
  11. 11.
    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 26:582–591CrossRefPubMedGoogle Scholar
  12. 12.
    Massimino M, Gandola L, Giangaspero F, Sandri A, Valagussa P, Perilongo G, Garre ML, Ricardi U, Forni M, Genitori L, et al (2004) Hyperfractioned radiotherapy and chemotherapy for childhood ependymoma: final results of the first prospective AIEOP (Associazione Italiana di Ematologia-Oncologia Pediatrica) study. Int J Radiat Oncol Biol Phys 58:1336–1345CrossRefPubMedGoogle Scholar
  13. 13.
    McLendon RE, Bentley RC, Parisi JE, Tien RD, Harrison JC, Tarbell NJ, Billitt AL, Gualtieri RJ, Friedman HS (1997) Malignant supratentorial glial-neuronal neoplasms: report of two cases and review of the literature. Arch Pathol Lab Med 121:485–492PubMedGoogle Scholar
  14. 14.
    Perry A, Scheithauer BW, Macaulay RJ, Raffel C, Roth KA, Kros JM (2002) Oligodendrogliomas with neurocytic differentiation. A report of 4 cases with diagnostic and histogenetic implications. J Neuropathol Exp Neurol 61:947–955PubMedGoogle Scholar
  15. 15.
    Powell SZ, Yachnis AT, Rorke LB, Rojiani AM, Eskin TA (1996) Divergent differentiation in pleomorphic xanthoastrocytoma: evidence for a neuronal element and possible relationship to ganglion cell tumors. Am J Surg Pathol 20:80–85CrossRefPubMedGoogle Scholar
  16. 16.
    Prayson RA, Abramovich CM (2000) Glioneuronal tumor with neuropil-like islands. Hum Pathol 31:1435–1438CrossRefPubMedGoogle Scholar
  17. 17.
    Teo JG, Gultekin SH, Bilsky M, Gutin P, Rosenblum MK (1999) A distinctive glioneuronal tumor of adult cerebrum with neuropil-like (including “rosetted”) islands. Report of 4 cases. Am J Surg Pathol 23:502–510CrossRefPubMedGoogle Scholar
  18. 18.
    Wiestler OD, Schiffer D, Coons SW, Prayson RA, Rosenblum MK. (2000) Ependymoma. In: Kleihues P, Cavanee WK, (eds) Pathology and genetics of tumours of the nervous system, 3rd edn. WHO/IARC, Lyon, pp 72–76Google Scholar

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • Marco Gessi
    • 1
  • Carla Marani
    • 1
  • Jennian Geddes
    • 3
  • Antonella Arcella
    • 2
  • Giovanna Cenacchi
    • 4
  • Felice Giangaspero
    • 1
    • 2
    • 5
  1. 1.Department of Experimental Medicine and PathologyUniversity of Rome “La Sapienza”RomeItaly
  2. 2.IRCCS NeuromedPozzilliItaly
  3. 3.Department of Histopathology and Morbid AnatomyRoyal London HospitalLondonUK
  4. 4.Department of Radiology and PathologyUniversity of BolognaBolognaItaly
  5. 5.Neuropatologia c/o NeurochirurgiaPoliclinico Umberto IRomaItaly

Personalised recommendations