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Primitive neuroectodermal tumours (PNETs) located in the spinal canal; the relevance of classification as central or peripheral PNET

Case report of a primary spinal PNET occurrence with a critical literature review

  • Clinical--Patient Studies
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Summary

Intraspinal location of central PNET (cPNET) is very rare. We present a case, critically review all publications of primary intraspinal cPNET occurrence and discuss tendencies in clinical presentation. In several previous attempts to summarise, authors often confused cPNET with peripheral PNET (pPNET). cPNET and pPNET are different entities with different immunohistochemical profiles and genetic backgrounds. Clinically, they are both aggressive tumours, but exhibit different characteristics in their local manifestation and metastatic spread. Survival rates are quite similar provided that treatment is applied according to the established protocols. Protocols in cPNET treatment differ from those for pPNET as regards the order of the treatment sub-modalities, specific chemotherapeutic regimen and intensity, radiation dose and its extent and consequently, the side effects. Therefore, failure to distinguish cPNET from pPNET leads to clinical guidance and treatment proposals based on false assumptions, which might effect outcomes. Often, distinguishing between cPNET and pPNET is easy, because they occur in different location. In the case of intraspinal tumour location, however, the differentiation is crucial because both primary cPNET and pPNET can occur intraspinally, even though this is rare. Nowadays, demonstrating the expression of MIC2 glycoprotein by immunocytochemical staining (CD99) showing the specific EWS-FLI1 chimeric gene presence in pPNET, offers an easy way of making a differential diagnosis between cPNET and pPNET.

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References

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

    Article  PubMed  CAS  Google Scholar 

  2. Cohn S, Diagnosis and classification of the small round cell tumors of childhood AJP 155:11–15 1999

    PubMed  CAS  Google Scholar 

  3. Cenacchi G, Giangaspero F, Emerging tumor entities and variants of CNS Neoplasms J Neuropath Exp Neurol 63:185–192 2004

    PubMed  Google Scholar 

  4. Russo C, Pellarin M, Tingby O, et al. Comparative genomic hybridization in patients with supratentorial and infratentorial primitive neuroectodermal tumors Cancer 86:331–339 1999

    Article  PubMed  CAS  Google Scholar 

  5. Weidner N, Tjoe J, Immunohistochemical profile of monoclonal antibody O13: antibody that recognizes glycoprotein p30/32MIC2 and is useful in diagnosing Ewing’s sarcoma and peripheral neuroepithelioma Am J Surg Pathol 18:486–494 1994

    Article  PubMed  CAS  Google Scholar 

  6. Winters JL, Geil J, O´Conner W, Immunohistology, cytogenetics and molecular studies of small round cell tumors of childhood Ann Clin Lab Sc 25:66–75 1995

    CAS  Google Scholar 

  7. McNeil DE, Cote TR, Clegg L, Rorke LB, Incidence and trends in pediatric malignancies medulloblastoma/ primitive neuroectodermal tumor: a SEER update. Surveillance Epidemiologiy and End Results Med Pediatr Oncol 39(3):190–194 2002

    Article  PubMed  Google Scholar 

  8. Enzinger FM, Weiss SW, 1995 Primitive neuroectodermal tumors and related lesions. In: Enzinger FM, Weiss SW, (eds), Soft Tissue Tumors. (3). Mosby Year Book, St Louis 929–964

    Google Scholar 

  9. Kovar H, Ewing’s sarcoma and peripheral primitive neuroectodermal tumors after their union Curr Opin Oncol 10:334–342 1998

    Article  PubMed  CAS  Google Scholar 

  10. Pomeroy SL, Tamayo P, Gaasenbeek M, et al. Prediction of central nervous system embryonal tumour outcome based on gene expression Nature (London) 415:436–442 2002

    Article  CAS  ADS  Google Scholar 

  11. Jallo GJ, Bong-Soo K, Epstein F, The current management of intramedullary neoplasms in children and young adults Ann Neurosurg 1(1):1–13 2001

    Google Scholar 

  12. Hadfield MG, Quezado MM, Williams RL, et al. Ewing’s family of tumors involving structures related to the central nervous system: a review Pediatr Dev Path 3:203–210 2000

    Article  CAS  Google Scholar 

  13. Innocenzi G, Raco A, Cantore G, et al. Intramedullary astrocytomas and ependymomas in the pediatric age group: a retrospective study Childs Nerv Syst 12:776–780 1996

    Article  PubMed  CAS  Google Scholar 

  14. Fletscher CDM, 1995 Peripheral neural tumors. In: Fletscher CDM, (ed), Diagnostic Histopathology Of Tumors. Churchill Livingstone, Edinburgh 1239–1241

    Google Scholar 

  15. Ishii N, Hiraga H, Sawamura Y, et al. Alternative EWS-FLI1 fusion gene and MIC2 expresssion in peripheral and central primitive neuroectodermal tumors Neuropathology 21:40–44 2001

    Article  PubMed  CAS  Google Scholar 

  16. Kleihues P, Louis DN, Scheithauer BW, et al. The WHO classification of tumors of the nervous system J Neuropath Exp Neurol 61:215–225 2002

    PubMed  Google Scholar 

  17. Li MH, Holtas S, MR imaging of spinal intramedullary tumors Acta Radiol 32(6):505–513 1991

    Article  PubMed  CAS  Google Scholar 

  18. Lopez-Alberola R, Bhattacharjee M, Borne J, Lee DA, Atypical features of medulloblastoma on magnetic resonance: case study and review of literature J Child Neurol 18(1):64–672003

    Article  PubMed  Google Scholar 

  19. Eberhart CG, Kepner JL, Goldthwaite PT, et al. Histopathologic grading of medulloblastomas; a pediatric oncology group study Cancer 94:552–560 2002

    Article  PubMed  Google Scholar 

  20. Smith DR, Hardman JM, Earle KM, et al. Metastasizing neuroectodermal tumours of the central nervous system J Neurosurg 31:50–58 1969

    Article  PubMed  CAS  Google Scholar 

  21. Kosnik Ej, Boesel C, Bay J, et al. Primitive neuroectodermal tumours of the central nervous system in the children J Neurosurg 48:741–746 1978

    PubMed  Google Scholar 

  22. Kepes JJ, Belton K, Roessmann U, et al. Primitive neuroectodermal tumours of the cauda equina in adults with no detectable primary intracranial neoplasm: three case studies Clin Neuropathol 4:1–11 1985

    PubMed  CAS  Google Scholar 

  23. Sevick RJ, Johns RD, Curry BJ, et al. Primary spinal neuroectodermal tumour with extraneural metastasis AJNR 8:1151–1152 1987

    PubMed  CAS  Google Scholar 

  24. Liu HM, Yang WC, Garcia RL, et al. Intraspinal primitive neuroectodermal tumour arising from the sacral neve root J Comput Tomogr 11:350–354 1987

    Article  PubMed  CAS  Google Scholar 

  25. Jaksche H, Wockel W, Wernert N, Primary spinal medulloblastomas? Neurosurg Rev 11:259–265 1998

    Article  Google Scholar 

  26. Freyer DR, Hutchinson RJ, McKeever PE, Primary primitive neuroectodermal tumour of the spinal cord associated with neural tube defect Pediatric Neurosci 15:181–187 1989

    Article  CAS  Google Scholar 

  27. Ogasawara H, Kiva K, Kurisu K, et al. Intracranial metastasis from a spinal cord primitive neuroectodermal tumour : case report Surg Neurol 37:307–312 1992

    Article  PubMed  CAS  Google Scholar 

  28. McDermott VG, El-Jabbour, Sellar RJ, et al. Primitive neuroectodermal tumour of the cauda equina Neuroradiology 36:228–230 1994

    Article  PubMed  CAS  Google Scholar 

  29. Kwon OK, Wang KC, Ki IO, et al. Primary intramedullary spinal cord primitive neuroectodermal tumour with intracranial seeding in an infant Child’s Nerv Syst 12:633–636 1996

    Article  PubMed  CAS  Google Scholar 

  30. Deme S, Ang L, Skaf G, Rowed D, Primary intramedullary Primitive Neuroectodermal Tumor of the Spinal Cord: Case report and Review of the Literature Neurosurg 41(6):1417–1420 1997

    Article  CAS  Google Scholar 

  31. Koot RW, Henneveld H, Albrecht K, et al. Two children with unusual causes of torticollis: primitive neuroectodermal tumour and Grisel’s syndrome Ned Tijdschr Geneeskd 142:1030–1033 1998

    PubMed  CAS  Google Scholar 

  32. Papadatos D, Albrecht S, Mohr G, del Carpio R, Exophytic primitive neuroectodermal tumor of the spinal cord AJNR 19:787–789 1998

    PubMed  CAS  Google Scholar 

  33. Dorfmüller G, Würtz FG, Unschaden HW, Kleinert R, Ambros PF, Intraspinal primitive neuroectodermal tumour: report of two ases and review of literature Acta Neurochir 141:1169–1175 1999

    Article  Google Scholar 

  34. Isotalo PA, Agbi C, Davidson B, Girard A, Verma S, et al. Primary primitive neuroectodermal tumor of the cauda equina Hum Pathol 31(8):999–1001 2000

    Article  PubMed  CAS  Google Scholar 

  35. Virani MJ, Jain S, Primary intraspinal primitive neuroectodermal tumor (PNET): a rare occurrence Neurol India 50:75–80 2002

    PubMed  CAS  Google Scholar 

  36. Mawrin C, Synowitz HJ, Kirches E, Kutz E, Dietzman K, Weis S, Primary primitive neuroectodermal tumor of the spinal cord: case report and review of literature Clin Neurol Neurosurg 104:36–40 2002

    Article  PubMed  Google Scholar 

  37. Reihani-Kermani H, Amizadeh B, Primary intraspinal primitive neuroectodermal tumors: report of a case Arch Iranian Med 5(4):262–266 2002

    Google Scholar 

  38. Albrecht CF, Weiss E, Schulz-Schaeffer WJ, Albrecht T, Fauser S, et al. Primary intraspinal primitive neuroectodermal tumor: report of two cases and review of literature J Neur Onc 61:113–120 2003

    Article  Google Scholar 

  39. Izycka E, Debiec M, Wasag B, Wozniak A, Gasecki D, et al. A unique occurrence of a cerebral atypical teratoid/rhabdoid tumor in an infant and a spinal canal primitive neuroectodermal tumor in her father J Neur Onc 61:219–225 2003

    Article  Google Scholar 

  40. Akyüz M, Demiral AN, Gürer IE, Uçar T, Tuncer R, Case report. Primary primitive neuroectodermal tumor of the cauda equina with intracranial seeding Acta Neurochir 92(2):684–688 2004

    Google Scholar 

  41. Nikitakis N, Salama A, Malley BW, Ord RA, Papadimitrou JC: 2003 Malignant peripheral neuroectodermal tumor- peripheral neuroepithelioma of the head: a clinicopathologic study of five cases and review of literature. Head & Neck June 488–498

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Authors and Affiliations

  1. Academic Hospital of the Vrije Universiteit Brussel (AZVUB), Brussels, Belgium

    W.A. Kampman

  2. Erasmus Medical Center, Rotterdam, The Netherlands

    J.M. Kros, T.H.R. De Jong & M.H. Lequin

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  1. W.A. Kampman
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  2. J.M. Kros
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  3. T.H.R. De Jong
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  4. M.H. Lequin
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Correspondence to W.A. Kampman.

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Address for offprints: W.A. Kampman, P/a: Academisch Ziekenhuis VUB, Secretariaat pediatrie, Laarbeeklaan 101, 1090 Jette, Belgium; Tel.: +32-2-477-5781; Fax: +32-2-477-5735; E-mail: kampmanwendy@hotmail.com

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Kampman, W., Kros, J., De Jong, T. et al. Primitive neuroectodermal tumours (PNETs) located in the spinal canal; the relevance of classification as central or peripheral PNET. J Neurooncol 77, 65–72 (2006). https://doi.org/10.1007/s11060-005-9006-z

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  • DOI: https://doi.org/10.1007/s11060-005-9006-z

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