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Primary peripheral primitive neuroectodermal tumors of the spinal cord: report of two cases and review of the literature

  • Clinical–Patient Studies
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Abstract

Primary intraspinal peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare tumors with only seven reported cases in the literature. The histopathologic diagnosis of this tumor is complex and has led to a variety of treatment approaches. The distinction between central and peripheral type primary spinal cord PNETs has not always been made in the literature, leading to a paucity of data in this disease. We present here two young patients with primary intraspinal pPNET, their treatment and outcome. The first patient, a 27 year old male, presented with an intradural mass extending from L2 through L5, after multiple relapses, he is currently alive with disease after 72 months, the longest survival yet reported. The second patient, a 16 year old female, presented with an intradural mass at the cauda equina from L2 through L5, and is currently alive with responsive disease at 5 months after initial diagnosis. Here, we discuss the clinical course, the pathology and treatment for this disease and review the literature.

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References

  1. Kwon OK, Wang KC, Kim CJ, Kim IO, Chi JG, Cho BK (1996) Primary intramedullary spinal cord primitive neuroectodermal tumor with intracranial seeding in an infant. Childs Nerv Syst 12:633–636

    Article  PubMed  CAS  Google Scholar 

  2. Akyuz M, Demiral AN, Gurer IE, Ucar T, Tuncer R (2004) Primary primitive neuro-ectodermal tumor of cauda equina with intracranial seeding. Acta Neurochir (Wien) 146:525–528

    Article  CAS  Google Scholar 

  3. Albrecht CF, Weiss E, Schulz-Schaeffer WJ, Albrecht T, Fauser S, Wickboldt J, Hess CF (2003) Primary intraspinal primitive neuroectodermal tumor: report of two cases and review of the literature. J Neurooncol 61:113–120

    Article  PubMed  Google Scholar 

  4. Albright AL, Wisoff JH, Zeltzer P, Boyett J, Rorke LB, Stanley P, Geyer JR, Milstein JM (1995) Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors. A neurosurgical perspective from the children’s cancer group. Pediatr Neurosurg 22:1–7

    PubMed  CAS  Google Scholar 

  5. Aydin MV, Sen O, Ozel S, Kayaselcuk F, Caner H, Altinors␣N (2004) Primary primitive neuroectodermal tumor within the spinal epidural space: report of a case and review of the literature. Neurol Res 26:774–777

    Article  PubMed  Google Scholar 

  6. Deme S, Ang LC, Skaf G, Rowed DW (1997) Primary intramedullary primitive neuroectodermal tumor of the spinal cord: case report and review of the literature. Neurosurgery 41:1417–1420

    Article  PubMed  CAS  Google Scholar 

  7. Dorfmuller G, Wurtz FG, Umschaden HW, Kleinert R, Ambros PF (1999) Intraspinal primitive neuroectodermal tumor: report of two cases and review of the literature. Acta Neurochir (Wien) 141:1169–1175

    Article  CAS  Google Scholar 

  8. Freyer DR, Hutchinson RJ, McKeever PE (1989) Primary primitive neuroectodermal tumor of the spinal cord associated with neural tube defect. Pediatr Neurosci 15:181–187

    Article  PubMed  CAS  Google Scholar 

  9. Harimaya K, Oda Y, Matsuda S, Tanaka K, Chuman H, Iwamoto Y (2003) Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. Spine 28:E408–412

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  11. Jaksche H, Wockel W, Wernert N (1988) Primary spinal medulloblastomas? Neurosurg Rev 11:259–265

    Article  PubMed  CAS  Google Scholar 

  12. Kepes JJ, Belton K, Roessmann U, Ketcherside WJ (1985) Primitive neuroectodermal tumors of the cauda equina in␣adults with no detectable primary intracranial neoplasm—three case studies. Clin Neuropathol 4:1–11

    PubMed  CAS  Google Scholar 

  13. Liu HM, Yang WC, Garcia RL, Noh JM, Malhotra V, Leeds␣NE (1987) Intraspinal primitive neuroectodermal tumor arising from the sacral spinal nerve root. J Comput Tomogr 11:350–354

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  15. McDermott VG, el-Jabbour JN, Sellar RJ, Bell J (1994) Primitive neuroectodermal tumor of the cauda equina. Neuroradiology 36:228–230

    Article  PubMed  CAS  Google Scholar 

  16. Sevick RJ, Johns RD, Curry BJ (1987) Primary spinal primitive neuroectodermal tumor with extraneural metastases. AJNR Am J Neuroradiol 8:1151–1152

    PubMed  CAS  Google Scholar 

  17. Smith DR, Hardman JM, Earle KM (1969) Metastasizing neuroectodermal tumors of the central nervous system. J␣Neurosurg 31:50–58

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  19. Yavuz AA, Yaris N, Yavuz MN, Sari A, Reis AK, Aydin F (2002) Primary intraspinal primitive neuroectodermal tumor: case report of a tumor arising from the sacral spinal nerve root and review of the literature. Am J Clin Oncol 25:135–139

    Article  PubMed  Google Scholar 

  20. Weber DC, Rutz HP, Lomax AJ, Schneider U, Lombriser N, Zenhausern R, Goitein G (2004) First spinal axis segment irradiation with spot-scanning proton beam delivered in the treatment of a lumbar primitive neuroectodermal tumor. Case report and review of the literature. Clin Oncol (R Coll Radiol) 16:326–331

    CAS  Google Scholar 

  21. Kim YW, Jin BH, Kim TS, Cho YE (2004) Primary intraspinal primitive neuroectodermal tumor at conus medullaris. Yonsei Med J 45:533–538

    PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  23. Hadfield MG, Quezado MM, Williams RL, Luo VY (2000) Ewing’s family of tumors involving structures related to the central nervous system: a review. Pediatr Dev Pathol 3:203–210

    Article  PubMed  CAS  Google Scholar 

  24. Biegel JA, Rorke LB, Packer RJ, Sutton LN, Schut L, Bonner K, Emanuel BS (1989) Isochromosome 17q in primitive neuroectodermal tumors of the central nervous system. Genes Chromosomes Cancer 1:139–147

    Article  PubMed  CAS  Google Scholar 

  25. Tsokos M (1992) Peripheral primitive neuroectodermal tumors. Diagnosis, classification, and prognosis. Perspect Pediatr Pathol 16:27–98

    PubMed  CAS  Google Scholar 

  26. Siebenrock KA, Nascimento AG, Rock MG (1996) Comparison of soft tissue Ewing’s sarcoma and peripheral neuroectodermal tumor. Clin Orthop Relat Res 329:288–299

    Article  PubMed  Google Scholar 

  27. Ladanyi M, Lewis R, Garin-Chesa P, Rettig WJ, Huvos AG, Healey JH, Jhanwar SC (1993) EWS rearrangement in Ewing’s sarcoma and peripheral neuroectodermal tumor. Molecular detection and correlation with cytogenetic analysis and MIC2 expression. Diagn Mol Pathol 2:141–146

    PubMed  CAS  Google Scholar 

  28. Packer RJ, Goldwein J, Nicholson HS, Vezina LG, Allen JC, Ris MD, Muraszko K, Rorke LB, Wara WM, Cohen BH, Boyett JM (1999) Treatment of children with medulloblastomas with reduced-dose craniospinal radiation therapy and adjuvant chemotherapy: a children’s cancer group study. J␣Clin Oncol 17:2127–2136

    PubMed  CAS  Google Scholar 

  29. Krasin MJ, Rodriguez-Galindo C, Billups CA, Davidoff AM, Neel MD, Merchant TE, Kun LE (2004) Definitive irradiation in multidisciplinary management of localized Ewing sarcoma family of tumors in pediatric patients: outcome and prognostic factors. Int J Radiat Oncol Biol Phys 60:830–838

    Article  PubMed  Google Scholar 

  30. Barker LM, Pendergrass TW, Sanders JE, Hawkins DS (2005) Survival after recurrence of Ewing’s sarcoma family of tumors. J Clin Oncol 23:4354–4362

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric Hematology/Oncology Childrens Hospital Los Angeles, University of Southern California, Keck School of Medicine, 4650 Sunset #54, Los Angeles, CA, 90027, USA

    R. Perry, J. Finlay & S. Zacharoulis

  2. Department of Pathology, Childrens Hospital Los Angeles, University of Southern California, Keck School of Medicine, Los Angeles, CA, USA

    I. Gonzales

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  1. R. Perry
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  2. I. Gonzales
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  3. J. Finlay
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  4. S. Zacharoulis
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Correspondence to S. Zacharoulis.

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Perry, R., Gonzales, I., Finlay, J. et al. Primary peripheral primitive neuroectodermal tumors of the spinal cord: report of two cases and review of the literature. J Neurooncol 81, 259–264 (2007). https://doi.org/10.1007/s11060-006-9178-1

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  • DOI: https://doi.org/10.1007/s11060-006-9178-1

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