Journal of Neuro-Oncology

, Volume 86, Issue 2, pp 217–224 | Cite as

A supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature

  • Shigeo OhbaEmail author
  • Kazunari Yoshida
  • Yuichi Hirose
  • Eiji Ikeda
  • Takeshi Kawase
Clinical-patient studies


Supratentorial primitive neuroectodermal tumors (sPNET) occurring in adults are rare. Only 56 such cases have been previously reported. This report documents a 56-year-old male who presented with the chief complaint of right facial palsy. Magnetic resonance imaging (MRI) revealed left frontal and bilateral periventricular lesions. Surgery was performed for the frontal mass, which was histologically diagnosed to be sPNET. An immunohistochemistry assay for CD99, and a fluorescence in situ hybridization (FISH) assay for t(11;22) translocation revealed this PNET to be a central PNET. This case was the first case to detect a central PNET using both immunohistochemistry and the FISH assay in adult sPNET. Though radiation therapy was performed, an MRI performed 2.5 months after the surgery revealed a regrowth of the tumor. The patient died 5 months after surgery. This case report is accompanied by a review of 57 cases of adult sPNET.


Adult CD99 Central PNET FISH Supratentorial primitive neuroectodermal tumor t(11;22) 


  1. 1.
    Hart MN, Earle KM (1973) Primitive neuroectodermal tumors of the brain. Cancer 32:890–897PubMedCrossRefGoogle Scholar
  2. 2.
    Rorke LB, Hart MN, Mclendon RE (2000) Supratentorial primitive neurorctodermal tumor (PNET). In: Kleihues P, Cavenee WK (eds) Pathology & Genetics: tumors of the nervous system. LARC Press, Lyon, France, pp 141–144Google Scholar
  3. 3.
    Gaffney CC, Sloane JP, Bradley NJ, Bloom HJG (1985) Primitive neuroectodermal tumors of the cerebrum: pathology and treatment. J Neurooncol 3:23–33PubMedCrossRefGoogle Scholar
  4. 4.
    Balafouta MJ, Kouvaris JR, Miliadou AC, Papacharalampous XN, Kolokouris DV, Kouloulias VE, Mariolis AD, Vlahos LJ (2003) Primitive neuroectodermal tumor in a 60-year-old man: a case report and literature review. Br J Radiol 76:62–65PubMedCrossRefGoogle Scholar
  5. 5.
    Bellis EH, Salcman M, Bastian FO (1983) Primitive neuroectodermal tumor in a 57-years-old man. Surg Neurol 20:30–35PubMedCrossRefGoogle Scholar
  6. 6.
    Gnanalingham KK, Gillespie J, Richardson PL (2001) Magnetic resonance study of multiple primitive neuroectodermal tumors of the cerebrum, cerebellum and spinal cord in an adult. Clin Radiol 56:588–590PubMedCrossRefGoogle Scholar
  7. 7.
    Grant JW, Steart PV, Gallagher PJ (1988) Primitive neuroectodermal tumors of the cerebrum: a histological and immunohistochemical study of 10 cases. Clinical Neuropathology 7:228–233PubMedGoogle Scholar
  8. 8.
    Gyure KA, Prayson RA, Estes ML (1999) Extracerebellar primitive neuroectodermal tumors: a clinicopathologic study with bcl-2 and CD99 immunohistochemistry. Ann Diagn Pathol 3:276–280PubMedCrossRefGoogle Scholar
  9. 9.
    Kim DG, Lee DY, Paek SH, Chi JG, Choe G, Jung HW (2002) Supratentorial primitive neuroectodermal tumors in adults. J Neurooncol 60:43–52PubMedCrossRefGoogle Scholar
  10. 10.
    Kouyialis AT, Boviatsis EI, Karampelas IK, Korfias S, Korkolopoulou P, Sakas DE (2005) Primitive supratentorial neuroectodermal tumor in an adult. J Clin Neurosci 12:492–495PubMedCrossRefGoogle Scholar
  11. 11.
    Krampulz T, Hans VH, Oppel F, Dietrich U, Puchner MJ (2006) Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult: a case report. J Neurooncol 77:291–294PubMedCrossRefGoogle Scholar
  12. 12.
    Kuratsu J, Matsukado Y, Seto H, Nonaka N, Sueyoshi N (1986) Primitive neuroectodermal tumor arising from the thalamus of an adult-case report. Neurol Med Chir (Tokyo) 26:30–34CrossRefGoogle Scholar
  13. 13.
    Masuda K, Yutani C, Akutagawa K, Yamamoto S, Haysuyama H, Ishibashi-Ueda H, Imakita M, Manaka H, Takahashi J, Nagata I (2000) Cerebral primitive neuroectodermal tumor in an adult male. Acta Cytol 44:1050–1058PubMedGoogle Scholar
  14. 14.
    Miyazawa T, Ueno H, Hatashita S, Yagishita S (1994) “Undifferentiated” cerebral primitive neuroectodermal tumor in a Young adult: case report. Neurol Med Chir (Tokyo) 34:759–762Google Scholar
  15. 15.
    Pickuth D, Leutloff U (1996) Computed tomography and magnetic resonance imaging findings in primitive neuroectodermal tumors in adults. Br J Radiol 69:1–5PubMedCrossRefGoogle Scholar
  16. 16.
    Pigott TJD, Punt JAG, Lowe JS, Henderson MJ, Beck A, Gray T (1990) The clinical, radiological and histological features of cerebral primitive neuroectodermal tumours. Br J Neurosurg 4:287–298PubMedCrossRefGoogle Scholar
  17. 17.
    Prados MD, Edward MS, Chang SM, Russo C, Davis R, Rabbitt J, Page M, Lamborn K, Wara WM (1999) Hyperfractionated craniospinal radiation therapy for primitive neuroectodermal tumors: results of a phase II study. Int J Radiat Oncol Biol Phys 43:279–285PubMedCrossRefGoogle Scholar
  18. 18.
    Sanno N, Shimura T, Takumi I, Node Y, Aihara K, Osamura RY, Teramoto A (1998) An adult case of cerebral primitive neuroectodermal tumor. Acta Neurochir (Wien) 140:445–446CrossRefGoogle Scholar
  19. 19.
    Selassie L, Rigotti R, Kepes JJ, Towfighi J (1994) Adipose tissue and smooth muscle in a primitive neuroectodermal tumor of cerebrum. Acta Neuropathol 87:217–222PubMedCrossRefGoogle Scholar
  20. 20.
    Shingu T, Kagawa T, Kimura Y, Takada D, Moritake K, Hoshii Y (2005) Supratentorial primitive neuroectodermal tumor in an aged patient: case report. Neurol Med Chir (Tokyo) 45:530–535CrossRefGoogle Scholar
  21. 21.
    Visee S, Soltner C, Rialland X, Machet MC, Loussouarn D, Milinkevitch S, Pasco-Papon A, Mercier P, Rousselet MC (2005) Supratentorial primitive neuroectodermal tumors of the brain: multidirectional differentiation does not influence prognosis. A clinicopathological report of 18 patients. Histopathology 46:403–412PubMedCrossRefGoogle Scholar
  22. 22.
    Klisch J, Husstedt H, Hennings S, Velthoven VV, Pagenstecher A, Schumacher M (2000) Supratentorial primitive neuroectodermal tumors: diffusion-weighted MRI. Neuroradiology 42:393–398PubMedCrossRefGoogle Scholar
  23. 23.
    Majos C, Alonso J, Aguilera C, Serrallonga M, Acebes JJ, Arus C, Gili J (2002) Adult primitive neuroectodermal tumor: proton MR spectroscopic findings with possible application for differential diagnosis. Radiology 225:556–566PubMedCrossRefGoogle Scholar
  24. 24.
    Rorke LB (1983) The cerevellar medulloblastoma and its relationship to primitibe neuroectodermal tumors. J Neuropathol Exp Neurol 42:1–15PubMedGoogle Scholar
  25. 25.
    Katayama Y, Kimura S, Watanabe T, Yoshino A, Koshinaga M (1999) Peripheral-type primitive neuroectodermal tumor arising in the tentorium. J Neurosurg 90:141–144PubMedCrossRefGoogle Scholar
  26. 26.
    Stevenson AJ, Chatten J, Bertoni F, Miettinen M (1994) CD99 (p30/32MIC2) neuroectodermal/Ewing’s sarcoma antigen as an immunohistochemical marker: review of more than 600 tumors and the literature experience. Appl Immunohistochem 2:231–240Google Scholar
  27. 27.
    Mhawech-Fayceglia P, Hermann F, Penetrante R, Beck A, Sait S, Block AM, Odunsi K, Fisher J, Balos L, Cheney RT (2006) Diagnostic utility of FLI-1 monoclonal antibody and dual-colour, break-apart probe fluorescence in situ (FISH) analysis in Ewing’s sarcoma/primitive neuroectodermal tumor (EWS/PNET). A comparative study with CD99 and FLI-1 polyclonal antibodies. Histopathology 49:569–575CrossRefGoogle Scholar
  28. 28.
    Bridge RS, Rajaram V, Dehner LP, Pfeifer JD, Perry A (2006) Molecular diagnosis of Ewing sarcoma/primitive neuroectodermal tumor in routinely progressed tissue: a comparison of two FISH strategies and RT-PCR in malignant round cell tumors. Mod Pathol 19:1–8PubMedCrossRefGoogle Scholar
  29. 29.
    Cohen BH, Zeltzer PM, Boyett JM, Geyer JR, Allen JC, Finlay JL, McGuire-Cullen P, Milstein JM, Rorke LB, Stanley P (1995) Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a children’s cancer group randomized traial. J Clin Oncol 13:1687–1696PubMedGoogle Scholar
  30. 30.
    Dirks PB, Harris L, Hoffman HJ, Kumphreys RP, Drake JM, Rutka JT (1996) Supratentorial primitive neuroectodermal tumors in children. J Neuro-Oncol 29:75–84CrossRefGoogle Scholar
  31. 31.
    Yang HJ, Nam DH, Wang KC, Kim YM, Chi JG, Cho BK (1999) Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors. Childs Nerv Syst 15:377–383PubMedCrossRefGoogle Scholar
  32. 32.
    Janss AJ, Yachnis AT, Silber JH, Trojanowski JQ, Lee VM, Sutton LN, Perilongo G, Rorke LB, Phillips PC (1996) Glial differentiation predicts poor clinical outcome in primitive neuroectodermal brain tumor. Ann Neurol 39:481–489PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2007

Authors and Affiliations

  • Shigeo Ohba
    • 1
    Email author
  • Kazunari Yoshida
    • 1
  • Yuichi Hirose
    • 2
  • Eiji Ikeda
    • 3
  • Takeshi Kawase
    • 1
  1. 1.Department of NeurosurgeryKeio University School of MedicineShinjuku-kuJapan
  2. 2.Department of NeurosurgeryFujita Health UniversityToyoakeJapan
  3. 3.Department of PathologyKeio University School of MedicineShinjuku-kuJapan

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