Journal of Neuro-Oncology

, 94:283 | Cite as

Clinicopathologic features of recurrent dysembryoplastic neuroepithelial tumor and rare malignant transformation: a report of 5 cases and review of the literature

  • Wilson Z. Ray
  • Spiros L. Blackburn
  • Sandro Casavilca-Zambrano
  • Carlos Barrionuevo
  • Jose E. Orrego
  • Hugo Heinicke
  • Joshua L. Dowling
  • Arie Perry
Case Report

Abstract

Dysembryoplastic neuroepithelial tumors (DNETs) have traditionally been viewed as benign “quasihamartomatous” tumors widely considered curable with surgery alone. More recently, case reports have described malignant gliomas arising after irradiation and recurrences following subtotal or even gross total resection. Herein, we describe five cases of DNET with recurrences 2–7 years after resection. Although the radiology was often alarming (e.g., new ring enhancing mass), the pathology remained benign in most cases. Nonetheless, a probably radiation induced anaplastic astrocytoma was encountered in one case 7 years after therapy. These findings suggest that these patients may need closer follow-up than initially suggested, lending further support to the notion that this tumor behaves more like a benign neoplasm, rather than a dysplastic or hamartomatous lesion.

Keywords

Dysembryoplastic neuroepithelial tumor Recurrence Malignant transformation 

Notes

Acknowledgments

The authors are grateful to Dr. Edith Schmidt from the Sunrise Hospital in Las Vegas (currently at the Children’s National Medical Center in Washington, DC) for sharing case #4 with us in consultation and providing additional clinical information.

References

  1. 1.
    Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, Laws ER Jr, Vedrenne C (1988) Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. Neurosurgery 23:545–556. doi:10.1097/00006123-198811000-00002 PubMedCrossRefGoogle Scholar
  2. 2.
    Prayson RA, Estes ML (1992) Dysembryoplastic neuroepithelial tumor. Am J Clin Pathol 97:398–401PubMedGoogle Scholar
  3. 3.
    Vali AM, Clarke MA, Kelsey A (1993) Dysembryoplastic neuroepithelial tumour as a potentially treatable cause of intractable epilepsy in children. Clin Radiol 47:255–258. doi:10.1016/S0009-9260(05)81133-X PubMedCrossRefGoogle Scholar
  4. 4.
    Vogelgesang S, Kunert-Keil C, Cascorbi I, Mosyagin I, Schroder E, Runge U, Jedlitschky G, Kroemer HK, Oertel J, Gaab MR, Pahnke J, Walker LC, Warzok RW (2004) Expression of multidrug transporters in dysembryoplastic neuroepithelial tumors causing intractable epilepsy. Clin Neuropathol 23:223–231PubMedGoogle Scholar
  5. 5.
    Wolf HK, Zentner J, Hufnagel A, Campos MG, Schramm J, Elger CE, Wiestler OD (1993) Surgical pathology of chronic epileptic seizure disorders: experience with 63 specimens from extratemporal corticectomies, lobectomies and functional hemispherectomies. Acta Neuropathol 86:466–472. doi:10.1007/BF00228581 PubMedCrossRefGoogle Scholar
  6. 6.
    Daumas-Duport C, Varlet P, Bacha S, Beuvon F, Cervera-Pierot P, Chodkiewicz JP (1999) Dysembryoplastic neuroepithelial tumors: nonspecific histological forms—a study of 40 cases. J Neurooncol 41:267–280. doi:10.1023/A:1006193018140 PubMedCrossRefGoogle Scholar
  7. 7.
    Raymond AA, Halpin SF, Alsanjari N, Cook MJ, Kitchen ND, Fish DR, Stevens JM, Harding BN, Scaravilli F, Kendall B et al (1994) Dysembryoplastic neuroepithelial tumor. Features in 16 patients. Brain 117(Pt 3):461–475. doi:10.1093/brain/117.3.461 PubMedCrossRefGoogle Scholar
  8. 8.
    Josan V, Smith P, Kornberg A, Rickert C, Maixner W (2007) Development of a pilocytic astrocytoma in a dysembryoplastic neuroepithelial tumor. Case report. J Neurosurg 106:509–512PubMedGoogle Scholar
  9. 9.
    Fernandez C, Girard N, Paz Paredes A, Bouvier-Labit C, Lena G, Figarella-Branger D (2003) The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumor in children: a study of 14 cases. AJNR Am J Neuroradiol 24:829–834PubMedGoogle Scholar
  10. 10.
    Jensen RL, Caamano E, Jensen EM, Couldwell WT (2006) Development of contrast enhancement after long-term observation of a dysembryoplastic neuroepithelial tumor. J Neurooncol 78:59–62. doi:10.1007/s11060-005-9054-4 PubMedCrossRefGoogle Scholar
  11. 11.
    Hammond RR, Duggal N, Woulfe JM, Girvin JP (2000) Malignant transformation of a dysembryoplastic neuroepithelial tumor. Case report. J Neurosurg 92:722–725PubMedCrossRefGoogle Scholar
  12. 12.
    Nolan MA, Sakuta R, Chuang N, Otsubo H, Rutka JT, Snead OC III, Hawkins CE, Weiss SK (2004) Dysembryoplastic neuroepithelial tumors in childhood: long-term outcome and prognostic features. Neurology 62:2270–2276PubMedGoogle Scholar
  13. 13.
    Hennessy MJ, Elwes RD, Honavar M, Rabe-Hesketh S, Binnie CD, Polkey CE (2001) Predictors of outcome and pathological considerations in the surgical treatment of intractable epilepsy associated with temporal lobe lesions. J Neurol Neurosurg Psychiatry 70:450–458. doi:10.1136/jnnp.70.4.450 PubMedCrossRefGoogle Scholar
  14. 14.
    Rushing EJ, Thompson LD, Mena H (2003) Malignant transformation of a dysembryoplastic neuroepithelial tumor after radiation and chemotherapy. Ann Diagn Pathol 7:240–244. doi:10.1016/S1092-9134(03)00070-4 PubMedCrossRefGoogle Scholar
  15. 15.
    Perry A, Miller CR, Gujrati M, Scheithauer BW, Zambrano SC, Jost SC, Raghavan R, Qian J, Cochran EJ, Huse JT, Holland EC, Burger PC, Rosenblum MK (2009) Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases. Brain Pathol 19:81–90. doi:10.1111/j.1750-3639.2008.00167.x PubMedCrossRefGoogle Scholar
  16. 16.
    Daumas-Duport CPTHC, Shankar SK (2007) Dysembryoplastic neuroepithelial tumour. In: Louis D, Ohgaki H, Wiestler O, Cavenee W (eds) WHO classification of tumours of the central nervous system. International Agency for Research on Cancer (IARC), Lyon, pp 99–102Google Scholar
  17. 17.
    Perry AFC, Banerjee R, Brat DJ, Scheithauer BW (2003) Ancillary FISH analysis for 1p and 19q status: preliminary observations in 287 gliomas and oligodendroglioma mimics. Front Biosci 8:a1–a9. doi:10.2741/896 PubMedCrossRefGoogle Scholar
  18. 18.
    Raghavan RBJ, Perry A, Margraf L, Vono MB, Cai DX, Wyatt RE, Rushing EJ, Bowers DC, Hynan LS (2003) White CL 3rd Pediatric oligodendrogliomas: a study of molecular alterations on 1p and 19q using fluorescence in situ hybridization. J Neuropathol Exp Neurol 62:530–537PubMedGoogle Scholar
  19. 19.
    Maher CO, White JB, Scheithauer BW, Raffel C (2008) Recurrence of dysembryoplastic neuroepithelial tumor following resection. Pediatr Neurosurg 44:333–336. doi:10.1159/000138372 PubMedCrossRefGoogle Scholar
  20. 20.
    Schwartz AM, Ghatak NR (1990) Malignant transformation of benign cerebellar astrocytoma. Cancer 65:333–336. doi:10.1002/1097-0142(19900115)65:2<333::AID-CNCR2820650225>3.0.CO;2-3 PubMedCrossRefGoogle Scholar
  21. 21.
    Krieger MD, Gonzalez-Gomez I, Levy ML, McComb JG (1997) Recurrence patterns and anaplastic change in a long-term study of pilocytic astrocytomas. Pediatr Neurosurg 27:1–11. doi:10.1159/000121218 PubMedCrossRefGoogle Scholar
  22. 22.
    Jager B, Schuhmann MU, Schober R, Kortmann RD, Meixensberger J (2008) Induction of gliosarcoma and atypical meningioma 13 years after radiotherapy of residual pilocytic astrocytoma in childhood. Pediatr Neurosurg 44:153–158. doi:10.1159/000113120 PubMedCrossRefGoogle Scholar
  23. 23.
    Kuroiwa T, Ohta T, Tsutsumi A (1999) Malignant pilocytic astrocytoma in the medulla oblongata: case report. Brain Tumor Pathol 16:81–85. doi:10.1007/BF02478907 PubMedCrossRefGoogle Scholar
  24. 24.
    van der Wal EJ, Azzarelli B, Edwards-Brown M (2003) Malignant transformation of a chiasmatic pilocytic astrocytoma in a patient with diencephalic syndrome. Pediatr Radiol 33:207–210PubMedGoogle Scholar
  25. 25.
    Stuer C, Vilz B, Majores M, Becker A, Schramm J, Simon M (2007) Frequent recurrence and progression in pilocytic astrocytoma in adults. Cancer 110:2799–2808. doi:10.1002/cncr.23148 PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC. 2009

Authors and Affiliations

  • Wilson Z. Ray
    • 1
  • Spiros L. Blackburn
    • 1
  • Sandro Casavilca-Zambrano
    • 3
  • Carlos Barrionuevo
    • 3
  • Jose E. Orrego
    • 4
  • Hugo Heinicke
    • 4
  • Joshua L. Dowling
    • 1
  • Arie Perry
    • 2
  1. 1.Department of Neurological SurgeryWashington University School of MedicineSt. LouisUSA
  2. 2.Department of Pathology and Immunology, Division of NeuropathologyWashington University School of MedicineSt. LouisUSA
  3. 3.Department of PathologyInstituto Nacional de Enfermedades Neoplasicas (INEN)LimaPeru
  4. 4.Department of NeurosurgeryInstituto Nacional de Enfermedades Neoplasicas (INEN)LimaPeru

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