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Dysembryoplastic Neuroepithelial Tumors: Nonspecific Histological Forms – A Study of 40 Cases

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Abstract

Objective. To demonstrate that DNTs include a large morphological spectrum of tumors that cannot be histologically distinguished from conventional categories of gliomas.

Methods. All tumors from patients who underwent epilepsy surgery in Sainte-Anne hospital (Paris) that histologically resembled gliomas and did not conform to current histological criteria for DNTs or gangliogliomas were entered in the study.

Results. According to the WHO histological classification, the 40 tumors resembled: pilocytic astrocytomas (4 cases), astrocytomas (16 cases), anaplastic astrocytoma (1 case), oligodendrogliomas (10 cases), oligo-astrocytomas (8 cases) or anaplastic oligo-astrocytomas (1 case). However foci of cortical dysplasia could be observed in 47% of the cases. Clinical presentation and imaging features were strikingly similar to that observed in typical DNTs. Although surgical removal was incomplete in 28% of the cases and none of the patients underwent chemo or radiotherapy, none of the tumors recurred (mean follow-up: 7 years). Moreover, serial preoperative imaging in 26 patients (mean follow-up: 4.5 years) demonstrated that these lesions were perfectly stable.

Conclusions. Whatever the histological appearance of a glial tumor, the diagnosis of DNT must be considered when all the following criteria are associated: (1) partial seizures, with or without secondary generalization, beginning before the age 20 years, (2) no neurological deficit or stable congenital deficit, (3) cortical topography of the lesion as better demonstrated by MRI and (4) no mass effect on imaging.

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References

  1. Daumas-Duport C: Dysembryoplastic neuroepithelial tumours. Brain Pathol 3: 283–295, 1993

    PubMed  Google Scholar 

  2. Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, Laws ER, Vedrenne C: Dysembryoplasic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizure. Report of thirty-nine cases. Neurosurgery 23: 545–556, 1988

    PubMed  Google Scholar 

  3. Daumas-Duport C: Patterns of tumor growth and problems associated with histological typing of low-grade gliomas. In: Apuzzo LJ (ed): Benign Cerebral Gliomas. Park Ridge, AANS, 1995, vol. 1, pp 125–147

    Google Scholar 

  4. Kleihues P, Burger PC, Scheithauer BW: Histological Typing of Tumours of the Central Nervous System. 2nd ed., Springer-Verlag: Berlin, Heidelberg, New York 1993, p 23

    Google Scholar 

  5. Kleihues P, Burger PC, Scheithauer BW: The newWHOclassification of brain tumours. Brain Pathol 3: 255–268, 1993

    PubMed  Google Scholar 

  6. Talairach J, Bancaud J: Stereotaxic exploration and therapy in epilepsy. In: Viken PJ, Bruyn GW (eds): The Epilepsies. Handbook of Clinical Neurology. North Holland, Amsterdam, 1974, pp 758–782

    Google Scholar 

  7. Engel J, Van Ness PC, Rasmussen TB, Ojemann LM: Outcome with respect to epileptic seizures. In: Engel Jr, J (ed). Surgical Treatment of the Epilepsies, Raven Press, NewYork, 1993, pp 609–621

    Google Scholar 

  8. Daumas-Duport C: Dysembryoplastic neuroepithelial tumours in epilepsy surgery, In: Guerrini R (ed): Dysplasia of Cerebral Cortex and Epilepsy, 2nd ed., Raven Press, New York, 1995, pp 125–147

    Google Scholar 

  9. Lemesle M, Borsotti JP, Justrabo E, Giroud M, Dumas R: Tumeurs dysembryoplasiques neuroépithéliales. Rev Neurol 152: 451–457, 1996

    PubMed  Google Scholar 

  10. Prayson RA, Estes ML: Dysembryoplastic neuroepithelial tumor. Am J Clin Pathol 97: 398–401, 1992

    PubMed  Google Scholar 

  11. Raymond AA, Halpin SFS, Alsanjari N, Cook MJ, Kitchen ND, Fisch DR, Stevens JM, Harding BN, Scaravilli F, Kendall B, Shorvon SD, Neville BGR: Dysembryoplastic neuroepithelial tumour. Features in 16 patients. Brain 117: 461–475, 1994

    PubMed  Google Scholar 

  12. Vali AM, Clarke MA, Kelsey A: Dysembryoplastic neuroepithelial tumor as a potentially treatable cause of intractable epilepsy in children. Clin Radiol 47: 255–258, 1993

    PubMed  Google Scholar 

  13. Wolf HK, Wellmer J, Muller MB, Wiestler OD, Hufnagel A, Piestch T: Glioneuronal malformative lesions and dysembryoplastic neuroepithelial tumors in patients with chronic pharmacoresistantepilepsies. J Neuropathol Exp Neurol 54: 245–254, 1995

    PubMed  Google Scholar 

  14. Koeller KK, Dillon WP: Dysembryoplastic neuroepithelial tumors: MR appearance. AJNR 13: 1319–1325, 1992

    PubMed  Google Scholar 

  15. Kuroiwa T, Bergey GK, Rothman ML, Zoarski GH, Wolf A, Zagardo MT, Kristt DA, Hudson LP, Krumholz A, Barry E, Numaguchi Y: Radiologic appearance of the dysembryoplastic neuroepithelial tumor. Radiology 197: 233–238, 1995

    PubMed  Google Scholar 

  16. Leung SY, Gwi E, Ng HK, Fung CF, Yam KY: Dysembryoplastic neuroepithelial tumor. A tumor with small neuronal cells resembling oligodendroglioma. Am J Surg Pathol 18: 604–614, 1994

    PubMed  Google Scholar 

  17. Ostertun B, Wolf HK, Campos MG, Matus C, Solymosi L, Elger CE, Schramm J, Schild HH: Dysembryoplastic neuroepithelial tumors: MR and CT evaluation. AJNR 17: 419–430, 1996

    Google Scholar 

  18. Pasquier B, Bost F, Peoc'h M, Barnad R, Pasquier D: Données neuropathologiques dans l'épilepsie partielle pharmacor ésistante. Ann Pathol 16: 174–181, 1996

    PubMed  Google Scholar 

  19. Taratuto AL, Pomata H, Sevlever G, Gallo G, Monges J: Dysembryoplastic neuroepithelial tumor: morphological, immunocytochemical and deoxyribonucleic acid analyses in a pediatric series. Neurosurgery 36: 474–481, 1995

    PubMed  Google Scholar 

  20. Wolf HK, Wiestler OD: Surgical pathology of chronic epileptic seizure disorders. Brain Pathol 3: 371–380, 1993

    PubMed  Google Scholar 

  21. Kuroïwa T, Kishikawa T, Kato A, Ueno M, Kudo S, Tabuchi K: Dysembryoplastic neuroepithelial tumors: MR findings. J Comput Assist Tomogr 18: 352–356, 1994

    PubMed  Google Scholar 

  22. Hirose T, Scheithauer BW, Lopes MBS, Vanden Berg SR: Dysembryoplastic neuroepithelial tumor (DNT): an immunohistochemical and ultrastructural study. J Neuropathol Exp Neurol 53: 184–195, 1994

    PubMed  Google Scholar 

  23. Prayson RA, Estes ML, Morris HH: Coexistence of neoplasia and cortical dysplasia in patients presenting with seizures. Epilepsia 34: 609–615, 1993

    PubMed  Google Scholar 

  24. Wolf HK, Campos MGJ, Zentner J, Hufnagel A, Schramm J, Elger CE, Wiestler OD: Surgical pathology of temporal lobe epilepsy. Experience with 216 cases. J Neuropathol Exp Neurol 52: 499–506, 1993

    PubMed  Google Scholar 

  25. Haddad SF, Moore SA, Menezes AH, Van Gilder JC: Ganglioglioma: 13 years of experience. Neurosurgery 31: 171–178, 1992

    PubMed  Google Scholar 

  26. Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, Abbott JR, Miller DC: Central nervous system gangliogliomas. Clinical outcome. J Neurosurg 79: 867–873, 1993

    PubMed  Google Scholar 

  27. Prayson RA, Khajavi K, Comair YG: Cortical architectural abnormalities and MIB1 immunoreactivity in gangliogliomas: a study of 60 patients with intracranial tumors. J Neuropathol Exp Neurol 54: 513–520, 1995 280

    PubMed  Google Scholar 

  28. Cervera-Pierot P, Varlet P, Chodkiewicz JP, Daumas-Duport C: Dysembryoplastic neuroepithelial tumors in the caudate nucleus area. Neurosurgery 40: 1065–1070, 1997

    PubMed  Google Scholar 

  29. Kuchelmeister K, Demirel T, Schl¨orer E, Bergmann M, Gullotta F: Dysembryoplastic neuroepithelial tumour of the cerebellum. Acta Neuropathol 89: 385–390, 1995

    PubMed  Google Scholar 

  30. Kirkpatrick PJ, Honavar M, Janota I, Polkey CE: Control of temporal lobe epilepsy following en bloc resection of lowgrade tumors. J Neurosurg78: 19–25, 1993

    PubMed  Google Scholar 

  31. Fried I, Kim J, Spencer DD: Limbic and Neocortical Gliomas associated with intratable seizures: a distinct clinicopathological group. Neurosurgery 34: 815–823, 1994

    PubMed  Google Scholar 

  32. Jay V, Becker LE, Otsubo H, Hwang PA, Hoffman HJ, Harwood-Nash D: Pathology of temporal lobectomy for refractory seizures in children. Review of 20 cases including some unique malformative lesions. J Neurosurg 79: 53–61, 1993

    PubMed  Google Scholar 

  33. Morris HH, Estes ML, Gilmore R, Van-Ness PC, Barnett GH, Turnbull J: Chronic intractable epilepsy as the only symptom of primary brain tumor. Epilepsia 34: 1038–1043, 1993

    PubMed  Google Scholar 

  34. Raymond AA, Fish DR, Sisodiya SM, Alsanjari N, Stevens JM, Shorvon SD: Abnormalities of gyration, heterotopias, tuberous sclerosis, focal cortical dysplasia, microdysgenesis, dysembryoplastic neuroepithelial tumour and dysgenesis of the archicortex in epilepsy. Clinical, EEG and neuroimaging features in 100 adult patients. Brain 118: 629–660, 1995

    PubMed  Google Scholar 

  35. Cascino GD: Epilepsy and brain tumors: implications for treatment. Epilepsia 31: S37–S44, 1990

    PubMed  Google Scholar 

  36. Piepmeier JM, Christopher S, Spencer D, Byrne T, Kim J, Knisel J, Lacy J, Tsukerman L, Makuch R: Variations in the natural history and survival of patients with supratentorial low-grade astrocytomas. Neurosurgery 38: 872–879, 1996

    PubMed  Google Scholar 

  37. Gilles FH, Sobel EL, Tavare CJ, Leviton A, Hedley-Whyte ET: Age-related changes in diagnoses, histological features and survival in children with brain tumors: 1930–1979. Neurosurgery 37: 1056–1068, 1995

    PubMed  Google Scholar 

  38. Helseth A: The incidence of primary central nervous system neoplasms before and after computerized tomography availability. J Neurosurg 83: 999–1003, 1995

    PubMed  Google Scholar 

  39. Burger PC: The “ideal” classification of pediatric central nervous system neoplasms. Cancer56: 1865–1868, 1985

    PubMed  Google Scholar 

  40. Gilles FH, Sobel EL, Leviton A, Hedley-Whyte ET, Tavare CJ, Adelman LS, Sobel RA: Temporal trends among childhood brain tumour biopsies. J Neuro-Oncol13: 137–149, 1992

    Google Scholar 

  41. Rorke LB: Classification and grading of childhood brain tumours. Overview and statement of the problem. Cancer 56: 1848–1849, 1985

    PubMed  Google Scholar 

  42. Rorke LB, Gilles FH, Davis RL, Becker LE: Revision of the World Health Organization classification of brain tumors for childhood brain tumors. Cancer 56: 1869–1886, 1985

    PubMed  Google Scholar 

  43. Duffner PK, Cohen ME, Myers MH, Heise HW: Survival of children with brain tumors: SEER program, 1973–1980. Neurology 36: 597–601, 1986

    PubMed  Google Scholar 

  44. Marchese MJ, Chang CH: Malignant astrocytic gliomas in children. Cancer 65: 2771–2778, 1990

    PubMed  Google Scholar 

  45. Geyer JR, Finlay JL, Boyet JM, Wisoff J, Yates A, Mao L, Packer RJ: Survival of infants with malignant astrocytomes. A report from the childrens cancer group. Cancer 75: 1045–1050, 1995

    PubMed  Google Scholar 

  46. Westergaard L, Gjerris F, Kunken L: Prognostic parameters in benign astrocytomas. Acta Neurochir 123: 1–7, 1993

    Google Scholar 

  47. Tice H, Barnes PD, Goumnerova L, Scott RM, Tarbell NJ: Pediatric and adolescent oligodendrogliomas. AJNR 14: 1293–1300, 1993

    PubMed  Google Scholar 

  48. Hirsch JF, Sainte Rose C, Pierre-Kahn A, Pfister A, Hoppe-Hirsch E: Benign astrocytic and oligodendrocytic tumors of the cerebral hemispheres in children. J Neurosurg 70: 568–572, 1989

    PubMed  Google Scholar 

  49. Vinters HV, Amstrong DL, Babb DL, Daumas-Duport C, Robitaille Y, Bruton CJ, Farell MA: The neuropathology of human symptomatic epilepsy, In: Engel Jr(ed) Surgical treatment of the epilepsies, 2nd ed., Raven press, NewYork, 1993, pp 593–608

    Google Scholar 

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Authors and Affiliations

  1. Department of Pathology, Sainte-Anne Hospital, France

    Catherine Daumas-Duport, Pascale Varlet, Frédéric Beuvon & Pascale Cervera-Pierot

  2. Department of Pathology-Neuro-oncology, Cochin Port-Royal University, Paris, France

    Salim Bacha & Jean-Paul Chodkiewicz

  3. Department of Neurosurgery, Sainte-Anne Hospital, Paris, France

    Catherine Daumas-Duport, Pascale Varlet, Pascale Cervera-Pierot & Jean-Paul Chodkiewicz

Authors
  1. Catherine Daumas-Duport
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  2. Pascale Varlet
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  5. Pascale Cervera-Pierot
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  6. Jean-Paul Chodkiewicz
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Daumas-Duport, C., Varlet, P., Bacha, S. et al. Dysembryoplastic Neuroepithelial Tumors: Nonspecific Histological Forms – A Study of 40 Cases. J Neurooncol 41, 267–280 (1999). https://doi.org/10.1023/A:1006193018140

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