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Dysembryoplastic neuroepithelial tumors and cognitive outcome

Cure at a price?

  1. Ibrahim Qaddoumi MD, MS1,†,*,
  2. David W. Ellison MD, PhD2,
  3. E. Brannon Morris MD1,3,
  4. Alberto Broniscer MD1,
  5. Frederick Boop MD4,
  6. Thomas Merchant DO, PhD5,
  7. Shawna L. Palmer PhD6,
  8. Amar Gajjar MD1

Article first published online: 29 JUL 2010

DOI: 10.1002/cncr.25528

Issue

Cancer

Cancer

Volume 116, Issue 23, pages 5461–5469, 1 December 2010

Additional Information

How to Cite

Qaddoumi, I., Ellison, D. W., Morris, E. B., Broniscer, A., Boop, F., Merchant, T., Palmer, S. L. and Gajjar, A. (2010), Dysembryoplastic neuroepithelial tumors and cognitive outcome. Cancer, 116: 5461–5469. doi: 10.1002/cncr.25528

Author Information

  1. 1

    Division of Neuro-Oncology, Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee

  2. 2

    Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee

  3. 3

    Division of Neurology, Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee

  4. 4

    Division of Neurosurgery, Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee

  5. 5

    Division of Radiation Oncology, Department of Radiological Sciences, Memphis, Tennessee

  6. 6

    Department of Behavioral Medicine, St. Jude Children's Research Hospital, Memphis, Tennessee

  1. †

    Fax: (901) 521-9005

*Division of Neuro-Oncology, Department of Oncology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105

Publication History

  1. Issue published online: 23 NOV 2010
  2. Article first published online: 29 JUL 2010
  3. Manuscript Accepted: 11 JUN 2010
  4. Manuscript Revised: 20 MAY 2010
  5. Manuscript Received: 9 MAR 2010

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Keywords:

  • dysembryoplastic neuroepithelial tumors (DNETs);
  • pediatric;
  • recurrence;
  • neuropsychologic outcome

Abstract

BACKGROUND:

Dysembryoplastic neuroepithelial tumors (DNETs) are benign glioneuronal tumors that occur in children. These tumors are characterized by seizures, lack of neurologic deficits, and a seemingly benign course after resection.

METHODS:

A retrospective review was conducted of data relating to 11 children diagnosed with DNETs between January 1988 and December 2007 at St. Jude Children's Research Hospital. This report documented the clinical features, neurocognitive function, and treatment outcomes in this institutional series.

RESULTS:

The patient cohort included 8 boys and 3 girls (median age at diagnosis, 10 years); all patients presented with seizures: 4 complex partial, 3 generalized tonic-clonic, 2 absence, 1 partial simple, and 1 not classified. Of the 11 patients, 1 died of cardiac fibrosis, and tumors recurred or progressed in 4 (36%) patients. Seizure control was achieved in all patients but 1. Of the 9 patients who completed neuropsychologic testing, only 3 (33%) functioned at or above the expected level of same-age peers.

CONCLUSIONS:

The high recurrence and progression rates of DNETs and the high rate of abnormal neurocognitive test results noted in the current study highlight the need for regular follow-up and appropriate academic counseling of children with these tumors. Cancer 2010. © 2010 American Cancer Society

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