Desmoplastic infantile ganglioglioma with a malignant course (original) (raw)

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Case report

Eelco W. Hoving

Eelco W. Hoving Departments of Neurosurgery,

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M.D., Ph.D.

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Johan M. Kros

Johan M. Kros Department of Pathology, University Medical Center Rotterdam, The Netherlands

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Ellis Groninger

Ellis Groninger Pediatrics, Division of Pediatric Oncology, and

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Wilfred F. A. den Dunnen

Wilfred F. A. den Dunnen Pathology, University Medical Center Groningen; and

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M.D., Ph.D.

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• ABSTRACT

✓Desmoplastic infantile gangliogliomas (DIGs) are rare supratentorial tumors that arise in infancy. Despite the large size of these lesions, the prognosis is generally considered favorable after gross-total resection (GTR); however, in incidental cases tumor progression has been described. The authors report on a child harboring a DIG with a high-grade primitive cell population and glial differentiation. The progressive clinical course was determined by this cell component in spite of GTR and adjuvant chemotherapy. The significance of the presence of a high-grade primitive tumor component in the context of DIG is discussed.

Abbreviations used in this paper:

DIG = desmoplastic infantile ganglioglioma ; GFAP = glial fibrillary acidic protein ; GTR = gross-total resection ; LI = labeling index ; MR = magnetic resonance .