The great neurosurgical masquerader: 3 cases of desmoplastic infantile ganglioglioma

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Desmoplastic infantile ganglioglioma (DIG) is a rare, distinctive, supratentorial neoplasm with a generally favorable prognosis. Clinical, radiographic, and pathologic features can sometimes mimic those of a malignant tumor and other serious intracranial disorders. The author describes his experience with 3 cases of DIG, each of which initially masqueraded as another neurological disease with a very different prognosis. Case 1 was an infant boy referred for evaluation of a hemorrhagic infarction at birth. Case 2 was an infant girl referred for evaluation of a holohemispheric malignant neoplasm. Case 3 was an infant girl referred for evaluation of an intracranial mass believed to be a subdural empyema or possible sarcoma. In each case the lesion was resected and found to be a WHO grade I DIG. Each child has had a benign postoperative course. DIG can be mistaken for other serious neurological conditions including malignant neoplasm, cerebral infarction, and infection. It is prudent to consider this rare, low-grade resectable tumor in the differential diagnosis of atypical intracranial masses of childhood, as the impact on prognosis can be profound. The author discusses management strategies for DIG, including a role for molecular sequencing.

ABBREVIATIONS CBC = complete blood count; DIA = desmoplastic infantile astrocytoma; DIG = desmoplastic infantile ganglioglioma; GFAP = glial fibrillary acidic protein; NAA = N-acetylaspartate.

Article Information

Correspondence Alan R. Cohen: The Johns Hopkins University School of Medicine, Baltimore, MD. alan.cohen@jhmi.edu.

INCLUDE WHEN CITING Published online July 5, 2019; DOI: 10.3171/2019.5.PEDS19151.

Disclosures The author reports no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. Upper Row: Left posterior temporal hemorrhagic lesion interpreted at the referring hospital as a venous infarction at initial presentation. Noncontrast head CT on day 3 of life (A) and T2-weighted axial MRI on day 5 of life (B). Middle Row: Preoperative contrast-enhanced axial (C) and sagittal (D) T1-weighted MR images at age 8 months showing a large, heterogeneously enhancing, left posterior temporal mass. Lower Row: Postoperative contrast-enhanced axial (E) and sagittal (F) T1-weighted axial MR images 1 day after surgery, confirming gross-total tumor resection.

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    Case 1. A: Operative exposure of a large, firm, left temporal tumor that was adherent to the meninges. Anterior is to the right. B: Resected tumor. Figure is available in color online only.

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    Case 1. Photomicrograph showing DIG, WHO grade I, with ganglion cells in a background of spindle-shaped astrocytes. H & E, original magnification ×200. Figure is available in color online only.

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    Case 2. A: Fetal FIESTA sagittal MRI performed at 32 weeks gestation demonstrating a large, heterogeneous, left hemisphere mass. B–D: Preoperative postcontrast fast spoiled gradient echo axial, sagittal, and coronal MR images at birth, demonstrating a large, enhancing, solid and cystic left hemisphere tumor.

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    Case 2. A: Operative exposure of a large, left parietal, solid and cystic tumor adherent to the meninges. Anterior is to the right. B–E: Axial T2-weighted (B and C), T1-weighted contrast-enhanced (D), and sagittal T1-weighted contrast-enhanced (E) MR images obtained 1 day after surgery, confirming tumor resection. Figure is available in color online only.

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    Case 3. Pre- and postoperative MR images. A–C: Preoperative axial T2-weighted images showing marked right hemispheric edema. D–F: Preoperative contrast-enhanced axial, sagittal, and coronal images showing a solid, enhancing, right parietal parasagittal tumor with edema. G–I: Postoperative contrast-enhanced axial, sagittal, and coronal images obtained 4 months after surgery showing no residual enhancing tumor.

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    Case 3. Histopathological images showing a WHO grade I DIG. A: Image showing moderate cellularity with elongated astrocytes in a conspicuous storiform pattern. H & E, original magnification ×100. B: Higher magnification view. H & E, original magnification ×200. C: GFAP immunostaining showing abundance of elongated astrocytes. Original magnification ×200. D: Synaptophysin immunostaining labeling rare dysplastic ganglion cells. Original magnification ×200. Figure is available in color online only.

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