Abstract
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.
Original language | English (US) |
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Pages (from-to) | 258-266 |
Number of pages | 9 |
Journal | Journal of Neurosurgery: Pediatrics |
Volume | 24 |
Issue number | 3 |
DOIs | |
State | Published - 2019 |
Keywords
- BRAF
- Brain tumor
- Desmoplastic infantile ganglioglioma
- Diagnosis
- MRI
- Next generation sequencing
- Oncology
ASJC Scopus subject areas
- Surgery
- Pediatrics, Perinatology, and Child Health
- Clinical Neurology