Suprasellar central nervous system ganglioneuroblastoma: a case in a 9-year-old child and review of the literature

Purpose Intracranial ganglioneuroblastomas are incredibly rare neuroectodermal tumors with only 8 described cases total, 5 of those having imaging findings Methods Here we present a 9-year-old female patient with 4 months progressive headaches, personality changes, and vomiting. We also present a re...

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Veröffentlicht in:Child's nervous system 2020-11, Vol.36 (11), p.2845-2849
Hauptverfasser: Mrowczynski, Oliver D., Lane, Jessica R., Specht, Charles S., Greiner, Robert J., Iantosca, Mark R., Rizk, Elias B.
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Sprache:eng
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Zusammenfassung:Purpose Intracranial ganglioneuroblastomas are incredibly rare neuroectodermal tumors with only 8 described cases total, 5 of those having imaging findings Methods Here we present a 9-year-old female patient with 4 months progressive headaches, personality changes, and vomiting. We also present a review of the current literature of intracranial ganglioneuroblastomas. Results Imaging demonstrated a partially calcified suprasellar mass measuring 4.6 × 6.3 × 5 cm composed of both solid and cystic components, diagnosed to be a ganglioneuroblastoma, with mass effect on the lateral and 3rd ventricles, with a midline shift of right to left of 6-7 mm. She was treated with subtotal surgical resection, an intensive chemotherapeutic regimen, and radiation and has no residual disease on imaging 1 year and 4 months status post-surgery. Conclusion To our knowledge, this is the first case of a ganglioneuroblastoma to mimic a craniopharyngioma based upon imaging findings and suprasellar location. As these cases are extremely rare, an optimal therapeutic regimen has not been defined. However, a combination of surgical resection, chemotherapy, and radiation therapy can be effective, as shown here with successful treatment and no evidence of residual disease.
ISSN:0256-7040
1433-0350
DOI:10.1007/s00381-020-04597-4