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 |
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Format: | Artikel |
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. |
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ISSN: | 0256-7040 1433-0350 |
DOI: | 10.1007/s00381-020-04597-4 |