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

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Abstract

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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Correspondence to Oliver D. Mrowczynski.

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Mrowczynski, O.D., Lane, J.R., Specht, C.S. et al. Suprasellar central nervous system ganglioneuroblastoma: a case in a 9-year-old child and review of the literature. Childs Nerv Syst 36, 2845–2849 (2020). https://doi.org/10.1007/s00381-020-04597-4

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  • DOI: https://doi.org/10.1007/s00381-020-04597-4

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