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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container_end_page 2849
container_issue 11
container_start_page 2845
container_title Child's nervous system
container_volume 36
creator Mrowczynski, Oliver D.
Lane, Jessica R.
Specht, Charles S.
Greiner, Robert J.
Iantosca, Mark R.
Rizk, Elias B.
description 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.
doi_str_mv 10.1007/s00381-020-04597-4
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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. 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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. 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Medicine
Medicine & Public Health
Neurosciences
Neurosurgery
title Suprasellar central nervous system ganglioneuroblastoma: a case in a 9-year-old child and review of the literature
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