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.
Similar content being viewed by others
References
Wahl HR, Craig PE (1938) Multiple tumors of the sympathetic nervous system: report of a case showing a distinct ganglioneuroma, a neuroblastoma and a cystic calcifying ganglioneuroblastoma. Am J Pathol 14:797–808.5 Available: http://www.ncbi.nlm.nih.gov/pubmed/19970419. Accessed 17 April 2019
Gauchan E, Sharma P, Ghartimagar D, Ghosh A (2017) Ganglioneuroblastoma in a newborn with multiple metastases: a case report. J Med Case Rep 11:239 Available: http://www.ncbi.nlm.nih.gov/pubmed/28847309. Accessed 17 April 2019
Durity FA, Dolman CL, Moyes PD (1968) Ganglioneuroblastoma of the cerebellum. Case report. J Neurosurg 28:270–273 Available: https://thejns.org/view/journals/j-neurosurg/28/3/article-p270.xml. Accessed 17 April 2019
Gasparetto EL, Rosemberg S, Matushita H, Leite Cda C (2007) Ganglioneuroblastoma of the cerebellum: neuroimaging and pathological features of a case. Arq Neuropsiquiatr 65:338–340 Available: http://www.ncbi.nlm.nih.gov/pubmed/17607440. Accessed 17 April 2019
Mirza FA, Synder B, Smith VD, Vasquez RA (2018) Pediatric supratentorial ganglioneuroblastoma: case report and review of literature. World Neurosurg 113:261–266 Available: http://www.ncbi.nlm.nih.gov/pubmed/29496580. Accessed 17 April 2019
Packer RJ, Sutton LN, Rosenstock JG, Rorke LB, Bilaniuk LT, Zimmerman RA et al (1984) Pineal region tumors of childhood. Pediatrics 74:97–102 Available: http://www.ncbi.nlm.nih.gov/pubmed/6739222. Accessed 17 April 2019
Reubi JC, Lang W, Maurer R, Koper JW, Lamberts SW (1987) Distribution and biochemical characterization of somatostatin receptors in tumors of the human central nervous system. Cancer Res 47:5758–5764 Available: http://www.ncbi.nlm.nih.gov/pubmed/2889527. Accessed 17 April 2019
Sohma T, Tuchita H, Kitami K, Hotta H, Ishiguro M, Takeda T (1992) Cerebellopontine angle ganglioneuroblastoma. Neuroradiology 34:334–336 Available: http://www.ncbi.nlm.nih.gov/pubmed/1528448. Accessed 17 April 2019
Steenberge SP, Prayson RA (2014) Pediatric cerebral ganglioneuroblastoma. J Clin Neurosci 21:2023–2025 Available: https://linkinghub.elsevier.com/retrieve/pii/S0967586814004962. Accessed 17 April 2019
Yao P-S, Chen G-R, Shang-Guan H-C, Lin Q-S, Wang X-F, Zheng S-F et al (2017) Adult hippocampal ganglioneuroblastoma: case report and literature review. Medicine (Baltimore) 96:e8894 Available: http://www.ncbi.nlm.nih.gov/pubmed/29390424. Accessed 13 May 2019
Rha SE, Byun JY, Jung SE, Chun HJ, Lee HG, Lee JM (2003) Neurogenic tumors in the abdomen: tumor types and imaging characteristics. Radiographics 23:29–43 Available: http://www.ncbi.nlm.nih.gov/pubmed/12533638. Accessed 18 April 2019
Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B et al (1999) The international neuroblastoma pathology classification (the Shimada system). Cancer 86:364–372 Available: http://www.ncbi.nlm.nih.gov/pubmed/10421273. Accessed 18 April 2019
Shimada H, DeLellis RA TF (2017) Neuroblastic tumours of the adrenal gland. WHO Classif tumours Endocr organs IARC, Lyon, p 196–203
McLendon R, Judkins AR, Eberhart CG, Fuller GN, Sarkar C, Ng H-K et al (2016) Other CNS embryonal tumours. CNS ganglioneuroblastoma. WHO Classif tumours Cent Nerv Syst Revised. 4t:207–208
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
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
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-020-04597-4