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Central nervous system (CNS) neuroblastoma. A case-based update.
- Source :
-
Child's Nervous System . May2018, Vol. 34 Issue 5, p817-823. 7p. - Publication Year :
- 2018
-
Abstract
- Background: Primary central nervous system (CNS) neuroblastoma is a rare intracranial tumor affecting children mainly in the first years of life. It is usually a supratentorial tumor with a wide spectrum of clinical presentation, seizures, and focal neurological deficits being the most common presenting signs.Case description: A 2-year-old child was admitted to our ward after a generalized seizure. Neurological examination was normal. Radiological studies showed a small DWI hyperintense lesion of the right rectus gyrus. Follow-up brain MRI 8 months later showed a huge growth of the tumor (90 × 80 × 65 mm) with polycyclic and apparently defined margins, cystic components, and diffuse contrast enhancement. Complete tumor removal was performed in two planned surgical steps. Histological diagnosis was CNS neuroblastoma. At a follow-up of 8 months, the child is in good clinical and neurological condition and is completing chemotherapy treatment according to the SIOP PNET 4 protocol.Discussion and conclusion: A thorough review of the literature confirms that primary CNS neuroblastoma has to be considered a distinct entity. The disease related mortality is 12.5%, lower than the one usually reported for other previously described as PNETs tumors. The most relevant factors influencing prognosis are the possibility of obtaining a complete tumor removal and age more than 3 years, which allows to include radiotherapy among treatment options. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 02567040
- Volume :
- 34
- Issue :
- 5
- Database :
- Academic Search Index
- Journal :
- Child's Nervous System
- Publication Type :
- Academic Journal
- Accession number :
- 129020811
- Full Text :
- https://doi.org/10.1007/s00381-018-3764-3