Child's Nervous System

, Volume 34, Issue 5, pp 817–823 | Cite as

Central nervous system (CNS) neuroblastoma. A case-based update

  • Federico BianchiEmail author
  • Gianpiero Tamburrini
  • Marco Gessi
  • Paolo Frassanito
  • Luca Massimi
  • Massimo Caldarelli
Case-Based Update



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.


Neuroblastoma Infant Surgery Tumor 


Compliance with ethical standards

Conflict of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Pediatric Neurosurgery, Institute of NeurosurgeryCatholic University Medical SchoolRomeItaly
  2. 2.Histopathology DivisionCatholic University Medical SchoolRomeItaly

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