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EnglishBackground: 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.
Bianchi, F., Tamburrini, G., Gessi, M., Frassanito, P., Massimi, L., Caldarelli, M., Central nervous system (CNS) neuroblastoma. A case-based update, <<CHILDS NERVOUS SYSTEM>>, 2018; 34 (5): 817-823. [doi:10.1007/s00381-018-3764-3] [http://hdl.handle.net/10807/124171]
| Autori: | ||
| Titolo: | Central nervous system (CNS) neuroblastoma. A case-based update | |
| Digital Object Identifier (DOI): | http://dx.doi.org/10.1007/s00381-018-3764-3 | |
| URL: | link.springer.de/link/service/journals/00381/index.htm | |
| Data di pubblicazione: | 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. | |
| Lingua: | Inglese | |
| Rivista: | ||
| Citazione: | Bianchi, F., Tamburrini, G., Gessi, M., Frassanito, P., Massimi, L., Caldarelli, M., Central nervous system (CNS) neuroblastoma. A case-based update, <<CHILDS NERVOUS SYSTEM>>, 2018; 34 (5): 817-823. [doi:10.1007/s00381-018-3764-3] [http://hdl.handle.net/10807/124171] | |
| Appare nelle tipologie: | Articolo in rivista, Nota a sentenza |
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