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VALUTAZIONE DELL’OUTCOME POST-OPERATORIO IN PAZIENTI PEDIATRICI SOTTOPOSTI AD INTERVENTO NEURO-CHIRURGICO PER NEOPLASIA DEL SISTEMA NERVOSO CENTRALE
No full textPurpose: The aim of this study is to analyze the early outcome of the post-operatory (PO) period in terms of significant adverse events rate in neurosurgical pediatric patients with central nervous system tumors (CNS) at our institution, to identify possible risk factors for the occurrence of these events and to compare our results with published data.
Materials and methods: We prospectively recorded the PO data of children (0-16 years) with CNS tumors operated on between 2009 and 2018 at our institution and retrospectively reviewed them. We evaluated the occurrence of infections, seizures, hyponatremia, new neurological deficits, hemorrhages, ischemia, and cerebrospinal fluid related complications. We applied the consensus definitions proposed by the Canadian Pediatric Neurosurgery Study Group and by the CDC in order to obtain data comparable with previous studies. In our analysis we considered the adverse events which occurred during the inpatient stay or within 30 days of surgery.
Results: We reviewed the PO period of 78 procedures (54% M, mean age 9,1). 58% of children underwent tumor gross total resection, 31% partial removal and 11% a surgical biopsy. The tumor was supratentorial in 59%, infratentorial in 37% and located in the spinal cord in 4%. 73% of patients underwent a first surgical procedure while the rest a reintervention. Astrocytic tumor was the most frequent histological diagnosis (55 %). 6 % of children developed Hydrocephalus, 5 % a maior CSF leakage and 8 % a significant pseudomeningocele. 49 % of children developed fever and 87 % were treated with an antibiotic. in 20 % of procedures the PO period was associated with some kind of infection but only 6 % of children experienced a CNS or shunt infection. 19 % of patients developed a new moderate to severe neurological deficit and seizures occurred in 5 %. We recorded no hemorrhages, 4 % ischemic events and 23 % of patients experienced Hyponatremia but only 4 % a CSWS and 4 % a SIADH.
The mortality rate was 0% while 31% of operations were linked to at least one significant adverse event (SAE). On logistic regression analysis we found that radical procedures have a 79 % and supratentorial tumors a 87 % lower risk of SAE in the PO period in comparison with partial removal/biopsy and infratentorial/spinal (p 0,009 and p 0,003). The mean hospital and ICU PO length of stay in procedures linked with at least one SAE was 34.2 days (SD26.6) and 9.5 days (SD 19.3) respectively, which was statistically significantly longer than what we found in procedures with no SAE events (p<0.00001, p<0.005).
Conclusions:
Our SAE rate in a general tertiary hospital is high but comparable to other important international pediatric institutions using the same system. Earlier recognition of risk factors and post-operatory adverse events is fundamental for patient management and could affect long term outcome. This research will provide useful data to improve the quality of treatments delivered to children with CNS tumors after surgery, continuous surveillance is needed
Prevalence and significance of BRAF alterations in a pediatric population with low-grade gliomas
No full textBackground
Low-grade gliomas (LGG) are the most common brain tumours in children, represented by heterogeneous pathological entities. BRAF gene alterations have been recently identified as responsible of constitutive activation of the mitogen-activated protein kinase pathway (MAPK) and hence involved in the development of LGG in children. There is evidence that the BRAF V600E mutation is more common in supratentorial LGG while the KIAA1549:BRAF fusion in posterior fossa pilocytic astrocytoma (PA).
Objective
This case series describes the prevalence of BRAF alterations in LGG patients, trying to relate them to outcome.
Methods
Children aged 0–14 years, with a diagnosis of LGG, referred to a single neuro-oncologic centre, were retrospectively reviewed to analyze clinical and histopathological features related to BRAF alterations.
Results
A total of 35 patients were included (16 males, median age 85.5 ± 81.3 months). BRAF mutations were searched on 7/35 children (20 %) resulting positive in 5/7 (71 %). Two of them (40 %) showed the
KIAA1549:BRAF fusion. They were both pilocytic astrocytomas located in posterior fossa: 1/2 (50 %) was totally resected, showing stable disease (SD) 1 year after surgey; the other was not surgically treatable and
showed a progressive disease during chemotherapy. Three out of five (60 %) presented V600E mutation. All of them were supratentorial: 1 pilomyxoid astrocytoma, treated with two partial resections and subse-
quent chemotherapy, was in SD 5 years after diagnosis; 1 diffuse pleomorphic xanthoastrocytoma underwent three partial resections and showed SD after 5 years of vemurafenib; 1 glioneuronal tumor was in
SD after complete resection and radiotherapy.
Conclusion
Our data support the evidence that specific mutations of the BRAF pathway are related to site and histological subtype of brain tumors. This sample is too small to help supporting the hypothesis that these alterations
have a prognostic impact. Extent of surgery and localization seem to be the most important prognostic factors
Primary intramedullary spinal cord tumors in children: a single centre experience
No full textBackground
Primary spinal cord tumors are rare, accounting for 4 % to 8 % of all Central Nervous System (CNS) neoplasms in the pediatric population. Most of them are low grade tumors and prognosis is related to the extent
of surgical resection.
Objective
The aim of this study was to evaluate the clinical outcome of a small group of patients with intramedullary spinal tumors.
Methods
We retrospectively analyzed clinical and histopathological features, treatment modalities, survival rates of children aged 0–14 years with a diagnosis of spinal tumor treated at our department during the last 15 years.
Results
A total of 93 patients with a CNS tumor were included. Four of them (4.3%) had a spinal localization: 2 males and 2 girls, 3/4 (75%) 5 years of age at diagnosis (mean age 4.4 ± 3.6 years). Three (75 %) had cervical
localization, one (25 %) was localized in the lumbosacral tract. All cases underwent surgery: 2 biopsies (50 %), 1 partial resection (25 %) and 1 total resection (25 %). All of them were low-grade gliomas: 2 (50 %)
pilocytic astrocytomas, 1 (25 %) glioneuronal tumor and 1 ependymoma. All of the children with subtotal resection (biopsy and partial resection) underwent subsequent therapies: 2 were treated with radiotherapy (RT)
and chemotherapy (CT), 1 is under medical treatment (SIOP-LGG 2004 protocol). Regimens of CT included Carboplatin-Vincristin in pilocytic astrocytomas, and Cisplatin-Temozolomide in glioneuronal tumor. At
current time, all these patients had stable disease.
Conclusion
Our data support the evidence that the majority of intramedullary tumors are histologically benign and radical resection results in long-term survival. When total surgical resection is not achievable, excellent results in
survival rates could be obtained with a multimodal treatment combining RT and CT
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