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ObjectiveRadiation therapy is often advocated for residual or recurrent craniopharyngioma following surgical resection to prevent local recurrence. However, radiation therapy is not always effective and may render tumors more difficult to remove. If this is the case, patients may benefit more from reoperation if gross total resection can be achieved. Nevertheless, there is little data on the impact of radiation on reoperations for craniopharyngioma. In this study, we sought to analyze whether a history of previous radiation therapy (RT) affected extent of resection in patients with recurrent craniopharyngiomas subsequently treated with reoperation via endoscopic endonasal approach (EEA).MethodsThe authors reviewed a prospectively acquired database of EEA reoperations of craniopharyngiomas over 13 years at Weill Cornell, NewYork-Presbyterian Hospital. All procedures were performed by the senior author. The operations were separated into two groups based on whether the patient had surgery alone (group A) or surgery and RT (group B) prior to recurrence.ResultsA total of 24 patients (16 male, 8 female) who underwent surgery for recurrent craniopharyngioma were identified. The average time to recurrence was 7.64 ± 4.34 months (range 3–16 months) for group A and 16.62 ± 12.1 months (range 6–45 months) for group B (p < 0.05). The average tumor size at recurrence was smaller in group A (1.85 ± 0.72 cm; range 0.5–3.2) than group B (2.59 ± 0.91 cm; range 1.5–4.6; p = 0.00017). Gross total resection (GTR) was achieved in 91% (10/11) of patients in group A and 54% (7/13) of patients in group B (p = 0.047). There was a near significant trend for higher average Karnofsky performance status (KPS) score at last follow-up for group A (83 ± 10.6) compared with group B (70 ± 16.3, p = 0.056).ConclusionsWhile RT for residual or recurrent craniopharyngioma may delay time to recurrence, ability to achieve GTR with additional surgery is reduced. In the case of recurrent craniopharyngioma, if GTR can be achieved, consideration should be given to endonasal reoperation prior to the decision to irradiate residual or recurrent tumor.

A 37-year-old man presented to the neurosurgery clinic with diplopia, upward gaze palsy, and headache. He was found to have pupillary light–near dissociation on examination, as seen on a video. A midbrain lesion was seen on MRI, and biopsy showed a nonsecreting germinoma.

Purpose Longstanding overt ventriculomegaly in adults (LOVA) represents a form of chronic adulthood hydrocephalus with symptomatic manifestation in late adulthood. Based on the patency of the aqueduct, two different subcohorts of LOVA can be distinguished. Surgical treatments of this condition are also debated. Therefore, we analyzed preoperative characteristics and clinical outcome after different surgical treatments in a subgroup of LOVA patients with a patent aqueduct. Methods Eighteen LOVA patients with a patent aqueduct consecutively treated at our institution between July 2013 and December 2019 were analyzed for this study. Median age was 70 years. Preoperative radiological and clinical features, surgical procedures (ventriculo-peritoneal shunt or endoscopic third ventriculostomy), and outcomes were collected. Successful outcome was qualitatively defined as an improvement or a halt of progression of the presenting symptoms at follow-up, and quantitatively by changes in mRS and iNPHGS scales. Results Twelve patients underwent an ETV as a primary treatment, while 6 underwent VPS. A total of 22.2% of them were lost to follow-up. Median follow-up time was 38 months. Six patients (66.7%) in the ETV cohort achieved a successful outcome after treatment, with a complication rate of 11.1%. Two patients underwent rescue VPS after ETV failure with a good outcome. Four patients (100%) underwent primary VPS and achieved a satisfactory outcome after treatment, with a reported complications rate of 25%. Conclusion LOVA with patent aqueduct represents, in our opinion, a distinct clinical form of chronic hydrocephalus. For this subgroup, as well as for other forms of LOVA, ETV remains an acceptable first-line treatment option considering the good results, and the low complication rate, obtained in those patients and the hypothesis that hydrocephalus is due to an “intracisternal” obstruction.

•The literature lacks consensus on specific white matter tract alterations in Tourette Syndrome.•This study aims to investigate the microstructural integrity of the Frontal Aslant Tract in patients with Tourette Syndrome using Neurite Orientation Dispersion and Density Imaging (NODDI).•Our results show reduced Fractional Anisotropy and Neurite Density, as well as increased Mean Diffusivity, in the bilateral Frontal Aslant Tract of patients compared to healthy controls.•Moreover, this study suggests that diffusion metrics are mutually correlated: FA and ND are directly proportional, and both are inversely proportional to MD and ODI. Tourette syndrome (TS) is a neurodevelopmental disorder characterized by motor and phonic tics, affecting up to 1% of the adult population. While its etiology is unknown, increasing evidence highlights the key role of brain connectivity changes. Given its involvement in motor and speech inhibition, the Frontal Aslant Tract (FAT), a white matter tract connecting the posterior Superior and Inferior Frontal Gyri, may present structural alterations in individuals with TS. To investigate this hypothesis, a prospective case-control study was conducted from 2020 to 2023, enrolling 11 consecutive right-handed adults diagnosed with TS at a single referral center, alongside 12 age- and sex-matched healthy controls. We examined microstructural alterations of the FAT using metrics derived from two diffusion MRI techniques: Neurite Orientation Dispersion and Density Imaging (NODDI) and Constrained Spherical Deconvolution (CSD). We also examined correlations between diffusion metrics and clinical scores, as well as relationships among the diffusion metrics within both the TS and control groups. After False Discovery Rate corrections, patients’ bilateral FAT showed reduced Fractional Anisotropy (FA) and Neurite Density (ND), and higher Mean Diffusivity (MD), indicating a more isotropic water diffusion and a decreased density in axons and dendrites. No significant correlations between diffusion metrics and clinical measures were found. FA and ND were positively correlated, and they also showed negative correlations with MD and Orientation Dispersion Index (ODI). These results strongly indicate that patients with TS may have reduced integrity of the bilateral FAT, which could be considered a potential target of stimulation techniques in TS treatment. Data and Code Availability Statement: Raw Data will be made available upon a reasonable request to the corresponding author. [Display omitted]

Background: Skull base chordomas are rare tumors arising from notochord. Sphingolipids analysis is a promising approach in molecular oncology, and it has never been applied in chordomas. Our aim is to investigate chordoma behavior and the role of ceramides. Methods: Ceramides were extracted and evaluated by liquid chromatography and mass spectrometry in a cohort of patients with a skull base chordoma. Clinical data were also collected and correlated with ceramide levels. Linear regression and correlation analyses were conducted. Results: Analyzing the association between ceramides level and MIB-1, total ceramides and dihydroceramides showed a strong association (r = 0.7257 and r = 0.6733, respectively) with MIB-1 staining (p = 0.0033 and p = 0.0083, respectively). Among the single ceramide species, Cer C24:1 (r = 0.8814, p ≤ 0.0001), DHCer C24:1 (r = 0.8429, p = 0.0002) and DHCer C18:0 (r = 0.9426, p ≤ 0.0001) showed a significant correlation with MIB-1. Conclusion: Our lipid analysis showed ceramides to be promising tumoral biomarkers in skull base chordomas. Long- and very-long-chain ceramides, such as Cer C24:1 and DHCer C24:1, may be related to a prolonged tumor survival and aggressiveness, and the understanding of their effective biological role will hopefully shed light on the mechanisms of chordoma radio-resistance, tendency to recur, and use of agents targeting ceramide metabolism.

Thalamic gliomas represent a heterogeneous subset of deep-seated lesions for which surgical removal is advocated, although clear prognostic factors linked to advantages in performance status or overall survival are still lacking. We reviewed our Institutional Cancer Registry, identifying patients who underwent surgery for thalamic gliomas between 2006 and 2020. Associations between possible prognostic factors such as tumor volume, grade, the extent of resection and performance status (PS), and overall survival (OS) were evaluated using univariate and multivariate survival analyses. We found 56 patients: 31 underwent surgery, and 25 underwent biopsy. Compared to biopsy, surgery resulted positively associated with an increase in the OS (hazard ratio, HR, at multivariate analysis 0.30, 95% confidence interval, CI, 0.12–0.75). Considering the extent of resection (EOR), obtaining GTR/STR appeared to offer an OS advantage in high-grade gliomas (HGG) patients submitted to surgical resection if compared to biopsy, although we did not find statistical significance at multivariate analysis (HR 0.53, 95% CI 0.17–1.59). Patients with a stable 3-month KPS after surgery demonstrated to have a better prognosis in terms of OS if compared to biopsy (multivariate HR 0.17, 95% CI, 0.05–0.59). Age and histological grades were found to be prognostic factors for this condition (p = 0.04 and p = 0.004, respectively, chi-square test). Considering the entire cohort, p53 positivity (univariate HR 2.21, 95% CI 1.01–4.82) and ATRX positivity (univariate HR 2.69, 95% CI 0.92–7.83) resulted associated with a worse prognosis in terms of OS. In this work, we demonstrated that surgery aimed at tumor resection might offer a stronger survival advantage when a stable 3-month KPS after surgery is achieved.

Background Skull base chordomas (SBC) are rare malignant tumors and few factors have been found to be reliable markers for clinical decision making and survival prognostication. The aim of the present work was to identify specific prognostic factors potentially useful for the management of SBC patients. Methods A retrospective review of all the patients diagnosed and treated for SBC at the Fondazione IRCCS Istituto Neurologico “Carlo Besta” between January 1992 and December 2017 has been performed. Survival analysis was performed and a logistic regression model was used. Statistically significant predictors were rated based on their log odds in order to preliminarily build a personalized grading scale—the Peri-Operative Chordoma Scale (POCS). Results Fifty-nine primary chordoma patients were included. The average follow-up from the first treatment was 82.6 months (95% CI, 65.5–99.7). POCS was built over PFS and MR contrast enhancement (intense vs mild/no, value 4), preoperative motor deficit (yes vs no, value 3), and the development of any postoperative complications (yes vs no, value 2). POCS ranges between 0 and 9, with higher scores being associated with reduced likelihood of survival and progression-free state. Conclusions Our results show that preoperative clinical symptoms (motor deficits), surgical features (extent of tumor resection and surgeon’s experience), development of postoperative complications, and KPS decline represent significant prognostic factors. The degree of MR contrast enhancement significantly correlated to both OS and PFS. We also preliminarily developed the POCS as a prognostic grading scale which may help neurosurgeons in the personalized management of patients undergoing potential adjuvant therapies.

IntroductionSellar/parasellar tumors (SPTs) very rarely become symptomatic during pregnancy. No specific guidelines exist for their management, that is extremely challenging as mother and fetus health can be jeopardized.Materials and methodsData of patients with SPTs becoming symptomatic during pregnancy treated at two Italian referral Centers were retrospectively collected. Systematic literature review was also performed.ResultsOur series consisted of 6 cases, 3 meningiomas, 1 ACTH-secreting adenoma, 1 pituicytoma and 1 craniopharyngioma. Mean age at presentation was 33.6 ± 6.0 years. Five patients complained of visual disturbances, associated with headache in one case, that occurred between gestation week (GW) 22 and 34. In 5 cases, pregnancy was uneventful with the delivery of a healthy baby between GW 33 and 35, followed by endoscopic surgical tumor exeresis (n = 4) or proton bean therapy (n = 1). Another patient presented with stigmata typical of Cushing’s syndrome and rapidly worsening pre-eclampsia, that required pregnancy interruption and adenomectomy. Based on personal and literature cases, a practical algorithm was proposed to help clinicians dealing with these patients.ConclusionsSPTs becoming symptomatic in pregnancy deserve careful monitoring and multidisciplinary management. Overall, wait-and-see approach is suggested, reserving surgery to patients with rapidly progressive/life-threatening situations, significant risk of permanent neurological impairment or malignant lesions.

Despite the increasing popularity of flow diverters (FDs) as an endovascular option for intracranial aneurysms, the treatment of complex aneurysms still represents a challenge. Combined strategies using a flow-preservation bypass could be considered in selected cases. In this study, we retrospectively reviewed our series of patients with complex intracranial aneurysms submitted to bypass. From January 2015 to May 2022, 23 patients were selected. We identified 11 cases (47.8%) of MCA, 6 cases (26.1%) of ACA and 6 cases (26.1%) of ICA aneurysms. The mean maximal diameter was 22.73 ± 12.16 mm, 8 were considered as giant, 9 were fusiform, 8 presented intraluminal thrombosis, 10 presented wall calcification, and 18 involved major branches or perforating arteries. Twenty-five bypass procedures were performed in 23 patients (two EC–IC bypasses with radial artery graft, seventeen single- or double-barrel STA–MCA bypasses and six IC–IC bypasses in anterior cerebral arteries). The long-term bypass patency rate was 94.5%, and the total aneurysm exclusion was 95.6%, with a mean follow-up of 28 months. Median KPS values at last follow-up was 90, and a favorable outcome (KPS ≥ 70 and mRS ≤ 2) was obtained in 87% of the cases. The use of bypass techniques represents, in selected cases, a valid therapeutic option in the management of complex anterior circulation aneurysms when a simpler direct approach, including the use of FD, is considered not feasible.