Search Results (28)

Background: skull base chordoma (Ch) and chondrosarcoma (ChSa) are rare neoplasms prone to local relapse. Alongside surgery, proton therapy (PT) is a well-established treatment for them. Given the relatively long patient survival expectancy, post-treatment quality of life (QoL) is crucial. This study prospectively assessed long-term QoL in this collective. Methods: seventy-seven adult patients (median age, 50 years; male n = 31; 40.3%) with skull base Ch/ChSa completed at least two EORTC-QLQ-C30 and BN20 questionnaires during and after PT. Oncological outcomes and therapy-related toxicities were recorded during follow-up. QoL was analyzed, with post-treatment scores compared to each patient’s baseline and correlated to oncological outcomes. Results: median follow-up was 51 months (range, 1–94), with 5-year overall survival (5yOS) and local control (5yLC) rates of 88.8% and 82.8%, respectively. The time to local or distant failure ranged from 8 to 58 (median, 22) months. QoL deteriorated directly at completion of PT and two to three years thereafter, especially in patients with local or distant failure. From the fifth year onward, QoL improved again. Complete resection before PT correlated to better QoL at all time points. Disease progression was associated with overall worse QoL, higher neurological symptoms already before PT, and higher symptom burden one year thereafter. Males reported better QoL before and one year after PT than females. Conclusions: PT achieves excellent OS and LC in patients with skull base Ch/ChSa. QoL declines directly after PT but remains close to reference population values. From the fifth year onward, QoL improves again. Gender, resection status, and disease progression significantly affect QoL in these patients. Full article

Introduction: Tumors of the lateral skull base, particularly those arising from the external auditory canal and peri-retroauricular regions, present considerable surgical and reconstructive difficulties due to their intricate anatomy and aggressive nature. The anterolateral thigh (ALT) free flap offers a viable reconstruction option, enhancing oncological radicality while minimizing morbidity. Materials and Methods: A retrospective analysis was conducted on five cases of malignant tumors involving the external auditory canal and adjacent soft tissues, treated at a tertiary center from March 2023 to March 2025. All patients underwent radical resection, which included lateral or subtotal temporal bone resection, along with parotidectomy and neck dissection when necessary. Reconstruction was performed using myocutaneous anterolateral thigh free flaps. The study evaluated clinical outcomes, complications, and esthetic results. Results: The ALT flap was used in all cases without major postoperative complications. Two patients received adjuvant radiotherapy; one did not start treatment due to early recurrence. At follow-up, three patients were disease-free, while two had died from unrelated causes. Esthetic and functional outcomes were satisfactory in all patients. Conclusions: The ALT free flap is a universal and reliable option for the reconstruction of complex lateral skull base defects, especially in patients following the radical removal of tumors of the external auditory canal and peri-retroauricular areas. It offers excellent tissue coverage, facilitates adjuvant treatment, and has a low complication rate. While our experience supports its use in aggressive oncological resection, larger studies are needed to confirm these findings. Level of evidence: 4. Full article

Background: One-step cranioplasty combined with tumor removal is a recognized approach in neuro-oncology for patients with neoplastic skull invasion. The use of advanced technologies, including Mixed Reality (MR), has introduced new possibilities in surgical workflows. MR technology may provide additional benefits in preoperative planning, patient engagement, and intraoperative guidance. Can the proposed treatment algorithm, which includes Mixed Reality (MR) for preoperative planning and intraoperative navigation, demonstrate tangible utility and improve outcomes in the surgical management of skull-invasive tumors? Methods: A retrospective study was conducted on 14 patients treated at Cannizzaro Hospital, Catania, Italy, for skull-invasive tumors. The treatment algorithm incorporated tumor removal and one-step cranioplasty using custom-made titanium alloy meshes. Standard intraoperative navigation was compared with MR-based navigation. MR headsets and the Virtual Surgery Intelligence (VSI) platform were employed for preoperative planning, surgical guidance, and patient/family communication. Tumor types included nine meningiomas and five other tumor variants. Results: The integration of MR proved beneficial for preoperative planning, facilitating enhanced visualization of patient anatomy and aiding communication with patients and families. MR-assisted intraoperative navigation offered improved anatomical familiarity but demonstrated slightly lower accuracy compared with standard navigation. Postoperative outcomes were satisfactory across the cohort, with no significant complications reported. Conclusions: The study highlights the potential utility of the proposed treatment algorithm including MR technology in the surgical management of skull-invasive tumors. While MR provides enhanced visualization and preoperative engagement, standard navigation remains more precise during surgery. Nevertheless, MR serves as a valuable complementary tool, and its role in neuro-oncological workflows is expected to grow with technological advancements. Full article

Background/Objectives: Cranial reconstruction (cranioplasty) is a surgical procedure performed to restore skull function and aesthetics following trauma, oncological conditions, or congenital defects. Magnetic resonance imaging (MRI) is commonly used for the postoperative monitoring and diagnosis of patients with cranial implants. However, MRI artifacts caused by these implants can compromise imaging accuracy and diagnostic precision. This study aims to evaluate the extent of MRI artifacts caused by titanium and polyether ether ketone (PEEK) cranial implants and to identify optimal imaging sequences to minimize these artifacts. Methods: Phantom skull models with cranial defects of varying sizes (one-quarter, one-third, and one-half of the skull) were used to simulate real-world clinical conditions. The defects were filled with a water-based medium containing simulated brain tissue and tumor models. Custom 3D-printed titanium and PEEK cranial implants were fixed onto the phantom skulls and scanned using 1.5 T and 3 T MRI scanners. Various imaging sequences were tested, with a focus on optimizing parameters to reduce artifact formation. Turbo Spin Echo (TSE) sequences with fat saturation were implemented to assess their effectiveness in artifact reduction. Results: The study found that MRI artifacts varied based on the implant material, defect size, and magnetic field strength. A higher field strength (3 T) resulted in more pronounced artifacts. However, the use of TSE sequences with fat saturation significantly reduced artifacts and improved lesion visualization, enhancing diagnostic accuracy. Conclusions: This research highlights the importance of optimized MRI protocols when imaging patients with cranial implants. Proper selection of imaging sequences, particularly TSE with fat saturation, can mitigate artifacts and improve diagnostic precision, ultimately benefiting patient outcomes in clinical radiology. Full article

Pediatric primary brain tumors pose significant therapeutic challenges due to their aggressive nature and the critical environment of the developing brain. Traditional modalities like surgery, chemotherapy, and radiotherapy often achieve limited success in high-grade gliomas and embryonal tumors. Tumor-treating fields (TTfields), a non-invasive therapy delivering alternating electric fields, has emerged as a promising approach to disrupt tumor cell division through mechanisms such as mitotic disruption, DNA damage, and tumor microenvironment modulation. TTfields are thought to selectively target dividing tumor cells while sparing healthy, non-dividing cells. While TTfields therapy is FDA-approved for the management of glioblastoma and other cancers, its application in pediatric brain tumors remains under investigation. Preclinical studies reveal its potential in medulloblastoma and ependymoma models, while observational data suggest its safety and feasibility in children. Current research focuses on optimizing TTfields’ efficacy through advanced technologies, including high-intensity arrays, skull remodeling, and integration with immunotherapies such as immune checkpoint inhibitors. Innovative device-based therapies like magnetic field-based technologies further expand the treatment possibilities. As clinical trials progress, TTfields and related modalities offer hope for addressing unmet needs in pediatric neuro-oncology, especially for tumors in challenging locations. Future directions include biomarker identification, tailored protocols, and novel therapeutic combinations to enhance outcomes in pediatric brain tumor management. Full article

Pediatric cancers, while rare, pose unique challenges due to the heightened sensitivity of developing tissues and the increased risk of long-term radiation-induced effects. Radiotherapy (RT) is a cornerstone in pediatric oncology, but its application is limited by concerns about toxicity, particularly secondary malignancies, growth abnormalities, and cognitive deficits. CyberKnife (CK), an advanced robotic radiosurgery system, has emerged as a promising alternative due to its precision, non-invasiveness, and ability to deliver hypofractionated, high-dose RT while sparing healthy tissues. This narrative review explores the existing evidence on CK application in pediatric patients, synthesizing data from case reports, small series, and larger cohort studies. All the studies analyzed reported cases of tumors located in the skull or in the head and neck region. Findings suggest CK’s potential for effective tumor control with favorable toxicity profiles, especially for complex or inoperable tumors. However, the evidence remains limited, with the majority of studies involving small sample sizes and short follow-up periods. Moreover, concerns about the “dose-bath” effect and limited long-term data on stochastic risks warrant cautious adoption. Compared to Linac-based RT and proton therapy, CK offers unique advantages in reducing session numbers and enhancing patient comfort, while its real-time tracking provides superior accuracy. Despite these advantages, CK is associated with significant limitations, including a higher potential for low-dose scatter (often referred to as the “dose-bath” effect), extended treatment times in some protocols, and high costs requiring specialized expertise for operation. Emerging modalities like π radiotherapy further underscore the need for comparative studies to identify the optimal technique for specific pediatric cases. Notably, proton therapy remains the benchmark for minimizing long-term toxicity, but its cost and availability limit its accessibility. This review emphasizes the need for balanced evaluations of CK and highlights the importance of planning prospective studies and long-term follow-ups to refine its role in pediatric oncology. A recent German initiative to establish a CK registry for pediatric CNS lesions holds significant promise for advancing evidence-based applications and optimizing treatment strategies in this vulnerable population. Full article

Background: Trigeminal schwannomas (TSs) are uncommon tumors found along any segment of the fifth cranial nerve (CN V). Typically located at the skull base, these benign tumors carry substantial morbidity due to the extent of traditional surgical methods. Minimally invasive endoscopic surgery such as the endoscopic transorbital approach (ETOA) presents promising new avenues for treatment, with the transorbital approach emerging as a potentially successful alternative. Methods: This review systematically assesses the application of the ETOA in treating TSs. PubMed, Ovid MEDLINE, and Embase were thoroughly searched for articles detailing the use of the ETOA in clinical case studies. The outcomes of interest encompassed epidemiological profiling, surgical results, and complication rates. Results: This study included 70 patients with TSs (from six studies), with 22 males (31.4%) and 58 females (68.6%). Patients averaged 55 years and were monitored for around 16.4 months (on average). In most tumors, the middle cranial fossa was involved to some degree. The majority (87.2%) were large (3–6 cm) and underwent gross total resection (GTR) or near-total resection in 87.2% of patients. Preoperatively, sensory alterations were common, along with proptosis, neuropathic pain, and diplopia. Postoperatively, complications included ptosis, diplopia, sensory impairment, corneal keratopathy, masticatory difficulty, and neuralgia. The pure ETOA was the primary surgical technique used in 90% of cases, with no recurrence observed during the follow-up period. Conclusions: Using the ETOA to treat TSs demonstrated an oncologic control rate of 87.2%. Postoperative complications including ptosis, diplopia, and sensory disturbances have been observed, but careful monitoring and management can mitigate these problems. The ETOA emerges as a viable surgical option, especially for tumors involving the middle cranial fossa, capable of adapting to individual patient needs and demonstrating efficacy in TS management. Full article

Background/Objectives: Clivus metastases from distant neoplasms are uncommon occurrences both in clinical practice and the neurosurgical literature. Surgical management is debated, particularly about the role of surgery and the preferable approach. The aim of this study was to report our surgical experience and review the concerning literature. Methods: Our institutional registry was retrospectively reviewed, and patients who underwent surgical treatment for clival metastasis from 1998 to 2023 were included. A PRISMA systematic review of the literature was performed. Results: Four patients were enrolled, and all of them underwent an endoscopic endonasal approach (EEA). Three presented with cranial nerve (CN) VI palsy. The aim of surgery was biopsy in all cases. No complications were reported. Mean overall survival (OS) was 6 ± 1 months. The systematic review retrieved 27 papers reporting 39 patients who underwent the surgical treatment of clivus metastases. Most of them (79.5%) presented with CN palsies, and EEA was the preferred approach in 92.3% of the cases, to perform a biopsy in most patients (59%). Two hemorrhagic complications (5.1%) were reported, and the mean OS was 9.4 ± 5.6 months. Conclusions: Clival metastases are uncommonly observed, in most cases, during advanced stages of oncological disease. The aim of surgery should be the confirmation of diagnosis and symptomatic relief, balancing the risk–benefit ratio in a multidisciplinary context. EEA is the approach of choice, and it should be carried out in experienced tertiary skull base centers. Full article

Sinonasal and skull base malignancies represent a rare, heterogenous group of pathologies with an incidence of 0.556 per 100,000 persons in the population. Given the numerous critical anatomic structures located adjacent to the sinonasal cavity and skull base, surgery for tumors in this region requires careful pre-operative planning with the assistance of radiological imaging and intraoperative image guidance technologies to reduce the risk of complications. Virtual surgical planning (VSP) and three-dimensional models (3DMs) are adjunctive technologies which assist clinicians to better visualize patient anatomy using enhanced digital radiological images and physical stereolithographic models based on patients’ personal imaging. This review summarizes our institutional experience with VSP and 3DMs in sinonasal and skull base surgical oncology. A clinical case series is used to thematically illustrate the application of VSP and 3DMs in surgical ablation, reconstruction, patient communication, medical education, and interdisciplinary teamwork in sinonasal and skull base surgery. Full article

Background: Radiation necrosis (RN) is a possible late complication of stereotactic radiosurgery (SRS), but only a few risk factors are known. The aim of this study was to assess tumor location in correlation to the development of radiation necrosis for skull base (SB) and non-skull base tumors. Methods: All patients treated with radiosurgery for benign neoplasms (2004–2020) were retrospectively evaluated. The clinical, imaging and medication data were obtained and the largest axial tumor diameter was determined using MRI scans in T1-weighted imaging with gadolinium. The diagnosis of RN was established using imaging parameters. Patients with tumors located at the skull base were compared to patients with tumors in non-skull base locations. Results: 205 patients could be included. Overall, 157 tumors (76.6%) were located at the SB and compared to 48 (23.4%) non-SB tumors. Among SB tumors, the most common were vestibular schwannomas (125 cases) and meningiomas (21 cases). In total, 32 (15.6%) patients developed RN after a median of 10 (IqR 5–12) months. Moreover, 62 patients (30.2%) had already undergone at least one surgical resection. In multivariate Cox regression, SB tumors showed a significantly lower risk of radiation necrosis with a Hazard Ratio (HR) of 0.252, p < 0.001, independently of the applied radiation dose. Furthermore, higher radiation doses had a significant impact on the occurrence of RN (HR 1.372, p = 0.002). Conclusions: The risk for the development of RN for SB tumors appears to be low but should not be underestimated. No difference was found between recurrent tumors and newly diagnosed tumors, which may support the value of radiosurgical treatment for patients with recurrent SB tumors. Full article

Augmented reality (AR) involves the overlay of computer-generated images onto the user’s real-world visual field to modify or enhance the user’s visual experience. With respect to neurosurgery, AR integrates preoperative and intraoperative imaging data to create an enriched surgical experience that has been shown to improve surgical planning, refine neuronavigation, and reduce operation time. In addition, AR has the potential to serve as a valuable training tool for neurosurgeons in a way that minimizes patient risk while facilitating comprehensive training opportunities. The increased use of AR in neurosurgery over the past decade has led to innovative research endeavors aiming to develop novel, more efficient AR systems while also improving and refining present ones. In this review, we provide a concise overview of AR, detail current and emerging uses of AR in neurosurgery and neurosurgical training, discuss the limitations of AR, and provide future research directions. Following the guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA), 386 articles were initially identified. Two independent reviewers (GH and AC) assessed article eligibility for inclusion, and 31 articles are included in this review. The literature search included original (retrospective and prospective) articles and case reports published in English between 2013 and 2023. AR assistance has shown promise within neuro-oncology, spinal neurosurgery, neurovascular surgery, skull-base surgery, and pediatric neurosurgery. Intraoperative use of AR was found to primarily assist with surgical planning and neuronavigation. Similarly, AR assistance for neurosurgical training focused primarily on surgical planning and neuronavigation. However, studies included in this review utilize small sample sizes and remain largely in the preliminary phase. Thus, future research must be conducted to further refine AR systems before widespread intraoperative and educational use. Full article

Clival chordomas are rare but aggressive skull base tumors that pose significant treatment challenges and portend dismal prognosis. The aim of this study was to highlight the advantages and limitations of available treatments, to furnish prognostic indicators, and to shed light on novel therapeutic strategies. We conducted a retrospective study of clival chordomas that were surgically treated at our institution from 2003 to 2022; for comparison purposes, we provided a systematic review of published surgical series and, finally, we reviewed the most recent advancements in molecular research. A total of 42 patients underwent 85 surgeries; median follow-up was 15.8 years, overall survival rate was 49.9% at 10 years; meanwhile, progression-free survival was 26.6% at 10 years. A significantly improved survival was observed in younger patients (<50 years), in tumors with Ki67 ≤ 5% and when adjuvant radiotherapy was performed. To conclude, clival chordomas are aggressive tumors in which surgery and radiotherapy play a fundamental role while molecular targeted drugs still have an ancillary position. Recognizing risk factors for recurrence and performing a molecular characterization of more aggressive lesions may be the key to future effective treatment. Full article

Introduction: Pediatric skull base lesions occur rarely and are of various etiologies. Traditionally, open craniotomy has been the treatment of choice; however, nowadays, endoscopic approaches are increasingly applied. In this retrospective case series, we describe our experience in treating pediatric skull base lesions and provide a systematic overview of the literature on the treatment and outcome of pediatric skull base lesions. Methods: We conducted a retrospective data collection of all pediatric patients (<18 years) treated for a skull base lesion at the Division of Pediatric Neurosurgery, University Children’s Hospital Basel, Switzerland, between 2015 and 2021. Descriptive statistics and a systematic review of the available literature were additionally conducted. Results: We included 17 patients with a mean age of 8.92 (±5.76) years and nine males (52.9%). The most common entity was sellar pathologies (n = 8 47.1%), with craniopharyngioma being the most common pathology (n = 4, 23.5%). Endoscopic approaches, either endonasal transsphenoidal or transventricular, were used in nine (52.9%) cases. Six patients (35.3%) suffered from transient postoperative complications, while in none of the patients these were permanent. Of the nine (52.9%) patients with preoperative deficits, two (11.8%) showed complete recovery and one (5.9%) partial recovery after surgery. After screening 363 articles, we included 16 studies with a total of 807 patients for the systematic review. The most common pathology reported in the literature confirmed our finding of craniopharyngioma (n = 142, 18.0%). The mean PFS amongst all the studies included was 37.73 (95% CI [36.2, 39.2]) months, and the overall weighted complication rate was 40% (95% CI [0.28 to 0.53] with a permanent complication rate of 15% (95% CI [0.08 to 0.27]. Only one study reported an overall survival of their cohort of 68% at five years. Conclusion: This study highlights the rarity and heterogeneity of skull base lesions in the pediatric population. While these pathologies are often benign, achieving GTR is challenging due to the deep localization of the lesions and eloquent adjacent structures, leading to high complication rates. Therefore, skull base lesions in children require an experienced multidisciplinary team to provide optimal care. Full article

Skull base surgery has undergone significant progress following key technological developments. From early candle-lit devices to the modern endoscope, refinements in visualization techniques have made endoscopic skull base surgery (ESBS) a standard practice for treating a variety of conditions. The endoscope has also been integrated with other technologies to enhance visualization, including fluorescence agents, intraoperative neuronavigation with augmented reality, and the exoscope. Endoscopic approaches have allowed neurosurgeons to reevaluate skull base neuroanatomy from new perspectives. These advances now serve as the foundation for future developments in ESBS. In this narrative review, we discuss the history and development of ESBS, current visualization techniques, and future innovations. Full article