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Neuro-oncology Sep 2023Data on clinical outcomes for base of skull (BOS) chordomas in the pediatric population is limited. We report patient outcomes after surgery and proton radiotherapy...
BACKGROUND
Data on clinical outcomes for base of skull (BOS) chordomas in the pediatric population is limited. We report patient outcomes after surgery and proton radiotherapy (PRT).
METHODS
Pediatric patients with BOS chordomas were treated with PRT or combined proton/photon approach (proton-based; for most, 80% proton/20% photon) at the Massachusetts General Hospital from 1981 to 2021. Endpoints of interest were overall survival (OS), disease-specific survival, progression-free survival (PFS), freedom from local recurrence (LC), and freedom from distant failure (DC).
RESULTS
Of 204 patients, median age at diagnosis was 11.1 years (range, 1-21). Chordoma location included 59% upper and/or middle clivus, 36% lower clivus, 4% craniocervical junction, and 1% nasal cavity. Fifteen (7%) received pre-RT chemotherapy. Forty-seven (23%) received PRT, and 157 (77%) received comboRT. Median total dose was 76.7 Gy (RBE) (range, 59.3-83.3). At a median follow-up of 10 years (interquartile range, 5-16 years), 56 recurred. Median OS and PFS were 26 and 25 years, with 5-, 10-, and 20-year OS and PFS rates of 84% and 74%, 78% and 69%, and 64% and 64%, respectively. Multivariable actuarial analyses showed poorly differentiated subtype, radiographical progression prior to RT, larger treatment volume, and lower clivus location to be prognostic factors for worse OS, PFS, and LC. RT was well tolerated at a median follow-up of 9 years (interquartile range, 4-16 years). Side effects included 166 patients (80%) with mild/moderate acute toxicities, 24 (12%) patients with late toxicities, and 4 (2%) who developed secondary radiation-related malignancies.
CONCLUSION
This is the largest cohort of BOS chordomas in the literature, pediatric and/or adult. High-dose PRT following surgical resection is effective with low rates of late toxicity.
Topics: Adult; Humans; Child; Infant; Child, Preschool; Adolescent; Young Adult; Protons; Chordoma; Skull Base Neoplasms; Chondrosarcoma; Proton Therapy; Skull Base; Treatment Outcome; Follow-Up Studies
PubMed: 37029730
DOI: 10.1093/neuonc/noad068 -
Orbit (Amsterdam, Netherlands) Oct 2023
Topics: Humans; Orbit; Liposarcoma; Orbital Neoplasms; Orbit Evisceration
PubMed: 34470545
DOI: 10.1080/01676830.2021.1966814 -
Rhinology Dec 2023This meta-analysis aims to investigate the outcome of sinonasal adenoid cystic carcinoma (snAdCC). We followed PRISMA guidelines and included studies reporting 5-year... (Meta-Analysis)
Meta-Analysis
This meta-analysis aims to investigate the outcome of sinonasal adenoid cystic carcinoma (snAdCC). We followed PRISMA guidelines and included studies reporting 5-year overall survival (OS) rates for snAdCC. Eligible studies were identified through a literature search and assessed using JBI critical appraisal checklist. A total of 17 studies were included comprising 2259 patients (mean age: 58.1 years, 52.7% female, 47.3% male). The meta-analysis demonstrated that the 5-year OS, 10-year OS, and 5-year disease-free survival (DFS) were 68%, 40%, and 47.2%, respectively. Descriptive statistics on study level showed high rates of locally advanced tumor stages at diagnosis: 23% cT3, 53% cT4, 3.4% N+, and 4.2% M+. 29.7% of the tumors were in the nasal cavity, 67.6% in the paranasal sinuses. The maxillary, ethmoid, sphenoid, and frontal sinus were affected in 50.9%, 7.2%, 4%, and 0.5%, of cases. A combination of surgery and radiotherapy was used in 45.4% of the patients and 19.3% of patients received surgery only. In conclusion, these findings emphasize the significance of thorough surveillance for individuals with snAdCC to identify any potential recurrence or progression of the disease.
Topics: Humans; Male; Female; Middle Aged; Carcinoma, Adenoid Cystic; Paranasal Sinus Neoplasms; Nose Neoplasms; Retrospective Studies; Frontal Sinus
PubMed: 37703531
DOI: 10.4193/Rhin23.204 -
Neurosurgical Review Sep 2023Endoscopic transsphenoidal surgery is a novel surgical technique requiring specific training. Different models and simulators have been recently suggested for it, but no... (Review)
Review
Endoscopic transsphenoidal surgery is a novel surgical technique requiring specific training. Different models and simulators have been recently suggested for it, but no systematic review is available. To provide a systematic and critical literature review and up-to-date description of the training models or simulators dedicated to endoscopic transsphenoidal surgery. A search was performed on PubMed and Scopus databases for articles published until February 2023; Google was also searched to document commercially available. For each model, the following features were recorded: training performed, tumor/arachnoid reproduction, assessment and validation, and cost. Of the 1199 retrieved articles, 101 were included in the final analysis. The described models can be subdivided into 5 major categories: (1) enhanced cadaveric heads; (2) animal models; (3) training artificial solutions, with increasing complexity (from "box-trainers" to multi-material, ct-based models); (4) training simulators, based on virtual or augmented reality; (5) Pre-operative planning models and simulators. Each available training model has specific advantages and limitations. Costs are high for cadaver-based solutions and vary significantly for the other solutions. Cheaper solutions seem useful only for the first stages of training. Most models do not provide a simulation of the sellar tumor, and a realistic simulation of the suprasellar arachnoid. Most artificial models do not provide a realistic and cost-efficient simulation of the most delicate and relatively common phase of surgery, i.e., tumor removal with arachnoid preservation; current research should optimize this to train future neurosurgical generations efficiently and safely.
Topics: Humans; Animals; Endoscopy; Cadaver; Computer Simulation; Databases, Factual; Skull Base Neoplasms
PubMed: 37725193
DOI: 10.1007/s10143-023-02149-3 -
No Shinkei Geka. Neurological Surgery Mar 2024With the development of endoscopic and peripheral instruments, endonasal or transcranial endoscopic surgery for skull-base tumors has become more common. Preoperative...
With the development of endoscopic and peripheral instruments, endonasal or transcranial endoscopic surgery for skull-base tumors has become more common. Preoperative simulation makes it relatively easy to understand the anatomical relationship between skull base tumors and the surrounding vital structures, which vary with each case. This may lead to the avoidance of complications and an improvement in the removal rate. Especially in cases of skull base tumors where multiple surgical approaches are possible, the three-dimensional model can be used to confirm the surgical field for each approach and consider the most appropriate. With the development of endovascular treatment and radiotherapy, experience in craniotomy has decreased. Young neurosurgeons need to develop skills to learn as efficiently as possible from their limited experience. Therefore, it is extremely useful to provide an environment that allows for easier preoperative simulations.
Topics: Humans; Skull Base Neoplasms; Endoscopy; Nose; Craniotomy; Skull Base
PubMed: 38514122
DOI: 10.11477/mf.1436204917 -
Journal of Neuro-ophthalmology : the... Dec 2023
Topics: Humans; Chordoma; Skull Base Neoplasms; Head and Neck Neoplasms; Optic Neuritis; Skull
PubMed: 36728058
DOI: 10.1097/WNO.0000000000001575 -
Oral Oncology Nov 2023Although complete excision is the standard of care for ameloblastoma, a subset of recurrent and/or metastasizing ameloblastomas are difficult to treat surgically. Over... (Review)
Review
Although complete excision is the standard of care for ameloblastoma, a subset of recurrent and/or metastasizing ameloblastomas are difficult to treat surgically. Over the past decade, several recurrent mutations in the mitogen-activated protein kinase pathway genes have been identified in ameloblastoma, based on which the efficacy of targeted therapy has been investigated. However, most of the literature has focused on BRAF V600E mutations, the most common oncogenic mutations in ameloblastoma. Hence, this study aims to review the current knowledge of targetable genetic alterations in ameloblastoma from a broader perspective. In addition, the therapeutic potential of immunotherapy for ameloblastoma will be briefly discussed in the context of tumoral PD-L1 expression and the tumor immune microenvironment.
Topics: Humans; Ameloblastoma; Precision Medicine; Jaw Neoplasms; Proto-Oncogene Proteins B-raf; Mutation; Tumor Microenvironment
PubMed: 37816291
DOI: 10.1016/j.oraloncology.2023.106585 -
International Forum of Allergy &... Apr 2024The impact of sinonasal malignancies (SNMs) on quality of life (QOL) at presentation is poorly understood. The Sinonasal Outcome Test (SNOT-22) and University of... (Observational Study)
Observational Study
BACKGROUND
The impact of sinonasal malignancies (SNMs) on quality of life (QOL) at presentation is poorly understood. The Sinonasal Outcome Test (SNOT-22) and University of Washington Quality of Life (UWQOL) are validated QOL instruments with distinctive subdomains. This study aims to identify factors impacting pretreatment QOL in SNM patients to personalize multidisciplinary management and counseling.
METHODS
Patients with previously untreated SNMs were prospectively enrolled (2015-2022) in a multicenter observational study. Baseline pretreatment QOL instruments (SNOT-22, UWQOL) were obtained along with demographics, comorbidities, histopathology/staging, tumor involvement, and symptoms. Multivariable regression models identified factors associated with reduced baseline QOL.
RESULTS
Among 204 patients, presenting baseline QOL was significantly reduced. Multivariable regression showed worse total SNOT-22 QOL in patients with skull base erosion (p = 0.02). SNOT-rhinologic QOL was worse in women (p = 0.009), patients with epistaxis (p = 0.036), and industrial exposure (p = 0.005). SNOT extranasal QOL was worse in patients with industrial exposure (p = 0.016); worse SNOT ear/facial QOL if perineural invasion (PNI) (p = 0.027). Squamous cell carcinoma pathology (p = 0.037), palate involvement (p = 0.012), and pain (p = 0.017) were associated with worse SNOT sleep QOL scores. SNOT psychological subdomain scores were significantly worse in patients with palate lesions (p = 0.022), skull base erosion (p = 0.025), and T1 staging (p = 0.023). Low QOL was more likely in the presence of PNI on UW health (p = 0.019) and orbital erosion on UW overall (p = 0.03). UW social QOL was worse if palatal involvement (p = 0.023) or PNI (p = 0.005).
CONCLUSIONS
Our findings demonstrate a negative impact on baseline QOL in patients with SNMs and suggest sex-specific and symptom-related lower QOL scores, with minimal histopathology association. Anatomical tumor involvement may be more reflective of QOL than T-staging, as orbital and skull base erosion, PNI, and palate lesions are significantly associated with reduced baseline QOL.
Topics: Male; Humans; Female; Treatment Outcome; Quality of Life; Endoscopy; Skull Base; Skull Base Neoplasms; Chronic Disease; Rhinitis
PubMed: 37646428
DOI: 10.1002/alr.23261 -
American Journal of Rhinology & Allergy Sep 2023Leiomyomas are benign smooth muscle tumors that are rarely diagnosed in the nasal cavity and paranasal sinuses. (Review)
Review
BACKGROUND
Leiomyomas are benign smooth muscle tumors that are rarely diagnosed in the nasal cavity and paranasal sinuses.
OBJECTIVE
This systematic review summarizes the histopathologic and clinical tumor characteristics, surgical management, and follow-up of sinonasal leiomyomas.
METHODS
A systematic review of the literature on sinonasal leiomyoma was performed by applying the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Studies that met the inclusion criteria were assessed for level of evidence. Patient demographics, clinical and pathological tumor characteristics, primary intervention, and results of follow-up were evaluated.
RESULTS
Forty studies including 84 patients with sinonasal leiomyoma were identified. The tumor was most often located in the nasal cavity (47/84, 56%) originating from the inferior turbinate (32/84, 38%). Patients mostly presented with symptoms originating from an intranasal mass, including recurrent epistaxis (41/84, 49%), nasal obstruction (43/84, 51.2%), and localized facial or head pain (25/84, 29.8%). Surgery was performed in all cases. An endoscopic approach was most frequently chosen. Recurrence occurred only twice (2.4%). Morbidity was noted in 2 cases (2.4%) following postoperative bleeding and 1 (1.2%) case following a CSF leak.
CONCLUSION
Sinonasal leiomyomas are neoplasms of the smooth muscle manifesting clinically with recurrent epistaxis and nasal obstruction. Management goal is total resection with clear margins to avoid local recurrence.
Topics: Humans; Paranasal Sinus Neoplasms; Nasal Obstruction; Epistaxis; Follow-Up Studies; Leiomyoma; Nose Neoplasms
PubMed: 37093753
DOI: 10.1177/19458924231170464 -
Neurosurgery Dec 2023Tuberculum sellae meningiomas are resected via an expanded endonasal (EEA) or transcranial approach (TCA). Which approach provides superior outcomes is debated. The...
International Tuberculum Sellae Meningioma Study: Preoperative Grading Scale to Predict Outcomes and Propensity-Matched Outcomes by Endonasal Versus Transcranial Approach.
BACKGROUND AND OBJECTIVES
Tuberculum sellae meningiomas are resected via an expanded endonasal (EEA) or transcranial approach (TCA). Which approach provides superior outcomes is debated. The Magill-McDermott (M-M) grading scale evaluating tumor size, optic canal invasion, and arterial involvement remains to be validated for outcome prediction. The objective of this study was to validate the M-M scale for predicting visual outcome, extent of resection (EOR), and recurrence, and to use propensity matching by M-M scale to determine whether visual outcome, EOR, or recurrence differ between EEA and TCA.
METHODS
Forty-site retrospective study of 947 patients undergoing tuberculum sellae meningiomas resection. Standard statistical methods and propensity matching were used.
RESULTS
The M-M scale predicted visual worsening (odds ratio [OR]/point: 1.22, 95% CI: 1.02-1.46, P = .0271) and gross total resection (GTR) (OR/point: 0.71, 95% CI: 0.62-0.81, P < .0001), but not recurrence ( P = .4695). The scale was simplified and validated in an independent cohort for predicting visual worsening (OR/point: 2.34, 95% CI: 1.33-4.14, P = .0032) and GTR (OR/point: 0.73, 95% CI: 0.57-0.93, P = .0127), but not recurrence ( P = .2572). In propensity-matched samples, there was no difference in visual worsening ( P = .8757) or recurrence ( P = .5678) between TCA and EEA, but GTR was more likely with TCA (OR: 1.49, 95% CI: 1.02-2.18, P = .0409). Matched patients with preoperative visual deficits who had an EEA were more likely to have visual improvement than those undergoing TCA (72.9% vs 58.4%, P = .0010) with equal rates of visual worsening (EEA 8.0% vs TCA 8.6%, P = .8018).
CONCLUSION
The refined M-M scale predicts visual worsening and EOR preoperatively. Preoperative visual deficits are more likely to improve after EEA; however, individual tumor features must be considered during nuanced approach selection by experienced neurosurgeons.
Topics: Humans; Meningioma; Meningeal Neoplasms; Neuroendoscopy; Retrospective Studies; Treatment Outcome; Skull Base Neoplasms; Sella Turcica
PubMed: 37418417
DOI: 10.1227/neu.0000000000002581