Multimodal Intraoperative Image-Driven Surgery for Skull Base Chordomas and Chondrosarcomas.
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ABSTRACT:
SUBMITTER: Essayed WI
PROVIDER: S-EPMC8870528 | biostudies-literature | 2022 Feb
REPOSITORIES: biostudies-literature
Publications
Multimodal Intraoperative Image-Driven Surgery for Skull Base Chordomas and Chondrosarcomas.
Essayed Walid I WI Juvekar Parikshit P Bernstock Joshua D JD Rassi Marcio S MS Almefty Kaith K Zamani Amir Arsalan AA Golby Alexandra J AJ Al-Mefty Ossama O
Cancers 20220215 4
Given the difficulty and importance of achieving maximal resection in chordomas and chondrosarcomas, all available tools offered by modern neurosurgery are to be deployed for planning and resection of these complex lesions. As demonstrated by the review of our series of skull base chordoma and chondrosarcoma resections in the Advanced Multimodality Image-Guided Operating (AMIGO) suite, as well as by the recently published literature, we describe the use of advanced multimodality intraoperative i ...[more]
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Project description:Purpose Chordomas are malignant tumors of the axial spine and skull base, and they are notorious for their poor treatment response. Differentiating these tumors from comparatively less malignant chondrosarcomas is crucial for treatment and prognostication. Both tumor types differ in their developmental origin. Chordomas are considered to be derived from notochordal remnants and chondrosarcomas from mesenchymal cells. Here, we evaluated the differential expression of developmental transcription factors in these skull base tumors.Methods Histopathologically-confirmed tumor biopsies were obtained from 12 chordoma and 7 chondrosarcoma patients. Following RNA extraction, samples were submitted to real-time quantitative PCR (RT-qPCR) for the evaluation of 32 evolutionary conserved genes that are known to associate with notochord, mesoderm, and axial spine development. Gene expression levels were normalized to housekeeping genes ACTB and RS27a.Results Fifteen genes were either exclusively expressed (n = 12) or overexpressed (n = 3; 2.21-4.43 fold increase) in chordoma, compared to chondrosarcoma. Brachyury and CD24 were highly and exclusively expressed in chordoma. Other novel genes exclusive to chordomas included chordin, HOXA5 and ACAN. Vice versa, ten genes were either exclusively expressed (n = 2) or overexpressed (n = 8; 0.01-0.66 fold increase) in chondrosarcoma, compared to chordoma.Conclusion As chordoma patients demonstrate a worse prognosis compared to chondrosarcoma patients, the differential expression of chordin, HOXA5 and ACAN and CD24 could be relevant for the pathophysiology of chordomas and may have diagnostic and treatment value. Further study on role of these genes in tumorigenesis is therefore warranted.
| S-EPMC11832612 | biostudies-literature
Project description:Background Skull base chordoma and chondrosarcoma are exceptionally rare bone tumors with high propensity for local recurrence. Different postoperative radiation modalities are often used to improve the clinical efficacy. Proton therapy (PT) might be among the most promising ones because of the unique ballistic characteristics of high-energy particles. However, previous meta-analysis often included studies with combined radiation techniques. No systematic review to date has directly analyzed the survival and toxicity of pure PT for these two types of malignancies. Methods By following the PRISMA guidelines, a systematic search of three databases was conducted. Articles were screened and data were extracted according to a prespecified scheme. R 4.2.0 software was used to conduct the meta-analysis. Normal distribution test was used for the incidence rate of each subgroup. Results A total of seven studies involving 478 patients were included in this analysis. The quality of included articles ranged from moderate to high quality. All patients were histopathologically diagnosed with chordoma or chondrosarcoma, and the follow-up time of the cohort ranged from 21 to 61.7 months. For PT planning, the median target volume ranged from 15 cc to 40 cc, and the administered median dose varied from 63 to 78.4 GyRBE at 1.8–2.0 GyRBE per fraction. The 1-, 2-, 3-, 5-, and 7-year local control and overall survival rates were 100%, 93%, 87%, 78%, and 68%, and 100%, 99%, 89%, 85%, and 68%, respectively. The late grade 3 or higher toxicities were reported in only two involved articles. Conclusions Until now, medical centers worldwide have exerted PT to improve outcomes of skull base chordomas and chondrosarcomas. PT not combined with other radiation modalities showed favorable local control and survival with a low incidence of severe radiation-induced toxicities, which manifests promising clinical benefits. However, high-quality evidence is still limited, requiring future clinical trials and prospective studies in selected patients.
| S-EPMC9732011 | biostudies-literature
Project description:Skull-base chordoma and chondrosarcoma are rare radioresistant tumors treated with surgical resection and/or radiotherapy. Because of the established dosimetric and biological benefits of heavy particle therapy, we performed a systematic and evidence-based review of the clinical outcomes of patients with skull-base chordoma and chondrosarcoma treated with carbon ion radiotherapy (CIRT). A literature review was performed using a MEDLINE search of all articles to date. We identified 227 studies as appropriate for review, and 24 were ultimately included. The published data illustrate that CIRT provides benchmark disease control outcomes for skull-base chordoma and chondrosarcoma, respectively, with acceptable toxicity. CIRT is an advanced treatment technique that may provide not only dosimetric benefits over conventional photon therapy but also biologic intensification to overcome mechanisms of radioresistance. Ongoing research is needed to define the magnitude of benefit, patient selection, and cost-effectiveness of CIRT compared to other forms of radiotherapy.
| S-EPMC10605639 | biostudies-literature
Project description:Primary skull base chondrosarcomas (SBCs) can severely affect patients' quality of life. Surgical-resection and radiotherapy are feasible but may cause debilitating complications. We systematically reviewed the literature on primary SBCs. PubMed, EMBASE, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA guidelines to include studies of patients with primary SBCs. Clinical characteristics, management strategies, and treatment outcomes were analyzed. We included 33 studies comprising 1307 patients. Primary SBCs mostly involved the middle-fossa (72.7%), infiltrating the cavernous-sinus in 42.4% of patients. Cranial-neuropathies were reported in 810 patients (62%). Surgical-resection (93.3%) was preferred over biopsy (6.6%). The most frequent open surgical approaches were frontotemporal-orbitozygomatic (17.6%) and pterional (11.9%), and 111 patients (21.3%) underwent endoscopic-endonasal resection. Post-surgical cerebrospinal-fluid leaks occurred in 36 patients (6.5%). Radiotherapy was delivered in 1018 patients (77.9%): photon-based (41.4%), proton-based (64.2%), and carbon-based (13.1%). Severe post-radiotherapy complications, mostly hypopituitarism (15.4%) and hearing loss (7.1%) were experienced by 251 patients (30.7%). Post-treatment symptom-improvement (46.7%) and reduced/stable tumor volumes (85.4%) showed no differences based on radiotherapy-protocols (p = 0.165; p = 0.062). Median follow-up was 67-months (range, 0.1-376). SBCs recurrences were reported in 211 cases (16.1%). The 5-year and 10-year progression-free survival rates were 84.3% and 67.4%, and overall survival rates were 94% and 84%. Surgical-resection and radiotherapy are effective treatments in primary SBCs, with acceptable complication rates and favorable local tumor control.
| S-EPMC8656924 | biostudies-literature
Project description:Objectives To describe the potential uses of computed tomography image guidance in concert with the surgical robot for skull base surgery. Design An anatomical study was conducted. Setting Tertiary academic center. Participants Cadaveric skull. Main Outcome Measures The primary outcome measure was to measure the accuracy of robotic arm positioning to anatomical landmarks on a skull using image guidance and the surgical robot synchronously. Instruments with different angles of rotations were used. Estimated systematic error was calculated and compared with achieved errors. Clinical applications of metachronous image guidance and robotic system were discussed. Results The skull model approximated < 1 mm accuracy using standard image guidance instruments and the 0-degree robotic arm positioning. Increased angles of instruments from 20 to 60 degrees on the robotic system revealed more significant increases in error than estimated. Conclusions Image guidance may be useful for transoral robotic approaches. Precise movements are improved by limiting the angle of deviation. Future studies will help optimize the combined technologies before validating the study in clinical settings.
| S-EPMC4108486 | biostudies-other
Project description:BackgroundSkull-base chordomas are rare malignant bone cancers originating from the remnant of the notochord. Survival is variable, and clinical or molecular factors cannot reliably predict their outcomes. This study therefore identified epigenetic subtypes that defined new chordoma epigenetic profiles and their corresponding characteristics.MethodsMethylation profiles of 46 chordoma-resected neoplasms between 2008 and 2014, along with clinical information, were collected. K-means consensus clustering and principal component analysis were used to identify and validate the clusters. Single-sample gene set enrichment analysis, methylCIBERSORT algorithm, and copy number analysis were used to identify the characteristics of the clusters.ResultsUnsupervised clustering analysis confirmed two clusters with a progression-free survival difference. Gene set enrichment analysis indicated that the early and late estrogen response pathways and the hypoxia pathway were activated whereas the inflammatory and interferon gamma responses were suppressed. Forty-six potential therapeutic targets corresponding to differentially methylated sites were identified from chordoma patients. Subgroups with a worse outcome were characterized by low immune cell infiltration, higher tumor purity, and higher stemness indices. Moreover, copy number amplifications mostly occurred in cluster 1 tumors and the high-risk group. Additionally, the presence of a CCNE1 deletion was exclusively found in the group of chordoma patients with better outcome, whereas RB1 and CDKN2A/2B deletions were mainly found in the group of chordoma patients with worse outcome.ConclusionsChordoma prognostic epigenetic subtypes were identified, and their corresponding characteristics were found to be variable.
| S-EPMC9691996 | biostudies-literature
Project description:(1) Background: Our purpose is to describe the design of a phase II clinical trial on 5-fraction proton therapy for chordomas and chondrosarcomas of the skull base and to present early results in terms of local control and clinical tolerance of the first prospective series. (2) Methods: A dose of 37.5 GyRBE in five fractions was proposed for chordomas and 35 GyRBE in five fractions for chondrosarcomas. The established inclusion criteria are age ≥ 18 years, Karnofsky Performance Status ≥ 70%, clinical target volume up to 50 cc, and compliance with dose restrictions to the critical organs. Pencil beam scanning was used for treatment planning, employing four to six beams. (3) Results: A total of 11 patients (6 chordomas and 5 chondrosarcomas) were included. The median follow-up was 12 months (9-15 months) with 100% local control. Acute grade I-II headache (64%), grade I asthenia and alopecia (45%), grade I nausea (27%), and grade I dysphagia (18%) were described. Late toxicity was present in two patients with grade 3 temporal lobe necrosis. (4) Conclusions: Hypofractionated proton therapy is showing encouraging preliminary results. However, to fully assess the efficacy of this therapeutic approach, future trials with adequate sample sizes and extended follow-ups are necessary.
| S-EPMC10705113 | biostudies-literature
Project description:BackgroundHead and neck (H&N) sarcomas in children can poise numerous challenges to the surgical oncologist and require multidisciplinary input and meticulous surgical planning. The application of computer-assisted design/computer-assisted manufacturing (CAD/CAM) has been extensively examined in H&N reconstruction in adults, but its utility in ablative oncologic surgery in children warrants further examination. We present preliminary results utilizing CAD/CAM techniques to assist in planning tumor resections and the application of intra-operative radiation in children with skull-base sarcomas.MethodsA retrospective cohort review of all pediatric patients who presented to a tertiary care cancer center for surgical resection of a skull-base malignancy was performed between 1980 and 2021. All children under 18 years of age with diagnosis of a skull-base sarcoma as confirmed with imaging and pathology were analyzed.ResultsA total of 21 children were identified but only four children with skull-base sarcomas had diagnostic imaging available in whom computer-assisted volumetric analyses were generated. In these cases, CAD/CAM was used to plan surgical approaches and intraoperative radiotherapy, significantly aiding in treatment for these complicated pediatric cases.ConclusionCAD/CAM planning for oncologic resection has huge potential. Here we have shown its utility in pre-operative surgical planning and for administration of intraoperative radiation therapy. Future studies are needed to examine its value in facilitating intraoperative surgical management and patient outcomes, as well as cost effectiveness.
| S-EPMC11187774 | biostudies-literature
Project description:BackgroundData 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).MethodsPediatric 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).ResultsOf 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.ConclusionThis 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.
| S-EPMC10484173 | biostudies-literature
Project description:Objectives Intraoperative vagus nerve monitoring during skull base surgery has been reported with the use of an oral nerve monitoring endotracheal tube. However, the intraoral presence of an endotracheal tube can limit exposure by its location in the operative field during transfacial approaches and by limiting superior mobilization of the mandible during transcervical approaches. We describe a transnasal vagus nerve monitoring technique. Design and Participants Ten patients underwent open skull base surgery. Surgical approaches included transcervical (five), transfacial/maxillary swing (three), and double mandibular osteotomy (two). The vagus nerve was identified, stimulated, and monitored in all cases. Main Outcome Measures Intraoperative nerve stimulation, pre- and postoperative vagus nerve function through the use of flexible laryngoscopy in conjunction with assessment of subjective symptoms of hoarseness, voice change, and swallowing difficulty. Results Three patients had extensive involvement of the nerve by tumor with complete postoperative nerve deficit, one patient had a transient deficit following dissection of tumor off of nerve with resolution, and the remaining patients had nerve preservation. One patient experienced minor epistaxis during monitor tube placement that was managed conservatively. Conclusions Transnasal vagal nerve monitoring is a simple method that allows for intraoperative monitoring during nerve preservation surgery without limiting surgical exposure.
| S-EPMC4375050 | biostudies-other