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This article, titled "Targeted Chopstick Mononostril Approach for a Lower Clivus Chordoma," discusses a case study of a 47-year-old male who presented with nasopharyngitis and odynophagia that did not respond to typical treatment. Imaging revealed a large lesion at the craniocervical junction, which was successfully removed using a targeted endoscopic mononostril approach. The patient was diagnosed with a classic chordoma and referred for adjuvant proton-beam therapy, resulting in a positive outcome. The article provides details on the surgical technique, indications, alternative approaches, and risks associated with the procedure. [Extracted from the article]

Craniovertebral junction chordomas can represent a surgical challenge, especially when intradural extension and contact with neurovascular structures is involved. To date, there is still controversy on the choice of optimal surgical corridor for such lesions, with endoscopic endonasal, far lateral and anterolateral corridors being those most commonly used. In this operative video, we present a case of craniovertebral junction chordoma with significant bone destruction and intradural extension ( Fig. 1 ). The tumor was safely removed using an anterolateral approach with transposition of the vertebral artery to gain access to the lateral craniovertebral junction. The endoscope is demonstrated to help with intradural tumor removal, providing a wide angle of view through a relatively narrow dural opening ( Fig. 2 ). In this video, nuances of the anterolateral approach, chordoma removal strategies and closure technique are presented in detail. A gross total resection was achieved, and the postoperative course was uneventful. In summary, the anterolateral approach with endoscopic assistance can offer a safe and effective corridor for the surgical management of craniovertebral junction chordomas. The link to the video can be found at: https://youtu.be/pC0YxKgNoMY. [ABSTRACT FROM AUTHOR]

Background: The combined transpetrosal approach (CTPA) is a versatile technique suitable for challenging skull base pathologies. Despite the advantages provided by a wide surgical exposure, the soft tissue trauma, complex and time-consuming bony work, and cosmetic issues make it far from patient expectations. In this study, the authors describe a less invasive modification of the CTPA, the mini-combined transpetrosal approach (mini-CTPA), and perform a quantitative comparison between these two approaches. Methods: Five human specimens were used for this study. CTPA was performed on one side and mini-CTPA on the opposite side. The surgical freedom, petroclival and brainstem area of exposure, and maneuverability for 6 anatomical targets, provided by the CTPA and mini-CTPA, were calculated and statistically compared. The bony volumes corresponding to each anterior petrosectomy were also measured and compared. Three clinical cases with an operative video are also reported to illustrate the effectiveness of the approach. Results: The question-mark skin incision done along the muscle attachments permits an optimal cosmetic result. Even though the limited incision, the smaller craniotomy, and the less extensive bone drilling of mini-CTPA provide a smaller area of surgical freedom, the areas of exposure of petroclival region and brainstem were not statistically different between the two approaches. The antero-posterior maneuverability for the oculomotor foramen (OF), Meckel's cave (MC) and the REZ of trigeminal nerve, and the supero-inferior maneuverability for OF, MC, Dorello's canal, and REZ of CN VII are significantly reduced by the smaller opening. The bony volume of anterior petrosectomy resulted similar among the approaches. Conclusions: The mini-CTPA is an interesting alternative to the CTPA, providing comparable surgical exposure both for petroclival region and for brainstem. Although the lesser soft tissue dissection and bony opening decrease the surgical maneuverability, the mini-CTPA may reduce surgical time, potential approach-related morbidities, and improve cosmetic and functional outcomes for the patients. [ABSTRACT FROM AUTHOR]

Simple Summary: Chordomas are rare bone tumors characterized by a high recurrence rate. Presently, no medical treatment is available for advanced diseases due to the lack of molecular data and preclinical models. The current study showed the establishment and characterization of the largest panel chordoma xenografts, allowing pharmacological studies. In one PBRM1-mutated model, we demonstrated a strong therapeutic efficacy of the EZH2-inhibitor tazemetostat, encouraging further research on EZH2-inhibitors in chordomas. Chordomas are rare neoplasms characterized by a high recurrence rate and a poor long-term prognosis. Considering their chemo-/radio-resistance, alternative treatment strategies are strongly required, but their development is limited by the paucity of relevant preclinical models. Mutations affecting genes of the SWI/SNF complexes are frequently found in chordomas, suggesting a potential therapeutic effect of epigenetic regulators in this pathology. Twelve PDX models were established and characterized on histological and biomolecular features. Patients whose tumors were able to grow into mice had a statistically significant lower progression-free survival than those whose tumors did not grow after in vivo transplantation (p = 0.007). All PDXs maintained the same histopathological features as patients' tumors. Homozygous deletions of CDKN2A/2B (58.3%) and PBRM1 (25%) variants were the most common genomic alterations found. In the tazemetostat treated PDX model harboring a PBRM1 variant, an overall survival of 100% was observed. Our panel of chordoma PDXs represents a useful preclinical tool for both pharmacologic and biological assessments. The first demonstration of a high antitumor activity of tazemetostat in a PDX model harboring a PBRM1 variant supports further evaluation for EZH2-inhibitors in this subgroup of chordomas. [ABSTRACT FROM AUTHOR]

Objective  To investigate on the feasibility and safety of a new approach which consists of delaying instrumentation after destabilizing craniovertebral junction (CVJ) chordoma surgery, allowing proton beam radiotherapy to be performed in a metal-free tumoral cavity. Design  This is a retrospective series of a prospectively maintained database. Participants  Five consecutive patients operated on for a CVJ chordomas for which instrumentation after tumor resection was deferred to after radiotherapy treatment. Main Outcome Measures  The main outcome consisted of measurements of the following parameters: C0–C2 angle, atlanto-dens interval (ADI), condylar gap, and the position of the dens relative to McGregor's line and coronal inclination, performed at 3 different times for all patients: before tumor surgery (baseline), before instrumentation surgery, and after instrumentation surgery. Results  For all patients, CVJ parameters deteriorated during the delay period, but stayed within normal limits for most. Because of radiological instability, one patient necessitated instrumentation before receiving radiotherapy. All parameters except condylar gap were partially corrected after instrumentation. No new neurological symptom or evolving neck pain occurred during the delay period. Conclusion  Delayed instrumentation of CVJ chordomas can be a safe alternative that might lead to improved subsequent radiotherapeutical treatment. Patient's selection and close clinical and radiological follow-up are mandatory for the success of this approach. [ABSTRACT FROM AUTHOR]

Background: Surgical treatment of lesions involving the ventral craniovertebral junction (CVJ) and the lower clivus, traditionally involved complex lateral or transoral approaches to the skull base. However, mid or upper clivus involvement requires more extensive lateral approaches. Recently, the endoscopic endonasal approach (EEA) has become the standard for upper CVJ lesions and medial clival, and a valuable alternative for those tumors extending in its upper third as well as laterally. However, the EEA is associated with an increased risk of post-operative CSF leakage and infection when the tumor is characterized by an intradural extension. Furthermore, whenever the tumor has significant lateral and/or inferior extension below the odontoid process, the chances for a complete resection decrease. Method: To analyze the extent of exposure of a hybrid microscopic-endoscopic transcondylar antero-lateral approach to the CVJ and clival region, and to verify its effectiveness in terms of mid and upper clival access. Five silicone-injected cadaver heads were used. Following a standard antero-lateral approach, condylectomy and jugular tubercle drilling were performed, after which angled endoscopes were utilized to extend the bone resection to the clivus. A volumetric assessment of the amount of clival removal was carried out. A case of CVJ chordoma operated through this approach is presented. Results: The hybrid antero-lateral transcondylar approach provides adequate exposure of the ventral CVJ, up to the dorsum sellae and the sphenoid sinus, the contralateral petrous apex, and the contralateral paraclival internal carotid artery (ICA). Approximately 60% of the total clival volume can be removed with this approach. The main limitation is the limited visualization of the ipsilateral paraclival ICA and petrous apex. Conclusion: The hybrid antero-lateral transcondylar approach is a valuable surgical option for CVJ tumor extending from C2 to the mid and upper clivus. [ABSTRACT FROM AUTHOR]

Background and purpose To evaluate clinical results and safety of a dose adaptive protocol based on tumor volume coverage and critical structure constraints, for the treatment of skull base chordomas. Material and methods Between May 2006 and October 2012, 106 patients with skull base chordoma were treated by combined photon and proton irradiation. Prescribed dose levels were 68.4, 70.2, 72 and 73.8 Gy(RBE) in once daily fractionation of 1.8 Gy(RBE). Dose level and dosimetric constraints to organs at risk depended on postoperative residual Gross Tumor Volume (GTV) coverage. Local control (LC) and overall survival (OS) were evaluated using the Kaplan–Meier method. Results With a median follow-up of 61 months, the 2-year, 4-year, and 5-year LC rates were 88.6%, 78.3%, and 75.1%, respectively. GTV > 25 mL ( p  = 0.034 , HR = 2.22; 95%CI 1.06–4.62) was an independent unfavorable prognostic factor of LC. The 2-year, 4-year, and 5-year OS rates were 99%, 90.2%, and 88.3%, respectively. Grade 3–5 late toxicity was observed in 7 patients, resulting in 93% 5-year freedom from high-grade toxicity. Conclusions This study suggests that the probability of LC of skull base chordomas depends on postoperative GTV. The dose adaptive protocol achieves acceptable local control. Future studies should investigate whether further dose escalation to doses in excess of 74 Gy(RBE) would achieve better results. [ABSTRACT FROM AUTHOR]