Craniovertebral Junction Chordomas Research Articles

Anatomical determinants of occipitocervical fusion in skull base chordoma resection: a systematic review of the literature with illustrative cases.

Skull base chordomas are rare, locally osseo-destructive lesions that present unique surgical challenges due to their involvement of critical neurovascular and bony structures at the craniovertebral junction (CVJ). Radical cytoreductive surgery improves survival but also carries significant morbidity, including the potential for occipitocervical (OC) destabilization requiring instrumented fusion. The published experience on OC fusion after CVJ chordoma resection is limited, and the anatomical predictors of OC instability in this context remain unclear. PubMed and Embase were systematically searched according to the PRISMA guidelines for studies describing skull base chordoma resection and OC fusion. The search strategy was predefined in the authors' PROSPERO protocol (CRD42024496158). The systematic review identified 11 surgical case series describing 209 skull base chordoma patients and 116 (55.5%) who underwent OC instrumented fusion. Most patients underwent lateral approaches (n = 82) for chordoma resection, followed by midline (n = 48) and combined (n = 6) approaches. OC fusion was most often performed as a second-stage procedure (n = 53), followed by single-stage resection and fusion (n = 38). The degree of occipital condyle resection associated with OC fusion was described in 9 studies: total unilateral condylectomy reliably predicted OC fusion regardless of surgical approach. After lateral transcranial approaches, 4 studies cited at least 50%-70% unilateral condylectomy as necessitating OC fusion. After midline approaches-most frequently the endoscopic endonasal approach (EEA)-at least 75% unilateral condylectomy (or 50% bilateral condylectomy) led to OC fusion. Additionally, resection of the medial atlantoaxial joint elements (the C1 anterior arch and tip of the dens), usually via EEA, reliably necessitated OC fusion. Two illustrative cases are subsequently presented, further exemplifying how the extent of CVJ bony elements removed via EEA to achieve complete chordoma resection predicts the need for OC fusion. Unilateral total condylectomy, 50% bilateral condylectomy, and resection of the medial atlantoaxial joint elements were the most frequently described independent predictors of OC fusion in skull base chordoma resection. Additionally, consistent with the occipital condyle harboring a significantly thicker joint capsule at its posterolateral aspect, an anterior midline approach seems to tolerate a greater degree of condylar resection (75%) than a lateral transcranial approach (50%-70%) prior to generating OC instability.

Management strategies in clival and craniovertebral junction chordomas: a 29-year experience.

Chordomas represent one of the most challenging subsets of skull base and craniovertebral junction (CVJ) tumors to treat. Despite extensive resection followed by proton-beam radiation therapy, the recurrence rate remains high, highlighting the importance of developing efficient treatment strategies. In this study, the authors present their experience in treating clival and CVJ chordomas over a 29-year period. The authors conducted a retrospective study of clival and CVJ chordomas that were surgically treated at their institution from 1991 to 2020. This study focuses on three aspects of the management of these tumors: the factors influencing the extent of resection (EOR), the predictors of survival, and the outcomes of the endoscopic endonasal approaches (EEAs) compared with open approaches (OAs). A total of 265 surgical procedures were performed in 210 patients, including 123 OAs (46.4%) and 142 EEAs (53.6%). Tumors that had an intradural extension (p = 0.03), brainstem contact (p = 0.005), cavernous sinus extension (p = 0.004), major artery encasement (p = 0.01), petrous apex extension (p = 0.003), or high volume (p = 0.0003) were significantly associated with a lower EOR. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 52.1% and 75.1%, respectively. Gross-total resection and Ki-67 labeling index < 6% were considered to be independent prognostic factors of longer PFS (p = 0.0005 and p = 0.003, respectively) and OS (p = 0.02 and p = 0.03, respectively). Postoperative radiation therapy correlated independently with a longer PFS (p = 0.006). Previous surgical treatment was associated with a lower EOR (p = 0.01) and a higher rate of CSF leakage after EEAs (p = 0.02) but did not have significantly lower PFS and OS compared with primary surgery. Previously radiation therapy correlated with a worse outcome, with lower PFS and OS (p = 0.001 and p = 0.007, respectively). EEAs were more frequently used in patients with upper and middle clival tumors (p = 0.002 and p < 0.0001, respectively), had a better rate of EOR (p = 0.003), and had a lower risk of de novo neurological deficit (p < 0.0001) compared with OAs. The overall rate of postoperative CSF leakage after EEAs was 14.8%. This large study showed that gross-total resection should be attempted in a multidisciplinary skull base center before providing radiation therapy. EEAs should be considered as the gold-standard approach for upper/middle clival lesions based on the satisfactory surgical outcome, but OAs remain important tools for large complex chordomas.

Multicentric Chordoma With Initial Resection by Bilateral Transcondylar Approach: 2-Dimensional Operative Video.

Chordoma is a rare skull base tumor with malignant behavior.1-3 It invades locally with high recurrences, metastasizes distally, and seeds after interventions.1-4 Chordoma exemplifies the malignant progression doctrine as it accumulated genetic mutations. The natural history of untreated disease is 2.4 yr on average survival.5 Best tumor control is achieved by radical resection, followed by high doses radiation. Multicentric chordoma is an ill-defined challenging entity extremely rare in the literature. However, chordoma is known for distal metastasis, particularly to the lungs, iatrogenic cerebrospinal fluid (CSF) dissemination with drop metastasis, or surgical implantation. A subset of patients present with synchronous or metachronous regional or distal neuraxial lesions associated with the initial chordoma. Patients presenting with multicentric bony axial lesions and no extra-axial metastases point toward the multicentric chordoma concept rather than local, hematogenous, or CSF spread.6-12 Biopsy of these multicentric lesions can show a spectrum of abnormalities ranging from benign notochordal tumor to chordomas confirming the multicentric hypothesis.9 We present a patient who underwent a bilateral transcondylar approach for giant craniovertebral junction chordoma and then treated with radiation and a second lesion at the C6 transverse foramen. Six years later, she presented with a chordoma at the petrous apex. The patient consented to surgery and to the publications of her image. The participants and any identifiable individuals consented to publication of his/her image. Image at 1:39 reprinted with permission from Al-Mefty O, Operative Atlas of Meningiomas. Vol 1, © LWW, 1998.

Craniovertebral junction chordomas: Case series and strategies to overcome the surgical challenge.

Introduction:Chordomas are rare and malignant primary bone tumors. Different strategies have been proposed for chordomas involving the craniovertebral junction (CVJ) compared to other locations. The impossibility to achieve en bloc excision, the impact on stability and the need for proper reconstruction make their surgical management challenging.Objective:The objective is to discuss surgical strategies in CVJ chordomas operated in a single-center during a 7 years' experience (2013-2019).Methods:Adult patients with CVJ chordoma were retrospectively analyzed. The clinical, radiological, pathological, and surgical data were discussed.Results:A total number of 8 patients was included (among a total number of 32 patients suffering from skull base chordoma). Seven patients underwent endoscopic endonasal approach (EEA), and posterior instrumentation was needed in three cases. Three explicative cases were reported: EEA for midline tumor involving lower clivus and upper cervical spine (case 1), EEA and complemental posterior approach for occurred occipitocervical instability (case 2), C2 chordoma which required aggressive bone removal and consequent implant positioning, focusing on surgical planning (timing and type of surgical stages, materials and customization of fixation system) (case 3).Conclusion:EEA could represent a safe route to avoid injuries to neurovascular structure in clival locations, while a combined approach could be considered when tumor spreads laterally. Tumor involvement or surgical procedures could give raise to CVJ instability with the need of complementary posterior instrumentation. Thus, a tailored preoperative planning should play a key role, especially when aggressive bone removal and implant positioning are needed.

Hybrid antero-lateral transcondylar approach to the clivus: a laboratory investigation and case illustration.

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. 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. 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. The hybrid antero-lateral transcondylar approach is a valuable surgical option for CVJ tumor extending from C2 to the mid and upper clivus.

Anterolateral Approach with Endoscopic Assistance for Resection of a Craniovertebral Junction Chordoma.

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 .

Delayed Instrumentation Following Removal of Cranio-Vertebral Junction Chordomas: A Technical Note.

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.

Extracranial Surgery of the Skull Base and Craniovertebral Junction Chordomas

Methods: During the 1985–2015 period, 432 patients with skull base chordomas were operated. In 1985–2006, 207 patients were operated, 126 (61%) by transcranial approaches and 81 (39%) by extracranial approaches (transnasal and transoral). During the 2007–2015 period, 225 patients with skull base chordomas were operated, 58 (25.8%) by a variety of transcranial approaches, and 167 (74.2%) by extracranial. Twenty-eight patients underwent simultaneous combined operation: occipito spondylodesis and transoral removal of the tumor.

The algorithm for surgical treatment of skull base tumors invading the craniovertebral junction

Surgical treatment of skull base tumors invading the craniovertebral junction is a complex medical problem due to a high rate of adverse postoperative outcomes in these patients. The study aim was to optimize surgical treatment in patients with skull base tumors invading the craniovertebral junction. A comparative analysis of 2 groups of patients was performed. The study group included 28 patients with skull base and craniovertebral junction chordomas who underwent single-stage surgery, including posterior occipitospondylodesis and tumour resection using the transoral and combined transoral and transnasal approaches, in the period between 2000 and 2015. The control group included 21 patients with the same pathology who underwent microsurgery using the transoral approach without occipitospondylodesis in the period between 1990 and 2009. Most of the patients in both groups were operated on at late disease stages when they presented with gross clinical signs. The use of single-stage occipitospondylodesis and transoral skull base tumor removal significantly (compared to the control group) extends indications for surgical treatment of skull base tumors, which were previously considered unresectable, and provides better results. New surgical techniques significantly increase the completeness of tumor resection, decrease the rate of postoperative complications, accelerate the rehabilitation process, and improve the patient's quality of life.

Clival and Craniovertebral Junction Chordomas

This study primarily aims to evaluate the efficacy of gamma knife surgery in controlling growth progression rate of residual clival chordoma through retrospective outcome analysis of 12 consecutive patients.Data for 12 consecutive patients underwent GKS for post-operative residual histologically verified clival chordoma at our institution (IMC – Cairo- Egypt) from 2006 through end of 2017 were retrospectively reviewed and analyzed with mean follow-up period of 45 months (range12–120 months).In the last follow up MR, tumor growth control was achieved in 33.3% of patients (mean treated tumors volume was 2.7cc with mean peripheral prescription dose of 16 Gy), and 66.7% of patients reported lost tumor growth control (mean treated tumor volume was 9.2 cc with mean peripheral dose was 13.5 Gy). The overall tumor free progression with mean follow up period of 45mos was 33.3%. The Actuarial 2, 3 and 5 year tumor control rates after initial GKS was 35%, 30% and 25% respectively.Without satisfactory maximum tumor reduction and sufficient high peripheral prescription radiation tumor dose, it should not be expected that GKS could efficiently control the progression of residual clival chordoma, especially for long term.

Ventral Surgical Approaches to Craniovertebral Junction Chordomas

Chordomas are primarily malignant tumors encountered at either end of the neural axis; the craniovertebral junction and the sacrococcygeal junction. In this article, we discuss the surgical management of craniovertebral junction chordomas. In this paper, we discuss the surgical management of craniovertebral junction chordomas. The following approaches are illustrated: transoral-transpalatopharyngeal approach, high anterior cervical retropharyngeal approach, endoscopic transoral approach, and endoscopic transnasal approach. No single operative approach can be used for all craniovertebral chordomas. Therefore, the location of the tumor dictates which approach or approaches should be used.