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Patient with a clival chordoma with craniocervical junction involvement. Due to extensive involvement of adjacent soft tissue compartments in addition to the craniocervical junction, the patient underwent a radical resection via a 2-stage approach consisting of an endoscopic endonasal transclival/transpterygoid approach and a right sided extreme lateral approach with a vertebral artery transposition. Preoperative MRI scans shown in panels a, b and postoperative scans in panels c, d. Arrow denotes the parapharyngeal segment of the right internal carotid artery for reference. Figure property of the Department of Neurosurgery, The University of Texas M.D. Anderson Cancer Center
Source publication
Skull base primary malignancies represent a heterogeneous group of histologic diagnoses and sarcomas of the skull base are specific malignant tumors that arise from mesenchymal cells and can be classified by site of origin into bony and soft tissue sarcomas. The most common bony sarcomas include: chondrosarcoma, osteosarcoma, chordoma, Ewing’s sarc...
Citations
... However, an accurate diagnosis is challenging, as this condition may manifest with diverse and non-specific symptoms. Patients frequently present with chronic headache and various symptoms caused by tumor compression of brain tissues, blood vessels and nerves, but these symptoms also occur in patients with chordoma and other brain tumors (4)(5)(6). Furthermore, characteristics on computed tomography (CT) and magnetic resonance imaging (MRI) scans typically resemble those of meningioma and chordoma, further hampering an accurate preoperative diagnosis (7)(8)(9)(10)(11). ...
Intracranial primary chondrosarcomas are rare, accounting for <0.15% of all intracranial tumors, but exhibit a high risk of recurrence. Due to the rarity of this condition, it has proven difficult to establish efficacy-based treatment guidelines. The present study details a case of clivus chondrosarcoma exhibiting no recurrence following surgical resection using an endoscopic transsphenoidal approach and postoperative adjuvant radiotherapy. A 41-year-old female presented with primary symptoms of left eye esotropia, scotoma of the left nasal visual field and double vision. Preoperative cranial magnetic resonance imaging revealed a lesion on the clivus, which was initially diagnosed as chordoma. However, clivus chondrosarcoma was ultimately diagnosed based on intraoperative findings and postoperative histopathology. The tumor was totally resected and 25 doses of adjuvant radiotherapy with planning gross tumor volume (60 Gy) and planning clinical target volume (50 Gy) were administered for 5 weeks. The patient was discharged at 12 days post-surgery with no obvious postoperative complications. Over the 28-month follow-up period, there was no evidence of recurrence, which may be due to the successful use of combined gross total resection and adjuvant radiotherapy. Therefore, surgical resection using an endoscopic transsphenoidal approach and postoperative adjuvant radiotherapy is an effective method for treating intracranial clivus chondrosarcoma.
... 1,2 An image-guided biopsy is generally warranted for higher-grade or suspected metastatic lesions. 2 A biopsy may also be indicated when the lesion poses a treatment dilemma (e.g., observation versus radiation versus surgery) or when the patient is not a good surgical candidate. ...
Lesions within the skull base are the most challenging targets for percutaneous biopsy due to the likelihood of encountering a critical structure along any needle trajectory. Due to ICA proximity, the petrous apex is considered unsafe. We describe a novel percutaneous CT-guided approach for biopsying a petrous apex lesion via a contralateral mandibular condylar notch (subzygomatic approach). To our best knowledge, this approach has not been reported and can be safely employed with thorough planning.
... Chordoma is characterized by an aggressive nature and has a high risk of repeated recurrence despite multimodal treatments, including extensive surgical resection and high-dose radiotherapy [4]. In contrast, chondrosarcoma shows a benign clinical phenotype, with a high vulnerability to radiotherapy leading to a better prognosis than chordoma [5]. While the 10-year overall survival (OS) of chordoma is 32.3% [6], that of chondrosarcoma ranges from 89.9% to 100% [7]. ...
Chordoma and chondrosarcoma share common radiographic characteristics yet are distinct clinically. A radiomic machine learning model differentiating these tumors preoperatively would help plan surgery. MR images were acquired from 57 consecutive patients with chordoma (N = 32) or chondrosarcoma (N = 25) treated at the University of Tokyo Hospital between September 2012 and February 2020. Preoperative T1-weighted images with gadolinium enhancement (GdT1) and T2-weighted images were analyzed. Datasets from the first 47 cases were used for model creation, and those from the subsequent 10 cases were used for validation. Feature extraction was performed semi-automatically, and 2438 features were obtained per image sequence. Machine learning models with logistic regression and a support vector machine were created. The model with the highest accuracy incorporated seven features extracted from GdT1 in the logistic regression. The average area under the curve was 0.93 ± 0.06, and accuracy was 0.90 (9/10) in the validation dataset. The same validation dataset was assessed by 20 board-certified neurosurgeons. Diagnostic accuracy ranged from 0.50 to 0.80 (median 0.60, 95% confidence interval 0.60 ± 0.06%), which was inferior to that of the machine learning model (p = 0.03), although there are some limitations, such as the risk of overfitting and the lack of an extramural cohort for truly independent final validation. In summary, we created a novel MRI-based machine learning model to differentiate skull base chordoma and chondrosarcoma from multiparametric signatures.
... Cluster #1, #3 and #7 were the studies investigating the treatments of CS on different anatomical locations. We could learn from the 3 clusters labels that CS is commonly originate from these regions [51][52][53][54][55]. The common keywords in these 3 clusters were chemotherapy, radiotherapy, surgery, resection and follow-up, meaning that these studies were concentrating on the clinical e cacy and safety of preexisting treatments, and the prognosis of patients with CS. ...
Background The Chondrosarcoma (CS) is the second most frequent common malignant bone tumor, with variety in terms of histopathology, clinical behavior and prognostic outcome. Although the exploration of this topics is extensive, the management for advanced CS is still exceptional challenging. A comprehensive understanding of the profile of the research status and determination of novel directions is of urgent need.
Methods The Web of Science Core Collection (WOSCC) dataset was queried to collect all relevant publications related to CS on March 1, 2022. Bibliometric analyses of the acquired data were conducted with CiteSpace and VOSviewer software on these publications, to evaluate the collaborations among different countries, institutions and authors, as well as to explore the current hotspots and future directions in this field.
Results Ultimately, we identified and analyzed a total of 4181 records in English. There was a noticeable increasing annual trend in publications since 1970s. The United States contributed to almost half of the records included, with a leading position in this field. The universities and institutes from United States, European and Asian countries such as Leiden University, Harvard University, China Medical University, Mayo Clinic and Memory Sloan Kettering Cancer Center were the biggest nodes in every cluster of the collaboration network. Active collaborations among the countries were observed. As for the productive authors, Tang, Chih-Hsin featured the highest number of publications among all authors (91 documents), while Hascall, Vc had the highest average citations (79.35 times). Keywords co-occurrence analysis divided the CS studies into 9 clusters: human CS cell, skull base, p53 expression, long bone, cartilage matrix, chondroid chordoma, endoplasmic tissue stress, pelvic sarcoma and connective tissue growth factor. Together with the citation burst of references, the cluster analysis of keywords co-occurrence suggested that the basic studies on human CS cells to investigate the mutant genes, related signaling pathways and novel alternative modalities to treat refractory CS is an upsurge of research with great significance.
Conclusions The present bibliometric study summarizes the clinical research of CS on study type, most prolific countries, institutions, authors, keywords co-occurrence and references with citation burst. These findings may assist the new scholars to better understand the current status and research trends to guide future practice and directions.
... As described by Feuvret et al. [36], primary SBCs may also invade the anterior fossa, mostly causing vision impairment and hypopituitarism by directly compressing the optic apparatus or the pituitary stalk. However, such symptoms are non-specific and may also occur in patients with skull base chordomas and other sarcomas [8,56,57]. Likewise, radiological features of chordomas and SBCs may overlap, with similar localization and bone destructive patterns [58]. ...
... In view of the complex anatomy of primary SBCs, management strategies require multidisciplinary approaches encompassing neurosurgery, otolaryngology, maxillofacial surgery, ophthalmology, and radiation oncology [5,56,59]. A stand-alone biopsy is rarely pursued, carrying surgical risks comparable to resection whilst providing limited clinical benefit [23,24,36,39]. ...
... Tumor resection is preferred, having both a diagnostic and therapeutic role. As described for skull base chordomas and osteosarcomas, the selection of the best surgical approach depends on the tumor's bony epicenter and extension, aimed at safely exposing the lesion and the involved cranial nerves [8,56,57,60,61]. In our cohort, the endoscopic transnasal and the open frontotemporal-orbitozygomatic approaches were the most common, better addressing tumors involving the petrous apex and upper clivus and anteriorly invading the cavernous sinuses [35,39,40,43]. ...
Background:
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.
Methods:
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.
Results:
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%.
Conclusion:
Surgical-resection and radiotherapy are effective treatments in primary SBCs, with acceptable complication rates and favorable local tumor control.
... 17 Despite the advances in imaging and diagnostic techniques, surgical techniques that incorporate oncologic principles, conformal radiation facilities, potential targeted systemic therapies, and early access to coordinated multidisciplinary team, extensive skull base chondrosarcoma, especially in cases of intracranial extension portends worse prognosis and lower survival rates. 18,19 ...
In children and adolescents presenting with skull base sarcoma, treatment strategies will face challenging decisions due to the unique chemoresistant pathologies, limitations imposed by the not‐yet fully mature anatomical structures, and the small surgical site. In children and adolescents presenting with skull base sarcoma, treatment strategies will face challenging decisions due to the unique chemoresistant pathologies, limitations imposed by the not‐yet fully mature anatomical structures, and the small surgical site.
... Within the present series, two thirds of patients (20 of 30; 67%%) with locally recurrent disease after treatment either did not attempt salvage (8 of 30; 27%) or were unsuccessful (12 of 30; 40%) without further disease progression to date. It is clear that more innovative options are still needed for this population [20,21]. ...
... Although multimodality therapy incorporating upfront chemotherapy, radiotherapy, and surgery for poorly differentiated tumors may improve oncologic disease control, that issue was not assessed by the present series [32]. Data analyzing recurrent and metastatic disease are beginning to emerge, and, given the poor prognosis with tumor recurrence, future research should help determine which agents are the most efficacious for the development of novel agents [20,21]. ...
Purpose
To evaluate the effectiveness of external-beam proton therapy (PT) on local control and survival in patients with skull-base chordoma.
Materials and Methods
We reviewed the medical records of patients with skull-base chordoma treated with definitive or adjuvant high-dose PT and updated their follow-up when feasible. We assessed overall survival, disease-specific survival, local control, and freedom from distant metastasis. Radiotherapy toxicities were scored using the Common Terminology Criteria for Adverse Events, version 4.0.
Results
A total 112 patients were analyzed, of whom 105 (94%) received PT and 7 (6%) received combined proton-photon therapy between 2007 and 2019. Eighty-seven patients (78%) underwent a subtotal resection, 22 (20%) a gross total resection, and 3 (3%) a biopsy alone. The median radiotherapy dose was 73.8 Gy radiobiologic equivalent (GyRBE; range, 69.6-74.4). Ninety patients (80%) had gross disease at radiotherapy and 7 (6%) were treated for locally recurrent disease following surgery. Median follow-up was 4.4 years (range, 0.4-12.6); for living patients, it was 4.6 years (range, 0.4-12.6), and for deceased patients, 4.1 years (range, 1.2-11.2). At 5 years after radiotherapy, the actuarial overall survival, disease-specific survival, local control, and freedom from distant metastasis rates were 78% (n = 87), 83% (n = 93), 74% (n = 83), and 99% (n = 111), respectively. The median time to local progression was 2.4 years (range, 0.8-7). Local control and disease-specific survival by resection status was 95% versus 70% (P = 0.28) and 100% versus 80% (P = 0.06) for gross total, versus subtotal, resection or biopsy alone, respectively. There were no serious acute toxicities (grade ≥ 3) related to radiotherapy.
Conclusion
High-dose PT alone or after surgical resection for skull-base chordoma reaffirms the favorable 5-year actuarial local control rate compared with conventional techniques with acceptable late-complication–free survival. Outcomes following gross total resection and adjuvant PT were excellent. Further follow-up of this cohort is necessary to better characterize long-term disease control and late toxicities.
... Similarly, between 1979 and 2002, the relative survival rates of bone sarcomas in the United Kingdome increased from 43 % to 59 % in female patients and from 35 % to 58 % in male ones [21]. These findings could be due to the advancement in the treatment modalities of bone sarcomas over the years [26,27], improved diagnostic imaging [25], or modification in disease grading. ...
Introduction
Chondrosarcomas are extremely rare, locally invasive, and potentially mortal malignant cartilaginous tumors. In this study, we aimed to evaluate the incidence and survival rates and trends of skull base chondrosarcomas (SBC).
Methods
Data from SBC patients between 1975 and 2017 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. The age-adjusted incidence rates (AAR) were calculated for the overall cases and based on gender, age, race, and histology. Furthermore, the relative survival rates for one, three, and five years, and the rates stratified to the aforementioned selected variables were computed. Besides, we conducted a joint point regression analysis to calculate the annual percent change (APC) and its associated standard error (SE) for AAR and mortality.
Results
The AAR rate of SBC was 0.019 per 100,000. Higher AAR rates were observed in patients who were in the 65-74-year-age-group, females, Caucasians, and had none mesenchymal subtype. The relative one-year, three-year and five-year-survival rates were 99.58%, 93.67%, and 89.10%, respectively. Lower survival rates were noted in patients who were males, African Americans, and had a mesenchymal subtype. The trend analysis has shown a significant yearly increase (P < 0.001) in AAR of SBC (APC ± SE = 0.0005%±0.0001), along with a significant yearly decline in mortality rates (APC ± SE= -0.0202%±0.0029).
Conclusion
Despite the increase in AAR over time, there has been a significant decline in mortality rates over time, which might have been due to the advancement of treatment modalities, improvement in diagnostic imaging, and modification in disease grading.
Objective:
To investigate prognostic factors in patients with primary skull base chordoma (PSBC) to guide future therapeutic advances.
Methods:
This retrospective cohort study of 94 PSBC patients was conducted in 2 institutions from January 2006 to December 2013. Independent predictors for progression-free survival (PFS) and overall survival were established with multivariate Cox regression analysis.
Results:
Age (P = 0.006), extent of resection (P = 0.037), and radiotherapy (RT) (P = 0.027) were established as independent predictors for PFS in PSBC patients. Similarly, age (P = 0.002), extent of resection (P = 0.048), and RT (P = 0.015) were established as independent predictors for overall survival. Meta-analysis manifested that lower MIB-1 correlated with longer PFS in skull base chordoma patients (P < 0.001). RT doubled the 5-year PFS rate from 28.6 ± 12.1% to 61.6 ± 10.7% (P = 0.031) and increased the 5-year overall survival rate from 54.5 ± 13.8% to 84.2 ± 8.4% (P = 0.020) in the subtotal resection/partial resection and MIB-1 labeling index (STR/PR+MIB-1 LI) <2% subgroup. In contrast, in the STR/PR+MIB-1 LI ≥2% subgroup, the survival benefit of RT remained uncertain. Further analysis revealed no survival difference between different RT modalities in STR/PR PSBC patients.
Conclusions:
In PSBC patients, age, extent of resection, and adjuvant RT all are independent predictors for PFS. Lower MIB-1 LI is associated with longer PFS in PSBC patients. Adjuvant RT is necessary for PSBC patients who undergo STR/PR with MIB-1 LI <2%. Patients who undergo GTR or STR/PR with MIB-1 LI ≥2% seem nonresponsive to RT.
Background:
Recurrent skull base chondrosarcomas (CSA) are difficult to treat, and limited data are available to help guide subsequent therapy.
Objective:
To further characterize the natural history of CSA and identify treatment modalities that were most effective in prolonging progression-free (PFS) and disease-specific survival (DSS).
Methods:
We conducted a single-institution retrospective review of patients with recurrent skull base CSA from 1993 to 2021. Kaplan-Meier survival analyses for PFS and DSS were completed. Univariable and multivariable Cox proportional hazards regression models were used to identify patient-related, treatment-related, and disease-related factors that predicted PFS and DSS.
Results:
A total of 28 patients and 84 episodes of recurrence were included. One-year PFS was 70.6%, 5-year PFS was 28.9%, and 10-year DSS was 78.5%. The median time to first progression was 23.9 months (range, 2.8-282 months). In univariable Cox proportional hazards regression, male sex, higher grade histology, fourth or greater progression episode status, distal pattern of recurrence, and treatment of recurrence without surgery or with chemotherapy alone predicted worse PFS. Multivariable regression predicted shortened DSS in male patients (hazard ratio [HR] 0.16; P = .021) and higher-grade tumors (HR 0.22; P = .039). Treatment of recurrence with surgery was associated with, but did not significantly predict, improved DSS (HR 1.78; P = .11).
Conclusion:
Several patient and disease-specific factors were associated with shorter PFS and DSS in recurrent skull base chondrosarcoma. For recurrences amenable to resection, surgery is recommended for treatment of recurrent CSA. Local recurrence management without surgery results in shorter PFS and DSS.
