Original ArticleProposal and Validation of a Basic Progression Scoring System for Patients with Skull Base Chordoma
Introduction
Chordoma is a rare low-grade but local invasive bone malignancy that arises from persistent notochordal elements.1 The annual incidence of chordoma is 0.084 per 100,000 individuals, the prevalence is 0.4 per 100,000, and cranial chordoma represents 32% of the total incidence.2, 3, 4 The extremely low incidence and gradually progressing clinical course of chordoma lead to difficulties in early detection and delayed diagnosis and treatment. Its apparent tendency to recur locally but not metastasize highlights the importance of local control.
To date, the most valued method of treating cranial chordoma is safely and maximally resecting the tumor. Because it is difficult to completely remove the entity because of the limited space and its proximity to vital structures in the skull base, particle beam irradiation, rather than conventional radiotherapy techniques, also plays an important role in tumor control despite of the low-grade evidence.5, 6, 7, 8 No drugs have shown a statistically significant effect on treating chordoma, even though some clinical trials and case reports reported encouraging findings that 44% of advanced tumors could be controlled for more than 16 weeks.9 The median duration of progression-free survival (PFS) in a group of patients was 9 months.10
With a duration of PFS ranging from several months to decades, it is very complicated to predict when a tumor will recur. It is important for researchers to evaluate the effect of drug therapy on the basis of PFS. A reliable endpoint of clinical trial is needed desperately, which highlights the importance of predicting tumor progression. Moreover, once progression is well predicted, follow-ups could be more accurately scheduled to reduce the frequency of imaging and abate the emotional burden of patients.
Many researchers have focused on the prognostic factors of tumor recurrence or progression and made some findings. Despite some controversy, age, sex, resection degree, tumor location, tumor volume, previous treatment history, pathology, and adjuvant radiotherapy have shown statistically significant associations with tumor progression in respective studies.11, 12, 13, 14 No prognostic scale, however, was designed to systematically evaluate the PFS of patients with skull base chordoma. On the basis of the huge population of China and our specialty clinic for cranial tumors, a great number of patients with skull base chordoma have been admitted to our hospital, which gave us a chance to try to fill in this gap.
Section snippets
Patients and Methods
This study was approved by the Institution Review Board of the Beijing Tiantan Hospital affiliated with the Capital Medical University. Inclusion criteria included: 1) patients who received surgery between January 1, 2008, and December 31, 2013; 2) tumor's pathologic type was verified as chordoma by experienced neuropathologists. Exclusion criteria included: 1) patients who died within 3 months after operation; 2) patients without preoperative magnetic resonance imaging (MRI) scans; 3) patients
Results
Among 170 patients, 34 patients were ≤22 years of age, and 136 patients were older than 22 years old (median, 40 years of age; range, 5–67 years). The majority of patients were men (55.9%), and the ratio of men to women was 1.27. The volume of tumor ranged from 2.14 cm3 to 139.14 cm3, with a median volume of 22.61 cm3. The Pre-KPS of 12.9% of patients was less than 70. Among the whole series, 25 patients received adjuvant therapy within 6 months after operation. In the training set, 13 patients
Discussion
In this study, we found that age, treatment history, Pre-KPS, pathology, and MRI features were independent prognostic factors for tumor progression of patients with chordoma. The BPSC was well proposed and validated. Patients in the high-risk group showed the worst tumor control. To the best of our knowledge, this study is the first to analyze so many potential prognostic factors and design a progression scoring system for patients with skull base chordoma.
Conclusions
Age ≥22 years, treatment history, preoperative KPS <70, classical chordoma, and RCE/RT1 ≥1.7 and RT2/RT1 ≤4.3 of MRI features are risk factors for tumor progression of skull base chordoma. The BPSC is valid and reproducible. It can be used to counsel individual patients and screen high-risk patients for clinical trials of drug therapies.
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Natural Growth Dynamics of Untreated Skull Base Chordomas In Vivo
2020, World NeurosurgeryCitation Excerpt :However, this phenomenon requires further study into the pathologic morphology or the Ki-67 index.25 Chordomas have a high incidence of recurrence, and the literature has shown that factors related to the recurrence of skull base chordomas include female gender, older age, greater tumor size, increasing extent of tumor invasion, incomplete resection, presence of metastasis, higher Ki-67 index, and dedifferentiated histologic subtype.6,9-11 The present data showed that, compared with male patients, female patients showed higher growth rates, which was difficult to explain, and perhaps hormone levels did play a role in tumor progression.26
Radiomic analysis of multiparametric magnetic resonance imaging for differentiating skull base chordoma and chondrosarcoma
2019, European Journal of RadiologyCitation Excerpt :We believe that T2WI and CET1 may contain more useful information in differentiating skull base chordoma from chondrosarcoma compared to T1WI. Similarly, previous studies have also demonstrated that T2WI and CET1 were of outstanding value in the prognosis and grading of chordoma [29,30]. We hypothesized that a radiomics method could differentiate these two tumors, which on standard imaging exhibit both a high T2-weighted signal and heterogeneous contrast enhancement on CET1, and our results show that such a radiomics approach was feasible and successful.
Prognosis, survival, and surveillance
2018, Chordomas and Chondrosarcomas of the Skull Base and SpinePrognostic Factors in Skull Base Chordoma: A Systematic Literature Review and Meta-Analysis
2018, World NeurosurgeryCitation Excerpt :Of these, 19 did not investigate SBC prognostic factors; 10 analyzed spinal chordoma and SBC patients simultaneously; and 24 had ≥1 methodologic flaws, including no multivariate analysis and/or without definition of outcome parameters. An eventual 22 papers were included in this systematic review after we excluded these 53 articles.19-40 Of these, 4 did not present sufficient detailed data for statistical pooling, and were further excluded for subsequent meta-analysis.22,23,29,38
Expression of Cathepsin K in Skull Base Chordoma
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Conflict of interest statement: This study was supported in part by the National Natural Science Foundation of China (NSFC; grant no. 81472370 and 81541146), the Natural Science Foundation of Beijing Municipality (Beijing Natural Science Foundation; grant no. 7142052) and Beijing Municipal Science and Technology Commission (grant no. Z131107002213179).
Jun-Peng Ma and Kai-Bing Tian are co–first authors.