Prognostic factors in chordoma: Role of postoperative radiotherapy

doi:10.1016/0959-8049(95)00458-0

European Journal of Cancer

Volume 31, Issues 13–14, December 1995, Pages 2255-2259

https://doi.org/10.1016/0959-8049(95)00458-0 Get rights and content

Abstract

We have investigated prognostic factors for survival in a series of 26 patients with chordoma treated in Lyon, France, between 1979 and 1993. In this series, the median progression-free (PFS) and overall survival (OS) were 10 and 90 months, respectively. In univariate analysis, PFS, but not OS, was found significantly longer in males as compared to females (median: 19 versus 7 months, P = 0.05); and patients under 60 years of age had a longer PFS (median: 18 versus 6 months; P = 0.06) and OS (median: 108 versus 47+, P = 0.05) than older patients. A favourable prognostic subgroup including male patients under 60 years and a poor prognostic group including female patients and male over 60 years were thus defined (median PFS: 36 versus 6 months, P = 0.001; median OS: 108 versus 55+, P = 0.15). Primary treatment combining surgery and postoperative radiotherapy was associated with a longer PFS than surgery only (median: 36 versus 7 months, P = 0.002) in the whole series and in both prognostic subgroups.

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High Expression of TGF-β1 Predicting Tumor Progression in Skull Base Chordomas
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Citation Excerpt :
Women also showed shorter PFS than men.27 In a study of 62 patients with SBCs, female patients showed an increasingly poor prognosis28; an additional 2 studies also support this finding.29,30 Although the present study did not find sex differences of prognosis in SBCs, it did reveal an increased expression of TGF-β1 mRNA in SBCs from women.
To investigate the expression characteristics and prognostic value of transforming growth factor β1 (TGF-β1) in primary skull base chordomas (SBCs).
The mRNA expression levels of TGF-β1 were measured in 57 frozen samples from patients with primary SBCs. Clinical data collection, follow-up, correlations, and survival analyses were performed.
In the series of 57 patients (29 men and 28 women) with primary SBCs, the mean value of TGF-β1 mRNA was 1.713 with a median of 0.904. Twenty-four SBCs were soft type and 33 were hard type. The Mann–Whitney U test revealed that the expression level of TGF-β1 mRNA in hard type SBCs was significantly higher than the expression level found in the soft type (P = 0.03). The independent-samples median test suggested that the expression level of TGF-β1 mRNA in female patients' SBCs was significantly higher than that in male patients' SBCs (P = 0.01). Expression differences of TGF-β1 were not seen among different pathological subtypes, tumor blood supply, or degree of resection. The Spearman rank correlation coefficient clarified that TGF-β1 mRNA levels were not correlated with tumor diameter, preoperative Karnofsky Performance Status (KPS), postoperative KPS, follow-up KPS, age, or intraoperative blood loss. The multivariate Cox analysis revealed that pathological subtype (P = 0.008), expression level of TGF-β1 mRNA (P = 0.01), and tumor texture (P = 0.03) were all independent prognostic factors for tumor progression.
SBCs in female patients and SBCs with hard texture were prone to have high TGF-β1 mRNA expression. High expression of TGF-β1, hard tumor texture, and conventional subtype were all independent risk factors for tumor progression.
Development of Machine Learning Algorithms for Prediction of 5-Year Spinal Chordoma Survival
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Accurately prognosticating survival for patients with this pathology is difficult not only because of the rarity of these tumors, with an incidence of 0.08 per 100,000 persons, but also because few studies have a sufficient duration of patient follow-up to provide meaningful estimates of long-term survival.4 Previous prognostication models in spinal chordoma have used statistical modeling to identify predictive factors significantly associated with survival; however, none have translated these models into open access digital applications, and none have used techniques such as support vector machines or neural networks.4-19 Machine learning is a growing field of statistics and computer science that has provided important insights to industries ranging from advertising to finance and cyber security.20-22
Chordomas are locally invasive slow-growing tumors that are difficult to study because of the rarity of the tumors and the lack of significant volumes of patients with longitudinal follow-up. As such, there are currently no machine learning studies in the chordoma literature. The purpose of this study was to develop machine learning models for survival prediction and deploy them as open access web applications as a proof of concept for machine learning in rare nervous system lesions.
The National Cancer Institute's Surveillance, Epidemiology, and End Results program database was used to identify adult patients diagnosed with spinal chordoma between 1995 and 2010. Four machine learning models were used to predict 5-year survival for spinal chordoma and assessed by discrimination, calibration, and overall performance.
The 5-year overall survival for 265 patients with spinal chordoma was 67.5%. Variables used for prediction were age at diagnosis, tumor size, tumor location, extent of tumor invasion, and extent of surgery. For 5-year survival prediction, the Bayes Point Machine achieved the best performance with a c statistic of 0.80, calibration slope of 1.01, calibration intercept of 0.03, and Brier score of 0.16. This model for 5-year mortality prediction was incorporated into an open access application and can be found online (https://sorg-apps.shinyapps.io/chordoma/).
This analysis of patients with spinal chordoma demonstrated that machine learning models can be developed for survival prediction in rare pathologies and have the potential to serve as the basis for creation of decision support tools in the future.
Differences in Dural Penetration of Clival Chordomas Are Associated with Different Prognosis and Expression of Platelet-Derived Growth Factor Receptor-β
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It is still controversial whether age is a risk factor for poor prognosis. Some studies have reported age <40 years,12 >50 years,13 or >60 years14 to be risk factors for shorter OS. In our study, age was significantly correlated with OS and we found that patients <55 years old had longer OS.
We sought to compare the prognosis of clival chordomas with different dural penetration and establish the relationship between dural penetration and platelet-derived growth factor receptor (PDGFR)-β signaling pathway.
Tumors in Type I (33 cases) showed limited dural penetration, while those in Type II (34 cases) had more serious dural penetration. Cox multivariate regression analysis was used to analyze risk factors affecting survival. Kaplan-Meier analysis measured overall survival (OS) and progression-free survival (PFS). To determine the relationship between dural penetration and PDGFR-β signaling, expression of PDGFR-β, Akt, mammalian target of rapamycin (mTOR), and phosphatase and tensin homolog (PTEN) expression was compared using immunohistochemistry, quantitative reverse transcription polymerase chain reaction, and Western blotting.
Total resection was achieved in 9 cases in Type I and 11 in Type II. There were significant correlations between OS and dural penetration (P = 0.032) and age (P = 0.034). PFS correlated significantly with dural penetration (P = 0.022), gender (P = 0.001), and degree of resection (P = 0.001). Mean OS in Type I was significantly longer than in Type II (P = 0.046). Patients aged <55 years had longer OS than those aged ≥55 years (P = 0.004). Total resection was correlated with longer PFS (P = 0.011). Among patients with tumors totally resected, mean PFS in Type I was significantly longer than in Type II (P = 0.007). Expression of PDGFR-β in Type II was higher than in Type I.
Clival chordomas have different degrees of dural penetration. Patients with chordomas with serious dural penetration have poorer prognosis. Higher expression of PDGFR-β is related to more serious dural penetration of clival chordomas.
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2016, Handbook of Neuro-Oncology Neuroimaging: Second Edition
Primary spinal cord tumors (SCT) comprise a diverse group of neoplasms that are often of lower grade and less infiltrative than their counterparts in the brain. The most common SCT are extramedullary and include schwannomas, meningiomas, neurofibromas, sarcomas, dermoids, epidermoids, hemangioblastomas, and chordomas. Intramedullary tumors are less frequently diagnosed and consist mainly of ependymomas and astrocytomas. Surgical resection is the mainstay of treatment for most SCT, and can be curative in some cases. External beam radiation therapy is reserved for recurrent or progressive low-grade and all high-grade SCT. The standard regimen consists of 4500–5500 cGy administered in 180 cGy daily fractions. Chemotherapy should only be considered as adjunctive treatment for selected SCT, especially progressive or recurrent astrocytomas and ependymomas. Molecular therapeutic approaches are under development and will be applied to selected SCT in the near future.
Prognostic factors in spinal chordoma: A systematic review
2015, Clinical Neurology and Neurosurgery
Discovering reliable prognostic factors for spinal chordoma remains a challenge. We attempted to identify evidence-based prognostic factors in the literature since its inception and to establish pooled relative risks (RR) of such factors.
MEDLINE and Embase search (inception to December 2014). Two reviewers independently selected papers involving spinal chordoma prognostic factors, and studied them for methodological quality and valuable new factors. Subsequently, we attempted to pool the results.
Of 1465 citations, we studied 65 papers closely, and found several “new” prognostic factors. However, only eight papers were of adequate quality for analysis. Location in the upper cervical spine (pooled RR = 5.46, 95% confidence interval [CI]: 2.23–13.34), worse preoperative Frankel score (pooled RR = 2.77, 95% CI: 1.73–4.42), intralesional surgery (pooled RR = 2.68, 95% CI: 1.66–4.32), greater extent of invasion (pooled RR = 5.09, 95% CI: 1.49–17.41), and revision surgery (pooled RR = 2.42, 95% CI: 1.34–4.36) appeared to be independent factors for worse outcome.
Despite the wealth of literature available, disappointingly few papers are of sufficient quality for drawing valid conclusions related to spinal chordoma prognostic factors. The heterogeneity of the studies renders results pooling almost impossible. More accurate individual prognostication requires methodologically high-quality studies with more uniform study design and data reporting.

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Original paperPrognostic factors in chordoma: Role of postoperative radiotherapy

Abstract

Int J Radiat Oncol Biol Phys

Int J Radiat Oncol Biol Phys

Les tumeurs primitives durachis. Aspect oncologique inital: épidémiologie, classification anatomoprognostique et thérapeuticque

Neurochirurgie

Retrospective long-term follow-up analysis in 21 patients with chordomas of various sites treated at a single institution

J Neurosurg

Sarcoma of bone

Clinocopathologic study of sacrococcygeal chordoma

Cancer

Clinical and pathologic review of 48 cases of chordoma

Cancer

Spinal chordomas

J Neurosurg

Original paper
Prognostic factors in chordoma: Role of postoperative radiotherapy