Skull Base Chordoma and Chondrosarcoma: Influence of Clinical and Demographic Factors on Prognosis: A SEER Analysis

doi:10.1016/j.wneu.2014.07.005

World Neurosurgery

Volume 82, Issue 5, November 2014, Pages 806-814

https://doi.org/10.1016/j.wneu.2014.07.005 Get rights and content

Objective

Chordomas and chondrosarcomas are rare skull base tumors, with similar radiographic and clinical presentations. We investigated factors influencing long-term survival in these 2 tumors using the Surveillance Epidemiology and End Results (SEER) database.

Methods

Patients with chordoma (n = 416) and chondrosarcoma (n = 269) within the skull base from 1983 to 2009 were identified within the SEER database. Kaplan-Meier curves and Cox proportional hazards models were used to test associations with survival. t tests and χ² tests were used to compare groups.

Results

Chordoma and chondrosarcoma patients were similar demographically. Survival at 5 years was 65% for chordomas and 81.8% (P < 0.0001) for chondrosarcomas and at 10 years was 32.3% and 49.5% (P = 0.004). Multivariate analysis demonstrated chordomas had a worse prognosis even when we controlled for age and tumor size (hazard ratio 3.0, 95% confidence interval 1.9–4.7, P < 0.0001). For chordomas, multivariate analysis demonstrated increasing age and tumor size were significantly associated with reduced survival. For chondrosarcomas, multivariate analysis demonstrated older age, earlier decade of diagnosis, and mesenchymal subtype were significantly associated with reduced survival. Postoperative radiation was given to 42% and 41% of patients with chordomas and chondrosarcomas, respectively. The addition of radiation did not improve survival.

Conclusion

Consistent with previous case series, skull base chordomas have significantly worse prognosis than chondrosarcomas. Patients in the SEER database had worse survival overall compared with existing case series for both chordomas and chondrosarcomas, suggesting selection bias in the existing literature.

Introduction

Chordomas and chondrosarcomas are rare skull base tumors with a combined incidence of 0.02/100,000 a year, with similar radiographic and clinical presentations (32). Although rare, these tumors comprise a large proportion of skull base tumors and are locally invasive (4). Chondrosarcomas are thought to carry a better prognosis; however, data for outcomes of both tumors are varied, and comparative natural history data also are limited 1, 4, 9, 18, 23. The rarity of these skull base tumors limits understanding of the natural history of these tumors and hinders prospective study of antitumoral therapies.

Although the 2 have not been compared in rigorous studies, chordomas of the skull base generally are reported to have worse prognosis than chondrosarcomas 4, 18. For both tumors surgical resection remains the accepted primary treatment, and there are many reports that maximizing the extent of resection reduces recurrence risk and improves survival 1, 18, 23, 28. Postoperative radiation frequently is used in these tumors, particularly in cases of incomplete resection, and has been reported to reduce the risk of recurrence 5, 16. No randomized trials of radiation therapy in skull base chordomas or chondrosarcomas have been completed, and the variability in patterns of care in single-center series presents a challenge for meta-analytic methods in determining the impact of postoperative radiation therapy on outcome in these rare and frequently slow-growing tumors.

The Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute is a population-based registry that contains tumor data for approximately 10%–26% of the U.S. population. The goal of this study was to better understand the clinical and demographic factors influencing survival for skull base chordomas and chondrosarcomas by use of the SEER database to enable more informed clinical management of these lesions and to identify knowledge gaps to guide future trial design.

Section snippets

Patient Selection

Patients with a diagnosis of chordoma and chondrosarcoma were identified using the SEER database. Patients from the 1983–1988, 1988–2003, and 2004–2009 data sets were screened for a diagnosis of chordoma, chondroid chordoma, dedifferentiated chordoma, chondrosarcoma, chondroblastoma, myxoid chondrosarcoma, mesenchymal chondrosarcoma, and clear cell chondrosarcoma including histology codes 9220–9242 and 9370–9372. Chordomas and chondrosarcomas involving the skull (C41.0), brainstem (C71.7),

Results

A total of 685 patients were included in the study; 416 patients had chordomas and 269 had chondrosarcomas. Chordoma and chondrosarcoma patients were similar with regard to age, race, and sex (Table 1). Chondrosarcomas were more likely to be larger; 68.9% of chondrosarcomas were ≥4 cm at presentation compared with only 41.2% of chordomas (P < 0.0001). The rate of radical resection was slightly greater for patients with chondrosarcoma (36.8%) than those with chordoma (29%), although this

Discussion

Recent systematic reviews of the literature estimate the overall 5-year survival for patients with skull base chondrosarcoma to be 89% and progression-free survival 80% 4, 5. These rates compare favorably to the 5-year rates reported in a recent meta-analysis by Di Maio et al. (18) of patients with skull base chordoma of 78% overall survival and 51% progression-free survival. Surgery remains the initial treatment of choice for patients with skull base chordomas and chondrosarcomas. Di Maio

Conclusion

A study of the SEER data for patients with skull base chordomas and chondrosarcomas confirms the improved survival of patients with chondrosarcomas, despite the similarities in clinical presentation and radiographic appearance of these 2 tumors. In chondrosarcomas, older age, earlier decade of diagnosis, and mesenchymal subtype were associated with worse overall survival. In chordomas, older age and larger tumors were associated with worse prognosis. In both tumor types, survival was markedly

References (32)

M. Alonso-Basanta et al.
Proton beam therapy in skull base pathology
Otolaryngol Clin North Am
(2011)
O.G. Bloch et al.
A systematic review of intracranial chondrosarcoma and survival
J Clin Neurosci
(2009)
J.S. Hauptman et al.
Challenges in linear accelerator radiotherapy for chordomas and chondrosarcomas of the skull base: focus on complications
Int J Radiat Oncol Biol Phys
(2012)
E.B. Hug et al.
Proton radiation therapy for chordomas and chondrosarcomas of the skull base
Neurosurg Clin N Am
(2000)
D. Schulz-Ertner et al.
Effectiveness of carbon ion radiotherapy in the treatment of skull-base chordomas
Int J Radiat Oncol Biol Phys
(2007)
D. Schulz-Ertner et al.
Carbon ion radiotherapy of skull base chondrosarcomas
Int J Radiat Oncol Biol Phys
(2007)
B.P. Walcott et al.
Chordoma: current concepts, management, and future directions
Lancet Oncol
(2012)
D.C. Weber et al.
Results of spot-scanning proton radiation therapy for chordoma and chondrosarcoma of the skull base: the Paul Scherrer Institut experience
Int J Radiat Oncol Biol Phys
(2005)
K. Almefty et al.
Chordoma and chondrosarcoma: similar, but quite different, skull base tumors
Cancer
(2007)
M. Amichetti et al.
Proton therapy in chordoma of the base of the skull: a systematic review
Neurosurg Rev
(2009)

O.G. Bloch et al.

Cranial chondrosarcoma and recurrence

Skull Base

(2010)

M. Brada et al.

Current clinical evidence for proton therapy

Cancer J

(2009)

D.M. Bugoci et al.

Photon-based fractionated stereotactic radiotherapy for postoperative treatment of skull base chordomas

Am J Clin Oncol

(2012)

P.A. Forsyth et al.

Intracranial chordomas: a clinicopathological and prognostic study of 51 cases

J Neurosurg

(1993)

E. Gay et al.

Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients

Neurosurgery

(1995)

T. Hasegawa et al.

Gamma knife surgery for skull base chordomas and chondrosarcomas

J Neurosurg

(2007)

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Citation Excerpt :
However, most studies had focused solely on 1 tumor type. Skull base chondrosarcomas were thought to result in a better prognosis than chordomas.2,10 However, the survival of patients with chondrosarcoma versus chordoma in other locations has been less well explored.
Chondrosarcoma and chordoma are often grouped together because of their similar anatomic locations, clinical presentations, histopathological and radiological findings, and growth patterns. In the present study, we investigated the clinical and prognostic differences of chondrosarcomas and chordomas of the skull base and spine.
We accessed the Surveillance, Epidemiology, and End Results database to search for patients from 2000 to 2018 with chondrosarcomas and chordomas of the skull base and spine for inclusion in the present study.
We included 1346 and 1536 cases of chondrosarcoma and chordoma for analysis, respectively. Chondrosarcomas of the cranial base and spine were seen in younger patients and were associated with a larger tumor size compared with chordomas. Among the tumors of the skull base, chondrosarcomas were more common in women, with a male predominance found for chordomas. We also observed a male predilection for both spinal chondrosarcomas and chordomas. Distinct metastatic patterns were found for chondrosarcomas versus chordomas, and spinal chondrosarcomas showed a greater risk of distant metastases at presentation compared with spinal chordomas. Cranial base chondrosarcomas were associated with superior outcomes compared with chordomas. However, we demonstrated an opposite survival pattern for spinal chondrosarcomas and chordomas.
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: Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Peer-Review ReportSkull Base Chordoma and Chondrosarcoma: Influence of Clinical and Demographic Factors on Prognosis: A SEER Analysis

Objective

Methods

Results

Conclusion

Introduction

Section snippets

Patient Selection

Results

Discussion

Conclusion

Otolaryngol Clin North Am

J Clin Neurosci

Int J Radiat Oncol Biol Phys

Neurosurg Clin N Am

Int J Radiat Oncol Biol Phys

Int J Radiat Oncol Biol Phys

Lancet Oncol

Int J Radiat Oncol Biol Phys

Chordoma and chondrosarcoma: similar, but quite different, skull base tumors

Cancer

Proton therapy in chordoma of the base of the skull: a systematic review

Neurosurg Rev

Cranial chondrosarcoma and recurrence

Skull Base

Current clinical evidence for proton therapy

Cancer J

Photon-based fractionated stereotactic radiotherapy for postoperative treatment of skull base chordomas

Am J Clin Oncol

Intracranial chordomas: a clinicopathological and prognostic study of 51 cases

J Neurosurg

Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients

Neurosurgery

Gamma knife surgery for skull base chordomas and chondrosarcomas

J Neurosurg

Peer-Review Report
Skull Base Chordoma and Chondrosarcoma: Influence of Clinical and Demographic Factors on Prognosis: A SEER Analysis