High-dose proton-based radiation therapy in the management of spine chordomas: outcomes and clinicopathological prognostic factors

Rotondo, Ronny L.; Folkert, Wendy; Liebsch, Norbert J.; Chen, Yen Lin E.; Pedlow, Frank X.; Schwab, Joseph H.; Rosenberg, Andrew E.; Nielsen, G. Petur; Szymonifka, Jackie; Ferreira, A. R.; Hornicek, Francis J.; DeLaney, Thomas F.

doi:10.3171/2015.3.spine14716

Cited by 139 publications

(126 citation statements)

References 24 publications

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“…Moreover, prior studies have often focused on single, static 5-or 10-year overall survival or disease-free survival determined at the time of diagnosis [26,34,37]. In addition, prognostic factors or risk factors have been analyzed without taking into consideration the possibility of change in prognosis with time [23,34].…”

Section: Introductionmentioning

confidence: 99%

What Are the Conditional Survival and Functional Outcomes After Surgical Treatment of 115 Patients With Sacral Chordoma?

Guo

Yang

et al. 2017

Clinical Orthopaedics &Amp; Related Research

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Background Conditional survival is a measure of prognosis for patients who have already survived for a specific period of time; however, data on conditional survival after sacrectomy in patients with sacral chordoma are lacking. In addition, because sacral tumors are rare and heterogeneous, classifying them in a way that allows physicians to predict functional outcomes after sacrectomy remains a challenge. Questions/purposes (1) What is the overall survival and disease-free survival in patients treated by sacrectomy for chordoma? (2) What is the conditional survival probability and how do prognostic factors change over time in patients undergoing surgical resection for sacral chordoma? (3) What is the local recurrence rate after surgery, how was it treated, and what factors impact on local recurrence? (4) What is the postoperative motor, sensory, bowel, and bladder function by level of resection as determined by using a newly designed scoring method?Methods Between 2003 and 2012, our center treated 122 patients surgically for sacral chordoma. Of those, two died and five were lost before a minimum followup of 1 year was achieved, leaving 115 patients available for analysis in this retrospective study at a mean of 4.9 years (range, 1.3-10.8 years). Basically, single posterior or combined approaches were chosen based on the most cephalad extent of the tumor and resection level was normally at half or one sacral vertebrae above the tumor. The 5-year conditional survival rate was calculated based on Kaplan-Meier survival analysis. The effect of prognostic factors on conditional survival was also explored. A newly designed score method was proposed and adopted in the current study to critically evaluate the functional outcome after resection of the sacrum. Inter-and intraobserver reliability was tested by a preliminary study using kappa statistics and Spearman rank correlation coefficients. Significant interobserver (p\0.01) and intraobserver agreement (j[0.75) were found in nine items between each observer. Results The estimated 5-year overall survival rate was 81% (95% confidence interval [CI], 72%-90%) at diagnosis. The 5-year disease-free survival rate was 52% (95% CI, 43%-63%). The 5-year conditional overall survival decreased with each additional year in the first 4 years (81% at diagnosis versus 60% at the fourth year, p \ 0.0001) and increased slightly in the fifth year. Patients with adequate surgical margins displayed a higher 5-year survival than those with an inadequate margin (86% [95% CI, 76%-95%] versus 67% [95% CI, 48%-85%], p = 0.01) at diagnosis. Conditional survival estimates for patients who received operations elsewhere were lower than that of newly diagnosed patients treated by us at diagnosis (64% [95% CI, 46%-83%] versus 90% [95% CI, 82%-99%], p = 0.012), but with the numbers we had, we could not detect a difference in conditional survival between those treated elsewhere first compared with those initially treated by us at 5 years. The proposed score system for function evaluation was a...

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Section: Introductionmentioning

confidence: 99%

What Are the Conditional Survival and Functional Outcomes After Surgical Treatment of 115 Patients With Sacral Chordoma?

Guo

Yang

et al. 2017

Clinical Orthopaedics &Amp; Related Research

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“…The overall results of the series of Rotondo et al 22 are presented above in the surgical section. With respect to RT, patients received a median dose of 72.4 GyRBE (relative biological effectiveness) as a combination of photons and protons.…”

Section: Resultsmentioning

confidence: 98%

“…Rotondo et al 22 reviewed 126 cases of chordoma of the mobile spine and sacrum of which 32 comprised LR disease. For all patients, the 5-year rate of local control (LC) was higher after radical en bloc excision (72%) than that after intralesional resection (55%) ( P =0.016).…”

Section: Resultsmentioning

confidence: 99%

Management of Locally Recurrent Chordoma of the Mobile Spine and Sacrum

Ailon

Torabi

Fisher

et al. 2016

Spine

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Study Design Systematic review. Objective To determine evidence-based guidelines for the management of locally recurrent spinal chordoma. Summary of Background Data Chordoma of the spine is a low-grade malignant tumor with a strong propensity for local recurrence. Salvage therapy is challenging due to its relentless nature and refractoriness to adjuvant therapies. There are currently no guidelines regarding the best management of recurrent chordoma. Methods We combined the results of a systematic review with expert opinion to address the following research questions: (1) For locally recurrent chordoma of the spine without systemic disease, if surgery is planned, should en bloc resection be attempted if technically feasible with acceptable morbidity? (2) For locally recurrent chordoma without systemic disease, in which wide en bloc excision is not possible, what is the treatment of choice? (2) Should adjuvant or neoadjuvant radiation therapy be used in the treatment of locally recurrent chordoma? Results A total of nine surgical and seven radiation therapy articles met study criteria. Evidence quality was low or very low. Recurrent disease is associated with predominantly poor outcome, regardless of treatment modality. As for primary chor-doma, resection with wide margins appears to confer an advantage with respect to local control, although this effect is attenuated in the setting of relapse. Postoperative radiation therapy likely reduces the rate of further relapse. Conclusion (1) For locally recurrent chordoma of the spine without systemic disease, when surgery is planned, wide en bloc resection should be performed if technically feasible with acceptable morbidity. Strong recommendation, Low Quality of Evidence. (2) For locally recurrent chordoma without systemic disease, in which wide en bloc excision is not possible, partial resection is the treatment of choice. Weak recommendation, Very Low Quality of Evidence. (3) For the treatment of locally recurrent chordoma, high-dose conformal radiation therapy should be administered postoperatively to reduce the risk of further recurrence, and may be considered as a primary therapy. Strong recommendation, Very Low Quality of Evidence.

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“…Another systematic review also concluded that proton therapy improves long-term local control and survival in patients with skull base chordoma 65 . In tumors of the sacrum and spine, surgical resection coupled with proton therapy provided a 5-year overall survival and local control rate of 81% and 62%, respectively 66 . Carbon ion beam appears to be comparable to proton therapy.…”

Section: Treatmentmentioning

confidence: 99%

Recent advances in understanding and managing chordomas

et al. 2016

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Chordomas are rare primary bone tumors arising from embryonic remnants of the notochord. They are slow-growing, locally aggressive, and destructive and typically involve the axial skeleton. Genetic studies have identified several mutations implicated in the pathogenesis of these tumors. Treatment poses a challenge given their insidious progression, degree of local invasion at presentation, and high recurrence rate. They tend to respond poorly to conventional chemotherapy and radiation. This makes radical resection the mainstay of their treatment. Recent advances in targeted chemotherapy and focused particle beam radiation, however, have improved the management and prognosis of these tumors.

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High-dose proton-based radiation therapy in the management of spine chordomas: outcomes and clinicopathological prognostic factors

Cited by 139 publications

References 24 publications

What Are the Conditional Survival and Functional Outcomes After Surgical Treatment of 115 Patients With Sacral Chordoma?

What Are the Conditional Survival and Functional Outcomes After Surgical Treatment of 115 Patients With Sacral Chordoma?

Management of Locally Recurrent Chordoma of the Mobile Spine and Sacrum

Recent advances in understanding and managing chordomas

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