Management of chordoma of the sacrum and mobile spine

Chordoma is a low-grade notochordal tumor of the skull base, mobile spine and sacrum which behaves malignantly and confers a poor prognosis despite indolent growth patterns. These tumors often present late in the disease course, tend to encapsulate adjacent neurovascular anatomy, seed resection cavities, recur locally and respond poorly to radiotherapy and conventional chemotherapy, all of which make chordomas challenging to treat. Extent of surgical resection and adequacy of surgical margins are the most important prognostic factors and thus patients with chordoma should be cared for by a highly experienced, multi-disciplinary surgical team in a quaternary center. Ongoing research into the molecular pathophysiology of chordoma has led to the discovery of several pathways that may serve as potential targets for molecular therapy, including a multitude of receptor tyrosine kinases (e.g., platelet-derived growth factor receptor [PDGFR], epidermal growth factor receptor [EGFR]), downstream cascades (e.g., phosphoinositide 3-kinase [PI3K]/protein kinase B [Akt]/mechanistic target of rapamycin [mTOR]), brachyury—a transcription factor expressed ubiquitously in chordoma but not in other tissues—and the fibroblast growth factor [FGF]/mitogen-activated protein kinase kinase [MEK]/extracellular signal-regulated kinase [ERK] pathway. In this review article, the pathophysiology, diagnosis and modern treatment paradigms of chordoma will be discussed with an emphasis on the ongoing research and advances in the field that may lead to improved outcomes for patients with this challenging disease.

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Imaging Features of Aggressive Giant Cell Tumors of the Mobile Spine: Retrospective Analysis of 101 Patients From Single Center

Global Spine Journal ◽

10.1177/2192568220982280 ◽

2021 ◽

pp. 219256822098228

Author(s):

Bei Yuan ◽

Lihua Zhang ◽

Shaomin Yang ◽

Hanqiang Ouyang ◽

Songbo Han ◽

...

Keyword(s):

Giant Cell ◽

Vertebral Body ◽

Signal Intensity ◽

Imaging Features ◽

Single Center ◽

Giant Cell Tumors ◽

Ct Guided ◽

Cortical Destruction ◽

Mobile Spine ◽

T2 Weighted Images

Study Design: Retrospective study. Objectives: Giant cell tumors (GCTs) of the mobile spine can be locally aggressive. This study described and classified the typical and atypical appearance of aggressive spinal GCTs according to imaging findings to help the imaging diagnosis, especially for patients with rapid neurological deficit that may require emergent surgery without biopsy. Methods: Computed tomography (CT) and magnetic resonance imaging (MRI) scans of patients diagnosed with aggressive spinal GCTs at single center were reviewed. Results: Overall, 101 patients with 100 CT images and 94 MR images were examined. All lesions were osteolytic with cortical destruction; 95 lesions showed epidural extension; 90 were centered in the vertebral body; 82 showed pathological fracture and/or collapse of the vertebral body; 78 had pseudotrabeculation on CT; 80 showed low-to-iso signal intensity or heterogeneous high-signal intensity with cystic areas on the T2-weighted images; 9 showed fluid–fluid level on T2-weighted images; and 61 patients showed marked enhancement on contrast-enhanced CT and/or MRI. Forty-one lesions (40.6%) had at least 1 atypical radiographic feature: 19 involved ≥2 segments; 11 were centered in the posterior neural arch; 10 had a paravertebral mass over 2 segments; 16 showed partial margin sclerosis with partial cortical destruction on CT scans; and 3 showed mineralization within the tumor on CT. Eighty-eight patients underwent CT-guided biopsy with a diagnostic accuracy rate of 94.3%. Conclusions: Spinal GCTs might appear more radiologically atypical, and about 40% of the lesions may have at least 1 atypical feature. CT-guided biopsies are recommended for definitive diagnosis.

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Mobile spine chordoma: results of 166 patients from the AOSpine Knowledge Forum Tumor database

Journal of Neurosurgery Spine ◽

10.3171/2015.7.spine15201 ◽

2016 ◽

Vol 24 (4) ◽

pp. 644-651 ◽

Cited By ~ 30

Author(s):

Ziya L. Gokaslan ◽

Patricia L. Zadnik ◽

Daniel M. Sciubba ◽

Niccole Germscheid ◽

C. Rory Goodwin ◽

...

Keyword(s):

Local Recurrence ◽

Survival Data ◽

Large Scale ◽

Surgical Techniques ◽

En Bloc Resection ◽

Bloc Resection ◽

En Bloc ◽

Increased Risk ◽

Significant Difference ◽

Mobile Spine

OBJECT A chordoma is an indolent primary spinal tumor that has devastating effects on the patient's life. These lesions are chemoresistant, resistant to conventional radiotherapy, and moderately sensitive to proton therapy; however, en bloc resection remains the preferred treatment for optimizing patient outcomes. While multiple small and largely retrospective studies have investigated the outcomes following en bloc resection of chordomas in the sacrum, there have been few large-scale studies on patients with chordomas of the mobile spine. The goal of this study was to review the outcomes of surgically treated patients with mobile spine chordomas at multiple international centers with respect to local recurrence and survival. This multiinstitutional retrospective study collected data between 1988 and 2012 about prognosis-predicting factors, including various clinical characteristics and surgical techniques for mobile spine chordoma. Tumors were classified according to the Enneking principles and analyzed in 2 treatment cohorts: Enneking-appropriate (EA) and Enneking-inappropriate (EI) cohorts. Patients were categorized as EA when the final pathological assessment of the margin matched the Enneking recommendation; otherwise, they were categorized as EI. METHODS Descriptive statistics were used to summarize the data (Student t-test, chi-square, and Fisher exact tests). Recurrence and survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and multivariate Cox proportional hazard modeling. RESULTS A total of 166 patients (55 female and 111 male patients) with mobile spine chordoma were included. The median patient follow-up was 2.6 years (range 1 day to 22.5 years). Fifty-eight (41%) patients were EA and 84 (59%) patients were EI. The type of biopsy (p < 0.001), spinal location (p = 0.018), and if the patient received adjuvant therapy (p < 0.001) were significantly different between the 2 cohorts. Overall, 58 (35%) patients developed local recurrence and 57 (34%) patients died. Median survival was 7.0 years postoperative: 8.4 years postoperative for EA patients and 6.4 years postoperative for EI patients (p = 0.023). The multivariate analysis showed that the EI cohort was significantly associated with an increased risk of local recurrence in comparison with the EA cohort (HR 7.02; 95% CI 2.96–16.6; p < 0.001), although no significant difference in survival was observed. CONCLUSIONS EA resection plays a major role in decreasing the risk for local recurrence in patients with chordoma of the mobile spine.

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Chordoma of the Mobile Spine: Fifty Years of Experience

Spine ◽

10.1097/01.brs.0000200038.30869.27 ◽

2006 ◽

Vol 31 (4) ◽

pp. 493-503 ◽

Cited By ~ 258

Author(s):

Stefano Boriani ◽

Stefano Bandiera ◽

Roberto Biagini ◽

Patrizia Bacchini ◽

Luca Boriani ◽

...

Keyword(s):

Years Of Experience ◽

Mobile Spine

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Management of Locally Recurrent Chordoma of the Mobile Spine and Sacrum

Spine ◽

10.1097/brs.0000000000001812 ◽

2016 ◽

Vol 41 ◽

pp. S193-S198 ◽

Cited By ~ 29

Author(s):

Tamir Ailon ◽

Radmehr Torabi ◽

Charles G. Fisher ◽

Laurence D. Rhines ◽

Michelle J. Clarke ◽

...

Keyword(s):

Mobile Spine ◽

Locally Recurrent

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Surgical Management of Primary and Metastatic Sarcomas of the Mobile Spine 906

Neurosurgery ◽

10.1227/01.neu.0000309956.41884.3a ◽

2006 ◽

Keyword(s):

Surgical Management ◽

Mobile Spine

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Long-Term Outcomes of High-Dose Single-Fraction Radiosurgery for Chordomas of the Spine and Sacrum

Neurosurgery ◽

10.1093/neuros/nyz310_177 ◽

2019 ◽

Vol 66 (Supplement_1) ◽

Author(s):

Jenny Jin ◽

John Berry-Candelabra ◽

Y Josh Yamada ◽

Daniel Higginson ◽

Adam Schmitt ◽

...

Keyword(s):

Local Recurrence ◽

En Bloc Resection ◽

Bloc Resection ◽

High Dose ◽

En Bloc ◽

Intralesional Resection ◽

Single Fraction ◽

Mobile Spine ◽

Symptom Response

Abstract INTRODUCTION To evaluate outcomes of patients with primary chordomas treated with spine stereotactic radiosurgery (SRS) alone or in combination with surgery, drawing from a single-institution database to elucidate treatment options associated with durable radiographic control of these conventionally radioresistant tumors. Chordomas result in significant morbidity, with a high rate of local recurrence and potential for metastases. SRS as a primary treatment could save patients from extensive surgery. Spine SRS outcomes support exploration of its role in the durable control of these conventionally radioresistant tumors. METHODS Clinical records were reviewed for outcomes of patients with primary chordoma of the mobile spine and sacrum who underwent single-fraction SRS between 2006 and 2017. Radiographic local recurrence-free survival (LRFS), overall survival (OS), symptom response, and toxicity were assessed in relation to extent of surgery. RESULTS In total, 35 patients with de novo chordoma of the mobile spine (49%) and sacrum (51%) received SRS with a median post-SRS follow-up of 38.8 mo (range: 2.0-122.9). The median PTV dose was 24Gy (range: 18–24Gy). Overall, 12 patients (33%) underwent definitive SRS and 23 patients (66%) underwent surgery followed by adjuvant SRS. Surgical strategies included separation surgery prior to SRS, curettage/intralesional resection, and en bloc resection in 7, 6, and 10 patients, respectively. The 3- and 5-yr LRFS rates were 86.2% and 80.5% respectively. Among 32 patients receiving 24 Gy (91%), the 3- and 5-yr LRFS were 96.3% and 89.9%. The 3- and 5-yr OS rates were 90.0% and 84.3%. The symptom response rate to treatment was 88% for pain and radiculopathy. Extent or type of surgery was not associated with LRFS, OS, or symptom response rates (P > .05), but en bloc resection was associated with higher CTCAE v. 5.0 surgical toxicity compared to epidural decompression and curettage/intralesional resection (P = .03). The long-term = grade 2 SRS toxicity rate was 31%, including 17% grade 3 tissue necrosis, recurrent laryngeal nerve palsy, fracture, and secondary malignancy. CONCLUSION High-dose spine SRS offers the chance of durable radiographic control and effective symptom relief with acceptable toxicity for primary chordomas as either definitive or adjuvant therapy.

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