En Bloc Resection in Combination with High Dose Radiation Improves Patient Survival in Mobile Spine Chordoma

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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Spinal stereotactic body radiotherapy following intralesional curettage with separation surgery for initial or salvage chordoma treatment

Neurosurgical FOCUS ◽

10.3171/2016.9.focus16373 ◽

2017 ◽

Vol 42 (1) ◽

pp. E4 ◽

Cited By ~ 15

Author(s):

Dennis T. Lockney ◽

Timothy Shub ◽

Benjamin Hopkins ◽

Natalie A. Lockney ◽

Nelson Moussazadeh ◽

...

Keyword(s):

Local Control ◽

Salvage Therapy ◽

En Bloc Resection ◽

Bloc Resection ◽

Local Control Rate ◽

High Dose ◽

En Bloc ◽

Intralesional Curettage ◽

Mobile Spine

OBJECTIVE Chordoma is a rare malignant tumor for which en bloc resection with wide margins is advocated as primary treatment. Unfortunately, due to anatomical constraints, en bloc resection to achieve wide or marginal margins is not feasible for many patients as the resulting morbidity would be prohibitive. The objective of this study was to evaluate the efficacy of intralesional curettage and separation surgery followed by spinal stereotactic body radiation therapy (SBRT) in patients with chordomas in the mobile spine. METHODS The authors performed a retrospective chart review of all patients with chordoma in the mobile spine treated from 2004 to 2016. Patients were identified from a prospectively collected database. Initially 22 patients were identified with mobile spine chordomas. With inclusion criteria of cytoreductive separation surgery followed closely by SBRT and a minimum of 6 months of follow-up imaging, 12 patients were included. Clinical and pathological characteristics of each patient were collected and data were analyzed. Patients were divided into two cohorts—those undergoing intralesional resection followed by SBRT as initial chordoma treatment at Memorial Sloan Kettering Cancer Center (MSKCC) (Cohort 1) and those undergoing salvage treatment following recurrence (Cohort 2). Treatment toxicities were classified according to the Common Terminology Criteria for Adverse Events version 4.03. Overall survival was analyzed using Kaplan-Meier analysis. RESULTS The 12 patients had a median post-SBRT follow-up time of 26 months. Cohort 1 had 5 patients with median post-SBRT follow-up time of 65.9 months and local control rate of 80% at last follow-up. Only one patient had disease progression, at 48.2 months following surgery and SBRT. Cohort 2 had 7 patients who had been treated at other institutions prior to undergoing both surgery and SBRT (salvage therapy) at MSKCC. The local control rate was 57.1% and the median follow-up duration was 10.7 months. One patient required repeat irradiation. Major surgery- and radiation-related complications occurred in 18% and 27% of patients, respectively. Epidural spinal cord compression scores were collected for each patient pre- and postoperatively. CONCLUSIONS The combination of surgery and SBRT provides excellent local control following intralesional curettage and separation surgery for chordomas in the mobile spine. Patients who underwent intralesional curettage and spinal SBRT as initial treatment had better disease control than those undergoing salvage therapy. High-dose radiotherapy may offer several biological benefits for tumor control.

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Long-term outcomes of high-dose single-fraction radiosurgery for chordomas of the spine and sacrum

Journal of Neurosurgery Spine ◽

10.3171/2019.7.spine19515 ◽

2020 ◽

Vol 32 (1) ◽

pp. 79-88 ◽

Cited By ~ 2

Author(s):

Chunzi Jenny Jin ◽

John Berry-Candelario ◽

Anne S. Reiner ◽

Ilya Laufer ◽

Daniel S. Higginson ◽

...

Keyword(s):

Local Recurrence ◽

En Bloc Resection ◽

Bloc Resection ◽

High Dose ◽

En Bloc ◽

Single Fraction ◽

Mobile Spine ◽

Symptom Response ◽

Clinical Records

OBJECTIVEThe current treatment of chordomas is associated with significant morbidity, high rates of local recurrence, and the potential for metastases. Stereotactic radiosurgery (SRS) as a primary treatment could reduce the need for en bloc resection to achieve wide or marginal margins. Spinal SRS outcomes support the exploration of SRS’s role in the durable control of these conventionally radioresistant tumors. The goal of the study was to evaluate outcomes of patients with primary chordomas treated with spinal SRS alone or in combination with surgery.METHODSClinical records were reviewed for outcomes of patients with primary chordomas 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 the extent of surgery.RESULTSIn total, 35 patients with de novo chordomas of the mobile spine (n = 17) and sacrum (n = 18) received SRS and had a median post-SRS follow-up duration of 38.8 months (range 2.0–122.9 months). The median planning target volume dose was a 24-Gy single fraction (range 18–24 Gy). Overall, 12 patients (34%) underwent definitive SRS and 23 patients (66%) underwent surgery and either neoadjuvant or postoperative adjuvant SRS. Definitive SRS was selectively used to treat both sacral (n = 7) and mobile spine (n = 5) chordomas. Surgical strategies for the mobile spine were either intralesional, gross-total resection (n = 5) or separation surgery (n = 7) and for the sacrum en bloc sacrectomy (n = 11). The 3- and 5-year LRFS rates were 86.2% and 80.5%, respectively. Among 32 patients (91%) receiving 24-Gy radiation doses, the 3- and 5-year LRFS rates were 96.3% and 89.9%, respectively. The 3- and 5-year OS rates were 90.0% and 84.3%, respectively. The symptom response rate to treatment was 88% for pain and radiculopathy. The extent or type of surgery was not associated with LRFS, OS, or symptom response rates (p > 0.05), but en bloc resection was associated with higher surgical toxicity, as measured using the Common Terminology Criteria for Adverse Events (version 5.0) classification tool, than epidural decompression and curettage/intralesional resection (p = 0.03). The long-term rate of toxicity ≥ grade 2 was 31%, including 20% grade 3 tissue necrosis, recurrent laryngeal nerve palsy, myelopathy, fracture, and secondary malignancy.CONCLUSIONSHigh-dose spinal SRS offers the chance for durable radiological control and effective symptom relief with acceptable toxicity in patients with primary chordomas as either a definitive or adjuvant therapy.

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High-dose hypofractionated stereotactic body radiotherapy for spinal chordoma

Journal of Neurosurgery Spine ◽

10.3171/2021.2.spine202199 ◽

2021 ◽

pp. 1-10

Author(s):

Xuguang Chen ◽

Sheng-Fu L. Lo ◽

Chetan Bettegowda ◽

Daniel M. Ryan ◽

John M. Gross ◽

...

Keyword(s):

Stereotactic Body Radiotherapy ◽

En Bloc Resection ◽

Bloc Resection ◽

Surgical Margins ◽

High Dose ◽

Newly Diagnosed ◽

En Bloc ◽

Spinal Chordoma ◽

Mobile Spine

OBJECTIVE Spinal chordoma is locally aggressive and has a high rate of recurrence, even after en bloc resection. Conventionally fractionated adjuvant radiation leads to suboptimal tumor control, and data regarding hypofractionated regimens are limited. The authors hypothesized that neoadjuvant stereotactic body radiotherapy (SBRT) may overcome its intrinsic radioresistance, improve surgical margins, and allow preservation of critical structures during surgery. The purpose of this study is to review the feasibility and early outcomes of high-dose hypofractionated SBRT, with a focus on neoadjuvant SBRT. METHODS Electronic medical records of patients with spinal chordoma treated using image-guided SBRT between 2009 and 2019 at a single institution were retrospectively reviewed. RESULTS Twenty-eight patients with 30 discrete lesions (24 in the mobile spine) were included. The median follow-up duration was 20.8 months (range 2.3–126.3 months). The median SBRT dose was 40 Gy (range 15–50 Gy) in 5 fractions (range 1–5 fractions). Seventeen patients (74% of those with newly diagnosed lesions) received neoadjuvant SBRT, of whom 15 (88%) underwent planned en bloc resection, all with negative margins. Two patients (12%) developed surgical wound-related complications after neoadjuvant SBRT and surgery, and 4 (two grade 3 and two grade 2) experienced postoperative complications unrelated to the surgical site. Of the remaining patients with newly diagnosed lesions, 5 received adjuvant SBRT for positive or close surgical margins, and 1 received SBRT alone. Seven recurrent lesions were treated with SBRT alone, including 2 after failure of prior conventional radiation. The 2-year overall survival rate was 92% (95% confidence interval [CI] 71%–98%). Patients with newly diagnosed chordoma had longer median survival (not reached) than those with recurrent lesions (27.7 months, p = 0.006). The 2-year local control rate was 96% (95% CI 74%–99%). Among patients with radiotherapy-naïve lesions, no local recurrence was observed with a biologically effective dose ≥ 140 Gy, maximum dose of the planning target volume (PTV) ≥ 47 Gy, mean dose of the PTV ≥ 39 Gy, or minimum dose to 80% of the PTV ≥ 36 Gy (5-fraction equivalent doses). All acute toxicities from SBRT were grade 1–2, and no myelopathy was observed. CONCLUSIONS Neoadjuvant high-dose, hypofractionated SBRT for spinal chordoma is safe and does not increase surgical morbidities. Early outcomes at 2 years are promising, although long-term follow-up is pending.

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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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Quality of life after en bloc resection of tumors in the mobile spine

The Spine Journal ◽

10.1016/j.spinee.2015.03.042 ◽

2015 ◽

Vol 15 (8) ◽

pp. 1728-1737 ◽

Cited By ~ 20

Author(s):

Matthew W. Colman ◽

Syed M. Karim ◽

Santiago A. Lozano-Calderon ◽

Frank X. Pedlow ◽

Kevin A. Raskin ◽

...

Keyword(s):

Quality Of Life ◽

En Bloc Resection ◽

Bloc Resection ◽

En Bloc ◽

Mobile Spine

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Outcomes following attempted en bloc resection of cervical chordomas in the C-1 and C-2 region versus the subaxial region: a multiinstitutional experience

Journal of Neurosurgery Spine ◽

10.3171/2014.5.spine121045 ◽

2014 ◽

Vol 21 (3) ◽

pp. 348-356 ◽

Cited By ~ 26

Author(s):

Camilo A. Molina ◽

Christopher P. Ames ◽

Dean Chou ◽

Laurence D. Rhines ◽

Patrick C. Hsieh ◽

...

Keyword(s):

Cervical Spine ◽

Median Survival ◽

Tumor Recurrence ◽

Demographic Data ◽

En Bloc Resection ◽

Bloc Resection ◽

Upper Cervical Spine ◽

En Bloc ◽

Significant Difference ◽

Mobile Spine

Object Chordomas involving the mobile spine are ideally managed via en bloc resection with reconstruction to optimize local control and possibly offer cure. In the cervical spine, local anatomy poses unique challenges, limiting the feasibility of aggressive resection. The authors present a multi-institutional series of 16 cases of cervical chordomas removed en bloc. Particular attention was paid to clinical outcome, complications, and recurrence. In addition, outcomes were assessed according to position of tumor at the C1–2 level versus the subaxial (SA) spine (C3–7). Methods The authors reviewed cases involving patients who underwent en bloc resection of cervical chordoma at 4 large spine centers. Patients were included if the lesion epicenter involved the C-1 to C-7 vertebral bodies. Demographic data and details of surgery, follow-up course, exposure to adjuvant therapy, and complications were obtained. Outcome was correlated with presence of tumor in C1–2 versus subaxial spine via a Student t-test. Results Sixteen patients were identified (mean age at presentation 55 ± 14 years). Seven cases (44%) cases involved C1–2, and 16 involved the subaxial spine. Median survival did not differ significantly different between the C1–2 (72 months) and SA (60 months) groups (p = 0.65). A combined (staged anteroposterior) approach was used in 81% of the cases. Use of the combined approach was significantly more common in treatment of subaxial than C1–2 tumors (100% vs 57%, p = 0.04). En bloc resection was attempted via an anterior approach in 6% of cases (C1–2: 14.3%; SA: 0%; p = 0.17) and a posterior approach in 13% of cases (C1–2: 29%; SA: 0%; p = 0.09). The most commonly reported margin classification was marginal (56% of cases), followed by violated (25%) and wide (19%). En bloc excision of subaxial tumors was significantly more likely to result in marginal margins than excision of C1–2 tumors (C1–2: 29%; SA: 78%; p = 0.03). C1–2 tumors were associated with significantly higher rates of postoperative complications (C1–2: 71%; SA: 22%; p = 0.03). Both local and distant tumor recurrence was greatest for C1–2 tumors (local C1–2: 29%; local SA: 11%; distant C1–2: 14%; distant SA: 0%). Statistical analysis of tumor recurrence based on tumor location was not possible due to the small number of cases. There was no between-groups difference in exposure to postoperative adjuvant radiotherapy. There was no difference in median survival between groups receiving proton beam radiotherapy or intensity-modulated radiotherapy versus no radiation therapy (p = 0.8). Conclusions Compared with en bloc resection of chordomas involving the subaxial cervical spine, en bloc resection of chordomas involving the upper cervical spine (C1–2) is associated with poorer outcomes, such as less favorable margins, higher rates of complications, and increased tumor recurrence. Data from this cohort do not support a statistically significant difference in survival for patients with C1–2 versus subaxial disease, but larger studies are needed to further study survival differences.

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

Journal of Neurosurgery Spine ◽

10.3171/2015.3.spine14716 ◽

2015 ◽

Vol 23 (6) ◽

pp. 788-797 ◽

Cited By ~ 72

Author(s):

Ronny L. Rotondo ◽

Wendy Folkert ◽

Norbert J. Liebsch ◽

Yen-Lin E. Chen ◽

Frank X. Pedlow ◽

...

Keyword(s):

Radiation Therapy ◽

Prognostic Factors ◽

En Bloc Resection ◽

Bloc Resection ◽

Tumor Control ◽

High Dose ◽

Recurrence Rates ◽

En Bloc ◽

Primary Tumors ◽

Entire Cohort

OBJECT Spinal chordomas can have high local recurrence rates after surgery with or without conventional dose radiation therapy (RT). Treatment outcomes and prognostic factors after high-dose proton-based RT with or without surgery were assessed. METHODS The authors conducted a retrospective review of 126 treated patients (127 lesions) categorized according to disease status (primary vs recurrent), resection (en bloc vs intralesional), margin status, and RT timing. RESULTS Seventy-one sacrococcygeal, 40 lumbar, and 16 thoracic chordomas were analyzed. Mean RT dose was 72.4 GyRBE (relative biological effectiveness). With median follow-up of 41 months, the 5-year overall survival (OS), local control (LC), locoregional control (LRC), and distant control (DC) for the entire cohort were 81%, 62%, 60%, and 77%, respectively. LC for primary chordoma was 68% versus 49% for recurrent lesions (p = 0.058). LC if treated with a component of preoperative RT was 72% versus 54% without this treatment (p = 0.113). Among primary tumors, LC and LRC were higher with preoperative RT, 85% (p = 0.019) and 79% (0.034), respectively, versus 56% and 56% if no preoperative RT was provided. Overall LC was significantly improved with en bloc versus intralesional resection (72% vs 55%, p = 0.016), as was LRC (70% vs 53%, p = 0.035). A trend was noted toward improved LC and LRC for R0/R1 margins and the absence of intralesional procedures. CONCLUSIONS High-dose proton-based RT in the management of spinal chordomas can be effective treatment. In patients undergoing surgery, those with primary chordomas undergoing preoperative RT, en bloc resection, and postoperative RT boost have the highest rate of local tumor control; among 28 patients with primary chordomas who underwent preoperative RT and en bloc resection, no local recurrences were seen. Intralesional and incomplete resections are associated with higher local failure rates and are to be avoided.

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Sacroplasty Augmentation of Instrumented Pelvic Reconstruction After High Sacrectomy: A Technical Case Report

Operative Neurosurgery ◽

10.1093/ons/opab157 ◽

2021 ◽

Author(s):

Amanda N Sacino ◽

Sutipat Pairojboriboon ◽

Ian Suk ◽

Daniel Lubelski ◽

Robin Yang ◽

...

Keyword(s):

Mechanical Stability ◽

Pelvic Ring ◽

Surgical Techniques ◽

Extent Of Resection ◽

En Bloc Resection ◽

Bloc Resection ◽

En Bloc ◽

Mobile Spine ◽

Sacral Tumors ◽

Fibrous Tumor

Abstract BACKGROUND AND IMPORTANCE En bloc resection of sacral tumors is the most effective treatment to help prevent recurrence. Sacrectomy, however, can be destabilizing, depending on the extent of resection. Various surgical techniques for improving stability and enabling early ambulation have been proposed. CLINICAL PRESENTATION Here, we report a case in which we use PMMA (poly[methyl methacrylate]) to augment pelvic instrumentation to improve mechanical stability after sacrectomy for en bloc resection of a solitary fibrous tumor. CONCLUSION We highlight the use of sacroplasty augmentation of pelvic ring reconstruction to provide biomechanical stability without the need for fusion of any mobile spine segments, which allowed for early patient ambulation and no appreciable loss of range of motion or mobility.

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Reconstruction of the anterior craniocervical junction using an expandable cage after resection of a C1 chordoma in a 5-year-old child: case report

Journal of Neurosurgery Pediatrics ◽

10.3171/2019.2.peds18752 ◽

2019 ◽

Vol 24 (1) ◽

pp. 62-65

Author(s):

Lourdes C. Eco ◽

Alison Brayton ◽

William E. Whitehead ◽

Andrew Jea

Keyword(s):

Spinal Instrumentation ◽

En Bloc Resection ◽

Craniocervical Junction ◽

Lateral Approach ◽

Bloc Resection ◽

Benign Tumors ◽

En Bloc ◽

Expandable Cage ◽

Far Lateral Approach ◽

Mobile Spine

Chordomas are histologically benign tumors with local aggressive behavior. They arise from embryological remnants of the notochord at the clivus, mobile spine, and sacrum. Chordomas are rare tumors in the pediatric age group. Their surgical management is difficult, given their propensity for inaccessible anatomical regions, and proximity to critical neurovascular structures. While en bloc resection with surgical margins has been advocated as the preferred approach for chordomas, tumor characteristics and violation of adjacent anatomical boundaries may not allow for safe en bloc resection of the tumor. Here, the authors present the case of a C1 chordoma in a 5-year-old boy with epidural and prevertebral extension. The patient’s treatment consisted of a far-lateral approach for resection of the tumor and C1 arch, followed by circumferential reconstruction of the craniocervical junction with an expandable cage spanning the skull base to C2, and posterior occipitocervical spinal instrumentation. At 42 months after surgery, the patient remains neurologically intact with stable oncological status, and no evidence of craniocervical junction instrumentation failure.

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