Sacral Chordoma Research Articles

CORR Insights®: What are the Conditional Survival and Functional Outcomes After Surgical Treatment of 115 Patients with Sacral Chordoma?

CORR Insights®: What are the Conditional Survival and Functional Outcomes After Surgical Treatment of 115 Patients with Sacral Chordoma?

The Role of Surgical Diagnosis in The Choice of Anesthetic Technique

Spinal anesthesia is a regional anesthetic technique applied by the anesthesiologist, more than a hundred years. The growing popularity of ambulatory surgical procedures have led to more frequent use of spinal anesthesia. This is an anesthetic technique which enhances the satisfaction of the patient and surgeon with this aspect. Chordoma is a rare, slow growing primary malignant tumor of the axial skeleton. It is seen at sacrum and skull base in the spine especiallay sacrococcygeal area (%50). In this paper, we presented a rare case of sacral chordoma up our spinal anesthesia. Perioperative potential risks can be minimized by close monitoring and good preoperative evaluation. Thus; anesthesic technique which even thought as an unappropriate method can be prefered and we believe that diagnosis can remain in the secondary plan in this decision.

EP-1407: Surgical spacer for sacral chordoma carbon ion treatment at CNAO

EP-1407: Surgical spacer for sacral chordoma carbon ion treatment at CNAO

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

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. (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? 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 (κ > 0.75) were found in nine items between each observer. 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 able to distinguish different levels of resection. The overall functional results for the preservation of bilateral S1, S2, and S3 were 40 ± 8%, 60 ± 12%, and 82 ± 11%, respectively. Patients who had preservation of only one S3 nerve root had more severe incontinence (1.99 ± 0.79 versus 2.60 ± 0.63, p = 0.01) and more sensory loss (1.88 ± 0.82 versus 2.31 ± 0.59, p = 0.02) than those patients with preservation of bilateral S3 nerve roots. The 5-year conditional survival for sacral chordoma decreased with each additional year and began to improve after the fourth year. In addition, the effect of the surgical margin and influence of previous surgery on conditional survival were not linear over time. The level of nerve root resections corresponded with the overall function scores according to the proposed scoring method. This information and scoring system should be valuable in discussing outcomes of sacrectomy in patients with chordoma who are considering this operation and serve as the basis for further study. Level III, therapeutic study.

Chordomas, malignant spinal tumors: a 15-year experience

Abstract Objective: Spinal chordomas are rare, locally invasive, malignant neoplasm, representing 5% of all malignant tumors of the skeleton. In the majority of cases the segment involved is the sacrum. This study reviews our experience during the last 15 years at The Spinal Surgery Department of “Bagdasar-Arseni” Clinical Hospital, to determine the effects of various treatment methods on the overall course of this disease process. Methods: A retrospective study was performed, from 2000 to 2015, in which, 31 patients with spinal chordomas were evaluated at our institution. Results: Two thirds of patients were male, with a mean age of 57 years. Patients presented with local pain, radiculopathy and bladder-bowel dysfunction at hospital admission. There were 24 patients with sacral chordomas, 4 with cervical chordomas, 2 with thoracic and one with lumbar chordoma. All patients underwent at least one surgical procedure. The main goal of surgery was to achieve total resection of the tumor. Conclusion: Our study suggests that spinal chordomas are rare tumors with a high risk of tumor recurrence. If total resection is achieved, the risk of recurrence diminishes. If the tumor resection is subtotal, than recurrence appears in all cases. After tumor resection, the surgeon faces the problem of spinal instability that needs solving. Based on these findings, we think that, whenever possible, radical resection should be the treatment of choice for spinal chordomas.

Carbon Ion Radiation Therapy for Unresectable Sacral Chordoma: An Analysis of 188 Cases

To evaluate the results of carbon ion radiation therapy administered to 188 patients with unresectable primary sacral chordomas. One hundred eighty-eight patients were treated with carbon ion radiation therapy at a single institute between 1996 and 2013 and retrospectively analyzed. The median age was 66 years. The highest proximal invasion reached past S2 level in 137 patients. The median clinical target volume was 345 cm(3). One hundred six patients received 67.2 gray equivalents (GyE)/16 fractions (fr), 74 patients received 70.4 GyE/16 fr, 7 patients received 73.6 GyE/16 fr, and 1 patient received 64.0 GyE/16 fr. The median follow-up period was 62 months (range, 6.8-147.5 months). Seventy percent of patients were followed for 5 years or until death. The 5-year local control, overall survival, and disease-free survival rates were 77.2%, 81.1%, and 50.3%, respectively. Forty-one patients had a local recurrence. Sex, tumor volume, level of proximal invasion, and irradiated dose were unrelated to local control. There was grade 3 toxicity of the peripheral nerves in 6 patients and grade 4 toxicity of the skin in 2 patients. Ambulation remained in 97% of patients. Carbon ion radiation therapy was safe and effective for unresectable chordoma and provided good local control and survival while preserving ambulation.

Computer Navigation-aided Resection of Sacral Chordomas.

Background:Resection of sacral chordomas is challenging. The anatomy is complex, and there are often no bony landmarks to guide the resection. Achieving adequate surgical margins is, therefore, difficult, and the recurrence rate is high. Use of computer navigation may allow optimal preoperative planning and improve precision in tumor resection. The purpose of this study was to evaluate the safety and feasibility of computer navigation-aided resection of sacral chordomas.Methods:Between 2007 and 2013, a total of 26 patients with sacral chordoma underwent computer navigation-aided surgery were included and followed for a minimum of 18 months. There were 21 primary cases and 5 recurrent cases, with a mean age of 55.8 years old (range: 35–84 years old). Tumors were located above the level of the S3 neural foramen in 23 patients and below the level of the S3 neural foramen in 3 patients. Three-dimensional images were reconstructed with a computed tomography-based navigation system combined with the magnetic resonance images using the navigation software. Tumors were resected via a posterior approach assisted by the computer navigation. Mean follow-up was 38.6 months (range: 18–84 months).Results:Mean operative time was 307 min. Mean intraoperative blood loss was 3065 ml. For computer navigation, the mean registration deviation during surgery was 1.7 mm. There were 18 wide resections, 4 marginal resections, and 4 intralesional resections. All patients were alive at the final follow-up, with 2 (7.7%) exhibiting tumor recurrence. The other 24 patients were tumor-free. The mean Musculoskeletal Tumor Society Score was 27.3 (range: 19–30).Conclusions:Computer-assisted navigation can be safely applied to the resection of the sacral chordomas, allowing execution of preoperative plans, and achieving good oncological outcomes. Nevertheless, this needs to be accomplished by surgeons with adequate experience and skill.

Late local recurrence, at 19 and 17 years, of sacral chordoma treated by en bloc resection

Sacral chordoma (SC) is a malignant bone tumor with high risk of local recurrence (LR) even after en bloc resection, generally in the first 10years after resection. We report two cases of late LR, at 17 and 19years. Two male patients, aged 45 and 53years, presented with large SC needing a combined approach for en bloc resection. Surgical margins were safe for the first patient and borderline for the second. The patients had yearly follow-up. The first patient developed LR on the posterior wall of the right acetabulum and the second developed LR in the right sciatic notch, at 17 and 19years, respectively. These two cases of very late LR of SC advocate for yearly screening of patients even more than 20years after resection. Level of evidenceIV (case report).

Spinopelvic Reconstruction with Femoral Allograft and Vertical Rectus Abdominis Myocutaneous Flap after Total Sacrectomy in Recurrent Sacral Chordoma: A Case Report

Spinopelvic Reconstruction with Femoral Allograft and Vertical Rectus Abdominis Myocutaneous Flap after Total Sacrectomy in Recurrent Sacral Chordoma: A Case Report

Spinopelvic Reconstruction with Femoral Allograft and Vertical Rectus Abdominis Myocutaneous Flap after Total Sacrectomy in Recurrent Sacral Chordoma: A Case Report

Spinopelvic Reconstruction with Femoral Allograft and Vertical Rectus Abdominis Myocutaneous Flap after Total Sacrectomy in Recurrent Sacral Chordoma: A Case Report

Wide Resection of Sacral Chordoma Including Gluteus Maximus Muscles via Posterior Approach

Wide Resection of Sacral Chordoma Including Gluteus Maximus Muscles via Posterior Approach

Wide Resection of Sacral Chordoma Including Gluteus Maximus Muscles via Posterior Approach

Malign sacral tumors are treated by total sacrectomy and wide resection of soft tissue to reduce local recurrence and for cure. A 78-year-old male presented with low back pain and tenesmus. MRI showed a large tumor in sacrum and the biopsy result showed it was chordoma. “S” shaped incision was applied between L3 and Co2 and chordoma was taken out and gluteal maximus muscles were totally excised. General surgery opened the colostomy and plastic surgery was applied for the flap reconstruction after wound dehiscence. Total sacrectomy is a complicated and difficult surgery because of complex pelvic anatomy. Only posterior approach provides to come through the sacral tumors without anterior approach “laparotomy”. J Med Cases. 2016;7(1):15-17 doi: http://dx.doi.org/10.14740/jmc2254w

CORR(®) Tumor Board: Sacral Insufficiency Fractures are Common After High-dose Radiation for Sacral Chordomas Treated With or Without Surgery.

Beth Israel Deaconess Medical Center and Boston Children’s Hospital Preventing these fractures is important, since we know from our patients and other research [13] that the morbidity of sacral insufficiency fractures is tremendous. The fractures in combination with radiation osteitis are painful and limit patients’ abilities to mobilize, care for themselves, work, and other daily living activities. They are difficult to treat and slow to heal [13]. Patients may be cured of their tumors, but have lasting effects from the treatment on their quality of life. These tumors are rare, but further prospective multiinstitutional collaborative efforts [6, 9] can demonstrate the exact morbidity in this specific population. Given that these fractures are relevant clinically, what can we do to prevent them? Other work by some of the same investigators [10] has shown that high-dose radiation, in addition to en bloc resection, leads to the highest rates of local control in chordomas. Therefore, the argument could be made that radiation cannot be withheld to best improve local tumor control. Antiresorptive therapy, such as use of bisphosphonates, might offer some A Note from the Editor-in-Chief: We are pleased to present our newest column, CORR Tumor Board. This column provides multidisciplinary perspective on the themes raised in selected CORR tumor papers. In this column, we will discuss the implications of the highlighted article from the varied disciplines of the Tumor Board members: Orthopaedic surgery, pathology, and radiology. This month’s column discusses ‘‘Sacral Insufficiency Fractures are Common After High-dose Radiation for Sacral Chordomas Treated With or Without Surgery’’ by Osler and colleagues available at: DOI: 10.1007/s11999-0154566-5. The authors certify that they, or any members of their immediate families, have no funding or commercial associations (eg, consultancies, stock ownership, equity interest, patent/licensing arrangements, etc.) that might pose a conflict of interest in connection with the submitted article. All ICMJE Conflict of Interest Forms for authors and Clinical Orthopaedics and Related Research editors and board members are on file with the publication and can be viewed on request. The opinions expressed are those of the writers, and do not reflect the opinion or policy of CORR or The Association of Bone and Joint Surgeons. This CORR Tumor Board column refers to the article available at DOI: 10.1007/s11999-0154566-5.

Metastatic saccrococcygeal chordoma

We report a 46-year-old man who presented with a 2week history of worsening headaches and acute onset left sided hemiplegia. He had undergone a surgical resection of a sacral chordoma 13years prior, followed by adjuvant radiotherapy and chemotherapy. MRI revealed multiple enhancing lesions in the brain, and the two largest were resected. The histopathology was consistent with chordoma. Sacrococcygeal chordomas are locally invasive notochord-related sarcomas. They rarely metastasize to the brain, and only eight patients have been reported. While currently available adjuvant radiotherapy and systemic chemotherapeutic regimens can be implemented in the management of these rare patients, they have shown limited success. The newer strategies that are reported here have also been disappointing.

CORR Insights(®): Sacral Insufficiency Fractures are Common after High-dose Radiation for Sacral Chordomas Treated With or Without Surgery.

CORR Insights(®): Sacral Insufficiency Fractures are Common after High-dose Radiation for Sacral Chordomas Treated With or Without Surgery.

Analysis of Tumor Response in Mobile Spine and Sacral Chordoma Treated With Definitive High Dose Photon/Proton Radiation Therapy

Analysis of Tumor Response in Mobile Spine and Sacral Chordoma Treated With Definitive High Dose Photon/Proton Radiation Therapy

Expression of Survivin and VEGF in Sacral Chordoma Provides Potential Targets for Therapy

Expression of Survivin and VEGF in Sacral Chordoma Provides Potential Targets for Therapy

Sacral Insufficiency Fractures are Common After High-dose Radiation for Sacral Chordomas Treated With or Without Surgery.

Surgery with high-dose radiation and high-dose radiation alone for sacral chordomas have shown promising local control rates. However, we have noted frequent sacral insufficiency fractures and perceived this rate to be higher than previously reported. We wished (1) to characterize the incidence of sacral insufficiency fractures in patients with chordomas of the sacrum who received high-dose radiation, and (2) to determine whether patients treated with surgery plus high-dose radiation or high-dose radiation alone are more likely to experience a sacral fracture, and to compare time to fracture in these groups. Sixty-two patients who received high-dose radiation for sacral chordomas with (n = 44) or without surgical resection (n = 18) between 1992 and 2013 were included in this retrospective study. At our institution, sacral chordomas generally are treated by preoperative radiotherapy, followed by en bloc resection, and postoperative radiotherapy. Radiation alone, with an intent to cure, is offered to patients who otherwise are not good surgical candidates or patients who elect radiotherapy based on tumor location and the anticipated morbidity after surgery (such as sexual, bowel, or bladder dysfunction). MRI and CT scans were evaluated for evidence of sacral insufficiency fractures. Complete followup was available at a minimum of 2 years (or until fracture or death) for all 18 patients who underwent radiation alone, whereas 14% (six of 44 patients) in the surgery plus radiation group (9% [three of 33] after high sacrectomy and 27% [three of 11] after low sacrectomy) were lost to followup before 2 years. Sacral insufficiency fractures occurred in 29 of the 62 patients (47%). A total of 25 of 33 patients (76%) with high sacrectomy had fractures develop compared with zero of 11 (0%) after low sacrectomy, and four of the 18 patients (22%) who had high-dose radiation alone (p < 0.001). The fracture rate was greater in the high sacrectomy group than in the low sacrectomy group (p < 0.001) and the radiation only group (p < 0.001). There was no difference with the numbers evaluated in fracture probability between patients in the low-sacrectomy group and those treated with radiation alone (p = 0.112). The fracture-free survival probability was 0.99 for the low sacrectomy group at all times as there were no insufficiency fractures in this group; the 1-year fracture-free survival probability was 0.53 (95% CI, 0.35-0.69) after high sacrectomy, 0.83 (95% CI, 0.57-0.94) after radiation alone; the 2-year fracture-free survival probability was 0.36 (95% CI, 0.19-0.52) after high sacrectomy and 0.77 (95% CI, 0.50-0.91) after radiation alone; and the 5-year fracture-free survival probability was 0.14 (95% CI, 0.04-0.30) after high sacrectomy and 0.77 (95% CI, 0.50-0.91) after radiation alone. Acknowledging the limitations of potential differences in baseline and followup among treatment groups in our study, we found that almost ½ of our patients experienced an insufficiency fracture. We found that the fracture rate was greater in the surgery group compared with the radiation alone group and that high sacrectomy accounted for all fractures in the surgery group. These findings can be used to inform patients and also support the need for further research to elucidate the influence of high-dose radiation on bone quality. Level III, therapeutic study.

En bloc resection of middle-lower sacral chordomas under the microscope: a clinical study

Objective To investigate the application value of microneurosurgery in combination with en-bloc idea in the resection of middle-lower sacral chordomas. Methods The chordomas only involving under S3 (including S3) are known as the middle-lower sacral chordomas. Eleven patients with middle-lower sacral chordoma were treated surgically from October 2007 to December 2012. The en-bloc resection concept was used as a guiding principle during the operation; osteotomy, nerve anatomy, and tumor separation were performed by using microneurosurgical technique in the specific removal process. The effect of operation and complications were observed. Results In the case of keeping the capsule intact, the sacral chordomas were totally removed. The mean time of operation was 141 min, and the mean blood loss was 420 ml. The nerves were clearly identified under the microscope. The anterior nerve roots above bilateral S3 were retained completely in 9 cases, and the anterior nerve roots of the unilateral S3 were retained completely in 2 cases. The bowel and bladder functions were preserved completely in 10 cases at 6 months after procedure, and partially preserved in one case. The patients were followed up for a mean of 3.7 years. Two patients had local recurrence. Conclusions In the middle-lower sacral chordoma surgery, en-bloc resection in combination with microneurosurgery technique is conducive to en-bloc resection of sacral chordomas and protection of neurological function, and reduction of introperative blood loss. The recurrence rate is low. Key words: Chordoma; Sacrococcygeal region; Microsurgery; En bloc resection

Current Status of the Treatment for Sacral Chordomas and its Future Trends

Chordomas are rare slow-growing, low to intermediate-grade malignant neoplasms (less than 5% of all primary bone neoplasms); however they sometimes metastasise to distant organs. The 10-year overall survival rate is relatively low. The sacrococcygeal region is one of the most common sites. Intensive surgery is the first line for the treatment, since chordomas are resistant to chemotherapy. In turn, radiotherapy presents marginal effect. It has been reported that surgical margins govern local recurrence and survival. However, sacral chordomas represent a therapeutic challenge as a large number of sacral chordomas are often very large at the time of diagnosis and may comprise adjacent vital organs. Sacrec- tomy includes four surgical approaches; i.e., a combined sequential anterior and posterior approach, a combined simulta- neous anterior and posterior approach, a posterior approach alone and combined extended ilioinguinal and posterior ap- proaches. Because of its large invasion, various postoperative complications were considered. Bladder, bowel and sexual dysfunction and wound complications were major and important complications. The efficacy of adjuvant therapy for the treatment of sacral chordoma is under debate. Chemotherapy has not significant benefit to local control as well as overall survival in patients with chordomas. Standard dose of radiotherapy is not effective for chordomas, either. Some authors described that Hadron-based therapy has several advantages compared with conventional photon-based therapy. Recently some authors described efficacy of new molecular-targeting agents. The infrequency of this disease has prevented to es- tablish the optimal treatment. In this review, we summarized accumulating knowledge of the treatment for sacral chordo- mas and discuss future trends.