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Free access

Introduction. Chordoma: updates and advances

Paul A. Gardner, Sebastien Froelich, Ziya L. Gokaslan, Shannon M. MacDonald, Maria Peris Celda, Shaan M. Raza, and Georgios A. Zenonos

Free access

Multidisciplinary surgical considerations for en bloc resection of sacral chordoma: review of recent advances and a contemporary single-center series

Christian Schroeder, Weston C. de Lomba, Owen P. Leary, Rafael De la Garza Ramos, Julia S. Gillette, Thomas J. Miner, Albert S. Woo, Jared S. Fridley, Ziya L. Gokaslan, and Patricia L. Zadnik Sullivan

OBJECTIVE

Contemporary management of sacral chordomas requires maximizing the potential for recurrence-free and overall survival while minimizing treatment morbidity. En bloc resection can be performed at various levels of the sacrum, with tumor location and volume ultimately dictating the necessary extent of resection and subsequent tissue reconstruction. Because tumor resection involving the upper sacrum may be quite destabilizing, other pertinent considerations relate to instrumentation and subsequent tissue reconstruction. The primary aim of this study was to survey the surgical approaches used for managing primary sacral chordoma according to location of lumbosacral spine involvement, including a narrative review of the literature and examination of the authors’ institutional case series.

METHODS

The authors performed a narrative review of pertinent literature regarding reconstruction and complication avoidance techniques following en bloc resection of primary sacral tumors, supplemented by a contemporary series of 11 cases from their cohort. Relevant surgical anatomy, advances in instrumentation and reconstruction techniques, intraoperative imaging and navigation, soft-tissue reconstruction, and wound complication avoidance are also discussed.

RESULTS

The review of the literature identified several surgical approaches used for management of primary sacral chordoma localized to low sacral levels (mid-S2 and below), high sacral levels (involving upper S2 and above), and high sacral levels with lumbar involvement. In the contemporary case series, the majority of cases (8/11) presented as low sacral tumors that did not require instrumentation. A minority required more extensive instrumentation and reconstruction, with 2 tumors involving upper S2 and/or S1 levels and 1 tumor extending into the lower lumbar spine. En bloc resection was successfully achieved in 10 of 11 cases, with a colostomy required in 2 cases due to rectal involvement. All 11 cases underwent musculocutaneous flap wound closure by plastic surgery, with none experiencing wound complications requiring revision.

CONCLUSIONS

The modern management of sacral chordoma involves a multidisciplinary team of surgeons and intraoperative technologies to minimize surgical morbidity while optimizing oncological outcomes through en bloc resection. Most cases present with lower sacral tumors not requiring instrumentation, but stabilizing instrumentation and lumbosacral reconstruction are often required in upper sacral and lumbosacral cases. Among efforts to minimize wound-related complications, musculocutaneous flap closure stands out as an evidence-based measure that may mitigate risk.

Open access

Clinical criteria for filum terminale resection in occult tethered cord syndrome

Petra M. Klinge, Owen P. Leary, Philip A. Allen, Konstantina Svokos, Patricia Sullivan, Thomas Brinker, and Ziya L. Gokaslan

OBJECTIVE

Tethered cord syndrome (TCS) comprises three symptom categories: back/leg pain, bowel/bladder, and neurological complaints. MRI typically reveals a low-lying conus medullaris, filum terminale (FT) pathology, or lumbosacral abnormalities. FT resection is established in TCS but not in radiologically occult TCS (OTCS). This study aims to identify patients with OTCS who are likely to benefit from FT resection.

METHODS

The authors recruited 149 patients with OTCS (31 pediatric, 118 adult) treated with FT resection—including only cases with progressive TCS, negative spine MRI, and no concurrent neurological/urological conditions. A comprehensive questionnaire collected patient self-reported symptoms and clinical findings at the preoperative and at 3- and 12-month follow-up examinations. Based on questionnaire data, the authors extracted a 15-item symptoms and findings scale to represent the three TCS symptom categories, assigning 1 point for each item present.

RESULTS

OTCS presents without radicular/segmental sensorimotor findings, but with leg/back pain and conus dysfunction, in addition to leg fatigue and spasticity; the latter indicating an upper motoneuron pathology. The 15-item scale showed clinical improvement in 89% of patients at the 3-month follow-up and 68% at the 12-month follow-up. Multivariate analysis of the scale revealed that it accurately predicts outcome of FT resection in 82% of cases. Patients with a preoperative score exceeding 6 points are most likely to benefit from surgery.

CONCLUSIONS

By applying the study’s inclusion criteria and incorporating the novel 15-item scale, surgeons can effectively select candidates for FT resection in patients with OTCS. The observed outcomes in these selected patients are comparable to those achieved in degenerative spine surgery.

Restricted access

Treatment of intramedullary spinal cord tumors: a modified Delphi technique of the North American Spine Society Section of Spine Oncology

Presented at the 2023 AANS/CNS Joint Section on Disorders of the Spine and Peripheral Nerves

Andrew M. Hersh, Zach Pennington, Daniel Lubelski, Aladine A. Elsamadicy, Nicolas Dea, Atman Desai, Ziya L. Gokaslan, C. Rory Goodwin, Wesley Hsu, George I. Jallo, Ajit Krishnaney, Ilya Laufer, Sheng-Fu Larry Lo, Mohamed Macki, Ankit I. Mehta, Ali Ozturk, John H. Shin, Hesham Soliman, and Daniel M. Sciubba

OBJECTIVE

Intramedullary spinal cord tumors (IMSCTs) are rare tumors with heterogeneous presentations and natural histories that complicate their management. Standardized guidelines are lacking on when to surgically intervene and the appropriate aggressiveness of resection, especially given the risk of new neurological deficits following resection of infiltrative tumors. Here, the authors present the results of a modified Delphi method using input from surgeons experienced with IMSCT removal to construct a framework for the operative management of IMSCTs based on the clinical, radiographic, and tumor-specific characteristics.

METHODS

A modified Delphi technique was conducted using a group of 14 neurosurgeons experienced in IMSCT resection. Three rounds of written correspondence, surveys, and videoconferencing were carried out. Participants were queried about clinical and radiographic criteria used to determine operative candidacy and guide decision-making. Members then completed a final survey indicating their choice of observation or surgery, choice of resection strategy, and decision to perform duraplasty, in response to a set of patient- and tumor-specific characteristics. Consensus was defined as ≥ 80% agreement, while responses with 70%–79% agreement were defined as agreement.

RESULTS

Thirty-six total characteristics were assessed. There was consensus favoring surgical intervention for patients with new-onset myelopathy (86% agreement), chronic myelopathy (86%), or progression from mild to disabling numbness (86%), but disagreement for patients with mild numbness or chronic paraplegia. Age was not a determinant of operative candidacy except among frail patients, who were deemed more suitable for observation (93%). Well-circumscribed (93%) or posteriorly located tumors reaching the surface (86%) were consensus surgical lesions, and participants agreed that the presence of syringomyelia (71%) and peritumoral T2 signal change (79%) were favorable indications for surgery. There was consensus that complete loss of transcranial motor evoked potentials with a 50% decrease in the D-wave amplitude should halt further resection (93%). Preoperative symptoms seldom influenced choice of resection strategy, while a distinct cleavage plane (100%) or visible tumor-cord margins (100%) strongly favored gross-total resection.

CONCLUSIONS

The authors present a modified Delphi technique highlighting areas of consensus and agreement regarding surgical management of IMSCTs. Although not intended as a substitute for individual clinical decision-making, the results can help guide care of these patients. Additionally, areas of controversy meriting further investigation are highlighted.

Restricted access

Optimizing surgical management of facet cysts of the lumbar spine: systematic review, meta-analysis, and local case series of 1251 patients

Arjun Ganga, Owen P. Leary, Aayush Setty, Kevin Xi, Albert E. Telfeian, Adetokunbo A. Oyelese, Tianyi Niu, Joaquin Q. Camara-Quintana, Ziya L. Gokaslan, Patricia Zadnik Sullivan, and Jared S. Fridley

OBJECTIVE

Lumbar facet cysts (LFCs) can cause neurological dysfunction and intractable pain. Surgery is the current standard of care for patients in whom conservative therapy fails, those with neurological deficits, and those with evidence of spinal instability. No study to date has comprehensively examined surgical outcomes comparing the multiple surgical treatment options for LFCs. Therefore, the authors aimed to perform a combined analysis of cases both in the literature and of patients at a single institution to compare the outcomes of various surgical treatment options for LFC.

METHODS

The authors performed a literature review in accordance with PRISMA guidelines and meta-analysis of the PubMed, Embase, and Cochrane Library databases and reviewed all studies from database inception published until February 3, 2023. Studies that did not contain 3 or more cases, clearly specify follow-up durations longer than 6 months, or present new cases were excluded. Bias was evaluated using Cochrane Collaboration’s Risk of Bias in Nonrandomised Studies–of Interventions (ROBINS-I). The authors also reviewed their own local institutional case series from 2015 to 2020. Primary outcomes were same-level cyst recurrence, same-level revision surgery, and perioperative complications. ANOVA, common and random-effects modeling, and Wald testing were used to compare treatment groups.

RESULTS

A total of 1251 patients were identified from both the published literature (29 articles, n = 1143) and the authors’ institution (n = 108). Patients were sorted into 5 treatment groups: open cyst resection (OCR; n = 720), tubular cyst resection (TCR; n = 166), cyst resection with arthrodesis (CRA; n = 165), endoscopic cyst resection (ECR; n = 113), and percutaneous cyst rupture (PCR; n = 87), with OCR being the analysis reference group. The PCR group had significantly lower complication rates (p = 0.004), higher recurrence rates (p < 0.001), and higher revision surgery rates (p = 0.001) compared with the OCR group. Patients receiving TCR (3.01%, p = 0.021) and CRA (0.0%, p < 0.001) had significantly lower recurrence rates compared with those undergoing OCR (6.36%). The CRA group (6.67%) also had significantly lower rates of revision surgery compared with the OCR group (11.3%, p = 0.037).

CONCLUSIONS

While PCR is less invasive, it may have high rates of same-level recurrence and revision surgery. Recurrence and revision rates for modalities such as ECR were not significantly different from those of OCR. While concomitant arthrodesis is more invasive, it might lead to lower recurrence rates and lower rates of subsequent revision surgery. Given the limitations of our case series and literature review, prospective, randomized studies are needed.

Open access

Perception of frailty in spinal metastatic disease: international survey of the AO Spine community

Mark A. MacLean, Miltiadis Georgiopoulos, Raphaële Charest-Morin, C. Rory Goodwin, Ilya Laufer, Nicolas Dea, John H. Shin, Ziya L. Gokaslan, Laurence D. Rhines, John E. O’Toole, Daniel M. Sciubba, Michael G. Fehlings, Byron F. Stephens, Chetan Bettegowda, Sten Myrehaug, Alexander C. Disch, Cordula Netzer, Naresh Kumar, Arjun Sahgal, Niccole M. Germscheid, Michael H. Weber, and on behalf of the AO Spine Knowledge Forum Tumor

OBJECTIVE

Frailty has not been clearly defined in the context of spinal metastatic disease (SMD). Given this, the objective of this study was to better understand how members of the international AO Spine community conceptualize, define, and assess frailty in SMD.

METHODS

The AO Spine Knowledge Forum Tumor conducted an international cross-sectional survey of the AO Spine community. The survey was developed using a modified Delphi technique and was designed to capture preoperative surrogate markers of frailty and relevant postoperative clinical outcomes in the context of SMD. Responses were ranked using weighted averages. Consensus was defined as ≥ 70% agreement among respondents.

RESULTS

Results were analyzed for 359 respondents, with an 87% completion rate. Study participants represented 71 countries. In the clinical setting, most respondents informally assess frailty and cognition in patients with SMD by forming a general perception based on clinical condition and patient history. Consensus was attained among respondents regarding the association between 14 preoperative clinical variables and frailty. Severe comorbidities, extensive systemic disease burden, and poor performance status were most associated with frailty. Severe comorbidities associated with frailty included high-risk cardiopulmonary disease, renal failure, liver failure, and malnutrition. The most clinically relevant outcomes were major complications, neurological recovery, and change in performance status.

CONCLUSIONS

The respondents recognized that frailty is important, but they most commonly evaluate it based on general clinical impressions rather than using existing frailty tools. The authors identified numerous preoperative surrogate markers of frailty and postoperative clinical outcomes that spine surgeons perceived as most relevant in this population.

Free access

Verteporfin-loaded microparticles for radiosensitization of preclinical lung and breast metastatic spine cancer

Oluwaseun O. Akinduro, Paola Suarez-Meade, McKinley Roberts, Stephany Y. Tzeng, Rachel Sarabia-Estrada, Paula Schiapparelli, Emily S. Norton, Ziya L. Gokaslan, Panos Z. Anastasiadis, Hugo Guerrero-Cázares, Jordan J. Green, and Alfredo Quiñones-Hinojosa

OBJECTIVE

The vertebral column is the most common site for skeletal metastasis, often leading to debilitating pain and weakness. Metastatic cancer has unique genetic drivers that potentiate tumorigenicity. There is an unmet need for novel targeted therapy in patients with spinal metastatic disease.

METHODS

The authors assessed the effect of verteporfin-induced yes-associated protein (YAP) inhibition on spine metastatic cell tumorigenicity and radiation sensitivity in vitro. Animal studies used a subcutaneous xenograft mouse model to assess the use of systemic intraperitoneal verteporfin (IP-VP) and intratumoral verteporfin microparticles (IT-VP) to inhibit the tumorigenicity of lung and breast spinal metastatic tumors from primary patient-derived tissue.

RESULTS

Verteporfin led to a dose-dependent decrease in migration, clonogenicity, and cell viability via inhibition of YAP and downstream effectors cyclin D1, CTGF, TOP2A, ANDRD1, MCL-1, FOSL2, KIF14, and KIF23. This was confirmed with knockdown of YAP. Verteporfin has an additive response when combined with radiation, and knockdown of YAP rendered cells more sensitive to radiation. The addition of verteporfin to YAP knockdown cells did not significantly alter migration, clonogenicity, or cell viability. IP-VP and IT-VP led to diminished tumor growth (p < 0.0001), especially when combined with radiation (p < 0.0001). Tissue analysis revealed diminished expression of YAP (p < 0.0001), MCL-1 (p < 0.0001), and Ki-67 (p < 0.0001) in tissue from verteporfin-treated tumors compared with vehicle-treated tumors.

CONCLUSIONS

This is the first study to demonstrate that verteporfin-mediated inhibition of YAP leads to diminished tumorigenicity in lung and breast spinal metastatic cancer cells. Targeting of YAP with verteporfin offers promising results that could be translated to human clinical trials.

Free access

Continued underrepresentation of historically excluded groups in the neurosurgery pipeline: an analysis of racial and ethnic trends across stages of medical training from 2012 to 2020

Elijah M. Persad-Paisley, Sarah B. Andrea, Owen P. Leary, Orianna D. Carvalho, Victoria G. Zeyl, Amanda R. Laguna, Matthew N. Anderson, Belinda Shao, Steven A. Toms, Adetokunbo A. Oyelese, Ziya L. Gokaslan, and Katherine M. Sharkey

OBJECTIVE

US allopathic medical schools have experienced improvements in racial and ethnic diversity among matriculants in the past decade. It is not clear, however, whether better representation of historically excluded racial and ethnic groups at medical school entry impacts subsequent stages of the medical training pipeline leading into a specific field. The aim of this study was to examine these trends as they relate to the neurosurgical medical education pipeline and consider the drivers that sustain barriers for underrepresented groups.

METHODS

Race and ethnicity reports from the American Association of Medical Colleges were obtained on allopathic medical school applicants, acceptees, and graduates and applicants to US neurosurgical residency programs from 2012 to 2020. The representation of groups categorized by self-reported race and ethnicity was compared with their US population counterparts to determine the representation quotient (RQ) for each group. Annual racial composition differences and changes in representation over time at each stage of medical training were evaluated by estimating incidence rate ratios (IRRs) and 95% confidence intervals (CIs) using non-Hispanic Whites as the reference group.

RESULTS

On average, Asian and White individuals most frequently applied and were accepted to medical school, had the highest graduation rates, and applied to neurosurgery residency programs more often than other racial groups. The medical school application and acceptance rates for Black individuals increased from 2012 to 2020 relative to Whites by 30% (95% CI 1.23–1.36) and 42% (95% CI 1.31–1.53), respectively. During this same period, however, inequities in neurosurgical residency applications grew across all non-Asian racialized groups relative to Whites. While the incidence of active Black neurosurgery residents increased from 2012 to 2020 (0.6 to 0.7/100,000 Black US inhabitants), the prevalence of White neurosurgery residents grew in the active neurosurgery resident population by 16% more.

CONCLUSIONS

The increased racial diversity of medical school students in recent years is not yet reflected in racial representation among neurosurgery applicants. Disproportionately fewer Black relative to White US medical students apply to neurosurgery residency, which contributes to declining racial representation among all active neurosurgery resident physicians. Hispanic individuals are becoming increasingly represented in neurosurgery residency but continue to remain underrepresented relative to the US population. Ongoing efforts to recruit medical students into neurosurgery who more accurately reflect the diversity of the general US population are necessary to ensure equitable patient care.

Free access

Awake transforaminal endoscopic lumbar facet cyst resection: technical note and case series

Matthew J. Hagan, Albert E. Telfeian, Rahul Sastry, Rohaid Ali, Kai-Uwe Lewandrowski, Sanjay Konakondla, Sean Barber, Kendall Lane, and Ziya L. Gokaslan

OBJECTIVE

The aim of this study was to describe a minimally invasive transforaminal surgical technique for treating awake patients presenting with lumbar radiculopathy and compressive facet cysts.

METHODS

Awake transforaminal endoscopic decompression surgery was performed in 645 patients over a 6-year period from 2014 to 2020. Transforaminal endoscopic decompression surgery utilizing a high-speed endoscopic drill was performed in 25 patients who had lumbar facet cysts. All surgeries were performed as outpatient procedures in awake patients. Nine of the 25 patients had previously undergone laminectomies at the treated level. A retrospective chart review of patient-reported outcome measures is presented.

RESULTS

At the 2-year follow-up, the mean (± standard deviation) preoperative visual analog scale leg score and Oswestry Disability Index improved from 7.6 ± 1.3 to 2.3 ± 1.4 and 39.7% ± 8.1% to 13.0% ± 7.4%, respectively. There were no complications, readmissions, or recurrence of symptoms during the 2-year follow-up period.

CONCLUSIONS

A minimally invasive awake procedure is presented for the treatment of lumbar facet cysts in patients with lumbar radiculopathy. Approximately one-third of the treated patients (9 of 25) had postlaminectomy facet cysts.

Open access

Navigation-assisted resection of tumoral calcinosis of the lumbosacral spine: illustrative case

Oliver Y. Tang, Patricia Zadnik Sullivan, Teddi Tubre, Joshua Feler, Belinda Shao, Jesse Hart, and Ziya L. Gokaslan

BACKGROUND

Tumoral calcinosis is an uncommon disease resulting from dystrophic calcium phosphate crystal deposition, with only 7% of cases involving the spine, and it may diagnostically mimic neoplasms.

OBSERVATIONS

In this case, a 54-year-old woman with history of systemic scleroderma presented with 10 months of progressive left lumbosacral pain. Imaging revealed an expansile, 4 × 7-cm, well-circumscribed mass in the lumbosacral spine with L5–S1 neuroforaminal compression. Because intractable pain and computed tomography (CT)-guided needle biopsy did not entirely rule out malignancy, operative management was pursued. The patient underwent L4–S2 laminectomies, left L5–S1 facetectomy, L5 and S1 pediculectomies, and en bloc resection, performed under stereotactic CT-guided intraoperative navigation. Subsequently, instrumented fusion was performed with L4 and L5 pedicle screws and S2 alar-iliac screws. Pathological examination was consistent with tumoral calcinosis, with multiple nodules of amorphous basophilic granular calcified material lined by histiocytes. There was no evidence of recurrence or neurological deficits at 5-month follow-up.

LESSONS

Because spinal tumoral calcinosis may mimic neoplasms on imaging or gross intraoperative appearance, awareness of this clinical entity is essential for any spine surgeon. A review of all case reports of lumbosacral tumoral calcinosis (n = 14 from 1952 to 2016) was additionally performed. The case featured in this report presents the first known case of navigation-assisted resection of lumbosacral tumoral calcinosis.