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State of the Art and New Approaches to Spinal Cord...
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State of the Art and New Approaches to Spinal Cord Tumors

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Methods and Technologies Development".

Deadline for manuscript submissions: 31 October 2024 | Viewed by 5551

Special Issue Editors

Prof. Dr. Cédric Y. Barrey

E-Mail Website
Guest Editor
Department of Spine and Spinal Cord Surgery, Hôpital Pierre Wertheimer, Hospices Civils de Lyon, Claude Bernard University of Lyon I, 59 Boulevard Pinel, 69677 Lyon, France
Interests: neurosurgery; spine surgery; spinal cord
Dr. Gabriele Capo

E-Mail Website
Guest Editor
Humanitas Research Hospital, Milano, Italy
Interests: neurosurgery; spinal surgeon

Special Issue Information

Dear Colleagues,

In the last years, progresses have been made in the field of spinal cord tumors regarding not only the diagnosis and the management of these lesions, but also the identification of prognostic factors in accordance with a better understanding of their natural course.

Diagnosis times have been reduced in recent years thanks to easier access to MRI. New functional imaging methods such as spinal tractography (DTI) are developing and gaining ground in the preoperative evaluation of these patients.

Multidisciplinary teams are essential to optimize the management of these patients during the pre- and post-operative period; these teams can include rehabilitation doctors, neurologists and neurophysiologists, neurosurgeons specialized in spinal cord surgery, neuroradiologists, and neuro-oncologists and neuropathologists.

New knowledge of the somatotopic distribution of pyramidal tracts and multimodal neuromonitoring techniques allow for increased extent of resection with reduced morbidity. Multimodal intra-operative neuromonitoring including D-wave is now the gold standard, with intramedullary stimulation improving our understanding of spinal cord functions. 

The standard of care for most spinal cord tumors remains the surgical resection, which has improved with modernized operating microsurgery and has benefited from better codification. The quality of surgical resection remains one of the most significant prognostic factors. Radiotherapy and chemotherapy are reserved for high-grade and infiltrative tumors, but further developments in their application are desired bearing in mind the genetic and molecular findings that are emerging.

Research has shown the unique genetic expression profiles of spinal cord tumors and their genetic alterations compared with their intracranial counterparts. The WHO 2021 CNS tumors classification introduced the anatomical and molecular pattern in the definition of tumor type. A new generation of scientific papers based on this new classification are expected.

The role of predictive prognostic factors, such as the extent of surgery, preoperative neurological status and tumor histology, all have to be confirmed.

This Special Issue of Cancers aims to provide a state of the art that includes new updates by well-recognized experts and hyper-specialized teams in the field of spinal cord tumors.

Preoperative strategies for surgical planning, new insights about imaging, surgical techniques, intraoperative functional monitoring, and novel therapeutic drugs are especially welcome. We also encourage submission of articles concerning the most recent diagnostic methods as well as the profiles of molecular tumors. Submitted studies can be focused on prediction models of surgical outcome, novel and intraoperative video-assisted technology (exoscope/augmented reality), and new rehabilitation techniques.

We look forward to your valuable contribution.

Prof. Dr. Cédric Y. Barrey
Dr. Gabriele Capo
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cancers is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2900 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • neurosurgery
  • spinal cord tumors
  • ependymoma
  • microsurgery
  • neuromonitoring
  • neuroradiology
  • neurorehabilitation
  • neurohistology
  • neuro-oncology
  • intramedullary tumors

Published Papers (6 papers)

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Research

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12 pages, 2527 KiB  
Article
Management and Outcome of Recurring Low-Grade Intramedullary Astrocytomas
by Elly Chaskis, Martina Silvestri, Nozar Aghakhani, Fabrice Parker and Steven Knafo
Cancers 2024, 16(13), 2417; https://doi.org/10.3390/cancers16132417 - 30 Jun 2024
Viewed by 513
Abstract
Intramedullary astrocytomas (IMAs) are the second most frequent intramedullary tumors in adults. Low-grade IMAs (LG-IMA, WHO grade I and II) carry a better prognosis than high-grade IMAs (HG-IMAs). However, adjuvant treatment of LG-IMAs by radiotherapy (RT) and/or chemotherapy (CT) as well as treatment [...] Read more.
Intramedullary astrocytomas (IMAs) are the second most frequent intramedullary tumors in adults. Low-grade IMAs (LG-IMA, WHO grade I and II) carry a better prognosis than high-grade IMAs (HG-IMAs). However, adjuvant treatment of LG-IMAs by radiotherapy (RT) and/or chemotherapy (CT) as well as treatment of tumor recurrences remains controversial. The aim of our study was to evaluate the postoperative outcome of LG-IMAs and the management of recurring tumors. We retrospectively reviewed a series of patients operated on for IMA from 1980 to 2022 in a single neurosurgical department. We retrieved 40 patients who received surgery for intramedullary astrocytomas, including 30 LG-IMAs (22 WHO grade I; 5 WHO grade II; 3 “low-grade”) and 10 HG-IMAs (4 WHO grade III; 5 WHO grade IV; 1 “high-grade”). Of the patients with LG-IMAs, the extent of surgical resection was large (gross or subtotal resection >90%) in 30% of cases. Immediate postoperative radiotherapy and/or chemotherapy was proposed only to patients who underwent biopsy (n = 5), while others were initially followed-up. Over a median follow-up of 59 months (range = 13–376), 16 LG-IMA (53.3%) recurred with a mean delay of 28.5 months after surgery (range = 3–288). These included seven biopsies, five partial resections (PR), four subtotal resections (STR) but no gross total resections (GTR). Progression-free survival for LG-IMAs was 51.9% at 3 years and 35.6% at 5 and 10 years; overall survival was 96.3% at 3 years; 90.9% at 5 years and 81.9% at 10 years. There were no significant differences in terms of OS and PFS between WHO grade I and grade II tumors. However, “large resections” (GTR or STR), as opposed to “limited resections” (PR and biopsies), were associated with both better OS (p = 0.14) and PFS (p = 0.04). The treatment of recurrences consisted of surgery alone (n = 3), surgery with RT and/or CT (n = 2), RT with CT (n = 3), RT alone (n = 2) or CT alone (n = 2). In conclusion, although LG-IMAs are infiltrating tumors, the extent of resection (GTR or STR), but not WHO grading, is the main prognostic factor. The management of recurring tumors is highly variable with no conclusive evidence for either option. Full article
(This article belongs to the Special Issue State of the Art and New Approaches to Spinal Cord Tumors)
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11 pages, 792 KiB  
Article
Outcomes and Pattern of Care for Spinal Myxopapillary Ependymoma in the Modern Era—A Population-Based Observational Study
by Chenyang Wang, Michael K. Rooney, Christopher Alvarez-Breckenridge, Thomas H. Beckham, Caroline Chung, Brian S. De, Amol J. Ghia, David Grosshans, Nazanin K. Majd, Mary F. McAleer, Susan L. McGovern, Robert Y. North, Arnold C. Paulino, Subha Perni, Jay P. Reddy, Laurence D. Rhines, Todd A. Swanson, Claudio E. Tatsui, Martin C. Tom, Debra N. Yeboa and Jing Liadd Show full author list remove Hide full author list
Cancers 2024, 16(11), 2013; https://doi.org/10.3390/cancers16112013 - 25 May 2024
Viewed by 626
Abstract
(1) Background: Myxopapillary ependymoma (MPE) is a rare tumor of the spine, typically slow-growing and low-grade. Optimal management strategies remain unclear due to limited evidence given the low incidence of the disease. (2) Methods: We analyzed data from 1197 patients with spinal MPE [...] Read more.
(1) Background: Myxopapillary ependymoma (MPE) is a rare tumor of the spine, typically slow-growing and low-grade. Optimal management strategies remain unclear due to limited evidence given the low incidence of the disease. (2) Methods: We analyzed data from 1197 patients with spinal MPE from the Surveillance, Epidemiology, and End Results (SEER) database (2000–2020). Patient demographics, treatment modalities, and survival outcomes were examined using statistical analyses. (3) Results: Most patients were White (89.9%) with a median age at diagnosis of 42 years. Surgical resection was performed in 95% of cases. The estimated 10-year overall survival was 91.4%. Younger age (hazard ratio (HR) = 1.09, p < 0.001) and receipt of surgery (HR = 0.43, p = 0.007) were associated with improved survival. Surprisingly, male sex was associated with worse survival (HR = 1.86, p = 0.008) and a younger age at diagnosis compared to females. (4) Conclusions: This study, the largest of its kind, underscores the importance of surgical resection in managing spinal MPE. The unexpected association between male sex and worse survival warrants further investigation into potential sex-specific pathophysiological factors influencing prognosis. Despite limitations, our findings contribute valuable insights for guiding clinical management strategies for spinal MPE. Full article
(This article belongs to the Special Issue State of the Art and New Approaches to Spinal Cord Tumors)
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12 pages, 567 KiB  
Article
Multimodal Intraoperative Neurophysiological Monitoring in Intramedullary Spinal Cord Tumors: A 10-Year Single Center Experience
by Maria Pia Tropeano, Zefferino Rossini, Andrea Franzini, Gabriele Capo, Simone Olei, Mario De Robertis, Daniela Milani, Maurizio Fornari and Federico Pessina
Cancers 2024, 16(1), 111; https://doi.org/10.3390/cancers16010111 - 25 Dec 2023
Cited by 1 | Viewed by 842
Abstract
Objective: The study aimed at evaluating the efficacy and the ability of D-wave monitoring combined with somatosensory evoked potentials (SSEPs) and motor evoked potentials (MEPs) to predict functional outcomes in intramedullary spinal cord tumor (IMSCT) surgery. Methods: Between December 2011 and December 2020, [...] Read more.
Objective: The study aimed at evaluating the efficacy and the ability of D-wave monitoring combined with somatosensory evoked potentials (SSEPs) and motor evoked potentials (MEPs) to predict functional outcomes in intramedullary spinal cord tumor (IMSCT) surgery. Methods: Between December 2011 and December 2020, all patients harboring IMSCT who underwent surgery at our institution were prospectively collected in a surgical spinal registry and retrospectively analyzed. Patient charts and surgical and histological reports were analyzed. The multimodal IONM included SSEPs, MEPs, and—whenever possible—D-waves. All patients were evaluated using the modified McCormick and Frankel grade at admission and 3, 6, and 12 months of follow-up. Results: Sixty-four patients were enrolled in the study. SSEP and MEP monitoring was performed in all patients. The D-wave was not recordable in seven patients (11%). Significant IONM changes (at least one evoked potential modality) were registered in 26 (41%) of the 64 patients. In five cases (8%) where the SSEPs and MEPs lost and the D-wave permanently dropped by about 50%, patients experienced a permanent deterioration of their neurological status. Multimodal IONM (SSEP, MEP, and D-wave neuromonitoring) significantly predicted postoperative deficits (p = 0.0001), with a sensitivity of 100.00% and a specificity of 95.65%. However, D-waves demonstrated significantly higher sensitivity (100%) than MEPs (62.5%) and SSEPs (71.42%) alone. These tests’ specificities were 85.10%, 13.89%, and 17.39%, respectively. Comparing the area under ROC curves (AUCs) of these evoked potentials in 53 patients (where all three modalities of IONM were registered) using the pairwise t-test, D-wave monitoring appeared to have higher accuracy and ability to predict postoperative deficits with strong statistical significance compared with MEP and SSEP alone (0.992 vs. 0.798 vs. 0.542; p = 0.018 and p < 0.001). Conclusion: The use of multimodal IONM showed a statistically significant greater ability to predict postoperative deficits compared with SSEP, MEP, and D-wave monitoring alone. D-wave recording significantly increased the accuracy and clinical value of neurophysiological monitoring in IMSCT tumor resection. Full article
(This article belongs to the Special Issue State of the Art and New Approaches to Spinal Cord Tumors)
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Review

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22 pages, 3497 KiB  
Review
State-of-the-Art and New Treatment Approaches for Spinal Cord Tumors
by Chetan Kumawat, Toshiyuki Takahashi, Isao Date, Yousuke Tomita, Masato Tanaka, Shinya Arataki, Tadashi Komatsubara, Angel O. P. Flores, Dongwoo Yu and Mukul Jain
Cancers 2024, 16(13), 2360; https://doi.org/10.3390/cancers16132360 - 27 Jun 2024
Viewed by 336
Abstract
Spinal cord tumors, though rare, present formidable challenges in clinical management due to their intricate nature. Traditional treatment modalities like surgery, radiation therapy, and chemotherapy have been the mainstay for managing these tumors. However, despite significant advancements, challenges persist, including the limitations of [...] Read more.
Spinal cord tumors, though rare, present formidable challenges in clinical management due to their intricate nature. Traditional treatment modalities like surgery, radiation therapy, and chemotherapy have been the mainstay for managing these tumors. However, despite significant advancements, challenges persist, including the limitations of surgical resection and the potential side effects associated with radiation therapy. In response to these limitations, a wave of innovative approaches is reshaping the treatment landscape for spinal cord tumors. Advancements in gene therapy, immunotherapy, and targeted therapy are offering groundbreaking possibilities. Gene therapy holds the potential to modify the genes responsible for tumor growth, while immunotherapy harnesses the body’s own immune system to fight cancer cells. Targeted therapy aims to strike a specific vulnerability within the tumor cells, offering a more precise and potentially less toxic approach. Additionally, novel surgical adjuncts are being explored to improve visualization and minimize damage to surrounding healthy tissue during tumor removal. These developments pave the way for a future of personalized medicine for spinal cord tumors. By delving deeper into the molecular makeup of individual tumors, doctors can tailor treatment strategies to target specific mutations and vulnerabilities. This personalized approach offers the potential for more effective interventions with fewer side effects, ultimately leading to improved patient outcomes and a better quality of life. This evolving landscape of spinal cord tumor management signifies the crucial integration of established and innovative strategies to create a brighter future for patients battling this complex condition. Full article
(This article belongs to the Special Issue State of the Art and New Approaches to Spinal Cord Tumors)
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27 pages, 34546 KiB  
Review
It Looks Like a Spinal Cord Tumor but It Is Not
by Julien Fournel, Marc Hermier, Anna Martin, Delphine Gamondès, Emanuele Tommasino, Théo Broussolle, Alexis Morgado, Wassim Baassiri, Francois Cotton, Yves Berthezène and Alexandre Bani-Sadr
Cancers 2024, 16(5), 1004; https://doi.org/10.3390/cancers16051004 - 29 Feb 2024
Viewed by 1935
Abstract
Differentiating neoplastic from non-neoplastic spinal cord pathologies may be challenging due to overlapping clinical and radiological features. Spinal cord tumors, which comprise only 2–4% of central nervous system tumors, are rarer than non-tumoral myelopathies of inflammatory, vascular, or infectious origins. The risk of [...] Read more.
Differentiating neoplastic from non-neoplastic spinal cord pathologies may be challenging due to overlapping clinical and radiological features. Spinal cord tumors, which comprise only 2–4% of central nervous system tumors, are rarer than non-tumoral myelopathies of inflammatory, vascular, or infectious origins. The risk of neurological deterioration and the high rate of false negatives or misdiagnoses associated with spinal cord biopsies require a cautious approach. Facing a spinal cord lesion, prioritizing more common non-surgical myelopathies in differential diagnoses is essential. A comprehensive radiological diagnostic approach is mandatory to identify spinal cord tumor mimics. The diagnostic process involves a multi-step approach: detecting lesions primarily using MRI techniques, precise localization of lesions, assessing lesion signal intensity characteristics, and searching for potentially associated anomalies at spinal cord and cerebral MRI. This review aims to delineate the radiological diagnostic approach for spinal cord lesions that may mimic tumors and briefly highlight the primary pathologies behind these lesions. Full article
(This article belongs to the Special Issue State of the Art and New Approaches to Spinal Cord Tumors)
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Other

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13 pages, 2019 KiB  
Systematic Review
Unsatisfactory Neurological Outcome in an Intramedullary Thoracic Intermediate-Grade Melanocytoma—Systematic Review and Illustrative Case
by Marco Battistelli, Fulvio Grilli, Alessandro Rapisarda, Michele Di Domenico, Nicola Montano, Marco Gessi, Alessandro Olivi, Alessio Albanese and Filippo Maria Polli
Cancers 2024, 16(10), 1867; https://doi.org/10.3390/cancers16101867 - 14 May 2024
Viewed by 625
Abstract
Background: Intramedullary melanocytomas are exceedingly rare, with only twenty-four cases reported up to now. They present as local invasive tumors despite their benign biological behavior. Attempting a complete safe resection often results in severe post-operative neurological deficits, as in our case presented here. [...] Read more.
Background: Intramedullary melanocytomas are exceedingly rare, with only twenty-four cases reported up to now. They present as local invasive tumors despite their benign biological behavior. Attempting a complete safe resection often results in severe post-operative neurological deficits, as in our case presented here. Methods: A systematic review was conducted across the PubMed and Scopus databases including studies published till February 2024. Results: A total of 19 studies were included, encompassing 24 cases. A similar distribution between sexes was noted (M:F 13:11), with ages ranging from 19 to 79 years. The thoracic segment was most affected, and intermediate-grade melanocytoma (19 cases) was the most common histotype. Radiographically, intramedullary melanocytomas usually appear as hyperintense hemorrhagic lesions peripheral to the central canal with focal nodular enhancement. Intraoperatively, they are black–reddish to tan and are tenaciously adherent lesions. In the sampled studies, IONM employment was uncommon, and post-operative new-onset neurological deficits were described in 16 cases. Adjuvant RT was used in four cases and its value is debatable. Recurrence is common (10 cases), and adjuvant therapies (RT or repeated surgery) seem to play a palliative role. Case presentation: A 68-year-old woman presented with a three-year history of worsening spastic paraparesis and loss of independence in daily activities (McCormick grade 4). An MRI revealed an intramedullary tumor from Th5 to Th7, characterized by T1-weighted hyperintensity and signs of recent intralesional hemorrhage. Multimodal neuromonitoring, comprising the D-Wave, guided the resection of a black–tan-colored tumor with hyper-vascularization and strong adherence to the white matter. During final dissection of the lesion to obtain gross total resection (GTR), a steep decline in MEPs and D-Wave signals was recorded. Post-operatively, the patient had severe hypoesthesia with Th9 level and segmental motor deficits, with some improvement during neurorehabilitation. Histopathology revealed an intermediate-grade melanocytoma (CNS WHO 2021 classification). A four-month follow-up documented the absence of relapse. Conclusions: This literature review highlights that intramedullary T1 hyperintense hemorrhagic thoracic lesions in an adult patient should raise the suspicion of intramedullary melanocytoma. They present as locally aggressive tumors, due to local invasiveness, which often lead to post-operative neurological deficits, and frequent relapses, which overwhelm therapeutic strategies leading to palliative care after several years. Full article
(This article belongs to the Special Issue State of the Art and New Approaches to Spinal Cord Tumors)
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