|
1
|
Al-Mistarehi AH, Parker M, Xia Y, Hasanzadeh A, Horowitz MA, Raj D, Papali P, Davidar AD, Redmond KJ, Bettegowda C, Witham T, Bydon A, Theodore N, Lubelski D. Survival Factors in 1580 Adults with Spinal Ependymoma: Insights from a Multicenter Oncology Database. World Neurosurg 2024; 190:e920-e930. [PMID: 39142388 DOI: 10.1016/j.wneu.2024.08.036] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/21/2024] [Revised: 08/03/2024] [Accepted: 08/05/2024] [Indexed: 08/16/2024]
Abstract
BACKGROUND Using a multi-institutional oncology database, we investigate the survival rates and the impacts of demographic, clinical, and management characteristics on overall survival among adult patients diagnosed with spinal ependymoma. METHODS Utilizing the SEER registry, patients with histologically or radiologically confirmed ependymomas were included. Factors impacting overall survival were analyzed using Kaplan-Meier survival curves and log-rank statistical analyses. RESULTS A total of 1,580 patients were included. Their mean ± standard deviation age was 46.68 ± 15.96 years, and 51.1% were women. Gross total resection (GTR) was achieved in 66.4% of patients. The 5- and 10-year survival rates were 96.7% and 95.4%, respectively. A multivariable backward Cox regression showed that age ≥65 years was a significant predictor for mortality (hazard ratio [HR]: 3.93; 95% confidence interval [CI]: 2.21-7.00; P < 0.001). Likewise, tumor grade 3 (HR: 6.36; 95% CI: 1.95-20.76; P = 0.002), tumor grade 4 (HR: 7.74; 95% CI: 3.97-15.11; P < 0.001), presence of extra-neural metastasis (HR: 13.81; 95% CI: 3.67-51.96; P < 0.001), and receiving radiotherapy (HR: 2.50; 95% CI: 1.50-4.19; P < 0.001) were significant risk factors for mortality, while GTR was significantly associated with improved overall survival compared with subtotal resection or nonsurgical management (HR: 0.42; 95% CI: 0.25-0.73; P = 0.002). There were no significant effects for gender, race, marital status, income, residential area, chemotherapy, tumor size, and the presence of other benign or malignant tumors on the survival hazards (P > 0.05 for each). CONCLUSION Early diagnosis and surgical management of spinal ependymomas, such as GTR, were associated with remarkable survival benefits. Old age, high-grade spinal ependymoma, and extra-neural metastasis were associated with worse overall survival, whereas radiotherapy's role remains unclear.
Collapse
Affiliation(s)
| | - Megan Parker
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Yuanxuan Xia
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Alireza Hasanzadeh
- Advanced Diagnostic and Interventional Radiology Research Center, Medical School, Tehran University of Medical Sciences, Tehran, Iran
| | - Melanie Alfonzo Horowitz
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Divyaansh Raj
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Pritika Papali
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - A Daniel Davidar
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Kristin J Redmond
- Department of Radiation and Molecular Oncology, John Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Chetan Bettegowda
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Timothy Witham
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Ali Bydon
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Nicholas Theodore
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Daniel Lubelski
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
| |
Collapse
|
|
2
|
Esparragosa Vazquez I, Ducray F. The Role of Radiotherapy, Chemotherapy, and Targeted Therapies in Adult Intramedullary Spinal Cord Tumors. Cancers (Basel) 2024; 16:2781. [PMID: 39199553 PMCID: PMC11353198 DOI: 10.3390/cancers16162781] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2024] [Revised: 07/22/2024] [Accepted: 08/02/2024] [Indexed: 09/01/2024] Open
Abstract
Intramedullary primary spinal cord tumors are rare in adults and their classification has recently evolved. Their treatment most frequently relies on maximal safe surgical resection. Herein, we review, in light of the WHO 2021 classification of central nervous system tumors, the knowledge regarding the role of radiotherapy and systemic treatments in spinal ependymomas, spinal astrocytomas (pilocytic astrocytoma, diffuse astrocytoma, spinal glioblastoma IDH wildtype, diffuse midline glioma H3-K27M altered, and high-grade astrocytoma with piloid features), neuro-glial tumors (ganglioglioma and diffuse leptomeningeal glioneuronal tumor), and hemangioblastomas. In spinal ependymomas, radiotherapy is recommended for incompletely resected grade 2 tumors, grade 3 tumors, and recurrent tumors not amenable to re-surgery. Chemotherapy is used in recurrent cases. In spinal astrocytomas, radiotherapy is recommended for incompletely resected grade 2 astrocytomas and grade 3 or 4 tumors as well as recurrent tumors. Chemotherapy is indicated for newly diagnosed high-grade astrocytomas and recurrent cases. In hemangioblastomas not amenable to surgery, radiotherapy is an effective alternative option. Targeted therapies are playing an increasingly important role in the management of some intramedullary primary spinal cord tumor subtypes. BRAF and/or MEK inhibitors have demonstrated efficacy in pilocytic astrocytomas and glioneuronal tumors, belzutifan in von Hippel-Lindau-related hemangioblastomas, and promising results have been reported with ONC201 in diffuse midline glioma H3-K27M altered.
Collapse
Affiliation(s)
| | - François Ducray
- Neuro-Oncology Department, Hospices Civils of Lyon, 69500 Bron, France;
| |
Collapse
|
|
3
|
Kumawat C, Takahashi T, Date I, Tomita Y, Tanaka M, Arataki S, Komatsubara T, Flores AOP, Yu D, Jain M. State-of-the-Art and New Treatment Approaches for Spinal Cord Tumors. Cancers (Basel) 2024; 16:2360. [PMID: 39001422 PMCID: PMC11240441 DOI: 10.3390/cancers16132360] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/21/2024] [Revised: 06/19/2024] [Accepted: 06/24/2024] [Indexed: 07/16/2024] Open
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 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.
Collapse
Affiliation(s)
- Chetan Kumawat
- Department of Orthopedic Surgery, Okayama Rosai Hospital, 1-10-25 Chikkomidorimachi, Minami Ward Okayama, Okayama 702-8055, Japan
- Department of Orthopedic Surgery, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi 110060, India
| | - Toshiyuki Takahashi
- Spinal Disorder Center, Fujieda Heisei Memorial Hospital, 123-1 Mizuue Fujieda, Shizuoka 426-8662, Japan
| | - Isao Date
- Department of Neurosurgery, Okayama Rosai Hospital, 1-10-25 Chikkomidorimachi, Minami Ward Okayama, Okayama 702-8055, Japan
| | - Yousuke Tomita
- Department of Neurosurgery, Okayama Rosai Hospital, 1-10-25 Chikkomidorimachi, Minami Ward Okayama, Okayama 702-8055, Japan
| | - Masato Tanaka
- Department of Orthopedic Surgery, Okayama Rosai Hospital, 1-10-25 Chikkomidorimachi, Minami Ward Okayama, Okayama 702-8055, Japan
| | - Shinya Arataki
- Department of Orthopedic Surgery, Okayama Rosai Hospital, 1-10-25 Chikkomidorimachi, Minami Ward Okayama, Okayama 702-8055, Japan
| | - Tadashi Komatsubara
- Department of Orthopedic Surgery, Okayama Rosai Hospital, 1-10-25 Chikkomidorimachi, Minami Ward Okayama, Okayama 702-8055, Japan
| | - Angel O P Flores
- Department of Orthopedic Surgery, Okayama Rosai Hospital, 1-10-25 Chikkomidorimachi, Minami Ward Okayama, Okayama 702-8055, Japan
| | - Dongwoo Yu
- Department of Orthopedic Surgery, Okayama Rosai Hospital, 1-10-25 Chikkomidorimachi, Minami Ward Okayama, Okayama 702-8055, Japan
| | - Mukul Jain
- Department of Orthopedic Surgery, Okayama Rosai Hospital, 1-10-25 Chikkomidorimachi, Minami Ward Okayama, Okayama 702-8055, Japan
| |
Collapse
|
|
4
|
Davison MA, Lilly DT, Patel AA, Kashkoush A, Chen X, Wei W, Benzel EC, Prayson RA, Chao S, Angelov L. Clinical presentation and extent of resection impacts progression-free survival in spinal ependymomas. J Neurooncol 2024; 167:437-446. [PMID: 38438766 PMCID: PMC11096218 DOI: 10.1007/s11060-024-04623-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2024] [Accepted: 02/26/2024] [Indexed: 03/06/2024]
Abstract
PURPOSE Primary treatment of spinal ependymomas involves surgical resection, however recurrence ranges between 50 and 70%. While the association of survival outcomes with lesion extent of resection (EOR) has been studied, existing analyses are limited by small samples and archaic data resulting in an inhomogeneous population. We investigated the relationship between EOR and survival outcomes, chiefly overall survival (OS) and progression-free survival (PFS), in a large contemporary cohort of spinal ependymoma patients. METHODS Adult patients diagnosed with a spinal ependymoma from 2006 to 2021 were identified from an institutional registry. Patients undergoing primary surgical resection at our institution, ≥ 1 routine follow-up MRI, and pathologic diagnosis of ependymoma were included. Records were reviewed for demographic information, EOR, lesion characteristics, and pre-/post-operative neurologic symptoms. EOR was divided into 2 classifications: gross total resection (GTR) and subtotal resection (STR). Log-rank test was used to compare OS and PFS between patient groups. RESULTS Sixty-nine patients satisfied inclusion criteria, with 79.7% benefitting from GTR. The population was 56.2% male with average age of 45.7 years, and median follow-up duration of 58 months. Cox multivariate model demonstrated significant improvement in PFS when a GTR was attained (p <.001). Independently ambulatory patients prior to surgery had superior PFS (p <.001) and OS (p =.05). In univariate analyses, patients with a syrinx had improved PFS (p =.03) and were more likely to benefit from GTR (p =.01). Alternatively, OS was not affected by EOR (p =.78). CONCLUSIONS In this large, contemporary series of adult spinal ependymoma patients, we demonstrated improvements in PFS when GTR was achieved.
Collapse
Affiliation(s)
- Mark A Davison
- Department of Neurosurgery, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Daniel T Lilly
- Department of Neurosurgery, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Arpan A Patel
- Department of Neurosurgery, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Ahmed Kashkoush
- Department of Neurosurgery, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Xiaoying Chen
- Department of Quantitative Health Sciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Wei Wei
- Department of Quantitative Health Sciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Edward C Benzel
- Department of Neurosurgery, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Richard A Prayson
- Department of Anatomic Pathology, The Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Samuel Chao
- Department of Neurosurgery, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Lilyana Angelov
- Department of Neurosurgery, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA.
- Rose Ella Burkhardt Brain Tumor & Neuro-Oncology Center, Cleveland Clinic, Cleveland, OH, USA.
- Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA.
- Neurologic Oncology and Radiosurgery Fellowships, Neurological Surgery, CCLCM at CWRU, Rose Ella Burkhardt Brain Tumor & Neuro-Oncology Center, 9500 Euclid Ave., CA-51, 44195, Cleveland, OH, USA.
| |
Collapse
|
|
5
|
McFaline-Figueroa JR. Spinal Cord Neoplasms. Continuum (Minneap Minn) 2024; 30:99-118. [PMID: 38330474 DOI: 10.1212/con.0000000000001375] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/10/2024]
Abstract
OBJECTIVE This article discusses the diagnostic approach to patients with suspected neoplasms of the spinal cord and reviews the most common primary and metastatic spinal neoplasms and their presentations. LATEST DEVELOPMENTS Neoplasms of the spinal cord are rare entities that can involve the spinal cord parenchyma, the dura and leptomeninges, or the extradural space. The most common intramedullary spinal cord neoplasms are primary spinal cord tumors, including ependymomas, pilocytic astrocytomas, and diffuse midline gliomas. The most common primary neoplasms of the spine are intradural extramedullary spinal meningiomas, whereas primary neoplasms of the leptomeninges are rare. Advances in molecular characterization of spinal cord tumors and recent clinical trials of these rare entities are expanding the repertoire of systemic therapy options for primary spinal cord neoplasms. Metastases to the spine most often affect the extradural space. Metastatic epidural spinal cord compression is a neurologic emergency that requires a rapid, multidisciplinary response to preserve neurologic function. ESSENTIAL POINTS Neurologists should understand the diagnostic approach to neoplasms of the spinal cord. Knowledge of the most common spinal cord neoplasms will allow for appropriate management and optimal patient care.
Collapse
|
|
6
|
Cerretti G, Pessina F, Franceschi E, Barresi V, Salvalaggio A, Padovan M, Manara R, Di Nunno V, Bono BC, Librizzi G, Caccese M, Scorsetti M, Maccari M, Minniti G, Navarria P, Lombardi G. Spinal ependymoma in adults: from molecular advances to new treatment perspectives. Front Oncol 2023; 13:1301179. [PMID: 38074692 PMCID: PMC10704349 DOI: 10.3389/fonc.2023.1301179] [Citation(s) in RCA: 10] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/24/2023] [Accepted: 10/24/2023] [Indexed: 12/21/2024] Open
Abstract
Ependymomas are rare glial tumors with clinical and biological heterogeneity, categorized into supratentorial ependymoma, posterior fossa ependymoma, and spinal cord ependymoma, according to anatomical localization. Spinal ependymoma comprises four different types: spinal ependymoma, spinal ependymoma MYCN-amplified, myxopapillary ependymoma, and subependymoma. The clinical onset largely depends on the spinal location of the tumor. Both non-specific and specific sensory and/or motor symptoms can be present. Owing to diverse features and the low incidence of spinal ependymomas, most of the current clinical management is derived from small retrospective studies, particularly in adults. Treatment involves primarily surgical resection, aiming at maximal safe resection. The use of radiotherapy remains controversial and the optimal dose has not been established; it is usually considered after subtotal resection for WHO grade 2 ependymoma and for WHO grade 3 ependymoma regardless of the extent of resection. There are limited systemic treatments available, with limited durable results and modest improvement in progression-free survival. Thus, chemotherapy is usually reserved for recurrent cases where resection and/or radiation is not feasible. Recently, a combination of temozolomide and lapatinib has shown modest results with a median progression-free survival (PFS) of 7.8 months in recurrent spinal ependymomas. Other studies have explored the use of temozolomide, platinum compounds, etoposide, and bevacizumab, but standard treatment options have not yet been defined. New treatment options with targeted treatments and immunotherapy are being investigated. Neurological and supportive care are crucial, even in the early stages. Post-surgical rehabilitation can improve the consequences of surgery and maintain a good quality of life, especially in young patients with long life expectancy. Here, we focus on the diagnosis and treatment recommendations for adults with spinal ependymoma, and discuss recent molecular advances and new treatment perspectives.
Collapse
Affiliation(s)
- Giulia Cerretti
- Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, Italy
| | - Federico Pessina
- Department of Neurosurgery, IRCCS Humanitas Research Hospital, Milan, Italy
- Department of Biomedical Sciences, Humanitas University, Milan, Italy
| | - Enrico Franceschi
- Nervous System Medical Oncology Department, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy
| | - Valeria Barresi
- Department of Diagnostics and Public Health, University of Verona, Verona, Italy
| | - Alessandro Salvalaggio
- Department of Neuroscience, University of Padova, Padova, Italy
- Padova Neuroscience Center (PNC), University of Padova, Padova, Italy
| | - Marta Padovan
- Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, Italy
| | - Renzo Manara
- Department of Neuroscience, Azienda Ospedale-Università di Padova, Padua, Italy
- Department of Medicine - DIMED, University of Padova, Padua, Italy
| | - Vincenzo Di Nunno
- Nervous System Medical Oncology Department, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy
| | - Beatrice Claudia Bono
- Department of Neurosurgery, IRCCS Humanitas Research Hospital, Milan, Italy
- Department of Biomedical Sciences, Humanitas University, Milan, Italy
| | - Giovanni Librizzi
- Department of Neuroscience, Azienda Ospedale-Università di Padova, Padua, Italy
| | - Mario Caccese
- Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, Italy
| | - Marta Scorsetti
- Department of Radiotherapy and Radiosurgery, IRCCS Humanitas Research Hospital, Milan, Italy
| | - Marta Maccari
- Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, Italy
| | - Giuseppe Minniti
- Department of Radiological Sciences, Oncology and Anatomical Pathology, Sapienza University, Rome, Italy
- IRCCS Neuromed, Pozzilli, Italy
| | - Pierina Navarria
- Department of Radiotherapy and Radiosurgery, IRCCS Humanitas Research Hospital, Milan, Italy
| | - Giuseppe Lombardi
- Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, Italy
| |
Collapse
|
|
7
|
Noureldine MHA, Shimony N, Jallo GI. Benign Spinal Tumors. ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY 2023; 1405:583-606. [PMID: 37452955 DOI: 10.1007/978-3-031-23705-8_23] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 07/18/2023]
Abstract
Benign spinal intradural tumors are relatively rare and include intramedullary tumors with a favorable histology such as low-grade astrocytomas and ependymomas, as well as intradural extramedullary tumors such as meningiomas and schwannomas. The effect on the neural tissue is usually a combination of mass effect and neuronal involvement in cases of infiltrative tumors. The new understanding of molecular profiling of different tumors allowed us to better define central nervous system tumors and tailor treatment accordingly. The mainstay of management of many intradural spinal tumors is maximal safe surgical resection. This goal is more achievable with intradural extramedullary tumors; yet, with a meticulous surgical approach, many of the intramedullary tumors are amenable for safe gross-total or near-total resection. The nature of these tumors is benign; hence, a different way to measure outcome success is pursued and usually depends on functional rather than oncological or survival outcomes.
Collapse
Affiliation(s)
- Mohammad Hassan A Noureldine
- Department of Neurosurgery and Brain Repair, University of South Florida Morsani College of Medicine, Tampa, FL, USA
- Institute for Brain Protection Sciences, Johns Hopkins University School of Medicine, Johns Hopkins All Children's Hospital, Saint Petersburg, FL, USA
| | - Nir Shimony
- Institute of Neuroscience, Geisinger Medical Center, Geisinger Commonwealth School of Medicine, Danville, PA, USA
- Institute for Brain Protections Sciences, Johns Hopkins All Children's Hospital, Saint Petersburg, FL, USA
- Department of Surgery, St Jude Children's Research Hospital, Memphis, USA
| | - George I Jallo
- Institute for Brain Protections Sciences, Johns Hopkins All Children's Hospital, Saint Petersburg, FL, USA.
| |
Collapse
|
|
8
|
Extra-Neural Metastases of Late Recurrent Myxopapillary Ependymoma to Left Lumbar Paravertebral Muscles: Case Report and Review of the Literature. Brain Sci 2022; 12:brainsci12091227. [PMID: 36138961 PMCID: PMC9497216 DOI: 10.3390/brainsci12091227] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/12/2022] [Revised: 09/04/2022] [Accepted: 09/05/2022] [Indexed: 11/21/2022] Open
Abstract
Ependymomas are commonly classified as low-grade tumors, although they may harbor a malignant behavior characterized by distant neural dissemination and spinal drop metastasis. Extra-CNS ependymoma metastases are extremely rare and only few cases have been reported in the lung, lymph nodes, pleura, mediastinum, liver, bone, and diaphragmatic, abdominal, and pelvic muscles. A review of the literature yielded 14 other case reports metastasizing outside the central nervous system, but to our knowledge, no studies describe metastasis in the paravertebral muscles. Herein, we report the case of a 39-year-old patient with a paraspinal muscles metastasis from a myxopapillary ependymoma. The neoplasm was surgically excised and histologically and molecularly analyzed. Both the analyses were consistent with the diagnosis of muscle metastases of myxopapillary ependymoma. The here-presented case report is first case in the literature of a paraspinal muscles metastasis of myxopapillary ependymoma.
Collapse
|
|
9
|
Kanno H, Kanetsuna Y, Shinonaga M. Anaplastic myxopapillary ependymoma: A case report and review of literature. World J Clin Oncol 2021; 12:1072-1082. [PMID: 34909401 PMCID: PMC8641005 DOI: 10.5306/wjco.v12.i11.1072] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/23/2021] [Revised: 05/17/2021] [Accepted: 10/11/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Myxopapillary ependymoma (MPE) is a pathological grade I tumor that arises in the filum terminale. MPE with anaplastic features is extremely rare, and only 5 cases have shown malignancy at the time of recurrence.
CASE SUMMARY The patient (a 46-year-old woman) had undergone a MPE operation 30 years ago. After subtotal resection of the tumor located in L4-S1, it had a solid component that extended to the adjacent subcutaneous region. Histologically, the tumor consisted of a typical MPE with anaplastic features. The anaplastic areas of the tumor showed hypercellularity, a rapid mitotic rate, vascular proliferation, and connective tissue proliferation. Pleomorphic cells and atypical mitotic figures were occasionally observed. The MIB-1 index in this area was 12.3%. The immunohistochemical study showed immunoreactivity for vimentin, glial fibrillary acidic protein and S100. The morphological pattern and immunohistochemical profile were consistent with anaplastic MPE. The patient tolerated surgery well without new neurological deficits. She underwent local irradiation for the residual tumor and rehabilitation.
CONCLUSION Although extremely rare, anaplastic MPE occurs in both pediatric and adult patients, similar to other ependymomas. At a minimum, close monitoring is recommended, given concerns about aggressive biological potential. In the future, further study is needed to determine the WHO classification criteria and genetic indicators of tumor progression. The possibility of malignant transformation of MPE should be taken into account, and patients with MPE should be treated with care and follow-up.
Collapse
Affiliation(s)
- Hiroshi Kanno
- Department of Neurosurgery, International University of Health and Welfare Atami Hospital, Atami 413-0012, Shizuoka, Japan
| | - Yukiko Kanetsuna
- Department of Pathology, International University of Health and Welfare Atami Hospital, Atami 413-0012, Shizuoka, Japan
| | - Masamichi Shinonaga
- Department of Neurosurgery, International University of Health and Welfare Atami Hospital, Atami 413-0012, Shizuoka, Japan
| |
Collapse
|
|
10
|
Abstract
Spinal cord diseases in pediatric patients are highly variable in terms of presentation, pathology, and prognosis. Not only do they differ with respect to each other but so too with their adult equivalents. Some of the most common diseases are autoimmune (ie, multiple sclerosis, acute disseminated encephalomyelitis, and acute transverse myelitis), congenital (ie, dysraphism with spina bifida, split cord malformation, and tethered cord syndrome), tumor (ie, juvenile pilocytic astrocytoma, ependymoma, and hem-angioblastoma), and vascular (ie, cavernous malformations, arteriovenous malformations, and dural arteriovenous fistulas) in nature. These each require their own niche treatment paradigm and prognosis. Furthermore, presentation of different spinal cord diseases in children can be difficult to discern without epidemiologic and imaging data. Interpretation of these data is crucial to facilitating a timely and accurate diagnosis. Correspondingly, the aim of this review was to highlight the most pertinent features of the most common spinal cord diseases in the pediatric population.
Collapse
Affiliation(s)
- Victor M Lu
- Department of Neurological Surgery, Nicklaus Children's Hospital, Miami, FL.,Department of Neurological Surgery, University of Miami, Miami, FL
| | - Toba N Niazi
- Department of Neurological Surgery, Nicklaus Children's Hospital, Miami, FL.,Department of Neurological Surgery, University of Miami, Miami, FL
| |
Collapse
|
|
11
|
Challenges in diagnosis and management of adult spinal cord gliomas. Rev Neurol (Paris) 2021; 177:515-523. [PMID: 33896651 DOI: 10.1016/j.neurol.2021.02.384] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/06/2020] [Revised: 02/09/2021] [Accepted: 02/09/2021] [Indexed: 11/22/2022]
Abstract
Intramedullary spinal cord gliomas have very low incidence rates. They are associated with difficulties in diagnosis and treatment, and cause significant morbidity. Their clinical presentation and their appearance at magnetic resonance imaging are not specific. They can mimic inflammatory, infectious, vascular disorders or other neoplastic lesions. Primary treatment is surgery. Surgical resection can often be total for ependymomas, but difficult for infiltrating astrocytomas. Radiotherapy is indicated for malignant tumors, but remains controversial in some indications. Chemotherapy is reserved for recurrence, but small retrospective series are available. Genetic studies have revealed genetic alterations which could have a potential impact on treatment in the near future.
Collapse
|
|
12
|
Hussain I, Parker WE, Barzilai O, Bilsky MH. Surgical Management of Intramedullary Spinal Cord Tumors. Neurosurg Clin N Am 2020; 31:237-249. [PMID: 32147015 DOI: 10.1016/j.nec.2019.12.004] [Citation(s) in RCA: 22] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023]
Abstract
Intramedullary spinal cord tumors (IMSCT) comprise a rare subset of CNS tumors that have distinct management strategies based on histopathology. These tumors often present challenges in regards to optimal timing for surgery, invasiveness, and recurrence. Advances in microsurgical techniques and technological adjuncts have improved extent of resection and outcomes with IMSCT. Furthermore, adjuvant therapies including targeted immunotherapies and image-guided radiation therapy have witnessed rapid development over the past decade, further improving survival for many of these patients. In this review, we provide an overview of types, epidemiology, imaging characteristics, surgical management strategies, and future areas of research for IMSCT.
Collapse
Affiliation(s)
- Ibrahim Hussain
- Department of Neurological Surgery, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA; Department of Neurological Surgery, Weill Cornell Medical College, 525 E. 68th St, New York, NY 10065, USA.
| | - Whitney E Parker
- Department of Neurological Surgery, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA; Department of Neurological Surgery, Weill Cornell Medical College, 525 E. 68th St, New York, NY 10065, USA
| | - Ori Barzilai
- Department of Neurological Surgery, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA
| | - Mark H Bilsky
- Department of Neurological Surgery, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA; Department of Neurological Surgery, Weill Cornell Medical College, 525 E. 68th St, New York, NY 10065, USA
| |
Collapse
|
|
13
|
Brown DA, Goyal A, Takami H, Graffeo CS, Mahajan A, Krauss WE, Bydon M. Radiotherapy in addition to surgical resection may not improve overall survival in WHO grade II spinal ependymomas. Clin Neurol Neurosurg 2019; 189:105632. [PMID: 31862631 DOI: 10.1016/j.clineuro.2019.105632] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2019] [Revised: 10/27/2019] [Accepted: 12/06/2019] [Indexed: 11/19/2022]
Abstract
OBJECTIVES Spinal ependymomas are rare intramedullary neoplasms. The paucity of cases limits the ability to conduct large prospective studies. Current guidelines recommend maximal safe resection followed by adjuvant radiotherapy (RT) in cases of grade II spinal ependymomas with subtotal resections (STR) and all grade III spinal ependymomas. Herein we assess the impact of RT on survival in grades II and III spinal ependymomas. PATIENTS AND METHODS The National Cancer Database was queried for adult patients with WHO grades II or III spinal ependymomas diagnosed between 2004 and 2014 who underwent resection or biopsy. Kaplan-Meier and multivariable Cox regression models were used to determine the impact of radiotherapy on survival. RESULTS A total of 1058 patients met inclusion criteria. Most patients (85.9 %) received a biopsy/STR versus gross total resection (GTR, 14.1 %). Radiotherapy was preferentially performed in those with residual tumor (p = 0.001). We found a 10-fold increased hazard of death in grade III versus grade II tumors (HR: 10.33; 95 % CI: 5.01-21.3; p < 0.001). Age positively correlated with worsened survival (HR: 1.04; 95 % CI: 1.02-1.10; p < 0.001). Adjuvant RT did not reduce the hazard of death for the cohort overall (HR: 1.08; 95 % CI: 0.55-2.10; p = 0.810) or among those with grade II tumors (HR: 0.90; 95 % CI: 0.38-2.10; p = 0.810). We found no additional survival benefit of GTR compared to biopsy/STR (HR: 0.52; 95 % CI: 0.19-1.50; p = 0.217). CONCLUSION While RT may improve progression-free survival, it may not impact overall survival in surgically resected grade II and III spinal ependymomas. Future studies should evaluate the impact of RT on local recurrence and symptomatic improvement.
Collapse
Affiliation(s)
| | - Anshit Goyal
- Department of Neurosurgery, Mayo Clinic, Rochester, MN, USA; Mayo Clinic Neuro-Informatics Laboratory, Mayo Clinic, Rochester, MN, USA
| | - Hirokazu Takami
- Division of Neurosurgery, Toronto Western Hospital, Toronto, ON, USA
| | | | - Anita Mahajan
- Department of Radiation Oncology, Mayo Clinic, Rochester, MN, USA
| | | | - Mohamad Bydon
- Department of Neurosurgery, Mayo Clinic, Rochester, MN, USA; Mayo Clinic Neuro-Informatics Laboratory, Mayo Clinic, Rochester, MN, USA.
| |
Collapse
|
|
14
|
Azad TD, Jiang B, Bettegowda C. Molecular foundations of primary spinal tumors-implications for surgical management. ANNALS OF TRANSLATIONAL MEDICINE 2019; 7:222. [PMID: 31297387 DOI: 10.21037/atm.2019.04.46] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
Primary spinal tumors are rare lesions that require careful clinical management due to their intimate relationship with critical neurovascular structures and the significant associated risk of morbidity. While the advent of molecular and genomic profiling is beginning to impact the management of the cranial counterparts, translation for spinal tumors has lagged behind. Maximal safe surgical resection remains the mainstay of patients with primary spinal tumors, with extent of resection and histology the only consistently identified independent predictors of survival. Adjuvant therapy has had limited impact. To develop targeted neoadjuvant and adjuvant therapies, improve prognostication, and enhance patient selection in spinal oncology, a thorough understanding of the current molecular and genomic landscape of spinal tumors is required. In this review, we detail the epidemiology, current standard-of-care, and molecular features of the most commonly encountered intramedullary spinal cord tumors (IMSCT), intradural extramedullary (IDEM) tumors, and primary spinal column malignancies (PSCM). We further discuss current efforts and future opportunities for integrating molecular advances in spinal oncology with clinical management.
Collapse
Affiliation(s)
- Tej D Azad
- Department of Neurosurgery, Stanford University School of Medicine, Stanford, CA, USA
| | - Bowen Jiang
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA
| | - Chetan Bettegowda
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA
| |
Collapse
|
|
15
|
Abd-El-Barr MM, Huang KT, Moses ZB, Iorgulescu JB, Chi JH. Recent advances in intradural spinal tumors. Neuro Oncol 2019; 20:729-742. [PMID: 29216380 DOI: 10.1093/neuonc/nox230] [Citation(s) in RCA: 30] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022] Open
Abstract
Intradural spinal tumors are rare tumors of the central nervous system. Due to the eloquence of the spinal cord and its tracts, the compact architecture of the cord and nerves, and the infiltrative nature of some of these tumors, surgical resection is difficult to achieve without causing neurological deficits. Likewise, chemotherapy and radiotherapy are utilized more cautiously in the treatment of intradural spinal tumors than their cranial counterparts. Targeted therapies aimed at the genetic alterations and molecular biology tailored to these tumors would be helpful but are lacking.Here, we review the major types of intradural spinal tumors, with an emphasis on genetic alterations, molecular biology, and experimental therapies for these difficult to treat neoplasms.
Collapse
Affiliation(s)
- Muhammad M Abd-El-Barr
- Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Kevin T Huang
- Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Ziev B Moses
- Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - J Bryan Iorgulescu
- Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - John H Chi
- Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| |
Collapse
|
|
16
|
Rudà R, Reifenberger G, Frappaz D, Pfister SM, Laprie A, Santarius T, Roth P, Tonn JC, Soffietti R, Weller M, Moyal ECJ. EANO guidelines for the diagnosis and treatment of ependymal tumors. Neuro Oncol 2018; 20:445-456. [PMID: 29194500 PMCID: PMC5909649 DOI: 10.1093/neuonc/nox166] [Citation(s) in RCA: 183] [Impact Index Per Article: 26.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022] Open
Abstract
Ependymal tumors are rare CNS tumors and may occur at any age, but their proportion among primary brain tumors is highest in children and young adults. Thus, the level of evidence of diagnostic and therapeutic interventions is higher in the pediatric compared with the adult patient population.The diagnosis and disease staging is performed by craniospinal MRI. Tumor classification is achieved by histological and molecular diagnostic assessment of tissue specimens according to the World Health Organization (WHO) classification 2016. Surgery is the crucial initial treatment in both children and adults. In pediatric patients with intracranial ependymomas of WHO grades II or III, surgery is followed by local radiotherapy regardless of residual tumor volume. In adults, radiotherapy is employed in patients with anaplastic ependymoma WHO grade III, and in case of incomplete resection of WHO grade II ependymoma. Chemotherapy alone is reserved for young children <12 months and for adults with recurrent disease when further surgery and irradiation are no longer feasible. A gross total resection is the mainstay of treatment in spinal ependymomas, and radiotherapy is reserved for incompletely resected tumors. Nine subgroups of ependymal tumors across different anatomical compartments (supratentorial, posterior fossa, spinal) and patient ages have been identified with distinct genetic and epigenetic alterations, and with distinct outcomes. These findings may lead to more precise diagnostic and prognostic assessments, molecular subgroup-adapted therapies, and eventually new recommendations pending validation in prospective studies.
Collapse
Affiliation(s)
- Roberta Rudà
- Department of Neuro-Oncology, University of Turin and City of Health and Science University Hospital, Turin, Italy
| | - Guido Reifenberger
- Institute of Neuropathology, Medical Faculty, Heinrich Heine University Düsseldorf and German Cancer Consortium (DKTK), partner site Essen/Düsseldorf, Düsseldorf, Germany
| | - Didier Frappaz
- Département de Neuro-Oncologie, Centre Léon-Bérard, Institut d’Hématologie et Oncologie Pédiatrique et Adulte, Lyon, France
| | - Stefan M Pfister
- Division of Pediatric Neuro-oncology, German Cancer Research Center, DKTK, Heidelberg, Germany and Department of Pediatric Oncology, Hematology and Immunology, University Hospital Heidelberg, Heidelberg, Germany
| | - Anne Laprie
- Department of Radiation Oncology, Institut Universitaire du Cancer de Toulouse Oncopole, Toulouse, France
| | | | - Patrick Roth
- Department of Neurology and Brain Tumor Center, University Hospital Zurich and University of Zurich, Zurich, Switzerland
| | - Joerg Christian Tonn
- Department of Neurosurgery Ludwig-Maximilians-Universität and DKTK partner site, University of Munich, Munich, Germany
| | - Riccardo Soffietti
- Department of Neuro-Oncology, University of Turin and City of Health and Science University Hospital, Turin, Italy
| | - Michael Weller
- Department of Neurology and Brain Tumor Center, University Hospital Zurich and University of Zurich, Zurich, Switzerland
| | | |
Collapse
|
|
17
|
Abstract
STUDY DESIGN The expression of HOXB13 and HOXA9 proteins was detected. OBJECTIVE The purpose of this study was to investigate the molecular signature of spinal ependymoma (EPN) and astrocytoma, 2 most common types of intramedullary spinal tumor. SUMMARY OF BACKGROUND DATA Intramedullary spinal tumor is unusual. It leads to high neurological morbidity and mortality without treatment. Till now, its molecular feature has been elucidated up to a little extent. METHODS A total of 37 cases of spinal EPN, including 12 myxopapillary EPNs (MEPNs), 18 classic EPNs, and 7 anaplastic EPNs, and another 12 cases of astrocytoma were selected for this study. Immunohistochemical analysis of a large cohort of patients providing clinical tumor samples was performed to compare the expression of HOXB13 and HOXA9 not only between spinal EPN and astrocytoma but also among all 3 World Health Organization grades of spinal EPN. RESULTS The results showed that HOXB13 and HOXA9 were selectively expressed in spinal EPN instead of astrocytoma. Furthermore, we found the strongest positive response of HOXB13 in MEPN whereas that of HOXA9 was ubiquitously detected in all subgroups of EPN. CONCLUSION Both specificity and sensitivity of HOXB13 in MEPN indicated that HOXB13 might be a diagnostic marker to distinguish MEPN from other 2 types of EPN and a promising therapeutic target for MEPN. The strong immunoreactivity of HOXA9 in spinal EPN suggested an indispensable role in the progression of spinal EPN, and further research on its molecular function will provide new clues for the development of treatment options. LEVEL OF EVIDENCE N /A.
Collapse
|
|
18
|
Theeler BJ, Gilbert MR. Investigating therapies in ependymoma. Expert Opin Orphan Drugs 2016. [DOI: 10.1080/21678707.2016.1191347] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
Affiliation(s)
- Brett J. Theeler
- Department of Neurology, Walter Reed National Military Medical Center, Neurology and John P. Murtha Cancer Center, Bethesda, MD, USA
| | - Mark R. Gilbert
- Neuro-Oncology Branch, National Institutes of Health, Bethesda, MD, USA
| |
Collapse
|
|
19
|
Celano E, Salehani A, Malcolm JG, Reinertsen E, Hadjipanayis CG. Spinal cord ependymoma: a review of the literature and case series of ten patients. J Neurooncol 2016; 128:377-86. [PMID: 27154165 DOI: 10.1007/s11060-016-2135-8] [Citation(s) in RCA: 71] [Impact Index Per Article: 7.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2015] [Accepted: 05/01/2016] [Indexed: 02/06/2023]
Abstract
Spinal cord ependymoma (SCE) is a rare tumor that is most commonly low-grade. Complete surgical resection has been established as first-line treatment and can be curative. However, SCEs tend to recur when complete tumor resection is not possible. Evidence supporting the use of adjuvant radiation and chemotherapy is not definitive. We review the most recent literature on SCE covering a comprehensive range of topics spanning the biology, presentation, clinical management, and outcomes. In addition, we present a case series of ten SCE patients with the goal of contributing to existing knowledge of this rare disease.
Collapse
Affiliation(s)
- Emma Celano
- Emory University School of Medicine, Atlanta, GA, USA
| | | | | | - Erik Reinertsen
- Emory University School of Medicine, Atlanta, GA, USA.,Department of Biomedical Engineering, Georgia Institute of Technology, Atlanta, GA, USA
| | - Constantinos G Hadjipanayis
- Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, Mount Sinai Beth Israel Philips Ambulatory Care Center, 10 Union Square, 5th Floor, Suite 5E, New York, NY, 10003, USA.
| |
Collapse
|
|
20
|
Abstract
Tumours of the spinal cord, although rare, are associated with high morbidity. Surgical resection remains the primary treatment for patients with this disease, and offers the best chance for cure. Such surgical procedures, however, carry substantial risks such as worsening of neurological deficit, paralysis and death. New therapeutic avenues for spinal cord tumours are needed, but genetic studies of the molecular mechanisms governing tumourigenesis in the spinal cord are limited by the scarcity of high-quality human tumour samples. Many spinal cord tumours have intracranial counterparts that have been extensively studied, but emerging data show that the tumours are genetically and biologically distinct. The differences between brain and spine tumours make extrapolation of data from one to the other difficult. In this Review, we describe the demographics, genetics and current treatment approaches for the most commonly encountered spinal cord tumours--namely, ependymomas, astrocytomas, haemangioblastomas and meningiomas. We highlight advances in understanding of the biological basis of these lesions, and explain how the latest progress in genetics and beyond are being translated to improve patient care.
Collapse
|
|
21
|
Chamberlain MC, Tredway TL. Adult primary intradural spinal cord tumors: a review. Curr Neurol Neurosci Rep 2011; 11:320-8. [PMID: 21327734 DOI: 10.1007/s11910-011-0190-2] [Citation(s) in RCA: 189] [Impact Index Per Article: 13.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
Primary spinal cord tumors constitute 2% to 4% of all central nervous system neoplasms and are characterized based on their location as intramedullary, intradural extramedullary, and extradural. A contemporary literature review of primary intradural spinal cord tumors was performed. Among intramedullary tumors, ependymomas are more common and often can be surgically resected. However, astrocytomas infiltrate the spinal cord and complete resection is rare. Intradural extramedullary tumors include schwannomas, neurofibromas, and meningiomas and are usually amenable to surgical resection. Radiotherapy is reserved for malignant variants and recurrent gliomas, whereas chemotherapy is administered for recurrent primary spinal cord tumors without surgical or radiotherapy options. Early recognition of the signs and symptoms related to primary spinal cord tumors facilitates timely discovery, treatment, potentially minimizes neurologic morbidity, and may improve outcome. Treatment consists of surgical resection, and predictors of outcome include preoperative functional status, histologic grade of tumor, and extent of surgical resection.
Collapse
Affiliation(s)
- Marc C Chamberlain
- Department of Neurology/Division of Neuro-OncologyFred Hutchinson Cancer Research Center, Seattle Cancer Care Alliance, University of Washington, 825 Eastlake Avenue East, POB 19023, MS G4940, Seattle, WA 98109-1023, USA.
| | | |
Collapse
|
|
22
|
Kim DJ, Kim TW, Kim Y, Park KH. Clear cell ependymoma occurring in the cauda equina. J Korean Neurosurg Soc 2010; 48:153-6. [PMID: 20856665 DOI: 10.3340/jkns.2010.48.2.153] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2009] [Revised: 02/15/2010] [Accepted: 08/03/2010] [Indexed: 11/27/2022] Open
Abstract
The authors present a rare case of clear cell ependymoma that developed in the cauda equina. A 54-year-old man was admitted to hospital with intermittent lower back pain. A neurological examination conducted on admission revealed no sensory or motor disturbance. Deep tendon reflexes in both lower extremities were normal. Magnetic resonance images demonstrated a 1.0 cm-sized intradural mass at the filum terminale. Gross total resection was performed via total laminectomy of L1 and L2. The tumor was confirmed to be clear cell ependymoma by histopathologic examination. His symptom was relieved after surgery.
Collapse
Affiliation(s)
- Dong Joon Kim
- Department of Neurosurgery, Seoul Veterans Hospital, Seoul, Korea
| | | | | | | |
Collapse
|
|
23
|
Temozolomide for malignant primary spinal cord glioma: an experience of six cases and a literature review. J Neurooncol 2010; 101:247-54. [DOI: 10.1007/s11060-010-0249-y] [Citation(s) in RCA: 43] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2010] [Accepted: 05/18/2010] [Indexed: 10/19/2022]
|
|
24
|
Abstract
Primary spinal cord tumors represent 2-4% of all neoplasms of the CNS. Primary spinal cord tumors are anatomically separable into two broad categories: intradural intramedullary and intradural extramedullary. Intramedullary tumors are comprised predominantly of gliomas (infiltrative astrocytomas and ependymomas). Resective surgery can usually be accomplished with spinal ependymomas owing to separation of tumor from spinal cord and, when complete, require no further therapy. By contrast, spinal cord gliomas infiltrate the myelon and, consequently, surgery is nearly always incomplete. Involved-field radiotherapy is most often administered after partial resection. Intradural extramedullary tumors are either peripheral nerve sheath tumors (neurofibromas or schwanommas) or meningiomas. In either instance, complete resection may be accomplished and is often curative. Radiotherapy is reserved for rare malignant variants and for patients in whom surgery is contraindicated. Chemotherapy is administered for recurrent primary spinal cord tumors without other options, that is, reoperation or re-irradiation. Problematic, however, is the lack of clinical trials in general for these CNS tumors and for spinal cord tumors in particular. Consequently, treatment is similar to that for intracranial tumors with a similar histology. Early recognition of the signs and symptoms of primary spinal cord tumors allows for early treatment, potentially minimizes neurologic morbidity and improves outcome. Primary treatment is surgery in essentially all spinal cord tumors, and predictors of outcome include preoperative functional status, histological grade of tumor and extent of surgical resection.
Collapse
Affiliation(s)
- Sean Grimm
- University of Washington, Department of Neurology/Division of Neuro-Oncology, Fred Hutchinson Cancer Research Center, Seattle Cancer Care Alliance, 825 Eastlake Avenue E, POB 19023, MS G4940, Seattle, WA 98109-1023, USA
| | | |
Collapse
|
|
25
|
Abstract
STUDY DESIGN Clinically based systematic review. OBJECTIVE To define optimal clinical care for primary intramedullary spinal cord tumors using a systematic review with expert opinion. METHODS Focused questions on the treatment of primary intramedullary spinal cord tumors were refined by a panel of spine oncology surgeons, medical and radiation oncologist. Keyword were searched through Medline database and pertinent abstracts and manuscripts obtained. The quality of literature was rated as high, moderate, low, or very low. Using the GRADE evidence based review system the proposed questions were answered using the literature review and expert opinion. These treatment recommendations were then rated as either strong or weak based on the quality of evidence and clinical expertise. RESULTS The literature searches revealed low and very low quality evidence with no prospective or randomized studies. The MEDLINE search engine returned 9000 articles which was restricted to articles about human subjects and written in the English language. The subsequent search resulted in a return of: "spinal cord tumor" (5053), "ependymoma" (580), "astrocytoma" (420), and "glioma" (235) articles. Seventeen articles referenced timing of surgical intervention and symptomatology for intramedullary spinal cord tumors. One hundred fifty-eight chemotherapy and 183 radiation therapy articles for intramedullary spinal cord tumors were reviewed. CONCLUSION The most important factor in determining the IMSCT patient's long-term neurologic and functional outcome after surgery is the patient's preoperative neurologic status. However, this must be taken in the context of the underlying tumor histology. Therefore, resection is reserved for progressive neurologic decline and serial monitoring for asymptomatic individuals. Adjuvant therapy is an option for high grade astrocytomas (WHO grades 3-4).
Collapse
|
|
26
|
Schor NF. Pharmacotherapy for adults with tumors of the central nervous system. Pharmacol Ther 2008; 121:253-64. [PMID: 19091301 DOI: 10.1016/j.pharmthera.2008.11.003] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2008] [Accepted: 11/07/2008] [Indexed: 11/18/2022]
Abstract
Tumors of the adult central nervous system are among the most common and most chemoresistant neoplasms. Malignant tumors of the brain and spinal cord collectively account for approximately 1.3% of all cancers and 2.2% of all cancer-related deaths. Novel pharmacological approaches to nervous system tumors are urgently needed. This review presents the current approaches and challenges to successful pharmacotherapy of adults with malignant tumors of the central nervous system and discusses novel approaches aimed at overcoming these challenges.
Collapse
Affiliation(s)
- Nina F Schor
- Departments of Pediatrics, Neurology, and Neurobiology & Anatomy, University of Rochester Medical Center, Rochester, NY, USA.
| |
Collapse
|
|
27
|
Meneses MS, Leal AG, Periotto LB, Milano JB, Coelho-Net M, Sobral AC, Ramina R. Primary filum terminale ependymoma: a series of 16 cases. ARQUIVOS DE NEURO-PSIQUIATRIA 2008; 66:529-33. [DOI: 10.1590/s0004-282x2008000400017] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/18/2007] [Accepted: 05/20/2008] [Indexed: 11/21/2022]
Abstract
Filum terminale ependymomas are slow growing tumors of the cauda equina with a high incidence in young adults. Although a complete microsurgical resection can lead to a cure, recurrence is not uncommon. Sixteen cases of filum terminale ependymomas treated at the Instituto de Neurologia de Curitiba were analyzed. Eleven patients were females and 5 males, their age ranging from 7 to 84 years. Symptoms and signs included lumbar pain (31.25%), radicular pain (56.25%) and neurological deficits (12.5%). In three cases, patients had previously undergone surgery in other hospitals. All were tested through MRI and were operated on. Two underwent a laminoplasty and 14 a laminectomy. The last 8 patients of this series had neuro-physiological monitoring during surgery. In all patients a total microsurgical resection was achieved. Histologically, 2 cases were cellular ependymomas and 14 cases myxopapillary ependymomas. There was no recurrence during a 2 to 84 month follow-up period.
Collapse
|
|
28
|
Newton HB, Ray-Chaudhury A, Malkin MG. Overview of Pathology and Treatment of Primary Spinal Cord Tumors. HANDBOOK OF NEURO-ONCOLOGY NEUROIMAGING 2008:36-49. [DOI: 10.1016/b978-012370863-2.50007-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 07/19/2023]
|
|
29
|
Abstract
Optimal management of ependymomas includes surgical resection and evaluation of the extent of central nervous system involvement using cerebrospinal fluid cytology and craniospinal contrast-enhanced MRI. In instances of measurable residual disease, reoperation should be considered because survival of patients with ependymomas is significantly improved by performance of a complete resection. In patients not considered for further surgery and with residual disease, limited-field radiotherapy is usually administered. The role of craniospinal irradiation in patients with local disease and no evidence of metastasis is controversial because most tumor recurrences are local and at the site of the primary tumor. No clear role for adjuvant chemotherapy has been demonstrated. When used, chemotherapy for ependymomas has been administered primarily to children aged younger than 3 years as adjuvant therapy and to patients with recurrent disease who are not considered surgical candidates as salvage therapy. Recurrent ependymomas are managed by reoperation of tumors that are surgically accessible, by radiotherapy if not previously administered, and by salvage chemotherapy. The role of stereotactic radiotherapy administered as radiosurgery or brachytherapy is unclear because all reports are anecdotal. Because salvage chemotherapy is not curative, no standard therapy exists, and a variety of chemotherapy agents and drug schedules have been investigated.
Collapse
Affiliation(s)
- Sajeel Chowdhary
- Department of Interdisciplinary Oncology, H. Lee Moffitt Cancer Center, University of South Florida, Neuro-Oncology Program, 12902 Magnolia Drive, Tampa, FL 33612, USA
| | | | | |
Collapse
|
|
30
|
Lee J, Parsa AT, Ames CP, McCormick PC. Clinical management of intramedullary spinal ependymomas in adults. Neurosurg Clin N Am 2006; 17:21-7. [PMID: 16448904 DOI: 10.1016/j.nec.2005.11.001] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Affiliation(s)
- Janet Lee
- Department of Neurological Surgery, University of Utah, Salt Lake City, 30 N, 1900 E, RM 3B409, UT 84112, USA
| | | | | | | |
Collapse
|
|
31
|
Abstract
Ependymomas are uncommon neoplasms of the central nervous system (CNS), and as a consequence, few randomized, clinical trials have been performed, thereby limiting treatment guidelines. A review of the literature would permit the following conclusions regarding treatment. The best management of newly diagnosed ependymoma entails a complete resection corroborated by postoperative contrast-enhanced magnetic resonance imaging (MRI). If an incomplete resection is documented, a second attempt at gross total resection should be considered, given the prognostic significance of complete resection. Small volume residual disease is best managed with involved-field radiotherapy unless postoperative staging (cerebrospinal fluid cytology, neuraxis MRI) documents metastatic disease, which is best managed by craniospinal irradiation. The role of chemotherapy is uncertain and in general would be reserved for patients having previously failed surgery and radiotherapy. Disease-free survival following recurrence is unusual (<15% at 5 years) and suggests intensification of initial adjuvant treatment may best prevent relapse.
Collapse
Affiliation(s)
- Marc C Chamberlain
- Department of Neurology, USC/Norris Cancer Center, 1441 Eastlake Avenue, Suite 3459, Los Angeles, CA 90033-0804, USA.
| |
Collapse
|