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Nature Reviews. Neurology May 2013Tumours of the spinal cord, although rare, are associated with high morbidity. Surgical resection remains the primary treatment for patients with this disease, and... (Review)
Review
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.
Topics: Animals; Genome; Genomics; Humans; Mutation; Spinal Cord Neoplasms
PubMed: 23528542
DOI: 10.1038/nrneurol.2013.48 -
Medicine May 2020Intramedullary cervical spinal cord teratomas (ICTs) are extremely rare, and diagnosis and treatment are challenging. We conducted a systematic review of the literature...
BACKGROUND
Intramedullary cervical spinal cord teratomas (ICTs) are extremely rare, and diagnosis and treatment are challenging. We conducted a systematic review of the literature on the diagnosis and treatment of ICT.
METHOD
The presentation, imaging manifestations, diagnosis, management, surgery findings, prognosis and histology were reviewed following Preferred Reporting Items for Systematic Reviews and Meta Analyses guidelines. English-language studies and case reports published from inception to 2018 were retrieved. Data on presentation, imaging characteristics, diagnosis, management, surgery findings, outcomes, and histopathology were extracted.
RESULTS
Ten articles involving 10 patients were selected. The lesions were located in the upper cervical vertebrae in 4 cases, whereas in the lower cervical vertebrae in the remaining 6 cases. In 5 cases, the lesions were located on the dorsal side of the spinal cord, and in the center of the spinal cord in the remaining 5 cases. Quadriparesis (60%), paraplegia (30%), monoplegia (10%), and neck pain (50%) were the main presentations. The lesion appeared as a intramedullary heterogeneous signal during an MRI scan, and the lesion signal would be partially enhanced after the contrast medium was applied. All patients underwent surgical intervention through a posterior approach. Neurological function improved postoperatively in all patients. Two patients with pathology confirmed to be immature teratomas experienced recurrence.
CONCLUSION
ICTs are extremely rare entities that are mainly located in the center or dorsal part of the spinal cord which mainly manifest as quadriplegia and neck pain. MRI is a useful modality that provides diagnostic clues. Surgery from a posterior approach is the primary treatment, and the effect of adjuvant therapy remains uncertain. The prognosis is mainly related to the pathological nature of the tumor and not the method of resection.
Topics: Cervical Vertebrae; Humans; Spinal Cord Neoplasms; Teratoma
PubMed: 32358400
DOI: 10.1097/MD.0000000000020107 -
The Journal of Spinal Cord Medicine Jan 2013Due to advances in acute oncological treatment, patients with spinal cord tumors exhibit improved survival. However, these patients have not received the full benefits... (Review)
Review
CONTEXT
Due to advances in acute oncological treatment, patients with spinal cord tumors exhibit improved survival. However, these patients have not received the full benefits of rehabilitation services to address their neurological deficits and rehabilitation goals.
OBJECTIVE
To evaluate the epidemiology and pathophysiology of spinal cord tumors, address methods of acute oncological management, review treatment for neurological sequelae, and understand the implications as they relate to rehabilitation.
METHODS
An extensive literature review was performed regarding the epidemiology, pathophysiology, acute oncological management, neurological sequelae, and rehabilitation for patients with spinal cord tumors. Databases used included pubmed.gov and OVID, as well as individual journal and textbook articles.
RESULTS
Access to treatment should be increased given improved survival and functional deficits for patients with spinal cord tumors. Individuals can benefit from inpatient rehabilitation programs, in spite of increased medical co-morbidity and neurological deficits. Specific areas of improvement include functionality, mood, quality of life, and survival. Adjustments to treatment plans must incorporate medical complications from cancer and its treatment, perceived quality of life, and prognosis.
CONCLUSIONS
Patients with spinal cord tumors who participate in rehabilitation programs show general improvement in function, mood, quality of life, and survival. Adaptations to care plans should be made to accommodate medical co-morbidities from cancer and its treatment, patient perceptions, and prognosis.
Topics: Humans; Prognosis; Quality of Life; Recovery of Function; Spinal Cord Neoplasms
PubMed: 23433329
DOI: 10.1179/2045772312Y.0000000015 -
Journal of Veterinary Internal Medicine Jan 2022Magnetic resonance imaging is the method of choice for diagnosing spinal cord neoplasia, but the accuracy of designating the relationship of a neoplasm to the meninges...
BACKGROUND
Magnetic resonance imaging is the method of choice for diagnosing spinal cord neoplasia, but the accuracy of designating the relationship of a neoplasm to the meninges and agreement among observers is unknown.
OBJECTIVES
To determine agreement among observers and accuracy of diagnosis compared with histology when diagnosing lesion location based on relationship to the meninges.
ANIMALS
Magnetic resonance images from 53 dogs with intradural extramedullary and intramedullary spinal neoplasms and 17 dogs with degenerative myelopathy.
METHODS
Six observers were supplied with 2 sets of 35 images at different time points and asked to designate lesion location. Agreement in each set was analyzed using kappa (κ) statistics. We tabulated total correct allocations and calculated sensitivity, specificity, and likelihood ratios for location designation from images compared with known histologic location for lesions confined to 1 location only.
RESULTS
Agreement in the first set of images was moderate (κ = 0.51; 95% confidence interval [CI], 0.43-0.58) and in the second, substantial (κ = 0.69; 95% CI, 0.66-0.79). In the accuracy study, 180 (75%) of the 240 diagnostic calls were correct. Sensitivity and specificity were moderate to high for all compartments, except poor sensitivity was found for intradural extramedullary lesions. Positive likelihood ratios were high for intradural extramedullary lesions and degenerative myelopathy.
CONCLUSIONS AND CLINICAL IMPORTANCE
Overall accuracy in diagnosis was reasonable, and positive diagnostic calls for intradural extramedullary lesions and negative calls for intramedullary lesions are likely to be helpful. Observers exhibited considerable disagreement in designation of lesions relationship to the meninges.
Topics: Animals; Dog Diseases; Dogs; Magnetic Resonance Imaging; Spinal Cord; Spinal Cord Diseases; Spinal Cord Neoplasms
PubMed: 34859507
DOI: 10.1111/jvim.16327 -
The Veterinary Clinics of North... Sep 2010The objective of this article is to review the recent literature that reports on the most common diseases affecting the spinal cord of cats, and to draw some general... (Review)
Review
The objective of this article is to review the recent literature that reports on the most common diseases affecting the spinal cord of cats, and to draw some general conclusions that will be useful to formulate diagnosis and prognosis for feline spinal patients. The most common types of feline spinal cord diseases documented were inflammatory/infectious diseases, and feline infectious peritonitis was the most common disease, representing approximately 50% of all feline myelitis. Neoplasms were documented in approximately 25% of cases; lymphosarcoma was the most common tumor affecting the spinal cord of cats, with reported prevalence between 28% and 40%. Cats diagnosed with spinal lymphosarcoma were significantly younger (median age 4 years) than cats with other spinal cord tumors (median age 10 years). Cats with clinical signs of intervertebral disc disease had a median age of 8 years, and 67% had Hansen type I disc protrusions. The most commonly affected intervertebral disc was at the L4 to L5 intervertebral disc space. Fibrocartilaginous embolism-affected older cats (median age 10 years), seemed to predominate in the cervicothoracic intumescence, and clinical signs were markedly lateralized, especially when the cervical region was affected.
Topics: Animals; Cat Diseases; Cats; Diagnosis, Differential; Female; Male; Myelitis; Prognosis; Spinal Cord Diseases; Spinal Cord Neoplasms
PubMed: 20732602
DOI: 10.1016/j.cvsm.2010.05.005 -
Physical Medicine and Rehabilitation... Feb 2017Spinal tumors are classically grouped into 3 categories: extradural, intradural extramedullary, and intradural intramedullary. Spinal tumors may cause spinal cord... (Review)
Review
Spinal tumors are classically grouped into 3 categories: extradural, intradural extramedullary, and intradural intramedullary. Spinal tumors may cause spinal cord compression and vascular compromise resulting in pain or neurologic compromise. They may also alter the architecture of the spinal column, resulting in spinal instability. Oncologic management of spinal tumors varies according to the stability of the spine, neurologic status, and presence of pain. Treatment options include surgical intervention, radiation therapy, chemotherapy, and hormonal manipulation. When combined with this management, rehabilitation can serve to relieve symptoms, improve quality of life, enhance functional independence, and prevent further complications in patients.
Topics: Humans; Pain Management; Quality of Life; Spinal Cord Neoplasms
PubMed: 27912991
DOI: 10.1016/j.pmr.2016.08.007 -
Modern Pathology : An Official Journal... Sep 2019Primary high-grade infiltrating gliomas of the spinal cord are rare, with prior series including limited numbers of cases and reporting poor outcomes. Additionally, the...
Primary high-grade infiltrating gliomas of the spinal cord are rare, with prior series including limited numbers of cases and reporting poor outcomes. Additionally, the molecular profile of high-grade infiltrating gliomas of the spinal cord has not been well characterized. We identified 13 adult patients whose surgery had been performed at our institution over a 26-year-period. Radiologically, nine cases harbored regions of post-contrast enhancement. Existing slides were reviewed, and when sufficient tissue was available, immunohistochemical stains (IDH1-R132H, H3-K27M, H3K27-me3, ATRX, p53 and BRAF-V600E), and a targeted 150-gene neuro-oncology next-generation sequencing panel were performed. The 13 patients included 11 men and 2 women with a median age of 38 years (range = 18-69). Histologically, all were consistent with an infiltrating astrocytoma corresponding to 2016 WHO grades III (n = 5) and IV (n = 8). By immunohistochemistry, six cases were positive for H3K27M, all showing concomitant loss of H3K27-me3. Next-generation sequencing was successfully performed in ten cases. Next-generation sequencing studies were successfully performed in four of the cases positive for H3K27M by immunohistochemistry, and all were confirmed as H3F3A K27M-mutant. Additional recurrent mutations identified included those of TERT promoter (n = 3), TP53 (n = 5), PPM1D (n = 3), NF1 (n = 3), ATRX (n = 2), and PIK3CA (n = 2). No HIST1H3B, HIST1H3C, IDH1, IDH2, or BRAF mutations were detected. Ten patients have died since first surgery, with a median survival of 13 months and 1 year of 46%. Median survival was 48.5 months for H3K27M-positive cases, compared to 1 month for those with TERT promoter mutation and 77 months for those harboring neither (p = 0.019). Median survival for cases with TP53 mutations was 11.5 months and for those with PPM1D mutations was 84 months. Our findings suggest that high-grade infiltrating gliomas of the spinal cord in adults represent a heterogeneous group of tumors, with variable outcomes possibly related to their molecular profiles.
Topics: Adolescent; Adult; Aged; Biomarkers, Tumor; Female; Glioma; Humans; Male; Middle Aged; Spinal Cord Neoplasms; Young Adult
PubMed: 31028365
DOI: 10.1038/s41379-019-0271-3 -
Journal of Neuro-oncology Jul 2016Spinal 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... (Review)
Review
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.
Topics: Adult; Cohort Studies; Ependymoma; Female; Follow-Up Studies; Humans; Male; Middle Aged; Spinal Cord; Spinal Cord Neoplasms; Young Adult
PubMed: 27154165
DOI: 10.1007/s11060-016-2135-8 -
Spinal Cord Series and Cases Nov 2021Gliofibroma is a rare tumor that develops in the brain and spinal cord. Due to the rarity of its nature, its pathophysiology and appropriate treatment remain elusive. We...
INTRODUCTION
Gliofibroma is a rare tumor that develops in the brain and spinal cord. Due to the rarity of its nature, its pathophysiology and appropriate treatment remain elusive. We report a case of intramedullary spinal cord gliofibroma that was surgically treated multiple times. This report is of great significance because this is the first case of recurrence of this tumor.
CASE PRESENTATION
A 32-year-old woman complained of gait disturbance and was referred to our institution. At the age of 13 years, she was diagnosed with intramedullary gliofibroma and underwent gross total resection (GTR) in another hospital. Based on imaging findings, tumor recurrence was suspected at the level of cervical spinal cord, and surgery was performed. However, the resection volume was limited to 50% because the boundary between the tumor and spinal cord tissue was unclear and intraoperative neuromonitoring alerted paralysis. At 1 year postoperatively, the second surgery was performed to try to resect the residual tumor, but subtotal resection was achieved at most. At 2 years after the final surgery, no tumor recurrence was observed, and neurologic function was maintained to gait with cane.
DISCUSSION
Although complete resection is desirable for this rare tumor at the initial surgery, there is a possibility to recur even after GTR with long-term follow-up. During surgical treatment for tumor recurrence, fair adhesion to the spinal cord is expected, and reoperation and/or adjuvant therapy might be considered in the future if the tumor regrows and triggers neurological deterioration.
Topics: Adolescent; Adult; Astrocytoma; Female; Humans; Neurosurgical Procedures; Spinal Cord Neoplasms; Treatment Outcome
PubMed: 34741004
DOI: 10.1038/s41394-021-00461-y -
JPMA. the Journal of the Pakistan... Sep 2023Spinal subependymoma (SSE) is a rare intramedullary, benign tumour. Surgical excision isthe preferred approach. However, the interwoven pattern of neural tissue within... (Review)
Review
Spinal subependymoma (SSE) is a rare intramedullary, benign tumour. Surgical excision isthe preferred approach. However, the interwoven pattern of neural tissue within the tumour dictates the extent of resection. Where gross total resection is linked with possible neurological deficits, subtotal resection or close observation may support better functional outcomes. The evidence for the management of SSE is based mostly on case reports. Herein, we review the existing literature regarding treatment options and clinical outcomes of spinal subependymoma.
Topics: Humans; Glioma, Subependymal; Spinal Cord Neoplasms; Neurosurgical Procedures
PubMed: 37817716
DOI: 10.47391/JPMA.23-66