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1.
World Neurosurg ; 181: e1001-e1011, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37956902

RESUMEN

OBJECTIVE: The aim of this study, a retrospective database analysis, was to assess the impact of baseline cannabis use disorder (CUD) on perioperative complication outcomes in patients undergoing primary 1- to 2-level anterior cervical diskectomy and fusion (ACDF) surgery. METHODS: The PearlDiver Database was queried from January 2010 to December 2021 for patients who underwent primary 1- to 2-level ACDF surgery for degenerative spine disease. Patients with CUD diagnosis 6 months before the index ACDF surgery (i.e., CUD) were propensity matched with patients without CUD (i.e., control in a ratio of 1:1, employing age, gender, and Charlson Comorbidity Index as matching covariates). Univariate and multivariable analysis models with adjustment of confounding variables were used to evaluate the risk of CUD on perioperative complications between the propensity-matched cohorts. RESULTS: The 1:1 matched cohort included 838 patients in each group. Following multivariate analysis, CUD was demonstrated to be associated with an increased incidence of hospital readmission at 90 days (odds ratio [OR] = 2.64, 95% confidence interval: [1.19 to 6.78], [P = 0.027]) and revision surgery at 1 year postoperative (OR = 3.36, 95% confidence interval: [1.17 to 14.18], [P = 0.049]). CUD was additionally associated with reduced risk of overall medical complications at both 6 months and 1 year postoperative (OR = 0.55, [P = 0.021], and OR = 0.54, [P = 0.015], respectively). CONCLUSIONS: These findings indicate that isolated baseline CUD is associated with an increased risk of hospital readmission at 90 days postoperative and cervical spine reoperation at 1 year after primary 1- to 2-level ACDF surgery with a decrease in overall medical complications, cardiac arrhythmias, and acute renal failure.


Asunto(s)
Abuso de Marihuana , Fusión Vertebral , Trastornos Relacionados con Sustancias , Humanos , Estudios Retrospectivos , Fusión Vertebral/efectos adversos , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Vértebras Cervicales/cirugía , Discectomía/efectos adversos , Trastornos Relacionados con Sustancias/complicaciones , Abuso de Marihuana/complicaciones , Abuso de Marihuana/cirugía
2.
Transl Cancer Res ; 12(5): 1325-1334, 2023 May 31.
Artículo en Inglés | MEDLINE | ID: mdl-37304540

RESUMEN

Background: Primary spinal anaplastic meningioma (PSAM) is a very rare entity in the spinal canal. Therefore, the clinical features, treatment strategy, and long-term outcomes remain poorly studied. Case Description: Clinical data of six patients with PSAM treated at one single institution were retrospectively analyzed and all previously reported cases in the English literature were reviewed. There were three male and three female patients with a median age of 25 years. The duration of symptoms before initial diagnosis ranged from one week to one year. PSAMs occurred at cervical level in four, cervicothoracic in one and thoracolumbar in one. In addition, PSAMs presented isointensity on T1 weighted imaging (WI), hyperintensity on T2WI, and hetero- or homogeneously marked enhancement with contrast. Eight operations were performed in six patients. Simpson II resection was achieved in four (50%), Simpson IV in three (37.5%), Simpson V in one (12.5%). Adjuvant radiotherapy was performed in five patients. With a median survival time of 14 months (4-136 months), three patients had recurrence, two experienced metastases, and four died of respiratory failure. Conclusions: PSAMs are a rare disease, and there is limited evidence as to the management of these lesions. They may metastasize, recur, and portend a poor prognosis. A close follow-up and further investigation are therefore necessary.

3.
J Clin Neurosci ; 113: 77-85, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-37224612

RESUMEN

BACKGROUND: Syringo-subarachnoid shunt (SSS) is often considered a rescue procedure or a second-line treatment option for syringomyelia. However, the clinical efficacy of SSS in treating this condition remains controversial. OBJECTIVE: To evaluate the long-term outcome of the SSS and its relationship with the syrinx area, as well as to investigate the factors that influence the prognosis. METHODS: This retrospective study included twenty-seven consecutive patients who underwent SSS between 2014 and 2020. The study evaluated several independent variables such as age, sex, duration of progressive symptoms, morphological characteristics of the syrinx, changes in the syrinx area, and Chiari malformation. The long-term follow-up (>2 years) Japanese Orthopaedic Association (JOA) score was used to assess neurological function and outcome. Statistical analysis was performed using a stepwise logistic regression test. RESULTS: All patients were followed up for an average of 48.6 ± 14.8(26.8 to 78.0) months. Follow-up magnetic resonance imaging showed syrinx collapse to different degrees occurred in 96.3% (26 of 27) patients. The JOA score was improvedinonly6patients (22.2%), remained stable in 5 patients (18.5%),and deteriorated in 16 patients(59.3%).A logistic regression test showed that the tension syrinx (odds ratio 0.111) and early shunting intervention (odds ratio 0.138) were favorable independent prognostic factors. CONCLUSIONS: It is important to note that the shrinkage of the syrinx does not necessarily translate to an improvement in clinical outcomes. Therefore, the decision to perform SSS should be made with caution and considered as a last resort.


Asunto(s)
Malformación de Arnold-Chiari , Anomalías Cardiovasculares , Siringomielia , Humanos , Siringomielia/diagnóstico por imagen , Siringomielia/cirugía , Pronóstico , Estudios Retrospectivos , Derivaciones del Líquido Cefalorraquídeo/métodos , Malformación de Arnold-Chiari/complicaciones , Malformación de Arnold-Chiari/diagnóstico por imagen , Malformación de Arnold-Chiari/cirugía , Imagen por Resonancia Magnética/métodos , Resultado del Tratamiento
4.
Eur Spine J ; 32(7): 2459-2467, 2023 07.
Artículo en Inglés | MEDLINE | ID: mdl-37027035

RESUMEN

PURPOSE: Myxopapillary ependymoma (MPE) was classified as grade 2 tumor in the 2021 World Health Organization central nervous system classification because of its high recurrence probability. This study aimed to investigate predictive factors and management of tumor recurrence. METHODS: Seventy-two patients with spinal MPE underwent initial surgical treatment at our hospital between 2011 and 2021. Kaplan-Meier curves and Cox regression were used to analyze the correlation between clinical variables and progression-free survival (PFS). RESULTS: The median age at diagnosis was 33.5 years (range 8-60 years). Twenty-one patients (29.2%) had preoperative spinal drop metastases. Gross total resection (GTR) was performed in 37 patients (51.4%). The median follow-up was 7.2 years, and the follow-up rate was 88.9% (64 of 72 cases). Twelve of the 64 patients (18.9%) relapsed, and preoperative drop metastasis occurred in 7 patients (58.3%). The estimated 5-year and 10-year PFS rates were 82% and 77%, respectively. Univariate analysis showed that GTR was associated with improved PFS (hazard ratio [HR] 0.149, p = 0.014), while preoperative drop metastasis (HR 3.648, p = 0.027) and tumor involvement sacrococcygeal region (HR 7.563, p = 0.003) were associated with tumor recurrence. Adjuvant radiotherapy (RT) was significantly associated with improved PFS in patients with preoperative drop metastasis (p = 0.039). CONCLUSION: Complete surgical resection under the premise of protecting neurological function is an important factor in reducing spinal MPE recurrence. Adjuvant RT is recommended when the tumor invades the capsule with preoperative drop metastasis or adhesion to the nerve and cannot reach GTR.


Asunto(s)
Ependimoma , Neoplasias de la Médula Espinal , Humanos , Niño , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Recurrencia Local de Neoplasia/epidemiología , Pronóstico , Neoplasias de la Médula Espinal/patología , Radioterapia Adyuvante , Ependimoma/cirugía , Estudios Retrospectivos
5.
Neurosurg Focus ; 54(3): E5, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36857791

RESUMEN

OBJECTIVE: Foramen magnum decompression with duraplasty (FMDD) is one of the most frequently utilized surgical treatments for Chiari malformation type I (CMI) in adults. However, its long-term outcomes remain controversial. The object of this study was to evaluate the long-term outcomes of FMDD in adults with CMI. METHODS: In total, 297 adults with CMI who had undergone FMDD at the authors' institution between 2011 and 2020 were included in this retrospective study. Long-term (> 1 year) outcomes were evaluated using the Chicago Chiari Outcome Scale (CCOS), visual analog scale (VAS), and Japanese Orthopaedic Association (JOA) scale. RESULTS: The median patient age was 44 years (range 18-65 years). The mean clinical follow-up period was 67 months (range 14-123 months). Compared with preoperative conditions, the postoperative syringomyelia regression rate was 91.3% (242/265), and the cerebellar tonsil ascended in 18.2% of patients (54/297), was stable in 64.3% (191/297), and continuously descended in 17.5% (52/297). Long-term clinical follow-up data were acquired from 267 patients. According to the CCOS score, the patient's condition improved in 62.5% of cases (167/267), was stable in 31.8% (85/267), and worsened in 5.6% (15/267). According to the VAS score, the patient's condition improved in 59.5% of cases (110/185), remained unchanged in 27.6% (51/185), and worsened in 13.0% (24/185) among the follow-up patients with relevant data. According to the JOA score, the patient's condition improved in 40.1% of cases (107/267), remained unchanged in 50.2% (134/267), and worsened in 9.7% (26/267). Notably, there was no association between clinical outcomes and syringomyelia regression (p = 0.227) or changes in the cerebellar tonsillar position (p = 0.323). CONCLUSIONS: FMDD is a simple, safe, and effective surgical procedure for adult CMI that yields significant and sustained improvement in clinical and radiological outcomes. However, clinical improvement does not always correlate with syringomyelia regression and cerebellar tonsillar shift.


Asunto(s)
Malformación de Arnold-Chiari , Siringomielia , Humanos , Adulto , Adolescente , Adulto Joven , Persona de Mediana Edad , Anciano , Foramen Magno , Estudios Retrospectivos , Descompresión
6.
Front Oncol ; 13: 1083085, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36824145

RESUMEN

Objective: Primary spinal anaplastic ependymoma (PSAE) is an extremely rare disease. We aim to report the largest PSAE cohort, evaluate the treatments, and investigate the prognostic factors for progression-free survival (PFS). Methods: Clinical data collected from the authors' institute and literature articles were pooled and described. Survival analysis and multivariable Cox regression analysis were performed to evaluate therapies and investigate prognostic factors for PFS. Results: Our cohort included 22 females and 16 males, with a median age of 33 years. PSAE developed mostly on cervical and cervicothoracic levels. The median length measured 3 segments. Half of PSAE were intramedullary. Pain was the most common symptom. The median duration of symptoms was 6 months. Neurological statuses were improved in 76% following treatments, whereas clinical tumor progression occurred in 41.7%. The estimated median progression-free survival was 132 months, and the estimated median survival was 192 months. The median Ki-67 index was 15%. Patients aged less than or equal to 25 experienced worse neurological statuses and more repeated progression. Age less than or equal to 25 (HR 10.312, 95%CI 1.535-69.260, p=0.016), gross total resection (HR 0.116, 95%CI 0.020-0.688, p=0.018), and radiotherapy (HR 0.084, 95%CI 0.009-0.804, p=0.032) are three prognostic factors for tumor progression. Conclusion: Tumor progression remains a big concern in the clinical course of PSAE. Being aged above 25, undergoing GTR, and accepting adjuvant radiotherapy put patients at lower risk for tumor progression. Younger patients might have worse neurological statuses compared with those aged over 25.

7.
BMC Neurol ; 22(1): 63, 2022 Feb 22.
Artículo en Inglés | MEDLINE | ID: mdl-35193508

RESUMEN

BACKGROUND: Neurocysticercosis is a neuroinfectious disease caused by the larval stage of the tapeworm Taenia solium. Isolated spinal cysticercosis is rare, with limited cases having been reported in the literature. This entity poses great diagnostic and therapeutic challenges. METHODS: This retrospective study included seven patients pathologically diagnosed with spinal cysticercosis. The clinical manifestations, radiological features on magnetic resonance imaging (MRI), treatment, and outcomes were analyzed. RESULTS: This case series consisted of four male and three female patients, with an average age of 34.9 ± 10.9 years. Clinically, six patients manifested with localization-related myelopathy. There were four solid lesions, one cystic-solid lesion, and three cystic lesions. The solid and cystic-solid lesions showed characteristic MRI features: 1) within the lesion, there was a mural nodule with isointensity on T1WI and iso- to hyperintensity on T2WI; 2) the signals at the periphery of the mural nodule were variable, ranging from hypointense to hyperintense on T2WI; and 3) ring-like or cyst wall enhancement could be present, and dot-like enhancement could be noted in the mural nodule. Complete resection of the responsible lesion was achieved in all patients, and oral albendazole was administered in a patient with one more suspected homologous lesion. After a mean follow-up period of 56.7 ± 35.1 months, the patient's symptoms mostly regressed. CONCLUSION: Spinal cysticercosis is an extremely rare cause of myelopathy. Characteristic MRI features can facilitate preoperative diagnosis. Clinicians should be aware of this entity, and it should be included in the differential diagnosis of myelopathy.


Asunto(s)
Cisticercosis , Neurocisticercosis , Enfermedades de la Médula Espinal , Adulto , Cisticercosis/diagnóstico , Cisticercosis/diagnóstico por imagen , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Neurocisticercosis/complicaciones , Neurocisticercosis/diagnóstico por imagen , Estudios Retrospectivos , Enfermedades de la Médula Espinal/complicaciones , Enfermedades de la Médula Espinal/diagnóstico por imagen , Columna Vertebral , Adulto Joven
8.
Neurospine ; 19(1): 65-76, 2022 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-35130420

RESUMEN

OBJECTIVE: Intramedullary spinal cord metastasis from lung cancer (ISCM-LC) are increasing in prevalence. We aim to investigate its clinical features, treatments and prognosis. METHODS: We reported 6 ISCM-LC cases and conducted a systematic review. Descriptive summarization, survival analysis, and multivariate Cox regression analysis were performed to comprehensively study the disease. RESULTS: All 6 patients had surgery. One used chemotherapy and the other had targeted drugs. Two patients died of ISCM-LC, 1 died of pulmonary embolism, 1 was alive, and 2 were lost to follow-up. We identified 197 ISCM-LC cases in literature with a mean age of 58 years and male preponderance. Small cell lung cancer accounted for 39.1%. The median interval from lung cancer to ISCM-LC was 7 months. Limb weakness was the most common symptom, and 45% cases progressed rapidly. Concomitant brain, leptomeningeal, and vertebral metastasis occurred to 55.8%, 20%, and 19.5%, respectively. Peritumoral edema appeared in 83.3%. Through survival analysis, we found sex, extraspinal metastasis, pathology, and improved symptoms affected the overall survival. Additionally, gross total resection (GTR) shared similar effectiveness with non-GTR, and other treatments following surgery hardly added extra effect. Surgery, improved symptoms, and sex were 3 independent prognostic factors after adjusting for confounding. The estimated median survival time was 5 months. CONCLUSION: The overall survival of ISCM-LC remains poor. Surgery is an independent protective factor for survival. Surgery should be considered once tolerated, and GTR might not be necessary. In addition, female patients with improved symptoms after intervention might have better overall survival.

9.
J Neurophysiol ; 126(5): 1740-1750, 2021 11 01.
Artículo en Inglés | MEDLINE | ID: mdl-34644182

RESUMEN

We reported that a high level of autophagy was initiated by oxygen-glucose deprivation (OGD) and was maintained in neurons even after oxygen-glucose deprivation followed by reoxygenation (OGD/R), accompanied by neuronal apoptosis. This study focused on autophagy-induced apoptosis and its signaling network, especially the role of endoplasmic reticulum stress (ERS). Analysis of primary cultured cortical neurons from mice showed that the autophagy-induced apoptosis depended on caspase-8 and -9 but not on caspase-12. This finding did not mean that the endoplasmic reticulum did not participate in this process. Increases in the levels of endoplasmic reticulum (ER) biomarkers and binding immunoglobulin protein (BiP) were induced by autophagy in OGD/R-treated neurons. In addition, as an apoptotic transcription factor induced by ER stress, C/EBP homologous protein (CHOP) expression was significantly increased in neurons after OGD/R. This result suggested that the autophagy-BiP-CHOP-caspase (8 and 9)-dependent apoptotic signaling pathway at least partly participated in autophagy-induced apoptosis in primary cortical neurons. It revealed that ER induced apoptosis in neurons suffering from OGD/R injury in an ER stress-CHOP-dependent manner rather than a caspase-12-dependent manner. However, more research on signaling or cross-linking networks and intermediate links is needed. The realization of caspase-12-independent BiP-CHOP neuronal apoptosis pathway has expanded our understanding of the neuronal apoptosis network, which may eventually provide endogenous interventional strategies for OGD/R injury after stroke.NEW & NOTEWORTHY ER stress induced by autophagy mediates caspase-8- and caspase-9-dependent apoptosis pathways by regulating CHOP in neurons exposed to OGD/R. We hypothesized that the autophagy-BiP-CHOP-caspase (8 and 9)-dependent apoptotic signaling pathway at least partly participated in autophagy-induced apoptosis in primary cortical neurons.


Asunto(s)
Apoptosis/fisiología , Autofagia/fisiología , Caspasa 12/metabolismo , Corteza Cerebral/metabolismo , Estrés del Retículo Endoplásmico/fisiología , Neuronas/metabolismo , Factor de Transcripción CHOP/metabolismo , Animales , Células Cultivadas , Glucosa/metabolismo , Ratones , Oxígeno/metabolismo
10.
Neurosurgery ; 89(2): 300-307, 2021 07 15.
Artículo en Inglés | MEDLINE | ID: mdl-34015818

RESUMEN

BACKGROUND: "Diffuse midline glioma, H3 K27M-mutant" (DMG) mainly arises within the pontine, thalamic, and spinal cord regions. Because of the rarity of spinal cord gliomas, the general knowledge surrounding DMGs is mainly based on pontine and thalamic gliomas, whereas tumor location tends to influence the clinicopathological features and prognosis. OBJECTIVE: To determine the clinicopathological characteristics and molecular profiles of DMGs located in the spinal cord. METHODS: The clinical and molecular pathologic features and prognosis were comprehensively analyzed in a series of 44 patients with spinal cord DMGs. RESULTS: The median age was 36 yr, and 88.7% of patients (39/44) were adults (≥18 yr). Histopathologically, malignant grades included grade II (16 cases), grade III (20 cases), and grade IV (8 cases). Compared with patients with histological grade IV, patients with lower histological grade (grade II/III) were older (37 vs 24 yr, P = .020) and were associated with longer overall survival (24.1 vs 8.6 mo, P = .007). All 30 tested tumors were isocitrate dehydrogenase (IDH) wild type, and 96% of cases (22/23) presented with unmethylated O6-methylguanine-DNA methyltransferase. Univariate and multivariate analyses showed that histological grade and presurgery McCormick Scale scores were independent prognostic factors for overall survival, whereas extensive surgical resection and chemoradiotherapy were not significantly associated with improved survival. The most frequent anatomic locations were the cervical enlargement (C4-T1, n = 16) and conus medullaris (T12-L1, n = 13), which exhibited distinctive clinical characteristics and molecular features. CONCLUSION: The findings provide guidelines for the evidence-based practice of the specialized management of spinal cord DMGs.


Asunto(s)
Neoplasias Encefálicas , Glioma , Adulto , Glioma/diagnóstico , Glioma/genética , Glioma/terapia , Histonas/genética , Humanos , Mutación/genética , Pronóstico , Médula Espinal
11.
Neurosurg Rev ; 44(3): 1665-1673, 2021 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32767042

RESUMEN

Ependymomas occurring in the upper cervical spinal cord (above the level of the C4 segment) are rare entities with great therapeutic challenges. This study was aimed to investigate the clinicoradiological characteristics and the prognosis in a large cohort of upper cervical ependymomas from a single institution. This retrospective study enrolled 155 patients with primary ependymomas in the upper cervical spinal cord. The pre- and post-operative clinical and magnetic resonance imaging profiles were collected. The neurological outcomes and survival events were evaluated, and potential independent risk factors were analyzed. There were 82 females and 73 males, with an average age of 43.1 ± 11.3 years. Immediately post-operatively, 118 (76.1%) patients experienced neurological deterioration and 32 (20.7%) patients remained unchanged. Three months after surgery, 61 (39.4%) patients showed deteriorated neurological functions compared to the pre-operative baseline levels. After an average follow-up period of 56.0 ± 24.7 months, the neurological functions were worse than the baseline status in 37 (23.9%) patients and improved in 33 (21.3%) patients, respectively. Logistic regression analysis identified that lower age (≤ 42 years) and lower pre-operative MMS (I-II) were independent protective factors for predicting favorable neurological functions. Multivariate Cox regression analysis revealed that incomplete resection was the only independent risk factor associated with a shorter progression-free survival. Age and pre-operative functional status affect the long-term neurological outcomes, and incomplete resection was associated with a shorter survival. Our findings indicate that gross total resection should be the goal of surgical treatment of upper cervical ependymomas.


Asunto(s)
Médula Cervical/diagnóstico por imagen , Médula Cervical/cirugía , Ependimoma/diagnóstico por imagen , Ependimoma/cirugía , Neoplasias de la Médula Espinal/diagnóstico por imagen , Neoplasias de la Médula Espinal/cirugía , Adulto , Anciano , Estudios de Cohortes , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Procedimientos Neuroquirúrgicos/métodos , Estudios Retrospectivos , Factores de Riesgo , Resultado del Tratamiento , Adulto Joven
12.
Cancer Med ; 9(19): 6996-7006, 2020 10.
Artículo en Inglés | MEDLINE | ID: mdl-32777166

RESUMEN

BACKGROUND: Due to their rarity, the clinicopathological characteristics and prognostic factors of spinal cord gliomas are still unclear. Here, we aimed to clarify these issues in a cohort of 108 spinal cord astrocytomas. METHODS: We characterized the clinicopathological characteristics, including 2016 World Health Organization (WHO) grade, age, sex, location, segment length, resection, pre- and postsurgery, Modified McCormick Scale (MMS), radio- and chemotherapy, and Ki-67 and H3 K27M mutations, in 108 spinal cord astrocytomas through heatmaps. The Cox regression analysis and Kaplan-Meier curves were used to study the prognostic value of these clinicopathological features. RESULTS: There are a total 38 H3 K27M-mutant tumors, including 31 cases with histological grade II/III tumors. The age of low-grade astrocytoma patients (WHO grade I/II, n = 54) was significantly younger (27.0 vs 35.5 years, P = .001) than those with high-grade tumors (WHO grade III/IV, n = 54). All patients underwent surgical resection with neurophysiological monitoring, and the surgery did not result in significant changes in MMS. The presurgery MMS was associated with overall survival in the high-grade subgroup (P = .008) but not in the low-grade subgroup (P = .312). While, the high content of resection improved the survival of only patients with low-grade astrocytomas (P = .016) but not those with high-grade astrocytomas (P = .475). Both the low-grade and high-grade astrocytomas had no obvious benefit from neither adjuvant chemotherapy nor radiotherapy (all P > .05). CONCLUSIONS: We characterized the clinicopathological characteristics and their prognostic values in 108 spinal cord astrocytomas, which could help with evidence-based management of spinal cord astrocytomas.


Asunto(s)
Astrocitoma/cirugía , Procedimientos Neuroquirúrgicos , Neoplasias de la Médula Espinal/cirugía , Adolescente , Adulto , Astrocitoma/genética , Astrocitoma/mortalidad , Astrocitoma/patología , Biomarcadores de Tumor/genética , Quimioterapia Adyuvante , Niño , Preescolar , Femenino , Histonas/genética , Humanos , Masculino , Persona de Mediana Edad , Mutación , Clasificación del Tumor , Procedimientos Neuroquirúrgicos/efectos adversos , Procedimientos Neuroquirúrgicos/mortalidad , Radioterapia Adyuvante , Estudios Retrospectivos , Neoplasias de la Médula Espinal/genética , Neoplasias de la Médula Espinal/mortalidad , Neoplasias de la Médula Espinal/patología , Factores de Tiempo , Resultado del Tratamiento , Adulto Joven
13.
Onco Targets Ther ; 13: 4649-4663, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32547095

RESUMEN

INTRODUCTION: Chordoma is a malignant tumor predominantly involving the skull base and vertebral column. This study aimed to investigate the molecular characteristics of PTEN and CDKN2A in conventional and chondroid chordomas and their correlation with clinical prognosis. MATERIALS AND METHODS: A total of 42 patients were enrolled, including 26 patients with conventional chordoma and 16 patients with chondroid chordoma. Clinicopathological profiles and tissue specimens were collected. Gene sequencing and fluorescence in situ hybridization were performed to identify genetic alterations in the PTEN and CDKN2A genes. Immunohistochemical staining was used for semiquantitative evaluation of PTEN and CDKN2A expression. RESULTS: Gene sequencing revealed an intron SNP (c.80-96A>G) and a missense mutation (c.10G>A; p.Gly4Arg) in the PTEN gene and a missense mutation (c.442G>A; p.Ala148Thr) in the CDKN2A gene. Loss of the PTEN locus was identified in 25 (59.5%) cases, and loss of the CDKN2A locus was found in 28 (66.7%) cases. There was no significant correlation between progression-free survival (PFS)/overall survival (OS) and loss of PTEN or CDKN2A. The patients with lower PTEN expression showed significantly shorter PFS and OS than those with higher expression, while there was no significant difference in PFS or OS between patients with lower CDKN2A expression and those with higher CDKN2A expression. CONCLUSION: Our findings delineated the genetic landscape and expression of PTEN and CDKN2A in chordomas. PTEN expression may serve as a prognostic and predictive biomarker for chordomas.

14.
Oncogene ; 39(24): 4711-4727, 2020 06.
Artículo en Inglés | MEDLINE | ID: mdl-32404987

RESUMEN

Chordoma, a rare neoplasm derived from intraosseous notochordal remnants, is unresponsive to conventional chemotherapy and radiotherapy. Sonic Hedgehog (Shh) is a crucial fetal notochord-secreted morphogen that directs notochordal development. The aim of this study was to determine the functional roles and therapeutic potential of Shh-Gli1 signaling in chordomas. Tissue samples and clinical profiles were collected from 42 patients with chordoma. The chordoma cell lines U-CH1 and MUG-Chor1 were used for functional experiments. Shh-Gli1 signaling pathway genetic alterations were screened, and the functions of the identified novel variants were analyzed using in silico analyses, real-time quantitative PCR, and minigene assays. Ligand-dependent Shh-Gli1 signaling activation was assessed using single- and dual-label immunostaining, western blot analysis, and a Shh-responsive Gli-luciferase reporter assay. The small-molecule inhibitor vismodegib was used to target Shh-Gli1 signaling in vitro and in vivo. Overall, 44 genetic alterations were identified, including four novel variants (c.67_69dupCTG in SMO, c.-6_-4dupGGC and c.3306 + 83_3306 + 84insG in PTCH1, and c.183-67_183-66delinsA in SUFU). Shh, PTCH1, SMO, SUFU, and Gli1 were extensively expressed in chordomas, and higher Gli1 expression correlated with poorer prognosis. A luciferase reporter assay and dual-label immunostaining indicated the occurrence of juxtacrine ligand-dependent Shh-Gli1 signaling activation. Vismodegib significantly inhibited cell proliferation and induced apoptosis and G1/S cell cycle arrest. In vivo investigation demonstrated that vismodegib effectively inhibited chordoma xenograft growth. This current preclinical evidence elucidates the therapeutic potential of Shh-Gli1 signaling pathway targeting for chordoma treatment. Vismodegib may be a promising targeted agent, and further clinical trials are warranted.


Asunto(s)
Anilidas/farmacología , Cordoma , Sistemas de Liberación de Medicamentos , Regulación Neoplásica de la Expresión Génica , Proteínas Hedgehog , Piridinas/farmacología , Transducción de Señal , Proteína con Dedos de Zinc GLI1 , Animales , Línea Celular Tumoral , Cordoma/tratamiento farmacológico , Cordoma/genética , Cordoma/metabolismo , Cordoma/patología , Femenino , Puntos de Control de la Fase G1 del Ciclo Celular/efectos de los fármacos , Puntos de Control de la Fase G1 del Ciclo Celular/genética , Proteínas Hedgehog/genética , Proteínas Hedgehog/metabolismo , Humanos , Masculino , Ratones , Ratones Endogámicos NOD , Ratones SCID , Mutación , Células 3T3 NIH , Puntos de Control de la Fase S del Ciclo Celular/efectos de los fármacos , Puntos de Control de la Fase S del Ciclo Celular/genética , Transducción de Señal/efectos de los fármacos , Transducción de Señal/genética , Ensayos Antitumor por Modelo de Xenoinjerto , Proteína con Dedos de Zinc GLI1/genética , Proteína con Dedos de Zinc GLI1/metabolismo
15.
World Neurosurg ; 137: 235-238, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-32081826

RESUMEN

BACKGROUND: Subependymoma in the spinal cord is very rare and usually occurs in the cervical cord. We report an exceptional case of subependymoma that occurred at the conus medullaris with cystic formation. This article reviews the literature on subependymoma in the conus medullaris; discusses its clinical manifestations, imaging findings, and differential diagnoses; and offers an opinion about the cystic formation of the subependymoma. CASE DESCRIPTION: A 69-year-old woman experienced progressive limb weakness with a somatosensory abnormality for 3 months. Preoperative magnetic resonance imaging showed a cystic intramedullary lesion at the conus medullaris with a well-defined margin. A preliminary diagnosis of epidermoid cyst was made based on the imaging findings. During the operation, cystic formation of the tumor was found, and the tumor was completely removed. Pathology showed an uneven proliferation of glial cells, consistent with subependymal morphology, and the tumor was confirmed as subependymoma. CONCLUSIONS: We present an extremely rare case of cystic formation in subependymoma at the conus medullaris. Subependymoma should be included in the differential diagnosis of intramedullary cystic lesions. The breakdown of the blood-brain barrier and excessive extravasation may be potential mechanisms of cystic formation.


Asunto(s)
Glioma Subependimario/cirugía , Neoplasias de la Médula Espinal/cirugía , Médula Espinal/patología , Médula Espinal/cirugía , Anciano , Barrera Hematoencefálica/diagnóstico por imagen , Barrera Hematoencefálica/patología , Diagnóstico Diferencial , Quiste Epidérmico/diagnóstico por imagen , Quiste Epidérmico/cirugía , Femenino , Glioma Subependimario/diagnóstico por imagen , Glioma Subependimario/patología , Humanos , Imagen por Resonancia Magnética , Neuroglía/patología , Neoplasias de la Médula Espinal/diagnóstico por imagen , Neoplasias de la Médula Espinal/patología
16.
World Neurosurg ; 137: e570-e576, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-32081827

RESUMEN

BACKGROUND: Surgical resection of intramedullary tumors remains technically challenging. The role of intraoperative ultrasound and contrast-enhanced ultrasound (CEUS) in these surgeries has not yet been well defined. This study was aimed to evaluate the potential of intraoperative ultrasonography, especially CEUS, in visualizing intramedullary spinal cord tumors, and to assess the values for improving surgical outcomes. METHODS: This prospective study recruited 14 patients with intramedullary spinal cord tumor. All patients underwent tumor resection with intraoperative ultrasound and CEUS. The first ultrasonographic scanning was performed before the dural incision. After the dural incision, further ultrasonographic scanning was performed over the surface of the spinal cord. During and after the tumor removal, repeat ultrasonographic scanning was conducted to assess the extent of surgical resection. Follow-up data were collected and analyzed. RESULTS: Seven patients had small tumors involving 1 or 2 spinal segments, and 7 patients showed relatively large tumors involving ≥3 spinal segments. Intraoperatively, ultrasonography was able to visualize the lesion clearly in all cases. Using intraoperative ultrasound with CEUS, the tumor boundaries were clearly defined. After an average follow-up period of 15.93 months, neurologic function improved in 10 cases (71.4%) and remained unchanged in 4 cases (28.6%); there was no deterioration in individual neurologic function. CONCLUSIONS: Intraoperative ultrasound is of great value for localizing lesions and determining the extent of dural opening and myelotomy, therefore reducing the invasiveness of spinal surgery for intramedullary tumors. CEUS helps to delineate the tumor margins and perfusion features.


Asunto(s)
Monitoreo Intraoperatorio/métodos , Neoplasias de la Médula Espinal/cirugía , Neoplasias de la Columna Vertebral/cirugía , Ultrasonografía , Adulto , Medios de Contraste , Femenino , Humanos , Masculino , Persona de Mediana Edad , Procedimientos Neuroquirúrgicos , Estudios Prospectivos , Neoplasias de la Médula Espinal/diagnóstico por imagen , Neoplasias de la Columna Vertebral/diagnóstico por imagen , Adulto Joven
17.
Neurosurgery ; 86(4): 548-556, 2020 04 01.
Artículo en Inglés | MEDLINE | ID: mdl-31081882

RESUMEN

BACKGROUND: Limited data existed to guide the management of intraspinal neurenteric cysts (ISNECs). OBJECTIVE: To evaluate the risk factors for progression-free survival (PFS), elucidate the radiological features of ISNECs, and propose a treatment protocol. METHODS: From 2003 to 2015, 121 patients with pathologically confirmed ISNECs treated at our institute were included in this study. Pertinent risk factors were evaluated. RESULTS: Gross total resection (GTR) was achieved in 55 (44.6%) patients; 106 (87.6%), 12 (9.9%), and 3 (2.5%) ISNECs were classified as Wilkins A, B, and C, respectively. After a median follow-up duration of 64.2 mo, recurrence occurred in 25 (22.7%) patients, with a median PFS time of 43.1 mo. The actuarial PFS rates at 5 and 10 yr were 73.2% and 66.2%, respectively. The actuarial overall survival rates at 5 and 10 yr were 100% and 97.6%, respectively. Non-GTR (hazard ratio [HR], 5.836; 95% confidence interval [CI], 1.698-20.058; P = .005), Wilkins B/C (HR, 3.129; 95% CI, 1.009-9.702; P = .048), and a history of surgical resection (HR, 3.690; 95% CI, 1.536-8.864; P = .004) were adverse factors. CONCLUSION: GTR and Wilkins A were favorable factors for PFS. If tolerable, GTR alone was advocated as an optimal treatment. Because of the benign nature and favorable prognosis, non-GTR was an alternative if GTR failed. Close follow-up was needed because of the recurrent tendency of ISNEC. Future study with a large cohort is necessary to verify our findings.


Asunto(s)
Defectos del Tubo Neural/mortalidad , Defectos del Tubo Neural/patología , Defectos del Tubo Neural/cirugía , Adolescente , Adulto , Niño , Estudios de Cohortes , Femenino , Humanos , Masculino , Procedimientos Neuroquirúrgicos/métodos , Pronóstico , Supervivencia sin Progresión , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia , Adulto Joven
18.
Neurosurg Rev ; 43(5): 1351-1356, 2020 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31473875

RESUMEN

Spinal myxopapillary ependymoma (SME) is a rare pathological variant of ependymoma, which most commonly occurs in the cauda equina and filum terminale of the spinal cord. Although SME is considered as a benign entity, histologically corresponding to WHO grade I, local recurrence and metastasis have been reported in many cases. The purpose of this large-scale, single-center study was to investigate the clinical characteristics and surgical outcomes of SME. A total of 34 consecutive patients diagnosed with SME were enrolled in this retrospective study. All patients underwent magnetic resonance imaging (MRI) and were treated with surgical resection. Individual clinical data were collected, and surgical outcomes were evaluated during the follow-up period. There were 21 males and 13 females, with an average age of 29.97 years. Clinical symptoms included back pain (82.4%), weakness (44.1%) and numbness (20.6%) in extremities, and sphincter dysfunction (26.5%). The tumor locations included lumbar segments (52.9%), thoracolumbar segments (23.5%), lumbosacral segments (17.6%), and thoraco-lumbo-sacral segments (5.9%). On MRI, all SMEs appeared hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging, with remarkable enhancement. Gross total resection was achieved in 18 patients, and subtotal resection (STR) was achieved in 16 patients. After an average follow-up period of 41.5 months, recurrence was noted in 5 patients (5 of 16) who underwent STR. SMEs have some characteristic features on MRI which can facilitate the preoperative diagnosis. Complete surgical resection is the best treatment of choice with a favorable outcome. In cases of incomplete resection, postoperative radiotherapy may be an effective alternative.


Asunto(s)
Ependimoma/cirugía , Procedimientos Neuroquirúrgicos/métodos , Neoplasias de la Médula Espinal/cirugía , Adolescente , Adulto , Niño , Terapia Combinada , Ependimoma/diagnóstico por imagen , Ependimoma/radioterapia , Femenino , Humanos , Región Lumbosacra/cirugía , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neoplasia Residual/cirugía , Pronóstico , Estudios Retrospectivos , Neoplasias de la Médula Espinal/diagnóstico por imagen , Neoplasias de la Médula Espinal/radioterapia , Resultado del Tratamiento , Adulto Joven
19.
Neurosurg Rev ; 43(5): 1315-1322, 2020 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31422571

RESUMEN

Chordoid gliomas are extremely rare entities, which are generally considered occurring exclusively in the third ventricle. Despite the low-grade histological grade, aggressive behaviors have been reported in literatures. Due to the low morbidity, the origins, clinical, and radiological features, management and prognosis are still yet to be well elucidated. We retrospectively reviewed the clinical profiles from a series of 6 patients with chordoid gliomas. All patients underwent surgical treatment, and the diagnoses were based on histopathological examinations. Magnetic resonance imaging (MRI) was performed perioperatively. Follow-up outcomes were presented. This case series consisted of three male and three female patients (age range 27-67 years; mean age 43.3 years). MRI results showed tumors in the third ventricle (4/6), temporal-parietal-occipital lobe involving the lateral ventricle (1/6), and cerebellar hemisphere (1/6). Three tumors were solid, and the others were cystic-solid. Hydrocephalus was present in one patient. The T1-weighted imaging showed hypo- to isointensity, and T2-weighted imaging showed iso- to hyperintensity; enhancement was homogeneous (4/6) or heterogeneous (2/6). Diffusion-weighted imaging showed no evidence of restricted diffusion. Magnetic resonance spectrum showed an elevated choline value and reduced N-acetylaspartate value. Gross total resection was achieved in all patients, and during an average follow-up period of 35.8 months, no recurrence was noted. Chordoid gliomas can occur outside the third ventricle with a great diagnostic challenge. The MRI characteristics suggest a low-grade tumor, and the accurate diagnosis depends on pathological criteria. Complete surgical resection is associated with a favorable outcome.


Asunto(s)
Neoplasias del Ventrículo Cerebral/patología , Neoplasias del Ventrículo Cerebral/cirugía , Glioma/patología , Glioma/cirugía , Procedimientos Neuroquirúrgicos , Tercer Ventrículo/patología , Tercer Ventrículo/cirugía , Adulto , Anciano , Ácido Aspártico/análogos & derivados , Ácido Aspártico/metabolismo , Cerebelo/diagnóstico por imagen , Neoplasias del Ventrículo Cerebral/diagnóstico por imagen , Colina/metabolismo , Imagen de Difusión por Resonancia Magnética , Femenino , Glioma/diagnóstico por imagen , Humanos , Hidrocefalia/etiología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/patología , Pronóstico , Estudios Retrospectivos , Tercer Ventrículo/diagnóstico por imagen , Resultado del Tratamiento
20.
Cancer Manag Res ; 11: 10321-10326, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31849521

RESUMEN

BACKGROUND: Solitary fibrous tumor (SFT) is a benign neoplasm arising in the soft tissue, which can occur anywhere in the body, while it is predominantly found in the visceral pleura. Spinal SFT is quite uncommon, with limited cases having been reported in the literature; especially, SFT occurring in the intramedullary site is extremely rare. CASE PRESENTATION: We present a case of a 35-year-old woman presenting with progressive numbness and weakness in the legs and urinary incontinence. Magnetic resonance imaging (MRI) showed an intramedullary lesion with bright enhancement. A diagnosis of spinal hemangioblastoma was suspected, and thus a three-dimensional computed tomographic angiography reconstruction was requested, which also demonstrated an angiomatous lesion. The tumor was completely resected under neurophysiological monitoring. However, histopathological and immunohistochemical examinations revealed an SFT. No adjuvant radiotherapy or chemotherapy was scheduled. The symptoms were relieved completely, and no recurrence or progression was noted during the follow-up. CONCLUSION: Though SFT has been considered similar to malignant hemangiopericytoma and the histological classification has always been controversial, the intramedullary location and benign behavior in the present case add to the current understandings of this extremely rare entity.

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