Clinical management and prognosis of spinal myxopapillary ependymoma: a single-institution cohort of 72 patients.

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.

Long-term outcomes of foramen magnum decompression with duraplasty for Chiari malformation type I in adults: a series of 297 patients.

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.

Long-term outcome and prognostic factors of syringo-subarachnoid shunt for syringomyelia.

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.

Spinal Cord Diffuse Midline Gliomas With H3 K27m-Mutant: Clinicopathological Features and Prognosis.

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.

Clinicopathological characteristics and survival of spinal cord astrocytomas.

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.

Therapeutic Strategies and Prognostic Factors Based on 121 Spinal Neurenteric Cysts.

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.

[Upper limbs motor maps in cortex and plasticity after the anatomical hemispherectomy].

OBJECTIVE: To locate motor functional area of patients who undergone modified anatomical hemispherectomy in order to analysis the plasticity of upper limbs motor. METHODS: The patients who undergone modified anatomical hemispherectomy were performed BOLD sequences, to locate functional cortical areas in their residual brain. RESULTS: 6 patients have performed examination of BOLD sequences by 3.0-T MRI.5 of them obtained contralateral upper limb motor areas in their residual brain, and 3 of them obtained ipsilateral and contralateral upper limb motor area map in cortex. The ipsilateral upper limb motor areas in the M1, SMA and posterior parietal cortex. CONCLUSIONS: The patients who undergone modified anatomical hemispherectomy is an excellent model to investigate mechanism of plasticity in the developing brain. Functional magnetic resonance (fMRI) provided fine spatial detail of brain responses, would describe the motor functional area of cortical maps. These patients exist ipsilateral motor areas in their residual mono hemisphere. The study indicated there maybe have somewhat extent of correlation between the surgical procedure and the outcome of neuroplasticity.

[Motor evoked potential of both upper extremities long term after anatomical hemispherectomy].

OBJECTIVE: To explore the function and motor pathway of remained cerebral hemisphere by studying motor evoked potential of both upper extremities on patients long term after anatomical hemispherectomy. METHODS: Five patients after anatomical hemispherectomy, who were marked 5 dispersive sites on head to perform transcranial magnetic stimulation. Recording motor evoked potential of target muscles (brachioradialis muscle and abductor pollicis brevis) of both upper extremities respectively when muscle resting and contracting. RESULTS: Only affected abductor pollicis brevis of case 2 and only affected brachioradialis muscle of case 4 and 5 recorded motor evoked potential when muscle resting. Motor evoked potential of some cases can be recorded simultaneously in homonymous muscles of both sides when muscle resting or contracting. CONCLUSIONS: There exists motor cortex that controls movement of ipsilateral limbs and also ipsilateral motor pathway of corticospinal connection at patients after anatomical hemispherectomy. It also means that the motor function of affected limbs has potency to recover well after hemispherectomy. The mirror movement after hemispherectomy is possible relate to overlap of both limbs' motor cortex.

Lymphoblastic Lymphoma Involving Multiple Vertebrae.

Acute lymphoblastic lymphoma is a malignant hematologic disease in childhood but rarely initially involves epidural compartment in adults. A 20-year-old male presented with progressive osphyalgia with constipation. Contrasted magnetic resonance imaging showed multiple vertebrae of hypointense T1 signals and an intraspinal epidural lesion. Subtotal resection was achieved. Histopathology suggested malignant B-cell lymphoma with Ki-67 of 90% and positivity of leukocyte common antigen. A bone marrow biopsy was unequivocally diagnostic of B-cell acute lymphoblastic lymphoma followed by chemotherapy (methotrexate) and partial recovery was observed. The marrow biopsy was necessary if without hypercalcemia and abnormal peripheral blood examination.

Intradural Extramedullary Bronchogenic Cyst: Clinical and Radiologic Characteristics, Surgical Outcomes, and Literature Review.

OBJECTIVE: An intradural extramedullary bronchogenic cyst (IEBC) is a congenital malformation and an extremely rare type of endodermal cyst. This study aims to report the clinical and radiologic characteristics and surgical outcomes of IEBCs and to review the available literature. METHODS: The medical records of 6 patients (3 female) with pathologically confirmed bronchogenic cysts involving the spinal cord between 2009 and 2016 were retrospectively reviewed, and follow-up evaluations were performed. RESULTS: Of the 6 symptomatic lesions, 4 were located in the cervical vertebra, 1 at the lumbar vertebra, and the remaining 1 at the craniocervical junction. Radiographs showed signals similar to cerebral spinal fluid on all magnetic resonance imaging sequences without contrast enhancement. Total resection was achieved in 3 patients. Histopathology revealed simple and pseudostratified respiratory epithelium with benign subepithelial mucous glands and fat components neighboring the cyst. After a mean follow-up duration of 49.2 months, 2 asymptomatic residual lesions regrew after nontotal resection. In previous literature including 19 cases, most IEBCs (84.2%) tended to be homogeneous and well demarcated on radiologic images, and 85.7% were not contrast enhancing. Cervical or upper thoracic segments were predilection sites with intradural extramedullary localization. The majority of IEBCs had similar benign histological features. The recurrence rate was 15.4% after nontotal resection but nil after total resection. CONCLUSIONS: IEBCs displayed an indolent course, and the most effective management with positive outcomes was total resection. Nontotal resection, cystic fenestration, and biopsy, which were associated with recurrence, should be avoided.

Primary spinal epidural cavernous hemangioma: clinical features and surgical outcome in 14 cases.

OBJECT: The aim of this study was to investigate the clinical characteristics, imaging features, differential diagnosis, treatment options, and prognosis for primary spinal epidural cavernous hemangiomas. METHODS: Fourteen patients with pathologically diagnosed non-vertebral origin cavernous hemangiomas who had undergone surgery at Beijing Tiantan Hospital between 2003 and 2012 were identified in the hospital's database. The patients' clinical data, imaging characteristics, surgical treatment, and postoperative follow-up were analyzed retrospectively. RESULTS: There were 9 males and 5 females with an average age of 51.64 years. The primary epidural cavernous hemangiomas were located in the cervical spine (2 cases), cervicothoracic junction (2 cases), thoracic spine (8 cases), thoracolumbar junction (1 case), and lumbar spine (1 case). Hemorrhage was confirmed in 4 cases during surgery. Preoperatively 5 lesions were misdiagnosed as schwannoma, 1 was misdiagnosed as a meningioma, and 1 was misdiagnosed as an arachnoid cyst. Preoperative hemorrhages were identified in 2 cases. Three patients had recurrent cavernous hemangiomas. The initial presenting symptoms were local pain in 5 cases, radiculopathy in 6 cases, and myelopathy in 3 cases. Upon admission, 1 patient had radicular symptoms and 13 had myelopathic symptoms. The average symptom duration was 18 months. All patients underwent surgery; complete resection was achieved in 8 cases, subtotal resection in 4 cases, and partial resection in 2 cases. Postoperative follow-up was completed in 10 cases (average follow-up 34 months); 1 patient died, 5 patients showed clinical improvement, and 4 patients remained neurologically unchanged. CONCLUSIONS: Total surgical removal of spine epidural cavernous hemangiomas with a chronic course is the optimum treatment and carries a good prognosis. Secondary surgery for recurrent epidural cavernous hemangioma is technically more challenging. In patients with profound myelopathy from acute hemorrhage, even prompt surgical decompression can rarely reverse all symptoms.

[A novel Krit-1 mutation in Han family with cerebral cavernous malformation].

OBJECTIVE: To detect the mutations of Krit-1 gene that cause familial cerebral cavernous malformation (CCM) in the Han ethnic origin. METHODS: The subjects were hospitalized in the Department of Neurosurgery, Tiantan Hospital affiliated to Capital University of Medical Sciences. Two families (A and B) and 8 apparently sporadic individuals affected with CCM were screened for mutations of Krit-1 gene. Members of the family CCM have a wide range in age of onset with seizures, headaches and skin lesions. The gene was screened by PCR amplification of 16 exons and mutation was detected by direct sequencing. RESULTS: In family A samples, analysis of the Krit-1 gene revealed a new point mutation in exon 14 [a heterozygous C to G transition at nucleotide 1 289 (counting from the start codon or nt 2 308 counting from the first nt of the mRNA, aligned according to Gene Bank AF388384)] which predicts the substitution of a premature termination codon for Serine at codon 430 (S430X), belonging a nonsense point mutation. No mutation was identified in one of family A members as well as in any of the sporadic individuals with the exception of a single nucleotide polymorphism. CONCLUSIONS: Report the first family in the Han with CCM having a novel mutation in the CCM1 gene on the continent of Asia. The newly identified mutation creates a premature termination codon and is predicted to produce a truncated Krev1 interaction-trapped 1 protein, KRIT1. This result allows efficient presymptomatic molecular diagnosis.

Surgical strategies and outcomes of spinal ependymomas of different lengths: analysis of 210 patients: clinical article.

OBJECT: The aim of this study was to investigate the surgical strategies and outcomes for spinal ependymomas of different lengths. METHODS: The authors used data from 210 patients with spinal ependymomas (WHO Grades II and III) in this 10-year retrospective study (January 1999 to December 2008), dividing them into 3 different groups according to length (spinal ependymomas < 5 cm, 5-10 cm, and > 10 cm). All patients underwent tumor resection. The basic characteristics of the patients were reviewed and the functional status was assessed using the McCormick classification. RESULTS: There were 89, 81, and 40 patients, respectively, in the 3 groups (< 5 cm, 5-10 cm, and > 10 cm). Grosstotal resections (GTRs) were performed in 172 patients (81.9% overall, or 86.5%, 79.0%, and 77.5% in the 3 groups, respectively). Subtotal and partial resections were achieved in 38 patients (18.1%). Eight patients with medulla oblongata or upper cervical cord tumors received a tracheotomy postoperatively. The follow-up period ranged from 56 to 176 months. One hundred thirty-five patients (76.7%) experienced improvement, (88.2%, 83.8%, and 34.4% in the < 5 cm, 5-10 cm, and > 10 cm groups, respectively). Thirty-three patients (18.8%) maintained their pretreatment status, and 8 patients (4.5%) showed deterioration following tumor resection at 6 months. Tumor recurrence or progression was observed in 6 (2.9%) of the 210 patients. Among the 6 patients, recurrent tumors were located in the conus (n = 3), thoracic (n = 1), and medullocervical cord (n = 2). CONCLUSIONS: Radical resection of spinal ependymomas could be performed in most patients, and the rate of GTR was significantly different in the different-length groups (< 10 cm vs > 10 cm, p = 0.032). Patients with longer tumors had worse surgical results compared with those with small tumors (p < 0.001), and more postoperative neuropathic pain and proprioceptive deficits could usually be observed in patients harboring larger tumors. Early diagnosis and timely operation are critical to achieving better neurological outcomes. For tumors with dense adhesions, complete removal should be performed cautiously because of the significant incidence of neurological deterioration.

Long-term P300 in hemispherectomized patients.

BACKGROUND: In the years around 1990, in Beijing Tiantan Hospital Affiliated to Capital Medical University many children with infantile hemiplegia and intractable epilepsy were treated with further modified anatomical hemispherectomy. We report the follow up of the first six cases. To make good use of these precious clinical data and make clear their neuropsychological state, we performed neuropsychological and neurophysiological measurements in these patients, who were at a median of 17.8 years after hemispherectomy. METHODS: Oddball task was given to the patients and to a normal control group to collect the peak latency (PL) and peak amplitude (PA) of event-related potentials (ERPs)-P300. The P300 data of the two groups were analyzed and the P300 patterns of the six patients are presented. The baseline characteristics and long-term follow-up of the six hemispherectomized patients, especially the long-term seizure control and cognitive function after surgery, are described. RESULTS: Five patients had no seizures and one was almost seizure-free during the years after surgery. Clear P300 was obtained from every electrode in the patients. Differences of P300 between patients and normal control group had no statistical significance. And the maximum PA was at the site of electrode Pz or Cz which was consistent with that of the control group and with previous findings. CONCLUSIONS: Further modified anatomical hemispherectomy has preferable long-term antiepileptic effects. The P300 results of the patients mean that the basic cognitive function of the patients has no difference from the control group. This reflects the plasticity of the hemisphere to some extent and increases the affirmation of the long-term curative effects of further modified anatomical hemispherectomy from both neuropsychological and neurophysiological aspects.