Background:Desmoplastic infantile astrocytomas/ganglioglioma (DIA/DIG) arising in the suprasellar region are extremely rare, and their clinical features, optimal management, and outcomes remain unclear. We aimed to summarize the clinical manifestations, treatment strategies, and prognosis of this rare tumor entity. Patients and methods:This retrospective case series included 5 institutional cases and 13 literature cases of suprasellar DIA/DIGs. The clinical and radiological characteristics, therapies, and outcomes of this rare tumor were examined. Results:Our case series comprised 2 males and 3 females, with a median age of 6 months. Three patients underwent partial resection, and 2 had subtotal resection. During follow-up, the residual tumors in 2 patients showed spontaneous regression, 1 tumor progressed, and 2 remained stable. The literature review, including our cases, yielded 18 suprasellar DIA/DIG. Of these, 10 were male and 8 were female, with a median age of 4 months (range, 1-85 months). Ten patients had solitary suprasellar tumors and 8 had multifocal lesions. Over a median follow-up of 25 months, tumor progression of the suprasellar lesions was seen in 9 patients. Notably, spontaneous regression of the suprasellar tumors occurred in 2 patients. Conclusions:Despite being WHO grade I, suprasellar DIA/DIG can demonstrate multifocal CNS disease and high risk of progression after subtotal resection. DIA/DIG should be considered in the differential diagnosis of suprasellar lesions in infants and long-term close monitoring is warranted.
The choroid plexus (ChP) serves as the principal origin of cerebrospinal fluid (CSF). CSF hypersecretion due to ChP inflammation has emerged as an important pathogenesis of hydrocephalus recently. Nevertheless, the precise mechanisms of ChP inflammation and the ensuing CSF hypersecretion in hydrocephalus remain ill-defined. In the present study, we elucidate the critical role of macrophages in the pathogenesis of ChP inflammation. Specifically, we identify the chemokine CCL2, released by ChP epithelial cells, recruits CCR2+ monocytes to the ChP thereby inciting hydrocephalus pathogenesis. The accumulated ChP macrophages increase the inflammation in ChP epithelial cells through TNF-α/TNFR1/NF-κB signaling cascade, thereby leading to CSF hypersecretion. Strikingly, augmentation of ChP‒CCL2 using an adeno-associated viral approach (AAV) exacerbates macrophage recruitment, activation, and ventriculomegaly in rat PHH models. Systemic application of Bindarit, a specific CCL2 inhibitor, significantly inhibits ChP macrophage infiltration and activation and reduces CSF secretion rate. Furthermore, the administration of CCR2 antagonist (INCB 3284) reduces ChP macrophage accumulation and ventriculomegaly. This study not only unveils the ChP CCL2‒CCR2 signaling in the pathophysiology of hydrocephalus but also unveils Bindarit as a promising therapeutic choice for the management of posthemorrhagic hydrocephalus.
Critical-size skull defects caused by trauma, infection, and tumor resection raise great demands for efficient bone substitutes. Herein, a hybrid cross-linked hierarchical microporous hydrogel scaffold (PHCLS) was successfully assembled by a multistep procedure, which involved (i) the preparation of poly(lactic-co-glycolic)/nanohydroxyapatite (PLGA-HAP) porous microspheres, (ii) embedding the spheres in a solution of dopamine-modified hyaluronic acid and collagen I (Col I) and cross-linking via dopamine polyphenols binding to (i) Col I amino groups (via Michael addition) and (ii) PLGA-HAP (via calcium ion chelation). The introduction of PLGA-HAP not only improved the diversity of pore size and pore communication inside the matrix but also greatly enhanced the compressive strength (5.24-fold, 77.5 kPa) and degradation properties to construct a more stable mechanical structure. In particular, the PHCLS (200 mg, nHAP) promoted the proliferation, infiltration, and angiogenic differentiation of bone marrow mesenchymal stem cells in vitro, as well as significant ectopic angiogenesis and mineralization with a storage modulus enhancement of 2.5-fold after 30 days. Meanwhile, the appropriate matrix microenvironment initiated angiogenesis and early osteogenesis by accelerating endogenous stem cell recruitment in situ. Together, the PHCLS allowed substantial skull reconstruction in the rabbit cranial defect model, achieving 85.2% breaking load strength and 84.5% bone volume fractions in comparison to the natural cranium, 12 weeks after implantation. Overall, this study reveals that the hierarchical microporous hydrogel scaffold provides a promising strategy for skull defect treatment.
Objective:To explore the clinical features, treatment and outcomes of hemangioblastomas in the cerebellopontine angle (CPA) region.Methods:A retrospective analysis was conducted on the clinical data of 31 patients with hemangioblastoma in the CPA region admitted to the Department of Neurosurgery, West China Hospital of Sichuan University from January 2003 to January 2019. All 31 patients underwent tumor resection, and 10 patients underwent preoperative endovascular embolization. Outpatient follow-up was performed every 3 months after surgery, and every 6 months after 1 year. Follow-up content included changes in clinical symptoms of patients and re-examination of enhanced brain MRI to determine whether there was tumor recurrence.Results:Among the 31 patients, 20 were males and 11 were females; the median disease duration was 15.3 months (0.5-72.0 months). The main clinical manifestations were headache (25 cases, 80.6%), hearing loss (19 cases, 61.3%) and tinnitus (13 cases, 41.9%). Ten patients (32.3%) were diagnosed with Von Hippel-Lindau (VHL) syndrome. Preoperative contrast-enhanced MRI showed solid tumors in 18 cases (58.1%) and cystic tumors in 13 cases (41.9%). Ten cases underwent preoperative endovascular embolization. Among the 27 patients with complete surgical data, 22 patients (81.5%) underwent surgery via retrosigmoid approach, and 5 patients (18.5%) underwent surgery via the far lateral approach. One patient had severe bleeding during tumor resection, the tumor was tightly adhered to the cranial nerves, and only subtotal resection was performed. Postoperative re-examination of enhanced brain MRI showed that 30 patients (96.8%) underwent total tumor resection, and 1 patient (3.2%) underwent subtotal tumor resection. There were 4 cases (12.9%) of new-onset facial paralysis, 4 cases (12.9%) of posterior cranial nerve palsy, 2 cases (6.5%) of trigeminal nerve damage, 2 cases (6.5%) of abducens nerve palsy, 1 case (3.2%) of intracranial hemorrhage, 1 case (3.2%) of cerebrospinal fluid leakage and 1 case (3.2%) of pseudomeningocele. At discharge, preoperative symptoms improved in 22 patients, remained unchanged in 6 patients, and worsened in 3 patients. The follow-up time of 31 patients was 39.1±23.4 months (3-144 months). By the last follow-up, preoperative symptoms improved in 25 cases, did not improve in 3 cases, and aggravated in 3 cases. Except for 2 patients with new-onset facial paralysis after surgery whose facial nerve function did not improve, the neurological functions of the rest of the patients with new-onset cranial nerve dysfunction after surgery recovered to normal. Five patients(16.1%) had tumor recurrence in situ during follow-up, including 3 patients with VHL syndrome.Conclusions:Hemangioblastomas in the CPA region are clinically rare, and there are slightly more male patients. Headache, hearing loss and tinnitus are often the first symptoms. Surgery is an effective treatment and preoperative endovascular embolization can help reduce surgical risks and intraoperative bleeding. Although it is a benign tumor, long-term follow-up is necessary after surgery.
Far lateral approach (FLA), together with its modified University to perform this retrospective study (No.2021approaches, is a fundamental surgical approach for surgical resection of lesions located at the ventral or ventrolateral side of the foramen magnum. The key procedure in FLA is exposing the vertebral artery (VA) especially the V3 segment. When performing an FLA, most neurosurgeons choose to expose the suboccipital triangle (formed by the rectus capitis posterior major, the superior oblique, and the inferior oblique) as a key anatomical landmark initially and then expose the V3 segment. However, on the one hand, this muscular anatomical landmarks-suboccipital triangle varies in depth, morphology, and location in different patients, which increases the risk of VA injury during surgery. On the other hand, this procedure could lead to exposing difficulty due to the obstruction of overlying layers of muscles and the distribution of blood vessels, which could prolong the time for craniotomy fairly. The course of VA could vary, such as looping backward and bulge posteriorly between the lips of the suboccipital triangle, where it could be damaged if one expects it to be found in the depth of the suboccipital triangle. Thus, neurosurgeons have the potential for VA injury and a rather prolonged time to successfully expose VA.
BACKGROUND:The pineal tumor was once considered as a restricted area for surgery. Such cases are rare, with many different opinions on surgical treatment. This study aimed to review our experience of tumor treatment in the pineal region and explore the optimal treatment strategy. METHODS:The clinical data of 72 patients with pineal tumors from January 1997 to May 2015 (18 years) were retrospectively analyzed. Preoperative preparation, pathology type, tumor resection rate, surgical approach, and follow-up outcomes were used as the indicators to evaluate the treatment efficacy. RESULTS:The Krause approach was used in 46 cases, the Poppen approach in 10 cases, and the transcallosal-lateral ventricle-choroid fissure approach in 16 cases. The postoperative pathological results were as follows: 24 cases of germinoma, 11 of teratoma, 15 of glioma, 6 of meningioma, 11 of Pineocytoma, 2 of cholesteatoma, 2 of cavernous hemangioma, and 1 of choriocarcinoma. Further, the study included 64 cases of total surgical resections, 8 of subtotal resections, and 2 deaths. The follow-up period was from 7 months to 10 years. Further, 51 (70.8%) patients were followed up. The multivariate regression model showed that the surgical method and the pathological type contributed significantly to predicting outcomes. CONCLUSIONS:The type of pathology, extent of excision, and surgical approach had a significant impact on the prognosis of patients. The transcallosal-lateral ventricle-choroid fissure approach for large and medium-sized pineal tumors near the posterior part of the third ventricle had good efficacy.
Objective:Acoustic neuroma (AN) is a common benign tumor. Little is known of neuropsychological studies in patients with acoustic neuroma, especially cognitive neuropsychology, and the neuropsychological abnormalities of patients affect their life quality. The purpose of this study was to explore the changes in the cognitive function of patients with acoustic neuroma, and the possible mechanism of these changes by structural magnetic resonance imaging.Materials and methods:We used a neuropsychological assessment battery to assess cognitive function in 69 patients with acoustic neuroma and 70 healthy controls. Then, we used diffusion tensor imaging data to construct the structural brain network and calculate topological properties based on graph theory, and we studied the relation between the structural brain network and cognitive function. Moreover, three different subnetworks (short-range subnetwork, middle-range subnetwork, and long-range subnetwork) were constructed by the length of nerve fibers obtained from deterministic tracking. We studied the global and local efficiency of various subnetworks and analyzed the correlation between network metrics and cognitive function. Furthermore, connectome edge analysis directly assessed whether there were differences in the number of fibers in the different brain regions. We analyzed the relation between the differences and cognitive function.Results:Compared with the healthy controls, the general cognitive function, memory, executive function, attention, visual space executive ability, visual perception ability, movement speed, and information processing speed decreased significantly in patients with acoustic neuroma. A unilateral hearing loss due to a left acoustic neuroma had a greater impact on cognitive function. The results showed that changes in the global and local metrics, the efficiency of subnetworks, and cognitively-related fiber connections were associated with cognitive impairments in patients with acoustic neuroma.Conclusion:Patients exhibit cognitive impairments caused by the decline of the structure and function in some brain regions, and they also develop partial compensation after cognitive decline. Cognitive problems are frequent in patients with acoustic neuroma. Including neuropsychological aspects in the routine clinical evaluation and appropriate treatments may enhance the clinical management and improve their life quality.
Aim Multifocal desmoplastic infantile ganglioglioma/astrocytoma (DIA/DIG) has rarely been reported. Here, two cases have been presented, reviewing the literature and proposed treatment algorithms for this rare tumor. Patients and Methods We report two patients diagnosed with multifocal DIA/DIGs in West China Hospital. In addition, a literature review was performed, in October 2019, on case reports of DIA/DIGs with multifocal lesions. The clinical and radiological features, treatment, and outcome of this rare disease were discussed. Results DIA/DIGs with multifocal locations were rare, and only thirteen cases (including ours) had been reported. This series included 8 males and 5 females with a mean age of 31.4 ± 45.7 months (range, 3-144 months). The supratentorial hemisphere, suprasellar region, posterior cranial fossa, and spinal cord were frequently involved. Ten patients (76.9%) received surgical resection for the symptomatic lesions and three patients (23.1%) underwent biopsy. Seven patients received chemotherapy postoperatively. Six individuals had tumor recurrences during the follow-up period, while three patients had tumors that spontaneously regressed. Finally, two patients died of tumor progression and one patient died of respiratory insufficiency and hypothalamic dysfunction. Conclusions Multifocal DIA/DIGs have more aggressive clinical behavior and poor outcome despite benign histology. DIA/DIGs should be included in the differential diagnosis of multifocal brain tumors in children. The mainstay of treatment is surgical resection; adjuvant treatment with chemotherapeutic drugs is unknown and requires additional research.
Lymphoplasmacyte-rich meningioma (LRM) is a rare histologic subtype of meningioma. Creeping-growth pattern is uncommon in meningioma, and the mechanism is unclear. Here, we report a 44-year-old man presented with extremities weakness for 2 months and incontinence for 2 weeks. Head and neck MRI revealed diffuse creeping-growth nodular meningeal masses with skull base, tentorium, sella area, and C1-6 vertebral plane involvement. An operation was carried out, cervical and lower clivus part of the lesion was resected, but gross total resection could not be achieved due to the widespread lesions. Pathologic examination revealed the diagnosis of LRM. The patient is free from progression clinically 3 months postoperatively. We also conducted a systematic literature review about LRM with creeping-growth pattern. A total of only nine cases (including the present case) of creeping-growth LRMs were included and analyzed in terms of clinical manifestations, radiological features, treatment, and outcome. LRMs show a higher rate (7.5%) of creeping-growth pattern than other types of meningiomas. The average creeping length of all creeping-growth LRMs was 11.4 ± 10.9 cm (range, 3–30 cm). Most cases (66.7%) had obvious peritumoral edema. Total removal rate is low (33.3%), and two of them (22.2%) received biopsy, followed by steroids treatment (or further immunosuppressive drugs therapy) and radiotherapy. The recurrence rate is higher than conventional LRMs (22.2 vs. 11.3%), and one patient (11.1%) died 11 months after treatment. Creeping-growth pattern in LRM may be considered as a general radiologic variant. The recurrence rate is higher compared with LRM with round/swelling pattern. We speculated that the pathogenesis of creeping growth in LRM may be associated with damage of lymphatic systems of the central nervous system.
目的 探讨中重度颅脑损伤(traumatic brain injury,TBI)患者28 d死亡影响因素及中性粒细胞与淋巴细胞比值(neutrolphil to lymphocyte ratio,NLR)联合格拉斯哥昏迷量表(Glasgow coma scale,GCS)评分对其28 d死亡预测价值.方法 回顾性分析中重度TBI 628例的临床资料,根据28 d生存情况,将其分为病死组112例和存活组516例.采用单因素和多因素Logistic回归分析对中重度TBI患者28 d死亡影响因素进行分析,应用受试者工作特征(ROC)曲线分析NLR、GCS评分和NLR联合GCS评分对中重度TBI患者28 d死亡的预测价值.结果 单因素分析结果 显示,病死组入院时NLR、活化部分凝血酶原时间和C反应蛋白高于存活组,血红蛋白低于存活组;急诊手术治疗、坠落伤、瞳孔对光反应双阴、中线移位>5 mm、环池受压所占比例以及急性生理学及慢性健康状况评分系统Ⅱ评分高于存活组,GCS评分低于存活组,差异有统计学意义(P<0.05或P<0.01).多因素Logistic回归分析结果 显示,NLR>10.36、中线移位>5 mm和环池受压是中重度TBI患者28 d死亡的危险因素,GCS评分9~12分是中重度TBI患者28 d死亡的保护因素(P<0.01).ROC曲线分析结果 显示,NLR联合GCS评分预测中重度TBI 28 d死亡的曲线下面积(AUC)为0.892高于NLR、GCS评分单独预测中重度TBI 28 d死亡的AUC 0.745和0.827,且敏感度和特异度均较高.结论 NLR>10.36、中线移位>5 mm和环池受压是中重度TBI患者28 d死亡的危险因素,GCS评分9~12分是中重度TBI患者28 d死亡的保护因素.NLR联合GCS评分对中重度TBI患者28 d死亡具有较高预测价值.
Peritumoral cysts are commonly detected in the central nervous system tumors, especially hemangioblastomas (HBs). However, the molecular mechanisms driving their formation and propagation are still unknown. We conducted an integrated lipidomics and transcriptomics analysis on solid and cystic HB samples in order to elucidate the changes in the lipid profile and expression of lipid metabolism-related genes during cyst formation. Transcriptomic analysis revealed differential expression of several genes between the solid and cystic HBs, and those associated with lipid metabolism, such as ADCY4, MGLL, ACOT2, DGKG, SHC1 and LPAR2, were markedly dysregulated in the cystic HBs. The lipidomic analysis further showed a significant reduction in the abundance of triacylglycerol, ceramide, lysophosphatidylcholine and lysophosphatidylethanolamine, and an increase in phosphatidylcholine and phosphatidylethanolamine levels in the cystic HBs. Furthermore, bioinformatics analysis revealed altered lipid biosynthesis, glycerophospholipid metabolism and phospholipase activity in the cystic HBs. Taken together, our findings indicate that cyst formation in HBs is related with aberrant lipid metabolism.
目的 探讨基础远外侧入路即髁后及部分经髁入路,在以枕骨大孔腹侧为中心的病变手术的使用范围、方法及效果.方法 回顾性分析四川大学华西医院神经外科2016年4月—2019年10月,采用基础远外侧入路手术治疗的30例枕骨大孔腹侧病变患者的临床资料.结果 本组30例患者中,脑膜瘤27例、神经鞘瘤2例、动脉瘤1例;肿瘤全切者28例,部分切除者1例(脑膜瘤),1例动脉瘤顺利夹闭.术后,3例患者出现后组颅神经功能障碍,其中2例患者术后3个月内恢复.1例患者出现肢体轻偏瘫,4例出现皮下积液,均经治疗后消失.无死亡病例.结论 基础远外侧入路即髁后及部分经髁入路,是处理以枕骨大孔腹侧为中心病变的经典且安全有效的手术入路.
Background: Hemangioblastomas (HBs) are rare and understudied. The impact of age on clinical features, optimal treatment, and postoperative outcome in HBs has not been systemically studied. Materials and Methods: We performed an institutional study in the West China Hospital, including patients diagnosed with hemangioblastomas between October 2011 and October 2016. Clinical data and surgical outcomes were compared among three different age groups (0-20, 21-59, and >= 60 years). We also studied surgical prognostic factors. Results: 187 patients (102 males and 85 females) were identified. HBs in spinal locations were more prevalent in children and adolescents, while the most common site in young and old-age adults was the cerebellum (P = 0.023). Risk of Von Hippel-Lindau syndrome (VHL) was negatively correlated with patient age (P = 0.003). 47.6 % of patients aged 0-20, 23 % of patients aged 21-59, and 6.45 % of patients aged >= 60 years were diagnosed with VHL. Older aged patients had a significantly lower risk of VHL and had a lower tumor recurrence rate. Surgical resection was an adequate and sufficient procedure in all age groups with a favorable surgical outcome (P = 0.823). Solid tumor, VHL disease, and localization in the brainstem were associated with poor outcome in patients with hemangioblastomas. Conclusions: Our study showed that HBs in elderly patients were mostly included in the cerebellum and had a solid configuration and large size, while HBs in children were mainly localized in the spinal cord. VHL disease was more common in children and adolescents but rare in patients older than 60 years. The present study found no excess surgical mortality and risks for HBs in elderly patients or children than in younger adults.
听神经瘤手术目前已从拯救患者生命提升至提高患者的生命质量,因而术中保护面神经对于患者术后的生命质量至关重要。术中面神经电生理监测对于保护面神经具有重要价值,不仅可实时精确辨认和定位面神经,还可评估面神经功能的完整性。目前的神经电生理监测技术主要包括:触发肌电图、自发肌电图及经颅面神经运动诱发电位。本文将对听神经瘤术中面神经电生理监测技术的基本原理、技术进展及其技术陷阱进行综述。
CONTEXT:A well-established cell line of hemangioblastomas (HBs) is still lacking. AIM:This study aims to explore a stable way to establish primary cell lines of HB stromal cells and investigate the morphological and molecular features of these cells. PATIENTS AND METHODS:Specimens of HBs from 13 patients were collected for establishment of primary cell lines of stromal cells. The details on cell culture were described, and the characterizations of cultured cells were conducted by morphological observation, immunocytochemical staining of inhibin-α, brachyury, CD133, CD34, GFAP, CD31, NeuN, CD45, Oligo2, and transmission electron microscopy. RESULTS:Eleven cases were successfully cultured with a success rate of 84.6%. The cultured cells survived for 10 generations with an estimated doubling time of 77.2 ± 5.89 h. Light microscopy revealed that these cells showed vigorous growth status and presented as polygons or trigons with significant heterogeneity. The immunocytochemical staining showed that inhibin-α, brachyury, CD133, and CD34 were expressed in all the cultured cells, whereas the expression of GFAP, CD31, NeuN, CD45, and Oligo2 was all negative. Transmission electron microscopy confirmed that the cultured cells were stromal cells with typical lipid droplets. The phenomenon of lysosomal autophagy was commonly observed without apoptotic cells in late stage. CONCLUSION:Appropriate selection of tumor specimens, short duration of devascularization, ideal digestion time, and nutritious medium are critical points for establishment of primary cell line of HB stromal cells. Stromal cells from both von Hippel-Lindau disease-related HBs and sporadic HBs might originate from embryologically arrested hemangioblasts.
Pediatric cerebellar glioblastomas (pcGBMs) are rare and their characteristics remain ill-defined. We conducted a retrospective analysis of pediatric cerebellar glioblastomas who underwent surgery from 2008 to 2019 in our department. Besides, we performed a literature review of the literature data on pcGBMs. Ten children with mean age of 9.4 years were included. During the follow-up, six patients died with mean survival time of 11.7 months, four patients survived with mean follow-up of 28 months. Seven patients underwent molecular analysis, no patients detected IDH1 mutations, four patients (57.1%) had H3K27M mutations, and two patients (28.6%) had MGMT promoter methylation. The literature review identified 38 pcGBMs cases (including ours), with mean age of 8.84 ± 4.20 years (range, 1–16 years). Increased ICP was the commonest sign. Eighteen (47.4%) patients underwent GTR and fifteen (45.5%) patients received STR. Postoperative radiation (RT) was conducted in 28 patients (75.7%) and 23 patients (65.7%) received chemotherapy. During the follow-up, 25 patients died with mean survival time of 12.21 months and 11 patients survived with average follow-up of 29.3 months. Kaplan-Meier survival depicted chemotherapy (P < 0.001) or radiation (P < 0.001) had positive impact on overall survival. Multivariate analysis revealed chemotherapy was a significant predictor of survival with a hazard ratio of 3.264 (P = 0.038). Our study found mean overall survival time for pcGBMs patients was 12.21 months. PcGBMs may have distinct molecular features, with higher incidence of H3K27M mutation and were always IDH1 wild-type. We recommend the routine postoperative radiotherapy and chemotherapy in pcGBMs.
Aim Survival rates and prognostic factors of cortical ependymomas (CEs) remain elusive. This study aimed to perform a comprehensive analysis of prognostic factors, treatment, and outcomes for patients with CEs based on institutional and literature case series. Materials and Methods Thirty patients with CEs from our department were included in this study. Furthermore, a systemic review of the literature yielded an additional 106 patients with CEs. Clinical data including patient age, sex, symptoms, tumor location, World Health Organization (WHO) grade, extent of surgery, radiation, recurrence, and survival were recorded and statistically analyzed. Results From January 2009 to October 2019, 30 (4.2%) cases were diagnosed as CEs in our department. These series consisted of 19 males and 11 females, 10 continuous patients after 2017 screened for C11orf95-RELA fusion, and 9 patients (90%) were RELA fusion positive. During the follow-up period, nine (30%) patients depicted tumor recurrence or progression; four (13.3%) patients died of tumor progression. The literature review yielded 106 CE cases, with additional 30 cases of our own collected for further analysis. Of these 136 cases, the frontal lobe (40%) was the most common location, and the average age was 22.6 +/- 17.6 years. Anaplastic histology/WHO grade III tumors were identified in 68 (50%) patients. Statistically analysis demonstrated that extent of surgery and WHO tumor grade were significant prognostic factors in Kaplan-Meier log-rank testing and Cox proportional hazards models. Gross total resection (GTR) predicted longer progression-free survival (PFS) [P= 0.013, hazard ratio (HR) = 3.012, 95% confidence interval (CI) = 1.257-7.213] and overall survival (OS) (P= 0.003, HR = 5.322, 95% CI = 1.751-16.178). WHO grade III tumors had worse PFS (P= 0.002, HR = 5.17, 95% CI = 1.804-14.816) and OS (P= 0.025, HR = 5.640, 95% CI = 1.248-25.495). Conclusion CEs accounted for only 3.5 to 5.7% of ependymomas, with seizures the most common symptom and the frontal lobe the most frequent location. CEs may have higher rate of RELA fusions, but generally favorable prognosis. The extent of surgery and WHO tumor grade were significant prognostic factors for PFS and OS in multivariate analysis. GTTR or WHO grade II tumors had better overall outcome in patients with CEs.
Objectives: Hemangioblastomas (HBs) in the elderly are very uncommon and have rarely been studied. This retrospective study aimed to identify clinical features, optimal treatment, surgical outcomes and long-term prognostic factors in these rare lesions. Patients and methods: We performed a retrospective analysis of HBs patients over 65 years old who underwent surgery from 2008 to 2018 at our department. Clinical data was retrospectively reviewed and statistically analyzed. Results: Thirty-three elderly patients with a mean age of 68.76 years were included in this study. Cerebellum, brainstem, and spinal cord locations accounted for 72.7 %, 18.2 % and 9.1 %. Two patients (6.1 %) were diagnosed as von Hippel-Lindau (VHL) syndrome. After mean follow-up of 37.95 +/- 22.12 months, clinical symptoms improved in 22 patients (67 %), unchanged in seven patients (21 %) and aggravated in 4 patients (12 %). Only 1(3 %) patient experienced local recurrence during follow-up. Univariate analysis showed tumor size (P = 0.044) and tumor characteristic (cystic or solid) (P = 0.034) were significantly related to long-term outcomes, while multiple logistic regression analysis depicted tumor characteristics were exclusively correlated with outcomes (P = 0.04). Conclusions: Our study suggests elderly hemangioblastomas may be different from their younger counterparts in that they often display solid configuration with large size and include more cerebellar tumors. HBs should be included in the differential diagnosis of elderly patients presenting with cerebellar mass. Despite many challenges involved, surgical removal of HBs in this age group is a safe procedure with acceptable risks. They may do not require as frequent follow-up as younger counterparts due to the low associations with VHL disease and tumor recurrence rate.