Cauda equina ependymomas: surgical treatment and long-term outcomes in a series of 125 patients

2021 ◽  
pp. 1-12
Author(s):  
Nicolò Marchesini ◽  
Nicola Tommasi ◽  
Franco Faccioli ◽  
Giampietro Pinna ◽  
Francesco Sala

OBJECTIVE Cauda equina ependymoma (CEE) is a rare tumor for which little information is available on the oncological and clinical outcomes of patients. In this study the authors aimed to address functional, oncological, and quality-of-life (QOL) outcomes in a large series of consecutive patients operated on at their institution during the past 20 years. METHODS The records of 125 patients who underwent surgery between January 1998 and September 2018 were reviewed. Analyzed variables included demographic, clinical, radiological, surgical, and histopathological features. Neurological outcomes were graded according to the McCormick and Kesselring scales. The QOL at follow-up was evaluated by administering the EQ-5DL questionnaire. RESULTS On admission, 84% of patients had a McCormick grade of I and 76.8% had a Kesselring score of 0. At follow-up (clinical 8.13 years; radiological 5.87 years) most scores were unchanged. Sacral level involvement (p = 0.029) and tumor size (p = 0.002) were predictors of poor functional outcome at discharge. Tumor size (p = 0.019) and repeated surgery (p < 0.001) were predictors of poor outcome. A preoperative McCormick grade ≥ III and Kesselring grade ≥ 2 were associated with worse outcomes (p = 0.035 and p = 0.002, respectively). Myxopapillary ependymoma (MPE) was more frequent than grade II ependymoma (EII). The overall rate of gross-total resection (GTR) was 91.2% and rates were significantly higher for patients with EII (98%) than for those with MPE (84%) (p = 0.0074). On multivariate analysis, the only factor associated with GTR was the presence of a capsule (p = 0.011). Seventeen patients (13.7%) had recurrences (13 MPE, 4 EII; 76.4% vs 23.6%; p = 0.032). The extent of resection was the only factor associated with recurrence (p = 0.0023) and number of surgeries (p = 0.006). Differences in progression-free survival (PFS) were seen depending on the extent of resection at first operation (p < 0.001), subarachnoid seeding (p = 0.041), piecemeal resection (p = 0.004), and number of spine levels involved (3 [p = 0.016], 4 [p = 0.011], or ≥ 5 [p = 0.013]). At follow-up a higher proportion of EII than MPE patients were disease free (94.7% vs 77.7%; p = 0.007). The QOL results were inferior in almost all areas compared to a control group of subjects from the Italian general population. A McCormick grade ≥ 3 and repeated surgeries were associated with a worse QOL (p = 0.006 and p = 0.017). CONCLUSIONS An early diagnosis of CEE is important because larger tumors are associated with recurrences and worse functional neurological outcomes. Surgery should be performed with the aim of achieving an en bloc GTR. The histological subtype was not directly associated with recurrences, but some of the features more commonly encountered in MPEs were. The outcomes are in most cases favorable, but the mean QOL perception is inferior to that of the general population.

2015 ◽  
Vol 39 (2) ◽  
pp. E8 ◽  
Author(s):  
Mohammed Abdulaziz ◽  
Grant W. Mallory ◽  
Mohamad Bydon ◽  
Rafael De la Garza Ramos ◽  
Jason A. Ellis ◽  
...  

OBJECT While extent of resection has been shown to correlate with outcomes after myxopapillary ependymoma (MPE) resection, the effect of capsular violation has not been well studied. The role of adjuvant radiation also remains controversial. In this paper the authors' goals were to evaluate outcomes following resection of MPE based on intraoperative capsular violation and to explore the role of adjuvant radiotherapy in cases of capsular violation. METHODS A retrospective review of patients undergoing resection of MPE at 2 academic institutions between 1990 and 2013 was performed. Cases with dissemination at presentation, less than 12 months of follow-up, or incomplete records were excluded. Extent of resection was defined as en bloc if all visible tumor was removed without capsular violation, gross-total resection (GTR) if all visible tumor was removed, but with capsular violation, and subtotal resection (STR) if a known residual was left at the time of surgery. Postoperative MR images were reviewed to confirm the extent of resection. Primary outcomes were progression-free survival (PFS) and overall recurrence rates. The effects of extent of resection, capsular violation, and adjuvant radiotherapy on recurrence rates and PFS were analyzed using Kaplan-Meier statistics. Associations between recurrence and preoperative variables were evaluated using Fisher exact methods and t-tests where appropriate. RESULTS Of the 107 patients reviewed, 58 patients (53% were male) met inclusion criteria. The mean age at surgery was 40.8 years (range 7–68 years). The median follow-up was 51.5 months (range 12–243 months). Extent of resection was defined as en bloc in 46.5% (n = 27), GTR in 34.5% (n = 20), and STR in 18.9% (n = 11). No recurrences were noted in the en bloc group, compared with 15% (n = 3) and 45% (n = 5) in the GTR and STR groups. En bloc resection was achieved most frequently in tumors involving the conus. Twelve patients (20%) underwent adjuvant radiotherapy following either STR or GTR. The overall recurrence rate was 13.8% (n = 8), and the 5-year PFS was 81%. Capsular violation was associated with a higher recurrence rate (p = 0.005). Adjuvant radiotherapy showed a nonsignificant trend of lower recurrence rates (16.7% vs 31.6%, p = 0.43) and longer PFS at 5 years (83.3% vs 49.9%, p = 0.16) in cases of capsular violation. CONCLUSIONS A strong correlation between capsular violation and recurrence was found following removal of MPE and should be assessed when defining extent of resection in future studies. Although the use of adjuvant radiotherapy in cases of capsular violation showed a trend toward improved PFS, further investigation is needed to establish its role as salvage therapy also appears to be effective at halting disease progression.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e13034-e13034
Author(s):  
Menal Bhandari ◽  
Ajeet K Gandhi ◽  
Pramod Kumar Julka ◽  
Chitra Sarkar ◽  
Dayanand Sharma ◽  
...  

e13034 Background: This study assesses the impact of 6 cycles of adjuvant TMZ (conventional arm) versus 12 cycles (Extended arm) on Progression free survival (PFS), evaluate the toxicity and correlate the outcome with EGFR, P53 and MIB I labelling Index. Methods: Between December 2010 to October 2012, 36 post operative patients of Glioblastoma between age 18-65 years and Karnofsky Performance Score (KPS) ≥ 70 were included. Patients were randomized to receive Radiation with a dose of 60 Gray in 30 fractions over 6 weeks at 2 gray/fraction with concomitant TMZ (75 mg/m2/day) and Adjuvant therapy with either 6 or 12 cycles of TMZ(150 mg/m2 for 5 days, 28 days cycle). Patients were then assessed monthly clinically and imaged with MRI/CT every 3 monthly or when symptomatic. Toxicity was assessed using CTCAE version 3.0. Statistical Analysis was done using SPSS version 17.0.Kaplan Meier method was used for analysis of survival and log rank test was used for assessing the impact of variables on survival. Results: Of 36 patients, 18 patients were treated in each arm. Median age and KPS in both the arms was 47 years and 80 respectively. 44 % patients in the conventional arm and 50% patients in the Extended arm underwent complete surgical resection. 22% patients in the conventional arm and 28% in the extended arm did not complete their intended treatment. Grade ¾ Thrombocytopenia was seen in 16% in the extended arm and 0% in the conventional arm.EGFR, P 53 and MIB 1 >20% was seen in 26%, 45% and 20% patients respectively, overall. Median follow up was 18 months for both the arms (Range 10-23 months).At last follow up,8 patients in each arm had progression. Median PFS was 10 months vs.18.4 months (p 0.47) in conventional and extended arm respectively. On Univariate analysis, patients with KPS ≤ 80 had poorer survival than those >80 (Median PFS 9.5 Months vs. 16.9 Months; p 0.02).Age, extent of resection, EGFR, P53, MIB 1 did not significantly alter survival in the two treatment groups. Conclusions: Our study showed that schedule of extended Temozolomide is well tolerated by patients and tend to have better progression free survival. Further prospective randomized studies are needed to validate the findings of our study.


2015 ◽  
Vol 39 (2) ◽  
pp. E7 ◽  
Author(s):  
Jörg Klekamp

OBJECT Ependymomas of the filum terminale provide specific surgical challenges due to their often enormous size, contact with nerve roots of the cauda equina and conus, and potential for subarachnoid dissemination. This study presents treatment results for these tumors over a 30-year period. METHODS Among 1447 patients with tumors of the spinal canal treated between 1980 and 2014, 618 patients presented with extramedullary tumors. Of these, 42 patients (25 males, 17 females) demonstrated a myxopapillary ependymoma in the lumbosacral region. Thirty-four patients underwent 36 operations for 39 such tumors. The mean patient age was 38 ± 14 years (range 11−73 years), with an average clinical history of 37 ± 67 months. Patients were followed through outpatient visits and questionnaires, with a mean follow-up of 10 years (127 ± 100 months). Twenty-seven operations were performed to treat de novo tumors and the remainder were undertaken on recurrent tumors. Short-term results were determined for individual symptoms, and tumor recurrence rates were calculated with Kaplan-Meier statistical analyses. RESULTS Subarachnoid dissemination was observed in 11 patients and was related to previous surgery in 9 patients and associated with extensive tumors in 2 patients. Gross-total resections (GTR) were achieved in 28 operations (77.7%) and subtotal resections in the remainder. Subtotal resections were restricted to unencapsulated ependymomas (61.5%). Radiotherapy was employed after 6 operations on unencapsulated tumors, with 5 of these also demonstrating subarachnoid seeding. Permanent surgical morbidity affected 3 patients who experienced permanent worsening of bladder function, while 7 patients showed no postoperative changes, and the remaining 26 operations were followed by improvements. Long-term outcome depended on the amount of resection and the presence of a tumor capsule. Eight of 9 tumor recurrences affected unencapsulated tumors, of which 3 had undergone GTR. The overall recurrence rates were 6.6%, 19.0%, and 37.0% after 1, 10, and 20 years, respectively. For unencapsulated ependymomas, the corresponding rates were 15.6%, 32.5%, and 66.2% after 1, 10, and 20 years, respectively, with significantly lower rates of 9.1% after 10 and 20 years for encapsulated tumors. Postoperative radiotherapy tended to prolong the recurrence-free interval for patients with unencapsulated tumors. Five patient deaths occurred during follow-up, of which 2 deaths were tumor related and occurred at 216 and 287 months after surgery. CONCLUSION Extramedullary ependymomas are slow-growing tumors in the lumbosacral region, sometimes with an indolent course for long periods of time. Despite their delicate location and often enormous size, surgical morbidity in experienced hands is low, with good chances for postoperative clinical improvements and very low recurrence rates after GTR for encapsulated tumors. The role of postoperative radiotherapy remains controversial. Radiotherapy may be considered after incomplete resections of unencapsulated tumors and/or for patients with subarachnoid dissemination.


2021 ◽  
Vol 12 ◽  
Author(s):  
Dan Cao ◽  
Yong Chen ◽  
Zhengqian Guo ◽  
Yibo Ou ◽  
Jian Chen

Objective: This study aimed to explore the immediate postoperative and long-term outcomes of central neurocytoma (CN) based on 15 years of experience in our institution.Methods: This single-institution study collected data of 43 patients with CN who underwent surgery between 2005 and 2020. We reviewed data of clinical, immediate postoperative outcome, and long-term outcome of patients. More specifically, we divided complications into neurological and regional complications groups.Results: Among the 43 patients with CN who underwent surgery, the transcortical (72.1%) or transcallosal (25.6%) approach was used. There were 18 patients (41.9%) who complained about postoperative neurological complications, including motor weakness (25.6%), memory deficit (18.6%), aphasia (7.0%), and seizure (4.7%). In addition, 18 patients suffered postoperative regional complications such as hydrocephalus (2.3%), hematoma (34.9%), infection (4.7%), and subcutaneous hydrops (2.3%). Only one-quarter of patients had suffered permanent surgical complications. The majority of patients recovered from the deficit and could turn back to normal life. There were no significant differences in the clinical outcomes between transcortical and transcallosal approaches. At a median follow-up of 61.8 months, the 5-year overall survival and progression-free survival were 87.0 and 74.0%, respectively. A multivariate Cox model analysis showed that the extent of resection was not related to progression-free survival. However, the extent of resection was significantly associated with overall survival, and gross total resection decreased the risk of death.Conclusions: Patients with CN show favorable outcomes after surgery. The transcortical and transcallosal approaches have similar postoperative complication rates and long-term follow-up outcomes. In terms of long-term prognosis, maximal safety resection should be the first choice of CN.


2020 ◽  
Vol 1 (2) ◽  
pp. 103-109
Author(s):  
Gyanendra Joshi ◽  
Binod Bijukachhe ◽  
Javed Ahmad Khan

Introduction: To report the treatment results of 19 patients who underwent excision of intradural extramedullary (IDEM) spinal tumors. Materials & Methods:  This is a retrospective study. Patients’ records were retrieved from the electronic database of Grande International Hospital. There were 19 IDEM spinal tumors excised over a period of 6 years between January 2013 and August 2019 by a single surgeon. There were 11 (57.9%) males and 8 (42.1%) females with an average age of 48.37±21.87 years (range, 5-79 years). The mean postoperative follow-up period was 12.87±14.88 months (range, 15 days - 60 months). The histopathological findings, locations of the tumors, and clinical results were analyzed. Neurological findings were evaluated using ASIA grading system and pain was evaluated using VAS score. Results:  Histopathologically the tumors were: 8 meningioma (42.1%), 4 schwannoma (21.1%), 4 neurofibroma (21.1%), 1 dermoid cyst (5.3%), 1 lipoma (5.3%), and 1 myxopapillary ependymoma (5.3%). Tumor locations were: dorsal in 10 (52.6%), lumbar in 5 (26.3%), lumbosacral in 2 (10.5%), dorsolumbar in 1 (5.3%), and cervical in 1 (5.3%) patient. Neurologic status of 7 patients was normal and 12 had neurologic involvement with 3 patients having Cauda Equina Syndrome (CES) preoperatively. At the final follow-up, 3-grade, 2-grade and 1-grade improvement in ASIA score was observed in 1, 6 and 1 cases, respectively. There was no change in the ASIA grade in 1 case. All 3 cases of CES achieved full neural recovery. Preoperative VAS score was 9.21±1.08 and improved significantly to 0.74±1.62 after surgery. Neurological improvement was seen in 91.67% with complete recovery in 75% of the cases and not a single case deteriorated neurologically post-surgery. Conclusion:  Most intradural-extramedullary spinal cord tumors are mostly benign and good clinical results can be obtained when adequately treated with surgery.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 2013-2013
Author(s):  
Marta Domenech ◽  
Carles Fabregat-Franco ◽  
Carlos Mesia ◽  
Anna Esteve ◽  
Sonia Del Barco Berron ◽  
...  

2013 Background: We previously presented our results of the GEINO 1401 trial that randomized patients diagnosed with glioblastoma and treated with chemoradiotherapy and adjuvant temozolomide (TMZ) followed by six cycles of TMZ, to receive an extended use of TMZ up to 12 cycles or to control. We found no differences in 6-months neither progression free survival (PFS) nor overall survival (OS). In this report we actualize our results and analyse long-term survivor patients (LTSP). Methods: The trial NCT02209948 randomized (ratio 1:1) 159 patients diagnosed with glioblastoma who had been treated with standard therapy to stop treatment or to continue up to 12 cycles of TMZ. Patients were stratified based on their O6-methylguanine-DNA-methyltransferasa (MGMT) methylation status and presence or absence of measurable disease at inclusion. We update here OS outcomes and analyse the data of LTSP defined as an OS over 30 months from diagnosis. Results: At a median follow-up of 20 months, 82.4% of the patients had died and 89.9% had progressed. The median OS from randomization was 22.0 months for the control arm and 18.2 for the experimental arm: HR 0.957 (95%CI 0.806-1.136, p = 0.615). At 2 years from randomization there were a 61% of survivors in the TMZ group and 62% in the control group. There were a 49.7% of LTSP showing no differences between TMZ and control group. We found a higher prevalence of methylated MGMT in LTSP, but no differences were shown in patients with or without measurable disease at inclusion, status of IDH and the use of bevacizumab after progression. Conclusions: Adding 6 cycles of TMZ after the first 6 adjuvant cycles confers no additional benefit in OS. Nearly 50% of the patients included in GEINO 1401 who had been previously treated with TMZ 6 cycles without progressing were LTSP. Clinical trial information: NCT02209948.


2012 ◽  
Vol 166 (6) ◽  
pp. 1061-1068 ◽  
Author(s):  
D S Olsson ◽  
M Buchfelder ◽  
K Wiendieck ◽  
N Kremenevskaja ◽  
B-Å Bengtsson ◽  
...  

ObjectiveMost patients who have been treated for craniopharyngioma (CP) are GH deficient (GHD). GH replacement therapy (GHRT) may stimulate tumour regrowth; and one of the concerns with long-term GHRT is the risk of tumour progression. Therefore, the objective was to study tumour progression in CP patients on long-term GHRT.DesignCase–control study.Patients and methodsThe criteria for inclusion of cases were: i) GHD caused by CP; ii) GHRT >3 years; and iii) regular imaging. This resulted in 56 patients (mean age at diagnosis 25±16 years) with a mean duration of GHRT of 13.6±5.0 years. As controls, 70 CP patients who had not received GHRT were sampled with regard to follow-up, gender, age at diagnosis and initial radiation therapy (RT).ResultsThe 10-year tumour progression-free survival rate (PFSR) for the entire population was 72%. There was an association (hazard ratio, P value) between PFSR and initial RT (0.13, <0.001) and residual tumour (3.2, <0.001). The 10-year PFSR was 88% for the GHRT group and 57% for the control group. Substitution with GHRT resulted in the following associations to PFSR: GHRT (0.57, 0.17), initial RT (0.16, <0.001), residual tumour (2.6, <0.01) and gender (0.57, 0.10). Adjusted for these factors, the 10-year PFSR was 85% for the GHRT group and 65% for the control group.ConclusionsIn patients with CP, the most important prognostic factors for the PFSR were initial RT and residual tumour after initial treatment. Long-term GHRT did not affect the PFSR in patients with CP.


Neurosurgery ◽  
1984 ◽  
Vol 14 (2) ◽  
pp. 204-210 ◽  
Author(s):  
Helen S. L. Chan ◽  
Laurence E. Becker ◽  
Harold J. Hoffman ◽  
Robin P. Humphreys ◽  
Bruce E. Hendrick ◽  
...  

Abstract Seven of fourteen children with spinal cord ependymoma had myxopapillary tumors of the filum terminale. These tumors made up 15.9% of all primary spinal neuroectodermal tumors in children (44 cases) seen during a 62-year period (1919 to 1981). Their clinical presentation, radiological features, pathological findings, treatment, and outcome are reported. Six of the seven patients were known to be alive at the time of writing. The seventh patient was lost to follow-up after 3 years without tumor recurrence. Of 5 patients whose primary mode of treatment was operation alone, 3 had intraspinal or intracranial recurrences. Despite tumor recurrences, 2 patients were long term survivors after further operation and irradiation, whereas the third patient recently received craniospinal irradiation for intracranial tumor recurrence. The 2 patients who did not have tumor recurrence after operation alone had been followed for 3 and 7 years, respectively. Two children with subtotal tumor resection and spinal irradiation had no recurrences at 1 and 17 years, respectively. Our data suggest that this unusual subtype of spinal ependymoma is not uncommon during childhood and has a good prognosis. All patients with this tumor require prolonged follow-up for tumor recurrence after operation and irradiation.


2013 ◽  
Vol 18 (1) ◽  
pp. 76-84 ◽  
Author(s):  
Kazuyoshi Nakanishi ◽  
Nobuhiro Tanaka ◽  
Naosuke Kamei ◽  
Toshio Nakamae ◽  
Bun-ichiro Izumi ◽  
...  

Object The pathophysiology of occult tethered cord syndrome (OTCS) with no anatomical evidence of a caudally shifted conus and a normal terminal filum is hard to understand. Therefore, the diagnosis of OTCS is often difficult. The authors hypothesized that the posterior displacement of the terminal filum may become prominent in patients with OCTS who are in a prone position if filum inelasticity exists, and they investigated prone-position MRI findings. Methods Fourteen patients with OTCS and 12 control individuals were examined using T2-weighted axial MRI with the patients in a prone position on a flat table. On each axial view, the distance between the posterior and anterior ends of the subarachnoid space (A), the distance between the posterior end of the subarachnoid space and the terminal filum (B), the distance between the posterior end of the subarachnoid space and the dorsal-most nerve among the cauda equina (C), and the distance between the posterior end of the subarachnoid space and the ventral-most nerve (D) were measured. The location ratios of the terminal filum, the dorsal-most nerve, and the ventral-most nerve were calculated by the ratio of A to B (defined as TF = B/A), A to C (defined as DN = C/A), and A to D (defined as VN = D/A), respectively. Patients underwent sectioning of the terminal filum with the aid of a surgical microscope. The low-back pain Japanese Orthopaedic Association score was obtained before surgery and at the final follow-up visit. Results On prone-position axial MRI, the terminal filum was separated from the cauda equina and was shifted caudally to posterior in the subarachnoid space in all patients with OTCS. The locations of the caudal cauda equina shifted to ventral in the subarachnoid space. The TF values in the OTCS group were significantly lower than those in the control group at the L3–4 (p = 0.023), L-4 (p = 0.030), L4–5 (p = 0.002), and L-5 (p < 0.001) levels. In contrast, the DN values in the OTCS group were significantly higher than those of the control group at the L-2 (p = 0.003), L2–3 (p = 0.002), L-3 (p < 0.001), L3–4 (p < 0.001), L-4 (p = 0.007), L4–5 (p = 0.003), and S-1 (p = 0.014) levels, and the VN values in the OTCS group were also significantly higher than those of the control group at the L2–3 (p = 0.022), L-3 (p = 0.027), L3–4 (p = 0.002), L-4 (p = 0.011), L4–5 (p = 0.019), and L5–S1 (p = 0.040) levels. Sections were collected during surgery for histological evaluation, and a decreased elasticity within the terminal filum was suggested. Improvements in the Japanese Orthopaedic Association score were observed at the final follow-up in all patients. Conclusions The authors' new method of using the prone position for MRI shows that the terminal filum is located significantly posterior and the cauda equina is located anterior in patients with OTCS, suggesting a difference in elasticity between the terminal filum and cauda equina.


Neurosurgery ◽  
2013 ◽  
Vol 73 (5) ◽  
pp. 777-790 ◽  
Author(s):  
Xiao-Qiang Wang ◽  
Hong Chen ◽  
Lin Zhao ◽  
Shi-Ting Li ◽  
Jie Hu ◽  
...  

Abstract BACKGROUND: Papillary meningioma (PM) is an uncommon meningioma subtype, and the clinical characteristics remain unclear. OBJECTIVE: To determine the clinical characteristics and prognosis of PM. METHODS: The clinical data of 30 PM patients were collected, the samples were reexamined, and the patients' prognoses were based on clinical observations and calculated according to the Kaplan-Meier method. RESULTS: The 30 patients included 16 males and 14 females (median: 34.0 years upon initial diagnosis). Of the 48 intracranial operations in the 30 patients, total removal was attained in 34 surgeries, and subtotal removal in 14 surgeries. Radiotherapy was provided in 20 patients. In 40 specimens with follow-up, 29 attained the positive aggressive factors. Six tumors showed positive progestogen receptor (PR) combined with negative Bcl-2. The median follow-up period was 39.0 months. Tumor recurrence occurred in 18 patients (median: 17.0 months); the recurrence rates following total removal and subtotal removal were 57.1% and 100%, respectively. Fourteen patients died of the recurrence. In the univariate analyses, positive aggressive factors (P = .021), positive PR combined with negative Bcl-2 immunoreactivity (P = .011), the extent of resection (P = .001), and radiotherapy (P = .002) were significantly related to progression-free survival. The MIB-1 labeling index was not significantly related to progression-free survival (P = .88). CONCLUSION: PM is a rare subtype of meningioma with a tendency of recurrence. The extent of resection is an important prognosis factor. The presence of positive histopathological index increases the recurrence risk. Positive PR combined with negative Bcl-2 immunoreaction might predict a good prognosis. Postoperative radiotherapy may play a vital role in prolonging the time to tumor recurrence.


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