Intramedullary Ependymoma Research Articles

A rare case of Intramedullary spinal ependymoma presenting as isolated neck pain

A rare case of Intramedullary spinal ependymoma presenting as isolated neck pain

Variety of preoperative MRI changes in spinal cord ependymoma of WHO grade II: a case series.

To report a case series of surgically proven spinal ependymomas of WHO grade II in which there were changes in the preoperative MRI characteristics over time. A total of 71 patients with spinal cord ependymoma of WHO grade II underwent surgery. There were ten cases in which surgery was performed at an average of 2.2years after the tumor was found. Cystic components, syringomyelia, hemorrhage "cap sign," Gd enhancement pattern, characteristic changes in MRI, MIB-1 index, and neurological assessment during the preoperative period were examined. Cases with a huge cyst showed further enlargement of the cyst on the caudal and rostral sides with hemosiderin formation over time and changes in the pattern of Gd enhancement. In contrast, cases without initial cyst did not show cyst formation, and nodular homogeneous lesion remained without changes in Gd enhancement. Regarding neurological status, two cases with cyst enlargement and hemosiderin formation had worsened non-independent gait preoperatively. MRI in cases of spinal ependymomas of WHO grade II showed characteristics such as hemorrhage and cyst formation that varied over time. In particular, cases with cyst and hemosiderin showed tumor enlargement, including enlargement of lesions on the caudal and rostral sides and enlargement of Gd-enhanced lesions. These characteristics might influence gait ability during preoperative period. We emphasize that early surgery is still the standard of care for cervical intramedullary ependymoma, and our findings in this study should not be interpreted to indicate that such early surgery is not necessary in symptomatic cases. These slides can be retrieved under electronic supplementary material.

Predictors of Functional Outcome after Spinal Ependymoma Resection.

Aim:Spinal ependymomas are among the most common intramedullary neoplasms in both adults and children. While surgical resection is the golden treatment standard, the role chemotherapy and radiotherapy have in patients with spinal ependymomas remains unclear. The aim of this study is to determine the predictors of functional outcome following spinal ependymoma resection to single out patients that may require adjuvant therapy.Methods:We conducted a retrospective study on patients that underwent spinal ependymoma resection in our institution in a 10-year period. Magnetic resonance imaging of the spine was used to set the diagnosis of an intradural/intramedullary neoplasm. All patients underwent either gross tumor resection or tumor mass reduction. Histological diagnosis and histopathological grading of spinal ependymoma were done for all collected samples. Patients’ general and neurological examination were performed early after the surgery (within the 1st week) and in a 6-month follow-up.Results:A total of 51 intradural and intramedullary ependymoma resection surgeries on 43 patients were performed. There were slightly more male patients (57%) and the average patient age was 41 years. About 76.5% of patients presented with a tumor affecting one vertebrae level, while 23.5% presented with tumors expanding over two or more spinal regions. Gross tumor resection was achieved in 80% of cases, while 25% of procedures were performed on a recurring ependymomas. Most of the tumors (57%) were classified as G2 histological grade, while 8% were anaplastic ependymomas. In 80% of cases, early postoperative patient status was either better or equivalent to the preoperative one, while in a 6-month follow-up, up to 60% of cases showed a significant improvement over the preoperative status. Different demographic and clinical parameters were not proven to be predictors of postsurgical patient outcome including age, gender, and initial neurological presentation. Interestingly, most tumor characteristics were also not associated with postoperative functional outcome (histological grade, number of vertebrae levels affected, whether it is a primary or recurrent tumor). Even the scope of surgical procedure did not affect the functional outcome. The spinal region affected by the tumor was proven to be a predictor of early postoperative outcome (ρ= 0.346, P = 0.033), with lumbar spine being associated with the best outcomes. As expected, the scope of the surgery and whether gross tumor resection or tumor mass reduction was performed were the only significant predictors of tumor recurrence (ρ= 0.391, P = 0.005).Conclusions:Spinal ependymoma resection is an efficient procedure that improves the patient outcomes. Spinal region affected by the tumor is likely to be the most important predictor of functional outcome, while the procedure scope seems to be the most important predictor of tumor recurrence.

Osteopathic Evaluation and Post-Surgical Rehabilitation Approach in a Patient With Myelopathy and Tetraparesis Related to Cervical Ependymoma: A Case Report

Abstract Neck pain with associated upper limb symptomatology presents commonly in primary care, musculoskeletal specialty, and osteopathic manipulative medicine (OMM) clinics. Thorough evaluation to determine the presence of red flags prior to providing treatment is a prerequisite for OMM providers. In the present case report, a 48-year-old right-hand-dominant woman with chronic neck pain and weakness in the left arm was found to have cervical myelopathy due to intramedullary ependymoma. Urgent surgical consultation facilitated appropriate spinal decompression followed by in-patient rehabilitation. To the author’s knowledge, this is the first case reported involving the use of osteopathic manipulative treatment (OMT) in the sub-acute rehabilitation phase following spinal cord tumor surgical decompression.

REMOVAL OF THE CERVICAL SPINAL CORD EPENDYMOMA

The paper describes long-term results of surgical treatment of a female patient with intramedullary ependymoma at the cervical level. Long relapse-free period was reported after the radical removal of intramedullary ependymoma. A complication developed after multilevel laminectomy required anterior fusion. Analysis of patient’s neurological symptoms and level of long-term social adaptation showed her full independence and preservation of working capacity.

Evidence-based review and guidelines for the management of myxopapillary and intramedullary ependymoma

Evidence-based review and guidelines for the management of myxopapillary and intramedullary ependymoma

Intramedullary ependymoma: long-term outcome after surgery.

Overall (OS) and progression-free survival (PFS) of patients undergoing spinal ependymoma resection has been frequently reported. Contrarily, OS and PFS of purely intramedullary ependymomas have not been clearly determined yet. The data of 37 patients undergoing resection of an intramedullary ependymoma (IE) from January 2000 to December 2016 were analysed retrospectively. The mean age was 46years. The male:female ratio was 24:13. The median duration of symptoms was 12months. Sixty-two per cent of ependymomas were in the cervical, 24% in the thoracic, and 14% in the conus region in our series. The median volume was 1.3ml. A syrinx was found in 49% and a cyst in 32%. GTR was achieved in 89%, STR in three (8%), and PR in one patient (3%). Median follow-up was 114months. PFS was 87%, 82%, and 82% at 5, 10, and 15years, respectively. OS was 97%, 88%, and 63% at 5, 10, and 15years, respectively. There was a significant difference in PFS depending on the extent of resection and in OS depending on the pre-operative clinical status. There was no significant difference in OS and PFS regarding the other examined influencing factors. GTR resection was the most important factor influencing PFS. According to our results OS of IEs is much worse than that of spinal ependymomas. Our analysis confirms that patients with good pre-operative (McCormick grade 1 and 2) clinical status have significantly better OS than patients with McCormick grade 3 and higher.

Spinal intramedullary ependymoma: surgical approaches and outcome.

Intramedullary ependymomas are uncommon tumors that can occur within the medullary substance of the spinal cord. Despite this difficult location, they are typically benign tumors that can most often be removed completely with an acceptable surgical risk. Therefore, the recommended management approach is usually surgical excision. This review will consider the historical context in which surgeons began treating these tumors and then review the more recent literature that guides their current management.

Surgical treatment of intramedullary ependymomas

ObjectThe aims of this study were to present the results of surgery for intramedullary ependymomas (IEs), analyze complication and recurrence rates, and analyze factors that might influence outcome. MethodsThe charts of 29 consecutive patients (women – 8, men – 21; mean age – 38 years; range: 18–72) operated for IE were retrospectively analyzed. Mean follow-up was 9 years. Eighteen tumors (62%) were located in the cervical or cervicothoracic spine, and average tumor length was four spinal levels. Twenty patients (69%) presented with neurological deficit. ResultsGross total resections (GTRs) comprised 87% of cases, subtotal resections (STRs) 10%, and partial resections 3%. The neurological outcome on postoperative day 1 was as follows: modified McCormick scale (mMS) grade I – 6%, grade II – 21%, grade III – 21%, grade IV – 31%, and grade V – 21%; at follow-up, outcomes were mMS grade I – 42%, grade II – 34%, grade III – 10%, and grade V – 14% of patients. Compared to the preoperative period, 69% of patients deteriorated postoperatively; however, 62% improved or remained without deficit in follow-up, and deterioration persisted in 24%. The functional results were significantly worse when the intraoperative monitoring potentials dropped below 50% (p=0.005) and if the tumor involved >3 spinal levels (p=0.039). Fourteen postoperative complications in 10 patients (34%) included respiratory failure (14%), pneumonia (7%), urinary infection (10%), bed sores (10%), and CSF leak (7%). Two tumors progressed after STR, with progression-free survival times of 5 and 14 years. No recurrence was observed after GTR. ConclusionsTotal tumor resection is the treatment of choice in cases of IEs: no tumor re-growth occurred after total resection, 86% of patients were independent at follow-up, and the 10-year survival rate was 79%.

Surgical strategies of intramedullary medullocervical ependymomas: a report of 120 cases

Objective To explore the clinical characteristics and microsurgical skills of intramedullary medullocervical ependymomas for increasing the rate of gross total resection (GTR) and decreasing the disabling rate. Methods A total of 120 patients with medullocervical ependymomas were admitted to Department of Neurosurgery, Changzheng Hospital, the Second Military Medical University of PLA, from January 2001 to November 2016. A retrospective analysis was conducted regarding their clinical manifestations, imaging characteristics, microsurgical manipulations, extent of resection and postoperative follow-up data. In all cases, the tumor resection was performed using posterior suboccipital midline approach. Results Among the 120 patients, GTR was achieved in 111 (92.5%) cases and subtotal resection was performed in 9 (7.5%) cases. The maximal tumor size was 25.0×2.0×2.0 cm and affected all cervical levels, and the minimal size of the tumor was 1.0×1.0×0.6 cm. Follow-up was successfully conducted in 106 (95.5%) patients and for 3 to 108 months with an average of (23.0±18.5) months. The patient's sensory and motor functions were relieved or remained unchanged post surgery in 83 (78.3%) cases and aggravated in 22 (20.8%) cases. One (0.9%) death occurred. Four patients (3.8%) were reoperated on due to tumor recurrence. The neurological function was graded based on the McCormick system as 60 (56.6%) cases in grade I, 28 (26.4%) cases in grade II, 16 cases (15.1%) in grade III and 1(0.9%) case in grade IV. Conclusion For most intramedullary medullocervical ependymomas, proper surgical strategies and qualified operational skills, combined with imaging and neuroelectrophysiological technologies could be applied to realize the GTR of tumors and preservation of neurological functions. Key words: Medulla oblongata; Spinal cord; Ependymoma; Microsurgery; Prognosis

Differential Diagnosis of Quadriparesis in an Otherwise Healthy Adult Male by a Physical Therapist

HISTORY: A 52-year-old male was referred to Physical Therapy (PT) by his primary care physician for progressive quadriparesis related to suspected peripheral neuropathy (PN). The patient was healthy and active until two years prior when he began experiencing weakness and paresthesias in both hands. This progressed to his right lower extremity, then all four extremities, and by the time of the PT evaluation, the patient was no longer able to stand. He also endorsed several episodes of bladder incontinence, constipation, and painful muscle spasms. He was scheduled for imaging of the spine and a follow-up with his physician in two months. Review of the medical record revealed that the patient was evaluated by a physical therapist nine months prior. His presentation then was consistent with the working diagnosis of PN. However, a sinister diagnosis was suspected due to the involvement of all four extremities without risk factors for polyneuropathy. At that time, no further steps were taken as the patient did not follow up in clinic. PHYSICAL EXAMINATION: The patient presented in a wheelchair, though was unable to propel or stand without assistance due to significantly worsening weakness. Neurologic exam revealed several upper motor neuron signs including hypertonicity and hyperreflexia. This presentation was no longer consistent with the working diagnosis of PN (see table).Table: No title available.DIFFERENTIAL DIAGNOSIS: 1. Cervical spine stenosis with myelopathy 2. Space occupying lesion in cervical region 3. Progressive motor neuron disease TESTS AND RESULTS: Cervical/Thoracic spine MRI: 12.4 cm intradural intramedullary mass from C5 to T5, likely related to multiple tumoral cystic areas, with syrinx from T3 to T4 FINAL DIAGNOSIS: Intradural intramedullary ependymoma extending from C5 to T5 with associated syrinx TREATMENT AND OUTCOMES: Based on the examination and presence of multiple red flags, the physical therapist referred the patient to the Emergency Department for further evaluation.

Application of intra-operative yellow fluorescence to intramedullary spinal cord ependymoma resection

Objectives To study the application of intraoperative yellow fluorescence to intramedullary spinal cord ependymoma resection. Methods The clinical data of 83 in-patients with spinal cord tumors were retrospectively analyzed, who were admitted to Department of Neurosurgery, Beijing Tsinghua Changgung Hospital from December 2014 to December 2015. Among them, the diagnosis of intramedullary spinal cord ependymoma was histologically confirmed in 56 cases (39 men and 17 women), whose mean age was 43.5±4.2 years old (range: 31～65 years). All cases in this study received preoperative MRI scan to demonstrate the location, size and segment of tumors.Fluorescein sodium(3～4 mg/kg) was administered intravenously 30 minutes prior to anesthesia. Tumor resection was performed under alternation between conventional white light and yellow fluorescent light source. At 3 months post operation, MRI scans were conducted for observation of residual tumors, and spinal cord function was evaluated according to the McCormick scale. The mean follow-up period was 3 months. Results The rate of gross total resection (GTR)was 100.0% in this series of 56 cases. Among them, 47 cases were significantly stained under fluorescein sodium model (correlated with preoperative MRI enhancement) and received fluorescence-guided tumor resection.The other 9 cases with little staining (including 2 cases with little preoperative MRI enhancement and 4 cases demonstrating uneven MRI enhancement due to tumor apoplexy) underwent resection with conventional white light. Compared with 7-day and 1-month follow-ups, the 3-month postoperative improvement rates of sensory and motor functions were 83.0%(44/53)and 82.4%(42/51), respectively(P 0.05). Based on the McCormick grading system, 3.6%(2), 55.4%(31), 25.0%(14)and 16.1%(9)of the 56 patients were preoperatively categorized as gradeⅠ, Ⅱ, Ⅲ and Ⅳ, respectively. At 3 months post operation, 7.2%(32), 21.4%(12), 14.3%(8) and 7.1%(4) of the patients were categorized as gradeⅠ, Ⅱ, Ⅲ and Ⅳ, respectively, which demonstrated significant difference compared with preoperative conditions (P<0.01). The patients preoperatively categorized as grade Ⅰ and Ⅱ reported good surgical outcomes, while grade Ⅲ and Ⅳ patients did not. MRI results demonstratedno residual tumors or recurrence 3 months post operation. Conclusion Fluorescein sodium could be used for intramedullary ependymoma resection and contribute to complete excision of tumors. Key words: Spinal cord neoplasms; Ependymoma; Fluoresceins; Neurosurgical procedures

Gross total resection rates of grade II/III intramedullary ependymomas using the surgical strategy of en-bloc resection without intra-operative neurophysiological monitoring

Introduction: Grade II and III intramedullary ependymomas [IME] are circumscribed with a plane of cleavage that should facilitate high gross total resection rates (GTR). Gross total resection of grade II/III IME is superior to subtotal resection (STR) and radiotherapy (RTx) for progression-free and overall survival. We sought to compare our GTR with other series that have utilised standard intraoperative monitoring techniques and we explored factors that may influence rates of resection.Materials and methods: Database search and retrospective chart and radiological review of all grade II or III spinal ependymomas over a 10-year period from the senior authors practice. Comprehensive PubMed search to identify similar series that identified histology, McCormick Function scores (MCC) preoperatively and post-operatively, surgical strategy and use of intraoperative monitoring. Standard statistical analysis was performed.Results: Seventeen patients were identified: 16 grade II and one grade 3. GTR was 94.12%. Factors that correlated with a decline in MCC were longitudinal extension of the tumour (p = 0.0238) and presentation with motor signs and symptoms (p = 0.0223). There was no statistical difference between preoperative factors that influence post-operative outcomes in the current study when compared with other published series. There was no statistical difference between preoperative and postoperative MCC scores between our series and other published series.Discussion: The current series with a GTR of 94.12% compares favourably with other published series with GTRs of 55.8–84% with no significant difference in functional outcomes. Series with low GTRs should examine their operative strategy or false-positive alarm rates which may lead to higher STRs. This series should be viewed as a unique opportunity to benchmark GTRs of circumscribed intramedullary tumours.

Intramedullary ependymoma: Microsurgical resection technique

Spinal ependymomas are predominantly slow-growing lesions constituting approximately 30–88% of primary spinal intramedullary tumors. They usually present as circumscribed lesions, with regular margins and a clear surgical plane. Gross-total resection is often feasible and potentially curative but neurosurgeons should keep in mind that the ultimate goal of surgery is the preservation of spinal cord function. We present the surgical technique to safely resect an intramedullary ependymoma using a posterior median sulcus approach. A brief description of current management of this pathology is also presented.

Spinal myxopapillary ependymoma in an adult male presenting with recurrent acute low back pain: a case report.

BackgroundSpinal intramedullary ependymomas are very rare and occur more commonly in the cervical and upper thoracic regions. These neoplasms tend to manifest in young adulthood, and patients typically present with mild clinical symptoms without objective evidence of neurologic deficits. The mean duration of symptoms is 40 months until the lesion is diagnosed.Case PresentationA 48-year-old male police officer was referred to a chiropractic clinic by a general practitioner for the evaluation of recurrent acute low back pain (LBP). Although the first episode of LBP was resolved, the clinical examination during the second episode revealed subtle changes that warranted referral to magnetic resonance imaging (MRI). The MRI revealed a spinal myxopapillary ependymoma.ConclusionBecause the primary symptoms of spinal intramedullary ependymomas can mimic ordinary LBP presentations, in particular lumbar intervertebral disc herniations, clinicians need to be sensitive to subtle changes in the clinical presentation of LBP patients. Prompt referral to advanced medical imaging such as MRI and early neurosurgical intervention is key to achieve best possible outcomes for patients with spinal intramedullary ependymomas.

Spinal ependymomas. Part 1: Intramedullary ependymomas.

OBJECT Ependymomas represent the most common intramedullary tumor in adults. Despite their usually well-defined dissection plane, surgical morbidity has been documented to be considerably higher compared with other intramedullary entities. This study presents an analysis of risk factors for surgical morbidity and data on long-term results for intramedullary ependymomas. METHODS Among 1447 patients with tumors of the spinal canal treated between 1980 and 2014, 309 patients presented with intramedullary tumors. One hundred patients with intramedullary ependymomas underwent 102 operations. Mean age was 44 ± 15 years (range 8-74 years). Patients were followed by outpatient visits and questionnaires, with a mean follow-up of 77 ± 91 months. Short-term results were determined for individual symptoms and the McCormick Scale, whereas tumor recurrence rates were calculated with Kaplan-Meier statistics. RESULTS Compared with cervical ependymomas, those of the thoracic spine were associated with more severe motor deficits and gait problems at presentation. A total of 86.3% of patients with intramedullary ependymomas underwent gross-total resection (GTR). A low preoperative McCormick grade and first surgery were the strongest predictors for a GTR. Postoperatively, 67.6% of patients demonstrated a worse neurological state at discharge from the hospital. This deterioration was transient for 40.1% of the patients and permanent for 27.5%. In the long term, the McCormick grade remained unchanged from the preoperative grade in 74.5% of patients, while it was improved in 5.9% of patients and increased after surgery in 19.6% of patients. According to a multivariate analysis, the risk of permanent morbidity increased with a thoracic level of the ependymoma, advanced age, a long clinical history, presence of a tumor hemorrhage, and surgery on a recurrent tumor. In the long term, tumor recurrence rates correlated significantly with the amount of resection (4.2% and 18.5% in 20 years after GTR and partial resections, respectively). Postoperative neuropathic pain syndromes affected 37.0% of patients, whereas 4% demonstrated a postoperative myelopathy related to cord tethering at the level of surgery. CONCLUSIONS Intramedullary ependymomas are tumors best treated surgically. A complete resection indicates cure for the overwhelming majority of these patients. Surgery should be performed early by neurosurgeons who deal with these lesions on a regular basis to achieve high GTR rates. Permanent surgical morbidity varies most according to tumor location and patient age.

Progression free survival and functional outcome after surgical resection of intramedullary ependymomas

We present a 15year institutional analysis of the factors affecting progression free survival (PFS) and overall survival (OS) in patients undergoing attempted resection of adult intramedullary spinal cord ependymomas. Intramedullary spinal cord tumors are rare but important clinical entities, and ependymomas are the most commonly encountered intramedullary tumor. In total, 53 adult patients over the span of 15years were analyzed for OS, PFS, and the effects of plane of dissection (POD) and gross total resection (GTR) on functional and long term outcomes. The mean age was 45years and median follow-up was 54months. The follow-up neurological outcome and modified McCormick scale were used to determine the functional outcome. Kaplan–Meier curves were used to calculate progression and survival. The overall ability to achieve GTR was significantly correlated to identification of an intraoperative POD (p<0.001). There was a trend towards increased PFS with the ability to achieve a GTR. There was no significant difference in the pre- and postoperative functional outcome scores. The ability to achieve a GTR is strongly correlated to the identification of a POD in ependymomas. There is a trend towards an increased probability of PFS in intramedullary spinal cord tumors when GTR is achieved. The resection of these tumors is likely to halt, but not reverse, neurological deterioration.

Dissemination of intramedullary anaplastic ependymomas

Two cases of 6 years old and 20 years of ages were operated for intramedullary anaplastic ependymomas. A gross total microsurgical excision of their tumors was achieved in both the cases, barring the infiltrated nerve roots of one case (6 years old, child). Standard doses of irradiation were offered to both the cases following surgery. The dissemination of disease occurred at six months and one year and 10 months duration in both. An extensive spread of ependymoma involved full spinal cord and brain stem in the first case, while malignant meningitis, mild hydrocephalus and a distal recurrence occurred in another case. It was concluded that (1) anaplastic ependymomas have high potential for dissemination anywhere in neuraxis (2) dissemination may occur without a local recurrence of tumor at primary site (3) gross total microsurgical excision and irradiation does not offer resistance to dissemination.

INTRAMEDULLARY EPENDYMOMAS: LONG-TERM RESULTS OF SURGICAL TREATMENT

Objective. To study the long-term results of surgical treatment of intramedullary ependymomas. Material and Methods . Spinal ependymomas were detected in 91 (20.2 %) cases of a total number of operated patients. Intramedullary location of the tumor was diagnosed in 41 (45.0 %) patients. Ependymoma was benign in 78.1 % of cases and anaplastic in 9 cases (21.9 %). Radical removal of the tumor was achieved in 36 (87.8 %) patients, subtotal resection was performed in 5 cases (12.2 %). Results . Gross neurological symptoms persisted in the long-term period in 6 (24.0 %) patients. Structural changes in the spine associated with surgical intervention was found only in 12.0 % of cases, relapses and continued growth of the tumor - in 16.1 %. Early postoperative mortality was 4.8 % (2 patients out of 41 died). In the late postoperative period, four more patients died, so that the overall mortality was 14.6 %. Five-year survival rate of patients corresponded to 80.6 %. Conclusion . Radical surgery is an effective treatment for intramedullary ependymomas. The prognosis of intramedullary ependymoma may be considered as favorable in case of radical removal of the tumor, its benign nature, and good functional outcome of surgical treatment.

Management of cervical intramedullary ependymomas with a unilateral approach: 3-dimensional operative video.

Management of cervical intramedullary ependymomas with a unilateral approach: 3-dimensional operative video.