Intramedullary Ependymoma Research Articles

Clinic and treatment of intramedullary ependymomas

Objective — to improve the results of treatment of patients with intramedullary ependymomas. Materials and methods. The results of treatment of 29 patients (14 men and 15 women aged 18 to 64 years) with intramedullary ependymomas operated on at the Romodanov Institute of Neurosurgery of the National Academy of Medical Sciences of Ukraine were analysed. All patients who were scheduled for surgery underwent magnetic resonance imaging (MRI) with paramagnetic contrast agent. The preoperative and postoperative neurological status was assessed using the modified McCormick scale. Results. Microsurgical technique for removal of spinal cord tumors was used in all 29 observations. According to the volume of tumor removal, operations with total (12 observations), subtotal (9 observations) and partial (8 observations) removal were distinguished. Removal of at least 95 % of the tumor volume, which was confirmed by visualization with a microscope during surgery and early postoperative MRI, was considered total. At the time of discharge from the hospital, in 11 patients (37.9 %) the neurological condition improved compared to the preoperative period, in 13 (44.8 %) it remained unchanged, and in 5 (17.2 %) there was an increase in neurological symptoms. 12 patients were examined 12—36 months after the operation. Improvement compared to the preoperative period was found in 10 patients, and no changes in 2. There were no cases of worsening of neurological symptoms during this period. MRI performed before discharge from the hospital showed almost normal spinal cord diameter in the area of surgical procedures, and MRI performed ≥ 3 months after total tumour removal showed atrophic changes in the spinal cord with ependymoma localisation at the thoracic level. Conclusions. Intramedullary ependymomas can be totally removed, and surgical intervention must be carried out immediately after the diagnosis before the appearance of signs of increasing neurological deficit. Thanks to the use of microsurgical techniques and an intraoperative microscope, it is possible to achieve positive functional results during the total removal of intramedullary tumors.

Commentary: C4 to C7 Laminoplasty for Resection of an Intradural Intramedullary Ependymoma: 2-Dimensional Operative Video.

Commentary: C4 to C7 Laminoplasty for Resection of an Intradural Intramedullary Ependymoma: 2-Dimensional Operative Video.

Removal of a Large Laterally Located Spinal Cord Intramedullary Ependymoma Through a Lateral Myelotomy: 2-Dimensional Operative Video.

Removal of a Large Laterally Located Spinal Cord Intramedullary Ependymoma Through a Lateral Myelotomy: 2-Dimensional Operative Video.

Case report of selumetinib as a novel therapy in a neurofibromatosis type 2-associated ependymoma

We report partial response (PR) to novel therapy with selumetinib in a patient with Neurofibromatosis type 2 (NF2). A 25-year-old male presented with bilateral vestibular schwannomas, spinal cord intramedullary ependymomas, cranial and spinal meningiomas, spinal nerve root mixed schwannoma-neurofibromas, and peripheral nerve sheath tumors. He tested negative for germline NF2, SWItch/Sucrose Non-Fermentable-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1), and leucine zipper like transcription regulator 1 (LZTR1) mutations. Molecular analysis of a resected cervical spine schwannoma-neurofibroma demonstrated an isolated somatic SMARCB1 mutation. Due to progression of all tumors, he was treated medically with both everolimus (10 mg/day) and selumetinib (25 mg/kg BID), but rapidly transitioned to selumetinib monotherapy due to everolimus toxicity. Three months of treatment resulted in PR in one spinal ependymoma and stable disease in other tumors. This partial response was quantified by the differences in units of intensity in pre- and post-treatment MR image. To the best of our knowledge, this is the first reported case for using selumetinib in NF2-associated tumors or ependymomas.

Association of early surgery and absence of ataxia with full recovery after spinal intramedullary ependymoma resection.

Spinal intramedullary ependymomas (IEs) represent a well-defined tumor entity usually warranting resection. Factors that determine full long-term neurological recovery after resection are seldomly reported on in larger clinical series. In this study, the authors aimed to highlight the neurological outcome of patients with IEs after resection, with a focus on full neurological recovery, and to explore possible risk factors for the absence of neurological amelioration to an optimal function after surgical treatment. A single-center retrospective analysis of all patients undergoing surgery for IEs between 2007 and 2021 was performed. Data collection included patient demographics, symptoms, clinical findings, histopathological diagnosis, surgical procedures, complications, and neurological outcome. Patients harboring a favorable outcome (modified McCormick Scale [mMS] grade of I) were compared with patients with a less favorable outcome (mMS grade ≥ II) at the final follow-up. In total, 72 patients with a histologically diagnosed IE were included. IEs in those patients (41 males, 31 females; median age 51 [IQR 40-59] years) mostly occurred in the cervical (n = 40, 56%) or thoracic (n = 23, 32%) spine. Upon admission, motor deficits or gait deficits (mMS grade ≥ II) were present in 29 patients (40%), with a median mMS grade of II (IQR I-II). Gross-total resection was achieved in 60 patients (90%), and the rate of surgical complications was 7%. Histopathologically, 67 tumors (93%) were classified as WHO grade 2 ependymomas, 3 (4%) as WHO grade 1 subependymomas, and 2 (3%) as WHO grade 3 anaplastic ependymomas. After a mean follow-up of 863 ± 479 days, 37 patients (51%) had a fully preserved neurological function and 62 patients (86%) demonstrated an mMS grade of I or II. Comparison of favorable with unfavorable outcomes revealed an association of early surgery (within a year after symptom onset), the absence of ataxia or gait disorders, and a low mMS grade with full neurological recovery at the final follow-up. A subgroup of patients (n = 15, 21%) had nonresolving deterioration at the final follow-up, with no significant differences in relevant variables compared with the rest of the cohort. The data presented solidify the role of early surgery in the management of spinal IEs, especially in patients with mild neurological deficits. Furthermore, the presence of gait disturbance or ataxia confers a higher risk of incomplete long-term recovery after spinal ependymoma resection. Because a distinct subgroup of patients had nonresolving deterioration, even when presenting with an uneventful history, further analyses into this subgroup of patients are required.

Thoracic laminectomy and midline myelotomy for resection of a spinal ependymoma.

Spinal cord ependymomas comprise 25% of all intramedullary tumors and are typically treated with resection. A man in his mid-60s presented with imbalance and sensory deficits in both lower extremities, and a spinal thoracic intramedullary ependymoma spanning the levels T2 and T3 was diagnosed. After a laminectomy was performed, the tumor was microsurgically resected, and the patient demonstrated no neurological deficits on postoperative examination. Subsequent MRI showed complete resection of the tumor. This video showcases a thoracic intramedullary ependymoma resected using careful microdissection into the median raphe as a safe entry zone to preserve neurological function.

Spinal cord tractography and neuromonitoring-based surgical strategy for intramedullary ependymoma.

Because the spinal cord contains a rich concentration of longitudinal and transversal fibers in a very small area, intramedullary surgery could result in a high likelihood of morbidity. In this video, the authors demonstrate the microsurgical technique and surgical skills used to perform excision of an intramedullary ependyma. The authors also present tools (electrophysiology and neuroimaging) that are useful for surgical decision-making and planning, and thus are used intraoperatively, that allow safer and more effective resection of an intramedullary tumor.

P11.85.B LONG-TERM OUTCOME AFTER INCOMPLETE RESECTION OF INTRAMEDULLARY GRADE II EPENDYMOMAS WITHOUT ADJUVANT RADIOTHERAPY

Abstract BACKGROUND Ependymomas are the most frequent intramedullary tumors in adults. While gross total resection is the aim of surgery, tumor infiltration might limit resection. In case of subtotal removal, the adjuvant management remains unclear. The aim of our study was to assess the need for adjuvant radiotherapy after incomplete resection of grade II intramedullary ependymomas (IME-II). MATERIAL AND METHODS We retrospectively reviewed all cases of IME-II operated in a single tertiary neurosurgical center from 2009 to 2018. Patients with anaplastic or myxopapillary ependymomas, and patients with a follow-up of less than 3 years were excluded. RESULTS We included 46 patients: 19 (41.3%) had gross total resection, 21 (45.7%) had subtotal resection and 6 (13%) had partial resection. None of the patients underwent adjuvant radiotherapy. Over a median follow-up of 79 months (range = 36-186), 7 patients presented a radiological tumor progression with a mean delay of 50.9 months (range =18-85), of which 2 were symptomatic (4.3%). Progression-free survival (PFS) was 90.1% at 5 years and 76.8% at 10 years. Extent of resection was the only significant risk factor for secondary tumor progression (p=0.012). 4 of the 7 patients with recurring IME-II were treated: 3 patients had a second surgery, leading to 2 GTR and 1STR, followed by radiotherapy in one case, and 1 patient underwent radiotherapy alone. CONCLUSION In this study, the rate of symptomatic progression and retreatment after incomplete resection of IME-II without adjuvant radiotherapy was low, supporting a conservative approach in such cases.

Giant sacral schwannoma in a neurofibromatosis type 2 patient

BackgroundNeurofibromatosis type 2 is an autosomal dominant disorder, mainly characterized by multiple neurological lesions, such as schwannomas, meningiomas, neurofibromas and intramedullary ependymomas. Schwannomas are usually small circumscribed lesion. Sacral location of a schwannoma with cystic change is a very rare finding. We are presenting one such case with giant cystic schwannoma with fluid–fluid levels in sacral region.Case presentationWe present a case of 13-year-old female patient, presenting with pelvic pain and gradually progressive bilateral lower limb weakness. On MRI, giant cystic schwannoma with internal fluid–fluid levels was noted in sacral region, extending anteriorly into the presacral region, causing mass effects on pelvic organs, which explained the cause of her symptoms. She also showed the presence of bilateral vestibular schwannoma and multiple small cerebral lesions, leading to the diagnosis of neurofibromatosis type 2.ConclusionsOur current case of neurofibromatosis type 2, diagnosed by presence of bilateral vestibular schwannoma, shows atypically large sacral cystic schwannomas and cerebral subcortical lesions, probably representing glial microhamartomas. Sacral schwannomas can be of giant size with cystic changes and fluid–fluid levels, mimicking aneurismal bone cyst, as in current case.

Long-Term Outcomes after Incomplete Resection of Intramedullary Grade II Ependymomas: Is Adjuvant Radiotherapy Justified?

Ependymomas are the most common intramedullary tumors in adults. While gross total resection is the aim of surgery, tumor infiltration might limit resection. In cases of subtotal removal, the necessary adjuvant management remains unclear. The aim of our study was to assess the need for adjuvant radiotherapy after an incomplete resection of grade II intramedullary ependymomas (IME-II). We retrospectively reviewed all cases of IME-II operated upon at a single tertiary neurosurgical center from 2009 to 2018. Patients with anaplastic or myxopapillary ependymomas, and patients with a follow-up of less than three years, were excluded. We included 46 patients: 19 (41.3%) had a gross total resection; 21 (45.7%) had a subtotal resection; and 6 (13%) had a partial resection. None of the patients underwent adjuvant radiotherapy. Over a median follow-up of 79 months (range = 36-186), seven patients presented a radiological tumor progression with a mean delay of 50.9 months (range = 18-85), of which two were symptomatic (4.3%). Progression-free survival (PFS) was 90.1% at 5 years and 76.8% at 10 years. The extent of the resection was the only significant risk factor for secondary tumor progression (p = 0.012). Four of the seven patients with recurring IME-II were treated: three patients had a second surgery, leading to two GTR and one STR, followed by radiotherapy in one case, and one patient underwent radiotherapy alone. In this study, the rate of symptomatic progression and retreatment after incomplete resection of IME-II without adjuvant radiotherapy was low, suggesting a conservative approach in such cases.

The Primtif Spinal Cord Localization of Ependymoma about 2 Cases Treated by Surgery and Adjuvant Radiotherapy and Review of the Literature

Primary intramedullary ependymomas are rare tumors, characterized by a slow evolution. We report 2 cases of primary intramedullary ependymomas diagnosed following sensory-motor disorders of the 2 lower limbs, both patients underwent incomplete surgery followed by adjuvant radiotherapy and surveillance at the Mohammed VI University Hospital in Marrakech.

Resection of a ventral intramedullary spinal cord ependymoma through an anterior cervical approach: illustrative case.

Although posterior myelotomy leaves patients with dorsal column deficits, few reports have explored the anterior cervical approach for cervical intramedullary tumors. The authors describe the resection of a cervical intramedullary ependymoma through an anterior approach with a two-level corpectomy and fusion. A 49-year-old male presented with a C3-5 ventral intramedullary mass with polar cysts. Because of the ventral location of the tumor and the added benefit of avoiding a posterior myelotomy and dorsal column deficits, an anterior C4-5 corpectomy offered a direct route and excellent visualization of the ventrally located tumor. After a C4-5 corpectomy, microsurgical resection, and C3-6 anterior fusion with a fibular allograft filled with autograft, the patient remained neurologically intact. Magnetic resonance imaging (MRI) on postoperative day (POD) 1 confirmed gross-total resection. The patient was extubated on POD 2 and was discharged home on POD 4 with a stable examination. At 9 months, the patient developed mechanical neck pain refractory to conservative treatment and underwent a posterior fusion to address pseudarthrosis. MRI at 15 months showed no evidence of tumor recurrence with the resolution of neck pain. An anterior cervical corpectomy provides a safe corridor to access ventral cervical intramedullary tumors and avoids posterior myelotomy. Although the patient required a three-level fusion, we believe the tradeoff of decreased motion compared to dorsal column deficits is preferred.

Predictors of Progression-Free Survival in Patients With Spinal Intramedullary Ependymoma: A Multicenter Retrospective Study by the Neurospinal Society of Japan.

Ependymoma is the most common spinal intramedullary tumor. Although clinical outcomes have been described in the literature, most of the reports were based on limited numbers of cases or been confined to institutional experience. The objective of this study was to analyze more detailed characteristics of spinal intramedullary ependymoma (SIE) and provide clinical factors associated with progression-free survival (PFS). This retrospective observational multicenter study included consecutive patients with SIE in the cervical or thoracic spine treated surgically at a total of 58 institutions between 2009 and 2020. The results of pathological diagnosis at each institute were confirmed, and patients with myxopapillary ependymoma, subependymoma, or unverified histopathology were strictly excluded from this study. Outcome measures included surgical data, surgery-related complications, postoperative systemic adverse events, postoperative adjuvant treatment, postoperative functional condition, and presence of recurrence. This study included 324 cases of World Health Organization grade II (96.4%) and 12 cases of World Health Organization grade III (3.6%). Gross total resection (GTR) was achieved in 76.5% of cases. Radiation therapy (RT) was applied after surgery in 16 cases (4.8%), all of which received local RT and 5 of which underwent chemotherapy in combination. Functional outcomes were significantly affected by preoperative neurological symptoms, tumor location, extent of tumor resection, and recurrence. Multivariate regression analysis suggested that limited extent of tumor resection or recurrence resulted in poor functional outcomes. Multiple comparisons among the groups undergoing GTR, subtotal resection and biopsy, or partial resection of the tumor showed that the probability of PFS differed significantly between GTR and other extents of resection. When GTR can be safely obtained in the surgery for SIE, functional maintenance and longer PFS can be expected.

Massive Intramedullary Ependymoma: A Case Report

Introduction: Intramedullary ependymoma (IE) is adults' most common intramedullary spinal tumor. Tumors usually extend one to eight segments in the cervical region. In this case report, we reported a patient with massive IE spanning from the fourth ventricle to the T4 segment of the spinal cord, which surgically treated with laminectomy from occiput to T4 Case Presentation: A 42-year-old man who is a known case of IE with progressive upper extremities paraesthesia and gait disturbance. Four years ago he refused surgery and presented with dysphagia. The patient's MRI demonstrated an intramedullary spinal cord tumor extending from the fourth ventricle to T4. Conclusions: massive spinal ependymoma is a rare, benign, slow-growing tumor, and patients present symptoms years before diagnosis. Upon confirmation of the diagnosis, the tumor must be surgically removed.

Laser fluorescence spectroscopy in surgical treatment of intramedullary tumors: experience of the Burdenko Neurosurgery Center

To analyze efficacy of fluorescence navigation technique and laser fluorescence spectroscopy in surgical treatment of various spinal cord tumors. There were more than 1000 patients with intramedullary spinal cord tumors between 2001 and 2022. Intraoperative fluorescence navigation with laser spectroscopy was used in 47 cases. All patients underwent examination before and after surgery. We analyzed somatic, neurological and functional status (McCormick scale). MRI of the spinal cord and intraoperative fluorescence spectroscopy (OPMI Pentero 900 microscope with a fluorescent module, Carl Zeiss, Germany) were performed. To induce visible fluorescence, we used 5-ALA-Alasens (NIOPIK, Russia). Laser spectroscopy was carried out on a LESA-01-BIOSPEC analyzer (Biospec JSC, Russia). Morphological analysis of intramedullary spinal cord tumors was carried out in the laboratory of neuromorphology of the Burdenko Neurosurgery Center. In our sample, fluorescence navigation and laser fluorescence spectroscopy were used only in surgical treatment of intramedullary tumors. Laser fluorescence spectroscopy was valuable to identify fragments of intramedullary ependymoma and astrocytoma in 86% of cases, visual fluorescence - only in 81% of cases. Advisability of this technique for low-grade astrocytomas is unclear and requires further study. Visual fluorescence combined with laser spectroscopy is a promising method for intraoperative imaging of tumor remnants. This approach can improve safety, quality and results of surgical treatment.

Current management in the treatment of intramedullary ependymomas in children.

Current management of pediatric intramedullary ependymoma is extrapolated from adult series since large studies in children are unavailable. This has led us to share our experience with this rare tumor and compare it to the literature and to review and highlight important aspects of current management and point out inconsistencies. This is a retrospective analysis of patients with intramedullary ependymoma managed at our institution between 2004 and 2021. During the study period, 5 patients were treated for intramedullary ependymoma. Cases of myxopapillary ependymoma were excluded. The mean age of our cohort was 11.2years. We identified 4 cases of grade II ependymoma and 1 case of grade III ependymoma. Gross tumor removal (GTR) was achieved in two patients (40%) of patients. One patient was treated with radiotherapy for recurrence and two patients received chemotherapy. There were no cases of recurrence among patients treated with GTR, but in all patients treated with STR. Eighty percent of patients either improved or stayed stable neurologically. During follow-up (mean 73months), 2 patients died of disease. GTR and tumor grade remain the key prognostic factor of long-term tumor-free survival. Many questions prevail regarding outcomes, correct use of adjuvant therapy, and prognostic factors.

Impact of tumor-associated syrinx on outcomes following resection of primary ependymomas of the spinal cord.

To interrogate the association of tumor-associated syrinxes with postoperative neurological and oncological outcomes in patients surgically treated for WHO grade 2 spinal ependymomas. Adults treated for primary spinal intramedullary ependymomas between 2000 and 2020 were identified and data were gathered on preoperative neurological exam, radiographic characteristics, operative details, and postoperative neurological outcome. Neurological status was graded on the modified McCormick Scale (MMS). Neurological worsening immediately postoperatively and at last follow-up were defined by ≥ 1 MMS grade deterioration. Decision-tree analyses were also performed to identify independent predictors of new neurological deficits. Seventy patients were identified; mean age 45.4 ± 12.7; 60% male. Forty-eight patients (68.6%) had tumor-associated syrinxes, were more common among males (68.8%) and cervical lesions (68.8 vs. 31.8%; P = 0.005). Postoperatively patients with syrinxes had better MMS (P = 0.035) and were less likely to require a gait aid (39.6 vs. 81.8; P = 0.002). This latter difference persisted to last follow-up (22.9 vs. 59.1%; P = 0.006). On decision-tree analysis the strongest predictors of long-term neurological worsening were advanced age (≥ 63 years) and worse baseline neurological function. Worsened neurological status in the immediate postoperative period was best predicted by thoracic localization, the presence of a hemosiderin cap, and longer craniocaudal extension. For spinal ependymomas, tumor-associated syrinxes may portend decreased risk for immediate postoperative neurologic deficits but do not predict long-term neurological outcomes (MMS) or odds of successful gross total resection. Thoracic localization appears to best predict new immediate postoperative deficits, and worse baseline neurological function and advanced age best predict long-term deficits.

Resection of Cervical Intramedullary Ependymoma Under D-Wave and SSEP/MEP Monitoring without the Use of Coagulation.

Resection of Cervical Intramedullary Ependymoma Under D-Wave and SSEP/MEP Monitoring without the Use of Coagulation.

Intramedullary spinal cord and filum tumours—long-term outcome: single institution case series

BackgroundIntramedullary spinal cord tumours are rare and account for about 2–4% of primary CNS tumours. Ependymomas and astrocytomas are most frequent. The aim of this study was to evaluate the long-term neurological outcome, quality of life (QoL), survival, need for additional treatment and frequency of neuropathic pain in a patient group treated at a tertiary university hospital.MethodRetrospective descriptive study of 52 long-term survivors with intramedullary or filum tumours consenting to participate in this study. Fifty-six operations were performed in 48 patients. Clinical and radiological follow-up period was 113 and 117 months, respectively.ResultsGood neurological outcome (ASIA score D or E, modified McCormick grade 1 or 2) was achieved in 88%. We found two negative prognostic factors in regards of severe disability which were large craniocaudal tumour size (p = 0.004) and histologic verified astrocytomas (p = 0.002). SF-36 results showed significantly lower results on all five subdomains concerning physical function, whereas scores for mental health and role emotional showed no significant differences compared to Norwegian norms.Ten patients including all astrocytoma patients, one primitive neuroectodermal tumour and three recurrent tumours of filum terminale had adjuvant therapy. None of the patients with intramedullary ependymoma had adjuvant therapy.Neuropathic pain was present in 54% of patients at the last follow-up.ConclusionThis series shows that good results can be obtained with surgery for intramedullary tumours, even without perioperative neurophysiological monitoring. Multicentre studies are needed for further evaluation of negative and positive prognostic factors to further improve outcome.

Anatomical Limitation of Posterior Spinal Myelotomy for Intramedullary Hemorrhage Associated with Ependymoma or Cavernous Malformation of the High Cervical Spine.

Spinal intramedullary tumors such as ependymoma or vascular lesions such as cavernous malformation are often at risk of intramedullary hemorrhage. Surgical procedures involving the high cervical spinal cord are often challenging. This technical note included four patients who presented with acute, subacute, or gradual onset of spinal cord dysfunction associated with intramedullary hemorrhage at the C1 or C1/2 level of the high cervical spine. The mean age was 46.3 years (16-74 years). All patients underwent posterior spinal cord myelotomy of the posterior median sulcus or posterolateral sulcus. It was not to exceed the caudal opening of the fourth ventricle (foramen of Magendie) and was assumed to be as high as the caudal medulla oblongata. Total removal of the intramedullary ependymoma or cavernous malformation occurred in three of four cases, and the remaining case had subtotal removal of the ependymoma. None of the patients showed postoperative deterioration of the neurological condition. Pathological examination of all cases revealed intramedullary hemorrhage was associated with ependymoma or cavernous malformation. Posterior spinal myelotomy should be limited to the caudal opening of the fourth ventricle (foramen of Magendie), that is the caudal medulla oblongata, to avoid the significant deterioration after surgery.