Clinical Analysis of 21 Cases of Spinal Cord Ependymoma : Positive Clinical Results of Gross Total Resection

Object Reports on spinal cord ependymoma in children are rare. The aim of this study was to evaluate the clinical spectrum, treatment, and outcome of children with primary ependymoma of the spinal cord who were registered in the database of the pediatric German brain tumor studies Hirntumor (HIT) '91 and HIT 2000. Methods Between 1991 and 2007, 29 patients (12 male and 17 female, median age at diagnosis 13.6 years) with primary spinal cord ependymoma (myxopapillary ependymoma WHO Grade I, II, and III tumors in 6, 17, and 6 patients, respectively) were identified. Four patients had neurofibromatosis Type 2. Results With a median follow-up of 4.2 years (range 0.48–15 years), 28 patients (96.6%) were alive. Seven patients (24.1%) developed progressive disease or relapse, 2 after gross-total resection (GTR) and 5 after incomplete resection or biopsy. One patient with anaplastic ependymoma (WHO Grade III) died 65 months after diagnosis of disease progression. Primary adjuvant treatment (radiotherapy, chemotherapy, or both) was used in 8 (50%) of 16 patients following GTR and in 9 (82%) of 11 patients who underwent less than a GTR. Three additional patients were treated adjuvantly following progression. Estimated progression-free survival and overall survival rates at 5 years were 72.3% (95% CI 50%–86%) and 100%, respectively. Progression-free survival at 5 years is 84.4% (95% CI 50%–96%) for patients following GTR compared with 57.1% (95% CI 25%–69%) for patients who achieved a less than GTR (p = 0.088, log-rank test). A high relapse incidence (4 of 6) was observed among patients with myxopapillary ependymoma. Conclusions Gross-total resection is the mainstay of treatment for patients with primary spinal cord ependymoma and may be achieved in about 50% of the patients using modern surgical techniques. Primary adjuvant treatment was commonly used in children with spinal cord ependymoma irrespective of the extent of resection or tumor grade. The impact of adjuvant treatment on progression-free and overall survival has to be investigated in a prospective trial.

Download Full-text

Microsurgical Resection of Cervical Intradural Juxtamedullary Solitary Fibrous Tumor: 2-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opaa232 ◽

2020 ◽

Vol 19 (5) ◽

pp. E532-E532

Author(s):

Paolo Perrini ◽

Davide Tiziano Di Carlo ◽

Nicola Montemurro ◽

Nicola Benedetto ◽

Valerio Ortenzi ◽

...

Keyword(s):

Spinal Cord ◽

Nerve Root ◽

Tumor Resection ◽

Gross Total Resection ◽

Total Resection ◽

Neurophysiologic Monitoring ◽

Intramedullary Lesion ◽

Dural Attachment ◽

Fibrous Tumor ◽

Nerve Root Involvement

Abstract Solitary fibrous tumors (SFTs) are uncommon mesenchymal lesions originally described as pleura-based neoplasms. Intradural juxtamedullary SFTs are rare, hard, and scarcely vascularized and generally present a conspicuous extramedullary exophytic component without dural attachment and nerve root involvement. Gross-total resection is the mainstay of treatment, although the absence of an arachnoidal plane and the firm adherence to the spinal cord make resection challenging. We describe the case of a 74-yr-old female patient presenting with a history of progressive spastic tetraparesis due to a cervical juxtamedullary SFT. The patient was not able to walk and magnetic resonance imaging (MRI) of the cervical spine demonstrated a possible intramedullary lesion at C2-C3 with homogeneous enhancement after gadolinium injection. Given the progressive nature of symptoms, the patient elected to have surgical resection of the tumor. The patient underwent C2-C3 laminoplasty and tumor resection under neurophysiologic monitoring. The tumor presented extremely hard without dural attachment or nerve root involvement and was progressively debulked using microsurgical techniques and ultrasonic aspirator. The identification of a plane between the mass and the spinal cord white matter allowed for a gross total resection. Permanent pathological analysis eventually demonstrated SFT. The patient's neurological condition was unchanged postoperatively. MRI performed 2 mo after the operation demonstrated gross total resection of the lesion. At the 6-mo follow-up visit, the patient was able to walk with assistance. The patient signed the Institutional Consent Form to undergo the surgical procedure and to allow the use of her images and videos for any type of medical publications.

Download Full-text

The Clinical Results of Radiation Therapy for Spinal Cord Ependymoma with Adverse Prognostic Features: A Single-Institution Experience

International Journal of Radiation Oncology*Biology*Physics ◽

10.1016/j.ijrobp.2018.07.788 ◽

2018 ◽

Vol 102 (3) ◽

pp. e232-e233

Author(s):

H.K. Byun ◽

H.I. Yoon ◽

J.H. Cho ◽

C.O. Suh

Keyword(s):

Spinal Cord ◽

Radiation Therapy ◽

Clinical Results ◽

Single Institution ◽

Prognostic Features ◽

Spinal Cord Ependymoma

Download Full-text

Surgical Technique and Outcomes in the Treatment of Spinal Cord Ependymomas: Part II: Myxopapillary Ependymoma

Operative Neurosurgery ◽

10.1227/neu.0b013e3181fdf912 ◽

2011 ◽

Vol 68 (suppl_1) ◽

pp. ons90-ons94 ◽

Cited By ~ 4

Author(s):

Elisa J Kucia ◽

Peter H Maughan ◽

Udaya K Kakarla ◽

Nicholas C Bambakidis ◽

Robert F Spetzler

Keyword(s):

Spinal Cord ◽

Radiation Therapy ◽

Gross Total Resection ◽

Subtotal Resection ◽

Myxopapillary Ependymoma ◽

Postoperative Radiation ◽

Total Resection ◽

Postoperative Radiation Therapy ◽

Duration Of Symptoms

Abstract BACKGROUND: Myxopapillary ependymomas usually occur in the filum terminale of the spinal cord. OBJECTIVE: This report summarizes our experience treating myxopapillary ependymomas. METHODS: The records of 34 patients (14 men, 20 women; mean age 45.5 years; age range, 14-88 years) who underwent resection of a myxopapillary ependymoma between 1983 and 2006 were reviewed for age, sex, tumor location, symptoms at diagnosis, duration of symptoms, treatment before presentation, extent of surgical resection, adjuvant therapy, length of follow-up, evidence of recurrence, and complications. Neurological examinations performed at presentation, immediately after surgery, and last follow-up were graded according to the McCormick grading scale. RESULTS: The average duration of symptoms before diagnosis was 22.2 months. The most common symptom was pain followed by weakness, bowel/bladder symptoms, and numbness. The rate of gross total resection was 80%. All patients with a subtotal resection (20%) underwent postoperative radiation therapy. Presentation and outcomes of patients who underwent subtotal resection followed by radiation therapy were compared with those who underwent gross total resection. There was no significant difference in neurological grade between the groups at presentation or final follow-up. The overall recurrence rate was 10% (3/34 patients). CONCLUSION: The goal of surgical treatment of myxopapillary ependymomas is resection to the greatest extent possible with preservation of function. In cases of subtotal resection, postoperative radiation therapy may improve outcome. If neurological function is maintained at treatment, these indolent lesions allow years of good function.

Download Full-text

Treatment of pediatric Grade II spinal ependymomas: a population-based study

Journal of Neurosurgery Pediatrics ◽

10.3171/2014.9.peds1473 ◽

2015 ◽

Vol 15 (3) ◽

pp. 243-249 ◽

Cited By ~ 16

Author(s):

Yimo Lin ◽

Andrew Jea ◽

Stephanie C. Melkonian ◽

Sandi Lam

Keyword(s):

Spinal Cord ◽

Overall Survival ◽

Radiation Therapy ◽

Pediatric Patients ◽

Treatment Patterns ◽

Gross Total Resection ◽

Subtotal Resection ◽

Total Resection ◽

Spinal Ependymoma ◽

Grade Ii

OBJECT Grade II spinal cord ependymomas occurring in pediatric patients are exceptionally rare neoplasms. In this paper the authors use a national cancer database to determine patient demographics, treatment patterns, and associated outcomes of this cohort. METHODS The Surveillance Epidemiology and End Results (SEER) database was used to analyze subjects younger than 18 years with histologically confirmed diagnoses of Grade II spinal cord ependymoma from the years 1973 to 2008. Descriptive data on the demographic characteristics of this cohort and the associated treatment patterns are shown. The Kaplan-Meier method was used to estimate overall survival at 1, 2, 5, and 10 years. RESULTS This cohort comprised 64 pediatric subjects with Grade II spinal ependymoma. The median age was 13 years, nearly half of the patients were male, and most were white (84%). The median follow-up was 9.2 years. Overall survival at 5 and 10 years was 86% and 83%, respectively. Gross-total resection was achieved in 57% of subjects, and radiation therapy was administered to 36%. Radiation therapy was administered to 78% of subjects after subtotal resection but only to 19% of patients after gross-total resection; this difference was significant (p < 0.001). In a multivariate regression model analyzing sex, age at diagnosis, year of diagnosis, radiotherapy, and extent of resection, female sex was found to be an independent predictor of decreased mortality (HR 0.15 [95% CI 0.02–0.94], p = 0.04). CONCLUSIONS These data show long-term outcomes for pediatric patients with Grade II spinal ependymoma. Radiotherapy was more likely to be administered in cases of subtotal resection than in cases of gross-total resection. Female sex is associated with decreased mortality, while other demographic or treatment modalities are not.

Download Full-text

Spinal Cord Ependymomas With MYCN Amplification Show Aggressive Clinical Behavior

Journal of Neuropathology & Experimental Neurology ◽

10.1093/jnen/nlz064 ◽

2019 ◽

Vol 78 (9) ◽

pp. 791-797 ◽

Cited By ~ 4

Author(s):

Amy A Swanson ◽

Aditya Raghunathan ◽

Robert B Jenkins ◽

Martina Messing-Jünger ◽

Torsten Pietsch ◽

...

Keyword(s):

Spinal Cord ◽

Adolescent And Young Adult ◽

World Health ◽

Gross Total Resection ◽

Mycn Amplification ◽

Chromosomal Microarray Analysis ◽

Adjuvant Radiation ◽

Low Grade ◽

Adult Women ◽

Total Resection

Abstract Adult spinal cord ependymomas are typically low grade and have a relatively favorable clinical course following gross total resection. We report 4 cases of anaplastic spinal cord ependymoma with MYCN amplification, an exceptionally rare finding. All cases occurred in the spinal cord of adolescent and young adult women and had morphological and immunohistochemical features of anaplastic ependymomas (World Health Organization grade III). Chromosomal microarray analysis demonstrated amplification of 2p24 (including MYCN) in all cases. One patient died 6 months after surgery. Another patient recently had removal of metastatic nodules in the thoracic region, following gross total resection and adjuvant radiation therapy of a lumbar ependymoma 1 year previously. One patient responded well after chemotherapy but died after multiple relapses 82 months after diagnosis. We found MYCN amplification reported in 2 other ependymomas, both anaplastic and arising in the spinal cord of adult females (Brain Pathol 2001;11:133–43). One patient had multiple recurrences in the spinal cord and an intracranial metastasis. Although MYCN amplification is rare in ependymomas, the current and previously reported cases suggest that this is associated with higher-grade histology, spinal location, and often unfavorable prognosis. The clinical significance and therapeutic implications of MYCN amplification in ependymomas require further evaluation.

Download Full-text

Spinal cord ependymomas and myxopapillary ependymomas in the first 2 decades of life: a clinicopathological and immunohistochemical characterization of 19 cases

Journal of Neurosurgery Pediatrics ◽

10.3171/2012.2.peds11285 ◽

2012 ◽

Vol 9 (6) ◽

pp. 646-653 ◽

Cited By ~ 33

Author(s):

James H. Stephen ◽

Angela J. Sievert ◽

Peter J. Madsen ◽

Alexander R. Judkins ◽

Adam C. Resnick ◽

...

Keyword(s):

Spinal Cord ◽

Protein Expression ◽

Pediatric Patients ◽

Pathological Diagnosis ◽

Gross Total Resection ◽

Low Grade ◽

Total Resection ◽

Significant Difference ◽

Grade Ii

Object Primary spinal cord ependymomas (EPNs) are rare in children, comprising classical WHO Grade II and III tumors and Grade I myxopapillary ependymomas (MEPNs). Despite their benign histology, recurrences and neural-axis dissemination have been reported in up to 33% MEPNs in the pediatric population. Treatment options beyond resection are limited, and little is known about their tumorigenesis. The purpose of this study was to explore the tumor biology and outcomes in a consecutive series of pediatric patients treated at a single institution. Methods The authors performed a retrospective clinicopathological review of 19 patients at a tertiary referral children's hospital for resection of a spinal cord ependymoma. The population included 8 patients with a pathological diagnosis of MEPN and 11 patients with a pathological diagnosis of spinal EPN (10 cases were Grade II and 1 case was Grade III). The upregulation of the following genes HOXB13, NEFL, PDGFRα, EGFR, EPHB3, AQP1, and JAGGED 1 was studied by immunohistochemistry from archived paraffin-embedded tumor samples of the entire cohort to compare the expression in MEPN versus EPN. Results Gross-total resection was achieved in 75% of patients presenting with MEPNs and in 100% of those with EPNs. The average follow-up period was 79 months for the MEPN subset and 53 months for Grade II/III EPNs. Overall survival for both subsets was 100%. However, event-free survival was only 50% for patients with MEPNs. Of note, in all cases involving MEPNs that recurred, the patients had undergone gross-total resection on initial surgery. In contrast, there were no tumor recurrences in patients with EPNs. Immunohistochemistry revealed no significant differences in protein expression between the two tumor types with the exception of EPHB3, which demonstrates a tendency to be positive in MEPNs (6 reactive tumors of 9) rather than in EPN (2 reactive tumors of 10). Conclusions The authors' experience shows that, following a gross-total resection, MEPNs are more likely to recur than their higher-grade counterpart, EPNs. This supports the recommendation for close long-term radiological follow-up of pediatric patients with MEPNs to monitor for recurrence, despite the tumor's low-grade histological feature. No significant difference in the protein expression of HOXB13, NEFL, PDGFRα, EGFR, EPHB3, AQP1, and JAGGED 1 was present in this selected cohort of pediatric patients.

Download Full-text

Surgical Technique and Outcomes in the Treatment of Spinal Cord Ependymomas, Part 1: Intramedullary Ependymomas

Operative Neurosurgery ◽

10.1227/neu.0b013e318208f181 ◽

2011 ◽

Vol 68 (suppl_1) ◽

pp. ons57-ons63 ◽

Cited By ~ 16

Author(s):

Elisa J Kucia ◽

Nicholas C Bambakidis ◽

Steve W Chang ◽

Robert F Spetzler

Keyword(s):

Spinal Cord ◽

Radiation Therapy ◽

Surgical Resection ◽

Complication Rate ◽

Gross Total Resection ◽

Subtotal Resection ◽

Total Resection ◽

Tertiary Center ◽

The Mean

Abstract BACKGROUND: Intramedullary spinal ependymomas are rare tumors. OBJECTIVE: To provide a large retrospective review in the modern neuroimaging era from a tertiary center where aggressive surgical resection is favored. METHODS: Charts of intramedullary spinal ependymomas treated between 1983 and 2006 were reviewed. RESULTS: Sixty-seven cases were reviewed. The mean age was 45.6 years (range, 11-78 years) with a male-to-female ratio of 2:1. The most common location was the cervical spine, followed by the thoracic and lumbar spine. The average duration of symptoms was 33 months, with the most common symptom being pain and/or dysesthesias, followed by weakness, numbness, and urinary or sexual symptoms. Gross total resection was achieved in 55 patients and a subtotal resection was performed in 12 patients; 9 patients were treated with adjuvant radiation therapy. Mean follow-up was 32 months. The mean McCormick neurological grade at last follow-up was 2.0. The preoperative outcome correlated significantly with postoperative outcome (P < .001). A significant number of patients who initially worsened improved at their 3-month follow-up examination. Outcomes were significantly worse in patients undergoing subtotal resection with or without radiation therapy (P < .05). There were 3 recurrences. The overall complication rate was 34%. The primary complications were wound infections or cerebrospinal fluid leaks. CONCLUSION: Spinal cord ependymomas are difficult lesions to treat. Aggressive surgical resection is associated with a high overall complication rate. However, when gross total resection can be achieved, overall outcomes are excellent and the recurrence rate is low.

Download Full-text

Intramedullary spinal cord ependymoma and astrocytoma: intraoperative frozen-section diagnosis, extent of resection, and outcomes

Journal of Neurosurgery Spine ◽

10.3171/2018.7.spine18230 ◽

2019 ◽

Vol 30 (1) ◽

pp. 133-139 ◽

Cited By ~ 5

Author(s):

Hiroki Hongo ◽

Keisuke Takai ◽

Takashi Komori ◽

Makoto Taniguchi

Keyword(s):

Spinal Cord ◽

Diagnostic Accuracy ◽

Frozen Section ◽

Progression Free Survival ◽

Final Diagnosis ◽

Gross Total Resection ◽

Frozen Sections ◽

Total Resection ◽

Intramedullary Spinal Cord ◽

Frozen Section Diagnosis

OBJECTIVEThe intraoperative differentiation of ependymomas from astrocytomas is important because neurosurgical strategies differ between these two tumor groups. Previous studies have reported that the diagnostic accuracy of intraoperative frozen sections of intracranial central nervous system (CNS) tumors is higher than 83%–97%, whereas that for spinal intramedullary tumors remains unknown. Herein, authors tested the hypothesis that intraoperative frozen-section diagnosis is the gold standard for a differential diagnosis of intramedullary spinal cord tumors.METHODSThe clinical characteristics, intraoperative histological diagnosis from frozen sections, extent of tumor resection, progression-free survival (PFS), and overall survival (OS) of 49 cases of intramedullary spinal cord ependymomas (n = 32) and astrocytomas (n = 17) were retrospectively evaluated.RESULTSThe frozen-section diagnosis and final diagnosis with permanent sections agreed in 23 (72%) of 32 cases of ependymoma. Of the 9 cases of ependymoma in which the frozen-section diagnosis disagreed with the final diagnosis, 4 were incorrectly diagnosed as astrocytoma and the other 5 cases had a nonspecific diagnosis, such as glioma. Nonetheless, gross-total resection was achieved in 6 of these 9 cases given the presence of a dissection plane. The frozen-section diagnosis and final diagnosis agreed in 12 (71%) of 17 cases of astrocytoma. Of the 5 cases of astrocytoma in which the frozen-section diagnosis disagreed with the final diagnosis, 1 was incorrectly diagnosed as ependymoma and the other 4 had a nonspecific diagnosis. Gross-total resection was achieved in only 1 of these 5 cases.A relationship between the size of tumor specimens and the diagnostic accuracy of frozen sections was not observed. Ependymal rosettes and perivascular pseudorosettes were observed in 30% and 57% of ependymomas, respectively, but were absent in astrocytomas.Progression-free survival and OS were both significantly longer in cases of ependymoma than in cases of astrocytoma (p < 0.001). Gross-total resection was achieved in 69% of ependymomas and was associated with longer PFS (p = 0.041). In the astrocytoma group, gross-total resection was achieved in only 12% and there was no relationship between extent of resection and OS. Tumor grades tended to correlate with OS in astrocytomas (p = 0.079).CONCLUSIONSThe diagnostic accuracy of intraoperative frozen sections was lower for intramedullary spinal cord ependymomas and astrocytomas in the present study than that for intracranial CNS tumors reported on in the literature. Surgical strategies need to be selected based on multiple factors, such as clinical characteristics, preoperative imaging, frozen-section diagnosis, and intraoperative findings of the tumor plane.

Download Full-text

Resection of Spinal Meningioma Using Ultrasonic BoneScalpel Microshaver: Cases, Technique, and Review of the Literature

Operative Neurosurgery ◽

10.1093/ons/opaa223 ◽

2020 ◽

Author(s):

Saeed Sam Sadrameli ◽

Tiffany Michelle Chan ◽

Jonathan Jinhee Lee ◽

Virendra R Desai ◽

Paul J Holman

Keyword(s):

Spinal Cord ◽

Power Level ◽

Mass Effect ◽

Complete Recovery ◽

Gross Total Resection ◽

Spinal Tumors ◽

Total Resection ◽

Cord Injury ◽

Leg Weakness ◽

Spinal Meningiomas

Abstract BACKGROUND Meningiomas of the spinal canal comprise up to 40% of all spinal tumors. The standard management of these tumors is gross total resection. The outcome and extent of resection depends on location, size, patient's neurologic status, and experience of the surgeon. Heavily calcified spinal meningiomas often pose a challenge for achieving gross total resection without cord injury. OBJECTIVE Here, we report our experience with the BoneScalpel Micro-shaver to resect heavily calcified areas of spinal meningiomas adherent to the spinal cord without significant cord manipulation, achieving gross total resection and outstanding clinical results. METHODS 79 and 82-yr-old females presented with progressive leg weakness, paresthesias, and gait instability. Magnetic resonance imaging of the thoracic spine showed a homogenous enhancing intradural extramedullary mass with mass effect on the spinal cord. Midline bilateral laminectomy was performed, and the dura was open in midline. The lateral portion of the tumor away from the spinal cord was resected with Cavitron Ultrasonic Surgical Aspirator while the BoneScalpel Micro-shaver (power level 5 and 30% irrigation) was brought into the field for the calcified portion of the tumor adherent to the spinal cord. RESULTS Gross total resection was achieved for both cases. At the 2-wk postoperative visit, both patients reported complete recovery of their leg weakness with significant improvement in paresthesias and ataxia. CONCLUSION The ultrasonic osteotome equipped with a microhook tip appears to be a safe surgical instrument allowing for effective resection of spinal meningiomas or other heavily calcified spinal masses not easily removed by usual surgical instrumentation.

Download Full-text