|
1
|
Kanno H, Kanetsuna Y, Shinonaga M. Anaplastic myxopapillary ependymoma: A case report and review of literature. World J Clin Oncol 2021; 12:1072-1082. [PMID: 34909401 PMCID: PMC8641005 DOI: 10.5306/wjco.v12.i11.1072] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/23/2021] [Revised: 05/17/2021] [Accepted: 10/11/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Myxopapillary ependymoma (MPE) is a pathological grade I tumor that arises in the filum terminale. MPE with anaplastic features is extremely rare, and only 5 cases have shown malignancy at the time of recurrence.
CASE SUMMARY The patient (a 46-year-old woman) had undergone a MPE operation 30 years ago. After subtotal resection of the tumor located in L4-S1, it had a solid component that extended to the adjacent subcutaneous region. Histologically, the tumor consisted of a typical MPE with anaplastic features. The anaplastic areas of the tumor showed hypercellularity, a rapid mitotic rate, vascular proliferation, and connective tissue proliferation. Pleomorphic cells and atypical mitotic figures were occasionally observed. The MIB-1 index in this area was 12.3%. The immunohistochemical study showed immunoreactivity for vimentin, glial fibrillary acidic protein and S100. The morphological pattern and immunohistochemical profile were consistent with anaplastic MPE. The patient tolerated surgery well without new neurological deficits. She underwent local irradiation for the residual tumor and rehabilitation.
CONCLUSION Although extremely rare, anaplastic MPE occurs in both pediatric and adult patients, similar to other ependymomas. At a minimum, close monitoring is recommended, given concerns about aggressive biological potential. In the future, further study is needed to determine the WHO classification criteria and genetic indicators of tumor progression. The possibility of malignant transformation of MPE should be taken into account, and patients with MPE should be treated with care and follow-up.
Collapse
Affiliation(s)
- Hiroshi Kanno
- Department of Neurosurgery, International University of Health and Welfare Atami Hospital, Atami 413-0012, Shizuoka, Japan
| | - Yukiko Kanetsuna
- Department of Pathology, International University of Health and Welfare Atami Hospital, Atami 413-0012, Shizuoka, Japan
| | - Masamichi Shinonaga
- Department of Neurosurgery, International University of Health and Welfare Atami Hospital, Atami 413-0012, Shizuoka, Japan
| |
Collapse
|
|
2
|
Huynh TR, Lu C, Drazin D, Lekovic G. Myxopapillary ependymoma with anaplastic features: A case report with review of the literature. Surg Neurol Int 2018; 9:191. [PMID: 30294495 PMCID: PMC6169347 DOI: 10.4103/sni.sni_422_17] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2017] [Accepted: 06/04/2018] [Indexed: 12/17/2022] Open
Abstract
Background: Myxopapillary ependymoma (MPE) with anaplastic features is extremely rare, with only three case reports in the literature. Case Description: We report the case of a MPE with anaplastic features in a 24-year-old female who presented with a dominant lumbar mass along with intracranial and sacral metastases. Upon gross total resection of the dominant tumor located at L2-L3, it appeared to arise from the filum terminale, and had a solid component in addition to soft or necrotic areas. Histologically, the tumor was composed of the two classic components of MPE: (1) low-grade ependymal cells surrounding blood vessels, producing the papillary appearance and (2) perivascular myxoid material between blood vessels and ependymal cells, creating the myxopapillary appearance. The high-grade anaplastic component showed hypercellularity, brisk mitotic rate, and vascular proliferation, with frequent pleomorphic cells and atypical mitotic figures. It was positive for vimentin and glial fibrillary acidic protein (GFAP); negative for epithelial membrane antigen (EMA), CAM5.2, creatine kinase 7 (CK7), CK20; and the MIB-1 index (Ki-67) was 8–38%. Ten months after initial resection, follow-up magnetic resonance imaging revealed new lesions in (1) the hypothalamus, (2) the left pons, and (3) the left medial temporal lobe, which were treated with radiosurgery. Eight months later (18 months from initial surgery), the patient underwent thoracic laminectomy for a large leptomeningeal metastasis at T6 and T8. Conclusion: The present case of MPE with anaplastic features is the fourth case on record in the medical literature.
Collapse
Affiliation(s)
- Tridu R Huynh
- Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California, USA
| | - Conrad Lu
- Department of Pathology, St. Vincent Medical Center, Los Angeles, California, USA
| | - Doniel Drazin
- Swedish Neuroscience Institute, Swedish Medical Center, Seattle, Washington, USA
| | | |
Collapse
|
|
3
|
Zhu F, Ding J, Li Y, Mao D, He X, Chen W, Lou L, Ding Z. Benign ependymoma with extensive intracranial and spinal cerebrospinal fluid dissemination: case report and literature review. Br J Neurosurg 2017. [PMID: 28633540 DOI: 10.1080/02688697.2017.1340584] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
Abstract
Myxopapillary ependymoma (MPE) is a rare variant of ependymoma that is most commonly located in the cauda equina and filum terminale. We present a case of 23-year-old man diagnosed with MPE in the fourth ventricle and sacral canal area with extensive disseminated lesions along the cerebrospinal ventricular system. Additionally, a molecular pathological diagnosis was performed. The patient underwent a craniotomy and a lumbar laminectomy. In the course of 18 months of follow-up, the patient have recovered very well.
Collapse
Affiliation(s)
- Fangmei Zhu
- a Department of Radiology, Tongde Hospital of Zhejiang Province , Hangzhou , China
| | - Jurong Ding
- b School of Automation and Information Engineering, Sichuan University of Science and Engineering , Zigong , China
| | - Yumei Li
- c Department of Radiology , Zhejiang Provincial People's Hospital , Hangzhou , China
| | - Dewang Mao
- c Department of Radiology , Zhejiang Provincial People's Hospital , Hangzhou , China
| | - Xianglei He
- d Department of Pathology , Zhejiang Provincial People's Hospital , Hangzhou , China
| | - Wanyuan Chen
- d Department of Pathology , Zhejiang Provincial People's Hospital , Hangzhou , China
| | - Lin Lou
- e Department of Neurosurgery , Zhejiang Provincial People's Hospital , Hangzhou , China
| | - Zhongxiang Ding
- c Department of Radiology , Zhejiang Provincial People's Hospital , Hangzhou , China
| |
Collapse
|
|
4
|
Trivedi D, Xiong Z. Anaplastic myxopapillary ependymoma in an infant: Case report and literature review. Intractable Rare Dis Res 2017; 6:128-131. [PMID: 28580214 PMCID: PMC5451745 DOI: 10.5582/irdr.2017.01015] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2017] [Revised: 04/17/2017] [Accepted: 04/21/2017] [Indexed: 11/05/2022] Open
Abstract
A 7-month-old boy presented with gastrointestinal disturbance, mild neurologic deficit of the left lower extremity and levo-scoliosis of the thoracic spine. Magnetic resonance imaging demonstrated a large intramedullary lesion involving the thoracic spine, from level T1 to T11. Histologic analysis showed a glial tumor with fibrillary processes arranged in radial pattern around mucoid fibrovascular cores with a high proliferative index (focally up to 80%) and prominent vascular endothelial hyperplasia. These findings were consistent with an anaplastic myxopapillary ependymoma. Subtotal resection was performed via a T3-T10 laminoplasty. A ventricular shunt was placed, and the patient subsequently received chemoradiation therapy. To date, this is the second case of a myxopapillary ependymoma with high-grade anaplastic features and the first case in an infant reported in the literature.
Collapse
Affiliation(s)
- Darshan Trivedi
- Department of Pathology and Laboratory Medicine, Tulane University School of Medicine, New Orleans, LA, USA
| | - Zhenggang Xiong
- Department of Pathology and Laboratory Medicine, Tulane University School of Medicine, New Orleans, LA, USA
| |
Collapse
|
|
5
|
Yener U, Güdük M, Ekşi MŞ, Aytar MH, Sav A, Özgen S. Concomitant Double Tumors of Myxopapillary Ependymoma Presented at Cauda Equina-Filum Terminale in Adult Patient. KOREAN JOURNAL OF SPINE 2016; 13:33-6. [PMID: 27123029 PMCID: PMC4844659 DOI: 10.14245/kjs.2016.13.1.33] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/29/2015] [Revised: 01/28/2016] [Accepted: 01/29/2016] [Indexed: 11/21/2022]
Abstract
A 32-year-old man presented with gradually increasing bilateral buttock pain. He had intermittent claudication. Multiple, homogenously enhanced intradural extramedullary lesions at L2-L3 and L5-S1 levels were observed on magnetic resonance imaging. The tumors were debulked and were removed in piecemeal pattern until they had completely been resected. Histopathological examination of the surgical specimens confirmed that both tumors were myxopapillary ependymomas (MPE). MPE presenting as concomitant double tumor at conus-cauda-filum level are very rare. This kind of presentation could not be directly considered as dissemination, since both tumors were in the site of classical origin of MPE. Ten cases of double spinal MPEs have been reported to date. Including the present case, analysis of the 11 patients revealed some facts. There is a male predominance, which is opposite to the ependymomas that are commonly observed in females. Median age at presentation is 15 years. Most pronounced symptom is low back pain that sometimes radiates to lower extremities. Surgical approach was aimed in all tumors, which could be succeeded in all tumors except one. Adjuvant radiation therapy was applied in 5 patients. No recurrences have been reported after surgery or surgery + radiotherapy regimens.
Collapse
Affiliation(s)
- Ulaş Yener
- Department of Neurosurgery, Acıbadem University Faculty of Medicine, Istanbul, Turkey
| | - Mustafa Güdük
- Department of Neurosurgery, Acıbadem University Faculty of Medicine, Istanbul, Turkey
| | - Murat Şakir Ekşi
- Department of Orthopedic Surgery-Spine Center, University of California at San Francisco, CA, USA
| | - Murat Hamit Aytar
- Department of Neurosurgery, Acıbadem University Vocational School of Health Services, Istanbul, Turkey
| | - Aydın Sav
- Department of Pathology, Acıbadem University Faculty of Medicine, Istanbul, Turkey
| | - Serdar Özgen
- Department of Neurosurgery, Acıbadem University Faculty of Medicine, Istanbul, Turkey
| |
Collapse
|
|
6
|
Abstract
Electron microscopy is a useful diagnostic technique in order to confirm or establish a definitive diagnosis in brain tumors that may have an atypical histological pattern, which requires a concrete diagnosis. In ependymomas, electron microscopy reveals morphological characters that have a pathognomonic diagnostic value, therefore allowing a definitive diagnosis. The main fine structural criteria of ependymomas consist of the numerous microvilli and cilia, which are incorporated in the cell body or extended freely in the intracellular space; the centriole or blepharoplast, which is located in the basis of the cilia; the large number of the fragmented microtubules in the perikaryon and the cellular processes (any small cellular projection into the neutrophil or intracellular space); the junctional apparatus between the cells, such as zonula adherens, zonula occludens and puncta adherentia; the basement membrane-like structure, seen in papillary ependymomas and ependymomas of the filum terminale; and the elongated cells in the loose intracellular space, commonly seen in myxopapillary ependymomas.
Collapse
Affiliation(s)
- Stavros J Baloyannis
- Aristotelian University Department of Neurology, Thessaloniki & Research Institute for Alzheimer's disease, Iraklion Langada, Greece.
| | | |
Collapse
|
|
7
|
The nature of double concomitant myxopapillary ependymoma: report of a case. Childs Nerv Syst 2014; 30:527-30. [PMID: 23904042 DOI: 10.1007/s00381-013-2251-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/16/2013] [Accepted: 07/22/2013] [Indexed: 10/26/2022]
Abstract
Myxopapillary ependymomas are almost exclusively seen at the conus medullaris/filum terminale/cauda equina region, usually as solitary space-occupying lesions. The authors report the case of a 14-year-old boy with double concomitant myxopapillary ependymoma, proximal and caudal on the filum terminale in which a totally gross removal was achieved in two stages. This presentation is rare and, so far, we have known just three similar cases that were previously reported in children. The true nature of these lesions is controversial, and while some argue that they are related to metastatic seeding, others consider them independent lesions developing synchronously. A review on dissemination of spinal myxopapillary ependymomas was done.
Collapse
|
|
8
|
Oh MC, Tarapore PE, Kim JM, Sun MZ, Safaee M, Kaur G, Aranda DM, Parsa AT. Spinal ependymomas: benefits of extent of resection for different histological grades. J Clin Neurosci 2013; 20:1390-7. [PMID: 23768966 DOI: 10.1016/j.jocn.2012.12.010] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2012] [Accepted: 12/08/2012] [Indexed: 11/26/2022]
Abstract
Although the World Health Organization (WHO) categorizes spinal ependymomas into three histological grades, difference in surgical outcomes between WHO grades I and II tumors are unclear. For these benign tumors, prognosis may be best determined by factors other than tumor grade alone, such as extent of resection. To analyze the effects of the extent of resection on different grades of spinal ependymomas, we performed a comprehensive literature review to identify adult spinal ependymoma patients who received surgical resection with a clearly identifiable WHO grade. A total of 175 patients were identified. While grade III tumors carried the worst prognosis as expected (p<0.001), grade I and II tumors did not differ significantly in outcomes following surgery. Overall, gross total resection (GTR, 68.7%, 114/166) provided significantly improved progression-free survival (PFS, p<0.001) and overall survival (OS, p=0.022) compared to the subtotal resection group. Surprisingly, the highest GTR rate was achieved for grade II tumors (78.8%, 78/99; p<0.001) followed by grade I (58.9%, 33/56) and grade III tumors (27.3%, 3/11). Interestingly, PFS was significantly improved by GTR for grade II tumors (p<0.001), but not for grade I (p=0.705). Similar trends, although not statistically significant, were found for OS. Our results show that while GTR provides the best overall outcomes, GTR is most effective for classic grade II ependymomas, but not for grade I ependymomas. Despite having a lower WHO grade, myxopapillary ependymomas have a lower GTR rate, and benefit less from GTR.
Collapse
Affiliation(s)
- Michael C Oh
- Department of Neurological Surgery, University of California San Fransisco, 505 Parnassus Avenue, San Francisco, CA 94117, USA
| | | | | | | | | | | | | | | |
Collapse
|
|
9
|
Oh MC, Kim JM, Kaur G, Safaee M, Sun MZ, Singh A, Aranda D, Molinaro AM, Parsa AT. Prognosis by tumor location in adults with spinal ependymomas. J Neurosurg Spine 2013; 18:226-35. [PMID: 23311515 DOI: 10.3171/2012.12.spine12591] [Citation(s) in RCA: 48] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
OBJECT Ependymomas are primary central nervous system tumors that occur more frequently in the spines of adults than they do there in children. Previous studies consist mainly of retrospective single-institutional experiences or case studies. In this study, a comprehensive literature review was performed on reported cases of spinal ependymoma treated with resection to determine whether tumor location along the spinal axis conveys important prognostic information. METHODS A PubMed search was performed to identify all papers that included data on patients with spinal ependymoma. Only cases involving adult patients who underwent ependymoma resection with a clearly reported tumor location were included for analysis. Tumor locations were separated into 6 groups: cervicomedullary, cervical, cervicothoracic, thoracic, thoracolumbar, and conus + cauda equina. Kaplan-Meier survival and Cox regression analyses were performed to determine the effect of tumor location on progression-free survival (PFS) and overall survival (OS). RESULTS A total of 447 patients who underwent resection of spinal ependymomas with clearly indicated location of tumor were identified. The most common locations of spinal ependymomas were the cervical (32.0%) and conus + cauda equina (26.8%) regions. The thoracolumbar and cervicomedullary regions had the fewest tumors (accounting for, respectively, 5.1% and 3.4% of the total number of cases). The conus + cauda equina and thoracolumbar regions had the highest percentage of WHO Grade I tumors, while tumors located above these regions consisted of mostly WHO Grade II tumors. Despite the tendency for benign grades in the lower spinal regions, PFS for patients with spinal ependymomas in the lower 3 regions (thoracic, thoracolumbar, conus + cauda equina) was significantly shorter (p < 0.001) than for those with tumors in the upper regions (cervicomedullary, cervical, cervicothoracic), but the difference in OS did not achieve statistical significance (p = 0.131). CONCLUSIONS Spinal ependymomas along different regions of spinal axis have different characteristics and clinical behaviors. Tumor grade, extent of resection, and PFS varied by tumor location (upper vs lower spinal regions), while OS did not. Recurrence rates were higher for the lower spinal cord tumors, despite a greater prevalence of lower WHO grade lesions, compared with upper spinal cord tumors, suggesting that tumor location along the spinal axis is an important prognostic factor.
Collapse
Affiliation(s)
- Michael C Oh
- Departments of Neurological Surgery, University of California, SanFrancisco, CA 94117 USA
| | | | | | | | | | | | | | | | | |
Collapse
|
|
10
|
Gepp RDA, Couto JMC, Silva MDD, Quiroga MRS. Mortality is higher in patients with leptomeningeal metastasis in spinal cord tumors. ARQUIVOS DE NEURO-PSIQUIATRIA 2013; 71:40-5. [PMID: 23288021 DOI: 10.1590/s0004-282x2012005000019] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/03/2011] [Accepted: 07/30/2012] [Indexed: 11/22/2022]
Abstract
UNLABELLED Spinal cord tumors are a rare neoplasm of the central nervous system (CNS). The occurrence of metastases is related to poor prognosis. The authors analyzed one series of metastasis cases and their associated mortality. METHODS Clinical characteristics were studied in six patients with intramedullary tumors with metastases in a series of 71 surgical cases. RESULTS Five patients had ependymomas of which two were WHO grade III. The patient with astrocytoma had a grade II histopathological classification. Two patients required shunts for hydrocephalus. The survival curve showed a higher mortality than the general group of patients with no metastases in the CNS (p<0.0001). CONCLUSION Mortality is elevated in patients with metastasis and greater than in patients with only primary lesions. The ependymomas, regardless of their degree of anaplasia, are more likely to cause metastasis than spinal cord astrocytomas.
Collapse
|
|
11
|
Khalatbari MR, Jalaeikhoo H, Hamidi M, Moharamzad Y. Craniospinal dissemination of filum myxopapillary ependymoma following spinal trauma: case report and literature review. Childs Nerv Syst 2013; 29:149-52. [PMID: 23011448 DOI: 10.1007/s00381-012-1927-1] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/25/2012] [Accepted: 09/07/2012] [Indexed: 11/26/2022]
Abstract
BACKGROUND Myxopapillary ependymoma is a rare benign variant of ependymoma, occurring most commonly in the cauda equina/filum terminale of the spinal cord. It is rare in pediatric patients. Dissemination of the myxopapillary ependymoma along the neuraxis is uncommon both in adults and pediatric patients. METHODS We report a 16-year-old boy with dissemination of the myxopapillary ependymoma along the neuraxis following spinal trauma. The possible mechanisms of tumor dissemination are discussed with a review of the literature.
Collapse
Affiliation(s)
- Mahmoud Reza Khalatbari
- Department of Neurosurgery, Arad Hospital, Somayeh St., between Dr. Shariati & Bahar Ave., Tehran, Iran, 1445613131.
| | | | | | | |
Collapse
|
|
12
|
Chakraborti S, Govindan A, Alapatt JP, Radhakrishnan M, Santosh V. Primary myxopapillary ependymoma of the fourth ventricle with cartilaginous metaplasia: a case report and review of the literature. Brain Tumor Pathol 2011; 29:25-30. [PMID: 21837503 DOI: 10.1007/s10014-011-0059-8] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2011] [Accepted: 07/10/2011] [Indexed: 02/08/2023]
Abstract
Myxopapillary ependymoma (MPE), which is a benign histological subtype of ependymoma, is found predominantly in the cauda equina region. It occurs rarely in the brain and mostly as a metastatic deposit from a spinal lesion. The occurrence of primary intracranial MPE is exceptional, with only 11 cases reported to date. We report an additional case of intracranial MPE, which is the third reported case in the fourth ventricle. The tumor manifested in a 50-year-old lady, who presented with features of raised intracranial pressure. A gross total resection of the tumor was achieved. Histologically, the tumor had characteristic features of MPE with focal metaplastic cartilaginous deposit. On further evaluation, there was no evidence of a primary tumor in the spinal cord. Intracranial MPE needs further evaluation by craniospinal MRI to exclude an unrecognized primary in the spinal region, which could warrant surgical attention.
Collapse
Affiliation(s)
- Shrijeet Chakraborti
- Department of Neuropathology, National Institute of Mental Health and Neurosciences, Hosur Road, Bangalore, Karnataka, 560 029, India.
| | | | | | | | | |
Collapse
|
|
13
|
Tumori spinali e intrarachidei. Neurologia 2011. [DOI: 10.1016/s1634-7072(11)70656-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
|
|
14
|
Kucia EJ, Maughan PH, Kakarla UK, Bambakidis NC, Spetzler RF. Surgical technique and outcomes in the treatment of spinal cord ependymomas: part II: myxopapillary ependymoma. Neurosurgery 2010; 68:90-4; discussion 94. [PMID: 21099714 DOI: 10.1227/neu.0b013e3181fdf912] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
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.
Collapse
Affiliation(s)
- Elisa J Kucia
- Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, Arizona, USA
| | | | | | | | | |
Collapse
|
|
15
|
Mridha AR, Sharma MC, Sarkar C, Suri V, Rishi A, Garg A, Suri A. Myxopapillary ependymoma of lumbosacral region with metastasis to both cerebellopontine angles: report of a rare case. Childs Nerv Syst 2007; 23:1209-13. [PMID: 17643247 DOI: 10.1007/s00381-007-0423-5] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/27/2007] [Indexed: 10/23/2022]
Abstract
INTRODUCTION Myxopapillary ependymomas are low grade tumours that are known to recur locally even after complete excision, but metastasis to distant sites is extremely uncommon. CASE REPORT We report an unusual case of lumbo-sacral myxopapillary ependymoma in a 13-year-old boy with metastasis to both cerebellopontine angles. To the best of our knowledge, this is the youngest patient of metastatic myxopapillary ependymoma.
Collapse
Affiliation(s)
- A R Mridha
- Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
| | | | | | | | | | | | | |
Collapse
|
|
16
|
Tzerakis N, Georgakoulias N, Kontogeorgos G, Mitsos A, Jenkins A, Orphanidis G. Intraparenchymal myxopapillary ependymoma: case report. Neurosurgery 2005; 55:981. [PMID: 15934181 DOI: 10.1227/01.neu.0000137278.84588.06] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022] Open
Abstract
OBJECTIVE AND IMPORTANCE Myxopapillary ependymoma is a histological variant of ependymoma found in the cauda equina region. The most characteristic histological feature of myxopapillary tumors is the abundance of intercellular and perivascular mucin and the arborizing vasculature, which tends to form papillae. Primary intracerebral myxopapillary ependymomas are extremely rare; only three cases have been reported in the previous literature. CLINICAL PRESENTATION A 68-year-old man presented with disorientation and dizziness caused by a cystic left frontal intraparenchymal lesion. This proved to be a myxopapillary ependymoma. Similarities to previously reported cases are discussed, as are the findings on magnetic resonance imaging. There is also a literature review of the histological findings, natural history, and outcome of surgically treated myxopapillary ependymoma. INTERVENTION The lesion was totally removed. After surgery, the patient was neurologically intact and had an uneventful recovery. CONCLUSION This is the fourth reported case of histologically proven primary myxopapillary intracranial ependymoma.
Collapse
Affiliation(s)
- Nikolaos Tzerakis
- Department of Neurosurgery, G. Gennimatas General Hospital, Athens, Greece.
| | | | | | | | | | | |
Collapse
|
|
17
|
|
|
18
|
Awaya H, Kaneko M, Amatya VJ, Takeshima Y, Oka S, Inai K. Myxopapillary ependymoma with anaplastic features. Pathol Int 2004; 53:700-3. [PMID: 14516321 DOI: 10.1046/j.1440-1827.2003.01546.x] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
A case of myxopapillary ependymoma with anaplastic features in 15-year-old boy is reported. The tumor was located in the intradural space extending to the 12th thoracic to 2nd lumbar vertebral level. It was excised with the accompanying spinal arch of the T12 to L2 vertebra. At operation, the tumor was not attached to the surrounding soft and bony tissues. The tumor, measuring 49 x 19 x 15 mm, was brownish-yellow in color and involved the conus medullaris and filum terminale. Histologically, the tumor was composed of biphasic features of a hypercellular papillary growth area and a hypocellular myxoid area. In the papillary growth area, ependymal rosettes and perivascular pseudorosettes were observed. These findings were consistent with those of a myxopapillary ependymoma, although multiple foci of punctate necrosis within the tumor and proliferation of endothelial cells showing glomeruloid structures were observed. Many mitotic figures were also observed. In addition, the Ki-67 labeling index of tumor cells was 10.1%. These findings are unusual for myxopapillary ependymoma, and therefore, it appeared that the diagnosis of myxopapillary ependymoma with anaplastic features was appropriate.
Collapse
Affiliation(s)
- Hirokazu Awaya
- Department of Pathology, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan.
| | | | | | | | | | | |
Collapse
|