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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.
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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
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Abstract
Metastases are the most common intracranial tumors in adults. Lung cancer, melanoma, renal cell carcinoma, and breast cancer are the most common primary tumors that metastasize to the brain. Improved detection of small metastases by MRI, and improved systemic therapy for primary tumors, resulted in increased incidence of brain metastasis. Advances in neuroanesthesia and neurosurgery have significantly improved the safety of surgical resection of brain metastases. Surgical approach and active management have become applicable for many patients. Subsequently, brain metastases diagnosis no longer equals palliative treatment. Moreover, the demand for diagnosing brain masses has increased with its associated challenges.
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Affiliation(s)
- Saber Tadros
- Laboratory of Pathology, National Cancer Institute, 10 Center Drive, Building 10, Room 3N248, Bethesda, MD 20814, USA.
| | - Abhik Ray-Chaudhury
- Surgical Neurology Branch, National Cancer Institute, 10 Center Drive, Building 10, Room 3D-03, MSC1414, Bethesda, MD 20892-3704, USA
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Spinal Myxopapillary Ependymoma: The Sapienza University Experience and Comprehensive Literature Review Concerning the Clinical Course of 1602 Patients. World Neurosurg 2019; 129:245-253. [PMID: 31152881 DOI: 10.1016/j.wneu.2019.05.206] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/05/2019] [Revised: 05/22/2019] [Accepted: 05/23/2019] [Indexed: 01/18/2023]
Abstract
BACKGROUND Spinal myxopapillary ependymoma (sMPE) is an uncommon primary spinal neoplasm infiltrating the spinal cord, conus medullaris (CM), and nerve roots. It is associated with low resection and high recurrence rates. The purpose of this literature review is to evaluate the exact impact of the involvement of the CM and the role played by gross total resection (GTR) on overall survival (OS). METHODS The English literature was systematically investigated using MEDLINE, the NIH Library, PubMed, and Google Scholar search engines with relevant queries. Case series reporting details concerning OS, GTR, and CM involvement rate were included, with a differential statistical weight given by the number of patients enrolled. A final cohort of 1602 clinical records was analyzed according to the 3 selected end point variables. RESULTS The average age was 36.44 ± 3.41 years, and the CM was involved in 28.4% ± 28.2% of cases. The average GTR rate was 53.94% ± 22.20%. Five- and 10-year OS rates were respectively available in 1170 and 1167 cases, with an average 5- and 10-year OS rate of 94.99% ± 3.87% and 92.31% ± 5.73%. By means of analyses performed both on aggregated and disaggregated data a strong positive statistical connection between GTR and increased OS was demonstrated despite the real clinical advantage could range as low as around 1% of increased OS rate. CONCLUSIONS Given the indolent sMPE behavior, it is difficult to evaluate the exact impact of GTR and CM involvement on OS; however, GTR could be associated with a limited survival advantage, whereas CM involvement could be associated with a survival disadvantage.
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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.
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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
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Jatana KR, Jacob A, Slone HW, Ray-Chaudhury A, Welling DB. Spinal Myxopapillary Ependymoma Metastatic to Bilateral Internal Auditory Canals. Ann Otol Rhinol Laryngol 2017; 117:98-102. [DOI: 10.1177/000348940811700204] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Objectives We report a rare case of spinal myxopapillary ependymoma metastatic to both internal auditory canals (IACs) and its implications for diagnosing neurofibromatosis type 2 (NF2). Methods We present a detailed clinical history, magnetic resonance imaging (MRI), intraoperative photographs, and histopathologic findings from a patient with bilateral IAC lesions, and review the diagnostic criteria for NF2. Results An 11-year-old boy with surgically resected spinal myxopapillary ependymoma, treated with total spine irradiation for recurrence, later showed bilaterally enhancing IAC lesions on T1-weighted MRI with contrast. The diagnosis of NF2 with bilateral vestibular schwannomas was entertained. Close examination of T2-weighted MRI, however, demonstrated the masses to be isointense to cerebrospinal fluid. This finding raised the possibility of other, more unusual IAC lesions. The patient underwent sequential suboccipital craniotomies for tissue diagnosis, and both IAC lesions were found to be myxopapillary ependymomas. Conclusions This is the youngest reported patient with metastatic myxopapillary ependymoma. Although vestibular schwannomas account for the majority of contrast-enhancing T1-weighted IAC lesions, other uncommon lesions may present in a similar manner. A T2 fast-spin echo screening MRI would have missed this patient's lesions. Therefore, both T1-weighted MRI with or without contrast and T2-weighted MRI may be necessary to distinguish vestibular schwannoma from other, more unusual IAC lesions.
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Affiliation(s)
- Kris R. Jatana
- Departments of Otolaryngology–Head and Neck Surgery, The Ohio State University, Columbus, Ohio
| | - Abraham Jacob
- Departments of Otolaryngology–Head and Neck Surgery, The Ohio State University, Columbus, Ohio
| | - H. Wayne Slone
- Departments of Radiology, The Ohio State University, Columbus, Ohio
| | | | - D. Bradley Welling
- Departments of Otolaryngology–Head and Neck Surgery, The Ohio State University, Columbus, Ohio
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Primary Seeding of Myxopapillary Ependymoma: Different Disease in Adult Population? Case Report and Review of Literature. World Neurosurg 2016; 99:812.e21-812.e26. [PMID: 28040529 DOI: 10.1016/j.wneu.2016.12.022] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2016] [Revised: 12/07/2016] [Accepted: 12/08/2016] [Indexed: 02/08/2023]
Abstract
Myxopapillary ependymoma (MPE) is a slow-growing tumor, occurring most often in adults. It originates from the filum terminale in the area of the conus medullaris and cauda equina and is considered a benign lesion. Despite this classification, however, recurrence after both partial and gross total resection is well known. In the pediatric population, primary MPE seeding and generally more aggressive clinical course is well documented and treated through gross total resection, if possible, followed by irradiation. In adults, however, primary MPE seeding is rarely seen. There are few prior reports describing primary metastases into multiple spinal locations in an adult before resection of an MPE. The reason for this difference among pediatric and adult MPE remains unclear. We present the case of a 32-year-old man with primary seeding of an MPE into multiple lumbosacral areas. The patient underwent gross total resection of the lesions and had an uneventful postoperative course. Primary seeding could be a sign of aggressive behavior in this tumor. Complete craniospinal magnetic resonance imaging studies should be done before and after surgery in patients who present with a multifocal primary MPE. Furthermore, patients with a history of primary tumor seeding of MPE should be thoroughly evaluated radiologically. Unlike in pediatric populations, the need for postoperative irradiation in adults is less clear and further studies-particularly genetic ones-are warranted.
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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.
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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.
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Affiliation(s)
- Shrijeet Chakraborti
- Department of Neuropathology, National Institute of Mental Health and Neurosciences, Hosur Road, Bangalore, Karnataka, 560 029, India.
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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.
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Affiliation(s)
- A R Mridha
- Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
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Dickerman RD, Reynolds AS, Gilbert E, Morgan B. The importance of early postoperative radiation in spinal myxopapillary ependymomas. J Neurooncol 2007; 82:323-5. [PMID: 17206476 DOI: 10.1007/s11060-006-9287-x] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/19/2006] [Accepted: 10/23/2006] [Indexed: 11/25/2022]
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Plans G, Brell M, Cabiol J, Villà S, Torres A, Acebes JJ. Intracranial retrograde dissemination in filum terminale myxopapillary ependymomas. Acta Neurochir (Wien) 2006; 148:343-6; discussion 346. [PMID: 16362177 DOI: 10.1007/s00701-005-0693-1] [Citation(s) in RCA: 41] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/13/2005] [Accepted: 10/18/2005] [Indexed: 12/26/2022]
Abstract
Myxopapillary ependymomas (ME) are considered benign tumours (WHO grade I) of the central nervous system with long term survival rates and a tendency to local recurrence. However an aggressive course has occasionally been described, leading to CSF dissemination and even systemic metastases. We describe the case of a 23-year-old man diagnosed with intracranial subarachnoid dissemination of a filum terminale ME three years after the initial diagnosis. We have performed a careful review of the literature on CSF dissemination in ME and finally propose treatment of these cases.
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Affiliation(s)
- G Plans
- Department of Neurosurgery, University of Barcelona, Hospital Universitari de Bellvitge, Spain.
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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.
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Affiliation(s)
- Hirokazu Awaya
- Department of Pathology, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan.
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