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Yang CG, Xue RF, Yang LX, Jieda XL, Xiang W, Zhou J. Ventricular system-unrelated cerebellar ependymoma: A case report. World J Clin Cases 2024; 12:5814-5820. [PMID: 39247726 PMCID: PMC11263058 DOI: 10.12998/wjcc.v12.i25.5814] [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: 04/26/2024] [Revised: 06/10/2024] [Accepted: 07/02/2024] [Indexed: 07/12/2024] Open
Abstract
BACKGROUND An ependymoma is a glial tumor that usually occurs in or near the ventricle, close to the ependyma. It rarely occurs exclusively in the brain parenchyma without being associated with the ventricle. CASE SUMMARY Here, we report a rare case of a cerebellar ependymoma completely located in the brain parenchyma. A previously healthy 32-year-old female with a 1-month history of dizziness was admitted to our hospital. During hospitalization, magnetic resonance imaging of the brain revealed a space-occupying lesion measuring 57 mm × 41 mm × 51 mm in the right cerebellar hemisphere and inferior cerebellar vermis. The patient underwent surgical resection for the right cerebellar mass. Postoperative pathological examination revealed an ependymoma. At 1 year follow-up, the patient was doing well and showed no recurrence. CONCLUSION We conducted a literature review and summarized three theories regarding ependymomas located exclusively in the brain parenchyma, which are key to the diagnosis of intraparenchymal cerebellar ependymomas. Surgery and postoperative radiotherapy are the primary treatment options for ependymomas.
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Affiliation(s)
- Chao-Ge Yang
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Rui-Feng Xue
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Lu-Xia Yang
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Xiao-Lin Jieda
- Department of Neonatology, Children's Medical Center, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Wei Xiang
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Jie Zhou
- Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
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Bohara S, Shrestha S, Thapa A. A rare presentation of extra-axial supratentorial ependymoma with subdural hematoma mimicking a parasagittal meningioma. BRAIN & SPINE 2023; 3:101715. [PMID: 37383435 PMCID: PMC10293308 DOI: 10.1016/j.bas.2023.101715] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/03/2022] [Revised: 01/09/2023] [Accepted: 01/18/2023] [Indexed: 06/30/2023]
Abstract
Introduction Primary extra-axial ependymomas, though rare, the majority of such lesions are identified as WHO grade III ependymomas. These ependymomas may mimick a meningioma on radiological investigations which can be confirmed by histopathology. Research Question We show in this case report a rare presentation of extra-axial supratentorial ependymoma with concomitant subdural hematoma mimicking a parasagittal meningioma. Material and Methods A 59 years lady with no known comorbidities presented with weakness of right half of body and decreased speech for 2 days. She was aphasic. Contrast MRI brain revealed an extra-axial dural-based homogenously enhancing lesion in the left anterior 1/3rd parasagittal area with left frontotemporoparietal chronic subdural hematoma. With a provisional diagnosis of meningioma, the patient was subjected to bifrontal open-book craniotomy with gross total excision of lesion with periosteal graft duraplasty and acrylic cranioplasty. Left sided frontotemporal subacute SDH with thin greenish yellow membrane was present. In post operative period, patient quickly became E4V5M6 with power of 4/5 in the right half of body which was same as compared to the preoperative period. Results The biopsy of the mass, however, revealed features suggestive of extra-axial supratentorial ependymoma (WHO Grade III). Immunohistochemistry supported the diagnosis of supratentorial ependymoma, NOS. The patient was then referred for further chemoradiation. Discussion and Conclusion We report the first case of extra-axial supratentorial ependymoma mimicking a parasagittal meningioma occurring with adjacent subdural hematoma. Clinical and imaging background along with a complete pathological examination with immunohistochemical study is essential to confirm the diagnosis of rare brain tumours.
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Aftahy AK, Barz M, Krauss P, Liesche F, Wiestler B, Combs SE, Straube C, Meyer B, Gempt J. Intraventricular neuroepithelial tumors: surgical outcome, technical considerations and review of literature. BMC Cancer 2020; 20:1060. [PMID: 33143683 PMCID: PMC7640680 DOI: 10.1186/s12885-020-07570-1] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2020] [Accepted: 10/26/2020] [Indexed: 02/06/2023] Open
Abstract
Background Intraventricular neuroepithelial tumors (IVT) are rare lesions and comprise different pathological entities such as ependymomas, subependymomas and central neurocytomas. The treatment of choice is neurosurgical resection, which can be challenging due to their intraventricular location. Different surgical approaches to the ventricles are described. Here we report a large series of IVTs, its postoperative outcome at a single tertiary center and discuss suitable surgical approaches. Methods We performed a retrospective chart review at a single tertiary neurosurgical center between 03/2009–05/2019. We included patients that underwent resection of an IVT emphasizing on surgical approach, extent of resection, clinical outcome and postoperative complications. Results Forty five IVTs were resected from 03/2009 to 05/2019, 13 ependymomas, 21 subependymomas, 10 central neurocytomas and one glioependymal cyst. Median age was 52,5 years with 55.6% (25) male and 44.4% (20) female patients. Gross total resection was achieved in 93.3% (42/45). 84.6% (11/13) of ependymomas, 100% (12/21) of subependymomas, 90% (9/10) of central neurocytomas and one glioependymal cyst were completely removed. Postoperative rate of new neurological deficits was 26.6% (12/45). Postoperative new permanent cranial nerve deficits occurred in one case with 4th ventricle subependymoma and one in 4th ventricle ependymoma. Postoperative KPSS was 90% (IR 80–100). 31.1% of the patients improved in KPSS, 48.9% remained unchanged and 20% declined. Postoperative adverse events rate was 20.0%. Surgery-related mortality was 2.2%. The rate of shunt/cisternostomy-dependent hydrocephalus was 13.3% (6/45). 15.4% of resected ependymomas underwent adjuvant radiotherapy. Mean follow-up was 26,9 (±30.1) months. Conclusion Our surgical findings emphasize satisfactory complete resection throughout all entities. Surgical treatment can remain feasible, if institutional experience is given. Satisfying long-term survival and cure is possible by complete removal. Gross total resection should always be performed under function-remaining aspects due to mostly benign or slow growing nature of IVTs. Further data is needed to evaluate standard of care and alternative therapy options in rare cases of tumor recurrence or in case of patient collective not suitable for operative resection.
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Affiliation(s)
- A Kaywan Aftahy
- Department of Neurosurgery, Technical University Munich, Medical Faculty, School of Medicine, Klinikum rechts der Isar, Ismaninger Str. 22, 81675, Munich, Germany.
| | - Melanie Barz
- Department of Neurosurgery, Technical University Munich, Medical Faculty, School of Medicine, Klinikum rechts der Isar, Ismaninger Str. 22, 81675, Munich, Germany
| | - Philipp Krauss
- Department of Neurosurgery, Technical University Munich, Medical Faculty, School of Medicine, Klinikum rechts der Isar, Ismaninger Str. 22, 81675, Munich, Germany
| | - Friederike Liesche
- Department of Neuropathology, Technical University Munich, School of Medicine, Klinikum rechts der Isar, Institute of Pathology, Munich, Germany
| | - Benedikt Wiestler
- Department of Neuroradiology, Technical University Munich, School of Medicine, Klinikum rechts der Isar, Munich, Germany
| | - Stephanie E Combs
- Department of Radiation Oncology, Technical University Munich, School of Medicine, Klinikum rechts der Isar, Munich, Germany.,Department of Radiation Sciences (DRS) Helmholtz Zentrum Munich, Institute of Innovative Radiotherapy (iRT), Munich, Germany.,German Cancer Consortium (DKTK), Partner Site Munich, Munich, Germany
| | - Christoph Straube
- Department of Radiation Oncology, Technical University Munich, School of Medicine, Klinikum rechts der Isar, Munich, Germany
| | - Bernhard Meyer
- Department of Neurosurgery, Technical University Munich, Medical Faculty, School of Medicine, Klinikum rechts der Isar, Ismaninger Str. 22, 81675, Munich, Germany
| | - Jens Gempt
- Department of Neurosurgery, Technical University Munich, Medical Faculty, School of Medicine, Klinikum rechts der Isar, Ismaninger Str. 22, 81675, Munich, Germany
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Adult cerebellopontine angle ependymoma presenting as an isolated cisternal mass: A case report. J Med Imaging Radiat Sci 2020; 51:689-693. [PMID: 32888857 DOI: 10.1016/j.jmir.2020.08.007] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/08/2019] [Revised: 08/02/2020] [Accepted: 08/09/2020] [Indexed: 11/23/2022]
Abstract
INTRODUCTION Ependymomas represent approximately 2%-8% of all primary intracranial brain tumors. The occurrence of extra-axial posterior fossa ependymomas in adults is rare. CASE AND OUTCOMES We report a case of extra-axial cerebellopontine (CP) angle ependymoma in an adult patient, managed through gross total resection (GTR) and adjuvant radiotherapy. At her one-year postoperative visit, the patient remained clinically stable without any symptoms or focal neurological deficit and a follow up MRI showed no evidence of tumor recurrence. DISCUSSION Only six cases of adult cerebellopontine angle ependymomas have been reported in the English literature, with the left side affected more commonly. Including this case, the mean age of the reported cases of adult extra-axial CP angle ependymoma is 44.14 years (range 22-66 years). Men accounted for five out of seven cases (71.4%). Maximal surgical resection is the mainstay of treatment in extra-axial CP angle ependymomas. Among seven reported cases, five received GTR and two had subtotal resection (STR). Patients were followed an average of 13.6 months (range 2-30 months) and only two patients with STR died during the follow-up period (6 weeks and 2 months after surgery). Six of the seven reported cases (including this one) received adjuvant radiotherapy. CONCLUSION Although rare, extra-axial CP angle ependymomas should be considered as a differential diagnosis to other lesions of the CPA. Radical resection, whenever possible, is usually associated with a good outcome. Adjuvant radiotherapy remains an optional treatment with an unknown impact on overall and progression-free survival.
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Nambirajan A, Malgulwar PB, Sharma MC, Singh A, Pathak P, Satyarthee GD, Garg A. C11orf95-RELAfusion present in a primary intracranial extra-axial ependymoma: Report of a case with literature review. Neuropathology 2016; 36:490-495. [DOI: 10.1111/neup.12299] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/15/2015] [Revised: 02/15/2016] [Accepted: 02/15/2016] [Indexed: 12/26/2022]
Affiliation(s)
- Aruna Nambirajan
- Department of Pathology; All India Institute of Medical Sciences; New Delhi India
| | - Prit Benny Malgulwar
- Department of Pathology; All India Institute of Medical Sciences; New Delhi India
| | - Mehar C. Sharma
- Department of Pathology; All India Institute of Medical Sciences; New Delhi India
| | - Anutosh Singh
- Neurosurgery; All India Institute of Medical Sciences; New Delhi India
| | - Pankaj Pathak
- Department of Pathology; All India Institute of Medical Sciences; New Delhi India
| | | | - Ajay Garg
- Neuroradiology; All India Institute of Medical Sciences; New Delhi India
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Yang Y, Tian KB, Hao SY, Wu Z, Li D, Zhang JT. Primary Intracranial Extra-Axial Anaplastic Ependymomas. World Neurosurg 2016; 90:704.e1-704.e9. [PMID: 26947725 DOI: 10.1016/j.wneu.2016.02.095] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2016] [Revised: 02/23/2016] [Accepted: 02/24/2016] [Indexed: 11/30/2022]
Abstract
BACKGROUND Ependymomas are usually located in the ventricular system or in the central canal of the spinal cord; intracranial extra-axial ependymomas (IEAEs) are rare. To date, only 17 cases of IEAEs have been reported. CASE DESCRIPTION We report 2 cases with 3 IEAEs (anaplastic) that were initially misdiagnosed. In Case 1 (47-year-old male), the para-falcine lesion was initially refractory to radiosurgery and gross total resection (GTR) was required due to relentless growth. The lesion had adhered to the falx and was well demarcated from the surrounding cortex. It was then correctly diagnosed as an anaplastic ependymoma on the basis of histopathology, and the patient received radiotherapy. No recurrence was observed after the 53-month follow-up. In Case 2 (30-year-old male), 2 IEAEs underwent staged surgeries and were identified as extra-axial lesions without connection to the ventricular system. Near total resection (NTR) and GTR were achieved in the right temporal and right occipital lesions, respectively, but the patient declined radiotherapy. The residual tumor after NTR regrew rapidly, and aggressive resection was performed followed by radiotherapy. No further recurrence was observed after 28 months. The previous 17 cases were male predominant (76.5%) without correct preoperative diagnoses; no recurrence was observed after total resection in the 9 patients reported in the literature. CONCLUSIONS IEAEs are rare and have a wide spectrum of clinical and radiological phenotypes. Preoperative diagnosis is difficult. Favorable outcomes for IEAEs can be achieved by GTR plus radiotherapy. Multiple IEAEs benefit from tailored staged surgical resection plus radiotherapy.
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Affiliation(s)
- Yang Yang
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China; China National Clinical Research Center for Neurological Diseases; Center of Brain Tumor, Beijing Institute for Brain Disorders; Beijing Key Laboratory of Brian Tumor
| | - Kai-Bing Tian
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China; China National Clinical Research Center for Neurological Diseases; Center of Brain Tumor, Beijing Institute for Brain Disorders; Beijing Key Laboratory of Brian Tumor
| | - Shu-Yu Hao
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China; China National Clinical Research Center for Neurological Diseases; Center of Brain Tumor, Beijing Institute for Brain Disorders; Beijing Key Laboratory of Brian Tumor
| | - Zhen Wu
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China; China National Clinical Research Center for Neurological Diseases; Center of Brain Tumor, Beijing Institute for Brain Disorders; Beijing Key Laboratory of Brian Tumor
| | - Da Li
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China; China National Clinical Research Center for Neurological Diseases; Center of Brain Tumor, Beijing Institute for Brain Disorders; Beijing Key Laboratory of Brian Tumor.
| | - Jun-Ting Zhang
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China; China National Clinical Research Center for Neurological Diseases; Center of Brain Tumor, Beijing Institute for Brain Disorders; Beijing Key Laboratory of Brian Tumor.
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Ye J, Zhu J, Yan J, Chen P, Wan Z, Chen F, Zhang L, Qian J, Luo C. Analysis on therapeutic outcomes and prognostic factors of intracranial ependymoma: a report of 49 clinical cases in a single center. Neurol Sci 2015. [PMID: 26216494 DOI: 10.1007/s10072-015-2347-2] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
Although intracranial ependymoma is relatively rare, it is often associated with great clinical aggressiveness and poor overall survival. There are controversies over factors affecting the prognosis of the disease. The aim of this retrospective study was to evaluate factors that may affect the therapeutic outcome and prognosis of intracranial ependymoma by reviewing the medical records of 49 patients who were surgically treated in our hospital between 2001 and 2014. Univariate and multivariate analyses were performed to identify prognostic variables relative to patient and tumor characteristics, and treatment modalities. All 49 patients (24 men and 25 women; mean age 27.6 years) underwent surgical resection, of whom 14 patients also underwent postoperative radiotherapy. Local recurrence was found in 15 (48.8 %) patients, and 22 (51.2 %) patients died during the follow-up periods. The 5-year recurrence rate was 65 % and the survival rate was 51 %. The results of statistical analysis suggested that preoperative extraventricular drainage and surgical resection extent were prognostic factors related to progression-free survival, and that age, surgical resection extent and histological grade were closely associated with survival. Interestingly, there was a significant correlation between the symptom of hydrocephalus and age (P = 0.010), and patients with a better clinical status (KPS ≥ 80) were significantly associated with a lower WHO grade (P = 0.007). In conclusion, we confirmed that surgical resection extent was the major independent factor affecting both recurrence and survival of patients with intracranial ependymoma, while age and WHO grade were prognostic factors affecting survival but not recurrence.
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Affiliation(s)
- Jingliang Ye
- Department of Neurosurgery, Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China.,Department of Neurosurgery, CPLA No.98 Hospital, Huzhou, 313000, China
| | - Junle Zhu
- Department of Neurosurgery, Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China
| | - Jiayi Yan
- Department of Radiology, Shanghai Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200025, China
| | - Peiqin Chen
- Department of Neurosurgery, Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China
| | - Zhiping Wan
- Department of Neurosurgery, Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China
| | - Feng Chen
- Department of Neurosurgery, Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China
| | - Lei Zhang
- Department of Neurosurgery, Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China
| | - Jun Qian
- Department of Neurosurgery, Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China
| | - Chun Luo
- Department of Neurosurgery, Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China.
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Khilji MF, Hamid RS, Qureshi A. Supratentorial extraventricular anaplastic ependymoma in a child. BMJ Case Rep 2014; 2014:bcr-2014-203750. [PMID: 24623545 DOI: 10.1136/bcr-2014-203750] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
A young child presented to the emergency department of a tertiary care hospital with on and off headache, focal seizures involving the left side of the body, weakness of left upper and lower limbs and vomiting for 2 weeks. Examination showed an alert child with grade 4/5 powers in left upper and lower limbs. Blood investigations were normal. An urgent CT of the brain showed intra-axial mass in the right frontal cerebral cortex, superolateral to the right lateral ventricle. MRI of the brain showed supratentorial extraventricular mass of 5.20 × 3.70 × 3.80 cm, in the right frontal cortex, emitting heterogeneous signals on T1, T2 and fluid-attenuated inversion recovery sequences and impression of astrocytoma, ependymoma or choroid plexus papilloma was made. Complete surgical resection of mass was performed. Histopathology of the mass proved it as WHO grade III anaplastic ependymoma. The child made an uneventful postoperative recovery and radiotherapy was followed.
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Smith AB, Smirniotopoulos JG, Horkanyne-Szakaly I. From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics 2013; 33:21-43. [PMID: 23322825 DOI: 10.1148/rg.331125192] [Citation(s) in RCA: 98] [Impact Index Per Article: 8.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
A variety of neoplasms may arise in the ventricular system. Intraventricular neoplasms may be discovered as an incidental finding at cross-sectional imaging or may manifest with varied symptoms depending on their location, including symptoms of increased intracranial pressure. These lesions may arise from various ventricular structures, including the ependymal lining (eg, ependymoma), subependymal layer (eg, subependymoma), or choroid plexus (eg, choroid plexus neoplasms), or they may have a cell of origin that has yet to be determined (eg, chordoid glioma). Other neoplasms involving the ventricular system include central neurocytoma, subependymal giant cell tumor, meningioma, rosette-forming glioneuronal tumor, and metastases. The differential diagnosis for intraventricular neoplasms can be broad, and many of them have similar patterns of signal intensity and contrast enhancement at imaging. However, the location of the lesion in the ventricular system-along with knowledge of the patient's age, gender, and underlying conditions-will help narrow the differential diagnosis.
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Affiliation(s)
- Alice Boyd Smith
- Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences, 4301 Jones Bridge Rd, Bethesda, MD 20814-4799, USA.
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