• Journal of Korean Neurosurgical Society
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• v.54 no.6
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• pp.521-524
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• 2013

Ependymoma can spread via cerebrospinal fluid, but late spinal recurrences of intracranial tumor are very rare. We describe a case of a 33-year-old male who presented with multiple, delayed, recurrent lesions in the spinal cord from an intracranial ependymoma. The patient underwent gross total resection and postoperative radiation therapy 14 years prior to visit for a low grade ependymoma in the 4th ventricle. The large thoraco-lumbar intradural-extramedullary spinal cord tumor was surgically removed and the pathologic diagnosis was an anaplastic ependymoma. An adjuvant whole-spine radiation therapy for residual spine lesions was performed. After completion of radiation therapy, a MRI showed a near complete response and the disease was stable for three years.

• Journal of Yeungnam Medical Science
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• v.37 no.2
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• pp.128-132
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• 2020

Tanycytic ependymoma is a rare variant of ependymoma that commonly affects the cervical and thoracic spinal cord. It usually arises as intramedullary lesions, and extramedullary cases are extremely rare. We report a case of a 44-year-old woman who was diagnosed with tanycytic ependymoma in her lumbar spine at level 2-3. The tumor mass developed in an intradural extramedullary location. Histopathologically, tanycytic ependymoma can be misdiagnosed as schwannoma or pilocytic astrocytoma. Immunohistochemical findings such as strong positivity for glial fibrillary acidic protein, perinuclear dot-like positive patterns for epithelial membrane antigen, and focal positivity for S100 protein are helpful in diagnosing tanycytic ependymoma. It is important to be aware of this rare tumor to ensure appropriate patient management and accurate prognosis.

• Journal of Korean Neurosurgical Society
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• v.40 no.3
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• pp.164-168
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• 2006

Objective : The goal of treatment for spinal cord ependymoma is complete removal without postoperative neurological deficit. The authors analyzed the surgical results and factors influencing the postoperative prognosis. Methods : Fifty-one cases of primary spinal cord ependymoma, surgically treated between 1979 and 2003, were retrospectively analyzed. The mean follow-up period was 44 months. Results : Gross total removal was achieved in 42 patients and incomplete removal in nine. The proportion of complete surgical removals was influenced by tumor location and histology. Disease progression was observed in five cases [9.8%], the mean progression free interval after surgical removal was 48 months and the 5-year progression free rate was 68%. Disease progression was found in none of the 42 cases who underwent complete removal, and in 5 of 9 cases who hadincomplete removal group [P<0.001]. Statistically significant disease-progression factors by multivariate analysis were the surgical extent of removal [P=0.012]. preoperative functional status [P=0.032] the presence of intratumoral cysts [P=0.007] and postoperative radiation therapy [P=0042]. Of those patients who underwent incomplete removal, radiation therapy was found to significantly improve the clinical result [P=0042]. Conclusion : In the surgical treatment of spinal cord ependymoma, preoperative functional status, the presence of intratumoral cysts, the extent of removal, and postoperative radiation therapy were found to be significant prognostic factors of postoperative outcome.

• The Korean Journal of Cytopathology
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• v.10 no.1
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• pp.73-78
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• 1999

Myxopapillary ependymoma generally arise in the conus medullaris and filum terminale of adult spinal cord. These tumors are readily recognized due to unique histopathologic features, however, their cytologic features are not well described. When only a tiny sample is obtained, cytologic examination using crush preparation may be a useful diagnostic tool to help appropriate intraoperative diagnosis. We present the crush cytologic features of myxopapillary ependymoma arising in thoracic and lumbar spinal cord of a 13-year-old boy. The patient had complained of paraparesis and back pain for 1 month. The MRI image revealed a relatively well demarcated intramedullary mass in T11-L1 levels. Crush preparation for cytology were peformed by biopsy material. Crush cytologic findings revealed high cellularity and small sized branching papillary clusters on fibrillary or mucinous background. The tumor cells had uniform round or elongated nuclei. The cytoplasmic process of tumor cells were attached to the vascular wall. Between the tumor cells and vascular walls, the perivascular collar of globoid acellular stroma with metachromatic reaction on toluidin blue stain was noted. The crush preparation of myxopapillary ependymoma is considered as a simple and highly accurate diagnostic tool for differentiation from other intramedullary neoplasms of central nervous system.

• Journal of Korean Neurosurgical Society
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• v.61 no.2
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• pp.233-242
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• 2018

Objective : A spinal cord subependymoma is an uncommon, indolent, benign spinal cord tumor. It is radiologically similar to a spinal cord ependymoma, but surgical findings and outcomes differ. Gross total resection of the tumor is not always feasible. The present study was done to determine the clinical, radiological and pathological characteristics of spinal cord subependymomas. Methods : We retrospectively reviewed the medical records of ten spinal cord subependymoma patients (M : F=4 : 6; median 38 years; range, 21-77) from four institutions. Results : The most common symptoms were sensory changes and/or pain in eight patients, followed by motor weakness in six. The median duration of symptoms was 9.5 months. Preoperative radiological diagnosis was ependymoma in seven and astrocytoma in three. The tumors were located eccentrically in six and were not enhanced in six. Gross total resection of the tumor was achieved in five patients, whereas subtotal or partial resection was inevitable in the other five patients due to a poor dissection plane. Adjuvant radiotherapy was performed in two patients. Neurological deterioration occurred in two patients; transient weakness in one after subtotal resection and permanent weakness after gross total resection in the other. Recurrence or regrowth of the tumor was not observed during the median 31.5 months follow-up period (range, 8-89). Conclusion : Spinal cord subependymoma should be considered when the tumor is located eccentrically and is not dissected easily from the spinal cord. Considering the rather indolent nature of spinal cord subependymomas, subtotal removal without the risk of neurological deficit is another option.

• Journal of Korean Neurosurgical Society
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• v.50 no.1
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• pp.57-59
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• 2011

Intradural extramedullary (IDEM) ependymomas occur very rarely and little has been reported about their clinical characteristics. The authors present a case of a 57-year-old woman with an IDEM ependymoma. She was referred for the evaluation of a 4-month history of increasing neck pain and muscular weakness of the left extremities. Magnetic resonance imaging (MRI) of the cervical spine demonstrated an IDEM tumor with spinal cord compression. At the time of surgery, an encapsulated IDEM tumor without a dural attachment or medullary infiltration was noted, but the tumor capsule adherent to the spinal cord and root was left in place to minimize the risk of neurological sequelae. Histologic examination revealed a benign classic ependymoma. The post-operative course was uneventful and radiotherapy was performed. The patient showed an excellent clinical recovery, with no recurrence after 5 years of follow-up.

• Journal of Korean Neurosurgical Society
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• v.59 no.2
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• pp.83-90
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• 2016

Ependymomas occur in both the brain and spine. The prognosis of these tumors sometimes differs for different locations. The genetic landscape of ependymoma is very heterogeneous despite the similarity of histopathologic findings. In this review, we describe the genetic differences between spinal ependymomas and their intracranial counterparts to better understand their prognosis. From the literature review, many studies have reported that spinal cord ependymoma might be associated with NF2 mutation, NEFL overexpression, Merlin loss, and 9q gain. In myxopapillary ependymoma, NEFL and HOXB13 overexpression were reported to be associated. Prior studies have identified HIC-1 methylation, 4.1B deletion, and 4.1R loss as common features in intracranial ependymoma. Supratentorial ependymoma is usually characterized by NOTCH-1 mutation and p75 expression. TNC mutation, no hypermethylation of RASSF1A, and GFAP/NeuN expression may be diagnostic clues of posterior fossa ependymoma. Although MEN1, TP53, and PTEN mutations are rarely reported in ependymoma, they may be related to a poor prognosis, such as recurrence or metastasis. Spinal ependymoma has been found to be quite different from intracranial ependymoma in genetic studies, and the favorable prognosis in spinal ependymoma may be the result of the genetic differences. A more detailed understanding of these various genetic aberrations may enable the identification of more specific prognostic markers as well as the development of customized targeted therapies.

• Journal of the Korean Society of Radiology
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• v.82 no.5
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• pp.1341-1345
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• 2021

Spinal ependymomas are generally located in the intramedullary compartment in adults. Intradural extramedullary spinal ependymomas are extremely rare. Spinal ependymomas show various contrast enhancements on MRI. In this study, we report a rare case of a 52-year-old female who had a pathologically confirmed intradural extramedullary ependymoma that showed no enhancement on MRI.

• Radiation Oncology Journal
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• v.7 no.2
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• pp.197-203
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• 1989

Of 34 evaluated patients with primary spinal cord tumors, 32 were irradiated at our institution between 1969 and 1983. The results are reported of 32 patients, 16 with ependymoma and 16 with astrocytoma, who were treated with post-operative external beam radiotherapy following biopsy or subtotal resection Twenty-nine patients received $45\~55Gy$ megavoltage beam irradiation in $5\~6$ weeks and the remaining three patients received less than 40Gy. Spinal cord was in the irradiated field for six patients who received more than 50Gy. The minimum follow-up was five years. Five and ten year acturaial survival rates for entire group of patients were $73\%(22/30)\;and\;50\%(8/16)$, including three patients who were salvaged by surgery after radiation failures. Corresponding five and ten year relapse free survival rates were $60\%(18/30)\;and\;32\%(6/19)$, respectively. Of the 29 patients who recived more than 45Gy, relapse free survival at five years was $63\%(17/27)$. Treatment failed in 13 patients and all of those failures were in the irradiated portal. Patients with ependymomas have significantly better relapse free survival than those with astrocytomas, $80\%\;vs.\;40\%$ (p<0.05). There was significant difference in survival between patients with tumors involving the cervical spine and those with tumors in the other loactions, $45\%$ vs. $89\%(p<0.05)$. There was no significant differnece in survival between patients with cauda equina tumors and those with tumors at spinal cord, $100\%\;vs.\;68\%(p>0.05)$. No radiotherapy related neurological deficit was noted with a maximum 20 year follow-up. This study confirms that external beam radiotherapy is a safe and effective treatment modality for primary spinal cord tumors.

• Journal of Korean Neurosurgical Society
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• v.47 no.2
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• pp.102-106
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• 2010

Objective : To evaluate the clinical results of gross total resection in the surgical approach to spinal ependymoma. Methods : Between June 1995 and May 2009,13 males and 8 females (mean age 34) diagnosed with intramedullary or extramedullary spinal ependymoma were surgically treated at our centre. The neurological and functional state of each patient were evaluated according to the modified McCormick scale. Results : The average follow-up duration was 54 months (ranging from 12 to 168 months). The locations of the lesions were : thoracic region (4, 19%), lumbar region (7, 34%), cervical region (4, 19%), cervicothoracic region (3, 14%) and conus medullaris (3, 14%). Four patients (19%) had deterioration of neurological function in the early postoperative period. The neurological function of three patients was completely recovered at the 6th postoperative month, while that of another patient was recovered at the 14th month. In the last assessment of neurological function, 20 patients (95%) were assessed as McCormick grade 1. No perioperative complications developed in any of our patients. In one patient's 24-month assessment, tumour recurrence was observed. Re-operation was not performed and the patient was taken under observation. Conclusion : Two determinants of good clinical results after spinal ependymoma surgery are a gross total resection of the tumour and a good neurological condition before the operation. Although neurological deficits in the early postoperative period can develop as a result of gross total tumour resection, significant improvement is observed six months after the operation.