• Journal of Korean Neurosurgical Society
• /
• 제54권6호
• /
• pp.521-524
• /
• 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
• /
• 제37권2호
• /
• pp.128-132
• /
• 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
• /
• 제40권3호
• /
• pp.164-168
• /
• 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.

• 대한세포병리학회지
• /
• 제10권1호
• /
• pp.73-78
• /
• 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
• /
• 제61권2호
• /
• pp.233-242
• /
• 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
• /
• 제50권1호
• /
• pp.57-59
• /
• 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
• /
• 제59권2호
• /
• pp.83-90
• /
• 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.

• 대한영상의학회지
• /
• 제82권5호
• /
• pp.1341-1345
• /
• 2021

척수 뇌실막세포종은 일반적으로 성인에서 척수 내에 위치한다. 경막내 수외 척수 뇌실막세포종은 극히 드물다. 대부분의 척수 뇌실막세포종은 MRI에서 다양한 조영증강을 보인다. 저자는 MRI에서 조영증강을 보이지 않으며, 병리학적으로 확인된 52세 여자 환자의 경막내 수외 뇌실막세포종을 경험하여 이에 대해 보고하고자 한다.

• Radiation Oncology Journal
• /
• 제7권2호
• /
• pp.197-203
• /
• 1989

1969년부터 1983년까지 버지니아 의대부속병원에서 방사선 치료를 받은 34예의 원발성 척수암중, 조직검사 또는 아절제술을 시행한 후 수술후 방사선 요법으로 치료받은 32예 (Astrocytoma 16, ependymoma 16)에 대한 치료실적을 보고하고자 한다. 29예에서는 $45\~55Gy$를 5내지 6주간에 조사 받았으며 나머지 3예에서는 40Gy이하의 선량을 조사받았다. 50Gy 이상 조사된 6예는 모두 척수가 조사야에 포함되었다. 본 연구의 최저 추적 기간은 5년이었다. 방사선 치료 실패 후 재수술로 치료된 3예를 포함하여, 모든 예에서 생존율은 5년과 10년에서 각각 $73\%\;와\;50\%$이었다. 각각의 투병생존율은 $60\%\;와\;32\%$이었다. 45Gy 이상의 선량을 받은 29예에서는 5년 무병생존이 $63\%$이었다. 13예에서 치료실패를 경험했으며 치료실패는 조사야에서만 관찰되었다. Ependymoma가 astrocytoma보다 통계적으로 유의하게 무병생존율이 높았다 $(45\%\;:\; 89\%(p<0.05))$, 원발부위에 있어 경추가 포함된 예에서 그렇지 않은 예보다 생존율이 저하되었다. 원발부위의 cauda equina포함여부는 예후에 영향이 없었다. 최고 20년간 추적결과 방사선 치료에 의한 신경성 합병증은 관찰되지 않았다. 본 연구는 원발성 척수암의 치료에 있어서 방사선 치료가 안전하고 효과적임을 확인하였다.

• Journal of Korean Neurosurgical Society
• /
• 제47권2호
• /
• pp.102-106
• /
• 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.