• Journal of Korean Neurosurgical Society
• /
• v.59 no.2
• /
• 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.

• Journal of Yeungnam Medical Science
• /
• v.37 no.2
• /
• 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
• /
• v.54 no.6
• /
• 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 Korean Neurosurgical Society
• /
• v.50 no.3
• /
• pp.244-247
• /
• 2011

Two-thirds of ependymomas arise in the infratentorial or intraventricles, whereas one-third are located supratentorially. But supratentorial "cortical" ependymomas are very rare. We report a case of a cortical ependymoma in a 21-month-old boy. The patient presented with simple partial seizures. This tumor was located in the postcentral gyrus and he had gross total excision. Microscopy and immunohistochemistry showed grade II differentiation ependymoma.

• Asian Pacific Journal of Cancer Prevention
• /
• v.15 no.2
• /
• pp.867-870
• /
• 2014

Purpose: This study used receiver operating characteristic curve to analyze Surveillance, Epidemiology and End Results (SEER) ependymoma data to identify predictive models and potential disparity in outcome. Materials and Methods: This study analyzed socio-economic, staging and treatment factors available in the SEER database for ependymoma. For the risk modeling, each factor was fitted by a Generalized Linear Model to predict the outcome ('brain and other nervous systems' specific death in yes/no). The area under the receiver operating characteristic curve (ROC) was computed. Similar strata were combined to construct the most parsimonious models. A random sampling algorithm was used to estimate the modeling errors. Risk of ependymoma death was computed for the predictors for comparison. Results: A total of 3,500 patients diagnosed from 1973 to 2009 were included in this study. The mean follow up time (S.D.) was 79.8 (82.3) months. Some 46% of the patients were female. The mean (S.D.) age was 34.4 (22.8) years. Age was the most predictive factor of outcome. Unknown grade demonstrated a 15% risk of cause specific death compared to 9% for grades I and II, and 36% for grades III and IV. A 5-tiered grade model (with a ROC area 0.48) was optimized to a 3-tiered model (with ROC area of 0.53). This ROC area tied for the second with that for surgery. African-American patients had 21.5% risk of death compared with 16.6% for the others. Some 72.7% of patient who did not get RT had cerebellar or spinal ependymoma. Patients undergoing surgery had 16.3% risk of death, as compared to 23.7% among those who did not have surgery. Conclusion: Grading ependymoma may dramatically improve modeling of data. RT is under used for cerebellum and spinal cord ependymoma and it may be a potential way to improve outcome.

• Journal of Korean Neurosurgical Society
• /
• v.39 no.5
• /
• pp.382-384
• /
• 2006

We report a rare case of primary intradural extramedullary myxopapillary ependymoma of the spinal cord. A 45-year-old woman was admitted to the author's institution with a history of progressive paraparesis[grade IV/IV] with back pain. Neurologic examination revealed decreased sensation below T12 sensory dermatome level. Magnetic resonance imaging[MRI] revealed an intermediate enhanced intradural extramedullary tumor extending from T12. Total resection of the tumor was achieved by T12 laminectomy. Intraoperatively, there was no finding of attachment to rootlet and dura. Histopathological examination identified the tumor as a myxopaillary ependymoma. To the best of my knowledge, this is the first reported case of primary intradural extramedullary ependymoma in Korea.

• Journal of Korean Neurosurgical Society
• /
• v.57 no.6
• /
• pp.487-490
• /
• 2015

We report a 10-year-old boy with supratentorial cortical ependymoma that rapidly grew in the course of 3 years. He suffered generalized seizures when he was 5 years old; MRI showed a small cortical lesion in the right postcentral gyrus. MRI performed 2 years later revealed no changes. For the next 3 years he was free of seizures. However, at the age of 10 he again suffered generalized seizures and MRI disclosed a large parietal tumor. It was resected totally and he remains free of neurological deficits. The histopathological diagnosis was ependymoma. Pediatric supratentorial cortical ependymomas are extremely rare. We recommend including cortical ependymoma as a differential diagnosis in pediatric patients with cortical mass lesions presenting with seizures and careful follow-up even in the absence of symptoms because these tumors may progress.

• The Korean Journal of Cytopathology
• /
• v.10 no.1
• /
• 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
• /
• v.48 no.2
• /
• pp.153-156
• /
• 2010

The authors present a rare case of clear cell ependymoma that developed in the cauda equina. A 54-year-old man was admitted to hospital with intermittent lower back pain. A neurological examination conducted on admission revealed no sensory or motor disturbance. Deep tendon reflexes in both lower extremities were normal. Magnetic resonance images demonstrated a 1.0 cm-sized intradural mass at the filum terminale. Gross total resection was performed via total laminectomy of L1 and L2. The tumor was confirmed to be clear cell ependymoma by histopathologic examination. His symptom was relieved after surgery.

• Journal of Korean Neurosurgical Society
• /
• v.39 no.5
• /
• pp.378-381
• /
• 2006

A 65-year-old woman presented with a history of severe lower back pain on forward-flexion for 2 months duration. Magnetic resonance Imaging revealed a high signal mass with a tail on T1-weighted images at the L3 level. A total surgical resection was performed via a posterior approach with the aid of a microscope. Histopathological examination of the tumor revealed two pathological components : lipoma and myxopapillary ependymoma. The presence of dual histological components in one spinal cord tumor is rare. There are no prior reports of both types of cells [adipose and ependymal] grown simultaneously in a single tumor of the filum terminale in the medical literature. We report a unique case of the co-existence of lipoma and myxopapillary ependymoma within the same tumor located at the filum terminale and review related literature.