Intracranial Anaplastic Ependymoma Research Articles

OPHTHALMOLOGICAL AND NEUROLOGICAL MANIFESTATIONS OF A DOG WITH INTRACRANIAL ANAPLASTIC EPENDYMOMA. CASE REPORT

The objective is to report ependymoma associated with uveitis in a four-month-old male boxer crossbreed. The dog initially presented in both eyes conjunctival hyperemia, blepharospasm, projection of the nictitating membrane, and photophobia, characteristic of uveitis.Traumatic uveitis was rejected. Six days after onset of ophthalmic symptoms, vomiting, incoordination, and behavior changes occurred. Neurological symptoms and eyebrow contraction suggesting head pain and brain injury were investigated by computed tomography (CT) nine days after, when the dog showed spasms of the limbs and neck followed by respiratory arrest with reversal and stabilization. The scans revealed extensive amorphic neoformation in the diencephalon, midbrain, and within the right lateral ventricle, along with sinistral displacement of the cerebral sickle. The animal was euthanized, and necropsy of the head revealed hydrocephalus and an intracerebral tumor mass consistent with the CT imaging. Histopathological evaluation by hematoxylin and eosin staining revealed tissue alterations in several CNS segments, showing several pseudorosettes in the neuropil, mitosis, and a high degree of cell atypia, indicating ependymoma.Inflammatory, hemorrhagic, and necrotic tissue lesions were observed in the brainstem and cerebellum due to compression by tumor tissue and hydrocephalus. The neoplasia was phenotyped by Immunohistochemistry (IHC), and tested positive for the tumoral markers vimentin and glial fibrillary acid protein, confirming intracranial anaplastic ependymoma. Behavior changes and neurological signs resulted from vascular, inflammatory, and degenerative processes in the neuropil caused by neoplasm compression and invasion of brain tissue. Although dogs with ependymoma often present with neurological disease ,in the present case, blepharospasm was the first symptom noticed by the owner, and it persisted until euthanasia. According to the literature, and confirmed in the evolution of the current case, the symptoms are related to tumor location and extent and to secondary lesions due to tumor expansion. Clinical symptomatology and complementary laboratory testing, CT, necropsy, histology, and IHC characterized ependymoma, a rare condition in young dogs.

MRI Features of Intracranial Anaplastic Ependymomas: A Comparison of Supratentorial and Infratentorial Lesions.

Background: Several previous reports of anaplastic ependymomas have described their imaging features, and most of these studies were case reports. However, no studies have compared the magnetic resonance imaging (MRI) features between the infratentorial and supratentorial anaplastic ependymomas.Objective: The goal of this study was to explore MRI characteristics for intracranial anaplastic ependymomas.Material and Methods: We retrospectively reviewed the demographics of 165 patients and MRI findings of 60 patients with supratentorial (SAEs) and infratentorial anaplastic ependymomas (IAEs) before surgery. The demographics and MRI features for SAEs and IAEs were compared and evaluated.Results: Among the 60 patients, most SAEs (91.7%) were extraventricular, whereas most IAEs (91.7%) were intraventricular. Of sixty intracranial anaplastic ependymomas, most lesions were well-defined (n = 45) and round-like (n = 36). On T1-weighted imaging, compared with the gray matter, the SAEs exhibited heterogeneous signal intensity, whereas IAEs exhibited iso-hypointense signals. T2 signals exhibited greater associations with hyperintense signals in IAEs; however, SAEs showed hyperintense or hypointense–hyperintense. On diffusion-weighted imaging (DWI), almost all solid tissues of SAEs appeared as hyperintense, whereas IAEs exhibited iso-hypointense signals. Peritumoral edema and intratumoral hemorrhage occurred more frequently in SAEs. Almost all anaplastic ependymomas exhibited heterogeneous enhancement. Cysts or necrosis was associated with 56 anaplastic ependymomas; however, large cysts were more prevalent in SAEs. On magnetic resonance spectroscopy (MRS), the mean choline/creatine (Cho/Cr) and choline/N-acetyl-aspartate (Cho/NAA) ratio of anaplastic ependymomas were (6.58 ± 4.26) and (8.84 ± 6.34), respectively, representing typical high-grade tumors.Conclusion: We demonstrate the conventional and functional MRI features of intracranial anaplastic ependymomas, including DWI and MRS. MRI characteristics, such as location, cyst, diffusion restriction, and peritumoral edema, differed between supratentorial and infratentorial locations. Cho/Cr and Cho/ NAA ratios of anaplastic ependymomas are increased.

"Drop" Metastases from an Operated Case of Intracranial Anaplastic Ependymoma Identified on Fluoro-2-deoxyglucose Positron Emission Tomography/Computed Tomography.

The seeding of tumor through cerebrospinal fluid (CSF) from primary intracranial tumors is very rare, often goes undetected, and is usually identified only on autopsy. CSF cytology along with magnetic resonance imaging constitutes the standard approach of diagnosing this grave condition. Use of fluoro-2-deoxyglucose positron emission tomography/computed tomography (PET/CT) in indentifying spinal metastases from primary intracranial malignancies is very limited and has been reported in patients with metastatic glioblastoma multiforme and medulloblastomas. We present a rare case of metastatic anaplastic ependymoma to show the potentially clinically utility of PET/CT in diagnosing leptomeningeal or the so-called “drop” metastases.

MS-09 * PROGNOSTIC IMPLICATIONS OF HISTOLOGICAL CLEAR CELLS IN HIGH GRADE INTRACRANIAL EPENDYMAL TUMORS: A RETROSPECTIVE ANALYSIS FROM A TERTIARY CARE HOSPITAL IN PAKISTAN

Clear Cell variant in Ependymal tumors is rare. We aim to compare the features & clinical outcomes of Clear Cell variant with the Classic pathology of high grade intracranial Ependymal tumors in Pakistan. A retrospective cohort study conducted at the Department of Neurosurgery, Aga Khan University. The medical record files from 2003 - 2013 were reviewed, MRI/C.T scans and Histopathological slides of WHO grade III Ependymal tumors were reviewed. Patients lost to follow-up were contacted. Analysis was done on SPSS 20.There were 10 cases of clear cell variant and 23 cases of classic Anaplastic Ependymoma. The median age of Clear Cell variant was 52 years (range 24years-72 years),whereas of Anaplastic Ependymoma was 37 years (range 3 months to 65 years). Symptoms included Headache 66% in CCE and 63% in AE, Seizures 30% in CCE and 22% in AE, and raised ICP in 30% in CCE and 54% of AE. CCE was Supratentorial in 70% cases, intracranial Anaplastic Ependymoma was supratentorial in 58% of cases. Gross Total Resection was achieved in 55% cases in CCE and 26% cases in AE. Subtotal resection was done in 45% cases in CCE and 65% in AE. Radiotherapy was given in 60% patients in CCE and in 50% in AE. Recurrence was there in 80% cases of CCE and 55% in AE. Repeat Surgery was done in 30% cases of CCE and 34% cases of AE. Median Progression free survival was 8 months(range 3-28 months), and Overall survival was 10 months(range 3-41 months) in CCE. Median Progression free survival was 4 months (range 0.5-53 months), Overall survival was 14 months (range 1.5- 36 months) in AE. The median Progression free survival was better in Clear Cell variant than Anaplastic Ependymoma, this could be explained by a higher Gross total resection in CCE, whereas the median Overall survival was worse in Clear Cell variant group.

MERLIN EXPRESSION IN PEDIATRIC ANAPLASTIC EPENDYMOMAS REAL TIME PCR STUDY

The most common genetic abnormalities of ependymomas involve the chromosome 22 where there is the oncosuppressor gene neurofibromin 2 (NF2). NF2 mutations are primarily encountered in spinal lesions. In contrast, NF2 alterations do not seem related to tumor grade. We studied the NF2 expression through a real-time polymerase chain reaction in 25 pediatric anaplastic ependymomas. We compared the NF2 expression in neoplastic and non-neoplastic tissues, in supratentorial and infratentorial ependymomas and in primitive and non-primitive tumors (recurrences and metastases). Statistical analysis did not prove significant differences. Our results suggest that NF2 alterations are not typical of intracranial anaplastic ependymomas.

Dissemination limits the survival of patients with anaplastic ependymoma after extensive surgical resection, meticulous follow up, and intensive treatment for recurrence

The extent of resection is the most consistent factor affecting outcome of intracranial ependymomas. The outcomes in patients with intracranial anaplastic ependymomas who underwent more than subtotal resection and intensive treatment for recurrence were reviewed retrospectively. Between 1989 and 2007, 18 patients underwent more than subtotal resection at Tohoku University Hospital. Adjuvant chemoradiation therapy was conducted in 16 patients. Meticulous follow-up was performed for early detection of recurrence. Intensive therapy including surgery and chemoradiation therapy was added for recurrence. Median survival in all 18 patients was 136 months. The 5- and 10-year survival rates were 59.1% and 50.7%, respectively. Eight patients died during the observation period, five of dissemination, but none of uncontrollable local recurrence. High resection rate, meticulous follow-up, and intensive treatment for recurrence improved the survival of patients with anaplastic ependymoma. Dissemination was the life-determining factor in this series of patients.

Treatment of a recurrent intracranial anaplastic ependymoma – A case report

Objective: Ependymomas represent about 10% of intracranial neoplasms in children. The proportion of cases defined as anaplastic is about 25%. Due to the survival rate after 5 years of 40–50%, as well as the tumor's high recurrence rates, further research into the efficacy of treatment strategies and adjuvant therapy is necessary. Extent of resection remains the most important determinant of survival in patients with ependymomas. Patient/Methods: We report a case of malignant ependymoma (WHO Grade III) first diagnosed in the age of 17 months in a child who has so far lived for over 9 years after onset of our interdisciplinary treatment Results: A 17 months old boy with vomiting presented with a tumor located in the fourth ventricle. The tumor was resected radically. Neuropathological examination revealed the diagnosis of anaplastic ependymoma, which was confirmed by two reference pathologies. He underwent a chemotherapy following the HIT-SKK-92 protocol and radiotherapy (54Gy). After 2 cycles MTX, Vincristin, Cyclophosphamid, Carboplatin, Etoposid and intrathecal MTX, a follow-up MRI revealed leucencephalopathy requiring immediate termination of MTX therapy. Within 27 months he developed supratentorial metastasis in both lateral ventricles and underwent multiple craniotomies for tumor resection as well as 2 stereotactic radiosurgeries for 3 targets (20Gy) and chemotherapy following the HIT-2000 and CWS-96 GPOH protocol. The last operation was in 10/2007. Currently, the patient is receiving temozolamide for 1 year. Besides a treated precocious puberty there are no major neurological and only mild neuropsychological deficits. Conclusion: Despite a poor prognosis due to the malignant ependymoma and the age of less than 3 years at onset this case demonstrates that the interdisciplinary treatment with a combination of surgery, chemotherapy and radiosurgery can achieve local control of anaplastic ependymoma without dramatic.

Loss of heterozygosity on chromosome 22 in human ependymomas.

Ependymomas are glial tumors of the brain and spinal cord. The most frequent genetic change in sporadic ependymomas is monosomy 22, suggesting the presence of an ependymoma tumor suppressor gene on chromosome 22. Thirty-three pairs of matched normal and tumor specimens from ependymoma patients were genotyped using 12 polymorphic microsatellite markers spanning the long arm of chromosome 22. Allelic deletion was found in 12 of 33 tumors (36.4%). Eight tumors showed partial deletions and 4 tumors exhibited loss of the entire arm of 22q. We identified two common regions of deletion: one at 22q11.21-12.2 flanked by markers D22S420 and D22S300, and a second candidate region at 22q13.1-13.3 between D22S274 and D22S1149. The size of each region was 21.1 and 2.4 cM, respectively. Thus, our results suggest that one or more tumor suppressor genes associated with ependymoma may be present on chromosome 22. Comparison of these results with clinicopathological data indicate that allelic losses on 22q tend to occur more frequently in intracranial anaplastic ependymomas in children and intraspinal ependymomas in adults.