Humans ; Ependymoma/pathology* ; Nuclear Receptor Coactivator 1/metabolism*

Ependymomas usually develop from neuroectodermal organs. Here, we present an ependymoma arising from the pelvic cavity. A 27-year-old Korean female was admitted to the hospital with a sensation of abdominal fullness. Imaging studies revealed a huge heterogeneous nodular mass in the pelvis and lower abdomen. Laparotomy showed that two large masses with multiple nodules were located between the uterus and rectum and uterus and bladder, respectively. Histologically, the tumor was characterized by compact columnar neoplastic cells divided by fibrovascular septae. The neoplastic cells formed true ependymal rosettes and perivascular pseudorosettes. Immunohistochemical staining showed a strong positive reaction for glial fibrillary acidic protein (GFAP) and vimentin and a partial positive reaction for S100 and EMA. The tumor was thus diagnosed as an ependymoma arising from the pelvic cavity. The patient was treated with a debulking operation and chemotherapy based upon the in vitro chemosensitivity test results. The patient was free of cancer for 4 years following surgery. This is a rare case of extraneural ependymoma for which an in vitro chemosensitivity test was critical in determining the multidisciplinary approach for treatment.
Adult ; Ependymoma/drug therapy/*pathology ; Female ; Humans ; Pelvic Neoplasms/drug therapy/*pathology

Ependymomas usually develop from neuroectodermal organs. Here, we present an ependymoma arising from the pelvic cavity. A 27-year-old Korean female was admitted to the hospital with a sensation of abdominal fullness. Imaging studies revealed a huge heterogeneous nodular mass in the pelvis and lower abdomen. Laparotomy showed that two large masses with multiple nodules were located between the uterus and rectum and uterus and bladder, respectively. Histologically, the tumor was characterized by compact columnar neoplastic cells divided by fibrovascular septae. The neoplastic cells formed true ependymal rosettes and perivascular pseudorosettes. Immunohistochemical staining showed a strong positive reaction for glial fibrillary acidic protein (GFAP) and vimentin and a partial positive reaction for S100 and EMA. The tumor was thus diagnosed as an ependymoma arising from the pelvic cavity. The patient was treated with a debulking operation and chemotherapy based upon the in vitro chemosensitivity test results. The patient was free of cancer for 4 years following surgery. This is a rare case of extraneural ependymoma for which an in vitro chemosensitivity test was critical in determining the multidisciplinary approach for treatment.
Adult ; Ependymoma/drug therapy/*pathology ; Female ; Humans ; Pelvic Neoplasms/drug therapy/*pathology

Humans ; Ependymoma/secondary* ; Brain Neoplasms/pathology* ; Skin Neoplasms ; Melanoma ; Spinal Cord Neoplasms/pathology*

Clear cell ependymoma was included in the World Health Organization classification of the nervous system in 1993, and all the reported cases, except for two in the spinal cord, were located in the brain, mainly in the supratentorial compartment. Astrocytomas outnumber ependymomas in the spinal cord, and the two entities partly share cytologic findings such as long, bipolar glial processes and oval to round nuclei resembling those seen in pilocytic astrocytoma. Here, we report the first Korean case of intramedullary clear cell ependymoma of the spinal cord, which is the third case situated in the spinal cord in the literature. The crush smear revealed round-to-oval nuclei with occasional nuclear eosinophilic inclusion and rare nuclear grooves. Cytoplasm had fluffy eosinophilic glial processes, and acellular fibrillary zone. On hematoxylin-eosin stain, oval to round tumor cells had large central nuclei with indistinct nucleoli and a moderate amount of clear cytoplasm, i.e. perinuclear halo, mimicking oligodendroglioma. Perivascular pseudorosettes and ependymal clefts were rarely found. In retrospect, perinuclear halo was absent on crush smears. Ultrastructurally, they had extensive surface microvilli and edematous cytoplasm filled with abundant glial filaments and microlumens with or without microvilli. Intercellular long cell junctions of the zipper-like zonula adherens type were found.
Aged ; Ependymoma/metabolism/*pathology/ultrastructure ; Female ; Humans ; Immunohistochemistry ; Microscopy, Electron, Transmission ; Spinal Cord Neoplasms/metabolism/*pathology/ultrastructure

BACKGROUND: The purpose of this study was to evaluate the prognostic factors and outcomes in patients with ependymoma to management plans. METHODS: Between 1997 and 2013, 33 patients with 25 ependymomas (WHO grade II) and eight anaplastic ependymomas (WHO grade III) were pathologically diagnosed. Six were pediatric patients (mean age, 6.15 years; range, 1.3–11 years), while 27 were adults (mean age, 47.5 years; range, 19–70 years). Of those, there were 12 adult patients with totally resected ependymomas without anaplastic pathology and adjuvant treatment. Prognostic factors were assessed in ependymoma patients. Prognostic factors were studied using Kaplan-Meier estimates in subgroups. RESULTS: For six pediatric patients, the progression-free survival (PFS) was 43.7±13.5 months, and the overall survival (OS) was 58.1±13.7 months. For 27 adult patients, the PFS was 125.6±14.3 months, and the OS was 151.2±12.5 months. Age demonstrated a statistically significant effect on PFS (p=0.03) and OS (p=0.03). In adult ependymomas, the extent of tumor removal significantly affected PFS (p=0.03) and trended towards an effect on OS (p=0.06). Out of 12 patients with totally resected ependymomas without anaplastic pathology and adjuvant treatment, one patient showed tumor recurrence during follow-up (mean, 93.5 months; range, 27.9–162.7 months). CONCLUSION: Adult patients with ependymomas were found to have better survival rates compared to pediatric patients. We suggest that totally resected adult ependymomas without anaplastic pathology could be observed without any adjuvant treatment, regardless of the tumor location.
Adult ; Disease-Free Survival ; Ependymoma* ; Follow-Up Studies ; Humans ; Pathology ; Prognosis ; Radiotherapy ; Recurrence ; Retrospective Studies* ; Survival Rate

Papillary ependymoma is a rare variant of ependymoma and often gives rise to confusion with choroid plexus papilloma because of topographic, light microscopic and ultrastructural similarities. Here, we report two cases of papillary ependymomas regarding their unique clinicopathologic features and differential points from choroid plexus papilloma. Brain MRI revealed a large mass in the left lateral ventricle in one case and a 3cm sized mass in the pineal area and the 3rd ventricle in the other. Microscopically, the tumor was characterized by papillary and tubular structures. Immunohistochemically, the tumor cells in both cases expressed cytokeratins(CK22 and CAM 5.2) but did not express glial fibrillary acidic protein(GFAP), vimentin, epithelial membrane antigen, and S100 protein. This is a very unusual immunohistochemical feature for papillary ependymoma. Ultrastructurally, the tumor showed a mosaic pattern of tumor cells with frequent intercellular microrosettes having a few stubby microvilli, a few cilia and zonulae adherentes. The cytoplasmic processes were markedly reduced compared to conventional ependymoma. The cytoplasm did not contain intermediate filaments. Interestingly, the mitochondria showed abnormal features with a pleomorphic shape and abnormal cristae in both cases. These ultrastructural features enabled differentiation between papillary ependymoma and choroid plexus papilloma in addition to the light microscopic findings.
Adult ; Carcinoma, Papillary/*pathology/surgery ; Case Report ; Diagnosis, Differential ; Ependymoma/*pathology/surgery ; Fatal Outcome ; Female ; Follow-Up Studies ; Glioma/*pathology ; Human ; Magnetic Resonance Imaging ; Middle Age

Dysembryoplastic neuroepithelial tumors (DNETs) arise mostly in the supratentorial cerebral cortex. A very rare case of intraventricular DNET with diffuse ependymal involvement, which causes spinal drop metastasis, is presented.
Adult ; Diagnosis, Differential ; Ependymoma/*pathology/radiotherapy ; Female ; Humans ; Lumbosacral Region/*pathology ; *Magnetic Resonance Imaging ; Neuroepithelial Cells/pathology ; Spinal Neoplasms/radiotherapy/*secondary

Adolescent ; Brain Neoplasms ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Ganglioglioma ; metabolism ; pathology ; surgery ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Immunohistochemistry ; Male ; Tubulin ; metabolism ; Vimentin ; metabolism

Brain tumors are the leading cause of cancer death in children, with ependymoma being the third most common and posing a significant clinical burden. Its mechanism of pathogenesis, reliable prognostic indicators, and effective treatments other than surgical resection have all remained elusive. Until recently, ependymoma research was hindered by the small number of tumors available for study, low resolution of cytogenetic techniques, and lack of cell lines and animal models. Ependymoma heterogeneity, which manifests as variations in tumor location, patient age, histological grade, and clinical behavior, together with the observation of a balanced genomic profile in up to 50% of cases, presents additional challenges in understanding the development and progression of this disease. Despite these difficulties, we have made significant headway in the past decade in identifying the genetic alterations and pathways involved in ependymoma tumorigenesis through collaborative efforts and the application of microarray-based genetic (copy number) and transcriptome profiling platforms. Genetic characterization of ependymoma unraveled distinct mRNA-defined subclasses and led to the identification of radial glial cells as its cell type of origin. This review summarizes our current knowledge in the molecular genetics of ependymoma and proposes future research directions necessary to further advance this field.
Adult ; Animals ; Brain Neoplasms ; genetics ; pathology ; Cell Transformation, Neoplastic ; Child, Preschool ; Chromosome Aberrations ; Ependymoma ; genetics ; pathology ; Epigenesis, Genetic ; Gene Expression Profiling ; Humans ; Neuroglia ; pathology