Giant cell ependymoma-report of three cases and review of the literature. 2012

Jian Yi Li, and Jose I Lopez, and Suzanne Z Powell, and Stephen W Coons, and Gregory N Fuller
Department of Pathology and Lab. Medicine, North Shore and Long Island Jewish Health System, Hofstra North Shore-LIJ School of Medicine, Lake Success, NY 11042, USA. jli2@nshs.edu

Ependymomas constitute the most common type of primary spinal cord tumors, and are subclassified as myxopapillary ependymoma, classic ependymoma, and anaplastic ependymoma. Ependymomas can be further subclassified based on morphologic phenotype: cellular, papillary, tanycytic, clear cell, pigmented and epithelioid. Giant cell ependymoma (GCE), a rare variant, has recently been described. Reported cases have exhibited a wide anatomic distribution, including spinal cord, cerebrum and cerebellum. We report here three cases of GCE, arising from cerebrum in a 5-year-old girl, spinal cord in a 34-year-old female and cerebellum in an 86-year-old female respectively. Histologically those cases showed prominent pleomorphic giant cells with focal perivascular pseudorosettes in all cases. Tumor cells were immunopositive for GFAP and EMA. Only the first case was qualified for anaplastic ependymoma. No recurrence was noted in these three cases after 57, 46 and 6 months of follow-up respectively. By reviewing the literature, GCEs arising from spinal cord and cerebellum tended to have low-grade morphology while supratentorially located GCEs tended to have anaplastic features. GCEs were preferentially located in extraventricular regions. Anaplastic GCEs in adult population seemed to pursue a more aggressive behavior. Gross total resection should still be the main treatment for GCEs.

UI MeSH Term Description Entries
D002528 Cerebellar Neoplasms Primary or metastatic neoplasms of the CEREBELLUM. Tumors in this location frequently present with ATAXIA or signs of INTRACRANIAL HYPERTENSION due to obstruction of the fourth ventricle. Common primary cerebellar tumors include fibrillary ASTROCYTOMA and cerebellar HEMANGIOBLASTOMA. The cerebellum is a relatively common site for tumor metastases from the lung, breast, and other distant organs. (From Okazaki & Scheithauer, Atlas of Neuropathology, 1988, p86 and p141) Benign Cerebellar Neoplasms,Cerebellar Cancer,Malignant Cerebellar Neoplasms,Cerebellar Neoplasms, Benign,Cerebellar Neoplasms, Malignant,Cerebellar Neoplasms, Primary,Cerebellar Tumors,Neoplasms, Cerebellar,Neoplasms, Cerebellar, Benign,Neoplasms, Cerebellar, Malignant,Neoplasms, Cerebellar, Primary,Primary Neoplasms, Cerebellum,Benign Cerebellar Neoplasm,Cancer, Cerebellar,Cerebellar Cancers,Cerebellar Neoplasm,Cerebellar Neoplasm, Benign,Cerebellar Neoplasm, Malignant,Cerebellar Neoplasm, Primary,Cerebellar Tumor,Cerebellum Primary Neoplasm,Cerebellum Primary Neoplasms,Malignant Cerebellar Neoplasm,Neoplasm, Benign Cerebellar,Neoplasm, Cerebellar,Neoplasm, Cerebellum Primary,Neoplasm, Malignant Cerebellar,Primary Cerebellar Neoplasm,Primary Cerebellar Neoplasms,Primary Neoplasm, Cerebellum,Tumor, Cerebellar
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004806 Ependymoma Glioma derived from EPENDYMOGLIAL CELLS that tend to present as malignant intracranial tumors in children and as benign intraspinal neoplasms in adults. It may arise from any level of the ventricular system or central canal of the spinal cord. Intracranial ependymomas most frequently originate in the FOURTH VENTRICLE and histologically are densely cellular tumors which may contain ependymal tubules and perivascular pseudorosettes. Spinal ependymomas are usually benign papillary or myxopapillary tumors. (From DeVita et al., Principles and Practice of Oncology, 5th ed, p2018; Escourolle et al., Manual of Basic Neuropathology, 2nd ed, pp28-9) Ependymoma, Myxopapillary,Ependymoma, Papillary,Anaplastic Ependymoma,Cellular Ependymoma,Clear Cell Ependymoma,Papillary Ependymoma,Anaplastic Ependymomas,Ependymoma, Anaplastic,Ependymomas,Ependymomas, Anaplastic,Ependymomas, Myxopapillary,Ependymomas, Papillary,Myxopapillary Ependymoma,Myxopapillary Ependymomas,Papillary Ependymomas
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000369 Aged, 80 and over Persons 80 years of age and older. Oldest Old
D013120 Spinal Cord Neoplasms Benign and malignant neoplasms which occur within the substance of the spinal cord (intramedullary neoplasms) or in the space between the dura and spinal cord (intradural extramedullary neoplasms). The majority of intramedullary spinal tumors are primary CNS neoplasms including ASTROCYTOMA; EPENDYMOMA; and LIPOMA. Intramedullary neoplasms are often associated with SYRINGOMYELIA. The most frequent histologic types of intradural-extramedullary tumors are MENINGIOMA and NEUROFIBROMA. Intradural-Extramedullary Spinal Cord Neoplasms,Intramedullary Spinal Cord Neoplasms,Intramedullary Spinal Cord Neoplasms, Primary,Neoplasms, Spinal Cord,Primary Intramedullary Spinal Cord Neoplasms,Primary Spinal Cord Neoplasms, Intramedullary,Spinal Cord Neoplasms, Benign,Spinal Cord Neoplasms, Intradural-Extramedullary,Spinal Cord Neoplasms, Intramedullary,Spinal Cord Neoplasms, Malignant,Spinal Cord Neoplasms, Primary Intramedullary,Tumors, Spinal Cord,Intradural Extramedullary Spinal Cord Neoplasms,Neoplasm, Spinal Cord,Spinal Cord Neoplasm,Spinal Cord Neoplasms, Intradural Extramedullary,Spinal Cord Tumor,Spinal Cord Tumors,Tumor, Spinal Cord
D014408 Biomarkers, Tumor Molecular products metabolized and secreted by neoplastic tissue and characterized biochemically in cells or BODY FLUIDS. They are indicators of tumor stage and grade as well as useful for monitoring responses to treatment and predicting recurrence. Many chemical groups are represented including HORMONES; ANTIGENS; amino and NUCLEIC ACIDS; ENZYMES; POLYAMINES; and specific CELL MEMBRANE PROTEINS and LIPIDS. Biochemical Tumor Marker,Cancer Biomarker,Carcinogen Markers,Markers, Tumor,Metabolite Markers, Neoplasm,Tumor Biomarker,Tumor Marker,Tumor Markers, Biochemical,Tumor Markers, Biological,Biochemical Tumor Markers,Biological Tumor Marker,Biological Tumor Markers,Biomarkers, Cancer,Marker, Biochemical Tumor,Marker, Biologic Tumor,Marker, Biological Tumor,Marker, Neoplasm Metabolite,Marker, Tumor Metabolite,Markers, Biochemical Tumor,Markers, Biological Tumor,Markers, Neoplasm Metabolite,Markers, Tumor Metabolite,Metabolite Markers, Tumor,Neoplasm Metabolite Markers,Tumor Markers, Biologic,Tumor Metabolite Marker,Biologic Tumor Marker,Biologic Tumor Markers,Biomarker, Cancer,Biomarker, Tumor,Cancer Biomarkers,Marker, Tumor,Markers, Biologic Tumor,Markers, Carcinogen,Metabolite Marker, Neoplasm,Metabolite Marker, Tumor,Neoplasm Metabolite Marker,Tumor Biomarkers,Tumor Marker, Biochemical,Tumor Marker, Biologic,Tumor Marker, Biological,Tumor Markers,Tumor Metabolite Markers
D015726 Giant Cells Multinucleated masses produced by the fusion of many cells; often associated with viral infections. In AIDS, they are induced when the envelope glycoprotein of the HIV virus binds to the CD4 antigen of uninfected neighboring T4 cells. The resulting syncytium leads to cell death and thus may account for the cytopathic effect of the virus. Giant Cells, Multinucleated,Multinucleated Giant Cells,Polykaryocytes,Syncytium,Syncytia,Cell, Giant,Cell, Multinucleated Giant,Cells, Giant,Cells, Multinucleated Giant,Giant Cell,Giant Cell, Multinucleated,Multinucleated Giant Cell,Polykaryocyte

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