Oncocytic ependymoma: a new morphological variant of high-grade ependymal neoplasm composed of mitochondrion-rich epithelioid cells.
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Oncocytic ependymoma: a new morphological variant of high-grade ependymal neoplasm composed of mitochondrion-rich epithelioid cells. / Vajtai, Istvan; von Gunten, Michael; Fung, Christian; Brekenfeld, Caspar; Kappeler, Andreas; Reinert, Michael M.
in: PATHOL RES PRACT, Jahrgang 207, Nr. 1, 1, 2011, S. 49-54.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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T1 - Oncocytic ependymoma: a new morphological variant of high-grade ependymal neoplasm composed of mitochondrion-rich epithelioid cells.
AU - Vajtai, Istvan
AU - von Gunten, Michael
AU - Fung, Christian
AU - Brekenfeld, Caspar
AU - Kappeler, Andreas
AU - Reinert, Michael M
PY - 2011
Y1 - 2011
N2 - Oncocytomas are defined as tumors containing in excess of 50% large mitochondrion-rich cells, irrespective of histogenesis and dignity. Along the central neuraxis, oncocytomas are distinctly uncommon but relevant to the differential diagnosis of neoplasia marked by prominent cytoplasmic granularity. We describe an anaplastic ependymoma (WHO grade III) with a prevailing oncocytic component that was surgically resected from the right fronto-insular region of a 43-year-old female. Preoperative imaging showed a fairly circumscribed, partly cystic, contrast-enhancing mass of 2 cm × 2 cm × 1.7 cm. Histology revealed a biphasic neoplasm wherein conventional ependymal features coexisted with plump epithelioid cells replete with brightly eosinophilic granules. Whereas both components displayed an overtly ependymal immunophenotype, including positivity for S100 protein and GFAP, as well as "dot-like" staining for EMA, the oncocytic population also tended to intensely react with the antimitochondrial antibody 113-1. Conversely, failure to bind CD68 indicated absence of significant lysosomal storage. Negative reactions for both pan-cytokeratin (MNF 116) and low molecular weight cytokeratin (CAM 5.2), as well as synaptophysin and thyroglobulin, further assisted in ruling out metastatic carcinoma. In addition to confirming the presence of "zipper-like" intercellular junctions and microvillus-bearing cytoplasmic microlumina, electron microscopy allowed for the pervasive accumulation of mitochondria in tumor cells to be directly visualized. A previously not documented variant, oncocytic ependymoma, is felt to add a reasonably relevant novel item to the differential diagnosis of granule-bearing central nervous system neoplasia, in particular oncocytic meningioma, granular cell astrocytoma, as well as metastatic deposits by oncocytic malignancies from extracranial sites.
AB - Oncocytomas are defined as tumors containing in excess of 50% large mitochondrion-rich cells, irrespective of histogenesis and dignity. Along the central neuraxis, oncocytomas are distinctly uncommon but relevant to the differential diagnosis of neoplasia marked by prominent cytoplasmic granularity. We describe an anaplastic ependymoma (WHO grade III) with a prevailing oncocytic component that was surgically resected from the right fronto-insular region of a 43-year-old female. Preoperative imaging showed a fairly circumscribed, partly cystic, contrast-enhancing mass of 2 cm × 2 cm × 1.7 cm. Histology revealed a biphasic neoplasm wherein conventional ependymal features coexisted with plump epithelioid cells replete with brightly eosinophilic granules. Whereas both components displayed an overtly ependymal immunophenotype, including positivity for S100 protein and GFAP, as well as "dot-like" staining for EMA, the oncocytic population also tended to intensely react with the antimitochondrial antibody 113-1. Conversely, failure to bind CD68 indicated absence of significant lysosomal storage. Negative reactions for both pan-cytokeratin (MNF 116) and low molecular weight cytokeratin (CAM 5.2), as well as synaptophysin and thyroglobulin, further assisted in ruling out metastatic carcinoma. In addition to confirming the presence of "zipper-like" intercellular junctions and microvillus-bearing cytoplasmic microlumina, electron microscopy allowed for the pervasive accumulation of mitochondria in tumor cells to be directly visualized. A previously not documented variant, oncocytic ependymoma, is felt to add a reasonably relevant novel item to the differential diagnosis of granule-bearing central nervous system neoplasia, in particular oncocytic meningioma, granular cell astrocytoma, as well as metastatic deposits by oncocytic malignancies from extracranial sites.
KW - Adult
KW - Diagnosis, Differential
KW - Humans
KW - Female
KW - Immunohistochemistry
KW - Magnetic Resonance Imaging
KW - Immunophenotyping
KW - Microscopy, Electron, Transmission
KW - Adenoma, Oxyphilic/immunology/pathology/ultrastructure
KW - Brain Neoplasms/immunology/pathology/ultrastructure
KW - Ependymoma/immunology/pathology/ultrastructure
KW - Epithelioid Cells/pathology/ultrastructure
KW - Glial Fibrillary Acidic Protein/immunology/metabolism
KW - Intercellular Junctions/ultrastructure
KW - Mitochondria/pathology/ultrastructure
KW - Neoplasm Recurrence, Local/pathology/radiotherapy
KW - S100 Proteins/immunology/metabolism
KW - Adult
KW - Diagnosis, Differential
KW - Humans
KW - Female
KW - Immunohistochemistry
KW - Magnetic Resonance Imaging
KW - Immunophenotyping
KW - Microscopy, Electron, Transmission
KW - Adenoma, Oxyphilic/immunology/pathology/ultrastructure
KW - Brain Neoplasms/immunology/pathology/ultrastructure
KW - Ependymoma/immunology/pathology/ultrastructure
KW - Epithelioid Cells/pathology/ultrastructure
KW - Glial Fibrillary Acidic Protein/immunology/metabolism
KW - Intercellular Junctions/ultrastructure
KW - Mitochondria/pathology/ultrastructure
KW - Neoplasm Recurrence, Local/pathology/radiotherapy
KW - S100 Proteins/immunology/metabolism
M3 - SCORING: Journal article
VL - 207
SP - 49
EP - 54
JO - PATHOL RES PRACT
JF - PATHOL RES PRACT
SN - 0344-0338
IS - 1
M1 - 1
ER -