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.

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@article{8df72959ff134432be0209b84c94dbea,
title = "Oncocytic ependymoma: a new morphological variant of high-grade ependymal neoplasm composed of mitochondrion-rich epithelioid cells.",
abstract = "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.",
keywords = "Adult, Diagnosis, Differential, Humans, Female, Immunohistochemistry, Magnetic Resonance Imaging, Immunophenotyping, Microscopy, Electron, Transmission, Adenoma, Oxyphilic/immunology/*pathology/ultrastructure, Brain Neoplasms/immunology/*pathology/ultrastructure, Ependymoma/immunology/*pathology/ultrastructure, Epithelioid Cells/pathology/*ultrastructure, Glial Fibrillary Acidic Protein/immunology/metabolism, Intercellular Junctions/ultrastructure, Mitochondria/pathology/*ultrastructure, Neoplasm Recurrence, Local/pathology/radiotherapy, S100 Proteins/immunology/metabolism, Adult, Diagnosis, Differential, Humans, Female, Immunohistochemistry, Magnetic Resonance Imaging, Immunophenotyping, Microscopy, Electron, Transmission, Adenoma, Oxyphilic/immunology/*pathology/ultrastructure, Brain Neoplasms/immunology/*pathology/ultrastructure, Ependymoma/immunology/*pathology/ultrastructure, Epithelioid Cells/pathology/*ultrastructure, Glial Fibrillary Acidic Protein/immunology/metabolism, Intercellular Junctions/ultrastructure, Mitochondria/pathology/*ultrastructure, Neoplasm Recurrence, Local/pathology/radiotherapy, S100 Proteins/immunology/metabolism",
author = "Istvan Vajtai and {von Gunten}, Michael and Christian Fung and Caspar Brekenfeld and Andreas Kappeler and Reinert, {Michael M}",
year = "2011",
language = "English",
volume = "207",
pages = "49--54",
journal = "PATHOL RES PRACT",
issn = "0344-0338",
publisher = "Urban und Fischer Verlag GmbH und Co. KG",
number = "1",

}

RIS

TY - JOUR

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 -