Clinical presentation, immunohistochemistry and electron microscopy indicate neurofibromatosis type 2-associated gliomas to be spinal ependymomas.
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Clinical presentation, immunohistochemistry and electron microscopy indicate neurofibromatosis type 2-associated gliomas to be spinal ependymomas. / Hagel, Christian; Stemmer-Rachamimov, Anat O; Bornemann, Antje; Schuhmann, Martin; Nagel, Christoph; Huson, Susan; Evans, D Gareth; Plotkin, Scott; Matthies, Cordula; Kluwe, Lan; Mautner, Viktor Felix.
in: NEUROPATHOLOGY, Jahrgang 32, Nr. 6, 6, 2012, S. 611-616.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Clinical presentation, immunohistochemistry and electron microscopy indicate neurofibromatosis type 2-associated gliomas to be spinal ependymomas.
AU - Hagel, Christian
AU - Stemmer-Rachamimov, Anat O
AU - Bornemann, Antje
AU - Schuhmann, Martin
AU - Nagel, Christoph
AU - Huson, Susan
AU - Evans, D Gareth
AU - Plotkin, Scott
AU - Matthies, Cordula
AU - Kluwe, Lan
AU - Mautner, Viktor Felix
PY - 2012
Y1 - 2012
N2 - Neurofibromatosis type 2 (NF2) is a hereditary tumor syndrome. The hallmark of NF2 is bilateral vestibular schwannoma. In addition, glioma is one of the diagnostic criteria of NF2. In this retrospective study the clinical presentation and histopathological features of 12 spinal gliomas from NF2 patients were assessed. Ten tumors were previously diagnosed as ependymomas and two as astrocytomas. However, upon re-evaluation both astrocytomas expressed epithelial membrane antigen in a dot-like fashion and in one case it was possible to perform electron microscopy revealing junctional complexes and cilia typical for ependymoma. The findings suggest that NF2-associated spinal gliomas are ependymomas. Based on the fact that NF2-associated gliomas are almost exclusively spinal and that no NF2 mutations have been found in sporadic cerebral gliomas, we suggest that "glioma" in the current diagnostic criteria for NF2 should be specified as "spinal ependymoma".
AB - Neurofibromatosis type 2 (NF2) is a hereditary tumor syndrome. The hallmark of NF2 is bilateral vestibular schwannoma. In addition, glioma is one of the diagnostic criteria of NF2. In this retrospective study the clinical presentation and histopathological features of 12 spinal gliomas from NF2 patients were assessed. Ten tumors were previously diagnosed as ependymomas and two as astrocytomas. However, upon re-evaluation both astrocytomas expressed epithelial membrane antigen in a dot-like fashion and in one case it was possible to perform electron microscopy revealing junctional complexes and cilia typical for ependymoma. The findings suggest that NF2-associated spinal gliomas are ependymomas. Based on the fact that NF2-associated gliomas are almost exclusively spinal and that no NF2 mutations have been found in sporadic cerebral gliomas, we suggest that "glioma" in the current diagnostic criteria for NF2 should be specified as "spinal ependymoma".
KW - Adult
KW - Diagnosis, Differential
KW - Humans
KW - Male
KW - Female
KW - Middle Aged
KW - Adolescent
KW - Young Adult
KW - Child
KW - Immunohistochemistry/methods
KW - Ependymoma/ultrastructure
KW - Microscopy, Electron/methods
KW - Neurofibromatosis 2/diagnosis/genetics/pathology
KW - Spinal Cord Neoplasms/ultrastructure
KW - Adult
KW - Diagnosis, Differential
KW - Humans
KW - Male
KW - Female
KW - Middle Aged
KW - Adolescent
KW - Young Adult
KW - Child
KW - Immunohistochemistry/methods
KW - Ependymoma/ultrastructure
KW - Microscopy, Electron/methods
KW - Neurofibromatosis 2/diagnosis/genetics/pathology
KW - Spinal Cord Neoplasms/ultrastructure
M3 - SCORING: Journal article
VL - 32
SP - 611
EP - 616
JO - NEUROPATHOLOGY
JF - NEUROPATHOLOGY
SN - 0919-6544
IS - 6
M1 - 6
ER -
