Intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligodendroglioma-like features
Intramedullary glioma are rare and their biological behaviour can differ from their cerebral counterparts. Pilomyxoid astrocytoma (PMA, WHO grade II), predominantly occur in the hypothalamic/chiasmatic region of infants and children. The few reported cases of pediatric intramedullary PMA displayed a...
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doaj-ced129ffcdc5483090394d46357efb9f2020-11-25T03:48:09ZengSAGE PublishingRare Tumors2036-36052036-36132012-05-0142e30e3010.4081/rt.2012.e302151Intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligodendroglioma-like featuresSabina Eigenbrod0Niklas Thon1Nathalie Jansen2Hendrik Janssen3Janina Mielke4Michael Ruiter5Christian la Fougère6Aurelia Peraud7Rupert Egensperger8Hans Kretzschmar9Center for Neuropathology, Ludwig-Maximilians-University, MunichDepartment of Neurosurgery, Ludwig-Maximilians-University, MunichDepartment of Nuclear Medicine, Ludwig-Maximilians-University, MunichDepartment of Clinical Radiology, Ludwig-Maximilians-University, MunichCenter for Neuropathology, Ludwig-Maximilians-University, MunichCenter for Neuropathology, Ludwig-Maximilians-University, MunichDepartment of Nuclear Medicine, Ludwig-Maximilians-University, MunichDepartment of Neurosurgery, Ludwig-Maximilians-University, MunichCenter for Neuropathology, Ludwig-Maximilians-University, MunichCenter for Neuropathology, Ludwig-Maximilians-University, MunichIntramedullary glioma are rare and their biological behaviour can differ from their cerebral counterparts. Pilomyxoid astrocytoma (PMA, WHO grade II), predominantly occur in the hypothalamic/chiasmatic region of infants and children. The few reported cases of pediatric intramedullary PMA displayed a particularly aggressive behavior. Here, we report a diagnostically challenging case of a five year old female patient presenting with intramedullary glioma and local tumor recurrence three years later. Twelve years after the initial manifestation, a second tumor was found intracerebrally. We performed a comprehensive histological, molecular pathological and imaging analysis of the tumors from both localizations. The results revealed a metastasizing PMA with unique histological and genetic features. Our study indicates that PMA comprise a heterogeneous group including aggressive subtypes which may not be compatible with the current classification according to WHO grade II. Furthermore, the case emphasizes the increasing relevance of molecular pathological markers complementing classic histological diagnosis.http://www.pagepress.org/journals/index.php/rt/article/view/4063Intramedullary gliomapilomyxoid astrocytomametastasis1p19q |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Sabina Eigenbrod Niklas Thon Nathalie Jansen Hendrik Janssen Janina Mielke Michael Ruiter Christian la Fougère Aurelia Peraud Rupert Egensperger Hans Kretzschmar |
spellingShingle |
Sabina Eigenbrod Niklas Thon Nathalie Jansen Hendrik Janssen Janina Mielke Michael Ruiter Christian la Fougère Aurelia Peraud Rupert Egensperger Hans Kretzschmar Intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligodendroglioma-like features Rare Tumors Intramedullary glioma pilomyxoid astrocytoma metastasis 1p19q |
author_facet |
Sabina Eigenbrod Niklas Thon Nathalie Jansen Hendrik Janssen Janina Mielke Michael Ruiter Christian la Fougère Aurelia Peraud Rupert Egensperger Hans Kretzschmar |
author_sort |
Sabina Eigenbrod |
title |
Intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligodendroglioma-like features |
title_short |
Intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligodendroglioma-like features |
title_full |
Intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligodendroglioma-like features |
title_fullStr |
Intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligodendroglioma-like features |
title_full_unstemmed |
Intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligodendroglioma-like features |
title_sort |
intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligodendroglioma-like features |
publisher |
SAGE Publishing |
series |
Rare Tumors |
issn |
2036-3605 2036-3613 |
publishDate |
2012-05-01 |
description |
Intramedullary glioma are rare and their biological behaviour can differ from their cerebral counterparts. Pilomyxoid astrocytoma (PMA, WHO grade II), predominantly occur in the hypothalamic/chiasmatic region of infants and children. The few reported cases of pediatric intramedullary PMA displayed a particularly aggressive behavior. Here, we report a diagnostically challenging case of a five year old female patient presenting with intramedullary glioma and local tumor recurrence three years later. Twelve years after the initial manifestation, a second tumor was found intracerebrally. We performed a comprehensive histological, molecular pathological and imaging analysis of the tumors from both localizations. The results revealed a metastasizing PMA with unique histological and genetic features. Our study indicates that PMA comprise a heterogeneous group including aggressive subtypes which may not be compatible with the current classification according to WHO grade II. Furthermore, the case emphasizes the increasing relevance of molecular pathological markers complementing classic histological diagnosis. |
topic |
Intramedullary glioma pilomyxoid astrocytoma metastasis 1p19q |
url |
http://www.pagepress.org/journals/index.php/rt/article/view/4063 |
work_keys_str_mv |
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