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Massive Intracranial Extracerebral Glioneuronal Heterotopia with Parapharyngeal Extension
Author(s) -
Rodriguez Michelle Evon,
Baumgartner James,
Covinsky Michael,
Hochhauser Leo,
Fuller Gregory N.,
Wang Min
Publication year - 2007
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.21.5.a402
Subject(s) - heterotopia (medicine) , pathology , choroid plexus , synaptophysin , medicine , anatomy , immunohistochemistry , central nervous system , endocrinology
Intracranial extracerebral glioneuronal heterotopias are rare pediatric lesions. We report a case with follow up throughout a two‐year period. This female child was diagnosed in utero at 30 weeks gestational age with an intracranial extracerebral mass that deformed the intracranial structures. Postnatal radiological studies revealed a large dumbbell‐shaped right intracranial mass with parapharyngeal extension. Pre‐operative biopsies and eventual tumor debulking revealed disorganized glioneuronal tissue with focal hypercellularity. There was a focal area with a high MIB‐1 (Ki‐67) labeling index and scattered mitoses. The majority of proliferating cells were positive for glial fibrillary acidic protein, with areas of synaptophysin positivity. An area of choroid plexus‐like tissue that was positive for synaptophysin, CK7 and prealbumin was also observed in the heterotopia. This extraordinary case supports the hypothesis of mis‐separation or mis‐detachment of neuroectodermal cells during embryogenesis as the pathogenetic basis of intracranial extracerebral glioneuronal heterotopias.