Primary cerebellopontine angle craniopharyngioma in a patient with Gardner syndrome

Case report and review of the literature

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✓The authors report on the case of a craniopharyngioma arising in the cerebellopontine angle (CPA) in a patient with Gardner syndrome. Although familial adenomatous polyposis (FAP) is associated with intracranial neoplasms, the current case is only the third reported craniopharyngioma in a patient with Gardner syndrome. Two of these tumors, including that of the current case, originated in the CPA, an unusual location for craniopharyngiomas. The literature concerning FAP and its associations with intracranial neoplasia, as well as the pathogenesis of craniopharyngiomas in the posterior fossa, is discussed.

Abbreviations used in this paper:CNS = central nervous system; CPA = cerebellopontine angle; FAP = familial adenomatous polyposis; MR = magnetic resonance.

Article Information

Address reprint requests to: Kristian Aquilina, F.R.C.S., Academic Centre Room 1, Frenchay Hospital, Frenchay Park Road, Bristol, BS16 1LE, United Kingdom. email: kristianaquilina@hotmail.com.

© AANS, except where prohibited by US copyright law.

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Figures

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    Postcontrast axial MR images through the upper (A) and lower (B) posterior fossa demonstrating a cystic, peripherally enhancing tumor arising from the right CPA (arrows); one of the subcutaneous fibromas is visible in the right preauricular region (arrowhead).

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    Postcontrast midsagittal MR image demonstrating the craniopharyngioma extending inferiorly to the foramen magnum (arrow).

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    Photomicrograph showing tumor tissue with features consistent with the diagnosis of adamantinomatous craniopharyngioma. H & E, original magnification × 100.

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    A: Photograph of the resected subcutaneous collagenoma from the left occipital region. B: Photomicrograph of the fibroma showing a relatively acellular connective tissue stroma consisting of thick collagen fibers. H & E, original magnification × 100.

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