Brainstem Glioma in PHACES Syndrome: Case Report and Review of the Literature

Introduction

The PHACES syndrome is a neurocutaneous disorder whose acronym describes a constellation of congenital abnormalities including posterior fossa malformations, hemangiomas of the face and scalp, arterial malformations, coarctation of the aorta with other cerebrovascular and cardiac defects, eye anomalies, as well as sternal clefting or superumbilical raphe. Numerous posterior fossa anomalies associated with PHACES, such as Dandy-Walker malformation, have been previously described in the literature[1]. Recently, a case of cerebellar pilocytic astrocytoma in association with PHACES was described, introducing the possibility that neoplasms may occur in addition to the other posterior fossa malformations[2]. Here, we present the case of a 16-year-old adolescent boy with PHACES syndrome who was found to have a brainstem glioma. The patient had been treated at our neurosurgical center three years earlier for a giant fusiform internal carotid artery (ICA) aneurysm ipsilateral to the side of his facial hemangioma and was the subject of a previous case report[3]. Review of the literature revealed only two previous cases of gliomas[2] [4] in association with this condition. We believe this is the first case of brainstem glioma associated with PHACES syndrome and that it provides further evidence for an association between neoplasms and PHACES syndrome.

References

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