Brainstem glioma in PHACES syndrome: case report and review of the literature.

摘要

The PHACES syndrome is a neurocutane-ous 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 [2j. Here, we present the case of a 16-year-old adolescent boy with PHACES syndrome who was found to have a brain-stem 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].