Associated vascular malformations and neoplasms in children

Primary CNS neoplasms can rarely be associated with abnormal vascularity which is more striking than usual hypervascularity or neovascularization. These lesions are sometimes reported as angiogliomas, angiomatous astrocytomas, or descriptively as two distinct pathological entities. This vascular pattern has been reported with astrocytomas, oligodendrogliomas, neurilemmomas, meningiomas, pituitary adenomas, gangliogliomas, hemangioblastomas, mixed gliomas, gliosarcomas, and craniopharyngiomas. These lesions are classically found to be extremely vascular, both radiographically and at surgery. The true nature of these lesions is a point of controversy with some interpreting them merely as intensely vascularized tumors. Most agree, however, that unlike neovascularity in astrocytomas, the vascularity in these lesions does not carry any significant prognostic importance. In this report we present six such cases from The Children's Hospital of Philadelphia seen in the pediatric population. Four patients had vascular malformations associated with diffuse astrocytomas, and in 2 patients they were associated with subependymal giant cell astrocytomas. Five of the lesions were supratentorial, and 1 was located in the posterior fossa. The history and theories surrounding these lesions are explored.