Lateral ventricle pleomorphic xanthoastrocytoma concurrent with Dandy-Walker complex: A case report

Abstract

Introduction: Dandy-Walker complex and pleomorphic xanthoastrocytomas are both rare disease entities that typically manifest early in life and are associated with congenital etiological factors. Dandy-Walker complex is a cerebellar malformation associated with a series of anatomical changes. The disease onset is usually at birth or during infancy. Late onset in adulthood is uncommon. Pleomorphic xanthoastrocytoma is a rare WHO grade II astrocytic tumor affecting mainly young adults. Concomitant occurrence of Dandy-Walker complex and pleomorphic xanthoastrocytoma has not been previously reported.Patient concerns and diagnosis: A 30-year-old woman with a previous history of unconfirmed resected lateral ventricle meningioma presented with severe headache for 1 day. Imaging examination revealed a mass in the right lateral ventricle with heterogeneous signal patterns, changes in the posterior fossa corresponding to a Dandy-Walker variant, and mild hydrocephalus.Interventions and outcomes: Surgical complete resection of the mass was achieved. postoperative histopathological examination confirmed WHO grade II pleomorphic xanthoastrocytoma. Three years postsurgery, ventriculoperitoneal shunt was performed due to worsening of hydrocephalus. The patient has since remained symptom-free.Conclusion: This is the first report of concomitant occurrence of Dandy-Walker complex and pleomorphic xanthoastrocytoma. The association of neurological congenital malformation with intracranial neoplasms may be multifactorial, with underlying role of genetic mutations or chromosome alterations.

A Late Diagnosis of Pilocytic Astrocytoma in a Dandy-Walker Complex: Case Report

“…Astrocytic tumors are produced from neuroepithelial tissue and are classi ed as grade I due to their wellcircumscribed and sluggish growth. They can, however, be located anywhere along the neuraxis [4]. A particular etiology can be discovered in a limited percentage of cases; documented etiologies include chromosome 3, 9, 13, and 18 abnormalities and mutations of the FOXC1, ZIC1, and ZIC4 genes [5][6].…”