Nonanaplastic pleomorphic xanthoastrocytoma with meningeal dissemination presenting with bilateral visual loss

Pleomorphic xanthoastrocytoma (PXA) is a brain neoplasm included in the astrocytic group, exceptionally manifesting with meningeal dissemination. We described a 27-year-old patient presented with acute bilateral visual loss and papilledema with normal brain computed tomography scan, initially mimicking idiopathic intracranial hypertension (IIH). Brain and spinal cord magnetic resonance imaging (MRI) study revealed a subtle area of hyperintensity of the gyri surrounding the left central sulcus, and contrast enhancement of the thoracic leptomeninges. Brain biopsy of the parietal lesion revealed nonanaplastic PXA. Treatment with temozolomide was given. Yearly control MRI demonstrated new brain lesions and marked progression of leptomeningeal spinal enhancement. In spite of this, the patient has remained stable with no new symptoms. Nonanaplastic PXA may present with widespread meningeal dissemination with acute visual loss and papilledema mimicking IIH, and no clinical progression at 3 years.