Pleomorphic xanthoastrocytoma inside lateral ventricle: a rare case report and literature review

Pleomorphic xanthoastrocytoma (PXA) is a relatively rare, low grade astrocytic tumor that usually affects children as well as young adults. The reported cases were predominantly located superficially in the temporal lobe. To our knowledge, so far only two cases of PXA occurring in lateral ventricle were reported in English literature. Herein, we present the third case of PXA intra-lateral ventricle in a 28-year-old Chinese male. Histologically, the tumor was relatively well circumscribed and consisted of spindle-shaped, ovoid, and multinuclear giant cells admixed with scattered eosinophilic granular bodies, inflammatory cells, and xanthomatous cells. Immunohistochemically, the tumor cells were strongly positive for S-100, GFAP, oligo-2 and vimentin, focally positive for synaptophysin and CD34, and negative for cytokeratin, EMA, NeuN and IDH1. Ki-67 proliferation index was approximately 2%. A BRAF V600E mutation was then identified in the tumor. Based on morphologic features, the immunohistochemical staining and BRAF V600E mutation, the tumor was diagnosed as a PXA. Because of the presence of the bizarre multinuclear giant cells and xanthomatous cells and the unusual location, PXA was easily misdiagnosed as a high-grade tumor. It should be noted that PXA was also an important differential diagnosis for intraventricular tumors.