Rare-36. pleomorphic xanthoastrocytoma with anaplastic features: a case report

Pleomorphic xanthoastrocytoma (PXA) is a rare WHO Grade II neoplasm that often presents with seizures in children and young adults and has a supratentorial, typically temporal, predilection. The latest WHO classification includes a rare subtype with anaplastic features. These tumors demonstrate high mitotic activity of > 5 mitosis per 10 high powered field, focal positivity of CD-34, synaptophysin, MIB-1 labeling index, and P53 reactivity. Histologically, they exhibit spindle cells admixed with multinucleated giant cells showing lipidized vacuolated cytoplasm, intranuclear cytoplasmic inclusion, and eosinophilic granular bodies. Focal areas of necrosis are often seen. They are thought to arise from a malignant transformation of lower grade PXA, and the underlying molecular events behind this are not understood, though BRAF may play a role. Prognosis is significantly poorer than in the lower grade variants. There have been fewer than 11 cases reported in literature and thus a consensus on optimal treatment has not been reached. Herein, we present a case of PXA with anaplastic features in a 37 year-old woman and discuss her clinical course along with treatment, which included Temozolomide, Bevacizumab, and Optune Tumor Treating Fields.
Source: Neuro-Oncology - Category: Cancer & Oncology Authors: Tags: RARE TUMORS Source Type: research