A pediatric case of anaplastic pleomorphic xanthoastrocytoma (Grade 3)

Abstract

Pleomorphic xanthoastrocytoma (PXA) is a glial tumor which accounts for less than 1% of astrocytomas. PXA most commonly occurs in the temporal lobe in the outer cortex of brain and usually seen in young adults and children. As per 2020 WHO classification of CNS tumors, PXA with a mitotic activity ≥5 mitoses/10 HPF is defined as anaplastic pleomorphic xanthoastrocytoma (APXA), WHO grade 3. Here we present a rare case of Anaplastic Pleomorphic Xanthoastrocytoma developing in a female child of 11 years of age.A 11 years old female child presented with symptoms of continuous headache, giddiness, fever and vomiting since last 15 days. Computed Tomography of her brain revealed a soft tissue density mass lesion in right temporal lobe involving the insular cortex and capsuloganglionic region. The radiological differential diagnosis was a neoplastic lesion, most likely glioma. The patient underwent surgery for removal of temporal lobe tumor and the resected tumor bits were sent for histopathological assessment. Microscopically, many multinucleated lipidized giant cells were seen having eccentrically pushed nuclei, conspicuous nucleoli and abundant eosinophilic granular cytoplasm. A second population of few spindled cells with less amount of cytoplasm was also noted in the intervening parenchyma. Immunohistochemical workup showed the tumor to be positive for GFAP, S-100, Synaptophysin, p53 and CD-68. The tumor was negative for CD34. Ki-67 index was 15-20%. A final diagnosis of Anaplastic Pleomorphic Xanthoastrocytoma (grade 3) was made.APXA is a rare astrocytic tumor and has relatively more aggressive radiological and histomorphological features than pleomorphic xanthoastrocytomas and therefore it needs to be identified and treated separately.