Pleomorphic xanthoastrocytoma with anaplastic features: A rare case report and review of literature with reference to current management

Abstract

Pleomorphic xanthoastrocytoma (PXA) is an uncommon tumor constitutes less than 1% of all astrocytic glial neoplasms was first reported in 1979. PXA commonly occurs in young patients and manifests itself first as seizures followed by focal neurological deficits. The role of radiotherapy or chemotherapy has not yet been established because of the relative infrequency of this disease. PXA is classified as grade II tumor in the WHO classification of tumors of the CNS. In literature 9 to 20 % PXA may undergo malignant change at recurrence or may display at the time of initial presentation. Malignant transformation is mainly associated with high mitotic activity and necrosis. The criteria for PXA with anaplastic features was five or more mitotic activity per 10 high power fields, necrosis, microvascular proliferation, marked cellular anaplasia, and high Ki-67 labeling indices. PXA with anaplastic features management is highly controversial as very sparse literature is available. We are reporting a case of PXA with anaplastic features with atypical radiology and tried to review the up to date literature regarding this rare tumor.

Anaplastic Pleomorphic Xanthoastrocytoma Presenting with Musical Hallucination

“…Most notable findings on histological examination of APXA are pleomorphic, xanthomatous tumour cells with intracytoplasmic lipid vacuoles. Multinucleated giant cells, increased mitotic activity, hyperchromatism, cell atypia, nuclear irregularities, and necrosis may be seen [ 14 ]. Absence of palisading necrosis and endothelial microvascular proliferation enables pathologist distinguish these tumours from majority of glioblastomas [ 15 ].…”

“…Due to the rarity of this tumour and dearth of literature a standard management guideline has not been established. Standard surgical excision followed by radiotherapy or chemotherapy including new alkylating agents such as temozolomide has not been successful as noted with PXA [ 14 , 22 ]. Temozolomide and BRAF inhibitor vemurafenib are effective in PXA irrespective of the V600E tumour status [ 23 ].…”

“…Temozolomide and BRAF inhibitor vemurafenib are effective in PXA irrespective of the V600E tumour status [ 23 ]. Gross total resection remains the goal of treatment although reports of improved survival have been documented with stereotactic radiosurgery [ 14 , 22 ]. This article emphasizes the need to always consider organic lesions in the differential diagnosis of patients with auditory hallucinations.…”

Pleomorphic Xanthoastrocytoma With Anaplastic Features- A Rare Case Report and Review of Literature

“…Eighteen cases of PXA showing anaplastic features at the first presentation have been reported in the literature till now. [6,9,10,11,12] In Gianni et al study the significance of the mitotic index, the presence of necrosis and the extent of resection were analysed and found that the mitotic index and the extent of resection were the main predictors for recurrence free survival and overall survival rates. [2] Data to support the role of adjuvant treatment are scanty and sparse.…”

Clinico-Histomorphological and Immunohistochemical Profile of Anaplastic Pleomorphic Xanthoastrocytoma: Report of Five Cases and Review of Literature

“…anaplastic transformation in recurrent conventional PXA 4 . APXA has a poor prognosis and rarely can undergo transformation to glioblastoma 5 . All the cases of APXA were evaluated for their clinicoradiological, histomorphological and immunohistochemical (IHC) findings.…”