Synchronous multicentric pleomorphic xanthoastrocytoma with anaplastic features

Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumor with a relatively favorable prognosis. However, PXA cases having several recurrent patterns with poor prognosis have been reported in recent years, and a new concept of "PXA with anaplastic features" has been proposed. The present case was of a 55-year-old male who presented with weakness and numbness of right upper and lower limbs since 3 months along with difficulty in walking as well as difficulty in speaking since then. He also complained of headache since 9 months. The magnetic resonance imaging study revealed two nodular, homogeneously enhancing lesions, approximately 1 cm in size in the right cerebral hemisphere. Clinical and radiological examinations were suggestive of a metastatic neoplasm. A right frontal craniotomy was performed for excisional biopsy of both lesions. Histopathological findings showed that the tumor was PXA with strong pleomorphism, xanthomatous changes, extensive areas of tumor necrosis, and increased mitotic activity. From these findings, the histopathological diagnosis "PXA with anaplastic features" was given. Synchronous multicentric PXA presents unique challenges in that gross total resection would impose significant surgical morbidity; histological homogeneity among the lesions cannot be confirmed; and the well-described potential for anaplastic transformation may be increased with multiple lesions. The optimal treatment for patients with this rare and challenging diagnosis awaits further study.