Pleomorphic Xanthoastrocytoma

Abstract

Pleomorphic xanthoastrocytoma is a rare, but clinically and histologically distinctive supratentorial glioma in young patients. Such a case, occurring in the right frontal lobe, is presented. The patient, an 8-year-old girl, had been well until February 1984 when she developed left facial spasm. Neurological examination on admission in May 1984 revealed left facial palsy with frequent facial spasm. Computed tomography (CT) demonstrated a small right frontal mass associated with a large area of low density in the surrounding brain. A right fronto-temporal craniotomy revealed a firm and solid tumor, 3 cm in diameter, involving the overlying leptomeninges. The tumor was well demarcated and was totally removed. Histologically the tumor showed marked cellular pleomorphism and a dense network of collagen and reticulin fibers, but there was no mitosis or necrosis. Some of the tumor cells contained lipid droplets. Glial fibrillary acidic protein was demonstrated in the cytoplasm of many tumor cells by immunoperoxydase study. The patient has been well without any neurological deficits postoperatively. CT showed no evidence of recurrence. Twenty cases of pleomorphic xanthoastrocytoma have been reported in the literature. The clinical features are distinctive, for instance, preponderance in young patients, good prognosis, superficial location of the tumor with marked pleomorphism in pathology. However, late recurrence, mostly with malignant change occurred in 6 of the 20 cases. It is suggested that long term follow-up is necessary.