[Pleomorphic xanthoastrocytoma. Apropos of 3 new cases. Review of the literature].

Three cases of pleomorphic xanthoastrocytomas (P.X.A.), a low grade leptomeningeal glioma are reported. Prominent histological features used for diagnosis were a cellular pleomorphism of G.F.A.P. positive cells, with intracytoplasmic lipidic vacuols. A reticulinic network and mononuclear cells infiltrates have been observed. A weak mitotic activity and lack of necrosis and of endothelial cells proliferation were significant additional features necessary for diagnosis. Our cases were observed during the surgical management of young patients with resistant epilepsy. Neuroradiological examinations showed a tumor superficially located within the temporal or the parietal lobe. This tumor could be calcified and/or cystic. Operative aspects showed a firm and non-encapsulated leptomeningeal tumor with possible various colors. Our patients were seizures-free after surgery even during the follow-up. From the currently reported cases clinical follow-up ranging for 1.5 to 3 years is not sufficient to predict a favorable carcinologic prognosis. P.X.A. is an uncommon tumor and less than 50 cases are reported throughout the literature. This tumor affecting young subjects mainly during the second decade is revealed in the majority of cases (3/4) by epileptic seizures, less frequently by a deficit or by an intracranial hypertension. The great majority of clinical events are observed before 20 years. The functional prognosis is rather good after surgery with a disappearance of epileptic fits in about 50% of the cases. Throughout the literature the prognosis of this tumor seems to be comparable to low-grade astrocytomas. Optimal management of P.X.A. seems to be primary surgical resection with later surgery for residual or recurrent tumor. The role of radiotherapy in the management of P.X.A. is at this time uncertain.