Pleomorphic xanthoastrocytoma. Ultrastructural and immunohistochemical study of a case with a rapidly fatal outcome following surgery

Abstract

In 1979, researchers described a series of young patients with clinically and histologically distinctive supratentorial gliomas which were designated pleomorphic (meningocerebral) xanthoastrocytomas (PXA). Significantly, patients with these neoplasms were reported to have a relatively favorable prognosis. The authors present a new case of PXA in a 32‐year‐old man. This case is unique for two reasons: (1) a relatively rapid fatal outcome with death 21 months after diagnosis; and (2) the presence, at autopsy, of extensive recurrent tumor with features of a malignant astrocytoma. Detailed electron microscopic and immunohistochemical studies, supporting the proposed subpial astrocytic origin of PXA, are presented. Literature pertaining to PXA is reviewed. This report illustrates the unique features of PXA and demonstrates its potential for aggressive behavior. Cancer 52:2055‐2063, 1983.

Intraventricular pleomorphic xanthoastrocytoma: a case report

“…In contrast to mesenchimal tumors and giant cell glioblastomas, PXAs are associated with an intermediate prognosis. Malignant progression of PXAs, although rare, has been documented, with mitotic index and extent of resection as main predictors of outcome (10,16,19,24,36,37). PXA typically presents as a well-demarcated solid-cystic mass involving the superficial temporal lobe, with intense contrast enhancement of the solid portion of tumor in most imaging modalities (13,39).…”

Massive Intracranial Hemorrhage Associated With Pleomorphic Xanthoastrocytoma -Case Report-

“…Pleomorphic xanthoastrocytoma is well known for the presence of prominent basal lamina surrounding the tumor cells, which, in addition to its tendency to involve the leptomeninges, suggests subpial astrocytes as the possible origin of this rare tumor. 8,18,19,21) Invasion of vessel walls has not been recognized as a clinicopathological feature of pleomorphic xanthoastrocytoma, presumably because this tumor mostly involves the cerebral cortex and is usually not located in the proximity of major vessels. The present case is an important example of this interesting phenomenon, which may be helpful in the management of pleomorphic xanthoastrocytoma.…”

Malignant transformation of pleomorphic xanthoastrocytoma: case report

“…However, in cases proven to be fatal, they usually have undergone transition to anaplastic astrocytoma or glioblastoma. Recurrences may show a histological pattern analogous to the original tumor, but increasing anaplasia may also be set [4][5][6][7][8][9][10][11] . Pleomorphism may cease to be a feature and closely packed smaller cells may come to dominate the tumor.…”

“…Our case is in accordance with these observations. The patient presented a typical PXA that recurred as glioblastoma five months later, comparable to the cases reported by Kepes et al 3,4 , WeldonLinne et al 11 and one case described by Tonn et al 10 .…”