Kepes J J, Rubinstein L J, Ansbacher L, Schreiber D J
Department of Pathology and Oncology, University of Kansas College of Health Sciences and Hospital, Kansas City 66103.
Acta Neuropathol. 1989;78(6):585-93. doi: 10.1007/BF00691285.
Pleomorphic xanthoastrocytoma (PXA), a tumor most often presenting superficially over the cerebral hemisphere of young subjects, has certain morphological similarities to fibrous histiocytoma (or fibrous xanthoma) of the meninges and brain, namely the occurrence of lipid-laden neoplastic cells and, frequently, a dense reticulin fiber network. The detection of glial fibrillary acidic (GFA) protein in the tumor cells helped to establish its astrocytic derivation, but it has been advanced that, in spite of this agreed observation, the tumor should still be regarded as a fibrous xanthoma of meningeal origin. Although many patients have a long symptom-free postoperative survival, local recurrences at varying intervals after surgery have been noted in some instances. Weldon-Linne et al. first reported that such a recurrence had the morphology of a small-cell glioblastoma. We are reporting three further examples of locally recurrent neoplasms in patients whose original meningocerebral tumors had the typical features of PXA; the recurrences (developing 7 months, 7 years and 15 years, respectively, after surgery) were small-cell glioblastomas. The rich reticulin network present in the initial tumor was mostly lost in the recurrences. This anaplastic evolution further confirms the astrocytic nature of the PXA.
多形性黄色星形细胞瘤(PXA)是一种最常出现在年轻患者大脑半球表面的肿瘤,它在形态上与脑膜和脑的纤维组织细胞瘤(或纤维黄色瘤)有某些相似之处,即存在富含脂质的肿瘤细胞,且常常有密集的网状纤维网络。肿瘤细胞中胶质纤维酸性(GFA)蛋白的检测有助于确定其星形细胞来源,但有人提出,尽管有这一公认的观察结果,该肿瘤仍应被视为起源于脑膜的纤维黄色瘤。尽管许多患者术后有较长时间的无病生存期,但在某些情况下,术后不同时间间隔出现了局部复发。韦尔登 - 林内等人首先报道,这种复发具有小细胞胶质母细胞瘤的形态。我们报告了另外三例原发性脑膜脑肿瘤具有PXA典型特征的患者局部复发肿瘤的病例;复发肿瘤(分别在术后7个月、7年和15年出现)均为小细胞胶质母细胞瘤。最初肿瘤中丰富的网状纤维网络在复发肿瘤中大多消失。这种间变演化进一步证实了PXA的星形细胞性质。