Grant J W, Steart P V, Gallagher P J
Department of Pathology, Southampton University General Hospital.
Clin Neuropathol. 1988 Sep-Oct;7(5):228-33.
Four males and 6 females, 5-66 (mean 27) years, with supratentorial primitive neuroectodermal tumors (PNETs) have been treated in one center in a 5-year period. Symptoms and signs had been present from 4 days to 8 years and were highly variable. The frontal hemisphere was involved in 6 cases. Seven patients have died but with a combination of surgery and radiotherapy 3 patients have survived 20 months or longer. All tumors were composed of rounded, hyperchromatic undifferentiated cells but there were focal areas of astrocytic (5 cases), mesenchymal (4 cases) or neuronal differentiation (1 case). In 9 of the 10 cases a proportion of the undifferentiated round cells stained with antibodies to GFAP. Seven of these also showed nuclear staining for S100 protein and 6 of these, plus the tumor that did not stain for GFAP, had cytoplasmic staining for neuron specific enolase. Six tumors stained with antibodies to vimentin but there was no staining for common leukocyte antibody, cytokeratin or myoglobin. These results, in particular those with GFAP, indicate that PNETs undergo differentiation even in areas of apparent morphological uniformity. Furthermore, immunohistochemistry is useful in distinguishing PNETs from other "small blue cell tumors" such as malignant lymphoma and undifferentiated secondary carcinoma.
在5年期间，一个中心共治疗了4名男性和6名女性，年龄在5至66岁（平均27岁），患有幕上原始神经外胚层肿瘤（PNETs）。症状和体征出现的时间从4天到8年不等，且差异很大。6例累及额叶。7例患者死亡，但通过手术和放疗相结合，3例患者存活了20个月或更长时间。所有肿瘤均由圆形、深染的未分化细胞组成，但存在星形细胞分化灶（5例）、间充质分化灶（4例）或神经元分化灶（1例）。在10例病例中的9例中，一部分未分化的圆形细胞用抗GFAP抗体染色。其中7例还显示S100蛋白核染色，其中6例以及未用GFAP染色的肿瘤，神经元特异性烯醇化酶呈细胞质染色。6例肿瘤用抗波形蛋白抗体染色，但普通白细胞抗体、细胞角蛋白或肌红蛋白无染色。这些结果，特别是GFAP的结果，表明即使在形态学上明显均匀的区域中，PNETs也会发生分化。此外，免疫组织化学有助于将PNETs与其他“小蓝细胞肿瘤”，如恶性淋巴瘤和未分化的继发性癌区分开来。
