摘要
目的 探讨外周性原始神经外胚层瘤 (PPNET)和尤文肉瘤 (ES)的临床病理、免疫组化特点及诊断标准。方法 用HE染色、PAS染色和免疫组化方法对 19例PPNET和ES进行观察 ,3例标本做电镜检查。结果 12例PPNET ,年龄 2～ 11岁 ,平均 6 5岁 ;男 8例 ,女 4例 ,病变位于四肢 5例、胸壁 3例 ,脊柱旁 3例和腹膜后 1例。 7例ES ,年龄 4～ 10岁 ,平均 6 3岁 ;男 3例 ,女 4例 ,病变位于股骨 3例 ,肩胛骨 3例 ,左小腿 1例。镜检 :PPNET瘤细胞小 ,排列呈实性片状、分叶状、腺泡状和索条状 ,12例均见Homer Wright菊形团 ;ES瘤细胞形态与排列和PPNET相似 ,但胞浆透明 ,未见Homer Wright菊形团。免疫组化 :PPNET和ES均表达 12E7,每例PPNET都表达 2项以上神经标志 ,而ES仅表达 1项。结论 PPNET和ES为儿童常见的小细胞恶性肿瘤 ,Homer
Objective To investigate the pathological and immunohistochemical features, and diagnostic criteria of peripheral primitive neuroectodermal tumor (PPNET) and Ewing's sarcoma (ES). Methods HE and PAS stains and immunohistochemistry were used to analyze 12 cases of PPNET and 7 ES, in which 3 cases were studied with electron microscope. Results Range of age in 12 patients with PPNET was from 2 to 11 years old ( mean 6.5), with 8 male and 4 female. Location of the tumor was in the extremities (5 cases), chest wall (3 cases), paraspinal regions (3 cases) and retroperitoneum (1 case). Of the 7 cases of ES, age range was from 4 to 10 years old, with 3 male and 4 female, and, 3 tumors were located in the femur, 3 in the scapula and 1 in the leg. Microscopically, both PPNET and ES were composed of small cells, arranged in solid sheet, lobular, alveolar or corded structures. Homer-Wright rosette was seen in all cases of PPNET, but not in ES. Immunohistochemically, 12E7 was expressed in all cases of both PPNET and ES. Two or more neurogenic markers were demonstrated in all PPNET cases, but only one case of ES. Conclusions Both PPNET and ES are malignant small cell tumors, commonly occurred in children, derived from the neural crest. Presence of the Homer-Wright rosette and at least two neural markers are required in the diagnosis of PPNET.
出处
《诊断病理学杂志》
CSCD
2000年第4期249-251,I066,共4页
Chinese Journal of Diagnostic Pathology
关键词
外周性原始神经外胚层瘤
尤文肉瘤
诊断
病理
Peripheral primitive neuroectodermal tumor Ewing's tumor Immunogistochemistry Diagnosis
