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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 Wright菊形团和神经标志至少2项阳性为诊断PPNET的条件
Objective To investigate the clinical pathology, immunohistochemical characteristics and diagnostic criteria of peripheral primitive neuroectodermal tumors (PPNET) and Ewing’s sarcoma (ES). Methods 19 cases of PPNET and ES were observed by HE staining, PAS staining and immunohistochemistry. Three specimens were examined by electron microscopy. Results 12 patients with PPNET, aged 2 to 11 years, mean age 65 years; 8 males and 4 females, lesions located in 5 limbs, 3 cases of chest wall, 3 cases of paraspinal and 1 case of retroperitoneum. 7 cases of ES, aged 4 to 10 years old, averaged 63 years old; 3 males and 4 females, lesions located in the femur in 3 cases, 3 cases of scapula, left leg in 1 case. Microscopic examination: The PPNET tumor cells were small and arranged in solid slices, lobulated, alveolar and cords. Homer Wright chrysanthemum clusters were found in all 12 cases; the morphology and arrangement of ES tumors were similar to PPNET, but cytoplasm. Transparent, no homer Wright daisy-shaped group. Immunohistochemistry: Both PPNET and ES expressed 12E7, and each case of PPNET expressed more than 2 neural markers, whereas ES only expressed 1 item. Conclusions PPNET and ES are common small cell malignancies in children. Homer Wright has at least 2 positive clusters and neural markers for the diagnosis of PPNET.