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目的 分析儿童外周原始神经外胚层肿瘤 (pPNET)临床及病理特征 ,提高对本病的诊治水平。方法 回顾分析解放军总医院 1997年 9月至 2 0 0 2年 12月收治 9例儿童pPNET的临床、病理资料。结果 肿瘤原发部位为胸部 4例 ,左肩胛骨、左前臂、左盆腔、左下肢、腰骶椎各 1例。其中 6例手术 ,3例行肿物穿刺活检。 9例患儿病理标本除常规石蜡切片和HE染色外 ,均做了免疫组织化学检查 ,其中 3例行电镜观察。所有患儿均进行化疗 ,6例手术者 3例合并放疗 ,1例行自体外周血造血干细胞移植。 6例分别于发病后 1、5、7、19、2 4、2 5个月死亡 ;2例正在治疗中 ;1例无瘤生存 6 3个月。结论 pPNET是一种极度恶性的软组织小圆形细胞肿瘤 ,多见于胸部 ,其诊断必须依靠免疫组织化学检查或电镜检查。Homer Wright菊形团和神经标志至少 2项阳性为诊断 pP NET的条件。
Objective To analyze the clinical and pathological features of peripheral primate neuroectodermal tumors (pPNET) in children and to improve the diagnosis and treatment of this disease. Methods The clinical and pathological data of 9 cases of children with pPNET admitted to PLA General Hospital from September 1997 to December 2002 were retrospectively analyzed. Results The primary site of the tumor was chest in 4 cases, left shoulder blade, left forearm, left pelvis, left lower limb and lumbosacral vertebra in 1 case. Among them, 6 cases were operated on and 3 cases were performed biopsy. In addition to routine paraffin sections and HE staining, all the 9 cases of pathological specimens were examined by immunohistochemistry, of which 3 cases were observed by electron microscope. All patients were treated with chemotherapy, 6 cases of surgery combined with radiotherapy and 1 case of autologous peripheral blood stem cell transplantation. 6 cases died at 1, 5, 7, 19, 24, 25 months after onset, 2 cases were under treatment and 1 case had no tumor to survive for 6 to 3 months. Conclusion pPNET is an extremely malignant soft tissue small cell tumor that is more common in the chest. Its diagnosis must rely on immunohistochemistry or electron microscopy. Homer Wright Chrysalis and neurological markers at least two positive for the diagnosis of pP NET conditions.