儿童Askin肿瘤

目的 分析儿童Askin瘤临床、病理特征，提高对本病的认识水平。方法 报告本院1996～2002年收治的4例儿童硒肿瘤临床、病理资料。结果 儿童病例中以年长儿为主，多以胸痛、胸腔肿物为首发症状。组织学上以稀少的纤维血管间质将小圆、卵圆瘤细胞围绕成巢状或小叶状结构。网染细胞间网状纤维少或无，PAS染色部分病例( )。免疫组化中CD99、Syn阳性率高。电镜下可见神经分泌颗粒。4例中2例行术后化疗、放疗。另2例活检后化疗。3例因肺转移、脑转移、化疗并发症分别于发病后24、25、5个月死亡，1例带瘤生存。结论 Askin瘤为一种位于胸肺部的高度恶性的神经内分泌肿瘤，易误诊为神经母细胞瘤、淋巴瘤、横纹肌肉瘤、肺结核及肺脓肿等，需包括手术、术前或术后化疗及放疗的综合治疗。更有效的治疗方法仍有待于探讨。

Askin Tumors in Children

Objective To analyze the clinicopathological features and enhance the knowledge of Askin tumor in children. Methods The clinicopathological features of 4 cases of children with Askin tumor were described. Results Clinically, the tumor was often seen in elder child patients with painful thoracopulmonary masses.Histopathologically, the nest-like or lobular structures composed of small round-to-oval cells were surrounded by sparse fibrovascular stroma. Among tumor cells there were no or few resticular fibers,however in the surrounding of tumor masses the fibers were found. PAS staining in the tumor in a part of cases was positive. The positive rates of CD99 and Syn detected by immunohistochemistry were high. Neurosecretory granules can be observed by electron microscopy. Two patients received both chemotherapy and radiotherapy three patients died of metastatic tumor of lung and brain and chemotherapy complication in 24,25 and 5 months, respectively. Conclusions Askin tumor is a highly malignant neurosecretory tumor in thoracopulmonary region, which often be misdiagnosed as neuroblastoma, lymphoma, embryonal rhabdomyo sarcoma, tuberculosis and lung abscess, and requires a synthetical treatment plan consisting of pre-and postoperative chemotherapy, radiotherapy and surgical resection. The treatment methods of Askin tumor needs to be further studied.