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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～ 2 0 0 2年收治的 4例儿童Askin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 2 4、2 5、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
Objective To analyze the clinical and pathological features of Askin’s tumor in children and to raise awareness of the disease. Methods The clinical and pathological data of 4 children with Askin tumors admitted from 1996 to 2002 in our hospital were reported. Results The majority of children were elderly children with chest pain and thoracic tumor as the first symptom. Histologically, sparsely organized fibrovascular stromal cells, small round, oval cells around the nests or lobular structure. Reticulated reticular fibers less or no network, PAS staining in some cases (+). Immunohistochemical CD99, Syn positive rate. Electron microscope shows neurosecretory particles. Two of the four patients received postoperative chemotherapy and radiotherapy. The other two cases after biopsy chemotherapy. Three cases died of pulmonary metastases, brain metastases and chemotherapy complications at 24, 25, 5 months after onset, and one case had tumorigenicity. Conclusions Askin’s tumor is a highly malignant neuroendocrine tumor located in the chest and lung. It is easily misdiagnosed as neuroblastoma, lymphoma, rhabdomyosarcoma, pulmonary tuberculosis and lung abscess, including surgery, preoperative or postoperative chemotherapy and radiotherapy Comprehensive treatment. More effective treatment remains to be explored