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胸部原始神经外胚层肿瘤(附10例报告) 被引量:14

Thoracic primitive neuroectodermal tumor
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摘要 目的提高对胸部原始神经外胚层肿瘤(PNET)的认识,改进治疗效果。方法回顾性分析1999年至2004年手术切除并经病理证实的10例胸部PNET治疗结果。结果肿瘤位于胸腔内6例、胸壁2例,心包内和后纵隔各1例。胸内肿瘤完全摘除2例,大部分切除2例,肿瘤并肺叶切除2例。2例胸壁肿瘤合并受累肋骨切除胸壁重建,1例行肿瘤及心包部分切除,1例后纵隔哑铃形肿瘤由胸外科和神经外科同期切除。全组无手术死亡或住院死亡。病理标本免疫组化检查,10例CD99均为阳性,LCA均为阴性。术后4例接受化疗,4例放化疗,2例未行任何辅助治疗。术后随访6年,死亡7例,生存最长27个月,最短9个月,平均17个月。3例生存已超过12个月者仍在随访中。结论胸部PNET恶性程度高,进展快,术前诊断率低。诊断与鉴别诊断需要病理组织学和免疫组化确定。彻底摘除肿瘤及受累组织是治疗的重要环节,术后放化疗有助延长生存期。提高生存率还需要寻找更有效的方法。 Objectve To enhance the understanding of chest primitive neuroectedennal tumor (PNET) and improve the results of management. Methods The results of 10 cases of chest PNET, which had been surgically treated and pathologically confirmed from 1999 to 2004 were retrospectively analyzed. Results There were 6 intrathoraeic tuners, 2 in the chest wall, 1 in the pericardium, and 1 in the posterior mediastinum. Two intrathoraeic tumors were completely resected, 2 partially resected, tuner and the involved lung were removed in 2 eases. Two had tumor with the affected ribs removed and the chest wall reconstructed. Tumor and the invaded pericardium were removed in 1 case. The PNET in the posterior mediastinum, invading into spinal canal as a bell tuner, was excised by the thoracic surgeon cooperating with neuresurgcon. There was no hospitalization death. All the specimens demonstrated positive reaction to CD99 and negative to LCA. 4 cases received postoperatively adjuvant chemotherapy, 4 radiotherapy and chemotherapy, the other 2 eases had none adjuvant therapy. 7 patients died during 6 years of follow-up, with 9 to 27 months survival. 3 patients are alive and have over 12 months survival so far. Conclusion PNET is an aggressive and invasive malignant tumor with poor prognosis. Difficulties exist in diagnosis. Besides common microscopic examination, pathological histology and inmmnochemistry, even electron mimic examination may be required in differential diagnosis. Complete removal of tumor and involved tissue plays an important role, postoperative adjuvant therapy, including radiotherapy or combination with chemotherapy, may prolong survival. Medalities that are more effective should be developed to improve the treatment results.
出处 《中华胸心血管外科杂志》 CSCD 北大核心 2006年第2期102-104,共3页 Chinese Journal of Thoracic and Cardiovascular Surgery
关键词 胸部肿瘤 胸外科手术 神经外胚瘤 诊断 综合疗法 Thoracic neoplasms Thoracic surgical procedures Neroectodennal tumors Diagnosis Combined modality therapy
作者简介 进修医师
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参考文献18

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