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目的:探讨累及胸部的外周原始神经外胚层肿瘤(primitive neuroectodermal tumor,PNET)的临床特点、病理学特点、诊断标准、治疗与预后,进而提高胸部PNET的诊疗水平。方法:回顾性分析4例胸部PNET的临床资料。本组纵隔PNET3例,其中2例肿瘤侵犯上腔静脉,行肿瘤大部切除,1例在胸腔镜下行纵隔包块探查活检术;右肺PNET1例,行锁骨上包块活检术。结果:4例术后病理和免疫组化检查均诊断为PNET。2例未行任何治疗,1例行全身化疗,1例行局部放疗。未接受治疗2例生存期分别为6、7个月;接受全身化疗者生存期为12个月;接受局部放疗者截止2006年8月已生存4个月,结论:胸部PNET可发生于任何年龄,原发病灶体积较小时即可发生远处淋巴结转移;临床误诊率高,需依靠病理及免疫组化确诊。由于胸部PNET恶性程度较高,局部浸润与远处转移迅速,因此手术切除率较低,预后较差,需寻找更为有效的治疗方法。
Objective: To explore the clinical features, pathological features, diagnostic criteria, treatment and prognosis of peripheral primitive neuroectodermal tumor (PNET) involving the thorax, and to improve the diagnosis and treatment of chest PNET. Methods: A retrospective analysis of 4 cases of chest PNET clinical data. The group of mediastinal PNET 3 cases, of which 2 cases of tumor invasion of the superior vena cava, most of the tumor resection, 1 case of mediastinum cystoscopy underwent biopsy; right lung PNET 1 cases, supraclavicular mass biopsy. Results: Four cases were diagnosed as PNET by pathology and immunohistochemistry. 2 cases without any treatment, 1 case of systemic chemotherapy, 1 case of local radiotherapy. Two patients who did not receive treatment had survival rates of 6 and 7 months respectively. Patients who underwent systemic chemotherapy had a survival of 12 months. Patients who underwent local radiotherapy had survived for 4 months as of August 2006. Conclusion: PNET in the chest can occur at any age , The primary lesion volume can occur when the distant lymph node metastasis; clinical misdiagnosis rate, need to rely on pathology and immunohistochemistry diagnosis. Due to the high degree of chest PNET malignant, rapid local infiltration and distant metastases, so the lower the surgical resection rate, the prognosis is poor, need to find a more effective treatment.