肺大细胞神经内分泌癌的诊断及治疗

Diagnosis and Treatment of Pulmonary Large-cell Neuroendocrine Carcinoma

肺大细胞神经内分泌癌(pulmonary large-cell neuroendocrine carcinoma,LCNEC)是一种罕见的肺部上皮性恶性肿瘤,1991年被首次报道,2004年病理分型正式划归支气管肺神经内分泌肿瘤。该病多见于高龄吸烟男性,临床表现无特异性,可有呼吸道相关症状,副肿瘤综合征罕见,CT常表现为外周型结节影,可有分叶、毛刺及胸膜牵拉。LCNEC恶性程度高,患者诊断时约60%~80%出现淋巴结转移,40%出现远处转移,恶性程度接近小细胞肺癌。手术联合术后辅助治疗是Ⅰ、Ⅱ、Ⅲa期患者的主要治疗手段,化疗建议PE方案(顺铂联合依托泊苷)。转移及术后复发可能是预后不良的主要原因,肿瘤驱动基因及进一步靶向药物治疗尚有待进一步研究。

Pulmonary large-cell neuroendocrine carcinoma(LCNEC),first reported in 1991,is a rare pulmonary epithelial malignancy.It was not until 2004 that LCNEC was officially classified pathologically as the bronchopulmonary neuroendocrine tumor.Most of the patients are elderly male smokers.Their clinical manifestations are not specific.They may have respiratory tract-related symptoms.Paraneoplastic syndrome is rare.CT usually shows peripheral nodules,including lobulation,burr,and pleural traction.LCNEC was reported as a highly aggressive malignancy,with lymph node metastasis occurring in about 60%-80%of patients and distant metastasis in about 40%of patients,similar to the degree of malignancy of small cell lung cancer.Surgery combined with postoperative adjuvant therapy is the main treatment for stageⅠ,Ⅱ,andⅢa patients.PE regimen is recommended for chemotherapy.Metastasis and recurrence may be the main causes of poor prognosis.Tumordriven genes and targeted drug therapy need further study.