原发性肺淋巴上皮瘤样癌的临床病理特点及预后分析

发布时间：2018-11-14 09:44

【摘要】：目的肺原发性淋巴上皮瘤样癌(Lymphoepithelioma-like carcinoma,LELC)是一类少见疾病,临床上对其了解和认识不足,容易出现漏诊和误诊,本文旨在对肺原发性淋巴上皮瘤样癌患者的临床病理、影像学、基因特点进行归纳总结,对淋巴转移及预后相关因素进行分析,由此进一步加深对原发性肺淋巴上皮瘤样癌这一少见疾病的认识,有助于临床上对该少见疾病的诊治决策和预后评估。方法本研究回顾性分析了 2009年6月至2017年3月期间浙江大学医学院附属第一医院收住的肺原发性淋巴上皮瘤样癌患者,收集临床病理资料,标本送基因检测,归纳总结其临床病理、影像学、基因检测、免疫组化方面的特征,并对患者进行随访,进一步分析预后相关因素。结果共纳入23例原发性肺淋巴上皮瘤样癌患者,男性居多(65.2%),中位年龄59岁,吸烟者占43.4%,患者以咳嗽咳痰(39.1%)和胸痛(21.7%)症状起病多见。影像学上以周围型多见(66.7%),大多呈实质性占位,平均最大径为2.99cm,位置离纵隔相近,大多密度不均(66.7%),不规则(72.2%),可伴毛刺分叶征象,可合并阻塞性肺炎(27.8%)、胸腔积液等征象,且增强后往往呈不均匀强化。23例患者中,有1例经评估后无手术指征,另22例则行手术切除。免疫组化中,阳性表达的有p63、CK(pan)、CK14及CK5/6,阴性表达为主的标记物有CgA、Syn、CK7、TTF1、CD56及CDD,,EBER阳性率高达87.5%(14/16);14例基因检测标本中,仅有1例EGFR20突变。22例术后患者中,5例出现复发转移,约半数接受术后辅助治疗。分析发现60岁以上的患者出现淋巴结转移的风险更低(p=0.008),预后更好,在CT影像中如果肿块压迫或包绕支气管,患者淋巴结转移风险增加(p=0.041),而淋巴结转移是预后不良因素之一,此外,肿瘤病理上是否合并坏死也是影响复发转移和长期生存的重要因素(p[PFS]=0.023,p[OS]=0.005)。结论原发性肺淋巴上皮瘤样癌患者起病时常有肺部症状,CT影像上以肺实性占位多见,往往为单发、近纵隔周围型病灶,边界不规则,可伴毛刺分叶,钙化坏死少见,常常呈不均匀轻中度强化,如肿瘤包绕或压迫支气管,则淋巴结转移风险增加。治疗方面推荐综合治疗,即在手术治疗基础上辅以放化疗。患者年龄60岁以下、肿瘤存在淋巴结转移、合并坏死是预后的不良因素。
[Abstract]:Objective Primary lymphoepitheliomatous carcinoma (Lymphoepithelioma-like carcinoma,LELC) of the lung is a rare disease, which is not well understood and understood in clinic, and is liable to be misdiagnosed and misdiagnosed. The purpose of this article is to study the clinicopathological features of primary lymphoepitheliomatous carcinoma of the lung. The imaging and gene characteristics were summarized, and the related factors of lymphatic metastasis and prognosis were analyzed, which further deepened the understanding of the rare disease of primary lymphoepitheliomatous carcinoma of the lung. It is helpful for the diagnosis and treatment and prognosis evaluation of this rare disease. Methods from June 2009 to March 2017, patients with primary lymphoepitheliomatous carcinoma of the lung were collected from the first affiliated Hospital of Zhejiang University Medical College. The clinicopathological, imaging, genetic and immunohistochemical features of the patients were summarized, and the prognostic factors were analyzed. Results A total of 23 patients with primary lymphoepitheliomatous carcinoma were included, the male being the majority (65.2%), the median age was 59 years old, the smokers accounted for 43.44.The patients with cough and sputum (39.1%) and chest pain (21.7%) were more likely to have symptoms of cough and sputum (39.1%) and chest pain (21.7%). Most of the images were peripheral type (66.7%), most of them were solid occupying, the average maximum diameter was 2.99 cm, the location was close to the mediastinum, the density was uneven (66.7%) and irregular (72.2%). Obstructive pneumonia (27.8%), pleural effusion and other signs were often enhanced inhomogeneously after enhancement. Of the 23 cases, 1 case had no indication of operation after evaluation, the other 22 cases underwent surgical excision. In immunohistochemistry, the positive rate of CgA,Syn,CK7,TTF1,CD56 and CDD,,EBER was 87.5% (14 / 16). Only one case of EGFR20 mutation was detected in 14 gene samples, 5 of 22 patients had recurrence and metastasis, and about half of them received postoperative adjuvant therapy. It was found that patients over 60 years old had a lower risk of lymph node metastasis (p0. 008) and better prognosis. In CT imaging, the risk of lymph node metastasis was increased if the mass was compressed or wrapped around the bronchus (p0. 041). Lymph node metastasis is one of the poor prognostic factors. In addition, whether the tumor is associated with necrosis or not is also an important factor affecting recurrence, metastasis and long-term survival (p [PFS] = 0.023 p [OS] = 0.005). Conclusion Pulmonary symptoms often occur in patients with primary lymphocytoma-like carcinoma of the lung. Pulmonary solid lesions are more common on CT images, which are usually solitary, near mediastinal lesions, with irregular borders, can be accompanied by prickles and lobes, and calcified necrosis is rare. Uneven mild and moderate enhancement, such as tumor wrapping or compression of the bronchus, increases the risk of lymph node metastasis. Comprehensive treatment is recommended for treatment, that is, radiotherapy and chemotherapy are added to the surgical treatment. Patients under 60 years of age, tumor lymph node metastasis, associated with necrosis is a poor prognostic factor.
【学位授予单位】：浙江大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R734.2

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