【摘要】：目的肺原發(fā)性淋巴上皮瘤樣癌(Lymphoepithelioma-like carcinoma,LELC)是一類少見疾病,臨床上對(duì)其了解和認(rèn)識(shí)不足,容易出現(xiàn)漏診和誤診,本文旨在對(duì)肺原發(fā)性淋巴上皮瘤樣癌患者的臨床病理、影像學(xué)、基因特點(diǎn)進(jìn)行歸納總結(jié),對(duì)淋巴轉(zhuǎn)移及預(yù)后相關(guān)因素進(jìn)行分析,由此進(jìn)一步加深對(duì)原發(fā)性肺淋巴上皮瘤樣癌這一少見疾病的認(rèn)識(shí),有助于臨床上對(duì)該少見疾病的診治決策和預(yù)后評(píng)估。方法本研究回顧性分析了 2009年6月至2017年3月期間浙江大學(xué)醫(yī)學(xué)院附屬第一醫(yī)院收住的肺原發(fā)性淋巴上皮瘤樣癌患者,收集臨床病理資料,標(biāo)本送基因檢測(cè),歸納總結(jié)其臨床病理、影像學(xué)、基因檢測(cè)、免疫組化方面的特征,并對(duì)患者進(jìn)行隨訪,進(jìn)一步分析預(yù)后相關(guān)因素。結(jié)果共納入23例原發(fā)性肺淋巴上皮瘤樣癌患者,男性居多(65.2%),中位年齡59歲,吸煙者占43.4%,患者以咳嗽咳痰(39.1%)和胸痛(21.7%)癥狀起病多見。影像學(xué)上以周圍型多見(66.7%),大多呈實(shí)質(zhì)性占位,平均最大徑為2.99cm,位置離縱隔相近,大多密度不均(66.7%),不規(guī)則(72.2%),可伴毛刺分葉征象,可合并阻塞性肺炎(27.8%)、胸腔積液等征象,且增強(qiáng)后往往呈不均勻強(qiáng)化。23例患者中,有1例經(jīng)評(píng)估后無手術(shù)指征,另22例則行手術(shù)切除。免疫組化中,陽性表達(dá)的有p63、CK(pan)、CK14及CK5/6,陰性表達(dá)為主的標(biāo)記物有CgA、Syn、CK7、TTF1、CD56及CDD,,EBER陽性率高達(dá)87.5%(14/16);14例基因檢測(cè)標(biāo)本中,僅有1例EGFR20突變。22例術(shù)后患者中,5例出現(xiàn)復(fù)發(fā)轉(zhuǎn)移,約半數(shù)接受術(shù)后輔助治療。分析發(fā)現(xiàn)60歲以上的患者出現(xiàn)淋巴結(jié)轉(zhuǎn)移的風(fēng)險(xiǎn)更低(p=0.008),預(yù)后更好,在CT影像中如果腫塊壓迫或包繞支氣管,患者淋巴結(jié)轉(zhuǎn)移風(fēng)險(xiǎn)增加(p=0.041),而淋巴結(jié)轉(zhuǎn)移是預(yù)后不良因素之一,此外,腫瘤病理上是否合并壞死也是影響復(fù)發(fā)轉(zhuǎn)移和長(zhǎng)期生存的重要因素(p[PFS]=0.023,p[OS]=0.005)。結(jié)論原發(fā)性肺淋巴上皮瘤樣癌患者起病時(shí)常有肺部癥狀,CT影像上以肺實(shí)性占位多見,往往為單發(fā)、近縱隔周圍型病灶,邊界不規(guī)則,可伴毛刺分葉,鈣化壞死少見,常常呈不均勻輕中度強(qiáng)化,如腫瘤包繞或壓迫支氣管,則淋巴結(jié)轉(zhuǎn)移風(fēng)險(xiǎn)增加。治療方面推薦綜合治療,即在手術(shù)治療基礎(chǔ)上輔以放化療�；颊吣挲g60歲以下、腫瘤存在淋巴結(jié)轉(zhuǎn)移、合并壞死是預(yù)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.
【學(xué)位授予單位】：浙江大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R734.2

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