Lai Shi-Cong, Seery Samuel, Zhang Wei, Liu Ming, Zhang Guan, Wang Jian-Ye
Department of Urology, Beijing Hospital, National Center of Gerontology, Beijing 100730, China.
School of Humanities and Social Sciences, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
World J Clin Cases. 2020 Feb 26;8(4):771-781. doi: 10.12998/wjcc.v8.i4.771.
Lymphoepithelioma-like carcinomas (LELCs) are rare, malignant epithelial tumors, generally considered a subtype of squamous cell carcinoma. LELCs are undifferentiated and can occur in multiple tissues, although LELCs in the urinary tract are extremely rare. As such, evidence does not provide clinicians with guidelines for the best practices. Even though this is a rare disease, it is associated with high morbidity and mortality. Therefore, we must learn to differentiate LELC types and identify risk factors for early identification.
To develop an evidence base to guide clinicians treating primary LELCs of the upper urinary tract (UUT-LELC).
We performed a systematic review of all reports on UUT-LELC from the first published case in 1998 until October 2019, according to the PRISMA. A database was then developed by extracting data from previously published reports in order to analyze interactions between clinical characteristics, pathological features, interventions and outcomes. Survival was analyzed using Kaplan-Meier estimates, which were compared using log rank tests.
A total of 28 previously published cases were identified for inclusion. The median age was 72 years with a male to female ratio of 4:3. Pure type LELCs were most common with 48.3% ( = 14), followed by 37.9% ( = 11) predominant LELCs and 3.4% ( = 1) focal LELCs. Epstein-Barr virus testing was negative in all cases. Fourteen patients received radical nephroureterectomy (RNU)-based intervention. Twenty-three patients survived with no evidence of further metastasis, although six died before the median 18 mo follow-up point. Survival analysis suggests pure histological subtypes, and patients who receive complete tumor resection have more favorable prognoses. As always in cancer care, early identification generally increases the probability of interventional success.
The most effective treatment for UUT-LELC is RNU-based therapy. Since cases are few in number, case reporting must be enhanced and publishing encouraged to both save and prolong lives.
淋巴上皮瘤样癌（LELC）是一种罕见的恶性上皮性肿瘤，通常被认为是鳞状细胞癌的一种亚型。LELC未分化，可发生于多种组织，尽管尿路中的LELC极为罕见。因此，现有证据并未为临床医生提供最佳实践指南。尽管这是一种罕见疾病，但它与高发病率和高死亡率相关。因此，我们必须学会区分LELC类型并识别危险因素以便早期诊断。
建立循证依据，以指导临床医生治疗上尿路原发性LELC（UUT-LELC）。
根据PRISMA，我们对1998年首例发表至2019年10月期间所有关于UUT-LELC的报告进行了系统评价。然后通过从先前发表的报告中提取数据建立数据库，以分析临床特征、病理特征、干预措施和结果之间的相互作用。使用Kaplan-Meier估计法分析生存率，并使用对数秩检验进行比较。
共纳入28例先前发表的病例。中位年龄为72岁，男女比例为4:3。纯型LELC最常见，占48.3%（n = 14），其次是占37.9%（n = 11）的优势型LELC和占3.4%（n = 1）的局灶型LELC。所有病例的爱泼斯坦-巴尔病毒检测均为阴性。14例患者接受了以根治性肾输尿管切除术（RNU）为基础的干预。23例患者存活，无进一步转移迹象，尽管6例在中位随访18个月前死亡。生存分析表明，组织学纯亚型以及接受肿瘤完整切除的患者预后更佳。在癌症治疗中，一如既往，早期诊断通常会增加干预成功的概率。
UUT-LELC最有效的治疗方法是以RNU为基础的治疗。由于病例数量较少，必须加强病例报告并鼓励发表，以挽救生命并延长生存期。
