Published online Feb 26, 2020. doi: 10.12998/wjcc.v8.i4.771
Peer-review started: October 20, 2019
First decision: December 4, 2019
Revised: December 11, 2019
Accepted: December 22, 2019
Article in press: December 22, 2019
Published online: February 26, 2020
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% (n = 14), followed by 37.9% (n = 11) predominant LELCs and 3.4% (n = 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.
Core tip: Lymphoepithelioma-like carcinomas in the urinary tract are extremely rare, hence there is little evidence to provide clinical guidelines. Of the 28 participants included in this systematic review of case reports, 23 patients survived with no evidence of further metastasis. 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, although this evidence base must be developed with more rigorous testing and case reporting.