Submandibular Lymphoepithelioma-like Carcinoma-A Case Report and Review of Literatures

顎下腺淋巴上皮瘤樣癌－病例報告與文獻回顧

劉康帆(Kang-Fan Liu) ； 劉定國(Ting-Kuo Liu) ； 吳家佑(Chia-Yu Wu)

《臺灣口腔顎面外科學會雜誌》 32卷3期 (2021/09) Pp. 243-251

摘要

Lymphoepithelioma-like carcinoma (LELC) of the salivary gland is a rare malignant tumor, characterized by extensive lymphoid infiltration in the stroma with morphologic features resemble undifferentiated nasopharyngeal carcinoma (NPC). The tumor is also strongly association with Epstein-Barr virus (EBV) infection. LELC can occur throughout the body, it has also been found in other organs, such as lung, stomach, bladder, breasts, uterus and skin. Primary LELC of the salivary gland is extremely rare, it comprises only 0.4% of salivary gland malignancies, with 83.5% of the tumor occur in parotid gland, 15% in submandibular gland and scarcely seen in sublingual gland. In this article, we present a case of submandibular gland LELC in a 53 years-old male. The clinical diagnosis, pathological characteristics and treatment modalities were reported. Through literatures review, we discuss the pathogenesis of LELC and other EBV related malignancies as well as their prognostic factors. The role of genomic expressions especially EBERs and BART miRNA in the regulation carcinogenesis of NPC and their possibility of the development of biomarker and precision treatment. The significance of the positivity of expression of PD-L1 in LELC of lung and its potential for the development of future immunotherapy were also reviewed.

關鍵字

Lymphoepithelioma-like carcinoma ； Epstein-Barr virus related malignancies ； Salivary gland tumor ； Submandibular gland

並列摘要

唾液腺淋巴上皮瘤樣癌是一種罕見的口腔癌，其組織學特徵為廣泛性的間質淋巴樣浸潤，和未分化的鼻咽癌難以區分。淋巴上皮瘤樣癌常見於亞洲人種，且與EB病毒感染有強烈的關聯。原發性淋巴上皮瘤樣癌可生長於其他器官，如肺、胃、乳房、子宮、膀胱、皮膚等。原發性唾液腺淋巴上皮瘤樣癌非常罕見，僅佔唾液腺惡性腫瘤的0.4％，其中腮腺佔83.5％，顎下腺15％，而舌下腺則更罕見。本文報告一53歲男性病例，為顎下腺淋巴上皮瘤樣癌。報告中論及臨床診斷，病理特徵及治療方法。經由文獻回顧，此病及其他EB病毒相關之惡性腫瘤之病因學及預後因子均予以討論。此外，EB病毒之基因體編碼表現物如EBERS及BART miRNA在鼻咽癌腫瘤病因學之地位以及應用在發展腫瘤生物標記及精準治療之可能性亦予以探討。尤有進者，肺部之淋巴上皮瘤樣癌之腫瘤細胞之PD-L表現度之研究，亦予以關注以展望未來此相近似腫瘤在免疫治療方面的發展，以應用於末期及轉移案例。

並列關鍵字

淋巴上皮瘤樣癌 ； EB病毒相關惡性腫瘤 ； 唾液腺腫瘤 ； 顎下腺