Lymphoepithelioma‐like carcinoma of the esophagus: Report of a case with non‐progressive behavior

Abstract

Lymphoepithelioma-like carcinoma (LELC) of the esophagus is extremely rare and the prognosis has been described to be favorable. Herein, the case is reported of esophageal LELC showing non-progressive course for 1 year. The patient was a 70-year-old Japanese man with a submucosal tumor in the lower esophagus. The tumor was endoscopically regarded as a benign leiomyoma. One year after the first endoscopic examination, the size of the lesion remained unchanged despite no treatment. Histologically, the tumor showed undifferentiated carcinoma associated with dense lymphocytic and plasma cell infiltration. Lymphoid follicles surrounded the tumor cell nests. Immunohistochemically, Ki-67 labeling index of the tumor cells were 76.0%, and there were few single-strand DNA-positive apoptotic cells. In situ hybridization using a digoxigen-labeled Epstein-Barr virus (EBV)-encoded small RNA (EBER)-1 riboprobe failed to detect the presence of EBV infection. Physicians should be aware that esophageal LELC endoscopically mimics benign submucosal tumor. This case may demonstrate that esophageal LELC is a slow-growing tumor and it is related to immune reaction.

Difficult Preoperative Diagnosis of Lymphoepithelioma-Like Carcinoma of the Esophagus

“…None were reported to have Epstein-Barr virus (EBV) infection, despite a close association between EBV infection and nasopharyngeal or gastric LELC. 2,3 The typical EGD findings were SMT covered with normal-appearing mucosa, and histologic diagnosis of the primary biopsy tended to be poorly differentiated SCC or no malignancy. 2–5 This is not surprising because the histologic characteristics are observed mainly in the submucosa, where undifferentiated cancer cells exist in the stroma, while nondysplastic epithelium is overlaid.…”

Esophageal Lymphoepithelioma-Like Carcinoma with Unique Daisy-Like Appearance

“…LELC tumors can arise in a multitude of locations such as the thyroid and breast, as well as gastrointestinal sites such as the biliary system, stomach, and colon. While the histologic features are similar, clinical presentation, age, sex, stage at presentation, size, and overall survival vary dramatically between sites [ 8 ]. LELC has a better prognosis compared to conventional gastrointestinal adenocarcinoma [ 9 , 10 ].…”

“…LELC has a better prognosis compared to conventional gastrointestinal adenocarcinoma [ 9 , 10 ]. Seventeen LELC cases arising in the esophagus have been reported in the English language literature [ 8 ].…”

“…The most characteristic endoscopic appearance of esophageal LELC is submucosal tumor covered with intact or ulcerative esophageal mucosa and a depressed middle portion. Exceptional cases may have simple ulcerative morphology, polypoid lesion, or solely reddish mucosal irregularity [ 8 ]. In our case, the lesion was polypoid and the accompanying reddish mucosal irregularity was established from the lesion to the level of the cardioesophageal junction.…”

“…Limited information exists in the literature regarding the long-term follow-up and management of LELC because of its low incidence. Nakasono et al [ 8 ] reported a case of esophageal LELC with non-progressive behavior. Contrary to that report, our case had a rapid recurrence after polypectomy with concomitant evident lymphadenopathies, which can indicate poor prognosis.…”