Endoscopic submucosal dissection combined with radiotherapy for T1b esophageal lymphoepithelioma-like carcinoma: A case report

Abstract

BACKGROUND

Esophageal lymphoepithelioma-like carcinoma is a rare, poorly differentiated neoplasm lacking standardized endoscopic treatment. It is prone to misdiagnosis as a subepithelial lesion, with false-negative biopsy results. Tiny superficial lesions may still exhibit submucosal invasion. Reports on endoscopic submucosal dissection for this disease are limited, and the necessity of adjuvant radiotherapy remains controversial in the absence of unified clinical guidelines.

CASE SUMMARY

A 59-year-old man with a history of total gastrectomy had a 1.0-cm superficial esophageal lesion. Endoscopic evaluation suggested submucosal invasion, while computed tomography imaging ruled out metastatic disease. En bloc endoscopic submucosal dissection was subsequently performed. Pathological examination confirmed T1b-SM2 lymphoepithelioma-like carcinoma with negative margins and no evidence of Epstein–Barr virus infection. The patient received adjuvant radiotherapy at a dose of 6000 cGy. Regular follow-up with endoscopy and thoracic computed tomography has shown no signs of local or distant recurrence.

CONCLUSION

Minute esophageal lymphoepithelioma-like carcinoma may harbor a risk of deep invasion. Radiotherapy may be considered as subsequent therapy, though further evidence is needed.

Key Words: Esophageal lymphoepithelioma-like carcinoma; Endoscopic submucosal dissection; T1b esophageal carcinoma; Radiotherapy; Epstein–Barr virus; Case report

Core Tip: Esophageal lymphoepithelioma-like carcinoma is a rare entity prone to misdiagnosis. We report a case of tiny T1b esophageal lymphoepithelioma-like carcinoma treated with en bloc endoscopic submucosal dissection followed by adjuvant radiotherapy. Despite the small lesion size, submucosal invasion was present. No recurrence was observed during 3 years of follow-up. This case suggests that even small superficial lymphoepithelioma-like carcinoma may harbor invasive potential. Although adjuvant radiotherapy was administered in accordance with esophageal cancer guidelines, a unified treatment consensus for this rare pathology remains lacking and requires further investigation.

INTRODUCTION

Esophageal lymphoepithelioma-like carcinoma (LELC) is rare but treatable via either surgical or endoscopic resection[1,2]. However, there is no consensus regarding whether additional treatment should be administered when esophageal LELC invades the submucosa. Our case might add some value.

CASE PRESENTATION

Chief complaints

A 59-year-old man with a history of total gastrectomy had a 1.0-cm superficial esophageal lesion. He underwent gastric cancer surgery 2 years prior, and esophageal mucosal lesions were detected 9 months prior.

History of present illness

Two years previously, the patient presented with postprandial upper abdominal distension and discomfort. He was diagnosed with early gastric cancer at a local hospital and underwent total gastrectomy, with an uneventful postoperative recovery. Nine months previously, routine follow-up gastroscopy at the local hospital revealed esophageal mucosal lesions. Esophageal biopsy pathology showed focal mild-to-moderate squamous epithelial dysplasia (Figure 1).

Figure 1 Pathology of the endoscopically resected specimen. A: Beneath the mucosal epithelium, large areas of tumor parenchyma diffusely infiltrate in sheet-like and nested patterns; B and C: The tumor invades the lamina propria and muscularis mucosae, but the overlying epithelial cells are non-neoplastic; D and E: The tumor stroma is densely infiltrated by abundant mature lymphocytes and plasma cells, forming the characteristic architecture of “tumor nests embedded in abundant lymphoid stroma”; F: Tumor cells are large, with pale eosinophilic cytoplasm and ill-defined cell borders. The nuclei are round to oval, with thick and irregular nuclear membranes. Chromatin is pale and vacuolated, with prominent nucleoli; binucleated or multinucleated cells are observed in some areas. Mitotic figures are frequently seen, accompanied by marked cellular atypia. The white scale bars represent 40 μm, 60 μm, 10 μm, 5 μm, and 20 μm in B–F, respectively.

History of past illness

The patient had no significant past medical history.

Personal and family history

The patient had no significant personal or family history.

Physical examination

No abnormalities were noted.

Laboratory examinations

The serum carbohydrate antigen 72-4 concentration was elevated to 11.70 U/mL.

Imaging examinations

A contrast-enhanced computed tomography scan of the whole abdomen showed that the gastric silhouette was absent. Striped hyperdensities were observed in the surgical bed. Mild wall thickening was noted at the gastroenterostomy stoma. The duodenal stump demonstrated no definite intestinal wall thickening. On a contrast-enhanced chest computed tomography scan, no wall thickening was identified at the esophageal anastomosis (Figure 2). No abnormally enhanced masses or space-occupying lesions were detected.

Figure 2 Endoscopic and radiographic findings before surgery. A: Under white-light imaging, a pale type 0-IIa lesion is observed; B: Narrow-band imaging shows intact mucosa with no obvious demarcation, presumably indicating a submucosal origin; C: Magnifying endoscopy shows irregular B2i-like vessels; D: Lugol’s iodine staining shows a pale-stained area at the top of the lesion; E: Postoperative anatomy after total gastrectomy; F: Negative findings on computed tomography prompted the use of minimally invasive endoscopic submucosal dissection.

FINAL DIAGNOSIS

The final diagnosis was an esophageal mucosal lesion of undetermined nature. The patient had undergone total gastrectomy.

TREATMENT

En bloc endoscopic submucosal dissection was performed for the esophageal lesion. Macroscopic pathological examination showed that the lesion measured 0.4 cm × 0.4 cm. Microscopic examination after hematoxylin–eosin staining showed a nest of poorly differentiated squamous cell carcinoma infiltrated by dense lymphocytes and covered with normal epithelium. The cancer invaded 500 μm into the submucosa, with clear margins.

OUTCOME AND FOLLOW-UP

The patient received adjuvant radiotherapy at a dose of 6000 cGy. No recurrence was observed during 3 years of follow-up.

DISCUSSION

Esophageal LELC is rare. It is generally treated with either surgical or endoscopic resection. To date, at least 6 cases of superficial esophageal LELC ranging from 0.6 cm to 1.2 cm in size have been treated with endoscopic submucosal dissection[3]. However, there is no consensus regarding whether additional treatment should be administered after endoscopic resection when LELC invades the submucosa. According to the Japan Esophageal Society guidelines on esophageal cancer, additional therapy, including surgical resection, chemotherapy, or radiotherapy, should be administered when pathological examination indicates submucosal invasion[4]. Therefore, the management of esophageal LELC could follow the guidelines for esophageal squamous cell carcinoma, in which subsequent chemoradiotherapy is recommended for tumors invading the muscularis mucosae and submucosa. Our patient received adjuvant radiotherapy at a dose of 6000 cGy. No recurrence was observed during 3 years of follow-up. Endoscopic therapy combined with radiotherapy may serve as a standard treatment regimen for LELC with submucosal invasion, but more clinical cases need to be accumulated to evaluate its efficacy.

CONCLUSION

Minute esophageal LELC may harbor a risk of deep invasion. Radiotherapy may be considered as subsequent therapy, though further evidence is needed.

ACKNOWLEDGMENTS

We greatly appreciate Professor Bing Hu from West China Hospital for performing the endoscopic submucosal dissection.