Non-Epstein-Barr virus-associated double primary lymphoepithelioma-like carcinoma of the esophagus and stomach: a case report and literature review

Abstract

A 55-year-old Chinese male was admitted to the hospital for epigastralgia and dysphagia with a two month history, and hematemesis and melena with a two-day history. Two lesions were found in the esophagus and stomach by esophago--gastroduodenoscopy and computed tomography. The patient underwent subtotal esophagectomy and gastrectomy, esophagogastric anastomosis above the aortic arch, and thoracic-abdominal two-field lymph node dissection. Pathological and immumohistochemical studies showed that both lesions had the same form of poorly differentiated carcinoma with dense lymphoid stroma, which was diagnosed as lymphoepithelioma-like carcinoma (LELC). No metastatic relationship was found between the two tumors. Therefore, the case was double primary lymphoepithelioma-like carcinoma of the esophagus and stomach. Epstein-Barr virus (EBV) in the two tumors were negative by EBV-encoded small RNA1 (EBER-1) in situ hybridization. No adjuvant therapy was performed due to his poor physical condition post-operatively, and no evidence of tumor recurrence or metastasis was found during the next 14 months of follow-up. Esophageal and gastric LELC are rare, especially the former, which has a specific geographical distribution. Literature reported cases showed upper gastrointestinal LELC were highly malignant with good prognosis, and EBV was detected less in esophageal LELC cases but more commonly in gastric LELC cases. Upper gastrointestinal LELC lesions are usually singular, and no synchronous lesions were reported in the literature. Our case is the first LELC to present as double primary lymphoepithelioma- like carcinoma of both esophagus and stomach simultaneously, which demonstrates that LELC can be multifocal in the upper gastrointestinal tract.

Keywords: Esophagus; diagnosis; double primary; lymphoepithelioma-like carcinoma; stomach.

Figure 1

Esophago-gastroduodenoscopy revealed an elevated lesion in the left posterior esophagus with a central ulcer 30-35 cm from the upper incisors (A) and an ulcerative lesion in the cardia, fundus and anterior and posterior lesser curvature of the stomach with a soiled and purulent surface (B).

Figure 2

Computed tomography of the chest and abdomen revealed a thickened wall and almost occluded lumen that was located at the middle and lower thoracic portion of the esophagus (A) and a thickened cardiac wall demonstrating protrusion into the gastric cavity (B), no enlarged lymph nodes were found around them (120 KV, 200 mA, WW: 350, WL: 40).

Figure 3

(A) 4 × 2.5 × 1.2 cm ulcerative and infiltrative type of tumor was observed in the resected esophageal specimen (A) and another 7 × 5 × 2.5 cm ulcerative and infiltrative type of tumor was observed in the resected gastric specimen (B).

Figure 4

Microscopy revealed the esophageal lesion (A-C) and the gastric lesion (D-F) had similar form, and that they were both sub-mucosal carcinoma that had invaded full layer, including the serosa. Low power view (seen in panels A and D above): The carcinoma cells were poorly differentiated, with a sparse nest-like distribution, and a small palisading arrangement. HE staining; × 40 magnification. Medium power view (seen in panels B and E above): Massive mature lymphocytes were seen around the carcinoma nests, including small lymphocytes and plasmocytes. HE staining; × 100 magnification. High power view (C and F): The carcinoma cells were enlarged, with very large and atypical oval nuclei with vesicular chromatin and prominent nucleoli. HE staining; × 200 magnification.