High-grade large cell neuroendocrine carcinoma of the esophagus: a case report and review of the literature

Abstract

Background: Neuroendocrine carcinomas are extremely rare in the esophagus as they represent less than 0.04% of all neuroendocrine tumors. To date, only 14 cases of poorly differentiated, high-grade esophageal NEC have been described in the literature. The majority of these patients presented with typical dysphagia symptoms. Due to its rarity, no standardized guidelines have been proposed to treat esophageal neuroendocrine carcinoma, although general recommendations suggest surgery with adjuvant chemoradiotherapy as the treatment of choice.

Case presentation: A 67-year-old previously healthy White male presented with a year-long intermittent nonspecific retrosternal discomfort, with the absence of any other symptoms. Esophagogastroduodenoscopy revealed an ulcerative mass in his lower esophagus, with concern of malignancy. Endoscopic ultrasound-guided biopsy revealed poorly differentiated neuroendocrine carcinoma of the esophagus with metastasis to a diaphragmatic lymph node. He was treated with neoadjuvant chemoradiation followed by surgery, and he has been in remission for over 5 years.

Conclusion: Here, we review the literature and report a unique case of a patient with a vague presentation of esophageal neuroendocrine carcinoma as he enters his sixth year of survival following neoadjuvant chemoradiotherapy.

Keywords: Esophageal cancer; Large cell carcinoma; Neuroendocrine tumor.

Fig. 1

A Esophagogastroduodenoscopy and B endoscopic ultrasound showing the tumor

Fig. 2

A On low magnification, the biopsy showed islands of tumor separated by fibrovascular septae [hematoxylin and eosin (H&E) ×4]. B The neoplastic cells have large, hyperchromatic nuclei, irregular nuclear membranes, inconspicuous nucleoli, and scant amount of amphophilic cytoplasm (H&E ×20). The presence of significant nuclear molding on the right side of the image was also noted. The tumor cells demonstrate strong and diffuse expression of CD56 (C) and synaptophysin (D)