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.

An Uncommon Encounter: A Comprehensive Case Report of an Advanced Esophageal Neuroendocrine Carcinoma

“…The incidences vary according to geographic location: 0.5-5.9%, 0.8-2.8%, and 1-2.8% of all esophageal cancers in China, Japan, and the Western world, respectively [ 4 ]. E-NECs mainly afflict elderly individuals (range 42-85 years), with a predilection for the male sex (80%) [ 3 ]. The symptoms' onset is 1-12 months prior to the diagnosis.…”

“…E-NECs are mainly located in the middle-to-lower esophageal segments, due to the greater availability of Merkel cells and endocrine cells in the cardiac glands in the middle and lower esophagus, respectively. These malignant cells may be functional; however, most of them are non-functional, particularly the poorly differentiated ones [ 3 ]. E-NECs are usually an incidental finding on UGIE, with the visualization of a single lesion.…”

“…However, the lesion may appear as an ulcerated or fungating mass, with deep infiltration of the esophageal wall [ 4 ]. The symptoms vary according to the size, location, secretory activity, and metastasis of the tumor [ 3 ]. Dysphagia and weight loss are the most common presenting symptoms, while dysphonia, odynophagia, retrosternal and epigastric pain, dyspnea, and gastrointestinal bleeding are also reported [ 1 , 4 ].…”

“…Morphologically, neuroendocrine tumors (NETs) are either small cell (about 90%) or large cell (about 10%). With an incidence rate of 0.44 per million a year, they account for 0.04% of all NECs, and 0.4 - 2% of all esophageal cancers [ 3 ]. Over the last few decades, their incidence has increased, mainly owing to clinician awareness and improved diagnostic modalities.…”

A Poorly Differentiated Esophageal Neuroendocrine Carcinoma With Brain Metastasis

“…6 Furthermore, esophageal NECs might be discovered incidentally during endoscopic examinations. 2 Esophageal tumors can be divided based on their locations into upper (15-24 cm from the incisor), middle (24-32 cm from the incisor), and lower (32-40 cm from the incisor). 10 One study found that esophageal NECs are mostly found in the lower esophagus.…”