Hemet Global Medical Center Hemet, CA, United States
Sasank Nakka, MD Hemet Global Medical Center, Hemet, CA
Introduction: Neuroendocrine carcinoma (NEC) of the esophagus is a rare and highly aggressive disease with a reported incidence between 0.4% and 2% among all malignancies of the esophagus. The prognosis of NEC of the esophagus is poor, as the tumor is often at advanced disease at diagnosis. The therapeutic strategy for NEC of the esophagus has not been well defined due to the small number of cases reported in the literature thus far.
Case Description/Methods: Three patients presented to a rural community hospital and/or an affiliated outpatient gastroentereology private practice office with chief complaints of dysphagia and weight loss. "Patient 1" was a 67 year old male with history of hypertension, obesity, smoking, and NSAID use. Presenting symptoms were a 6 week history of epigastric abdominal pain, GERD, and dysphagia with weight loss. EGD with biopsy showed Gastric fundus tumor and esophageal tumor Grade 3 neuroendocrine carcinoma, small cell type. "Patient 2" was a 76 year old male non-smoker with history of hypertension, GERD and barrett's esophagus who presented with dysphagia and 40lb weight loss. EGD with biopsies showed Invasive high-grade neuroendocrine carcinoma of the gastric cardia/fundus and esophagus. "Patient 3" was a 70 year old male non-smoker with history of obesity and underlying Barrett's esophagus with repeated endoscopies showing non-dysplasia. However, he presented approximately six years later with worsening dysphagia. Repeat EGD with biopsy showed progression to neuroendocrine carcinoma of the esophagus. In all three cases the patients underwent surgical resection and/or chemotherapy or radiation. Despite all efforts and treatments, all three succumbed to their illness.
Discussion: The cellular origin of NEC of the esophagus remains unknown. Although the therapeutic strategy for NEC of the esophagus is still not established, it is important to precisely diagnose it because its treatment might be completely different from that of esophageal squamous cell carcinoma and adenocarcinoma. Furthermore, the co-existence of squamous cell carcinoma or adenocarcinoma further complicates treatment. The standard therapy for NEC of the esophagus is not standardized, although the multi-modality treatments including surgery, chemotherapy and radiotherapy are recommended.
In conclusion, further study may be important for clarifying the molecular mechanisms underlying NEC of the esophagus, as well as studies for establishing the optimal treatment for this rare and dismal disease.
Sasank Nakka indicated no relevant financial relationships.
Sasank Nakka, MD. P0361 - Three Rare Cases of Neuroendocrine Carcinoma of the Esophagus in a Rural Community, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.