Esophageal Neuroendocrine Carcinoma Presenting as a Rare Cause of Dysphagia

Abstract

Introduction: Neuroendocrine carcinomas comprise 0.4-2.0% of all esophageal malignancies and are a rare cause of dysphagia. The prognosis is generally poor as the tumor is usually advanced at the time of symptomology and diagnosis. The following is a case of a 60-year-old male patient with solid food dysphagia found to have a large, friable, gastroesophageal junction tumor during esophagogastroduodenoscopy (EGD). Biopsies confirmed a high-grade neuroendocrine carcinoma with a Ki-67 (proliferative index) of 90%.Figure: Frond-like esophageal tumor seen in the distal esophagus.Figure: Large ulcerated tumor extending into the gastric cardia, retroflex view.Figure: Neuroendocrine carcinoma consisting of a poorly differentiated, high-grade malignant neoplasm of intermediate to large cells with a trabecular architecture resembling neuroendocrine tumor within gastric mucosa (magnification 100x).Case: A 60-year-old male presented with a six-month history of daily solid food dysphagia and a 10lb intentional weight loss. His past medical history was significant for stage 1 squamous cell carcinoma of the larynx diagnosed four years prior and treated with chemotherapy. He had a 30 pack-year smoking history and continued to smoke half a pack of cigarettes daily. His only medication was an ipratropium/albuterol 20-100mg inhaler for chronic obstructive pulmonary disease. EGD demonstrated a large frond-like villous tumor occupying 50% of the circumference of the distal third of the esophagus extending 3cm proximal of the Z-line (figure 1). There was a pseudo lumen within the tumor that led into the stomach, and on retroflexed view there was a 5 cm ulcerated tumor extending into the cardia (figure 2). Biopsy results showed squamous and oxyntic-gastric mucosa containing sheets and packets of pleomorphic cells (figure 3). The cells demonstrated variable immunohistochemical positivity for synaptophysin, chromogranin and CD56. Immunohistochemistry for Ki-67 revealed a proliferation index of greater than 90%, classifying the tumor as Grade 3 or high-grade neuroendocrine carcinoma, denoting poor prognosis per the current National Comprehensive Cancer Network guidelines. Subsequent computed tomography supported findings of a 7.1 x 6.8 x 4.8 cm gastroesophageal junction mass with prominent regional lymph nodes without distant metastasis. The patient was treated with neoadjuvant chemotherapy of cisplatin, etoposide, and dexamethasone and concurrent radiation therapy prior to surgical resection. Discussion: Esophageal neuroendocrine tumors are a very rare cause of dysphagia and represent 0.4-2.0% of all esophageal malignancies. They are not well known by gastroenterologists and present as a mimicker of esophageal adenocarcinoma. Poorly differentiated (high-grade) extrapulmonary neuroendocrine carcinomas are not well-defined or documented frequently within the medical literature. National cancer guidelines suggest a treatment similar to small-cell lung cancer with platinum-based chemotherapy and radiation therapy either as neoadjuvant therapy or palliative therapy if metastatic disease is present. Generally, there is a poor prognosis of esophageal neuroendocrine tumors due to the advanced nature at diagnosis, but in the absence of metastatic disease, are treated with curative intent.