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INTRODUCTION:Neuroendocrine carcinoma (NEC) of the esophagus is a rare, poorly understood disease with high mortality rates. We present a de novo case of NEC of the esophagus responsive to multi-modality therapy facilitated by early endoscopic evaluation and clinical suspicion. Our objective is to raise awareness on rarer causes of dysphagia in order to improve time to treatment and patient outcomes.CASE DESCRIPTION/METHODS:A 63-year-old male with a past medical history significant for GERD and tobacco use, presented with a complaint of a one month history of progressive dysphagia to solids. He denied any history of presenting symptoms and had no prior endoscopic examination in the past. Vitals, laboratory workup, and physical exam including chest and abdominal exam were normal. In the setting of progressive dysphagia the patient underwent Esophagogastroduodenoscopy (EGD) revealing a distal esophageal mass (Figure 1). Subsequent Computed Tomography (CT) and Positron Emission Tomography (PET) scan, showed an esophageal mass approximately 4.7 cm x 8.5 cm without any distant metastasis (Figure 2). Biopsy revealed poorly differentiated, discohesive cells indicative of malignant, high-grade neoplasm with neuroendocrine features. Immunohistochemical testing was positive for CD56, synaptophysin, AE1/AE3, and CK8/18. Our patient was referred to a multidisciplinary team and underwent local radiation and chemotherapy of Cis-platinum and Etoposide with excellent response. Surveillance EGD with random biopsies and PET-CT following treatment at 1 year from diagnosis showed no evidence of residual malignancy (Figure 3). However, surveillance EGD at year 2, showed irregular Z lines and biopsy revealed small nests of atypical cells with neuroendocrine features. PET-CT and EUS were negative for any metabolically active neoplasm, and the patient declined further treatment.DISCUSSION:Esophageal NEC is rare with only 0.4 and 2% of all diagnosed NETs being found in the esophagus. Our case presents a patient with limited disease surviving past the median survival time of 17-23 months. Our patient was responsive to local radiation and Cis-platinum and Etoposide management. However, surveillance EGD two years after initial diagnosis showed recurrent malignancy. Poor patient outcomes and the limited number of reported cases have made it difficult to define first-line therapeutic strategies. Albeit rare, increased clinical suspicion and early endoscopic evaluation can hasten time to treatment and improve patient outcomes.