S2096 Development of Achalasia in Chronic Intestinal Pseudo-Obstruction (CIPO)

Abstract

Introduction: Neuroendocrine carcinoma (NEC) of the esophagus is a rare malignancy and represents approximately 0.4-2% of all esophageal cancer. Histologically, two types of NEC of the esophagus have been described: small cell NEC and large cell NEC. Small cell NEC is the more common type, comprising almost 95% of all NEC of the esophagus. Case Description/Methods: We present a 52-year-old Hispanic male with 30 pack year smoking history and chronic alcohol abuse presented with sore throat and worsening dysphagia to both liquids and solids for 3 weeks. Physical examination revealed anterior cervical swelling which was tender to palpation. The oropharynx looked moist with mild tonsillar erythema. Complete blood chemistry showed mild leucocytosis. COVID-19 PCR and streptococcal Ag test came back negative. The computer tomography (CT) of the neck with contrast revealed an esophageal mass at the level of T1- T2 with paratracheal extension associated with enlarged paratracheal lymph nodes. The endoscopic evaluation revealed a large bloody, exophytic, friable, and necrotic mass in the oropharynx which was partially obstructing the airway. A large, circumferential, ulcerating and partially obstructing mass was found in the proximal esophagus. Immunohistochemistry was positive for synaptophysin and chromogranin and negative for CK5/6 and p63. KI-67 was greater than 90%. The biopsies were consistent with a neuroendocrine malignancy. The decision was made to start him on cisplatin, etoposide with the addition of Durvalumab and radiation therapy. Discussion: Neuroendocrine tumors of the esophagus have an aggressive nature, with no current standard treatment guidelines. From the limited literature available, The treatment strategy is dictated by the extent of disease with surgery being the preference in limited disease and a combination of chemotherapy, immunotherapy, and radiation therapy for the extended disease. The most commonly used chemotherapy is cisplatin with etoposide and the addition of immunotherapy, durvalumab. An alternative chemotherapy regimen that may be used if patients exhibit intolerance to cisplatin includes carboplatin with etoposide and the addition of durvalumab. In our case, we used cisplatin, Etoposide, Durvalumab and radiotherapy which is protocol for small cell carcinoma of lung.Although many recent advances were made in NEC management, there are no definitive guidelines. As of now, despite all aggressive measures, NEC of the esophagus has a poor overall prognosis.