Chagasic Megaesophagus–Associated Carcinoma: Clinical Pattern and Outcomes

Abstract

PURPOSE: Approximately 4% to 10% of patients diagnosed with Chagas-induced megaesophagus disease develop esophageal carcinoma. However, the natural history and clinical pattern of this entity are not well described. METHODS: Herein, we retrospectively analyzed 593 patients with esophageal carcinoma treated at a single Brazilian institution. We identified 32 patients with Chagas disease, of whom 11 had megaesophagus. The epidemiologic profile and oncological treatment outcomes were evaluated. RESULTS: Although baseline characteristics were similar among the three groups, patients with Chagas megaesophagus–associated carcinoma (CMAC) presented with a lower rate of smoking. This factor reinforced the concept that achalasia is the predominant risk factor for cancer development. The CMAC group had a higher rate of tumor in situ (two of 11 patients) compared with the other groups. These patients were treated with endoscopic resection, and no recurrence was detected. Eight of 11 patients with CMAC were diagnosed with locally advanced disease. Patients with locally advanced CMAC presented with a median progression-free survival of 7.8 months and a median overall survival of 9.1 months. CONCLUSION: If CMAC is not promptly detected, it has a dismal prognosis, indicating that a high index of suspicion of esophageal carcinoma is required for patients with Chagasic megaesophagus. Additional studies are needed to improve the surveillance and treatment approaches for this neglected disease.