Achalasia Patients at Increased Risk for Esophageal Cancer

Achalasia can cause food retention, leading to bacterial overgrowth, irritation, and chronic inflammation, a proposed pathophysiological process that may lead to squamous cell carcinoma (SCC). On the other hand, treatments for achalasia, including myotomy or dilation, can lead to GERD in 10% to 30% of patients. In turn, this can predispose patients to Barrett’s esophagus (BE), the precursor disease to esophageal adenocarcinoma (EAC). The aim of this long-term cohort study was to assess the risk of both squamous cell carcinoma and adenocarcinoma of the esophagus in patients with achalasia. 

In a single-center study in northern Italy, 566 patients (mean age, 48.1 years; 46% male) diagnosed with primary achalasia between January 1973 and December 2018 were followed for an average of 15.5 years following diagnosis. Of these, 450 (79.5%) patients underwent surgical myotomy with or without previous pneumatic dilation, 86 (15.2%) were treated with pneumatic dilation, and 30 (5.3%) were treated with oral medications. Upper endoscopy was performed at 1, 3, and 5 years after treatment and every 3 years thereafter. Random biopsy specimens were obtained in a 4-quadrant fashion every 3 cm above the gastroesophageal junction as well as from any suspicious lesions. Twenty patients developed esophageal cancer (SCC, 15; EAC, 5), providing an annual incidence rate of 0.24% (SCC, 0.18%; EAC, 0.06%). All 20 patients had been treated with surgical myotomy. The overall risk of esophageal cancer (both SCC and EAC) was significantly greater in patients with achalasia than the general population (standardized incidence ratio, 104.2; 95% confidence interval, 63.7-161).