From the Guidelines
Achalasia is a significant risk factor for developing esophageal squamous cell carcinoma (ESCC), with a 5-fold higher risk compared to the general population. The relationship between achalasia and the risk of developing ESCC is well-established, with chronic inflammation and stasis of food in the esophagus leading to repeated irritation of the esophageal lining, bacterial overgrowth, and fermentation, producing potentially carcinogenic compounds that damage the esophageal mucosa 1. Key factors that contribute to this increased risk include:
- Duration of symptoms
- Degree of food retention
- Chronic inflammation and stasis of food in the esophagus Patients with achalasia should be aware of the risk of oesophageal cancer, and endoscopic surveillance should be strongly considered for all patients to monitor for disease progression and to identify potential complications from asymptomatic GERD 1. Although specific intervals for endoscopy have not been established, a surveillance plan can help tailor interventions that may delay or thwart progression to end-stage achalasia. The goal of implementing a surveillance plan is to ideally monitor for structural changes, clearance of the esophagus, and mucosal changes, and to identify potential complications early on 1.
From the Research
Risk Factors for Esophageal Squamous Cell Carcinoma (ESCC)
The relationship between achalasia and the risk of developing esophageal squamous cell carcinoma (ESCC) is supported by several studies.
- Achalasia is associated with an increased risk of esophageal carcinoma, including ESCC 2.
- The incidence of ESCC in achalasia patients is significantly higher than in the general population, with a reported incidence of 312.4 cases per 100,000 patient-years at risk 2.
- A study of 241 achalasia patients found that 9 developed carcinoma, with a prevalence of 3.7% and an incidence of 88 per 100,000 population, which is 14.5 times that of the age-adjusted and sex-adjusted general population 3.
- Another study found that patients with achalasia have an excess risk of developing both squamous cell carcinoma and adenocarcinoma of the esophagus, with a significantly greater risk than the general population 4.
- The risk of ESCC in achalasia patients is estimated to be 50 times higher than in the general population, with the disease manifesting 20-25 years after achalasia symptom onset 5.
- A matched cohort study using the nationwide Veterans Affairs Achalasia Cohort found that individuals with achalasia had a 5-fold higher risk of esophageal cancer, including ESCC, compared to those without achalasia 6.
Comparison of Options
Based on the evidence, achalasia (option c) is a risk factor for esophageal SCC.
- Barrett's esophagus (option a) is a risk factor for esophageal adenocarcinoma, but not specifically for ESCC.
- GORD (option b) is a risk factor for esophageal adenocarcinoma, but its relationship with ESCC is not as well established as that of achalasia.