PD05.01. Unveiling the Risk of Esophageal Squamous Cell Carcinoma in Achalasia: End-Stage Disease Carries a Markedly Higher Prevalence and Incidence

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Abstract Topic Esophageal Cancer: Other Background Esophageal achalasia is a rare motility disorder associated with an increased risk of esophageal squamous cell carcinoma (ESCC), with reported prevalence ranging from 0.4% to 9.2%. The risk is particularly elevated in end-stage disease, characterized by longstanding stasis and sigmoidal-shaped megaesophagus, and is not always mitigated by surgical myotomy. Despite this, current international guidelines do not recommend routine endoscopic surveillance. We report data from a single-center cohort of patients undergoing laparoscopic Heller-Dor (LHD) for end-stage achalasia, alongside a systematic review and meta-analysis aimed at estimating the overall ESCC risk in achalasia, with emphasis on advanced disease. Methods All consecutive patients with radiologically confirmed end-stage achalasia treated with LHD at the University of Padova (2000–2025) were included. Preoperative assessment consisted of endoscopy, barium swallow, and manometry. Postoperative follow-up included endoscopy one year after surgery and every two years thereafter, aimed at detecting malignant degeneration. Treatment failure was defined as an Eckardt score ≥3 or the need for retreatment. A systematic review and meta-analysis of the literature was also performed according to PRISMA guidelines. Results Among 1,782 patients who underwent LHD for esophageal achalasia, 115 presented with radiological stage IV (M:F 68:47; median age 55 years, IQR 42–63). After a median follow-up of 8.2 years (IQR 5.1–15.6), symptom relief was achieved in 85% of cases. Four patients (3.5%) developed ESCC, three of whom more than 10 years after surgery; only one case was diagnosed at an early stage. One patient developed cancer after failed surgical outcome with persistent dysphagia. In contrast, no ESCC was observed in the remaining 1,667 patients with stage I–II–III achalasia (M:F 937:730; median age 49 years) after a median follow-up of 10.2 years (IQR 7.4-12.3). The systematic review confirmed an overall ESCC prevalence of ~2%, with an incidence of 173/100,000 patient-years. Stratified analysis showed a markedly higher risk in end-stage disease (724 vs 114/100,000 patient-years; rate ratio 6.34, 95% CI 3.24–12.38). Conclusion Our single-center experience, supported by meta-analysis data, demonstrates a significantly increased risk of ESCC in patients with end-stage achalasia, persisting even after surgical treatment. These findings highlight the need for targeted postoperative endoscopic surveillance focused on malignant degeneration in this high-risk subgroup. Further prospective studies are warranted to define evidence-based surveillance strategies.