Between 1980 and 1988, we treated 1,521 patients with squamous cell carcinoma of the esophagus and 336 patients with adenocarcinoma of the gastric cardia. Between 1967 and 1988, 244 patients with esophageal achalasia were also observed. Among 1,857 patients with cancer, achalasia was present in 21 cases (1.1%). In 18 patients the mean and median interval between the diagnosis of achalasia and cancer was 11.5 and 8 years, respectively. In 3 cases achalasia was detected during the work-up for esophageal cancer. The previous treatment for achalasia administered elsewhere was as follows: balloon dilatation in 6 cases, myotomy and Nissen repair in 2, and distal esophageal resection in 1. Thirteen patients (61.9%) underwent resection, resulting in 1 postoperative death, and a mean and median survival of 23.3 and 13 months, respectively. Push intubation was performed in 4 cases, chemotherapy in 2, a by-pass procedure in 1, endoscopic Nd:YAG laser in 1, while 1 further patient did not receive any treatment for the carcinoma, but only balloon dilatation of the LES. The mean follow-up of the 244 patients with primary esophageal achalasia was 44.6 months (range 1-108), and only 1 patient developed an esophageal cancer, giving an incidence of 18.6 cases per 100,000 per year.
Conclusions: in our experience, achalasia is present in a minority of patients with esophageal cancer, and larger prospective controlled trials are needed to assess the true incidence of malignant degeneration in the achalasic patient.