Does Achalasia Predispose to Cancer of the Esophagus?* S~ren Aggestrup, M.D., F.C.C.E; lens Christian Holm, B.Se.; and Hans Rahbek S~rensen, M.D., F.C.C.R
In a follow-up study of 147 patients with achalasia of the esophagus treated by myotomy, 146 patients were traced (58 female and 88 male patients aged 4 to 83 years [median, 46 years]). The living persons were contacted in writing or by telephone. The mean follow-up time after the operation was 23.2 years (range, 6 to 41 years). The cause of death was established for 71 patients. There were three postoperative deaths and two deaths following recurrence. In comparison with the Danish population, the 66 remaining
hundred years ago, the association between O neachalasia and cancer of the esophagus was de-
scribed, and in 1969, Just-Viera and Haight! found 167 instances suitable for review. The reported incidence was between 1 and 20 percent, but most series were small. The largest series \\'as described by Ellis, 2 who in a follow-up of 85 patients found that of 24 patients with an established cause ofdeath, seven died ofesophageal cancer. A recent reappraisal questions the concept of a connection between achalasia and esophageal carcinoma, and a follow-up study of 100 patients \vith achalasia disclosed no instances of cancer of the esophagus. 3 However, it is our opinion that the design and statistical evaluation of this study is inadequate for the theory to be discarded. We therefore made a followup of our 147 patients with achalasia operated on by a myotomy. MATERIAL AND METHOD
The study is a follo\\'-up of 147 patients. The criteria for entering patients in the study were that they had diagnosed cardiospasm or achalasia of the esophagus and a history of myotomy. One hundred forty-seven patients who satisfied the criteria were treated in the period 1949 to 1984 at the departments of the University Hospital, Rigshospitalet, in Denmark. One patient living abroad \\'as not found for the follow-up. All the other patients have been traced. There were 58 female and 88 male patients aged 4 to 83 years
(median age, 46 years; mean age, 48.9 years). Seventy-five patients are still alive and have been contacted in \\'riting or by telephone. Seventy-one have died and we have obtained information on the causes of death from death certificates,
*From the Department of Thoracic Surgery, University Hospital Rigshospitalet, Copenhagen, Denmark. Manuscript received October 21; revision accepted January 31. Reprint requests: Dr. Aggestrup, Department of Thoracic Surgery, Rigshospitalet, 2100 Copenhagen 0, DennUlrk
patients were found to have a relatively higher cancer mortality (33.8 percent). Contrary to the expected less than one, ten of 23 patients who died of cancer had a malignant tumor in the esophagus. The mortality rate after 30 years was 66.1 percent, 11.9 percent of the deaths caused by esophageal cancer. It is concluded that there is a connection between achalasia and cancer of the esophagus that ought to be considered in the treatment and follow-up of patients with achalasia. (Chest 1992; 102:1013-16) where possible supplemented by medical records, autopsy reports, histologic examinations, and reports from general practitioners. Twenty-seven patients had had an autopsy. The follow-up time has been calculated from the date ofoperation. The mean follow-up time was 23.2 years (range, 6 to 41 years), \\,hich is equivalent to 3,390 observation years in 146 patients. The time interval from the first symptom to the operation was mentioned in 143 records but was not exact in all cases; it ranged from nil to 45 years \\'ith a median value of more than three and less than four years. Statistics Survival has been calculated by the actuarial method and contingency tables have been evaluated by the x2 test. P values of p>0.05). Table 2 shows the different sites of the malignant tumors. Ten of 23 patients who died of cancer had a malignant tumor in the esophagus. Table 3 provides information about those ten patients. The data on the size of the esophagus derive from the radiologists' reports; roentgenograms were not available. The interval from the time ofthe operation to the occurrence of death was longer than 19 years for patients younger than 60 years. During the years 1970 to 1979, the number of new cases of esophageal cancer in Denmark per year was 120 among male patients and 62 among female patients. 6 The five-year survival is less than 5 percent, so almost 180 patients die of cancer of the esophagus each year. Cancer of the esophagus accounted for about 1.5 percent of all cancer deaths in the above period, 1.7 percent in 1965 and 1.2 percent in 1985 (see table 1). As a consequence, the expected number of deaths caused by esophageal cancer among our 66 patients is less than one (0.33). The difference between the expected "less than one patient" and the observed ten patients is highly significant (X2 710.0, p
In a follow-up study of 147 patients with achalasia of the esophagus treated by myotomy, 146 patients were traced (58 female and 88 male patients aged 4 to 83 years [median, 46 years]). The living persons were contacted in writing or by telephone. The mean follow-up time after the operation was 23.2 years (range, 6 to 41 years). The cause of death was established for 71 patients. There were three postoperative deaths and two deaths following recurrence. In comparison with the Danish population, the 66 remaining
hundred years ago, the association between O neachalasia and cancer of the esophagus was de-
scribed, and in 1969, Just-Viera and Haight! found 167 instances suitable for review. The reported incidence was between 1 and 20 percent, but most series were small. The largest series \\'as described by Ellis, 2 who in a follow-up of 85 patients found that of 24 patients with an established cause ofdeath, seven died ofesophageal cancer. A recent reappraisal questions the concept of a connection between achalasia and esophageal carcinoma, and a follow-up study of 100 patients \vith achalasia disclosed no instances of cancer of the esophagus. 3 However, it is our opinion that the design and statistical evaluation of this study is inadequate for the theory to be discarded. We therefore made a followup of our 147 patients with achalasia operated on by a myotomy. MATERIAL AND METHOD
The study is a follo\\'-up of 147 patients. The criteria for entering patients in the study were that they had diagnosed cardiospasm or achalasia of the esophagus and a history of myotomy. One hundred forty-seven patients who satisfied the criteria were treated in the period 1949 to 1984 at the departments of the University Hospital, Rigshospitalet, in Denmark. One patient living abroad \\'as not found for the follow-up. All the other patients have been traced. There were 58 female and 88 male patients aged 4 to 83 years
(median age, 46 years; mean age, 48.9 years). Seventy-five patients are still alive and have been contacted in \\'riting or by telephone. Seventy-one have died and we have obtained information on the causes of death from death certificates,
*From the Department of Thoracic Surgery, University Hospital Rigshospitalet, Copenhagen, Denmark. Manuscript received October 21; revision accepted January 31. Reprint requests: Dr. Aggestrup, Department of Thoracic Surgery, Rigshospitalet, 2100 Copenhagen 0, DennUlrk
patients were found to have a relatively higher cancer mortality (33.8 percent). Contrary to the expected less than one, ten of 23 patients who died of cancer had a malignant tumor in the esophagus. The mortality rate after 30 years was 66.1 percent, 11.9 percent of the deaths caused by esophageal cancer. It is concluded that there is a connection between achalasia and cancer of the esophagus that ought to be considered in the treatment and follow-up of patients with achalasia. (Chest 1992; 102:1013-16) where possible supplemented by medical records, autopsy reports, histologic examinations, and reports from general practitioners. Twenty-seven patients had had an autopsy. The follow-up time has been calculated from the date ofoperation. The mean follow-up time was 23.2 years (range, 6 to 41 years), \\,hich is equivalent to 3,390 observation years in 146 patients. The time interval from the first symptom to the operation was mentioned in 143 records but was not exact in all cases; it ranged from nil to 45 years \\'ith a median value of more than three and less than four years. Statistics Survival has been calculated by the actuarial method and contingency tables have been evaluated by the x2 test. P values of p>0.05). Table 2 shows the different sites of the malignant tumors. Ten of 23 patients who died of cancer had a malignant tumor in the esophagus. Table 3 provides information about those ten patients. The data on the size of the esophagus derive from the radiologists' reports; roentgenograms were not available. The interval from the time ofthe operation to the occurrence of death was longer than 19 years for patients younger than 60 years. During the years 1970 to 1979, the number of new cases of esophageal cancer in Denmark per year was 120 among male patients and 62 among female patients. 6 The five-year survival is less than 5 percent, so almost 180 patients die of cancer of the esophagus each year. Cancer of the esophagus accounted for about 1.5 percent of all cancer deaths in the above period, 1.7 percent in 1965 and 1.2 percent in 1985 (see table 1). As a consequence, the expected number of deaths caused by esophageal cancer among our 66 patients is less than one (0.33). The difference between the expected "less than one patient" and the observed ten patients is highly significant (X2 710.0, p
![[Therapy of esophagus achalasia].](/assets/img/hold.png)
