Digestive Diseases and Sciences, Vol. 35, No. 12 (December 1990), pp. 1549-1552

CASE REPORT

Esophageal Adenocarcinoma in a Patient with Surgically Treated Achalasia

PHILIP GOODMAN, MD, LARRY D. SCOTT, MD, REGINA R. VERANI, MD, and CHARLES C. BERGGREEN, MD

KEY WORDS: achalasia; adenocarcinoma; Barrett's esophagus; esophagomyotomy; esophagus.

Esophageal adenocarcinoma is a recognized se- quela of Barrett's esophagus, an acquired epithelial metaplasia associated with chronic reflux esophagi- tis (1). Gastroesophageal reflux may complicate surgical treatment of achalasia with the Heller myotomy in about 18-31% of patients so treated (2, 3). As such, these patients also would be at in- creased risk for development of Barrett's esopha- gus and, in turn, adenocarcinoma. There are few reports documenting this occurrence, however. Squamous cell carcinoma of the esophagus may occur with increased frequency in the patient with achalasia, but this is thought to follow the esopha- gitis that can occur in these patients from retention and stasis of secretions, ingested food, and other potentially noxious compounds (4-6). We report here a patient who underwent a Heller myotomy for achalasia 17 years before he presented with re- newed dysphagia secondary to esophageal adeno- carcinoma arising in a segment of Barrett's esoph- agus.

CASE REPORT

A 54-year-old man presented with increasing dyspha- gia. He had undergone Heller myotomy for achalasia 17 years previously. Recurrent symptoms prompted a sec- ond myotomy five years later. He did well until two to three years prior to admission when dysphagia recurred, becoming complete for solids and partial for liquids. The

patient noted a 50-pound weight loss over the past six months.

An esophagram showed marked dilatation of the esophagus proximal to a nodular filling defect at the gastroesophagealjunction (Figure 1). At endoscopy, the esophagus was dilated and atonic; a large amount of liquid and debris was removed by lavage. There was a 4-cm friable multinodular circumferential mass in the most distal portion of the esophagus with the squamo- columnar junction visualized proximal to the mass. The endoscope was passed through the lesion into the stom- ach without much difficulty and on a retroflexed view the mass could not be seen in the stomach. Multiple biopsies of the mass revealed a moderately well differ- entiated adenocarcinoma. Computed tomography (CT) of the chest and abdomen confirmed the findings on

Manuscript received May 21, 1990; revised manuscript re- ceived July 27, 1990; accepted July 30, 1990.

From University of Texas Medical Branch, Department of Radiology, Galveston, Texas 77550; University of Texas Medi- cal School at Houston, Department of Internal Medicine, Divi- sion of Gastroenterology, Houston, Texas 77030; and University of Texas Medical School at Houston, Department of Pathology, Houston, Texas 77030.

Address for reprint requests: Dr. P. Goodman, Department of Radiology, University of Texas Medical Branch, Galveston, Texas 77550-2780.

Fig 1. Upright film of the distal esophagus shows marked esophageal dilatation proximal to an irregular nodular filling defect (arrows). Multiple surgical clips are noted.

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Fig 2. CT at the level of the gastroesophageal junction shows a lobulated mass (curved arrows) almost obliterating the esophageal lumen (arrow).

esophagram and showed no evidence of metastasis (Figure 2).

An esophagectomy and gastric pull-through proce- dure were performed. Pathologic examination of the esophagus showed a fungating neoplasm measuring 4 x 3 x 2 cm, involving almost the entire circumference of the distal esophagus. Histological examination showed a moderate to poorly differentiated adenocarcinoma with a focal signet ring cell pattern (Figure 3). The adenocarcinoma extended focally through the mus cular wall into the adjacent fibroadipose tissue and involved the gastroesophageal junction. Gastric epithe- lium just below the gastroesophageal mucosal junction showed columnar epithelium with a villiform surface

and scattered goblet cells within glands (Figure 4); these changes were felt to be typical of Barrett's esoph- agus.

Postoperatively, the patient's condition gradually dete- riorated, He developed increasing respiratory decompen- sation and showed signs of sepsis, which remained unre- sponsive to multiple antibiotics. A water-soluble contrast study of the gastric pull-through did not reveal a leak, and several CT scans of the chest and abdomen showed no abnormal fluid collections. Exploratory surgery 12 days after esophagogastrectomy failed to reveal a source of sepsis. The patient died two weeks later secondary to multiple organ system failure, An autopsy was not per- formed.

Fig 3. Light microscopy of the esophageal tumor showing neoplastic cells focally arranged in a glandular pattern. Normal esophageal mucosa is observed (arrow). Hematoxylin and eosin, x 100.

Fig 4. Section from esophagus showing the normal stratified squamous epithelium (left) and the specialized columnar epithe- lium. The mucosal glands show goblet cells (arrows); chronic inflammation is also observed. Hematoxylin and eosin, 100.

1 550 Digestive Diseases and Sciences, Vol. 35, No. 12 (December 1990)

ADENOCARCINOMA AND ACHALASIA

DISCUSSION

Primary achalasia is an esophageal motility dis- order caused by degeneration of the intrinsic auto- nomic nerves in the wall of the esophagus. This results in decreased or absent peristalsis in the smooth muscle portion of the esophagus and failure of complete relaxation of the lower esophageal sphincter (LES) (5). Dysphagia is the most common symptom and usually presents in the third or fourth decade of life. Esophagram typically shows a di- lated nonpropulsive esophagus, which tapers smoothly at its most distal portion (7). The current treatment of achalasia is to disrupt or tear the smooth muscle comprising the LES, either by forcefully stretching it with a pneumatic dilator or by performing a surgical myotomy (3). While sphincter function does not improve, the lowered basal pressure in the LES provides less resistance to passage of a swallowed bolus.

A delayed but important complication of achala- sia is the development of squamous cell carcinoma. This occurs in approximately 3-7% of patients and presumably represents malignant degeneration sec- ondary to chronic stasis of food and retention esophagitis. Squamous cell carcinoma is usually detected many years after the onset of achalasia with an average range of 17-28 years. This may involve any segment of the esophagus, although the upper and middle thirds are most often affected. Prognosis is poor since the tumor is often quite large before dysphagia occurs. Squamous cell carcinoma also has been reported in cases where esophageal dilatation and food retention persisted following sur- gical myotomy for achalasia (4-6, 8, 9).

Although most esophageal carcinomas complicat- ing achalasia are squamous cell type, adenocarci- noma associated with Barrett's esophagus has been reported following surgical myotomy for achalasia (2, 10). Since myotomy reduces basal pressure in the LES, as already noted, the barrier to acid reflux is also reduced; decreased or absent clearance of refluxed material resulting from loss of propulsive motility in the esophageal body also occurs. Thus, postmyotomy gastroesophageal reflux and reflux esophagitis may be responsible for the development of Barrett's esophagus and, in turn, adenocarci- noma in patients with surgically treated achalasia (2, 10-13). Indeed, reflux and subsequent esopha- gifts are common late complications of surgical myotomy for achalasia, occurring in about 18-31% and 5-11% of patients, respectively (2, 3). Numer-

ous studies have concluded that chronic reflux esophagitis can cause Barrett's esophagus, which represents metaplasia of the normal esophageal squamous epithelium to an intestinal-like columnar epithelium. In the general population, this occurs in about 10% of patients with reflux esophagitis. Adenocarcinoma arises in areas of Barrett's epithe- lium in about 7-15% of such patients (1, 14). Al- though antireflux procedures may be performed in combination with myotomy in order to decrease the incidence of reflux in patients following myotomy, these procedures have had variable results and their use is controversial (3).

As with squamous cell carcinoma developing in achalasia, detection of adenocarcinoma may be difficult. Since significant esophageal dilatation may persist despite surgical myotomy, symptoms of obstruction secondary to tumor occur relatively late and prognosis is usually poor. Radiographic and endoscopic diagnosis of an esophageal tumor may be easily obscured by large amounts of retained food and fluid in the dilated esophagus, making it essential to lavage the esophagus prior to examina- tion (10).

Primary or metastatic adenocarcinoma of the gastric cardia may produce an achalasia-like picture with radiographic, endoscopic, and manometric similarities (7, 15). However, achalasia in this pa- tient was clinically typical and was first diagnosed 17 years previously. Moreover, the specialized co- lumnar epithelium from which this tumor arose is characteristic of Barrett's esophagus (16). Thus, adenocarcinoma should be considered in patients with achalasia who present with recurrent dyspha- gia many years after myotomy.

SUMMARY

Although squamous cell carcinoma of the esoph- agus occurs with increased incidence in primary achalasia, esophageal adenocarcinoma has been considered rare in this condition. We report a patient with long-standing achalasia in whom adenocarcinoma of the esophagus occurred many years after Heller esophagomyotomy, presumably related to Barrett's esophagus complicating gastro- esophageal reflux disease.

REFERENCES

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11. Kortan P, Warren RE, Gardner J, Ginsberg RJ, Diamant NE: Barrett's esophagus in a patient with surgically treated achalasia. J Clin Gastroenterol 3:357-360, 1981

12. Agha FP, Keren DF: Barrett's esophagus complicating achalasia after esophagomyotomy. A clinical, radiologic, and pathologic study of 70 patients with achalasia and related motor disorders. J Clin Gastroenterol 9:232-237, 1987

13. Feczko PJ, Ma CK, Halpert RD, Batra SK: Barrett's meta- plasia and dysplasia in post myotomy achalasia patients. Am J Gastroenterol 78:265-268, 1983

14. Agha FP: Progression of Barrett's mucosa to adenocarci- noma after antireflux surgery: Radiologic-pathologic corre- lation. Dysphagia 2:117-122, 1987

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