Adenocarcinoma of the gastroesophageal junction presenting as achalasia

Primary achalasia of the is a disease of unknown etiology characterized by failure of the lower esophageal sphincter to relax in response to Mansour F. Maleki, M.D.* and the absence of peristaltic activity in Bertram Fleshier, M.D. the body of the esophagus. Occasionally, carcinoma Edgar Achkar, M.D. of the and other tumors involving the gastroesophageal junctional area may have the pre- Department of senting symptoms of achalasia.1"6 It may be difficult to differentiate between primary and secondary Khalil Sheibani, M.D. achalasia, but it is important from the standpoint Division of Laboratory Medicine of selecting appropriate therapy. We report two cases of adenocarcinoma of the stomach presenting initially as achalasia. Case 1. A 36-year-old black male was admitted with a 3- to 4-week history of intermittent accom- panied by vague substernal pain. The dysphagia was initiated by both solid foods and liquids. Cold liquids were particularly difficult to swallow. There was no history of , vomiting, , or between meals. A laparotomy had been performed 12 years earlier for a gunshot wound of the abdomen, but there were no other unusual events in the history. The physical examination was unremarkable. A bar- ium meal showed dilatation of the entire esophagus with distal narrowing and delay of the passage of barium. The * Associate Professor of Medicine Uni- end of the esophagus was tapered and the mucosa ap- versity of Isfahan, Iran. International peared normal (Fig. I). The roentgenographic diagnosis Scholar, The Cleveland Clinic Foun- was achalasia. On endoscopic examination, the esopha- dation. gus was dilated and no peristaltic movements were ob- 137

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Fig. 1. Barium meal showing dilatation of the esophagus with a tapered narrowing at the esophagogastric junction (case 1). Roentgenographic diagnosis was achalasia. served. The esophagogastric junction was not found, because the neoplasm had mas- tight, but the instrument could be passed sively invaded the submucosa. Ganglion cells through to the stomach with slight pressure. within Auerbach's plexus were found with Retrograde views of the cardia revealed no difficulty, and most of the few identifiable abnormalities and the remainder of the collections demonstrated invasion and de- stomach and duodenum was normal except struction by malignant cells (Fig. 3). The for minimal hypertrophy of the gastric mu- patient survived 19 months after surgery. No cosa. The endoscopic diagnosis was achalasia autopsy was obtained. and hypertrophic . specimens Case 2. A 57-year-old black male had a obtained from sites at the esophagogastric history of an 18 kg (40 pound) weight loss in junction that appeared normal showed sig- the previous 5 months, beginning shortly net cell adenocarcinoma. A gastroesophageal after the death of his wife. Initially there was resection and pyloroplasty were performed. no anorexia. One month before admission, The resected segment of esophagus was esti- the patient noted that food was sticking mated at 5 cm during operation, but on substernally, anorexia had developed, and pathologic examination only 2 cm of esoph- regurgitation of food 30 to 60 minutes after agus was contained in the specimen and the a meal became increasingly frequent. These proximal line of resection was involved with symptoms were accompanied by epigastric tumor. pain at night. There was no history of gas- Histopathologic examination revealed a trointestinal symptoms. A barium meal poorly differentiated, highly invasive muci- showed a dilated esophagus with lengthen- nous adenocarcinoma with transmural in- ing and tapering of the distal end (Fig. 4). vasion of the gastroesophageal segment (Fig. The stomach was normal except for a de- 2). Ganglion cells of Meissner's plexus were formity of the distal antrum and pylorus.

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Fig. 2. Photomicrograph from the gastroesophageal junction demonstrating massive neoplastic infiltration (case 1) (hematoxylin and eosin stain, X400).

The roentgenographic diagnosis was achal- geographic areas, achalasia is produced asia with possible scarring from previous by the action of the neurotoxin of . At , the Trypanosoma cruzi. No other agents esophagus contained about 200 cc of thick, have been identified that produce such mucoid material. The esophagus was dilated degeneration. and the instrument could not be passed into Clinically and roentgenographically, the stomach. The patient underwent dilata- achalasia has been described in associ- tion with Maloney Bougies up to No. 48 ation with carcinoma involving the car- French. Pneumatic dilation was attempted, but was unsuccessful. A week later a second dioesophageal junction. In these cases, attempt at pneumatic dilation was also un- the question arises as to whether the successful and the patient was referred for preceded and produced the surgery. Carcinoma of the upper half of the achalasia or whether the cancer devel- stomach involving the gastroesophageal oped as a complication of achalasia. The junction was found. The tissue diagnosis was latter phenomenon is recognized as a adenocarcinoma of the signet cell type. No late and relatively unusual complication resection was done. The patient did well for in long-standing achalasia.8-10 It is not 4 months, bu't did not return for follow-up. possible to determine absolutely which Discussion disorder came first, but in the two cases Achalasia is a motor disorder of the described here, and other reported cases, esophagus caused by degeneration of the evidence supports the idea that car- the cells of Auerbach's plexus. In most cinoma was the primary lesion. The fac- cases, the cause is unknown. In certain tors favoring carcinoma as the primary

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Fig. 3. Photomicrograph demonstrating partial destruction of a residual ganglion cell collection hv invading malignant cells (arrows). Esophageal line of resection 12 mm proximal to the gastroesophageal junction (case 1) (periodic acid-Schiff stain, X400). lesion were the short duration of symp- as a cause of achalasia should be consid- toms (4 weeks and 5 months in our two ered, particularly in patients older than cases, respectively) and the relatively 50 years with a brief history and severe rapid progression of disease once it was weight loss. In our two patients the discovered. Idiopathic achalasia, by history was brief, but weight loss, at contrast, is a relatively indolent disor- least in the first patient, was not severe, der, which results in little increase in and the disease occurred at ages 36 and mortality. Recent onset of symptoms, 57 respectively. Others have reported however, occurs in as many as 12% of carcinoma with the picture of achalasia patients with idiopathic achalasia." in patients in their 30s.1'6 Thus carci- Thus a short history, although sugges- noma as a cause must be considered in tive, does not reliably differentiate a patient with achalasia at any age. achalasia apparently due to carcinoma Differentiation between idiopathic from the idiopathic form. achalasia and achalasia secondary to It has been suggested that carcinoma carcinoma of the cardia may be diffi-

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1, is difficult to observe. Biopsy is defin- itive when positive, but may not be helpful, since it is not unusual for an endoscopic biopsy to fail to identify neo- plastic material and to simply sample tissue from surrounding areas of necrosis or inflammation. The pathogenesis of the achalasia in adenocarcinoma of the cardia is not well defined. One possible mechanism pro- ducing the aperistalsis involves infiltra- tion of esophageal myenteric and nerve plexuses with neoplastic cells. In the first case reported here all identifiable areas of Auerbach's plexus were infiltrated and destroyed up to and including the line of resection. Similar results and speculations have been reported by oth- ers.4' 12 Some have rejected the mecha- nism mainly on the basis of failure to demonstrate such invasion in certain cases.1' How much destruction of Auerbach's plexus is needed to produce achalasia is unknown. It has been estimated that as

Fig. 4. Barium meal showing a dilated esophagus much as 90% destruction may be re- with lengthening and tapering at the distal end quired.1'5 Since serial sections and care- (case 2). Roentgenographs diagnosis was achala- ful counting of ganglion cells in cases of sia. carcinoma with achalasia have not been done, it is not possible to be definitive cult. Each of our patients had the typi- about direct myenteric invasion and the cal roentgenographic features of achal- pathophysiologic mechanism. asia although each had a neoplasm. A second possible mechanism con- Thus history, physical examination, and cerns the idea that achalasia may occur roentgenography may not establish the as a reaction to distal obstruction. Such diagnosis. Endoscopy with biopsy re- a hypothesis is suggested by reversal of mains the most helpful means of elimi- achalasia in a treated sarcoma14 and nating the diagnosis of carcinoma in a restoration of normal follow- patient with apparent achalasia. How- ing pneumatic dilatation in idiopathic ever, in the two cases reported here, and achalasia. However, achalasia is rare in other reported cases,1_i endoscopy ini- in the many instances of distal obstruc- tially failed to establish the diagnosis of tion due to cancer and stricture and true carcinoma. This may occur because the reversibility of idiopathic achalasia, carcinomatous lesion is not seen, the with the exception cited, is unknown. upper stomach is not well visualized, or Finally, it has been suggested that the carcinoma primarily is one that in- achalasia in cancer represents a periph- filtrates the submucosa and, as in case eral manifestation of cancer analogous

Downloaded from www.ccjm.org on October 2, 2021. For personal use only. All other uses require permission. 15 2 Cleveland Clinic Quarterly Vol. 46, No. 4 to the distant disorders of the central manifestation of carcinoma of the cardia. J and peripheral nervous system associ- Indian Med Assoc 60: 431-432, 1973. ated with tumors.3 No direct evidence 7. Ferreira-Santos R: Aperistalsis of the esopha- gus and colon ( and megaco- exists to support this hypothesis, and lon) etiologically related to Chagas' disease. achalasia has not been reported in can- Am J Dig Dis 6: 700-726, 1961. cers that do not directly involve the area 8. Ellis FG: The natural history of achalasia of of the esophagus or stomach. the cardia. Proc R Soc Med 53: 663-666, 1960. 9. Wychulis AR, Woolam GL, Andersen HA, et References al: Achalasia and carcinoma of the esophagus. 1. Tucker HJ, Snape WJ Jr, Cohen SC: Achal- JAMA 215: 1638-1641, 1971. asia secondary to carcinoma; manometric and 10. Just-Viera JO, Haight C: Achalasia and car- clinical features. Ann Intern Med 89: 315- cinoma of the esophagus. Surg Gynecol Ob- 318, 1978. stet 128: 1081-1095, 1969. 2. McCallum RW: Esophageal achalasia sec- 11. Lawrance K, Shoesmith JH: A review of the ondary to gastric carcinoma. Am J Gastroen- treatment of cardiospasm. Thorax 14: 211- terol 71: 24-29, 1979. 215, 1959. 3. Shulze KS, Goresky CA, Jabbari M, et al: 12. Kolodny M, Schräder ZR, Rubin W, et al: Esophageal achalasia associated with gastric Esophageal achalasia probably due to gastric carcinoma; lack of evidence for widespread carcinoma. Ann Intern Med 69: 569-573, plexus destruction. Can Med Assoc J 112: 1968. 857-864, 1975. 13. Cassella RR, Brown AL Jr, Sayre GP, et al: 4. Bessent CT, Lopez CA, Cocco AE: Case re- Achalasia of the esophagus; pathologic and port; carcinoma of the EG junction mimick- etiologic considerations. Ann Surg 160: 474- ing achalasia. Md State Med J 22: 47-50, 486, 1964. 1973. 14. Davis JA, Kantrowitz PA, Chandler HL, et 5. Herrera AF, Colon J, Valdes-Dapena A, et al: al: Reversible achalasia due to reticulum-cell Achalasia or carcinoma? The significance of sarcoma. N Engl J Med 293: 130-132, 1975. the Mecholyl test. Am J Dig Dis 15: 1073- 15. Mellow MH: Return of esophageal peristalsis 1081, 1970. in idiopathic achalasia. Gastroenterology 70: 6. Ullal SR, Rao NR: Achalasia. An unusual 1148-1151, 1976.

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