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      Extragastroesophageal Malignancy-Associated Secondary Achalasia: A Rare Association of Pancreatic Cancer Rendering Alarm Manifestation

      case-report

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          Abstract

          Secondary achalasia or pseudoachalasia is a rare esophageal motor abnormality, which mimics primary achalasia; it is not easily distinguishable from idiopathic achalasia by manometry, radiological examination, or endoscopy. Although the majority of reported pseudoachalasia cases are associated with neoplasms at or near the esophagogastric (EG) junction, other neoplastic processes or even chronic illnesses such as rheumatoid arthritis can lead to the development of pseudoachalasia, for example, mediastinal masses, gastrointestinal (GI) tumors of the liver and biliary tract, and non-GI malignancies. Therefore, even if a patient presents with the typical findings of achalasia, we should be alert to the possibility of other GI malignancies besides EG tumors. For instance, pancreatic cancer was found in the case reported here; only four such cases have been reported in the literature. A 47-year-old man was admitted to our center with a 3-month history of dysphagia. His endoscopic and esophageal manometric findings were compatible with primary achalasia. However, unresponsiveness to diverse conventional achalasia treatments led us to suspect secondary achalasia. An active search led to a diagnosis of pancreatic mucinous cystadenocarcinoma invading the gastric fundus and EG junction. This rare case of pseudoachalasia caused by pancreatic carcinoma emphasizes the need for suspecting GI malignancies other than EG tumors in patients refractory to conventional achalasia treatment.

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          Most cited references18

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          Comparison of pseudoachalasia and achalasia.

          Malignancies involving the gastric cardia or distal esophagus can result in a clinical syndrome termed pseudoachalasisa that mimics idiopathic achalasia. If not promptly recognized, pseudoachalasia can result in inappropriate pneumatic dilatation of the lower esophageal sphincter segment and delay appropriate treatment of the underlying malignancy. During the past 14 years, six patients with pseudoachalasia and 161 patients with primary idiopathic achalasia were encountered. Pseudoachalasia occurred mainly in the elderly and represented about 9 percent of these patients over 60 years of age with suspected achalasia. Five of the six pseudoachalasia cases were secondary to adenocarcinoma that originated in the gastric fundus, and one was caused by a squamous cell carcinoma of the distal esophagus. Conventional esophageal manometry did not discriminate achalasia from pseudoachalasia. On the other hand, esophagogastroscopy with biopsy resulted in a diagnosis of pseudoachalasia in five of these cases and in 24 of 32 cases reported previously. Ominous endoscopic findings are mucosal ulceration or nodularity, reduced compliance of the esophagogastric junction, or an inability to pass the endoscope into the stomach. Radiographic evaluation, particularly in conjunction with amyl nitrite inhalation, was also useful in discriminating pseudoachalasia from primary achalasia. It is concluded that pseudoachalasia generally mimics idiopathic achalasia imperfectly and can usually be diagnosed prior to surgery by fastidious endoscopic and radiographic examination.
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            Achalasia - An Update

            Achalasia is an esophageal motility disorder of unknown cause, characterized by aperistalsis of the esophageal body and impaired lower esophageal sphincter relaxation. Patients present at all ages, primarily with dysphagia for solids/liquids and bland regurgitation. The diagnosis is suggested by barium esophagram and confirmed by esophageal manometry. Achalasia cannot be cured. Instead, our goal is to relieve symptoms, improve esophageal emptying and prevent the development of megaesophagus. The most successful therapies are pneumatic dilation and surgical myotomy. The overall success rate of graded pneumatic dilation is 78%, with women and older patients responding best. Laparoscopic myotomy, usually combined with a partial fundoplication, has an overall success rate of 87%. Young patients, especially men, are the best candidates for surgical myotomy. Botulinum toxin injection into the lower esophageal sphincter and smooth muscle relaxants are usually reserved for older patients or those with co-morbid illness. The prognosis for achalasia patients to return to near normal swallowing is good, but the disease is rarely "cured" with a single procedure and intermittent touch-up procedures may be required.
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              Pseudoachalasia: a case series and analysis of the literature.

              Pseudoachalasia frequently cannot be distinguished from idiopathic achalasia by manometry, radiologic examination or endoscopy. Mechanisms proposed to explain the clinical features of pseudoachalasia include a circumferential mechanical obstruction of the distal esophagus or a malignant infiltration of inhibitory neurons within the myenteric plexus. Between January 1980 and December 2002, the clinical features of 5 patients with pseudoachalasia and 174 patients with primary achalasia, diagnosed in a single center, were compared. A literature analysis of the etiology of pseudoachalasia for the time period 1968 to December 2002 was performed. The search concentrated on the databases and online catalogues PubMed, Web of Science, Cochrane Library and Current Contents Connect. In our case series, patients with pseudoachalasia reported a shorter duration of symptoms and tended to be older than patients with primary achalasia. Conventional manometry, endoscopy and radiologic examination of the esophagus proved to be of little value in distinguishing between the diseases. In the majority of cases only surgical exploration revealed the underlying cause. A coincidence of primary achalasia and disorders of the gastroesophageal junction was excluded by showing return of peristalsis following treatment. The analysis of the literature showed a total of 264 cases of pseudoachalasia in 122 publications. Most cases of were due to malignant disease (53.9% primary and 14.9% secondary malignancy), followed by benign lesions (12.6%) and sequelae of surgical procedures at the distal esophagus or proximal stomach (11.9%). In rare instances, the disease was an expression of a paraneoplastic process due to distant neuronal involvement rather than to local invasion with destruction of the myenteric plexus (2.6%). The diagnosis of pseudoachalasia is difficult to establish by conventional diagnostic measures. The main distinguishing feature of secondary versus primary achalasia is the complete reversal of pathologic motor phenomena following successful therapy of the underlying disorder.
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                Author and article information

                Journal
                Clin Endosc
                Clin Endosc
                CE
                Clinical Endoscopy
                The Korean Society of Gastrointestinal Endoscopy
                2234-2400
                2234-2443
                July 2015
                24 July 2015
                : 48
                : 4
                : 328-331
                Affiliations
                Department of Internal Medicine, CHA Bundang Medical Center, CHA University, Seongnam, Korea.
                Author notes
                Correspondence: Kwang Hyun Ko. Department of Internal Medicine, CHA Bundang Medical Center, CHA University, 59 Yatap-ro, Bundang-gu, Seongnam 463-712, Korea. Tel: +82-31-780-5214, Fax: +82-31-780-5219, bluehipp@ 123456cha.ac.kr
                Article
                10.5946/ce.2015.48.4.328
                4522426
                26240808
                4fd80b3a-27bc-4d49-a6f3-01c835c0a3fb
                Copyright © 2015 Korean Society of Gastrointestinal Endoscopy

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 04 August 2014
                : 10 August 2014
                : 10 August 2014
                Categories
                Case Report

                Radiology & Imaging
                esophageal achalasia,pseudoachalasia,esophagogastric tumor,pancreatic neoplasms

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