Clin Endosc.  2015 Jul;48(4):328-331. 10.5946/ce.2015.48.4.328.

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

• Kim HM
• Chu JM
• Kim WH
• Hong SP
• Hahm KB
• Ko KH

• KMID: 1964277
• DOI: http://doi.org/10.5946/ce.2015.48.4.328

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.

Keyword

Esophageal achalasia; Pseudoachalasia; Esophagogastric tumor; Pancreatic neoplasms

MeSH Terms

Arthritis, Rheumatoid
Biliary Tract
Chronic Disease
Cystadenocarcinoma, Mucinous
Deglutition Disorders
Diagnosis
Endoscopy
Esophageal Achalasia*
Gastric Fundus
Humans
Liver
Manometry
Middle Aged
Neoplastic Processes
Pancreatic Neoplasms*

Figure

• 

  Fig. 1 Esophagoscopic finding suggestive of primary achalasia. A dilated esophagus filled with liquid and some solid foods is visible (A, B), which is compatible with the findings of primary achalasia. There was no evidence of extrinsic compression or any cardiac lesion on retroversion of endoscopy (C).

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  Fig. 2 Esophageal manometry findings suggestive of primary achalasia. Manometry shows a total absence of peristalsis in the body of the esophagus (vacant arrow) and a hypertonic lower esophageal sphincter with incomplete relaxation (filled arrow).

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  Fig. 3 Botulinum toxin injection therapy to relieve achalasia. In total, 4 mL of botulinum toxin was injected at four points in the esophagogastric junction with 1 mL injected at each point. The photographs show the condition of the esophagogastric junction before (A) and after (B) the botulinum toxin injections.

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  Fig. 4 Abdominal and pelvic computed tomography findings showing a huge mass extending into the gastric fundus. An approximately 7.5-cm sized irregular infiltrative solid mass with multiple septated cystic portions originating from the pancreas had invaded the gastric body and extended to the gastric fundus (arrow).

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  Fig. 5 Esophageal stent insertion to relieve dysphagia of secondary achalasia. Endoscopic image of an esophageal stent (uncovered, 5 cm; Taewoong Medical) placed across the esophagogastric junction into the proximal stomach.

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