Cancer of the oesophagus in achalasia

Abstract

A 54-year old man was admitted to the emergency department for chronic dysphagia for solid as well for liquids of several years duration, with increasing severity for two weeks.The patient also had shortness of breath grade III for two weeks. The medical history of the patient was uneventful, except for surgery on the oesophagus 26 years previously, probably for achalasia. No follow-up was performed. At the time of admission, the patient doesn't take any medication. He doesn't smoke and consumes one or two alcohol units a day.Clinical examination fails to reveal any specific findings. Blood analysis showed a decrease of the hemoglobin level (10.4 g/dL), a slightly elevated leukocytosis (10400/µL) and elevated C-reactive protein level (127 mg/L), as well as hyponatriemia and hypochloremia.Chest X-ray shows cardiomegaly and a dilated proximal oesophagus with a marked decrease in the diameter distally.Fibroscopy shows an ulcerated tumoral lesion of 6 cm at 40 cm, biopsies showing an infiltrating epidermoid carcinoma.Bronchoscopy was negative. PET-scan confirms the presence of a large, irregular, hypermetabolic mass in the lower oesophagus and enlarged hypermetabolic mediastinal lymph nodes.Barium X-ray confirms the predominantly enlarged upper oesophagus (4 cm) with stasis of liquids and absence of peristalsis. The distal part of the oesophagus is narrowed showing a pattern compatible with treated achalasia (Fg. A). On the right posterior border of the distal one third of the oesophagus the large ulcerated lesion of approximately 8 cm with typical neo plastic pattern is demonstrated on the lateral view (Fig. B). Contrast enhanced computerized tomography of the thorax confirms the presence of the ulcerated mass and shows an enlarged lymph node in the mediastinum and the right hilus (Fig. C). Treatment consisted of radio-chemotherapy. CommentAchalasia is a primary oesphageal motility disorder affecting mainly adults of both sexes equally and predominantly presenting with dysphagia, sometimes with regurgitation.The treatment of achalasia is symptomatic and designed to improve the oesophageal emptying. The most effective treatments are pneumatic balloon dilatation and cardiomyotomy, follow-up being based on X-ray and manometry. Late complications are related to recurrent dysphagia after treatment. Perihiatal scarring, peptic stricture or obstructing tumors can all cause recurrent symptoms. The most important late complication is the occurrence of oesophageal cancer, patients having similar symptoms in recurring achalasia and in oesophageal cancer. Therefore, mostly cancers are diagnosed in an advanced stage.The relationship between achalasia and cancer was first described in 1872 by Fagge. An increased risk of developing oesophageal carcinoma in patients with long standing achalasia has been reported by several authors, with incidences varying widely from 1.7 to 20%.It has been established that the consumption of alcohol and tobacco increases the risk of developing esophageal cancers due to the content of carcinogens i...

Imaging in the Evaluation of Endoscopic or Surgical Treatment for Achalasia

“…Upper endoscopy is usually performed to rule out cancer or a peptic stricture and, particularly in patients older than 50 years with dysphagia and weight loss, attention should be paid on the possible presence of a tumor underlying achalasia (pseudoachalasia) [ 10 , 11 ]. Cytohistological samples should always be taken in the cardiac region and in the suspicious areas, to find possible neoplastic degeneration [ 12 , 13 ].…”