Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014848 (achalasia)
 2,804 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twelve patients with benign and malignant esophageal diseases were treated by transhiatal esophagectomy, without thoracotomy, using abdominal-mediastinal dissection conducted by videolaparoscopy. A cervical approach was used to retrieve the esophagus and to perform the esophagogastric anastomosis. The procedure was indicated in patients with advanced achalasia of the esophagus, severe reflux stenosis, squamous cell carcinoma, and adenocarcinoma of the esophagus. Three pleural perforations occurred during surgery. Blood loss was minimal. One patient required conversion to open surgery, two patients were submitted to chest drainage, and three had transitory dysphonia. One patient had an anastomotic leak with subsequent stenosis requiring endoscopic dilatation. No mortality occurred in this small series.
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PMID:Laparoscopic transhiatal esophagectomy with esophagogastroplasty. 773 33

Achalasia of the esophagus is presumed by many to be a premalignant lesion leading to an increased risk of squamous cell carcinoma. There is disagreement, however, as to the precise risk of malignant degeneration and there is no consensus as to either the need for close surveillance of achalasia patients or the surveillance technique that should be employed. A review of the available literature on the subject has disclosed a wide range of reported cancer risks in achalasia patients, from zero to 33 times that of the normal population. Cancers, when discovered, are often unresectable and the median survival when they are resectable is low. A personal experience with 241 achalasia patients treated during the past quarter of a century disclosed that 9 had carcinoma, for a prevalence of 3.7%. Carcinoma developed in 3 of these 9 while they were under our observation. This translates into one cancer per 1,138 patient-years of follow-up, an incidence of 88 per 100,000 population, and a risk 14.5 times that of the age-adjusted and sex-adjusted general population. Because of the low postresection survival rate if treatment is delayed until carcinoma of the esophagus becomes symptomatic, closer surveillance of achalasia patients is recommended than has been the case. Because it seems unlikely that close endoscopic surveillance will prove to be cost-effective, periodic (every 2 to 3 years) blind brush biopsy warrants further study as a means of surveillance.
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PMID:Achalasia and squamous cell carcinoma of the esophagus: analysis of 241 patients. 777 59

Although the risk of cancer is increased in patients with achalasia, biomarkers of an increased cancer risk have not been evaluated. In an esophagectomy specimen of a patient with achalasia-associated squamous cell carcinoma, normal mucosal and carcinomatous samples were systematically taken for flow cytometry and histology. The distribution of DNA aneuploidy and dysplasia was mapped within the resected specimen. Four of 10 tumor samples and 4 of 16 normal mucosal samples of the esophagus showed additional aneuploid stem lines. Gastric mucosa only showed diploid DNA histograms. S-phase fraction in normal esophageal samples (7.8% +/- 1.1%) was lower than in dysplastic and carcinomatous samples (8.8% +/- 2.4%; P = NS). Areas of mild to moderate dysplasia were detected in the esophageal mucosa adjacent to the neoplasm. This report shows the potential applicability of flow cytometry in the surveillance of patients with achalasia. However, prospective endoscopic studies with long follow-up periods are required before flow cytometric and histological parameters can be used as biomarkers of an increased cancer risk in achalasia.
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PMID:Achalasia-associated squamous cell carcinoma of the esophagus: flow-cytometric and histological evaluation. 783 97

Achalasia is characterized by failure of relaxation of the lower esophageal sphincter and absence of progressive peristalsis in the esophageal body. Few data are available regarding the morphologic features of achalasia, in particular its histologic progression. The esophagi of 42 patients with achalasia treated with total thoracic esophagectomy were examined histologically in order to systematically identify morphologic features of clinically unresponsive achalasia and to determine what could be learned about the disease's evolution. In all cases, myenteric ganglion cells within the esophageal body were markedly diminished, with 20 specimens having none. Twenty specimens had residual ganglion cells in the proximal esophagus, and 15 specimens had a few randomly distributed ganglion cells in the mid- and distal portions of the esophagus. Inflammation within myenteric nerves, present in all cases, generally consisted of a mixture of lymphocytes and eosinophils, occasionally with plasma and mast cells. Focal replacement of myenteric nerves by collagen occurred in all cases, and there was almost complete replacement in several cases. Actual destruction of the residual ganglion cells was not seen. The resected esophagi also shared extramyenteric morphologic features. Some features probably stemmed from physiologic obstruction, such as muscular hypertrophy, mainly of the muscularis propria (all cases), with secondary degeneration and fibrosis (29 cases), and eosinophilia of the muscularis propria (22 cases). Other changes, probably resulting from chronic stasis of ingested materials in the lumen, included diffuse squamous hyperplasia (all cases), lymphocytic mucosal esophagitis (28 cases), lymphocytic inflammation of the lamina propria and submucosa with prominent germinal centers (all cases), and submucosal periductal or glandular inflammation with complete loss of submucosal glands in half of the cases. One patient had high-grade squamous dysplasia, and another had superficially invasive squamous cell carcinoma. A third group of changes was probably due to previous esophagomyotomy, including abnormal gastroesophageal reflux, as shown by pH reflux testing (13 cases) and Barrett's mucosa (four cases). In one case of Barrett's there was low-grade dysplasia. Clinically unresponsive, surgically resected achalasia has almost total loss of ganglion cells, and widespread destruction of myenteric nerves has already occurred. The only active component is myenteric inflammation. However, it cannot be determined whether this inflammation is a manifestation of ongoing nerve destruction or whether it is a secondary phenomenon.
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PMID:Achalasia. A morphologic study of 42 resected specimens. 814 27

A 67 year old male caucasian clerical worker with a background of long-standing gastro-oesophageal reflux-like dyspepsia and bronchiectasis presented to a tertiary hospital gastroenterology unit with a recent onset of dysphagia. An initial diagnosis of achalasia was made and within 1 year an established verrucous carcinoma of the upper oesophagus had developed. The tumour was inoperable due to tracheal invasion and therefore palliative treatment was given. The patient developed a tracheo-oesophageal fistula and died of pneumonia. Thus, verrucous squamous cell carcinoma of the oesophagus can occur with achalasia.
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PMID:Verrucous carcinoma of the oesophagus and achalasia. 843 56

In the upper aerodigestive tract, carcinogenesis in squamous cell mucosa is characterized by a tendency to field carcinogenesis leading to multicentricity of lesions and synchronous or metachronous multiple tumoral lesions, namely multifocality. During pretherapy broncho-esophagoscopy carried out on ENT-cancer patients, the rate of synchronous second primary cancer is 24%. In 85% of the cases, these second primaries are detected at an early stage (in situ, microinvasive or submucosal carcinoma) and do not give rise to symptoms. Early diagnosis of cancer of the upper aerodigestive tract is possible provided that high risk patients are recognized and screening endoscopy of the whole mucosa is performed in every high risk patient. On the other hand, squamous cell carcinoma and adenocarcinoma may occur with increased frequency in patients with esophageal lesions such as achalasia, caustic stenosis and Barrett's esophagus. The premalignant potential of these three entities is discussed.
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PMID:[Precancerous conditions of the esophagus]. 851 40

Cancer of the oesophagus has great diversity in geographical distribution and incidence. The rate of oesophageal cancer has been increasing in some areas and the reasons for this are not clear. This review outlines fascinating epidemiological aspects and the risk factor for squamous cell carcinoma of the oesophagus. While in the Western world the effects of alcohol and tobacco are substantial preconditions, worldwide other factors, such as diet, nutritional deficiencies, environmental exposure and infectious agents (especially papillomavirus and fungi), play a significant role. Chronic irritation of the oesophagus appears to participate in the process of carcinogenesis, particularly in patients with thermal and/or mechanical injury, achalasia, oesophageal diverticulum, chronic lye stricture, radiation therapy, injection sclerotherapy and gastric resection before the appearance of oesophageal tumour. The association of Plummer-Vinson syndrome, coeliac disease, tylosis and scleroderma with oesophageal cancer has also been reviewed.
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PMID:Risk factors for squamous cell carcinoma of the oesophagus. 905 60

An accurate preoperative diagnosis of an esophageal motor disorder, including its location (the LES, the body of the esophagus, or both), is essential before proceeding with esophagomyotomy. The operative procedure should be performed with careful attention to certain technical details to ensure completeness of the myotomy, to prevent later healing of the myotomy, and to avoid radical cardiomyotomy that might facilitate the subsequent development of GER. Potential hazards are associated with performing a 360 degrees antireflux procedure in the presence of an aperistaltic esophagus. Early operation before the development of megaesophagus is recommended. The excellent results achieved by resection coupled with antrectomy and Roux-en-Y diversion suggest its wider application to patients with one or more previous failed myotomies, particularly patients with stricture and megaesophagus. Long-term surveillance of patients with achalasia is mandatory in view of the known risk of late development of squamous cell carcinoma.
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PMID:Failure after esophagomyotomy for esophageal motor disorders. Causes, prevention, and management. 924 98

Carcinosarcoma of the esophagus is a rare malignant neoplasm that consists of both carcinomatous and sarcomatous elements. The histogenesis of the sarcomatous component is generally considered to result from metaplasia of carcinomatous cells toward mesenchymal differentiation. True carcinosarcoma, characterized as a collision between a carcinoma and a sarcoma, is extremely rare. We describe a patient with primary achalasia who developed a true carcinosarcoma of the esophagus in which clonal differences between carcinomatous and sarcomatous elements were genetically and immunohistochemically demonstrated. A polypoid tumor located in the middle third of the esophagus developed in a 51-year-old man with longstanding achalasia. The tumor was predominantly composed of spindle-shaped sarcomatous cells. Squamous cell carcinoma in situ and islands of well-differentiated squamous cell carcinoma in the sarcomatous element were histologically observed. The sarcomatous element was immunoreactive for both mesenchymal and myoid markers. The carcinomatous component expressed type I and type II cytokeratins as well as epithelial membrane antigen. Analysis for chromosomal loss of heterozygosity performed in multiple microdissected samples of each sarcomatous and carcinomatous element revealed distinct genetic clonalities. These differences in immunohistochemical and genetic clonalities suggest that the tumor composed of squamous cell carcinoma and leiomyosarcoma originated separately from epithelial and mesenchymal precursors.
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PMID:Esophageal carcinosarcoma: a genetic analysis. 928 91

Cancer of the oesophagus is a challenging clinical problem. Overall survival is poor, but patients who present early are eminently curable. Most cancers of the middle and upper oesophagus are squamous cell carcinoma. Adenocarcinoma is the most common cancer of the third of the oesophagus; this is not surprising when the usual distribution of Barrett's mucosa is considered. The geographical variation in the prevalence of oesophagus cancer is important. In most parts of the world, alcohol consumption and tobacco usage are the principal risk factors. Other risk factors have been identified in "the high-risk areas": a diet high in nitrosamines, deficient in trace elements, in vitamins (C.A, E) and the hereditary conditions like: Barrett's oesophagus, achalasia, caustic strictures.
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PMID:[The etiopathogenesis of esophageal cancer]. 945 31


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