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)

Squamous cell carcinoma (SCC) of the esophagus is an often-lethal disease that most commonly presents in an advanced stage with dysphagia in elderly patients. Known risk factors include alcohol and tobacco abuse, lye stricture, and achalasia. Screening protocols for high-risk patients are practiced in Japan but not in the United States. The diagnosis usually is made based on the results of esophagogastroduodenoscopy and contrast upper gastrointestinal radiographs. Staging is determined using computed tomography scanning and esophageal ultrasound, the latter rapidly being accepted as a superior method. Treatment is based on the stage of disease at presentation. Lesions without metastatic spread or mediastinal invasion generally should be treated with esophagectomy. Dysphagia associated with advanced lesions is difficult to treat, but may be palliated by surgery, radiation therapy, chemotherapy, laser ablation, peroral dilation, or esophageal stenting. Despite numerous medical advances, little headway has been made in managing and treating SCC, and a multidisciplinary approach is recommended.
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PMID:Squamous cell carcinoma of the esophagus: a review and update. 977 5

Endoscopic ultrasound (EUS) of the esophagus has been used primarily in staging biopsy-proven cancers. Its use as a primary diagnostic modality for esophageal malignancy has not been previously described. We report our recent experience in four patients with dysphagia and endoscopic biopsies negative for malignancy, including one patient with clinical and manometric features suggestive of achalasia. In all cases, EUS revealed a large infiltrating tumor invading through the esophageal wall into the surrounding tissues, and in one case into the aorta. Computed tomography suggested the possibility of a tumor in only one of the cases. Two patients underwent esophagectomy and were found to have adenocarcinoma. Two patients underwent repeat biopsy with alternative aggressive biopsy techniques and were found to have squamous cell carcinoma. We conclude that EUS is useful in the diagnosis of esophageal cancer and should be performed in selected patients with esophageal strictures whose biopsies are negative for malignancy; i.e., those with suspicious endoscopic or radiographic appearance, atypical presentation (e.g., profound weight loss, short duration of symptoms, or advanced age), and failure to respond to treatment.
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PMID:Biopsy-negative malignant esophageal stricture: diagnosis by endoscopic ultrasound. 982 Apr 10

Oesophageal achalasia was treated with modified Heller's oesophagomyotomy in 51 patients (19 males, 32 females) via thoracotomy in 47 cases and thoracoscopy in 4 cases. A Belsey Mark IV antireflux procedure was added to transthoracic oesophagomyotomy in two cases, because of extended cardiomyotomy. There were no hospital deaths. The overall improvement rate was 93.5%, with excellent results in 80.6%. Postoperative follow-up averaged 7.4 years. In all four cases of thoracoscopic oesophagomyotomy, simultaneous oesophagoscopy was performed to facilitate the procedure. One patient required repeat surgery 2 months later because of inadequate myotomy. Thirty-one patients, including three with severe gastro-oesophageal reflux, received long-term medication. Barrett's oesophagus developed in two of the 31 patients (6.5%) 4.7 and 7.6 years, respectively, after myotomy and squamous cell carcinoma was diagnosed in a 44-year-old woman 2.2 years postoperatively. The study suggests that transthoracic oesophagomyotomy without antireflux procedure can provide excellent long-term relief of dysphagia in oesophageal achalasia and carries a low risk of serious postoperative complications.
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PMID:Transthoracic oesophagomyotomy in the treatment of achalasia--a 15-year experience. 1062 44

AIMS: The management and surveillance of achalasia remains controversial at the present time. The aim of this study was therefore to evaluate the results of endoscopic management and subsequent surveillance of patients with achalasia presenting to a specialist unit. METHODS: A prospective cohort of 40 patients with a radiological and manometric diagnosis of achalasia who presented to this unit between 1991 and 1998 were studied; the male : female ratio was 1 : 1 and the median age 38 (range 15-84) years. Twenty-one patients presented de novo, seven had previously undergone cardiomyotomy and 12 were referred following unsuccessful dilatation. RESULTS: Some 36 patients were treated with balloon dilatation (Microvasive achalasia balloon, 35/40 mm). Results were graded 1-4 (1, asymptomatic; 2, symptomatic but significantly improved; 3, symptomatic with no change; and 4, symptomatic but worse); 29 of 36 patients were grade 1 at subsequent follow-up and the remaining seven were grade 2 (median follow-up 17 (range 5-96) months). There was a single complication of oesophageal perforation which was treated conservatively with full recovery. Following intervention, patients were enrolled in a prospective surveillance programme of chromoendoscopy at 2-year intervals; in a total of 74 patient-years' follow-up, two superficial squamous cell carcinomas (SCCs) and one adenocarcinoma (following cardiomyotomy) were detected, giving a relative risk of one cancer in 25 patient-years. CONCLUSIONS: Balloon dilatation is a safe and effective treatment for achalasia even in patients who have had previous unsuccessful dilatations or cardiomyotomy. There is a high risk of SCC and the adenocarcinoma may have resulted from previous refluxogenic therapy, so all patients with achalasia should be followed up with surveillance endoscopy.
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PMID:Achalasia: management, outcome and surveillance in a specialist unit 1071 70

We evaluated diagnostic X-ray and endoscopic examination findings in 486 patients with esophageal achalasia. Concerning the association between the duration of disease and the X-ray dilatation type, the duration was 1-4 years in more than 50% of patients with the Sp type, a mean of 8.5 years in those with the F type, and frequently more than 10 years in those with the S type. Endoscopy is generally used to determine the presence or absence of abnormal movement. In achalasia, the endoscope can be inserted into the stomach despite resistance at the stenotic site, and the mucosal surface is normal. Squamous cell carcinoma as a complication was observed in 21 patients (4.3%). The carcinoma complication rate was higher with a longer duration of disease and a longer observation period. The mean total course including the postoperative course was 27 years. Long-term and periodic X-ray and endoscopic observation of the disease course is important, and iodine staining is indispensable for early detection of esophageal cancer.
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PMID:[X-ray and endoscopic diagnosis of esophageal achalasia]. 1084 93

Achalasia of the esophagus is a benign disease caused by dyskinesia of the lower esophagus and cardia and is presumed to be a premalignant lesion leading to an increased risk of squamous cell carcinoma. We analyzed six surgically or endoscopically resected carcinomas among 54 cases of esophageal achalasia using histological and immunohistochemical procedures. The mean interval between the diagnosis of achalasia and carcinoma was 21.5 years. Four of the six cases were superficial early-stage cancers whilst the other two were advanced cancers invading the adventitia. Histological mapping of the resected esophageal specimens demonstrated marked hyperplastic changes of stratified squamous epithelium and multiple foci of dysplastic changes. The squamous cell carcinomas showed well-differentiated type with low-grade atypia, closely associated with dysplastic foci. Immunohistochemical staining for p53, p21, p16 and epidermal growth factor receptor suggested that the dysplastic epithelium was a borderline lesion between hyperplasia and in situ carcinoma. Our observations suggested that esophageal food stasis induces chronic hyperplastic esophagitis and eventually malignant transformation of esophageal epithelial cells, associated with dysplasia-carcinoma sequence.
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PMID:Clinicopathological studies of esophageal carcinoma in achalasia: analyses of carcinogenesis using histological and immunohistochemical procedures. 1126 44

This article discusses the risk of esophageal squamous cell carcinoma in patients with achalasia. A broad review of the literature is provided with incidence and prevalence data. Clinical features, pathophysiologic, diagnostic, and prognostic aspects are discussed. Information is also provided on how different treatment methods impact this disease. Finally, recommendations on surveillance intervals are made, based on an evidence-based approach.
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PMID:Risk and surveillance intervals for squamous cell carcinoma in achalasia. 1131 71

Reported incidence rates of carcinoma in patients with achalasia and the prevalence of achalasia in patients with esophageal cancer vary widely in the literature. The prognosis of an "achalasia-carcinoma" is generally considered poor, although systematic studies assessing the incidence, prevalence, and prognosis of patients with "achalasia-carcinoma" are scant. We investigated the incidence of esophageal cancer in a large series of patients with known achalasia, assessed the prevalence of achalasia in patients presenting with esophageal cancer, and evaluated the prognosis of these patients compared to that of patients with esophageal cancer without achalasia. Between 1982 and 1998 a total of 124 patients with primary achalasia were treated and followed at our department. During the same time period 1366 patients presented with esophageal cancer (879 esophageal squamous cell carcinomas, 487 adenocarcinomas). Of the 124 patients with primary achalasia, 4 developed a carcinoma during a mean follow-up of 5.6 years (i.e., an incidence of one carcinoma per 173.6 patient-years of follow-up). Altogether, 13 of 879 patients (1.5%) presenting with esophageal squamous cell carcinoma and 1 of 487 patients (0.2%), presenting with esophageal adenocarcinoma had a history of primary achalasia. Seven patients with achalasia-carcinoma (50%) had early-stage disease (stage I, IIA, or IIB). There was no difference in the prognosis of patients with resected achalasia-carcinoma versus those with esophageal carcinoma but no achalasia. Thus in our population of patients with long-standing achalasia the risk for developing an esophageal cancer was increased about 140-fold over that of the general population. With liberal use of surveillance, carcinoma could often be detected at an early stage in these patients, with a prognosis that was not worse than that of patients with squamous cell esophageal cancer but no achalasia.
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PMID:Achalasia and esophageal cancer: incidence, prevalence, and prognosis. 1137 10

Achalasia is an esophageal motor disorder in which the primary morphologic changes are found in the myenteric plexus. However, a number of secondary alterations are characteristically found in esophagectomy specimens, including the mucosa. In addition, these patients are at increased risk of developing esophageal squamous cell carcinoma. We studied the squamous mucosal alterations in 35 esophagectomy specimens from patients with end-stage achalasia and compared them with those found in the squamous mucosa near the esophagogastric junction from pediatric autopsies (</=18 years) from patients with no known esophageal disease. A representative block was immunostained for p53 (DO7), CD3, and CD20. p53 immunoreactivity was graded as follows: 0 = no staining; 1+ = rare basal cell staining; 2+ = extensive basal cell staining; 3+ = suprabasilar staining. Intraepithelial lymphocyte counts were performed by counting five high power fields (HPF) and calculating an average/HPF. Ages of achalasia patients at esophagectomy ranged from 21 to 78 years (mean 56 years), including 20 men and 15 women. Disease duration ranged from 1 to 44 years (mean 17 years). In all cases the squamous mucosa from achalasia patients was markedly hyperplastic with papillomatosis and basal cell hyperplasia. p53 staining in the squamous mucosa from achalasia patients was significantly more common than in controls (32 of 35 [91%] vs 1 of 17 [6%]; p <0.05). In all achalasia cases CD3+ cells far outnumbered CD20+ cells. There was a significantly greater number of CD3+ cells in achalasia cases (range 32-239/HPF; mean 107/HPF) compared with controls (range 0.8-12/HPF; mean 6/HPF) (p <0.05). In conclusion, the squamous mucosa in esophagectomy specimens from patients with end-stage achalasia shows significant alterations including marked squamous hyperplasia, an increased frequency of p53 immunoreactivity, and increased numbers of CD3+ cells when compared with controls. These changes may be related to the increased risk of squamous cell carcinoma in these patients.
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PMID:Squamous mucosal alterations in esophagectomy specimens from patients with end-stage achalasia. 1168 58

Achalasia is a disease of unknown origin in which there is a denervation of the myenteric plexus on the smooth muscle of the lower oesophageal sphincter, causing a cardial stenosis and a loss of efficacy of oesophageal peristalsis. The predominant symptoms are dysphagia for solids and liquids and regurgitation of the retained food. Occasionally, there may be oesophageal haemorrhage as a consequence of oesophagitis and stasis ulcers. An important but uncommon complication is the development of oesophageal cancer, which is typically squamous cell carcinoma. We report an exceptional case of a 77-year-old woman with a long-term achalasia and mega-oesophagus who presented four episodes of upper gastrointestinal bleeding in a 2 month period. The patient underwent surgical resection of the 10 cm of distal oesophagus, performing a partial fundoplication, and the pathological study revealed an oesophageal infiltration by a low-grade non-Hodgkin's lymphoma. After an insidious outcome, she died on the 47th day after admission.
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PMID:Relapsing upper bleeding in non-Hodgkin's oesophageal lymphoma associated with achalasia. 1450 23


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