5-Fluorouracil and cisplatin as palliative treatment of advanced oesophageal squamous cell carcinoma. A multicentre randomised controlled trial. The French Associations for Surgical Research

OBJECTIVE: To compare chemotherapy with no chemotherapy as palliative treatment for oesophageal squamous cell carcinoma. DESIGN: Randomised study. SETTING: Multicentre trial in France. SUBJECTS: Of 161 patients with histologically confirmed oesophageal squamous cell carcinoma located more than 5 cm from the mouth of the oesophagus, five were withdrawn because of protocol violation. The remaining 156 patients, 149 men and 7 women, mean (SD) age 58 (9) years range 36 to 77, were randomly allocated to either a control group without chemotherapy (n = 84) or a group treated by chemotherapy (n = 72). Patients were divided into four strata: I = complete resection of the tumour but with lymph node involvement (n = 62); II = incomplete resection of tumour leaving gross tumour behind (n = 58); III = no resection because of local or regional invasion (n = 22) ; and IV = no resection because of distant metastasis (n = 14). Exclusion criteria were histologically confirmed tracheobronchial involvement, oesophagotracheal fistula, Karnosky score < 50, cerebral metastases, or hepatic metastases occupying more than 30% of the liver, peritoneal carcinomatosis, associated or previously treated ear-nose-throat carcinoma, or complete resection of tumour without lymph node involvement. INTERVENTIONS: 5 fluorouracil (5FU) and cisplatin (CDDP) were given in 5-day courses, once every 28 days, for a maximum of eight cycles. 5 FU, 1 g/m2, was infused for 24 hours after a water overload, during five days. Cisplatin was given either in one dose of 100 mg/m2 at the beginning of the cycle or 20 mg/m2/day over three hours for five days. Duration of treatment ranged from 6-8 months. OUTCOME MEASURES: Median and actuarial survival. The subsidiary endpoint was quality of survival judged by complications of treatment, swallowing disorders, and the duration of ability to feed normally. RESULTS: There was no difference in survival, either overall (median = 12 months) or in any of the strata. There were however significantly more patients with neurological (p < 0.003), haematological (p < 0.0001), and renal (p < 0.0002) complications in the treated group compared with the control group. Four patients (6%) died of complications of chemotherapy. The course of swallowing disorders did not differ between the two groups. The duration of autonomous oral feeding was exactly the same in both groups (median = 10.5 months). CONCLUSION: The results suggest that 5FU and CDDP do not help in patients with squamous cell carcinoma of the oesophagus whether or not the tumour has been resected.     

Clinical photodynamic therapy for superficial cancer in the oesophagus and the bronchi: 514 nm compared with 630 nm light irradiation after sensitization with Photofrin II

Photodynamic therapy (PDT) for cancer in the oesophagus and bronchi with red (630 nm) light may occasionally lead to wall perforation and fistula. Therefore, we investigated the clinical use of a less penetrating wavelength (514 nm) for the curative treatment of nine superficial carcinomas in the oesophagus and bronchi after photosensitization with Photofrin II. Tumours without infiltration beyond the submucosa in the oesophagus and beyond the lamina propria in the bronchi were considered as superficial cancers. The outcome and complications were compared with those of 13 superficial cancers treated with PDT and 630 nm light. In addition, we evaluated histologically the extent of the long-term tissue damage and scarring following treatment of six oesophageal cancers with either green or red light. At first endoscopic control, 7-10 days after PDT, tissue necrosis simply matched the illuminated area, without evidence of selective tumour damage. Six of nine tumours treated with 514 nm light had a complete response compared with nine of 13 after 630 nm irradiation. No perforation or fistula occurred in either treatment group. However, severe chest pain and fever with or without pleural effusion, consistent with occult perforation, were observed in three patients after 630 nm illumination in the oesophagus. Histologically, fibrous scarring in the three distinct sites treated with green light was limited to the superficial layers of the oesophagus. After red light treatment, transmural fibrosis with marked thinning of the oesophageal wall was evident in two of the three specimens available for inspection. These results indicate that PDT with 514 nm light has the potential to cure superficial cancer in the oesophagus and bronchi with essentially the same probability of success as red light. In the oesophagus, green light prevents deep tissue damage, thus reducing the risk of perforation.     

Esophageal cancer on the increase. Is Barrett esophagus the cause?

At the Dept of Surgery, Lund University, during the 10-year period 1985-95, 54 patients with adenocarcinoma of the gastro-oesophageal junction (17 with Barrett's epithelium, and 37 without) underwent oesophageal resection: oesophagectomy and gastric pull-up (n = 10), extended total gastrectomy (n = 37), or oesophageal resection and interposition of colon (n = 2) or jejunum (n = 5). Hospital mortality was 3.7% (2/54), and the mean duration of hospitalisation 13 days (range, 9-42). Long-term survival was significantly better in the Barrett's oesophagus subgroup than in the carcinoma of the cardia (non-Barrett's oesophagus) subgroup, the respective rates being 50% vs. 10% (p = 0.0052; Log rank test). The better survival in the Barrett's oesophagus subgroup is probably to be explained by the earlier stage of disease among these patients, in turn due to a history of gastro-oesophageal reflux, whereas the predominant symptom in the cardia carcinoma subgroup was dysphagia.     

The etiopathogenesis of esophageal cancer

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.     

Protein kinases A and C phosphorylate purified Ca2+-ATPase from rat cortex, cerebellum and hippocampus

Previous knowledge on risk factors for oral, pharyngeal, laryngeal, and esophageal cancer has been based mainly on case-control studies. In the present study, the impact of alcohol consumption, tobacco smoking, and dietary factors on upper aerogastric tract cancer risk was studied in a cohort of 10,960 Norwegian men followed from 1968 through 1992, in which period a total of 71 upper aerogastric tract cancers occurred. The relative risk (RR) of cancer was 3.9 (95 percent confidence interval [CI] = 2.1-7.1) for the highest consumption group of alcohol and 4.7 (CI = 1.7-13.2) for the highest smoking level, compared with the respective reference groups. Among the dietary items, high consumption of oranges was associated with reduced cancer risk (RR = 0.5, CI = 0.3-1.0), as was high consumption of bread (RR = 0.2, CI = 0.1-0.5). Frequent consumption of beef and bacon increased relative cancer risk bordering on significance. The present results are largely in accordance with previous studies. The decreased risk associated with a high intake of bread deserves further investigation.     

Local production and action of adrenomedullin in the rat adrenal zona glomerulosa

The objective of this study was to compare the pre-hospital health care process, clinical characteristics at admission and survival of patients with a digestive tract cancer first admitted to hospital either electively or via the emergency department. The study involved cross-sectional analysis of information elicited through personal interview and prospective follow-up. The setting was a 450-bed public teaching hospital primarily serving a low-income area of Barcelona, Catalonia, Spain. Two hundred and forty-eight symptomatic patients were studied, who had cancer of the oesophagus (n = 31), stomach (n = 70), colon (n = 82) and rectum (n = 65). The main outcome measures were stage, type and intention of treatment and time elapsed from admission to surgery; the relative risk of death was calculated using Cox's regression. There were 161 (65%) patients admitted via the emergency department and 87 (35%) electively. The type of physician seen at the first pre-hospital visit had more often been a general practitioner in the emergency than in the elective group (89% vs 75%, P < 0.01). Emergency patients had seen a lower number of physicians from symptom onset until admission, but two-thirds had made repeated visits to a primary care physician. Emergency patients were less likely to have a localized tumour and a diagnosis of cancer at admission, and surgery as the initial treatment. Median survival was 30 months for elective patients and 8 months for emergency patients (P < 0.001), and the relative risk of death (RR) was 1.83 (95% confidence interval, CI, 1.32-2.54). After adjustment for strong prognostic factors, emergency patients continued to experience a significant excess risk (RR = 1.58; CI 1.10-2.27). In conclusion, in digestive tract cancers, admission to hospital via the emergency department is a clinically important marker of a poorer prognosis. Emergency departments can only partly counterbalance deficiencies in the effectiveness of and integration among the different levels of the health system.     

Cancer of the oesophagus

Oesophageal cancer is the fourth most common tumour in developing countries, comprising mainly squamous cell tumours, although the incidence of adenocarcinoma has increased enormously over the last decades. Surgical resection has long been acknowledged as the mainstay of treatment, and developments in surgical technique are reviewed. The roles of radiotherapy and chemotherapy in the management of oesophageal cancer remain unclear, especially as the majority of studies to date have been uncontrolled trials. We present an analysis of 601 patients who underwent resection for carcinoma of the oesophagus between 1970 and 1994 in the Department of Clinical Surgery, St James's Hospital, Dublin. The analysis shows clearly that, while peri-operative mortality continues to improve, conventional surgery offers little prospect of cure in the majority of cases. We have therefore embarked upon a prospective controlled trial of neoadjuvant chemoradiotherapy followed by surgery versus surgery alone in patients with adenocarcinoma or squamous cell tumours of the oesophagus. Preliminary results indicate that multi-modality treatment may have a valuable role to play in the treatment of carcinoma of the oesophagus.     

100 years guarding the health of the border guards

A Heller-Dor procedure was performed by laparotomy (group A: n = 8) or by laparoscopy (group B: n = 12) after failure of one to 17 sessions of intraluminal dilatations (n = 13) or as a primary treatment of oesophageal achalasia (n = 7). The oesophagomyotomy was extended over the thoracic oesophagus by thoracoscopy in two patients having vigorous achalasia. Injury to the oesophageal mucosa occurred in two group A patients who had previously been dilated. At follow-up (range: 1 to 113 months), 6 patients of group A (75%) and 10 of group B(83.3%) had no residual dysphagia. The four patients (group A: n = 2; group B: n = 2) who complained of heartburn prior to the operation were asymptomatic, only one group A patient developed symptoms of reflux, and oesophageal pH-monitoring was normal in the 6 group B patients investigated at follow-up. The laparoscopic approach reduces the magnitude of the operation, and the magnified overview permits precise dissection of the intraparietal adhesions which may develop after numerous sessions of dilatation.     

Risk of second primary cancer in two-year survivors of small cell lung cancer]

A total of 498 patients with small cell lung cancer received chemotherapy with or without chest irradiation at Osaka Prefectural Habikino Hospital from October 1977 through December 1991. Sixty-one who survived for more than two years were evaluated to determine the incidence and anatomic patterns of redevelopment of small cell lung cancer and development of second primary cancers. The numbers of expected cancers were estimated by cumulating person years of observation from 2 years after the start of treatment for small cell lung cancer to the date of death. Second primary cancers were observed in seven patients (four cases of non-small cell lung cancer, two of gastric cancer, and one of prostate cancer). The risk of a second primary cancer was 3.2 times greater than in the general population (95% Cl: 1.3-6.6). the relations between occurrence of a second primary cancer and family history of cancer, smoking history, smoking cessation after treatment of small cell lung cancer, and thoracic irradiation were studied. Occurrence of a second primary cancer correlated with family history (relative risk 7.5, 95% Cl: 1.5-22) and smoking cessation (relative risk 3.2, 95% Cl: 1.2-6.9). Long-term survivors were more likely to have a second primary cancer than a relapse of small cell lung cancer. Therefore, long-term survivors should be closely monitored for second primary cancers. Meta-analyses of studies done at several institutions may provide more detailed information on the occurrence of second primary cancers after small cell lung cancer.     

Computed tomography and positron emission tomography in the pre-operative staging of oesophageal carcinoma

Because patients with carcinoma of the oesophagus usually present with advanced disease and surgery has a high mortality with cure in less than 10% of patients, pre-operative staging to select appropriate patients is necessary. Computed tomography (CT) plays an important role in staging but has well recognized limitations. Positron emission tomography (PET) which provides physiological information may therefore be a better alternative. OBJECTIVE: To compare the findings of CT and positron emission tomography (PET) with 2-[18fluorine]-fluoro-2-deoxy-D-glucose (FDG) in the pre-operative staging of oesophageal carcinoma. MATERIALS AND METHODS: Twenty-five patients with biopsy proven oesophageal cancer had pre-operative staging using CT and FDG-PET. The studies were read independently and full histological confirmation was obtained in 19 patients. Four parameters were studied: the primary tumour, peri-oesophageal lymph nodes, liver metastases and left gastric lymph nodes. RESULTS: PET visualized all primary tumours; CT missed one. CT identified 4/8 patients with involved peri-oesophageal nodes and PET 3/8. CT identified 5/9 patients with left gastric adenopathy and PET 1/9. PET visualized a liver metastasis missed on CT and appeared to be better in assessing residual tumour. PET did identify distant metastases not seen on CT in seven patients. CONCLUSIONS: The two techniques are both effective in showing the primary tumour and about equally sensitive in the demonstration of peri-oesophageal nodes. PET is probably more sensitive than CT for the detection of distant metastases.     

Oesophageal cancer coexisting with colorectal lesions

OBJECTIVE: To investigate the incidence of colorectal lesions in patients who present with oesophageal cancer. DESIGN: Prospective open study. SETTING: University hospital, Japan. SUBJECTS: 135 of 218 patients who presented with squamous cell carcinoma of the oesophagus during the seven year period 1988-1994 were randomly allocated to have a barium enema examination. INTERVENTIONS: Barium enema examinations, and if colorectal lesions were found, colonoscopy and biopsy. MAIN OUTCOME MEASURE: Incidence of coexistent colorectal lesions. RESULTS: No abnormal findings were found in 52 (39%), diverticula were present on 37 (27%), benign polypoid lesions in 51 (38%), and malignant lesions in 6 (4%). We examined the clinical and histopathological details of all patients to see if it was possible to distinguish the patients at high risk of developing oesophageal and colorectal cancer but could find no differences among the groups. CONCLUSION: Asymptomatic colorectal lesions are relatively common in patients with squamous cell carcinoma of the oesophagus in Japan.     

K-ras point mutations are rare events in premalignant forms of Barrett's oesophagus

OBJECTIVE: In Barrett's adenocarcinomas, in contrast to squamous oesophageal carcinomas, K-ras point mutations are thought to be a frequent event. The frequency of K-ras point mutations in premalignant forms of Barrett's oesophagus (metaplasia, dysplasia) leading to adenocarcinoma with increased risk is currently not known. To establish the frequency of K-ras mutations in premalignant forms of Barrett's oesophagus, we investigated oesophageal biopsy specimens with Barrett's metaplastic and dysplastic epithelium for point mutations in the K-ras gene/codons 12, 13. DESIGN: A total of 412 biopsies from patients with Barrett's oesophagus were histologically classified into biopsies with metaplasia (n = 252), dysplasia (n = 105) and adenocarcinoma (n = 11), as well as biopsies distant from disease (normal, n = 37 and hyperplastic squamous epithelium, n = 7). METHODS: DNA from biopsy specimens was amplified by polymerase chain reaction (PCR) with a modified primer for generating a restriction site in the case of wild type in codon 12. Wild-type or point mutations in the K-ras gene/codons 12, 13 were detected by restriction fragment length analysis of the PCR products. RESULTS: Point mutations in K-ras/codon 12 were found in 9 biopsies (n = 1 in metaplasia, n = 4 in dysplasias, n = 4 in adenocarcinomas). All the other biopsies showed the wild type of K-ras/codon 12. No K-ras/codon 13 mutation (GGCgly-->GACasp) was observed. CONCLUSION: Mutations in K-ras/codon 12 were rarely found in premalignant forms of Barrett's oesophagus. Whereas the screening for K-ras point mutations in metaplastic sites of Barrett's epithelium seems not to be of practical value, the screening for mutations in dysplastic lesions might be helpful to estimate the individual risk for progression of Barrett's epithelium to adenocarcinoma. A further evaluation in larger numbers of patients is needed.     

A gene for FG syndrome maps in the Xq12-q21.31 region

AIMS: An increased risk of adenocarcinoma of the oesophagus has been demonstrated in patients with long segments of Barrett's mucosa. The risk of cancer associated with short segments of metaplasia of the oesophagogastric junction is not known. METHODS AND RESULTS: We report a case of early adenocarcinoma of the oesophagus arising on short tongues of Barrett's mucosa associated with an oesophageal cyst. The patient was a 68-year-old man with no previous clinical history of gastro-oesophageal reflux disease. The fortuitous discovery of an oesophageal cyst lead to the diagnosis of short tongues of Barrett's mucosa with high-grade dysplasia. On pathological examination of the resected specimen, an early adenocarcinoma had developed in Barrett's mucosa, localized just above the oesophageal cyst. CONCLUSIONS: As oesophageal cysts can cause symptoms suggestive of reflux, we hypothesize that this association may not be fortuitous.     

Human skin irritation studies of a lecithin microemulsion gel and of lecithin liposomes

OBJECTIVES: (1) To examine the prevalence and extent of intramural metastasis in squamous cell carcinomas of the oesophagus so as to delineate the resection margins for these tumours; (2) to devise an appropriate method for measurement of these lesions which takes into account of the contraction of the specimens after resection. METHODS: Oesophagectomy specimens were prospectively collected from 96 patients (87 males, nine females) with primary oesophageal squamous cell carcinoma over a two year period. The sizes of the tumours were measured in situ, after resection and after application of muscle relaxant (to regain their in situ length). The specimens were then serially sectioned for histological examination. RESULTS: The sizes of the tumours measured after application of muscle relaxant roughly corresponded to those measured in situ. Intramural metastasis was observed in 26% of the cases. Sixty four per cent (16 cases) of these were on the oral side, 72% (18 cases) on the gastric side, and 25% (nine cases) on both sides of the tumours. The most distant extent of intramural metastasis from the primary tumour was from 0.5 cm to 7.7 cm (mean = 3.4 cm) on the oral side, and 0.5 to 9.5 cm (mean 4 cm) on the gastric aspect of the tumour. Intramural metastasis was seen only in patients in whom the primary cancer had deep muscle infiltration. Multiple neoplastic lesions could be detected in 33% of the patients. Both intramural metastasis and multiple neoplastic lesions were associated with extensive lymph node infiltration. However, they had different histological features and extent of infiltration. CONCLUSIONS: Intramural metastasis was frequently observed in oesophageal squamous cell carcinoma. This implies that excision with wide margins should be considered for local control of the disease.     

Smoking tobacco, oral snuff, and alcohol in the etiology of squamous cell carcinoma of the head and neck: a population-based case-referent study in Sweden

BACKGROUND: This case-referent study was conducted to elucidate the role of selected exogenous agents in the etiology of head and neck cancer. The factors studied were tobacco smoking, alcohol intake, the use of moist oral snuff, dietary factors, occupational exposures, and oral hygiene. In this first report, the authors discuss the impact of tobacco smoking, the use of oral snuff, and alcohol consumption. METHODS: The study base was approximately 2 million person-years at risk and consisted of Swedish males age 40-79 years living in 2 geographic regions during the years 1988-1990. A total of 605 cases were identified in the base, and 756 controls were selected by stratified random sampling from population registries covering the base. RESULTS: Among those who were tobacco smokers at the time of the study, the relative risk of head and neck cancer was 6.5% (95% confidence interval, 4.4-9.5%). After cessation of smoking, the risk gradually declined, and no excess risk was found after 20 years. The relative risk associated with alcohol consumption of 50 grams or more per day versus less than 10 grams per day was 5.5% (95% confidence interval, 3.1-9.6%). An almost multiplicative effect was found for tobacco smoking and alcohol consumption. CONCLUSIONS: Tobacco smoking and alcohol intake had a strong interactive effect on the risk of squamous cell carcinoma of the head and neck. Moderate alcohol intake (10-19 grams per day) had little or no effect among nonsmokers. No increased risk was found for the use of Swedish oral snuff.     

Obesity and cancer risk: a Danish record-linkage study

A cohort of 43,965 obese persons was accrued on the basis of discharge registrations from Danish hospitals, and incidence of cancer in the cohort was compared to that in the Danish population as a whole using indirect standardisation for age and period. Increased incidence was observed for cancer of the uterine corpus independently of age [114 cases, relative risk (RR) = 2.0, confidence interval 1.6-2.4], and for breast cancer in women above the age of 70 (133 cases, RR = 1.2). These findings are consistent with previous studies. In younger women, breast cancer occurred less frequently and ovarian cancer occurred more frequently than expected. Increased incidence was observed for cancers of the oesophagus (26 cases, RR = 1.9) and the liver (58 cases, RR = 1.9), probably reflecting an increased prevalence of excessive alcohol consumption in the cohort. Increased incidence was furthermore observed for cancers of the pancreas (101 cases, RR = 1.7), the prostate (96 cases, RR = 1.3) and the colon (195 cases, RR = 1.2), which may indicate the existence of risk factors which are common to obesity and to these cancers, for example, dietary habits. Kidney cancer was increased in women only. Overall, the incidence of cancer was increased by 16% in the cohort. The results were essentially unchanged by restriction to the subcohort of 8207 persons in whom obesity was the primary discharge diagnosis, and were also similar in the first year of follow-up after hospital discharge. Selection bias is, therefore, not likely to have influenced the results.     

Histochemistry of mucin secreting components in mucoepidermoid and adenosquamous carcinoma of the oesophagus

AIMS: To determine the direction of differentiation of the mucin secreting components in a rare group of oesophageal tumours--oesophageal squamous cell carcinomas with prominent mucin secreting components (mucoepidermoid carcinomas and adenosquamous carcinomas). METHODS: In a review of 617 cases of primary carcinoma of the oesophagus, 16 cases of squamous cell carcinoma with prominent mucin secreting components were studied using a battery of histochemical techniques. RESULTS: The mucin produced by these tumours was mixed and included a variable content of enzyme labile sialomucin (positive for mucicarmine, periodic acid Schiff, and alcian blue, and sensitive to sialidase digestion and negative for high iron diamine-alcian blue). Retrospective analysis of endoscopic biopsy specimens taken from these tumours showed that mucin was present in five (42%) cases. CONCLUSIONS: The glandular component of this group of tumours histochemically differentiated in the direction of oesophageal glands: examination of the mucin secreting component in squamous cell carcinoma in resected specimens is therefore required for recording the true incidence of this type of tumour.     

Review: Barrett's oesophagus in Taiwan

Barrett's oesophagus is the eponym applied to the columnar epithelium-lined lower oesophagus which is acquired as a complication of chronic gastro-oesophageal reflux (GER). Various complications seen in the Barrett's oesophagus, such as peptic ulcer, stricture, adenocarcinoma are named as Barrett's ulcer, Barrett's stricture-and Barrett's carcinoma, respectively. It is now generally accepted that Barrett's oesophagus is an acquired condition resulting from chronic repetitive GER. The frequency of Barrett's oesophagus seems to be higher in Caucasian than in Oriental or Negro populations. There is a tendency towards increasing prevalence rates all over the world, including Taiwan, due to the Westernization of diet, rapid growth in the elderly population, obesity etc. Almost 6% of the patients who manifest heartburn in GI clinics in Taiwan now suffer from GER, which is almost similar to the 7% reported by Nabel, (USA) in 1976. During the last 30 years, the incidence of esophageal adenocarcinoma has increased rapidly. Patients with Barrett's oesophagus have an increased risk of developing oesophageal adenocarcinoma and should be kept under surveillance. Regular follow-up, at least twice a year or preferably, every 2-3 months, for those patients with SCE using endoscopic surveillance and biopsy for those with severe dysphasia (oesophageal columnar intraepithelial neoplasia) in the surrounding area to detect Barrett's oesophagus cancer, is very important.     

Loss of heterozygosity in sporadic oesophageal tumors in the tylosis oesophageal cancer (TOC) gene region of chromosome 17q

From the genotyping of UK and US tylotic families with a high risk of oesophageal cancer we have previously localized the tylosis-associated cancer susceptibility gene (TOC gene, tylosis oesophageal cancer gene) to a 1 cM region on the long arm of chromosome 17 (Kelsell et al., 1996). In the present study we investigated loss of heterozygosity (LOH) patterns of 35 sporadic squamous cell carcinomas of the oesophagus using six polymorphic microsatellite markers encompassing this locus. Twenty-four of the 35 cases (69%) revealed LOH at one or more loci. Deletion was most frequently observed with the marker D17S801 (64% LOH, informative cases), which shows significant linkage to the TOC locus. The LOH analysis in sporadic oesophageal cancer we report here is thus consistent with the hypothesis that the tylosis oesophageal cancer susceptibility gene is also involved in the pathogenesis of a proportion of sporadic squamous cell carcinomas of the oesophagus.     

False positive MIBG scan

OBJECTIVE: An increased age-related incidence of oesophageal cancer in people with intellectual disability has been suggested by studies in the Netherlands. Gastro-oesophageal reflux disease (GORD), as documented by pH testing, occurs frequently in the intellectually disabled population, being found in nearly 50% of those with an IQ less than 50, while Barrett's oesophagus is found in about 15-26%. DESIGN: We compared the age-related incidence of oesophageal cancer in institutionalized, intellectually disabled individuals in the Netherlands with the age-related incidence in the general Dutch population. METHODS: Data were provided by the Netherlands Cancer Registry. The patient's institute physician was asked to complete a questionnaire about the diagnosis, which was endoscopically and histologically confirmed. RESULTS: The incidence of oesophageal carcinoma was 20 in 168,000 person-years. The expected incidence for oesophageal cancer, based on age-related incidence in the general population, was 7.0, resulting in a standardized morbidity ratio in the population with intellectual disability of 2.9 (confidence limits, 1.8-4.1; P < 0.001). Endoscopic findings were as follows: in 18/20 intellectually disabled carcinoma patients an adenocarcinoma was found; the remaining two patients had a squamous cell carcinoma. Barrett's epithelium was observed in nine patients (45%), eight (42%) of whom showed a peptic stricture as well. In 15 (75%) cancer patients reflux oesophagitis was found, accompanied in 14 cases by a hiatal hernia. CONCLUSION: A standardized morbidity ratio for oesophageal carcinoma of 2.9 was found in the intellectually disabled population as compared to the general population. Early detection and treatment of GORD in the population with intellectual disability is of paramount importance to prevent the development of Barrett's dysplasia and carcinoma.