Effort, safety, and findings of routine preoperative endoscopic evaluation of morbidly obese patients undergoing bariatric surgery.

BACKGROUND: Obesity is becoming an epidemic health problem and is associated with concomitant diseases, such as sleep apnea syndrome and gastroesophageal reflux disease (GERD). There is no standardized diagnostic workup for the upper gastrointestinal tract in obese patients; many patients have no upper gastrointestinal symptoms, and few data are available on safety of endoscopy in morbidly obese patients. METHODS: Sixty-nine consecutive diagnostic upper gastrointestinal endoscopies in morbidly obese patients (26 men, 43 women; mean age 43.4 +/- 10.9 years) were prospectively evaluated from January to December 2008 in an outpatient setting before bariatric procedures. Sedation was administered with propofol. Data on sedation, critical events, and examination times were recorded, as well as pathological findings. RESULTS: The patients' mean body mass index was 47.6 +/- 7.9 (range, 35.1-73.3) kg/m(2); 17.4% reported GERD symptoms. The mean duration of the endoscopy procedure (including sedation) was 20.3 +/- 9.3 (range, 5-50) min, and the whole procedure (including preparation and postprocessing) took 58.2 +/- 19 (range, 20-120) min. The mean propofol dosage was 380 +/- 150 (range, 80-900) mg. Two patients had critical events that required bronchoscopic intratracheal O(2) insufflation due to severe hypoxemia (<60% SaO: (2)). Nearly 80% of patients had pathological findings in the upper gastrointestinal tract. Only 20% reported upper gastrointestinal symptoms. Pathologic conditions were found in the esophagus in 23.2% of the patients, in the stomach in 78.2%, and in the duodenum in 11.6%. The prevalence of Helicobacter pylori infection was 8.7%. CONCLUSIONS: Upper gastrointestinal endoscopy can be performed safely. However, careful monitoring and anesthesiological support are required for patients with concomitant diseases and those receiving sedation. Because 80% of the patients with pathological findings were asymptomatic, every morbidly obese patient should undergo endoscopy before bariatric surgery because there may be findings that might change the surgical strategy.

Transient lower esophageal sphincter relaxation in morbid obesity.

BACKGROUND: There is strong evidence that morbid obesity is often accompanied by gastroesophageal reflux. Gastroesophageal reflux is caused predominantly by transient lower esophageal sphincter relaxations (TLESRs). Only few data are available about TLESRs in patients with stage III obesity (body mass index > 35). The aim of this study was to analyze the frequency and types of TLESRs in patients with morbid obesity in different physiological stages (postprandial: upright and recumband) compared to patients with normal weight gastroesophageal reflux disease (GERD) and diffuse esophagus spasm (DES). METHODS: In order to measure TLESRs in obese patients with and without GERD, three subgroups were prospectively performed: group I consisted of seven healthy controls, group II consisted of seven obese patients, group III consisted of seven non-obese patients with GERD, and in group IV, five patients were recruited with diffuse esophageal spasm. All participants underwent both conventional water-perfused stationary esophagus manometry and a 24-h ambulatory esophagus manometry, 24-h ambulatory pH monitoring, and esophago-gastroscopy. In order to measure the lower esophageal sphincter pressure (LESP) over a prolonged time under physiological conditions, a special solid-state sleeve catheter was used. Additionally, all patients were interviewed using a standardized questionnaire. RESULTS: Compared to normal subjects, patients with morbid obesity and patients with gastroesophageal reflux show a substantial increase of TLESRs in the postprandial phase. There was a tendency towards more TLESRs per hour in patients with DES than in healthy subjects, but the difference was not statistically significant. The types of TLESRs differed with the LESP. The majority of isolated TLESRs were complete and incomplete. Some of the isolated TLESRs were accompanied by contractions of the tubular esophagus. CONCLUSION: Morbid obesity is associated with gastroesophageal reflux. The frequency of TLESRs has significantly increased compared to healthy subjects and does not differ statistically from patients with GERD. Isolated TLESRs are mostly incomplete in patients with a hypotonic LES.

Neoadjuvant continuous infusion of weekly 5-fluorouracil and escalating doses of oxaliplatin plus concurrent radiation in locally advanced oesophageal squamous cell carcinoma: results of a phase I/II trial.

Oxaliplatin and 5-fluorouracil have a significant activity in locally advanced oesophageal squamous cell cancer (OSCC). However, their optimal dosage and efficacy when combined with concurrent radiotherapy as neoadjuvant treatment are unknown. This non-randomised, phase I/II study aimed to define the maximum tolerated dose (MTD) and assessed the histopathological tumour response rate to neoadjuvant oxaliplatin in weekly escalating doses (40, 45, 50 mg m(-2)) and continuous infusional 5-fluorouracil (CI-5FU; 225 mg m(-2)) plus concurrent radiotherapy. Patients had resectable OSCC. Resection was scheduled for 4-6 weeks after chemoradiotherapy. During phase I (dose escalation; n=19), weekly oxaliplatin 45 mg m(-2) plus CI-5FU 225 mg m(-2) was established as the MTD and was the recommended dosage for phase II. Oesophageal mucositis was the dose-limiting toxicity at higher doses. During phase II, histopathological responses (<10% residual tumour cells within the specimen) were observed in 10 of 16 patients (63%; 95% confidence interval: 39-82%). Overall, 16 of the 25 patients (64%) who underwent resection had a histopathological response; tumour-free resection (R0) was achieved in 80%. Neoadjuvant weekly oxaliplatin 45 mg m(-2) plus CI-5FU 225 mg m(-2) with concurrent radiotherapy provides promising histological response rates and R0 resection rates in locally advanced OSCC.

[Curative vs palliative strategies in locoregional recurrence of gastrointestinal malignancies]. / Therapeutische Strategien bei lokoregionalen Rezidiven gastrointestinaler. Tumoren Palliation oder Kuration?

Locoregional recurrence is a relevant problem in surgical oncology. Intraluminal local relapse only occurs when the primary resection is carried through with inappropriate safety margins. If possible, a second surgical resection with wider margins is the treatment of choice. When the primary resection was appropriately done, extraluminal relapse in the original tumor bed indicates the primary tumor was already in an advanced stage. The indication for a second resection must be considered carefully. A realistic prospect of long-term tumor control exists only when the second resection yields tumor-free margins and is combined with chemo- and radiotherapy. There is usually no curative treatment option for recurrences in the draining lymph node region, the so-called fourth dimension.

Esophageal cancer: patient evaluation and pre-treatment staging.

Improvements in the overall survival of patients with esophageal cancer can in the future only be achieved by tailored therapeutic strategies which are based on the individual histologic tumor type, tumor location, tumor stage at the time of presentation, consideration of established prognostic factors and the physiologic status of the patient. The major aim of every diagnostic strategy is to assess whether a complete macroscopic and microscopic tumor resection (i.e. an R0 resection) can be achieved by primary surgical approach with a high degree of likelihood. This requires histologic classification of the tumor type (squamous cell cancer or adenocarcinoma), the exclusion of distant solid organ metastases, localization of the primary tumor in relation to the tracheobronchial tree, and determination of the T-category and the surrounding structures of the primary tumor. This is currently achieved by a combination of contrast radiography, endoscopy with biopsy, endoscopic ultrasonography and CT scan. PET scanning will in the future be more widely used in esophageal cancer staging because it appears to be superior to current imaging modalities in the exclusion of distant solid organ and lymph node metastases and allows early assessment of response of the primary tumor to neoadjuvant treatment. Systematic risk analysis with a dedicated composite scoring system is essential to assess the physiologic status of the patient and reduce postoperative mortality. Only hospitals with a sufficient case load of esophageal cancer patients ('hospital volume') and a dedicated interest in the management of this disease ('centers of excellence') can provide the required expertise and standards for patient evaluation and tailored therapy.

Responders benefit from neoadjuvant radiochemotherapy in esophageal squamous cell carcinoma: results of a prospective phase-II trial.

BACKGROUND: We present the results of a prospective phase-II-study of neoadjuvant combined radiochemotherapy followed by surgical resection in patients with histological proven locally advanced squamous cell carcinoma of the esophagus located at or above the level of the tracheal bifurcation. METHODOLOGY: Between February 1995 and March 2000 a total of 76 patients with esophageal squamous cell carcinoma (uT3/4N0/+-categories) received simultaneous combined neoadjuvant radiochemotherapy consisting of a continuous intravenous infusion of 5-fluorouracil (300 mg/m2/day) 7 day per week concurrently with conventional fractioned external beam radiation therapy (2 Gy/day), five fractions per week up to a total dose of 30 Gy. RESULTS: Radiochemotherapy related acute severe toxicity rate (CTC-grade-III) occurred in 34 patients, two patients died. Sixty-four patients underwent surgery with a complete resection in 48 patients. Three patients died during a 90-day post-operative course. The histopathological workup revealed no viable residual tumour cells in eight patients (ypCR) and according to the modified criteria of Mandard in 26 patients a histopathological response. Twenty-two of these patients underwent a R0-resection. The median follow-up time was 5.4 years with an overall median survival time of 20.6 months. The median survival in the 26 responders was 32.3 months versus 19.5 months in 38 non-responders (p=0.03). CONCLUSIONS: Patients with locally advanced squamous cell carcinoma of the esophagus, who respond to preoperative neoadjuvant combined radiochemotherapy, seem to have more benefit from subsequent resection than non-responding patients.

New aspects of prognostic factors in adenocarcinomas of the small bowel.

BACKGROUND/AIMS: Primary small bowel tumors are rare and the prognosis is generally considered to be poor. Histologically chiefly adenocarcinomas are reported. The surgeon is challenged in their treatment, because of the infrequency, unspecific symptoms and delay in diagnosis. Retrospectively we investigated the surgical therapy, combined morbidity, survival rates and prognostic factors in a large series of primary adenocarcinomas of the small bowel at a single surgical center. METHODOLOGY: Between 1985 and 1998, 94 patients with a primary tumors of the small bowel (malignant n = 62 [65.9%], benign n = 32 [34.1%]) were operated on. The subgroup of the adenocarcinomas (n = 22) were considered for this study. RESULTS: The median follow-up is 8.4 years (range: 0.9-14.2 years). Sixteen patients had a follow-up more than 5 years. The main surgical procedure was a small bowel segment resection. Morbidity was 13.6% (only in patients with a duodenal tumors) and the 30-day mortality 5.6%. The estimated 2-year-survival rate was 66%, the 5-year-survival rate 45%. Univariate analysis identified the presence of the residual tumor (R-status) (P = 0.004), tumor stage according to the UICC (P = 0.01), lymph node metastasis (P = 0.007), distant metastasis (P = 0.001), lymphangiosis carcinomatosa (P = 0.001) and vascular invasion (P = 0.0008) as prognostic factors. CONCLUSIONS: A complete macroscopic and microscopic tumor resection including a systemic lymph node dissection has to be the aim of any curative surgical approach in patients with adenocarcinoma of the small bowel.

Treatment of acute abdominal pain in the emergency room: a systematic review of the literature.

Appropriate pain therapy prior to diagnosis in patients with acute abdominal pain remains controversial. Several recent studies have demonstrated that pain therapy does not negatively influence either the diagnosis or subsequent treatment of these patients; however, current practice patterns continue to favour withholding pain medication prior to diagnosis and surgical treatment decision. A systematic review of PubMed, Web-of-Science and The-Cochrane-Library from 1929 to 2011 was carried out using the key words of 'acute', 'abdomen', 'pain', 'emergency' as well as different pain drugs in use, revealed 84 papers. The results of the literature review were incorporated into six sections to describe management of acute abdominal pain: (1) Physiology of Pain; (2) Common Aetiologies of Abdominal Pain; (3) Pre-diagnostic Analgesia; (4) Pain Therapy for Acute Abdominal Pain; (5) Analgesia for Acute Abdominal Pain in Special Patient Populations; and (6) Ethical and Medico-legal Considerations in Current Analgesia Practices. A comprehensive algorithm for analgesia for acute abdominal pain in the general adult population was developed. A review of the literature of common aetiologies and management of acute abdominal pain in the general adult population and special patient populations seen in the emergency room revealed that intravenous administration of paracetamol, dipyrone or piritramide are currently the analgesics of choice in this clinical setting. Combinations of non-opioids and opioids should be administered in patients with moderate, severe or extreme pain, adjusting the treatment on the basis of repeated pain assessment, which improves overall pain management.

Multi-Parametric MRI-Directed Focal Salvage Permanent Interstitial Brachytherapy for Locally Recurrent Adenocarcinoma of the Prostate: A Novel Approach.

Even with the technological advances of dose-escalated IMRT with the addition of the latest image guidance technologies, local failures still occur. The combination of MRI-based imaging techniques can yield quantitative information that reflects on the biological properties of prostatic tissues. These techniques provide unique information that can be used for tumor detection in the treated gland. With the advent of these improved imaging modalities, it has become possible to more effectively image local recurrences within the prostate gland. With better imaging, these focal recurrences can be differentially targeted with salvage brachytherapy minimizing rectal and bladder toxicity. Here we report a novel use of MRI-directed focal brachytherapy after local recurrence. This technique offers a unique opportunity to safely and successfully treat recurrent prostate cancer, previously treated with definitive radiation therapy. The use of multi-parametric MRI-directed focal salvage permanent interstitial brachytherapy for locally recurrent adenocarcinoma of the prostate is a promising strategy to avoid more aggressive and expensive treatments that are associated with increased morbidity, potentially improving survival at potentially lower costs.

[Peritoneal carcinomatosis]. / Peritonealkarzinose.

Cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (HIPEC) is now an established therapy with a curative option for patients with gastrointestinal and gynecological peritoneal carcinomatosis as well as for primary peritoneal carcinomatous tumors. Decisive for the prognosis is a complete cytoreduction, which in most cases necessitates multi-organ resection in addition to a partial or subtotal parietal peritonectomy (PE). The highest priority is given to maintain an adequate quality of life for the patient while performing maximum tumor resection. The morbidity following PE and HIPEC in experienced centers lies between 25% and 35% with a mortality risk of <5%. Consideration must be given not only to the technical surgical aspects and the intraoperative decision-making but also to the intraoperative management, intensive care therapy, pain therapy, management of complications, physiotherapy and many more. The greatest challenge in the management of peritoneal carcinomatosis is still patient selection. Computed tomography imaging together with (18)fluorodeoxyglucose positron emission tomography (FDG-PET) plays an important role in the assessment of operability.

[Peritoneal carcinomatosis of colorectal cancer: cytoreductive surgery and hyperthermic intraperitoneal chemotherapy]. / Peritoneal Metastasiertes Kolorektales Karzinom: Zytoreduktive Chirurgie und Hypertherme Intraperitoneale Chemotherapie.

Peritoneal carcinomatosis caused by colorectal carcinoma is still considered as the end-stage of disease. A multi-modal therapeutic concept including maximal cytoreduction followed by intraperitoneal hyperthermic chemotherapy (HIPEC) has the potential to cure selected patients. In case of peritoneal carcinomatosis palliative systemic treatment is no longer the state of the art. This article addresses aspects of the disease, the rationale behind peritonectomy with HIPEC, and the surgical management of peritoneal carcinomatosis.

[Esophageal squamous cell carcinoma: pre-operative combined radiochemotherapy from a surgical oncological viewpoint]. / Plattenepithelkarzinom des Osophagus : Präoperative kombinierte Radiochemotherapie aus chirurgisch-onkologischer Sicht.

Esophageal squamous cell carcinomas (ESCCs) are an interdisciplinary challenge in terms of diagnosis, multimodal and/or surgical treatment procedures and also postoperative management as they are often associated with multiple comorbidities. The gold standard for a curative treatment approach is radical surgery, which is standardized and can be carried out with acceptable morbidity and mortality rates. ESCCs are usually diagnosed at locally advanced tumor stages and neoadjuvant treatment procedures are therefore used. Patients who respond to neoadjuvant therapy (responders) have a significantly better survival rate. The neoadjuvant studies that are currently available need to be critically assessed as they do not include response as an end point. In this context the WHO clinical response evaluation that has been used up to now is questionable. The histopathological findings with percentage proportions of residual tumor cells represent the gold standard for evaluating the response. Positron-emission tomography as a response criterion is predictive for the histopathological response and survival. ESCC patients who are classified as non-responders do not appear to benefit from surgical resection. In the future the results of a response evaluation and of pretreatment molecular biological tests could have a place in the process of pretherapeutic and peritherapeutic oncological decision-making in patients with ESCCs.

[Selection criteria for peritonectomy with hyperthermic intraoperative chemotherapy (HIPEC) in peritoneal carcinomatosis]. / Selektion zur Peritonektomie mit hyperthermer intraoperativer Chemotherapie (HIPEC) bei Peritonealkarzinose.

BACKGROUND: Cytoreductive peritonectomy with hyperthermic intraoperative chemotherapy (HIPEC) is an established therapy for patients with gastrointestinal, gynaecological metastasised peritoneal carcinomatosis as well as primary peritoneal carcinomatous tumours. METHODS: On the basis of a literature review and our personal experience, selection criteria for peritonectomy are discussed. RESULTS: Computed tomography (CT) scans and diagnostic laparoscopy are not sufficient for the diagnosis of peritoneal carcinomatosis. The combination of fluorodeoxyglucose positron emission tomography (FDG-PET) and CT seems to be the most reliable diagnostic imaging method. In our institution, all patients undergo PET / CT prior to peritonectomy. CONCLUSION: The PET / CT scan may play an important role in forecasting the operability of patients with peritoneal carcinomatosis.

The predictive value of molecular markers (p53, EGFR, ATM, CHK2) in multimodally treated squamous cell carcinoma of the oesophagus.

Pretherapeutic identification of oesophageal squamous cell carcinomas that will respond to neoadjuvant chemoradiotherapy is an important attempt for improvement of patient's prognosis. In the current study, pretherapeutic biopsies from 94 oesophageal squamous cell carcinomas (cT3, cN0/+, cM0) in patients who underwent neoadjuvant chemoradiotherapy (RCTx: 45 Gy plus cisplatin and 5-fluorouracil) and subsequent oesophagectomy in the setting of a single-centre prospective treatment trial were investigated by means of immunohistochemistry. Expression of proteins involved in DNA repair and/or cell-cycle regulation, that is p53, p53 (phosphorylated at Ser15), EGFR, ATM protein kinase (phosphorylated at Ser1981) and checkpoint kinase 2 (CHK2) (phosphorylated at Thr68) was correlated with the response to RCTx and with overall survival. Tumours that were positive for CHK2 expression more frequently showed clinically determined regression after RCTx (69.4%) than tumours that were negative for CHK2 expression (32.1%; P=0.0011), whereas other parameters did not correlate with tumour regression. Expression of ATM correlated with expression of CHK2 (P=0.0061) and p53-phospho (P=0.0064). Expression of p53 correlated with expression of p53-phospho (P<0.0001). In contrast to clinical and histopathological response evaluation, none of the molecular parameters under investigation correlated with overall survival. In conclusion, expression analysis of p53, EGFR CHK2 and ATM has no predictive value in multimodally treated oesophageal squamous cell carcinoma.

Squamous cell carcinoma and Zenker diverticulum.

Squamous cell carcinoma in a Zenker diverticulum is a very rare condition. We report a case of a patient with a Zenker carcinoma, who was primarily functionally inoperable and therefore received neoadjuvant radiochemotherapy before cardiac bypass surgery. After a complicated course with cardiogenic shock and myocardial infarction, a re-evaluation of functional risk analysis and the tumor situation revealed operability. Subsequently, partial hypopharyngectomy and partial cervical esophageal resection with lymphadenectomy was performed. Reconstruction of the gastrointestinal continuity was made by interposition of a free small bowel graft and microvascular anastomosis. The postoperative course showed a small anastomotic leakage of the hypopharyngeal-small bowel anastomosis, which was successfully treated conservatively.

Expression of cyclo-oxygenase 1 and 2, prostaglandin E synthase and transforming growth factor beta1, and their relationship with vascular endothelial growth factors A and C, in primary adenocarcinoma of the small intestine.

BACKGROUND: Primary adenocarcinomas of the small intestine are rare. The prostaglandin biosynthetic pathway plays a major role in carcinogenesis and is linked with angiogenesis in various tumours. Promotion of tumour growth by transforming growth factor (TGF) beta may be mediated through the prostaglandin pathway. METHODS: Expression of cyclo-oxygenase (COX) 1 and 2, prostaglandin E synthase (PGES), TGF-beta1 and vascular endothelial growth factor (VEGF) A and C genes was analysed in 54 primary adenocarcinomas of the small intestine and corresponding normal intestinal mucosa. All patients had undergone surgical resection without previous antineoplastic therapy. Target gene expression was analysed at the mRNA level by reverse transcriptase-polymerase chain reaction and correlated with clinicopathological parameters as well as survival. COX-2 protein expression was examined by immunohistochemistry. RESULTS: Expression of COX-2 protein was detected immunohistochemically in 98 per cent of the carcinomas. COX-1, COX-2, VEGF-A, VEGF-C, PGES and TGF-beta1 mRNA expression varied markedly in different tumours, but all were overexpressed compared with levels in normal intestinal mucosa. There were significant associations between levels of COX-1, COX-2, TGF-beta1 and PGES mRNAs and those of VEGF-A and VEGF-C. CONCLUSION: Correlations between levels of mRNA for COX-1, COX-2, TGF-beta1 and PGES and those for proangiogenic factors VEGF-A and VEGF-C suggest a role for these factors in the propagation of primary adenocarcinomas of the small intestine.

[Impalement injury of the neck]. / Pfählungsverletzung des Halses.

In comparison to the United States or South Africa, penetrating injuries of the neck are rare in Europe. Most of these traumas are due to sharp perforation mechanisms. We report on a 43-year-old man who was admitted to the emergency room because of an impressive transcervical penetrating neck trauma inflicted by a chisel. He survived the trauma since the chisel missed all important structures of the neck. The diagnostic strategy to evaluate the dimension of the trauma was primarily based on endoscopic and surgical exploration.

Lymphatic vessel invasion is an independent prognostic factor in patients with a primary resected tumor with esophageal squamous cell carcinoma.

BACKGROUND: Little data exist about the prognostic role of a lymphatic vessel invasion (LVI) in patients with esophageal carcinoma. The objective of this study was to clarify the presence and prognostic impact of LVI in a large group of patients resected for esophageal squamous cell carcinoma (SCC) at one surgical center. METHODS: Three hundred sixty-six patients, who had a primary resection for SCC, were analyzed by univariate and multivariate analysis. Follow-up was complete for 93.7% patients with a median follow-up of 8.3 years. RESULTS: The total rate of LVI was 39.1% (n = 143). Univariate analysis revealed a significant relation between LVI and different T classifications (P = 0.001), N classifications (P < 0.0001), M classifications (P < 0.0001), International Union Against Cancer (UICC) stages (P < 0.0001), and residual tumor (P < 0.0001). Multivariate analysis of the patients with R0-resected tumors proved LVI as an independent prognostic factor. The 2-, 5- and 10-year survival rates in patients with LVI were 28.5%, 11.1%, and 9.2% compared with 63.4%, 46.6%, and 27%, respectively, without LVI (P < 0.0001). Patients with LVI had a median survival time of 11.4 months compared with 28.6 months without LVI (P < 0.0001). Patients with R0-resected tumors without LVI had a median survival time of 54.1 months compared with 12.1 months in patients with LVI (P < 0.0001) and compared with 11.3 months in patients with R1-resected tumors P < 0.0001). CONCLUSIONS: These data clearly show that LVI is an independent prognostic factor in patients with SCC and confirm the importance of a systematic pathohistologic workup. The prognosis of patients with R0-resected tumors with LVI is equal to patients with an incomplete tumor resection. This supports the inclusion of LVI in the UICC classification system for esophageal carcinoma.

Achalasia and esophageal cancer: incidence, prevalence, and prognosis.

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.