Duodenal adenomatosis in familial adenomatous polyposis.

BACKGROUND: The prevalence of duodenal carcinoma is much higher in familial adenomatous polyposis (FAP) than in the background population, and duodenal adenomatosis is found in most polyposis patients. AIMS: To describe the long term natural history of duodenal adenomatosis in FAP and evaluate if cancer prophylactic surveillance of the duodenum is indicated. METHODS: A prospective five nation study was carried out in the Nordic countries and the Netherlands. PATIENTS: A total of 368 patients were examined by gastroduodenoscopy at two year intervals during the period 1990-2001. RESULTS: At the first endoscopy, 238 (65%) patients had duodenal adenomas at a median age of 38 years. Median follow up was 7.6 years. The cumulative incidence of adenomatosis at age 70 years was 90% (95% confidence interval (CI) 79-100%), and of Spigelman stage IV 52% (95% CI 28-76%). The probability of an advanced Spigelman score increased during the study period (p<0.0001) due to an increasing number and size of adenomas. Two patients had asymptomatic duodenal carcinoma at their first endoscopy while four developed carcinoma during the study at a median age of 52 years (range 26-58). The cumulative incidence rate of cancer was 4.5% at age 57 years (95% CI 0.1-8.9%) and the risk was higher in patients with Spigelman stage IV at their first endoscopy than in those with stages 0-III (p<0.01). CONCLUSIONS: The natural course of duodenal adenomatosis has now been described in detail. The high incidence and increasing severity of duodenal adenomatosis with age justifies prophylactic examination, and a programme is presented for upper gastrointestinal endoscopic surveillance.

Ranitidine as adjuvant treatment in colorectal cancer.

BACKGROUND: Results from short-term studies of histamine type 2 (H2) receptor antagonists on survival of patients with solid tumours are debatable. In this study the efficacy of the H2-receptor antagonist ranitidine on long-term survival of patients with colorectal cancer was evaluated. METHODS: Patients scheduled for elective resection of primary tumours were consecutively included in a randomized double-blind placebo-controlled clinical study designed to evaluate the effect of ranitidine on survival. Before skin incision ranitidine 100 mg or placebo was given intravenously twice daily followed by oral ranitidine 150 mg or placebo twice daily for 5 years. Adjuvant cytotoxic or radiation therapy was not given. An observer-blinded interim analysis performed after 40 months showed that there was no effect of ranitidine on overall survival, and the study was discontinued in accordance with the protocol. The patient cohort has been followed continuously without loss of any patient, and a final statistical analysis was performed on an intention-to-treat basis after more than 5 years; this included a subgroup analysis of perioperative blood transfusion and postoperative infectious complications. RESULTS: The median observation period of the 740 patients included was 6.8 (range 5.4-7.9) years. A univariate analysis of all 740 patients and of the subgroup of 560 who underwent curative resection showed no significant effect of ranitidine on survival. Furthermore, ranitidine had no survival benefit in curatively resected patients who received a perioperative blood transfusion (n = 358), but it improved the survival of non-transfused patients (n = 202; hazard ratio (HR) 0.6 (95 per cent confidence interval (c.i.) 0.4 to 0.9), P = 0.02) and of non-transfused patients who did not develop postoperative infectious complications (n = 170; HR 0.6 (95 per cent c.i. 0.4 to 0.9), P = 0.01). In multivariate analysis of patients who had a curative resection, including Dukes' stage, age, gender, tumour location, blood transfusion, postoperative infectious complications and treatment, ranitidine still had an independent, beneficial effect on survival (HR 0.6 (95 per cent c.i. 0.4 to 1.0), P = 0.04) within the subgroup of patients who did not receive perioperative blood transfusion and did not develop postoperative infectious complications. CONCLUSION: Ranitidine may prolong the survival of patients who undergo curative resection of colorectal cancer and who do not receive perioperative blood transfusion and do not develop postoperative infectious complications.

Pancreaticoduodenectomy for periampullary cancer in patients more than 70 years of age.

BACKGROUND/AIMS: To assess the indications for and results of pancreaticoduodenectomy in patients more than 70 years old with periampullary cancer. METHODOLOGY: Thirty-four consecutive patients older than 70 years with periampullary cancer. The surgical procedure was pancreaticoduodectomy (Whipple's operation) with an extensive dissection of lymph nodes and the connective tissue in the peripancreatic region. Main outcome measures were postoperative morbidity and mortality, median and 5-year survival rates. RESULTS: Postoperative medical complications occurred in 24% and surgical complications in 53% of the patients. Four patients (12%) died in the postoperative period (within 30 days), and 3 patients (9%) died later in the postoperative course. The cumulative and age corrected 5-year survival rate for the remaining patients was 26%. Fifteen patients died of recurrence, and 7 patients of other causes. Five patients are still alive more than 5 years after surgery. In patients with noncurative operation the median survival time was 1 1/2 years, which is longer than would be expected from other palliative procedures. Apart from a moderately increased postoperative mortality the results were similar to those reported for younger patients. CONCLUSIONS: Pancreaticoduodenectomy should be considered in patients older than 70 years with resectable periampullary cancer. A 5-year survival rate of 20-35% can be obtained. Palliative resection may be indicated in patients in good general condition, as resection gives the best palliation and longer survival than other palliative methods.

[Recurrence and survival after conventional low anterior resection for rectal cancer]. / Recidiv og overlevelse efter konventionel lav anterior resektion for cancer recti.

INTRODUCTION: The aim of the study was to evaluate the incidence of recurrence of local cancer, distant metastases and survival after conventional low anterior resection for cure in patients with rectal carcinoma, on the basis of the poor prognosis after colorectal cancer in Denmark. MATERIAL AND METHODS: Consecutive patients operated on in the nine Danish departments of surgical gastroenterology in 1992-1993. Retrospective collection of data on recurrence of local cancer, distant metastases, and over-all survival at the end of 1996. RESULTS: Of 268 patients, 77 (29%) developed recurrent local cancer and/or distant metastases. Forty-eight (18%) had local recurrence with a cumulative 5-year rate of 39%. Distant metastases were seen in 54 (20%). The local recurrence rate increased with increasing Dukes' tumour stage and was higher after operation by a non-specialist (30%) than by a consultant, another specialist, or a surgeon under training and supervised by a consultant (15-17%) (p = 0.04). Multiple regression showed that the recurrence rate was independent of tumour localisation, blood loss, transfusion, anastomotic leakage, and status of the surgeon. The cumulative crude 5-year survival was 50% and independent of the status of the surgeon. DISCUSSION: Our relatively high local recurrence rate and the results in the literature after total mesorectal excision (TME) indicate that the conventional technique should be replaced by TME, which has become the recommended method in recent years. Furthermore, we propose a changed strategy in the treatment of rectal cancer. The patients should be treated in fewer departments with established teams of rectal cancer specialists taking part in all operations for rectal cancer.

[Surgical treatment of stomach cancer at a university hospital. A retrospective survey of the period 1990-1995]. / Kirurgisk behandling af cancer ventriculi på et universitetshospital. En retrospektiv opgørelse for perioden 1990-1995.

AIM: A retrospective study of 69 cases of gastric cancer seen during the period from 1/1-1990 to 31/12-1994 treated in a University Hospital. The aim of the study was to describe morbidity, mortality and identify independent prognostic variables for mortality. METHOD: Patient data were recovered from the hospital's central database. Mortality was chosen as end-parameter. Univariate log-rank-test identified statistically significant variables which were then analysed by Cox backward stepwise regressional analysis. MATERIAL: Sixty-nine patients were available for analysis, median age 73 years. Fifty-one patients underwent operation. Eighteen patients did not have a surgical procedure due to disseminated disease. The overall postoperative morbidity was 25% and postoperative mortality 10%. The overall five-year survival rate was 8%, 12% for operated patients, 35% after radical and 0% after non-radical or omitted surgery. Age, radicality of operation, type of operation, Borrmann's tumour classification, and degree of depth of local infiltration were identified as significant factors for survival. Cox's analysis identified type of operation (p = 0.0002) and Borrmann's tumour classification (p = 0.001) as independent variables. DISCUSSION: The overall five-year survival is low and has not changed over two decades in Denmark, whereas mortality and morbidity rates have improved. It should be recommended that: The treatment of gastric cancer must be centralised in order to develop preoperative examinations, operative technique and the necessary routine for the surgeons. All gastric ulcers must be considered malignant and biopsies taken accordingly.

Effects of the combination of blood transfusion and postoperative infectious complications on prognosis after surgery for colorectal cancer. Danish RANX05 Colorectal Cancer Study Group.

BACKGROUND: The frequency of postoperative infectious complications is significantly increased in patients with colorectal cancer receiving perioperative blood transfusion. It is still debated, however, whether perioperative blood transfusion alters the incidence of disease recurrence or otherwise affects the prognosis. METHODS: Patient risk variables, variables related to operation technique, blood transfusion and the development of infectious complications were recorded prospectively in 740 patients undergoing elective resection for primary colorectal cancer. Endpoints were overall survival (n = 740) and time to diagnosis of recurrent disease in the subgroup of patients operated on with curative intention (n = 532). The patients were analysed in four groups divided with respect to administration or not of perioperative blood transfusion and development or non-development of postoperative infectious complications. RESULTS: Overall, 19 per cent of 288 non-transfused and 31 per cent of 452 transfused patients developed postoperative infectious complications (P< 0.001). The median observation period was 6.8 (range 5.4-7.9) years. In a multivariate analysis, risk of death was significantly increased among patients developing infection after transfusion (n = 142) compared with patients receiving neither blood transfusion nor developing infection (n = 234): hazard ratio 1.38 (95 per cent confidence interval (c.i.) 1.05-1.81). Overall survival of patients receiving blood transfusion without subsequent infection (n = 310) and patients developing infection without preceding transfusion (n = 54) was not significantly decreased. In an analysis of disease recurrence the combination of blood transfusion and subsequent development of infection (hazard ratio 1.79 (95 per cent c.i. 1.13-2.82)), localization of cancer in the rectum and Dukes classification were independent risk factors. CONCLUSION: Blood transfusion per se may not be a risk factor for poor prognosis after colorectal cancer surgery. However, the combination of perioperative blood transfusion and subsequent development of postoperative infectious complications may be associated with a poor prognosis.

Preoperative plasma plasminogen activator inhibitor type-1 and serum C-reactive protein levels in patients with colorectal cancer. The RANX05 Colorectal Cancer Study Group.

BACKGROUND: Preoperative plasma plasminogen activator inhibitor-1 (PAI-1) is a prognostic variable in patients with colorectal cancer. It has been suggested, however, that plasma PAI-1 is a nonspecific prognostic parameter similar to the acute-phase reactant C-reactive protein (CRP). In the present study we analyzed the association between plasma PAI-1 and serum CRP in patients scheduled for elective resection of colorectal cancer. In addition, the prognostic value of PAI-1 and CRP was studied in this patient cohort. METHODS: PAI-1 and CRP were analyzed in citrated plasma and serum, respectively, obtained preoperatively from 594 patients. Patients who required preoperative blood transfusion received SAGM blood, in which soluble PAI-1 is not present. None of the patients received pre- or postoperative adjuvant chemotherapy, and all were followed in the outpatient clinic for at least 5 years or until death. The association of PAI-1 and CRP, respectively, with survival was tested using the median value of PAI-1 and the upper normal limit for CRP. Analyses were performed by inclusion of all patients, and in the subgroup of patients, who underwent curative resection. RESULTS: The median follow-up period was 6.8 (5.4-7.9) years. The median value of plasma PAI-1 was 35.8 ng/ml, and values greater than 94 nmol/L identified patients with increased CRP levels. Comparison of the molecules showed that PAI-1 was weakly correlated with CRP (r = .26; P <.0001). Both molecules showed a Dukes independent distribution. In univariate survival analyses high levels of PAI-1 were found associated with poor prognosis and low levels with good prognosis (P = .02, HR: 1.3). Similarly, high levels of CRP were found associated with poor prognosis and low levels with good prognosis (P <.0001, HR: 1.9). In a multivariate statistical analysis including Dukes classification, gender, age, tumor location, perioperative blood transfusion, PAI-1 and CRP, plasma PAI-1 was a dependent prognostic variable, while serum CRP (P <.0001; HR: 1.4; 95% CI: 1.3-1.5) was found to be a Dukes independent prognostic variable. Similar analyses, excluding patients with Dukes' D disease showed serum CRP to be an independent prognostic variable (P <.0001; HR: 1.3: 95% CI: 1.2-1.5). CONCLUSIONS: This study did not show a strong correlation between plasma PAI-1 and serum CRP in patients with colorectal cancer. Serum CRP was found to be a Dukes independent prognostic variable in this patient cohort, and was found to identify a subgroup of curatively resected patients at risk for short survival.

Independent prognostic value of eosinophil and mast cell infiltration in colorectal cancer tissue.

Overall peritumoural inflammatory cell infiltration is a prognostic variable in solid tumours, but the survival-related impact of the individual cell types within the infiltrate has still not been fully evaluated and compared with the conventional disease classification. In the present study, the prognostic value of individual white cell counts in the peritumoural inflammatory infiltrate in colorectal cancer was assessed. Intra-operative tumour tissue samples from 584 patients undergoing elective surgery for colorectal cancer were included. None of the patients received pre- or post-operative adjuvant chemotherapy. Tissue blocks were cut from the periphery of the tumours and embedded in paraffin. All blocks included both tumour tissue and normal bowel tissue. Serial sections of 4 microm were analysed for tumour tissue inflammatory cell infiltration using a computer- and video-assisted microscope, which allowed semi-automated quantification of cells within a fixed area. Total white cells and individual counts of eosinophils, neutrophils, mast cells, lymphocytes, and plasma cells were evaluated in every tumour specimen. Stratification into four groups with similar numbers of events was used to dichotomize the cell counts with respect to survival. The median observation period was 61 (49-75) months. In a multivariate analysis including Dukes' stage, gender, age, peri-operative blood transfusion, tumour location, and counts of specific inflammatory cells, only advanced Dukes' stage ( p< 0.0001), high age ( p=0.0003), and tumour location in the rectum predicted poor survival, while high counts of eosinophils ( p=0.006) and mast cells ( p=0.02) predicted good survival. Tumour-associated eosinophilia and mastocytosis appear to be independent prognostic variables in colorectal cancer. Future studies should investigate the potential biological role of tumour tissue eosinophils and mast cells in the modulation of tumour growth.

Percutaneous transhepatic cholelithotripsy for difficult common bile duct stones.

BACKGROUND AND STUDY AIMS: A study was carried out to assess the feasibility of a new rapid technique for percutaneous transhepatic access to the biliary tract with endoscopic lithotripsy (percutaneous transhepatic cholelithotripsy). PATIENTS AND METHODS: 14 patients with biliary stones resistant to endoscopic retrograde cholangiography and extracorporeal shock wave lithotripsy underwent cholelithotripsy, utilizing a new dilation kit with massive teflon dilators covered by "peel-away sheets". RESULTS: Successful lithotripsy was performed in all patients by laser lithotripsy through a choledochoscope or ureteroscope in ten patients and by stone removal by basket in the remaining four patients. The procedure was carried out using local anesthesia in the last 11 patients. Except for two patients with transient cholangitis, no complications occurred. CONCLUSIONS: Difficult bile duct and intrahepatic stones can be treated successfully with a simple percutaneous transhepatic cholelithotripsy procedure including local anesthesia, dilation and stone clearance.

The effect of ranitidine on postoperative infectious complications following emergency colorectal surgery: a randomized, placebo-controlled, double-blind trial.

OBJECTIVE AND DESIGN: To study the potential effect of ranitidine on postoperative infectious complications following emergency colorectal surgery. A randomized, placebo-controlled, double-blind trial was carried out in three university clinics and two county hospitals in Denmark. PATIENTS AND TREATMENT: One hundred and ninety-four consecutive patients undergoing acute colorectal surgery for perforated and/or obstructed large bowel were randomized in a double-blind fashion to receive ranitidine 100 mg i.v. twice a day commencing at induction of anesthesia and continued for five days (group I) or i.v. placebo (group II). All patients were given 1.5 g metronidazole plus 3.0 g cefuroxime at the time of surgery. Patients with perforation of the colon or rectum were given metronidazole and cefuroxime for further 3 days. All patients were assessed daily until discharge from the hospital. Thirty patients were withdrawn from the study (for reasons such as other diagnosis, refused to continue, medication not given as prescribed). MAIN OUTCOME MEASURES: Patients were observed for signs of infectious complications; such as wound infection, intra-abdominal abscess, septicemia, and pneumonia. RESULTS: Both groups were similar with respect to age, sex, weight, duration of surgery, blood transfusions, and site of the procedure, as well as the histologic nature of the underlying disease process. However, the Mannheim Peritonitis Index (MPI) was significantly higher in group I compared with group II (p < 0.05). Wound infection, intraabdominal abscess, septicemia, and pneumonia were 12.9%, 5.2%, 3.8% and 14%, respectively in group I. In group II, the infectious complications were 16.1%, 6.8%, 6.9% and 22%, respectively. Twelve patients (13.8%) in the placebo group developed more than one complication compared with 5 patients (6.5%) in the ranitidine group. CONCLUSION: Ranitidine may have a beneficial effect on postoperative infectious complications in patients following acute colorectal surgery.

[How much is the workload of surgeons in Scandinavia?]. / Hvor mye opererer kururgene i Norden?

According to figures presented at the Bianniul General Meeting of the Scandinavian Surgical Society, the mean number of operations performed per surgeon at some clinics of different sizes in Denmark, Finland, Iceland, Norway and Sweden ranged from 90 to 240 in 1996. This corresponds to 2.6 to 8.5 hours actual operating time, though figures are misleading since time spent assisting at operations, or on endoscopies, minor diagnostics and outpatient procedures, and essential pre- and post-operative tasks is not included. This level of operative activity is considered barely sufficient for training surgeons or for maintaining surgical skills. Surgeons could devote more time to surgery if a greater proportion of their non-surgical workload was taken over by other hospital staff, which would also reduce the number of surgeons required.

[Anastomotic leakage after low anterior resection for rectal cancer]. / Anastomoselaekage efter lav anterior resektion for cancer recti.

A series of 377 consecutive patients were operated upon with low anterior resection for rectal cancer in the nine Danish departments of surgical gastroenterology during 1992-1993. A retrospective analysis was carried out to calculate the frequency of anastomotic leakage and to evaluate factors of potential influence on the development of leakage according to the literature. Sixty-three patients (17%) developed leakage, which was followed by an increased mortality within the first three postoperative months. Only two variables significantly influenced the leakage rate: male gender was associated with a higher leakage rate (p = 0.02), whereas departments with a low number of rectal cancer surgeons had a low rate of anastomotic leakage (p = 0.02). In conclusion, the rather high frequency of anastomotic leakage calls for further clinical and pathogenetic research in this field. Until then, we recommend the routine use of a peroperative leakage test and selective use of prophylactic ostomy in cases of unsatisfactory anastomosis. Furthermore, it is recommended that low anterior resection for rectal cancer is limited to few surgeons in each department in order to ensure a uniform quality and hopefully also thereby reduce the rate of anastomotic leakage.

[Prognosis in familial adenomatous polyposis. Results from the Polyposis Registry]. / Prognosen ved familiaer adenomatøs polypose. Resultater fra polyposeregistret.

Regional and national polyposis registries have been established all over the world over last decades, with the aim to improve survival in patients with familial adenomatous polyposis (FAP). The Danish Polyposis Register was founded in 1971 and coordinates screening and prophylactic treatment. At the end of 1992 the register included 321 histologically verified FAP patients: 142/205 probands (69%) had colorectal cancer at the time of diagnosis of FAP versus only 2/116 call-up cases (2%). The 10-year cumulative crude survival was 94% among call-up cases versus only 41% among probands (p < 0.00001), and the survival increased significantly after establishment of the Polyposis Register in 1971. We conclude that centralized registration with identification and prophylactic examination of relatives at risk results in a substantial improvement of the prognosis.

Subcutaneous oxygen and carbon dioxide tensions during head-up tilt-induced central hypovolaemia in humans.

The effect of hypovolaemic shock on subcutaneous oxygen and carbon dioxide tensions was studied in man. Subcutaneous oxygen (Psc,O2) and carbon dioxide (Psc,CO2) tensions were monitored, during 50 degrees head-up tilt (anti-Trendelenburg's position)-induced central hypovolaemia, in two females and eight males, using a silicone tonometer on the lateral upper aspect of the right arm. All cardiovascular variables remained stable at rest. Incremental tilting to 50 degrees increased heart rate (HR) and mean arterial pressure (MAP) (p < 0.01), while stroke volume (SV), cardiac output (CO) and central venous saturation (SvO2) decreased (p < 0.05). Presyncopal symptoms appeared after 28 (8-48) min (mean and range) as HR decreased from 82 (63-108) to 52 (36-70) beats min-1 (p < 0.05), MAP from 88 (61-106) to 46 (37-54) mmHg and SvO2 from 0.68 (0.56-0.76) to 0.58 (0.39-0.70) (p < 0.01). On return of the tilt table to the horizontal position HR, MAP, SV, CO and SvO2 immediately re-established resting values. The Psc,O2 was 83 (72-102) mmHg at rest and during tilting it increased to 89 (82-111) mmHg followed by a decrease to 72 (58-97) mmHg (p < 0.01) at the appearance of presyncopal symptoms. Psc,O2 returned to the pretilt level over 45 (30-60) min of recovery. Arterial oxygen, carbon dioxide and Psc,CO2 did not change significantly. Subcutaneous oxygen tension decreases during marked central hypovolaemia and it returns slowly to the resting level during recovery. Psc,O2 is a more sensitive marker of impaired tissue oxygenation than arterial oxygen pressure.

Prophylaxis against postoperative complications in gastroenterology.

Gastrointestinal surgery results in pain, profound endocrine metabolic changes and organ dysfunction, immunosuppression and decreased resistance to infection, fatigue and convalescence. The main pathogenetic mechanism is the surgical stress response, which may be reduced by minimal invasive (laparoscopic) surgical techniques and afferent neural and perhaps humoral mediator blockade. Subsequently, these techniques have been documented as reducing a variety of postoperative morbidity parameters. A unifying concept for control of the postoperative period is presented as a combined effort to enhance preoperative information, stress reduction and sufficient functional pain relief allowing early mobilization and oral nutrition. Preliminary data, in combination with laparoscopic surgery, suggest that this approach improves outcome significantly.

The incidence rate of familial adenomatous polyposis. Results from the Danish Polyposis Register.

Based on the Danish Polyposis Register epidemiological calculations on familial adenomatous polyposis (FAP) were carried out. The mean annual incidence was 1.85 x 10(-6) during the years 1971-1992, and the prevalence increasing to about 32 x 10(-6) at the end of 1992. FAP patients constituted a decreased percentage of all Danish patients with colorectal cancer (0.07% in 1980-1992). The completeness of registration was 97% in 1983-1992. The results are similar to Finnish estimates based on the same direct method of calculation, and as both series are based on almost complete national polyposis registration in well-registered populations we regard our results to be close to the true incidence rate.

Effect of ranitidine and low-dose interleukin-2 in vitro on NK-cell activity in peripheral blood from patients with liver metastases from colorectal cancer.

Peripheral venous blood from 12 patients with colorectal cancer and eight healthy volunteers was used to identify the lowest in vitro dose of human, recombinant interleukin-2 (rIL-2) with immunoactivity on NK-cell lysis of K562 tumour cells. Subsequently, this dosage of 200 units/ml rIL-2, which may respond to 10(6) units in vivo, was used alone or in combination with ranitidine (0.02 mg/ml, which may correspond to 100 mg in vivo) to improve in vitro NK-cell activity in peripheral blood from 25 patients with liver metastases from colorectal cancer. A standard 4-hour Cr51-release assay of K562 tumour cells was used for the analyses. Spontaneous NK-cell activity was 19.0% (6.5-33.2), while ranitidine-induced NK-cell activity was 23.6% (7.8-46.2), and without statistical difference from spontaneous activity. Recombinant IL-2-induced NK-cell activity was 37.1% (11.1-71.7) (P < 0.05 compared to spontaneous activity), and rIL-2 plus ranitidine-induced NK-cell activity was 52.7% (18.9-85.6) (P < 0.05 compared to spontaneous and to rIL-2-induced activity, respectively). These results suggest a synergistic increase of low-dose rIL-2-induced NK-cell activity by ranitidine. Therefore, the combination of low-dose rIL-2 and ranitidine may be beneficial to improve post-operative immune competence, and should be considered in future adjuvant treatment regimens of cancer patients.

Centralized registration, prophylactic examination, and treatment results in improved prognosis in familial adenomatous polyposis. Results from the Danish Polyposis Register.

BACKGROUND: Over the last few decades numerous regional and national registers have been established all over the world with the aim of improving survival in familial adenomatous polyposis (FAP). The Danish Polyposis Register was founded in 1971 and coordinates the screening and subsequent prophylactic colectomy of FAP patients. METHODS: The crude cumulative survival in 321 patients (205 probands and 116 call-up cases) with verified FAP was calculated in accordance with the life-table method. RESULTS: At the time of diagnosis of FAP only 2 of 116 (2%) had colorectal cancer versus 142 of 205 probands (69%). The 10-year cumulative survival was 94% (95% confidence limits, 89-99) in call-up cases compared with only 41% (34-49) in probands (p < 0.00001), and survival improved significantly (p < 0.00001) after the establishment of the Danish Polyposis Register. CONCLUSION: The establishment of a centralized polyposis register has resulted in a substantial improvement of the prognosis in FAP.