Sulindac and tamoxifen in the treatment of desmoid tumours in patients with familial adenomatous polyposis.
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Journal Article↗
Biomedical subjects
S Bülow
Publications and source records attributed to S Bülow.
Decision analysis in the surgical treatment of patients with familial adenomatous polyposis: a Dutch-Scandinavian collaborative study including 659 patients.
BACKGROUND AND AIMS: The choice of colorectal surgery in patients with familial adenomatous polyposis lies between the morbidity of proctocolectomy and ileum-pouch-anal anastomosis (IPAA) and the mortality from rectal cancer after total colectomy and ileorectal anastomosis (IRA). The aims of the present study were: (1) to assess the risk of dying from rectal cancer after IRA, (2) to compare the life expectancy between patients with an IRA and those with an IPAA, and (3) to investigate whether regular endoscopic examination of the rectum leads to detection of cancer at an earlier stage. METHODS: Clinical and pathological data on 659 patients who underwent colectomy and ileorectal anastomosis were collected from four national polyposis registries-that is, in Denmark, Finland, Sweden, and the Netherlands. Data were analysed using survival analysis methods. Decision analysis was used to compare the life expectancy between patients with an IRA and those with an IPAA. RESULTS: A total of 47 patients developed rectal cancer after IRA. The risk of dying from rectal cancer was 12.5% (95% confidence interval 7.1--17.9%) by age 65. Compared with IRA, IPAA would lead to an increase in life expectancy of 1.8 years. Seventy five per cent of patients with rectal cancer had a negative rectoscopy within 12 months before the diagnosis. CONCLUSION: IRA is associated with substantial mortality due to rectal cancer. Follow up examinations of the rectum does not have sufficient preventive effect on morbidity and mortality of rectal cancer.
Adenomatous Polyposis Coli↗
[Desmoid tumor in familial adenomatous polyposis].
INTRODUCTION: Desmoid tumors (DT) are rare benign tumors that do not metastasize, but tend to invade locally. DT are frequently seen in patients with familial adenomatous polyposis (FAP), and diagnosis and treatment are often difficult. METHOD: The article presents the clinical picture, diagnosis and treatment of DT in patients registered in the Danish Polyposis Register by the end of 1999. RESULTS: Twenty-seven of 486 patients (6%) had DT. Eighteen patients were alive at the time of evaluation. DT were found in the mesentery in 42%, in the abdominal wall in 40%, in the retroperitoneum in 8% and only 10% on the extremities. Fifty percent of the patients had complications (intestinal obstruction, hydronephrosis or fistulas), and 2/9 deaths were caused by DT. Ninety-three percent were treated with surgery, NSAIDs, antioestogenic drugs, chemotherapy or radiotherapy, but all modalities proved disappointing, except for treatment with a combination of the NSAID sulindac and tamoxifen. Five patients treated with this combination showed extensive and long lasting response. DISCUSSION: Surgical excision is recommended in patients with DT in the abdominal wall. First line treatment of mesenteric DT is Clinoril in combination with tamoxifen. Elective surgery may be considered in patients with a small well-defined DT with no signs of invasion of vital structures, and in patients with imminent bowel ischaemia or obstruction. The prognosis for mesenteric DT is grave, and improvement of the therapeutic strategy awaits current international studies.
Abdominal Neoplasms↗
Asthma mortality in Danish children and young adults, 1973-1994: epidemiology and validity of death certificates.
Several reports indicate that asthma mortality has increased during the last few decades. International comparisons reveal some striking differences in the pattern of asthma mortality. The authors investigated the asthma mortality rate in the Danish child and youth population 1973-1994 and studied the validity of death certificates. The authors reviewed all death certificates coded as asthma death in the International Classification of Diseases (ICD 8-ICD 10 (1994)) and adjacent respiratory code numbers for the age group 1-19 yrs. Hospital records and autopsy reports were assessed to validate the cause of death. Age-standardized and age-specific mortality rates were calculated. From 1973 to 1987 there was a significant upward trend in the mortality. On subdivision, this trend was limited to the age group 15-19 yrs. Generally the mortality rate decreased from 1988 to 1994. Four per cent coded as asthma were false positive. Twelve per cent were false negative asthma deaths, wrongly coded as due to other causes. Only 62% of all true positive death caused by asthma were appropriately coded. The number of false negative certifications increased with increasing autopsy frequency. Asthma mortality rates in Denmark increased in adolescents during 1973-1987 and decreased from 1988 to 1994. A possible explanation may be an increased awareness of asthma symptoms combined with a steadily improved treatment of asthma. Even in children and young adults under the age of 20 yrs, validity problems still make comparisons between countries difficult; even interpretation of national trends requires caution.
Adolescent↗
Ileorectal anastomosis is appropriate for a subset of patients with familial adenomatous polyposis.
BACKGROUND & AIMS: This study reevaluates the risk of rectal cancer and the frequency of subsequent proctectomy for nonmalignant causes in patients with familial adenomatous polyposis (FAP) who have undergone colectomy with ileorectal anastomosis (IRA). Potential risk factors for rectal cancer in this setting are also examined, and recommendations for the choice of surgical procedure are made. METHODS: The national polyposis registries in Denmark, Finland, The Netherlands, and Sweden included 659 patients undergoing surgery with IRA in 1940-1997. Kaplan-Meier analysis and Cox regression analysis were performed to evaluate cumulative risk, survival, and predictive risk factors. RESULTS: Rectal carcinoma was diagnosed in 47 patients, with a cumulative 40-year risk of 0.32. The cumulative risk according to chronologic age was 0.30 at age 60, and higher in patients undergoing surgery above age 25 (P = 0.0016). Chronologic age was the only independent risk factor (P = 0.0016). The cumulative 5-year survival rate after rectal carcinoma was 0.60. The apc mutation was known in 167 patients, of whom 7 had rectal cancer. The cumulative 40-year risk of secondary proctectomy was 0.70, and higher in patients with a mutation in codon 1250-1500 than outside this region (P = 0.005). However, all 7 rectal cancers were found in the latter group. None of the 18 patients with attenuated FAP (mutation in codon 0-200 or >1500) had a secondary proctectomy. CONCLUSIONS: IRA is recommended in (1) young patients with few rectal adenomas and a family history of a mild phenotype and (2) patients with attenuated FAP (a mutation in codon 0-200 or >1500), provided there is acceptance of life-long rectal surveillance. Patients with many rectal polyps and/or a family history of severe polyposis should be offered a restorative proctocolectomy with an ileal pouch-anal anastomosis.
Adenoma↗
Causes of death in familial adenomatous polyposis.
BACKGROUND: The prognosis in familial adenomatous polyposis (FAP) has improved over the past decades owing to a reduction in the prevalence of colorectal cancer, resulting from effective early screening. During the same period several polyposis registers have recorded an increasing number of deaths due to duodenal/periampullary cancer and desmoid tumours. The aim of this study was to examine the causes of death with special emphasis on duodenal/periampullary cancer. METHODS: The material consisted of 328 patients (144 females and 184 males) registered from I January 1943 to 31 December 1992 in the Danish Polyposis Register. The standard mortality rate (SMR) was calculated for known major causes of death, using the entire Danish population as background population. The attributable risk was also calculated for selected death causes. RESULTS: One hundred and thirty-three patients had died, SMR being 4.98 (95% confidence limits, 4.17-5.90). There were significantly lower SMRs in the call-up group than in the proband group. The late cohort (1943-1992) had lower SMRs than the early group (1889-1942). SMR was significantly increased for death due to colorectal cancer (145), duodenal cancer (214), and ovarian cancer (30). No deaths due to desmoids were observed in the examination period. The attributable risk for colorectal cancer was 29% and for duodenal cancer only 0.6%. CONCLUSION: Colorectal cancer is the most frequent cause of death in polyposis patients, followed by duodenal/periampullary cancer, but the latter is still a rare cause of death in FAP.
Adenomatous Polyposis Coli↗
[Local treatment of anal fissure with nitroglycerin ointment].
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Administration, Topical↗
[Carcinoid tumors of the colon and rectum].
The article gives a review of the literature concerning carcinoid tumours of the colon and rectum. Carcinoid tumours of the rectum are more common than of the colon, although these tumours are rarities. The carcinoid tumour grows slowly and the patients can survive for years with the disease. Treatment of the rectal carcinoid depends on the size and invasiveness of the primary tumour, as a non-invasive tumour less than 2 cm in diameter can be locally excised. For carcinoids of the colon, the treatment of tumours of less than 2 cm and without invasion will be local excision. For larger and/or invasive tumours in both colon and rectum the treatment is resection. The prognosis for colonic carcinoids is worse than for rectal carcinoids. Adjuvant treatment forms are briefly described. Follow-up programmes for these patients are generally long (over five years) and include recto-/colonoscopy, and search for metastatic spread.
Carcinoid Tumor↗
[Local recurrence after low anterior resection of rectal cancer].
Among 75 consecutive patients operated upon with anterior resection for rectal adenocarcinoma during a five year period, 29 (39%) developed local cancer recurrence. The total cumulative five-year survival was 49%, but only 17% in those with a local cancer recurrence. The most important risk factors for development of local recurrence were tumour fixation, intraoperative blood transfusion and surgical routine. Local recurrence was seen in 4/23 (17%) after operation performed by a consultant, 1/5 (20%) after a consultant-supervised operation and 24/47 (51%) after operation by a senior registrar (p < 0.02). As a consequence we recommend that operation for rectal cancer should only be performed or supervised by a few specialists in colorectal surgery.
Adenocarcinoma↗
[Patients' perception of symptoms in colorectal cancer. A cause of delay in diagnosis and treatment].
A questionnaire was given to a series of 50 patients referred for colorectal cancer to the Department of Surgical Gastroenterology at Hvidovre Hospital, Copenhangen. The patients were asked about the length of the period from debut of symptoms to the first visit to their family doctor (patient's delay), detailed symptoms and their views and attitudes towards the significance of these symptoms. More than half of the patients first saw their own doctor more than three months after the first symptom: 17/33 (52%) with a change in bowel habits, 12/28 (43%) with rectal bleeding, 11/19 (58%) with abdominal pain and 4/6 (67%) with a palpable mass. Only 7/44 (15%) thought that cancer was the cause of their symptoms, and a similar minority was afraid of an operation or an ostomy. We conclude that the main cause of patient's delay is a lack of knowledge among the patients about the significance of bowel symptoms, rather than fear of cancer, operation or an ostomy. On this basis we recommend that an information campaign about cancer-related bowel symptoms (bleeding, change of bowel habits) should be carried out with the aim of reducing patient's delay and thereby possibly also improving the prognosis through an early diagnosis.
Colonic Neoplasms↗
[Anastomotic leakage after low anterior resection for rectal cancer].
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.
Adenocarcinoma↗
Is screening for thyroid carcinoma indicated in familial adenomatous polyposis? The Leeds Castle Polyposis Group.
Forty-five polyposis patients with thyroid carcinoma constituted 1.2% of the patients in the Leeds Castle Polyposis Group database. The patients were diagnosed during 1959-1995; 44 were females at a median age of 25 years (range 10-40) and 37 (82%) had papillary carcinoma. At the end of 1995 only one patient (9%) had died from thyroid carcinoma, and the ten-year cumulative survival was 84% (95% confidence limits 72-97). Due to the low incidence of thyroid carcinoma in FAP and the good prognosis we do not recommend a regular thyroid screening in polyposis, as this is unlikely to result in a reduction of the mortality, but will only aggravate existing cancrophobia in these strained patients.
Adenomatous Polyposis Coli↗
Decision analysis in the management of duodenal adenomatosis in familial adenomatous polyposis.
BACKGROUND: Patients with familial adenomatous polyposis are not only at high risk of developing adenomas in the colorectum but a substantial number of patients also develop polyps in the duodenum. Because treatment of duodenal polyps is extremely difficult and it is unknown how many patients ultimately develop duodenal cancer, the value of surveillance of the upper digestive tract is uncertain. AIMS: (1) To assess the cumulative risk of duodenal cancer in a large series of polyposis patients. (2) To develop a decision model to establish whether surveillance would lead to increased life expectancy. METHODS: Risk analysis was performed in 155 Dutch polyposis families including 601 polyposis patients, and 142 Danish families including 376 patients. Observation time was from birth until date of last contact, death, diagnosis of duodenal cancer, or closing date of the study. RESULTS: Seven Dutch and five Danish patients developed duodenal cancer. The lifetime risk of developing this cancer by the age of 70 was 4% (95% confidence interval 1-7%) in the Dutch series and 3% (95% confidence interval 0-6%) in the Danish series. Decision analysis showed that surveillance led to an increase in life expectancy by seven months. CONCLUSIONS: Surveillance of the upper digestive tract led to a moderate gain in life expectancy. Future studies should evaluate whether this increase in life expectancy outweighs the morbidity of endoscopic examination and proximal pancreaticoduodenectomy.
Adenomatous Polyposis Coli↗
Hereditary non-polyposis colorectal cancer: clinical features and survival. Results from the Danish HNPCC register.
BACKGROUND: Hereditary non-polyposis colorectal cancer (HNPCC) is a dominantly inherited syndrome characterized by the development of colorectal cancer (CRC) and other carcinomas. Our aim was to evaluate tumour parameters and survival in HNPCC. METHODS: One hundred and eight Danish HNPCC patients were compared with 870 patients with sporadic colorectal cancer. RESULTS: The median age at CRC diagnosis was 41 years in the HNPCC group. HNPCC patients had significantly more carcinomas located to the right colon (68% against 49% in controls), more synchromous tumours (7% versus 1%), more metachronous CRC after 10 years (29% versus 5%), more localized carcinomas (62% versus 39%), and significantly higher crude cumulative 5-year survival (56% versus 30%). CONCLUSIONS: CRC in HNPCC behaves differently compared to sporadic cases concerning age of onset, frequency of multiple lesions, and location. The metastatic tendency is less than in sporadic CRC and the survival is better.
Adult↗
[Prognosis in familial adenomatous polyposis. Results from the Polyposis Registry].
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.
Adenomatous Polyposis Coli↗
[Hereditary colorectal cancer].
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Adenomatous Polyposis Coli↗
[Surgery for ulcerative colitis. Treatment with proctocolectomy, stapled ileum-J-pouch, stapled pouch-anal anastomosis and temporary ileostomy].
Thirty-two patients with ulcerative colitis, median age 29 (range 14-49), were submitted to restorative proctocolectomy. Twenty-five patients had a three-stage procedure and seven had a two-stage procedure. A stapled J-pouch was formed, and a pouch-anal anastomosis was created by the double stapling technique. A temporary end ileostomy was closed through peristomal incision after three months. There were no pouch failures and no cases of pouch-anal anastomosis leakage. In one patient secondary mucosectomy and neo-anastomosis became necessary due to severe inflammation of remnant rectal mucosa. Five patients were operated for small bowel obstruction, and two had to have a dilatation of a slight stricture of the pouch-anal anastomosis. In two patients the final diagnosis was verified or probable Crohns disease, of whom one developed recurrence of a previous rectovaginal fistula. Twenty-seven patients have had the ileostomy closed for more than one month, 25 of these (93%) were fully continent three months after ileostomy closure and later on. After one year the patients had median five (range 3-9) bowel movements per day. It is concluded that restorative proctocolectomy with a stapled J-pouch-anal anastomosis and a temporary end ileostomy for ulcerative colitis carries few complications and provides a good functional result.
Adolescent↗
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.
Adenomatous Polyposis Coli↗