Survival of patients with Stage III colon cancer is improved in hereditary non-polyposis colorectal cancer compared with sporadic cases. A Danish registry based study.

AIM: Patients with hereditary non-polyposis colorectal cancer (HNPCC) seem to have a better prognosis than those with sporadic colorectal cancer (CRC). The aim was to compare survival after Stage III CC in patients with HNPCC with those having sporadic CC. METHOD: A total of 230 patients with hereditary cancer from the Danish HNPCC Register and 3557 patients with sporadic CC from the Danish Colorectal Cancer Database, diagnosed during May 2001-December 2008, were included. HNPCC patients were classified according to mismatch repair mutation status and family pedigree. Sporadic cases had no known family history of cancer. Patient characteristics, geographical differences and survival data were analysed. RESULTS: The overall survival (OS) was better in HNPCC patients compared with sporadic CC after stratification for sex and age (P = 0.02; CI 1.04-1.7). The 5-year survival was 70% in HNPCC patients compared with 56% in sporadic CC (P < 0.001). No survival difference was found between HNPCC subgroups but a tendency to better OS was seen in patients with Lynch syndrome. No geographical differences in OS were found. The median follow-up was 3.9 (0-9.5) years for HNPCC vs 3.2 (0-9.6) years for sporadic CC. CONCLUSION: HNPCC patients with Stage III CC have a better OS compared with sporadic CC. No significant difference in OS was found within HNPCC subgroups.

[Registration of hereditary non-polyposis colorectal cancer]. / Registrering af hereditaer non-polypøs kolorektal cancer.

Hereditary non-polyposis colorectal cancer is a dominant inherited disease, with development of colorectal cancer (CRC) and other cancers too. About 3% of all CRC-cases belong to an HNPCC-family. Mutations responsible for the disease has been identified in five genes, all of them involved in DNA mismatch repair. Since the establishment of the Danish HNPCC Register 345 families have been referred, and 101 of these families had HNPCC. Median age of onset for CRC was 50 years: Approximately 60% of the tumors were situated on the right side, 9% had synchronous tumors and the risk of metachronous tumours was 40% in 20 years. Tumours were localised in 64% of the cases, and the 5-year crude survival rate was better in HNPCC compared to sporadic CRC. The number of CRC diagnosed as Dukes A in HNPCC has risen since 1990, and the survival after CRC in HNPCC has increased. The disease specific mutation is identified in 21 families, from which 152 persons at risk have had a molecular genetic diagnose, and 83 could afterwards evade screening protocols. Few years after the establishment of the HNPCC-registry, it is indicated that information, registration and screening lead to an earlier diagnosis and a better prognosis after CRC.

[Hereditary non-polyposis colorectal cancer]. / Hereditaer non-polyposis kolorektal cancer.

Hereditary non-polyposis colorectal cancer (HNPCC) probably constitutes 5% of all the cases of sporadic colorectal cancer. At present, the diagnosis can only be established on the basis of a family history which should fulfill the "Amsterdam criteria": 1) Colorectal cancer in at least three family members, 2) One family member must be a close relative of the other two, and 3) The diagnosis must have been established prior to the age of 50 years in at least one relative. Other forms of cancer also occur in the HNPCC syndrome, particularly endometrial cancer. The syndrome has a dominant inheritance and, therefore, all close relatives should be submitted to control examinations for the most important forms of cancer associated with the syndrome.

The pelvic floor muscles: muscle thickness in healthy and urinary-incontinent women measured by perineal ultrasonography with reference to the effect of pelvic floor training. Estrogen receptor studies.

Maintenance of urinary continence is multifactorial and depends mainly on detrusor control and urethral closure function. The closure forces can be categorized as permanent closure forces active at rest, and adjunctive closure forces active during physical activities. The efficiency of these forces depends on the structural components in the urethral wall, the position of the bladder neck and proximal urethra, the periurethral striated muscles, and the pelvic floor muscles. By means of pudendal blockade and simultaneous recordings of pressure and cross-sectional area in the urethra, it has been demonstrated that the striated periurethral muscles and the pelvic floor muscles are of paramount importance for the closure function. This emphasizes the importance of well-functioning pelvic floor muscles to obtain continence, and probably explains the rationale for the effect of pelvic floor training in treating urinary incontinence. This study presents a review of the literature on female urinary incontinence, continence mechanisms, pelvic floor muscles, and pelvic floor training. Furthermore, a review of the literature on estrogen receptors in the pelvic floor muscles is given. Perineal ultrasonography, a method for visualization and measurement of thickness of the pelvic floor muscle, was developed and evaluated. This method was used to gain information on the thickness of the pelvic floor muscles in younger physiotherapists, healthy women, and women suffering from urinary incontinence, and to evaluate the effect of pelvic floor training. Additionally, a study of the Pelvic floor muscles was performed to assess the presence of estrogen receptors. Muscle thickness seems to decrease with age. In women over age 60 years, a significantly thinner pelvic floor muscle was found compared to younger women. The muscle increment during contraction decreased significantly with age, probably reflecting a stronger pelvic floor or a better awareness of pelvic floor function in the younger women. Incontinent women had a thinner pelvic floor muscle compared to healthy women. Hypertrophy of the muscles was demonstrated in urinary-incontinent women after pelvic floor training, and the difference in thickness of the muscles in these women before training compared to healthy women was eliminated by training. pelvic floor training reduced the use of incontinence appliances and urinary leakage both in stress and urge-incontinent women. Subjectively, 60% of the women gained a positive effect of the training. In spite of the fact that training increased muscle thickness and the increment of muscle thickness during contraction, no correlation between these parameters and subjective improvement or reduced urine loss in the pad weighing test could be demonstrated. Training may strengthen the pelvic floor without effect on the multifactorial continence mechanism in cases where urinary incontinence is caused by destruction of the urethral attachment to the surrounding tissue. No estrogen receptors were found in the nuclei of striated muscle cells in biopsies from levator ani muscles, using an immunohistochemical technique. Thus, the effect of estrogen treatment on the striated pelvic floor muscles is doubtful. A possible effect of estrogen treatment of urinary incontinence must be mediated via other structures than the pelvic floor muscles.

Iatrogenic psoas abscess. Case report.

A case of iatrogenic pneumococcus psoas abscess is reported. The etiology was probably repeated local anaesthetic blockades in the lumbogluteal structures because of lumbago.

Barrett's oesophagus.

Barrett's oesophagus is defined as the occurrence of columnar epithelium extending for more than 3 cm up into the tubular part of the oesophagus. The average age at the time of diagnosis is 55 years. The condition is most often seen in men and is rare among negroid populations. The condition is caused by a combination of pronounced gastro-oesophageal reflux, hypersecretion of acid by the stomach, motoric and sensory dysfunction in the oesophagus, as well as increased aggressiveness of the refluxed material. The diagnosis is made by endoscopy, taking biopsies. Three types of histological epithelium occur: specialized columnar epithelium, junctional-type epithelium and gastric fundus-type epithelium. Barrett's oesophagus is a premalignant condition. Severe dysplasia is correlated with the development of oesophageal adenocarcinoma. The incidence of the latter varies between 1:441 and 1:52 per patient-year. The treatment of Barrett's oesophagus is either medical treatment or surgery. The medical treatment includes H2 receptor antagonists or omeprazole. Antireflux surgery is indicated in cases resistant to medical treatment. Resection is the only possible curative treatment when severe dysplasia or adenocarcinoma is present. Recommendations are made, based on the available literature, as to the treatment and follow-up of patients with Barrett's oesophagus.

Hepatoblastoma in two cousins in a family with adenomatous polyposis. Report of two cases.

Two cases of hepatoblastoma in cousins in a family with familial adenomatous polyposis (FAP) are reported. Twenty-five cases of hepatoblastoma with family history of FAP have been documented in the literature, but there has never been a report of two cases of hepatoblastoma in the same polyposis family.

Malignant mesenchymoma of the scrotum.

Paratesticular sarcomas are rare, especially the malignant mesenchymoma. To our knowledge only four cases of paratesticular malignant mesenchymoma have been described previously. All were localized to the spermatic cord. We present a case of malignant mesenchymoma in the scrotum free of the spermatic cord.

Bricker's ileal conduit urinary diversion with a simple non-refluxing uretero ileal anastomosis.

Fifty consecutive patients had ileal conduits constructed with a technically and quick simple antireflux ureteroileal anastomosis. Complications related to the ureteral implantation were studied retrospectively, and at follow-up (8 months-12 years later, median 3 years) conduit dysfunction and ureteral reflux were assessed in 18 patients out of the 25 patients who were still alive. Early complications and signs of late upper urinary tract deterioration were similar to those found after other operative techniques had been used. One patient had a postoperative urinary leak from the uretero ileal anastomosis. which was treated successfully by two weeks drainage. Hydronephrosis deteriorated in 18 (26%) of the renal units, remained unchanged in 39 (57%) and improved in 11 (16%). Increases in plasma creatinine concentrations up to 200 mumol/l were found in eight patients, and in one patient it increased from 300 to 420 mumol/l. Partial ureteral reflux was present in three (2 patients) of 33 ureters studied and minimal conduit dysfunction was found in 8 patients. In conclusion we find this method of urinary diversion to be quick, easy, and safe.

Mandibular osteomas in sporadic colorectal carcinoma. A genetic marker.

Pantomography of the mandible was performed in 98 patients with sporadic colorectal adenocarcinoma. Twenty-eight patients (29%) had osteomas versus 5% in a control group (P < 0.001). Mandibular osteomas are found in most patients with the premalignant dominant syndrome familial adenomatous polyposis. Sporadic colorectal cancer examinations of married couples have shown that diet has only a moderate influence on the development of colorectal cancer, whereas pedigree studies indicate a genetic component. On this basis we conclude that mandibular osteomas are probably genetic markers of the development of sporadic colorectal carcinoma.