Professor MRB Keighley
The optimum prophylactic operation for cancer in familial adenomatous polyposis
University Department of Surgery, Queen Elizabeth Hospital, Birmingham, England
Familial adenomatous polyposis is a Mendelian dominant with an inevitable risk of colon cancer if left untreated. However, the risk of colon cancer and the time at which cancer develops varies with the genetic mutation on chromosome 5, which also influences the incidence of extra-intestinal disease and other factors which could determine the most appropriate surgical operation.
There is thus enormus heterogeneity in familial adenomatous polyposis in terms of colorectal polyps, other of potential malignancy and desmoids. The number of colorectal polyps veries with the sepcific site of mutation as do the malignant potential of the polyps and the distribution of the polyps. Furthermore, the risk of bile induced cancer in the bile duct, duodenum and small bowel also is influenced by the mutation on the APC gene. In addition, the risk of gastric polyps of thyroid cancer, central ner-vous system tumours, bone tumours and subcutaneous tumours is also influenced by the genetic predisposition and site of mutation. Finally, the risk of desmoids either in the wound, in the retroperitoneal tissues or in the pelvis is genetically determined. Since all of these issues play a role in deciding upon the most appropriate surgical operation in FAP, more genetic information and its impact on prognosis is needed.
Quite apart from genetic information, there are other issues that need to be taken into consideration when deciding upon the most appropriate operation for familial adenomatous polyposis. These include patient intelligence, patient expectations, reliability of individuals in attending follow up, body image and the fact the their operation is a prophylactic procedure when patients have no symptoms must also be considered.
The principal surgical options in the ablation of the large bowel for prevention of colorectal cancer include: a) proctocolectomy and permanent ileostomy, b) subtotal colectomy and ileorectal anastomosis, which leaves the rectal stump still at risk and c) restorative proctocolectomy and ileopouch anal anastomosis. However for ileopouch anal anastomosis to completely protect against malignancy a mucosectomy of the anal canal is needed. Having said that, the risk of cancer occurring in the anal canal is extremely low and it could, therefore, be argued that the risk is so small that a mucosectomy is not necessary and that it is perfectly justified to perform an operation with better functional results by stapled ileoanal anastomosis without anal mucosectomy.
Powyżej zamieściliśmy fragment artykułu, do którego możesz uzyskać pełny dostęp.