Aim: To evaluate the risk factors and long-term prognosis of metachronous rectal cancer in the remnant rectum of patients with familial adenomatous polyposis (FAP). Patients and Methods: Sixty-five patients (49 families) who underwent prophylactic surgery, including bowel resection, for FAP between January 1976 and August 2022 at Hamamatsu University Hospital were included and divided into two groups based on the presence of metachronous rectal cancer. Risk factors for metachronous rectal cancer development were analysed in cases treated with total colectomy with ileorectal anastomosis (IRA) and stapled total proctocolectomy with ileal pouch anal anastomosis (IPAA) (IRA, n=22; stapled IPAA n=20; total, n=42). Results: The median surveillance period was 169 months. Twelve patients developed metachronous rectal cancer (IRA, n=5; stapled IPAA, n=7), of which six with advanced cancer died. Patients who temporarily dropped out of surveillance were significantly more likely to have metachronous rectal cancer (metachronous vs. non-metachronous rectal cancer: 33.3% vs. 1.9%, p<0.01). The mean duration of surveillance suspension was 87.8 months. Cox regression analysis revealed that temporary surveillance drop-out independently affected the risk (p=0.04). The overall survival associated with metachronous rectal cancer was 83.3% at 1 year and 41.7% at 5 years. Overall survival was significantly worse in advanced cancer than in early cancer cases (p<0.01). Conclusion: Temporary drop-out from surveillance was a risk factor for metachronous rectal cancer development, and advanced cancer had a poor prognosis. Continuous surveillance of patients with FAP, without temporary drop-out, is strongly recommended.
The patient was a 38-year-old woman who had been diagnosed as having ileocolic Crohn's disease in 1994 and undergone resection of the ileocecal region and a part of the small intestine for ileal stenosis in 2004; the length of her residual small intestine was 230 cm. As symptoms of intestinal stenosis occurred despite postoperative medical therapy, in 2014, the patient was treated conservatively for 3 month, being prescribed exclusive intake of enteral formulas, followed by partial resection of the small intestine, including the anastomotic site of the previous operation. Prophylactic antibiotics were used only on the day of the surgery. While the preoperative blood tests showed no abnormalities of the coagulation profile, the PT and APTT were mildly prolonged one day after the surgery and markedly prolonged 6 days after the surgery (PT 72.8 seconds, APTT 98.3 seconds). Vitamin K deficiency was suspected, although there were no clinical symptoms. Her coagulation ability improved rapidly after intravenous infusion of a vitamin K preparation. Patients with Crohn's disease have multiple risk factors for vitamin deficiencies; however, virtually no previous reports have documented vitamin K deficiency-induced coagulopathy that appeared perioperatively. We report a case of vitamin K deficiencyinduced coagulopathy that occurred in the early postoperative period.
Backgrounds
: This study aimed to evaluate the changes in the rates of genetic counseling and genetic testing as well as the diagnosis rate of Lynch syndrome (LS)-associated colorectal cancer before and after multistep approach with multidisciplinary team in Japanese.
Methods
In September 2016, we started universal screening for LS by mismatch repair protein immunohistochemistry and prospectively collected the records. Following patient interviews, we started multistep approach with multidisciplinary team (MA) in January 2020. MA consists of six surgeons, one genetic counselor, one medical geneticist, and six pathologists. MA is set up to compensate for patients’ lack of knowledge about genetic diseases and make case selection for elderly colorectal cancer patients with deficient mismatch repair (dMMR). MA is designed as a system that could be performed by a small number of medical genetic specialists. A total of 522 patients were included during the study duration, 323 and 199 patients in the pre-MA (P-MA) and MA groups, respectively.
Results
The frequency of dMMR in all patients was 10.0%. The patient interview results indicated a significant lack of patient education regarding genetic diseases. The rates of genetic counseling and genetic testing was significantly higher in MA group than in P-MA group (genetic counseling: MA 34.6% vs. P-MA 7.7%, p = 0.04; genetic testing: MA 30.8% vs. P-MA 3.8%, p = 0.02). Moreover, the diagnosis rate of LS-associated colorectal cancer was significantly higher in MA group (2.5%) than in P-MA group (0.3%) (P = 0.03). In addition, MA could be performed without problems despite the small number of medical and human genetics specialists.
Conclusions
MA has achieved appropriate pickup of suspected hereditary colorectal cancer patients and complemented the lack of knowledge about genetic diseases. The introduction of MA increased LS-associated colorectal cancer after universal screening. MA is an appropriate LS screening protocol for Japanese patients who lag behind in medical and human genetics education.
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