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An alternative to prophylactic colectomy for colon cancer prevention in HNPCC syndrome
  1. H F A Vasen1,
  2. W H de Vos tot Nederveen Cappel2
  1. 1The Netherlands Foundation for the Detection of Hereditary Tumours, Leiden, the Netherlands
  2. 2Department of Gastroenterology, Leiden University Medical Centre, Leiden, the Netherlands
  1. Correspondence to:
    Dr H F A Vasen
    The Netherlands Foundation for the Detection of Hereditary Tumours, Rijnsburgerweg 10, 2333 AA, Leiden, the Netherlands;

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The surgical option for treatment of a patient with screen detected colorectal cancer (CRC) from a family with hereditary non-polyposis colorectal cancer (HNPCC) is subtotal colectomy or segmental resection. Using decision analysis, we showed that subtotal colectomy performed at a young age leads to an increased life expectancy (LE) of 1–2.3 years. Based on these results and the high risk of developing a second CRC, we concluded that if CRC is detected in a young patient participating in a surveillance programme, colectomy with ileorectal anastomosis seems to be the treatment of choice.

A French Committee on HNPCC commented on our study.1 Firstly, they stated that using quality adjusted LE would be a more accurate approach. We agree completely but studies on quality of life (QOL) did not specifically consider HNPCC patients. In HNPCC, QOL after segmental resection may be decreased by the need for colonoscopy (versus rectoscopy after colectomy) and the fear of a second tumour. Secondly, the committee considered our five year survival rates optimistic. The five year survival rates for HNPCC patients with Dukes’ B cancer varied in the literature from 70% to 91% and those for patients with Dukes’ C from 19% to 70%.2–6 These survival rates are similar to those used in our analysis. Thirdly, the committee mentioned that the overall five year survival of patients with CRC in HNPCC is approximately 55%. They stated that if the decision for an extended resection is made before the pathological staging of the tumour is known, 45% of patients will sustain a substantial decrease in QOL with no counterpart in quantity (that is, LE). The committee referred to the survival (55%) of symptomatic CRC in HNPCC. In our study, we discussed the surgical options for patients with CRC detected during surveillance. In our table 1, we showed the stage distribution of screen detected CRC based on our study and the Finnish series.7 As 86% had local cancer, the five year survival will be higher than 55%. Fourthly, the committee indicated that only a very small proportion of patients will be identified with CRC by the age of 27 years and that the increased LE for patients with CRC diagnosed at age 47 years was only one year. Half of the patients with screen detected CRC will be diagnosed before the age of 50 years and will have a substantial increase of LE of 1–2.3 years. Fifthly, the committee stressed that different indications should be made in men and women because of their different risks for metachronous cancer as well as for the competing risk of endometrial cancer. Although female mutation carriers may have a lower risk of CRC than male carriers, it has not been shown that they also have a lower risk of a second CRC. In fact, among HNPCC patients that developed a second tumour, we found more females than males.8 Female mutation carriers do indeed have a high risk of developing endometrial cancer but this cancer is only a rare cause of death in HNPCC.

As stated by the committee, it is difficult for a patient diagnosed with CRC to decide between an increase in LE and a potential decrease in their QOL. An increased LE is a somewhat theoretical concept that entails additional years at the end of one’s life while the negative impact on QOL of subtotal colectomy will start from the first postoperative day. On the other hand, it may be even more difficult for a physician to explain to a patient that has developed CRC under surveillance that after segmental resection, surveillance of the remaining colon will prevent cancer development. It is possible that this patient will be happy after removal of the colon as now they are at a substantially lower risk of developing a second CRC. We agree that the patient’s choice is pivotal in decisions on prophylactic surgery, after being fully informed of the pros and cons of the surgical options.


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  • Conflict of interest: None declared.

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