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Pending large prospective studies, the extension of the current surveillance program in MSH2 mutation carriers with the inclusion of the urinary tract should be considered.

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Assessment of clinical findings can improve the rate of detection of mutations of DNA mismatch-repair genes in families suspected of having hereditary nonpolyposis colorectal cancer.

Neuroendocrine tumours account for only 0.5% of all malignancies. The incidence is approximately 2/100,000 with a female preponderance under the age of 50 years due to appendiceal location. The main primary sites are the gastrointestinal tract (62–67%) and the lung (22–27%). Presentation with metastatic disease accounts for 12–22%. In the last decades, the incidence has been rising. This might be due to more awareness, improved diagnostic tools or a change in definition. Most neuroendocrine tumours are mainly sporadic, but association with the multiple endocrine neoplasia type 1 syndrome and clustering within families is known. Also an increased risk of secondary cancers has been reported, but numbers are small. The 5-year survival is mainly associated with stage: 93% in local disease, 74% in regional disease and 19% in metastatic disease. In metastatic disease, survival increased since 1992, when treatment with octreotide became largely available in the Netherlands.