Table 6

Surveillance protocol for patients with CMMR-D proposed by the European consortium

Type of cancerLower age limitProcedure/interval
Brain tumoursFrom age 2 yearsMRI, 1×/6–12 months
Digestive tract cancer
 SBCFrom age 10 yearsVCE, upper gastrointestinal endoscopy*; 1×/year
 CRCFrom age 8 yearsIleocolonoscopy; 1×/year;
Haematological malignancies
 NHL/other lymphomaFrom age 1 yearClinical examination 1×/6 months
Optional: abdominal ultrasound 1×/6 months
 LeukaemiaFrom age 1 yearBlood count 1×/6smonths
LS-associated cancers†From age 20 yearsGynaecological examination, transvaginal US, Pipelle curettage (1×/year),
Urine cytology, dipstick (1×/year)
All cancersParents and patients should be advised to contact their doctor in case of unusual signs or symptoms. A pamphlet should be available with information about the signs/symptoms that may occur.
  • *At the same time as colonoscopy under general anaesthesia.

  • †See: Revised guidelines for the clinical management of Lynch syndrome: HFA Vasen et al Gut 2013.

  • CMMR-D, constitutional mismatch repair-deficiency; CRC, colorectal cancer; LS, Lynch syndrome; SBC, small bowel cancer; NHL, non-Hodgkin lymphoma; VCE, video capsule endoscopy.