Table 1

 Clinical features of patients studied

No.SexAge at diagnosisColorectal adenomasa Colorectal cancer(s)Family history of colorectal tumoursRelatives with malignancy of any site or other colorectal tumoursb
First degreeSecond degreeThird degree
1F41100+0Y1: Co3: Br; Ga Co+Co+Ads (3)+En2: Co+Ads (3); Ads (?)
2M31240Y2: Co+Ga; Co1: Co
3F1950–1000N1: Oe
4F595c 1N
5M55700Y2: Co; Ads (?)
6F353, 3, 1, 0, 4, 00Y1: Co1: Lu
8F57701Y3: Co; Ga; Ca n/s
9F491, 14, 1, 23Y1: Co
10F7850+0Y2: Co; Ads (?)1: Ads (?)
12M631, “Multiple”, 1, 1, 10Y1: Co3: Co; Co; Ads (?)1: Co+Ads (?)
13M651d 0Y3: Co+Co+Co; GI?; Ca n/s1: Ga
14M5612, 2, 11Y7: Co; Co; Co; Co; Co+Pa+Ads (20); Pa; Ads (6)6: Co+Ads (4); Ads (8); Ad; Ads (6); Ki+Ad; Br2: Ad; Ad
15M52“Multiple”0N3: Co; Co; Vu1: Co
16F577, 5, 1, 4, 12Y1: B1
17M461, 1, 1, 40Y3: Co; Ads (?); Ads (?)
18M541e 0Y1: Co2: Br; Ca n/s
19F6340–500Y3: Co; Co; Ca n/s1: Ca n/s1: Ca n/s
20M5000Y1: Co
21M5160Y2: Co; Co3: Co; Co; Ca n/s
22M6032f Y2: En; Pr4: Co; Co; Br; NHL1: Br
23F4840Y2: Pa; Lu4: Co; Br; Me; Lk
24M4960Y2: Co; Ads (?)1: Co1: Ga
25M5140Y4: Ads (?); Ads (?); Ads (?); Ads (?)2: Ads (?); Ads (?)
26g F631h 0Y2: Co; Co1: Lui
27g F2900j N
  • aEither (i) total number of adenomas found or (ii) if separated by commas, numbers of adenomas removed on successive occasions, or (iii) “multiple” if exact number of adenomas not reported, but with notes or personal comunication indicating that the number of adenomas lay within the range consistent with a diagnosis of AAPC.

  • bNumber of affected relatives is shown. Different individuals’ tumours are separated by a semicolon; tumours in the same individual are separated by +.

  • Cancer types: Co, colorectal; Br, breast; Pr, prostate; Lu, bronchus; Lk, leukaemia; Ki, kidney (RCC); NHL, non-Hodgkin’s lymphoma; Bl, urinary bladder (TCC); Me, melanoma;Vu, vulva; Oe, oesophagus; Pa, pancreas; En, endometrium; GI, gastrointestinal; Ca n/s, cancer site not specified/unknown; Ads, colorectal adenomas, with total numbers in parentheses; (?) indicates multiple adenomas, but precise number not known.

  • cAlso multiple intra-abdominal desmoid tumours.

  • d“Upper GI polyposis” also noted.

  • eMucinous adenoma of the appendix; patient studied owing to unusual tumour suggestive of precursor of HNPCC.

  • fSynchronous cancers of duodenum, caecum, and sigmoid colon.

  • gPatients additional to sample of 25, studied owing to unusual tumours.

  • hSerrated adenoma.

  • iCarcinoid tumour.

  • jMultiple desmoid tumours in leg and back.