FIGURE 1.

FIGURE 2.

FIGURE 3.

Cancer rate after primary ileal pouch anal anastomosis (IPAA)
| First author and publication date (ref.) | No of patients | Age at cancer diagnosis (years) | Time to cancer (years) | Interval since last surveillance endoscopy and findings | Endoscopic findings at diagnosis | Location | Staging of cancer and status |
|---|---|---|---|---|---|---|---|
| Aelvoet et al., 202355 | 3/111 (2.7%) | / | / | / | / | / | Pouch excision |
| Pasquer et al., 202158 | 1/92 (1.1) | 30 | / | 1 month | Pouch body | Endoscopic resection | |
| Ganschow et al., 201861 | 1 | / | 27 | / | / | Pouch body | Resection and reconstruction of a new pouch - alive |
| Walsh et al., 201663 | 1 | 54 | / | Regular annual surveillance | New endoscopy due to anemia and rectal blood loos | Anastomosis | T3N2Mx, resection and ileostomy, alive during last FUP. |
| Wasmuth et al., 201367 | 1 | / | 11 | / | / | Rectal cuff | Resection and ileostomy - alive |
| Boostrom et al., 201366 | 1 | / | 23.7 | / | / | Pouch body | Transanal resection - alive |
| Ozdemir et al., 201338 | 4 | / |
| Regular annual surveillance | / | All ATZ |
|
| Makni et al., 201269 | 1 | 26 | 10 | 8 months | Polyps, LGD? | Pouch body? | Pouch excision – died 12 months later dissemination |
| Tonelli et al., 201251 | 2 |
| 10 |
|
|
| Excision with ileostomy, T3N0M0, died 6 months later dissemination/Excision with ileostomy, T2N0M0, alive after 56 month FUP |
| voon Roon et al., 201170 | 1 | / | 13 | / | / | Pouch body | Excision of a pouch – died 2 years of disseminated disease |
| Banasiewicz et al., 201133 | 5 | / | / | / | / | Pouch body | / |
| Ault et al., 200971 | 2 |
|
|
| Pain and blood per rectum, 3 cm mass/Sacral pain, bleeding ulcer | Pouch body / Pouch body | T2N1Mx, died of AMI prior treatment / Metastatic disease, chemotherapy |
| Tajika et al., 200983 | 2 |
|
|
| 30×25 mm cancer / Polyposis and 25 × 25 mm polyp | Pouch body/Kock’s pouch body |
|
| Lee et al., 200871 | 1 | / | 7 | / | Ulcerating tumor | Pouch body | T4N1M0, APR ileostomy. Developed metastases 2 years later. |
| Friederich et al., 200832 | 4 |
|
|
| / | All pouch body |
|
| Linehan et al., 200772 | 1 | 40 | 10 | / | Pelvic pain, discharge | Pouch body (patient had ileostomy but pouch was left in situ) | Excision. At last FUP patient was well. |
| Ulas et al., 200674 | 1 | / | 9 | / | / | Anastomosis | Dukes B, APR, metachronous cancer after 1 year |
| Campos et al., 200519 | 1 | / | 12 | No FUP | Presented with rectal bleeding | Pouch body | T2N0Mx, APR and ileostomy, patient well at 6 years FUP. |
| Vroueraets et al., 200475 | 2 |
|
| 5 years normal, then 2 and 1 years (both multiple LGD adenomas refused surgery) / Regular FUP every 2 years | Presented after 1 year with rectal bleeding /Normal. Routine biopsies at subsequent FUP revealed adenoca. |
| T2N0M0, APR, alive 1 year later / T4N0M0, APR, alive 8 months later |
| Cherki et al., 200376 | 1 | 35 | 3.5 | 1.5 years | / | Pouch body | T3N1M1, resection with ileostomy, died 1 month later |
| Ooi et al., 200336 | 2 |
|
|
|
|
| T3NOMO, APR, ileostomy, died 2.5 years later dissemination / T2N0M0, transanal excision with ileostomy (refused APR), died 4 years later, dissemination |
| Brown et al., 200178 | 1 | 44 | 7 years 4 months | Under FUP every 6 months | / | Anastomosis | / |
| Vuilleumier et al., 200079 | 1 | 38 | 7 | No FUP | / | Anastomosis | Resection with ileostomy – died 12 months later dissemination |
| Palkar et al., 199715 | 1 | 39 | 4.7 | 3 months | ? | Pouch body | T4NOM? - alive |
| Kim et al., 199715 | 1 | / | / | / | / | Pouch body? | / |
| Bassuini et al., 199680 | 1 | 31 | 3 | No FUP | / | Pouch body | / |
| Von Herbay et al., 199614 | 1 | 33 | 8 | Anastomosis | T1N0M0 | ||
| Hoehner et al., 19947 | 1 | 34 | 20 | / | / | Anastomosis | / |
Rate of adenoma development in the neoterminal ileum in patients after ileorectal anastomosis (IRA) and ileal pouch anal anastomosis (IPAA)
| First author and publication date (ref.) | Adenomas in the neoterminal ileum – after primary IPAA; n (%) | Cumulative risk for development of neoterminal adenomas | Years since surgery | Risk factor for adenomas in neoterminal ileum | Rate of adenomas in the neoterminal ileum – after primary IRA; n (%) | Years since surgery |
|---|---|---|---|---|---|---|
| Tajika et al., 201916 | 4/24 (16.7) | 4.4% at 20 years and 36% at 30 years after primary surgery | 23.1 ± 5.8 | 0/14 (0.0) | ||
| Boostrom et al., 201366 | 4/33 polyps (12.0) | |||||
| Pommaretet et al., 201335 | 9/118 (6.5) | Presence of pouch adenomas (OR, 2.16, P = 0.007) | ||||
| Booij et al., 201018 | 5/34 (14.7) 2 patients had resection of neo-terminal ileum, one due to LGD and other due to HGD adenoma. | |||||
| Gleeson et al., 200830 | 3/13 (23.1) | Median 6.5 (0–15) | 4/16 (25.0) | Median 12 (1–29) | ||
| Moussata et al., 200717 | Mean 17.6 +-7.8(6–35) Mean from colectomy to diagnosis: 16.4+-8.5 (5–30) | 10/21 (47.6) of which 2 were advanced adenomas. | ||||
| Groves et al., 200534 | 2/20 (10.0) | 6 (1–14) | 1/47 (2.0%) | 12 (0–39) | ||
| Thompson-Fawcett et al., 200177 | 1/24 (4.2) | Median 7 (1–19) |
Patient characteristics and rate of rectal remnant cancer rate in patients after ileorectal anastomosis (IRA)
| First author and publication date | Proportion of man; n / (%) | APC mutation Underwent n/(%); Positive in; n/(%) | Follow-up (years/months) since surgery | Years since surgery to cancer diagnosis | Age at surgery | Age at cancer diagnosis | Rectal remnant cancer rate; n/(%) | Cumulative risk for rectal cancer | Rectal cancer mortality |
|---|---|---|---|---|---|---|---|---|---|
| Colletti et al., 202258 | 57.4% | 93.6% / | / | Median of 13 years | / | / | 47 / 715 (6.57) | / | 14/47 (29.8%) at median follow up of 13 years. |
| Pasquer et al., 202159 | 95 (48.2) | / | / | / | / | / | 12 / (6.1); 1 was metastatic, 2 were resected endoscopically, 10 surgically | / | / |
| Maehata et al., 201520 | 16 (59.3) |
| 21.1 (3–35) | / | Median 27 years (9–66) | / | 10/27 (37.0); 6/10 cancers were TisN0M0 | 8% at 10 years; 19% at 20 years; 57% at 30 years | 3/27 (11.1) |
| Koskenvuo et al., 201322 | 59 (42.1) | / | Median 15 years (0–44) | / | Mean 36 years (18–71) | Cumulative risk 2% at 40 years age; 7% at 50; 13% at 60 years age and 16 % at 70 years age. | 18/140 (13%) | 3% at 5 years; 4% at 10 years; 11% at 20 years; 24% at 30 years after IRA | 10/140 (7%); 5-year survival 55%. Cumulative risk for death due to rectal cancer after IRA: 2% at 5 years, 3% at 10 years and 9% at 30 years. |
| Booij et al., 201018 | 19 (44.2) | / | / | / | Median 16 (7–25) | / | 3/34 (8.8) | / | 2/34 (5.8) |
| Sinha et al., 201026 | 232 (54.3) |
| Median 15 years (7–25) | / | Median 21 years (11–67) | / | 48/427 (11.2%) | / | / |
| Yamaguchi et al., 200925 | 35 (59.3) | / | Median 8.9 years | / | Median 30 years (13–65) | / | 17/59 (30%) | / | 5-year survival 94%; 10-year survival 94%. |
| Nieuwenhuis et al., 200927 | / | / | / | / | / | / | / | 3.7% for group 1; 9.3% for group 2; 8.3% for group 3.% | / |
| Campos et al., 200819 | / | / | 91.1 (3–557) | / | Mean 45.8 years | Mean 50.6 years | 6/36 (16.7) | 17.2% at 5 years; 24.1% at 10 years; 43.1% after 15 years | / |
| Gleeson et al., 200830 | / | / | FUP initiated median 12 (1–29) years after surgery | / | / | 40 and 59 years. | 2/16 (12.5) | / | / |
| Bullow et al., 200824 | 401 (51.7) | / | Median 7 years (0–13). Patients were operated between 1950–2006 | Median 27 (7–75) | / | 60/776 (7.7%) (56/576; 10% and 4/200; 2%) | 10-year cumulative risk 4.4% [95% CI 2.6–6.2] in pre-pouch era; 10-year cumulative risk 2.5% [95% CI 0–5.5] in pouch era; | / | |
| Moussata et al., 200717 They only watched ileal muocas above the IRA | 10 (47.6) |
| Mean 8.4 years ± 5 since colectomy | / | / | / | 0/21 (0.0) | / | / |
| Church et al., 200338 | 92 / (46.7) | / | Pre-pouch era: 212 months (IQR 148 months); Pouch era: 60 months (IQR 80 months) | / | Median age 23 years (IQR 15.5 years pre-pouch and 17 years pouch) | / | 8 (12.9%) in the pre-pouch era and 0 in pouch era. | / | / |
| Bertario et al., 200023 | 206/371 (55.5) |
| Median 81 months | Median 102 months (1–26 years) | Mean 32 years | / | 27/371 (7.3) |
| 6/371 (1.6) |
| Jenner et al., 199821 | 25/55 (45.0) | 55/(100.0) | Median 10 (1–31) | / | Mean age 30 (13–62) | Median 41 | 7/55 (12.7) | / | / |
Patient characteristics and rate of adenomas in patients after primary ileal pouch anal anastomosis (IPAA)
| First author and publication date | Sex (man); n (%) | APC mutation Underwent; n (%); Positive in; n (%) | Distinguish between pouch body and rectal cuff | Follow-up (months/years) | Time from surgery to first adenomas (years) | Age at surgery (years) | Rate of adenomas (≥ 1 polyp) | Size of adenomas, mm | Histology of adenomas; n (%) | Number of Adenomas |
|---|---|---|---|---|---|---|---|---|---|---|
| Aelvoet AS et al., 202355 | 81 (56) |
| Yes | Median 152 (77–240) | 15% at 5 years; 48% at 10 years; 85% at 20 years. | Median 24 (18–32) | Median 5 (3–15) | Tubular adenomas 31 (28%), Tubulovillous 26 (23%), Villous 5 (5%) | Prepouch ileum 4(2–13), Pouch body 20 (5–50), rectal cuff 6 (3–10) | |
| Tajika et al., 201926 | 16 (47.1) | / | Yes | Median 21.6 (3.7–8.8) | 32 (35.9) of patients showed progression of pouch adenomas during FUP | Median 34.6 (17–52) | 24/34 (70.6) | 2–40 mm | 6 advanced adenomas (25.0) | 1–300 |
| Ganschow et al., 201861 | 100 (52.1) |
| No | Median 12.8 (9–17) for patients with pouch adenomas and (2.5–12.2) for patients without pouch adenomas; | 32 (35.9) of patients showed progression of pouch adenomas during FUP | 27.5 years (10.2–58.5) | 90/192 (46.9) at a median of 8.5 years (0.9–25.1) after IPAA. 5 years after IPAA 84.9% patients free of adenoma; 15 years after 40.4% and 20 years after 21.9% patients were free of adenomas. | 53/192 (58.9) ≤ 4 mm; 24/192 (26.7) 5 – 10 mm; 13/192 (14.4) ≥ 10 mm | Tubular adenomas in 69/192 (76.7); tubulovillous adenomas in 16/192 (17.8); villous in 5/192 (5.6) | 46/192 (51.1) had < 4; 14/192 (15.6) 5–10; 30/192 (33.3) > 10 adenomas |
| Goldstein et al., 201563 | 24 (41.0) | Yes | Mean 11.6 years +-14.6 years | Median adenoma free time interval since surgery; Cuff 10.8 years Pouch 16.9 years | Mean 30.8 years +-10.8 years | 35/59 (59.0);
| / | All LGD | / | |
| Zahid et al., 201519 | 14 (51.8) | No | Mean 9.2 years | Median; 72 months (18–249) | Median 31 years (14–65) | 12/27 (44.0) | / | Only 1 polyp HGD (< 99%) | / | |
| Kennedy et al., 201466 | 43 (45.0) | Watched only anastomosis | Mean 7.6 (0 – 24) | Mean 15.4 (4–20) | 9/95 (9.4) | |||||
| Pommaretet et al., 201336 |
| / | Median 15 years | 25 years (9−61 years) | 57/118 (48.3) | > 10 mm:12 | 94% LGD; 6% HGD |
| ||
| Boostrom et al., 201366 | 52 (44.5) | Yes | 125 months (25–423 months) | 12.4 years (15–405 months) | 26 years (4–60 years) | 30/117 (25.6) | 5.9 mm (2 mm to 20 mm) | 22 LGD, 8 tubulovillous | / | |
| Wasmuth et al., 201367 | 34 (55.7) | / | Yes (body and anastomosis) | Cumulative rate of adenomas at 28 years 17% for mucosectomy group and 75% at 15 years in a group without mucosectomy (P < 0.0001) | 20 (10–49) |
| ||||
| Tonelli et al., 201251 | / | 45 (65%) | No | Median 133 months (12–288 months) | Mean 7 years (1–15 years) | 33 years (17–63 years) | 25 (36.0) | Mean 3 mm (1–40) | Adenomas, dysplasia not specified | Mean 8 (1–47) |
| Yan et al., 201268 | 30 (71.5) | / | Yes | Median 7.2 (2.2–20) | 29 (16–65) | At the anastomosis 6/33 (18.2) | / | / | / | |
| Banasiewicz et al., 201133 | 79 (47.9) | / | / | Endoscopies performed 2–19 years since surgery. |
| 21/165 (12.7) | LGD - 21/32 (65.6); HGD - 11/32 (34.4) | |||
| Gleeson et al., 200831 | / | / | Yes | / | FUP began median 6.5 (0–15) after surgery | / | 13/13 (100): 10/13 pouch body; 2/13 anastomosis; 3/13 ileum above anastomosis | < 5 mm | / | 5–30 |
| Friederich et al., 200832 | 119 (56.0) | / | / | Mean 7.9 (0.4–20.3 years) |
| Mean 30.0 years (10–62.6 years) | 47/212 (35%) | / | / | / |
| Campos et al., 200817 | / | / | No | 50.8 (5–228) | 3/26 (11.5) | |||||
| Moussata et al., 200725 | 12 (57.1) |
| Yes (only polyps in the ileal mucosa of the pouch body are described) | Mean 5.4 +- 2.6 (1–11) | Mean 4.7+-3.3 years (1–14) | 17/23 (74.0) | Mean size 5.2 mm +-3.4 mm; 3 polyps were > 10 mm. | LGD 16/17 (94.1); HGD 1/17 (5.9) | / | |
| Groves et al., 200535 | 35 (58.3) | / | Between pouch and above anastomosis ileum | 6 years (1–17 years) | / | 32.5 years (13–66 years) | 34/60 (57%) of which 5 were > 10 mm / 11 were advance adenomas | Mean size 5 mm (1–40 mm) | / | Median number 4 |
| Thompson-Fawcett et al., 200177 | / |
| Only pouch body | / | / | / | 20/33 (60.0) adenomas | 1–3 mm | / | Median 10 (1–100) Also lymphoid hyperplasia included |
Summary of recommendations from the international guidelines
| First author and publication date (ref.) | Endoscopic surveillance – patients with IRA | Indications for secondary proctectomy patients with IRA | Endoscopic surveillance – patients with IPAA |
|---|---|---|---|
| Vasen et al., 20087 | Every 3 to 6 months | Multiple large adenomas (> 5 mm) Adenomas with dysplasia | Every 6 to 12 months |
| Balmaña et al., 2013, ESMO8 | Every 12 months | No recommendations | Every 12 months |
| Stoffel et al., 2015, ASCO9 | Every 6 to 12 months | No recommendations | Every 6 months to 5 years (Intervals should be determined on a case-by-case basis and may be even shorter than 1 year for some individuals) |
| Sygnal et al., 2015, ACG10 | Every 12 months | No recommendations | Every 12 months |
| Herzig et al., 2017, ASCRS4 | Every 12 months | No recommendations | Every 12 months |
| Van Leerdam ME et al., 2019, ESGE53 | Every 12 to 24 months | No recommendations | Every 12 to 24 months |
| Yang J et al., 2020, ASGE54 | 6 months after surgery with 6 to 12 months further surveillance interval | 12 months after surgery with 12 to 24 months further surveillance interval. 6 months if advance adenoma |
Characteristics of included studies
| First author and publication date (ref.) | No. of patients | Country | Setting | Study design | Surgery performed (period) | Study population |
|---|---|---|---|---|---|---|
| Aelvoet et al., 202355 | 144 (111 IPAA, 33 ileostomy) | The Netherlands | Single | Cohort/Retrospective | / | IPAA, ileostomy |
| Tatsuta et al., 202356 | 65 (22 IRA, 20 IPAA) | Japan | Single | Cohort/Retrospective | 1976–2022 | IRA, IPAA |
| Anele et al., 202257 | 199 (199 IRA) | United Kingdom | Single | Cohort/Retrospective | 1990–2017 | IRA |
| Colletti et al., 202258 | 715 (715 IRA) | Italy | Multicentre | Retrospective analysis of the Registry | 1977–2021 | IRA |
| Pasquer et al., 202159 | 289 (197 IRA, 92 IPAA) | France | Multicentre | Retrospective analysis of the Registry | 1965–2015 | IRA, IPAA |
| Ardoino et al., 202060 | 925 (585 IRA, 340 IPAA) | Italy | Multicenter | Retrospective analysis of the Registry | 1947–2015 | IRA, IPAA |
| Tajika et al., 201916 | 47 (14 IRA, 25 IPAA, 8 ileostomy) | Japan | Single | Cohort/Retrospective | 1965–2017 | IRA, IPAA and ileostomy |
| Ganschow et al., 201861 | 192 | Germany | Singe | Cohort/Prospective and retrospective analysis of Polyposis Registry | Endoscopy data collected during 2010–2013 | IPAA |
| Kariv et al., 201762 | 45 | Israel | Single | Cohort/Retrospective | 1986–2013 | IPAA |
| Patel et al., 201642 | 21 (6 IRA, 5 IPAA, 10 intact colon) | Indianapolis, USA | Single | Cohort/Retrospective | Endoscopies performed between 2004–2016 | IRA, IPAA and intact colon |
| Walsh et al., 201663 | 1 | Ireland | Single | Case report | 1987 | IPAA - cancer |
| Maehata et al., 201520 | 27 | Japan | Single | Cohort/Retrospective | 1990–2004 | IRA |
| Ganschow et al., 201550 | 100; 50 hand-sewn and 50 stapled anastomoses | Germany | Single | Cohort/Prospective | ? | Hand-sewn vs. stapled anastomosis |
| Goldstein et al., 201563 | 59 | Israel | Single | Cohort/Retrospective | 1986–2013 | IPAA |
| Zahid et al., 201564 | 27 | Australia | Single | Cohort/Retrospective | 1984–2011 | IPAA |
| Kennedy et al., 201465 | 95; 85 hand-sewn and 1 stapled anastomosis | Rochester, Mayo Clinic, USA | Single | Cohort/Retrospective | 1987–2011 | IPAA |
| Koskenvuo et al., 201322 | 140 | Finland | Single | Cohort/Retrospective | 1963–2012 | IRA |
| Pommaret et al., 201335 | 118 | France | Single | Cohort/Retrospective | / | IPAA and IRA |
| Boostrom et al., 201366 | 117 | Rochester, Mayo Clinic, USA | Single | Cohort/Retrospective | 1972–2007 | IPAA |
| Ozdemir et al., 201337 | 260; 86 hand-sewn and 175 stapled anastomoses | Cleveland, USA | Single | Analysis of polyposis registry | 1983–2010 | Hand-sewn vs. stapled anastomosis |
| Wasmuth et al., 201367 | 61; 39 hand-sewn with mucosectomy and 22 without of which 15 were stapled and 7 hand-sewn anastomoses | Norway | Multicenter | Analysis of polyposis registry | 1986–2008 | IPAA (mucosectomy vs. no-mucosectomy) |
| Yan et al., 201268 | 42 (33 IPAA; 6 IRA ?) | China | Single | Cohort/Retrospective | 1988–2008 | IPAA and IRA |
| Makni et al., 201269 | 1 | Tunisia | Single | Case report | 1996 | IPAA - cancer |
| Tonelli et al., 201251 | 69 | Italy | Single | Cohort/Prospective data collection | 1984–2008 | IPAA |
| von Roon et al., 201170 | 140; 44 hand-sewn and 76 stapled anastomoses | UK | Single | Retrospective analysis of St. Mark’s Hospital Polyposis Registry | 1978–2007 | Hand-sewn vs. stapled anastomosis |
| Banasiewicz et al., 201132 | 165 | Poland | Bicenter | Bicenter/Retrospective analysis | 1985–2009 operated, Clinical data from endoscopy FUP between 2004–2009 | IPAA |
| Booij et al., 201018 | 43 (34 IRA) | The Netherlands | Single | Cohort/Retrospective | 1977–2005 | IRA and IPAA |
| Sinha et al., 201026 | 427 | UK | Single | Retrospective analysis of St. Mark’s Hospital Polyposis Registry | 1990–2008 | IRA |
| Ault et al., 200971 | 2 | Los Angeles, USA | Single | Case series | 1990, 1993 | IPAA - cancer |
| Nieuwenhuis et al., 200927 | 475 | Denmark, Finland, Sweden, Netherlands | Multicenter | Analysis of polyposis registry | / | IRA |
| Yamaguchi et al., 200925 | 59 | Japan | Single | Cohort/Retrospective | 1962–2007 | IRA |
| Friederich et al., 200831 | 212; 71 hand-sewn with mucosectomy and 115 stapled anastomoses | The Netherlands | Single | Analysis of National Polyposis Registry | 1985–2005 | IPAA |
| Campos et al., 200819 | 36 | Brasil | Single | Cohort/Retrospective | 1977–2006 | IRA and IPAA |
| Bullow et al., 200824 | 776; 576 operated in pre-pouch period and 200 in pouch period starting in 1990 | Denmark, Finland, Sweden, Netherlands | Multicenter | Analysis of polyposis registry | 1950–2006 | IRA |
| Gleeson et al., 200830 | 16 | Rochester, Mayo Clinic, USA | Single | Cohort/Retrospective analysis | 1964–2003(Analysis of endoscopies between 1992–2006) | IPAA and IRA |
| Lee et al., 200872 | 1 | Korea | Single | Case report | 1998 | IPAA - cancer |
| Linehan et al., 200773 | 1 | Ireland | Single | Case report | 1997 | IPAA - cancer |
| Valanzano et al., 200728 | 25 | Italy | Single | Cohort/Prospective | 1986–2004 | IRA |
| Moussata et al., 200717 | 21 | France | Single | Cohort/Retrospective | / | IPAA and IRA |
| Ulas et al., 200674 | 1 | Turkey | Single | Case report | 1993 | IPAA - cancer |
| Campos et al., 200519 | 1 | Brazil | Single | Case report | / | IPAA - cancer |
| Groves et al., 200534 | 60 | UK | Single | Retrospective analysis of St. Mark’s Hospital Polyposis Registry | / | IPAA |
| Vroueraets et al., 200475 | 2 | The Netherlands | Single | Case report | 1990, 1991 | IPAA – cancer |
| Ooi et al., 200336 | 2 | Cleveland, USA | Single | Case report | / | IPAA – cancer |
| Church et al., 200338 | 197; 62 operated in pre-pouch period and 135 in pouch period starting in 1983 | Cleveland, USA | Single | Analysis of polyposis registry | 1950–1999 | IRA |
| Cherki et al., 200376 | 1 | France | Single | Case report | / | IPAA - cancer |
| Thompson-Fawcett et al., 200177 | 33 | Canada | Single | Cohort/Prospective | / | IPAA |
| Church et al., 200115 | 213 (165 IRA) | Cleveland, USA | Single | Analysis of polyposis registry | / | IRA and IPAA |
| Brown et al., 200178 | 1 | Singapore | Single | Case report | / | IPAA - cancer |
| Bertario et al., 200023 | 371 | Italy | Multicenter | Retrospective analysis of Hereditary tumor registry | 1955–1997 | IRA |
| Vuilleumier et al., 200079 | 1 | UK | Single | Case report | 1990 | IPAA - cancer |
| Jenner et al., 199821 | 55 | Australia | Single | Analysis of polyposis registry | ?–1994 | IRA |
| Bassuini et al., 199680 | 1 | UK | Single | Case report | 1991 | IPAA - cancer |
| Hoehner et al., 19946 | 1 | Iowa, USA | Single | Case report | / | IPAA - cancer |