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Patel RV, Curtius K, Man R, Fletcher J, Cuthill V, Clark SK, von Roon AC, Latchford A. Long-term outcomes of pouch surveillance and risk of neoplasia in familial adenomatous polyposis. Endoscopy 2023; 55:836-846. [PMID: 36807005 PMCID: PMC10465241 DOI: 10.1055/a-2038-0541] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/14/2022] [Accepted: 02/15/2023] [Indexed: 02/23/2023]
Abstract
BACKGROUND Long-term pouch surveillance outcomes for familial adenomatous polyposis (FAP) are unknown. We aimed to quantify surveillance outcomes and to determine which of selected possible predictive factors are associated with pouch dysplasia. METHODS Retrospective analysis of collected data on 249 patients was performed, analyzing potential risk factors for the development of adenomas or advanced lesions ( ≥ 10 mm/high grade dysplasia (HGD)/cancer) in the pouch body and cuff using Cox proportional hazards models. Kaplan-Meier analyses included landmark time-point analyses at 10 years after surgery to predict the future risk of advanced lesions. RESULTS Of 249 patients, 76 % developed at least one pouch body adenoma, with 16 % developing an advanced pouch body lesion; 18 % developed an advanced cuff lesion. Kaplan-Meier analysis showed a 10-year lag before most advanced lesions developed; cumulative incidence of 2.8 % and 6.4 % at 10 years in the pouch body and cuff, respectively. Landmark analysis suggested the presence of adenomas prior to the 10-year point was associated with subsequent development of advanced lesions in the pouch body (hazard ratio [HR] 4.8, 95 %CI 1.6-14.1; P = 0.004) and cuff (HR 6.8, 95 %CI 2.5-18.3; P < 0.001). There were two HGD and four cancer cases in the cuff and one pouch body cancer; all cases of cancer/HGD that had prior surveillance were preceded by ≥ 10-mm adenomas. CONCLUSIONS Pouch adenoma progression is slow and most advanced lesions occur after 10 years. HGD and cancer were rare events. Pouch phenotype in the first decade is associated with the future risk of developing advanced lesions and may guide personalized surveillance beyond 10 years.
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Affiliation(s)
- Roshani V. Patel
- Polyposis Registry, St Mark’s Hospital, Harrow, UK
- Department of Surgery and Cancer, Imperial College London, London, UK
| | - Kit Curtius
- Barts Cancer Institute, Queen Mary University of London, London, UK
- Division of Biomedical Informatics, Department of Medicine, University of California San Diego, La Jolla, California, USA
| | - Ripple Man
- Polyposis Registry, St Mark’s Hospital, Harrow, UK
| | - Jordan Fletcher
- Polyposis Registry, St Mark’s Hospital, Harrow, UK
- Department of Surgery and Cancer, Imperial College London, London, UK
| | | | - Susan K. Clark
- Polyposis Registry, St Mark’s Hospital, Harrow, UK
- Department of Surgery and Cancer, Imperial College London, London, UK
| | | | - Andrew Latchford
- Polyposis Registry, St Mark’s Hospital, Harrow, UK
- Department of Surgery and Cancer, Imperial College London, London, UK
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Le Cosquer G, Buscail E, Gilletta C, Deraison C, Duffas JP, Bournet B, Tuyeras G, Vergnolle N, Buscail L. Incidence and Risk Factors of Cancer in the Anal Transitional Zone and Ileal Pouch following Surgery for Ulcerative Colitis and Familial Adenomatous Polyposis. Cancers (Basel) 2022; 14:530. [PMID: 35158797 PMCID: PMC8833833 DOI: 10.3390/cancers14030530] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/28/2021] [Revised: 01/17/2022] [Accepted: 01/18/2022] [Indexed: 12/29/2022] Open
Abstract
Proctocolectomy with ileal pouch-anal anastomosis is the intervention of choice for ulcerative colitis and familial adenomatous polyposis requiring surgery. One of the long-term complications is pouch cancer, having a poor prognosis. The risk of high-grade dysplasia and cancer in the anal transitional zone and ileal pouch after 20 years is estimated to be 2 to 4.5% and 3 to 10% in ulcerative colitis and familial polyposis, respectively. The risk factors for ulcerative colitis are the presence of pre-operative dysplasia or cancer, disease duration > 10 years and severe villous atrophy. For familial polyposis, the risk factors are the number of pre-operative polyps > 1000, surgery with stapled anastomosis and the duration of follow-up. In the case of ulcerative colitis, a pouchoscopy should be performed annually if one of the following is present: dysplasia and cancer at surgery, primary sclerosing cholangitis, villous atrophy and active pouchitis (every 5 years without any of these factors). In the case of familial polyposis, endoscopy is recommended every year including chromoendoscopy. Even if anal transitional zone and ileal pouch cancers seldom occur following proctectomy for ulcerative colitis and familial adenomatous polyposis, the high mortality rate associated with this complication warrants endoscopic monitoring.
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Affiliation(s)
- Guillaume Le Cosquer
- Department of Gastroenterology and Pancreatology, CHU Toulouse-Rangueil (University Hospital Centre) and Toulouse University, UPS, 31059 Toulouse, France; (G.L.C.); (C.G.); (B.B.)
| | - Etienne Buscail
- Department of Surgery, CHU Toulouse-Rangueil and Toulouse University, UPS, 31059 Toulouse, France; (E.B.); (J.-P.D.); (G.T.)
- IRSD, Toulouse University, INSERM 1022, INRAe, ENVT, UPS, 31300 Toulouse, France; (C.D.); (N.V.)
| | - Cyrielle Gilletta
- Department of Gastroenterology and Pancreatology, CHU Toulouse-Rangueil (University Hospital Centre) and Toulouse University, UPS, 31059 Toulouse, France; (G.L.C.); (C.G.); (B.B.)
| | - Céline Deraison
- IRSD, Toulouse University, INSERM 1022, INRAe, ENVT, UPS, 31300 Toulouse, France; (C.D.); (N.V.)
| | - Jean-Pierre Duffas
- Department of Surgery, CHU Toulouse-Rangueil and Toulouse University, UPS, 31059 Toulouse, France; (E.B.); (J.-P.D.); (G.T.)
| | - Barbara Bournet
- Department of Gastroenterology and Pancreatology, CHU Toulouse-Rangueil (University Hospital Centre) and Toulouse University, UPS, 31059 Toulouse, France; (G.L.C.); (C.G.); (B.B.)
| | - Géraud Tuyeras
- Department of Surgery, CHU Toulouse-Rangueil and Toulouse University, UPS, 31059 Toulouse, France; (E.B.); (J.-P.D.); (G.T.)
| | - Nathalie Vergnolle
- IRSD, Toulouse University, INSERM 1022, INRAe, ENVT, UPS, 31300 Toulouse, France; (C.D.); (N.V.)
| | - Louis Buscail
- Department of Gastroenterology and Pancreatology, CHU Toulouse-Rangueil (University Hospital Centre) and Toulouse University, UPS, 31059 Toulouse, France; (G.L.C.); (C.G.); (B.B.)
- Centre for Clinical Investigation in Biotherapy, CHU Toulouse-Rangueil and INSERM U1436, 31059 Toulouse, France
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Yang J, Gurudu SR, Koptiuch C, Agrawal D, Buxbaum JL, Abbas Fehmi SM, Fishman DS, Khashab MA, Jamil LH, Jue TL, Law JK, Lee JK, Naveed M, Qumseya BJ, Sawhney MS, Thosani N, Wani SB, Samadder NJ. American Society for Gastrointestinal Endoscopy guideline on the role of endoscopy in familial adenomatous polyposis syndromes. Gastrointest Endosc 2020; 91:963-982.e2. [PMID: 32169282 DOI: 10.1016/j.gie.2020.01.028] [Citation(s) in RCA: 90] [Impact Index Per Article: 18.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/17/2020] [Accepted: 01/18/2020] [Indexed: 02/08/2023]
Abstract
Familial adenomatous polyposis (FAP) syndrome is a complex entity, which includes FAP, attenuated FAP, and MUTYH-associated polyposis. These patients are at significant risk for colorectal cancer and carry additional risks for extracolonic malignancies. In this guideline, we reviewed the most recent literature to formulate recommendations on the role of endoscopy in this patient population. Relevant clinical questions were how to identify high-risk individuals warranting genetic testing, when to start screening examinations, what are appropriate surveillance intervals, how to identify endoscopically high-risk features, and what is the role of chemoprevention. A systematic literature search from 2005 to 2018 was performed, in addition to the inclusion of seminal historical studies. Most studies were from worldwide registries, which have compiled years of data regarding the natural history and cancer risks in this cohort. Given that most studies were retrospective, recommendations were based on epidemiologic data and expert opinion. Management of colorectal polyps in FAP has not changed much in recent years, as colectomy in FAP is the standard of care. What is new, however, is the developing body of literature on the role of endoscopy in managing upper GI and small-bowel polyposis, as patients are living longer and improved endoscopic technologies have emerged.
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Affiliation(s)
- Julie Yang
- Division of Gastroenterology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, USA
| | - Suryakanth R Gurudu
- Division of Gastroenterology and Hepatology, Mayo Clinic, Scottsdale, Arizona, USA
| | - Cathryn Koptiuch
- Department of Population Sciences, Huntsman Cancer Institute, University of Utah School of Medicine, Salt Lake City, Utah, USA
| | - Deepak Agrawal
- Department of Internal Medicine, Dell Medical School, University of Texas at Austin, Austin, Texas, USA
| | - James L Buxbaum
- Division of Gastrointestinal and Liver Diseases, Keck School of Medicine of University of Southern California, Los Angeles, California, USA
| | - Syed M Abbas Fehmi
- Department of Gastroenterology, University of California, San Diego, California, USA
| | - Douglas S Fishman
- Section of Pediatric Gastroenterology, Hepatology and Nutrition, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA
| | - Mouen A Khashab
- Division of Gastroenterology and Hepatology, Johns Hopkins University, Baltimore, Maryland, USA
| | - Laith H Jamil
- Section of Gastroenterology and Hepatology, Beaumont Hospital-Royal Oak, Royal Oak, Michigan, USA
| | - Terry L Jue
- Department of Gastroenterology, The Permanente Medical Group, San Francisco, California, USA
| | - Joanna K Law
- Department of Gastroenterology and Hepatology, Digestive Disease Institute, Virginia Mason Medical Center, Seattle, Washington, USA
| | - Jeffrey K Lee
- Department of Gastroenterology, Kaiser Permanente San Francisco Medical Center, San Francisco, California, USA
| | - Mariam Naveed
- Advent Health Medical Group, Gastroenterology/Hepatology, Advent Health Hospital Altamonte Springs, Altamonte Springs, Florida, USA
| | - Bashar J Qumseya
- Department of Gastroenterology, University of Florida, Gainsville, Florida, USA
| | - Mandeep S Sawhney
- Division of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA
| | - Nirav Thosani
- Division of Gastroenterology, Hepatology and Nutrition, McGovern Medical School, UTHealth, Houston, Texas, USA
| | - Sachin B Wani
- Division of Gastroenterology and Hepatology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - N Jewel Samadder
- Division of Gastroenterology and Hepatology, Mayo Clinic, Scottsdale, Arizona, USA
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Ishida H, Yamaguchi T, Tanakaya K, Akagi K, Inoue Y, Kumamoto K, Shimodaira H, Sekine S, Tanaka T, Chino A, Tomita N, Nakajima T, Hasegawa H, Hinoi T, Hirasawa A, Miyakura Y, Murakami Y, Muro K, Ajioka Y, Hashiguchi Y, Ito Y, Saito Y, Hamaguchi T, Ishiguro M, Ishihara S, Kanemitsu Y, Kawano H, Kinugasa Y, Kokudo N, Murofushi K, Nakajima T, Oka S, Sakai Y, Tsuji A, Uehara K, Ueno H, Yamazaki K, Yoshida M, Yoshino T, Boku N, Fujimori T, Itabashi M, Koinuma N, Morita T, Nishimura G, Sakata Y, Shimada Y, Takahashi K, Tanaka S, Tsuruta O, Yamaguchi T, Sugihara K, Watanabe T. Japanese Society for Cancer of the Colon and Rectum (JSCCR) Guidelines 2016 for the Clinical Practice of Hereditary Colorectal Cancer (Translated Version). J Anus Rectum Colon 2018; 2:S1-S51. [PMID: 31773066 PMCID: PMC6849642 DOI: 10.23922/jarc.2017-028] [Citation(s) in RCA: 30] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/26/2017] [Accepted: 09/15/2017] [Indexed: 02/07/2023] Open
Abstract
Hereditary colorectal cancer accounts for less than 5% of all colorectal cancer cases. Some of the unique characteristics that are commonly encountered in cases of hereditary colorectal cancer include early age at onset, synchronous/metachronous occurrence of the cancer, and association with multiple cancers in other organs, necessitating different management from sporadic colorectal cancer. While the diagnosis of familial adenomatous polyposis might be easy because usually 100 or more adenomas that develop in the colonic mucosa are in this condition, Lynch syndrome, which is the most commonly associated disease with hereditary colorectal cancer, is often missed in daily medical practice because of its relatively poorly defined clinical characteristics. In addition, the disease concept and diagnostic criteria for Lynch syndrome, which was once called hereditary non-polyposis colorectal cancer, have changed over time with continual research, thereby possibly creating confusion in clinical practice. Under these circumstances, the JSCCR Guideline Committee has developed the "JSCCR Guidelines 2016 for the Clinical Practice of Hereditary Colorectal Cancer (HCRC)," to allow delivery of appropriate medical care in daily practice to patients with familial adenomatous polyposis, Lynch syndrome, or other related diseases. The JSCCR Guidelines 2016 for HCRC were prepared by consensus reached among members of the JSCCR Guideline Committee, based on a careful review of the evidence retrieved from literature searches, and considering the medical health insurance system and actual clinical practice settings in Japan. Herein, we present the English version of the JSCCR Guidelines 2016 for HCRC.
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Affiliation(s)
- Hideyuki Ishida
- Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitma Medical University, Kawagoe, Japan
| | - Tatsuro Yamaguchi
- Department of Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan
| | - Kohji Tanakaya
- Department of Surgery, Iwakuni Clinical Center, Iwakuni, Japan
| | - Kiwamu Akagi
- Department of Cancer Prevention and Molecular Genetics, Saitama Prefectural Cancer Center, Saitama, Japan
| | - Yasuhiro Inoue
- Department of Gastrointestinal and Pediatric Surgery, Division of Reparative Medicine, Institute of Life Sciences, Mie University Graduate School of Medicine, Tsu, Japan
| | - Kensuke Kumamoto
- Department of Coloproctology, Aizu Medical Center, Fukushima Medical University, Aizuwakamatsu, Japan
| | - Hideki Shimodaira
- Department of Clinical Oncology, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan
| | - Shigeki Sekine
- Division of Pathology and Clinical Laboratories, National Cancer Center, Hospital, Tokyo, Japan
| | - Toshiaki Tanaka
- Department of Surgical Oncology, The Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Akiko Chino
- Division of Gastroenterology, The Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan
| | - Naohiro Tomita
- Department of Surgery, Hyogo College of Medicine, Nishinomiya, Japan
| | - Takeshi Nakajima
- Endoscopy Division/Department of Genetic Medicine and Service, National Cancer Center Hospital, Tokyo, Japan
| | | | - Takao Hinoi
- Department of Surgery, Institute for Clinical Research, National Hospital Organization Kure Medical Center and Chugoku Cancer Center, Kure, Japan
| | - Akira Hirasawa
- Department of Obstetrics and Gynecology, Keio University School of Medicine, Tokyo, Japan
| | - Yasuyuki Miyakura
- Department of Surgery Saitama Medical Center, Jichi Medical University, Saitama, Japan
| | - Yoshie Murakami
- Department of Oncology Nursing, Faculty of Nursing, Toho University, Tokyo, Japan
| | - Kei Muro
- Department of Clinical Oncology, Aichi Cancer Center Hospital, Nagoya, Japan
| | - Yoichi Ajioka
- Division of Molecular and Diagnostic Pathology, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan
| | | | - Yoshinori Ito
- Department of Radiation Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Yutaka Saito
- Endoscopy Division, National Cancer Center Hospital, Tokyo, Japan
| | - Tetsuya Hamaguchi
- Division of Gastrointestinal Medical Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Megumi Ishiguro
- Department of Translational Oncology, Tokyo Medical and Dental University Graduate School, Tokyo, Japan
| | - Soichiro Ishihara
- Department of Surgical Oncology, The Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Yukihide Kanemitsu
- Colorectal Surgery Division, National Cancer Center Hospital, Tokyo, Japan
| | - Hiroshi Kawano
- Department of Gastroenterology, St. Mary's Hospital, Fukuoka, Japan
| | - Yusuke Kinugasa
- Department of Colon and Rectal Surgery, Shizuoka Cancer Center, Shizuoka, Japan
| | - Norihiro Kokudo
- Hepato-Pancreato-Biliary Surgery Division, Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
| | - Keiko Murofushi
- Radiation Oncology Department, The Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan
| | - Takako Nakajima
- Department of Clinical Oncology, St. Marianna University School of Medicine, Kawasaki, Japan
| | - Shiro Oka
- Gastroenterology and Metabolism, Hiroshima University Hospital, Hiroshima, Japan
| | | | - Akihiko Tsuji
- Department of Clinical Oncology, Faculty of Medicine, Kagawa University, Takamatsu, Japan
| | - Keisuke Uehara
- Division of Surgical Oncology, Department of Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Hideki Ueno
- Department of Surgery, National Defense Medical College, Saitama, Japan
| | - Kentaro Yamazaki
- Division of Gastrointestinal Oncology, Shizuoka Cancer Center, Shizuoka, Japan
| | - Masahiro Yoshida
- Department of Hemodialysis and Surgery, Chemotherapy Research Institute, International University of Health and Welfare, Ichikawa, Japan
| | - Takayuki Yoshino
- Department of Gastroenterology and Gastrointestinal Oncology, National Cancer Center Hospital East, Chiba, Japan
| | - Narikazu Boku
- Division of Gastrointestinal Medical Oncology, National Cancer Center Hospital, Tokyo, Japan
| | | | - Michio Itabashi
- Department of Surgery, Institute of Gastroenterology, Tokyo Women's Medical University, Tokyo, Japan
| | - Nobuo Koinuma
- Department of Health Administration and Policy, Tohoku Medical and Pharmaceutical University, Sendai, Japan
| | - Takayuki Morita
- Department of Surgery, Cancer Center, Aomori Prefectural Central Hospital, Aomori, Japan
| | - Genichi Nishimura
- Department of Surgery, Japanese Red Cross Kanazawa Hospital, Ishikawa, Japan
| | - Yuh Sakata
- CEO, Misawa City Hospital, Misawa, Japan
| | - Yasuhiro Shimada
- Division of Clinical Oncology, Kochi Health Sciences Center, Kochi, Japan
| | - Keiichi Takahashi
- Department of Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan
| | - Shinji Tanaka
- Department of Endoscopy, Hiroshima University Hospital, Hiroshima, Japan
| | - Osamu Tsuruta
- Division of GI Endoscopy, Kurume University School of Medicine, Fukuoka, Japan
| | - Toshiharu Yamaguchi
- Department of Gastroenterological Surgery, The Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan
| | | | - Toshiaki Watanabe
- Department of Surgical Oncology, The Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
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Surveillance using capsule endoscopy is safe in post-colectomy patients with familial adenomatous polyposis: a prospective Japanese study. Fam Cancer 2015; 15:75-83. [PMID: 26450841 DOI: 10.1007/s10689-015-9844-6] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
The utility of capsule endoscopy (CE) for the surveillance of small intestinal lesions in familial adenomatous polyposis (FAP) patients has been reported. However, few studies have investigated the safety of CE in FAP patients who have undergone colon surgery. We aimed to assess the safety of surveillance CE in post-colectomy FAP patients and the endoscopic findings associated with small intestinal lesions. We assessed the safety of CE surveillance of small intestinal lesions in 41 FAP patients who had undergone colectomies. Forty-two CEs were performed in 41 patients at our facility from April 2012 to July 2014. CE was conducted safely and none of the capsules were retained, despite the inclusion of patients who had undergone several abdominal surgeries previously. Thirty-nine out of 42 capsules (93 %) were retrieved within the examination timeframe; hence, the retrieval rate was favorable. The findings from this study indicate that surveillance CE can be safely conducted in post-colectomy FAP patients.
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Bizzotto A, Riccioni ME, Landi R, Marmo C, Barbaro B, Costamagna G. Small-Bowel Tumors, Polyps, and Polyposis Syndromes. ENDOSCOPY IN SMALL BOWEL DISORDERS 2015:175-198. [DOI: 10.1007/978-3-319-14415-3_13] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/05/2025]
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Tajika M, Niwa Y, Bhatia V, Tanaka T, Ishihara M, Yamao K. Risk of ileal pouch neoplasms in patients with familial adenomatous polyposis. World J Gastroenterol 2013; 19:6774-6783. [PMID: 24187452 PMCID: PMC3812476 DOI: 10.3748/wjg.v19.i40.6774] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/26/2013] [Revised: 07/17/2013] [Accepted: 08/20/2013] [Indexed: 02/06/2023] Open
Abstract
Restorative proctocolectomy is the most common surgical option for patients with familial adenomatous polyposis (FAP). However, adenomas may develop in the ileal pouch mucosa over time, and even carcinoma in the pouch has been reported. We therefore reviewed the prevalence, nature, and treatment of adenomas and carcinoma that develop after proctocolectomy in the ileal pouch mucosa in patients with FAP. In 25 reports that were reviewed, the incidence of adenomas in the ileal pouch varied from 6.7% to 73.9%. Several potential factors that favor the development of pouch polyposis have been investigated, but many remain controversial. Nevertheless, it seems certain that the age of the pouch is important. The risk appears to be 7% to 16% after 5 years, 35% to 42% after 10 years, and 75% after 15 years. On the other hand, only 21 cases of ileal pouch carcinoma have been recorded in the literature to date. The diagnosis of pouch carcinoma was made between 3 to 20 years (median, 10 years) after pouch construction. Although the risk of malignant transformation in ileal pouches is probably low, it is not negligible, and the long-term risk cannot presently be well quantified. Regular endoscopic surveillance, especially using chromoendoscopy, is recommended.
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Smith JC, Schäffer MW, Ballard BR, Smoot DT, Herline AJ, Adunyah SE, M'Koma AE. Adenocarcinomas After Prophylactic Surgery For Familial Adenomatous Polyposis. ACTA ACUST UNITED AC 2013; 4:260-270. [PMID: 23875116 DOI: 10.4236/jct.2013.41033] [Citation(s) in RCA: 37] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
The incidence of familial adenomatous polyposis (FAP) is one in 7,000 to 12,000 live births. Virtually, all surgically untreated patients with FAP inevitably develop colorectal-cancer in their lifetime because they carry the adenomatous polyposis coli gene. Thus prophylactic proctocolectomy is indicated. Surgical treatment of FAP is still controversial. There are however, four surgical options: ileorectal anastomosis, restorative proctocolectomy with ileal pouch-anal anastomosis, proctocolectomy with ileostomy, and proctocolectomy with continent-ileostomy. Conventional proctocolectomy options largely lie between colectomy with ileorectal anastomosis or ileal pouch-anal anastomosis. Detractors of ileal pouch-anal anastomosis prefer ileorectal anastomosis because of better functional results and quality of life. The functional outcome of total colectomy with ileorectal anastomosis is undoubtedly far superior to that of the ileoanal pouch; however, the risk for rectal cancer is increased by 30%. Even after mucosectomy, inadvertent small mucosal residual islands remain. These residual islands carry the potential for the development of subsequent malignancy. We reviewed the literature (1975-2012) on the incidence, nature, and possible etiology of subsequent ileal-pouch and anal transit zone adenocarcinoma after prophylactic surgery procedure for FAP. To date there are 24 studies reporting 92 pouch-related cancers; 15 case reports, 4 prospective and 5 retrospective studies. Twenty three of 92 cancers (25%) developed in the pouch mucosa and 69 (75%) in anal transit zone (ATZ). Current recommendation for pouch surveillance and treatment are presented. Data suggest lifetime surveillance of these patients.
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Affiliation(s)
- Joan C Smith
- Laboratory of Inflammatory Bowel Disease Research, Division of Biomedical Sciences, Department of Biochemistry and Cancer Biology, Meharry Medical College School of Medicine, Nashville, Tennessee
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Koornstra JJ. Small bowel endoscopy in familial adenomatous polyposis and Lynch syndrome. Best Pract Res Clin Gastroenterol 2012; 26:359-68. [PMID: 22704577 DOI: 10.1016/j.bpg.2012.01.022] [Citation(s) in RCA: 38] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/04/2011] [Accepted: 01/19/2012] [Indexed: 01/31/2023]
Abstract
Patients with familial adenomatous polyposis (FAP) and patients with Lynch syndrome have an increased risk of developing small intestinal neoplasia. In both conditions, the lifetime risk to develop small bowel cancer is estimated to be around 5%. In FAP, this risk is associated with the degree of duodenal polyposis, classically assessed by the Spigelman classification. For this reason, gastroduodenal surveillance with forward-viewing and side-viewing endoscopy is generally recommended. Studies using video capsule endoscopy and balloon-assisted enteroscopy in FAP patients have revealed that jejunal and ileal polyps occur frequently in FAP, especially in those with extensive duodenal polyposis. Nevertheless, the clinical relevance of small bowel polyps beyond the duodenum appears to be limited. Compared to FAP, little is known about the prevalence and natural history of small bowel neoplasia in Lynch syndrome. Surveillance of the small bowel is not recommended in Lynch syndrome, although recent data using capsule endoscopy provided promising results.
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Affiliation(s)
- Jan Jacob Koornstra
- Department of Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
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Asgeirsson T, Mascarenas C, Kaiser AM. Screening and Surveillance Strategies in Hereditary Colon and Rectal Cancer. SEMINARS IN COLON AND RECTAL SURGERY 2011; 22:88-94. [DOI: 10.1053/j.scrs.2010.12.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
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Abstract
BACKGROUND Familial adenomatous polyposis (FAP) is the most common inherited polyposis syndrome characterized by the development of hundreds of colorectal adenomatous polyps. The aim of this study was to review cases of FAP diagnosed at The Children's Hospital of Philadelphia in a 16-year period. METHODS Medical records of patients diagnosed as having FAP between 1990 and 2005 were reviewed. The collected data included disease presentation, genetic profile, extraintestinal manifestations, surveillance, and treatment. RESULTS We identified 12 patients with FAP. The age range at presentation was 7 to 18 years. Seven (68%) patients presented due to symptoms, the most common of which was rectal bleeding (6 patients, 86%). The youngest age at which polyps were detected was 7 years. Eight patients (67%) had positive family history. Three patients had Gardner syndrome and 1 presented in infancy with hepatoblastoma. Four patients had adenomatous polyposis coli gene mutation identified. One patient was diagnosed as having rectal carcinoma in situ. Six patients (50%) had gastric fundic gland polyposis and 6 had duodenal adenomatous changes. Capsule endoscopy was performed in 3 patients; 1 had multiple polyps in the duodenum and the jejunum. Seven patients (58%) underwent total colectomy with no serious complications. CONCLUSIONS FAP is a rare condition but with significant risk of cancer and comorbidity. In this series, patients commonly presented to medical attention due to their symptoms. The youngest patient with polyps detected was 7 years old. We identified 1 patient with rectal cancer in situ and high proportion of patients with duodenal adenomatous lesions. Majority of patients underwent early colectomy.
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Capsule endoscopy in small-bowel surveillance of patients with hereditary polyposis syndromes. Int J Colorectal Dis 2010; 25:1377-82. [PMID: 20544205 DOI: 10.1007/s00384-010-0982-x] [Citation(s) in RCA: 31] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 05/27/2010] [Indexed: 02/04/2023]
Abstract
PURPOSE Familial adenomatous polyposis (FAP) and Peutz-Jeghers syndrome (PJS) are hereditary polyposis syndromes with a high risk for benign small-bowel polyps and cancer. The aim of this study was to assess the prevalence of small-bowel polyps beyond the duodenum in patients with FAP and PJS and to examine the clinical value and the optimal interval of capsule endoscopy (CE) for the surveillance of small-bowel polyps in patients with FAP. METHODS Between 2002 and 2009, standard gastroscopy, duodenoscopy, and CE were performed on 19 consecutive patients with hereditary polyposis syndromes (FAP n=15; PJS n=4). The number, size, and location of polyps detected by CE were assessed. Five FAP patients had repeated CEs in intervals of 2-7 years. RESULTS In 13 of the 15 (87%) FAP patients, small-bowel polyps were detected by CE ranging from estimated <5 mm to >10 mm in size. Thereof, in four patients, medium-sized (5-10 mm) or large-sized (>10 mm) polyps were seen-all of them located in the proximal jejunum. In three FAP patients with repeated CEs, the latest CE displayed medium- and large-sized polyps in the proximal jejunum, whereas previous CEs had detected only small-sized (<5 mm) polyps. In three of the four PJS patients, large-sized small-bowel polyps were visualized by CE which could then be removed by double-balloon enteroscopy (DBE) or surgical resection. CONCLUSION CE is an effective and safe method for small-bowel surveillance in FAP and PJS.
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Wibmer AG, Kroesen AJ, Gröne J, Slavova N, Weinhold A, Buhr HJ, Ritz JP. Predictors of permanent ileostomy after restorative proctocolectomy. Br J Surg 2010; 97:1561-6. [PMID: 20632324 DOI: 10.1002/bjs.7135] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
BACKGROUND Proctocolectomy with ileal pouch-anal anastomosis (IPAA) is a surgical approach for ulcerative colitis and familial adenomatous polyposis. This study evaluated predictors of the need for a permanent ileostomy to identify patients at high risk of IPAA failure. METHODS This was a retrospective analysis of patients who underwent proctocolectomy and IPAA between 1997 and 2008. A logistic regression model was used for multivariable analysis of potential risk factors. RESULTS Proctocolectomy was combined with IPAA in 185 patients, of whom 169 had a loop ileostomy formed. IPAA and ileostomy closure were successful in 162 patients (87.6 per cent). Reasons for not closing the ileostomy included pouch failure (16 patients), patient choice (5) and death (2). Thus one in eight patients had a permanent ileostomy after planned IPAA. Age was the major predictor of the need for a permanent ileostomy in multivariable analysis (P = 0.002) with a probability of more than 25 per cent in patients aged over 60 years. However, advancing age was associated with colitis, co-morbidity, obesity and corticosteroid use. CONCLUSION The probability of the need for a permanent ileostomy after IPAA increases with age.
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Affiliation(s)
- A G Wibmer
- Department of General, Visceral, Vascular and Thoracic Surgery, Charité, Campus Benjamin Franklin, Berlin, Germany
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Abstract
Total proctocolectomy and ileal pouch-anal anastomosis is the operation of choice for patients with familial adenomatous polyposis. With this operation comes the risk of developing ileal pouch polyps. Although rare, ileal pouch carcinomas may also occur within the pouch. Periodic endoscopic surveillance of the retained rectum and ileal pouch is recommended. Endoscopic polypectomy of medium and large polyps should be performed. Sulindac is effective in the reduction and often in the elimination of numerous smaller pouch polyps. Future studies are necessary to determine the role of sulindac and other chemotherapeutic agents in preventing the development of these polyps.
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Affiliation(s)
- David P O'Brien
- Colon and Rectal Surgery, Gastrointestinal and Minimally Invasive Surgery Division, The Oregon Clinic, PC, Portland, OR 97210, USA.
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Tescher P, Macrae FA, Speer T, Stella D, Gibson R, Tye-Din JA, Srivatsa G, Jones IT, Marion K. Surveillance of FAP: a prospective blinded comparison of capsule endoscopy and other GI imaging to detect small bowel polyps. Hered Cancer Clin Pract 2010; 8:3. [PMID: 20361877 PMCID: PMC2859487 DOI: 10.1186/1897-4287-8-3] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/21/2009] [Accepted: 04/04/2010] [Indexed: 12/21/2022] Open
Abstract
Background Familial adenomatous polyposis (FAP) is a hereditary disorder characterized by polyposis along the gastrointestinal tract. Information on adenoma status below the duodenum has previously been restricted due to its inaccessibility in vivo. Capsule Endoscopy (CE) may provide a useful adjunct in screening for polyposis in the small bowel in FAP patients. This study aims to evaluate the effectiveness of CE in the assessment of patients with FAP, compared to other imaging modalities for the detection of small bowel polyps. Method 20 consecutive patients with previously diagnosed FAP and duodenal polyps, presenting for routine surveillance of polyps at The Royal Melbourne Hospital were recruited. Each fasted patient initially underwent a magnetic resonance image (MRI) of the abdomen, and a barium small bowel follow-through study. Capsule Endoscopy was performed four weeks later on the fasted patient. An upper gastrointestinal side-viewing endoscopy was done one (1) to two (2) weeks after this. Endoscopists and investigators were blinded to results of other investigations and patient history. Results Within the stomach, upper gastrointestinal endoscopy found more polyps than other forms of imaging. SBFT and MRI generally performed poorly, identifying fewer polyps than both upper gastrointestinal and capsule endoscopy. CE was the only form of imaging that identified polyps in all segments of the small bowel as well as the only form of imaging able to provide multiple findings outside the stomach/duodenum. Conclusion CE provides important information on possible polyp development distal to the duodenum, which may lead to surgical intervention. The place of CE as an adjunct in surveillance of FAP for a specific subset needs consideration and confirmation in replication studies. Trial Registration Australian New Zealand Clinical Trials Registry ACTRN12608000616370
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Affiliation(s)
- Paul Tescher
- Department of Colorectal Medicine and Genetics, The Royal Melbourne Hospital, Melbourne, Australia.
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Prevalence of adenomas and carcinomas in the ileal pouch after proctocolectomy in patients with familial adenomatous polyposis. J Gastrointest Surg 2009; 13:1266-73. [PMID: 19333660 DOI: 10.1007/s11605-009-0871-1] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/07/2008] [Accepted: 03/06/2009] [Indexed: 02/06/2023]
Abstract
PURPOSE Restorative proctocolectomy has become the most common surgical option for patients with familial adenomatous polyposis (FAP). However, adenomas may develop in the ileal pouch mucosa over time, and even carcinoma in the pouch has been reported. Our aim was to evaluate the prevalence, nature, and etiology of ileal pouch and nonpouch adenomas and carcinoma in patients with FAP. PATIENTS AND METHODS This was a retrospective study of 31 FAP patients with Kock's continent ileostomy (Kock; n = 8), ileorectal anastomosis (IRA; n = 7), and ileal pouch-anal anastomosis (IPAA) (n = 16). All patients were followed with a standardized protocol including chromoendoscopy and biopsies of visible polyps in the ileal pouch and nonpouch mucosa. RESULTS Sixteen of 24 pouch patients (Kock and IPAA) developed adenomas in the ileal pouch mucosa, and all patients with IRA developed adenomas in the rectal mucosa. The prevalence of ileal adenomas was significantly higher in pouch patients than in IRA patients (P = 0.002). Only one patient with Kock showed adenoma in the prepouch area. Two cases of adenocarcinomas and one case of advanced adenoma were found in the ileal pouch mucosa. CONCLUSION Our results show a high frequency of adenomas in the ileal pouch mucosa, with evolution into carcinoma in some patients. Regular endoscopic surveillance of the pouch is recommended at a frequency similar to that for the rectal mucosa after IRA in pouch patients with FAP.
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Abstract
While the overall incidence of pouchitis is low, extensive research continues at clinical and experimental levels in attempts to unravel its etiology. The ileal pouch and pouchitis together represent a unique in vivo opportunity to study mucosal adaptation and inflammation in depth. In the recent past, molecular data relating to pouchitis has significantly expanded. These data provide invaluable insight into intracellular and extracellular events that underpin mucosal adaptation and inflammation. Advances in classification, risk factor evaluation, and prevention have meant that a review of this data, as well as its relationship to our current understanding of pouchitis, is both timely and warranted. Therefore, the aim of this review is to summarize recent data in the context of the established literature.
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Affiliation(s)
- John Calvin Coffey
- Department of Academic Surgery, Cork University Hospital, Cork, Ireland.
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Progression to advanced neoplasia is infrequent in post colectomy familial adenomatous polyposis patients under endoscopic surveillance. Fam Cancer 2008; 8:33-8. [DOI: 10.1007/s10689-008-9203-y] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2007] [Accepted: 07/06/2008] [Indexed: 01/27/2023]
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