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Alves Martins BA, Shamsiddinova A, Worley GHT, Hsu YJ, Cuthill V, Hawkins M, Sinha A, Jenkins JT, Miskovic D, Clark SK, Faiz OD. Employing innovation to enhance the safety and reliability of restorative surgical techniques for patients with familial adenomatous polyposis at a national referral centre. Tech Coloproctol 2024; 28:150. [PMID: 39520507 DOI: 10.1007/s10151-024-03021-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/24/2024] [Accepted: 09/21/2024] [Indexed: 11/16/2024]
Abstract
INTRODUCTION Restorative proctocolectomy (RPC) and total colectomy with ileorectal anastomosis (TC-IRA) are traditional surgical options for individuals with familial adenomatous polyposis (FAP). Re-appraisal and modification to these techniques, such as near-total colectomy with ileo-distal sigmoid anastomosis (NT-IDSA) and RPC with robotic intracorporeal single-stapled anastomosis (RPC-RiSSA), have been implemented in recent years. This study aimed to evaluate the early postoperative outcomes associated with novel techniques employed in a single centre for restorative surgery in patients with FAP. METHODS A retrospective analysis was conducted using data from patients with FAP who underwent prophylactic restorative surgery between January 2008 and December 2022 at St Mark's Hospital. RESULTS Two hundred fifty-three individuals underwent restorative surgery over the 15-year period; 102/253 (40.3%) underwent TC-IRA, 84 (33.2%) had NT-IDSA, and 67 (26.5%) underwent RPC. Laparoscopic approach was the most common (88.2%) operative access. Seventeen patients (6.7%) underwent robotic operations. For robotic-assisted procedures, no conversions were reported. No anastomotic leaks or 30-day reoperations were reported in the NT-IDSA group compared to 8% (0/84 vs 8/102, p = 0.009) and 11% (0/84 vs 11/102, p = 0.002), respectively, in the TC-IRA group. Regarding RPC, following the introduction of robotic RPC-RiSSA in 2019, no anastomotic leakage was observed compared with 9% (0/11 vs 5/56, p = 0.3) in those undergoing conventional RPC. CONCLUSION Our institution has transitioned from TC-IRA to NT-IDSA since 2014 and conventional RPC to RPC-RiSSA in 2019. To date, since refinement of the techniques there have been no anastomotic failures amongst these cohorts. The reported results may offer future horizons for patients undergoing similar procedures for alternative colorectal diseases.
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Affiliation(s)
- B A Alves Martins
- Department of Colorectal Surgery, St Mark's Hospital, London North West University Healthcare NHS Trust, London, UK
- Department of Colorectal Surgery, University Hospital of Brasília, Brasília, Brazil
| | - A Shamsiddinova
- Department of Colorectal Surgery, St Mark's Hospital, London North West University Healthcare NHS Trust, London, UK
- Department of Surgery and Cancer, Imperial College, London, UK
| | - G H T Worley
- Department of Colorectal Surgery, St Mark's Hospital, London North West University Healthcare NHS Trust, London, UK
| | - Y-J Hsu
- Department of Colorectal Surgery, St Mark's Hospital, London North West University Healthcare NHS Trust, London, UK
- Division of Colon and Rectal Surgery, Chang Gung Memorial Hospital, Taoyuan City, Taiwan
| | - Victoria Cuthill
- St Mark's Centre for Familial Intestinal Cancer, London North West University Healthcare NHS Trust, London, UK
| | - M Hawkins
- St Mark's Centre for Familial Intestinal Cancer, London North West University Healthcare NHS Trust, London, UK
| | - A Sinha
- Department of Colorectal Surgery, St Mark's Hospital, London North West University Healthcare NHS Trust, London, UK
- Department of Surgery and Cancer, Imperial College, London, UK
- St Mark's Centre for Familial Intestinal Cancer, London North West University Healthcare NHS Trust, London, UK
| | - J T Jenkins
- Department of Colorectal Surgery, St Mark's Hospital, London North West University Healthcare NHS Trust, London, UK
| | - D Miskovic
- Department of Colorectal Surgery, St Mark's Hospital, London North West University Healthcare NHS Trust, London, UK
| | - S K Clark
- Department of Colorectal Surgery, St Mark's Hospital, London North West University Healthcare NHS Trust, London, UK
- Department of Surgery and Cancer, Imperial College, London, UK
- St Mark's Centre for Familial Intestinal Cancer, London North West University Healthcare NHS Trust, London, UK
| | - O D Faiz
- Department of Colorectal Surgery, St Mark's Hospital, London North West University Healthcare NHS Trust, London, UK.
- Department of Surgery and Cancer, Imperial College, London, UK.
- St Mark's Centre for Familial Intestinal Cancer, London North West University Healthcare NHS Trust, London, UK.
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Sommovilla J, Shepard D, Liska D. Management of Desmoid Disease in Familial Adenomatous Polyposis. Clin Colon Rectal Surg 2024; 37:185-190. [PMID: 38606047 PMCID: PMC11006445 DOI: 10.1055/s-0043-1770731] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/13/2024]
Abstract
Desmoid disease, though technically a benign condition, is nevertheless a leading cause of morbidity and mortality in patients with familial adenomatous polyposis (FAP). Desmoid disease impacts approximately 30% of FAP patients, with several known risk factors. It runs the gamut in terms of severity-ranging from small, slow-growing asymptomatic lesions to large, focally destructive, life-threatening masses. Desmoids usually occur following surgery, and several patient risk factors have been established, including female sex, family history of desmoid disease, 3' APC mutation, and extraintestinal manifestations of FAP. Desmoid disease-directed therapy is individualized and impacted by desmoid stage, severity, postsurgical anatomy, and consequences of disease. Medical therapy consists of options in multiple classes of drugs: nonsteroidal anti-inflammatory drugs, hormonal therapy, tyrosine kinase inhibitors, and cytotoxic agents. Surgical excision is sometimes an option, but can be limited by common location of disease at the root of the small bowel mesentery. Palliative surgical treatments are often considered in management of desmoid disease. Intestinal transplantation for severe desmoid disease is an emerging and promising option, though long-term data on efficacy and survival is limited.
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Affiliation(s)
- Joshua Sommovilla
- Department of Colon and Rectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Sanford R Weiss MD Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
| | - Dale Shepard
- Department of Hematology and Medical Oncology, Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio
| | - David Liska
- Department of Colon and Rectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Sanford R Weiss MD Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
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Banerjee S, Burke CA, Sommovilla J, Anderson CE, O'Malley M, LaGuardia LA, Vazquez Villasenor AC, Macaron C, Liska D. Risk of Proctectomy After Ileorectal Anastomosis in Familial Adenomatous Polyposis in the Modern Era. Dis Colon Rectum 2024; 67:427-434. [PMID: 38064246 DOI: 10.1097/dcr.0000000000003157] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/09/2024]
Abstract
BACKGROUND Prophylactic surgery for familial adenomatous polyposis has evolved over several decades. Restorative proctocolectomy with IPAA provides an alternative to total abdominal colectomy with ileorectal anastomosis. We have previously shown that the rate of proctectomy and rectal cancer after total abdominal colectomy with ileorectal anastomosis in the "pre-pouch era" was 32% and 13%, respectively. OBJECTIVE To determine the rate of proctectomy and rectal cancer among familial adenomatous polyposis patients and relative rectal sparing (fewer than 20 rectal polyps) selected for total abdominal colectomy with ileorectal anastomosis in the modern era. DESIGN Retrospective cohort study. SETTING Single tertiary care institution with a hereditary colorectal cancer registry. PATIENTS Patients with familial adenomatous polyposis who underwent total abdominal colectomy with ileorectal anastomosis between 1993 and 2020. MAIN OUTCOME MEASURES Incidence of proctectomy for any indication and rectal cancer. RESULTS A total of 197 patients with a median age of 24 years (range, 10-67) were included. The median follow-up after total abdominal colectomy with ileorectal anastomosis was 13 years (interquartile range, 6-17). Sixteen patients (8%) underwent proctectomy. Indications included rectal cancer in 6 patients (3%; 2 stage I and 4 stage III), polyps with high-grade dysplasia in 4 (2%), progressive polyp burden in 3 (1.5%), defecatory dysfunction in 2 (1%), and anastomotic leak in 1 (0.5%). Among 30 patients (18%) with 20 or more rectal polyps at the time of total abdominal colectomy with ileorectal anastomosis, 8 patients (26%) underwent proctectomy and 3 patients developed rectal cancer (10%). Among 134 patients (82%) with fewer than 20 polyps, 8 patients (6%) underwent proctectomy and 3 patients developed rectal cancer (2%). Number of rectal polyps at the time of total abdominal colectomy with ileorectal anastomosis was associated with the likelihood of proctectomy (OR 1.1, p < 0.001) but not incident rectal cancer ( p = 0.3). LIMITATION Retrospective data collection. CONCLUSIONS Patients with familial adenomatous polyposis selected for total abdominal colectomy with ileorectal anastomosis by rectal polyp number have low rates of proctectomy and rectal cancer compared to historical controls. With appropriate selection criteria and surveillance, total abdominal colectomy with ileorectal anastomosis remains an important and safe treatment option for patients with familial adenomatous polyposis. See Video Abstract . RIESGO DE PROCTECTOMA DESPUS DE ANASTOMOSIS ILEORRECTAL EN POLIPOSIS ADENOMATOSA FAMILIAR EN LA ERA MODERNA ANTECEDENTES:La cirugía profiláctica para la poliposis adenomatosa familiar (PAF) ha evolucionado durante varias décadas. La proctocolectomía restauradora con anastomosis anal con bolsa ileal (IPAA) proporciona una alternativa a la colectomía abdominal total con anastomosis ileorrectal (TAC/IRA). Anteriormente hemos demostrado que la tasa de proctectomía y cáncer de recto después de TAC/IRA en la era "pre-bolsa" era del 32% y el 13%, respectivamente.OBJETIVO:Determinar la tasa de proctectomía y cáncer de recto entre pacientes con PAF y pacientes con preservación rectal relativa (<20 pólipos rectales) seleccionados para TAC/IRA en la era moderna.DISEÑO:Estudio de cohorte retrospectivo.ÁMBITO:Institución única de atención terciaria con un registro de cáncer colorrectal hereditario.PACIENTES:Pacientes con PAF que se sometieron a TAC/IRA entre 1993 y 2020.MEDIDAS DE RESULTADO PRINCIPALES:Incidencia de proctectomía por cualquier indicación y cáncer de recto.RESULTADOS:Se incluyeron 197 pacientes con una mediana de edad de 24 años (rango 10-67). La mediana de seguimiento tras TAC/IRA fue de 13 años (RIC 6-17). 16 pacientes (8%) fueron sometidos a proctectomía. Las indicaciones incluyeron cáncer de recto en 6 (3%) (2 en estadio I y 4 en estadio III); pólipos con displasia de alto grado en 4 (2%); carga progresiva de pólipos en 3 (1,5%), disfunción defecatoria en 2 (1%); y fuga anastomótica en 1 (0,5%). Entre 30 pacientes (18%) con ≥20 pólipos rectales en el momento de TAC/IRA, 8 pacientes (26%) se sometieron a proctectomía y 3 pacientes desarrollaron cáncer de recto (10%). Entre 134 pacientes (82%) con <20 pólipos, 8 pacientes (6%) se sometieron a proctectomía y 3 pacientes desarrollaron cáncer de recto (2%). El número de pólipos rectales en el momento de TAC/IRA se asoció con la probabilidad de proctectomía (OR 1,1, p <0,001), pero no con la incidencia de cáncer de recto (p = 0,3).LIMITACIÓN:Recopilación de datos retrospectivos.CONCLUSIÓN:Los pacientes con PAF seleccionados para TAC/IRA por el número de pólipos rectales tienen tasas bajas de proctectomía y cáncer de recto en comparación con los controles históricos. Con criterios de selección y vigilancia adecuados, TAC/IRA sigue siendo una opción de tratamiento importante y segura para los pacientes con PAF. (Pre-proofed version ).
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Affiliation(s)
- Sudeep Banerjee
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
| | - Carol A Burke
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Sanford R. Weiss MD Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Department of Gastroenterology, Hepatology and Nutrition, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
| | - Joshua Sommovilla
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Sanford R. Weiss MD Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
| | - Cristan E Anderson
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Sanford R. Weiss MD Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
| | - Margaret O'Malley
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Sanford R. Weiss MD Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
| | - Lisa A LaGuardia
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Sanford R. Weiss MD Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
| | - Ana C Vazquez Villasenor
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Sanford R. Weiss MD Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
| | - Carole Macaron
- Sanford R. Weiss MD Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Department of Gastroenterology, Hepatology and Nutrition, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
| | - David Liska
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
- Sanford R. Weiss MD Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
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Campos FG, Martinez CAR, Bustamante-Lopez LA, Mendonça RLDS, Kanno DT. Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development? Clinics (Sao Paulo) 2023; 78:100144. [PMID: 36476966 PMCID: PMC9723922 DOI: 10.1016/j.clinsp.2022.100144] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/25/2022] [Revised: 11/03/2022] [Accepted: 11/07/2022] [Indexed: 12/07/2022] Open
Abstract
OBJECTIVE Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk patients and modification of operative strategy may be crucial. AIM The objective of this study was to estimate the desmoid tumor risk in relation to various surgical and clinical variables. METHODS Patients who had undergone polyposis since 1958 were included in the study. After exclusion criteria were met, those who had developed desmoid tumors were selected to undergo further evaluation. RESULTS The study revealed that the risk of developing desmoid tumors was associated with various factors such as sex ratio, colectomy, and reoperations. On the other hand, the type of surgery, family history, and surgical approach did not affect the risk of developing desmoid tumors. The data collected from 146 polyposis patients revealed that 16% had desmoid polyps. The sex ratio was 7:1, and the median age at colectomy was 28.6 years. Family history, multiple abdominal operations, and reoperations were some of the characteristics that were common in desmoid patients. CONCLUSION Recognition of clinical (female sex) and surgical (timing of surgery and previous reoperations) data as unfavorable variables associated with greater risk may be useful during the decision-making process.
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Affiliation(s)
- Fábio Guilherme Campos
- Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, SP, Brazil
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Efficacy of Wholistic Turmeric Supplement on Adenomatous Polyps in Patients with Familial Adenomatous Polyposis-A Randomized, Double-Blinded, Placebo-Controlled Study. Genes (Basel) 2022; 13:genes13122182. [PMID: 36553450 PMCID: PMC9777742 DOI: 10.3390/genes13122182] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2022] [Revised: 11/05/2022] [Accepted: 11/18/2022] [Indexed: 11/24/2022] Open
Abstract
Several studies have demonstrated that curcumin can cause the regression of polyps in familial adenomatous polyposis (FAP), while others have shown negative results. Wholistic turmeric (WT) containing curcumin and additional bioactive compounds may contribute to this effect. We performed a double-blinded, randomized, controlled trial to assess the efficacy of WT in FAP patients. Ten FAP patients were randomly assigned to receive either WT or placebo for 6 months. Colonoscopies were performed at baseline and after 6 months. The polyp number and size, as well as the cumulative polyp burden, were assessed. No differences were noted between the groups in terms of changes from the baseline's polyp number, size, or burden. However, stratifying the data according to the right vs. left colon indicated a decrease in the median polyp number (from 5.5 to 1.5, p = 0.06) and polyp burden (from 24.25 mm to 11.5 mm, p = 0.028) in the left colon of the patients in the WT group. The adjusted left polyp number and burden in the WT arm were lower by 5.39 (p = 0.034) and 14.68 mm (p = 0.059), respectively. Whether WT can be used to reduce the polyp burden of patients with predominantly left-sided polyps remains to be seen; thus, further larger prospective trials are required.
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Abstract
BACKGROUND Desmoid disease is a leading cause of morbidity and mortality in patients with familial adenomatous polyposis. Abdominal desmoid disease usually follows total proctocolectomy with IPAA or total abdominal colectomy with ileorectal anastomosis. Sex, extraintestinal manifestations, and a 3'-mutation location have been identified as risk factors, but surgical risk factors are poorly understood. We hypothesized that pouch construction creates a higher risk of desmoid formation due to the increased stretch of the small-bowel mesentery. OBJECTIVE This study aimed to investigate the surgical risk factors for desmoid formation. DESIGN This was a retrospective, single-center, registry-based cohort study. SETTINGS This study was conducted at a single academic institution with a prospectively maintained hereditary colorectal cancer database between 1995 and 2015. PATIENTS All patients with familial polyposis (total 345) who underwent either proctocolectomy with a pouch or colectomy with an ileorectal anastomosis during the study period and met inclusion criteria were selected. MAIN OUTCOME MEASURES The development of symptomatic abdominal desmoid disease was the primary end point. Associations between desmoid formation and resection type, surgical approach, and other patient factors were analyzed. RESULTS A total of 172 (49%) patients underwent proctocolectomy/ileoanal pouch, whereas 173 (51%) underwent total colectomy/ileorectal anastomosis. Overall, 100 (28.9%) developed symptomatic desmoids after surgery. On univariable analysis, open surgery and pouch surgery were associated with desmoid development, along with extracolonic manifestations, family history of desmoids, mutation location, and a high desmoid risk score. On multivarible analysis, proctocolectomy with pouch was most strongly associated with desmoid disease ( p < 0.01). LIMITATIONS This study was limited by its retrospective nature, the lack of uniform desmoid screening, and the variable duration of follow-up. Unanalyzed confounding factors include polyposis severity and number of surgeries. CONCLUSIONS Patients with polyposis who underwent total proctocolectomy with pouch by any approach had significantly greater risk of developing desmoid disease than total colectomy with ileorectal anastomosis, even when accounting for other risk factors. See Video Abstract at http://links.lww.com/DCR/B822 .RESULTADOS DE LOS PACIENTES SOMETIDOS A RESECCIÓN INTESTINAL ELECTIVA ANTES Y DESPUÉS DE LA IMPLEMENTACIÓN DE UN PROGRAMA DE DETECCIÓN Y TRATAMIENTO DE ANEMIA. ANTECEDENTES Se sabe que los pacientes anémicos que se someten a una cirugía electiva de cáncer colorrectal tienen tasas significativamente más altas de complicaciones posoperatorias y peores resultados. OBJETIVO Mejorar las tasas de detección y tratamiento de la anemia en pacientes sometidos a resecciones electivas de colon y recto a través de una iniciativa de mejora de calidad. DISEO Comparamos una cohorte histórica de pacientes antes de la implementación de nuestro programa de detección de anemia y mejora de la calidad del tratamiento con una cohorte prospectiva después de la implementación. ENTORNO CLINICO Hospital de atención terciaria. PACIENTES Todos los pacientes adultos con un nuevo diagnóstico de cáncer de colon o recto sin evidencia de enfermedad metastásica entre 2017 y 2019. INTERVENCIONES Detección de anemia y programa de mejora de la calidad del tratamiento. PRINCIPALES MEDIDAS DE RESULTADO El resultado primario fue el costo hospitalario por ingreso. RESULTADOS Un total de 84 pacientes se sometieron a resección electiva de colon o recto antes de la implementación de nuestro proyecto de mejora de calidad de la anemia y 88 pacientes se sometieron a cirugía después. En la cohorte previa a la implementación, 44/84 (55,9 %) presentaban anemia en comparación con 47/99 (54,7 %) en la cohorte posterior a la implementación. Las tasas de detección (25 % a 86,4 %) y tratamiento (27,8 % a 63,8 %) aumentaron significativamente en la cohorte posterior a la implementación. El costo total medio por admisión se redujo significativamente en la cohorte posterior a la implementación (costo medio $16 827 vs. $25 796, p = 0,004); esta reducción significativa se observó incluso después de ajustar los factores de confusión relevantes (proporción de medias: 0,74, IC del 95 %: 0,65 a 0,85). El vínculo mecánico entre el tratamiento de la anemia y la reducción de costos sigue siendo desconocido. No hubo diferencias significativas en las tasas de transfusión de sangre, complicaciones o mortalidad entre los grupos. LIMITACIONES El diseño de antes y después está sujeto a sesgos temporales y de selección. CONCLUSIONES Demostramos la implementación exitosa de un programa de detección y tratamiento de anemia. Este programa se asoció con un costo por admisión significativamente reducido. Este trabajo demuestra el valor y los beneficios posibles de la implementación de un programa de detección y tratamiento de la anemia. Consulte Video Resumen en http://links.lww.com/DCR/C15 . (Traducción- Dr. Francisco M. Abarca-Rendon ).
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Flahive CB, Onwuka A, Bass LM, MacFarland SP, Minneci PC, Erdman SH. Characterizing Pediatric Familial Adenomatous Polyposis in Patients Undergoing Colectomy in the United States. J Pediatr 2022; 245:117-122. [PMID: 34560095 DOI: 10.1016/j.jpeds.2021.09.021] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/22/2021] [Revised: 08/16/2021] [Accepted: 09/15/2021] [Indexed: 10/20/2022]
Abstract
OBJECTIVE To characterize a multi-institutional cohort of pediatric patients who underwent colectomy for familial adenomatous polyposis (FAP). STUDY DESIGN In this retrospective cohort study, diagnosis and procedure codes were used to identify patients who underwent colectomy for FAP within the Pediatric Health Information System (PHIS). The inclusion criteria were validated at 3 children's hospitals and applied to PHIS to generate a cohort of patients with FAP between 2 and 21 years who had undergone colectomy between 2009 and 2019. Demographics, clinical and surgical characteristics, and endoscopic procedure trends as identified through PHIS are described. Descriptive and comparative statistics were used to analyze data. RESULTS Within the PHIS, 428 pediatric patients with FAP who underwent colectomy were identified. Median age at colectomy was 14 years (range 2-21 years); 264 patients (62%) received an ileal pouch anal anastomosis and 13 (3%) underwent ileorectal anastomosis. Specific anastomotic surgical procedure codes were not reported for 151 patients (35%). Endoscopic assessment at the surgical institution occurred in 40% of the cohort before colectomy and in 22% of the cohort following colectomy. CONCLUSIONS In this cohort, colectomy took place at an earlier age than suggested in published guidelines. Ileal pouch anal anastomosis is the predominant procedure for pediatric patients with FAP who underwent colectomy in US pediatric centers. Endoscopic assessment trends before and after surgery suggest that the surgical institution plays a limited role in the care of this population.
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Affiliation(s)
- Colleen B Flahive
- Division of Gastroenterology Hepatology and Nutrition, Department of Pediatrics, Nationwide Children's Hospital, Columbus, OH.
| | - Amanda Onwuka
- Center for Surgical Outcomes Research, Abigail Wexner Research Institute and Department of Surgery, Nationwide Children's Hospital, Columbus, OH
| | - Lee M Bass
- Division of Gastroenterology Hepatology and Nutrition, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL
| | - Suzanne P MacFarland
- Division of Oncology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA
| | - Peter C Minneci
- Center for Surgical Outcomes Research, Abigail Wexner Research Institute and Department of Surgery, Nationwide Children's Hospital, Columbus, OH
| | - Steven H Erdman
- Division of Gastroenterology Hepatology and Nutrition, Department of Pediatrics, Nationwide Children's Hospital, Columbus, OH
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Anele CC, Xiang J, Martin I, Hawkins M, Man R, Clark SK, Faiz OD, Latchford A. Regular endoscopic surveillance and polypectomy is effective in managing rectal adenoma progression following colectomy and ileorectal anastomosis in patients with familial adenomatous polyposis. Colorectal Dis 2022; 24:277-283. [PMID: 34741380 DOI: 10.1111/codi.15981] [Citation(s) in RCA: 17] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/11/2021] [Revised: 10/28/2021] [Accepted: 11/01/2021] [Indexed: 01/04/2023]
Abstract
AIM Total colectomy with ileorectal anastomosis (TC-IRA) is a surgical option for patients with familial adenomatous polyposis (FAP). Regular endoscopic surveillance of the rectum is recommended to prevent rectal cancer. We aimed to document polyp progression in the rectum following TC-IRA and evaluate the role of polypectomy during surveillance. METHOD Patients with FAP who underwent TC-IRA between 1990 and 2017 were identified. Demographic, endoscopic and genetic data were retrieved. Cumulative rectal adenoma (polyp) counts were obtained, whilst accounting for any polypectomies during the study period. The rate of polyp progression and factors influencing secondary proctectomy were evaluated. RESULTS One hundred and ninety-nine patients fulfilled our inclusion criteria, of which 44% were male. The median age at colectomy was 19 (range 11-70) years and median preoperative rectal polyp count was 7 (range 0-50). All patients had an APC pathogenic variant, of which 151 (79%) were 5' of the mutation cluster region (MCR), 19 (10%) in the MCR, six (3%) were 3' of the MCR and 15 (8%) had a gross deletion. After a median follow-up of 8.6 (range1-27) years and a median of 11 (range 2-37) flexible sigmoidoscopies per patient, the median rate of polyp progression was 5.5 polyps/year (range 0-70.2). There was no evidence of polyp regression. Eight (4%) patients underwent secondary proctectomy for neoplasia, of which one (0.5%) had rectal adenocarcinoma. A total of 13,527 polyps were removed, a median of 35 polyps/patient (range 0-829). The rate of polyp progression was not significantly associated with genotypic or phenotypic factors. CONCLUSION Progression of rectal adenoma burden following TC-IRA appears to be slow and dependent on the length of follow-up. In the modern era of stringent endoscopic surveillance and therapeutic procedures such as cold snare polypectomy, the rate of secondary proctectomy and the risk of rectal cancer after TC-IRA are very low. These findings are important when counselling patients with regard to the choice of surgery for FAP and implementing endoscopic surveillance.
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Affiliation(s)
- Chukwuemeka C Anele
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK.,Department of Surgery and Cancer, Imperial College, London, UK
| | - Jinpo Xiang
- Department of Medicine, Imperial College, London, UK
| | - Isabel Martin
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK
| | - Menna Hawkins
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK
| | - Ripple Man
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK
| | - Susan K Clark
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK
| | - Omar D Faiz
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK
| | - Andrew Latchford
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK
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9
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Gilad O, Rosner G, Brazowski E, Kariv R, Gluck N, Strul H. Management of pouch related symptoms in patients who underwent ileal pouch anal anastomosis surgery for adenomatous polyposis. World J Clin Cases 2021; 9:9847-9856. [PMID: 34877323 PMCID: PMC8610899 DOI: 10.12998/wjcc.v9.i32.9847] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/12/2021] [Revised: 07/23/2021] [Accepted: 09/23/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Adenomatous polyposis syndromes (APS) patients with ileal pouch anal anastomosis (IPAA) suffer frequent symptoms with scarce signs of inflammation, distinct from ulcerative colitis patients. While the management of pouchitis in ulcerative colitis patients is well established, data regarding response to treatment modalities targeting pouch-related disorders in APS patient population is scarce.
AIM To assess clinical, endoscopic and histologic response to various treatment modalities employed in the therapy of pouch related disorders.
METHODS APS patients who underwent IPAA between 1987-2019 were followed every 6-12 mo and pouch-related symptoms were recorded at every visit. Lower endoscopy was performed annually, recording features of the pouch, cuff and terminal ileum. A dedicated gastrointestinal pathologist reviewed biopsies for signs and severity of inflammation. At current study, files were retrospectively reviewed for initiation and response to various treatment modalities between 2015-2019. Therapies included dietary modifications, probiotics, loperamide, antibiotics, bismuth subsalicylate, mebeverine hydrochloride, 5-aminosalicylic acid compounds and topical rectal steroids. Symptoms and endoscopic and histologic signs of inflammation before and after treatment were assessed. Pouchitis disease activity index (PDAI) and its subscores was calculated. Change of variables before and after therapy was assessed using Wilcoxon signed rank test for continuous variables and using McNemar's test for categorical variables.
RESULTS Thirty-three APS patients after IPAA were identified. Before treatment, 16 patients (48.4%) suffered from abdominal pain and 3 (9.1%) from bloody stools. Mean number of daily bowel movement was 10.3. Only 4 patients (12.1%) had a PDAI ≥ 7. Mean baseline PDAI was 2.5 ± 2.3. Overall, intervention was associated with symptomatic relief, mainly decreasing abdominal pain (from 48.4% to 27.2% of patients, P = 0.016). Daily bowel movements decreased from a mean of 10.3 to 9.3 (P = 0.003). Mean overall and clinical PDAI scores decreased from 2.58 to 1.94 (P = 0.016) and from 1.3 to 0.87 (P = 0.004), respectively. Analyzing each treatment modality separately, we observed that dietary modifications decreased abdominal pain (from 41.9% of patients to 19.35%, P = 0.016), daily bowel movements (from 10.5 to 9.3, P = 0.003), overall PDAI (from 2.46 to 2.03, P = 0.04) and clinical PDAI (1.33 to 0.86, P = 0.004). Probiotics effectively decreased daily bowel movements (from 10.2 to 8.8, P = 0.007), overall and clinical PDAI (from 2.9 to 2.1 and from 1.38 to 0.8, P = 0.032 and 0.01, respectively). While other therapies had minimal or no effects. No significant changes in endoscopic or histologic scores were seen with any therapy.
CONCLUSION APS patients benefit from dietary modifications and probiotics that improve their pouch-related symptoms but respond minimally to anti-inflammatory and antibiotic treatments. These results suggest a functional rather than inflammatory disorder.
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Affiliation(s)
- Ophir Gilad
- Department of Gastroenterology and Hepatology, Tel-Aviv Sourasky Medical Center, Tel Aviv 6423906, Israel
| | - Guy Rosner
- Department of Gastroenterology and Hepatology, Tel-Aviv Sourasky Medical Center, Tel Aviv 6423906, Israel
| | - Eli Brazowski
- Department of Pathology, Tel-Aviv Sourasky Medical Center, Tel Aviv 6423906, Israel
| | - Revital Kariv
- Department of Gastroenterology and Hepatology, Tel-Aviv Sourasky Medical Center, Tel Aviv 6423906, Israel
| | - Nathan Gluck
- Department of Gastroenterology and Hepatology, Tel-Aviv Sourasky Medical Center, Tel Aviv 6423906, Israel
| | - Hana Strul
- Department of Gastroenterology and Hepatology, Tel-Aviv Sourasky Medical Center, Tel Aviv 6423906, Israel
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10
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Incidence and Management of Rectal Cuff and Anal Transitional Zone Neoplasia in Patients With Familial Adenomatous Polyposis. Dis Colon Rectum 2021; 64:977-985. [PMID: 33960328 DOI: 10.1097/dcr.0000000000001967] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND Rectal cuff and anal transitional zone neoplasia is an increasing challenge in patients with familial adenomatous polyposis who have undergone restorative proctocolectomy. Its real incidence, range of severity, and treatment efficacy are poorly documented. OBJECTIVE We sought to document the evolution of rectal cuff and anal transitional zone neoplasia and describe its management. DESIGN This is a retrospective cohort study collecting prospectively recorded data. SETTINGS This study involved a hereditary colorectal cancer group in a large academic medical center. PATIENTS All patients undergoing primary restorative proctocolectomy at this institution were included. INTERVENTIONS Surveillance pouchoscopy and treatment of rectal cuff/anal transitional zone neoplasia were performed. MAIN OUTCOME MEASURES The primary outcomes measured were the presence and the severity of rectal cuff/anal transitional zone neoplasia. Excision by cautery, snare, mucosectomy, or redo pouch was evaluated. RESULTS A total of 165 patients were included: 52% were male (86/165) with a median age at restorative proctocolectomy of 31.0 years (SD 12.8). In 117 of 165, the proctocolectomy was their first operation and, in 48 of 165, it followed a colectomy. Of the patients, 83% (137/165) had stapled anastomosis; 17% had mucosectomy with handsewn anastomosis; and 14% (23/165) were treated with sulindac at some point during their surveillance. Median follow-up was 10.1 years (interquartile range, 4.5-17.2) and the median number of pouchoscopies per patient was 4 (interquartile range, 2-8). Seventy-eight of 165 (47.3%) developed rectal cuff/anal transitional zone adenomas, more in the stapled group (52.3%; 72/137) than in the handsewn group (21.4%; 6/28; p < 0.005). Median time to adenoma was 4.5 years (interquartile range, 2.4-8.9). Three patients developed cancer under surveillance, and, in 3 other patients, cancers developed when screenings lapsed. Five patients developed symptomatic anal stenosis secondary to repeated surgeries (median, 9 procedures; range, 2-10). LIMITATIONS There was no quality-of-life measurement in patients who developed rectal cuff/anal transitional zone neoplasia. CONCLUSIONS Rectal cuff/anal transitional zone adenomas are more common than previously reported. Mild polyposis can be controlled endoscopically, but repeated procedures in a higher stage are associated with risk of anal stenosis. Compliance with surveillance is essential to avoid cancer. See Video Abstract at http://links.lww.com/DCR/B594. INCIDENCIA Y TRATAMIENTO DE LA NEOPLASIA DEL REMANENTE RECTAL Y DE LA ZONA DE TRANSICIN ANAL EN PACIENTES CON POLIPOSIS ADENOMATOSA FAMILIAR ANTECEDENTES:La neoplasia del remanente rectal y de la zona de transición anal presenta un desafío mayor en pacientes con poliposis adenomatosa familiar tratados con una proctocolectomía restaurativa. Su incidencia real, el espectro de la gravedad y la eficacia del tratamiento están mal documentados.OBJETIVO:Buscamos documentar la evolución de la neoplasia del remanente rectal y de la zona de transición anal y describir su tratamiento.DISEÑO:Estudio de cohorte retrospectivo que recabó datos registrados prospectivamente.AMBITO:Un grupo con cáncer colorrectal hereditario de un importante centro médico académico.PACIENTES:Todos los pacientes operados por primera vez de proctocolectomía restaurativa en nuestra institución.INTERVENCIONES:Endoscopía del pouch para vigilancia y tratamiento de la neoplasia del remanente rectal / zona de transición anal.PRINCIPALES VARIABLES ANALIZADAS:La presencia y la gravedad de la neoplasia del remanente rectal / zona de transición anal. Resección con cauterio, asa, mucosectomía o rehacer el pouch.RESULTADOS:Se incluyeron un total de 165 pacientes: 52% eran hombres (86/165) con una mediana de edad al momento de la proctocolectomía restaurativa de 31,0 años (DE 12,8). En 117/165 la proctocolectomía fue su primera cirugía y en 48/165 fue posterior a una colectomía. En 83% (137/165) tenía una anastomosis con engrapadora, 17% tenía mucosectomía y anastomosis con sutura manual (HS). El 14% de los pacientes (23/165) fueron tratados con sulindac en algún momento durante su vigilancia. La mediana de seguimiento fue de 10,1 años (IQR: 4,5, 17,2) y la mediana del número de endoscopías del pouch por paciente fue de 4. (IQR: 2, 8) 78/165 (47,3%) desarrollaron adenomas en la zona de transición anal /remanente rectal, mayor en el grupo con engrapadaora (52,3%; 72/137) comparado con el grupo con sutura manual (21,4%; 6/28) (p <0,005). La mediana del tiempo hasta el adenoma fue de 4,5 años (IQR: 2,4, 8,9). Tres pacientes que se encontraban en vigilancia desarrollaron cáncer y en 3, otros cánceres se desarrollaron transcurrida la vigilancia. 5 pacientes desarrollaron estenosis anal sintomática secundaria a múltiples cirugías (mediana de 9 procedimientos; rango 2-10).LIMITACIONES:Falta de medición de la calidad de vida en pacientes que desarrollaron neoplasia del remanente rectal / zona de transición anal.CONCLUSIONES:Los adenomas de la zona de transición anal / remanente rectal son más comunes de lo reportado anteriormente. La poliposis leve se puede tratar por endoscopía, pero procedimientos repetidos en estadíos mayores se asocian con el riesgo de estenosis anal. El apego a la vigilancia es fundamental para evitar el cáncer. Consulte Video Resumen en http://links.lww.com/DCR/B594. (Traducción-Dr. Lisbeth Alarcon-Bernes).
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11
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Determinants of Pouch-Related Symptoms, a Common Outcome of Patients With Adenomatous Polyposis Undergoing Ileoanal Pouch Surgery. Clin Transl Gastroenterol 2021; 11:e00245. [PMID: 33031194 PMCID: PMC7544178 DOI: 10.14309/ctg.0000000000000245] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Total proctocolectomy with ileal pouch anal anastomosis (IPAA) is performed in patients with adenomatous polyposis syndromes (APSs). Data regarding pouch outcomes in APS are scarce. The purposes of this study were to determine the prevalence of pouch-related symptoms in patients with APS and to identify the contributing factors.
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12
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Japanese Society for Cancer of the Colon and Rectum (JSCCR) guidelines 2020 for the Clinical Practice of Hereditary Colorectal Cancer. Int J Clin Oncol 2021; 26:1353-1419. [PMID: 34185173 PMCID: PMC8286959 DOI: 10.1007/s10147-021-01881-4] [Citation(s) in RCA: 95] [Impact Index Per Article: 23.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/23/2020] [Accepted: 01/10/2021] [Indexed: 12/14/2022]
Abstract
Hereditary colorectal cancer (HCRC) accounts for < 5% of all colorectal cancer cases. Some of the unique characteristics commonly encountered in HCRC cases include early age of onset, synchronous/metachronous cancer occurrence, and multiple cancers in other organs. These characteristics necessitate different management approaches, including diagnosis, treatment or surveillance, from sporadic colorectal cancer management. There are two representative HCRC, named familial adenomatous polyposis and Lynch syndrome. Other than these two HCRC syndromes, related disorders have also been reported. Several guidelines for hereditary disorders have already been published worldwide. In Japan, the first guideline for HCRC was prepared by the Japanese Society for Cancer of the Colon and Rectum (JSCCR), published in 2012 and revised in 2016. This revised version of the guideline was immediately translated into English and published in 2017. Since then, several new findings and novel disease concepts related to HCRC have been discovered. The currently diagnosed HCRC rate in daily clinical practice is relatively low; however, this is predicted to increase in the era of cancer genomic medicine, with the advancement of cancer multi-gene panel testing or whole genome testing, among others. Under these circumstances, the JSCCR guidelines 2020 for HCRC were prepared by consensus among members of the JSCCR HCRC 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 2020 for HCRC.
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13
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Koskenvuo L, Ryynänen H, Lepistö A. Timing of prophylactic colectomy in familial adenomatous polyposis. Colorectal Dis 2020; 22:1553-1559. [PMID: 32441460 DOI: 10.1111/codi.15151] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/03/2020] [Accepted: 04/26/2020] [Indexed: 12/28/2022]
Abstract
AIM The aim was to evaluate the timing of prophylactic colectomy in patients with familial adenomatous polyposis (FAP) in Finland. METHOD All Finnish FAP patients were included from the years 1963-2018. Among the 452 FAP patients studied, 252 were called up as relatives of the proband. Of these, 180 underwent prophylactic colectomy. Patient data including mutation characteristics and surgical technique were collected. We assessed the timing of prophylactic colectomy in relation to the time of diagnosis, time of decision for surgery and age. RESULTS There were 94 (52%) colectomies with ileorectal anastomosis, 79 (44%) proctocolectomies with ileal pouch-anal anastomosis and seven (4%) proctocolectomies with an ileostomy. Median age at diagnosis and at the time of surgery was 20.9 and 25.2 years, respectively. The median time from decision to operate to undergoing surgery was 4 months. There were eight (4%) cancers in the colectomy specimens, but none was found during the annual follow-ups after the first-round endoscopy. After genetic testing became available, the diagnosis was made primarily by either genetic testing in 52 (49%) or endoscopy in 54 (51%) patients at a median age of 17.8 and 19.3 years, respectively. Thirty-eight (21%) patients died during follow-up, 23 (61%) for FAP-related reasons and only six from rectal cancer. The relative survival of 30 years from diagnosis was 84% and 78% in men and women, respectively. CONCLUSIONS For those who underwent surgery, timing of prophylactic surgery was successful as there were no unexpected colorectal cancers found in colectomy specimens among the screened patients. Two major improvements over the study period have changed the treatment of FAP patients: one is the introduction of the ileal pouch-anal anastomosis procedure and the other is the availability of genetic testing. FAP-related extra-colonic malignancies affect the relative survival of these patients after prophylactic surgery.
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Affiliation(s)
- L Koskenvuo
- Department of Gastroenterological Surgery, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
| | - H Ryynänen
- Finnish Cancer Registry, Institute for Statistical and Epidemiological Cancer Research, Helsinki, Finland
| | - A Lepistö
- Department of Gastroenterological Surgery, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
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14
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Anele CC, Xiang J, Martin I, Hawkins M, Clark SK, Faiz OD, Latchford A, Hyer W. Polyp Progression in Paediatric Patients With Familial Adenomatous Polyposis: A Single-centre Experience. J Pediatr Gastroenterol Nutr 2020; 71:612-616. [PMID: 33093367 DOI: 10.1097/mpg.0000000000002845] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
OBJECTIVES Prophylactic colectomy at a premalignant stage is the cornerstone of management of familial adenomatous polyposis (FAP). Before surgery, colonoscopy surveillance is recommended in children with FAP. This study aimed to examine the natural history of FAP in children by evaluating adenoma progression and factors influencing timing of colectomy. METHOD Patients with FAP younger than 18 years at first surveillance colonoscopy and who had undergone more than 1 colonoscopy were identified. Demographic, endoscopic, genetic, and surgical data were retrieved. Cumulative adenoma (polyp) counts were obtained while accounting for any polypectomies during the study period. The rate of polyp progression and factors influencing the timing of colectomy were evaluated. RESULTS Eighty-four patients (50% boys; mean age at first colonoscopy 13 years [standard deviation 1.97]) were identified, of which 83 had a family history of FAP. At first colonoscopy, 67 (79%) had <100 adenomas and 29 (35%) had colonic polyps identified despite rectal sparing. The median rate of polyp progression per patient was 12.5 polyps/year (range 0-145). Of the 45 (54%) patients who had undergone surgery, 41 (91%) underwent colectomy with ileorectal or ileodistal sigmoid anastomosis. Polyp progression did not alter the choice of surgical intervention in any patient. CONCLUSION Our results suggest that adenoma number remains relatively stable in the majority of children under surveillance. Tailored surveillance intervals according to phenotype are a more appropriate strategy as recommended by recently published guidelines.
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Affiliation(s)
- Chukwuemeka C Anele
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow.,Department of Surgery and Cancer.,Surgical Epidemiology, Trials and Outcome Centre (SETOC)
| | - Jinpo Xiang
- Department of Medicine, Imperial College London, London
| | - Isabel Martin
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow.,Department of Surgery and Cancer, Imperial College London, London, UK
| | - Menna Hawkins
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow
| | - Susan K Clark
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow.,Department of Surgery and Cancer, Imperial College London, London, UK
| | - Omar D Faiz
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow.,Surgical Epidemiology, Trials and Outcome Centre (SETOC).,Department of Surgery and Cancer, Imperial College London, London, UK
| | - Andrew Latchford
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow.,Department of Surgery and Cancer, Imperial College London, London, UK
| | - Warren Hyer
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow
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15
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Anele CC, Nachiappan S, Sinha A, Cuthill V, Jenkins JT, Clark SK, Latchford A, Faiz OD. Safety and efficacy of laparoscopic near-total colectomy and ileo-distal sigmoid anastomosis as a modification of total colectomy and ileorectal anastomosis for prophylactic surgery in patients with adenomatous polyposis syndromes: a comparative study. Colorectal Dis 2020; 22:799-805. [PMID: 31943692 DOI: 10.1111/codi.14964] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/07/2019] [Accepted: 12/15/2019] [Indexed: 02/08/2023]
Abstract
AIM Colectomy in patients with adenomatous polyposis (AP) syndromes demands good oncological and surgical outcome. Total colectomy with ileorectal anastomosis (TC-IRA) is one surgical option for these patients. Anastomotic leakage rates of 11% have been reported following TC-IRA. Ileo-distal sigmoid anastomosis (IDSA) is a recent modification of our practice. Our aim was to compare postoperative outcome in patients with AP following near-total colectomy with IDSA (NT-IDSA) and TC-IRA at a single institution. METHOD A prospectively maintained database was reviewed to identify patients with AP who underwent laparoscopic NT-IDSA and TC-IRA. Patient demographics, early morbidity and mortality and outcome of endoscopic surveillance were evaluated. RESULTS A total of 191 patients with AP underwent laparoscopic colectomy between 2006 and 2017, of whom 139 (72.8%) underwent TC-IRA and 52 (27.2%) NT-IDSA. The median age at surgery in the TC-IRA and NT-IDSA groups was 20 years (IQR 17-45) and 27 years (IQR 19-50), respectively. Grade II complications were comparable between the two groups. There were no anastomotic leakages in the NT-IDSA group compared with 15 (10.8%) in the TC-IRA group (P = 0.0125) and no reoperation in the NT-IDSA group compared with 17 (12.2%) in the TC-IRA group (P = 0.008). The frequency of polypectomies per flexible sigmoidoscopy was comparable between the two groups. CONCLUSION This study demonstrates that laparoscopic NT-IDSA for polyposis is associated with a significant improvement in anastomotic leakage rates and surgical outcome. It is too soon to tell whether NT-IDSA alters the need for further intervention, either endoscopic polypectomy or further surgery.
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Affiliation(s)
- C C Anele
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK.,Surgical Epidemiology, Trials and Outcome Centre (SETOC), St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK.,Department of Surgery and Cancer, Imperial College London, London, UK
| | - S Nachiappan
- Surgical Epidemiology, Trials and Outcome Centre (SETOC), St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK.,Department of Surgery and Cancer, Imperial College London, London, UK
| | - A Sinha
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK.,Department of Surgery and Cancer, Imperial College London, London, UK
| | - V Cuthill
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK
| | - J T Jenkins
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK.,Department of Surgery and Cancer, Imperial College London, London, UK
| | - S K Clark
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK.,Department of Surgery and Cancer, Imperial College London, London, UK
| | - A Latchford
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK.,Department of Surgery and Cancer, Imperial College London, London, UK
| | - O D Faiz
- The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK.,Surgical Epidemiology, Trials and Outcome Centre (SETOC), St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow, UK.,Department of Surgery and Cancer, Imperial College London, London, UK
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16
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Vitellaro M, Piozzi G, Signoroni S, Ricci MT, Ciniselli CM, Cardani A, Vecchi I, Mancini A, Magarotto A, Verderio P, Massimino M, Ferrari A, Biasoni D. Short-term and long-term outcomes after preventive surgery in adolescent patients with familial adenomatous polyposis. Pediatr Blood Cancer 2020; 67:e28110. [PMID: 31802619 DOI: 10.1002/pbc.28110] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/27/2019] [Revised: 11/14/2019] [Accepted: 11/16/2019] [Indexed: 01/24/2023]
Abstract
BACKGROUND APC gene pathogenic variants are characterized by a lifetime risk of nearly 100% to develop a colorectal carcinoma. International guidelines suggest a prophylactic surgery in the second decade. METHODS A descriptive analysis was performed evaluating a surgical series of adolescent patients with familial adenomatous polyposis (FAP) enrolled in the prospectively maintained hereditary polyposis registry. RESULTS Thirty-eight adolescent patients (median age 16 years; range, 7-19) underwent laparoscopic prophylactic surgery. APC gene pathogenic variants were detected in all patients, and six patients were proband. No patients were converted to open surgery. Median postoperative stay was five days (4-16). Early postoperative complications were one dural puncture and one anastomotic leakage. Regarding late complications, we observed one patient having small bowel obstruction 56 months after surgery. Pathological reports showed one patient with pTis adenocarcinoma in two separate sites; 33 patients with low-grade dysplasia, four with high-grade dysplasia. One patient developed a desmoid tumor 37 months after surgery. After a median follow-up of 40.5 months, no patients died or had a second abdominal surgery because of cancer in rectal stump. CONCLUSIONS Rectal sparing surgery was the first choice in the major respect of patients' quality of life. Laparoscopic prophylactic surgery for FAP is well accepted from adolescents. It represents a safe option due to the low incidence of post-surgical desmoids and quick postoperative recovery.
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Affiliation(s)
- Marco Vitellaro
- Hereditary Digestive Tract Tumors Unit, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.,Colorectal Surgery Section, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Guglielmo Piozzi
- Colorectal Surgery Section, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Stefano Signoroni
- Hereditary Digestive Tract Tumors Unit, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Maria Teresa Ricci
- Hereditary Digestive Tract Tumors Unit, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Chiara Maura Ciniselli
- Bioinformatics and Biostatistics Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Anna Cardani
- Department of Critical and Supportive Therapy, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Irene Vecchi
- Department of Critical and Supportive Therapy, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Andrea Mancini
- Diagnostic and Therapeutic Endoscopy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Andrea Magarotto
- Diagnostic and Therapeutic Endoscopy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Paolo Verderio
- Bioinformatics and Biostatistics Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Maura Massimino
- Pediatric Oncology Section, Department of Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Andrea Ferrari
- Pediatric Oncology Section, Department of Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Davide Biasoni
- Pediatric Surgery Unit, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
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17
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Abstract
Familial adenomatous polyposis (FAP) is an autosomal dominant genetic disease with a high tendency to develop colorectal cancer. The timing and choice of preventive surgical interventions should be based on the number, size and severity of adenomas, combined with a variety of considerations, in order to effectively prevent cancer and make patients easy to accept. Total proctocolectomy and ileal pouch-anal anastomosis (IPAA) procedure, which could minimize the residual rectal mucosa and reduce the risk of adenoma recurrence, has become the first choice for patients with FAP. Besides, laparoscopic IPAA has obvious advantages such as cosmetic appearance, quick recovery, little adhesion and high pregnancy rate. Patients with FAP should be managed by experienced surgeons working in specialized medical centers in order to get the most reasonable treatment at the best time and achieve long-term effective outcomes.
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Affiliation(s)
- Kai-Yu Li
- Department of General Surgery, Tianjin Medical University General Hospital, Tianjin 300052, China
| | - Gang Liu
- Department of General Surgery, Tianjin Medical University General Hospital, Tianjin 300052, China
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18
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A Decision Analysis for Rectal-Sparing Familial Adenomatous Polyposis: Total Colectomy With Ileorectal Anastomosis Versus Proctocolectomy With IPAA. Dis Colon Rectum 2019; 62:27-32. [PMID: 30394986 DOI: 10.1097/dcr.0000000000001186] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
BACKGROUND There are different approaches for the surgical management of rectal-sparing familial adenomatous polyposis with variable impacts on both quality of life and survival. OBJECTIVE The aim of this study was to quantify the trade-offs between total proctocolectomy with IPAA versus total colectomy with ileorectal anastomosis using decision analysis. DESIGN We created a disease simulation Markov model to simulate the clinical events after IPAA and ileorectal anastomosis for rectal-sparing familial adenomatous polyposis in a cohort of individuals at the age 30 years. We used available literature to obtain different transition probabilities and health-states utilities. The output parameters were quality-adjusted life-years and life-years. Deterministic and probabilistic sensitivity analyses were performed. SETTINGS A decision analysis using a Markov model was conducted at a single center. PATIENTS Patients with rectal-sparing familial adenomatous polyposis at age 30 years were included. Rectal-sparing familial adenomatous polyposis is defined as the presence of 0 to 20 polyps that can be removed endoscopically. MAIN OUTCOME MEASURES Quality-adjusted life-years were measured. RESULTS Our model showed that the mean quality-adjusted life-years for IPAA was 25.12 and for ileorectal anastomosis was 27.12 in base-case analysis. Mean life-years for IPAA were 28.81 and 28.28 for ileorectal anastomosis. A 1-way sensitivity analysis was performed for all of the parameters in the model. None of the deterministic sensitivity analyses changed the model results across the range of plausible values. Probabilistic analysis identified that, in 86.9% of scenarios, ileorectal anastomosis had improved quality-adjusted life-years compared with IPAA. LIMITATIONS The study was limited by characteristics inherent to modeling studies. CONCLUSIONS Ileorectal anastomosis was found to be preferable for patients with rectal-sparing familial adenomatous polyposis when quality of life is taken into consideration. This model was robust based on both deterministic and probabilistic sensitivity analyses. These data should be taken into consideration when counseling patients regarding a surgical approach in rectal-sparing familial adenomatous polyposis. See Video Abstract at http://links.lww.com/DCR/A715.
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Sarvepalli S, Burke CA, Monachese M, Leach BH, Laguardia L, O'Malley M, Kalady MF, Church JM. Natural history of colonic polyposis in young patients with familial adenomatous polyposis. Gastrointest Endosc 2018; 88:726-733. [PMID: 29864420 DOI: 10.1016/j.gie.2018.05.021] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/18/2018] [Accepted: 05/26/2018] [Indexed: 12/11/2022]
Abstract
BACKGROUND AND AIMS Proctocolectomy prevents colorectal cancer in familial adenomatous polyposis (FAP). Colorectal polyp progression is one of the indications for surgery. No data exist regarding the natural history of colorectal polyposis in young patients with FAP. This study examined the rate of polyposis progression and factors associated with it. METHODS Patients with FAP <30 years old who had undergone ≥2 colonoscopies since 2000 were identified. Rate of polyposis progression was calculated by review of polyp counts obtained from baseline and last colonoscopy, accounting for any polyps removed during the observation period. Endoscopic and non-endoscopic factors affecting the rate of polyposis progression were evaluated. Multivariate analysis was performed to identify factors associated with rate of polyposis progression. RESULTS One hundred sixty-eight patients (52% female; median age, 13.5 years) were included. Median rate of polyposis progression was 25.4 polyps/year (interquartile range, 9.5-69.8). Highest median rate of polyposis progression (89 polyps/year) was associated with mutation in codon 1309. The rate of polyposis progression was independently associated with the location of mutation in the adenomatous polyposis coli gene, the number of polyps at the initial colonoscopy, and exposure to chemoprevention. Of the 39.9% of patients who underwent surgery, an increase in polyp number was the most common indication (53.7%). CONCLUSIONS The rate of polyposis progression in young patients with FAP varies with a median of about 25 new polyps per year. Progression is associated with distinct factors, which can be used in discussion with patients regarding the need for and timing of prophylactic colorectal surgery.
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Affiliation(s)
- Shashank Sarvepalli
- Department of Hospital Medicine, Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Carol A Burke
- Department of Gastroenterology and Hepatology, Digestive Disease and Surgical Institute, Cleveland Clinic, Cleveland, Ohio, USA; Department of Colorectal Surgery; Digestive Disease and Surgical Institute, Cleveland Clinic, Cleveland, Ohio, USA; Sanford R. Weiss, MD, Center for Hereditary Colorectal Neoplasia, Cleveland Clinic, Cleveland, Ohio, USA
| | - Marc Monachese
- Department of Internal Medicine, Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Brandie H Leach
- Department of Colorectal Surgery; Digestive Disease and Surgical Institute, Cleveland Clinic, Cleveland, Ohio, USA; Sanford R. Weiss, MD, Center for Hereditary Colorectal Neoplasia, Cleveland Clinic, Cleveland, Ohio, USA; Genomic Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Lisa Laguardia
- Sanford R. Weiss, MD, Center for Hereditary Colorectal Neoplasia, Cleveland Clinic, Cleveland, Ohio, USA
| | - Margaret O'Malley
- Sanford R. Weiss, MD, Center for Hereditary Colorectal Neoplasia, Cleveland Clinic, Cleveland, Ohio, USA
| | - Matthew F Kalady
- Department of Colorectal Surgery; Digestive Disease and Surgical Institute, Cleveland Clinic, Cleveland, Ohio, USA; Sanford R. Weiss, MD, Center for Hereditary Colorectal Neoplasia, Cleveland Clinic, Cleveland, Ohio, USA
| | - James M Church
- Department of Colorectal Surgery; Digestive Disease and Surgical Institute, Cleveland Clinic, Cleveland, Ohio, USA; Sanford R. Weiss, MD, Center for Hereditary Colorectal Neoplasia, Cleveland Clinic, Cleveland, Ohio, 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: 32] [Impact Index Per Article: 4.6] [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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Remzi FH, Lavryk OA, Ashburn JH, Hull TL, Lavery IC, Dietz DW, Kessler H, Church JM. Restorative proctocolectomy: an example of how surgery evolves in response to paradigm shifts in care. Colorectal Dis 2017; 19:1003-1012. [PMID: 28481467 DOI: 10.1111/codi.13699] [Citation(s) in RCA: 40] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2016] [Accepted: 02/13/2017] [Indexed: 12/12/2022]
Abstract
AIM Surgical technique constantly evolves in response to the pressure of progress. Ileal pouch anal anastomosis (IPAA) is a good example. We analysed the effect of changes in practice on the technique of IPAA and its outcomes. METHOD Patients undergoing primary IPAA at this institution were divided into three groups by date of the IPAA: those operated from 1983 to 1993, from 1994 to 2004 and from 2005 to 2015. Demographics, patient comorbidity, surgical techniques, postoperative outcomes, pouch function and quality of life were analysed. RESULTS In all, 4525 patients had a primary IPAA. With each decade, increasing numbers of surgeons were involved (decade I, 8; II, 16; III, 31), patients tended to be sicker (higher American Society of Anesthesiologists score) and three-staged pouches became more common. After an initial popularity of the S pouch, J pouches became dominant and a mucosectomy rate of 12% was standard. The laparoscopic technique blossomed in the last decade. 90-day postoperative morbidity by decade was 38.3% vs 50% vs 48% (P < 0.0001), but late morbidity decreased from 74.2% through 67.1% to 30% (P < 0.0001). Functional results improved, but quality of life scores did not. Pouch survival rate at 10 years was maintained (94% vs 95.2% vs 95.2%; P = 0.06). CONCLUSION IPAA is still evolving. Despite new generations of surgeons, a more accurate diagnosis, appropriate staging and the laparoscopic technique have made IPAA a safer, more effective and enduring operation.
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Affiliation(s)
- F H Remzi
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute Cleveland, Cleveland Clinic, Cleveland, Ohio, USA
| | - O A Lavryk
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute Cleveland, Cleveland Clinic, Cleveland, Ohio, USA
| | - J H Ashburn
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute Cleveland, Cleveland Clinic, Cleveland, Ohio, USA
| | - T L Hull
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute Cleveland, Cleveland Clinic, Cleveland, Ohio, USA
| | - I C Lavery
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute Cleveland, Cleveland Clinic, Cleveland, Ohio, USA
| | - D W Dietz
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute Cleveland, Cleveland Clinic, Cleveland, Ohio, USA
| | - H Kessler
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute Cleveland, Cleveland Clinic, Cleveland, Ohio, USA
| | - J M Church
- Department of Colorectal Surgery, Digestive Disease and Surgery Institute Cleveland, Cleveland Clinic, Cleveland, Ohio, USA
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HEREDITARY COLORECTAL CANCER REGISTRY: A CLEVELAND CLINIC FOUNDATION EXPERIENCE. REVISTA MÉDICA CLÍNICA LAS CONDES 2017. [DOI: 10.1016/j.rmclc.2017.06.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
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J C, M O, L L, D C, X X, H H, L A, G D, B J, K H, B L, J M, C B, M K. REGISTRO DE CÁNCER COLORRECTAL HEREDITARIO: UNA EXPERIENCIA DE “CLEVELAND CLINIC FOUNDATION”. REVISTA MÉDICA CLÍNICA LAS CONDES 2017. [DOI: 10.1016/j.rmclc.2017.07.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022] Open
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Chittleborough TJ, Warrier SK, Heriot AG, Kalady M, Church J. Dispelling misconceptions in the management of familial adenomatous polyposis. ANZ J Surg 2017; 87:441-445. [PMID: 28266097 DOI: 10.1111/ans.13919] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2016] [Revised: 12/18/2016] [Accepted: 12/26/2016] [Indexed: 12/15/2022]
Abstract
Patients with familial adenomatous polyposis require surgical intervention at some point in their lives. The diagnosis is often apparent from their phenotype and family history, however, this is not always the case. Many factors can influence the surgical strategy although the polyposis burden and distribution remain the main consideration. While prophylactic removal of the rectum and colon is often required, sparing the rectum at the index surgery is safe in select patients. This article aims to dispel misconceptions in the diagnosis and treatment of patients with familial adenomatous polyposis.
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Affiliation(s)
- Timothy J Chittleborough
- Department of Cancer Surgery, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.,Department of Surgery, The University of Melbourne, Melbourne, Victoria, Australia
| | - Satish K Warrier
- Department of Cancer Surgery, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.,Department of Colorectal Surgery, Alfred Health, Melbourne, Victoria, Australia
| | - Alexander G Heriot
- Department of Cancer Surgery, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.,Department of Surgery, The University of Melbourne, Melbourne, Victoria, Australia
| | - Matthew Kalady
- Department of Colorectal Surgery, Digestive Diseases and Surgery Institute, Cleveland Clinic, Cleveland, Ohio, USA.,Sanford R. Weiss, MD Center for Hereditary Colorectal Neoplasia, Cleveland Clinic, Cleveland, Ohio, USA
| | - James Church
- Department of Colorectal Surgery, Digestive Diseases and Surgery Institute, Cleveland Clinic, Cleveland, Ohio, USA.,Sanford R. Weiss, MD Center for Hereditary Colorectal Neoplasia, Cleveland Clinic, Cleveland, Ohio, USA
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Ueno H, Kobayashi H, Konishi T, Ishida F, Yamaguchi T, Hinoi T, Kanemitsu Y, Inoue Y, Tomita N, Matsubara N, Komori K, Ozawa H, Nagasaka T, Hasegawa H, Koyama M, Akagi Y, Yatsuoka T, Kumamoto K, Kurachi K, Tanakaya K, Yoshimatsu K, Watanabe T, Sugihara K, Ishida H. Prevalence of laparoscopic surgical treatment and its clinical outcomes in patients with familial adenomatous polyposis in Japan. Int J Clin Oncol 2016; 21:713-722. [PMID: 26820718 DOI: 10.1007/s10147-016-0953-5] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/04/2015] [Accepted: 01/07/2016] [Indexed: 01/01/2023]
Abstract
BACKGROUND Laparoscopic surgery is becoming the preferred technique for most colorectal interventions. This study aimed to clarify the time trend of surgical treatment for familial adenomatous polyposis (FAP) and its relevance to clinical outcomes in Japan over a 13-year period. METHODS This was a multicenter retrospective cohort study comprising 23 specialist institutions for colorectal disease and a cohort of 282 FAP patients who underwent total colectomy or proctocolectomy during 2000-2012. Patient clinical backgrounds and surgical outcomes were compared between the first and second halves of the study period. RESULTS The proportion of surgical types adopted over the entire study period was 46, 21, 30, and 3 % for ileoanal anastomosis (IAA), ileoanal canal anastomosis, ileorectal anastomosis, and permanent ileostomy, respectively. FAP patients undergoing laparoscopic surgery have increased since 2008 and reached 74 % in the past 3 years. In particular, the number of patients undergoing laparoscopic proctocolectomy with IAA increased approximately four-fold from the first to the second half of the study period. A laparoscopic approach was increasingly used in patients with coexisting colorectal malignancies. Despite this trend, surgical results of the laparoscopic approach between the two study periods showed similar morbidity, pouch operation and stoma closure completion rates. No postoperative mortality was observed in this series, and laparoscopic surgery was comparable to open surgery in terms of stoma closure rate, incidence of intra-abdominal/abdominal desmoid tumors, and postoperative survival rate in both study periods. CONCLUSION Laparoscopic approach is increasingly being adopted for prophylactic FAP surgery in Japan and may provide clinically acceptable practical outcomes.
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Affiliation(s)
- Hideki Ueno
- Department of Surgery, National Defense Medical College, Tokorozawa, Saitama, 359-8513, Japan.
| | - Hirotoshi Kobayashi
- Center for Minimally Invasive Surgery, Tokyo Medical and Dental University, Tokyo, Japan
| | - Tsuyoshi Konishi
- Gastroenterological Center, Department of Gastroenterological Surgery, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan
| | - Fumio Ishida
- Digestive Disease Center, Showa University Northern Yokohama Hospital, Yokohama, Japan
| | - Tatsuro Yamaguchi
- Department of Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan
| | - Takao Hinoi
- Department of Gastroenterological and Transplant Surgery, Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Yukihide Kanemitsu
- Division of Colorectal Surgery, National Cancer Center Hospital, Tokyo, Japan
| | - Yasuhiro Inoue
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, Tsu, Japan
| | - Naohiro Tomita
- Department of Surgery, Hyogo College of Medicine, Nishinomiya, Japan
| | | | - Koji Komori
- Department of Gastroenterological Surgery, Aichi Cancer Center Hospital, Nagoya, Japan
| | - Heita Ozawa
- Department of Surgery, Tochigi Cancer Center, Utsunomiya, Japan
| | - Takeshi Nagasaka
- Department of Gastroenterological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan
| | | | - Motoi Koyama
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, Hirosaki, Japan
| | - Yoshito Akagi
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Toshimasa Yatsuoka
- Department of Gastroenterological Surgery, Saitama Cancer Center, Saitama, Japan
| | - Kensuke Kumamoto
- Department of Organ Regulatory Surgery, Fukushima Medical University School of Medicine, Fukushima, Japan
| | - Kiyotaka Kurachi
- Department of Surgery 2, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Kohji Tanakaya
- Department of Surgery, Iwakuni Clinical Center, Iwakuni, Japan
| | - Kazuhiko Yoshimatsu
- Department of Surgery, Tokyo Women's Medical University Medical Center East, Tokyo, Japan
| | - Toshiaki Watanabe
- Department of Surgical Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | | | - Hideyuki Ishida
- Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan
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A proposed staging system and stage-specific interventions for familial adenomatous polyposis. Gastrointest Endosc 2016; 84:115-125.e4. [PMID: 26769407 PMCID: PMC5570515 DOI: 10.1016/j.gie.2015.12.029] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/16/2015] [Accepted: 12/28/2015] [Indexed: 12/11/2022]
Abstract
BACKGROUND AND AIMS It is not possible to accurately count adenomas in many patients with familial adenomatous polyposis (FAP). Nevertheless, polyp counts are critical in evaluating each patient's response to interventions. However, the U.S. Food and Drug Administration no longer recognizes the decrease in polyp burden as a sufficient chemoprevention trial treatment endpoint requiring a measure of "clinical benefit." To develop endpoints for future industry-sponsored chemopreventive trials, the International Society for Gastrointestinal Hereditary Tumors (InSIGHT) developed an FAP staging and intervention classification scheme for lower-GI tract polyposis. METHODS Twenty-four colonoscopy or sigmoidoscopy videos were reviewed by 26 clinicians familiar with diagnosis and treatment of FAP. The reviewers independently assigned a stage to a case by using the proposed system and chose a stage-specific intervention for each case. Our endpoint was the degree of concordance among reviewers staging and intervention assessments. RESULTS The staging and intervention ratings of the 26 reviewers were highly concordant (ρ = 0.710; 95% credible interval, 0.651-0.759). Sixty-two percent of reviewers agreed on the FAP stage, and 90% of scores were within ±1 stage of the mode. Sixty percent of reviewers agreed on the intervention, and 86% chose an intervention within ±1 level of the mode. CONCLUSIONS The proposed FAP colon polyposis staging system and stage-specific intervention are based on a high degree of agreement on the part of experts in the review of individual cases of polyposis. Therefore, reliable and clinically relevant means for measuring trial outcomes can be developed. Outlier cases showing wide scatter in stage assignment call for individualized attention and may be inappropriate for enrollment in clinical trials for this reason.
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Advances in Hereditary Colorectal and Pancreatic Cancers. Clin Ther 2016; 38:1600-21. [PMID: 27045993 DOI: 10.1016/j.clinthera.2016.03.017] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2016] [Revised: 03/05/2016] [Accepted: 03/08/2016] [Indexed: 12/19/2022]
Abstract
PURPOSE Innovations in genetic medicine have led to improvements in the early detection, prevention, and treatment of cancer for patients with inherited risks of gastrointestinal cancer, particularly hereditary colorectal cancer and hereditary pancreatic cancer. METHODS This review provides an update on recent data and key advances that have improved the identification, understanding, and management of patients with hereditary colorectal cancer and hereditary pancreatic cancer. FINDINGS This review details recent and emerging data that highlight the developing landscape of genetics in hereditary colorectal and pancreatic cancer risk. A summary is provided of the current state-of-the-art practices for identifying, evaluating, and managing patients with suspected hereditary colorectal cancer and pancreatic cancer risk. The impact of next-generation sequencing technologies in the clinical diagnosis of hereditary gastrointestinal cancer and also in discovery efforts of new genes linked to familial cancer risk are discussed. Emerging targeted therapies that may play a particularly important role in the treatment of patients with hereditary forms of colorectal cancer and pancreatic cancer are also reviewed. Current approaches for pancreatic cancer screening and the psychosocial impact of such procedures are also detailed. IMPLICATIONS Given the availability of new diagnostic, risk-reducing, and therapeutic strategies that exist for patients with hereditary risk of colorectal or pancreatic cancer, it is imperative that clinicians be vigilant about evaluating patients for hereditary cancer syndromes. Continuing to advance genetics research in hereditary gastrointestinal cancers will allow for more progress to be made in personalized medicine and prevention.
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Koskenvuo L, Mustonen H, Renkonen-Sinisalo L, Järvinen HJ, Lepistö A. Comparison of proctocolectomy and ileal pouch-anal anastomosis to colectomy and ileorectal anastomosis in familial adenomatous polyposis. Fam Cancer 2016; 14:221-7. [PMID: 25504366 DOI: 10.1007/s10689-014-9773-9] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Abstract
Prophylactic surgical options for familial adenomatous polyposis (FAP) are either colectomy and ileorectal anastomosis (IRA) or proctocolectomy and ileal pouch-anal anastomosis (IPAA). The aim of this study was to analyse the short-term and long-term outcomes of these two operative techniques. All patients with FAP in Finland have been prospectively recorded in a database since 1963 were retrospectively reviewed in this analysis. Altogether 140 (61%) colectomies with IRA and 88 (39%) proctocolectomies with IPAA have been performed. Complications occurred in 28 (21%) patients after IRA and in 26 (30%) patients after IPAA. There were 15 (11%) severe complications for IRA and 5 (6%) for IPAA. Twenty-one (15%) patients of the IRA group ended up in conventional ileostomy whereas 3 (3.4%) patients of the IPAA group had their ileal reservoir converted to an ileostomy (p = 0.01). Cumulative survival for IRA was lower than for the IPAA (p = 0.03), but if accounting only for operations made after the IPAA era had commenced, there was no significant difference. IPAA was associated with improved long-term survival without an increase in postoperative complications. The risk of death after colectomy and IRA seemed to be predominantly related to the remaining risk of rectal cancer. Therefore, we favour proctocolectomy with IPAA as the prophylactic surgical procedure for FAP with intermediate or severe polyposis.
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Affiliation(s)
- L Koskenvuo
- Department of Gastrointestinal and General Surgery, Helsinki University Central Hospital, P.O. Box 340, 00029, HUS, Helsinki, Finland,
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ACG clinical guideline: Genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol 2015; 110:223-62; quiz 263. [PMID: 25645574 PMCID: PMC4695986 DOI: 10.1038/ajg.2014.435] [Citation(s) in RCA: 1082] [Impact Index Per Article: 108.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/01/2014] [Accepted: 12/01/2014] [Indexed: 02/06/2023]
Abstract
This guideline presents recommendations for the management of patients with hereditary gastrointestinal cancer syndromes. The initial assessment is the collection of a family history of cancers and premalignant gastrointestinal conditions and should provide enough information to develop a preliminary determination of the risk of a familial predisposition to cancer. Age at diagnosis and lineage (maternal and/or paternal) should be documented for all diagnoses, especially in first- and second-degree relatives. When indicated, genetic testing for a germline mutation should be done on the most informative candidate(s) identified through the family history evaluation and/or tumor analysis to confirm a diagnosis and allow for predictive testing of at-risk relatives. Genetic testing should be conducted in the context of pre- and post-test genetic counseling to ensure the patient's informed decision making. Patients who meet clinical criteria for a syndrome as well as those with identified pathogenic germline mutations should receive appropriate surveillance measures in order to minimize their overall risk of developing syndrome-specific cancers. This guideline specifically discusses genetic testing and management of Lynch syndrome, familial adenomatous polyposis (FAP), attenuated familial adenomatous polyposis (AFAP), MUTYH-associated polyposis (MAP), Peutz-Jeghers syndrome, juvenile polyposis syndrome, Cowden syndrome, serrated (hyperplastic) polyposis syndrome, hereditary pancreatic cancer, and hereditary gastric cancer.
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Campos FG. Surgical treatment of familial adenomatous polyposis: Dilemmas and current recommendations. World J Gastroenterol 2014; 20:16620-16629. [PMID: 25469031 PMCID: PMC4248206 DOI: 10.3748/wjg.v20.i44.16620] [Citation(s) in RCA: 58] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/20/2014] [Revised: 07/14/2014] [Accepted: 08/28/2014] [Indexed: 02/06/2023] Open
Abstract
Familial adenomatous polyposis (FAP) is an autosomal dominant inherited syndrome characterized by multiple adenomatous polyps (predisposing to colorectal cancer development) and numerous extracolonic manifestations. The underlying genetic burden generates variable clinical features that may influence operative management. As a precancerous hereditary condition, the rationale of performing a prophylactic surgery is a mainstay of FAP management. The purpose of the present paper is to bring up many controversial aspects regarding surgical treatment for FAP, and to discuss the results and perspectives of the operative choices and approaches. Preferably, the decision-making process should not be limited to the conventional confrontation of pros and cons of ileorectal anastomosis or restorative proctocolectomy. A wide discussion with the patient may evaluate issues such as age, genotype, family history, sphincter function, the presence or risk of desmoid disease, potential complications of each procedure and chances of postoperative surveillance. Therefore, the definition of the best moment and the choice of appropriate procedure constitute an individual decision that must take into consideration patient’s preferences and full information about the complex nature of the disease. All these facts reinforce the idea that FAP patients should be managed by experienced surgeons working in specialized centers to achieve the best immediate and long-term results.
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Vitellaro M, Sala P, Signoroni S, Radice P, Fortuzzi S, Civelli EM, Ballardini G, Kleiman DA, Morrissey KP, Bertario L. Risk of desmoid tumours after open and laparoscopic colectomy in patients with familial adenomatous polyposis. Br J Surg 2014; 101:558-65. [PMID: 24493089 DOI: 10.1002/bjs.9411] [Citation(s) in RCA: 50] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/25/2013] [Indexed: 02/06/2023]
Abstract
BACKGROUND Desmoid tumour (DT) is a main cause of death after prophylactic colectomy in patients with familial adenomatous polyposis (FAP). The purpose of this study was to evaluate the impact of prophylactic laparoscopic colectomy on the risk of developing DT in patients with FAP. METHODS The database of a single institution was reviewed. Patients with classical FAP with defined genotype who underwent either open or laparoscopic colectomy between 1947 and 2011 were included in the study. The impact of various demographic and clinical features on the risk of developing DT was assessed. RESULTS A total of 672 patients underwent prophylactic colectomy: 602 by an open and 70 by a laparoscopic approach. With a median (range) follow-up of 132 (0-516) months in the open group and 60 (12-108) months in the laparoscopic group, 98 patients (16·3 per cent) developed DT after an open procedure compared with three (4 per cent) following laparoscopic surgery. The estimated cumulative risk of developing DT at 5 years after surgery was 13·0 per cent in the open group and 4 per cent in the laparoscopic group (P = 0·042). In multivariable analysis, female sex (hazard ratio (HR) 2·18, 95 per cent confidence interval 1·40 to 3·39), adenomatous polyposis coli mutation distal to codon 1400 (HR 3·85, 1·90 to 7·80), proctocolectomy (HR 1·67, 1·06 to 2·61), open colectomy (HR 6·84, 1·96 to 23·98) and year of surgery (HR 1·04, 1·01 to 1·07) were independent risk factors for the diagnosis of DT after prophylactic surgery. CONCLUSION Laparoscopic surgery decreased the risk of DT after prophylactic colectomy in patients with FAP.
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Affiliation(s)
- M Vitellaro
- Colorectal Surgery Unit, Department of Surgery, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Nazionale dei Tumori, Milan, Italy; Department of Surgery, New York Presbyterian Hospital - Weill Cornell Medical College, New York, USA
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Anal transitional zone neoplasia in patients with familial adenomatous polyposis after restorative proctocolectomy and IPAA: incidence, management, and oncologic and functional outcomes. Dis Colon Rectum 2013; 56:808-14. [PMID: 23739186 DOI: 10.1097/dcr.0b013e31829005db] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND Restorative proctocolectomy and IPAA in patients with familial adenomatous polyposis may leave residual anal transitional zone mucosa that is prone to neoplasia. OBJECTIVE The aim of this study was to evaluate the long-term control of neoplasia at the IPAA, the functional outcomes, and the influence of anastomotic technique on these results. DESIGN : This research is a retrospective cohort study from a prospective database. SETTING The investigation took place in a high-volume specialized colorectal surgery department. PATIENTS Patients with familial adenomatous polyposis who underwent IPAA between 1983 and 2010 were included. MAIN OUTCOME MEASURES The primary outcomes measured were functional outcomes, quality of life, and the incidence of neoplasia in the anal transitional zone. RESULTS Eighty-six patients underwent mucosectomy and 174 underwent stapled anastomosis with mean 155 ± 99 and 95 ± 70 months follow-up. Eighteen patients (20.9%) in the mucosectomy group and 59 patients (33.9%) in the stapled group developed anal transitional zone adenomas (p = 0.03). One of 86 (1.2%) patients undergoing mucosectomy and 3 of 174 (1.7%) patients undergoing stapled anastomosis developed cancer in the anal transitional zone (p > 0.05). Three of these patients underwent an abdominoperineal resection, but one who refused abdominoperineal resection underwent transanal excision with neoileoanal anastomosis. Patients undergoing a mucosectomy had a significantly higher rate of anastomotic stricture, but other complications were similar. Incontinence, seepage, and pad usage were higher in the mucosectomy group. Cleveland global quality-of-life score was 0.8 ± 0.2 in patients with handsewn anastomoses and 0.8 ± 0.3 in patients with a stapled anastomoses (p > 0.05). LIMITATIONS This study was limited by its nonrandomized retrospective design. CONCLUSIONS Risk for the development of adenomas in the anal transitional zone is higher after a stapled IPAA than after a mucosectomy with handsewn anastomosis. However, control of anal transitional zone neoplasia results in a similar risk of cancer development. Because the stapled procedure is associated with better long-term functional outcomes than a mucosectomy, stapled IPAA is the preferable procedure for most patients with familial adenomatous polyposis.
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Turina M, Pavlik CM, Heinimann K, Behrensmeier F, Simmen HP. Recurrent desmoids determine outcome in patients with Gardner syndrome: a cohort study of three generations of an APC mutation-positive family across 30 years. Int J Colorectal Dis 2013; 28:865-72. [PMID: 23114473 DOI: 10.1007/s00384-012-1600-x] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 10/15/2012] [Indexed: 02/04/2023]
Abstract
PURPOSE Screening of Gardner syndrome (GS) patients is tailored towards prevention of colorectal cancer (CRC). However, many patients suffer from desmoid tumors, which are challenging to treat due to invasive growth and local recurrence. The aims of our study were to determine the effectiveness of screening in GS and analyze outcome of desmoid tumors by treatment modality. METHODS This was a cohort study of a family of 105 descendants with GS. All family members who agreed were screened by endoscopy, and colorectal resection was performed upon pending malignancy. Resectable desmoids were excised, whereas large tumors were treated by a combination of brachytherapy (BT) and radiotherapy (RT). Main outcome measures were the incidence of CRC and overall and disease-specific mortality (ClinicalTrial.gov ID NCT01286662). RESULTS Thirty-seven of 105 family members have GS. Preventive colorectal resections were performed in 16 patients (15 %), with one death due to gastric cancer. In four patients who denied screening endoscopy, invasive tumors of the colon (three patients) and stomach developed. Of 33 desmoid tumors, 10 (30 %) were located in the mesentery, 17 (52 %) in the abdominal wall, and 6 (18 %) in extra-abdominal sites. Excision of 12 desmoids was performed in eight patients. Four desmoids were treated by BT and RT and showed full or partial remission. CONCLUSIONS Provided adequate screening, good long-term control of colorectal tumors is achievable. However, desmoid tumors determine survival and quality of life in many patients. Our data suggest good local control using a combination of brachytherapy/radiotherapy in large desmoids unsuitable for surgical resection.
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Affiliation(s)
- Matthias Turina
- Department of Colorectal Surgery, Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland, OH 44195, USA.
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Warrier SK, Kalady MF. Familial adenomatous polyposis: challenges and pitfalls of surgical treatment. Clin Colon Rectal Surg 2012; 25:83-9. [PMID: 23730222 PMCID: PMC3423882 DOI: 10.1055/s-0032-1313778] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Surgical management of familial adenomatous polyposis (FAP) is complex and requires both sound judgment and technical skills. Because colorectal cancer risk approaches 100%, prophylactic colorectal surgery remains a cornerstone of management. Both patient factors and disease characteristics influence surgical decision-making regarding the timing of prophylactic surgery, the extent of resection, and types of reconstruction. Making appropriate choices can be challenging and there is continued debate regarding optimal strategies. This chapter reviews the controversies in colorectal surgery for FAP.
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Affiliation(s)
- Satish K. Warrier
- Department of Colorectal Surgery, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio.
- Sanford R. Weiss, M.D. Center for Hereditary Colorectal Neoplasia, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio.
| | - Matthew F. Kalady
- Department of Colorectal Surgery, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio.
- Sanford R. Weiss, M.D. Center for Hereditary Colorectal Neoplasia, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio.
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Rudert M, Holzapfel BM, Jakubietz MG. Adjuvant Radiotherapy. DEUTSCHES ARZTEBLATT INTERNATIONAL 2011; 108:553. [PMID: 21912576 DOI: 10.5372/1905-7415.0504.074] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 09/18/2023]
Abstract
Abstract
Background: Familial adenomatous polyposis (FAP) is an inherited colorectal cancer predisposing syndrome that has an autosomal dominant mode of inheritance with complete penetrance. Although the disease-causing gene of FAP, the adenomatous polyposis coli (APC) gene, is well understood, genetic testing for FAP remains uncommon in Thailand, possibly because of its high cost.
Objective: Present a family in southern Thailand that had benefited from this test.
Subject and methods: The proband was a 31-year-old man who had rectal cancer with profuse adenomatous polyposis of his colon.
Results: APC mutation screening revealed a novel mutation at codon 1249 (TGC1249TAA) that could predict a premature stop codon. On screening of three siblings in the same generation who were alive and 11 members of the descendant generation, four cases were positive for the mutation. The positive members were confirmed to have a type of diffuse colonic polyposis by endoscopy, and a prophylactic colectomy was performed without complications for every affected individual.
Conclusion: A genetic test is cost-effective. It should be considered for FAP, especially when the number of at-risk family members is high.
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Carmichael JC, Mills S. Surgical Management of Familial Adenomatous Polyposis. SEMINARS IN COLON AND RECTAL SURGERY 2011. [DOI: 10.1053/j.scrs.2010.12.010] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
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Laparoscopic colectomy and restorative proctocolectomy for familial adenomatous polyposis. Surg Endosc 2010; 25:1866-75. [PMID: 21136106 DOI: 10.1007/s00464-010-1478-z] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2010] [Accepted: 10/15/2010] [Indexed: 01/21/2023]
Abstract
BACKGROUND Familial adenomatous polyposis (FAP) is a dominantly inherited syndrome. Risk of cancer begins to increase after age 20 years if not treated. The purpose of this study was to evaluate the feasibility and short- and long-term outcomes after laparoscopic prophylactic surgery for FAP. METHODS Fifty-five patients with FAP were identified through the Hereditary Colorectal Tumor Registry from 2003 to 2009. Patients with laparoscopic total colectomy (TC)/IRA or proctocolectomy (TPC)/ileal pouch-anal anastomosis IPAA were included. Patients with previous colon or abdominal major surgery, malignancy, and desmoids before surgery were excluded. Main outcomes were: 30 days anastomotic leak and pouch failure; long-term desmoids and malignant recurrence. RESULTS Of the 55 patients, 32 were men, median age was 28 years, and mean body mass index was 23. Median follow-up time was 36 (range, 5-77) months. Forty-four patients had laparoscopic TC/IRA and ten had laparoscopic TPC/IPAA. One patient was converted to open surgery and received an open TPC/IPAA. Incision length was 7 (range, 5-14) cm. Anastomotic leak was 3 (5.4%: 2 laparoscopic and 1 open), and pouch failure was 0. Median postsurgical length of stay was 7 (range, 4-24) days. Desmoids occurred in three patients (5.4%), and there was no malignant recurrence within the follow-up period. Pathology revealed severe dysplasia in ten patients and adenocarcinoma in nine (8 laparoscopic and 1 open). Long-term small-bowel obstruction was 2 (3.6%). One mortality due to liver metastases occurred at 24 months. CONCLUSIONS Laparoscopic prophylactic treatment of FAP appears to be safe and feasible and may be an appealing alternative to open surgery. If the goal of prophylactic FAP surgery is to avoid cancer occurrence, laparoscopic surgery could be an important advancement.
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de Campos FGCM, Nicácio De Freitas I, Imperiale AR, Seid VE, Perez RO, Nahas SC, Cecconello I. [Colorectal cancer in familial adenomatous polyposis: Are there clinical predictive factors?]. Cir Esp 2010; 88:390-7. [PMID: 21056411 DOI: 10.1016/j.ciresp.2010.05.013] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2010] [Revised: 04/20/2010] [Accepted: 05/09/2010] [Indexed: 01/02/2023]
Abstract
BACKGROUND Familial Adenomatous Polyposis (FAP) is a hereditary disorder with multiple colorectal polyps that exhibit an almost inevitable risk of colorectal cancer (CRC) in untreated patients. GOALS To evaluate clinical features related to CRC risk at diagnosis. MATERIAL AND METHODS Charts from 88 patients were reviewed to collect information regarding age, family history, symptoms, polyposis severity and association with CRC. RESULTS 41 men (46.6%) and 47 women (53.4%) were assisted. CRC was detected in 53 patients (60.2%), with a frequency of 9.1% under 20 years, 58% between 21-40 and 85% over 41 years of age. Average age of patients without CRC was lower at treatment (29.5 vs. 40.0 years; p=0.001). Family history was reported by 58 patients (65.9%), whose average age did not differ from those who didn't report it (33.4 vs. 34.4; p=0.17). Asymptomatic patients comprised 10.2% of the total; in this group, CRC incidence was much lower when compared to those presenting symptoms (1.1% vs. 65.8%; p=0.001). Patients without CRC presented a shorter length of symptoms (15.2 vs. 26.4 months; p=0.03) and less frequent weight loss (11.4% vs. 33.9%; p=0.01). At colonoscopy, polyposis was classified as attenuated in 12 patients (14.3%), who presented greater average age (48.2 vs. 33.3 years; p=0.02) and equal CRC incidence (58.3% vs. 58.3%; p=0.6) when compared to those with classic polyposis. CONCLUSIONS The risk of CRC in FAP patients 1) increases significantly after the second decade; 2) is associated with higher age, weight loss, presence and duration of simptomatology; 3) is similar in patients with attenuated or classic phenotype.
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Sinha A, Tekkis PP, Rashid S, Phillips RKS, Clark SK. Risk factors for secondary proctectomy in patients with familial adenomatous polyposis. Br J Surg 2010; 97:1710-5. [DOI: 10.1002/bjs.7202] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/28/2023]
Abstract
Abstract
Background
Colectomy and ileorectal anastomosis (IRA) or restorative proctocolectomy are performed for prophylaxis in familial adenomatous polyposis (FAP). After IRA patients may require secondary proctectomy for worsening polyposis or rectal cancer. Outcomes after IRA were evaluated and risk factors predictive of progressive rectal disease identified.
Methods
Parametric survival analysis was used to identify predictors of progressive rectal disease in all patients undergoing an IRA for FAP at a single centre. Hazard ratios (HRs) were calculated for phenotype, genotype, sex, age at surgery and presence of colonic cancer.
Results
Of 427 patients who underwent IRA, 48 (11·2 per cent) developed rectal cancer and 77 (18·0 per cent) required proctectomy for worsening polyposis over a median follow-up of 15 (range 7–25) years. By the age of 60 years half of the patients retained their rectum. Rectal polyp count exceeding 20 (HR 30·99, 95 per cent confidence interval 9·57 to 100·32; P < 0·001), APC mutation codon 1250–1450 (HR 3·91, 1·45 to 10·51; P = 0·007), colonic polyp count 500 or more (HR 2·18, 1·24 to 3·82; P = 0·006) and age less than 25 years at the time of surgery (HR 1·99, 1·17 to 3·37; P = 0·011) were independent predictors of progressive rectal disease.
Conclusion
The risk of proctectomy after IRA for FAP is based on patient genotype, phenotype and age at surgery.
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Affiliation(s)
- A Sinha
- Polyposis Registry, St Mark's Hospital and Imperial College, Harrow, UK
| | - P P Tekkis
- Department of Surgery, Imperial College and the Royal Marsden Hospital, London, UK
| | - S Rashid
- Polyposis Registry, St Mark's Hospital and Imperial College, Harrow, UK
| | - R K S Phillips
- Polyposis Registry, St Mark's Hospital and Imperial College, Harrow, UK
| | - S K Clark
- Polyposis Registry, St Mark's Hospital and Imperial College, Harrow, UK
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