Long-term survival between total colectomy versus proctocolectomy in patients with FAP: a registry-based,observational cohort study

Abstract

Background:

The best surgical choice for patients with familial adenomatous polyposis (FAP) is still debated. No prospective trials have been carried out to evaluate the pros and cons of the recommended procedures: total colectomy (ileorectal anastomosis [IRA]) vs restorative proctocolectomy (ileal pouch–anal anastomosis [IPAA]). The aim of this study was to provide a basis for tailored precision surgery in patients with FAP.

Methods:

We conducted a retrospective review of patients with FAP who underwent surgery and were registered in a dedicated database in Milan, Italy. Twenty-year survival related to surgical approach and prognostic factors were investigated using a Cox regression model.

Results:

A total of 925 patients underwent surgery between 1947 and 2015: 340 (36.8%) IPAA and 585 (63.2%) IRA. Colorectal cancer (CRC) at surgery was diagnosed in 28.6% of patients and a pathogenic APC variant was identified in 88%. During a median follow-up of 129 months, 150 patients died. The survival probability was significantly higher in the IRA than the IPAA group: 0.82 vs 0.75 (hazard ratio [HR] 0.6, 95% confidence interval [CI] 0.42–0.84). Multivariable regression modeling adjusted for propensity scores showed a similar difference, although no longer significant. Multivariable analysis indicated as independent risk factors CRC (HR 4.68, 95% CI 3.04–7.20) and age at surgery (HR 1.03, 95% CI 1.02–1.06). Among patients without cancer, the main risk factor for shorter survival was older age (HR 1.06, 95% CI 1.04–1.09).

Conclusion:

The study confirms excellent long-term results of surgical approaches with IRA and IPAA, suggesting that the best surgical choice may be an individually and clinically tailored approach, preferably at a young age.

Keywords

Familial adenomatous polyposis ileal pouch–anal anastomosis ileorectal anastomosis long-term survival prophylactic surgery

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References

Church

Familial adenomatous polyposis. Surg Oncol Clin N Am 2009; 18: 585–598.

Groden

Thliveris

Samowitz

et al. Identification and characterization of the familial adenomatous polyposis coli gene. Cell 1991; 66: 589–600.

Joslyn

Carlson

Thliveris

et al. Identification of deletion mutations and three new genes at the familial polyposis locus. Cell 1991; 66: 601–613.

Kinzler

Nilbert

et al. Identification of FAP locus genes from chromosome 5q21. Science 1991; 253: 661–665.

Nishisho

Nakamura

Miyoshi

et al. Mutations of chromosome 5q21 genes in FAP and colorectal cancer patients. Science 1991; 253: 665–669.

Stoffel

Mangu

Limburg

et al. Hereditary colorectal cancer syndromes: American Society of Clinical Oncology clinical practice guideline endorsement of the familial risk–colorectal cancer: European Society for Medical Oncology clinical practice guidelines. J Oncol Pract 2015; 11: e437–441.

Signoroni

Vitellaro

Sala

et al. Biomarkers in familial adenomatous polyposis: role and significance. Front Biosci (Schol Ed) 2010; 2: 413–421.

Syngal

Brand

Church

et al. ACG clinical guideline: Genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol 2015; 110: 223–262; quiz 263.

Guillem

Wood

Moley

et al. ASCO/SSO review of current role of risk-reducing surgery in common hereditary cancer syndromes. Ann Surg Oncol 2006; 13: 1296–1321.

10.

Aziz

Athanasiou

Fazio

et al. Meta-analysis of observational studies of ileorectal versus ileal pouch-anal anastomosis for familial adenomatous polyposis. Br J Surg 2006; 93: 407–417.

11.

Campos

FG.

Surgical treatment of familial adenomatous polyposis: dilemmas and current recommendations. World J Gastroenterol 2014; 20: 16620–16629.

12.

Provenzale

Gupta

Ahnen

et al. Genetic/familial high-risk assessment: colorectal, version 1.2016, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw 2016; 14: 1010–1030.

13.

Vasen

Moslein

Alonso

et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut 2008; 57: 704–713.

14.

Vitellaro

Sala

Signoroni

et al. Risk of desmoid tumours after open and laparoscopic colectomy in patients with familial adenomatous polyposis. Br J Surg 2014; 101: 558–565.

15.

Schemper

Smith

TL.

A note on quantifying follow-up in studies of failure time. Control Clin Trials 1996; 17: 343–346.

16.

Marubini

Valsecchi

MG.

Analysing Survival Data from Clinical Trials and Observational Studies. Chichester, UK: John Wiley & Sons; 1995.

17.

Cox

DR.

Regression models and life-tables. J R Stat Soc Ser B (Methodological) 1972; 34: 187–220.

18.

D’Agostino

Jr.

Propensity score methods for bias reduction in the comparison of a treatment to a non-randomized control group. Stat Med 1998; 17: 2265–2281.

19.

Austin

PC.

An introduction to propensity score methods for reducing the effects of confounding in observational studies. Multivariate Behav Res 2011; 46: 399–424.

20.

Harrell

FE.

Regression Modeling Strategies: With Applications to Linear Models, Logistic Regression, and Survival Analysis. New York: Springer; 2001.

21.

Hakulinen

Seppa

Lambert

PC.

Choosing the relative survival method for cancer survival estimation. Eur J Cancer 2011; 47: 2202–2210.

22.

Barrow

Khan

Lalloo

et al. Systematic review of the impact of registration and screening on colorectal cancer incidence and mortality in familial adenomatous polyposis and Lynch syndrome. Br J Surg 2013; 100: 1719–1731.

23.

Winawer

Fletcher

Rex

et al. Colorectal cancer screening and surveillance: clinical guidelines and rationale: update based on new evidence. Gastroenterology 2003; 124: 544–560.

24.

Setti-Carraro

Nicholls

RJ.

Choice of prophylactic surgery for the large bowel component of familial adenomatous polyposis. Br J Surg 1996; 83: 885–892.

25.

Olsen

Juul

Bulow

et al. Female fecundity before and after operation for familial adenomatous polyposis. Br J Surg 2003; 90: 227–231.

26.

Bertario

Russo

Radice

et al. Genotype and phenotype factors as determinants for rectal stump cancer in patients with familial adenomatous polyposis: Hereditary Colorectal Tumors Registry. Ann Surg 2000; 231: 538–543.

27.

Nieuwenhuis

Bulow

Bjork

et al. Genotype predicting phenotype in familial adenomatous polyposis: a practical application to the choice of surgery. Dis Colon Rectum 2009; 52: 1259–1263.

28.

Vasen

van der Luijt

Slors

et al. Molecular genetic tests as a guide to surgical management of familial adenomatous polyposis. Lancet 1996; 348: 433–435.

29.

Paul

McGannon

et al. APC genotype, polyp number, and surgical options in familial adenomatous polyposis. Ann Surg 1998; 227: 57–62.

30.

Koskenvuo

Renkonen-Sinisalo

Jarvinen

et al. Risk of cancer and secondary proctectomy after colectomy and ileorectal anastomosis in familial adenomatous polyposis. Int J Colorectal Dis 2014; 29: 225–230.

31.

Vasen

van Duijvendijk

Buskens

et al. Decision analysis in the surgical treatment of patients with familial adenomatous polyposis: a Dutch-Scandinavian collaborative study including 659 patients. Gut 2001; 49: 231–235.

32.

Bulow

Vasen

Jarvinen

et al. Ileorectal anastomosis is appropriate for a subset of patients with familial adenomatous polyposis. Gastroenterology 2000; 119: 1454–1460.

33.

Church

Burke

McGannon

et al. Risk of rectal cancer in patients after colectomy and ileorectal anastomosis for familial adenomatous polyposis: a function of available surgical options. Dis Colon Rectum 2003; 46: 1175–1181.

34.

De Cosse

Bulow

Neale

et al. Rectal cancer risk in patients treated for familial adenomatous polyposis: The Leeds Castle Polyposis Group. Br J Surg 1992; 79: 1372–1375.

35.

Heiskanen

Jarvinen

HJ.

Fate of the rectal stump after colectomy and ileorectal anastomosis for familial adenomatous polyposis. Int J Colorectal Dis 1997; 12: 9–13.

36.

Iwama

Mishima

Factors affecting the risk of rectal cancer following rectum-preserving surgery in patients with familial adenomatous polyposis. Dis Colon Rectum 1994; 37: 1024–1026.

37.

Penna

Kartheuser

Parc

et al. Secondary proctectomy and ileal pouch-anal anastomosis after ileorectal anastomosis for familial adenomatous polyposis. Br J Surg 1993; 80: 1621–1623.

38.

Sinha

Tekkis

Rashid

et al. Risk factors for secondary proctectomy in patients with familial adenomatous polyposis. Br J Surg 2010; 97: 1710–1715.