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Serrated polyposis syndrome – risk management

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Serrated polyposis syndrome (SPS), previously known as hyperplastic polyposis (HP), is a syndrome of unknown genetic basis defined by the occurrence of multiple serrated polyps in the large intestine and associated with an increased risk of colorectal cancer (CRC).r Conventional adenomas of the large intestine may be part of the syndrome and their presence relates to the CRC risk. Up to 40% of individuals with SPS have a family history of CRC. Occasionally more than one family member may develop characteristic serrated polyps and meet the diagnostic criteria for SPS.

Individuals who fulfil clinical criteria for SPS rarely have a heritable cause for CRC due to a pathogenic variant in known CRC predisposition genes, and review by a clinical genetics service or familial cancer centre is recommended.

This risk management guideline has been developed for individuals who meet clinical criteria for SPS and may or may not have a personal history of CRC. While the care of individuals affected by CRC should be individualised based on their clinical situation, colonoscopy screening for individuals with SPS is more frequent than usually advised following a CRC diagnosis.

  • Individuals who meet the World Health Organization (WHO) 2019 criteria for SPS:r 
    • at least 5 serrated polyps proximal to the rectum all ≥5 mm, with at least 2 ≥10 mm* (WHO criterion 1 2019) OR
    • >20 serrated polyps of any size but distributed throughout the colon, with at least 5 proximal to the rectum* (WHO criterion 2 2019)
  • The previous WHO 2010 criteria included an extra criterion: individuals with serrated polyps proximal to the rectum with a relative who meets clinical diagnostic criteria for SPS

* The polyp count is cumulative over multiple colonoscopies and any histological subtype of serrated polyp is included in the final count.r 

Exclusion criteria

  • Individuals with genetic cause for polyposis
  • Individuals without polyps who have a first-degree relative with SPS (see Support and information)
Cancer/carcinoma in situ Risk for this group General population lifetime risk
Colorectal cancer (CRC)

Lifetime risk is unknown

Prevalence is 15-35%**rr

5-year CRC incidence while under colonoscopy surveillance: 1% (95% CI 0-2.9%)rr

 7.39%***
CRC OR high-risk polyps****

5-year advanced neoplasia incidence while under surveillance:**** 44% (37-51%)r

WHO I 2010 (now WHO criterion 1 2019): 53% (39-65%)r

WHO III 2010 (now WHO criterion 2 2019): 26% (16-35%)r

WHO I+III 2010 (now WHO criteria 1+2 2019): 59% (40-72%)r

 Unable to obtain

WHO – World Health Organization
** This data does not take into account the impact of surveillance.
*** Source: Australian Institute of Health and Welfare (AIHW) 2020 Cancer Data in Australia; Canberra: AIHW. <https://www.aihw.gov.au/reports/cancer/cancer-data-in-australia/contents/cancer-risk-data-visualisation>. (2016 data)
**** Following endoscopic clearance of polyps, figures quote risk of advanced neoplasia (AN) during 5 years of colonoscopy surveillance (performed at yearly or 2-yearly intervals based on presence of significant polyps). 'Advanced neoplasia' was defined as colorectal cancer, advanced serrated polyps or advanced tubular adenomas. 

The choice of risk management strategy should take into account current age, other health issues and age-related cancer risk.

The impact of lifestyle on cancer risk should be discussed e.g. exercise regularly, maintain healthy weight, have a healthy diet, limit alcohol intake, do not smoke and avoid excessive sun exposure.

Cancer/tumour type Recommendations
Colorectal cancer (CRC) Surgical
  • Total colectomy is not recommended unless the number and size of polyps makes it impossible to remove all polyps >5 mm in which case colectomy and ileorectal anastomosis should be considered
  • If CRC is diagnosed in a patient with SPS, option of partial colectomy and debulking of any remaining colorectal polyps OR total colectomy to avoid frequent colonoscopy depending upon patient preference
Surveillance
  • Clearance phase at presentation with polyposis: Removal of all relevant polyps## with repeat colonoscopy at 3- to 6-monthly intervals until 2 colonoscopies are clear of polyps. May require 2 to 4 colonoscopiesr
  • Surveillance phase: Colonoscopy every 1 to 2 years with polypectomy of all polyps >5 mm or smaller polyps with appearance of adenoma/sessile serrated lesions by optical aspect. This may be personalised to yearly or 2-yearly depending on surveillance findingsr with annual colonoscopy only if certain types or numbers of polyps are found###
  • Repeat colonoscopy may be advised if there is inadequate bowel preparation
Risk-reducing medication
  • Unless contraindicated, aspirin should be actively considered to reduce the risk of CRC. A low dose (100-300 mg per day) is recommended for at least 2.5 years from age 50 years

## All sessile serrated lesions (SSLs), traditional serrated adenomas (TSAs) and conventional adenomas, as well as all hyperplastic polyps (HPs) ≥5 mm in diameter.
### Annual colonoscopy if there are: >1 advanced serrated SP (TSA and/or >10 mm and/or containing dysplasia), >1 advanced adenoma (>10 mm and/or >25% villous histology), >5 SSLs irrespective of size and/or adenomas (irrespective of size) and/or HPs >5 mm.

There is some evidence that colonoscopy and removal of polyps reduces the risk of cancer for individuals with SPS when endoscopic colonoscopy is performed by a specialised centre.r

There is evidence that aspirin/NSAIDs reduce the risk of serrated polyps though there is no evidence aspirin lowers CRC risk for individuals with SPS.

The metachronous CRC rate is low (1.3% [95% CI 0-3.2%])r for patients under surveillance colonoscopy in expert centres. Therefore, while partial colectomy is an option, individuals with SPS should be informed yearly to 2-yearly surveillance colonoscopy will be advised for any remaining large bowel.

In general, first degree blood relatives (parents/brothers/sisters/children) are at increased risk of CRC, estimated as at least 3 times higher risk. For individuals without polyps who have a first degree relative with SPS, it is reasonable to commence colonoscopy from age 40 years, or 10 years younger than the youngest age of SPS diagnosis in the family. Future surveillance in relatives is guided by colonoscopy findings. If no polyps are detected for relatives, then 5-yearly colonoscopy as advised for those at moderate risk of CRC is reasonable. If polyps are found, then more frequent colonoscopy is advised based on types and numbers of polyps.

Informing family members about hereditary cancer

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Version 7

Date Summary of changes
13/02/2023

The following sections of the document were updated to align with the new eviQ cancer genetics risk management template:

  • Cancer/tumour risk management guidelines:
    • Template sentence updated
    • Lifestyle factors sentence moved above the table. 

Protocol version number increased to V.7.

Version 6

Date Summary of changes
19/05/2021

Protocol reviewed at October 2020 reference committee meeting. Discussions continued via email and MS Teams. New 2020 eviQ template changes applied. Approved for publication with the following changes made:

  • Summary:
    • 'In addition to multiple serrated polyps,' deleted
    • 'Occasionally SPS can occur in more more than one family member' changed to 'Occasionally more than one family member may develop characteristic serrated polyps and meet the diagnostic criteria for SPS'
    • New paragraph starting 'Individuals who fulful...' inserted
    • Final two paragraphs: major wording changes
  • Target group: 
    • Bullet 1: 'World Health Organization (WHO) 2019' inserted
    • ​New bullet 2 inserted: 'The previous WHO 2010 criteria included an extra criterion: individuals with serrated polyps proximal to the rectum with a relative who meets clinical diagnostic criteria for SPS'
    • Exclusion criteria bullet 1: changed from 'Individuals with other polyposis syndromes' to 'Individuals with genetic cause for polyposis'
    • New note inserted
  • Lifetime risk of cancer/tumour: Table and notes completely rewritten, hover box ('WHO 2019 serrated polyposis criteria') inserted
  • Cancer/tumour risk management guidelines:
    • Heading changed to 'Cancer/tumour risk management guidelines'
    • Sentence added above table 'The choice of risk management strategy should take into account current age, other health issues and residual cancer risk.'
    • 'Colorectal' changed to 'Colorectal cancer (CRC)'
    • Surgical: new paragraph 2 added
    • Surveillance: completely rewritten
    • Notes 2 and 3 to table added
  • Evidence for risk management guidelines: 
    • Paragraph 1: inserted 'for individuals with', removed 'in', deleted sentence beginning 'However, there is no evidence...'
    • New paragraphs 2 and 3 inserted
  • Support and information: major wording changes

Protocol version number increased to V.6. Review in 2 years.

Version 5

Date Summary of changes
04/11/2019

Protocol underwent minor review at May 2019 reference committee meeting. Approved for publication with the following changes made:

  • Target group: criteria updated to reflect 2019 WHO criteria
  • Protocol template changes applied
  • "Mutation" changed to "pathogenic variant" throughout document for consistency among eviQ cancer genetics protocols per agreement among the cancer genetics reference committees' chairs. Definition of "pathogenic variant" added as a pop-up.

Protocol version number increased to V.5. For major review in 1 year.

Version 4

Date Summary of changes
15/07/2014

Discussed at March 6, 2013 reference committee meeting and discussion continued via email:

  • Approved and published.
  • Review second yearly.
31/07/2015 Link to AGSA changed to Genetic Alliance Australia.
07/01/2016 Sentence added to Risk Management template: "The impact of lifestyle on cancer risk should be discussed".
14/04/2016 Sentence added to Risk Management template: "This risk management guideline has been developed for individuals who have NOT been diagnosed with a relevant cancer/tumour.  The care of affected individuals should be individualised based on their clinical situation, and the monitoring they need as part of their treatment and post-treatment follow up". 
17/01/2017

Protocol reviewed and discussed at cancer genetics reference committee meeting 20 October 2016.

Changes to Cancer risk management guidelines and Evidence for risk management guidelines made. Next review in two years.  
31/05/2017 Transferred to new eviQ website. Version number changed to V.2.
29/03/2019

Protocol underwent minor review at Nov 2018 RCM and discussion continued over email. Approved with the following changes made:

  • Summary: Sentence added from updated RM template.
  • Lifetime risk of cancer: General population risk of CRC updated.
  • Cancer risk management guidelines: Recommendations updated.
  • Support and information: First degree relative colonoscopy recommendations updated.
  • References: updated.

Version changed to V.3 as updates signal practice change. Still to undergo scheduled major review this year. 
28/08/2019 Protocol title changed from 'Risk management for serrated polyposis syndrome' to 'Serrated polyposis syndrome – risk management' in accordance with Cancer Genetics Reference Committees' consensus. Version number increased to V.4.

The information contained in this document is based on the highest level of available evidence and consensus of the eviQ reference committee regarding their views of currently accepted approaches to care or treatment. Any clinician seeking to apply or consult this document is expected to use independent clinical judgement in the context of individual clinical circumstances to determine any patient's care or treatment. While eviQ endeavours to link to reliable sources that provide accurate information, eviQ and the Cancer Institute NSW do not endorse or accept responsibility for the accuracy, currency, reliability or correctness of the content of linked external information sources. Use is subject to eviQ’s disclaimer available at www.eviQ.org.au

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https://www.eviq.org.au/p/1373

31 Mar 2023