PJS - gastrointestinal cancer prevention from surveillance?

The following abstract from St. Mark's in London shares some intriguing suggestions. Can imaging, scopes, and surgery to find and remove polyps reduce our cancer risk? And, is our cancer risk from the polyps? Or are polyps a symptom of an underlying problem (ie. our gene defect)?  For those curious to learn more, I've included links to related articles in my commentary.

First, the group at St. Mark's followed a large number of patients (63) for a long time (average 10 years).

They note 776 procedures performed to assess the GI tract. I guess this is a combination of scopes + imaging tests (PillCam, barium x-rays, MR enteroscopy). This is an average of 12 per patient over an average 10 year span.

Then they say that these 63 patients had 78 procedures, most (71) were surgeries + 5 double balloon enteroscopy (DBE) + 1 push enteroscopy. I'm curious whether DBE will gradually replace some of the surgical procedures for those hard to reach small bowel polyps.

The authors say that none of the surgeries were emergency surgeries. And 20 were performed as a result of baseline investigations, 12 arose from investigations of symptoms, and 39 were due to surveillance of asymptomatic patients. This is good news -planned surgery is much safer for patients (and easier for surgeons) than emergency procedures.

Six complications arose from endoscopy or surgical intervention and five major surgeries were needed to manage those complications.

 Of the 2461 polyps removed, only 6 polyps contained atypia or dysplasia. None were cancer!

Not only that, none of the patients in the study were diagnosed with luminal (I think they mean gastrointestinal) cancer.

The authors conclude that GI surveillance in Peutz-Jeghers syndrome is relatively safe and avoids the need for emergency surgery for small-bowel polyps.

The lack of GI cancers may reflect that surveillance and polypectomy have prevented cancer from  developing, although the detection of neoplasia or dysplasia is uncommon.

In the past I've shared some of the controversy among PJS specialists about whether our increased cancer risk (which includes many other organs than the gi tract) is directly or indirectly related to polyps. Some researchers call PJS polyps an epi-phenomena (a secondary symptom to a malignant condition) http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3175351/ Other researchers have maintained that removing polyps significantly reduces our GI cancer risk. http://www.ncbi.nlm.nih.gov/books/NBK1826/

This group seems to be saying both - that polyps from their patients weren't cancerous and only a small minority were even abnormal (atypical or dysplastic). But that patients who had polyps removed may have had cancers prevented - this based on lack of cancer diagnoses in their patients.

I do want to note, we've had reports from families with PJS who go to St. Mark's that have mentioned a variety of cancer diagnoses including breast, pancreas, gi tract and other.

Additionally, Spigelman's article from 1989 offered this SUMMARY Among 72 patients with the Peutz-Jeghers syndrome malignant tumours have developed in 16 (22%) of whom all but one have died. There were nine gastrointestinal and seven non- gastrointestinal tumours. The relative risks of death from gastrointestinal cancer and all cancers were 13(95%CI2.7-38.1) and 9(95%CI4.2-173) respectively. The chance of dying of cancer by the age of 57 was 48%. There is  evidence for a hamartoma/carcinoma sequence in the Peutz-Jeghers syndrome, suggesting that the gene locus involved is relevant tot he development of malignancy in general.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1434341/pdf/gut00224-0090.pdf

Still, this is good news. If we can reduce GI cancer risk with scopes and surgical removal polyps, then we reduce our overall cancer risk.

+++++

Abstract:

Dis Colon Rectum. 2011 Dec;54(12):1547-51.

Peutz-jeghers syndrome: intriguing suggestion of gastrointestinal cancer

prevention from surveillance.

Latchford AR, Neale K, Phillips RK, Clark SK.

1 Department of Gastroenterology, Derriford Hospital, Plymouth, United Kingdom 2

Polyposis Registry, St. Mark's Hospital, Harrow, United Kingdom.

BACKGROUND: : Peutz-Jeghers syndrome is characterized by GI polyps and

mucocutaneous pigmentation and carries an increased risk of GI cancer. GI polyps

may bleed or cause intussusception. Luminal GI surveillance is recommended, but

there are few data detailing outcomes from GI surveillance in Peutz-Jeghers

syndrome.

OBJECTIVE: : This study aimed to assess outcomes from GI surveillance in patients

with Peutz-Jeghers syndrome.

DESIGN: : This study is a retrospective review, using hospital and registry notes

and endoscopy and histology reports.

SETTING: : The investigation was conducted at a tertiary referral center.

PATIENTS: : All patients with Peutz-Jeghers syndrome who were followed up at St

Mark's hospital were included.

MAIN OUTCOME MEASURES: : The primary outcomes measured were surveillance

procedures performed, complications, and long-term outcomes.

RESULTS: : Sixty-three patients from 48 pedigrees were included; the median age

when patients were first seen was 20 years (range, 3-59). Only baseline

investigations were performed in 12 patients. The remaining patients were

followed up for 683 patient years, a median of 10 years (range, 2-41). Seven

hundred seventy-six procedures were performed to assess the GI tract. These led

to 5 double-balloon enteroscopies, 1 push enteroscopy, and 71 surgical

procedures. Of the surgical procedures, 20 were performed as a result of baseline

investigations, 12 arose from investigations of symptoms, and 39 were due to

surveillance of asymptomatic patients. No emergency surgical interventions were

performed. No luminal GI cancers were diagnosed. Of the 2461 polypectomies

performed, 6 polyps contained atypia or dysplasia. Six complications arose from

endoscopy or surgical intervention, requiring 5 laparotomies to manage these

complications.

CONCLUSION: : GI surveillance in Peutz-Jeghers syndrome is relatively safe and

avoids the need for emergency surgery for small-bowel polyps. The lack of GI

cancers may reflect that surveillance and polypectomy have prevented cancer from

developing, although the detection of neoplasia or dysplasia is uncommon.

PMID: 22067184  [PubMed - in process]