Thursday, March 10, 2016

Pancreatic cancer screening in PJS

Dear PJS people, 

Tell me your pancreatic cancer screening routine (or lack of) and I’ll tell you where you live…and maybe even guess your correct age.

This sounds humorous, but it should be a very serious topic for all of us. Pancreatic cancer risk in people with PJS is astronomical – our risk is possibly over 100 times that of the general population.

While pancreatic cancer is rare in the general population and generally affects older people, in PJS it is fairly common and often affects younger folks and even those who don’t have complicating risk factors like smoking.

Different research groups study different populations of PJS people to arrive at the high-risk estimate – the geographical and historical differences are startling.

More startling to me, a person living with PJS who’s read the medical literature for decades, is how all over the map the cancer screening guidelines for pancreatic cancer are.

A large European expert review advised against it:

Question: Is pancreatic cancer surveillance useful in PJS?

Routine surveillance for pancreatic cancer in PJS using current methods is not proven to be of benefit and is not cost effective. It should only be performed in the setting of a clinical research study.

2010 article from 2007 conference of European PJS specialists
Peutz–Jeghers syndrome: a systematic review and recommendations for Management
free full text


Similar guidelines from St. Mark’s in London, England:

We do not, however, recommend:

::: Pelvic or testicular ultrasound scans.
::: CT scans of the pancreas.
::: The use of tumour markers.

The reason why we do not recommend these scans and tests is that they are not accurate screening tools. For example, all young women develop small cystic areas on their ovaries to do with their monthly cycle. We have found that the frequent interventions caused by the scans of the pelvis caused more harm than good. Likewise, CT scans supply radiation to the body (which might help induce cancer) and do not reliably pick up early cancers.

2014 Intro to PJS for affected and their loved ones from St. Mark’s Hospital Polyposis Registry in London


And from Australia:

Routine screening of pancreas has recently been investigated and the authors found that surveillance should only be performed in defined research protocols as surveillance has not yet proven to reduce mortality [47]. Perth, W. Australia

2014 Introductory article PJS and JPS and similar polyposis syndromes


Pancreatic screening seems promising, but it is in the Netherlands nowadays only performed in light of an ongoing prospective trial since there are still many unanswered questions regarding pancreatic screening. These include whether early detection of (pre-cursor) lesions leads to an improved patient outcome, and also focus on the best way to manage detected lesions.


But, patients in the USA are offered dramatically different pancreatic cancer screening guidelines:

Pancreatic - Annual abdominal ultrasound or annual/every-other-year endoscopic ultrasound from age 25-30 years onwards.

Patients with PJS appear to have an increased risk for pancreatic-biliary cancer.[40] In a cohort study by Korsse et al that included 144 patients with PJS, 7 patients (5%) developed pancreatic cancer (median age, 54 y), 2 patients (3%) developed distal bile duct cancer, and 2 developed or ampullary cancer (median age, 55 y). The cumulative risk for pancreatic cancer was 26% at age 70 years; the relative risk was 76 (P < .001). The investigators reported a cumulative risk for pancreatic-biliary cancer of 32% at age 70 years, with a relative risk of 96 (P < .001).[40]

Imaging studies of the liver and the pancreas are indicated because of the risk of pancreatic cancer as well as of gallbladder polyps and cancer. These imaging studies may include ultrasonography and CT scanning with pancreatic details or magnetic resonance cholangiopancreatography (MRCP).

2015 eMedicine article on PJS:


And Johns Hopkins in Baltimore, Maryland is running  its fifth CAPS (cancer of the pancreas screening study) that includes locations in Connecticut, Maryland, Massachusetts, New York, Ohio, and Pennsylvania.
Participants with Peutz-Jeghers Syndrome should be at least 30 years old, and have at least 2 of 3 criteria diagnostic of Peutz-Jeghers syndrome (characteristic intestinal hamartomatous polyps, mucocutaneous melanin deposition, or family history of Peutz-Jeghers syndrome), or, known STK11 gene mutation carrier


So, are PJS people in the USA getting better medical care or just more aggressive, possibly futile, medical care?

Having observed the development of these guidelines over the past 16 years, I think reviews are mixed.

No medical journal articles to date suggest that pancreatic cancer screening in PJS people saves lives, leads to early diagnosis or successful treatment. None suggest that either the financial or possible costs are worth it. There aren’t even case reports, never mind population studies, with reliable statistics to suggest that USA guidelines are better than European or Australian guidelines.

The question of cancer screening in the general population (those at low- or average risk without symptoms) is in doubt throughout the world. A recent Smart Patients conversation has brought to light many concerns to what seems like a simple equation:

Here’s a quote I wrote that topic:

“We tend to think - early detection leads to cure leads to lives saved. It sounds like a no-brainer and has been the basic message from disease and patient advocacy organizations for decades. It's a political hot potato too.

“But, life and death experience just doesn't bear out our simplistic formulas for many, many reasons - including lead time bias; over diagnosis; over treatment; risks of harm from screening procedures and subsequent invasive procedures; lack of effective care/cure for a wide variety of conditions including many cancers; lack of any proof that early diagnosis leads to improved survival (disease-specific or overall survival); the numbers needed to screen to come up with one diagnosis or save one life; lack of clarity and innumeracy in practitioners and patients leading to poor choices; confusion of relative risk (RR) and absolute risk (AR) estimates; the fear and worry that arise when a possible cancer is diagnosed (many tests find many non-cancers, non-life threatening cancers, non-curable cancers and even non-treatable cancers); and false negative findings (missed cancers) provide false reassurance.

“I didn't mention financial expense, because for many patients, this topic boils down to, ‘they're trying to save money by throwing us to the wolves. They're just denying us necessary and basic services and medical care.’”

(free registration required)

A difficulty all PJS people face is the rarity of our condition and that most of us see gastroenterologists infrequently to manage our polyp care and GPs or PCPs more frequently to manage our overall medical care. Nobody is likely to focus on our pancreatic cancer risk and screening (or our reproductive tract cancer risk, but that’s a separate problem). And it’s unlikely that anyone will keep up with the wide range of screening guidelines to help us make the best medical decisions for ourselves and our loved ones.

There is no solution to this problem, though it’s worth exploring.

You are welcome to print and share this with your doctors. I’ve included references and links to medical literature for them.

Hope we can explore this together too.

What have you had done, where, at what age, what conversation came before, any questions or concerns, any good or bad results?

We’ve so much yet to learn. 

Please join us at to share your stories, questions and concerns. Free registration required.

Healing regards, dear PJS friends,

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