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
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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
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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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4112971/
xxx
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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.
xxx
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.
Details:
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:
xxx
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
xxx
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 https://www.smartpatients.com/communities/peutz-jeghers-syndrome to share your stories, questions and concerns. Free registration required.
Please join us at https://www.smartpatients.com/communities/peutz-jeghers-syndrome to share your stories, questions and concerns. Free registration required.
Healing regards, dear PJS friends,
Stephanie
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