Decision analysis in the management of duodenal adenomatosis in familial adenomatous polyposis

Abstract

Patients with familial adenomatous polyposis are not only at high risk of developing adenomas in the colorectum but a substantial number of patients also develop polyps in the duodenum. Because treatment of duodenal polyps is extremely difficult and it is unknown how many patients ultimately develop duodenal cancer, the value of surveillance of the upper digestive tract is uncertain.
(1) To assess the cumulative risk of duodenal cancer in a large series of polyposis patients. (2) To develop a decision model to establish whether surveillance would lead to increased life expectancy.
Risk analysis was performed in 155 Dutch polyposis families including 601 polyposis patients, and 142 Danish families including 376 patients. Observation time was from birth until date of last contact, death, diagnosis of duodenal cancer, or closing date of the study.
Seven Dutch and five Danish patients developed duodenal cancer. The lifetime risk of developing this cancer by the age of 70 was 4% (95% confidence interval 1-7%) in the Dutch series and 3% (95% confidence interval 0-6%) in the Danish series. Decision analysis showed that surveillance led to an increase in life expectancy by seven months.
Surveillance of the upper digestive tract led to a moderate gain in life expectancy. Future studies should evaluate whether this increase in life expectancy outweighs the morbidity of endoscopic examination and proximal pancreaticoduodenectomy.

Full text

Gut
1997;
40:
716-719
Decision
analysis
in
the
management
of
duodenal
adenomatosis
in
familial
adenomatous
polyposis
H
F
A
Vasen,
S
Biulow,
T
Myrh0j,
L
Mathus-Vliegen,
G
Griffioen,
E
Buskens,
B
G
Taal,
F
Nagengast,
J
F
M
Slors,
P
de
Ruiter
The
Netherlands
Foundation
for
the
Detection
of
Hereditary
Tumours,
Leiden
H
F
A
Vasen
Department
of
Surgical
Gastroenterology,
The
Danish
Polyposis
Register,
Hvidovre
University
Hospital,
Hvidovre,
Denmark
S
Bulow
T
Myrhoj
Department
of
Gastroenterology,
Academic
Medical
Centre,
Amsterdam
L
Mathus-Vliegen
Department
of
Gastroenterology,
Leiden
University
Hospital,
Leiden
G
Griffioen
Department
of
Clinical
Epidemiology,
Utrecht
University
Hospital,
Utrecht
E
Buskens
Department
of
Gastroenterology,
Netherlands
Cancer
Institute,
Amsterdam
B
G
Taal
Department
of
Gastroenterology,
Nijmegen
University
Hospital,
Nijmegen
F
Nagengast
Department
of
Surgery,
Academic
Medical
Centre,
Amsterdam
J
F
M
Slors
Department
of
Surgery,
Medical
Centre
ALhnaar
P
de
Ruiter
Correspondence
to:
H
F
A
Vasen,
MD,
PhD,
The
Netherlands
Foundation
for
the
Detection
of
Hereditary
Tunours,
c/o
University
Hospital,
Rijnsburgerweg
10,
Building
no.50,
2333
AA
Leiden,
The
Netherlands.
Accepted
for
publication
28
January
1997
Abstract
Background-Patients
with
familial
ad-
enomatous
polyposis
are
not
only
at
high
risk
of
developing
adenomas
in
the
colorectum
but
a
substantial
number
of
patients
also
develop
polyps
in
the
duo-
denum.
Because
treament
of
duodenal
polyps
is
extremely
difficult
and
it
is
unknown
how
many
patients
ultimately
develop
duodenal
cancer,
the
value
of
surveillance
of
the
upper
digestive
tract
is
uncertain.
Aims-(l)
To
assess
the
cumulative
risk
of
duodenal
cancer
in
a
large
series
of
poly-
posis
patients.
(2)
To
develop
a
decision
model
to
establish
whether
surveillance
would
lead
to
increased
life
expectancy.
Methods-Risk
analysis
was
performed
in
155
Dutch
polyposis
families
including
601
polyposis
patients,
and
142
Danish
families
including
376
patients.
Obser-
vation
time
was
from
birth
until
date
of
last
contact,
death,
diagnosis
of
duodenal
cancer,
or
closing
date
of
the
study.
Results-Seven
Dutch
and
five
Danish
patients
developed
duodenal
cancer.
The
lifetime
risk
of
developing
this
cancer
by
the
age
of
70
was
40/0
(95%/o
confidence
interval
1-7%)
in
the
Dutch
series
and
30/o
(95%
confidence
interval
0
60/o)
in
the
Danish
series.
Decision
analysis
showed
that
surveillance
led
to
an
increase
in
life
expectancy
by
seven
months.
Conclusions-Surveillance
of
the
upper
digestive
tract
led
to
a
moderate
gain
in
life
expectancy.
Future
studies
should
evaluate
whether
this
increase
in
life
expectancy
outweighs
the
morbidity
of
endoscopic
examination
and
proximal
pancreaticoduodenectomy.
(Gut
1997;
40:
716-719)
Keywords:
familial
adenomatous
polyposis,
duodenal
cancer,
surveillance,
decision
analysis,
pancreaticoduodenectomy.
Familial
adenomatous
polyposis
(FAP)
or
Bussey-Gardner
polyposis
is
an
autosomal
dominant
disease
due
to
a
mutated
aden-
omatous
polyposis
coli
(APC)
gene
and
is
characterised
by
the
development
of
hundreds
of
adenomas
in
the
colon.1`3
Since
the
disease
was
first
recognised,
there
have
been
numerous
reports
of
other
lesions
outside
the
colon.
The
spectrum
of
lesions
reported
in
FAP
includes
multiple
osteomas
of
the
cranium
and
mandibles,
multiple
epidermoid
cysts
of
the
skin,
dental
abnormalities,
desmoid
tumours
of
the
abdominal
wall
and
abdomen,
bilateral
patches
of
congenital
hypertrophy
of
the
retina
pigment
epithelium,
and
fundic
gland
polyposis.4
I
During
the
1970s,
an
increasing
number
of
case
reports
of
FAP
patients
with
malignancy
of
the
periampullary
region
and
proximal
duo-
denum
appeared.6
'
These
reports
focused
attention
on
the
upper
gastrointestinal
(GI)
tract
and
led
to
series
of
reports
on
gastroduo-
denoscopy
of
groups
of
polyposis
patients.
Most
recorded
that
at
least
two
thirds
of
the
polyposis
patients
also
had
duodenal
aden-
omas.8-l'
The
first
question
to
arise
at
that
time
was:
do
the
adenomas
of
the
duodenum
follow
the
adenoma-carcinoma
sequence
observed
in
the
colorectum?
At
present,
there
is
ample
evidence
suggesting
that
this
is
the
case.
Duodenal
or
periampullary
adenocarcinoma
has
been
found
to
occur
in
patients
with
FAP
at
a
much
higher
frequency
compared
with
the
general
population16
17
and
40%
of
patients
with
duodenal
cancer
have
synchronous
duo-
denal
adenomas.'8
Despite
this
information
it
is
still
unknown
how
many
patients
with
duodenal
polyps
ultimately
develop
duodenal
cancer.
As
such
information
should
be
available
before
the
introduction
of
a
large
scale
surveillance
pro-
gramme
for
patients
with
polyposis,
we
evalu-
ated
the
lifetime
risk
of
duodenal
cancer
in
a
large
series
of
patients
with
polyposis
from
the
Polyposis
Registries
in
The
Netherlands
and
Denmark.
In
addition,
a
decision
analysis
model
was
developed
for
prediction
of
whether
surveillance
of
the
upper
GI
tract
would
lead
to
an
increased
life
expectancy.
Methods
THE
DUTCH
AND
DANISH
POLYPOSIS
REGISTRIES
Families
suspected
of
FAP
are
referred
to
the
registries
from
all
parts
of
both
countries.19
Personal
data,
results
of
investigation,
patho-
logy
reports,
and
results
of
treatment
are
collected
for
the
registries.
The
criteria
used
for
the
diagnosis
of
an
FAP
family
were
that
there
should
be
at
least
one
relative
with
more
than
100
colorectal
adenomas,
or
that
linkage
or
mutation
analysis
had
proven
that
the
APC
gene
was
responsible
for
the
disease
in
the
family.
RISK
ANALYSIS
For
risk
assessment,
patients
with
polyposis
were
studied
with
respect
to
risk
of
the
716
group.bmj.com on July 13, 2011 - Published by gut.bmj.comDownloaded from

Short
segment
Barrett's
oesophagus
Life
expectancy
(years)
Probability
of
finding
stage
IV
duodenal
adenomatosis:
0.11
Probability
of
not
finding
stage
IV
duodenal
adenomatosis:
0.89
Probability
of
dying
of
surgery:
0.05
Probability
of
surviving
surgery:
0.95
---
15
---
40
40
.3
years
Probability
of
duodenal
cancer:
No
0.04
surveillance
Probability
of
no
duodenal
----
cancer:
0.96
Decision
tree
for
a
30
year
old
man
with
familial
adenomatous
polyposis.
development
of
duodenal
cancer
from
birth
until
death.
The
data
were
analysed
by
life
table
analysis
methods.
Observation
time
was
until
date
of
last
contact,
death,
date
of
diagnosis
of
a
duodenal
cancer,
or
closing
date
of
the
study,
31
December
1995.
DECISION
ANALYSIS
We
applied
the
technique
of
decision
analysis
to
a
hypothetical
male
polyposis
patient,
30
years
of
age,
who
had
undergone
colectomy
and
ileorectal
anastomosis.
The
first
step
was
to
identify
all
the
alternative
actions,
treat-
ments,
and
outcomes
that
could
occur
for
the
patient
in
question.
On
the
basis
of
this,
a
decision
model
(shown
in
the
Figure)
that
displays
these
elements
in
their
proper
time
sequence
was
developed.
Points
where
the
tree
branches
("nodes")
are
square
("choice
nodes")
when
they
imply
a
decision
under
the
control
of
the
physician,
and
round
("chance
nodes")
if
a
chance
outcome
occurs.
Results
RISK
ANALYSIS
On
31
December
1995,
the
Dutch
Popyposis
Register
included
about
200
families
with
FAP.
Data
collection
was
completed
in
the
first
155
families
and
these
families
were
selected
for
the
present
study.
The
155
families
included
711
patients
with
FAP.
The
diagnosis
of
FAP
was
confirmed
by
pathology
and/or
medical
reports
in
601
patients.
One
hundred
and
eighteen
patients
died;
the
cause
of
death
is
known
in
910%
of
the
patients.
Among
the
601
patients,
seven
developed
duodenal
cancer
(including
one
suspected
case).
The mean
age
at
diag-
nosis
of
duodenal
cancer
was
47
years
(range
39-53).
The
cumulative
risk
of
developing
duodenal
cancer
by
age
70
was
4%
(95%
confidence
interval
l-7%).
The
number
of
patients
at
risk
by
age
70
was
27.
On
31
December
1995,
the
Danish
Poly-
posis
Register
included
142
FAP
families
with
a
completed
data
collection,
including
454
patients
of
whom
376
had
a
histologically
verified
FAP.
The
cause
of
death
is
known
for
all
160
deceased
patients.
In
five
patients
data
were
insufficient.
Of
the
remaining
371
affected
patients,
five
developed
duodenal
cancer;
the
mean
age
at
diagnosis
was
51
years
(range
43-77).
The
cumulative
risk
of
devel-
oping
duodenal
cancer
by
age
70
was
3%
(95%
confidence
interval
O-6%).
The
number
of
patients
at
risk
by
age
70
was
nine.
DECISION
ANALYSIS
The
decision
tree
for
the
30
year
old
polyposis
patient
with
corresponding
probabilities
is
shown
in
the
Figure.
We
assumed
that
the
life
expectancy
of
a
30
year
old
polyposis
patient
would
be
shortened
due
to
desmoid
disease,
the
mortality
due
to
secondary
rectal
surgery,
and
the
mortality
due
to
rectal
cancer.
There-
fore,
we
estimated
that
the
average
life
expec-
tancy
of
this
30
year
old
patient
would
be
40
years
instead
of
45
years.
For
staging
of
duodenal
polyposis
in
most
studies
use
was
made
of
the
so-called
Spigelman
classifi-
cation.'4
This
staging
system
is
based
on
a
set
of
arbritary
scores
using
postulated
adenoma/
cancer
risk
factors.
These
are
the
architecture
("villousness"),
the
degree
of
dysplasia,
and
the
size
and
number
of
the
duodenal
polyps.
Stage
I
represents
minor
disease
and
stage
IV
indicates
major
or
advanced
duodenal
poly-
posis
(Table
I).
When
stage
IV
duodenal
polyposis
is
found,
surgical
intervention
may
be
considered.
The
probability
of
finding
Spigelman
stage
IV
is
based
on
findings
of
two
prospective
studies
on
the
natural
history
of
duodenal
adenomatosis.
22
40
717
group.bmj.com on July 13, 2011 - Published by gut.bmj.comDownloaded from

Vasen,
Bulow,
Myrhey,
Mathus-Vliegen,
Griffioen,
Buskens,
et
al
TABLE
I
Classification
of
duodenal
adenomas
according
to
Spigelman'4
Points
Polyps
1
2
3
Number
<4
5-20
>20
Size
(mm)
0-4
5-10
>10
Histology
Tubular
Tubulo-villous
Villous
Dysplasia
Mild
Moderate
Severe
Spigelman
stage
I:
1-4;
stage
II:
5-6;
stage
III:
7-8;
stage
IV:
9-12
points.
One
analysis
was
conducted
at
the
St
Mark's
Polyposis
Registry
in
London'4
and
the
other
in
five
European
countries.'5
The
British
study
showed
that
11
(1
1%)
out
of
102
FAP
patients
had
stage
IV
duodenal
polyposis.
In
the
European
multicentre
study,
27
patients
out
of
310
(9%)
had
stage
IV
duodenal
polyposis.
In
the
multicentre
study,
7%
of
the
patients
aged
between
20
and
40
years,
and
11%
of
those
aged
between
40
and
60
years
had
stage
IV
duodenal
adenomatosis
(personal
communication,
S
Bulow).
The
cumulative
risk
of
developing
stage
IV
adenomatosis
is
therefore
at
least
110%.
The
mean
age
of
the
patients
identified
with
Spigelman
stage
IV
was
51
years
in
the
British
study
and
38
years
in
the
multicentre
study.
On
these
grounds
we
estimated
that
the
average
age
of
patients
who
reached
stage
IV
duodenal
polyposis
would
be
45
years.
If
a
pancreaticoduodenotomy
is
performed
and
the
patient
dies
as
a
result
of
complications
of
this
procedure,
the
aver-
age
life
expectancy
of
a
30
year
old
patient
would
amount
to
15
years.
The
periopera-
tive
mortality
of
pancreaticoduodenectomy
has
declined
during
the
past
decade
and
is
now
about
5%.
The
cumulative
risk
of
duodenal
cancer
by
age
70
in
the
present
series
is
3-4%.
The
mean
age
at
diagnosis
of
duodenal
cancer
in
this
study
and
in
three
others
was
about
50
years.
The
life
expectancy
of
a
patient
who
develops
duodenal
cancer
is
estimated
at
two
years.
Hence,
the
life
expectancy
of
the
hypothetical
30
year
old
patient
is
on
average
22
years
if
he
develops
duodenal
cancer.
We
then
worked
our
way
back
through
the
decision
tree
by
"folding
it
back"
from
right
to
left.
By
multiplying
the
life
expectancy
by
the
probabilities
of
occurrence
of
each
option,
and
summing
them
for
each
branch,
we
could
TABLE
ii
The
impact
of
various
probabilities
of
developing
duodenal
cancer,
stage
IVduodenal
adenomatosis,
and
perioperative
mortality
on
life
expectancy
Life
expectancy
(y)
SurveiUlance
No
surveillance
Probability
of
duodenal
cancer
(%/o)
4
39.9
39.3
10
399
38-2
15
39
9
37-3
Probability
of
stage
IV
duodenal
adenomatosis
(%)
1
1
39-9
39-3
15
39-8
39-3
20 39
7
39-3
Probability
of
perioperative
mortality
(%/6)
2
399
393
4
39.9
39.3
6
39-8
39-3
assign
life
expectancies
to
the
various
nodes.
The
calculations
showed
that
the
option
of
surveillance
led
to
an
increase
in
life
expec-
tancy
by
seven
months.
The
key
variables
-
the
cumulative
risk
of
stage
IV
duodenal
adenomatosis,
duodenal
cancer,
and
the
risk
of
mortality
due
to
pancreaticoduodenotomy
-
were
varied
over
a
plausible
range
to
assess
their
impact
on
the
outcome
of
the
model
(Table
II).
The
probability
of
developing
duodenal
cancer
appeared
to
be
the
most
important
variable.
Discussion
After
the
realisation
that
a
majority
of
patients
with
polyposis
develop
adenomas
in
the
duo-
denum,
many
investigators
recommended
surveillance
of
the
upper
GI
tract.
However,
before
establishing
such
a
surveillance
pro-
gramme,
a
more
critical
evaluation
of
the
pros
and
cons
of
surveillance
should
be
performed.
In
particular,
the
difficulties
for
effective
treatment
posed
by
duodenal
adenomas
make
the
benefit
of
surveillance
of
the
upper
GI
tract
questionable.
In
the
assessment
of
population
screening,
the
criteria
formulated
by
Wilson
and
Jungner20
are
usually
applied.
These
criteria
are
also
appropriate
in
the
assessment
of
surveillance
of
high
risk
groups
such
as
patients
with
polyposis.
According
to
these
criteria,
the
natu-
ral
history
of
duodenal
adenomas
should
be
known,
a
curative
treatment
should
be
avail-
able,
and
there
should
be
evidence
that
early
treatment
leads
to
an
improved
prognosis.
With
respect
to
the
natural
history
of
duodenal
adenomas,
the
most
urgent
question
is
"do
the
duodenal
polyps
have
the
same
malignant
potential
as
the
colonic
polyps?"
Earlier
studies'7
indicated
that
the
relative
risk
of
duodenal
cancer
in
FAP
was
very
high,
but
such
information
is
less
useful
in
the
decision
making
process,
because
the
incidence
of
duodenal
cancer
in
the
general
population
is
extremely
low.
Much
more
important
would
be
to
know
the
lifetime
risk
of
developing
duodenal
cancer.
The
present
study
revealed
that
the
cumulative
risk
of
duodenal
cancer
was
less
than
5%
by
the
age
of
70.
Although
prospective
studies
are
needed
to
confirm
our
findings,
such
studies
have
the
disadvantage
that
the
screening
examinations
will
inevitably
lead
to
early
detection
of
premalignant
disease
and
to
early
surgical
intervention,
which
will
interfere
with
the
assessment
of
the
duodenal
cancer
risk.
The
treatment
of
duodenal
adenomas
in
our
patients
is
limited
by
a
number
of
factors.
Endoscopic
snaring
may
be
made
impossible
by
the
presence
of
large
numbers
of
polyps
or
by
the
usual
sessile
nature
of
the
polyps.
Endoscopic
electrocoagulation,
if
repeated
very
often,
will
lead
to
considerable
scarring,
which
in
the
periampullary
area
might
cause
strictures.
Laser
ablation
of
polyps
via
the
endoscope
can
be
used,
but
carries
the
risk
of
duodenal
perforation.
Polyp
removal
by
(sur-
gical)
duodenotomy
consisting
of
submucosal
infiltration
and
local
excision
of
all
polyps
is
718
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Short
segment
Barrett's
oesophagus
not
recommended,
because
a
recent
study
has
shown
recurrence
in
all
patients
treated
by
this
technique
within
a
short
time.2"
To
summarise,
the
only
curative
treatment
appears
to
be
a
proximal
pancreaticoduodenotomy.
Such
an
operation
has
considerable
potential
morbidity
and
mortality
which
makes
the
indication
for
and
the
timing
of
surgery
extremely
difficult.
Criteria
of
size,
rapid
growth,
polyp
induration,
or
consistently
severe
dysplasia
or
villous
change
suggest
that
intervention
is
necessary.
In
the
above
mentioned
British
study,
among
the
10
patients
with
stage
IV
adenomatosis
at
the
first
endoscopy,
one
developed
duodenal
cancer
and
two
other
patients
are
suspected
of
having
this
type
of
cancer.22
Thus
surgery
may
be
considered
in
patients
that
consistently
have
stage
IV
duodenal
adenomatosis.
Evidence
that
early
treatment
leads
to
improvement
of
the
prognosis
is
not
yet
available,
and
it
will
probably
take
a
long
time
to
collect
such
information.
The
best
way
to
demonstrate
the
benefits
of
surveillance
would
be
by
randomised
controlled
studies
showing
a
higher
survival
rate.
Such
studies
will,
how-
ever,
be
difficult
to
carry
out
in
view
of
the
extremely
high
risk
of
premalignant
duodenal
disease.
Therefore,
we
decided
to
apply
de-
cision
analysis
to
predict
whether
surveillance
might
lead
to
an
increase
in
life
expectancy.
The
calculations
showed
that
surveillance
increased
the
life
expectancy
by
seven
months
if
surgery
was
performed
after
detection
of
stage
IV
adenomatosis.
Sensitivity
analysis
showed
that
the
probability
of
duodenal
cancer
had
the
strongest
effect
on
the
outcome
com-
pared
with
the
probability
of
developing
duo-
denal
adenomatosis
stage
IV
or
perioperative
mortality.
To
summarise,
the
present
analysis
revealed
that
surveillance
may
lead
to
a
moderate
gain
in
life
expectancy.
Therefore,
before
starting
surveillance
of
the
upper
digestive
tract,
it
is
important
to
explain
to
the
patients
that
the
risk
of
developing
duodenal
cancer
is
relatively
low
and
that
the
only
curative
treatment
for
severe
duodenal
adenomatosis
is
a
major
operation
with
substantial
morbidity
and
mortality
(in
addition
to
the
morbidity
from
duodenoscopy).
On
the
basis
of
this
infor-
mation
the
patients
may
be
able
to
decide
whether
the
potential
gain
in
life
expectancy
outweighs
the
adverse
effects
of
surveillance
and
treatment.
If
the
patient
prefers
to
be
under
surveillance,
the
screening
protocol
should
start
by
the
age
of
30
years.
Starting
at
an
earlier
age
can
be
considered
to
offer
no
clinical
benefit,
as
reports
of
duodenal
cancer
before
this
age
are
extremely
rare.
The
recom-
mended
interval
between
examinations
is
one
to
three
years
depending
on
the
findings.
Ideally,
the
results
should
be
collected
in
a
uniform
manner
at
a
regional
or
national
registry
which
will
permit
future
evaluation.
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doi: 10.1136/gut.40.6.716
1997 40: 716-719Gut
H F Vasen, S Bülow, T Myrhøj, et al.
adenomatous polyposis.
duodenal adenomatosis in familial
Decision analysis in the management of
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