An unusual case of asymptomatic non-urothelial bladder tumour

Abstract

Introduction:
Non-urothelial tumours are rare and account for less than 5% of all bladder tumours. Bladder paragangliomas also known as extra-adrenal pheochromocytomas are of the non-urothelial subgroup. We present an unusual case of asymptomatic bladder paraganglioma.

Case report:
A 77year old lady presented with acute abdominal pain was found to have an incidental enhancing nodule in the bladder. During cystoscopy and transurethral resection the patient experienced significant fluctuations in blood pressure that required anaesthetic adjustments. Review of histology confirmed a diagnosis of bladder paraganglioma.

Discussion:
Most bladder paraganglioma cases present with sympathomimetic related symptoms and microscopic haematuria but our patient was asymptomatic which resulted in inadequate pre-operative optimisation and high anaesthetic risk. Majority of paragangliomas are benign but there is a 20-40% chance of malignancy. The management options will predominantly depend on whether disease is localised, regional, metastatic or recurrent in nature.

Conclusion:
Due to the non-specific nature of disease, variability of presentations and rare incidence, bladder paragangliomas are often not part of the urologists' differential diagnoses. In our opinion, establishing guidelines should assist to achieve a balance between anaesthetic risks, cystoscopy and follow up.

Full text

CASE
REPORT
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International
Journal
of
Surgery
Case
Reports
27
(2016)
18–20
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Journal
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ourna
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An
unusual
case
of
asymptomatic
non-urothelial
bladder
tumour
Daniel
Chinga,
Eleni
Anastasiadisb,
Sarb
Sandhub,∗
aFiona
Stanley
Hospital,
Western
Australia,
Australia
bDepartment
of
Urology,
Kingston
Hospital
NHS
Foundation
Trust,
United
Kingdom
a
r
t
i
c
l
e
i
n
f
o
Article
history:
Received
27
March
2016
Received
in
revised
form
9
July
2016
Accepted
10
July
2016
Available
online
14
July
2016
Keywords:
Urinary
bladder
neoplasms
Non-urothelial
bladder
tumour
Paraganglioma
Extra-adrenal
Cystoscopy
and
transurethral
resection
Case
report
a
b
s
t
r
a
c
t
INTRODUCTION:
Non-urothelial
tumours
are
rare
and
account
for
less
than
5%
of
all
bladder
tumours.
Bladder
paragangliomas
also
known
as
extra-adrenal
pheochromocytomas
are
of
the
non-urothelial
subgroup.
We
present
an
unusual
case
of
asymptomatic
bladder
paraganglioma.
CASE
REPORT:
A
77
year
old
lady
presented
with
acute
abdominal
pain
was
found
to
have
an
incidental
enhancing
nodule
in
the
bladder.
During
cystoscopy
and
transurethral
resection
the
patient
experienced
significant
fluctuations
in
blood
pressure
that
required
anaesthetic
adjustments.
Review
of
histology
confirmed
a
diagnosis
of
bladder
paraganglioma.
DISCUSSION:
Most
bladder
paraganglioma
cases
present
with
sympathomimetic
related
symptoms
and
microscopic
haematuria
but
our
patient
was
asymptomatic
which
resulted
in
inadequate
pre-operative
optimisation
and
high
anaesthetic
risk.
Majority
of
paragangliomas
are
benign
but
there
is
a
20-40%
chance
of
malignancy.
The
management
options
will
predominantly
depend
on
whether
disease
is
localised,
regional,
metastatic
or
recurrent
in
nature.
CONCLUSION:
Due
to
the
non-specific
nature
of
disease,
variability
of
presentations
and
rare
incidence,
bladder
paragangliomas
are
often
not
part
of
the
urologists’
differential
diagnoses.
In
our
opinion,
estab-
lishing
guidelines
should
assist
to
achieve
a
balance
between
anaesthetic
risks,
cystoscopy
and
follow
up.
©
2016
Published
by
Elsevier
Ltd
on
behalf
of
IJS
Publishing
Group
Ltd.
This
is
an
open
access
article
under
the
CC
BY-NC-ND
license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
1.
Introduction
Bladder
tumours
can
be
subdivided
into
urothelial
and
non-
urothelial
tumours.
They
usually
present
as
urothelial
tumours,
with
non-urothelial
tumours
accounting
for
less
than
5%
of
all
blad-
der
tumours.
Bladder
paragangliomas
(also
known
as
extra-adrenal
pheochromocytomas)
are
of
the
non-urothelial
subgroup
[1].
We
present
an
unusual
case
of
asymptomatic
bladder
paraganglioma.
2.
Case
report
A
77
year
old
lady
presented
to
the
emergency
department
with
acute
abdominal
pain.
A
Computed
Tomography
(CT)
abdomen
and
pelvis
with
contrast
performed
demonstrated
an
inciden-
tal
enhancing
nodule
anteriorly
in
the
bladder
measuring
2.2
cm
(Figs.
1
and
2).
The
patient
was
referred
to
the
urology
multi-
disciplinary
team
(MDT)
which
decided
further
investigation
was
warranted
with
cystoscopy
and
a
transurethral
resection.
During
the
resection
under
general
anaesthesia,
the
patient
experienced
significant
fluctuations
in
blood
pressure
(BP)
that
∗Corresponding
author
at:
Consultant
Urologist,
Department
of
Urology,
Kingston
Hospital,
Galsworthy
Road,
KT2
7QB,
United
Kingdom.
E-mail
address:
Sarbjinder.sandhu@kingstonhospital.nhs.uk
(S.
Sandhu).
required
intermittent
breaks
and
pharmacological
adjustment.
Macroscopically
the
tumour
was
a
tan
brown
lesion
with
normal
smooth
bladder
mucosa.
Review
of
histology
confirmed
a
diagnosis
of
a
bladder
paragan-
glioma
(Figs.
3,
4
and
5).
CD56
and
chromogranin
immunostains
are
commonly
used
neuroendocrine
markers.
The
brown
staining
on
both
immunostains
shows
evidence
for
neuroendocrine
cells.
As
the
patient
had
multiple
intra-abdominal
surgeries
and
there
was
no
evidence
of
metastatic
spread
on
the
CT
scan,
the
MDT’s
decision
was
for
3
monthly
follow-up
with
surveillance
cystoscopies
post-
resection
and
depending
on
clinical
features
for
further
biochemical
and
radiological
investigations
as
needed.
3.
Discussion
The
incidence
of
bladder
paragangliomas
is
less
than
0.05%
of
all
bladder
tumours
[2]
and
less
than
1%
of
all
paragangliomas
[3].
The
mean
patient
age
was
43.3
years
(range
11-84)
and
has
an
almost
equal
male
to
female
ratio
of
1.07:1
[4].
A
systemic
review
found
106
patients
and
demonstrated
that
majority
of
cases
presented
with
catecholamine
secretion
lead-
ing
to
sympathomimetic
related
symptoms.
The
most
common
symptoms
at
presentation
in
decreasing
order
are
hypertension,
headaches,
haematuria
and
syncope/palpitations
[3].
They
are
often
triggered
during
micturition
hence
termed
‘micturition
attacks’
and
http://dx.doi.org/10.1016/j.ijscr.2016.07.008
2210-2612/©
2016
Published
by
Elsevier
Ltd
on
behalf
of
IJS
Publishing
Group
Ltd.
This
is
an
open
access
article
under
the
CC
BY-NC-ND
license
(http://creativecommons.
org/licenses/by-nc-nd/4.0/).

CASE
REPORT
–
OPEN
ACCESS
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et
al.
/
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27
(2016)
18–20
19
Fig.
1.
Coronal
view
showing
bladder
enhancing
nodule.
Fig.
2.
Axial
section
demonstrating
bladder
enhancing
nodule.
Fig.
3.
Low
power
view
with
Hematoxylin
&
Eosin
stain
demonstrating
the
solid
tumour
compressing
muscle.
Fig.
4.
CD56
stain
with
brown
colour
highlighting
positive
paraganglioma
tumour
cells.
No
necrotic
areas
or
mitoses
were
seen.
Antigen
Ki-67
is
less
than
5%.
Fig.
5.
Chromogranin
immunostain
with
brown
colour
demonstrating
positive
neu-
roendocrine
cells.
are
reported
in
52.8%
of
patients
[3].
Patients
were
reported
to
have
haematuria
in
47-60%
of
cases
and
most
were
of
microscopic
nature
[3,5].
The
literature
dictates
that
only
27%
of
patients
had
no
indication
of
hormonal
activity
[6],
and
3%
of
cases
were
inciden-
tal
findings
[3].
Our
patient
had
moderate
hypertension
controlled
with
one
anti-hypertensive,
did
not
experience
any
other
symp-
toms
and
had
a
negative
urine
specimen.
In
an
asymptomatic
incidental
bladder
lesion
it
is
almost
impossible
to
diagnose
bladder
paraganglioma
without
histological
diagnosis.
Suspicious
cases
are
usually
investigated
by
measur-
ing
the
level
of
catecholamine
and
its
metabolites
(metanephrine
and
vanillylmandelic
acid
secretion)
in
blood
and
urine
[7,8].
A
flexible
cystoscopy
may
be
warranted
as
a
form
of
preliminary
investigation
especially
in
symptomatic
patients.
CT
or
Magnetic
Resonance
Imaging
can
identify
the
location
and
extent
of
the
inva-
sion.
Advanced
methods
with
the
use
of
nuclear
medicine
scanning
using
radioisotope 123-
or 131-iodine-metaiodinebenzylguinidine
and
Positron
Emission
Tomography
should
be
considered,
and
is
the
imaging
modality
of
choice
for
localizing
small
tumours
[9–11].
Prior
to
any
surgical
intervention,
careful
pre-operative
man-
agement
is
required.
A
pre-operative
treatment
period
of
1-3
weeks
with
alpha-adrenergic
blockade
is
recommended
to
avoid
intra/post-operative
complications
such
as
hypertensive
crisis,
arrhythmias,
myocardial
infarction
and
pulmonary
oedema.
Beta-

CASE
REPORT
–
OPEN
ACCESS
20
D.
Ching
et
al.
/
International
Journal
of
Surgery
Case
Reports
27
(2016)
18–20
adrenergic
blockade
can
be
considered
pre-operatively,
especially
in
patients
with
persistent
arrhythmias
or
tachycardia,
but
must
never
be
initiated
before
alpha-adrenergic
blockade
because
doing
so
leads
to
unopposed
alpha-adrenergic
vasoconstriction
which
can
lead
to
a
life-threatening
crisis
[12].
Good
symptomatic
con-
trol
and
a
target
low
BP
for
age
are
features
of
adequate
medical
optimisation.
The
macroscopic
appearance
of
tan
brown
lesion
seen
on
cys-
toscopy
is
not
pathognomic
for
any
form
of
bladder
tumour.
According
to
literature,
majority
of
paragangliomas
are
benign
with
a
20-40%
of
chance
of
malignancy
[13].
There
is
no
single
parame-
ter
that
can
predict
the
malignant
potential
of
paragangliomas.
In
practice,
the
only
reliable
criterion
for
malignancy
is
the
presence
of
distant
metastases
[12].
At
present,
novel
molecular
biomarkers
are
being
investigated
for
their
utility
in
defining
malignant
tumours.
The
management
options
will
predominantly
depend
on
whether
disease
is
localised,
regional,
metastatic
or
recurrent
in
nature.
Patients
with
disease
dissemination
will
require
man-
agement
with
a
combination
of
surgery,
chemotherapy
and/or
radiotherapy
[14,15].
There
is
currently
no
consensus
on
the
management
of
localised/regional
cases.
Some
studies
advocate
a
partial
or
complete
cystectomy
as
there
is
a
chance
of
recurrence
from
chromaffin
cells
in
the
sympathetic
plexus
of
detrusor
muscle,
and
a
transurethral
resection
approach
does
not
adequately
excise
the
lesion.
Most
however
agree
that
regular
follow-up
is
necessary
[13].
Prognostic
factors
that
can
be
used
as
a
guide
include
large
size
(>5
cm
diameter),
local
tumour
extension,
and
the
presence
of
necrotic
areas
[12,13].
4.
Conclusion
Due
to
the
non-specific
nature
of
disease,
variability
of
presenta-
tions
and
rare
incidence,
bladder
paragangliomas
are
often
not
part
of
the
urologist’s
differential
diagnoses.
This
article
hopes
to
add
to
the
limited
cases
of
asymptomatic
incidental
bladder
paragan-
gliomas
in
the
literature
and
illustrate
the
importance
of
suspecting
its
possibility
to
reduce
operative
risk.
The
involvement
of
the
MDT
is
essential
to
ensure
patient
safety.
We
support
the
notion
that
all
cases
necessitate
long-term
follow
up
which
will
be
dictated
by
the
extent
of
disease
involvement.
In
our
opinion,
establishing
guide-
lines
should
assist
to
achieve
a
balance
between
anaesthetic
risks,
cystoscopy
and
follow
up.
Further
research
is
required
to
establish
best
practice
in
localised
bladder
paraganglioma
management.
Conflicts
of
interest
All
authors
declare
that
there
is
no
conflict
of
interest.
Funding
This
research
received
no
specific
grant
from
any
funding
agency
in
the
public,
commercial,
or
not
-
for
-
profit
sectors.
Ethical
Approval
Not
applicable.
Consent
Written
informed
consent
was
obtained
from
the
patient
for
their
anonymised
information
to
be
published
in
this
article.
Author
contribution
DC
and
EA
researched
literature
and
conceived
the
study.
DC
wrote
the
first
draft
of
the
manuscript.
All
authors
reviewed
and
edited
the
manuscript
and
approved
the
final
version
of
the
manuscript.
Guarantor
SS
is
the
guarantor
of
the
study.
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and
conditions,
which
permits
unrestricted
non
commercial
use,
distribution,
and
reproduction
in
any
medium,
provided
the
original
authors
and
source
are
credited.