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Mucin-containing Rokitansky-Aschoff sinuses with extracellular mucin deposits simulating mucinous carcinoma of the gallbladder.
[carcinoma of the gallbladder]
Rokitansky-
Aschoff
sinuses
(
R-As
)
are
epithelial
invaginations
that
extend
down
the
gallbladder
wall
through
the
smooth
muscle
gaps
and
by
this
pathway
they
reach
the
subserosal
connective
tissue
.
We
report
here
5
cases
of
mucin-containing
R-As
that
ruptured
,
and
as
a
result
,
extracellular
mucin
escaped
into
the
adjacent
stroma
.
They
were
compared
with
10
mucinous
carcinomas
of
the
gallbladder
.
Three
cases
of
mucin-containing
R-As
accompanied
by
abundant
extracellular
mucin
deposits
with
epithelial
strips
,
glands
and
papillary
structures
were
misinterpreted
as
mucinous
carcinomas
.
Because
of
this
,
the
patients
were
subjected
to
a
second
unnecessary
radical
surgical
procedure
.
In
the
remaining
2
cases
,
the
extracellular
mucin
associated
with
R-As
was
acellular
and
regarded
as
focal
.
Three
patients
were
men
and
2
women
whose
ages
ranged
from
2
to
71
years
(
average
age
52
y
)
.
The
youngest
patient
was
a
2
-
year
-old
boy
in
whom
the
mucin-containing
R-As
were
discovered
incidentally
by
magnetic
resonance
imaging
.
He
was
later
diagnosed
with
metachromatic
leukodystrophy
.
The
remaining
4
patients
complained
of
right
upper
quadrant
pain
and
had
a
thickened
gallbladder
wall
identified
by
computed
tomography
and
magnetic
resonance
imaging
.
There
were
gallstones
in
3
patients
.
The
R-As
located
in
the
lamina
propria
contained
mucin
but
were
not
cystically
dilated
whereas
those
located
in
the
subserosa
were
mucin-filled
and
often
cystically
dilated
.
Detached
fragments
of
biliary
epithelium
,
small
glands
,
and
papillary
structures
lacking
cytologic
atypia
and
mitotic
figures
were
identified
in
the
abundant
mucin
deposits
located
in
the
subserosa
of
3
cases
.
The
overlying
surface
gallbladder
epithelium
exhibited
papillary
hyperplasia
with
focal
intestinal
metaplasia
in
2
patients
,
one
of
which
had
metachromatic
leukodystrophy
.
The
lack
of
reactivity
for
carcinoembryonic
antigen
and
p
53
and
the
low
proliferative
activity
as
measured
by
MIB-
1
labeling
index
provided
additional
support
to
the
benign
nature
of
the
lesion
.
Clues
to
separate
mucin-containing
R-As
from
mucinous
carcinoma
are
provided
.
All
5
patients
with
mucin-containing
R-As
are
disease
free
8
months
to
7
years
after
surgery
(
median
follow-up
of
39
mo
)
.
Diseases
Validation
Diseases presenting
"pain"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
aniridia
aromatase deficiency
carcinoma of the gallbladder
cholangiocarcinoma
coats disease
congenital diaphragmatic hernia
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
dentin dysplasia
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
focal myositis
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
lamellar ichthyosis
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
neuralgic amyotrophy
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
phenylketonuria
pleomorphic liposarcoma
primary hyperoxaluria type 1
proteus syndrome
pyomyositis
scrub typhus
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
trochlear dysplasia
typhoid
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wolf-hirschhorn syndrome
This symptom has already been validated