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Papillary cystadenoma of the epididymis and broad ligament: morphologic and immunohistochemical overlap with clear cell papillary renal cell carcinoma.
[von hippel-lindau disease]
Papillary
cystadenoma
is
an
uncommon
epithelial
tumor
,
originating
within
the
head
of
the
epididymis
and
broad
ligament
.
When
the
lesion
is
bilateral
,
it
is
associated
with
von
Hippel-
Lindau
disease
.
Its
resemblance
to
metastatic
clear
cell
renal
cell
carcinoma
(
RCC
)
has
been
noted
in
the
literature
.
Owing
to
the
emergence
of
additional
markers
for
RCCs
,
we
have
evaluated
the
immunohistochemical
staining
patterns
of
a
series
of
7
papillary
cystadenomas
using
CK
7
,
RCC
,
PAX
8
,
carbonic
anhydrase
IX
,
and
AMACR
.
Six
of
the
cases
involved
the
epididymis
,
and
1
involved
the
broad
ligament
.
The
patients
ranged
in
age
from
20
to
65
years
old
.
All
of
the
tumors
were
unilateral
and
not
known
to
be
associated
with
von
Hippel-
Lindau
disease
.
The
lesions
were
composed
of
cystic
structures
,
which
focally
contained
papillary
fibrovascular
cores
lined
by
cuboidal
to
columnar
bland-appearing
cells
with
clear
cytoplasm
.
Another
component
was
the
presence
of
tubules
,
which
focally
had
elongated
profiles
.
Reverse
polarity
,
wherein
the
nuclei
are
oriented
toward
the
luminal
surface
with
subnuclear
vacuoles
,
was
present
focally
in
4
cases
and
more
extensively
in
a
fifth
case
.
Features
associated
with
malignancy
,
such
as
mitotic
figures
,
nuclear
pleomorphism
,
and
necrosis
,
were
not
identified
.
All
lesions
were
strongly
positive
for
CK
7
and
negative
for
RCC
.
Carbonic
anhydrase
IX
was
positive
in
all
tumors
(
diffusely
positive
in
6
,
patchy
in
1
)
with
lack
of
staining
in
the
apical
portion
of
the
cytoplasm
(
ie
,
cup-shaped
staining
)
.
PAX
8
showed
diffuse
positivity
in
6
of
the
7
lesions
,
with
one
of
the
epididymal
cases
showing
negative
staining
.
AMACR
staining
was
negative
in
5
of
the
7
cases
and
showed
only
focal
,
weak
staining
in
the
remaining
2
cases
.
The
current
study
more
specifically
demonstrated
that
papillary
cystadenoma
does
not
resemble
clear
cell
RCC
.
Rather
,
papillary
cystadenomas
of
the
epididymis
and
broad
ligament
have
identical
morphology
and
immunohistochemical
staining
to
clear
cell
papillary
RCC
.
The
diagnosis
of
papillary
cystadenoma
can
be
established
as
clear
cell
papillary
RCC
to
date
has
not
exhibited
metastatic
behavior
.
Diseases
Validation
Diseases presenting
"papillary cystadenomas"
symptom
von hippel-lindau disease
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