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Unusual virilization in girls with juvenile granulosa cell tumors of the ovary is related to intratumoral aromatase deficiency.
[aromatase deficiency]
Hyperandrogenism
is
a
rare
symptom
of
juvenile
ovarian
granulosa
cell-
tumors
(
JGCTO
)
.
This
study
aimed
to
determine
whether
hyperandrogenism
was
related
to
overexpression
of
SOX
9
,
decreased
expression
of
FOXL
2
or
absent
aromatase
expression
in
tumor
with
particular
scheme
of
expression
of
P
450
scc
and
P
450
c
17
alpha
.
T
hrough
a
nationwide
study
including
the
French
Society
of
Pediatric
Oncology
,
6
/
30
patients
with
JGCTO
presented
with
clinical
hyperandrogenism
and
high
plasma
testosterone
.
Tumor
specimens
underwent
immunofluorescence
(
SOX
9
,
FOXL
2
)
and
immunochemistry
(
aromatase
,
P
450
scc
,
P
450
c
17
alpha
)
.
Results
were
compared
with
patients
without
hyperandrogenism
.
S
OX
9
was
expressed
in
the
granulosa
cell
nucleus
in
2
/
6
cases
but
also
in
9
/
24
tumors
without
hyperandrogenism
(
p
=
n
.
s
.
)
.
FOXL
2
was
absent
or
decreased
in
3
/
6
cases
of
JGCTO
with
hyperandrogenism
with
no
statistical
difference
from
the
group
without
this
symptom
.
In
6
/
6
patients
,
the
intratumoral
expression
of
aromatase
was
absent
(
n
=
5
)
or
dramatically
reduced
(
n
=
1
)
.
In
contrast
,
15
/
24
patients
without
virilization
exhibited
conserved
aromatase
expression
in
their
tumor
(
p
<
0
.
05
)
.
A
variable
number
of
tumoral
cells
expressed
P
450
scc
while
some
interstitial
cells
were
focally
immunopositive
for
P
450
c
17
alpha
.
Unusual
virilization
in
girls
with
JGCTO
is
not
explained
by
a
dysregulation
in
SOX
9
or
FOXL
2
expression
,
but
is
related
to
a
localized
defect
of
aromatase
expression
in
granulosa
cells
and
to
the
ability
of
interstitial
cells
to
produce
testosterone
.
