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Pulmonary Metastases of a Uterine Smooth Muscle Tumour with Undefined Malignancy Potential.
[lymphangioleiomyomatosis]
Smooth
muscle
neoplasms
with
atypical
proliferative
behaviour
,
but
without
clear
histopathological
malignancy
represent
a
diagnostic
and
therapeutic
challenge
,
as
distinction
from
a
sarcoma
can
be
difficult
and
no
guaranteed
treatment
recommendations
are
available
due
to
the
rarity
of
these
changes
.
In
the
event
of
uncertain
primary
histology
,
even
metastases
can
not
be
assessed
as
malignancy
criteria
,
but
may
contribute
to
the
clarification
of
the
histology
.
Similarities
with
other
smooth
muscle
proliferations
,
such
as
lymphangioleiomyomatosis
,
are
striking
.
The
diagnostic
difficulties
and
treatment
options
are
explained
based
on
the
example
of
a
59
-
year
-old
patient
,
in
whom
a
retroperitoneal
mass
and
pulmonary
lesion
of
such
a
tumour
occurred
4
years
after
a
hysterectomy
.
Even
though
the
genesis
and
histological
diagnostics
have
not
been
conclusively
clarified
,
slow
growth
and
a
low
recurrence
rate
for
post-menopausal
patients
allow
for
a
wait-and-see
approach
,
whereby
the
option
for
anti-hormonal
treatment
exists
in
the
event
of
positive
evidence
of
hormone
receptors
.
Diseases
Validation
Diseases presenting
"uncertain primary histology"
symptom
lymphangioleiomyomatosis
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