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PEComa of the nasal cavity with worrisome histologic features and benign behavior: a case report.
[lymphangioleiomyomatosis]
PEComas
(
perivascular
epithelioid
cell
tumors
)
are
a
family
of
neoplastic
lesions
that
share
overlapping
ultrastructure
and
morphological
and
immunohistochemical
appearance
and
include
angiomyolipoma
,
lymphangioleiomyomatosis
,
and
clear
cell
"
sugar
"
tumor
of
the
lung
,
as
well
as
similar
tumors
that
occur
in
a
variety
of
visceral
,
cutaneous
,
and
soft
tissue
sites
throughout
the
body
.
A
40
-
year
-old
woman
came
to
medical
attention
because
of
epistaxis
and
because
of
unilateral
nasal
obstruction
of
3
months
'
duration
.
Endoscopic
examination
revealed
a
well-demarcated
exophytic
lesion
attached
to
the
anterior
portion
of
the
middle
turbinate
.
The
lesion
was
superficially
located
,
and
therefore
amenable
to
complete
surgical
excision
.
Seven
years
after
surgery
,
the
patient
is
alive
and
well
,
without
evidence
of
local
recurrence
or
metastastic
disease
.
Based
on
morphological
and
immunohistochemical
appearance
,
a
diagnosis
of
PEComa
with
worrisome
histologic
features
was
rendered
.
In
the
present
study
,
we
describe
a
PEComa
that
occurred
in
the
nasal
cavity
and
discuss
the
behavior
of
this
entity
.
The
importance
of
recognizing
this
disease
will
ensure
its
consideration
in
the
differential
diagnosis
of
tumors
of
the
head
that
have
similar
morphological
features
.
The
histogenesis
of
PEComa
still
remains
elusive
,
and
collection
of
additional
cases
with
a
prolonged
follow-up
will
be
important
in
accurately
determining
the
behavior
of
these
distinctive
tumors
.
Diseases
Validation
Diseases presenting
"epistaxis"
symptom
heparin-induced thrombocytopenia
lymphangioleiomyomatosis
waldenström macroglobulinemia
This symptom has already been validated