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A random Abstract
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Mast cell sarcoma: a rare and potentially under-recognized diagnostic entity with specific therapeutic implications.
[cutaneous mastocytosis]
Mast
cell
sarcoma
is
a
rare
,
aggressive
neoplasm
composed
of
cytologically
malignant
mast
cells
presenting
as
a
solitary
mass
.
Previous
descriptions
of
mast
cell
sarcoma
have
been
limited
to
single
case
reports
,
and
the
pathologic
features
of
this
entity
are
not
well
known
.
Here
,
we
report
three
new
cases
of
mast
cell
sarcoma
and
review
previously
reported
cases
.
Mast
cell
sarcoma
has
a
characteristic
morphology
of
medium-sized
to
large
epithelioid
cells
,
including
bizarre
multinucleated
cells
,
and
does
not
closely
resemble
either
normal
mast
cells
or
the
spindle
cells
of
systemic
mastocytosis
.
One
of
our
three
cases
arose
in
a
patient
with
a
remote
history
of
infantile
cutaneous
mastocytosis
,
an
association
also
noted
in
one
previous
case
report
.
None
of
our
three
cases
were
correctly
diagnosed
as
mast
cell
neoplasms
on
initial
pathological
evaluation
,
suggesting
that
this
entity
may
be
under-recognized
.
Molecular
testing
of
mast
cell
sarcoma
has
not
thus
far
detected
the
imatinib-resistant
KIT
D
816
V
mutation
,
suggesting
that
recognition
of
these
cases
may
facilitate
specific
targeted
therapy
.
Diseases
Validation
Diseases presenting
"neoplasm"
symptom
adrenal incidentaloma
alexander disease
aromatase deficiency
carcinoma of the gallbladder
cholangiocarcinoma
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
erdheim-chester disease
esophageal adenocarcinoma
esophageal carcinoma
familial hypocalciuric hypercalcemia
focal myositis
hodgkin lymphoma, classical
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
kabuki syndrome
liposarcoma
lymphangioleiomyomatosis
pleomorphic liposarcoma
primary effusion lymphoma
proteus syndrome
pyomyositis
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wolf-hirschhorn syndrome
This symptom has already been validated