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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
"cytologically malignant mast cells"
symptom
cutaneous mastocytosis
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