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Malignant transformation of mastocytoma developed on skin mastocytosis into cutaneous mast cell sarcoma.
[cutaneous mastocytosis]
Mastocytosis
is
a
group
of
disorders
characterized
by
abnormal
mast
cell
proliferation
,
involving
the
skin
in
80
%
of
cases
.
Cutaneous
mastocytosis
,
which
appears
in
childhood
in
60
%
of
cases
,
usually
has
a
benign
course
with
a
gradually
regressive
evolution
before
puberty
.
Mast
cell
sarcomas
,
part
of
the
systemic
forms
of
mastocytosis
,
are
very
rare
tumors
characterized
by
a
destructive
growth
of
highly
atypical
mast
cells
,
with
secondary
spread
,
poor
prognosis
,
and
low
survival
rates
.
We
report
the
first
known
case
of
primary
cutaneous
mast
cell
sarcoma
due
to
the
transformation
of
a
benign
solitary
mastocytoma
in
an
adult
suffering
from
an
unregressive
localized
cutaneous
mastocytosis
.
Histologic
characteristics
of
the
tumor
,
mutation
analysis
,
and
c-
Kit
expression
were
compared
with
available
data
.
Wide
surgical
excision
of
the
tumor
followed
by
adjuvant
local
radiotherapy
were
performed
,
and
for
the
first
time
the
use
of
imatinib
was
attempted
,
as
neoplastic
mast
cells
expressed
the
CD
117
marker
.
However
,
they
failed
to
control
the
progression
of
sarcoma
.
To
date
,
no
treatment
is
known
to
be
effective
for
this
disease
,
which
is
associated
with
short
-term
survival
of
the
patients
.
Diseases
Validation
Diseases presenting
"abnormal mast cell proliferation"
symptom
cutaneous mastocytosis
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