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Mast cell sarcoma in an infant: a case report and review of the literature.
[cutaneous mastocytosis]
Mast
cell
diseases
comprise
a
spectrum
of
disorders
including
cutaneous
mastocytosis
,
indolent
or
aggressive
systemic
variants
including
leukemia
,
and
unifocal
tumor
formations
such
as
benign
extracutaneous
mastocytoma
or
aggressive
mast
cell
sarcoma
(
MCS
)
.
Many
mast
cell
diseases
are
associated
with
aberrancy
of
c-
KIT
proto-oncogene
resulting
in
tyrosine
kinase
activity
,
typically
exhibiting
point
mutation
in
codon
816
.
MCS
is
an
exceedingly
rare
clinicopathologic
entity
characterized
by
a
unifocal
accumulation
of
neoplastic
mast
cells
that
grow
in
a
locally
destructive
manner
.
We
report
a
case
in
a
2
-
year
-old
boy
who
was
initially
diagnosed
at
8
months
of
age
with
atypical
cutaneous
mastocytoma
of
the
right
ear
with
subsequent
aggressive
,
destructive
growth
pattern
;
features
that
were
most
consistent
with
MCS
.
So
far
,
MCS
has
been
documented
in
the
literature
in
at
least
6
human
cases
.
To
the
best
of
our
knowledge
,
our
case
represents
the
first
MCS
in
an
infant
.
Thorough
multimodal
approach
with
strict
follow-up
is
relevant
in
appropriately
diagnosing
this
rare
entity
,
particularly
in
differentiating
this
lesion
from
other
neoplasms
that
are
more
likely
to
occur
in
infancy
.