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Cowden syndrome: mucocutaneous lesions as precursors of internal malignancy.
[cowden syndrome]
Cowden
syndrome
is
an
autosomal-dominant
hereditary
cancer
syndrome
with
high
variability
and
susceptibility
.
It
is
characterized
by
multiple
hamartomas
and
neoplasms
of
ectodermal
,
endodermal
and
mesodermal
origin
affecting
many
organs
and
also
by
the
increased
risk
of
development
of
internal
malignancies
.
A
62
-
year
-old
woman
was
referred
to
our
Maxillofacial
Unit
with
a
hamartomatous
mass
of
the
left
lateral
tongue
which
had
slowly
grown
and
was
obstructing
normal
speech
and
restricting
oral
intake
.
The
patient
had
a
known
history
of
Cowden
syndrome
and
underwent
excision
of
the
lesion
under
general
anaesthetic
.
Orofacial
mucocutaneous
features
are
very
common
in
multiple
hamartoma
and
neoplasia
syndrome
with
almost
up
to
90
%
of
the
patients
being
affected
.
These
cutaneous
and
mucosal
lesions
,
which
are
predominantly
benign
,
often
manifest
prior
to
the
development
of
the
internal
malignant
tumours
associated
with
the
syndrome
.
The
prompt
identification
of
Cowden
syndrome
's
plethoric
signs
and
symptoms
can
lead
to
appropriate
surveillance
and
multidisciplinary
management
.
Oral
manifestations
are
frequent
,
prominent
and
usually
precede
the
establishment
of
malignant
tumours
of
visceral
organs
;
hence
,
the
maxillofacial
surgeon
or
general
dentist
may
have
a
significant
role
in
the
recognition
of
the
disease
.
Overall
prognosis
is
dependent
on
prevention
or
early
treatment
of
internal
malignancies
;
consequently
,
early
diagnosis
together
with
frequent
follow-up
forms
the
cornerstone
of
management
.
