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Familial follicular cell-derived thyroid carcinoma.
[werner syndrome]
Follicular
cell-derived
well-differentiated
thyroid
cancer
,
papillary
(
PTC
)
and
follicular
thyroid
carcinomas
comprise
95
%
of
all
thyroid
malignancies
.
Familial
follicular
cell-derived
well-differentiated
thyroid
cancers
contribute
5
%
of
cases
.
Such
familial
follicular
cell-derived
carcinomas
or
non-medullary
thyroid
carcinomas
(
NMTC
)
are
divided
into
two
clinical-pathological
groups
.
The
syndromic-associated
group
is
composed
of
predominately
non-thyroidal
tumors
and
includes
Pendred
syndrome
,
Warner
syndrome
,
Carney
complex
(
CNC
)
type
1
,
PTEN
-
hamartoma
tumor
syndrome
(
PHTS
;
Cowden
disease
)
,
and
familial
adenomatous
polyposis
(
FAP
)
/
Gardner
syndrome
.
Other
conditions
with
less
established
links
to
the
development
of
follicular
cell-derived
tumors
include
ataxia-
telangiectasia
syndrome
,
McCune
Albright
syndrome
,
and
Peutz-
Jeghers
syndrome
.
The
final
group
encompasses
syndromes
typified
by
NMTC
,
as
well
as
pure
familial
(
f
)
PTC
with
or
without
oxyphilia
,
fPTC
with
multinodular
goiter
,
and
fPTC
with
papillary
renal
cell
carcinoma
.
This
heterogeneous
group
of
diseases
does
not
have
the
established
genotype-phenotype
correlations
known
as
in
the
familial
C-
cell-derived
tumors
or
medullary
thyroid
carcinomas
(
MTC
)
.
Clinicians
should
have
the
knowledge
to
identify
the
likelihood
of
a
patient
presenting
with
thyroid
cancer
having
an
additional
underlying
familial
syndrome
stemming
from
characteristics
by
examining
morphological
findings
that
would
alert
pathologists
to
recommend
that
patients
undergo
molecular
genetic
evaluation
.
This
review
discusses
the
clinical
and
pathological
findings
of
patients
with
familial
PTC
,
such
as
FAP
,
CNC
,
Werner
syndrome
,
and
Pendred
syndrome
,
and
the
heterogeneous
group
of
familial
PTC
.
Diseases
Validation
Diseases presenting
"and fptc with papillary renal cell carcinoma"
symptom
cowden syndrome
pendred syndrome
werner syndrome
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