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Multiplicity of hormone-secreting tumors: common themes about cause, expression, and management.
[familial hypocalciuric hypercalcemia]
Multiplicity
of
hormone-secreting
tumors
occurs
in
a
substantial
portion
of
hormone-excess
states
.
Multiplicity
increases
the
difficulty
of
management
and
drives
the
selection
of
special
strategies
.
This
is
a
synthesis
from
publications
about
tumor
development
and
expression
,
and
also
about
types
of
clinical
strategy
for
hormone-secreting
tumors
.
Comparisons
were
made
between
patient
groups
with
solitary
tumors
vs
those
with
multiple
tumors
.
Major
themes
with
clinical
relevance
emerged
.
Usually
,
tumor
multiplicity
develops
from
a
genetic
susceptibility
in
all
cells
of
a
tissue
.
This
applies
to
hormone-secreting
tumors
that
begin
as
either
polyclonal
(
such
as
in
the
parathyroids
of
familial
hypocalciuric
hypercalcemia
)
or
monoclonal
tumors
(
such
as
in
the
parathyroids
of
multiple
endocrine
neoplasia
type
1
[
MEN
1
]
)
.
High
penetrance
of
a
hereditary
tumor
frequently
results
in
bilaterality
and
in
several
other
types
of
multiplicity
.
Managements
are
better
for
the
hormone
excess
than
for
the
associated
cancers
.
Management
strategies
can
be
categorized
broadly
as
ablation
that
is
total
,
subtotal
,
or
zero
.
Examples
are
discussed
for
each
category
,
and
1
example
of
each
category
is
named
here
:
1
)
total
ablation
of
the
entire
tissue
with
effort
to
replace
ablated
functions
(
for
example
,
in
C-
cell
neoplasia
of
multiple
endocrine
neoplasia
type
2
)
;
2
)
subtotal
ablation
with
increased
likelihood
of
persistent
disease
or
recurrent
disease
(
for
example
,
in
the
parathyroid
tumors
of
MEN
1
)
;
or
3
)
no
ablation
of
tissue
with
or
without
the
use
of
pharmacotherapy
(
for
example
,
with
blockers
for
secretion
of
stomach
acid
in
gastrinomas
of
MEN
1
)
.
Tumor
multiplicity
usually
arises
from
defects
in
all
cells
of
the
precursor
tissue
.
Even
the
optimized
managements
involve
compromises
.
Still
,
an
understanding
of
pathophysiology
and
of
therapeutic
options
should
guide
optimized
management
.
Diseases
Validation
Diseases presenting
"tumor development"
symptom
congenital adrenal hyperplasia
dystrophic epidermolysis bullosa
esophageal squamous cell carcinoma
familial hypocalciuric hypercalcemia
liposarcoma
lymphangioleiomyomatosis
oral submucous fibrosis
primary effusion lymphoma
severe combined immunodeficiency
wolf-hirschhorn syndrome
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