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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
.
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familial hypocalciuric hypercalcemia
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