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The utility of cortical-sparing adrenalectomy in pheochromocytomas associated with genetic syndromes.
[von hippel-lindau disease]
Management
of
pediatric
patients
with
pheochromocytomas
associated
with
genetic
syndromes
,
such
as
Von
Hippel-
Lindau
(
VHL
)
disease
,
is
complex
owing
to
the
need
for
continuous
surveillance
,
high
rates
of
recurrence
,
multiple
operations
during
childhood
,
and
the
possibility
of
lifelong
exogenous
steroid
replacement
.
We
report
the
successful
treatment
of
two
siblings
with
VHL
who
underwent
a
total
of
six
cortical
-sparing
procedures
without
requiring
steroid
therapy
.
Diseases
Validation
Diseases presenting
"cortical-sparing procedures"
symptom
von hippel-lindau disease
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