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Surgical management of pancreatic neuroendocrine neoplasms.
[von hippel-lindau disease]
Pancreatic
neuroendocrine
neoplasms
are
relatively
rare
entities
,
representing
approximately
1
%
to
2
%
of
all
pancreatic
tumors
.
Owing
to
their
rarity
as
well
as
their
relatively
indolent
natural
history
,
treatment
approaches
are
not
yet
standardized
.
A
formal
pancreatic
resection
is
usually
mandatory
for
large
and
localized
sporadic
pancreatic
tumors
or
in
the
presence
of
symptoms
.
However
,
in
small
and
asymptomatic
lesions
,
a
conservative
approach
consisting
in
a
careful
wait-and-see
policy
is
going
to
appear
as
more
appropriate
,
particularly
when
,
to
remove
the
lesion
,
an
aggressive
surgical
procedure
is
required
,
such
as
pancreaticoduodenectomy
or
distal
splenopancreatectomy
,
depending
on
the
localization
of
the
tumor
.
Surgery
has
also
a
significant
role
in
locally
advanced
and
metastatic
forms
.
In
the
setting
of
MEN
1
syndrome
or
Von
-
Hippel
Lindau
disease
,
the
tumor
size
and
the
possible
symptoms
should
be
considered
in
the
evaluation
of
a
proper
treatment
.
Diseases
Validation
Diseases presenting
"depending on the localization of the tumor"
symptom
von hippel-lindau disease
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