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Retinoblastoma frontiers with intravenous, intra-arterial, periocular, and intravitreal chemotherapy.
[coats disease]
In
this
report
,
we
explore
retinoblastoma
diagnostic
accuracy
and
review
chemotherapy
alternatives
for
retinoblastoma
using
intravenous
,
intra-
arterial
,
periocular
,
and
intravitreal
routes
.
A
review
of
2775
patients
referred
for
management
of
retinoblastoma
,
disclosed
78
%
with
confirmed
retinoblastoma
and
22
%
with
simulating
lesions
,
termed
pseudoretinoblastomas
.
Children
≤
2
years
old
showed
leading
pseudoretinoblastomas
of
persistent
fetal
vasculature
,
Coats
disease
,
and
vitreous
haemorrhage
,
whereas
those
>
5
years
showed
simulators
of
Coats
,
toxocariasis
,
and
familial
exudative
vitreoretinopathy
.
The
diagnosis
of
retinoblastoma
should
be
established
before
planning
therapeutic
strategy
.
Chemotherapy
strategy
depends
on
tumour
laterality
and
stage
of
disease
.
If
bilateral
retinoblastoma
,
intravenous
chemotherapy
(
IVC
)
is
important
as
first
-line
therapy
for
control
of
intraocular
disease
,
prevention
of
metastasis
,
and
reduction
in
prevalence
of
pinealoblastoma
and
long
-term
second
malignant
neoplasms
.
Bilateral
groups
D
and
E
retinoblastoma
receive
additional
subtenon
's
carboplatin
boost
for
improved
local
control
.
If
unilateral
disease
is
present
,
then
intra-
arterial
chemotherapy
(
IAC
)
is
often
considered
.
IAC
can
be
salvage
therapy
following
chemoreduction
failure
.
Unilateral
retinoblastoma
of
groups
D
and
E
are
managed
with
enucleation
or
globe-conserving
IVC
and
/
or
IAC
.
Intravitreal
chemotherapy
is
cautiously
reserved
for
recurrent
vitreous
seeds
following
other
therapies
.
In
conclusion
,
the
strategy
for
retinoblastoma
management
with
chemotherapy
depends
on
tumour
laterality
and
stage
of
disease
.
Bilateral
retinoblastoma
is
most
often
managed
with
IVC
and
unilateral
retinoblastoma
with
IAC
,
but
if
advanced
stage
,
combination
IVC
plus
IAC
or
enucleation
.
Diseases
Validation
Diseases presenting
"stage of disease"
symptom
adrenomyeloneuropathy
coats disease
dedifferentiated liposarcoma
gm1 gangliosidosis
hodgkin lymphoma, classical
liposarcoma
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