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A rare case of endolymphatic sac tumour: clinicopathologic study and surgical management.
[von hippel-lindau disease]
Objective
.
Endolymphatic
sac
tumor
(
ELST
)
is
a
rare
neoplasm
arising
from
the
intrapetrous
portion
of
the
endolymphatic
sac
,
either
isolated
or
in
association
with
the
von
Hippel-
Lindau
disease
.
We
report
a
sporadic
case
of
ELST
with
an
overview
of
the
literature
and
a
discussion
of
clinic-radiological
,
histopathologic
,
and
surgical
findings
.
Case
Report
.
A
young
woman
presented
with
a
progressive
hearing
loss
in
the
left
ear
.
Otoscopy
showed
a
reddish
,
bleeding
hypotympanic
mass
.
CT
demonstrated
an
expansile
lytic
mastoid
lesion
extending
to
the
middle
ear
,
with
bone
erosion
.
MRI
confirmed
a
lesion
of
increased
signal
on
T
1
-
weighted
sequences
.
The
patient
underwent
a
canal
wall-down
tympanoplasty
with
complete
removal
of
the
tumor
.
Histopathology
was
consistent
with
a
papillary
ELST
.
Immunohistochemistry
was
positive
for
cytokeratin
and
chromogranin
A
.
Conclusion
.
This
paper
highlights
the
rarity
of
ELST
,
the
need
for
an
accurate
neuroradiological
and
immunohistochemical
study
at
the
early
stages
,
and
the
timeliness
of
surgical
treatment
.
