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A rare case of sinonasal glomangiopericytoma post operative accidental diagnosis and managment - A case report

Authors:
Nitin Sharma at Northeastern Illinois University
Nitin Sharma
Dushyant Mandlik at HCG Cancer Center, Ahmedabad
Dushyant Mandlik
  • HCG Cancer Center, Ahmedabad
Purvi Patel
Purvi Patel
Purvi Patel
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Parin Patel
Parin Patel
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Abstract and Figures

Introduction: Glomangiopericytoma is a rare neoplasm of low malignant potential. It is a rare type of haemangiopericytoma located in nasal cavity. This neoplasm has good prognosis and complete surgical excision is treatment of choice. This case report is representing one such neoplasm. This reporting is done in line with the SCARE criteria (Agha et al., 2018 [1]). Case presentation: We presenting a case of 54 year old male patient from upper socioeconomic status who presented at our institution with history of surgery (endoscopic sinus surgery with Septoplasty) 15 days back. A Final histopathology report suggested glomangiopericytoma. Since primary surgery was not done as per oncologic principals, patient was advised for adjuvant radiation. At our institute patient was evaluated again. Revision surgery was done. Patient was discharged next day. Discussion: Glomangiopericytoma is a rare neoplasm with incidence of less than 0.5% of all neoplasms of sinonasal cavity. Prognosis is very good after complete surgical excision. It often confuses clinicians with nasal polyps. Here also patient was operated initially considering as benign polyposis outside. Patient was re-operated again to ensure the complete clearance. Conclusion: This is the typical case of converting dual modality treatment to single modality with the help of knowledge, communication, transparent team work. This also a rare type of neoplasm and by reporting this rare case we are contributing to data pool of nasal tumors where lack of reporting is major obstacle in the formation of uniform treatment guidelines.
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CASE
REPORT
–
OPEN
ACCESS
International
Journal
of
Surgery
Case
Reports
62
(2019)
54–57
Contents
lists
available
at
ScienceDirect
International
Journal
of
Surgery
Case
Reports
j
ourna
l
h
om
epage:
www.casereports.com
A
rare
case
of
sinonasal
glomangiopericytoma
post
operative
accidental
diagnosis
and
managment—A
case
report
Nitin
Sharmaa,∗,
Dushyant
Mandlika,
Purvi
Patela,
Parin
Patela,
Aditya
Joshipuraa,
Mitesh
Patela,
Srinal
Mankiwalaa,
Ashutosh
Vatsyayana,
Tulika
Dubeya,
Kintan
Sanghvia,
Diva
Shaha,
Shubhada
Kanherea,
Shailesh
Talatib,
Kaustubh
Patela
aHCG
Cancer
Center,
Sola,
Ahmedabad,
Gujarat,
India
bHemato
Oncology
Clinic,
Ahmedabad,
Gujarat,
India
a
r
t
i
c
l
e
i
n
f
o
Article
history:
Received
25
April
2019
Received
in
revised
form
27
June
2019
Accepted
30
June
2019
Available
online
9
August
2019
Keywords:
Glomangiopericytoma
Haemangiopericytoma
Nasal
Tumor
Endoscopic
sinus
surgery
a
b
s
t
r
a
c
t
INTRODUCTION:
Glomangiopericytoma
is
a
rare
neoplasm
of
low
malignant
potential.
It
is
a
rare
type
of
haemangiopericytoma
located
in
nasal
cavity.
This
neoplasm
has
good
prognosis
and
complete
surgical
excision
is
treatment
of
choice.
This
case
report
is
representing
one
such
neoplasm.
This
reporting
is
done
in
line
with
the
SCARE
criteria
(Agha
et
al.,
2018
[1]).
CASE
PRESENTATION:
We
presenting
a
case
of
54
year
old
male
patient
from
upper
socioeconomic
status
who
presented
at
our
institution
with
history
of
surgery
(endoscopic
sinus
surgery
with
Septoplasty)
15
days
back.
A
Final
histopathology
report
suggested
glomangiopericytoma.
Since
primary
surgery
was
not
done
as
per
oncologic
principals,
patient
was
advised
for
adjuvant
radiation.
At
our
institute
patient
was
evaluated
again.
Revision
surgery
was
done.
Patient
was
discharged
next
day.
DISCUSSION:
Glomangiopericytoma
is
a
rare
neoplasm
with
incidence
of
less
than
0.5%
of
all
neoplasms
of
sinonasal
cavity.
Prognosis
is
very
good
after
complete
surgical
excision.
It
often
confuses
clinicians
with
nasal
polyps.
Here
also
patient
was
operated
initially
considering
as
benign
polyposis
outside.
Patient
was
re-operated
again
to
ensure
the
complete
clearance.
CONCLUSION:
This
is
the
typical
case
of
converting
dual
modality
treatment
to
single
modality
with
the
help
of
knowledge,
communication,
transparent
team
work.
This
also
a
rare
type
of
neoplasm
and
by
reporting
this
rare
case
we
are
contributing
to
data
pool
of
nasal
tumors
where
lack
of
reporting
is
major
obstacle
in
the
formation
of
uniform
treatment
guidelines.
©
2019
Published
by
Elsevier
Ltd
on
behalf
of
IJS
Publishing
Group
Ltd.
This
is
an
open
access
article
under
the
CC
BY
license
(http://creativecommons.org/licenses/by/4.0/).
1.
Case
This
is
a
case
of
65
year
old
male
who
presented
in
outpa-
tient
department
in
tertiary
care
institute.
He
was
known
case
of
bronchial
asthma
and
had
history
of
myocardial
infarction
seven
years
back.
Presently
he
was
on
anti-platelet
medication.
Patient
was
operated
outside
for
septoplasty
and
functional
endoscopic
sinus
surgery
(FESS)
25
days
back.
Patient
was
referred
by
medical
oncologist
for
surgical
opinion
about
further
management.
Present-
ing
symptoms
were
pain
in
nasal
cavity
with
occasional
bloody
discharge.
The
previous
surgery
was
done
by
otolaryngologist.
CT
scan
done
prior
to
the
first
surgery
was
reported
as
polypoidal
mucosal
thickening
in
sphenoid
sinus
with
complete
opacifica-
tion.
There
was
hyperdensity
without
any
abnormal
enhancement
but
mild
focal
extension
of
soft
tissue
was
seen
in
sphenoeth-
moidal
recess
left
side
and
protruding
into
nasopharynx.
There
∗Corresponding
author.
E-mail
address:
nitinentkota@yahoo.com
(N.
Sharma).
was
neither
erosion
of
seller
floor
nor
intracranial
extension.
No
histopathology
report
was
done
prior
to
the
surgery.
The
final
histopathology
report
after
the
surgery
was
reported
as
respiratory
mucosa
with
a
submucosal
tumor
with
plasmacytoid
and
focally
spindled
morphology
in
myxoid
to
vascularized
stroma
and
differ-
ential
diagnosis
was
given
as
minor
salivary
gland
neoplasm,
nasal
tumor
and
extraosseous
myeloma
(Figs.
1
and
2).
His
Immunohistocytochemistry
(IHC)
was
reported
positive
for
SMA
but
negative
for
desmin,CD-34,
S-100
protein,
cytokeratin
and
EMA
and
concluded
as
glomangiopericytoma.
Immunohisto
cyto-
chemistry
(IHC)
was
done
at
one
more
place
where
tumor
was
reported
positive
for
SMA
and
beta
catenin
but
negative
for
synap-
tophysin,
P40,
P63,
S100,
CK7,
MUM1,
DESMIN,
CD
99,
CD34
and
CD31
and
concluded
as
borderline/low
grade
malignant
soft
tissue
tumor:
sinonasal
glomangiopericytoma.
After
this
report
patient
was
referred
to
the
radiation
oncologist
for
adjuvant
radiation
as
primary
surgeon
was
not
sure
of
complete
excision.
Through
a
com-
mon
medical
oncologist
friend
patient
was
referred
to
us.
At
our
centre
on
examination
we
found
operated
cavity
full
of
granula-
tion
tissue.
It
was
difficult
to
establish
complete
surgical
excision
https://doi.org/10.1016/j.ijscr.2019.06.066
2210-2612/©
2019
Published
by
Elsevier
Ltd
on
behalf
of
IJS
Publishing
Group
Ltd.
This
is
an
open
access
article
under
the
CC
BY
license
(http://creativecommons.org/licenses/
by/4.0/).
CASE
REPORT
–
OPEN
ACCESS
N.
Sharma
et
al.
/
International
Journal
of
Surgery
Case
Reports
62
(2019)
54–57
55
Fig.
1.
Respiratory
epithelium
and
subepithelial
sheets
of
oval
tumor
cells
arrange
themselves
around
blood
vessels.
(H&E
X
40).
Fig.
2.
Oval
tumor
cells
around
blood
vessels
show
very
minimal
atypia.
(H&E
X
100).
Fig.
3.
Post
first
surgery
magnetic
resonance
imaging
with
contrast
coronal
section
of
paranasal
sinus.
clinically
which
was
very
important
in
this
case.
Patient
underwent
MR
scan
to
rule
out
the
presence
of
any
residual
disease.
The
MRI
was
reported
as
ill
defined
low
signal
intensity
areas
in
posterior-
superior
aspect
of
left
nasal
cavity
and
sphenoethmoidal
recess
most
likely
the
postoperative
granulation
tissue/fibrosis.
Still
they
advised
for
clinical
correlation
(Figs.
3–5).
Now
the
challenges
were.
1
We
wanted
to
avoid
radiation
but
that
can
be
done
only
after
the
establishment
of
complete
clearance.
2
Radiologists
were
not
sure
about
complete
excision.
3
It
was
very
difficult
to
differentiate
between
the
residual
disease
and
postoperative
granulation
tissue
clinically.
Fig.
4.
Post
first
surgery
magnetic
resonance
imaging
with
contrast
axial
section
of
paranasal
sinus.
Fig.
5.
Endoscopic
image
after
first
surgery
and
before
second
(revision)
surgery
showing
granulation
tissue
at
posterior
part
of
septum,
floor
of
sphenoid
sinus
and
adjacent
lateral
nasal
wall.
4
One
opinion
was
to
wait
till
granulation
tissue
heals
but
in
that
case
patient
was
losing
the
golden
time
for
commencement
of
radiation.
5
Going
again
in
recently
operated
cavity
is
itself
challenge
but
only
complete
clearance
offers
the
best
chance
of
cure.
All
these
issues
were
discussed
in
tumor
board
where
it
was
decided
to
establish
that
there
was
no
residual
tumor.
This
case
was
also
discussed
with
the
patient
and
his
son.
After
through
dis-
cussion
and
fully
informed
consent
it
was
decided
to
go
for
revision
surgery
with
frozen
section
to
achieve
360◦clearance.
As
per
plan
patient
was
operated
again
endoscopically
and
entire
granulation
tissue
was
excised
and
subjected
to
frozen
section
along
with
normal
mucosal
margins.
Frozen
section
was
reported
negative
for
residual
disease.
Entire
hospital
stay
was
uneventful
and
patient
was
discharged
on
second
postoperative
day.
Final
histopathology
report
was
negative
for
residual
disease
and
all
the
margins
and
tumor
bed
mucosa
was
free
from
tumor.
CASE
REPORT
–
OPEN
ACCESS
56
N.
Sharma
et
al.
/
International
Journal
of
Surgery
Case
Reports
62
(2019)
54–57
Fig.
6.
Endoscopic
image
after
one
year
(revision
surgery)
showing
well
healed
mucosa
in
nasal
cavity
at
posterior
part
of
septum,
floor
of
sphenoid
sinus,
and
adjacent
lateral
nasal
wall.
Patient
was
now
advised
for
observation
instead
on
adjuvant
radi-
ation.
Since
then
patient
is
in
regular
follow
with
for
last
9
months
with
us
and
he
is
disease
free.
Patient
had
also
underwent
post
sec-
ond
surgery
MRI
which
was
reported
negative
for
disease
(Fig.
6).
2.
Discussion
Any
mass
in
a
nasal
cavity
is
very
thrilling
for
endoscopic
sur-
geon.
Mild
symptoms
and
causal
approach
of
both
clinicians
and
patient
create
room
for
surprises.
Most
of
the
time
unilateral
soft,
insensitive
and
grayish
mass
is
nasal
polyp
but
not
always.
Here
we
present
a
case
where
a
nasal
mass
with
same
characteristics
was
operated
by
a
endoscopic
surgeon
considering
this
as
sinonasal
polyp
and
later
in
final
histopathology
report
it
turned
out
as
glo-
mangiopericytoma.
The
incidence
of
glomangiopericytoma
is
less
than
0.5%
of
all
sinonasal
tumors
[2].
Glomangiopericytoma
as
name
suggests
originates
from
pericytes
[2].
Pericytes
are
cells
in
the
walls
of
capillaries
[3].
Glomangioperi-
cytoma
behaves
as
indolent
tumor
and
confuses
the
clinicians
due
to
appearance
similar
to
inflammatory
polyps
[4].
The
first
case
was
reported
in
1942
by
Staut
and
Murray
and
was
classified
as
hemangiopericytoma
[5].
In
1905
WHO
recognised
this
as
a
separate
entity
[4].
As
ini-
tial
surgery
was
done
considering
lesion
as
inflammatory
polyp.
Although
it
was
done
with
computed
tomography
of
paranasal
sinus
but
without
contrast
study
and
without
prior
biopsy
of
nasal
mass.
No
intraoperative
frozen
section
was
sent.
Patient
and
relatives
were
not
explained
about
possibility
of
lesion
being
malignant
on
final
histopathology
and
a
second
wide
excision
or
adjuvant
treatment.
Final
histopathology
and
immunohistochem-
istry
turned
out
surprise
as
glomangiopericytoma.
Treatment
of
choice
for
glomangiopericytoma
is
complete
surgical
excision
of
tumor
[6]
and
adjuvant
treatment
is
generally
required
if
tumor
is
excised
incompletely.
In
series
of
104
patient
Thompson
has
reported
survival
rate
of
74.2
percent
at
5
years
with
recurrence
rate
is
17
percent
[7].
In
this
case
neither
tumor
margins
nor
com-
plete
excision
was
sure
that
is
why
adjuvant
treatment
was
advised
by
treating
surgeon.
To
avoid
the
adjuvant
treatment
and
morbidity
associated
with
it
with
the
consent
of
the
patient
revision
surgery
was
done.
In
second
surgery
complete
excision
with
clear
margins
was
ensured.
Thus
we
saved
the
patient
from
adjuvant
treatment
and
it’s
morbidity.
There
are
several
reasons
for
is
to
report
this
case.
On
reviewing
literature
we
did
find
too
many
cases
of
gloman-
giopericytoma.
Many
more
case
reports
are
required
to
generate
adequate
data
pool.
This
is
the
example
of
teamwork
where
the
final
goal
of
the
team
was
to
do
best
for
the
patient
and
we
did
it.
So
for
us
the
take
home
message
from
this
case
is
that
there
should
be
a
comprehensive
care
for
any
tumor
then
only
we
can
provide
best
to
the
patient.
3.
Conclusion
Glomangiopericytoma
is
rare
indolent
kind
of
neoplasm.
The
treatment
of
choice
for
glomangiopericytoma
is
surgery.
The
best
can
only
be
achieved
if
you
work
in
team.
More
and
more
reporting
of
cases
will
help
in
making
definitive
guidelines
for
the
treatment
for
glomangiopericytoma.
Funding
We
wish
to
confirm
that
there
has
been
no
significant
financial
support
for
this
work
that
could
have
influenced
its
outcome.
Ethical
approval
Not
applicable
for
this
case
report.
Consent
I
wish
to
confirm
that
the
written
and
informed
consent
from
the
patient
has
been
taken
from
the
patient
to
publish
this
case
report.
Author
contribution
Dr.
Nitin
Sharma-
performed
the
surgery.
Prepare
the
case
report.
Dr.
Dushyant
Mandlik
-
communication
with
the
patient
and
preparation
of
case
report.
Dr.
Purvi
Patel
-
post
operative
care.
Dr.
Parin
Patel
-
planning.
Dr.
Aditya
Joshipura-post
operative
care.
Dr.
Mitesh
Patel
-
post
operative
care.
Dr.
Srinal
Mankiwala
-
assistant
surgeon.
Dr.
Ashutosh
Vatsayayan
-
assistant
surgeon.
Dr.
Tulika
Dubey-
arranging
pathology
pictures.
Dr.
Kintan
Sanghvi
-
pathology
report
and
pictures.
Dr.
Diva
Shah
-
radiology
work
up
and
pictures.
Dr.
Shubhada
Kanhere
-
pathology
pictures.
Dr.
Shailesh
Talati
-
planing
and
management.
Dr.
Kaustubh
Patel
-
planning
the
management
and
concept.
Registration
of
research
studies
Not
applicable
for
this
case
report.
Guarantor
Dr.
Dushyant
Mandlik.
Provenance
and
peer
review
Not
commissioned,
externally
peer-reviewed.
CASE
REPORT
–
OPEN
ACCESS
N.
Sharma
et
al.
/
International
Journal
of
Surgery
Case
Reports
62
(2019)
54–57
57
Declaration
of
Competing
Interest
We
wish
to
draw
the
attention
of
the
Editor
to
the
following
facts
which
may
be
considered
as
potential
conflicts
of
interest
and
to
significant
financial
contributions
to
this
work.
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http://dx.doi.org/10.3332/
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[5]
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Panagiotis
Asimakopoulos,
et
al.,
Sinonasal
glomangiopericytoma:
is
anything
new?
Ear
Nose
Throat
J.
95
(2)
(2016)
E1–E6,
http://dx.doi.org/10.1177/
014556131609500202.
[7]
Lester
D.R.
Thompson,
et
al.,
Sinonasal-type
hemangiopericytoma,
Am.
J.
Surg.
Pathol.
27
(6)
(2003)
737–749,
http://dx.doi.org/10.1097/00000478-
200306000-00004.
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Access
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... 2,3 GPC tumors have low malignant potential and are reported to represent less than 0.5% of sinonasal neoplasms. [4][5][6][7][8][9] The definition of the disease has a complex history since the first report in 1942 describing this as hemangiopericytoma. 10 While initially thought to be within the spectrum of vascular tumors ranging from capillary hemangioma to glomus tumors, GPC has been classified as a distinct entity by the World Health Organization since 2005. ...
... 10 While initially thought to be within the spectrum of vascular tumors ranging from capillary hemangioma to glomus tumors, GPC has been classified as a distinct entity by the World Health Organization since 2005. 1,6,7,11,12 Based on early reports in the literature, cases were described as typically in older patients in their sixtieth and seventieth decades of life and commonly presented with nasal obstruction and/or epistaxis. 4,5,11 The standard treatment course is complete surgical excision, with the risk of recurrence described as low (16-17%). ...
A case report of sinonasal glomangiopericytoma: An important reminder to always collect specimen
Article
Full-text available
Objectives To present a case report of sinonasal glomangiopericytoma (GPC) in a female patient in her thirties and to highlight the importance of collecting pathology specimens even in routine sinus surgery cases. Methods A case report detailing the diagnosis of GPC in a female in her thirties, including her initial presentation, treatment, and follow-up, along with a brief review of the literature. Results Pathology of the collected specimen revealed sinonasal GPC along with chronic rhinosinusitis. Immunohistochemistry was positive for SMA, beta-catenin, and cyclin D1; and negative for STAT6, ERG, pankeratin, SOX10, and S100. Conclusion This diagnosis expands the knowledge around the demographic profile of GPC patients. GPC should be included in the differential diagnosis of sinonasal masses, even in younger patients. The case highlights the importance of collecting the entire pathology specimen in all cases, even of ones that seem routine and benign.
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... Recurrences are generally attributed to incomplete removal during initial surgery [84]. To reduce the likelihood of recurrence, adjuvant radiotherapy can be employed following surgery [89]. To date, no specific clinical trials have been conducted for SGP. ...
Integrated Molecular and Histological Insights for Targeted Therapies in Mesenchymal Sinonasal Tract Tumors
Article
Full-text available
  • Feb 2024
  • Curr Oncol Rep
Purpose of Review This review aims to provide a comprehensive overview of mesenchymal sinonasal tract tumors (STTs), a distinct subset of STTs. Despite their rarity, mesenchymal STTs represent a unique clinical challenge, characterized by their rarity, often slow progression, and frequently subtle or overlooked symptoms. The complex anatomy of the sinonasal area, which includes critical structures such as the orbit, brain, and cranial nerves, further complicates surgical treatment options. This underscores an urgent need for more advanced and specialized therapeutic approaches. Recent Findings Advancements in molecular diagnostics, particularly in next-generation sequencing, have significantly enhanced our understanding of STTs. Consequently, the World Health Organization has updated its tumor classification to better reflect the distinct histological and molecular profiles of these tumors, as well as to categorize mesenchymal STTs with greater accuracy. The growing understanding of the molecular characteristics of mesenchymal STTs opens new possibilities for targeted therapeutic interventions, marking a significant shift in treatment paradigms. Summary This review article concentrates on mesenchymal STTs, specifically addressing sinonasal tract angiofibroma, sinonasal glomangiopericytoma, biphenotypic sinonasal sarcoma, and skull base chordoma. These entities are marked by unique histopathological and molecular features, which challenge conventional treatment approaches and simultaneously open avenues for novel targeted therapies. Our discussion is geared towards delineating the molecular underpinnings of mesenchymal STTs, with the objective of enhancing therapeutic strategies and addressing the existing shortcomings in the management of these intricate tumors.
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... De expressie van SMA, een marker voor gladde spiercellen, is eveneens een aanwijzing voor de herkomst. Minder dan 0,5% van de sinonasale tumoren betreft een glomangiopericytoom. 4,5 Een glomangiopericytoom kan uitgaan van het septum, 1 zoals in deze casus werd beschreven, de neusholte 6 en de sinussen. 3,7,8 De etiologie is nog niet geheel duidelijk maar een associatie met trauma, hypertensie, zwangerschap en corticosteroïdgebruik wordt verondersteld. ...
Het glomangiopericytoom -een zeldzame oorzaak van recidiverende epistaxis
Article
Full-text available
  • Nov 2022
An 83-year-old patient was referred to the outpatient clinic for ear, nose and throat surgery at the Tergooi Hospital because of recurrent epistaxis. Anterior rhinoscopy showed a haemorrhagic poly-pous abnormality in the right nasal cavity. Imaging showed an extensive soft tissue mass, filling almost the entire nasal cavity, with suspicion of dehiscence of the lateral nasal wall and invasive growth. Histo-logical examination revealed a glomangiopericyto-ma. The patient was referred to the University Medical Center Utrecht for treatment. Per-operatively there were no signs of bone destruction, and the glomangiopericytoma was macroscopically radically resected via an uncomplicated endonasal tumour resection. Histological examination of the complete specimen did not show malignancy. Glo-mangiopericytoma is a rare sinonasal tumour, which should be considered in the differential diagnosis of recurrent epistaxis due to a polypous nasal abnormality. Performing a biopsy early in the work-up, allows this lesion (which has indolent biological behaviour) to be rapidly diagnosed.
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... [9][10][11] Cases of misdiagnosis as benign nasal polyps have been previously reported. 12 The management of SNGPC consists mainly of total surgical resection. This is typically performed endoscopically, 13 but open approaches via medial maxillectomy have also been reported in advanced cases. ...
Sinonasal Glomangiopericytoma with Prolonged Postsurgical Follow-Up
Article
Full-text available
  • Jul 2022
Sinonasal glomangiopericytoma is a rare vascular tumor of the respiratory epithelium. Treatment consists mainly of surgical resection, though there is no consensus regarding the use of adjuvant therapies or preoperative endovascular embolization. The postsurgical prognosis is favorable, though there is a high risk of delayed recurrence. Here, we present the case of a patient who underwent endoscopic resection of a sinonasal glomangiopericytoma and a review of the literature.
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Our Experience with Sinonasal Glomangiopericytoma in North India: A Case Series
Article
  • Jul 2023
  • Indian J Otolaryngol
Glomangiopericytoma (GPC) is a rare benign sinonasal tumor originating from Zimmerman's Pericytes surrounding capil-laries and accounting for less than 0.05% of all sinonasal tumors. Glomangiopericytoma has low malignant potential (5-10%) and is mostly diagnosed in the 6th or 7th decade of age with slight female preponderance. We presented here a case series of 5 patients with sinonasal GPC. This research was conducted at a tertiary healthcare centre in North India. In our case series, all the patients were evaluated and underwent endoscopic surgical resection. All patients underwent digital subtraction angiography (DSA) and preoperative embolization. The coblation technique used for haemostasis proved very effective and time-saving. All patients exhibited cytoplasmic SMA positivity (a marker of GPC) and CD34 negativity, while one patient exhibited a high Ki-67 index (> 10%), which is a predictor of aggressive tumor behavior. None of the patients showed any recurrence in follow-up. We recommend performing complete endoscopic surgical excision to prevent recurrence. The use of DSA, preoperative embolization, and intraoperative use of the coblation technique provides a cleaner surgical field and reduced operating time.
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A rare case of glomangiopericytoma in the nasal cavity: A case report in light of recent literature
Article
Full-text available
  • Apr 2022
Introduction and importance Glomangiopericytoma (GPC) is a rare sinonasal tumor that behaves benignly with a long overall survival rate. It accounts for fewer than 0.5% of all sinonasal tumors. Case presentation We report the case of a 64-year-old man who presented with recurrent episodes of epistaxis. Rhinoscopy revealed a left posterior nasal septal mass with active oozing. Computed tomography (CT) showed a well-defined soft tissue lesion in the left nasal cavity measuring 1.95 × 1.51 cm. Complete endoscopic resection was successfully performed. Histopathological findings favored the diagnosis of GPC as it revealed tumor cells positive for smooth muscle actin and β-catenin with immunopositivity for CD34. Clinical discussion Presenting symptoms of GPC are predominated by epistaxis and nasal obstruction. Since CT and MRI merely lead to a presumptive diagnosis, histopathological findings are indispensable. Complete surgical excision of GPC remains the treatment of choice with excellent prognosis, especially when immunohistochemistry is positive for actin and CD34 immunostaining is negative. Conclusion GPC is a rare indolent tumor of pericytes that has a macroscopic appearance of a nasal polyp, which may result in uncertainty in the initial diagnosis. In most cases, GPC warrants only local excision. This case report adds to the literature and helps galvanize the developing clinical guidelines for diagnosis and treatment.
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Sino-nasal hemangiopericytoma: a case series and systematic literature review
Article
Full-text available
  • Aug 2022
  • Eur Arch Oto Rhino Laryngol
Background Hemangiopericytoma is a rare tumor of the sino-nasal tract. Its clinical behavior is controversial. Whereas some describe an indolent course, others consider it to be an aggressive lesion with a tendency toward rapid local recurrence. Here, we describe our experience in the management of sino-nasal hemangiopericytoma (SN-HPC), comparing our experience with the current literature, and evaluating signs and tools to improve diagnosis and treatment. Methods All cases of SN-HPC between 2010 and 2020 were extracted and reviewed from our institutional electronic medical records. SN-HPC cases from PubMed and EMBASE between 2010 and 2020 were analyzed in a systematic literature review using the preferred reporting items for systematic review and meta-analysis (PRISMA) guidelines. Data regarding demographics, presentation, diagnosis, treatment, and outcome were collected. Results We identified four cases of SN-HPC in the nasal cavity in our institution and an additional 53 cases in previous reports. The mean age at the time of diagnosis was 59 years, with a 1.2:1 male to female ratio. SN-HPC mostly appears unilaterally, arising in the ethmoid sinus (42.1%). The most common presenting symptoms were epistaxis (47.3) and nasal obstruction (47.3%). Both computed tomography (CT) and magnetic resonance imaging (MRI) were required for diagnosis and for tailoring the treatment plan. Endoscopic surgical excision was used in 85.9% of the patients, and in 15.7%, an additional preoperative embolization was performed, which was associated with septal necrosis in one patient (2.6%). The recurrence rate was 7%. Conclusion Although previous reports attribute an aggressive tumoral behavior to SN-HPC, our experience and the literature review support a more indolent course with low recurrence rates following complete endoscopic resection. Preoperative embolization can be useful in certain cases, but due to potential complications, it should not be routinely indicated.
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Sinonasal Glomangiopericytoma: Review of Imaging Appearance and Clinical Management Update for a Rare Sinonasal Neoplasm
Article
Full-text available
  • Dec 2021
Introduction: Glomangiopericytoma (GPC) is a rare tumor in the nasal cavity or paranasal sinuses with low malignant potential. Initially deemed a hemangiopericytoma, in 2005 it was classified as a distinct entity by the World Health Organization (WHO). Case Presentation: A male patient in his early 60s presented with new-onset right arm and leg weakness/numbness, who was incidentally found to have a left ethmoid sinus mass with extension in the olfactory fossa. On CT and MRI, the mass enhanced with well-defined borders and eroded the bone, but without dural enhancement. The mass was surgically excised, and pathology confirmed the diagnosis of glomangiopericytoma by microscopic appearance and staining. Discussion: Glomangiopericytoma has less than 0.5% incidence of all neoplasms of the sinonasal cavity, making it rare. Most diagnosed patients are in their 6th or 7th decade of age, with a slight female predominance. Treatment is complete surgical excision, with excellent prognosis, although there is up to 17% local recurrence. Despite the non-specific appearance on CT and MRI, imaging can help provide differential diagnosis, tumor extent, size, and reassuring non-aggressive characteristics of the tumor prior to surgery. GPC tumors are relatively resistant to radiation and chemotherapy. Conclusion: It is important to recognize glomangiopericytoma in the differential of masses of the nasal cavities or paranasal sinuses, as they rarely warrant aggressive treatment beyond local excision. Each reported case of glomangiopericytoma helps to build guidance for imaging and treatment since GPC is rare and not well-represented in the medical literature.
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Sinonasal glomangiopericytoma treated with preoperative embolisation and endoscopic sinus surgery
Article
Full-text available
  • Nov 2016
Sinonasal glomangiopericytoma is a benign rare tumour of pericytes that accounts for less than 0.5% of all sinonasal tumours. It is an indolent tumour with a macroscopic appearance of common inflammatory polyps. We report the case of a 55-year-old male who presented with right nasal obstruction. CT and MRI examinations demonstrated a soft-tissue mass that obstructed mainly the right nasal cavity. Biopsy revealed glomangiopericytoma. The tumour was treated with preoperative embolisation followed by complete endoscopic resection. Very few cases have been reported to be treated in this way.
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What is a pericyte?
Article
Full-text available
  • Oct 2015
Pericytes, spatially isolated contractile cells on capillaries, have been reported to control cerebral blood flow physiologically, and to limit blood flow after ischaemia by constricting capillaries and then dying. Paradoxically, a recent paper dismisses the idea of pericytes controlling cerebral blood flow, despite confirming earlier data showing a role for pericytes. We show that these discrepancies are apparent rather than real, and depend on the new paper defining pericytes differently from previous reports. An objective definition of different sub-classes of pericyte along the capillary bed is needed to develop novel therapeutic approaches for stroke and disorders caused by pericyte malfunction. Pubmed: http://www.ncbi.nlm.nih.gov/pubmed/26661200
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An unusual mass of nasal cavity: recurrent glomangiopericytoma
Article
Full-text available
  • Jul 2015
Hemangiopericytomas are rare tumors of sinonasal region. In this article, we described a case of recurrent mass in right nasal cavity which presented with nasal obstruction and intermittent epistaxis. Patient had a surgical history on the same nasal region without any histopathological document. Abnormal bleeding was observed during surgery and mass was partially removed for histopathological investigation. The result was glomangiopericytoma of the sinonasal region. Although extended surgery was recommended to the patient including preoperative and perioperative measures for bleeding problem, patient preferred to follow-up rather than removal. The clinical progress and review of glomangiopericytoma have also been discussed.
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The SCARE 2018 statement: Updating consensus Surgical CAse REport (SCARE) guidelines
Article
  • Oct 2018
  • Int J Surg
Introduction: The SCARE Guidelines were published in 2016 to provide a structure for reporting surgical case reports. Since their publication, SCARE guidelines have been widely endorsed by authors, journal editors, and reviewers, and have helped to improve reporting transparency of case reports across a range of surgical specialties. In order to encourage further progress in reporting quality, the SCARE guidelines must themselves be kept up to date. We completed a Delphi consensus exercise to update the SCARE guidelines. Methods: A Delphi consensus exercise was undertaken. All members of the previous Delphi group were invited to participate, in addition to researchers who have previously studied case reports, and editors from the International Journal of Surgery Case Reports. The expert group was sent an online questionnaire where they were asked to rate their agreement with proposed changes to each of the 24 items. Results: 56 people agreed to participate and 45 (80%) invitees completed the survey which put forward modifications to the original guideline. The collated responses resulted in modifications. There was high agreement amongst the expert group. Conclusion: A modified and improved SCARE checklist is presented, after a Delphi consensus exercise was completed. The SCARE 2018 Statement: Updating Consensus Surgical CAse REport (SCARE) Guidelines.
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Sinonasal Glomangiopericytoma: Is Anything New?
Article
More than 100 cases of sinonasal hemangiopericytoma have been reported in the literature, but only a handful of cases of nasal glomangiopericytoma. In this article, we report a case of a nasal glomangiopericytoma that was treated with endonasal surgical excision. We also attempt to clarify the confusion that attends to the nomenclature surrounding the terms glomangiopericytoma and hemangiopericytoma, which are often used interchangeably. Although glomangiopericytomas are histologically similar to sinonasal hemangiopericytomas, they sometimes behave in a different clinical manner. To further enhance our understanding of nasal glomangiopericytomas, more cases need to be reported. This may improve our ability to establish specific treatment modalities for this type of neoplasm and to predict clinical outcomes.
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Sinonasal-Type Hemangiopericytoma
Article
  • Jun 2003
  • AM J SURG PATHOL
Sinonasal-type hemangiopericytoma is an uncommon upper aerodigestive tract tumor of uncertain cellular differentiation. We report 104 cases of sinonasal-type hemangiopericytoma diagnosed between 1970 and 1995 from the files of the Armed Forces Institute of Pathology. There were 57 females and 47 males ranging in age from 5 to 86 years (mean 62.6 years). The most common clinical presentation was airway obstruction (n = 57) and/or epistaxis (n = 54), with symptoms averaging 10 months in duration. The tumors involved the nasal cavity alone (n = 47) or also a paranasal sinus (n = 26), were polypoid, and measured an average of 3.1 cm. Histologically, the tumors were submucosal and unencapsulated and showed a diffuse growth with fascicular (n = 37) to solid (n = 50) to focally whorled (n = 7) patterns. The tumor cells were uniform in appearance with minimal pleomorphism and had spindle-shaped (n = 82) to round/oval (n = 18) nuclei with vesicular to hyperchromatic chromatin and eosinophilic to amphophilic to clear-appearing cytoplasm with indistinct cell borders. Multinucleated (tumor) giant cells were identified in a minority of cases (n = 5). Mitotic figures were inconspicuous and necrosis was absent. The tumors were richly vascularized, including staghorn-appearing vessels that characteristically had prominent perivascular hyalinization (n = 92). An associated inflammatory cell infiltrate that included mast cells and eosinophils was noted in the majority of cases (n = 87). The immunohistochemical profile included reactivity with vimentin (98%), smooth muscle actin (92%), muscle specific actin (77%), factor XIIIa (78%), and laminin (52%). Surgery was the treatment of choice for all of the patients; adjunctive radiotherapy was given to four patients. Recurrences developed in 18 patients within 1–12 years from diagnosis. Ninety-seven patients were either alive (n = 51, mean 16.5 years) or dead (n = 46, mean 9.6 years) but free of disease. Four patients had disease at the last follow-up: three died with disease (mean 3.6 years) and one patient is alive with disease (28.3 years). Recurrent tumor (17.8%) can be managed by additional surgery. The majority of sinonasal-type hemangiopericytomas behave in a benign manner with excellent long-term prognosis (88% raw 5-year survival) following surgery alone. Sinonasal-type hemangiopericytomas have a characteristic light microscopic appearance with an immunophenotypic profile resembling that of glomus tumors. Almost from its initial description by Stout and Murray as a tumor primarily composed of pericytic cells, 42 the diagnosis of hemangiopericytoma (HPC) as a specific tumor type has been questioned. This skepticism has been predicated on the absence of any differentiating features by light microscopy coupled with the fact that the diagnosis of HPC often rests on its architectural features, specifically the presence of ramifying or branching pattern of its vascular component. However, this “specific” vascular pattern is found in a wide array of neoplasms of divergent differentiation such that a diagnosis of HPC is made only after excluding other tumors with similar histologic features. To the argument that HPC does not exist as an independent entity 15 comes the identification of the solitary fibrous tumor (SFT), a tumor originally described as a pleural-based tumor but now known to occur throughout the body. 19,23,43 Solitary fibrous tumor shares a perivascular pattern and CD34-positive tumor cells with HPC, suggesting common cellular differentiation between these tumor types. The questionable existence of soft tissue HPC as a specific entity is similarly posed relative to sinonasal tract HPC. The reasons for this skepticism are multifactorial, not the least of which include differences in the light microscopic features and overall biologic behavior between these sinonasal tract lesions and soft tissue HPC. As a result, the specific direction of differentiation for the sinonasal tract HPCs is still uncertain as attested to by the “hemangiopericytoma-like” designation with the implication that these tumors are related yet distinct from soft tissue HPC. 8 We undertook this study in an attempt to clarify some of the controversial issues surrounding the sinonasal-type HPC, specifically trying to suggest its possible link to other perivascular lesions. As we will discuss, our preferred terminology for this tumor is sinonasal-type hemangiopericytoma (SNTHPC).
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Sinonasal-type hemangiopericytoma: A clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation
Article
  • Jul 2003
  • AM J SURG PATHOL
Sinonasal-type hemangiopericytoma is an uncommon upper aerodigestive tract tumor of uncertain cellular differentiation. We report 104 cases of sinonasal-type hemangiopericytoma diagnosed between 1970 and 1995 from the files of the Armed Forces Institute of Pathology. There were 57 females and 47 males ranging in age from 5 to 86 years (mean 62.6 years). The most common clinical presentation was airway obstruction (n = 57) and/or epistaxis (n = 54), with symptoms averaging 10 months in duration. The tumors involved the nasal cavity alone (n = 47) or also a paranasal sinus (n = 26), were polypoid, and measured an average of 3.1 cm. Histologically, the tumors were submucosal and unencapsulated and showed a diffuse growth with fascicular (n = 37) to solid (n = 50) to focally whorled (n = 7) patterns. The tumor cells were uniform in appearance with minimal pleomorphism and had spindle-shaped (n = 82) to round/oval (n = 18) nuclei with vesicular to hyperchromatic chromatin and eosinophilic to amphophilic to clear-appearing cytoplasm with indistinct cell borders. Multinucleated (tumor) giant cells were identified in a minority of cases (n = 5). Mitotic figures were inconspicuous and necrosis was absent. The tumors were richly vascularized, including staghorn-appearing vessels that characteristically had prominent perivascular hyalinization (n = 92). An associated inflammatory cell infiltrate that included mast cells and eosinophils was noted in the majority of cases (n = 87). The immunohistochemical profile included reactivity with vimentin (98%), smooth muscle actin (92%), muscle specific actin (77%), factor XIIIa (78%), and laminin (52%). Surgery was the treatment of choice for all of the patients; adjunctive radiotherapy was given to four patients. Recurrences developed in 18 patients within 1-12 years from diagnosis. Ninety-seven patients were either alive (n = 51, mean 16.5 years) or dead (n = 46, mean 9.6 years) but free of disease. Four patients had disease at the last follow-up: three died with disease (mean 3.6 years) and one patient is alive with disease (28.3 years). Recurrent tumor (17.8%) can be managed by additional surgery. The majority of sinonasal-type hemangiopericytomas behave in a benign manner with excellent long-term prognosis (88% raw 5-year survival) following surgery alone. Sinonasal-type hemangiopericytomas have a characteristic light microscopic appearance with an immunophenotypic profile resembling that of glomus tumors.
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