Ami, Mazita

Min Han, Kong

Sabir Husin Athar, Primuharsa Putra

Shi Nee, Tan

Elsevier - Publisher Connector 

Postauricular neurofibroma – a rare occurrence 

ARTICLE IN PRESS+Model
Braz J Otorhinolaryngol. 2016;xxx(xx):xxx--xxx
www.bjorl.org
Brazilian Journal of
OTORHINOLARYNGOLOGY
CASE REPORT
Postauricular  neuroﬁbroma  -- a rare  occurrence
Neuroﬁbroma  pós-auricular  --  uma  ocorrência  rara
Tan Shi Neea, Mazita Amib, Kong Min Hanc, Primuharsa Putra Sabir Husin Athard,∗
a Department  of  Otorhinolaryngology,  Head  &  Neck  Surgery,  School  of  Medicine,  KPJ  Healthcare  University  College,  Malaysia
b Ear,  Nose  &  Throat  --  Head  &  Neck  Consultant  Clinic,  KPJ  Klang  Specialist  Hospital/KPJ  Healthcare  University  College,  Selangor,
Malaysia
c Department  of  Otorhinolaryngology,  Head  &  Neck  Surgery,  Universiti  Kebangsaan  Malaysia  Medical  Center,  Kuala  Lumpur,
Malaysia
d Ear,  Nose  &  Throat  --  Head  &  Neck  Consultant  Clinic,  KPJ  Seremban  Specialist  Hospital/KPJ  Healthcare  University  College,
Negeri Sembilan,  MalaysiaReceived  18  June  2015;  accepted  14  September  2015
H
s
i
o
l
t
m
r
t
g
s
i
c
o
f
t
h
a
p
DIntroduction
Neuroﬁbromas  are  a  type  of  benign  tumor  developed  from
the  myelin  sheath  of  the  peripheral  nervous  system  and
frequently  seen  in  neuroﬁbromatosis  type  1  (NF1).  Neuroﬁ-
broma  of  the  head  and  neck  regions  is  not  uncommon.1
There  were  only  a  few  cases  of  isolated  external  ear  neu-
roﬁbroma  without  neuroﬁbromatosis  (NF)  reported,  as  they
rarely  affect  the  external  ear.1 It  is  believed  that  neuroﬁbro-
matosis  is  autosomal  dominant;  the  type  1  neuroﬁbromatosis
is  due  to  disruptive  mutation  of  the  NF1  gene,  which  codes
for  protein  neuroﬁbromin.
Case report
A  38-year-old  Malay  male  with  no  comorbidity  came  to  our
otorhinolaryngology,  head  and  neck  clinic  with  the  com-
plaint  of  a  right  postauricular  swelling  for  the  past  two  years.
 Please cite this article as: Nee TS, Ami M, Han KM, Athar PPSH.
Postauricular neuroﬁbroma -- a rare occurrence. Braz J Otorhino-
laryngol. 2015. http://dx.doi.org/10.1016/j.bjorl.2015.09.001
∗ Corresponding author.
E-mail: putrani@yahoo.co.uk (P.P. Sabir Husin Athar).
N
m
t
http://dx.doi.org/10.1016/j.bjorl.2015.09.001
1808-8694/© 2015 Associac¸ão Brasileira de Otorrinolaringologia e Ciru
reserved.e  was  more  concerned  about  the  cosmetic  disﬁguring.  The
welling  was  insidious  at  the  onset  and  progressively  increas-
ng  in  size.  It  did  not  cause  any  pain.  There  was  no  history
f  bleeding  from  the  swelling  and  no  history  of  any  such
esions  in  the  body.  No  signiﬁcant  family  history.  Examina-
ion  of  the  swelling  showed  presence  of  a  single  4  cm  ×  5  cm
ass,  ﬁrm,  with  regular  surface  over  the  post  auricular
egion.  Subsequently,  we  performed  a  surgical  removal  of
he  mass  for  this  patient  via  a  postauricular  approach  under
eneral  anesthesia.  The  mass  was  partially  adherent  to  the
kin.  After  removing  the  excess  skin  which  was  adherent  to
t,  the  entire  mass  was  completely  excised.  Histopathologi-
al  report  (Fig.  1)  revealed  that  the  lesion  was  composed
f  spindle-shaped  cells  with  fusiform  nuclei  arranged  in
ascicles.  No  cellular  atypia  or  necrosis  seen.  These  his-
ological  features  are  suggestive  of  neuroﬁbroma.  Patient
as  an  uneventful  recovery,  and  a  good  cosmetic  result  was
chieved.  There  is  no  recurrence  during  follow  up  for  the
ast  year.
iscussioneuroﬁbromas  are  a  benign  neoplasm,  derived  from  the
yelin  sheath  of  the  peripheral  nerve.  They  may  occur  in
he  presence  of  neuroﬁbromatosis,  a  hereditary  condition.2
rgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights
BJORL-274; No. of Pages 2
ARTICLE IN+Model
2  
F
a
a
p
n
c
b
v
p
m
p
c
l
n
o
n
o
i
d
f
v
o
b
d
o
a
m
s
f
m
r
t
o
w
b
s
a
c
a
f
F
N
h
b
e
c
r
a
r
a
o
C
T
R
1
2
3
4igure  1  Microphotograph  showing  spindle  shaped  cells
rranged  in  short  fascicles,  whirling  and  storiform  pattern.
There  are  two  types  of  neuroﬁbromatosis:  type  1  (NF1)
nd  type  2  (NF2).
NF1  is  an  autosomal  dominant  type,  which  commonly
resents  with  peripheral  nerve  sheath  tumors  called  the
euroﬁbromas.3 NF1  is  caused  by  a  change  in  a  gene  on  the
hromosome  17.  NF2  usually  grows  into  the  spinal  cord  or
rain  and  is  caused  by  a  change  in  chromosome  22.  Bilateral
estibular  schwannomas  of  the  acoustic  nerve  and  multi-
le  meningiomas  are  characteristic,  rarely  with  cutaneous
anifestations.3,4
There  are  two  types  of  neuroﬁbromas:  dermal  and
lexiform.  Dermal  neuroﬁbroma  is  also  called  discrete  or
utaneous  neuroﬁbroma.  They  usually  develop  during  ado-
escence  and  adulthood.  They  tend  to  involve  the  terminal
erves  and  may  be  numerous,  but  there  is  no  apparent  risk
f  malignant  transformation.  However,  despite  their  benign
ature,  they  may  cause  signiﬁcant  cosmetic  problems  and
ccasionally  require  removal.
Conversely,  plexiform  neuroﬁbromas  are  usually  congen-
tal.  They  developed  in  childhood  and  are  often  extending
eeply  along  the  nerves  and  may  involve  all  levels  of  skin,
ascia,  muscle,  bone  and  even  viscera.  They  can  become
ery  large  and  may  cause  functional  impairment,  with  risk
f  malignant  transformation  in  6%  in  these  tumors.4,5
Change  in  the  genetic  material  that  causes  NF1  or  NF2  can
e  inherited  from  a  parent,  thus  referred  to  as  autosomal
ominant,  or  may  occur  due  to  spontaneous  mutation.1 In
ur  case  report,  there  was  no  family  history.  Solitary  lesions
re  not  usually  associated  with  the  presence  of  systemic
anifestations,  unlike  multiple  lesions,  which  are  commonly
5 PRESS
Shi  Nee  T  et  al.
een  in  patients  with  NF  or  von  Recklinghausen’s  disease.4
In  our  case  report,  the  patient  did  not  have  any  other
eatures  of  NF1.  Most  tumors  caused  by  NF  require  no  treat-
ent;  however,  when  these  tumors  cause  pain,  growing
apidly,  disﬁguring  or  impairing  function,  they  may  need
reatment.
Ghosh  S.K  et  al.  (2008)  reported  a  case  of  neuroﬁbroma
f  the  external  ear  where  he  removed  the  entire  lesion  along
ith  excess  skin,  and  a good  cosmetic  result  was  achieved.1
Shaida  AM  et  al.  (2007)  also  reported  a  case  of  neuroﬁ-
roma  of  the  pinna  in  which  surgical  excision  provided  a
atisfactory  outcome  and  resulted  in  an  excellent  functional
nd  cosmetic  outcome.5
In  our  case,  swelling  over  the  postauricular  region  causing
osmetic  deformity  was  the  chief  complaint,  and  we  were
ble  to  obtain  a  satisfactory  result  with  no  recurrence  on
ollow  up  for  a  year.
inal comments
euroﬁbromas  are  commonly  seen  in  a  patient  with  a
istory  of  neuroﬁbromatosis.  In  rare  cases  of  solitary  neuroﬁ-
roma,  depending  on  their  size  and  site  of  lesion,  complete
xcision  is  possible  with  any  signiﬁcant  functional  and
osmetic  deformity.  Here,  we  reported  a  rare  case  of  neu-
oﬁbroma,  a  nerve  sheath  tumor  which  presented  to  us  with
 swelling  behind  the  ear,  which  caused  cosmetical  disﬁgu-
ing.  Complete  excision  via  the  postauricular  approach  gives
 satisfactory  result  with  no  recurrence,  as  was  achieved  in
ur  case.
onﬂicts of  interest
he  authors  declare  no  conﬂicts  of  interest.
eferences
. Ghosh SK, Chakraborty D, Ranjan R, Barman D. Neuroﬁbroma of
the external ear -- a case report. Indian J Otolaryngol Head Neck
Surg. 2008;60:289--90.
. Singh S, Tandon S, Lahiri A, Sharma S. Neuroﬁbroma of the Pinna.
IJOHNS. 2014;3:66.
. Muir D, Neubauer D, Lim IT, Yachnis AT, Wallace MR. Tumorigenic
properties of neuroﬁbromin-deﬁcient neuroﬁbroma Schwann
cells. Am J Pathol. 2001;158:501--13.
. Wu J, Williams JP, Rizvi TA, Kordich JJ, Witte D, Meijer D,
et al. Plexiform and dermal neuroﬁbromas and pigmentation are
caused by Nf1 loss in desert hedgehog-expressing cells. Cancer
Cell. 2008;13:105--16.
. Shaida AM, Yung MW. Neuroﬁbroma of the pinna. Ear Nose Throat
J. 2007;86:36--7.


file:///data/remote/core/dit/data/elsevier/pdf/d05/aHR0cDovL2FwaS5lbHNldmllci5jb20vY29udGVudC9hcnRpY2xlL3BpaS9zMTgwODg2OTQxNTAwMjM2MA==.pdf

Postauricular neurofibroma – a rare occurrence

Abstract

Similar works

Full text

Available Versions

Elsevier - Publisher Connector

Elsevier - Publisher Connector