Cho, Kyu-Sup

Hong, Sung-Lyong

Lee, Chang-Hoon

Shin, Sung-Chan

Brazilian Journal of Otorhinolaryngology

Elsevier - Publisher Connector 

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araz J Otorhinolaryngol. 2016;82(5):614--617
www.bjorl.org
Brazilian Journal of
OTORHINOLARYNGOLOGY
ASE REPORT
rbital  metastasis  as  the primary  presentation  of
asopharyngeal carcinoma
etástase  orbital  como  apresentac¸ão  primária  de  carcinoma  de  nasofaringe
ung-Chan Shina, Sung-Lyong Honga, Chang-Hoon Leeb, Kyu-Sup Choa,∗
Pusan  National  University  Hospital,  Pusan  National  University  School  of  Medicine,  Department  of  Otorhinolaryngology  and
iomedical Research  Institute,  Busan,  South  Korea
Pusan  National  University  Hospital,  Pusan  National  University  School  of  Medicine,  Department  of  Pathology,  Busan,  South  Korea
eceived 17  March  2015;  accepted  27  April  2015
vailable  online  9  September  2015
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mass  and  right  cervical  lymph  node  (Fig.  1A).ntroduction
etastasis  to  the  orbit,  which  is  uncommon  due  to  the
haracter  of  the  orbital  volume  with  relative  stenosis,  is
stimated  to  account  for  1--13%  of  all  orbital  tumors.1
rbital  metastasis  is  believed  to  occur  in  approximately
--3%  of  patients  with  systemic  cancer.2 The  incidence
f  metastatic  orbital  tumors  varies  widely,  according  to
eographical  area  and  race;  the  most  common  primary  can-
ers  that  metastasize  to  the  orbit  are  breast,  prostate,
iver,  and  lung  cancer.1,2 Although  nasopharyngeal  carcinoma
NPC)  involves  the  orbits  through  direct  extension  to  the
rbital  apex,  metastasis  of  NPC  to  the  orbit  has  rarely  been
eported.  This  report  describes  two  cases  of  intraorbital,
xtrabulbar  metastases  from  NPC.
 Please cite this article as: Shin S-C, Hong S-L, Lee C-H, Cho K-S.
rbital metastasis as the primary presentation of nasopharyngeal
arcinoma. Braz J Otorhinolaryngol. 2016;82:614--7.
∗ Corresponding author.
E-mail: choks@pusan.ac.kr (K.-S. Cho).
a
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ttp://dx.doi.org/10.1016/j.bjorl.2015.04.006
808-8694/© 2015 Associac¸a˜o Brasileira de Otorrinolaringologia e Cirurgi
ccess article under the CC BY license (http://creativecommons.org/licase reports
ase  1
 52-year-old  male  with  abrupt-onset  hoarseness  visited  the
uthors’  clinic.  His  medical  history  was  otherwise  unremark-
ble.  A  1  ×  1  cm  hard,  ﬁxed  lymph  node  was  palpable  in  the
ight  level  II  area.  Flexible  ﬁberoptic  laryngoscopy  revealed
ight  vocal  cord  paralysis.  An  ulcerative  nasopharyngeal
ass  was  observed  by  nasal  endoscopy.  Computed  tomogra-
hy  (CT)  of  the  neck  revealed  an  enormous  nasopharyngeal
ass  extending  into  the  right  oropharyngeal,  masticator,
arotid,  prevertebral,  and  paravertebral  space,  with  mul-
iple  bilateral  cervical  lymph  nodes  metastases.  Transnasal
ndoscopic  biopsy  under  local  anesthesia  and  ﬁne  needle
spiration  on  the  right  level  II  lymph  node  were  per-
ormed.  Histopathologic  examination  revealed  keratinizing
quamous  cell  carcinoma  (SCC)  in  both  the  nasopharyngealThe  patient  received  docetaxel  and  cisplatin  chemother-
py.  However,  chemotherapy  was  stopped  after  the  ﬁrst
ycle  due  to  neutropenic  septic  shock.  Therefore,  early
a Ce´rvico-Facial. Published by Elsevier Editora Ltda. This is an open
enses/by/4.0/).
Orbital  metastasis  of  nasopharyngeal  carcinoma  615
Figure  1  Histopathologic  ﬁndings  of  case  1.  (A)  Inﬁltrating  keratinizing  carcinoma  of  the  nasopharynx  is  noted  beneath  normal
asop
ation
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wciliated columnar  pseudostratiﬁed  epithelium  (H&E,  ×200).  (B)  N
exhibits distinct  cytoplasmic  keratin  formation  with  pearl  form
radiotherapy  of  the  nasopharynx  and  cervical  lymph  nodes
(total  dose  =  70  Gy)  was  completed.  After  one  month,  mild
swelling  in  the  right  medial  canthal  area  was  noted.  Visual
acuity,  intraocular  pressure,  and  ocular  motility  were  within
normal  limits  and  there  was  no  exophthalmos.  Orbit  CT
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Figure  2  Computed  tomography  (CT)  and  magnetic  resonance  (MR
a soft  tissue  mass  with  indistinct  margins  on  the  inferomedial  side  o
mass displaying  mild  enhancement  on  post-contrast  T1  images  (C)  aharyngeal  keratinizing  carcinoma  inﬁltrating  orbital  soft  tissue
 (  )  (H&E,  ×400).
evealed  a  newly  developed  1.0  ×  0.9  cm  soft  tissue  mass
ith  indistinct  margins  on  the  inferomedial  side  of  the  right
rbit  (Fig.  2A  and  B).  Magnetic  resonance  (MR)  images  of
he  nasopharynx  revealed  an  oval-shaped  orbital  mass  dis-
laying  low  signal  intensity  on  T1-weighted  images  (T1WIs),
)  images  of  case  1.  Axial  (A)  and  coronal  (B)  CT  images  reveal
f  the  right  orbit  (  ).  MR  images  reveal  an  oval-shaped  orbital
nd  intermediate  signal  intensity  on  T2  images  (D).
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n16  
ntermediate  signal  intensity  on  T2WIs,  and  mild  enhance-
ent  on  gadolinium-T1WIs  (Fig.  2C  and  D).  This  was  followed
y  excisional  biopsy  that  conﬁrmed  the  diagnosis  of  ker-
tinizing  SCC  (Fig.  1B).  On  positron  emission  tomography
PET)/CT  scan,  multiple  areas  of  increased  ﬂuorodeoxyglu-
ose  (FDG)  uptake  were  detected  in  the  liver,  diagnosed  as
istant  liver  metastases.  Therefore,  the  patient  received  six
ycles  of  palliative  chemotherapy  with  TS-1  and  cisplatin.
lthough  primary  NPC  and  orbital  metastasis  did  not  recur,
he  patient  died  17  months  after  diagnosis  of  orbital  metas-
asis  due  to  liver  failure.
ase  2
 67-year-old  male  presented  with  abrupt-onset  ocular
ain  and  blurred  vision,  which  he  had  ﬁrst  noticed  one
onth  prior  to  presentation.  He  was  seen  by  a  neuro-
phthalmologist,  who  observed  visual  loss  to  no  light
erception  on  the  right.  The  patient’s  medical  history  was
therwise  unremarkable.  Nasal  endoscopy  revealed  a  mild
ontour  protrusion  without  mucosal  ulceration  or  necrosis  in
he  nasopharynx.  Neck  CT  revealed  a  soft  tissue  mass  in  the
xtraconal  and  intraconal  space  of  the  right  orbit.  Moreover,
symmetry  of  the  left  nasopharynx  and  metastatic  lymph
odes  were  present  in  the  left  level  II,  III,  IV,  and  retropha-
yngeal  areas  (Fig.  3).  On  PET/CT  scan,  the  main  mass  in
s
o
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igure  3  Computed  tomography  (CT)  images  of  case  2.  Axial  (A,  
he extraconal  and  intraconal  space  of  the  right  orbit.  Moreover,  asy
oted in  the  level  II  (black  arrow)  and  retropharyngeal  areas  (whiteShin  S-C  et  al.
he  right  orbit  was  of  a  hypermetabolic  nature  with  a  maxi-
um  standardized  uptake  value  (maxSUV)  of  3.5.  Abnormal
DG  uptake  was  present  in  multiple  left  neck  lymph  nodes,
dentical  to  CT  ﬁndings.  Transnasal  endoscopic  biopsy,  of
he  right  orbital  mass  and  left  nasopharyngeal  mucosa,
as  performed  under  general  anesthesia.  Histopathologic
xamination  of  both  specimens  revealed  undifferentiated
arcinoma  (Fig.  4).
The  patient  received  two  cycles  of  chemotherapy  with
ocetaxel  and  cisplatin.  However,  he  refused  further  treat-
ent  for  economic  reasons  and  died  six  months  after  orbital
etastasis  diagnosis.
iscussion
PC  is  a  tumor  that  arises  from  epithelial  cells  covering
he  surface  and  lining  of  the  nasopharynx.3 Three  subtypes
f  NPC  are  recognized  by  the  World  Health  Organization:
eratinizing  SCC,  nonkeratinizing  carcinoma,  and  undiffer-
ntiated  carcinoma.3 Although  NPC  commonly  metastasizes
o  cervical  lymph  nodes,  orbital  metastases  are  rare.  The
ajority  of  orbital  involvement  cases  involve  direct  inva-ion,  typically  via  the  pterygopalatine  fossa  and  inferior
rbital  ﬁssure,  but  occasionally  through  the  ethmoid  sinus
nd  sphenoid  sinus,  into  the  apex,  causing  proptosis  and
uscle  paralysis.4
C,  D)  and  coronal  (B)  images  reveal  a  soft  tissue  mass  (  )  in
mmetry  of  the  left  nasopharynx  and  metastatic  lymph  nodes  is
 arrow)  of  the  left  neck.
Orbital  metastasis  of  nasopharyngeal  carcinoma  617
Figure  4  Histopathologic  ﬁndings  of  case  2.  (A)  Inﬁltrating  undifferentiated  non-keratinizing  carcinoma  (*)  of  the  nasopharynx  is
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4noted beneath  normal  ciliated  columnar  pseudostratiﬁed  epithe
inﬁltrates orbital  soft  tissue  and  destroys  extraocular  striated  m
Orbital  metastatic  tumors  are  characterized  by  rather
abrupt  onset  of  diplopia,  blurred  vision,  and  pain.  A  visible
lump  may  also  be  present  beneath  the  eyelid  and  progress
is  relatively  rapid.  Examination  may  disclose  proptosis,  dis-
placement  of  the  globe,  blepharoptosis,  and  a  visible  or
palpable  mass.1,2 The  ﬁndings  in  the  present  cases  accord
with  previous  reports:  orbital  metastases  became  evident
ﬁve  months  after  NPC  diagnosis  in  one  patient  and  repre-
sented  the  ﬁrst  sign  of  NPC  in  the  other.
The  diagnosis  of  orbital  metastasis  should  be  suspected
when  a  patient  with  a  history  of  cancer  exhibits  the  afore-
mentioned  symptoms.  If  the  patient  has  no  history  of  cancer,
such  ﬁndings  should  prompt  a  systemic  survey  to  detect  a
primary  neoplasm  and  other  sites  of  metastasis.  Although
the  ultimate  diagnosis  of  orbital  metastasis  is  rendered  by
biopsy  or  ﬁne-needle  aspiration,  CT  or  MRI  orbital  imaging
studies  should  also  be  preformed.  CT  is  typically  employed
ﬁrst  because  it  provides  better  evaluation  of  bone.  However,
MRI  usually  provides  the  best  resolution  for  orbital  metas-
tasis  evaluation  because  the  majority  of  orbital  metastases
principally  affect  orbital  soft  tissues.  MRI  typically  reveals
an  inhomogeneous  low  signal  mass  on  T1  images,  increased
signal  intensity  on  T2  images,  and  a  degree  of  enhancement
with  contrast  agents.5 In  the  present  cases,  orbital  metasta-
sis  from  NPC  was  characterized  by  a  diffuse  or  well-deﬁned
soft  tissue  mass,  with  low  signal  intensity  on  T1WIs  and  inter-
mediate  signal  intensity  on  T2WIs  with  mild  enhancement.
The  main  aim  during  treatment  of  orbital  metastasis  is
to  alleviate  suffering  and  maintain  visual  function.  Radio-
therapy  is  the  mainstay  treatment  for  orbital  metastasis
from  NPC,  due  to  its  sensitivity,  but  chemotherapy  is  also
used  in  certain  patients.4 If  the  tumor  is  well-circumscribed
and  amenable  to  complete  removal,  it  should  be  treated
by  complete  excisional  biopsy.1 Prognosis  is  generally  poor,
5 (H&E,  ×200).  (B)  Nasopharyngeal  undifferentiated  carcinoma
es  (  )  (H&E,  ×400).
ecause  patients  are  typically  at  an  advanced  stage  of  dis-
ase.  The  overall  mean  survival  time  after  orbital  diagnosis
s  15  months;1 one  of  this  report’s  patients  died  after  17
onths,  the  other  after  six  months.
onclusion
lthough  NPC  with  orbital  metastasis  is  rare,  NPC  can
evelop  metastases  in  orbital  and  ocular  regions.  If  NPC
atients  complain  of  ophthalmological  symptoms  such  as
ocal  pain,  impaired  vision,  eyelid  swelling,  or  diplopia,  it  is
mportant  to  consider  orbital  or  ocular  metastatic  disease.
onﬂicts of interest
he  authors  declare  no  conﬂicts  of  interest.
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in Japan: a review of the literature. Ophthal Epidemiol.
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Orbital metastasis as the primary presentation of nasopharyngeal carcinoma 

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Orbital metastasis as the primary presentation of nasopharyngeal carcinoma

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