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4001
Objectives: To present a very
rare case of epithelial-
myoepithelial carcinoma (EMC) of
the nasopharynx and review the
literature.
Study design: Case presentation
and review of the English
literature.
Methods: PubMed literature
search
Results: A 62-year-old female
presented with 6-months history
of right nasal obstruction and
intermittent epistaxis. She was
found to have a posterior
nasopharyngeal mass that was
biopsied twice before EMC could
be diagnosed. She was then
taken for endoscopic tumor
debulking with adjuvant radiation
therapy.
Discussion: EMC is a rare
salivary gland tumor usually
affecting the parotid but can occur
in minor salivary glands. Only 8
cases of EMC of the sinonasal
tract have been reported.
Presentation usually involves
nasal obstruction and intermittent
epistaxis. The typical appearance
on exam is a smooth mucosal
swelling. The mean age at
presentation is the 5
th
decade with
a female predominance, which is
consistent with EMCs of the major
salivary glands. Radiology usually
shows a well-defined mass with
variable bony erosion. Diagnosis
depends on the surgical
pathologists ability to identify
epithelial ductal structures and
myoepithelial cells. EMC is
usually treated with wide local
excision (WLE) however, in the
nasopharynx, obtaining clear
margins is difficult, thus treatment
was augmented with adjuvant
radiation.
Conclusion: EMC is a very rare
salivary gland tumor and even
more rare in the sinonasal tract.
While this tumor is usually treated
with excision, in the nasopharynx,
clear margins are difficult to
achieve, thus, adjuvant radiation
therapy should be considered.
A Rare Case of Epithelial Myoepithelial Carcinoma of the Nasaopharynx
Bonnie Vorasubin, MD
a
; Arthur Wu, MD
a
; Chi Lai, MD
b
; Elliot Abemayor, MD, PhD
a
a
Division of Head and Neck Surgery, Department of Surgery, David Geffen School of Medicine at UCLA
b
Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA
(1 of which involved bilateral maxillary sinuses) and
2/8 cases reported EMC arising from the
nasopharynx. The average age of presentation was
54 years with a range of 22-70 years. There was a
female predominance with a female-to-male ratio of
2:1. The clinical presentation usually consisted of
nasal airway obstruction and intermittent epistaxis.
The exam usually revealed a friable, polypoid firm
mass with overlying smooth erythematous mucosa.
The mass was evaluated by CT in 6 cases and MRI
in 2 cases. In either modality, the typical
radiological finding was a non-to-faintly enhancing
well-defined soft tissue density usually without bony
destruction, though 3/9 cases noted EMC to exhibit
this destructive behavior. The diagnosis is
contingent on the histopathology under light
microscopy and confirmed with immunostains or
ultrastructural analysis under electron microscopy.
Under light microscopy, the epithelial cells exhibit
bimorphological differentiation into inner cuboidal
eosinophilic ductal cells and outer clear polygonal
cells. Each population of cells has a characteristic
immunostaining pattern summarized in Table 2.
The presented case lacked clear cells, thus making
the diagnostic interpretation more challenging
demonstrating the importance of
immunohistochemistry.
All cases were treated with WLE, while one case
was supplemented with XRT, which the literature
recommends if clear margins are unattainable or
the tumor size is >4cm. In the presented case, the
location of the EMC made clear surgical margins
difficult to obtain, thus the decision of the
institutions tumor board was to supplement
treatment with adjuvant XRT. 7/8 cases reported no
recurrence at follow up, which ranged from 12-55
mos. 1/8 cases did not mention outcome.
Recurrence rates have been reported at 30-50%
occurring 5 years from diagnosis while the
metastatic incidence is reported at 8-25% usually
occurring 15 years from diagnosis with the usual
site being the lungs. Given these very long time
frames, surveillance is recommended to extend to
20 years post diagnosis. Poor prognostic factors
are atypia in more than 20% of tumor cells and
aneuploidy.
The English literature was reviewed for existing
experiences with EMC of the sinonasal tract. A
pubmed search was conducted using the following
search terms: Nasal EMC, sinonasal EMC,
nasopharynx EMC
8 cases resulted from 1996-2008 which were
reviewed and are summarized in Table 1.
EMC are rare salivary gland tumors of low
malignant potential. There are 8 cases of EMC of
the sinonasal tract. These cases usually present
with nasal obstruction and epistaxis and the
diagnosis depends on the presence of epithelial
and myoepithelial cells on light microscopy and
confirmed with immunostains. While treatment is
usually WLE alone, in the case where clear margins
are difficult to obtain or the tumor is >4cm, adjuvant
XRT should be considered.
Epithelial-myoepithelial carcinomas (EMC) are very
rare salivary gland tumors comprising only 1% of all
salivary gland tumors. They were first described by
Donath et al in 1972 as a tumor of biphasic
differentiation into ductal and myeopithelial cells
and were thought to arise from the intercalated
ducts of salivary glands. They are a caricinoma of
low malignant potential with survival rates as high
as 93% at 5 years and 81% at 10 years. The
typical site of occurrence is in the major salivary
glands, more specifically the parotid; however,
these tumors have been reported to arise, in rare
cases, in other glandular tissue including breast,
lacrimal gland, trachea and the sinonasal tract.
INTRODUCTION
METHODS AND MATERIALS
Donath, K, Seifert, G, & Schmitz, R. (1972). [Diagnosis and ultrastructure of the tubular
carcinoma of salivary gland ducts. Epithelial-myoepithelial carcinoma of the intercalated ducts].
Virchows Archiv. A, Pathology, 356(1), 16-31.
Harada, H, Kashiwagi, S I, Fujiura, H, et al. (1996). Epithelial-myoepithelial carcinoma--report of
a case arising in the nasal cavity. The journal of laryngology & otology, 110(4), 397-400.
Imate, Y, Yamashita, H, Endo, S, et al. (2000). Epithelial-myoepithelial carcinoma of the
nasopharynx. ORL, 62(5), 282-5.
Jin, X L, Ding, C N, & Chu, Q. (1999). Epithelial-myoepithelial carcinoma arising in the nasal
cavity: a case report and review of literature. Pathology, 31(2), 148-51.
Kuran, G, Sagt, M, Akn, I, et al. (2008). Bilateral Epithelial-Myoepithelial Carcinoma: An
Extraordinary Tumor of the Paranasal Sinuses. Skull base, 18(2), 145-150.
Lee, H M, Kim, A R, & Lee, S H. (2000). Epithelial-myoepithelial carcinoma of the nasal cavity.
European archives of oto-rhino-laryngology, 257(7), 376-8.
Pradhan, SA, Khannan, R, Hazarika, B, et al. (2007) Sinonasal Epithelial-myoepithelial
carcinoma - A rare entity. Indian J Orolaryngol head neck surg, 59, 168-170
Seethala, R, Barnes, E L, & Hunt, J L. (2007). Epithelial-myoepithelial carcinoma: a review of the
clinicopathologic spectrum and immunophenotypic characteristics in 61 tumors of the salivary
glands and upper aerodigestive tract. The American journal of surgical pathology, 31(1), 44-57.
Sunami, K, Yamane, H, Konishi, K, et al. (1999). Epithelial-myoepithelial carcinoma: An unusual
tumor of the paranasal sinus. ORL, 61(2), 113-6.
Yamanegi, K, Uwa, N, Hirokawa, M, et al. (2008). Epithelial-myoepithelial carcinoma arising in
the nasal cavity. Auris nasus larynx, 35(3), 408-13.
CONCLUSIONS
Discussion
REFERENCES
Table 1. Summary of reviewed Cases.
Figure 2. Pictomicrographs of the nasopharyngeal biopsy specimen. A 100X H&E
stain. Note overlying sinonasal respiratory type mucosa (arrowheads). B 400x
H&E stain. Note the presence of moderatre cellular and nuclear atypia with
scattered mitotic figures (arrow) C 400x EMA immunostain. D 400x p63
immunostain.
Figure 1. Axial images on MRI (A) and PET/CT (B) of the presented case.
ABSTRACT
Nopawan Vorasubin, M.D.
David Geffen School of Medicine
Email: nvorasubin@mednet.ucla.edu
CONTACT
A 62-year-old female presented with a 6 months
history of right nasal airway obstruction and
intermittent epistaxis. On physical exam, she was
found to have a large mass in the right
nasopharynx. The mass was biopsied twice before
a diagnosis of EMC could be confirmed. An MRI
and PET/CT where done post biopsy that ruled out
distant disease (Figure 1). She was taken for
endoscopic cytoreductive surgery and referred for
adjuvant radiation therapy (XRT). At 6 months
follow up, she had no evidence of recurrent disease
(NED).
Case
Paper Pt Sxs Site Exam Rads Tx Outcome
Harada 56M NAO,
bloody
nasal d/c
L septum yellow-white
polypid friable
smooth mass
CT: soft tissue
density. No bony
destruction
WLE NED
Jin 62F NAO,
recurrent R
epistaxis.
R NP/NC mass covered w/
red congested
mucosa
CT: soft tissue
density, well
defined, no bony
destruction
WLE NED
x20mos
Sunami 65F R NAO R max
sinus
firmfixed mass
w/overlying
nonulcerated pale
mucosa
CT: large soft
tissue mass w/
bony wall
destruction
WLE NED
x24mos
Imate 68F AD otalgia R NP NP swelling MRI: sharply
defined margins
WLE NED
x55mos
Lee 22M NAO,
recurrent
epistaxis
R NC friable polypoid
mass
CT: soft tissue
mass w/ bony
destruction
WLE
+ XRT
NED
x44mos
Pradhan 29M L facial
swelling, L
NAO, L
epistaxis
L NC reddish polypoid
mass
CT: faintly
enhancing mass
WLE No mention
Yamanego 70F L recurrent
epistaxis
L NC hemorrhagic
polypoid soft
tumor
CT: tumor
destroying inferior
turbinate. No bony
destruction
WLE NED
x12mos
Kuran 54F R facial
swelling/pa
in
B max
sinus
firmfixed mass MRI: soft tissue
w/ bony
destruction
WLE NED
x30mos
A B
Cell type Inner cuboidal cell Outer clear cell
Immunostain Cytokeratin AE1/AE3
EMA
SMA
S100
P63
Conclusion Epithelial ductal Myoepithelial
Histopathology
The second nasopharyngeal biopsy was reviewed
and representative pictomicrographs are shown
(Figure 2). On H&E stain, the tumor appears to be
located in the deep submucosa with well-
circumscribed borders. The neoplastic cells form
islands and large nests of tubular structures, the
latter of which are surrounded by dense hyalinized
eosinophilic basement membrane (Figure 2A). On
higher magnification, the neoplastic cells
demonstrate moderate cellular and nuclear atypia
with scattered mitotic figures (Figure 2B). Unlike
typical EMCs that exhibit characteristic biphasic
cellular differentiation, which will be discussed later,
this particular case did not and a diagnosis of EMC
was evident only on immunohistochemical stains.
The inner luminal epithelial cells stained brightly
with EMA, a marker characteristic of ductal
epithelial cells (Figure 2C). The outer abluminal
cells were highlighted with P63 immunostain
(Figure 2D). The myoepithelial nature of these
cells were confirmed on SMA immunostain (not
shown). These staining patterns confirmed a
diagnosis of EMC
Literature Review
4/8 cases reported EMC arising from the nasal
cavity, 2/8 cases arose from the maxillary sinuses
Table 2. Summary of characteristic immunostaining patterns of each cell type in
EMC