AJR 2003; 180:1715-1722
© American Roentgen Ray Society
MR Imaging of Diverse Manifestations of Nasopharyngeal Carcinomas
Shy-Chyi Chin1,
Girish Fatterpekar2,
Cheng-Yu Chen1 and
Peter M. Som2,3
1 Department of Radiology, Tri-Service General Hospital, National Defense
Medical Center, Taipei, Taiwan.
2 Department of Radiology, The Mount Sinai Hospital, The Mount Sinai School of
Medicine of New York University, One Gustave Levy Pl., Box 1234, New York, NY
10029.
3 Department of Otolaryngology, The Mount Sinai Hospital, The Mount Sinai School
of Medicine of New York University, New York, NY 10029.
Received July 26, 2002;
accepted after revision November 14, 2002.
Address correspondence to P. M. Som.
Introduction
Nasopharyngeal cancer is a disease treated either by radiation therapy or
by combined radiation therapy and chemotherapy. Surgery does not play a
significant role in treatment, except to salvage nodal disease (and rarely the
primary tumor) that is unresponsive to irradiation. So far, no biochemical
markers have been identified that can either predict nasopharyngeal cancer or
estimate its severity. However, imaging studies play a crucial role in
delineating the tumor extent for planning initial treatment and then for
assessing treatment response. It is therefore important for the radiologist to
be aware of the various imaging appearances of nasopharyngeal cancers. This
pictorial essay illustrates some of the appearances of nasopharyngeal cancers
on MR imaging.
Epidemiologic and Clinical Aspects
Nasopharyngeal carcinoma is a multifactorial disease that may be the result
of interactions involving race, genetics, and environment. In addition,
regardless of histologic subtype, almost 100% of cases of nasopharyngeal
cancer have the Epstein-Barr virus–encoded RNA (nontranslated RNA that
is not associated with protein production). Familial clusters have been
reported, and there is a genetic susceptibility with HLA-A2, HLA-B17,
HLA-w46, and HLA-Bsin2
[1]. In Chinese patients,
cytogenetics has also shown a consistent loss of genetic material at loci
RAF-1 and D3 S3
[1]. In China, dietary factors
for nasopharyngeal cancer include nitrosamine-rich salted food. The
male-to-female ratio is 3:1, in contrast to other head and neck squamous cell
carcinomas that have a male-to-female ratio of 9:1
[1].
Although nasopharyngeal carcinoma can occur in all age groups, it is most
common in patients between 40 and 60 years old, and bimodal age peaks occur in
the second and sixth decades. In the United States, nasopharyngeal carcinoma
accounts for approximately 0.25% of all malignancies, and there is an age peak
among African Americans from 10 to 19 years old
[2]. In Southeast Asia,
nasopharyngeal cancers account for approximately 15–18% of all
malignancies, but childhood tumors are rare
[2]. However, in areas of
Africa, nasopharyngeal cancer accounts for 10–20% of childhood
malignancies [1].
Eighty-two percent of nasopharyngeal cancers arise in the posterolateral
recess of the pharyngeal wall (usually in the Rosenmüller's fossa), and
12% arise in the midline. In 6%, the nasopharyngeal mucosa appears normal at
endoscopy [1]. Between 60% and
72% of patients present with cervical nodal metastasis; however, there is no
direct relationship between tumor size and the presence of cervical nodal
metastases. Advanced disease is considered to be present when there are
findings of hearing loss, otalgia, or headache or evidence of cranial nerve
involvement (10–12%) [1].
At presentation, 5–11% of patients will have distant metastases, and
during the course of treatment, 50–60% of patients will develop
metastases. Almost 80% of metastases occur within 18 months after symptoms
first appear, and after the detection of distant metastases, the mean survival
is approximately 6 months
[1].
The World Health Organization classification of nasopharyngeal cancer
recognizes squamous cell carcinoma and nonkeratinizing carcinoma with two
subtypes: differentiated nonkeratinizing carcinoma and undifferentiated
carcinoma. However, more than one histologic type is present in 26% of the
cases.
The primary treatment is radiation therapy, but when induction chemotherapy
(5-fluorouracil cisplatin) is combined with radiation therapy, the incidence
of metastases drops from 34% (radiation therapy only) to approximately 19%
(radiation therapy plus chemotherapy). Similarly, disease-free survival rises
from 42% (radiation therapy only) to 69% (radiation therapy plus
chemotherapy), and the overall survival rises from 48% to 69%, respectively
[3,
4].
Features associated with a favorable outcome are female sex, an age younger
than 40 years at presentation, and findings of lymphoepithelioma at histology.
Features associated with a poor prognosis include symptoms for more than 1
year, findings of keratizing carcinoma at histology, lymph nodes in the lower
neck (supraclavicular fossa) that are positive for tumor, cranial nerve
involvement, and distant metastases. Features not appearing to have a
prognostic impact are unilateral or bilateral nodes in the upper neck, fixed
nodes, and involvement of skull base bone
[1,
5]. The two main staging
classifications for nasopharyngeal cancer, those of the American Joint
Committee on Cancer and Ho [6],
recognize these differences from the other head and neck squamous cell
carcinomas.
Nasopharyngeal tumors usually remain clinically silent when small and
confined to the nasopharynx. However, the locally aggressive nature of these
tumors makes adjacent structures vulnerable to invasion. Thus, extension into
the nasal cavity may present as epistaxis, nasal obstruction, or a nasal
quality to the voice. Extension into the eustachian tube may present as
hearing loss and serous otitis, whereas extension into the skull base with
involvement of the cavernous sinuses may present as headache and cranial nerve
palsies [7].
The clinical examination often provides information regarding the mucosal
extent of the tumor. However, deep extension and early skull base erosion may
not be easily assessed. In fact, as mentioned, 6% of nasopharyngeal cancer may
go undetected at endoscopy [8].
In contrast, imaging studies have a direct impact on treatment planning by
outlining the tumor extent not only on the mucosa but by delineating any deep
extension. Such accurate tumor mapping allows better tumor staging to be
achieved and better radiation ports to be designed
[9].
Imaging Features
In the adult, the normal imaging appearance of the nasopharynx consists of
a convex forward margin on either side of the midline posterior wall from the
longus capitis muscles. Just lateral to each muscle contour is the most
posterolateral recess of the upper nasopharynx, the Rosenmüller's fossa.
Ventral to each fossa is the soft-tissue prominence of the torus tubarius
containing the cartilaginous portion of the eustachian tube and the levator
veli palatini muscle. Ventral to the torus, between it and the posterior
margin of the medial pterygoid plate, is the opening of the eustachian tube.
Just lateral to this opening, arising from the pterygoid fossa between the
medial and lateral pterygoid plates, is the tensor veli palatini muscle. On
contrast-enhanced T1-weighted MR images, a thin enhancing mucosal line can be
seen (Fig. 1A). This line
should always be intact, and any violation of it indicates either the presence
of an aggressive infection or a tumor. In children and adolescents,
hyperplastic adenoidal tissue is present, always lying superficial to the
mucosal line. In children, reactive retropharyngeal lymphadenopathy is also
commonly seen (Fig. 1B).
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Fig. 1A. —Normal MR imaging appearance of nasopharynx. Axial
contrast-enhanced T1-weighted fat-suppressed MR image of healthy 15-year-old
girl obtained through nasopharynx shows longus capitis muscles (L),
Rosenmüller's fossa (small arrow), opening of eustachian tube
(large arrow), and torus tubarius (Tu) and tensor veli palatini (Te)
muscle. Note normal uniformly thin mucosal enhancement.
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Fig. 1B. —Normal MR imaging appearance of nasopharynx. Axial
contrast-enhanced T1-weighted fat-suppressed MR image of healthy 17-year-old
boy shows symmetric thickening of adenoidal tissue with curvilinear
enhancement (arrowhead) lying superficial to mucosa enhancement line.
Note reactive retropharyngeal lymph node (arrow).
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Focal or diffuse thickening of the nasopharyngeal mucosa and cervical
lymphadenopathy are the most common presentations of nasopharyngeal
carcinomas, both of which can be effectively shown on MR imaging
(Fig. 2). The lesions mostly
show hypointense to isointense signal intensity (relative to muscles) on
T1-weighted images, remain hypointense on T2-weighted images, and show
moderate to intense enhancement on contrast-enhanced sequences (Figs.
3A,
3B). In general, the
T2-weighted image is less than that of normal adenoidal tissue; however, often
this difference in signal intensity is not sufficient to confidently
differentiate between a carcinoma and normal adenoids. Biopsy remains the
final arbiter in these cases.
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Fig. 2. —56-year-old man with biopsy-proven nasopharyngeal cancer.
Axial contrast-enhanced T1-weighted fast spin-echo MR image obtained at level
of nasopharynx shows fullness and enhancement in region of left
Rosenmüller's fossa (arrow). Also seen is left metastatic
retropharyngeal lymph node (arrowhead). Note normal appearance of
nasopharynx on right side.
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Fig. 3A. —19-year-old man with biopsy-proven nasopharyngeal cancer.
Axial contrast-enhanced T1-weighted fast spin-echo MR image shows fairly
homogeneous nasopharyngeal mass (star). Although homogeneous nature
of enhancement does not always distinguish tumor from adenoidal hypertrophy
(B), such homogeneous appearance should alert one to possibility that
mass may be tumor. In addition, normal adenoidal hypertrophy in teenage
patient does not extend ventrally to nasal fossa, and bulky size of this mass
also should suggest that it is not routine adenoidal hypertrophy. Normal
longus capitis muscles are denoted by  .
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Fig. 3B. —19-year-old man with biopsy-proven nasopharyngeal cancer.
Axial T2-weighted MR image shows nasopharyngeal cancer to have
low-to-intermediate signal intensity. Signal intensity is lower than that
normally seen with adenoidal hypertrophy and suggests highly cellular nature
of mass. Ventral edge of mass (arrow) can be easily distinguished
from left inferior nasal turbinate.
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Because of its locally aggressive nature, nasopharyngeal cancer can
penetrate the tough pharyngobasilar fascia extending from the top of the
constrictor pharyngis superior muscle to the skull base. The tumor can also
directly breach the foramen of Morgagni in the pharyngobasilar fascia, a gap
in the upper ventral pharyngobasilar fascia through which the eustachian tube
and the levator veli palatini muscle extend. Nasopharyngeal cancer can also
violate the buccopharyngeal fascia as it encircles the pharyngeal muscles.
Once through this fascia, tumor can rapidly spread to the parapharyngeal,
masticator, and retropharyngeal spaces. Invasion of the skull base usually
occurs directly over the tumor site, and extension through the skull base most
often occurs via the foramen lacerum and the neural foramina of the middle
cranial fossa floor (Figs. 4A,
4B and
5A,
5B). Perineural tumor spread
primarily occurs after tumor has invaded into the pterygopalatine fossa, the
foramen ovale, and the hypoglossal canal. Further extension of tumor can
involve the orbit, cavernous sinus, and even the brain stem, all with a
concomitantly worse prognosis (Figs.
6A,
6B,
7,
8A,
8B,
9A,
9B).
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Fig. 4A. —35-year-old man with nasopharyngeal cancer and right hearing
impairment with extensive involvement of right masticator space and bilateral
involvement of oropharynx. Such involvement of oropharynx is considered to be
poor prognostic sign. Axial contrast-enhanced T1-weighted fast spin-echo MR
image shows enhancing nasopharyngeal tumor extending to contiguous portions of
right masticator space (short arrow). This indicates invasion through
buccopharyngeal fascia. Also note presence of bilateral retropharyngeal
metastatic lymph nodes (stars) and presence of fluid (long
arrow) in right mastoid cells. Complete obstruction of right eustachian
tube by tumor was present.
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Fig. 4B. —35-year-old man with nasopharyngeal cancer and right hearing
impairment with extensive involvement of right masticator space and bilateral
involvement of oropharynx. Such involvement of oropharynx is considered to be
poor prognostic sign. Coronal contrast-enhanced T1-weighted fast spin-echo MR
image shows extensive bilateral tumor involvement of oropharynx
(arrows). In this case, this represents both mucosal and submucosal
tumor spread.
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Fig. 5A. —59-year-old man with nasopharyngeal cancer after radiation
therapy. Posterolateral extension is most common route of nasopharyngeal
cancer spread. One of the most severe complications of nasopharyngeal cancer
is invasion of carotid sheath with pseudoaneurysm formation and eventual
blowout. Axial contrast-enhanced T1-weighted fast spin-echo MR image shows
well-defined enhancement of right internal carotid artery (arrow),
caliber of which is larger than normal left internal carotid artery.
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Fig. 5B. —59-year-old man with nasopharyngeal cancer after radiation
therapy. Posterolateral extension is most common route of nasopharyngeal
cancer spread. One of the most severe complications of nasopharyngeal cancer
is invasion of carotid sheath with pseudoaneurysm formation and eventual
blowout. Axial T2-weighted MR image shows flow void (arrow).
Angiogram confirmed that this was pseudoaneurysmal dilatation of internal
carotid artery. Such vascular complication is more common in patients who have
been treated with radiation therapy to head and neck region.
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Fig. 6A. —56-year-old man with regional nasopharyngeal cancer
recurrence. Axial contrast-enhanced T1-weighted fast spin-echo MR image
obtained at level of maxillary antrum shows enhancing mass in left nasopharynx
that has extended through sphenopalatine canal into pterygopalatine fossa
(arrow) and pterygomaxillary fissure (arrowhead).
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Fig. 6B. —56-year-old man with regional nasopharyngeal cancer
recurrence. Coronal contrast-enhanced T1-weighted fast spin-echo MR image
shows extension from pterygopalatine fossa cranially into region of inferior
and superior orbital fissures (arrows).
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Fig. 7. —42-year-old man with extensive nasopharyngeal cancer. Note
dominant mass in right cavernous sinus with involvement of ipsilateral foramen
ovale, V3 nerve enhancement, and sphenoid sinus invasion. Coronal
contrast-enhanced T1-weighted fast spin-echo MR image reveals invasion of
nasopharyngeal cancer into right cavernous sinus (arrows) with
encasement of internal carotid artery. Also note enhancing mass in sphenoid
sinus (star) and increased enhancement (compared with normal left
side) extending to right foramen ovale (arrowhead).
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Fig. 8A. —37-year-old man with long-standing history of nasopharyngeal
cancer who presented with recent complaints of tongue fasciculations after
radiation therapy. Axial contrast-enhanced T1-weighted fast spin-echo MR image
obtained through level of posterior fossa shows enhancement of small tumor in
posterior wall of nasopharynx (large arrow). This tumor also extends
to left nasopharyngeal wall and then laterally to involve left tensor and
levator veli palatini muscles (small arrows). Also note abnormal
enhancement in left hypoglossal nerve (arrowhead) from tumor in
posterior nasopharynx.
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Fig. 8B. —37-year-old man with long-standing history of nasopharyngeal
cancer who presented with recent complaints of tongue fasciculations after
radiation therapy. Axial T2-weighted MR image shows bright signal intensity
and posterior prolapse of left half of tongue (arrows), reflecting
early denervation atrophy.
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Fig. 9A. —50-year-old man with nasopharyngeal cancer and ataxia. Axial
contrast-enhanced T1-weighted fast spin-echo MR image shows destructive
enhancing nasopharyngeal mass invading clivus (black arrow) with
contiguous extension into ventral aspect of pons (white arrow).
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Fig. 9B. —50-year-old man with nasopharyngeal cancer and ataxia. Axial
T2-weighted MR image obtained at same level as in A reveals hypointense
mass (arrow) with surrounding vasogenic edema
(arrowheads).
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Nasopharyngeal cancers can occasionally be identified in unusual locations.
Although most often seen with tumors that have been previously irradiated,
such unusual sites can also occur with primary tumors. Thus, nasopharyngeal
cancer can be seen in the calvaria, dura, sellar, and suprasellar regions and
the brain stem (Figs. 10A,
10B and
11A,
11B). The current explanations
for these occurrences include radiation-induced fibrosis in both tumor and
adjacent normal tissue that causes obstruction of lymphatic channels and the
opening of collateral lymphatic drainage, incomplete irradiation portal
coverage of the tumor resulting in the persistent tumor invading further
distal sites, and possible hematogenous spread. The incidence of local,
regional, and systemic recurrences is associated with the primary tumor stage,
with tumors in an advanced stage at presentation being more likely to have a
late recurrence and to develop in unusual locations.
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Fig. 10A. —51-year-old man with nasopharyngeal cancer and visual field
deficits. Note tumor spread to sphenoid sinus, optic chiasm, pituitary fossa,
and optic tract. Sagittal reformatted contrast-enhanced T1-weighted MR image
from axial study shows contiguous enhancement of tumor in sphenoid sinus
(small arrow), pituitary fossa (arrowhead), and optic chiasm
(large arrow).
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Fig. 10B. —51-year-old man with nasopharyngeal cancer and visual field
deficits. Note tumor spread to sphenoid sinus, optic chiasm, pituitary fossa,
and optic tract. Axial fluid-attenuated inversion recovery MR image shows
hyperintense signal along proximal optic tracts bilaterally (arrows)
likely due to either tumor infiltration or radiation change.
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Fig. 11A. —13-year-old girl with nasopharyngeal cancer who was treated
with radiation therapy for more than 2 years. Findings of follow-up MR imaging
and clinical examination of nasopharynx were normal. Biopsy of involved
temporal bone documented undifferentiated carcinoma, identical to original
nasopharyngeal tumor. Metastatic spread to calvaria, adjacent dura, and
infratemporal fossa is presumably hematogeneous spread, although tumor seeding
may also account for dural disease. Coronal contrast-enhanced T1-weighted fast
spin-echo MR image shows enhancement of dura, calvaria, and overlying scalp
(arrows).
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Fig. 11B. —13-year-old girl with nasopharyngeal cancer who was treated
with radiation therapy for more than 2 years. Findings of follow-up MR imaging
and clinical examination of nasopharynx were normal. Biopsy of involved
temporal bone documented undifferentiated carcinoma, identical to original
nasopharyngeal tumor. Metastatic spread to calvaria, adjacent dura, and
infratemporal fossa is presumably hematogeneous spread, although tumor seeding
may also account for dural disease. Axial contrast-enhanced T1-weighted fast
spin-echo MR image shows tumor involving dura, calvaria, and scalp
(arrows).
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As mentioned, in 12% of cases, the nasopharyngeal cancer arises in the
midline nasopharynx. It can be predominantly submucosal or present as mucosal
thickening (Figs. 12A,
12B,
13A,
13B,
14). Clinical evaluation of
these patients may be easily confused with benign lesions such as a Thornwaldt
cyst or a retention cyst. Also, aggressive nasopharyngeal cancers can spread
into the middle ear cavity and the jugular fossa
[10,
11] (Figs.
15A,
15B and
16A,
16B).
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Fig. 12A. —66-year-old man with atypical presentation of nasopharyngeal
cancer. Axial contrast-enhanced T1-weighted fast spin-echo MR image obtained
at level of nasopharynx shows lobulated enhancing mass involving midline
nasopharynx with normal-appearing Rosenmüller's fossa.
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Fig. 12B. —66-year-old man with atypical presentation of nasopharyngeal
cancer. Coronal contrast-enhanced T1-weighted fast spin-echo MR image shows
mass (arrows) "hanging" in asymmetrical configuration
from roof of nasopharynx. Asymmetric appearance should alert radiologist that
this finding may not be residual adenoidal tissue and that biopsy should be
suggested if clinically indicated.
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Fig. 13A. —24-year-old man with sensation of nasal and aural fullness
and nasal-sounding voice caused by nasopharyngeal cancer. Axial T2-weighted MR
image shows lobulated homogeneous right-sided mass of fairly low signal
intensity (star). Mass extends from deep relative to nasopharyngeal
mucosa laterally into parapharyngeal space. Also, note fluid with right
mastoid air cells as result of obstruction to eustachian tube
(arrow).
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Fig. 13B. —24-year-old man with sensation of nasal and aural fullness
and nasal-sounding voice caused by nasopharyngeal cancer. Coronal
contrast-enhanced T1-weighted fast spin-echo MR image shows mass
(star) to have homogeneous enhancement.
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Fig. 14. —24-year-old man with left IX and X and bilateral XII cranial
nerve palsies. Note that although mucosal component is fairly symmetric in
appearance and could represent adenoidal tissue, there is enhancement of
longus capitus muscles indicating tumor invasion. Axial contrast-enhanced
T1-weighted fast spin-echo MR image shows enhancing nasopharyngeal soft-tissue
fullness (star) with enhancement of longus capitus muscles (black
arrows). Also note abnormal thickening and enhancement in hypoglossal
canals, worse on left side (white arrows), and tumor extension into
both carotid sheaths.
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Fig. 15A. —62-year-old woman with nasopharyngeal cancer, right-sided
hearing loss, and right X cranial nerve palsy. Axial contrast-enhanced
T1-weighted fast spin-echo MR image obtained at level of nasopharynx shows
enhancing lesion (arrowhead) in right nasopharyngeal lateral recess
invading ipsilateral carotid sheath (arrow). Clinically,
nasopharyngeal tumor was not conspicuous, and pathologic diagnosis was made
only after repeated biopsies.
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Fig. 15B. —62-year-old woman with nasopharyngeal cancer, right-sided
hearing loss, and right X cranial nerve palsy. Axial contrast-enhanced
T1-weighted fast spin-echo MR image obtained at higher level than A
shows abnormal enhancement along right clivus and petrooccipital fissure
(arrowhead) with contiguous dural enhancement of right petromastoid
region (arrows).
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Fig. 16A. —45-year-old man with biopsy-proven nasopharyngeal cancer and
right IX–XII cranial nerve palsies. Note spread of nasopharyngeal cancer
posterocephalad to ipsilateral jugular fossa and uncommon extension to
cerebellomedullary angle, presumably perisheath spread. Axial
contrast-enhanced T1-weighted fast spin-echo MR image shows enhancing mass
(arrowhead) in right nasopharyngeal roof with contiguous extension
toward ipsilateral jugular fossa, skull base, and cerebellomedullary cistern
(arrows)
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Fig. 16B. —45-year-old man with biopsy-proven nasopharyngeal cancer and
right IX–XII cranial nerve palsies. Note spread of nasopharyngeal cancer
posterocephalad to ipsilateral jugular fossa and uncommon extension to
cerebellomedullary angle, presumably perisheath spread. Sagittal reformatted
T1-weighted MR image shows enhancing mass (arrows) along course of
cranial nerves IX–XI.
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In conclusion, the aggressive nature of nasopharyngeal carcinoma can
sometimes produce unusual clinical and imaging appearances. It is imperative
for radiologists and clinicians to be aware of these appearances.
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H. E. Stambuk, S. G. Patel, K. M. Mosier, S. L. Wolden, and A. I. Holodny
Nasopharyngeal Carcinoma: Recognizing the Radiographic Features in Children
AJNR Am. J. Neuroradiol.,
June 1, 2005;
26(6):
1575 - 1579.
[Abstract]
[Full Text]
[PDF]
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Introduction
Epidemiologic and Clinical...
