DOI:10.2214/AJR.07.7031
AJR 2007; 189:S35-S45
© American Roentgen Ray Society
Imaging of Chronic and Exotic Sinonasal Disease: Review
Arash K. Momeni1,
Catherine C. Roberts2 and
Felix S. Chew3
1 Department of Radiology, David Grant Medical Center, Travis Air Force Base,
Fairfield, CA.
2 Department of Radiology, Mayo Clinic College of Medicine, 5777 E Mayo Blvd.,
Phoenix, AZ 85054.
3 Department of Radiology, University of Washington, Seattle, WA.
revised August 3, 2007;
accepted after revision August 29, 2007.
The opinions and assertions contained herein are the private views of the
authors and are not to be construed as official or as reflecting the views of
the Department of the Air Force or the Department of Defense.
Address correspondence to C. C. Roberts
(roberts.catherine{at}mayo.edu).
Abstract
Objective
Chronic sinusitis is one of the most commonly diagnosed illnesses in the
United States. The educational objectives of this review article are for the
participant to exercise, self-assess, and improve his or her understanding of
the imaging evaluation of sinonasal disease.
Conclusion
This article describes the anatomy, pathophysiology, microbiology, and
diagnosis of sinonasal disease, including chronic and fungal sinusitis,
juvenile nasopharyngeal angiofibroma, inverted papilloma, and
chondrosarcoma.
Keywords: chondrosarcoma • chronic sinusitis • CT • fungal sinusitis • inverted papilloma • juvenile nasopharyngeal angiofibroma • MRI
Introduction
Chronic sinusitis is one of the most commonly diagnosed illnesses in the
United States. It is estimated to affect more than 30 million individuals and
is increasing in incidence [1].
The number of office visits and the annual expenditures on prescription
medications for sinusitis rose from $50 million to $200 million from 1989 to
1992 alone. In addition to the economic impact, chronic sinusitis has a
significant impact on quality of life. It can lead to significant physical and
functional impairment even when compared with chronic debilitating diseases
such as congestive heart failure and chronic obstructive pulmonary disease
[2].
Sinusitis may be defined as an inflammatory process involving the mucous
membranes of the paranasal sinuses or the underlying bone. It is subdivided
into acute, subacute, and chronic on the basis of the duration of symptoms
[2]. Acute sinusitis is sudden
in onset and may last up to 4 weeks. Subacute is a continuum of the natural
progression of acute sinusitis and lasts 4–12 weeks. Chronic disease is
defined as inflammation of the mucosa of the paranasal sinuses and lasts for
at least 12 consecutive weeks
[2].
This review focuses on the anatomy, pathophysiology, microbiology, and
diagnosis of sinonasal disease, including chronic and fungal sinusitis,
juvenile nasopharyngeal angiofibroma, inverted papilloma, and
chondrosarcoma.
Anatomy and Pathophysiology
Understanding the normal anatomy and physiology of the paranasal sinuses is
important to understanding the pathogenesis of sinus disease. There are four
pairs of sinuses named for the bones of the skull they pneumatize. They are
the maxillary, ethmoid, frontal, and sphenoid sinus air cells and they are
lined by pseudostratified columnar epithelium-bearing cilia. The mucosa
contains goblet cells that secrete mucus, which aids in trapping inhaled
particles and debris.
The maxillary antrum consists of a roof, floor, and three walls: the
medial, anterior, and posterolateral. The roof and medial walls are shared
with the orbit and nasal cavity, forming the orbital floor and lateral wall of
the nose, respectively [3]. The
cilia in the maxillary antrum propel the mucous stream in a starlike pattern
from the floor toward the ostium, which is situated superomedially. From the
ostium, mucus is swept superiorly through the infundibulum, which is located
lateral to the uncinate process and medial to the inferomedial border of the
orbit (Figs. 1 and
2A,
2B). The uncinate process is a
sickle-shaped bone extension of the medial maxillary wall that extends
anterosuperiorly to posteroinferiorly
[4]. The uncinate process is
rarely pneumatized. The hiatus semilunaris, situated immediately superior to
the uncinate process, is a slitlike air-filled space anterior and inferior to
the largest ethmoid air cell, the ethmoidal bulla. It is clinically
significant because disease located here results in obstruction of the
ipsilateral maxillary antrum, anterior and middle ethmoid air cells, and
frontal sinus, whereas disease in the infundibulum results in isolated
obstruction of the ipsilateral maxillary sinus alone
[5].
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Fig. 1 —39-year-old woman with headache. Coronal unenhanced CT scan
shows normal sinus anatomy, including each maxillary ostium (arrows),
uncinate process (arrowheads), ethmoid bulla (B), middle nasal
turbinate (M), inferior nasal turbinate (I), and infraorbital ethmoid cells or
Haller cells (asterisks). Maxillary ostium enters infundibulum, which
is space between uncinate process and ethmoid bulla.
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Fig. 2A —59-year-old woman with headache. Noncontiguous axial
unenhanced CT images show normal paranasal sinus anatomy. At level of mid
globe, ethmoid (E) and sphenoid (S) sinus are visible, as well as middle
crania fossa (M) and lamina papyracea (LP). At level of mid face, maxillary
(M) sinuses have adjacent nasolacrimal duct (NLD), turbinates (T),
pterygopalatine fossa (PtPF), infratemporal fossa (ITF), and nasopharynx
(NP).
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Fig. 2B —59-year-old woman with headache. Noncontiguous axial
unenhanced CT images show normal paranasal sinus anatomy. At level of mid
globe, ethmoid (E) and sphenoid (S) sinus are visible, as well as middle
crania fossa (M) and lamina papyracea (LP). At level of mid face, maxillary
(M) sinuses have adjacent nasolacrimal duct (NLD), turbinates (T),
pterygopalatine fossa (PtPF), infratemporal fossa (ITF), and nasopharynx
(NP).
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The entire complex of the maxillary ostium, infundibulum, uncinate process,
hiatus semilunaris, ethmoid bulla, and middle meatus make up the ostiomeatal
unit or ostiomeatal complex. The ostiomeatal complex
(Fig. 3) acts as the common
drainage pathway of the frontal, maxillary, and anterior ethmoid air cells,
the patency of which is critical for normal sinus drainage and ventilation
[4].
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Fig. 3 —Anterior drawing of ostiomeatal complex. Arrows show
direction of mucociliary clearance. Potential areas of obstruction are denoted
with X. Location of anterior ethmoid artery (A) is important in endoscopic
sinus surgery. Graphic modified with permission from Mayo Foundation for
Education and Research.
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Obstruction of the ostiomeatal complex is commonly considered the
underlying cause of most cases of sinusitis because obstruction may result in
maxillary, ethmoidal, or frontal disease. Predisposing factors that induce
local inflammation of the sinonasal mucosa and occlude the ostiomeatal complex
include allergy, viral infections, and air pollutants
[6]. Mucosal swelling impairs
mucociliary clearance and results in sinus ostia obstruction. Sinus excretions
then pool and thicken, creating a nidus for superinfection.
The ethmoid sinuses are paired, discrete cells that may number 18 or more.
They are anatomically divided into anterior, middle, and posterior groups
according to the location of the draining ostia. There are two primary types
of cells: intramural and extramural. The intramural cells remain confined to
the ethmoid bone, whereas the extramural invade the adjacent bones of the
cranial vault or the face [3].
The ethmoid bulla is the air cell directly superior and posterior to the
infundibulum and hiatus semilunaris. A large ethmoidal bulla can obstruct the
infundibulum and hiatus semilunaris, leading to interference with the drainage
of the maxillary and anterior ethmoid sinuses through the ostiomeatal complex
[4].
The frontal sinuses drain inferomedially via the frontal recess, which is a
space between the inferomedial frontal sinus and the anterior part of the
middle meatus. The frontal sinus and the anterior ethmoid air cells together
drain directly into the middle meatus via the frontal recess, or less
commonly, into the superior ethmoidal infundibulum, before draining to the
middle meatus [4].
The sphenoid sinuses drain into the sphenoethmoidal recess, which lies
above the superior nasal concha, and the posterior ethmoid cells.
Pneumatization of the sphenoid sinuses is slow, but is usually complete by
puberty. Still, failure of pneumatization, resulting in a permanent infantile
appearance, is not uncommon
[3]. The sphenoid sinus is
usually septate, but the septum is midline in only 25% of patients
[7].
Microbiology
Unlike in acute sinus disease, the exact role of bacteria or other
organisms in the cause of chronic sinusitis remains unidentified
[8]. Acute illness is
classically caused by Haemophilus or Streptococcus species;
however, chronic disease may result from a number of disparate organisms. In a
study of 94 cases of endoscopically guided ethmoid sinus cultures from 50
adults with chronic sinusitis, the recovered organisms included
Staphylococcus aureus, gram-negative rods, Haemophilus
influenzae, group A streptococci, Streptococcus pneumoniae, and
Corynebacterium diphtheriae
[9]. Moreover, polymicrobial
infections are more common in patients with chronic sinusitis. The offending
organisms of polymicrobial disease include anaerobic bacteria, fungi, and
Pseudomonas aeruginosa
[10].
Chronic Sinusitis
Chronic sinusitis often develops secondary to acute disease that is
refractory to treatment. Clinically, few signs or symptoms reliably
differentiate acute from chronic disease because they present in a similar
fashion. The real distinction is based on the duration of symptoms: acute
disease is sudden in onset and may last up to 4 weeks, whereas chronic disease
lasts for at least 12 consecutive weeks. The most common presenting symptoms
for acute sinusitis include fever and toxicity; chronic sinusitis symptoms
include facial pressure, headache, nasal obstruction, postnasal drip, and
fatigue.
The location of the facial pain and pressure may also help localize the
infection to a particular sinus. Maxillary disease typically causes cheek
discomfort or pain in the upper teeth, whereas pain due to frontal sinusitis
is usually experienced in the forehead. Ethmoiditis may present with
tenderness over the medial canthal region; pain from sphenoid sinus
involvement is often retroorbital, but may radiate to the occipital and vertex
regions [11].
The two primary diagnostic imaging techniques for evaluating the paranasal
sinuses are CT and MRI. Radiography was once the most commonly ordered study;
however, CT has surpassed radiography in the evaluation process because of its
superior anatomic detail and, when a lower mA protocol is used, a radiation
dose similar to a standard four-view radiographic series
[12,
13]. CT is the imaging study
of choice in both adult and pediatric patients
[14].
The primary role of CT is to aid in the diagnosis and management of
recurrent and chronic disease and to define the anatomy before surgery. CT can
differentiate pathologic variations and show anatomic structures that are
inaccessible by physical examination or endoscopy. It is the method of choice
for defining the complex sinus anatomy because of its 3D high resolution. CT
is the technique of choice in the preoperative evaluation of the nose and
paranasal sinuses and is the gold standard for delineation of inflammatory
sinus disease resulting from obstruction
[15]. The use of a bone
algorithm provides excellent resolution of the complete ostiomeatal complex
and other anatomic factors that play a role in sinusitis. Coronal CT images
most closely correlate with the surgical approach
[16]. Therefore, it is the
preferred study for the surgeon performing functional endoscopic sinus surgery
(FESS) because coronal images simulate the appearance of the sinonasal cavity
from the perspective of the endoscope
[4]. If an MDCT scanner
acquiring near isotropic voxels is used, coronal reformatted images from axial
scans are as diagnostic as images acquired in the direct coronal plane. In
some cases, reformatted images are preferable to direct coronal images because
of decreased dental work artifacts, limited patient mobility, and decreased
radiation exposure [17].
The characteristic findings of sinus disease include air–fluid
levels, mucosal thickening, and opacification of the normally aerated sinus
lumen. The single distinguishing feature of acute sinusitis is the
air–fluid level as an isolated finding, whereas the only characteristic
finding in chronic sinusitis is sclerotic, thickened bone of the sinus wall
[18]
(Fig. 4). Mucosal thickening is
common to both acute and chronic sinusitis. The differential diagnosis of
sinus wall thickening includes fungal sinusitis (mycetoma), which often
coexists with chronic sinusitis.
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Fig. 4 —80-year-old woman with chronic sinusitis. Unenhanced coronal
CT scan shows extensive chronic thickening and sclerosis of maxillary sinus
walls (arrows) and mucosal thickening causing near-complete
opacification of sinuses. Intrasinus anatomy is distorted from prior bilateral
antrostomies and ethmoidectomies.
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According to Sonkens et al.
[19], there are five patterns
of inflammatory paranasal sinus disease: infundibular, ostiomeatal unit,
sphenoethmoidal recess, sinonasal polyposis and sporadic (unclassifiable). In
the infundibular pattern, disease is limited to the infundibulum and the
adjacent maxillary sinus; the frontal and ethmoid sinuses are preserved.
Pathophysiologic causes for this pattern are swollen mucosa, polypoid lesions,
and Haller cells. Infundibulotomy is the therapeutic method of choice and
produces excellent results in most cases. In the ostiomeatal unit pattern, the
middle meatus of the nasal cavity and the adjacent anterior and middle ethmoid
cells and the maxillary and frontal sinuses are involved. Pathophysiologic
causes for this pattern are swollen mucosa, polypoid lesion, concha bullosa,
septal deviation, and nasal tumor. Infundibulotomy, in combination with
ethmoid bullectomy, is often required. In the sphenoethmoidal recess pattern,
the sphenoid sinus and the ipsilateral posterior ethmoid cells are involved.
The level of obstruction is in the sphenoethmoidal recess. In the sinonasal
polyposis pattern, polypoid lesions fill the nasal cavity and the sinuses
bilaterally. In effect, this is a mixture of infundibular, ostiomeatal unit,
and sphenoethmoidal recess patterns. In the sporadic (unclassifiable) pattern,
the extent of the disease does not appear to be related to the known mucous
drainage patterns, and there may be retention cysts, mucoceles, and
postsurgical changes.
The extent of disease, according to one of the patterns above, is of
particular interest to the otorhinolaryngologist planning therapeutic
intervention. Therefore, it is critical to identify the technique of choice
for preoperative screening and evaluation. CT is superior to MRI for the
delineation of the fine bone structures of the infundibular complex, orbital
lamina, orbital floor, and cribriform lamina
[20]. Thus, CT is superior to
MRI in planning FESS [21].
With MRI, these bone structures have low signal intensity, making them
difficult to completely assess (Fig.
5). However, when evaluating for orbital or intracranial
complications of sinus disease or surgical intervention, MRI is superior to CT
[20,
22,
23].
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Fig. 5 —64-year-old man with history of sinus disease. Coronal
T1-weighted MR image shows normal low signal of bone. Orbital lamina and floor
(arrows, medial and inferior, respectively) and lateral maxillary
wall (arrowhead) appear as normal low-signal linear structures,
accentuated by adjacent high-signal fat. Soft tissue surrounding infundibulum
(asterisk) is well delineated, as are middle (M) and inferior (I)
turbinates.
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In their comparison of CT and MRI in the evaluation of inflammatory
paranasal disease, Hahnel et al.
[20] concluded that CT is
superior to MRI in the preoperative planning of FESS. However, MRI may be used
as a primary diagnostic instrument in screening for foci of septic disease
before implantation of organs or prostheses, in the diagnosis of complications
of sinus infection or FESS, and in patients with dental implants. Moreover,
MRI might be used to assess therapeutic success in patients with inflammatory
disease, with the advantage of avoiding radiation exposure to patients.
Therefore, MRI is an alternative to CT in the evaluation of the paranasal
sinuses, with its main limitations being a decreased ability to delineate bone
detail and its higher cost
[19].
Fungal Sinusitis
Fungal sinusitis encompasses a wide variety of infections, from relatively
innocuous to rapidly fatal. It should be considered in any patient with
chronic inflammation, particularly when the patient is immunocompromised or
has intractable symptoms despite adequate bacterial therapy
[24]. The two primary forms of
fungal disease with the associated imaging characteristics reviewed here
include allergic and invasive fungal sinusitis.
Allergic Fungal Sinusitis
Allergic fungal sinusitis is a benign, noninvasive disease caused by a
hypersensitivity reaction to fungi in the sinuses. The immune response is
predominantly an IgE-mediated type I hypersensitivity reaction; thus, most
patients with allergic fungal sinusitis have a history of atopy or asthma. The
involved sinuses contain brown or greenish-black material with the consistency
of peanut butter or cottage cheese. This material has been called
"allergic mucin" and contains laminated accumulations of intact
and degenerating eosinophils, Charcot-Leyden crystals, cellular debris, and
sparse hyphae rarely visualized without fungal stains. Obstruction of the
sinuses, usually due to local lesions such as nasal polyps, a deviated septum,
or inflamed mucosa from chronic sinusitis, is required to provide an
environment conducive for fungal growth
[24].
The most common causative agents of allergic fungal sinusitis are the
pigmented fungi, including Curvularia, Bipolaris, and
Pseudallescheria species, and the hyaline molds, such as
Aspergillus and Fusarium organisms. Five clinical features
aid in the diagnostic evaluation, including radiologically confirmed
sinusitis; the presence of mucin in a sinus; visualization of fungal hyphae in
the allergic mucin; absence of fungal invasion of the submucosa, blood
vessels, or bone; and the absence of diabetes, immunodeficiency disease, or
recent treatment with immunosuppressive drugs
[24].
Invasive Fungal Sinusitis
Invasive fungal sinusitis primarily occurs in immunosuppressed individuals
[23]. The most common causes
of immunosuppression in patients with invasive fungal sinusitis include
hematologic malignancies, solid organ or bone marrow transplantation,
chemotherapy-induced neutropenia, advanced AIDS, or diabetes mellitus
[24]. Infection may be
attributable to invasion by fungi that have colonized the sinuses or to
inhalation of fungal spores. Many patients with invasive fungal sinusitis have
a history of chronic sinusitis. Although some have anatomic abnormalities of
the sinuses, the anomalies are reported with equal frequency in the
asymptomatic population, making it controversial as to whether the anatomic
variants predispose patients to fungal colonization
[25].
Aspergillus, Fusarium, and Zygomycetes organisms and
pigmented molds are most often implicated in cases of invasive disease
[24]. Aspergillus
species are responsible for acute, fulminant disease with fever, facial pain,
nasal congestion, epistaxis, and changes in vision or mentation as common
clinical complaints. Diagnosis depends on histopathologic visualization of
fungal invasion by biopsy of the involved areas. Although not sufficiently
sensitive or specific to confirm diagnosis, imaging techniques such as CT and
MRI may show suggestions of fungal sinusitis.
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Fig. 6 —74-year-old man with diabetes and sinus infection caused by
Aspergillus species. Calcifications (arrows) are centrally
located in maxillary sinus. Abnormal soft tissue extends through medial wall
of maxillary sinus and enters left side of nasal cavity
(arrowheads).
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Benign fungal infections secondary to Aspergillus species, like
allergic fungal sinusitis, are characterized on CT by increased attenuation in
the sinuses and frequent bilateral involvement. Complete opacification with
expansion, erosion, or remodeling and thinning of the sinuses are
characteristic features of allergic fungal sinusitis. However, the signal
intensity on T2-weighted sequences is usually low
[4]. Intrasinus calcification
on CT is also characteristic of fungal sinusitis, particularly that caused by
Aspergillus species. Calcification may occur with other pathologic
processes, such as bacterial sinusitis, mucoceles, and neoplasms, but it is
uncommon in nonfungal inflammatory sinonasal disease
[26].
Intrasinus calcification on CT with aspergillosis is a characteristic
feature of fungal sinusitis and is present in 69–77% of cases
[27]. The shape and location
of calcification in nonfungal cases are different from those of fungal
sinusitis. Calcification in fungal cases is primarily centrally located in the
maxillary antrum (Fig. 6),
whereas the calcification in nonfungal cases is usually peripheral, near the
wall of the maxillary sinus (Fig.
7). Fine punctuate calcification has been identified only in
fungal sinusitis, although smooth, marginated, round, or eggshell
calcification has been found exclusively with nonfungal disease
[28]. Other noteworthy CT
features of fungal sinusitis include bone change of a sinus wall (as is seen
in chronic sinusitis), a focal mass with increased density in the sinus, and
infiltration of adjacent soft tissue or bone destruction in the case of
invasive fungal sinusitis. The differential diagnosis of an intrasinus
polypoid soft-tissue mass with bone remodeling also includes sinonasal
polyposis, sinonasal non-Hodgkin's lymphoma, and Wegener's granulomatosis, all
of which can be difficult to differentiate with imaging alone.
Invasive fungal infections have a propensity for orbital, cavernous sinus,
and neurovascular structure invasion. MRI plays a vital role in the diagnostic
evaluation of patients with aggressive fulminant fungal sinus infections by
aspergillosis or mucormycosis because of its ability to identify the spread of
mycotic infections from the turbinates to the sinuses, orbit, and intracranial
cavity [28]. Invasive fungal
sinusitis is typically hyperdense on CT and hypointense on MRI. Immediate
detection of this complication can lead to life- or orbit-saving therapy
[4].
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Fig. 8A —19-year-old man with juvenile nasopharyngeal angiofibroma who
presented with epistaxis. Coronal reformatted CT (A), unenhanced
T1-weighted MR (B), and noncontiguous enhanced T1-weighted MR
(C) images show large heterogeneously enhancing mass (arrows)
in posterior nasopharynx that involves sphenoid sinus, pterygoid process,
pterygopalatine fossa, and middle cranial fossa.
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Fig. 8B —19-year-old man with juvenile nasopharyngeal angiofibroma who
presented with epistaxis. Coronal reformatted CT (A), unenhanced
T1-weighted MR (B), and noncontiguous enhanced T1-weighted MR
(C) images show large heterogeneously enhancing mass (arrows)
in posterior nasopharynx that involves sphenoid sinus, pterygoid process,
pterygopalatine fossa, and middle cranial fossa.
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Fig. 8C —19-year-old man with juvenile nasopharyngeal angiofibroma who
presented with epistaxis. Coronal reformatted CT (A), unenhanced
T1-weighted MR (B), and noncontiguous enhanced T1-weighted MR
(C) images show large heterogeneously enhancing mass (arrows)
in posterior nasopharynx that involves sphenoid sinus, pterygoid process,
pterygopalatine fossa, and middle cranial fossa.
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Fig. 9A —19-year-old man with juvenile nasopharyngeal angiofibroma who
presented with epistaxis. Early (A) and late (B) anteroposterior
arterial phase right internal maxillary artery (IMA) injection angiograms
before embolization show marked vascularity of juvenile nasopharyngeal
angiofibroma (arrow).
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Fig. 9B —19-year-old man with juvenile nasopharyngeal angiofibroma who
presented with epistaxis. Early (A) and late (B) anteroposterior
arterial phase right internal maxillary artery (IMA) injection angiograms
before embolization show marked vascularity of juvenile nasopharyngeal
angiofibroma (arrow).
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Fig. 9C —19-year-old man with juvenile nasopharyngeal angiofibroma who
presented with epistaxis. Early arterial phase right IMA angiogram after
embolization shows marked reduction of vascularity (arrowhead).
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Juvenile Nasopharyngeal Angiofibroma
Juvenile nasopharyngeal angiofibroma is the most common benign tumor
arising from the nasopharynx, but it constitutes only 0.5% of all head and
neck neoplasms [29]. These are
locally aggressive benign tumors that classically present in adolescent boys
with epistaxis. They arise from the fibrovascular stroma of the nasal wall
adjacent to the sphenopalatine foramen. The mass characteristically fills the
nasopharynx (Fig. 8A,
8B,
8C) and invades the
pterygopalatine fossa where it bows the posterior wall of the maxillary sinus
anteriorly. Juvenile nasopharyngeal angiofibroma enhances markedly with
contrast-enhanced CT, differentiating it from the more rare lymphangioma and
from encephaloceles. Other masses that can be found in this location include
hypervascular polyps, rhabdomyosarcomas, germ cell tumors, and carcinomas.
The arterial supply of juvenile nasopharyngeal angiofibroma can arise from
internal or external carotid artery branches. It is important to have a high
clinical suspicion for this lesion because life-threatening hemorrhage may
result if a limited resection or biopsy is attempted. Preoperative angiography
and embolization (Fig. 9A,
9B,
9C) may reduce surgical blood
loss and improve the surgical field of view to facilitate a more complete and
uncomplicated surgical resection
[5,
30].
Contrast-enhanced CT and MRI are valuable in the evaluation of juvenile
nasopharyngeal angiofibroma, with surgical resection being the standard
treatment. Despite surgical and imaging enhancements, high recurrence rates
have been reported, especially when the juvenile nasopharyngeal angiofibroma
involves the skull base. A mean recurrence rate as high as 40–50% in
cases of skull base invasion has been reported
[31].
Recurrence of juvenile nasopharyngeal angiofibroma is typically due to the
growth of residual disease. Early detection of residual disease may allow
treatment and recurrence reduction. Kania et al.
[31] investigated the
diagnostic accuracy of contrast-enhanced CT to detect residual disease
immediately after surgical excision of juvenile nasopharyngeal angiofibroma.
They state the choice between contrast-enhanced CT and MRI to identify
residual disease is open to debate, but they chose CT because it depicts the
close interactions between the bone anatomy and residual disease better than
MRI and provides good soft-tissue definition.
The results of Kania et al.
[31] indicate that
contrast-enhanced CT enables early detection of residual disease in the early
postoperative stage after lesion excision involving the skull base. The
location of residual disease may be correlated with certain extension paths of
juvenile nasopharyngeal angiofibroma in the skull base, such as the sphenoid
sinus, the base of the pterygoids and clivus, and the cavernous sinus and
anterior fossa. To date, most postoperative imaging studies take place between
6 weeks and 6 months after surgery to identify tumors, but not to initiate
early management of persistent disease. Because minimally invasive revision
surgery may remove the residual disease in most instances, postoperative
imaging may be a novel approach in the evaluation process and may help reduce
the recurrence rate of juvenile nasopharyngeal angiofibroma.
Inverted Papilloma
Nasal papillomas are uncommon benign neoplasms, accounting for only
0.4–4.7% of all sinonasal tumors
[33]. The three most distinct
forms are fungiform, cylindric, and inverted papillomas, with inverted
papillomas accounting for approximately 47% of all nasal papillomas
[32]. Inverted papillomas are
benign epithelial neoplasms that classically arise from the lateral nasal wall
or maxillary sinus and have significant malignant potential. They most often
affect patients 40–70 years old and occur two to four times more often
in men than in women. The common symptoms are epistaxis, rhinorrhea, nasal
obstruction, anosmia, sinusitis, facial pain, and frontal headache
[33]. Proposed staging
classification includes stage I, limited to the nasal cavity alone; stage II,
limited to the ethmoid sinuses and the medial and superior portions of the
maxillary sinuses; stage III, extension to the lateral or inferior aspects of
the maxillary sinuses or extension into the frontal or sphenoid sinuses; and
stage IV, spread outside the nose and sinuses
[4].
Inverted papillomas can be associated with malignancy. Because an increased
association with squamous cell carcinoma exists, it is recommended that
inverted papillomas be surgically resected with wide mucosal margins. The
diagnosis is made by histologic examination of biopsy specimens. Grossly
inverted papillomas appear as a mucosal polypoid lesion with histologic
changes summarized as patchy severe squamous metaplasia on polypoid
protuberances of nasal mucosa and submucosa and in related ductal epithelium.
The polypoid protuberances are possibly formed by invagination of surface
mucosa in polyps [34].
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Fig. 10 —50-year-old man with intractable nasal congestion. Coronal CT
scan of paranasal sinuses shows complete opacification of right maxillary
sinus and abnormal soft tissue extending through infundibulum on right into
right nasal cavity (arrows). CT appearance is nonspecific and could
represent polyposis or inverted papilloma. Middle meatal antrostomy has been
performed on left (asterisk).
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Diagnostic imaging may help identify an inverted papilloma when a sinonasal
mass with polypoid morphology arises on the lateral nasal wall. CT may show a
nonspecific mass centered in the middle meatus with associated bone remodeling
(Fig. 10). The diagnosis is
evident in only approximately 20% of cases when CT contains stippled calcium.
On MRI, the lesion is isodense to muscle on T1-weighted images
(Fig. 11A) and isointense to
hypointense on T2-weighted images. Most other polypoid masses (sinonasal
polyposis, antrochoanal polyp) have high homogeneous signal intensity on
T2-weighted sequences. Inverted papillomas enhance
(Fig. 11B) and, in roughly 50%
of cases, the lesions are heterogeneous in both signal intensity and
enhancement. A convoluted cerebriform pattern on T2-weighted sequences or
enhanced T1-weighted sequences is typical of inverted papillomas
[4,
33]. Juvenile nasopharyngeal
angiofibromas tend to be located in the posterior nasal cavity of younger
patients. Nasal carcinomas are more likely to destroy bone rather than remodel
bone, as is seen with inverted papillomas.
The morphology of a sinonasal mass on MRI in a patient with biopsy-proven
inverted papilloma can help warn the surgeon of a probable associated
malignancy. Central necrosis in a sinonasal tumor requires consideration of an
associated malignancy, even when the preoperative biopsy identifies only an
inverted papilloma. Radiologists should be on the lookout for signs of locally
invasive disease that might otherwise go unappreciated and greatly alter the
surgical approach when a necrotic mass is present. MRI revealing a typical
convoluted cerebriform pattern can have small foci of in situ squamous cell
carcinoma [33].
Inverted papillomas can show an aggressive pattern of bone destruction
because they may cross the cribriform plate into the anterior cranial fossa.
They can erode the skull base similar to aggressive cancers and, because
signal intensity characteristics overlap those of malignancies, there is no
way to preoperatively predict the diagnosis. The lesion is especially
problematic for surgeons who treat the condition as if it were malignant by an
aggressive surgical approach. Unfortunately, despite aggressive operations,
the recurrence rate is 20–40%. Recurrences may be distinguished from
postoperative thickening by dynamic enhanced MRI; recurrences have earlier and
greater enhancement than granulation tissue
[4].
Convoluted cerebriform high signal on T2-weighted sequences or enhanced
T1-weighted sequences is typical of inverted papillomas
[4]. When inverted papilloma is
the biopsy result of a sinonasal mass, deviation from the convoluted pattern
strongly suggests an alternative diagnosis of concomitant inverted papilloma
and squamous cell carcinoma
[33].
Chondrosarcoma
Chondrosarcomas are uncommon malignant neoplasms of cartilaginous or
osseous origin that account for approximately 11–25% of all primary
sarcomatous neoplasms of bone and are most commonly found in the long bones
and pelvis. They account for less than 2% of all head and neck tumors, with
less than 10% occurring in the craniofacial region
[35], making craniofacial
chondrosarcoma a rare disease entity.
Craniofacial chondrosarcoma most commonly presents as a painless mass that
progresses to symptomatic, with complaints of impaired vision, nasal
obstruction, and dental abnormalities. In more rare cases it may also present
with swelling of the cheek, headaches, dysphagia, and sensory alterations in
the neck, shoulder, and arm. In part because of its rarity, epidemiologic risk
factors are poorly defined. The male-to-female ratio is approximately 1.2:1
[36]. Most craniofacial
chondrosarcomas occur in patients younger than 40 years and have been reported
to develop in association with malignant tumors such as osteosarcoma,
melanoma, fibrosarcoma, and leukemia, as well as benign conditions such as
Paget's disease and fibrous dysplasia
[35].
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|
Fig. 13 —61-year-old-man with chondrosarcoma of sphenoid sinus.
Enhanced axial CT scan shows irregularly enhancing mass in sinus (black
arrows). Intracranial extension is seen along right cavernous sinus
(arrowhead) and right cerebellopontine angle (white
arrow).
|
|
On CT, craniofacial chondrosarcoma appears as a lobulated mass containing
an irregular chondroid matrix with bone invasion and destruction
(Fig. 12). The signal density
of the chondroid matrix is less than that of the bone matrix, although regions
of bone density may be observed because of localized ossification. With
T1-weighted sequences, the administration of gadolinium results in curvilinear
septal enhancement of the fibrovascular tissues in chondrosarcoma tumors. The
unenhanced areas consist mostly of cartilage, mucoid tissue, or necrosis. The
chondroid matrix has high signal on T2-weighted sequences because of higher
water content, whereas the ossified regions have low signal due to immobile
protons [37,
38].
Delineation of chondrosarcoma boundaries relative to normal tissue can be
achieved with greater than 98% accuracy using CT and MRI
[36]. MRI is the best
technique for monitoring tumor recurrence and soft-tissue visualization,
thereby allowing differentiation of tumor from surrounding tissues or edema.
CT is superior in revealing bone erosion, particularly in the region of the
cribriform plate, orbit, pterygopalatine fossae, and infratemporal fossae.
Intracranial extension can also be detected as dural enhancement after the
administration of contrast material (Fig.
13). Distant metastasis is rare and most often involves the lung;
therefore, radionuclide bone scanning plays a limited role in the management
of chondrosarcoma [36].
Surgery is the standard of care for patients with chondrosarcoma of the
head and neck. Tumors are graded on the basis of cellular activity, nuclear
enlargement, and irregularity. Scores of 1, 2, and 3 correspond to low,
intermediate, and high grades, respectively. The prognosis is generally good
for low- and intermediate-grade chondrosarcoma, with tumor involvement at the
resection margin being the only poor prognostic factor other than high-grade
disease [36]. The overall
5-year disease-free survival for low-grade chondrosarcoma after complete
resection ranges between 54% and 77%
[35,
36], with the most common
cause of death being recurrence with local skull base invasion
[36].
Acknowledgments
We thank Craig Llewellyn at Madigan Army Medical Center, Tacoma,
Washington, and Nancy Fischbein at Stanford University, Palo Alto, California,
for contributing cases.
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Abstract
Introduction
