Transcript than Sinusitis and polyps. Malignant tumors of the paranasal sinuses

More than Sinusitis and polyps. Malignant
tumors of the paranasal sinuses: a
Radiopathological correlation
Aditya Bahel, DO; Ikjot Bhutani, MD; Rebecca Cornelius, MD
University of Cincinnati College of Medicine
Department of Radiology
• I have no financial disclosures.
Esthesioneuroblastoma
Coronal T1 Post Contrast and Sagittal T2 images demonstrate a large heterogeneously
enhancing mass in the region of the ethmoid sinus with intracranial extension. Note that the
center of the mass is at the level of cribriform plate and has a “snowman” shape, a
characteristic imaging feature for esthesioneuroblastoma (orange arrow). Along the superior
margins of the lesion, there is a marginal cystic component which has mass effect upon the
lateral ventricle (yellow arrow).
Esthesioneuroblastoma
Nests of monomorphic cells displaying a typical neuroendocrine
chromatin pattern (stippled or salt and pepper[arrow])
Esthesioneuroblastoma
• Rare tumor of neural crest origin that arises from the olfactory
mucosa in the superior nasal cavity
– Hence, the synonym, “Olfactory Neuroblastoma”
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Bimodal age distribution, 2nd and 6th decades
Very favorable prognosis, 75-77% overall 5 year survival rate
Commonly present with symptoms related to nasal obstruction
Demonstrates homogeneous enhancement
Dumbbell-shaped mass with the center of the lesion at the level of
the anterior skull base
Look for bony remodeling/destruction of the cribriform plate
May see speckled calcifications on CT
May also see cystic degeneration
Patients with this disease need long term follow-up as they may
recur late
Melanoma
T1 pre and post contrast axial images demonstrate a heterogeneous,
predominantly T1 hyperintense mass in the left aspect of the nasal cavity that
mildly enhances following the administration of gadolinium (red arrow) .
Melanoma
Nests of severely atypical melanocytes (red
arrow) are confined to the epidermis. This is
consistent with melanoma in-situ. Note the
submucosal melanophages (blue arrow).
Melanoma
• Very rare, accounting for only 4% of all sinonasal malignancies and <1% of
all cases of malignant melanoma
• Most commonly arises in the nasal cavity
– Anterior nasal septum, lateral nasal wall, and inferior turbinate are the
commonest sites
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Epistaxis is the most common presenting symptom
Poor prognosis, mean survival of ~24 months
Signal intensity on T1 owes to the paramagnetic effects of melanin
Lesions are often amelanotic
Typically hypointense on fluid-sensitive sequences, but appearance on T2
is variable
• Differential diagnosis for a T1 hyperintense mass in the sinonasal cavity
includes a hemorrhagic process, fungal disease, proteinaceous cyst,
mucocele, fatty lesion, or hemorrhagic metastasis
– Contrast should help differentiate melanoma from most of these lesions
– Fat suppression will distinguish fat-containing lesions
Osteosarcoma
Axial and Coronal Unenhanced CT images in a 20 year patient demonstrate an infiltrative
mass arising from the left maxillary sinus wall. There is complete resorption of the anterior
wall of the maxillary sinus with extension into the subcutaneous tissues. Note hyperdense
material within the mass, likely osteoid matrix formation (red arrow) . The coronal image
demonstrates aggressive, spiculated periosteal reaction along the maxillary ridge (blue
arrow).
Osteosarcoma
Sheets, cords and nests of variably
pleomorphic neoplastic spindle cells with
prominent nucleoli form new, immature
osteoid material (blue arrows).
Osteosarcoma
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Presents as painless swelling in maxilla or mandible
Mean age at diagnosis is 35 years
Male to female ratio, 2:1
Less aggressive in the paranasal sinuses than in long bones
Metastasize later on in disease
On imaging, look for aggressive, “spiculated” periosteal reaction
with immature bone formation
• Often involves adjacent soft tissues and may cross midline
• Variable T1/T2 signal, signal intensity dependent upon amount of
mineral formation
– Highly mineralized tumor: Low T1/Low T2
– Non mineralized tumor: Intermediate T1/High T2
• May be sclerotic, lytic, or mixed
Mucoepidermoid Carcinoma
Axial T2 image shows a circumferential mass within
the left maxillary sinus that is primarily
hypointense to the adjacent nasal mucosa (red
arrows).
Axial T1 post contrast image demonstrates an enhancing mass
occupying the left cavernous sinus and extending into the
prepontine cistern with resulting mass effect upon the pons (red
arrows) . Also note extension into the left orbit (blue arrow).
Intracranial & orbital extension are the result of CN V perineural
tumor spread and extension into the pterygopalatine fossa.
Mucoepidermoid Carcinoma
Axial CT shows extensive bony remodeling, hyperostosis and cortical
destruction along the left maxillary sinus wall (blue arrow).
Mucoepidermoid Carcinoma
Nests of epidermoid cells with a moderate amount
of eosinophilic cytoplasm and extracellular mucin,
consistent with a mucoepidermoid carcinoma. Note
the neoplasm involving the adjacent bony
trabeculae (blue arrow).
Mucoepidermoid Carcinoma
• Extremely rare in the paranasal sinuses
• Typically occurs in the major salivary glands of the oral cavity,
pharynx, and lacrimal glands
• Accounts for 0.6% of all salivary tumors and 4.8% of all
mucoepidermoid carcinomas
• Most common site in paranasal sinuses is maxillary antrum
• Can be preceded by mucosal dysplasia or carcinoma in situ
• Occurs twice as frequently in females than males
• More commonly involves the mandible than the maxilla
• 10 year cure rate approximately 90% for low grade tumors and 42%
for high grade tumors
• Bone invasion, old age, positive cervical nodes, and facial palsy
portend a poor prognosis
Neuroendocrine Tumor
Coronal CT demonstrates a large mass emanating from the right
maxillary sinus. There is erosion of the lateral, medial, and inferior
sinus walls with soft tissue extension (blue arrow).
Neuroendocrine Tumor
Axial T2 and Coronal T1 post contrast images again show a mass in the right
maxillary sinus that is heterogeneous in signal intensity on T2WI (red arrow).
There is heterogeneous enhancement within the lesion after the
administration of gadolinium (blue arrow).
Neuroendocrine tumor
Nests of cells with a high nuclear to cytoplasmic ratio, slightly
pleomorphic nuclei and the occasional prominent nucleolus.
Immunohistochemistry was consistent with a neuroendocrine
origin. Note the apoptotic bodies (red arrow).
Neuroendocrine tumor
• Rare malignancy of neuroectodermal origin, histologically distinct
from other sinonasal neuroectodermal tumors, however the
radiological distinction between these lesions can be difficult
• Average age at presentation: 47-50 years
• Typically present with advanced disease, 70% have stage IV disease
– 5 year survival rate 64%
• Tend to occur in superior nasal cavity and ethmoid sinuses
• Variable imaging appearance, but can have different patterns of
enhancement and most typically demonstrate bony destruction and
soft tissue infiltration
– May enhance to a lesser degree than esthesioneuroblastoma
• Reactive to several neuroendocrine markers
– CD57, S100, chromogranin, synaptophysin
Sinonasal Undifferentiated Carcinoma
Sagittal T2 image demonstrates a large mass arising from the ethmoids/superior nasal cavity.
It erodes through the skull base and demonstrates intracranial extension. Multifocal
hyperintense T2 foci within the lesion are consistent with cystic degeneration (blue arrow).
Sagittal and Coronal T1 post contrast images demonstrate variable areas of gadolinium
enhancement. Radiologic distinction between this lesion (Sinonasal Undifferentiated
Carcinoma) and Esthesioneuroblastoma would be difficult.
Sinonasal Undifferentiated Carcinoma
Nests and sheets of mildly pleomorphic cells with a high nuclear to cytoplasm
ratio and conspicuous nucleoli. Note the apoptotic bodies (blue arrows) and
rosette formation (red arrow). Immunohistochemistry was positive for
cytokeratin 8. This is consistent with a sinonasal undifferentiated carcinoma.
Sinonasal Undifferentiated Carcinoma
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Very aggressive tumor which has varying histology
Commonly present with symptoms related to nasal obstruction and/or orbital
involvement
Most commonly involve the nasal cavity and may extend into the anterior cranial
fossa
Usually have aggressive imaging features such as bony destruction, necrosis and
extensive soft tissue involvement
Very commonly metastasize to brain, bone, regional lymph nodes, and also liver
Masses are typically large at presentation (>4cm)
Imaging characteristics are nonspecific
– Often demonstrate avid to heterogeneous enhancement
– May have a cystic component
– Usually cannot be differentiated from other tumors that occur in the paranasal sinuses on
imaging alone
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Poor prognosis despite any/all aggressive therapy measures (surgery,
chemotherapy, radiotherapy)
Squamous Cell Carcinoma
Axial and coronal T1 post contrast images demonstrate an enhancing mass arising from the
right maxillary sinus. It extends medially into the right nasal passage and the inferior aspect
of the ethmoid air cells (red arrow), causing ostial obstruction. Pterygopalatine fossa
extension portends a poor prognosis (blue arrow).
Squamous Cell Carcinoma
Sheets of variably pleomorphic epithelioid cells with ample
eosinophilic to amphophilic cytoplasm. Distinct cell borders (blue
arrow) and scattered dyskeratotic cells (red arrow) are seen.
Squamous Cell Carcinoma
• Most common malignant tumor of the paranasal sinuses
• Patients typically present as if they have chronic sinusitis
• Commonest location in the paranasal sinuses is the maxillary
antrum
• HPV and thorotrast exposure increase risk
• Important to scrutinize adjacent structures for surgical planning
– Assessment for perineural involvement pivotal for surgical
management
• Assess foramen rotundum, pterygopalatine fossa
• Tends to enhance less than other sinonasal tumors
• May have areas of necrosis or hemorrhage
• Tumor has soft tissue density
– May mimic sinusitis
• Overall 5 year survival rate ~60%
Rhabdomyosarcoma
Sagittal T2 weighted MR image demonstrates a
relatively hypointense mass arising from the
ethmoids and extending into the frontal sinus.
Coronal T1 post contrast demonstrates a
homogeneously enhancing mass arising from
the nasal cavity and ethmoids.
Rhabdomyosarcoma
Sheets of “small blue cells” with features of malignancy: high
nuclear to cytoplasm ratio, nuclear pleomorphism and nuclear
folding (blue arrow). Immunohistochemistry was consistent
with rhabdomyosarcoma
Rhabdomyosarcoma
• Most common soft tissue sarcoma in children under 15
years of age, but is rare in adults.
• Ethmoid and maxillary sinuses are most common site
of head and neck rhabdomyosarcoma in adults
• Adults tend to have a worse prognosis than children
• Extent of bone erosion has been suggested to be an
important prognostic factor
• Commonly spread to lung, lymph nodes, marrow, and
also brain and meninges
• On imaging, commonly see a poorly defined mass with
osseous destruction
– Typically enhance and may be necrotic
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