More than Sinusitis and polyps 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” 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 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 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 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. Axial T2 image shows a circumferential mass within the left maxillary sinus that is primarily hypointense to the adjacent nasal mucosa (red arrows).

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 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 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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