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T1-weighted image c shows isointensity with remarkable contrast enhancement and a central unenhanced cystic area after contrast administration arrow d. The imaging feature of sinonasal schwannoma is non-specific, but a tubular tumor shape and target sign may be characteristic features and may be helpful for preoperative diagnosis and surgical planning [ 40 ]. Sinonasal meningioma, often blended with the surface squamous or respiratory epithelium, is arranged in lobules of whorled syncytial meningothelial cells [ 41 , 42 ]. Of the 15 histological types of meningioma, the meningothelial, transitional, metaplastic, and psammomatous types are common in sinonasal meningiomas.

Most of these meningiomas are grade 1 tumors, whereas grade 2 and 3 meningiomas are rare [ 41 ]. Primary extracranial meningiomas of the sinonasal tract with no direct cranial attachment are extremely rare, whereas secondary sinonasal meningiomas, which show direct extension, are much more common [ 41 ]. Symptoms are generally non-specific, including a mass or polyp and nasal obstruction. Sinonasal meningiomas most commonly involve the nasal cavity, followed by the frontal sinus [ 41 ].

Multimodality imaging of sinonasal undifferentiated carcinoma

Invasive meningioma chordoid meningioma, grade 2 [atypical grade for this region] in a year-old man. Coronal CT shows a huge homogeneous hyperdense mass lesion extending from the intracranial cavity to the ethmoid sinus and nasal cavity a. Axial b and sagittal T2-weighted images c show homogeneous hyperintensity and an intratumoral flow void arrows. Coronal contrast enhanced fat-suppressed T1-weighted image shows remarkable homogeneous enhancement d. Identification of hyperostosis may be useful for diagnosis of meningiomas. In the diagnosis of secondary extracranial meningioma, a characteristic feature that displays a different signal intensity and enhancement patterns between intra and extracranial components may be identified [ 45 ].

Ectopic pituitary adenoma EPA is a benign anterior pituitary gland neoplasm that does not involve the sella turcica [ 46 , 47 ]. Symptoms include nasal obstruction, sinusitis, rhinorrhea, discharge, and headache. Concomitantly, a normal pituitary gland is often identified in the normal location, and to date only 15 cases of sphenoid sinus EPAs associated with empty sella have been reported in the literature [ 49 , 50 ].

However, Yang et al. Ectopic pituitary adenoma in a year-old man with hyperthyroidism. Axial T2-weighted image shows a heterogeneous isointense mass lesion in the sphenoid sinus arrow a. Sagittal T1-weighted image shows isointensity b. Sagittal contrast enhanced T1-weighted image shows mild contrast enhancement arrow c. The normal pituitary gland can be also identified in the sella turcica arrowhead.

Rare Disease Database

Although rare, EPAs should always be considered in differential diagnosis of sphenoid sinus mass lesions in older adults regardless of coexistence with empty sella. Even if the pituitary gland is radiologically normal, the pharyngeal-cranial migration route must be reviewed in patients presenting with clinical and laboratory findings of a functioning pituitary adenoma, to exclude the possibility of EPAs.

Nasopharyngeal angiofibroma NA , which is also called juvenile nasopharyngeal angiofibroma, is a rare, benign, highly vascular, and locally aggressive neoplasm, composed of two components: vascular and stromal tissues. The blood vessels are of various sizes, shapes, and thicknesses, ranging from slit-like capillaries to irregularly dilated and branching vessels.

The stroma consists of bipolar or stellate fibroblastic cells, which appear to be arranged around the blood vessels [ 53 ]. Patients present with the classic triad of nasal obstruction, epistaxis, and nasopharyngeal mass. Most NAs arise from the pterygopalatine fossa and nasopharynx of the posterolateral nasal cavity wall [ 53 ]. Nasopharyngeal angiofibroma in a year-old man. CT shows a homogeneous isodense mass lesion in the pterygopalatine fossa a expanding the posterior wall of the maxillary sinus on the bone window arrow b. On MR imaging, the lesion shows hyperintensity on T2-weighted image c and isointensity on T1-weighted image d with remarkable contrast enhancement after contrast administration arrow e , which indicates hypervascularity.

Because of the hypervascularity, angiography is also diagnostic and useful for identifying the feeding artery usually the internal maxillary artery and preoperative embolization [ 53 ].

Program Operations Manual System (POMS)

Sinonasal inflammatory polyp often originates from the maxillary sinus. Inflammatory polyp is caused by allergy, vasomotor rhinitis, infections, cystic fibrosis, aspirin intolerance, or nickel exposure. Fluid accumulation in the lamina propria results in polyp formation. Furthermore, inflammatory polyp may be associated with mucocele or antral cyst formation in the maxillary sinus because of increased pressure in the antrum, resulting in expansion through an accessory or secondary ostium below the middle meatus.

Antrochoanal polyp is a relatively rare type of inflammatory polyp, which often arises from the ostium of the maxillary sinus, extends through the ostium, across the choana, and into the nasopharynx, and usually occurs unilaterally [ 35 ]. Patient age at presentation varies widely with no sex predilection.

Symptoms are non-specific, most commonly including nasal obstruction and rhinorrhea. Antrochoanal polyp in a year-old woman. CT shows a homogeneous isodense mass lesion in the left nasal cavity a.

Update on recently described tumors of the sinonasal tract - Dr. Montone (UPenn) #ENTpath

T2-weighted image shows a hyperintense mass lesion with partial hypointensity arrows b. Coronal c and sagittal d contrast enhanced fat-suppressed T1-weighted images show peripheral enhancement of the nasal lesion, which extends into the nasopharynx arrows. Although a large inflammatory polyp, especially antrochoanal polyp, may be misdiagnosed as other tumors, such as IPs, because of overlapping imaging features, peripheral enhancement may be useful for differentiation [ 55 ].

Sinonasal inflammatory polyps, including antrochoanal polyps, can coexist with a wide variety of pathologic entities ranging from infective granuloma diseases to malignant tumors [ 56 ]. Fungal disease of the paranasal sinuses is mainly categorized into two primary categories: invasive acute and chronic and noninvasive allergic and fungus ball [ 57 , 58 , 59 ]. Among these fungal diseases, fungus ball is a noninvasive dense conglomeration of fungal hyphae usually in a single sinus cavity. The disease tends to occur in older immunocompetent individuals.

Patients are usually asymptomatic, although they may present with mild pressure sensation involving one of the paranasal sinuses, nasal discharge, and cacosmia [ 58 ].

senrei-exorcism.com/images/android/cell-tracker-application-honor.php The maxillary sinuses are most commonly affected, followed by the sphenoid, frontal, and ethmoid sinuses [ 59 ]. Fungus ball in a year-old man. CT shows an isodense lesion with partial calcification arrow a. T2-weighted image shows severe hypointensity arrow b. T1-weighted image c shows hypointensity with mucosal contrast enhancement after contrast administration, whereas fungus ball itself does not show contrast enhancement d.

A hyperattenuating mass within an opacified single sinus in a non-atopic immunocompetent patient is consistent with fungus ball [ 58 ]. Granulomatosis with polyangiitis GPA , which was formerly called Wegener granulomatosis, is an idiopathic necrotizing granulomatous vasculitis. GPAs characteristically present with nasal involvement accompanied by pulmonary and renal disease. Histologic features are vasculitis, necrosis, and granulomatous inflammation [ 60 ].

The disease presents at any age, but is more common in the fourth and fifth decades of life with no sex predilection [ 60 ]. Granulomatosis with polyangiitis in a year-old man. CT shows hyperostosis of the maxillary sinus walls arrows a.

Treatment of Paranasal Sinus and Sinonasal Malignancy

Coronal STIR image shows destruction of the nasal septum arrow and mucosal thickening of the maxillary sinus b. A clinical history of cocaine usage is diagnostic of nasal cocaine necrosis. Hard palate defects are common with nasal cocaine necrosis and lymphoma. Osseous erosion underlying mucosal inflammation can be seen in lymphoma. In contrast, these findings are not characteristic of GPAs. Erosions affecting the nasal septum and turbinates are more specific for GPAs [ 60 ].

IgG4-related disease IgG4-RD is a rare systemic condition that has been found to be responsible for fibro-inflammatory lesions of numerous organs such as the pancreas, liver, biliary tract, salivary glands, lymph nodes, orbits, kidneys, and lungs [ 63 ].

It is currently reported that IgG4-RDs have a particular tropism for the trigeminal nerve, particularly its infraorbital branch [ 64 ]. These lesions often show homogeneously enhanced sinonasal soft tissue masses on CT and MR imaging and may be misdiagnosed as other sinonasal tumors [ 65 ].

In the head and neck region, mean patient age is in the sixth decade of life with no sex predilection [ 63 ]. IgG4-related disease in a year-old woman.


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Coronal CT shows an expanding right infraorbital canal arrow a. MR imaging shows enlargement of the right infraorbital nerve on coronal T2-weighted image arrow b and heterogeneous contrast enhancement after contrast administration arrow c. Differential diagnoses include aggressive malignant tumors with perineural invasion and malignant lymphoma [ 68 ].

Diagnosis may be challenging in cases of lymphoma, with a clinical presentation close to the orbital inflammation. However, lymphoma presents with a strongly restricted apparent diffusion coefficient, which may be helpful for differentiation [ 64 ]. Chronic expanding hematoma CEH is a non-neoplastic hemorrhagic lesion, which consists of a variable state of hematoma surrounded by an organized fibrous capsule [ 69 ]. Mechanisms for the formation of CEHs are the following: 1 repeated hemorrhage in the semiclosed lumen especially in the maxillary sinus forms a hematoma encapsulated by fibrosis; 2 the encapsulation prevents the absorption of the hematoma and induces vascularization, which causes rebleeding and increasing pressure within the hematoma; and 3 the progressive expansion of the hematoma causes the demineralization of adjacent structures [ 69 ].

Patients present between the second to eighth decades of life with no sex predilection. Symptoms are epistaxis, nasal obstruction, facial swelling, and rhinorrhea. CEHs usually occur in the maxillary sinus unilaterally. Because of their expanding features, CEHs are often misdiagnosed as malignant tumors. Complete removal of the hematoma with the fibrous capsule is not only the standard treatment but also diagnostically important to differentiate it from malignant tumors with massive hematoma.


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