2020.11.04 Lecture PNS tumor and tumorlike

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Paranasal sinus Tumors and tumor like lesions

Carolina Tramontini, M.D.Neuroradiologist

Clínica Reina Sofía

Clínica Infantil Santa María del Lago

Fundación Universitaria Sanitas

ECHNR, November 4th, 2020


I have no relevant disclosures for this lecture

• Introduction

• Benign lesions

• Malignant lesions

• Bone lesions

• External lesions

• Take home messages


• Introduction

• Benign lesions


• Bone lesions




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• Malignant tumors of the sinonasal tract are extremely rare

• The clinical presentation is non‐specific and often mimics

benign disease

• Delay in diagnosis is common

• 75% of all paranasal sinus tumors are Stage T3 or T4 at the

time of diagnosis

• Treatment depends on histological type, location and

extent

• Prognosis depends on location and stage

Introduction

Loevner L, Radiology Assistant 2009,Koeller K, Head and Neck Pathol, 2016Szewczyk-Bieda et al. Clinical Radiology ,2014

• Considerable overlap in imaging appearance

• Specific manifestations often are lacking

• Virtually all sinonasal tumors requiere histopathology for specific diagnosis

• “It is not about the histology but about answering the question 'is it tumor or not?’” (L Loevner)

Imaging in PNS tumors

• Role of imaging

Imaging in PNS tumors

Identification of the mass

Location and extension

Valuable guide for biopsy and surgical resection

CT• Excellent to determine anatomic landmarks and variants

• Effect of the lesions on adjacent bone structures

• Calcifications

• Density

MR• Distinguishes mass from fluid or mucosal thickening

• Mass characteristics

• Define extension into soft tissues, orbit or intracranial

Introduction

CT and MR are complementaryKoeller K, Head and Neck Pathol, 2016

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• Bone erosion or remodelling in benign or slowly growing tumors

• Osseous lytic destruction in more aggresive tumors (SCC)

• Bone sclerosis in chronicinflammation, radiation, osteomielitis

• Intratumoral calcifications– True tumoral or residual bone

– In chondroid and fibro‐osseous tumors, olfactory neuroblastoma and invertedpapilloma

CT

Koeller K, Head and Neck Pathol, 2016

• Fluid

• Inflammatory changes

• Low grade tumors

MR

Hypointensity in T2

Hyperintensity in T2

Superior to CT in identifying soft tissue tumor portion

• High cellularity

• Lack of fluid

Secretions do not have solid enhancement. If you have an enhancing mass, you must rule out tumor.

• Define invasion of the skull base

– Replacement of the high signal of the fatty marrow on T1WI by hypointense signal of the tumor

• Review for foraminal extension

– Perineural spread

– Direct invasion of the tumor

• Detect intracranial extension

MR

Loevner, The Radiology Assistant, 2009

• Different protein contents result in different signal intensities on T1 and T2W‐ images

• Fungus – Usually has a high protein content of more than 28%

– Can mimic an aerated sinus: low on T1‐ and T2WI

– You need CT to make the distinction

• Bright signal on T2 is a sign of benign disease– Fluid and mucosal disease usually have a high water content

MR

Loevner, The Radiology Assistant, 2009

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• Introduction

• Benign lesions


• Bone lesions




BenignTumors2017 WHO classification

Tatekawa et al, Japanese Journal of Radiology, 2018

Sinonasal papillomas

• Benign epithelial lesions arising from Schneiderian mucosa

• Three subtypes:

– Inverted 62%

– Oncocytic 6%

– Exophytic 32%

• Clinically: nasal obstruction, polyps, epistaxis, hyposmia, headache


Inverted papilloma

• The most frequent papilloma

• Inverted growth of the neoplastic epithelium into the underlying stroma

• Most frequent in the 5th and 6th decade

• 2,5 to 3 times more frequent in males

• Locally aggressive, multicentric, malignant transformation

• 1,9‐27% become SCC


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Inverted papillomaImaging

CT

• Location: lateral nasal wall and middle meatus

• Focal hyperostosis at the sites of origin

MR

• Lobulated shape

• Hyper on T2, iso ‐ hypo on T1

• Convoluted cerebriform pattern (CCP)



Lee et al, AJNR 2007

CT

• Focal hyperostosis at the site of origin

• Corresponded to site of origin in 89% correlating with surgical findings

• Two patterns

– Plaque like : lateral wall

– Cone shaped : paranasal sinuses and septum



Plaque like: lateral wall



Cone shaped: paranasal sinuses and septum

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MR

• Lobulated shape

• Hyper on T2, iso ‐ hypo on T1

• Convoluted cerebriform pattern (CCP)

Jeon et al, AJNR 2008


Convoluted cerebriform pattern (CCP)

• Present in 100% of IP (Jeon 2008)

• Present in 13% of malignant PNS tumors


Inverted papillomaSquamous cellcarcinoma


• Signs that indicate the presence of coexistent SCC


Focal loss of CCP

Necrotic lesion

Invasion to the surrounding tissue

Respiratory epitelial adenomatoidhamartoma ‐ REAH

• Benign neoplasm of the nasal cavity and PNS

• Submucosal glandular proliferation with polypoid appearance

• 3rd to 9th decade, median age is the 6th decade

• Male : female ratio is 7:1

• Most located at the anterior half of the olfactory clefts (OC) or posterior nasal septum

Tatekawa et al, Japanese Jurnal of Radiology, 2018

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Respiratory epitelial adenomatoidhamartoma ‐ Imaging

CT

• Homogeneous mass

• Enlargment of the OC without bone destruction

MR

• Well delineated soft tissue filling

• Homogeneous contrast enhancement


REAH‐ Imaging

CT

• Homogeneous mass

• Enlargment of the OC without bone destruction


Respiratory epitelial adenomatoidhamartoma ‐ Imaging

MR

• Well delineated soft tissue filling

• Homogeneous contrast enhancement


Other benign lesions

Salivary gland tumors• Pleomorphic adenoma

Benign soft tissue tumors• Leiomyoma (angioleiomyoma)

• Capillary hemangioma

• Cavernous hemangioma

• Schwannoma

Other tumorsMeningiomas

Ectopic pituitary adenoma


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Nerve sheat tumors

• Arise from nerve sheath cells

• Schwannomas and neurofibromas

• 25‐40% occur in the H&N

• <4% arise in the sinonasal region

• Usually originate from ophthalmic and maxillary branches of the trigeminal nerve

Koeller, Head and Neck Pathol 2016Szewczyk-Bieda et al, Clinical Radiology 2014

Nerve sheat tumorsCT

• Homogeneous soft‐tissue density

• Can be mistaken for inflammatory polyps or inspissated secretions

MRI• Well‐defined

• Intermediate signal on T1W, variable T2W signal

• Variable enhancement

Koeller, Head and Neck Pathol 2016Szewczyk-Bieda et al, Clinical Radiology 2014

Lobular capillary hemangioma

Szewczyk-Bieda et al, Clinical Radiology 2014

• AKA Granuloma gravidarum (misnomer)

• Capillary proliferation affecting mucous membranes of oral and nasal cavity

• At Kisselbachs plexus at the septum or inferior turbinate

• CT– Enhancing mass with hypoattenuating cap

– Bone destruction or remodelling

• MR– Early enhancement and flow voids

Pleomorphic adenoma

• Epithelial/ myoepithelial and mesenchymal stromal components

• 65% of PAs major salivary glands, 35% of PAs accessory salivary glands

• Nasal cavities and paranasal sinuses only rarely involved

• Intranasal PAs

– 3rd to 6th decade

– Slightly more frequent in females

– 80% of PAs arise in the nasal septal submucosa

– Malignant transformation in 2.4–10%

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Pleomorphic adenomaImaging

• Multilobulated appearance is one of the key findings

• CT

– Adjacent bony changes (displacement, expanding erosion)

– Intratumoral calcifications

• MR

– Shows iso‐ to hyperintensity on T2, hypointensity on T1

– Heterogeneous curvilinear contrast enhancement

• Clinical and diagnostic tips

– Complete excision is curative

– It is important to identify the origin of intranasal PAs

Benign mass lesion with CE Clinical and diagnostic tips


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Clinical and diagnostic tips

• Identify de hyperostotic region of origin to avoid inadecuate resection

• Focal loss of CCP, necrotic lesion, invasion to the surrounding tissue are signs that indicate the presence of coexistent SCC

• Significant widening of the OC (anterior half)


Inverted papilloma

REAHs

• Introduction

• Benign lesions


• Bone lesions




Malignant sinonasal tumors

• Nasal and PNS malignancies account for 1% of allmalignancies and 3% of H/N malignancies

• Malignant sinonasal tumors are more frequent thanbenign tumors

• 50% arise in nasal cavity, most of the rest in maxillaryand ethmoid sinuses

• Invasion of adjacent structures affects prognosis

Kawaguchi et al. Journal of Clinical Medicine, 2017Szewczyk-Bieda et al. Clinical Radiology ,2014Koeller K, Head and Neck Pathol, 2016

Malignant sinonasal tumors

Epithelial Non‐epithelial

Squamous cell carcinoma (SCC) Melanoma

Adenoid cystic carcinoma Lymphoma

AdenomaOlfactory neuroblastoma (Estesioneuroblastoma)

Adenocarcinoma Fibrous histiocytoma

Sinonasal Undifferentiated Carcinoma

Sarcomas (rabdomyosarcoma, fibrosarcoma

Fibromixoma, mixoma

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Squamous cell carcinoma SCC

• Is the most common histological subtype

• Accounts for more than half of all sinonasal malignant tumors

• 6th and 7th decade , male predominance

• Maxillary sinus >> nasal cavity >>ethmoid sinus.

• Formerly linked to cigarette smoking and occupational exposures

• In recent years increased positivity rate for HPV (in 32–62% of all sinonasal SCCs )

• Patients with a HPV‐positive sinonasal SCC have a better prognosis than patients with a HPV‐negative sinonasal SCC

Kawaguchi, J. Clin. Med. 2017

Squamous cell carcinoma SCCImaging

CT• Sinonasal soft tissue mass

with prominent bone destruction

• Invasion of the contralateral sinonasal area, orbital wall, infratemporal fossa, and skull base is sometimes observed



MR• Intermediate signal on T1

• Hypointense on T2 (to liquid)

• Larger tumors are heterogeneous (necrosis, hemorrhage)

• Moderate variable contrast enhancement

• Invasion into the anterior and middle cranial fossae



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Olfactory neuroblastoma

• AKA esthesioneuroblastoma

• Neuroectodermal tumor

• Any age, with peaks in the 2nd en 6th decades

• Accounts 3‐5% of intranasal neoplasms

• Arise from the specialized sensory neuroepithelial olfactory cells (cribriform plate, superior turbinate, upper third of the nasal septum)

• Direct tumor extension into paranasal sinuses, cribriform plate, skull base, orbit, and intracranial cavity is frequent.

• Cervical lymph node metastasis (20–25%) and distant metastases (10–40%)

Kawaguchi, J. Clin. Med. 2017.Szewczyk-Bieda et al. Clinical Radiology ,2014Koeller K, Head and Neck Pathol, 2016


CT

• Involves the superior portion of the nasal cavity and ipsilateral ethmoid and maxillary sinuses

• Well defined, homogeneous

• Expansile soft tissue mass

• Homogeneous enhancement

Szewczyk-Bieda et al. Clinical Radiology ,2014Koeller K, Head and Neck Pathol, 2016Kawaguchi, J. Clin. Med. 2017

CT is essential for the evaluation of osseous involvement of the cribriform plate, fovea ethmoidalis, and lamina papyracea.


MR

• Heterogeneous hypointensity compared to gray matter on T1

• Predominant hyperintensity on T2

• Homogeneous, often intense enhancement

• Commonly invades the orbits and extends intracranially

• Peritumoral cysts along the intracranial portion strongly suggests the diagnosis

Dublin A, Bobinsky M. J Neurol Surg B 2016 Szewczyk-Bieda et al. Clinical Radiology ,2014Koeller K, Head and Neck Pathol, 2016

• Head and neck region is the second most common site for extranodal lymphomas

• NHL is the second most common malignancy in the sinonasal tract after SCC.

• 6th 8th decade

• Male‐to‐female ratio 2:1

• Diffuse large B‐cell lymphoma (DLBCL) : maxillary sinus the most common site although may arise from the nasal cavity.

• NK/T‐cell lymphoma most commonly involves the nasal cavity

• B‐cell lymphoma has a better prognosis than T‐cell lymphoma.

Lymphoma


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CT

• Infiltrative or permeative bone changes

• Varying degrees of bone destruction

• Resorption or remodeling may be accompanied by sclerosis

MR

• NHLs: isointense on T1, slightly hyperintense on T2

• ADC lower than SCCs

• Usually homogeneously enhanced mass

• Necrotic areas within the tumor are occasionally observed in NK/T‐cell lymphoma

LymphomaImaging


Lymphoma

Case courtesy : Camilo Caicedo

Lymphoma


• Introduction

• Benign lesions


• Bone lesions




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Osseous and fibroosseous lesions

• Comprise nearly 25 % of nonepithelial tumors involving the sinonasal cavities

• Are very common incidental findings

• Often misinterpreted as tumor

• CT is usually diagnostic

Osseous and fibroosseous lesions

Most common skull base lesion

Fibrous dysplasia

Followed by osteomas

OsteomasUsually located in the sino‐nasal cavities

Fibromas Less common Can be

ossifying or non‐ossifying

Malignancies Are rare

Fibrous displasia

• Enfermedad idiopática, asintomática.

• En senos paranasales se asocia a obstrucción de vías de drenaje.

• Reemplazo de hueso medular por tejido fibrosoinmaduro, puede dar apariencia en vidrio esmerilado.

• Enfermedad poliostótica se asocia a síndrome deMcCune Albright (manchas café con leche y pubertad precoz).

• Enfermedad monostótica afecta principalmentela base del cráneo.

Fibrous displasia


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Osteoma

• Benign intraosseous proliferation of mature bone with predominant lamellar structure

• In 1% of individuals

• 5th‐6th decade

• M:F ratio 1.3‐2:1

• Most common within the frontal sinuses

• Followed by ethmoid sinuses

• Rarely involve maxillary or sphenoid sinuses

• Arises from the sinus wall

Schuknecht B, ECNR Antwerp, 2017

Osteoma• Osteoma is primarily composed

of mature bone similar to normal bone apart from increased bony elements and decreased marrow

• Hyperdense on CT

• Well defined

• May be completely inconspicuous on non‐contrast MRI

• Heterogeneous low‐to‐intermediate signal intensity

• Some Gd enhancement

Gupta et al, 2018Schuknecht B, ECNR Antwerp, 2017

Osteoblastoma

• Fibrous elements and osteoblasts

• Expansile

• Sclerotic or mixed lesion, cortical shell

• Frontoethmoid, orbit or jaw

• 2nd decade

• More frequent in males

Kim J, Kwon S. Medicine, 2017.Schuknecht B, ECNR Antwerp, 2017

Malignant bone lesions

Chondrosarcoma

• Origin in hyalina cartilage

• Lobulated, myxoid changes

• Osteolysis, cortical destruction

• Good prognosis with complete resection

Schuknecht B, ECNR Antwerp, 2017

Osteosarcoma

• Malignancy producing osteoid or bone

• Lobulated

• Lytic‐mixed or sclerotic lesion

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• Benign expansive and locally agressive bone lesionLesión ósea Middle aged women

• Frequently monostotic involvement of sphenoid or ethmoid bone

• Less frequent in maxilary sinus

• Well defined, central area of soft tissue density, surrounded by sclerotic border

• Always look for orbital and dental involvement

Ossifying fibroma

• Introduction

• Benign lesions


• Bone lesions




• CNS tumors

– Direct extension or due to embriologic remnants

– Include meningioma, craniopharyngioma, chordoma and nasal glioma

• Juvenile nasoangiofibroma

• Pituitary adenoma

External lesions

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• Malignant tumors of the NC and PNS are rare

• Clinical findings are non specific

• Role of imaging is

• CT and MR are complementary

Take home messages

Identification of the mass

Location and extension

Valuable guide for biopsy and surgical resection

• Always keep tumor and tumor like lesions in mind when reporting PNS

• Be familiar with the imaging characteristics of

– Inverted papilloma

–Osteoma

–SSC

–Olfactory neuroblastoma

Take home messages

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2020.11.04 Lecture PNS tumor and tumorlike

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