Paranasal Sinuses: Neoplastic Lesions

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1 Pravin Mundada Department of Radiology, Geneva University Hospital, Switzerland Paranasal Sinuses: Neoplastic Lesions ESHNR 2017 Lisbon, Portugal

2 Layout of the presentation Clinical & imaging features of commonly seen benign & malignant sinonasal tumors Role of various imaging modalities in diagnosis & treatment planning What does the surgeon expect from a radiological report?

3 Clinical overview Tumors of paranasal sinuses are rare Symptoms of benign & early malignant tumors mimic rhinosinusitis: stuffy nose, rhinorrhea, epiphora & epistaxis Symptoms of advanced malignant tumors are related to tumor spread: pain, facial swelling, cranial nerve palsies, anosmia & visual disturbances Often detected when they are large Imaging cannot give histopathological diagnosis in most instances Patient demographics & characteristic location of tumor may give diagnostic clue in some cases

4 Imaging armamentarium CT, MRI, FDG PET-CT, FDG PET-MRI, Angiography CT & MR compliment each other CT: detects bone remodelling & destruction, provides surgial roadmap MRI: soft tissue morphology, diffusion & perfusion parameters, direct & perineural spread FDG PET-CT/MRI is a problem solving tool & useful for detection of recurrence Angiography is useful for pre-surgical evaluation & embolisation

5 CT & MRI protocols CT: Pre & post-contrast volume acquisition in axial plane Multiplanar reconstructions in soft tissue algorithm (3mm slice thickness) & bone algorithm (1mm) MRI: T1W in axial & T2W in axial & coronal plane Axial T2FS & STIR coronal High-resolution post-gadolinium T1W & T1WFS sequences in axial & coronal planes, DWI (b-value 500 &1000)

6 Benign tumors and tumor-like conditions Osseous & fibrosseous Epithelial & other soft structures Osteoma Ossifying fibroma Fibrous dysplasia Inverted papilloma Juvenile angiofibroma Hemangioma Pleomorphic adenoma Nerve sheath tumor

7 What is your diagnosis? A 55 year-old male with nasal blockade & rhinorrhea

8 Inverted papilloma (IP) Benign but locally aggressive M>F, typically years 13 % bilateral & 4% multifocal IP 10% degenerate into or co-exist with SCC CT: Bone remodelling, conical focal hyperostosis, entrapped bone sign MRI: Post-contrast T1WFS: convoluted cerebriform appearance Bone destruction on CT & invasive-necrotic appearane on MRI: malignant degeneration

9 Inverted papilloma Dynamic Perfusion MRI can be used as a problem solving tool in certain cases It is useful in differentiating a small multifocal IP from enhancing normal mucosa a small recurrent IP from post surgical changes Treatment: Endoscopic resection/ mid-facial degloving/ medial maxillectomy + rhinotomy

10 Juvenile angiofibroma (JNA) Benign, locally invasive, highly vascular tumor Almost exclusively adolescent males (10-25 years) Centred in posterior nasal cavity near sphenopalatine foramen (SPF) CT: Avidly enahncing mass centred at SPF, bony remodelling +/- erosion, various extensions.

11 Juvenile angiofibroma MRI: Heterogeneous, intermediate to high signal on T2W, flow voids + Angiography: Tumor blush, internal maxillary artery is feeding artery Treatment: Complete surgical resection + pre surgical embolization/ combination of endoscopic + open resection/ radiation therapy

12 Osteoma Benign slow growing tumor Frontal & ethmoid sinus are common locations Sporadic Rarely autosomal dominant (Gardner syndrome) 3 grades: ivory, mature and mixed < 5% are symptomatic: obstruction to sinus drainage & mass effect

13 Ossifying fibroma (OF) Benign fibro-osseous tumor, locally agressive, F>M, years 4 - subtypes: juvenile, active, agressive & psammomatoid CT: Well-defined expansile soft tissue density mass with egg-shell ossified rim MRI: Variable appearance, may mimic aggressive pathology/ fibrous dysplasia, may show fluid-fluid levels Treatment: Complete surgical resection

14 Malignant tumors Osseous Epithelial and other soft tissue structure Sqamous cell carcinoma (SCC) Osteogenic sarcoma Chondrosarcoma Metastasis Esthesioneurobalstoma Undifferentiated carcinoma Adenoid cystcic carcinoma Mucoepidermoid carcinoma Non-Hodgkin lymphoma Melanoma

15 Squamous cell carcinoma (SCC) SCC accounts for 80% of all sinonasal cancers, of which about 75% are located in maxillary sinus M>F, years Risk factors: Noxious fume inhalation/ HPV/ inverted papilloma/ previous radiation/ immunosuppression 5 - year survival: For T1 stage about 100%, for T4a stage about 34% Better prognosis: Ethmoid sinus SCC, low tumor stage, HPV+ve, inverted papilloma Poor prognosis: Large tumor size, extension beyond sinus walls, nodal metastases, perineural spread

16 Squamous cell carcinoma (SCC) MRI: Intermediate-high signal on T2W, moderate enhancement, restricted diffusion, direct tumor extensions, perineural spread, retropharyngeal lymph nodes CT: Moderately enhancing mass/ irregular margins/ bone destruction Case 1 ase 2

17 T-Staging of maxillary sinus SCC T-stage Maxillary Sinus SCC Tis T1 T2 T3 T4a In situ Restricted mucosal lining Bone destruction, limited to hard palate and middle meatus Bone destruction of post wall of maxillary sinus; flor and medial wall of orbit; tumor extension to ethmoid sinus and pterygoid fossa Tumor growth in anterior orbit; pterygoid plate; infratemporal fossa; cribriform plate; frontal-sphenoid sinus; skin T4b Tumor growth into orbital apex; dura; brain; middle cranial fossa; cranial nerves other than V2; nasopharynx; clivus Treatment: Chemoradiotherapy + surgery

18 Adenoid cystic carcinoma (ACC) Malignant salivary gland-type adenocarcinoma, M>F, years Aggressive, high incidence of recurrence, distant metastasis & perineural spread Recurrence or metastasis can occur even decades after treatment. Poor prognosis MRI: On T2W-intermediate signal with areas of high signal, heterogeneous enhancement, restricted diffusion, perineural spread, CT: Solid or heterogeneously enhancing mass, bone destruction Treatment: Chemoradiotherapy + surgery ACC- Case-1 ACC-Case II

19 Mucoepidermoid carcinoma (MEC) Malignant salivary gland-type adenocarcinoma, rare, M>F, years. Aggressive & poor prognosis MRI: On T2W- intermediate to high signal, diffuse heterogeneous enhancement, perineural spread CT: Solid or heterogeneously enhancing mass, bone destruction Treatment: Chemoradiotherapy + surgery

20 An interesting case: A middle aged man with previous FESS for sinonasal polyposis underwent NECT at a private center for recurrent nasal blockade & anosmia This CT was reported as persistent sinonasal polyposis with rarefication of bones due to long standing inflammation

21 Esthesioneuroblastoma (ENB) Rare malignant neuroectodermal tumor arising from olfactory mucosa Bimodal distribution - 2 nd & 6 th decade Kadish Classification: A - nasal tumor, B - involvement of nasal cavity + sinuses, C - beyond sinuses CT: Cribriform plate destruction, bony remodelling of nasal cavity MRI: Intermediate-high signal on T2, dumb-bell shaped, avid enhancement, cysts in intracranial component, T2*GRE- blooming foci Treatment: Craniofacial resection + radiotherapy

22 What is your diagnosis? An elderly lady with long standing nasal blockade & recurrent epistaxis

23 Undifferentiated sinonasal carcinoma Rare non-squamous cell type tumor Elderly > 60 years Aggressive & high propensity for distant metastasis; perineural spread Unlike ENB, it extends beyond sinonasal cavity CT: Typically large enhancing mass, aggressive bony destruction, often erode cribriform plate, dumbbell shape due to intracranial component MRI: Low-intermediate T2 signal, areas of necrosis, heterogenous enhancement Treatment: Chemoradiotherapy & surgery

24 Non-Hodgkin lymphoma Mimics variety of neoplasms & inflammatory disorders Nasal cavity > sinuses, 6 th -7 th decade 3 types: B-cell, T-Cell & natural-killer T- cell (NKTL) MRI: Polypoid, Low-intermediate T2 signal, DWI - low ADC, perineural spread CT: Iso-hyperdense mass, bone remodelling +/- erosion FDG PET-CT: avid FDG uptake Treatment: Radiotherapy or chemoradiotherapy

25 Sinonasal malignant melanoma Neural crest malignancy arising from melanocytes 90% seen in Caucacians, M>F, years Propensity for systemic metastasis, poor prognosis, mean survival is 24 months Melanotic & amelanotic MRI: Melanotic: T1W-hyperintense, T2W-hypointense Amelanotic: T1W-intermediate, T2W-variable Both: T2*GRE +/- blooming CT: Lobular soft tissue mass, bone remodelling +/- erosion Treatment: Aggressive radical excision & radiotherapy

26 What is your diagnosis A 65-year-old male with recurrent sinusitis & recent episodes of epistaxis

27 What does the surgeon want to know about the tumor? Malignant or benign Tumor margins & direct extensions Perineural spread TNM staging

28 Tumor extension & impact on treatment SCC (stage T4a) SCC (stage T4a) JAF Masticator space Ectopic ENB( stage T4b) Cutaneous infiltration SCC (stage T4b) Foramen rotundum + Meckel cave + infratemporal fossa ENB (stage T4b) Skull base --cavernous sinus Intraorbital + hard palate Intradural

29 Perineural spread & its impact on treatment Depiction of perineural spread helps in individualisation of radiation field Perineural spread to foramen rotundum & cavernous sinus suggests low probablity of cure with radiotherapy PPF + Maxillary N PPF + Vidian N +GSPN + Facial N PPF + Greater palatine nerve Adenoid cystic carcinoma Rhabdomyosarcoma Mucoepidermoid carcinoma

30 TNM staging AJCC Cancer Staging System, 8th Edition

31 Take home messages CT & MRI complement each other in the diagnosis & management of sinonasal tumors An aggressive inverted papilloma may mimic a malignant tumor A highly vascular posterior nasal cavity tumor in an adolescent male is typical of a JAF Melanotic melanomas are hyperintense on T1 Otherwise, most malignant tumors have overlapping imaging features & imaging may not indicate a histopathologic diagnosis Description of tumor margins, extensions & perineural spread impacts treatment

32 Acknowledgements Prof. Dr. Minerva Becker Department of Radiology University Hospital, Geneva Switzerland

33 Thank you for your attention Matterhorn, 4478 metres