Inverted Papilloma and Other Benign Sino-Nasal Tumors

Inverted Papilloma and Other Benign Sino-Nasal Tumors

1. Benign Sino-Nasal Tumors Dr. Adhishesh Kaul Moderator: Dr. Nagarathna H K Associate Professor

2. Introduction

3. Types of Tumor Epithelial Tumors Inverted Papilloma Vascular Tumors Hemangioma ( Cavernous and Capillary) Bony Tumors Osteoma Ossifying fibroma Mesenchymatous Tumors Glioma Myxoma Leiomyoma Schwannoma

4. Inverted Papilloma

5. History and Basics Other Names Ringertz tumor Transitional cell papilloma Schneiderian papilloma 2 nd most common benign sinonasal tumor Type: Nasal epithelial benign tumor Discovered By: Ward : 1854 Invasiveness discovered by: Ringertz : 1938 Incidence: ~0.6/1lac population

6. Characteristics: 1. Bone invasion and remodelling 2. Recurrence 3. ~10% incidence of SCC (synchronous > metachronous) Time for transformation: Mean 52 months, Range 6 – 180 months Features Most common site: lateral nasal wall> maxillary sinus > ethmoids Male: Female – 2-3:1 Age: 5 th – 6 th decade Architecture: Endophytic Molecular mutation: EGFR activations, TGF α

7. Clinical Presentation Unilateral nasal obstruction Watery rhinorrhea Epistaxis Epiphora/ Proptosis/ Diplopia/ Headache

8. Histopathology Epithelium is hyperplastic (5 - 30 cell layers in thickness) may be of squamous, transitional or respiratory type Transmigrating neutrophils and neutrophilic microabscesses may be seen Stroma may have edema or chronic inflammation Seromucinous gland in the lamina propria is commonly decreased or absent

9. Staging (Krouse staging) T1: confined to nose without sinus extension T2: Involves OMC/ medial maxillary sinus/ ethmoids T3: Involving other areas of maxillary sinus/ sphenoid and/or frontal sinus T4: Extranasal / Extrasinus extension

10. Staging (Cannady Staging)

11. Diagnostic Workup Nasal Endoscopy CT/MRI Biopsy

12. Nasal Endoscopy Pale, polypoid lesion with a papillary appearance Lesion protrudes from the middle meatus

13. Small red bumps (granular mulberry appearance) Has undulations Firmer than polyp More vascular than polyp Not smooth like – polyp

14. Imaging Target AIM: extent and three-dimensional configuration of the lesion and to disclose its relationship with surrounding structures Methods: MRI with gadolinium enhancement CT

15. CT Scan

16. MRI Scan

17. Etiology – Various Theories Viral Infection Inflammation Environmental exposure

18. Viral Infection : HPV 1 st reported in 1980 Correlation ranges from 0% - 100% Subtypes associated with IP: 6, 11, 16, 18 Pathogenesis: HPV induces promoting agent in pathogenesis of papilloma, which leads to gene alteration (p53) resulting in development of IP

19. Chronic Inflammation IP is end stage of chronic inflammatory condition NOT A TRUE NEOPLASM HPE shows more inflammatory cells in IP compared to other sino -nasal papillomas

20. Environmental exposure Dietmer et al. 1996, case – control study 47 cases and 47 controls, found higher degree of occupational exposure to smoke, dust, aerosol Sham et a. 2010, case – control study 50 cases and 150 controls found outdoor and industrial exposure were associated with IP

21. Recurrence – Factors Location of attachment Completeness of resection in primary surgical resection Increased chance of recurrence in revision cases: 18.1% vs 4.1% complicated by scar tissue, absence of bony landmarks, residual disease

22. Rate of recurrence on basis of site of tumor location

23. Rate of recurrence on basis of Surgical Technique Meta-analysis comparing contemporary endoscopic (1992–2004) versus external approaches (1970–1995) demonstrated an improved recurrence rate in the endoscopic group (12% vs. 20%, respectively).

24. Other Risk Factors for Recurrence Tobacco usage Histological parameters hyperkeratosis hyperplasia mitotic index IHC markers

25. Treatment Goals Resection of tumor including tumor base Removal of bone/ burring the base NO NON-SURGICAL TREATMENT MODALITY Radiotherapy: Incase of malignant transformation Incompletely resectable tumors Multiple recurrent tumors

26. Surgical Approaches Transnasal approach (without endoscopes) Open approach (Radical Surgery) – Try to get around the tumor Medial Maxillectomy + Lateral Rhinotomy + Sublabial degloving Endoscopic

27. Open approaches Advantages Possibility of en -bloc resection Access areas with difficult endoscopic instrumentation Anterior maxillary sinus Region of Nasolacrimal duct superior and lateral frontal sinus

28. Anterior Maxillary Sinus Residual tumor in lateral frontal sinus

29. Drawbacks of Open Approaches Cosmetic CSF Leak Orbital Injury enophthalmos, ectropion, diplopia, orbital hemorrhage, rarely blindness Lacrimal Injuries epiphora, dacryocystitis, Mucocele Bleeding

30. Endoscopic approach advantages Improved precision for resection of involved areas Realization that site of attachment may be small and other structures can be spared Greatly improved visualization to determine site of attachment before resection is complete Improved follow up in office to detect and resect recurrences early

31. Contraindications for endoscopic approach massive involvement of the mucosa of the frontal sinus and/ or of a supraorbital cell transorbital extension concomitant presence of a malignancy that involves critical areas presence of significant scarring and anatomic distortion from previous surgery

32. Types of Endoscopic Approach Type 1: IP involving middle meatus, ethmoid, superior meatus, sphenoid sinus, or a combination of these structures; even lesions that protrude into the maxillary sinus without direct involvement of the mucosa are amenable to this approach

33. Type 2: which corresponds to an endoscopic medial maxillectomy, is indicated for tumors that originate within the naso -ethmoid complex and secondarily extend into the maxillary sinus or for primary maxillary lesions that do not involve the anterior and lateral walls of the sinus. The nasolacrimal duct can be included in the specimen to increase the exposure of the anterior part of the maxillary sinus.

34. Type 3: (endonasal denker / Sturman-Canfield operation) entails removal of the medial portion of the anterior wall of the maxillary sinus to enable access to all the antrum walls. It is therefore recommended for inverted papillomas that extensively involve the anterior compartment of the maxillary sinus

35. Endoscopic Medial Maxillectomy Resection of the lateral wall, including the inferior turbinate and nasolacrimal duct (NLD)

36. Drawbacks of EMM Empty nose syndrome Decreased efficiency of nasal heating and humidification in CT-based computational fluid dynamic Impaired stimulation of trigeminal cold receptors, involved in perception of nasal patency ( eg , the TRPM8 receptor) in the mucosa by mucosal cooling

37. Complications Bleeding Most common: Greater Palatine artery Management/ Prevention: Locate and bipolarize Low threshold for SPA ligation Bipolarize posterior end of anterior IT remanent Epiphora Management/ Prevention: Do divide cleanly , Do not leave bone fragments around lacrimal duct Parasthesia Palatal/ Teeth/ Infraorbital skin

38. Modifications of EMM Partial medial maxillectomy Inferior turbinate preservation NLD preservation Pre-Lacrimal Approach

39. Prelacrimal Approach (Endoscopic Modified Medial Maxillectomy) Access similar to that of EMM along with anterior maxillary sinus visualization Preservation of nasal morphology

40. Surgical Queries How to decide what resection to do? Physical examination Imaging What approach to use? Tumor base access Surgeon preference

41. Challenges – Frontal Sinus IP Incidence: 1 – 16% Approach: Recurrence: 22.4% ( Walgama et al 2012) Approach Location of IP Modified Lothrop Inferomedial Osteoplastic Flap Lateral, superior wall

42. Lothrop Procedure Traditional Procedure : first described in 1914, uses a combined external and transnasal approach to resect the median frontal sinus floor, superior nasal septum, and intersinus septum to drain the frontal sinus.  Modified Lothrop : Also known as draf III Remove entire sinus floor, including AS septum

43. Osteoplastic Flap

44. Challenges – Sphenoid Sinus IP Attachment over Optic Nerve, Carotid Artery - 4.76times greater risk of recurrence Intracranial Extension Orbital involvement

45. Invasion Intracranial : 17 patients studied by Wright, with 49.2 years of mean age, 60% had recurrent disease, with commonest side of invasion from frontal sinus and cribriform plate Orbital : 10 patients studied by Elner , with mean age 62 years, 100% with malignant transformation, 80% orbital exenteration, 30% intracranial extension

46. Prognostic Markers Increased risk of recurrence if involvement of sphenoid sinus, frontal sinus or maxillary sinus walls other than medial or extrasinus extension Major cause of recurrence is incomplete resection Risk of malignant transformation is ~9% in inverted papilloma (range: 5 - 15%) 

47. Endoscopic rate of recurrence ~ 12% Open procedure rate of recurrence ~18% Involvement of maxillary sinus floor and lateral recess required additional sublabial approach

48. Endoscopic Surveillance for Early Detection On posterior part of medial orbital wall

49. Indications for EMM Impaired Mucociliary function CF/ Wegeners disease/ Prior Caldwell-Luc with impaired mucocilary clearance Postoperative obstruction of normal ostium Osseo neogenesis in normal ostium from surgery/ prior orbital decompression of normal ostium Access in difficult airways Odontogenic infection/ Foreign bodies/ AC polyp with attachment in anterior or lateral wall Destroyed medial wall extensive mucocele with destruction of medial wall/ allergic fungal sinusitis

50. Osteoma

51. Benign, slow growing, osteoblastic lesion Incidence: 1% of population undergoing radiographs, 3% of population undergoing CT for Sinus symptoms Age: 2 nd to 5 th decade of life Male preponderance Site: Frontal > Ethmoid > Maxillary sinus > Sphenoids

52. Gross appearance: hard, white, multilobulated mass, Types: Ivory : lobulated, made of compact dense bone, and contains a minimal amount of fibrous tissue without evidence of haversian ducts . Mature : spongy, mature bone with bony trabeculae divided by a conspicuous amount of fibrous tissue; the lesion contains fibroblasts in different stages of maturation and a great number of collagen fibers, and the connective tissue may often contain distended thin-walled vessels. Mixed

53. Theories for development Embryogenic Theory : osteoma develops at the junction between the embryonic cartilaginous ethmoid and the membranous frontal bone Traumatic Theory : development of osteoma with a previous trauma Infective Theory : local inflammation may alter adjacent bone metabolism by activating osteogenesis

54. Imaging: CT Scan: Shows features of cortical bone, tapering to ground glass appearance in periphery Management: 1. Wait and watch in case asymptomatic 2. Excision: in case producing symptoms because of Obstruction of sinus clearance Compression on orbital structures/ optic nerve encroaching anterior skull base causing CSF leak, pneumocele , etc

55. Lobular Capillary Hemangioma

56. Rapidly growing lesion characterized by proliferation of capillaries arranged in lobules, separated by loose connective tissue stroma, infiltrated by inflammatory cells. Age: 10 years – 72 years No gender predisposition Etiology: Trauma, hormonal influences, viral oncogenes, microscopic arteriovenous malformations, angiogenic growth factors

57. DNE: red to purple mass, <1cm, associated with epistaxis CT: unilateral mass with soft tissue density MRI: T2: Hyperintensity T1: Hypointensity CE: vivid enhancement Definitive diagnosis: Histological examination Management: Endoscopic radical resection

58. Fibrous Dysplasia

59. It is a genetical developmental anomaly of the bone-forming mesenchyme with a defect in osteoblastic differentiation and maturation, leading to replacement of normal bony tissue by fibrous tissue of variable cellularity and immature woven bone. Fibrous dysplasia, lacks capsule and has presence of more immature bone without osteoblastic activity. Psammomatoid ossifying fibroma, is a variant, with numerous small ossicles in stroma, resembling psammoma bodies.

60. Types: Monostotic Polyostotic Disseminated Presentation: Pain and pressure symptoms Compress optic nerve/ orbit

61. CT Scan: associated with degree of mineralization of the tissue Early – High density of fiberous tissue, Lesion: Radiolucent to lytic appearance Late – Ground glass to sclerotic appearance MRI: T1 – Hypointense T2 – Variable CE - non homogeneous enhancement

62. Management: Resection to relieve symptoms Resection: Partial vs Radical Medical Management: Bisphosphonates – inhibit osteoclastic activity

63. Ossifying Fibroma

64. Occurrence: 3 rd and 4 th decade of life Race: more common in black women Psammomatoid variant affects young men more commonly with aggressive local behavior Presents as SOL in nasal cavity on endoscopy CT: well defined, multiloculated lesion, bordered by peripheral eggshell-like dense rim MRI: T2: Hyperintense T1: central part : intermediately intense to hyperintense outer shell : hypointense

65. Management: Radical resection i /v/o high rate of relapse, with local destruction and invasion of adjacent structures

66. Schwannoma

67. Neurogenic tumor arising from schwann cells of sheath of myelinated nerves Age: 6 years to 78 years No sex predisposition Origin: V2 and V3, Sympathetic fibers of carotid plexus Parasympathetic fibers of pterygopalatine ganglion

68. Well delineated, unencapsulated globular, firm to rubbery, yellow – tan mass polypoid mass filling the left nasal cavity with network of capillaries on the surface of the lesion, suggesting a diagnosis of hypervascularized tumor.

69. HPE: cellular Antoni A areas with Verocay bodies and hypocellular myxoid Antoni B areas IHC: S 100 protein

70. Imaging CT: Non diagnostic MRI: shows histologic features of lesion lesions with a prevalent Antoni A component have an intermediate signal on both T1- and T2-weighted predominant Antoni B pattern, which is related to a loose myxoid stroma, hyperintensity is observed on T2-weighted images Management: Radical surgery

71. References Scott Brown Ed 6-8 Jatin P Shah Cummings Mohan Bansal Hazarika AIIMS ENT Global ENT Outreach Sydney ENT Clinic: Prof Richard Harvey Seattle science foundation Pathology outlines