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Sino-nasal Malignancies PRESENTATION ENT
- 1.
- 2. tumours of nasalcavity Benign squamous papilloma inverted papilloma pleomorphic adenoma schwannoma meningioma hemangioma chondroma angiofibroma encephalocele glioma dermoid Malignant Carcinoma (Squamous cell Ca., Adenocarcinoma) malignant melanoma olfactory neuroblastoma haemangiopericytoma lymphoma solitary plasmacytoma various type of sarcoma
- 3. tumours of paranasalsinuses Benign -osteomas -fibrous dysplasia -ossifying fibromas -ameloblastoma Malignant -Carcinoma of maxillary sinus, ethmoid sinus, frontal sinus, sphenoid sinus. *80% SCC, rest adenocarcinoma, adenoid cystic carcinoma,melanoma, various type sarcomas
- 4. Tumors ofthe nasal cavity proper are approximately evenly divided between benign and malignant neoplasia, with inverting papilloma predominating in the benign group and squamous cell carcinoma in the malignant.
- 5. On theother hand, most sinus tumors are malignant with squamous cell carcinoma being the most prevalent. The maxillary sinus is most commonly involved with tumor, followed by the nasal cavity, the ethmoids, and then the frontal and sphenoid sinuses.
- 6. Papillomas 1.vestibular papillomas 2.schneiderian papillomas-derived from schneiderian mucosa (squamous) • fungiform- 50% nasal septum • cylindrical- 3% lateral wall/sinuses • inverted- 47% lateral wall
- 7. Inverted papillomas Etiologyis unclear. High recurrence rates. Associated with malignancy Male predominance Locally aggressive growth patterns,( technically difficult to remove ) There is also controversy over the appropriate surgical approach for tumor removal. There is a role for radiation therapy.
- 8. Inverted papillomas Papillomasdiffer from inflammatory polyps, which are more common, in that inflammatory polyps are associated with allergic rhinitis and are actually reactive lesions, not a tumor. Nasal papillomas are true neoplasms and, while their etiology is unclear, they are known to arise from the nasal respiratory epithelium, which undergoes metaplastic change and proliferation.
- 9. They arecommonly located in the nasal cavity and they typically involve an adjacent sinus. The most common location is the middle turbinate, but other common locations include the ethmoid sinus and maxillary sinus. They have even been found in the nasopharynx.
- 10. Most commonsymptoms are unilateral and include nasal obstruction, nosebleed and nasal discharge. It can be an incidental finding on examination. They are associated with malignancy, 5% to 15%. Inverted papillomas are more commonly associated with squamous cell carcinomas.
- 11. Cannady (2007) furtherrecommended a modification of the Krause staging for IP as follows: Modified Krause staging A – Inverted papilloma (IP) confined to the nasal cavity, ethmoid sinus, or medial maxillary wall B – Inverted papilloma (IP) with involvement of any maxillary wall (other than the medial wall) or frontal sinus or sphenoid sinus C – Inverted papilloma (IP) with extension beyond the paranasal sinuses
- 12. • Tumors ingroup A were amenable to complete endoscopic resection and low recurrence rates (RR) of (3%). • Tumors in group B could be resected endoscopically but often required adjunctive procedures (Caldwell-Luc approaches or osteoplastic frontal sinusotomy, respectively) for removal and RR of 20%. • Tumors in group C always required open approaches, and RR were closer to 35%. • Risk factors for the recurrence of inverted papillomas of the sphenoid sinus include the presence of dysplasia or carcinoma in situ (CIS) and attachment of the tumor over the optic nerve or carotid artery
- 13. The mainstay oftreatment is surgery, although radiation therapy can be involved. Surgery either a transnasal procedure with polypectomy or confined transnasal polypectomy with additional sinus procedure, such as Caldwell-Luc. The gold standard was medial maxillectomy via lateral rhinotomy or en bloc ethmoidectomy Radiation therapy - absolute indication for radiation therapy is when an inverted papilloma is associated with squamous cell carcinoma. Those -advanced incompletely resected or unresectable lesions that are biologically aggressive -patients where morbidity in resection would be more pronounced than morbidity of tumor radiation.
- 14. Sinonasal neoplasms Theseare rare, comprising less than 3% of all malignant aerodigestive tumors and less than 1% of all malignancies. typically affect Caucasion males in the fifth to seventh decades of life ,2:1 male preponderance.
- 15. Sinus Anatomy: Maxillary antrum Significance Superior – orbit, ethmoids Posterior – pterygoids, infratemporal fossa Ethmoid sinus Significance Superior – fovea, cribiform Medial – lamina papyracea
- 16. Sinus Anatomy: Sphenoidsinus Significance Superior – optic nerve, pituitary Lateral – ICA, cavernous sinus Inferior – vidian nerve
- 17. Frontal sinus Significance Inferior – orbit Posterior – anterior cranial fossa
- 18. Lymphatic Drainage Theanterior nose has the same lymphatic drainage as the external nose. These tend to spread to the submental or level I area, and also to level II and III The posterior nasal cavity,posterior ethmoids and sphenoids tends to drain to the retropharyngeal nodes as well as the lateral pharyngeal nodes, which eventually drain into the level II.
- 19. Etiology Up to44% are attributed to occupational exposures, including nickel, chromium, isopropyl oils, volatile hydrocarbons, and organic fibers that are found in the wood, shoe, and textile industries. In addition, human papilloma virus can be a cofactor Specific asssociations found include squamous cell carcinoma in nickel workers and adenocarcinoma in workers exposed to hardwood dusts and leather tanning.
- 20. The mostcommon entities are squamous cell carcinoma. The lateral nasal wall is the most common site of involvement, but SCC can also present in the sinuses(most common maxillary followed by ethmoid, frontal and sphenoid). Adenocarcinoma is the second most common malignancy in this area. It is most often in the ethmoids, has a male predominance, and is often seen in industrial workers.
- 21. Squamous cell carcinoma -mostcommon tumor (80%) -Location -maxillary sinus 70% -nasal cavity 20% -90% have local invasion by presentation -88% present in advanced stage (T3/T4) -Tx -surgical resection with postoperative radiation
- 22. Adenocarcinoma -2nd most commonmalignant tumor in in maxillary and ethmoid sinuses -present most often in the superior portion (strong asso. with occupational exposure) -High grade- solid growth pattern with poorly defined margin (30% present with metastasis) -Low grade- uniform and glandular with less incidence of perineural invasion/metastasis.
- 23. Adenoid cystic carcinoma -3rdmost common site is the nose/paranasal sinuses -perineural spread - anterograde & retrograde -despite aggressive surgical recection and radiotherapy,most grow insidously -neck metastasis is rare and usually a sign of local failure -postoperative RT is very important
- 24. Mucoepidermoid carcinoma -extremely rare -widespreadlocal invasion makes resection difficult, therefore radiation is often indicated
- 25. Hemangiopericytoma -pericytes of Zimmerman -presentas pale, rubbery, well circumscribed lesion resembling nasal polyp -Tx- surgical resection with postoperative RT for positive margin
- 26. Melanoma -0.5-1.5% of melanomaoriginates from nasal cavity and paranasal sinuses -anterior septum- most common site -treatment is wide excision with/without postoperative radiation therapy
- 27. Olfactory neuroblastoma Esthesioneuroblastoma -originates fromstem cells of neural crest origin that differentiate into olfactory sensory cells -aggressive behaviour -local failure 50-75% -metastatic disease develop in 20-30% -treatment is en-bloc surgical resectionwith postoperative RT
- 28. Sarcomas -osteogenic sarcomas • mostcommon primary malignancy of bone • mandible > maxilla • sunray radigraphic appearance -fibrosarcoma -chondrosarcoma
- 29. Rhabdomyosarcoma -most common paranasalsinus malignancy in children -non-orbital, parameningeal -triple therapy often necessary -agressive chemo/RT has improved survival from 51% to 81% in patients with cranial nerve deficits/skull/intracranial involvement
- 30. Lymphoma -Non-Hodgkins type -strong asso.with EBV -tumor is a destructive sinonasal lesion associated with obstructive symptoms, bone and soft-tissue destruction, and hemorrhage -Tx - radiation with/without chemotherapy -survival drops to 10% of recurrent lesion
- 31. Sinonasal Undifferentiated Carcinoma -aggressive locallydestructive lesion -dependent on pathological differentiation from melanoma, lymphoma, and olfactory neuroblastoma -preoperative radiation and chemotherapy offer improved survival
- 32. Metastatic tumors -Tumors thatmost frequently metastasize to this bony region are those that are well known to metastasize to other bones which include prostate, breast, kidney, lung, and thyroid. -RCC is the most common -palliative treatment only
- 33. Carcinoma of MaxillarySinus Clinical features • common in 40 - 60 age group, preponderance in males. • Early feature- epistaxis, nasal obstruction, recurrent sinusitis, cranial neuropathy, sinus pain, facial paresthesia, proptosis, diplopia, or an asymptomatic neck mass • Ominous signs and symptoms (eg, severe intractable headache, visual disturbances)
- 34. Signs and symptomsof maxillary sinus carcinoma fall into several major categories Oral Nasal Ocular, facial Auditory
- 35. Oral presentationsoccur in 25-35% and include pain involving the maxillary dentition,loosening of teeth, trismus, palatal and alveolar ridge fullness, and frank erosion into the oral cavity. Nasal findings are seen in up to 50% of patients and include obstruction, discharge, stuffiness, congestion, epistaxis, and extension into the nasal cavity.
- 36. Ocular findings occurin approximately 25% and arise from upward extension into the orbit, where unilateral tearing, diplopia, fullness of lids, pain, and exophthalmos are seen
- 37. Facial signsinclude infra-orbital nerve hypoesthesia, cheek swelling, pain, and facial asymmetry. Auditory complaints include hearing loss secondary to serous otitis media due to nasopharyngeal extension. With advanced disease, the classic triad of findings for carcinoma of the nasal cavity and paranasal sinuses may be present: These include Facial asymmetry A visible or palpable tumor bulge in the oral cavity Tumor visible in the nose with anterior rhinoscopy.
- 38. Classification Ohngren line,a line that is drawn from the angle of mandible to the medial canthus. Ohngren's classification indicates that tumors presented above this line, both superiorly and posteriorly (suprastructural), have a poorer prognosis than infrastructural.
- 39. American Joint Committeeon Cancer (AJCC) Staging System is the gold standard used for reporting, only for SCC.Histopathologically further divided into 3 (well, moderate and poorly differentiated, alsoto note on vascular and perineural invasion.) T1 tumors restricted to any one sub-site, with or without bony invasion. T2 tumors invading two sub-sites in single region or extending to involve adjacent regions of the nasoethmoidal complex,with or without bony invasion T3 tumors begin to have bony involvement, invade the medial wall or floor of the orbit,maxillary sinus, cribriform plate or palate. T4-A tumors involve any of the following; anterior orbital contents, skin of nose or cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or frontal sinuses. T4-B tumors involve any of the following; orbital apex, dura, brain, middle cranial nerves other than V2, nasopharynx or clivus.
- 40. • Lederman's classification.Uses 2 horizontal lines of Sebileau ; one passing through the floor of orbits and the other passing through floors of antra; thus dividing the area into; • a)suprastrucure - ethmoid, sphenoid, and frontal sinuses and olfactory area of the nose • b)mesostructure - maxillary sinus and respiratory part of nose • c)infrastructure - contain alveolar process • *further uses vertical lines, extending down the medial walls of orbit to separate ethmoid sinuses and nasal fossa from maxillary sinuses.
- 43. Investigations CT scansare excellent for determining bony erosion and extent of invasion.
- 44. MRI isexcellent for determining perineural spread, involvement of the dura, or involvement intracranially.
- 45. confirm diagnosisvia biopsy (often biopsy is performed after imaging rule out encephaloceles or other vascular issues) PET scan has been used to evaluate for residual tumor, recurrent tumor, and radiated treated fields. Angiography is not initially used, but can be used for vascular tumors to determine extent and vascularity as well as to allow for embolization prior to any surgical interventions.
- 46. Management (depend onhistology, location and extend of the disease) Adenocarcinoma: Treatment is controversial, but the literature indicates that craniofacial resection is the key.
- 47. Management SCC: Forthe treatment of early lesions, surgery, if the tumor is excised en bloc with good margins, and, if there is no evidence of perineural spread, then surgery is usually sufficient. If there are any questions about the margins or perineural invasion, the addition of radiation is indicated External Inferior medial Medial Radical
- 48. Management Combined modalitygenerally tends to be the gold standard: surgery with postoperative radiation therapy.
- 49. Management Also, theuse of chemotherapy is now being added with the goal of better local control and improvement in survival. Chemotherapy does have a role in palliation for large tumors that are nonresectable. If there is nodal disease of the neck with squamous cell carcinoma, a neck dissection is generally indicated
- 50. • Maxillary sinusca - surgical excision of the growth by total or extended maxillectomy (weber-fugerson's incision used in maxillectomy).radiotherapy can be given before or after the surgery • Ethmoid sinus ca -CF nasal obstruction, blood stained nasal discharge and retro-orbital pain -late features- broadening nasal root, lateral displacement of eyeball, diplopia. Extension through cribriform plate- cause meningitis
- 51. -early cases- preoperativeradiation, then lateral rhinotomy and total ethmoidectomy -cribriform plate involved-craniofacial resection (ant cranial fossa exposed) • Frontal sinus ca • uncommon, age 40-50,male • CF-pain and swelling of frontal region, may extend into orbit through ethmoid. Dura of ant cranial fossa involved if growth penetrates post wall of sinus • Tx- preop radiation then surgery (frontal sinusectomy with ethmoid and orbital exentration). • Sphenoid sinus ca • rare, radiotherapy is mainstay of tx
- 52. Unresectable tumors -superior extension: frontal lobes -lateral extension : cavernous sinus -posterior extension :prevertebral fascia -bilateral optic nerve involvement
- 53.