1 neoplasms of the nose and paranasal sinus university of texas medical branch steven t. wright,...
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Neoplasms of the Neoplasms of the Nose and Nose and
Paranasal SinusParanasal SinusUniversity of Texas Medical University of Texas Medical
BranchBranchSteven T. Wright, M.D.Steven T. Wright, M.D.
Anna M. Pou, M.D.Anna M. Pou, M.D.May 19, 2004May 19, 2004
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Neoplasms of Nose and Neoplasms of Nose and Paranasal SinusesParanasal Sinuses
Very rare 3%Very rare 3% Delay in diagnosis due to similarity Delay in diagnosis due to similarity
to benign conditionsto benign conditions Nasal cavityNasal cavity
½ benign½ benign ½ malignant½ malignant
Paranasal SinusesParanasal Sinuses MalignantMalignant
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Neoplasms of Nose and Neoplasms of Nose and Paranasal SinusesParanasal Sinuses
Multimodality treatmentMultimodality treatment Orbital PreservationOrbital Preservation Minimally invasive surgical Minimally invasive surgical
techniquestechniques
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EpidemiologyEpidemiology Predominately of older malesPredominately of older males Exposure:Exposure:
Wood, nickel-refining processesWood, nickel-refining processes Industrial fumes, leather tanning Industrial fumes, leather tanning
Cigarette and Alcohol consumptionCigarette and Alcohol consumption No significant association has been No significant association has been
shownshown
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LocationLocation
Maxillary sinusMaxillary sinus 70%70%
Ethmoid sinusEthmoid sinus 20%20%
SphenoidSphenoid 3%3%
FrontalFrontal 1%1%
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PresentationPresentation
Oral symptoms: 25-35%Oral symptoms: 25-35% Pain, trismus, alveolar ridge fullness, Pain, trismus, alveolar ridge fullness,
erosionerosion Nasal findings: 50%Nasal findings: 50%
Obstruction, epistaxis, rhinorrheaObstruction, epistaxis, rhinorrhea Ocular findings: 25%Ocular findings: 25%
Epiphora, diplopia, proptosisEpiphora, diplopia, proptosis Facial signsFacial signs
Paresthesias, asymmetryParesthesias, asymmetry
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RadiographyRadiography
CTCT Bony erosionBony erosion Limitations with periorbita involvementLimitations with periorbita involvement
MRIMRI 94 -98% correlation with surgical findings94 -98% correlation with surgical findings Inflammation/retained secretions: low T1, Inflammation/retained secretions: low T1,
high T2high T2 Hypercellular malignancy: low/intermediate Hypercellular malignancy: low/intermediate
on bothon both Enhancement with GadoliniumEnhancement with Gadolinium
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Benign LesionsBenign Lesions
PapillomasPapillomas OsteomasOsteomas Fibrous DysplasiaFibrous Dysplasia Neurogenic tumorsNeurogenic tumors
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PapillomaPapilloma
Vestibular papillomasVestibular papillomas Schneiderian papillomas derived Schneiderian papillomas derived
from schneiderian mucosa from schneiderian mucosa (squamous)(squamous) Fungiform: 50%, nasal septumFungiform: 50%, nasal septum Cylindrical: 3%, lateral wall/sinusesCylindrical: 3%, lateral wall/sinuses Inverted: 47%, lateral wallInverted: 47%, lateral wall
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Inverted PapillomaInverted Papilloma
4% of sinonasal tumors4% of sinonasal tumors Site of Origin: lateral nasal wallSite of Origin: lateral nasal wall UnilateralUnilateral Malignant degeneration in 2-13% Malignant degeneration in 2-13%
(avg 10%)(avg 10%)
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Inverted PapillomaInverted PapillomaResectionResection
Initially via transnasal resection:Initially via transnasal resection: 50-80% recurrence50-80% recurrence
Medial Maxillectomy via lateral rhinotomy:Medial Maxillectomy via lateral rhinotomy: Gold StandardGold Standard 10-20%10-20%
Endoscopic medial maxillectomy:Endoscopic medial maxillectomy: Key concepts:Key concepts:
Identify the origin of the papillomaIdentify the origin of the papilloma Bony removal of this regionBony removal of this region
Recurrent lesions:Recurrent lesions: Via medial maxillectomy vs. Endoscopic Via medial maxillectomy vs. Endoscopic
resectionresection 22%22%
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OsteomasOsteomas
Benign slow growing tumors of Benign slow growing tumors of mature bonemature bone
Location:Location: Frontal, ethmoids, maxillary sinusesFrontal, ethmoids, maxillary sinuses
When obstructing mucosal flow can When obstructing mucosal flow can lead to mucocele formationlead to mucocele formation
Treatment is local excisionTreatment is local excision
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Fibrous dysplasiaFibrous dysplasia
Dysplastic transformation of normal Dysplastic transformation of normal bone with collagen, fibroblasts, and bone with collagen, fibroblasts, and osteoid materialosteoid material
Monostotic vs PolyostoticMonostotic vs Polyostotic Surgical excision for obstructing Surgical excision for obstructing
lesionslesions Malignant transformation to Malignant transformation to
rhabdomyosarcoma has been seen rhabdomyosarcoma has been seen with radiationwith radiation
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Neurogenic tumorsNeurogenic tumors 4% are found within the paranasal sinuses4% are found within the paranasal sinuses SchwannomasSchwannomas NeurofibromasNeurofibromas Treatment via surgical resectionTreatment via surgical resection Neurogenic Sarcomas are very aggressive Neurogenic Sarcomas are very aggressive
and require surgical excision with post op and require surgical excision with post op chemo/XRT for residual disease.chemo/XRT for residual disease.
When associated with Von When associated with Von Recklinghausen’s syndrome: more Recklinghausen’s syndrome: more aggressive (30% 5yr survival).aggressive (30% 5yr survival).
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Malignant lesionsMalignant lesions Squamous cell carcinomaSquamous cell carcinoma Adenoid cystic carcinomaAdenoid cystic carcinoma Mucoepidermoid carcinomaMucoepidermoid carcinoma AdenocarcinomaAdenocarcinoma HemangiopericytomaHemangiopericytoma MelanomaMelanoma Olfactory neuroblastomaOlfactory neuroblastoma Osteogenic sarcoma, fibrosarcoma, Osteogenic sarcoma, fibrosarcoma,
chondrosarcoma, rhabdomyosarcomachondrosarcoma, rhabdomyosarcoma LymphomaLymphoma Metastatic tumorsMetastatic tumors Sinonasal undifferentiated carcinomaSinonasal undifferentiated carcinoma
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Squamous cell carcinomaSquamous cell carcinoma
Most common tumor (80%)Most common tumor (80%) Location:Location:
Maxillary sinus (70%)Maxillary sinus (70%) Nasal cavity (20%)Nasal cavity (20%)
90% have local invasion by 90% have local invasion by presentationpresentation
Lymphatic drainage:Lymphatic drainage: First echelon: retropharyngeal nodesFirst echelon: retropharyngeal nodes Second echelon: subdigastric nodesSecond echelon: subdigastric nodes
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TreatmentTreatment
88% present in advanced stages 88% present in advanced stages (T3/T4)(T3/T4)
Surgical resection with Surgical resection with postoperative radiationpostoperative radiation Complex 3-D anatomy makes margins Complex 3-D anatomy makes margins
difficultdifficult
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Adenoid Cystic Adenoid Cystic CarcinomaCarcinoma
33rdrd most common site is the most common site is the nose/paranasal sinusesnose/paranasal sinuses
Perineural spreadPerineural spread Anterograde and retrogradeAnterograde and retrograde
Despite aggressive surgical resection Despite aggressive surgical resection and radiotherapy, most grow and radiotherapy, most grow insidiously.insidiously.
Neck metastasis is rare and usually a Neck metastasis is rare and usually a sign of local failuresign of local failure
Postoperative XRT is very importantPostoperative XRT is very important
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Mucoepidermoid Mucoepidermoid CarcinomaCarcinoma
Extremely rareExtremely rare Widespread local invasion makes Widespread local invasion makes
resection difficult, therefore resection difficult, therefore radiation is often indicatedradiation is often indicated
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AdenocarcinomaAdenocarcinoma
22ndnd most common malignant tumor in the most common malignant tumor in the maxillary and ethmoid sinusesmaxillary and ethmoid sinuses
Present most often in the superior portionsPresent most often in the superior portions Strong association with occupational exposuresStrong association with occupational exposures
High grade: solid growth pattern with High grade: solid growth pattern with poorly defined margins. 30% present with poorly defined margins. 30% present with metastasismetastasis
Low grade: uniform and glandular with Low grade: uniform and glandular with less incidence of perineural less incidence of perineural invasion/metastasis.invasion/metastasis.
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HemangiopericytomaHemangiopericytoma Pericytes of ZimmermanPericytes of Zimmerman Present as rubbery, pale/gray, well Present as rubbery, pale/gray, well
circumscribed lesions resembling nasal polypscircumscribed lesions resembling nasal polyps Treatment is surgical resection with Treatment is surgical resection with
postoperative XRT for positive marginspostoperative XRT for positive margins
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MelanomaMelanoma 0.5- 1.5% of melanoma originates from 0.5- 1.5% of melanoma originates from
the nasal cavity and paranasal sinus.the nasal cavity and paranasal sinus. Anterior Septum: most common siteAnterior Septum: most common site Treatment is wide local excision Treatment is wide local excision
with/without postoperative radiation with/without postoperative radiation therapytherapy
END not recommendedEND not recommended AFIP: Poor prognosisAFIP: Poor prognosis
5yr: 11%5yr: 11% 20yr: 0.5%20yr: 0.5%
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Olfactory NeuroblastomaOlfactory NeuroblastomaEsthesioneuroblastomaEsthesioneuroblastoma
Originate from stem cells of neural Originate from stem cells of neural crest origin that differentiate into crest origin that differentiate into olfactory sensory cells.olfactory sensory cells.
Kadish ClassificationKadish Classification A: confined to nasal cavityA: confined to nasal cavity B: involving the paranasal cavityB: involving the paranasal cavity C: extending beyond these limitsC: extending beyond these limits
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Olfactory NeuroblastomaOlfactory NeuroblastomaEsthesioneuroblastomaEsthesioneuroblastoma
UCLA Staging systemUCLA Staging system T1: Tumor involving nasal cavity and/or T1: Tumor involving nasal cavity and/or
paranasal sinus, excluding the sphenoid paranasal sinus, excluding the sphenoid and superior most ethmoidsand superior most ethmoids
T2: Tumor involving the nasal cavity T2: Tumor involving the nasal cavity and/or paranasal sinus including and/or paranasal sinus including sphenoid/cribriform platesphenoid/cribriform plate
T3: Tumor extending into the orbit or T3: Tumor extending into the orbit or anterior cranial fossaanterior cranial fossa
T4: Tumor involving the brainT4: Tumor involving the brain
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Olfactory NeuroblastomaOlfactory NeuroblastomaEsthesioneuroblastomaEsthesioneuroblastoma
Aggressive behaviorAggressive behavior Local failure: 50-75%Local failure: 50-75% Metastatic disease develops in 20-Metastatic disease develops in 20-
30%30% Treatment:Treatment:
En bloc surgical resection with En bloc surgical resection with postoperative XRTpostoperative XRT
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SarcomasSarcomas
Osteogenic SarcomaOsteogenic Sarcoma Most common primary malignancy of Most common primary malignancy of
bone.bone. Mandible > MaxillaMandible > Maxilla Sunray radiographic appearanceSunray radiographic appearance
FibrosarcomaFibrosarcoma ChondrosarcomaChondrosarcoma
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RhabdomyosarcomaRhabdomyosarcoma
Most common paranasal sinus Most common paranasal sinus malignancy in childrenmalignancy in children
Non-orbital, parameningealNon-orbital, parameningeal Triple therapy is often necessaryTriple therapy is often necessary Aggressive chemo/XRT has improved Aggressive chemo/XRT has improved
survival from 51% to 81% in patients survival from 51% to 81% in patients with cranial nerve with cranial nerve deficits/skull/intracranial involvement.deficits/skull/intracranial involvement.
Adults, Surgical resection with Adults, Surgical resection with postoperative XRT for positive margins.postoperative XRT for positive margins.
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LymphomaLymphoma
Non-Hodgkins typeNon-Hodgkins type Treatment is by radiation, with or Treatment is by radiation, with or
without chemotherapywithout chemotherapy Survival drops to 10% for recurrent Survival drops to 10% for recurrent
lesionslesions
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Sinonasal Undifferentiated Sinonasal Undifferentiated CarcinomaCarcinoma
Aggressive locally destructive lesionAggressive locally destructive lesion Dependent on pathological Dependent on pathological
differentiation from melanoma, differentiation from melanoma, lymphoma, and olfactory lymphoma, and olfactory neuroblastomaneuroblastoma
Preoperative chemotherapy and Preoperative chemotherapy and radiation may offer improved radiation may offer improved survivalsurvival
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Metastatic TumorsMetastatic Tumors
Renal cell carcinoma is the most Renal cell carcinoma is the most commoncommon
Palliative treatment onlyPalliative treatment only
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Staging of Maxillary Sinus Staging of Maxillary Sinus TumorsTumors
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Staging of Maxillary Sinus Staging of Maxillary Sinus TumorsTumors
T1: limited to antral mucosa without bony T1: limited to antral mucosa without bony erosionerosion
T2: erosion or destruction of the T2: erosion or destruction of the infrastructure, including the hard palate infrastructure, including the hard palate and/or middle meatusand/or middle meatus
T3: Tumor invades: skin of cheek, posterior T3: Tumor invades: skin of cheek, posterior wall of sinus, inferior or medial wall of orbit, wall of sinus, inferior or medial wall of orbit, anterior ethmoid sinusanterior ethmoid sinus
T4: tumor invades orbital contents and/or: T4: tumor invades orbital contents and/or: cribriform plate, post ethmoids or sphenoid, cribriform plate, post ethmoids or sphenoid, nasopharynx, soft palate, pterygopalatine or nasopharynx, soft palate, pterygopalatine or infratemporal fossa or base of skullinfratemporal fossa or base of skull
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SurgerySurgery
Unresectable tumors:Unresectable tumors: Superior extension: frontal lobesSuperior extension: frontal lobes Lateral extension: cavernous sinusLateral extension: cavernous sinus Posterior extension: prevertebral fasciaPosterior extension: prevertebral fascia Bilateral optic nerve involvementBilateral optic nerve involvement
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SurgerySurgery Surgical approaches:Surgical approaches:
EndoscopicEndoscopic Lateral rhinotomyLateral rhinotomy Transoral/transpalatalTransoral/transpalatal Midfacial deglovingMidfacial degloving Weber-FergussonWeber-Fergusson Combined craniofacial approachCombined craniofacial approach
Extent of resectionExtent of resection Medial maxillectomyMedial maxillectomy Inferior maxillectomyInferior maxillectomy Total maxillectomyTotal maxillectomy
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TracheostomyTracheostomy
130 maxillectomies only 7.7% 130 maxillectomies only 7.7% required tracheostomyrequired tracheostomy
Of those not receiving tracheostomy Of those not receiving tracheostomy during surgery, only 0.9% during surgery, only 0.9% experienced postoperative airway experienced postoperative airway complicationscomplications
Tracheostomy is unnecessary except Tracheostomy is unnecessary except in certain circumstances (bulky in certain circumstances (bulky packing/flaps, mandibulectomy)packing/flaps, mandibulectomy)
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Treatment of the OrbitTreatment of the Orbit
Before 1970’s orbital exenteration Before 1970’s orbital exenteration was included in the radical resectionwas included in the radical resection
Preoperative radiation reduced Preoperative radiation reduced tumor load and allowed for orbital tumor load and allowed for orbital preservation with clear surgical preservation with clear surgical marginsmargins
Currently, the debate is centered on Currently, the debate is centered on what “degree” of orbital invasion is what “degree” of orbital invasion is allowed.allowed.
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Current indications for Current indications for orbital exenterationorbital exenteration
Involvement of the orbital apexInvolvement of the orbital apex Involvement of the extraocular musclesInvolvement of the extraocular muscles Involvement of the bulbar conjunctiva Involvement of the bulbar conjunctiva
or scleraor sclera Lid involvement beyond a reasonable Lid involvement beyond a reasonable
hope for reconstructionhope for reconstruction Non-resectable full thickness invasion Non-resectable full thickness invasion
through the periorbita into the through the periorbita into the retrobulbar fatretrobulbar fat
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ConclusionsConclusions
Neoplasms of the nose and Neoplasms of the nose and paranasal sinus are very rare and paranasal sinus are very rare and require a high index of suspicion for require a high index of suspicion for diagnosisdiagnosis
Most lesions present in advanced Most lesions present in advanced states and require multimodality states and require multimodality therapytherapy
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