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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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