Neoplasms of the Paranasal Neoplasms of the Paranasal SinusSinus

University of Esfahan MedicalJune 22, 2009 cummings,51

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are uncommon in the general population.are uncommon in the general population. most common are squamous cell most common are squamous cell

carcinomas, frequency of <1:200,000 per carcinomas, frequency of <1:200,000 per yearyear

diagnosis usually becomes evident only diagnosis usually becomes evident only after an advanced stage has been after an advanced stage has been reached, thus explaining the relatively reached, thus explaining the relatively poor prognosis. poor prognosis.

ANATOMYANATOMY

Maxillary Antrum Maxillary Antrum The most significant structures prognostically are The most significant structures prognostically are

above and behind the maxillary antrum.above and behind the maxillary antrum. Superiorly, orbit and the ethmoid sinus.Superiorly, orbit and the ethmoid sinus. Posterior, pterygoid plates, the pterygoid space, Posterior, pterygoid plates, the pterygoid space,

and the infratemporal fossa. and the infratemporal fossa. Anteriorly is the canine fossa,Anteriorly is the canine fossa, inferiorly the alveolus,inferiorly the alveolus, and medially the nasal cavity.and medially the nasal cavity.

Ethmoid SinusEthmoid Sinus greater concern is spread superiorly, where greater concern is spread superiorly, where

the fovea ethmoidalis and the cribriform plate the fovea ethmoidalis and the cribriform plate provide little barrier to intracranial spread.provide little barrier to intracranial spread.

The lateral wall consists of the thin bone of The lateral wall consists of the thin bone of the lamina papyracea, which also provides the lamina papyracea, which also provides little resistance to neoplastic invasion.little resistance to neoplastic invasion.

Sphenoid Sinus Sphenoid Sinus Located the sphenoid sinus relates to very Located the sphenoid sinus relates to very

complex and vital structures.complex and vital structures. superior, The optic nerve and the pituitary superior, The optic nerve and the pituitary

glandgland Laterally,the internal carotid artery and the Laterally,the internal carotid artery and the

cavernouscavernous Anterior to the sinus are posterior ethmoidal Anterior to the sinus are posterior ethmoidal

cells,cells, and inferiorly are the vidian nerve and the and inferiorly are the vidian nerve and the

nasopharynx.nasopharynx.

Frontal Sinus Frontal Sinus The least frequently involved by malignant The least frequently involved by malignant

tumortumor anteriorly by the soft tissues of the scalp, anteriorly by the soft tissues of the scalp,

inferiorly by the ethmoidal cells and the inferiorly by the ethmoidal cells and the orbitorbit

posteriorly by the anterior cranial fossa.posteriorly by the anterior cranial fossa. It is important to realize that all of the It is important to realize that all of the

sinuses have venous communications with sinuses have venous communications with intracranial veins, which provide yet intracranial veins, which provide yet another avenue for lethal spread of another avenue for lethal spread of neoplasm.neoplasm.

Lymphatics Lymphatics they do metastasize but that clinical they do metastasize but that clinical

evidence of early metastasis is absent, evidence of early metastasis is absent, because the primary drainage is to the because the primary drainage is to the lateral and retropharyngeal nodeslateral and retropharyngeal nodes

SYMPTOMSSYMPTOMS

early symptoms :early symptoms :

facial or dental pain,facial or dental pain,

nasal obstruction,nasal obstruction,

and epistaxis. and epistaxis. These early signs represent the impact These early signs represent the impact

of a necrotic infected mass situated of a necrotic infected mass situated within the paranasal sinus or nose. within the paranasal sinus or nose.

additional symptoms as additional symptoms as follows:follows:

    1.  1.  Diplopia or vision lossDiplopia or vision loss    2.       2.    EpiphoraEpiphora    3.       3.    Facial swelling and malocclusionFacial swelling and malocclusion

      4.    4.    TrismusTrismus signals signals    5.       5.    Neck massNeck mass, palpable metastatic , palpable metastatic adenopathy in the jugular chain adenopathy in the jugular chain

first nodes are located in the first nodes are located in the parapharyngeal region.parapharyngeal region.   6.       6.    Hearing lossHearing loss

      7.    7.    Facial numbnessFacial numbness

Hearing lossHearing loss usually results from usually results from nasopharyngeal extension of the tumor nasopharyngeal extension of the tumor causing serous otitis.causing serous otitis.

contraindication to surgery:contraindication to surgery:

nasopharyngeal extension of disease   nasopharyngeal extension of disease   

PHYSICAL FINDINGSPHYSICAL FINDINGS

    1.    1.    Nasal, facial, or intraoral massNasal, facial, or intraoral mass. The widening of . The widening of the upper alveolar ridge or development of loose, the upper alveolar ridge or development of loose, nonvital teeth may be the earliest sign of inferior bony nonvital teeth may be the earliest sign of inferior bony invasion. invasion.

A palatal mass and ulceration are evidence of more A palatal mass and ulceration are evidence of more advanced disease.advanced disease.   2.       2.    Proptosis.Proptosis.    3.       3.    Cranial nerve deficits.Cranial nerve deficits. The commonly involved The commonly involved cranial nerves are the CN II, CN III, CN IV, two cranial nerves are the CN II, CN III, CN IV, two branches of CN V (CN V1 and CN V2), and CN VI. branches of CN V (CN V1 and CN V2), and CN VI.

Involvement of cranial nerves is a manifestation of Involvement of cranial nerves is a manifestation of advanced disease and indicates a poor prognosis.advanced disease and indicates a poor prognosis.

DIAGNOSTIC ASSESSMENTDIAGNOSTIC ASSESSMENT Computed Tomography ScanComputed Tomography Scan CT is equal to tomography for assessment of bony CT is equal to tomography for assessment of bony

involvement. involvement. CT scanning may also be misleading in evaluation of CT scanning may also be misleading in evaluation of

the orbital floor.the orbital floor. helpfull involvement of the retroorbital and orbital apex helpfull involvement of the retroorbital and orbital apex

region.region. assessing infiltration of the nasopharynxassessing infiltration of the nasopharynx evaluation of intracranial extension is necessary, evaluation of intracranial extension is necessary,

contrast-enhanced CT scanningcontrast-enhanced CT scanning

AngiographyAngiography the lesion demonstrates enhancement the lesion demonstrates enhancement

during initial CT studyduring initial CT study it approximates the carotid system.it approximates the carotid system. evaluation of unusual tumors involving the evaluation of unusual tumors involving the

sphenoid sinus and skull basesphenoid sinus and skull base essential for assessment of tumor extent essential for assessment of tumor extent

and delineation of feeding vessels and as and delineation of feeding vessels and as an approach for embolization an approach for embolization

UltrasoundUltrasound B-mode scanning: is helpful in B-mode scanning: is helpful in assessing orbital massesassessing orbital masses

Positron Emission Tomography (PETPositron Emission Tomography (PET):): PET scanning is of little use in assessing PET scanning is of little use in assessing

tumor extent; tumor extent; play some role in assessing the presence of play some role in assessing the presence of

metastatic disease before oncologic surgery metastatic disease before oncologic surgery follow-up after concomitant chemoradiation. follow-up after concomitant chemoradiation. pretreatment PET provides the optimal pretreatment PET provides the optimal

baseline for posttreatment decision making.baseline for posttreatment decision making.

Magnetic Resonance Imaging (MRI)Magnetic Resonance Imaging (MRI)

The impact of MRI on the preoperative The impact of MRI on the preoperative assessment of tumor extent has been assessment of tumor extent has been dramatic. dramatic.

The particular benefit of MRI is its ability The particular benefit of MRI is its ability to differentiate tissue density and the to differentiate tissue density and the difference between tumor bulk and difference between tumor bulk and retained secretions in the sinuses. retained secretions in the sinuses.

BiopsyBiopsy

Tumors contained within the sinus cavities Tumors contained within the sinus cavities should be biopsied transnasally, should be biopsied transnasally,

possibility of a vascular tumor or an possibility of a vascular tumor or an encephalocele when biopsying unilateral nasal encephalocele when biopsying unilateral nasal masses. masses.

Aspiration cytology for diagnosis deep tumors Aspiration cytology for diagnosis deep tumors of the antrum. of the antrum.

useful for tumors that cause proptosis and useful for tumors that cause proptosis and present along the medial aspect of the orbit. present along the medial aspect of the orbit. Because the diagnosis in this setting includes Because the diagnosis in this setting includes benign lesions such as an orbital benign lesions such as an orbital pseudotumor, histologic evaluation before pseudotumor, histologic evaluation before surgical invasion is most appropriate.surgical invasion is most appropriate.

SURGICAL OPTIONSSURGICAL OPTIONS

External EthmoidectomyExternal Ethmoidectomy Indications :Indications : removal of benign tumors of the ethmoidal regionremoval of benign tumors of the ethmoidal region approach to biopsy and drainage for tumors of the approach to biopsy and drainage for tumors of the

sphenoethmoidal region and the medial orbit.sphenoethmoidal region and the medial orbit. Benefits Benefits allows excellent cosmesis and preservation of functional allows excellent cosmesis and preservation of functional

tissue.tissue. Limitations: will not provide en bloc excision for any but the Limitations: will not provide en bloc excision for any but the

most limited tumors (middle turbinate).most limited tumors (middle turbinate). there is a tendency to form a fistula to the nasal cavity there is a tendency to form a fistula to the nasal cavity

when this area is irradiated.when this area is irradiated.

Inferior Medial MaxillectomyInferior Medial Maxillectomy Indications Indications Inferior medial maxillectomy is designed for Inferior medial maxillectomy is designed for

resection of the medial wall of the antrum resection of the medial wall of the antrum and the inferior turbinate.and the inferior turbinate.

It is most often used for management of an It is most often used for management of an inverted papilloma. inverted papilloma.

Medial MaxillectomyMedial Maxillectomy

Indications Indications for larger benign or intermediate tumors for larger benign or intermediate tumors

involving the entire lateral nasal wall but involving the entire lateral nasal wall but without extension to the orbit, anterior without extension to the orbit, anterior cranial fossa, lateral maxilla, or alveolus.cranial fossa, lateral maxilla, or alveolus.

LimitationsLimitations Removal of all turbinate tissue results in an Removal of all turbinate tissue results in an

abnormal nasal cavity, often requiring abnormal nasal cavity, often requiring chronic management of crusting.chronic management of crusting.

Radical MaxillectomyRadical Maxillectomy Indications Indications the standard operation for advanced the standard operation for advanced

carcinoma of the maxilla. carcinoma of the maxilla. Complete radical maxillectomy includes Complete radical maxillectomy includes

removal of the maxilla along with the nasal removal of the maxilla along with the nasal bone, the ethmoid sinus, and in some bone, the ethmoid sinus, and in some instances, the pterygoid plates.instances, the pterygoid plates.

Surgical Approach Surgical Approach The Weber-Fergusson incision is used with The Weber-Fergusson incision is used with

extensions around the eyelids to preserve extensions around the eyelids to preserve those soft tissuesthose soft tissues

A skin or dermis graft is used to line the A skin or dermis graft is used to line the defect.defect.

A preformed obturator can be of great A preformed obturator can be of great assistance in the immediate postoperative assistance in the immediate postoperative period by acting as a support for packing.period by acting as a support for packing.

Limitations Limitations Even when orbital exenteration is included,Even when orbital exenteration is included, fails to provide when the tumor has escaped fails to provide when the tumor has escaped

superiorly (ethmoidal roof) or posteriorly superiorly (ethmoidal roof) or posteriorly (orbital apex, pterygoid region). (orbital apex, pterygoid region).

Craniofacial FrontoethmoidectomyCraniofacial Frontoethmoidectomy

Indications Indications designed to provide en bloc resection for designed to provide en bloc resection for

tumors of the ethmoidal and frontal regionstumors of the ethmoidal and frontal regions If the tumor crosses the midline, the If the tumor crosses the midline, the

procedure may be performed bilaterally.procedure may be performed bilaterally.

Benefits Benefits Craniofacial exposure provides direct visualization of the Craniofacial exposure provides direct visualization of the

cribriform plate and the fovea ethmoidalis and the potential cribriform plate and the fovea ethmoidalis and the potential for en bloc removal. for en bloc removal.

It also provides wide exposure to allow effective repair of It also provides wide exposure to allow effective repair of dural tears, thus decreasing the chance of a postoperative dural tears, thus decreasing the chance of a postoperative CSF leak and intracranial infection.CSF leak and intracranial infection.

would allow intraoperative irradiation or placement of a would allow intraoperative irradiation or placement of a radioactive implant.radioactive implant.

Limitations Limitations If the tumor extends to the sphenoid sinus, the cavernous If the tumor extends to the sphenoid sinus, the cavernous

sinus, or transdurally, en bloc resection cannot be sinus, or transdurally, en bloc resection cannot be achieved.achieved.

Extended Craniofacial ResectionExtended Craniofacial Resection Indications Indications Extensive tumors involving the anterior skull base, with Extensive tumors involving the anterior skull base, with

involvement of the pterygoid plates, involvement of the pterygoid plates, Bony Margins Bony Margins The posterior line of resection is defined by the oval foramen, the The posterior line of resection is defined by the oval foramen, the

round foramen, and the internal carotid artery. The excision may round foramen, and the internal carotid artery. The excision may extend through the sphenoid sinus and up to the contralateral extend through the sphenoid sinus and up to the contralateral optic nerve.optic nerve.

Limitations Limitations pterygoid plate erosion and cranial nerve invasion as indicators of pterygoid plate erosion and cranial nerve invasion as indicators of

inoperability.inoperability. it does not provide adequate en bloc resection for tumor at the it does not provide adequate en bloc resection for tumor at the

orbit apex, in the nasopharynx, or deeply infiltrating the pterygoid orbit apex, in the nasopharynx, or deeply infiltrating the pterygoid space.space.

Benign tumorsBenign tumors

PapillomaPapilloma Inverting papillomaInverting papilloma MeningiomaMeningioma NeuromaNeuroma HemangiomaHemangioma OsteomaOsteoma AmeloblastomaAmeloblastoma Fibrous dysplasiaFibrous dysplasia

OsteomasOsteomas frontal sinus> the ethmoid sinus > maxilla. frontal sinus> the ethmoid sinus > maxilla. When located away from the sinus osteum, an When located away from the sinus osteum, an

osteoma is silent ,followed with an interval osteoma is silent ,followed with an interval radiograph in 1 to 2 years radiograph in 1 to 2 years

when they interfere with sinus drainage or possibly when they interfere with sinus drainage or possibly when they impinge on the dura,they symptomizedwhen they impinge on the dura,they symptomized

If causes a mucocele, obliterative surgery or wide If causes a mucocele, obliterative surgery or wide drainage is recommended drainage is recommended

Chondromas develop anywhere in the sinonasal Chondromas develop anywhere in the sinonasal tracttract

resection should be more aggressive than with an resection should be more aggressive than with an osteoma, "fairly radical treatment.”osteoma, "fairly radical treatment.”

Schwannomas and NeurofibromasSchwannomas and Neurofibromas

arising from peripheral nerve components.arising from peripheral nerve components. A schwannoma is an isolated encapsulated lesion, A schwannoma is an isolated encapsulated lesion,

whereas a neurofibroma is woven into the nerve and whereas a neurofibroma is woven into the nerve and is often one of multiple lesions.is often one of multiple lesions.

cause symptoms :if may distort tissues by pressure or cause symptoms :if may distort tissues by pressure or by obstruction of a sinus osteum.by obstruction of a sinus osteum.

evaluation, a contrast-enhanced CT scan,demonstrate evaluation, a contrast-enhanced CT scan,demonstrate a characteristic irregular patchy appearancea characteristic irregular patchy appearance

managed by conservative local resection.managed by conservative local resection. malignant transformation occurs in about one of malignant transformation occurs in about one of

eight patients with multiple neurofibromatosis (von eight patients with multiple neurofibromatosis (von Recklinghausen's diseaseRecklinghausen's disease

Ossifying Fibromas and Ossifying Fibromas and CementomasCementomas

difficult to differentiate histologically from fibrous difficult to differentiate histologically from fibrous dysplasiadysplasia

ossifying fibromas may be diagnosed by ossifying fibromas may be diagnosed by including clinical and radiographic criteria. including clinical and radiographic criteria.

occur in primarily young adults,occur in primarily young adults, typified radiographically by a sclerotic bony typified radiographically by a sclerotic bony

margin that is evident at surgical resection, when margin that is evident at surgical resection, when the lesion often "shells out" easilythe lesion often "shells out" easily

Cementomas are described as a variant of Cementomas are described as a variant of ossifying ossifying

managed by local excision,managed by local excision, if any tumor is left, a recurrence is likely.if any tumor is left, a recurrence is likely.

Fibrous dysplasiaFibrous dysplasia Dysplastic transformation of normal bone Dysplastic transformation of normal bone

with collagen, fibroblasts, and osteoid with collagen, fibroblasts, and osteoid materialmaterial

Surgical excision for obstructing lesionsSurgical excision for obstructing lesions Malignant transformation to Malignant transformation to

rhabdomyosarcoma has been seen with rhabdomyosarcoma has been seen with radiationradiation

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Odontogenic TumorsOdontogenic Tumors

for only 1% of all jaw tumorsfor only 1% of all jaw tumors include ameloblastoma and the calcifying include ameloblastoma and the calcifying

epithelial tumor of Pindborg.epithelial tumor of Pindborg. They are locally aggressive,They are locally aggressive, Manage: resection with a small margin of Manage: resection with a small margin of

normal tissue normal tissue Most odontomas are emphasizing their Most odontomas are emphasizing their

benign naturebenign nature

INTERMEDIATE NEOPLASMSINTERMEDIATE NEOPLASMS

Inverted PapillomasInverted Papillomas usually present as polypoid unilateral nasal usually present as polypoid unilateral nasal

masses. masses. On occasion, they may be found in On occasion, they may be found in

association with allergic nasal polypassociation with allergic nasal polyp malignant change approximates 10%.malignant change approximates 10%. A lateral rhinotomy incision combined with A lateral rhinotomy incision combined with

medial maxillectomy to allow adequate medial maxillectomy to allow adequate margins is the classic approach.may be margins is the classic approach.may be removed endoscopically.removed endoscopically.

Inverting PapillomaInverting Papilloma

Usually arise from unilateral wall of nose and Usually arise from unilateral wall of nose and extend into ethmoid and maxillary sinusesextend into ethmoid and maxillary sinuses

No true invasion of surrounding structuresNo true invasion of surrounding structures Displacement, destruction by pressure necrosisDisplacement, destruction by pressure necrosis Complete excision necessary to prevent Complete excision necessary to prevent

recurrencerecurrence Radiotherapy ineffective, may promote Radiotherapy ineffective, may promote

malignant transformationmalignant transformation

HemangiomasHemangiomas

On rare occasions, a primary hemangioma will occur On rare occasions, a primary hemangioma will occur in the maxilla.in the maxilla.

pyogenic granuloma is mistaken for a true pyogenic granuloma is mistaken for a true hemangiomahemangioma

More frequently, the maxilla will become involved More frequently, the maxilla will become involved with a soft tissue hemangioma of the face or a with a soft tissue hemangioma of the face or a vascular malformation of the skull.vascular malformation of the skull.

Hemangiomas are typified by rarefaction on Hemangiomas are typified by rarefaction on radiographs, radiographs,

extirpation is indicated only for bleeding or major extirpation is indicated only for bleeding or major discomfortdiscomfort

HemangiopericytomasHemangiopericytomas typically occur in the nose but may involve the typically occur in the nose but may involve the

sinuses. sinuses. "If there is one common denominator for it is "If there is one common denominator for it is

the lack of uniformity in appearance, growth the lack of uniformity in appearance, growth and biological behavior." and biological behavior."

Clinical behavior from a slowly enlarging Clinical behavior from a slowly enlarging rubbery mass to an infiltrating aggressive rubbery mass to an infiltrating aggressive neoplasm.neoplasm.

are locally aggressive are locally aggressive likely to recur delayed recurrence, likely to recur delayed recurrence,

HemangiopericytomaHemangiopericytoma Pericytes of ZimmermanPericytes of Zimmerman Present as rubbery, pale/gray, well circumscribed lesions Present as rubbery, pale/gray, well circumscribed lesions

resembling nasal polypsresembling nasal polyps Treatment is surgical resection with postoperative XRT for Treatment is surgical resection with postoperative XRT for

positive marginspositive margins

MeningiomaMeningioma

Very rareVery rare Result of extracranial arachnoid cell clustersResult of extracranial arachnoid cell clusters Occur along cranial nerve tracts and skull Occur along cranial nerve tracts and skull

foraminaforamina Complete removal is curativeComplete removal is curative Radiation may stabilize inoperable casesRadiation may stabilize inoperable casesplainplain skull films may reveal hyperostosis of the skull films may reveal hyperostosis of the

ethmoidal region. ethmoidal region.

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, chondrosarcoma, Osteogenic sarcoma, fibrosarcoma, chondrosarcoma,

rhabdomyosarcomarhabdomyosarcoma LymphomaLymphoma Metastatic tumorsMetastatic tumors Sinonasal undifferentiated carcinomaSinonasal undifferentiated carcinoma

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RISK FACTORSRISK FACTORS

S.C.CS.C.C;aflatoxin found in certain foods and dust;;aflatoxin found in certain foods and dust; chromium,chromium, nickel, nickel, mustard gas,mustard gas, polycyclic hydrocarbons, and other organic polycyclic hydrocarbons, and other organic

chemicals, chemicals, mesothorium (Thorotrast), a radiopaque dye mesothorium (Thorotrast), a radiopaque dye

used as a contrast medium within the antrum.used as a contrast medium within the antrum. adenocarcinomaadenocarcinoma ;Wood dust has a particular ;Wood dust has a particular

association association Adenoid cystic carcinomas and Adenoid cystic carcinomas and

adenocarcinomas are next in approximately adenocarcinomas are next in approximately 10%10%

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 presentation90% have local invasion by presentation Lymphatic drainage:Lymphatic drainage:

– First echelon: retropharyngeal nodesFirst echelon: retropharyngeal nodes– Second echelon: subdigastric nodesSecond echelon: subdigastric nodes

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Maxillary SinusMaxillary Sinus

TT11 Tumor limited to maxillary sinus mucosa with no Tumor limited to maxillary sinus mucosa with no erosion or erosion or destruction of bonedestruction of bone

TT22 Tumor causing bone erosion or destruction including Tumor causing bone erosion or destruction including extension into the hard palate and/or middle nasal meatus, except extension into the hard palate and/or middle nasal meatus, except extension to posterior wall of maxillary sinus and pterygoid platesextension to posterior wall of maxillary sinus and pterygoid plates

TT33 Tumor invades any of the following: bone of the posterior Tumor invades any of the following: bone of the posterior wall of maxillary sinus, subcutaneous tissues, floor or medial wall wall of maxillary sinus, subcutaneous tissues, floor or medial wall of orbit, pterygoid fossa, ethmoid sinuses.of orbit, pterygoid fossa, ethmoid sinuses.

TT4a4a Tumor invades anterior orbital contents, skin of cheek, Tumor invades anterior orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinusesor frontal sinuses

TT4b4b Tumor invades any of the following: orbital apex, dura, Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than maxillary brain, middle cranial fossa, cranial nerves other than maxillary division of trigeminal nerve (Vdivision of trigeminal nerve (V22), nasopharynx, or clivus), nasopharynx, or clivus

Nasal Cavity and Ethmoid SinusNasal Cavity and Ethmoid Sinus

TT11 Tumor restricted to any one subsite, with or without bony Tumor restricted to any one subsite, with or without bony invasioninvasion

TT22 Tumor invading two subsites in a single region or extending Tumor invading two subsites in a single region or extending to involve an adjacent region within the nasoethmoidal complex, to involve an adjacent region within the nasoethmoidal complex, with or without bony invasionwith or without bony invasion

TT33 Tumor extends to invade the medial wall or floor of the orbit, Tumor extends to invade the medial wall or floor of the orbit, maxillary sinus, palate, or cribriform plate maxillary sinus, palate, or cribriform plate

TT4a4a Tumor invades any of the following: anterior orbital Tumor invades any of the following: anterior orbital contents, skin of nose or cheek, minimal extension to anterior contents, skin of nose or cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or frontal sinuses cranial fossa, pterygoid plates, sphenoid or frontal sinuses

TT4b4b Tumor invades any of the following: orbital apex, dura, brain, Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than (Vmiddle cranial fossa, cranial nerves other than (V22), nasopharynx, ), nasopharynx, or clivusor clivus

MRI and CT scanning should be required MRI and CT scanning should be required for staging of these neoplasms.for staging of these neoplasms.

ManagementManagement

Definitive treatment has includedDefinitive treatment has included surgery,surgery, irradiation,irradiation, and electrocoagulation in varying and electrocoagulation in varying

combinations. It does not seem that combinations. It does not seem that substantial improvements have been substantial improvements have been achieved in the past 50 yearsachieved in the past 50 years

Maxillary SinusMaxillary Sinus Early (TEarly (T11 and T and T22) lesions may be treated by ) lesions may be treated by

surgical resection (maxillectomy) or surgical resection (maxillectomy) or irradiationirradiation

Most patients have advanced (TMost patients have advanced (T33 or T or T44) disease ) disease when first seen, which most authors agree when first seen, which most authors agree requires combined therapy for optimal controlrequires combined therapy for optimal control

usually receiving the following types of usually receiving the following types of management: surgery for cure, surgery with management: surgery for cure, surgery with preoperative or postoperative irradiation, and preoperative or postoperative irradiation, and irradiation with surgical salvageirradiation with surgical salvage

Surgery AloneSurgery Alone Tomor isolated to the antral Tomor isolated to the antral mucosa.mucosa.

have taken a more aggressive surgical have taken a more aggressive surgical posture and reported dramatic posture and reported dramatic improvement in survival for patients with improvement in survival for patients with TT33 and T and T44 lesions lesions

Surgical management should include Surgical management should include consideration of immediate rehabilitation.consideration of immediate rehabilitation.

immediate rehabilitationimmediate rehabilitation

prosthetics. reconstruction of maxillary sinus defectsprosthetics. reconstruction of maxillary sinus defects skin graft ;skin graft ; 1-fibula osteocutaneous flap :midface reconstruction 1-fibula osteocutaneous flap :midface reconstruction

for lower maxillary defects. for lower maxillary defects. 2-when orbitozygomatic support is the objective,the 2-when orbitozygomatic support is the objective,the

scapula osteocutaneous flap is preferable. scapula osteocutaneous flap is preferable. reconstruction of the orbital floor, palate, and zygoma.reconstruction of the orbital floor, palate, and zygoma.[21[21

3-3- Iliac crest osteomyocutaneous flaps ;when requair Iliac crest osteomyocutaneous flaps ;when requair additional soft tissue bulk and boneadditional soft tissue bulk and bone

the major risks is "covering recurrent disease." the major risks is "covering recurrent disease." However, disease recurrence at this site, is essentially However, disease recurrence at this site, is essentially untreatable, so this may be a moot point.untreatable, so this may be a moot point.

Combined Irradiation and SurgeryCombined Irradiation and Surgery With TWith T33 tumors, little difference exists between tumors, little difference exists between

preoperative and postoperative irradiation.preoperative and postoperative irradiation. Postoperative irradiation control microscopically Postoperative irradiation control microscopically

positive resection margins.positive resection margins. preoperative irradiation seems to be the choice,more preoperative irradiation seems to be the choice,more

advanced lesions, where resectability is questionable,advanced lesions, where resectability is questionable, the management of advanced maxillary sinus tumors the management of advanced maxillary sinus tumors

is the need for orbital exenteration. indicates a is the need for orbital exenteration. indicates a doubling of survival for the management of ethmoid doubling of survival for the management of ethmoid neoplasms (57% vs 26%), neoplasms (57% vs 26%),

RadiotherapyRadiotherapy Difficulties in the use of standard radiation Difficulties in the use of standard radiation

therapy for paranasal sinus tumor arise from the therapy for paranasal sinus tumor arise from the fact that in air-filled spaces such as the sinuses, fact that in air-filled spaces such as the sinuses, the range of particles and thus the dosimetry is the range of particles and thus the dosimetry is subject to considerable unceratainty.subject to considerable unceratainty.

may cause significant morbidity to the eye and may cause significant morbidity to the eye and optic nerves, optic nerves,

or may result in significant underdosing of or may result in significant underdosing of portions ofthe posterior ethmoid and maxillary portions ofthe posterior ethmoid and maxillary sinusetsinuset

Chemoradiation TherapyChemoradiation Therapy Dramatic response with combined Dramatic response with combined

chemotherapy and radiation seems to be chemotherapy and radiation seems to be possible even in the presence of bulky possible even in the presence of bulky or unresectable disease,or unresectable disease,

Cervical Lymph NodesCervical Lymph Nodes cervical lymph nodes indicate a very cervical lymph nodes indicate a very

poor prognosis.poor prognosis. can be successfully managed with neck can be successfully managed with neck

dissection. dissection.

AdenocarcinomasAdenocarcinomas

have association with woodworkinghave association with woodworking commonly found in the upper nasal cavity and commonly found in the upper nasal cavity and

ethmoid sinus ethmoid sinus lethal by virtue of local progression. lethal by virtue of local progression. Treated with en bloc resection when possible.Treated with en bloc resection when possible. In contrast to adenoid cystic carcinomas, In contrast to adenoid cystic carcinomas,

adenocarcinomas have a low incidence of distant adenocarcinomas have a low incidence of distant metastasis, but the long-range survival is equally metastasis, but the long-range survival is equally poor.poor.

Management for esthesioneuroblastoma and Management for esthesioneuroblastoma and adenocarcinoma is generally similaradenocarcinoma is generally similar

craniofacial resection craniofacial resection postoperative radiation. postoperative radiation. The addition of chemotherapy to the management The addition of chemotherapy to the management

of advanced esthesioneuroblastoma (Tof advanced esthesioneuroblastoma (T33 and T and T44) is ) is now well established,now well established,

noted a decrease in survival with preservation of noted a decrease in survival with preservation of the orbital contents (26%) compared with orbital the orbital contents (26%) compared with orbital resection (57%).resection (57%).

Adenoid Cystic CarcinomaAdenoid Cystic Carcinoma 33rdrd most common site is the nose/paranasal most common site is the nose/paranasal

sinuses,sinuses, Common invalve antrumCommon invalve antrum Perineural spreadPerineural spread

– Anterograde and retrogradeAnterograde and retrograde Distant metastaseDistant metastase Neck metastasis is rare and usually a sign of Neck metastasis is rare and usually a sign of

local failurelocal failure Surgicaly and Postoperative XRT is very Surgicaly and Postoperative XRT is very

importantimportant

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ComplicationsComplications

complications increases when surgery breaches thecomplications increases when surgery breaches the intracranial intracranial space space //when high doses of irradiation are delivered to the orbit when high doses of irradiation are delivered to the orbit or intracranial contents.or intracranial contents.

SURGICAL COMPLICATION; SURGICAL COMPLICATION; meningitis, brain abscess, CSF leak, postoperative wound meningitis, brain abscess, CSF leak, postoperative wound

hemorrhage.hemorrhage.complication of sinus irradiation.complication of sinus irradiation. Cataracts are the most common Cataracts are the most common osteoradionecrosis,osteoradionecrosis, keratitis, keratitis, optic neuritis,optic neuritis, hypopituitarism.hypopituitarism. Injury to the optic nerve .After high-dose irradiation that uses Injury to the optic nerve .After high-dose irradiation that uses

fields that include the whole orbit, one can expect loss of fields that include the whole orbit, one can expect loss of vision in 100% of cases.vision in 100% of cases.

Therapeutic Decision MakingTherapeutic Decision Making

      1. 1.    Most tumors are at an advanced stage when first    Most tumors are at an advanced stage when first recognized and require aggressive multimodality recognized and require aggressive multimodality treatment.treatment.   2.    Survival and local disease control are better when the    2.    Survival and local disease control are better when the orbit is included in the therapeutic plan (i.e., exenteration orbit is included in the therapeutic plan (i.e., exenteration or irradiation).or irradiation).   3.    A significant chance of metastasis to the primary    3.    A significant chance of metastasis to the primary (pharyngeal) and secondary (cervical) nodes exists. They (pharyngeal) and secondary (cervical) nodes exists. They should be included in the primary treatment plan.should be included in the primary treatment plan.   4.    Higher success rates have been reported with    4.    Higher success rates have been reported with primary irradiation,primary irradiation,   5.    The best regimen may prove to be a combination of    5.    The best regimen may prove to be a combination of chemotherapy, irradiation, and limited surgical chemotherapy, irradiation, and limited surgical débridement.débridement.   6.    The concept of intraarterial chemotherapy for    6.    The concept of intraarterial chemotherapy for advanced skull base tumors was advocated advanced skull base tumors was advocated

SarcomasSarcomas

Osteogenic sarcomas and chondrosarcomas Osteogenic sarcomas and chondrosarcomas more commonly in the mandible than in the more commonly in the mandible than in the maxilla.maxilla.

They are usually lethal by local progression.They are usually lethal by local progression. successful therapy successful therapy is wide en bloc resection, is wide en bloc resection, Irradiation seems to be an effectiveIrradiation seems to be an effective

Osteogenic SarcomaOsteogenic Sarcoma

Hematopoietic and Lymphoid TumorsHematopoietic and Lymphoid Tumors

The paranasal sinuses are an uncommon primary site The paranasal sinuses are an uncommon primary site for lymphomatous tumorsfor lymphomatous tumors

most important role of the otolaryngologist is to most important role of the otolaryngologist is to provide adequate tissue in good condition for provide adequate tissue in good condition for histologic processinghistologic processing

Treatment is usually chemotherapy, although Treatment is usually chemotherapy, although irradiation alone is adequate for stage I and stage II irradiation alone is adequate for stage I and stage II Hodgkin's Hodgkin's

Malignant MelanomasMalignant Melanomas

Symptoms;Symptoms; black nasal dischargeblack nasal discharge and have a pigmented mass within the nose.and have a pigmented mass within the nose. Frequently regional or distant metastases exist at the Frequently regional or distant metastases exist at the

time of initial examination.time of initial examination. Although radical surgical removal is the standard of Although radical surgical removal is the standard of

carecare Irradiation has been considered inadequateIrradiation has been considered inadequate but but

adjustments in fractionation may provide better adjustments in fractionation may provide better results,results,

MelanomaMelanoma 1% originate in sinonasal cavity1% originate in sinonasal cavity 5th-8th decades5th-8th decades anterior septumanterior septum maxillary antrummaxillary antrum polypoid mass, polypoid mass, pigmentation?pigmentation? 5 yr = 38%5 yr = 38% 10 yr = 17%10 yr = 17%

PARANASAL SINUS NEOPLASMS IN PARANASAL SINUS NEOPLASMS IN CHILDRENCHILDREN

usually benign. usually benign. The common nasal sinus tumors are polyps arising on The common nasal sinus tumors are polyps arising on

an allergic basis or in association with cystic fibrosis. an allergic basis or in association with cystic fibrosis. – The most common true neoplasms are The most common true neoplasms are

fibroosseous lesions and tumors of dental originfibroosseous lesions and tumors of dental origin– malignant tumors are embryonal malignant tumors are embryonal

rhabdomyosarcomas, rhabdomyosarcomas, – usually managed surgically because: usually managed surgically because: – (1) most lesions are radioinsensitive;(1) most lesions are radioinsensitive;– (2) irradiation in excess of 3000 rad will have a (2) irradiation in excess of 3000 rad will have a

substantial effect on growth of the facial skeleton; substantial effect on growth of the facial skeleton; – (3) there is a significant risk of radiation-induced (3) there is a significant risk of radiation-induced

malignant transformation. malignant transformation.

Monostotic fibrous dysplasia Monostotic fibrous dysplasia developmental defect of bone developmental defect of bone in the pediatric age group (age 15 or younger) in the pediatric age group (age 15 or younger) common in the maxilla common in the maxilla sometimes requires wide local removal, including sometimes requires wide local removal, including

partial maxillectomy, partial maxillectomy,

Ossifying fibromas and cementomas,Ossifying fibromas and cementomas, in in contrast, are well delineated and may be managed by contrast, are well delineated and may be managed by local excision with a minimal margin.local excision with a minimal margin.

Rhabdomyosarcomas are the most common Rhabdomyosarcomas are the most common malignant neoplasm of the upper respiratory malignant neoplasm of the upper respiratory tract in the pediatric age tract in the pediatric age

surgical margins supplemented by irradiation surgical margins supplemented by irradiation and chemotherapy.and chemotherapy.

parameningeal involvement have a poor parameningeal involvement have a poor prognosisprognosis

This group of patients has benefited from a This group of patients has benefited from a combining intrathecal chemotherapy and combining intrathecal chemotherapy and craniospinal irradiation.craniospinal irradiation.

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