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Neoplasms of the Nose and Paranasal Sinuses

Kevin Katzenmeyer, MD
Anna Pou, MD
June 7, 2000
Sinonasal neoplasms are rare, comprising less than 3 o! all malignant aero"igestive tumors an"
less than # o! all malignancies$ Despite their in!re%uence, they represent &oth a "iagnostic an"
therapeutic challenge &ecause the presenting signs an" symptoms may &e in"istinguisha&le !rom &enign or
in!lammatory "isor"ers$ 'hese malignancies typically a!!ect (aucasion males in the !i!th to seventh
"eca"es o! li!e an" have a 2)# male prepon"erance$ Although the presentation may &e nonspeci!ic, certain
physical !in"ings are o!ten suggestive o! this "isease spectrum, such as cranial neuropathies, !acial
s*elling, an" epista+is$
'umors o! the nasal cavity proper are appro+imately evenly "ivi"e" &et*een &enign an"
malignant neoplasia, *ith inverting papilloma pre"ominating in the &enign group an" s%uamous cell
carcinoma in the malignant$ ,n the other han", most sinus tumors are malignant *ith s%uamous cell
carcinoma &eing the most prevalent$ 'he ma+illary sinus is most commonly involve" *ith tumor, !ollo*e"
&y the nasal cavity, the ethmoi"s, an" then the !rontal an" sphenoi" sinuses$
Epidemiology
-ecause cancer o! the paranasal sinuses is uncommon, it is more "i!!icult than usual to i"enti!y
promoting an" initiating !actors$ Despite this, up to .. are attri&ute" to occupational e+posures,
inclu"ing nic/el, chromium, isopropyl oils, volatile hy"rocar&ons, an" organic !i&ers that are !oun" in the
*oo", shoe, an" te+tile in"ustries$ 0n a""ition, human papillomavirus can &e a co!actor, an" in one series,
human papillomavirus 1 or #2 *as "ocumente" in 2. o! inverting papillomas an" . o! s%uamous cell
carcinomas$ Speci!ic asssociations !oun" inclu"e s%uamous cell carcinoma in nic/el *or/ers an"
a"enocarcinoma in *or/ers e+pose" to har"*oo" "usts an" leather tanning$ (igarette smo/ing an" heavy
alcohol consumption have long &een /no*n to increase the ris/ o! hea" an" nec/ malignancy$ 'heir
association *ith sinonasal neoplasms has &een har"er to esta&lish$ 0t has long &een thought that there *as
no association *ith sinonasal cancer &ut 2heng et al have propose" an increase" ris/ o! nasal cancer,
particularly in the ma+illary sinus$
Presentation
'he "iagnosis o! sinonasal malignancies is challenging$ 3ot only are they rare, &ut they are
"i!!icult to "istinguish !rom their &enign counterparts$ -enign sinonasal "isor"ers account !or a signi!icant
proportion o! visits to the otolaryngologist$ 'he similarites o! &enign an" malignant "isor"ers at initial
presentation lea"s to a signi!icant "elay in the "iagnosis o! malignancy$ 0t is estimate" that a span o! 1 to 4
months passes on average !rom the time o! initial symptoms until "iagnosis is esta&lishe"$ Key in"icators
o! malignancy such as cranial neuropathies an" proptosis are uncommon at initial presentation an" signi!y
a"vance" "isease$ A high in"e+ o! suspicion must &e maintaine" !or patients *ho "o not respon" to
me"ical treatment o! their sinonasal symptoms$
Signs an" symptoms o! ma+illary sinus carcinoma !all into several ma5or categories) oral, nasal,
ocular, !acial, an" au"itory$ ,ral presentations occur in 26736 an" inclu"e pain involving the ma+illary
"entition, trismus, palatal an" alveolar ri"ge !ullness, an" !ran/ erosion into the oral cavity$ 3asal !in"ings
are seen in up to 60 o! patients an" inclu"e o&struction, "ischarge, stu!!iness, congestion, epista+is, an"
e+tension into the nasal cavity$ ,cular !in"ings occur in appro+imately 26 an" arise !rom up*ar"
e+tension into the or&it, *here unilateral tearing, "iplopia, !ullness o! li"s, pain, an" e+ophthalmos are seen$
8acial signs inclu"e in!raor&ital nerve hypesthesia, chee/ s*elling, pain, an" !acial asymmetry$ Au"itory
complaints inclu"e hearing loss secon"ary to serous otitis me"ia "ue to nasopharyngeal e+tension$
9ith a"vance" "isease, the classic tria" o! !in"ings !or carcinoma o! the nasal cavity an"
paranasal sinuses may &e present$ 'hese inclu"e :#; !acial asymmetry, :2; a visi&le or palpa&le tumor
&ulge in the oral cavity, an" :3; tumor visi&le in the nose *ith anterior rhinoscopy$ All three *ill &e
present in .0 to 10 o! patients, &ut at least one *ill &e present in <0 at the time o! "iagnosis$
Diagnosis
'he physical e+amination shoul" &e thorough$ 'he sinonasal, ocular, an" neurologic systems
shoul" &e stu"ie" in "etail$ 0n particular, evi"ence o! in!raor&ital nerve hypesthesia, "iplopia, proptosis,
an" loose "entition shoul" &e care!ully evaluate"$ 3asal en"oscopy shoul" &e per!orme" a!ter a"e%uate
topical anesthesia so that e+am is not limite" &y patient "iscom!ort$
Any suspicious lesions note" on nasal en"oscopy shoul" &e &iopsie" &eing cognizant o! their
&lee"ing potential$ 0t is recommen"e" that &iopsy !ollo* ra"iographic e+am so that tissues are not
"istorte"$ Ma+illary sinus neoplasms are &iopsie" via antrostomies or nasoantral *in"o*s so that any
!uture partial ma+illectomy *ill remove the &iopsy site *ith the specimen$ A""itionally, any ma+illary
sinus *ith retaine" secretions *ill nee" "rainage via these routes i! ra"iation therapy is entertaine"$ -iopsy
via a (al"*ell7=uc approach is not recommen"e" &ecause o! the potential to see" the gingivo&uccal sulcus
an" chee/ s/in *ith tumor$
>a"iographic stu"ies are essential as the !ull e+tent o! a sinonasal neoplasm cannot &e esta&lishe"
even *ith mo"ern !i&eroptic technology$ Plain ra"iographs are rarely use" currently an" have &een
replace" &y compute" tomography :('; an"?or magnetic resonance imaging :M>0; as the initial stu"ies
per!orme"$ 'hese complementary stu"ies assist in the evaluation o! patients *ho have a malignancy o! the
nasal cavity or paranasal sinuses an" are the most e!!ective *ay to "elineate the e+tent o! tumor
e+tracranially an" intracranially$
As a general rule, malignant tumors "estroy &one, *hereas &enign processes cause thic/ening or
remo"eling o! a"5acent &one$ -one changes are "epicte" &etter *ith compute" tomography than *ith
magnetic resonance imaging$ All malignant tumors "o not "estroy &one, ho*ever$ Most sinonasal
sarcomas remo"el a&utting &one some*hat, as "o most minor salivary glan" carcinomas, e+trame"ullary
plasmacytomas, large cell lymphomas, ol!actory neuro&lastomas, an" hemangiopericytomas$
'he true value o! (' scanning is its a&ility to "etect &one erosion$ Key areas inclu"e the &ony
or&ital *alls, cri&i!orm plate, !ovea ethmoi"alis, posterior *all o! the ma+illary sinus, pterygopalatine
!ossa, the sphenoi" sinus, an" the posterior ta&le o! the !rontal sinus$ 'he accuracy o! (' in "etermining
tumor sprea" to these areas is on the or"er o! 46$ Despite the signi!icant amount o! in!ormation that can
&e gaine" !rom (', it has certain limitations) it cannot al*ays "etermine *hether tumor has inva"e" or 5ust
approache" the perior&ita an" it is "i!!icult to "i!!erentiate tumor !rom so!t tissue s*elling an" secretions
&ecause o! their similar "ensities$ (ontrast a"ministration o!!ers only marginal assistance *ith these t*o
"ilemnas$
M> imaging provi"es e+cellent "elineation o! tumor !rom surroun"ing in!lammatory tissue an"
secretions *ithin the sinuses$ 'his, along *ith its multiplanar a&ility an" lac/ o! ra"iation e+posure gives
a"vantage to M>0 in evaluation o! these lesions$ 'ypically, e"ema o! in!lame" tissue an" retaine"
secretions *oul" &e o! lo* intensity on '# an" high intensity on '2 secon"ary to increase" *ater content$
@o*ever, &ecause o! the o!ten chronic nature o! these secretions at the time o! "iagnosis a certain amount
o! !ree *ater *ill have &een a&sor&e" an" a varia&le pattern o! intensity may &e seen$ ,n the other han",
<6 o! sinonasal tumors are highly cellular *ith less *ater content giving lo* to interme"iate signal
intensity on &oth '# an" '2 imaging$ 0ntravenous in5ection o! ga"olinium provi"es a""itional in!ormation$
Most sinonasal tumors enhance in a "i!!use !ashion to an interme"iate "egree, *hereas in!lame" mucosa
enhances more intensely an" in a peripheral !ashion$ Perineural sprea" to tumor can also &e sho*n on M>0
*hich is most important i! a"enoi" cystic carcinoma is &eing stu"ie"$ (orrelation o! M> imaging an"
histologic !in"ings at surgery has &een sho*n to &e as high as <. *ith improvement to <4 *ith
ga"olinium$
Benign Lesions

Papillomas 7 A num&er o! "i!!erent papillomas arise *ithin the nose an" sinuses$ 0n the vesti&ule, a
s%uamous papilloma similar to that !oun" else*here on the s/in is !oun"$ 0n the sinonasal tract, three
"i!!erent types o! schnei"erian papillomas are recognize") inverte", !ungi!orm, an" cylin"ric$ 'he
!ungi!orm or everte" :60 o! cases; occurs e+clusively on the septum an" can cause o&struction an"
&lee"ing$ 'he cylin"ric papilloma :3 o! cases; can &e !oun" on the lateral *all an" in the sinuses$ 0t is
also compose" o! everte" !ron"s &ut also has cystic mucus7containing spaces$ 'hese can recur &ut their
malignant potential is %uestione"$ =astly, an" most importantly is the inverting papilloma :.7 o! cases;$
0ts etiology, malignant potential, an" optimal treatment are *i"ely "e&ate"$ 0ts site o! origin is usually the
lateral nasal *all in the region o! the mi""le meatus$ 0t has a ten"ency to recur, can &e locally "estructive,
an" is associate" *ith malignancy$ 0t is imperative that all specimens remove" "uring sinus surgery &e
evaluate" &y pathology so that these lesions "o not go un"iagnose"$ 'hese are almost al*ays unilateral, are
more common in men, an" are seen more commonly in the si+th an" seventh "eca"es$ 'hey are reporte" to
comprise 0$6 to . o! all sinonasal tumors$ Association *ith carcinoma is seen in 2 to #3 o! cases$
>ecurrence rate varies *i"ely !rom 0 to 40 "epen"ing on the initial metho" o! surgical control$ Me"ial
ma+illectomy via lateral rhinotomy is the gol"7stan"ar" to *hich all !orms o! therapy are measure"$
>ecently, many authors have a"vocate" en"oscopic e+cision !or control o! lesions that are accessi&le an"
easily !ollo*e" postoperatively$
Osteomas 7 'hese are &enign slo*7gro*ing tumors o! mature &one$ 0n or"er o! !re%uency, they occur in
the !rontal, ethmoi", an" ma+illary sinuses$ 'hey are e+tremely rare in the sphenoi" sinus$ 'hey are very
o!ten "iscovere" inci"entally &ut can &loc/ sinus "rainage lea"ing to mucocele !ormation or inva"e the
or&it lea"ing to proptosis$ 'hey are most o!ten seen in the #6 to .0 age group$ 'reatment i! necessary is &y
local e+cision *ith a margin o! normal &one$
Fibrous dysplasia 7 8i&rous "ysplasia is characterize" &y the replacement o! normal &one &y tissue
containing collagen, !i&ro&lasts, an" osteoi" material$ 0t most commonly occurs &e!ore the age o! 20$
S*elling o! one &one is the most common !eature, an" the ma+illa is more a!!ecte" than the man"i&le$ 0n
the ma+illa, it usually a!!ects the canine !ossa area or the zygomatic area$ 'he ra"iographic picture is !airly
typical an" "escri&e" as a groun"7glass appearance$ 0t can vary !rom a "i!!use uni!orm sclerosis that
!ollo*s the contour o! the &one to a ma+illary lesion that sho*s o&literation o! the ma+illary sinus *ith
involvement o! the in!raor&ital margin an" malar &ones$ 'he &est treatment is to "o as little as possi&le$
'hey are very vascular i! operate" on in the vascular phase$ 'he use o! ra"iation is not recommen"e" as
malignant trans!ormation has &een seen$
Neurogenic tumors 7 'hese tumors are commonly seen *ithin the hea" an" nec/ an" up to . o! these are
seen in the paranasal sinuses$ Sch*annomas have &een "escri&e" *ithin !acial &ones an" along the
&ranches o! the trigeminal nerve an" nerves o! the autonomic nervous system$ 'hey arise !rom the sur!ace
o! the nerve !i&ers an" sel"om un"ergo malignant "egeneration$ 3euro!i&romas arise !rom *ithin nerve
!i&ers, an" usually occur as part o! von >ec/linghausenAs "isease$ 'hey have &een "escri&e" *ithin !acial
&ones an" un"ergo malignant change in #6 o! cases$ 'hese tumors shoul" &e treate" &y complete
surgical e+cision unless vital surroun"ing structures are involve" in *hich partial e+cision is accepta&le$
Malignant Lesions
Squamous Cell Carcinoma 7 S%uamous cell carcinoma is the most common tumor a!!ecting the ma+illary
an" ethmoi" sinuses$ 0t has &een sai" to account !or up to 40 o! the tumors in this area$ 'he ma+illary
sinus is involve" 70 o! the time !ollo*e" &y the nasal cavity in 20 o! the cases *ith the ethmoi"s
comprising the remain"er$ Primary lesions !rom the !rontal an" sphenoi" sinuses are uncommon$ 0t is
primarily a "isease o! males an" presents most commonly in the si+th "eca"e$ Sprea" outsi"e the sinuses is
almost the rule at presentation$ More than <0 *ill have inva"e" through at least one *all o! the involve"
sinus *hen "iscovere"$ 0! metastasis "oes occur, the ma+illary tumors sprea" to the su&man"i&ular no"es
an" the ethmoi"al tumors o!ten are seen in the 5ugulo"igastric an" su&"igastric no"es$ 'he e+tent o!
"isease is more important than the "egree o! "i!!erentiation o! the tumor$
Adenoid Cystic Carcinoma 7 A"enoi" cystic carcinoma can arise *ithin the ma5or an" minor salivary
glan"s or mucous glan"s o! the oral cavity an" upper respiratory tract$ 'he most common site o!
occurrence is the palate, !ollo*e" &y the ma5or salivary glan"s an" the paranasal sinuses$ Despite agressive
surgical resection an" ra"iotherapy, most a"enoi" cystic carcinomas gro* insi"iously over several years,
resulting in multiple local recurrences an" "istant metastases$ 'he paranasal sinuses comprise #.7#7 o!
all cases$ Perineural sprea" along cranial nerves is &elieve" to &e responsi&le !or the high rates o! local
recurrence even *ith negative surgical margins$ -ecause o! this, it is imperative that all patients, regar"less
o! the status o! their margins, receive regular long7term !ollo*7up$ Distant metastases most commonly
occur in the lungs$ 3ec/ no"al metastases are e+tremely rare *ith this type o! tumor$
Mucoepidermoid Carcinoma 7 'hese are e+tremely rare in the sinuses an" ten" to present in later stages$
'he propensity !or *i"esprea" local invasion ma/es resection *ith negative margins "i!!icult so
com&ination treatment *ith ra"iation is o!ten recommen"e"$
Adenocarcinoma 7 'his is the secon" most common malignant tumor in the ma+illary an" ethmoi" sinuses
&eing seen in up to 6 to 20 o! cases$ 'hese ten" to &e more superiorly locate" *ith the ethmoi" sinuses
most commonly involve"$ Most are relate" to occupational e+posures as it is &elieve" that "ust particles
*ill travel along the mi""le tur&inate an" the larger particles *ill &e "eposite" there$ 'hey present
similarly to the s%uamous cell carcinoma an" are "ivi"e" histologically into high an" lo* gra"es$
Hemangiopericytoma 7 @emangiopericytoma is a *ell7recognize" &ut uncommon vascular tumor that
arises !rom the pericytes o! 2immerman$ 'hese are uni%ue cells !oun" spiraling aroun" the outsi"e o!
&loo" capillaries an" postcapillary venules$ =ess than one7thir" occur in the hea" an" nec/, *ith the
minority involving the sinonasal tract$ More than 40 o! these are sai" to involve the ethmoi"s$ Sinonasal
hemangiopericytomas are consi"ere" neither malignant nor &enign, &ut Binterme"iateB in &ehavior$
Metastases are rare$ (linically, intranasal hemangiopericytomas mani!est as pale, gray7*hite, *ell7
circumscri&e" masses *ith a so!t, ru&&ery consistency, resem&ling nasal polyps$ 3asal o&struction *ith
epista+is is common$ 'he mean age o! onset is 66, an" the gen"er "istri&ution is roughly e%ual$ 'reatment
is complete surgical resection !ollo*e" &y ra"iation i! the margin is positive$
Melanoma 7 -et*een 0$6 an" #$6 o! all melanomas are sai" to originate !rom the nasal cavity an"
paranasal sinuses, *here they constitute a&out 3$6 o! all sinonasal neoplasia$ 'he highest inci"ence is in
patients in their !i!th to eighth "eca"es$ 'he nasal cavity is most !re%uently involve" *ith the anterior
septum most commonly seen$ 'he ma+illary antrum is the most !re%uently seen sinus location$ 'ypically
it is seen as a polypoi" !leshy mass an" its pigmentation varies$ 0nitial metastasis to regional lymph no"es
is not prevalent *hich is mislea"ing as 6 an" #0 year survival is on the or"er o! 34 an" #7$ =ong7term
survival statistics are "i!!icult to esta&lish as patients succum& to their "isease even a!ter relatively long
"isease7!ree survivals o! up to 20 years$ Survival !rom mucosal melanoma is very poor especially !rom the
amelanotic variety *ith some stu"ies reporting 6 year survival as lo* as 1$
Olfactory neuroblastoma or esthesioneuroblastoma 7 'hese are rare lesions arising !rom in the upper part
o! the nasal cavity !rom stem cells o! neural crest origin that "i!!erentiate into ol!actory sensory cells$ 'he
tumor "i!!ers clinically !rom sympathetic neuro&lastoma in that all ages are a!!ecte" an" urinary CMA an"
@CA are not "etecta&le$ 0t occurs in t*o age pea/s, namely aroun" 20 an" 60$ 0t is a slo*7gro*ing tumor
that !orms an e+ophytic polypoi" or sessile mass *ith a congeste" appearance an" usually a smooth
sur!ace$ =arger tumors may &e ulcerate"$ 0nitially it is unilateral &ut e+tension to the opposite si"e o! the
nasal cavity an" paranasal sinuses occurs *ith gro*th$ Ka"ish propose" a clinical staging system *ith
group A tumors &eing con!ine" to the nasal cavity, group - involving the paranasal sinuses, an" group (
e+ten"ing &eyon" these limits$ >osettes o! the neuro&lastoma cells are the hallmar/ o! "iagnosis &ut the
histology varies *i"ely an" these tumors are sometimes classi!ie" mista/enly as un"i!!erentiate"
carcinoma$ 'he tumor mani!ests an aggressive &ehavior$ =ocal recurrences can &e e+pecte" in a&out 60 to
76 o! patients an" metastases in 20 to 30$ Most o!ten metastases are to regional lymph no"es, lungs,
an" &ones$
Osteogenic sarcoma 7 ,steosarcoma is the most common primary malignant tumor o! &one &ut is rare in
the sinuses$ ,nly a&out 6 occur in the hea" an" nec/ *here the man"i&le is more commonly a!!ecte"
than the ma+illa$ >a"iographically the sunray appearance is classic &ut is only seen in a&out 26 o! cases$
'here is a 307.0 chance o! "istant metastases an" the !ive7year survival is #6720$
Fibrosarcoma 7 'hese are e+tremely rare in the hea" an" nec/ an" even moreso in the paranasal sinuses$
'he most important "eterminant o! prognosis is the "egree o! "i!!erentiation$
Chondrosarcoma 7 'hese are seen in the thir" to !i!th "eca"es *ith an e%ual male)!emale inci"ence$
@istologic "i!!erentiation &et*een &enign an" malignant types can &e "i!!icult &ut the malignant variety
pre"ominates$ Distant metastases are rare$ Death is &y slo* erosive "estruction o! the s/ull &ase$
'reatment is o!ten ina"e%uate &ecause o! "i!!iculty in esta&lishing margins$
Rhabdomyosarcoma 7 'his is the most common paranasal sinus malignancy seen in chil"ren &ut can a!!ect
ol"er in"ivi"uals as *ell$ 'hey arise in the hea" an" nec/ in 36 to .6 o! cases$ 4 o! these are !oun" in
the sinonasal tract$ 'hree types are "escri&e") em&ryonal an" alveolar, *hich are most common in
chil"ren, an" pleomorphic, more commonly seen in a"ults$ 'riple therapy *ith surgery, chemotherapy, an"
irra"iation is o!ten necessary$
ymphoma 7 Sinonasal lymphoma has a &imo"al presentation, a!!ecting the very young &ut more !re%uently
seen in men &et*een the !i!th an" seventh "eca"es$ 'hey are usually o! the non7@o"g/ins type an" seventy
percent o! the lesions are stage . at presentation$ 'reatment is &y ra"iation, *ith or *ithout chemotherapy$
(hil"ren "o remar/a&ly &etter than a"ults *ho su!!er relapse !re%uently$ 0! there is recurrence, ultimate
survival "rops to aroun" #0$
!"tramedullary Plasmacytoma 7 'he large ma5ority o! these occur in the hea" an" nec/ *ith .0
"eveloping in the nasal cavity or paranasal sinuses$ 'ypically the lesion is B&enignB an" "oes not progress
to multiple myeloma$ (ervical lymph no"es can &e involve" in #0 to 26 o! cases$ Success!ul treatment
inclu"es e+cision or irra"iation$
Metastatic tumors 7 Metastases can occur !rom any*here *ithin the &o"y &ut usually occur !rom the
/i"ney$ >enal cell carcinoma lea"s &y a sizea&le margin as the most common in!raclavicular site o! sprea"$
3eoplasms o! the lungs, &reasts, an" the rest o! the urogenital an" gastrointestinal tract !ollo*$ 0t is
important to recognize these as metastatic lesions as ra"ical surgery is not appropriate an" only palliation
can &e o!!ere"$
Staging
,hngren in #<33 esta&lishe" an imaginary line e+ten"ing !rom the me"ial canthus to the angle o!
the man"i&le$ Dsing this the ma+illary sinus is "ivi"e" into a BsuprastructureB an" an Bin!rastructure$B @e
correctly reasone" that tumor a&ove this line is har"er to resect an" there!ore carries a *orse prognosis$
,ne caveat *ith this is that although in!erior e+tension through the palate is more easily resecte" than other
structures, some consi"er this to &e more correctly consi"ere" an oral cavity tumor *hich carries a higher
rate o! cervical no"e involvement$
(urrently, ma+illary sinus carcinoma is the only one that has &een given a '3M assignment &y the
American Joint (ommittee on (ancer$ 0t consists o! the !ollo*ing) '# 7 tumor limite" to the antral mucosa
*ith no erosion or "estruction o! &oneE '2 7 tumor *ith erosion or "estruction o! the in!rastructure,
inclu"ing the har" palate an"?or the mi""le nasal meatusE '3 7 tumor inva"es any o! the !ollo*ing) s/in o!
chee/, posterior *all o! ma+illary sinus, !loor or me"ial *all o! the or&it, anterior ethmoi" sinusE an" '. 7
tumor inva"es or&ital contents an"?or any o! the !ollo*ing) cri&i!orm plate, posterior ethmoi" or sphenoi"
sinuses, nasopharyn+, so!t palate, pterygoma+illary or temporal !ossae, or &ase o! s/ull$ 3o"al :3; an"
metastatic :M; "esignations are similar to other hea" an" nec/ malignancies$
Sarcomas are evaluate" &ase" on a histologic gra"ing system "etermine" &y the num&er o!
mitoses, "egree o! cellularity, amount o! stroma, "egree o! maturation, nuclear pleomorphism, an" presence
or a&sence o! necrosis$
Treatment
As state" previously, most sinonasal tumors present at a"vance" stages "ue to the relative lac/ o!
speci!ic symptoms i"enti!ying that malignancy is present$ Seventy7!ive percent o! tumors *ill &e o! '3 or
'. status at "iagnosis$ 'his lea"s to "i!!icult "ecisions regar"ing treatment$ >esection o! many o! these
lesions *ill lea" to permanent "is!igurement an" still may not provi"e much hope o! "isease control$ =ocal
control is the most "i!!icult to achieve in these tumors as they a&ut a num&er o! vital structures$ As many
as 10 o! patients recur locally$ 'he choice o! resection "epen"s on the patientAs *ishes an" the location
o! the tumor$ Dse o! preoperative or postoperative ra"iation is o!ten use" !or positive margins an"?or
attempts to limit the size o! resection$ (hemotherapy is usually reserve" !or palliation o! unresecta&le
lesions, metastatic lesions, or *ith recurrences$
Surgery
'he mainstay o! therapy !or sinonasal malignancy is en &loc surgical resection$ 'he speci!ic
approach is "etermine" &y location o! "isease an" histology$ Sisson "etaile" !our speci!ic criteria !or
unresecta&ility *hich inclu"e e+tension o! tumor to the !rontal lo&es :superior e+tension;, invasion o!
preverte&ral !ascia :posterior e+tension;, &ilateral optic nerve involvement, an" cavernous sinus e+tension
:lateral e+tension;$
3asal septal lesions can &e treate" &y *i"e7local e+cision o! the lesion$ Me"ial ma+illectomy *as
"escri&e" &y Sessions an" =arsen in #<77 an" is most amena&le to inverting papilloma or limite" lesions
involving the lateral nasal *all$ Ma+illectomy has &een the stan"ar" approach to*ar" sinus neoplasia *ith
&one cuts through the palate, lateral ma+illa, an" ethmoi"s &elo* the s/ull&ase$ 'umors *ith e+tensive
sprea" or tumors o! the ethmoi"s an" !rontal sinuses *ith involvement o! the s/ull&ase re%uire cranio!acial
resections *ith neurosurgical assistance$ 'here are numerous approaches an" proce"ures "escri&e" !or
these more complicate" resections *hich is &eyon" the scope o! this "iscussion$
,r&ital e+tension o! tumor spar/s "e&ate on the necessity o! e+enteration$ All !our o! the ma5or
paranasal sinuses lie a"5acent to the or&it an" !orm its &ony *alls$ 'hese &ony *alls are per!orate" &y
various !oramina that transmit nerves an" &loo" vessels, all o! *hich are potential routes o! invasion into
the eye$ 'he thin &one o! the lacrimal !ossa is o!ten the point o! entry !or tumor !rom the ethmoi"s into the
or&it$ 8ortunately, the eye is line" &y an inner periosteum or perior&ita *hich is highly resistant to tumor
invasion$ 0n the past, ra"ical ma+illectomy *ith or&ital e+enteration *as the proce"ure o! choice !or
tumors that ero"e into the or&it$ @arrison, in #<71, propose" or&ital e+enteration *henever there *as
preoperative proptosis, limitation o! e+traocular movement, or &ony erosion o! the or&it$ Fven in patients
*ho e+hi&ite" small areas o! &one erosion un"er*ent e+enteration &ecause o! the possi&ility o! resi"ual
tumor on the periosteum$ Sisson *as one o! the !irst to use preoperative irra"iation o! the or&it to prevent
e+enteration$ @e *as not a&le to alter survival &ut "i" "ecrease his num&er o! e+enterations$ Mc(ary an"
=evine have cite" e+perience *ith or&ital preservation an" recommen" preoperative irra"iation o! the or&it
to 60 Gy$ During resection they imme"iately evaluate the perior&ita *ith !rozen section stu"ies an" resect
perior&ita i! positive$ 'he perior&ital "e!ects are repaire" primarily, *ith temporalis !ascia gra!ts, or *ith
s/in gra!ts$ 'hey have ha" to e+enterate virtually no or&its an" report a"e%uate postoperative !unction$
Pterygopalatine !ossa invasion varies !rom #0 to 20 an" is a ris/ !actor !or local recurrence as
resection &ecomes very "i!!icult$ Som has gone so !ar as to suggest that pterygopalatine !ossa invasion
preclu"es the possi&ility o! local control$ Despite this, there are reports o! a"e%uate local control *ith
cranio!acial techni%ues inclu"ing resection o! the mi""le cranial !ossa$ >a"ical surgical resection an"
postoperative irra"iation are recommen"e" !or involvement o! this area$
3ec/ "issection is not routinely per!orme" an" shoul" &e reserve" !or patients *ith palpa&le
lympha"enopathy or ra"iographic evi"ence !or nec/ metastasis$ =ymphatic "rainage !rom the paranasal
sinuses is to the retropharyngeal, su&man"i&ular, an" 5ugulo"igastric no"es$ (ervical metastasis
appro+imates #0 at presentation thus not 5usti!ying elective "issection$ 'he rate o! !uture cervical
metastasis is much greater an" has &een !oun" to &e as high as .0 in some stu"ies &ut this is sel"om a
cause o! !ailure as these patients invaria&ly have recurre" locally as *ell$
>a"iation
Surgery as a single mo"ality is generally reserve" !or patients *ith histologically small tumors in
*hom clear margins are easily attaine" an" *hose tumors are locate" in areas rea"ily visualize" &y nasal
en"oscopy$ 0n clinical practice, this comprises !e* patients$ 'he use o! a"5uvant H>' is recommen"e" i!
surgical margins are positive or !or a"vance" tumors$ A"5uvant H>' is thought to augment surgical 67year
cure rates &y #0 to #6$ 'he use o! H>' alone as primary treatment mo"ality is no longer consi"ere" a
via&le option e+cept !or palliation$ 8ive year survival !or H>' alone is sai" to &e 23 an" !or surgery an"
H>' ..$ 'he use o! palliative high7"ose irra"iation alone !or unresecta&le "isease o!!ers 67year survival
rates o! #0 to #6$
'here is no convincing evi"ence as to the superiority o! preoperative versus postoperative
irra"iation$ Preoperative "oses are commonly in the 60 Gy range an" postoperative "oses in the 10 to 70
Gy range$ Most select postoperative irra"iation &ecause tumor margins are easier to "iscern an" *oun"
complications less$ Preoperative irra"iation is pre!erre" &y some *hen ra"iographic evaluation in"icates
tumor in pro+imity to the perior&ita or "ura mater$ 8rozen section sampling "uring the surgical proce"ure
can then "etermine *hether these structures have &een sterilize" an" or&ital e+enteration or craniotomy can
&e avoi"e"$
0rra"iation ports must &e care!ully "esigne" an" "amage to the central nervous system an" glo&e
must &e &alance" against the nee" to treat the neoplasm$ 0n those treate" &y irra"iation alone !or sinus
neoplasms, use!ul ipsilateral vision is lost in #2 to 20 o! patients an" &ilateral vision in 0 to 4$ Due to
"iplopia or other !actors, postoperative vision is not use!ul in #0 to 20 o! glo&es preserve" at surgery, an"
this percentage is "ou&le" &y irra"iation$

(hemotherapy
(hemotherapy is usually reserve" !or palliative treatment o! a"vance" or recurrent paranasal
malignant neoplasms$ 'he same protocols use" in other hea" an" nec/ malignancies are use" in sinonasal
tumors *ith platin7&ase" regimens !or s%uamous cell carcinoma, an" *ith "o+oru&icin or !luorouracil !or
glan"ular malignant neoplasms$ 'he use o! intra7arterial chemotherapy has ha" some promising results
!rom >o&&ins et al :41 response rate o! '. lesions; an" =ee et al :<# satis!actory response; &ut others
have sho*n no improvement$ 0ts use is investigational at present$ Patients at high ris/ !or recurrence such
as those presenting *ith positive margins, perineural sprea", or e+tracapsular sprea" in regional metastasis,
as *ell as patients *ho represent a poor surgical ris/ an" those *ho re!use surgery, coul" &e consi"ere" !or
enrollment in protocols that inclu"e com&inations o! ra"iation an" chemotherapy$
Conclusion
Paranasal sinus neoplasms constitute an enigma to hea" an" nec/ surgeons &ecause o! their
"i!!iculty in "iagnosis *ith such grave conse%uences !or the patient *hen !oun" in an a"vance" state$ A
high in"e+ o! suspicion lea"ing to more "etaile" stu"ies is there!ore necessary *hen patients "o not
respon" to routine me"ical management$ ,nce a paranasal sinus neoplasm is "iagnose", aggressive
multimo"ality therapy is o!ten necessary an" shoul" not &e "elaye"$

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