Congenital disorders of the Larynx - UCLA Head and Neck Surgery
2011-07-PATHO-Head and Neck Disorders 1
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Oral Soft Tissue
y Inflammatory/reactivelesions: Non neoplastic. Usuallyaresponsetochronic
irritation,ieill fitting dentures, bracesor
dentalcaries
o Fibrousproliferativelesionso Aphthousulcers (cankersores)o Glossitis
y Infectionsy Tumorsand pre cancerouslesions
Inflammatory/reactive lesions
y Fibrousproliferativelesionso Irritation fibroma: see diagram below
61% ofproliferativelesions Primarilyoccursinthe buccal
mucosaalongthe bitelineatthe
gingivodentalmargin
Consistsofanodularmassoffibroustissuewithfew
inflammatorycellscovered by
squamousmucosa Grossly,lesionissmooth. Takeson
coloroflocaltissue. Erosionsmay
bepresent.
o Pyogenicgranuloma: see diagrambelow
12% ofproliferativelesions Similartocapillaryhemangioma Regressesspontaneously,
particularlyafterpregnancyor
undergofibrosis
May developintoaperipheralossifyingfibroma
Vascularlesion,soreddishhue Commoninlowerlip Pedunculated,highlyvascular,
brightred lesionoccurringinthe
gingivalofchildren,youngadults,
and commonly,pregnantwomen
(pregnancytumor)
Histologically: overlyingepitheliumiseroded and proliferationof
capillarysized blood vessels
surrounded byinflammatorycells
o Peripheral ossifying fibroma 22% ofproliferativelesions Reactivegrowthofgingival (red,
ulcerated,nodularlesions)with
unknownetiology
Someariseasresultofmaturationoflongstandingpyogenicgranuloma,others donot;
may bemistakenclinicallyfor
pyogenicgranulomas
Peakincidence: youngfemaleso Peripheral giant cell granuloma (Giant
Cell Epulis)
5% ofproliferativelesions Similartopyogenicgranuloma butis
more bluishpurpleincolor
Lesionprotrudesfromgingivalatsitesofchronicinflammation
Covered bygingivalmucosa butmay beulcerated
Histologically: aggregatesofmultinucleated,foreign body type
giantcellsseparated bya
fibrovascularstroma.
Note: Dr.Pascualsaid thatthefrequencyofthe
abovelesionsis,in descendingorder:
y Irritation fibromay Pyogenicgranulomay Peripheral giant cell granulomay Peripheral ossifying fibroma
However,thepercentagesaboveweretakenfrom
the book. So,useyour judgmentshould this
questionappearontheexam.
y Canker Sores, aka Aphthous Ulcers: seediagram below
o Etiologyunknown, butsomesaymay bestressrelated
Subject: PathologyTopic: Head and Neck 1Lecturer: Dr. PascualDate of Lecture:July 26, 2011Transcriptionist: MopsterEditor: TelsioPaoPages: 9
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o Commonsuperficial,painful,oftenrecurrentulcerationsoftheoral
mucosa
o Lesionsappearassingleormultipleo Shallow,hyperemiculcerwiththinrim
ofexudatesand rimmed byanarrow
zoneoferythema
y Glossitis:see diagram belowo Inflammationoftongueo Beefyred tongueencountered in
certain deficiencystates (VitaminB12,
niacin,pyridoxine,riboflavin deficiency,
iron deficiencyanemia)
o Plummer Vinson Syndrome/PatersonKellySyndrome
Triad: glossitis,esophagealwebs,iron deficiencyanemia.
o Atrophyofpapillaethinningofmucosaexposureofunderlying
vasculaturebeefyred tongue
o Glossitiswithulcerativelesions(sometimesalongthelateral
bordersofthetongue),mayalso be
seenwith jagged cariousteeth,ill
fitting dentures,and rarely,with
syphilis,inhalation burns,or
ingestionofcorrosivechemicals
Infections
y Herpes Simplex virus infectionso CommonlyHSV1 butmayalso be
caused byHSV2o Morphology: vesiclefilled withserous
fluidrupturepainfululcer
o FindingsonTzancksmear: multinucleated giantcells intranuclearinclusions intercellularedema (acantholysis)
o Cold sores
Blistersand vesiclesaroundmucosalorifices (lips,nose)
Formed byintercellularedemaandballooning degenerationof
epidermalcells
o Gingivostomatitis Usuallyencountered inchildren;
HSV1
Vesiculareruptionextendingfromthetonguetotheretropharynxassociated withcervical
lymphadenopathy
o Recurrent herpetic stomatitis Occurseitheratthesiteofthe
primaryinoculationorinadjacent
mucosalareasthatareassociated
withthesameganglion
Taketheformofgroupsofsmall (1to 3 mm)vesiclesinthelips,hard
palate,nasalorifices
y Oral candidiasis (Thrush)o Mostcommonfungalinfectioninthe
oralcavity
o Candida albicanso Factorsthatincreaselikelihood of
infection:
Immunestatus StrainofC. albicanspresent Compositionofindividualsflora
o 3 clinicalforms: Pseudomembrane: can bescraped
offtorevealahyperemic base Erythematosus Hyperplastic
o Commonin: Diabetesmellitus Bonemarrowororgantransplant Neutropenia Chemotherapyinduced
immunosuppression
AIDS Useofbroad spectrumantibiotics
Tumors and pre cancerous lesions
y Smokingisthemostcommonantecedenty Leukoplakia: see 4 diagrams below
o Awhitepatchorplaquethatcannot bescraped off(which differentiatesitfrom
oralthrush)and cannot be
characterized clinicallyorpathologically
asanyother disease
o Allleukoplakiasmust beconsideredprecancerous
o Mayoccuranywhereintheoralcavity(favored locations: buccalmucosa,
floorofthemouth,ventralsurfaceof
tongue,gingival,palate)
o Morphology: Solitary,ormultiple,withindistinct
orsharply demarcated borders
May beslightlythickened andsmoothorwrinkled and fissured or
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mayappearasraised,sometimes
corrugated,verrucousplaques
o Histologically: hyperkeratosisacanthotic,orderlyormarkedly
dysplasticmucosalepithelium
y Erythroplakia: see both diagrams belowo Lesscommon butmoreominousthan
leukoplakia,incurringahigherriskof
malignanttransformation
o Red: intensesubepithelialinflammationwithvascular dilatation
o Markedly dysplasticepithelium,velvety,sometimeseroded area
o May beslightly depressed or blend inwithsurroundingarea.
o Histologically: Superficialerosionswith dysplasia,
carcinomainsitu,oralready
developed carcinoma
Intensesubepithelialinflammatoryreactionwithvascular
dilatationred appearance
y Speckled leukoerythroplakiao Intermediateformswiththe
characteristicsofbothleukoplakiaand
erythroplakia
y Squamous Cell Carcinomao 95% ofhead and neckcancersareSCC,
mostcommonlyintheoralcavity
o HNSCC: aggressive Multipleprimarytumors: field
cancerization
Riskfactors Smoked tobacco Alcohol HPVoncogenicvariants Familyhistoryofhead and neck
cancer
Actinicradiation: exposuretoUVradiation
Pipesmoking Betelchewing Chronicirritation
o Molecular biology Geneticalterationhave been
identified and related to
morphologicchangesinepithelium:
InactivationofP16genehyperplasia/hyperkeratos
is
Mutationofp53dysplasia Alterationsand deletionsat 4q,
6p, 8p, 11q, 13q, 14qfrank
malignancy
o SCC of the Oral Cavity Sites: floorofmouth,ventral
surfaceoftongue,lowerlip,soft
palate,gingival
Mayinvadetheunderlyingstromawithoutgoingthroughfull
thicknessCISstage
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Differentiation dependson degreeofkeratinization, but doesnot
correlatewith behavior
Tendstoinfiltratelocally beforedistantmetastasis
Spread and metastasis: Tendstoinfiltratelocally before
distantmetastasis
Metastasisisprimarily bylymphaticroute: Cervicallymphnodes Favored distantsites:
Mediastinallymphnodes
Lungs Liver Bones
o Prognostic determinants: Location: bestprognosislowerlip
(5yearSR = 90%) b/cofearlier
detection duetoitsobvious
visibility (asopposed to,say,the
backofthetongue).
Stage: depthofinvasion,lymphnodeinvolvement,metastasis
Grade Desmoplasticreaction: inthelip,
florid desmoplasiaisamarkerof
aggressive behavior
Tissueeosinophilia (favorablefactor)
LossofHantigen (precursorantigeninRBCswhichhelps determine
blood type)invasivenessand
distantspread
Overexpressionofp21genefavorableprognosisintongue
cancer
Amplificationof3q26-3 locuspoorprognosis
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Upper Airways: nose, sinuses, pharynx, larynx
Inflammation:
o Infectious Rhinitis:o Infectious rhinitis (common cold)
Adenovirus,echovirus,rhinovirus Thickened,edematousand red
mucosa
o Allergic rhinitis (hayfever) TypeIhypersensitivityreaction Edema,redness,mucusreaction Eosinophilsareprominentinthe
inflammatoryreaction
o Chronic rhinitis Resultsfromrepeated attacksof
acuterhinitiswith developmentof
bacterialinvasion
Superficial desquamationorulceration
Mixed inflammatoryinfiltrates:Neutrophils,lymphocytes,and
plasmacell
y Nasal polyps: see 2 diagrams belowo Focalprotrusionsofthenasalmucosa
secondarytorecurrentattacksof
rhinitis
o 3 -4 cm;edematousmucosa;hyperplasticorcysticmucousglands
o Histologically: Polypoid structureslined by
respiratoryepithelium
Inflammatoryinfiltrates:Neutrophils,eosinophils,plasma
cells,occasionallylymphocytes
y Sinusitiso Mostcommonlypreceded byacuteor
chronicrhinitis
o Occasionally,isanextensionofperiapicalinfection (maxillarysinusitis)
o Inflammatoryedemaofthemucosaimpairs drainageofthesinus
ifsuppurativeexudatesempyemaofthesinus
Accumulationofmucussecretionand cysticformationmucocele
Mayspread totheorbitand otherbonystructuresduralveinsinus
thrombosis
y Pharyngitis/tonsillitiso Usuallyafterorpartofaviral URTIo Etiology: rhinovirus,echovirus,
adenovirus, -hemolyticstreptococcus
o Reddeningtoslightedemaofthenasopharynx
o Enlarged,reddened (follicular)tonsils(duetoreactivelymphoid hyperplasia)
y Laryngitiso Mostcommon diseaseinthelarynxo Causes:
Partofageneralized URTI Heavyexposuretotobaccosmoke
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Partofasystemic disease (TB,diphtheria)
o Inchildren (whoselarynxaresmall),severeedemaand inflammationcause
obstruction
laryngeoepiglottitis (H. influenza, - hemolytic Strep)
laryngotracheobronchitis (croup)inspiratorystridor
Tumors
y Nasopharyngeal angiofibromao Veryvascularo Almostexclusivelyinadolescentmaleso Despiteits benignnature,maycause
seriousclinicalproblems becauseofits
tendencyto bleed profusely during
surgery
y Sinonasalpapilloma: see diagram belowo Lined bysquamousorcolumnar
epithelium
o HPVtype6and 11o 3 forms:
Septal(mostcommon) Inverted: benign butlocally
aggressive (no desmoplasia)can
invadetheorbitorrarely, develop
intocarcinoma. Called inverted
becauseitseemsto begrowing
inward instead ofoutward.
Cylindrical Figure below: inverted type
y Reactive nodules (vocal cord nodules andpolyps)
o Etiologicfactors Heavysmoking Greatstrainonvoice (singers)can
lead tochangeincharacterofvoice
orlead toprogressivehoarseness
o Smooth,rounded,sessileorpedunculated excrescences
o Covered bysquamousepithelium(keratotic,hyperplastic,orslightlydysplastic)
o Loosemyxoid CTcoreo Vocalcord polypstend to beunilateralo Vocalcord nodulestend to be bilateral
y Squamouspapilloma: see diagram belowo Ariseontruevocalcordso Singleinadults
o Multipleinchildrenjuvenilelaryngealpapillomatosis
o Etiology: HPVtype6and 11o Morphology:
Soft,raspberry likeexcresences,
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y Laryngeal carcinoma: see diagrams belowo Spectrumofhyperplasia dysplasia-
carcinomaisseen
o Riskfactors: tobacco,alcoholo Clinicalmanifestations: persistent
hoarsenessleadingtopain, dysphagia,
hemoptysis
o 95% ofthesecasesareofthesquamouscellcarcinomatype
o Smooth,white,orreddened focalthickeningso Verrucousorulcerated,whitetopinkor
graylesions
o 60% areconfined tothelarynx(intrinsic);ifoutsidethelarynxextrinsic
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Neck
y Branchial cysto Fromremnantsofbranchialarchesor
from developmentalsalivarygland
inclusionswithincervicallymphnodes
o 2 5cm;lined bystratified squamousorpseudostratified columnarepitheliumunderlain byintense
lymphoid aggregates
y Thyroglossal duct cysto 1 4 cm;lined bystratified squamous
epithelium (ifnearthetongue)or by
pseudostratified columnarepithelium
(ifitislower)
o Withinthecystwallarelymphoidaggregatesand remnantsofthyroid
tissue
y Carotid bodytumor (paraganglioma)o Referstoextra adrenaltumorsarising
fromparaganglia
o Developin 2 generallocations: Paravertebralparaganglia: with
sympatheticconnections;50%
elaborate
catecholamineschromaffinpositive
Aorticopulmonarychain (includingcarotid bodies,etc)
Parasympatheticconnections Infrequentlyrelease
catecholaminesnon-chromaffinparaganglioma
Senseoxygenand CO2tensionswithinadjacent
vesselschemodectoma
Red pinkto brown;
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Salivary glands
Inflammation
y Sialadenitiso Etiology: traumatic,viral, bacterialor
autoimmune (Sjogrens)
o Types: Acute suppurativesialdenitis:
bacterial
Viral sialadenitis: CMV,paramyxoviruses, EBV,
parainfluenzavirus,influenza
virus (mumps)
Granulomatoussialadenitis Often duetoresponsetofreed
ductalcontentsinobstructive
sialadenopathy
Infectiousinsome Chronic sclerosingsialadenitis
(submandibular gland)
Plasmacyticand lymphocyticperiductalinfiltrates Eventuallyathickfibrous
capsuleenclosesthesalivary
ducts
y Sialolithiasis: see diagram belowo 2 formsoflithiasisinthehead and neck
Angiolithiasis: lesscommon;moreofteninoralorperioralsofttissue
Sialolithiasis: exclusivelyinthesalivarygland
Mayoccurinanygland butmoreoftenthesubmandibular
gland becauseofthemore
torturouscourseofits ductand
itsmoremucinous
natureductal
obstructionglandularswelling
aftermeals
Histologically: Dilated ductsoftenwith
squamousmetaplasia
Variableacinar destructionchronicinflammationandfibrosis
y Cysts: mucocele: see both diagrams belowo ExtravasationMucocele most
common (85%)
Obstructionorruptureofsalivarygland causethesalivatoleakinto
thesurroundingstromamucous
filled pseudocystswithconnective
tissuecapsuleand nolining
epithelium
Mostcommonsites: lips (8
0
%) Ranula: referstomucocelearisingfrom damaged ductofthe
sublingualgland (plungingranula)
o Retention mucocele Salivaryglands Lined byepithelium (flat,cuboidal,
orstratified)
Tumors
y 65 8
0% occurintheparotid (1
0% insubmandibular)
y Likelihood ofmalignancyisinverselyproportionaltothesizeofthegland. Ie,the
smallerthegland thelargerthelikelihood
ofmalignancy.
y Malignant: 15 30% oftumorsintheparotid; 40%...
y F>M
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y Pleomorphic adenoma (mixed tumor): seediagrams below
o Mostcommonneoplasmofthesalivarygland
o 60% areintheparotido Painless,slowlygrowingmasso Derived from ductal (orepithelial)cells
and myoepithelailcells
o Recall: mixed tumorismorethanonetissue derived from 1 germlayero Carcinomaexpleomorphicadenomais
malignantcounterpart
o Gross: Rounded and well demarcated Encapsulated exceptinareaswhere
thecapsuleisnotfully developed
withtonguelikeprotrusionsinto
thesurroundingglandular
parenchyma,sotumorislikelyto
recurifitisnotremoved entirely.
o Histologically: Epithelialelements Mesenchymelikemyxoid tissue Chondroid orrarely, bonyislands
y Warthins tumor (papillarycystadenomalymphomatosum): see
diagrams belowo 2ndmostcommonsalivarygland tumoro Almostalwaysseenintheparotid glando Benigno M>F;5th 7th decadesoflifeo 10% aremultifocal; 10% are bilateralo Gross:
Round tooval 2 5cm
Encapsulated Palegreysurfacewithnarrowcystic
orcleft likespacesfilled with
mucinousorserousfluid
o Histologically: Papillaryexcrescencespapillary Cysticspacesarelined by double
liningofcells/polypoid
projectionscystadenoma Surfacelayerofcolumnarcells
(toward lumen)restingonalayerof
cuboidaltopolygonalcells
Beneaththisis densereactivelymphoid stromalymphomatosum
y Mucoepidermoid carcinomao Mostcommonprimarymalignant
tumorofthesalivarygland
o Occursmainlyintheparotid gland (60 70%) butaccountsforalargefractionof
salivarygland tumorsinotherglands,
especiallyminorglands
o Lowgradetumors: Locallyinvasive Rarelymetastasize
o Highgradetumors: Invasive; difficulttoexcise Highrecurrencerate Spreadsto distantsites
o Gross: Upto 8 cm No distinctcapsule Infiltrativeatmargins Palegray whiteparenchymawith
small,mucincontainingcysts
o Histologically: Squamous,mucousand
intermediatecellsincords,sheets
orcysticstructures (see below)
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TheLord islovingand merciful,slowto become
angryand fullofconstantlove.Psalm 145:
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