chapter 15 tumours of the head and neck

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    CHAPTER 15 - Tumors of the Head and Neck

    John J. Coleman III

    Mark R. ultan

    C!N"ENITA# #EI!N

    Th$ro%lossal &uct C$stsThe th$ro'd %land or'%'nates from the (har$n%eal floor at the foramen cecum dur'n%

    the fourth )eek of %estat'on. It enlar%es* +ecomes +'lo+ed* and descends ,entrall$ 'n

    the m'dl'ne of the neck 'n close a((ro'mat'on to the de,elo('n% h$o'd +one. &ur'n%

    th's descent the (atent d',ert'culum 's called the th$ro%lossal duct. The duct normall$

    resor+s +$ the tenth )eek of %estat'on. hen all or a (ort'on of th's duct (ers'sts*

    th$ro%lossal duct c$sts or s'nuses are formed.

    Class'call$ these c$sts (resent as m'dl'ne masses 'n ch'ldhood* althou%h the$ ha,e

    +een re(orted to +e as much as / cm from the m'dl'ne and ma$ (resent for the f'rst

    t'me 'n adults. E'%ht$ (ercent occur at or 0ust +elo) the h$o'd +one. A maneu,er to

    d'fferent'ate them from &el(h'an l$m(h nodes or other central masses 's to ha,e the(at'ent (rotrude the ton%ue. The le,el of the c$st 's ele,ated +$ (rotrus'on*

    demonstrat'n% 'ts em+r$olo%'c or'%'n from the +ase of the ton%ue. nl'ke +ranch'al

    cleft remnants* th$ro%lossal duct c$sts %enerall$ do not ha,e eternal s'nuses. A

    s'%n'f'cant (ro(ort'on do +ecome 'nfected* ho)e,er* usuall$ dur'n% the course of an

    u((er res('rator$ tract 'nfect'on. A((ro'matel$ 5 (ercent of the c$sts conta'n

    funct'onal th$ro'd t'ssue* and rare cases of th$ro%lossal duct carc'noma ha,e +een

    re(orted.

    The d'fferent'al d'a%nos's for m'dl'ne neck masses a+out the h$o'd +one 'ncludes

    l'n%ual th$ro'd t'ssue. In rare cases th's 's the (at'ent2s onl$ act',e th$ro'd %land.

    Therefore* the (resence of th$ro'd t'ssue 'n 'ts normal anatom'c locat'on must +e

    conf'rmed e'ther cl'n'call$ or +$ rad'oact',e scan +efore an$ m'dl'ne neck mass 's

    ec'sed* and careful (osto(erat',e o+ser,at'on for h$(oth$ro'd'sm 's 'm(erat',e.

    'strunk 's cred'ted )'th the de,elo(ment of a techn'3ue for ec's'n% th$ro%lossal

    duct c$sts and s'nuses that m'n'm'4es the r'sk of recurrence. He descr'+ed resect'on of

    the c$st )'th the central (ort'on of the h$o'd +one* follo)'n% the s'nus su(er'orl$ to

    'ts (resumed s'te of or'%'n* the foramen cecum* and ec's'n% 't 'n 'ts ent'ret$.

    ranch'al Cleft Anomal'es

    ranch'al cleft c$sts* s'nuses* and cart'la%'nous remnants result from the 'ncom(letefus'on of the +ranch'al clefts. The +ranch'al clefts* a((ear'n% 'n )eek 6 of em+r$on'c

    l'fe and normall$ 'n,olut'n% full$ +$ )eek 7* contr'+ute to the format'on of ,ar'ous

    head and neck structures 'n the de,elo('n% em+r$o. hen a (ort'on of a cleft (ers'sts*

    e('thel'um-l'ned c$sts or s'nuses* )'th or )'thout cutaneous o(en'n%s and

    cart'la%'nous rests* ma$ result. These anomal'es usuall$ (resent 'n the f'rst decade of

    l'fe +ut ma$ %o undetected unt'l adulthood. The ma0or't$ of +ranch'al cleft c$sts and

    s'nuses are l'ned +$ s3uamous e('thel'um* althou%h c'l'ated columnar e('thel'um has

    +een re(orted as )ell. ranch'al cleft carc'noma occurs rarel$ )hen there 's a h'stor$

    of a +ranch'al cleft c$st* the su+se3uent de,elo(ment of e('dermo'd cancer at that s'te*

    and no other (r'mar$ les'on. ranch'al cleft c$sts also conta'n l$m(ho'd t'ssue and

    ma$ enlar%e 'n res(onse to u((er res('rator$ tract 'nfect'ons.

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    The most common t$(es of +ranch'al cleft anomal'es are those of the second cleft and

    are found at the m'ddle and lo)er th'rds of the sternocle'domasto'd muscle.

    Cart'la%'nous rests from the f'rst +ranch'al cleft t$('call$ are su+cutaneous* usuall$

    a((ear med'al to the tra%us* and ma$ +e mana%ed +$ s'm(le ec's'on. C$sts and

    s'nuses often etend more dee(l$ 'nto the neck. The f'stulous tract courses su(er'orl$

    alon% the carot'd sheath and then med'all$ o,er the h$(o%lossal ner,e +et)een the'nternal and eternal carot'd arter'es to end at the (har$n ad0acent to the tons'llar

    fossa. A sta'rste( 'nc's'on 's somet'mes needed to follo) th's c'rcu'tous route. 8'rst

    +ranch'al cleft c$sts and s'nuses are located a+o,e the le,el of the h$o'd +one 0ust

    +elo) the +od$ of the mand'+le and etend su(erolaterall$ throu%h the (arot'd %land

    to end )'th'n the mem+ranous eternal aud'tor$ canal. Ec's'on of these c$sts and

    s'nuses 's recommended to a,o'd the com(l'cat'ons assoc'ated )'th recurrent

    'nfect'on. &'ssect'on must +e met'culous to a,o'd 'n0ur$ to the fac'al* h$(o%lossal*

    ,a%us* and l'n%ual ner,es and to the carot'd ,essels. Anomal'es of the th'rd +ranch'al

    cleft are rare. #'ke second cleft anomal'es* the$ ar'se anter'or to the m'ddle and lo)er

    th'rds of the sternocle'domasto'd muscle. Ho)e,er* the$ course +eh'nd the carot'd

    arter$ to end at the ($r'form s'nus.

    Heman%'omas and 9ascular Malformat'ons

    Con%en'tal ,ascular les'ons must +e clearl$ class'f'ed as heman%'omas or ,ascular

    malformat'ons 'n order to assess the'r (ro%nos's and esta+l'sh a((ro(r'ate

    mana%ement (lans. The d'st'nct'ons +et)een the t)o ha,e +een clar'f'ed on the +as's

    of cellular and cl'n'cal character'st'cs. Heman%'omas ha,e an 'ncreased m'tot'c

    act','t$ and as such ma$ +e cons'dered true neo(lasms. The$ are t$('call$ a+sent at

    +'rth or ma$ +e (resent as a fa'nt ,ascular +lush. &ur'n% the f'rst se,eral months of

    l'fe the$ ma$ under%o a ra('d (rol'ferat',e (hase* dur'n% )h'ch the$ somet'mes %ro)

    to lar%e s'4e. Althou%h the ma0or't$ of heman%'omas under%o s(ontaneous 'n,olut'on

    +$ the a%e of se,en* com(l'cat'ons such as ulcerat'on and +leed'n%* o+struct'on of the

    e$e )'th su+se3uent am+l$o('a* nasal a'r)a$ o+struct'on* and rarel$

    throm+oc$to(en'a :;asa+ach-Merr'tt s$ndrome< ma$ mandate earl$ sur%'cal

    resect'on. $stem'c deamethasone thera($* or the 'ntra,enous adm'n'strat'on of

    'nterferon-a* for a short course has +een found to arrest the %ro)th of lar%e les'ons

    dur'n% the'r (rol'ferat',e (hase. The use of 'ntrales'onal stero'd 'n0ect'ons or

    scleros'n% a%ents ma$ +e successful 'n ach'e,'n% tem(orar$ control of smaller

    heman%'omas 'n certa'n locat'ons such as the l'( or e$el'd. Photod$nam'c laser

    thera($ ma$ +e hel(ful 'n (re,ent'n% the onset of the (rol'ferat',e (hase of

    heman%'omas.

    9ascular malformat'ons* unl'ke heman%'omas* ha,e a normal rate of endothel'al cell

    turno,er. The$ result from con%en'tal errors 'n ,ascular mor(ho%enes's and are

    class'f'ed +$ the'r ,essel of 'n,ol,ement=ca('llar$* ,enous* arter'al* l$m(hat'c* or

    com+'ned. H'%h-flo) les'ons result from %ross a+normal't'es connect'n% the arter'al

    and ,enous s$stems and ma$ cause catastro(h'c (ro+lems of mass',e hemorrha%e*

    h'%h-out(ut con%est',e heart fa'lure* and hemol$t'c anem'a. Chron'c 'ncreased +lood

    flo) ma$ +e assoc'ated )'th skeletal a+normal't'es such as +one h$(ertro(h$ and

    d'stort'on. Ca('llar$* ,enous* and arter'al malformat'ons ma$ occur an$)here 'n the

    head and neck. #$m(hat'c malformat'ons :c$st'c h$%romas< class'call$ occur 'n the

    neck or the floor of the mouth. All malformat'ons are (resent at +'rth* althou%h the$

    ma$ not +e cl'n'call$ e,'dent unt'l the ectat'c ,essels suddenl$ d'late under hormonalor other (h$s'olo%'c 'nfluences. The$ normall$ %ro) (ro(ort'onall$ )'th the ch'ld and

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    do not re%ress s(ontaneousl$* unl'ke heman%'omas. Therefore* the mana%ement of

    malformat'ons often 's sur%'cal. Ind'cat'ons for earl$ sur%'cal resect'on 'nclude

    recurrent 'nfect'ons* o+struct',e s$m(toms :e.%.* res('rator$ d'stressc$st'c h$%romas*?

    are no) class'f'ed +$ the'r ma0or ,essel of 'n,ol,ement=ca('llar$* ,enous* or

    l$m(hat'c malformat'on. Accurate descr'(t'on 's cruc'al* s'nce the (ro%nos's andmana%ement of ,ascular +'rthmarks de(end d'rectl$ on the'r correct class'f'cat'on as

    heman%'omas or malformat'ons.

    In dee(-seated su+cutaneous ,ascular malformat'ons such as those somet'mes

    occurr'n% 'n the cheek or (har$n* (h$s'cal eam'nat'on ma$ +e 'nade3uate for

    accurate del'neat'on. 8urther e,aluat'on often 's necessar$ and ma$ 'nclude com(uted

    tomo%ra(h$ :CT< or ma%net'c resonance 'ma%'n% :MRI

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    The oral l'n'n% conta'ns countless mucous %lands* the m'nor sal',ar$ %lands. A

    su+mucosal accumulat'on of mucus results 'n a mucous retent'on c$st. 'nce the

    ma0or't$ of these c$sts ha,e no e('thel'al l'n'n%* 't 's +el'e,ed that the$ are most often

    caused +$ ru(ture of the duct s$stem* )'th etra,asat'on of mucus* and not +$ ductal

    o+struct'on* as )as (re,'ousl$ thou%ht. The most common locat'on for a mucous c$stor mucocele 's the la+'al mucosa of the lo)er l'( :8'%. 15-1

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    the$ t$('call$ occur as f'rm* su+mucosal s)ell'n%s 'n the m'ddle one-th'rd and ma$

    therefore m'm'c s3uamous cell carc'noma. ed%e ec's'on 's curat',e.

    The oral l'n'n% 's su+0ect to a num+er of ulcerat',e cond't'ons. The 'd'o(ath'c

    a(hthous ulcer 's the most common t$(e. The c$cle of (a'nful ulcerat'on and

    s(ontaneous heal'n% ma$ occur se,eral t'mes a $ear and (ers'st for man$ $ears. !thers'm'lar ulcers ha,e 'dent'f'a+le causat',e factors* 'nclud'n% ,'ral 'nfect'on )'th her(es

    s'm(le* nutr't'onal def'c'enc'es 'nclud'n% ,'tam'n * folate* or 'ron* and emot'onal

    stress. These ulcers often res(ond to to('cal stero'ds. Mult'(le (a'nful oral ulcerat'ons

    ma$ +e a man'festat'on of (em(h'%us ,ul%ar's. Th's ma$ +e accom(an'ed +$ se,ere*

    %eneral'4ed to'c't$. The d'sease t$('call$ occurs 'n the f'fth to se,enth decade of l'fe

    'n (at'ents of Med'terranean descent. The ulcers +e%'n as 'ntrae('thel'al +ullae that

    su+se3uentl$ ru(ture and ulcerate. The o,erl$'n% e('thel'um ma$ +e ru++ed off eas'l$

    :N'kolsk$2s s'%n

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    (ercent of ch'ldren )'th c$st'c f'+ros's ma$ also de,elo( nasal (ol$(s. The (ol$(s are

    often mult'(le* 'n,ol,'n% +oth s'des of the nasal ca,'t$ and the (aranasal s'nuses.

    The$ ma$ (resent )'th nasal o+struct'on* muco'd nasal d'schar%e* or anosm'a. Those

    that ar'se 'n the re%'on of the tur+'nates and ethmo'ds are ma'nl$ aller%'c 'n or'%'n*

    )h'le those of the (oster'or nasal ca,'t$ are most often 'nfect'ous. Med'cal

    mana%ement should 'nclude an e,aluat'on for aller%'es. As('r'n should +e sto((ed ora,o'ded* s'nce an assoc'at'on e'sts +et)een as('r'n use and the format'on of nasal

    (ol$(s. If the aller%'c e,aluat'on 's ne%at',e* an em('r'c tr'al of ant'm'cro+'als 's

    +e%un. tero'd nasal s(ra$s ma$ +e hel(ful. hen med'cal mana%ement fa'ls* sur%'cal

    'nter,ent'on ma$ +e necessar$. 'm(le (ol$(ectom$ carr'es a h'%h rate of recurrence*

    and more etens',e endosco('c or o(en ec's'on ma$ +e necessar$.

    Pa('llomatous %ro)ths ma$ occur on the nasal sk'n or )'th'n the nasal ca,'t$. The

    s3uamous (a('lloma 's an eo(h$t'c ,errucous les'on caused +$ the (a('lloma,'rus

    and 's (resent on the sk'n of the nasal s'lls* columella* or alae. It 's mana%ed as are

    other cutaneous )arts. The 'n,erted (a('lloma :also called schne'der'an (a('lloma*

    s3uamous (a('lloma* or (a('llomatos's< 's a (ol$(o'd mass occurr'n% on the lateralnasal )all* t$('call$ 'n m'ddle-a%ed men. The name 's der',ed from 'ts h'stolo%'c

    a((earance of an >'n,erted? (rol'ferat',e %ro)th (attern. The s'%n'f'cance of th's

    les'on l'es 'n 'ts (resentat'on )'th s$m(toms of nasal o+struct'on* 'ts recurrence rate

    of 5D (ercent )'th (ol$(ectom$ alone* and 'ts assoc'at'on )'th concurrent : (ercent