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Practice Essentials

Thyroid cancers represent approximately 1% of new cancer diagnoses in the United States eachyear. Thyroid malignancies are divided into papillary carcinomas (8%!" follic#lar carcinomas(1%!" med#llary thyroid carcinomas ($1%!" anaplastic carcinomas (1&%!" primary thyroidlymphomas (rare!" and primary thyroid sarcomas (rare!.

Signs and symptoms

Thyroid carcinoma most commonly manifests as a painless" palpa'le" solitary thyroid nod#le.Patients or clinicians discover most of these nod#les d#ring ro#tine palpation of the nec.

Signs and symptoms associated with malignancy in thyroid nod#les incl#de the following)

Solitary nod#les) *ost liely to 'e malignant in patients older than + years and in

patients yo#nger than , years

-ncreased rate of malignancy in males

od#lar growth

/apid growth) 0mino#s sign

Us#ally painless (nontender to palpation! s#dden onset of pain more strongly associatedwith 'enign disease (eg" hemorrhage into a 'enign cyst" s#'ac#te viral thyroiditis!

2ard and fixed nod#les

Diagnosis

The ey to the wor#p of the solitary thyroid nod#le is to differentiate malignant from 'enigndisease and" th#s" to determine which patients re3#ire intervention and which patients may 'emonitored serially. 2istory taing" physical examination" la'oratory eval#ation" and fineneedleaspiration 'iopsy (456! are the mainstays in the eval#ation of thyroid nod#les. -maging st#diescan 'e ad7#ncts in select cases.

Examination in patients s#spected of thyroid cancer incl#des the following)

Thoro#gh head and nec examination" incl#ding thyroid gland and cervical soft tiss#es -ndirect laryngoscopy

4irm cervical masses are highly s#ggestive of regional lymph node metastases. ocal foldparalysis implies involvement of the rec#rrent laryngeal nerve.

Procedures

456 is the most important diagnostic tool in eval#ating thyroid nod#les and sho#ld 'e the firstintervention. The following are the 9 possi'le res#lts from this proced#re)

6enign disease

*alignant disease

-ndeterminate for diagnosis

ondiagnostic

Up to $% of repeated 'iopsies res#lt in a definitive diagnosis. Patients whose findings areindeterminate or nondiagnostic despite repeat 'iopsy can #ndergo s#rgery for lo'ectomy fortiss#e diagnosis. ondiagnostic cases can also 'e monitored clinically" and radioiodine scanscan 'e #sef#l for determining the f#nctional stat#s of the nod#le" 'eca#se most hyperf#nctioningnod#les are 'enign.

Laboratory testing

The following la'oratory st#dies may 'e #sed to assess patients with s#spected thyroid cancer)

Ser#m thyroidstim#lating hormone concentration) Sensitive for

hyperthyroidism:hypothyroidism and for eval#ation of solitary thyroid nod#les


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Ser#m calcitonin:pentagastrinstim#lated calcitonin levels) Elevated levels highly

s#ggestive of med#llary thyroid carcinoma

Polymerase chain reaction (P;/! assay for germline m#tations in the /ET proto

oncogene) 4or diagnosis of familial med#llary thyroid carcinomaImaging studies

The following imaging st#dies may 'e #sed to eval#ate patients with s#spected thyroid cancer)

ec #ltrasonography) *ost common modality to eval#ate thyroid disease however"

limited #sef#lness for disting#ishing 'etween malignant and 'enign nod#les

Thyroid radioiodine imaging) To determine f#nctional stat#s of a nod#le '#t cannot

excl#de carcinoma

ec comp#ted tomography scanning or magnetic resonance imaging (avoid iodinated

contrast agents!) To eval#ate softtiss#e extension of large or s#spicio#s thyroid masses intothe nec" trachea" or esophag#s" and to assess metastases to the cervical lymph nodes norole in ro#tine management of solitary thyroid nod#les

Management

*alignant diagnoses re3#ire s#rgical intervention. Papillary thyroid carcinoma and med#llarythyroid carcinoma are often positively identified on the 'asis of 456 res#lts alone. ;ervicalmetastases discovered preoperatively or intraoperatively sho#ld 'e removed 'y means of en'loc lymphatic dissection of the respective cervical compartment (selective nec dissection! whilesparing the nonlymphatic str#ct#res.

Well-differentiated neoplasms

Patients with follic#lar neoplasm" as determined with 456 res#lts" sho#ld #ndergo s#rgery forthyroid lo'ectomy for tiss#e diagnosis. The extent of s#rgical therapy for welldifferentiatedneoplasms is controversial. Primary treatment for papillary and follic#lar carcinoma is s#rgicalexcision whenever possi'le. Total thyroidectomy has 'een the mainstay for treating welldifferentiated thyroid carcinoma. *odifications to total thyroidectomy incl#de s#'total

thyroidectomy to red#ce the ris of rec#rrent laryngeal nerve in7#ry and hypoparathyroidism.

Hrthle cell carcinomas

4or patients with 2


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=oxor#'icin or ;20P (ie" cyclophosphamide" hydroxyda#nomycin" 0ncovin >vincristine?"prednisone! is the commonly #sed chemotherape#tic regimen.

The treatment for thyroid sarcomas is total thyroidectomy. /adiation therapy may 'e #sed in anad7#nctive setting.

Postsurgical management

5fter total thyroidectomy" patients #ndergo radioiodine scanning to detect regional or distantmetastatic disease" followed 'y radioa'lation of any resid#al disease fo#nd. -n addition" patientsare given thyroid replacement therapy with T9 or triiodothyronine (T,!.

-n patients with anaplastic thyroid carcinoma" chemotherapy and radiation therapy are typicallyadministered in com'ination. Postoperative external'eam irradiation is effective in improvinglocal control this may also 'e #sed as primary treatment in #nresecta'le cases. ;hemotherapy(most commonly doxor#'icin! is added for palliation.

Image library

5lgorithm for the management of a solitary thyroid nod#le. 456 @ fine needleaspiration 'iopsy US @ #ltrasonography.

0verview

Thyroid malignancy occ#rs with relative infre3#ency in the United States" altho#gh 'enignthyroid disease is relatively common. 5ltho#gh patients with thyroid cancers generally have afavora'le prognosis compared with that of patients with many other solid t#mors" anestimated 1& patients died of thyroid cancer in the United States in 1AA8. ;ontemporarytreatment of patients with thyroid malignancy re3#ires a m#ltidisciplinary approach involvingan endocrinologist" a thyroid s#rgeon" a radiologist" and" on occasion" medical and radiationoncologists.

4or excellent patient ed#cation reso#rces" visit e*edicine2ealthBs Thyroid and *eta'olism;enter. 5lso" see e*edicine2ealthBs patient ed#cation articleThyroid Pro'lems.

5n image of 2#rthle cells can 'e seen 'elow.

5 monomorpho#s cell pop#lation of 2


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The incidence of thyroid malignancies is , times higher in women than in men. Theincidence of this disease peas in the third and fo#rth decades of life.

Thyroid cancers are divided into papillary carcinomas"follic#lar carcinomas" med#llarythyroid carcinomas (*T;s!" anaplastic carcinomas" primary thyroid lymphomas" and primarythyroid sarcomas. Papillary carcinoma represents 8% of all thyroid neoplasms. 4ollic#lar

carcinoma is the second most common thyroid cancer" acco#nting for approximately 1% ofcases. *T;s represent $1% of neoplasms. 5naplastic carcinomas acco#nt for 1&%.Primary lymphomas and sarcomas are rare.

Using data on 9AC US co#nties from the ational ;ancer -nstit#teBs S#rveillance"Epidemiolgy" and End /es#lts program" *orris et al fo#nd that the tripling of the incidence ofpapillary thyroid cancer in the past , decades is directly correlated with demographic andage'ased marers of access to health care" s#ggesting widespread overdiagnosis of thedisease. S#pporting this concl#sion" rates of mortality from thyroid cancer remained sta'led#ring this period.>1" &?

Etiology

Thyroid carcinomas arise from the & cell types present in the thyroid gland. Theendodermally derived follic#lar cell gives rise to papillary" follic#lar" and pro'a'ly anaplasticcarcinomas. The ne#roendocrinederived calcitoninprod#cing ; cell gives rise to *T;s.Thyroid lymphomas arise from intrathyroid lymphoid tiss#e" whereas sarcomas liely arisefrom connective tiss#e in the thyroid gland.

/adiation expos#re significantly increases the ris for thyroid malignancies" partic#larlypapillary thyroid carcinoma. This finding was o'served in children exposed to radiation afterthe n#clear 'om'ings in 2iroshima and agasai d#ring Dorld Dar --. 5dditional evidencewas gathered after atomic 'om's were tested in the *arshall -slands" after the accident atthe ;herno'yl n#clear power plant" and in patients who received lowdose radiation therapyfor 'enign disorders (eg" acne" adenotonsillar hypertrophy!. owdose radiation expos#refrom imaging st#dies has not 'een fo#nd to have a t#morigenic effect. /adiation targetingthe thyroid gland (eg" iodine1,1 a'lation of the thyroid! or highdose external'eamradiation therapy does not appear to increase the ris of papillary thyroid carcinoma. This ispres#ma'ly 'eca#se cell illing increases with these doses.

ow dietary intae of iodine does not increase the incidence of thyroid cancers overall.2owever" pop#lations with low dietary iodine intae have a high proportion of follic#lar andanaplastic carcinomas.

;linical Presentation

History

Thyroid carcinoma most commonly manifests as a painless" palpa'le" solitary thyroidnod#le. Patients or physicians discover most of these nod#les d#ring ro#tine palpation of thenec. Palpa'le thyroid nod#les are present in approximately 9C% of the general pop#lation"and most represent 'enign disease. 2ighresol#tion #ltrasonography reportedly depictsthyroid nod#les in 1A+C% of randomly selected individ#als. 5n estimated $1% of solitarythyroid nod#les are malignant. Palpa'le and nonpalpa'le nod#les of similar siFe have thesame ris of malignancy.

The patientBs age at presentation is important 'eca#se solitary nod#les are most liely to 'emalignant in patients older than + years and in patients yo#nger than , years. -n addition"thyroid nod#les are associated with an increased rate of malignancy in male individ#als.Growth of a nod#le may s#ggest malignancy. /apid growth is an omino#s sign.
http://emedicine.medscape.com/article/849000-overviewhttp://emedicine.medscape.com/article/849000-overviewhttp://emedicine.medscape.com/article/849000-overview


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*alignant thyroid nod#les are #s#ally painless. S#dden onset of pain is more stronglyassociated with 'enign disease" s#ch as hemorrhage into a 'enign cyst or s#'ac#te viralthyroiditis" than with malignancy.

2oarseness s#ggests involvement of the rec#rrent laryngeal nerve and vocal fold paralysis.=ysphagia may 'e a sign of impingement of the digestive tract. 2eat intolerance and

palpitations s#ggest a#tonomo#sly f#nctioning nod#les.

*ed#llary carcinoma can occ#r as part of m#ltiple endocrine neoplasia (*E! &5 or &6syndrome" as well as familial *T; (4*T;! syndrome. Patients with a family history ofthyroid cancer sho#ld 'e eval#ated with vigilance.

Physical examination

Physical examination sho#ld incl#de thoro#gh head and nec examination with caref#lattention to the thyroid gland and cervical soft tiss#es" as well as indirect laryngoscopy.

Solitary thyroid nod#les can vary from soft to hard. 2ard and fixed nod#les are mores#ggestive of malignancy than s#pple mo'ile nod#les are. Thyroid carcinoma is #s#ally

nontender to palpation. 4irm cervical masses are highly s#ggestive of regional lymph nodemetastases. ocal fold paralysis implies involvement of the rec#rrent laryngeal nerve.

Eval#ation and *anagement of the Solitary Thyroid od#leThe ey to the wor#p of the solitary thyroid nod#le is to differentiate malignant from 'enigndisease and" th#s" to determine which patients re3#ire intervention and which patients may'e monitored serially. 2istory taing" physical examination" la'oratory eval#ation" andfineneedle aspiration 'iopsy(456! are the mainstays in the eval#ation of thyroid nod#les.-maging st#dies can 'e ad7#ncts in select cases.

Fine-needle aspiration biopsy

456 is the most important diagnostic tool in eval#ating thyroid nod#les and sho#ld 'e thefirst intervention. The techni3#e is inexpensive and easy to perform" and it ca#ses fewcomplications.

To perform 456" comforta'ly position 'oth the patient and the physician. Extend thepatientBs nec slightly and palpate the nod#le with the nondominant hand. ;lean the sinwith alcohol and infiltrate the area with local anesthesia. Place a &1 to &$ga#ge needle onthe end of a syringe. *any physicians #se triggerstyle aspirating handles on the syringe.-ntrod#ce & m of air into the syringe" and place the needle into the sin. 5pply negativepress#re to the syringe" and pass the needle thro#gh the nod#le" which is identified 'y #singthe nondominant hand. 5fter several passes" release the negative press#re" and withdrawthe needle. Use the air remaining in the syringe to expel the specimen from the h#' and

needle onto a glass slide or into cytologic sol#tion for a cell 'loc. 4ix the slide in alcohol forPapanicolao# and hematoxylineosin staining. Some slides can 'e air dried and stained with/omanowsy stain (=iffH#ic!.

S#ccessf#l diagnosis 'y the cytologist depends on acc#rate sampling of the nod#le andspecimen cell#larity. 4or this reason" several a#thors recommend performing at least ,aspirations to ens#re ade3#acy of the specimen and to minimiFe falsenegative res#lts.Ultrasonographic g#idance can help to increase the acc#racy of 456. =anese et al reportincreased falsenegative rates with palpation 456 compared with #ltrasonographyg#ided456.

The 9 res#lts from 456 are 'enign disease" malignant disease" indeterminate for

diagnosis" and nondiagnostic. -n their review of several large series" Ghari' and Goellner(1AA,! fo#nd that +A% of 456 res#lts were 'enign" 9% were malignant" 1% were
http://emedicine.medscape.com/article/1819862-overviewhttp://emedicine.medscape.com/article/1819862-overviewhttp://emedicine.medscape.com/article/1819862-overviewhttp://emedicine.medscape.com/article/1819862-overviewhttp://emedicine.medscape.com/article/1819862-overview


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indeterminate" and 1C% were nondiagnostic. >,? Their falsepositive rate was &.A%" and theirfalsenegative rate was $.&%. Sensitivity and specificity were 8,% and A&%" respectively.

/es#lts of 456 determine the next step in managing the thyroid nod#le. 5 definitivediagnosis is o'tained in as many as $% of repeated 'iopsies. Patients whose findings arenondiagnostic despite repeat 'iopsy can #ndergo s#rgery for lo'ectomy for tiss#e diagnosis"

or they can 'e monitored clinically. -n these circ#mstances" radioiodine scans can 'e #sef#lfor determining the f#nctional stat#s of the nod#le" as most hyperf#nctioning nod#les are'enign.

-ndeterminate 'iopsy findings are la'eled s#spicio#s at some instit#tions. Dhen cell#larmaterial is ade3#ate for eval#ation '#t when malignant and 'enign disease cannot 'edifferentiated" 'iopsy res#lts can 'e la'eled s#spicio#s. Patients with a s#spicio#s diagnosissho#ld #ndergo lo'ectomy for definitive diagnosis.

*alignant diagnoses re3#ire s#rgical intervention. Papillary thyroid carcinoma and *T; areoften positively identified on the 'asis of 456 res#lts alone. -n patients with thesecarcinomas" definitive s#rgical planning can 'e #ndertaen at the o#tset. 2owever" it is

nearly impossi'le to disting#ish a follic#lar adenoma from a follic#lar carcinoma on the 'asisof 456 findings. Patients with follic#lar neoplasm" as determined with 456 res#lts" sho#ld#ndergo s#rgery for thyroid lo'ectomy for tiss#e diagnosis. These patients re3#ire completethyroidectomy if a malignancy is discovered on review of the pathology. Some controversyexists regarding the extent of thyroidectomy (total thyroidectomy" s#'total thyroidectomy" orlo'ectomy! for a partic#lar pathologic diagnosis. Each pathologic diagnosis and itscorresponding extent of thyroidectomy are disc#ssed 'elow.

;omplications of 456 are few and generally minor. The most common complications areminor hematoma" ecchymosis" and local discomfort. ;linically significant hematoma andswelling is exceedingly rare. -nadvertent p#nct#re of the trachea" carotid artery" or 7#g#larvein #s#ally does not ca#se clinically significant pro'lems and is managed with the

application of local press#re.

Laboratory ealuation

The ser#m thyroidstim#lating hormone (TS2! concentration is a highly sensitive meas#refor hyperthyroidism or hypothyroidism. 5 sensitive TS2 assay is #sef#l in the eval#ation ofsolitary thyroid nod#les. 5 low ser#m TS2 val#e s#ggests an a#tonomo#sly f#nctioningnod#le" which typically is 'enign. 2owever" malignant disease cannot 'e r#led o#t on the'asis of low or high TS2 levels.

0ther thyroid f#nction tests are #s#ally not necessary in the initial wor#p. Ser#mthyroglo'#lin meas#rements are not helpf#l diagnostically 'eca#se they are elevated in most'enign thyroid conditions.

Elevated ser#m calcitonin levels are highly s#ggestive of *T;. Ser#m calcitoninmeas#rement" which was once the mainstay in the diagnosis of 4*T;" has 'een replaced'y sensitive polymerase chain reaction (P;/! assays for germline m#tations inthe R!protooncogene. These m#tations are present in patients with *E &5" *E &6"and 4*T; (see Genetic testing for *E and 4*T; in the *ed#llary Thyroid ;arcinomasection!. 2owever" calcitonin and the more sensitive pentagastrinstim#lated calcitonin are#sed as t#mor marers to monitor patients who have 'een treated for *T;. 6eca#se of thelow incidence of *T; overall" testing of ser#m calcitonin is not a costeffective screeningtool in the primary wor#p of thyroid nod#les.

Imaging procedures

Ultrasonography is the imaging modality most commonly #sed to eval#ate thyroid disease.This noninvasive st#dy ena'les acc#rate eval#ation of the thyroid gland. 2owever" the


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#sef#lness of #ltrasonography for disting#ish 'etween malignant and 'enign nod#les islimited. Simple cysts fo#nd on sonograms are 'enign" '#t simple cysts are rarely fo#nd.;ysts are most commonly complex" with at least some solid component that co#ld potentiallyhar'or malignancy. *icrocalcifications noted on sonograms are associated with thyroidmalignancy. Ultrasonography is highly sensitive for thyroid nod#les and can depict nod#lesonly a few millimeters in siFe.

5 sonogram ordered to eval#ate a solitary nod#le often reveals additional nod#les of3#estiona'le clinical significance. The acc#racy of 456 res#lts increases whensonographic g#idance is #sed. Use of #ltrasonographyg#ided 456 can 'e #sef#l for'iopsy of small or diffic#lttopalpate thyroid nod#les as well as for 456 of nod#les inchildren. Ultrasonography can also 'e #sef#l for acc#rate meas#rement of thyroid nod#lesthat are 'eing monitored serially.

-n &1," researchers at the *ayo ;linic reported that recent dramatic increases in thediagnosis of lowris thyroid cancer in the United States are f#elled 'y the over#se of#ltrasonography.>9" $? The incidence of thyroid cancer in the United States has tripled in thepast , decades" from ,.+ per 1" in 1AC, to 11.+ per 1" in &A" maing it one of

the fastest growing diagnoses. The vast ma7ority of the thyroid t#mors 'eing detected aresmall lowris papillary thyroid cancers that are #nliely to ever progress eno#gh to ca#sesymptoms or death. That this represents overdiagnosis is s#pported 'y the o'servation thatthe death rate for these cancers has remained sta'le (.$ per 1" in 1ACA and in &A!"even with the increasing incidence.>9" $?

/adioiodine imaging can help in determining the f#nctional stat#s of a nod#le. onf#nctionalnod#les do not tae #p radiola'eled iodine1&, and appear as cold spots in the thyroid (coldnod#les!.>+? 2yperf#nctioning nod#les tae #p radioiodine and appear as hot spots (hotnod#les!. Darm nod#les appear similar to the s#rro#nding normal thyroid tiss#e. 2ot orwarm nod#les were historically tho#ght to 'e 'enign therefore" they did not re3#ire f#rthereval#ation for malignancy. 2owever" in a review of $ patients #ndergoing thyroidectomy

regardless of radioimaging findings" 5shcraft and an 2erle (1A81! fo#nd that 9% of hotnod#les har'ored malignancy.

;arcinoma cannot 'e excl#ded on the 'asis of radioiodine scans. Therefore" radioiodinescans are #s#ally not helpf#l for the ro#tine eval#ation of thyroid nod#les. -n selectsit#ations" radioiodine st#dies can 'e diagnostic ad7#ncts. Dhen res#lts of repeated 456 ofa nod#le are nondiagnostic" a radioiodine imaging can help in directing management if a hotnod#le is to 'e monitored clinically.

;T scanning and */- can 'e #sed to eval#ate softtiss#e extension of large or s#spicio#sthyroid masses into the nec" trachea" or esophag#s and to assess metastases to thecervical lymph nodes. These st#dies do not have a role in the ro#tine management of

solitary thyroid nod#les. The #se of iodinated contrast agents sho#ld 'e avoided in patientswith possi'le thyroid carcinoma 'eca#se they interfere with the postoperative #se ofradioactive iodine.

Dell=ifferentiated Thyroid ;arcinoma

Papillary carcinoma

"linical features

Papillary carcinoma is the most common thyroid malignancy" representing approximately8%. Papillary carcinoma and follic#lar carcinoma mae #p the welldifferentiated thyroidcarcinomas. Domen develop papillary cancer , times more fre3#ently than men do" and the

mean age at presentation is ,99 years.


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;ases can occ#r familially" either alone or in association with Gardner syndrome (familialadenomato#s polyposis!. 5s noted a'ove" radiation expos#re" especially d#ring childhood" isassociated with the development of papillary thyroid carcinoma. T#mors typically appearafter a latency period of a'o#t 1& years. -n addition" an increased incidence of papillarycancer is hypothesiFed among patients with 2ashimoto thyroiditis (chronic lymphocyticthyroiditis!. =espite this possi'ility" the rate of malignancy for a given nod#le in people with2ashimoto thyroiditis is similar to that of individ#als with a normal gland.

Papillary carcinoma is a slowgrowing t#mor that arises from the thyroxine (T9! andthyroglo'#linprod#cing follic#lar cells of the thyroid. The cells are TS2 sensitive and tae #piodine. They prod#ce thyroglo'#lin in response to TS2 stim#lation. This feat#re has 'othdiagnostic and therape#tic val#e for managing resid#al disease and rec#rrences afters#rgical excision (see Treatment and Prognosis 'elow!.

Pathology

0n gross pathologic examination" papillary carcinomas are whitish invasive neoplasms withilldefined margins. Under microscopy" the t#mors are #nencaps#lated neoplasms that

characteristically grow with papillae consisting of neoplastic epitheli#m overlyingfi'rovasc#lar stals. ery differentiated t#mors can have a complex ar'oriFing pattern. #cleihave an empty gro#ndglass appearance with characteristic n#clear grooves andpse#doincl#sions. *itoses are rare.

5nother histologic feat#re is the presence of psammoma 'odies" which occ#r in $% ofpapillary carcinomas. Psammoma 'odies are calcific concretions that have a circ#larlaminated appearance. They are fo#nd in the stroma of the t#mor. -n addition" many papillarycarcinomas contain areas that show a follic#lar growth pattern. 2owever" when the n#clearfeat#res in follic#lar areas are the same as those in papillary areas" the t#mor 'ehaves lie aclassic papillary carcinoma and sho#ld 'e designated as s#ch. Papillary carcinoma may 'em#lticentric" with foci present in 'oth the ipsilateral and contralateral lo'es.

Local in#asion

T#mors can grow directly thro#gh the thyroid caps#le to invade s#rro#nding str#ct#res.Growth into the trachea can occ#r" prod#cing hemoptysis. Extensive involvement can ca#seairway o'str#ction. The rec#rrent laryngeal nerves can 'ecome involved 'eca#se of theirproximity in the tracheoesophageal groove. Patients present with a hoarse" 'reathy voiceand" occasionally" dysphagia.

Regional and metastatic disease

5nother common feat#re of papillary carcinoma is its propensity to spread to the cervicallymph nodes. ;linically evident lymph node metastases are present in approximately one

third of patients at presentation. *icroscopic metastases are present in one half. The mostcommon site of lymph node involvement is in the central compartment (level +! locatedmedial to the carotid sheaths on 'oth sides" with extension from the hyoid 'one s#periorly tothe sternal notch inferiorly. The 7#g#lar lymph node chains (levels &9! are the next mostcommon sites of cervical node involvement. ymph nodes in the posterior triangle of thenec (level $! may also develop metastases. This finding has important implications on thetreatment algorithm for patients in this sit#ation (see Treatment and Prognosis 'elow and theimages 'elow!.


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5lgorithm for the management of a solitary thyroid nod#le. 456 @ fine needle aspiration 'iopsy US @ #ltrasonography.

5lgorithm for the management of malignant thyroid neoplasms. 456 @ fine needle aspiration 'iopsy I/T @ external'eam radiation therapy.

5pproximately $1% of patients develop distant metastases. =istant spread of papillarycarcinoma typically affects the l#ngs and 'one.

Follicular carcinoma

"linical features

4ollic#lar carcinoma is the second most common thyroid malignancy and represents a'o#t1% of thyroid cancers. 4ollic#lar carcinoma represents an increased portion of thyroidcancers in regions where dietary intae of iodine is low. Similar to papillary carcinoma"follic#lar carcinoma occ#rs , times more fre3#ently in women than in men. Patients withfollic#lar carcinoma are typically older than those with papillary carcinoma at presents. Themean age range at diagnosis is late in the fo#rth to sixth decades.

ie papillary carcinomas" follic#lar carcinomas arise from the follic#lar cells of the thyroid.The neoplastic cells are TS2 sensitive as well" taing #p iodine and prod#cing thyroglo'#linJa feat#re that is exploited diagnostically and therape#tically (see Postoperative radioiodinescanning and a'lation 'elow!.

Pathology


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0n gross pathology" the t#mors appear as ro#nd" encaps#lated" light 'rown neoplasms.4i'rosis" hemorrhage" and cystic changes are fo#nd in the lesions. Under microscopy" thet#mors contain neoplastic follic#lar cells" which overall can have a solid" tra'ec#lar" orfollic#lar growth pattern (that #s#ally prod#ces microfollicles!. The follic#lar cells in theset#mors do not have characteristic feat#res lie papillary carcinoma cells.

4ollic#lar carcinomas are differentiated from 'enign follic#lar adenomas 'y t#mor caps#leinvasion and:or vasc#lar invasion. 4or this reason" differentiating follic#lar adenomas fromfollic#lar carcinomas is extremely diffic#lt with 456 cytology and froFen section analysis.The t#mors are divided into minimally invasive and widely invasive lesions depending on thehistologic evidence of caps#le and vasc#lar invasion. -mm#nohistochemical staining forthyroglo'#lin and cytoeratins is nearly always positive.

Local in#asion

ocal invasion can occ#r as it does with papillary carcinoma" with the same presentingfeat#res (see ocal invasion for Papillary ;arcinoma" a'ove!.

"er#ical and distant metastasesUnlie papillary carcinoma" cervical metastases from follic#lar carcinomas are #ncommon.2owever" the rate of distant metastasis is significantly increased (approximately &%!. #ngand 'one are the most common sites.

Surgical treatment

The extent of s#rgical therapy for welldifferentiated neoplasms is controversial. Primarytreatment for papillary and follic#lar carcinoma is s#rgical excision whenever possi'le. Totalthyroidectomy has 'een the mainstay for treating welldifferentiated thyroid carcinoma. -nthis proced#re" all apparent thyroid tiss#e is s#rgically removed. *a7or complications in thisproced#re are rec#rrent laryngeal nerve in7#ry and hypoparathyroidism from inadvertent

damage or removal of the parathyroid glands. ;omplications associated with totalthyroidectomy are disc#ssed in the Techni3#e of Thyroidectomy section 'elow.

5fter total thyroidectomy" patients #ndergo radioiodine scanning to detect regional or distantmetastatic disease (see Postoperative radioiodine scanning and a'lation 'elow!" followed 'yradioa'lation of any resid#al disease fo#nd.

0ver the years" modifications to total thyroidectomy have 'een proposed in an effort tored#ce rec#rrent laryngeal nerve in7#ry and hypoparathyroidism associated with totalthyroidectomy. S#'total thyroidectomy has 'een proffered as an alternative to totalthyroidectomy. Dith s#'total thyroidectomy" a small portion of gross thyroid tiss#e oppositethe side of malignancy is left in place to minimiFe the ris of in7#ring the rec#rrent laryngealnerve and of inadvertently removing the parathyroid glands on that side. Patients #s#allyreceive postoperative radioiodine treatment in an attempt to a'late the remaining thyroidtiss#e.

Dith improved stratification of patients into prognostic gro#ps (see Prognostic factors 'elow!"some s#rgeons have proposed thyroid lo'ectomy with isthmectomy alone as definitivetreatment for patients at low ris for rec#rrent or metastatic disease. This approach remainsto 'e s#'stantiated as a feasi'le alternative to total thyroidectomy.

Using the S#rveillance" Epidemiology" and End /es#lts (SEE/! data'ase" one st#dycompared the overall s#rvival (0S! and ca#sespecific s#rvival (;SS! of &,"+$ s#'7ectswith papillary or follic#lar thyroid cancer treated with local excision" lo'ectomy" neartotalthyroidectomy" or total thyroidectomy. The 1year 0S and ;SS res#lts concl#ded that total

thyroidectomy res#lted in improved s#rvival over other techni3#es poorer o#tcomes wereassociated with age" stage T,:T9 disease" positive nodes" and t#mor siFe.>C?


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5ccording to a &A st#dy 'y 5sari et al" of &C patients with follic#lar thyroid carcinoma" the1&C patients with minim#m growth had no lymph node metastases. The a#thors state thatthyroidectomy is still recommended for all patients with follic#lar thyroid carcinoma" altho#ghpatients with widely invasive disease may need more aggressive s#rgical treatment. Patientswith minimal disease invasion have an excellent prognosis with limited need for nodals#rgery.>8?

Management o! nec"

The nec m#st 'e thoro#ghly examined for lymphatic metastases. Ultrasonography of thenec with partic#lar attention to the central compartment (level +! is an effective diagnosticapproach. 456 of s#spicio#s lymph nodes can 'e performed. ;ervical metastasesdiscovered preoperatively or intraoperatively sho#ld 'e removed 'y means of en 'loclymphatic dissection of the respective cervical compartment (selective nec dissection! whilesparing the nonlymphatic str#ct#res. Excision of single nodes" nown as 'erry picing" isinade3#ate therapy for metastatic disease. Elective nec dissection (removal of clinically'enign nec lymphatic tiss#e! in a welldifferentiated carcinoma is not indicated 'eca#sepostoperative radioiodine treatment effectively treats microscopic lymphatic metastases.

Postoperatie radioiodine scanning and ablation

6eca#se differentiated thyroid tiss#e and welldifferentiated thyroid carcinomas are TS2sensitive and 'eca#se they tae #p iodine" radioiodine preferentially targets resid#al normalor malignant tiss#e after thyroidectomy. Therefore" radioiodine can 'e given in diagnosticdoses to detect resid#al normal or neoplastic tiss#e in the 'ody and in therape#tic doses toa'late this tiss#e. 5fter thyroidectomy" #se of radioiodine scanning and a'lation has 'ecomecommonplace for diagnosing and treating resid#al thyroid tiss#e" as well as regional anddistant metastases from welldifferentiated thyroid carcinomas. Pretherape#tic iodine#ptaescanning is controversial 'eca#se of its cost and 'eca#se of concerns a'o#t1,1-ind#cedt#mor st#nning" which may decrease the effectiveness of radioiodine treatment.

5fter thyroidectomy" patients are given thyroid replacement therapy with T9 (Synthroid! ortriiodothyronine (T," ;ytomel!.1,1- or1&,- scanning is performed when the patient is in ahypothyroid state (TS2 K,$!. 5pproximately 9+ wees after thyroidectomy" hypothyroidcan 'e ind#ced 'y discontin#ing replacement (T9 for 9 wees or T, for & wees! to o'tainhigh ser#m TS2 levels. 5 diagnostic dose of1,1- or1&,- is given initially. Dhole'ody scanningis performed to detect any tiss#e taing #p radioiodine. -f any normal thyroid remnant ormetastatic disease is detected" a therape#tic dose of1,1- is administered to a'late the tiss#e.Posttreatment scanning sho#ld also 'e performed 'eca#se it may reveal metastatic diseasenot otherwise noted.

The role of recom'inant h#man TS2 (Thyrogen! in remnant a'lation contin#es to evolve.Thyrogen is approved for posts#rgical remnant a'lation in E#rope '#t not the United States.

6ar'aro et al fo#nd e3#ivalent res#lts in posts#rgical remnant a'lation when they comparedtraditional T9 withdrawal with the discontin#ation of T9 1 day 'efore TS2 stim#lation.Thyrogen stim#lation avoids the discomfort of patients having to discontin#e thyroidreplacement and is especially #sef#l in those #na'le to tolerate hypothyroidism or togenerate a high TS2 level.

-f a treatment dose of1,1- is re3#ired" diagnostic thyroid scanning is repeated while thepatient is in the hypothyroid state a'o#t + months after initial treatment. 5gain" if thediagnostic scan is positive" an additional therape#tic dose is given. This process is repeated#ntil the diagnostic scan is negative.

5 promising new development for follow#p thyroid scanning is the #se of recom'inant

h#man TS2 as opposed to withdrawing T9 to increase a#togeno#s TS2 levels. This


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approach avoids the discomfort of having to discontin#e thyroid replacement therapy forthese scans.

5 retrospective analysis of more than 1 patients with papillary thyroid cancer who#nderwent total thyroidectomies fo#nd that most patients with lowris local disease andsome with highris T, t#mors who did not receive radioiodine remnant a'lation after s#rgery

had high $year rec#rrencefree s#rvival rates. This s#ggests that physicians sho#ldcaref#lly consider whether the 'enefits o#tweigh the riss associated with radioiodineremnant a'lation when de'ating the possi'ility of employing this techni3#e in individ#alpatients.>A" 1?

#hyroid suppression

5fter thyroidectomy and radioiodine a'lation" patients with welldifferentiated thyroidcarcinoma are maintained on thyroids#ppression s#ppression. Patients tae T9 in dailydoses s#fficient to s#ppress TS2 prod#ction 'y the pit#itary. ow TS2 levels in the'loodstream red#ce t#moral growth rates and red#ce rec#rrence rates of welldifferentiatedthyroid carcinomas. The extent to which TS2 sho#ld 'e s#ppressed is controversial. *ost

a#thors recommend red#cing TS2 levels to .1 mU:. This level provides ade3#ate thyroids#ppression while avoiding deleterio#s cardiac and 'one effects of profo#nd thyroids#ppression.

Follo$-up care

Patients are reg#larly monitored every +1& months with serial radioiodine scanning andser#m thyroglo'#lin meas#rements after s#rgery and radioiodine therapy. Thyroglo'#lin is a#sef#l marer of t#mor rec#rrence 'eca#se welldifferentiated thyroid cancers synthesiFethyroglo'#lin. 2owever" it is #sef#l only after total thyroid a'lation. Ser#m thyroglo'#lin ismeas#red at the time of follow#p thyroid scanning" d#ring the withdrawal of thyroid hormoneor the administration of recom'inant TS2. Ser#m antithyroglo'#lin anti'odies are meas#redin addition to thyroglo'#lin 'eca#se their presence invalidates the assay. Thyroglo'#lin

anti'ody levels sho#ld 'e o'tained with each thyroglo'#lin meas#rement. /isingthyroglo'#lin level after thyroid a'lation s#ggests rec#rrence. Ultrasonography of the neccan also 'e #sed to detect regional rec#rrences.

Pharmacologic therapy

Sorafeni'(exavar! was approved in ovem'er &1, for differentiated thyroid cancer (=T;!that is refractory to radioactive iodine treatment. -n a st#dy of 91C patients with progressiveradioiodinerefractory =T;" treatment with sorafeni'" an orally active inhi'itor of EG4/1,and /af inases" significantly improved progressionfree s#rvival (1.8 months! comparedwith place'o ($.8 months!.>11" 1&?T#mor histology was $C% papillary" &$% follic#lar" and 1%poorly differentiated. The ma7ority of the patients (A+%! had metastatic disease" of which

C1% of the target lesions were in the l#ng" 9% in lymph nodes" and 19% in 'one.

5t the time of the report" median overall s#rvival had not yet 'een reached in either st#dyarm" and C% of place'o patients had started openla'el sorafeni'.>11" 1&?Th#s" all reportedresponses were partial) 1&.&% in the sorafeni' gro#p vs .$% in the place'o gro#p. The rateof sta'le disease for + months or longer was 9&% in the sorafeni' gro#p and ,,% in theplace'o gro#p.

Management o! recurrence

/ec#rrences are 'est treated with s#rgical excision if the disease is clinically evident ands#rgically accessi'le. onlocaliFed rec#rrences detected on the 'asis of elevatedthyroglo'#lin levels are treated with1,1-. 0n occasion" rec#rrent t#mors do not concentrate

iodine. Positron emission tomography (PET! may 'e helpf#l in localiFing disease in s#chcirc#mstances. Dhen s#rgical excision of rec#rrent disease is not feasi'le" external'eam
http://reference.medscape.com/drug/nexavar-sorafenib-342260http://reference.medscape.com/drug/nexavar-sorafenib-342260


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radiation therapy may 'e #sef#l. ;hemotherapy" #s#ally with doxor#'icin" is reserved fort#mors that do no respond to other treatments and for palliative care. /esponse rates of ,$9% are reported" tho#gh complete responses to chemotherapy are rare.

Prognostic !actors

The longterm diseasefree s#rvival with aggressive treatment and management is nearlyA% overall. 5 variety of factors are associated with prognosis" as listed 'elow.

5ge) The patientBs age at diagnosis is one of the most important prognostic feat#res

of welldifferentiated thyroid carcinoma. ;ancerrelated death is most liely to occ#r if thepatient is K9 years at the time of diagnosis. /ec#rrences are most common in patientswhose disease is diagnosed when they were L & years or K+ years.

Sex) *en are twice as liely as women to die from thyroid cancer.

SiFe) The siFe of the primary t#mor is related to s#rvival. Patients with primary

t#mors K9 cm have increased rec#rrence and cancerrelated mortality rates. 2istology) 0verall" papillary carcinoma is associated a ,year cancerrelated death

rate of +%. 4ollic#lar carcinoma has a ,year cancerrelated death rate of 1$%.

ocal invasion) -nvasion of s#rro#nding tiss#es o#tside of thyroid indicates 'iologicaggressiveness and significantly worsens the patientBs prognosis.

ymph node metastasis) ymph node metastasis does not appear to 'e as important

in the o#tcome of welldifferentiated thyroid carcinomas as in the o#tcome of most othersolid t#mors.

=istant metastasis) =istant metastasis at initial examination is associated with a +8.1

fold increase in the rate of diseasespecific death.

2


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2


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methionine s#'stit#tion in codon A18 of exon 1+. *#tations are descri'ed in exons 1, and19 in patients with 4*T;.

Genetic screening with sensitive P;/ assays for germline R!m#tations is ro#tinelyperformed in atris patients. ;hildren of parents nown to have *E or 4*T; are testedfor R!m#tations to g#ide therapy and f#t#re genetic co#nseling. -n addition" patients

presenting with sporadic *T; sho#ld #ndergoR!m#tational analysis to r#le o#t newspontaneo#s germline m#tations" which sho#ld prompt the testing of offspring for similarm#tations.

(iochemical testing !or M#%

6eca#se *T; cells prod#ce calcitonin" elevated ser#m calcitonin levels are diagnostic of*T;. 5ltho#gh ro#tine meas#rement of ser#m calcitonin has low yield in managing thesolitary thyroid nod#le 'eca#se of the #ncommon nat#re of *T;s" it is #sef#l in thes#rveillance of patients with a history of *T; and in managing familial forms. Stim#latingcalcitonin release 'y #sing intraveno#s pentagastrin increases the sensitivity of the test. 4orpentagastrinstim#lated calcitonin eval#ation" a 'aseline plasma calcitonin level is

meas#red" followed 'y the intraveno#s administration of pentagastrin .$ mg:g and serialmeas#rements of calcitonin 1.$ and $ min#tes after in7ection. Elevated 'asal or stim#latedcalcitonin levels a'ove the normal range for the la'oratory strongly s#ggest *T;.

Plasma calcitonin levels are commonly increased 'efore clinical evidence of *T; appears.5ltho#gh this finding was once the mainstay in diagnosing familial forms of *T;" res#lts ofgenetic testing have largely s#pplanted it. Plasma calcitonin testing is now #sed for the earlydetection of *T; in patients already nown to 'e at ris for *T; 'eca#se of their familyhistory and genetic res#lts. This level is most commonly #sed as a t#mor marer to identifyresid#al and metastatic disease after thyroidectomy to treat *T;.

Pathology

0n gross examination" *T;s are fairly well circ#mscri'ed" tho#gh they are #nencaps#lated.They are typically tannish pin and often contain yellow gran#lar regions" which representfocal calcification. *ost t#mors arise in the middle and #pper third of the thyroid lo'es"commens#rate with the location of the parafollic#lar ; cells in the thyroid gland. Sporadict#mors are #nilateral" and inherited forms #s#ally involve 'oth thyroid lo'es.

*T;s can have a varied microscopic appearance. The t#mors typically have a lo'#lar"tra'ec#lar" ins#lar" or sheetlie growth pattern. Some t#mors have a fi'rotic character.*alignant cells may appear ro#nd" polygonal" or spindle shaped. The cytoplasm iseosinophilic and finely gran#lar. -n the stroma" characteristic deposits of amyloid arecommonly o'served. This amyloid has typical green 'irefringence on ;ongo red staining"and this is a feat#re #ni3#e to *T; among thyroid malignancies. -mm#nohistochemical

stains for calcitonin and carcinoem'ryonic antigen are microscopically #sef#l fordifferentiating *T; from other t#mors.

5 #ni3#e feat#re to the familial cases of *T; is the finding of ;cell hyperplasia" which canhelp in disting#ishing familial cases from sporadic ones. ;cell hyperplasia is considered aprec#rsor to *T; and is #s#ally ad7acent to foci of *T;. The finding of ;cell hyperplasiawith *T; sho#ld raise the s#spicion for familial disease.

#reatment

6oth sporadic *T;s and 4*T;s are treated with total thyroidectomy and lymphaticdissection of the anterior compartment of the nec (level -!. -f the vasc#lat#re of theparathyroid gland is disr#pted" a#totransplantation of the parathyroid gland into the

sternocleidomastoid m#scle or the nondominant forearm is performed.


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*etastasis to the cervical lymph nodes is common in patients with *T;" partic#larly thosewith familial forms with m#lticentricity and 'ilaterality of the primary t#mor. ymph nodemetastases can occ#r in more than $% of patients. 6oth 'efore and at the time of s#rgery"the lateral 7#g#lar lymphatics sho#ld caref#lly 'e palpated for evidence of metastaticdisease. Selective nec dissection (sparing nonlymphatic str#ct#res when possi'le! of levels--" ---" -" and is performed when metastases are clinically evident.

Prophylactic thyroidectomy in patients $ith ME' )* and ME' )(

*T; is the most common ca#se of mortality in patients with *E &5 and *E &6" andmany patients who inherit these syndromes develop *T; in the first decade of life.Therefore" prophylactic thyroidectomy and centralcompartment lymphnode dissection is'eing performed in children with these syndromes. S#rgery is offered to patients when thediagnosis is made on the 'asis of R!m#tational analysis. ;hildren with R!m#tationswhose parents decline s#rgery sho#ld 'e monitored with ann#al meas#rement of calcitoninlevels. Thyroidectomy is performed when res#lts are a'normal.

Follo$-up care

5fter receiving treatment for *T;" patients are monitored with ann#al meas#rement ofser#m calcitonin levels for s#rveillance. Pentagastrinstim#lated calcitonin testing is nolonger widely availa'le. ;arcinoem'ryonic antigen is another t#moral marer associated withthe rec#rrence of *T;" and it may also 'e #sed for s#rveillance. Patients with elevatedlevels of calcitonin or carcinoem'ryonic antigen are eval#ated for rec#rrent disease. ec"a'dominal" and pelvic ;T or */- may 'e #sed to detect disease if metastasis or rec#rrenceis s#spected. Ultrasonography may 'e #sef#l to localiFe cervical disease. -n addition"radion#clide st#dies and selective veno#s catheteriFation with sampling of calcitonin levelscan 'e performed to localiFe rec#rrences. The role of PET is evolving.

/adiation therapy is #sed in an ad7#vant setting at some centers" and it can 'e #sed to treatpatients with s#rgically inopera'le rec#rrences and metastases. 6eca#se *T; does not

concentrate iodine" radioiodine therapy has no role in follow#p care or treatment.

5 variety of chemotherape#tic regimens have 'een #sed to treat metastatic disease. *T; isrelatively insensitive to chemotherapy" tho#gh partial responses have 'een o'tained. Todate" the most effective com'ination is dacar'aFine" vincristine" and cyclophosphamide.5dding doxor#'icin to this regimen" some investigators have gained a partial response rateof a'o#t ,$%.

andetani' (;aprelsa! and ca'oFantini' (;ometri3! are tyrosine inase inhi'itors approved'y the 4=5 for progressive" metastatic med#llary thyroid cancer. These agents target vario#styrosine inases incl#ding *ET" /ET" and EG4/&.

PrognosisThe overall prognosis for patients with *T; is worse than that of patients with welldifferentiated carcinoma. The reported 1year s#rvival rate is +$% overall. Mo#ng age" smallprimary t#mor" low stage of disease" and completeness of initial resection improve s#rvival.Patients with *E &6 have a prognosis s#'stantially worse than that of patients with *E&5" tho#gh the prognosis for 'oth gro#ps has improved with early diagnosis andintervention.

5naplastic ;arcinoma and 0ther Thyroid ;arcinomas

*naplastic #hyroid %arcinoma

"linical features


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5naplastic thyroid carcinoma is one of the least common thyroid carcinomas" acco#nting for1.+% of all thyroid cancers. 2owever" it has the most aggressive 'iologic 'ehavior of allthyroid malignancies and one of the worst s#rvival rates of all malignancies in general. iepapillary and follic#lar carcinomas" anaplastic thyroid carcinomas affect more women thanmen" with a femaletomale ratio of a'o#t &,)1. Patients with anaplastic thyroid carcinomaspresent later than those with other thyroid malignancies the former most typically present inthe sixth or seventh decade of life.>1,?

5naplastic thyroid carcinoma manifests as a rapidly growing thyroid mass in contrast to welldifferentiated carcinomas" which are comparatively slow growing. Patients commonlypresent with associated symptoms d#e to local invasion. 2oarseness and dyspnea res#ltingfrom the involvement of the rec#rrent laryngeal nerve and airway occ#r in as many as $%of patients.

Physical examination reveals a firm thyroid mass or masses that are most often larger than $cm at presentation. 5'o#t ,% of patients have vocal cord paralysis" and cervicalmetastases are palpa'le on examination in 9% of patients. 5t least one half of patientsalready have distant metastases at the time of diagnosis. The most common sites of

involvement are the l#ngs" 'ones" and 'rain.

Pathology

0n gross examination" anaplastic thyroid carcinoma is a large and invasive t#mor. 5reas offocal necrosis and hemorrhage may 'e present thro#gho#t the t#mor" giving a highlyvaria'le appearance. The t#mor often extends thro#gh the caps#le of the thyroid gland itself.5reas of welldifferentiated thyroid carcinoma are often fo#nd concomitantly" and anaplasticthyroid carcinoma is 'elieved to arise from a preexisting" welldifferentiated thyroidcarcinoma.

0n microscopic eval#ation" s3#amoid" spindle cell" and giant cell variants are o'served. 5ll ,

histologic variants show high mitotic activity" large foci of necrosis" and nota'le infiltration.-mm#nohistochemical stains are often positive for lowmolec#larweight eratins andoccasionally positive for thyroglo'#lin. /egarding their #ltrastr#ct#re" the neoplasms haveepithelial feat#res (eg" desmosomes" tight 7#nctions! that are helpf#l for differentiating themfrom sarcomas. Small cell carcinomas" which #s#ally represent lymphomas" may 'econf#sed with anaplastic thyroid carcinoma.

!reatment

The progression of disease is rapid" and most patients die from local airway o'str#ction orcomplications of p#lmonary metastases within 1 year despite all treatment efforts. Total ors#'total thyroidectomy is performed when the extent of the permits it. ec dissection isadded to manage palpa'le cervical metastases. ;omplete excision is often impossi'le

'eca#se many patients present with clinically significant local extension. Tracheal andlaryngeal resection is #s#ally not performed to remove disease 'eca#se of the poorprognosis in these circ#mstances. Tracheotomy is needed in cases with airway compromised#e to tracheal invasion. External'eam irradiation is effective in improving local control. -t isadded postoperatively or #sed as primary treatment in #nresecta'le cases. ;hemotherapy isadded for palliation. =oxor#'icin is the most commonly #sed chemotherape#tic agent.;hemotherapy and radiation therapy typically administered #sed in com'ination.

Prognosis

5naplastic thyroid carcinoma is poorly responsive to m#ltimodality therapy" and medians#rvival is 8.1 months. Mo#ng age" #nilateral t#mors" small t#mors (L $ cm!" no local invasion

of the s#rro#nding tiss#e" and a lac of cervical metastases are relatively favora'leprognostic indicators. Patients with these feat#res may have slightly prolonged s#rvival.


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ongterm s#rvival sho#ld prompt a reconsideration of the diagnosis of anaplastic thyroidcarcinoma the original t#mor is #s#ally fo#nd to 'e a variant of *T; or thyroid lymphoma.

Primary #hyroid Lymphoma

"linical features

Primary lymphomas of the thyroid gland represent approximately &$% of all thyroidmalignancies. *ost thyroid lymphomas are non2odgin 6cell t#mors. The next mostcommon histologic type is lowgrade malignant lymphoma of m#cosaassociated lymphoidtiss#e (*5T!. 2odgin lymphoma" 6#ritt cell lymphoma" and Tcell lymphoma have also'een reported.

The incidence peas in the sixth decade of life" and most diagnoses are made in patientsaged $8 years. Domen are more commonly affected than men" with a femaletomaleratio of 9)1. This t#mor is highly associated with chronic lymphocytic thyroiditis (2ashimotothyroiditis!. 5lmost all patients with primary thyroid lymphoma have either a clinical history orhistologic evidence of chronic lymphocytic thyroiditis. The ris of primary thyroid lymphomaincreases Cfold in patients with chronic lymphocytic thyroiditis compared with the generalpop#lation.

The most common clinical presentation is an enlarging thyroid mass. Patients may haveclinical or serologic evidence of hypothyroidism. ocal extension into the aerodigestive tractor s#rro#nding tiss#es may ca#se dysphagia" dyspnea" or symptoms of press#re in thenec. ocal fold paralysis and hoarseness s#ggest involvement of the rec#rrent laryngealnerve. /egional and distant lymphadenopathy is common.

=iagnosis is 'ased on the patientBs clinical presentation with a positive tiss#e diagnosis.456 may 'e #sef#l for diagnosing thyroid lymphoma" '#t it is considered less relia'le withthis lesion than with other thyroid malignancies. ymphoma may 'e diffic#lt to differentiatefrom chronic lymphocytic thyroiditis. S#rgical 'iopsy of the lesion is preferred for diagnosing

thyroid lymphoma. 6iopsy ena'les thoro#gh histochemical and imm#nohistochemicalanalysis to confirm the diagnosis. T#mor cells are positive for le#ocytecommon antigenand for 6 or Tcell marers depending on the type of t#mor.

Staging of thyroid lymphomas is important for therape#tic and prognostic p#rposes. Staginginvolves ;T scanning of the 'rain" nec" chest" a'domen" and pelvis" as well as 'onemarrow 'iopsy. *ost primary thyroid lymphomas are localiFed to the thyroid gland aloneand" therefore" classified as stage -E (localiFed to an extranodal site!. -nvolved regionallymph nodes increase the stage to --E.

!reatment and prognosis

Stage -E lymphomas may 'e treated with total thyroidectomy followed 'y postoperativeradiation therapy. S#rgical excision sho#ld not 'e performed if local infiltration intos#rro#nding tiss#es is evident. Stage --E lymphomas are treated with com'inedchemotherapy and radiation therapy. =oxor#'icin or ;20P (ie" cyclophosphamide"hydroxyda#nomycin" 0ncovin >vincristine?" prednisone! is the commonly #sedchemotherape#tic regimen.

*ost thyroid lymphomas are stage -E" which have a $year s#rvival rate of #p to 8$%.Spread 'eyond the thyroid gland red#ces the $year s#rvival rate to a'o#t ,$%. ymphomasat stages higher than this worsen the prognosis.

Sarcoma o! the #hyroid &land

Sarcomas that arise in the thyroid gland are #ncommon. They are aggressive t#mors thatmost liely arise from stromal or vasc#lar tiss#e in the gland. *alignancies that appear to 'e


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sarcomas sho#ld 'e differentiated from anaplastic thyroid carcinomas" which can appearsarcomato#s.

The treatment for thyroid sarcomas is total thyroidectomy. /adiation therapy may 'e #sed inan ad7#nctive setting. *ost sarcomas are #nresponsive to chemotherapy. /ec#rrence iscommon" as it is with sarcomas arising in other sites in the 'ody" and the patientBs overall

prognosis is poor.

Techni3#e of ThyroidectomyThyroid s#rgery is performed to diagnose or treat thyroid disease. The extent of s#rgeryranges from isthmectomy alone (for small nod#les tr#ly localiFed to the isthm#s! to s#'totalthyroidectomy" total thyroidectomy" or extended thyroidectomy. /adioiodine st#diesperformed after total thyroidectomy #s#ally show resid#al normal thyroid tiss#e despite totalthyroidectomy.

Principles of thyroid s#rgery are acc#rate exec#tion of the planned excision" avoidance ofin7#ry to the rec#rrent laryngeal nerve" avoidance of in7#ry to or devasc#lariFation of theparathyroid glands" and metic#lo#s hemostasis.

Indications

-ndications for thyroid lo'ectomy

6iopsy of a solitary thyroid nod#le s#ggestive of malignancy

*anagement of compressive or cosmetic symptoms d#e to a m#ltinod#lar

goiter *anagement of a welldifferentiated thyroid carcinoma in a lowris patient

(This is controversial.! -ndications for total thyroidectomy

*anagement of a welldifferentiated thyroid malignancy

*anagement of an *T;

*anagement of a sarcoma of the thyroid gland

*anagement of stage -Ethyroid lymphoma

*anagement of an o'str#ctive goiter (;onsider s#'total thyroidectomy.!

Preoperatie considerations

ocal fold mo'ility sho#ld always 'e determined 'efore thyroid s#rgery. -f lo'ectomy for'iopsy is planned" disc#ss the potential need for completion thyroidectomy with the patient.

+peratie technique

Positioning of the patient is important. Place the patient in a s#pine position with his or hernec extended 'y #sing a sho#lder roll. Plan a horiFontal incision in a nat#ral sin crease to

conto#r the c#rvat#re of the nec. The location sho#ld overly the thyroid gland" evenlyextending 'etween the anterior aspect of the sternocleidomastoid m#scles on 'oth sides.

Elevate sin flaps s#periorly and inferiorly in a s#'platysmal plane. (Platysma is often a'sentin the midline.! igate the anterior 7#g#lar veins only if they directly limit expos#re. Separatethe sternohyoid and sternothyroid m#scles in the median raphe" and retract them laterally toexpose the cricoid cartilage and thyroid isthm#s.

-n the anterior region" dissect the strap m#scles off the face of the thyroid lo'e ('ilaterally fortotal thyroidectomy!. S#pracaps#lar dissection is contin#ed #ntil the s#perior pole and itsvasc#lar pedicle are isolated. The s#perior pole vessels are divided and ligated. Thedissection contin#es laterally with division of the middle thyroid vein. The thyroid lo'e isgrad#ally medialiFed. ;aref#l 'l#nt dissection is performed to identify the rec#rrent laryngeal

nerve in the tracheoesophageal groove.


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5fter the rec#rrent nerve is identified" caref#lly follow the nerve s#periorly toward the larynx.The nerve passes closely to the 6erry ligament" '#t its position varies. 5fter the nerve isthoro#ghly identified in this region" divide the ligament to release the thyroid gland.

Neep the location of the parathyroid glands in mind d#ring lateral dissection. 5void dist#r'ingthe gland and vasc#lat#re as m#ch as possi'le. =issection close to the thyroid caps#le

minimiFes this ris. -f possi'le" ligate the inferior thyroid artery only after the vessels to theinferior parathyroid gland 'ranch.

-f a parathyroid gland is inadvertently removed" reimplant it in the sternocleidomastoidm#scle or on the volar s#rface of the forearm after slicing it into small pieces and maring itwith a s#rgical clip.

=#ring s#perior dissection" remem'er the near'y location of the external 'ranch of thes#perior laryngeal nerve" which innervates the cricothyroid m#scle. igation of the s#periorpole vessels tight to the thyroid in this area avoids inadvertent in7#ry to this nerve.

-f only lo'ectomy is planned" divide the thyroid isthm#s in the midline. igate the final soft

tiss#e attachments" and remove and la'el the lo'e. Send it to the pathology la'oratory.Dhen total thyroidectomy is performed" the s#rgeon may elect not to divide the thyroidisthm#s in the midline" '#t rather" to perform lateral dissection 'ilaterally. -dentify therec#rrent laryngeal nerves" and manage the inferior and s#perior vasc#lar pedicles similarly./emove the gland in 1 piece" la'el it" and send it for pathologic analysis.

-rrigate the wo#nd" and achieve metic#lo#s hemostasis. The decision to place a passive orclosed s#ction drain often depends on the s#rgeonBs 7#dgment. -f a drain is #sed" place it intothe wo#nd and 'ring it o#t laterally thro#gh the incision or thro#gh a separate sta' incision.The present a#thors have fo#nd the ro#tine #se of drains #nnecessary. /eapproximate thesternothyroid and sternohyoid" and caref#lly close the sin in layers.

Postoperatie care

-f a s#rgical drain is placed" maintain it #ntil its o#tp#t has diminished s#fficiently" #s#ally onthe first postoperative day. 2ypocalcemia may occ#r in patients who have #ndergone totalthyroidectomy. 5ssess for hypocalcemia 'y in3#iring a'o#t perioral paraesthesia. -n a patientwith hypocalcemia" tapping on prea#ric#lar region overlying the tr#n of the facial nerve mayca#se ipsilateral contraction of the face (;hovste sign!. *eas#re ioniFed calci#mpostoperatively. 2ypocalcemia may re3#ire calci#m and vitamin = s#pplementation. *anagepain with acetaminophen and narcotics as needed.

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