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In the
Name
of God
SURGICALTHYROIDDISEASES
Sadaf AlipourSurgical OncologistAssistant ProfessorArash Women’s HospitalTehran University of Medical Sciences
Our Subject:
What to operate?When to operate?How to operate?
Surgery Goals (general) An organ has a malfunction that cannot be
treated madically An organ is getting huge sizes and the
pressure effects cannot be treated medically
An organ harbors a mass/tumor that must be excised
There is a mal-placed or developmental anomaly that needs to be excised
Developmental Abnormalities
Thyroglossal Duct Cyst and Sinus The most commonly
encountered congenital cervical anomalies
Anywhere along the migratory path of the thyroid
80% in juxtaposition to the hyoid
Thyroglossal Duct Cyst and Sinus heterotopic thyroid tissue
in 20% usually asymptomatic occasionally infected by
oral bacteria: thyroid duct sinus secondary drainage of the cyst
Accompanied by minor inflammation of surrounding skin
Diagnosis of Thyroglossal Duct Cyst Clinically: usually by observing
a 1- to 2-cm, smooth, well-defined midline neck mass that moves upward with protrusion of the tongue.
Routine thyroid imaging not necessary
Thyroid scintigraphy and ultrasound: document the presence of normal thyroid tissue in the neck.
Surgery of Thyroglossal Duct Cyst and sinus
"Sistrunk operation“
en bloc cystectomy and excision of the central hyoid bone to minimize recurrence.
!!! of Thyroglossal Duct Cyst and sinus
1% contain cancer, 85% papillary Squamous, Hürthle cell, and anaplastic cancers also
reported but rare. Medullary thyroid cancers (MTCs) are not found in
thyroglossal duct cysts. Role of total thyroidectomy controversial Surgery advised in older patients with large tumors,
particularly if with additional thyroid nodules and evidence of cyst wall invasion or lymph node metastases.
Lingual Thyroid Failure of median
thyroid anlage to descend normally
May the only thyroid tissue present
Treatment in Lingual Thyroid
Necessary only if :
1) Obstructive symptoms:• choking • dysphagia• airway obstruction• hemorrhage
2) Hypothyroidism ( frequent) Medical treatment :
• exogenous thyroid hormone or
• RAI ablation followed by hormone replacement
Surgery in Lingual Thyroid Surgical excision rarely needed If required, should be preceded by evaluation of
normal thyroid tissue in the neck to avoid inadvertently rendering the patient hypothyroid
Ectopic Thyroid Found anywhere in the central neck compartment
› esophagus› trachea› anterior mediastinum› adjacent to the aortic arch› in the aortopulmonary window› within the upper pericardium› in the interventricular septum› Often, "tongues" of thyroid tissue seen extending off the
inferior poles particularly in large goiters
!!! in Ectopic Thyroid
When thyroid tissue lateral to carotid sheath and jugular vein (termed lateral aberrant thyroid): almost always metastatic thyroid cancer in lymph nodes
Even if not apparent on exam or US, the ipsilateral thyroid lobe contains a focus of papillary thyroid cancer (PTC), which may be microscopic.
Pyramidal Lobe In 50%, the distal end of the thyroglossal duct
persists : a pyramidal lobe projecting up from the isthmus, just to the left or right of the midline.
NL: not palpable
In thyroid hypertrophy : enlarged
and palpable
Surgery Goals (general) An organ has a malfunction that cannot be
treated madically An organ is getting huge sizes and the
pressure effects cannot be treated medically
An organ harbors a mass/tumor that must be excised
There is a mal-placed or developmental anomaly that needs to be excised
HyperthyroidismIncreased Hormone Synthesis (Increased RAIU)
Release of Preformed Hormone (Decreased RAIU)
Graves' disease (diffuse toxic goiter) Thyroiditis—acute phase of Hashimoto's thyroiditis, subacute thyroiditisToxic multinodular goiter
Plummer's disease (toxic adenoma)
Drug induced—amiodarone, iodine Factitious (iatrogenic) thyrotoxicosis
"Hamburger thyrotoxicosis"
Thyroid cancer Thyroiditis—acute phase of Hashimoto's thyroiditis, subacute thyroiditisStruma ovarii
Hydatidiform mole
TSH-secreting pituitary adenoma
Diffuse Toxic Goiter
Graves’ Disease
Diffuse Toxic Goiter (Graves' Disease)
Etiology of autoimmune process: not known
Thyroid gland diffusely and smoothly enlarged
Clinical Features of Graves Disease
Divided into › those related to hyperthyroidism › those specific to Graves' disease
Clinical Features ofhyperthyroidism in Graves Disease
Hyperthyroid symptoms:• heat intolerance• increased sweating• hair loss• weight loss despite
adequate caloric intake• palpitations• nervousness• fatigue• emotional lability
• tremors• diarrhea• women:
amenorrheadecreased fertilitymiscarriages
• children:rapid growthearly bone maturation
• older patients: AFCHF
Clinical Features of Hyperthyroidism in Graves Disease
On physical examination:
• weight loss• facial flushing • warm and moist skin • african americans:
darkening of skin• tachycardia or atrial
fibrillation • widening of the
pulse pressure
• fine tremor• muscle wasting• proximal muscle group
weakness• hyperactive tendon reflexes
Clinical Features Specific to Graves Disease
50% : ophthalmopathy spasm of the upper eyelid revealing the sclera above the
corneoscleral limbus (Dalrymple's sign) prominent stare, due to catecholamine excess lid lag (von Graefe's sign) periorbital edema conjunctival swelling and congestion (chemosis) keratitis proptosis limitation of upward and lateral gaze (from involvement of
the inferior and medial rectus muscles, respectively) blindness due to optic nerve involvement
Clinical Features Specific to Graves Disease
1-2%: dermopathy : deposition of glycosaminoglycans leading to thickened skin in pretibial region and dorsum of the foot
Gynecomastia common in young men Rare subperiosteal bone formation and swelling in
metacarpals (thyroid acropachy) Onycholysis, or separation of fingernails from their
beds
Clinical Features of Graves Disease
On physical examination:› thyroid usually diffusely
and symmetrically enlarged
›enlarged pyramidal lobe
›may bruit or thrill and loud venous hum in supraclavicular space
Diagnostic Tests for Graves’ disease Suppressed TSH Elevated /NL f T4 or T3 or f T3 If eye signs present, other tests generally not needed. If no eye findings: RAIU scan necessary Confirms diagnosis: elevated uptake and diffusely
enlarged gland Elevated Anti-Tg and anti-TPO in75%, non-specific Elevated TSH-R or thyroid-stimulating Abs (TSAb):in
90% :diagnostic MRI of orbits: useful in evaluating ophthalmopathy.
Treatment
May be treated by:›antithyroid drugs› thyroid ablation with radioactive 131I
›surgery
?
??
Antithyroid Drugs Generally used in preparation for RAI
ablation or surgery Drugs commonly used:
› propylthiouracil (PTU, 100 to 300 mg three times daily)
› methimazole (10 to 30 mg three times daily, then once daily)
Methimazole has a longer half-life and can be dosed once daily
RAI for Treatment of Graves’ Disease
The mainstay of Graves' disease treatment in North America.
Most often used in:› older patients with small or moderate-sized goiters
› relapse after medical or surgical therapy
› Contraindication of antithyroid drugs or surgery
Absolute contraindications to RAI:› pregnant or breastfeeding women
Relative contraindications :› young patients (i.e., especially children and adolescents)
› thyroid nodules› ophthalmopathy
RAI for Treatment of Graves’ Disease The major advantages:
› no surgery› reduced overall treatment costs› ease of treatment
Antithyroid drugs given until euthyroid Only 50% euthyroid 6 months after treatment The remaining still hyperthyroid or already hypothyroid After 1 year, 2.5% develop hypothyroidism each year The higher the initial dose of 131I, the earlier the onset and
the higher the incidence of hypothyroidism
Surgical Treatment of Graves’ Disease
Indications:(a) confirmed cancer or suspicious thyroid nodules
(b) young people
(c) pregnant or desire to conceive soon after treatment
(d) severe reactions to antithyroid medications
(e) large goiters causing compressive symptoms
(f) reluctant to RAI therapy Relative indications:
› Moderate to severe Graves' ophthalmopathy particularly in smokers
› desiring rapid control of hyperthyroidism with a chance of being euthyroid,
› poor compliance to antithyroid medications
Type of Surgery in Graves’Disease
total or near-total thyroidectomy› cancer› refuse RAI therapy › severe ophthalmopathy › life-threatening reactions to antithyroid
medications (vasculitis, agranulocytosis, or liver failure)
Subtotal thyroidectomy (leaving a 4- to 7-g remnant)› all remaining patients
Surgical Treatment of Graves’ Disease
Antithyroid drugs given up to the day of surgery to be euthyroid
Generally Lugol's iodide solution beginning 7 to 10 days preoperatively (three drops twice daily) to reduce vascularity and risk of precipitating thyroid storm
Toxic
Multinodular
Goiter
Toxic Multinodular Goiter Often a prior history of nontoxic
multinodular goiter Over several years, enough
thyroid nodules become autonomous to cause hyperthyroidism.
Usually in older people
Presentation in Toxic MNG
Symptoms and signs of hyperthyroidism similar to Graves’, extrathyroidal manifestations absent
Presentation of hyperthyroidism often insidious May hyperthyroidism only apparent when on low
dose thyroid hormone suppression for goiter May hyperthyroidism precipitated by iodide-
containing drugs (jodbasedow hyperthyroidism): contrast media amiodarone
Diagnostic Studies in Toxic MNG Blood tests are similar
to Graves:› Suppressed TSH
› Elevated free T4 or T3
RAIU increased: multiple nodules with increased uptake and suppression of the remaining gland
Treatment of Toxic MNG
Preferred treatment: Surgery Standard procedure: Subtotal
thyroidectomy Remnant size not crucial
because these require thyroid suppression to prevent recurrence
Treatment of Toxic MNG RAI reserved for :
Elderly with very poor operative risks if› no airway compression from the goiter
and› thyroid cancer not a concern.
Uptake is less than in Graves' disease: larger doses of RAI needed
RAI-induced thyroiditis may cause swelling and acute airway compromise, and leaves the goiter intact, with the possibility of recurrent hyperthyroidism
Toxic Adenoma
(Plummer's Disease)
Toxic Adenoma
RAIU: "hot" nodule with suppression of rest of thyroid
Recent growth of a long-standing nodule Hyperthyroidism from a single
hyperfunctioning nodule Symptoms of hyperthyroidism, typically in
younger patients Physical examination : solitary thyroid nodule
without palpable thyroid tissue on contralateral side
Treatment of Toxic Adenoma
Smaller nodules: › antithyroid medications › RAI
Surgery (lobectomy and isthmectomy): preferred for:› young patients› larger nodules
Thyroiditis
Thyroiditis
Classification:› acute› subacute› chronic
each associated with a distinct clinical presentation and histology.
Acute (Suppurative) Thyroiditis
Acute (Suppurative) Thyroiditis
Thyroid : resistant to infection Often preceded by upper respiratory tract
infection or otitis media More common in children 70%: Streptococcus and anaerobes
Presentation of Acute (Suppurative) Thyroiditis
Severe neck pain radiating to the jaws or ear
fever and chills
Odynophagia Dysphonia
May Complications :systemic sepsistracheal or esophageal rupture jugular vein thrombosis laryngeal chondritis or perichondritis sympathetic trunk paralysis
Diagnosis of Acute (Suppurative) Thyroiditis
Leukocytosis on blood tests FNAB for Gram's stain, culture, and cytology CT scans: delineate the extent of infection
A persistent pyriform sinus fistula should always be suspected in children with recurrent acute thyroiditis. A barium swallow demonstrates the anomalous tract with 80% sensitivity.
Treatment of Acute (Suppurative) Thyroiditis
Parenteral antibiotics Drainage of abscesses If pyriform sinus fistulae
› Complete resection of the sinus tract, including the area of the thyroid where the tract terminates, to prevent recurrence.
Painful Subacute Thyroiditis
Painful Subacute Thyroiditis
Exact etiology unknown, may be viral Most commonly in 30- to 40-yr women Sudden or gradual onset of neck pain, may
radiate toward mandible or ear Often history of preceding upper respiratory
tract infection Thyroid enlarged, exquisitely tender, and firm
Presentation of Painful Subacute Thyroiditis
Classically progresses through 4 stages:1- an initial hyperthyroid phase, due to
release of thyroid hormone
2- followed by a euthyroid phase
3- hypothyroidism which occurs in 20 to 30%
4- resolution and return to euthyroid state in >90%
A few patients develop recurrent disease.
Treatment of Painful SubacuteThyroiditis Primarily symptomatic.
› Aspirin and other NSAIDs for pain relief› Steroids may indicated in more severe cases› Short-term thyroid replacement may needed
Thyroidectomy reserved for the rare patient who has a prolonged course not responsive to medical measures or for recurrent disease
Painless Subacute Thyroiditis May be autoimmune may occur:
› sporadically
or› in postpartum period
at about 6 weeks after delivery in women with high TPO antibody titers in early pregnancy
Presentation of Painless Subacute Thyroiditis
Usually 30- 60 yr woman. Physical examination : normal sized or minimally
enlarged, slightly firm, nontender gland Clinical course parallels painful thyroiditis If symptomatic: may require beta blockers and thyroid
hormone replacement
Thyroidectomy or RAI ablation only indicated for the rare patient with recurrent, disabling episodes of thyroiditis.
Chronic Thyroiditis
Chronic Thyroiditis
Lymphocytic (Hashimoto's) Thyroiditis
Riedel's Thyroiditis
Hashimoto’s Thyroiditis
Hashimoto‘s Thyroiditis
The leading cause of hypothyroidism
Autoimmune process More common in 30-50 yr
women (male:female ratio 1:10 to 20 )
Clinical Presentation of Hashimoto
The most common presentation: mildly enlarged firm granular gland
or painless anterior neck mass
20%: hypothyroidism 5% : hyperthyroidism (Hashitoxicosis) physical examination: diffusely enlarged,
firm , lobulated gland enlarged pyramidal lobe often palpable
Diagnosis of Hashimoto's
Elevated TSH
and Thyroid autoantibodies
usually confirm the diagnosis FNAB if:
› solitary suspicious nodule
or› rapidly enlarging goiter
!!! of Hashimoto's
A complication of Hashimoto: Thyroid lymphoma
Rare Prevalence 80 times higher than
control population
Treatment of Hashimoto
In overtly hypothyroid patients:
thyroid hormone replacement, with a goal of maintaining normal TSH levels
Surgery occasionally indicated for:› suspicion of malignancy› goiters causing compressive symptoms› cosmetic deformity
Riedel's thyroiditis
Riedel's thyroiditis Rare = Riedel's struma or invasive fibrous thyroiditis Replacement of all or part of the thyroid
parenchyma by fibrous tissue, also invades into adjacent tissues
Etiology controversial, may be autoimmune Associated with other focal sclerosing
syndromes: mediastinal, retroperitoneal, periorbital, retro-orbital fibrosis and sclerosing cholangitis
Presentation in Riedel's Thyroiditis
In 30-60 yr Painless hard anterior neck mass, which progresses
over weeks to years Symptoms of compression:
› dysphagia› dyspnea› choking› hoarseness
May symptoms of hypothyroidism and hypopara as the gland is replaced by fibrous tissue
Diagnosis in Riedel's Thyroiditis
Hard, "woody" thyroid gland with fixation to surrounding tissues
Diagnosis needs to be confirmed by open thyroid biopsy, because the firm and fibrous nature of the gland renders FNAB inadequate
Treatment in Riedel's thyroiditis
Mainstay of treatment: surgery Chief goal of operation: decompression of
trachea by wedge excision of thyroid isthmus and make a tissue diagnosis
More extensive resections not advised due to infiltrative nature of fibrotic process
If hypothyroid: treated with thyroid hormone replacement
Surgery Goals (general) An organ has a malfunction that cannot be
treated madically An organ is getting huge sizes and the
pressure effects cannot be treated medically
An organ harbors a mass/tumor that must be excised
There is a mal-placed or developmental anomaly that needs to be excised
GOITER
Goiter
Any enlargement of thyroid gland is referred to as a goiter
May be › diffuse› uninodular› multinodular
Goiter - Clinical Features(1)
Mostly asymptomatic But often pressure sensation in neck Having to clear their throats frequently
(catarrh). As the goiters become very large:
compressive symptoms such as dyspnea and dysphagia
Dysphonia from RLN injury rare, except if cancerous
Goiter - Clinical Features(2)
Obstruction of venous return at the thoracic inlet from a substernal goiter results in a positive Pemberton's sign—facial flushing and dilatation of cervical veins upon raising the arms above the head
Sudden enlargement of nodules or cysts due to hemorrhage may cause acute pain.
Physical examination may reveal a soft, diffusely enlarged gland (simple goiter) or nodules of various size and consistency in case of a multinodular goiter.
Deviation or compression of the trachea may be apparent
Goiter -Diagnostic Tests Usually euthyroid: normal TSH and low-normal or
normal f T4 If some nodules develop autonomy: suppressed TSH or
hyperthyroid RAI scan: often patchy uptake with areas of hot and
cold nodules FNAB recommended in dominant nodule or one that is
painful or enlarging
!!! Carcinomas reported in 5 to 10% multinodular goiters
CT scans: evaluate extent of retrosternal extension and airway compression
Treatment of Goiter
Euthyroids with small diffuse goiters:
no treatment Euthyroids with large goiters:
exogenous hormone to reduce TSH Endemic goiters :
iodine administration
Surgical Treatment of Goiter
Surgical resection for goiters that:› continue to increase despite T4 suppression› cause obstructive symptoms› have substernal extension› have malignancy suspected or proven by FNAB› are cosmetically unacceptable
Subtotal thyroidectomy is the treatment of choice
Patients require lifelong T4 therapy to prevent recurrence.
Solitary Thyroid Nodule
Solitary Thyroid Nodule
Present in approximately 4% of people in USA
But thyroid cancer has a much lower incidence (40 new cases per 1 million)
Therefore, it is of utmost importance to determine which patients with solitary thyroid nodule would benefit from surgery.
Approach to Solitary Thyroid Nodule
Ask about: Time of onset Change in size Pain: unusual, raise
suspicion for Intrathyroidal
hemorrhage in a benign nodule
Thyroiditis Malignancy
History :• Exposure to ionizing
radiation• Family history of
thyroid and other malignancies associated with thyroid cancer.
DysphagiaDyspneaChokingHoarseness:may malignant
involvement of the RLNs
Physical Examination in STN
Best palpated from behind patient with neck in mild extension
The cricoid cartilage is an important landmark, as the isthmus is situated just below it
Nodules that are hard, or fixed to surrounding structures such as the trachea or strap muscles are more likely to be malignant
The cervical chain of lymph nodes should be assessed as well as the nodes in the posterior triangle
Diagnostic Approach in STN
FNAB has become the single most important test in the evaluation of thyroid masses
Can be performed with or without ultrasound guidance
Ultrasound guidance recommended for:› nodules difficult to palpate › cystic or solid-cystic nodules that recur after
aspiration
FNA in STN A 23-gauge needle is inserted into the thyroid
mass, and several passes are made while aspirating the syringe.
After releasing the suction on the syringe, the needle is withdrawn and the cells are immediately placed on prelabeled dry glass slides.
Some are immersed in a 70% alcohol solution and others are air dried.
FNA of STN
If a bloody aspirate is obtained, the patient should be repositioned in a more upright position and the biopsy repeated with a finer (25- to 30-gauge) needle
FNA of STN
After FNAB› benign (65%)cysts and colloid nodules, risk of malignancy <3%
› suspicious (20%)Risk of malignancy 20%.
› malignant (5%)› nondiagnostic (10%)
FNA of STN FNAB less reliable in history of head and
neck RT or FH of thyroid cancer, due to higher likelihood of multifocal lesions and occult cancer
If hyperthyroid, risk of malignancy 1% False-positive: 1% False-negative: 3%
Solitary thyroid nodule
FNA-B
Imaging in STN US helpful for
›nonpalpable nodules›differentiating solid from cystic
nodules› identifying adjacent LAP › following the size of benign
nodules diagnosed by US
Surgery of STN
When not malignant or suspicious, lobectomy if :› cyst persists after three aspirations › cysts >4 cm› complex cysts with solid and cystic
components› nodule enlarges on TSH suppression› compressive symptoms› cosmetic reasons
Surgery of STN
Exception :total or near-total thyroidectomy if› previous thyroid RT › family history of thyroid cancer
because high incidence of cancer
and decreased reliability of FNAB
Surgery Goals (general) An organ has a malfunction that cannot be
treated madically An organ is getting huge sizes and the
pressure effects cannot be treated medically
An organ harbors a mass/tumor that must be excised
There is a mal-placed or developmental anomaly that needs to be excised
THYROID
CANCER
Malignant Thyroid Disease
<1% of all malignancies
2% of women cancers
0.5% of men cancers
The most rapidly increasing cancer in women.
Specific Tumor Types
•Papillary Carcinoma •Follicular Carcinoma•Hurthle Cell Carcinoma •Medullary Carcinoma (MTC)•Anaplastic Carcinoma •Thyroid Lymphoma •Metastatic Carcinoma
Staging -1 Primary tumor (T) TX = Primary tumor cannot be assessed T0 = No evidence of primary tumor T1 = Tumor ≤2 cm in diameter, limited to thyroid T2 = Tumor >2 cm but <4 cm in diameter, limited to thyroid T3 = Tumor >4 cm in diameter, limited to thyroid, or any tumor with
minimal extrathyroidal invasion T4a = Any size tumor extending beyond capsule to invade
subcutaneous soft tissue, larynx, trachea, esophagus, or recurrent laryngeal nerve, or intrathyroidal anaplastic cancer
T4b = Tumor invading prevertebral fascia, or encasing carotid artery or mediastinal vessels; or extrathyroidal anaplastic cancer
Staging - 2 Regional lymph nodes (N) : NX = cannot be assessed N0 = No LN metastasis N1 = Regional LN metastasis
› N1a = Metastases to level VI (pretracheal, paratracheal, and prelaryngeal/Delphian lymph nodes)
› N1b = Metastases to unilateral, bilateral, or contralateral cervical or superior mediastinal lymph nodes
Staging - 3
Distant metastasis (M) MX = Distant metastases
cannot be assessed M1 = No distant metastasis
Papillary Thyroid Cancer
Papillary Tyroid Carcinoma 80% of all thyroid cancers The predominant thyroid cancer in
children and individuals exposed to external radiation
2:1 female-to-male ratio Mean age at presentation: 30 to 40
yr
Presentation of Papi l lary Tyroid Carcinoma
Mostly euthyroid Slow-growing painless mass in neck With locally advanced invasive
disease:› dysphagia› dyspnea› dysphonia
Spread of Papillary Tyroid Carcinoma
Lymph node metastases common, especially in children and young
Distant metastases uncommon initially, but may ultimately develop in 20%
The most common metastatic sites:› Lungs› Bone› Liver› Brain
Diagnosis and Prognosis of PTC
Diagnosis by FNAB of thyroid mass or lymph node
Once diagnosed on FNAB, neck US for evaluation of contralateral lobe and lymph node
excellent prognosis >95% 10-year survival rate
Surgical Treatment of PTC - 1
Total or near-total thyroidectomy When PTC diagnosed by FNAB,definive
surgery without frozen When probable PTC: thyroid lobectomy – if
PTC confirmed in final histology:completion thyroidectomy
If minimal PTCs (<1 cm) confined to thyroid without angioinvasion: no further med surgery needed
Surgical Treatment of PTC - 2
During thyroidectomy, enlarged central neck nodes should be removed
Biopsy-proven lymph node metastases in lateral neck : modified radical or functional neck
Follicular Thyroid
Carcinoma
Follicular Carcinoma - 1
10% of thyroid cancers more commonly in iodine-
deficient areasfemale-to-male ratio of 3:1mean age at presentation:50 yr
Presentation of Follicular Carcinoma
Usually present as solitary thyroid nodules Occasionally history of rapid size increase History of long-standing goiter Pain uncommon, unless hemorrhage Unlike papillary cancers, cervical LAP
uncommon initially (about 5%), although distant metastases may be present
In <1% hyperfunctioning, leading to thyrotoxicosis
Diagnosis of Follicular Carcinoma
FNAB unable to distinguish benign from carcinomas
Preoperative diagnosis of cancer difficult unless distant metastases present
Large follicular tumors (>4 cm) in older men: more likely to be malignant
Surgery of Follicular Carcinoma
When follicular lesion diagnosed by FNAB: lobectomy because at least 80% benign adenomas
Some recommend total thyroidectomy in older patients with follicular lesions >4 cm (50%risk of cancer).
Intraoperative frozen-section not helpful
Total thyroidectomy should be performed when thyroid cancer is diagnosed
No prophylactic nodal dissection because nodal involvement infrequent
If nodal metastases: therapeutic neck dissection
Prognosis of Follicular Carcinoma
Cumulative mortality :15% at 10 years and 30% at 20 years
Poor long-term prognosis predicted by:› age over 50 years old at presentation› tumor size >4 cm› higher tumor grade› marked vascular invasion› extrathyroidal invasion› distant metastases at time of diagnosis
Hürthle Cell Carcinoma
Hürthle Cell Carcinoma 3% of all thyroid malignancies Considered a subtype of follicular thyroid cancer Not diagnosed by FNAB More often multifocal and bilateral (30%) Do not take up RAI More likely to metastasize to local nodes (25%)
and distant sites Higher mortality rate (20% at 10 yr) Surgical rules similar to follicular neoplasms
Postoperative Management of Differentiated Thyroid Cancer
Thyroid Hormone T4 is necessary as:
› replacement therapy after total or near-total thyroidectomy
› suppression of TSH and reducing the growth stimulus for any possible residual thyroid cancer cells
TSH maintained at about 0.1 in low-risk and <0.1 in high-risk patients
Radioiodine Therapy Screening and treatment facilitated by
removal of all normal thyroid tissue, which effectively competes for iodine uptake
Metastatic differentiated thyroid cancer detected and treated by 131I in 75%
Early detection very important to improve prognosis
Radioiodine Therapy
T4 therapy discontinued weeks before scanning with 131I
T3 during this time period
T3 discontinued 2 weeks to allow TSH to rise
The usual protocol : a screening dose of 1 to 3 mCi of 123I and measuring uptake 24 hours later. After a total thyroidectomy, this value should be <1%
Follow-Up of Patients with Differentiated Thyroid Cancer
Thyroglobulin Measurement Tg: <2 ng/mL in total thyroidectomy under
T4therapy , and <5 when hypothyroid
Tg >2: metastatis or persistent normal thyroid tissue
Tg and anti-Tg antibody : measured initially at 6-month intervals and then annually
Medullary Thyroid
Carcinoma
MTC - 1 5% of thyroid malignancies Arises from the parafollicular or C cells C cells secrete calcitonin Concentrated superolaterally in thyroid lobes Most sporadical 25% with inherited syndromes ( familial MTC,
MEN2A, and MEN2B) All the inheriteds secondary to mutations in the
RET proto-oncogene
Syndrome Manifestations
MEN2A MTC, pheochromocytoma, primary hyperparathyroidism, lichen planus amyloidosis
MEN2B MTC, pheochromocytoma, Marfanoid habitus, mucocutaneous ganglioneuromatosis
Familial MTC MTC
MTC -2
Neck mass May palpable cervical LAP (15 to 20%) Pain or aching more common Local invasion may produce:
› dysphagia
› dyspnea
› dysphonia
Distant blood-borne metastases to the liver, bone (frequently osteoblastic), and lung occur later
MTC - 3 Female-to-male ratio is 1.5:1 50-60 yr Familial cases : younger age In extensive metastatic disease: diarrhea frequent 2 to 4% develop Cushing's syndrome as a result of
ectopic ACTH production Unilateral (80%) in sporadic disease Multicentric in familial cases Bilateral tumors in up to 90% of familial patients
Diagnosis of MTC
Established by :› History
› Physical examination
› Raised serum calcitonin
› Raised CEA levels
› FNAB cytology
Attention to FH because 25% have familial disease
Diagnosis of MTC
Because not possible to distinguish sporadic from familial disease at initial presentation, all new patients with MTC should be screened for:
› RET point mutations
›Pheochromocytoma
›HPT Calcitonin and CEA: identify patients with
persistent or recurrent MTC
Surgery in MTC
If harboring pheochromocytoma: operated on first
Total thyroidectomy: treatment of choice because of
high incidence of multicentricitymore aggressive coursethe fact that 131I therapy usually is not effective
Surgery in MTC
In locally recurrent or metastatic disease, tumor debulking to:
› ameliorate symptoms of flushing and diarrhea
›decrease risk of death from recurrent central neck or mediastinal disease
MTC Treatment
In carriers: Total thyroidectomy after confirmation of RET mutation› before age 6 in MEN2A
›before age 1 in MEN2B
to improves survival rates
Postop Follow-Up and Prognosis of MTC
Follow up: Annual measurements of calcitonin and CEA levels
10-year survival rate 80% but decreases to 45%
with lymph node involvement
Prognosis best in non-MEN familial MTC and worst in MEN2B.
Anaplastic Carcinoma
Anaplastic Carcinoma
1% of all thyroid malignancies
Women more commonly affected
The majority in 7th and 8th decades
Symptoms in Anaplastic Carcinoma
long-standing neck mass rapid enlargement may painful dysphonia dysphagia dyspnea
Signs in Anaplastic Carcinoma
Large tumor May be fixed to surrounding
structures May be ulcerated with areas of
necrosis Lymph nodes usually palpable May evidence of metastasis
Diagnosis of Anaplastic Carcinoma
Confirmed by FNAB Incisional biopsy occasionally
needed Isthmusectomy with or without
tracheostomy may be needed to alleviate tracheal obstruction
Treatment and Prognosis of Anaplastic Carcinoma
One of the most aggressive thyroid malignancies Few patients surviving 6 months beyond diagnosis All forms of treatment :disappointing If presentation as resectable mass: may small
improvement in survival with thyroidectomy, especially in younger individuals
Combined adjuvant RT and ChT in resectable disease: prolonged survival
Tracheostomy may needed for airway obstruction
Thyroid Lymphoma
Thyroid Lymphoma
<1% of thyroid malignancies Most: non-Hodgkin's B-cell type May part of a generalized lymphomatous
condition, but most develop in chronic lymphocytic thyroiditis
Usually symptoms similar to anaplastic carcinoma, although often painless
May presentation with acute respiratory distress
Diagnosis of Thyroid Lymphoma
Usually suggested by FNAB
Core needle or open biopsy may necessary for definitive diagnosis
Staging studies should be obtained to assess extent of extrathyroidal spread
Treatment of Thyroid Lymphoma
Rapid response to chemotherapy (CHOP—cyclophosphamide, doxorubicin, vincristine, and
prednisone), with improved survival Combined treatment with RT and ChT often
recommended Thyroidectomy and nodal resection for
alleviation of airway obstruction if:› no quick response to above regimens
› those who have completed the regimen before diagnosis
Prognosis of Thyroid Lymphoma
Overall 5-year survival rate: 50%
Much lower survival if extrathyroidal disease
Metastatic Carcinoma
Metastatic Carcinoma
Thyroid : rare site of metastases Most metastases from:
›Kidney
›Breast
›Lung
›Melanoma
Metastatic Carcinoma Often suggest the source of the metastatic
disease:› clinical examination
› review of the patient's history
Definitive diagnosis: usually by FNAB
Resection of the thyroid, usually lobectomy, may be helpful in many patients, depending on the status of their primary tumor.
Papillary Follicular Hurthle Medullary Anaplastic Lymphoma
Percent 80% 10% 3% 5% 1% 1%
Age 30-40 50 // 50-60, <Fa 60-80
Sex F/M=2/1 F/M=3/1 // F/M=1.5/1 F > M
Pain No No // Yes Yes No
LN Yes No Yes Yes Yes Yes
Metastasis No Yes Yes Yes Yes Yes...
Diagnosis FNA Fna-Sx // FNA,Calcit FNA FNA/Bx
Treatment Total Thy Lob-To Thy Lob-To Thy Total Thy Isth,Trach ChT
Prognosis Very good good Fair Fair-good Very poor Poor-good
multifocal No No Yes Yes,Fam ? ?
Points Child,RT Subtype Fol 25%fam,Yo B Non Ho
References:
Lal G, Clark OH.Thyroid, parathyroid, and adrenal. In: Brunicardi FC AD, Billiar TR, Dunn DL, Hunter JG, Matthews JB, Pollock RE, editor. Schwartz’s Principles of Surgery. 9th ed. USA: McGraw-Hill; 2010. p. 1343-1408
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