Transcript Thyroiditis

What
to operate?
When to operate?
How to operate?
 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
 The
most commonly
encountered
congenital cervical
anomalies
 Anywhere along the
migratory path of the
thyroid
 80% in juxtaposition
to the hyoid
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

Clinically: usually by observing
a 1- to 2-cm, smooth, welldefined 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.

"Sistrunk operation“
en bloc cystectomy and excision of the central
hyoid bone to minimize recurrence.

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.

 Failure
of median
thyroid anlage to
descend normally
 May
the only
thyroid tissue
present
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
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


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
 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.
 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:
 In
not palpable
thyroid hypertrophy : enlarged
and palpable
 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
Increased Hormone Synthesis
(Increased RAIU)
Release of Preformed Hormone
(Decreased RAIU)
Graves' disease (diffuse toxic goiter)
Thyroiditis—acute phase of
Hashimoto's thyroiditis, subacute
thyroiditis
Toxic multinodular goiter
Plummer's disease (toxic adenoma)
Drug induced—amiodarone, iodine
Factitious (iatrogenic) thyrotoxicosis
"Hamburger thyrotoxicosis"
Thyroid cancer
Struma ovarii
Hydatidiform mole
TSH-secreting pituitary adenoma
Thyroiditis—acute phase of
Hashimoto's thyroiditis, subacute
thyroiditis
 Etiology
of autoimmune process: not
known
 Thyroid gland diffusely and smoothly
enlarged
Divided
into
› those related to hyperthyroidism
› those specific to Graves' disease

Hyperthyroid symptoms:
• heat intolerance
• increased sweating
• hair loss
• weight loss despite
•
•
•
•
adequate caloric intake
palpitations
nervousness
fatigue
emotional lability
•tremors
•diarrhea
•women:
amenorrhea
decreased fertility
miscarriages
•children:
rapid growth
early bone maturation
•older patients:
 AF
CHF

On physical examination:
• weight loss
•
•
•
•
•
•fine tremor
facial flushing
•muscle wasting
warm and moist skin • proximal muscle group
african americans: weakness
•hyperactive tendon
darkening of skin
reflexes
tachycardia or atrial
fibrillation
widening of the
pulse pressure










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
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

 On
physical examination:
› thyroid usually diffusely
and symmetrically
enlarged
› enlarged pyramidal lobe
› may bruit or thrill and
loud venous hum in
supraclavicular space
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.

May
be treated by:
› antithyroid drugs
?
› thyroid ablation with
radioactive
› surgery
131I
?
 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
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


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

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
 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
 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
Often
a prior history of nontoxic
multinodular goiter
 Over several years, enough
thyroid nodules become
autonomous to cause
hyperthyroidism.
Usually in older people
 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 iodidecontaining drugs (jodbasedow hyperthyroidism):
 contrast media
 amiodarone
 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
Preferred
treatment: Surgery
Standard procedure: Subtotal
thyroidectomy
Remnant size not crucial
because these require thyroid
suppression to prevent
recurrence

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
 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
 Smaller
nodules:
› antithyroid medications
› RAI
 Surgery
(lobectomy and isthmectomy):
preferred for:
› young patients
› larger nodules
 Classification:
› acute
› subacute
› chronic
each associated with a distinct clinical
presentation and histology.
 Thyroid
: resistant to infection
 Often preceded by upper respiratory tract
infection or otitis media
 More common in children
 70%: Streptococcus and anaerobes

Severe neck
pain radiating
to the jaws or
ear
 fever and
chills
 Odynophagia
 Dysphonia
May Complications :
systemic sepsis
tracheal or esophageal
rupture
 jugular vein thrombosis
 laryngeal chondritis or
perichondritis
 sympathetic trunk
paralysis
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.
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.
 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
 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.
 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
 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
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.
Lymphocytic
(Hashimoto's) Thyroiditis
Riedel's Thyroiditis
The
leading cause of
hypothyroidism
Autoimmune process
More common in 30-50 yr
women (male:female ratio
1:10 to 20 )
 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
 Elevated TSH
and
 Thyroid autoantibodies
usually confirm the diagnosis
 FNAB
if:
› solitary suspicious nodule
or
› rapidly enlarging goiter
complication of Hashimoto:
Thyroid lymphoma
Rare
 Prevalence 80 times higher than
control population
A
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
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, retroorbital fibrosis and sclerosing cholangitis

 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
 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
 Mainstay
 Chief
of treatment: surgery
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
 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
Any
enlargement of
thyroid gland is
referred to as a goiter
May be
› diffuse
› uninodular
› multinodular
 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
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

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
 Euthyroids
with small diffuse goiters:
no treatment
 Euthyroids with large goiters:
exogenous hormone to reduce TSH
 Endemic goiters :
iodine administration
 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.
 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.

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.
Dysphagia
Dyspnea
Choking
Hoarseness:may malignant
involvement of the RLNs
 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
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
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.

A 23-gauge
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
After
FNAB
› benign (65%)
 cysts and colloid nodules, risk of
malignancy <3%
› suspicious (20%)
 Risk of malignancy 20%.
› malignant (5%)
› nondiagnostic (10%)
 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

US helpful for
› nonpalpable nodules
› differentiating solid from cystic
nodules
› identifying adjacent LAP
› following the size of benign
nodules diagnosed by US
 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
 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
 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
 <1%
 2%

of all malignancies
of women cancers
0.5% of men cancers
 The
most rapidly increasing cancer in
women.
•Papillary Carcinoma
•Follicular Carcinoma
•Hurthle Cell Carcinoma
•Medullary Carcinoma (MTC)
•Anaplastic Carcinoma
•Thyroid Lymphoma
•Metastatic Carcinoma
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

 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
Distant
metastasis (M)
MX = Distant metastases
cannot be assessed
M1 = No distant metastasis
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
Mostly euthyroid
Slow-growing painless mass in neck
With locally advanced invasive
disease:
› dysphagia
› dyspnea
› dysphonia
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
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
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
During thyroidectomy,
enlarged central neck
nodes should be removed
Biopsy-proven lymph
node metastases in lateral
neck : modified radical
or functional neck
10% of thyroid cancers
more commonly in iodinedeficient areas
female-to-male ratio of 3:1
mean age at presentation:50
yr
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
 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
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
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
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
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
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
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%
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
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
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
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
Established by :
› History
› Physical examination
› Raised serum calcitonin
› Raised CEA levels
› FNAB cytology
Attention to FH because 25% have familial
disease
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
If harboring pheochromocytoma:
operated on first
Total thyroidectomy: treatment of choice
because of
 high incidence of multicentricity
 more aggressive course
 the fact that 131I therapy usually is not
effective
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
 In carriers: Total thyroidectomy
after confirmation of RET mutation
› before age 6 in MEN2A
› before age 1 in MEN2B
to improves survival rates
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.
1% of all thyroid malignancies
Women more commonly affected
The majority in 7th and 8th decades
long-standing neck mass
rapid enlargement
may painful
dysphonia
dysphagia
dyspnea
Large tumor
May be fixed to surrounding
structures
May be ulcerated with areas of
necrosis
Lymph nodes usually palpable
May evidence of metastasis
Confirmed by FNAB
Incisional biopsy occasionally
needed
Isthmusectomy with or without
tracheostomy may be needed to
alleviate tracheal obstruction
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
<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
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
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
Overall 5-year survival rate: 50%
Much lower survival if
extrathyroidal disease
Thyroid : rare site of metastases
Most metastases from:
›Kidney
›Breast
›Lung
›Melanoma
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
 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. 13431408
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