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Chapter 57 / Endocrine 435

What is the prognosis with

 

the following procedures:

 

Complete excision?

90% 10-year survival

Incomplete excision?

25% 10-year survival

MULTIPLE ENDOCRINE NEOPLASIA

 

 

What is it also known as?

MEN syndrome

What is it?

Inherited condition of propensity to

 

develop multiple endocrine tumors

How is it inherited?

Autosomal dominant (but with a significant

 

degree of variation in penetrance)

Which patients should be

All family members of patients diagnosed

screened for MEN?

with MEN

MEN TYPE I

 

 

 

What is the common

Wermer’s syndrome (Think: Wermer

eponym?

Winner #1 type 1)

What is the gene defect in

Chromosome 11 (Think: 11 1)

MEN type I?

 

What are the most common

“PPP”:

tumors and their incidences?

Parathyroid hyperplasia ( 90%)

Pancreatic islet cell tumors ( 66%)

Gastrinoma: ZES (50%)

Insulinoma (20%)

Pituitary tumors ( 50%)

How can tumors for MEN-I be remembered?

How can the P’s associated with MEN-I be remembered?

What percentage of patients with MEN-I have parathyroid hyperplasia?

Think: type 1 Primary, Primary, Primary PPP Parathyroid, Pancreas, Pituitary

All the P’s are followed by a vowel: PA, PA, PI

90%

436 Section II / General Surgery

 

What percentage of patients

50%

with MEN-I have a

 

gastrinoma?

 

What other tumors

Adrenal (30%) and thyroid (15%)

(in addition to PPP) are

adenomas

associated with MEN-I?

 

MEN TYPE IIa

 

 

 

What is the common

Sipple’s syndrome (Think: Sipple

eponym?

Second #2 type 2)

What is the gene defect in

RET (Think: reT Two)

MEN type IIa?

 

What are the most common

“MPH”:

tumors and their incidences?

Medullary thyroid carcinoma (100%);

 

Calcitonin secreted

 

Pheochromocytoma ( 33%);

 

Catecholamine excess

 

Hyperparathyroidism ( 50%);

 

Hypercalcemia

How can the tumors

Think: type 2 2 MPH or 2 Miles

involved with MEN-IIa be

Per Hour MPH Medullary,

remembered?

Pheochromocytoma, Hyperparathyroid

How can the P of MPH be

Followed by the consonant “H”—

remembered?

PHEOCHROMOCYTOMA (remember,

 

the P’s of MEN-I are followed by vowels)

What percentage of

100%

patients with MEN-IIa have

 

medullary carcinoma of the

 

thyroid?

 

MEN TYPE IIb

 

 

 

What are the most common

“MMMP”:

abnormalities, their

Mucosal neuromas (100%)—in the

incidences, and symptoms?

nasopharynx, oropharynx, larynx,

 

and conjunctiva

 

Medullary thyroid carcinoma ( 85%)—

 

more aggressive than in MEN-IIa

 

Marfanoid body habitus (long/lanky)

 

Pheochromocytoma ( 50%) and

 

found bilaterally

How can the features of MEN-IIb be remembered?

How can you remember that MEN-IIb is marfanoid habitus?

What is the anatomic distribution of medullary thyroid carcinoma in MEN-II?

Chapter 57 / Endocrine 437

MMMP (Think: 3M Plastics)

Think: “TO BE marfanoid” II B marfanoid

Almost always bilateral (non–MEN-II cases are almost always unilateral!)

What are the physical

Mucosal neuromas (e.g., mouth, eyes)

findings/signs of MEN-IIb?

Marfanoid body habitus

 

Pes cavus/planum (large arch of foot/

 

flatfooted)

 

Constipation

What is the most common GI complaint of patients with MEN-IIb?

What percentage of pheochromocytomas in MEN-IIa/b are bilateral?

What is the major difference between MEN-IIa and MEN-IIb?

Constipation resulting from ganglioneuromatosis of GI tract

70% (but found bilaterally in only 10% of all patients diagnosed with pheochromocytoma)

MEN-IIa parathyroid hyperplasia MEN-IIb no parathyroid hyperplasia

(and neuromas, marfanoid habitus, pes cavus [extensive arch of foot], etc.)

What type of parathyroid disease is associated with MEN-I and MEN-IIa?

What percentage of patients with Z-E syndrome have MEN-I?

Hyperplasia (treat with removal of all parathyroid tissue with autotransplant of some of the parathyroid tissue to the forearm)

25%

438 Section II / General Surgery

C h a p t e r 58 Thyroid Gland

THYROID DISEASE

ANATOMY

Identify the following

1.

Pyramidal lobe

structures:

2.

Right lobe

 

3.

Isthmus

 

4.

Left lobe

Define the arterial blood

Two arteries:

supply to the thyroid.

1.

Superior thyroid artery (first branch

 

 

 

 

of the external carotid artery)

 

 

 

2.

Inferior thyroid artery (branch of

 

 

1

 

the thyrocervical trunk) (IMA

 

 

 

artery rare)

 

 

2

 

 

Thyrocervical

trunk

 

 

Chapter 58 / Thyroid Gland 439

What is the venous drainage

Three veins:

of the thyroid?

1.

Superior thyroid vein

 

2.

Middle thyroid vein

 

3.

Inferior thyroid vein

Name the thyroid lobe

Pyramidal lobe

appendage coursing toward

 

the hyoid bone from around

 

the thyroid isthmus.

 

What percentage of patients

50%

have a pyramidal lobe?

 

What veins do you first see

Anterior jugular veins

after opening the platysma

 

muscle when performing a

 

thyroidectomy?

 

Name the lymph node

Delphian lymph node group

group around the pyramidal

 

thyroid lobe.

 

What is the thyroid isthmus?

Which ligament connects the thyroid to the trachea?

What is the IMA (not I.M.A.) artery?

What percentage of patients have an IMA artery?

Midline tissue border between the left and right thyroid lobes

Ligament of Berry

Small inferior artery to the thyroid from the aorta or innominate artery

3%

440 Section II / General Surgery

 

Name the most posterior

Tubercle of Zuckerkandl

extension of the lateral

 

thyroid lobes.

 

Which paired nerves must be carefully identified during a thyroidectomy?

Recurrent laryngeal nerves, which are found in the tracheoesophageal grooves and dive behind the cricothyroid muscle; damage to these nerves paralyzes laryngeal abductors and causes hoarseness if unilateral, and airway obstruction if bilateral

What other nerve is at risk

Superior laryngeal nerve; if damaged,

during a thyroidectomy and

patient will have a deeper and quieter

what are the symptoms?

voice (unable to hit high pitches)

What is the name of the

Urban legend has it that it was Amelita

famous opera singer

Galli-Curci, but no objective data support

whose superior laryngeal

such a claim (Ann Surg 233:588, April

nerve was injured during

2001)

thyroidectomy?

 

PHYSIOLOGY

 

 

 

What is TRH?

Thyrotropin-Releasing Hormone

 

released from the hypothalamus; causes

 

release of TSH

What is TSH?

Thyroid-Stimulating Hormone released

 

by the anterior pituitary; causes release of

 

thyroid hormone from the thyroid

What are the thyroid hormones?

What is the most active form of thyroid hormone?

What is a negative feedback loop?

What is the most common site of conversion of T4 to T3?

T3 and T4

T3

T3 and T4 feed back negatively on the anterior pituitary (causing decreased release of TSH in response to TRH)

Peripheral (e.g., liver)

 

Chapter 58 / Thyroid Gland 441

What is Synthroid®

T4

(levothyroxine): T3 or T4?

 

What is the half-life of

7 days

Synthroid® (levothyroxine)?

 

What do parafollicular cells

Calcitonin

secrete?

 

THYROID NODULE

 

 

 

What percentage of people

5%

have a thyroid nodule?

 

What is the differential

Multinodular goiter

diagnosis of a thyroid

Adenoma

nodule?

Hyperfunctioning adenoma

 

Cyst

 

Thyroiditis

 

Carcinoma/lymphoma

 

Parathyroid carcinoma

Name three types of

1. Inflammatory lesions (e.g., abscess,

nonthyroidal neck masses.

lymphadenitis)

 

2. Congenital lesions (i.e., thyroglossal

 

duct [midline], branchial cleft cyst

 

[lateral])

 

3. Malignant lesions: lymphoma,

 

metastases, squamous cell carcinoma

What studies can be used to

U/S—solid or cystic nodule

evaluate a thyroid nodule?

Fine Needle Aspirate (FNA) S

 

cytology

 

123I scintiscan—hot or cold nodule

What is the DIAGNOSTIC

FNA

test of choice for thyroid

 

nodule?

 

What is the percentage of

5%

false negative results on

 

FNA for thyroid nodule?

 

442 Section II / General Surgery

What is meant by a hot versus a cold nodule?

What are the indications for a 123I scintiscan?

What is the role of thyroid suppression of a thyroid nodule?

In evaluating a thyroid nodule, which of the following suggest thyroid carcinoma:

History?

Nodule uptake of IV 131I or 99mT Hot—Increased 123I uptake functioning/

hyperfunctioning nodule Cold—Decreased 123I uptake

nonfunctioning nodule

1.Nodule with multiple “nondiagnostic” FNAs with low TSH

2.Nodule with thyrotoxicosis and low TSH

Diagnostic and therapeutic; administration of thyroid hormone suppresses TSH secretion, and up to half of the benign thyroid nodules will disappear!

1.Neck radiation

2.Family history (thyroid cancer, MEN-II)

3.Young age (especially children)

4.Male female

Signs?

1. Single nodule

 

2.

Cold nodule

 

3.

Increased calcitonin levels

 

4.

Lymphadenopathy

 

5.

Hard, immobile nodule

Symptoms?

1. Voice change (vocal cord paralysis)

 

2.

Dysphagia

 

3.

Discomfort (in neck)

 

4.

Rapid enlargement

What is the most common

Multinodular goiter

cause of thyroid enlargement?

 

 

What are indications for

Cosmetic deformity, compressive

surgery with multinodular

symptoms, cannot rule out cancer

goiter?

 

 

What is Plummer’s disease?

Toxic multinodular goiter

 

Chapter 58 / Thyroid Gland 443

MALIGNANT THYROID NODULES

 

 

What percentage of cold

25% in adults

thyroid nodules are

 

malignant?

 

What percentage of

1%

multinodular masses are

 

malignant?

 

What is the treatment of a

Most experts would remove the nodule

patient with a history of

surgically (because of the high risk of

radiation exposure, thyroid

radiation)

nodule, and negative FNA?

 

What should be done with

Send to cytopathology

thyroid cyst aspirate?

 

THYROID CARCINOMA

 

 

 

Name the FIVE main types

1. Papillary carcinoma: 80% (Popular

of thyroid carcinoma and

Papillary)

their relative percentages.

2. Follicular carcinoma: 10%

 

3. Medullary carcinoma: 5%

 

4. Hürthle cell carcinoma: 4%

 

5. Anaplastic/undifferentiated

 

carcinoma: 1% to 2%

What are the signs/

Mass/nodule, lymphadenopathy; most are

symptoms?

euthyroid

What comprises the

FNA, thyroid U/S, TSH, calcium level,

workup?

CXR, / scintiscan 123I

What oncogenes are

Ras gene family and RET proto-oncogene

associated with thyroid

 

cancers?

 

PAPILLARY ADENOCARCINOMA

 

 

 

What is papillary

Most common thyroid cancer (Think:

carcinoma’s claim to faim?

Papillary Popular) 80% of all

 

thyroid cancers

What is the environmental

Radiation exposure

risk?

 

444 Section II / General Surgery

What is the average age?

What is the sex distribution?

What are the associated histologic findings?

Describe the route and rate of spread.

131I uptake?

What is the 10-year survival rate?

What is the treatment for:1.5 cm and no history of neck radiation exposure?

1.5 cm, bilateral, cervical node metastasis OR a history of radiation exposure?

30–40 years

Female male; 2:1

Psammoma bodies (Remember, P Psammoma Papillary)

Most spread via lymphatics (cervical adenopathy); spread occurs slowly

Good uptake

95%

Options:

1.Thyroid lobectomy and isthmectomy

2.Near-total thyroidectomy

3.Total thyroidectomy

Total thyroidectomy

What is the treatment for: Lateral palpable cervical lymph nodes?

Central?

Do positive cervical nodes affect the prognosis?

What is a “lateral aberrant thyroid” in papillary cancer?

What postoperative medication should be administered?

What is a postoperative treatment option for papillary carcinoma?

Modified neck dissection (ipsilateral)

Central neck dissection

NO!

Misnomer—it is metastatic papillary carcinoma to a lymph node

Thyroid hormone replacement, to suppress TSH

Postoperative 131I scan can locate residual tumor and distant metastasis that can be treated with ablative doses of 131I

 

Chapter 58 / Thyroid Gland 445

What is the most common

Pulmonary (lungs)

site of distant metastases?

 

What are the “P’s” of

Papillary cancer:

papillary thyroid cancer (7)?

Popular (most common)

 

Psammoma bodies

 

Palpable lymph nodes (spreads most

 

commonly by lymphatics, seen in

 

33% of patients)

 

Positive 131I uptake

 

Positive prognosis

 

Postoperative 131I scan to

 

diagnose/treat metastases

 

Pulmonary metastases

FOLLICULAR ADENOCARCINOMA

 

 

What percentage of thyroid

10%

cancers does it comprise?

 

Describe the nodule

Rubbery, encapsulated

consistency.

 

What is the route of spread?

Hematogenous, more aggressive than

 

papillary adenocarcinoma

What is the male:female

1:3

ratio?

 

131I uptake?

Good uptake

What is the overall 10-year

85%

survival rate?

 

Can the diagnosis be made

No; tissue structure is needed for a

by FNA?

diagnosis of cancer

What histologic findings

Capsular or blood vessel invasion

define malignancy in

 

follicular cancer?

 

What is the most common

Bone

site of distant metastasis?

 

What is the treatment for

Total thyroidectomy

follicular cancer?

 

446 Section II / General Surgery

What is the postoperative treatment option if malignant?

What are the 4 “F’s” of follicular cancer?

Postoperative 131I scan for diagnosis/ treatment

Follicular cancer:

Far-away metastasis (spreads hematogenously)

Female (3 to 1 ratio)

FNA . . . NOT (FNA CANNOT diagnose cancer)

Favorable prognosis

HÜRTHLE CELL THYROID CANCER

What is it?

What percentage of thyroid cancers does it comprise?

What is the cell of origin?

131I uptake?

How is the diagnosis made?

Thyroid cancer of the Hürthle cells

5%

Follicular cells

No uptake

FNA can identify cells, but malignancy can be determined only by tissue histology (like follicular cancer)

What is the route of

Lymphatic hematogenous

metastasis?

 

What is the treatment?

Total thyroidectomy

What is the 10-year survival

80%

rate?

 

MEDULLARY CARCINOMA

 

 

 

What percentage of all

5%

thyroid cancers does it

 

comprise?

 

With what other conditions

MEN type II; autosomal-dominant

is it associated?

genetic transmission

Histology?

Amyloid (aMyloid Medullary)

What does it secrete?

Calcitonin (tumor marker)

What is the appropriate stimulation test?

Describe the route of spread.

How is the diagnosis made?

131I uptake?

What is the associated genetic mutation?

What is the female/male ratio?

What is the 10-year survival rate?

What should all patients with medullary thyroid cancer also be screened for?

If medullary thyroid carcinoma and pheochromocytoma are found, which one is operated on first?

Chapter 58 / Thyroid Gland 447

Pentagastrin (causes an increase in calcitonin)

Lymphatic and hematogenous distant metastasis

FNA

Poor uptake

RET proto-oncogene

Female male; 1.5:1

80% without LN involvement

45% with LN spread

MEN II: pheochromocytoma, hyperparathyroidism

Pheochromocytoma

What is the treatment?

Total thyroidectomy and median lymph

 

node dissection

 

Modified neck dissection, if lateral

 

cervical nodes are positive

What are the “M’s” of

Medullary cancer:

medullary carcinoma?

MEN II

 

aMyloid

 

Median lymph node dissection

 

Modified neck dissection if lateral

 

nodes are positive

ANAPLASTIC CARCINOMA

 

 

 

What is it also known as?

Undifferentiated carcinoma

What is it?

Undifferentiated cancer arising in 75%

 

of previously differentiated thyroid cancers

 

(most commonly, follicular carcinoma)

448 Section II / General Surgery

 

What percentage of all

2%

thyroid cancers does it

 

comprise?

 

What is the gender preference?

What are the associated histologic findings?

131I uptake?

How is the diagnosis made?

What is the major differential diagnosis?

What is the treatment of the following disorders:

Small tumors?

Airway compromise?

What is the prognosis?

MISCELLANEOUS

Women men

Giant cells, spindle cells

Very poor uptake

FNA (large tumor)

Thyroid lymphoma (much better prognosis!)

Total thyroidectomy XRT/chemotherapy

Debulking surgery and tracheostomy, XRT/chemotherapy

Dismal, because most patients are at stage IV at presentation (3% alive at 5 years)

What laboratory value must be followed postoperatively after a thyroidectomy?

Calcium decreased secondary to parathyroid damage; during lobectomy, the parathyroids must be spared and their blood supply protected; if blood supply is compromised intraoperatively, they can be autografted into the sternocleidomastoid muscle or forearm

What is the differential

Neck hematoma (remove sutures and

diagnosis of postoperative

clot at the bedside)

dyspnea after a

Bilateral recurrent laryngeal nerve

thyroidectomy?

damage

What is a “lateral aberrant

Misnomer: It is papillary cancer of a

rest” of the thyroid?

lymph node from metastasis

 

Chapter 58 / Thyroid Gland 449

BENIGN THYROID DISEASE

 

 

 

What is the most common

Graves’ disease

cause of hyperthyroidism?

 

What is Graves’ disease?

Diffuse goiter with hyperthyroidism,

 

exophthalmos, and pretibial myxedema

What is the etiology?

Caused by circulating antibodies that

 

stimulate TSH receptors on follicular

 

cells of the thyroid and cause deregulated

 

production of thyroid hormones (i.e.,

 

hyperthyroidism)

What is the female:male

6:1

ratio?

 

What specific physical

Exophthalmos

finding is associated with

 

Graves’?

 

How is the diagnosis made?

Increased T3, T4, and anti-TSH receptor

 

antibodies, decreased TSH, global uptake

 

of 131I radionuclide

Name treatment option

1. Medical blockade: iodide,

modalities for Graves’

propranolol, propylthiouracil (PTU),

disease.

methimazole, Lugol’s solution

 

(potassium iodide)

 

2. Radioiodide ablation: most popular

 

therapy

 

3. Surgical resection (bilateral subtotal

 

thyroidectomy)

What are the possible

Suspicious nodule; if patient is

indications for surgical

noncompliant or refractory to medicines,

resection?

pregnant, a child, or if patient refuses

 

radioiodide therapy

What is the major

Hypothyroidism

complication of radioiodide

 

or surgery for Graves’

 

disease?

 

What does PTU stand for?

PropylThioUracil

450 Section II / General Surgery

 

How does PTU work?

1. Inhibits incorporation of iodine into

 

T4/T3 (by blocking peroxidase

 

oxidation of iodide to iodine)

 

2. Inhibits peripheral conversion of T4

 

to T3

How does methimazole

Inhibits incorporation of iodine into

work?

T4/T3 only (by blocking peroxidase

 

oxidation of iodide to iodine)

TOXIC MULTINODULAR GOITER

 

 

 

What is it also known as?

Plummer’s disease

What is it?

Multiple thyroid nodules with one or

 

more nodules producing thyroid hormone,

 

resulting in hyperfunctioning thyroid

 

(hyperthyroidism or a “toxic” thyroid state)

What medication may bring on hyperthyroidism with a multinodular goiter?

How is the hyperfunctioning nodule(s) localized?

What is the treatment?

Amiodarone (or any iodine-containing medication/contrast)

131I radionuclide scan

Surgically remove hyperfunctioning nodule(s) with lobectomy or near total thyroidectomy

What is Pemberton’s sign? Large goiter causes plethora of head with raising of both arms

THYROIDITIS

What are the features of ACUTE thyroiditis?

What is the cause of ACUTE thyroiditis?

What is the treatment of ACUTE thyroiditis?

Painful, swollen thyroid; fever; overlying skin erythema; dysphagia

Bacteria (usually Streptococcus or Staphylococcus), usually caused by a thyroglossal fistula or anatomic variant

Antibiotics, drainage of abscess, needle aspiration for culture; most patients need definitive surgery later to remove the fistula

What are the features of SUBACUTE thyroiditis?

What is the cause of SUBACUTE thyroiditis?

What is the treatment of SUBACUTE thyroiditis?

What is De Quervain’s thyroiditis?

How can the differences between etiologies of ACUTE and SUBACUTE thyroiditis be remembered?

What are the common causative bacteria in acute suppurative thyroiditis?

Chapter 58 / Thyroid Gland 451

Glandular swelling, tenderness, often follows URI, elevated ESR

Viral infection

Supportive: NSAIDS, steroids

Just another name for SUBACUTE thyroiditis caused by a virus (Think: De QuerVain Virus)

Alphabetically: A before S, B before V (i.e., Acute before Subacute and Bacterial before Viral and thus: Acute Bacterial and Subacute Viral)

Streptococcus or Staphylococcus

What are the two types of chronic thyroiditis?

What are the features of Hashimoto’s (chronic) thyroiditis?

What is the claim to fame of Hashimoto’s disease?

What is the etiology of Hashimoto’s disease?

What lab tests should be performed to diagnose Hashimoto’s disease?

What is the medical treatment for Hashimoto’s thyroiditis?

1.Hashimoto’s thyroiditis

2.Riedel’s thyroiditis

Firm and rubbery gland, 95% in women, lymphocyte invasion

Most common cause of hypothyroidism in the United States

Autoimmune (Think: HashimOTO AUTO; thus, Hashimoto autoimmune)

Antithyroglobulin and microsomal antibodies

Thyroid hormone replacement if hypothyroid (surgery is reserved for compressive symptoms and/or if cancer needs to be ruled out)

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