Endocrine Flashcards

1
Q

Complications of adrenalectomy

A

IVC injury
devascularized kidney -> HTN
Nerve injury can mimic a hernia

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2
Q

Clinical finding in ectopic ACTH secretion

A

Bronze skin

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3
Q

Top 5 ectopic ACTH secreting tumors

A
SSC of Lung
PNET
Thymic neuroendocrine tumor
Pheo
MTC
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4
Q

What is size cutoff to safely do a MIS adrenalectomy?

A

< 6cm

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5
Q

What are perioperative medications for a bilateral adrenalectomy?

A

preop steroids

post-op steroids with mineralicosteroids (hydrocortisone + Florinef)

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6
Q

4 benefits of Radioactive iodine

A

Destroy microscopic foci of disease (adjuvant therapy)

Minimize the risk of development of de-novo papillary thyroid cancers in at-risk patients

Improve the specificity of thyroglobulin (Tg) as a tumor marker

Increase the specificity of 131-I scanning for detection of recurrent or metastatic disease

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7
Q

Who should not get radioactive iodine?

A

No high risk features (LVI, histology)

●Unifocal cancer <1 cm even with less than five lymph nodes measuring less than 2 mm)

●Multifocal cancer when all foci are <1 cm

●Intrathyroidal cancer <4 cm

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8
Q

Who should definitely get radioactive iodine

A

any gross extrathyroidal extension (T4)

any distant metastatic disease.

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9
Q

Who is intermediate risk who should consider getting RAI?

A

Tumors > 4cm
microscopic extrathyroidal extension
central and lateral LN involvement

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10
Q

contraindication to RAI

A

pregnancy and breastfeeding

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11
Q

preparations for RAI

A

total thyroidectomy
stop synthroid 3-4 weeks before
Must wait 1-3 months from last contrasted CT scan
low iodine diet for 1 week

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12
Q

alternative to stopping sythroid before RAI?

A

give patient recombinant TSH during therapy

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13
Q

RAI dose for genetic risk

A

(remnant ablation), 30 mCi

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14
Q

RAI dose for adjuvant therapy

A

~100 mCi are used

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15
Q

RAI dose for metastatic disease

A

100 to 200 mCi

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16
Q

Can you do RAI on dialysis?

A

yes

standard radioiodine dose followed by more frequent dialysis

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17
Q

how to follow patient after RAI?

A

Tg levels

I131 scan at 6-12 months if not decreasing

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18
Q

toxicities of RAI?

A

sialoadenitis
secondary cancers
infertility

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19
Q

Advice for radioactive patient after RAI:

A

The treated patient should remain 6 feet away from other people for about 24 hours after treatment.

especially avoid pregnant women and children

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20
Q

How long to delay pregnancy after RAI?

A

6 months

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21
Q

Staging for advanced thyroid cancer?

A

Contrast CT will delay RAI, but RAI is never an emergency…

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22
Q

Adjuvant therapies for parathyroid cancer?

A

Radiation is effective

[Munson Cancer 2003]

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23
Q

what is the rate of an intrathyroid parathyroid gland?

A

1-3%

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24
Q

What is the rate of an intrathyroid parathyroid cancer?

A

<1%

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25
Q

Parathyroid looks white/gray and is invading the RLN? what do you do?

A

Do en-bloc resection of parathyroid and ipsilateral thyroid lobe with RLN.

Leave the nodes alone if clinically benign.

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26
Q

What syndromes are associated with parathyroid cancer?

A

MEN

Jaw tumor syndrome

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27
Q

Mechanism of cinacalcet

A

Calciummemetic, blocks calcium receptor

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28
Q

Is a frozen section helpful for parathyroid cancer?

A

No need architecture showing invasion or a distant met to make the diagnosis

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29
Q

5 year survival in parathyroid cancer?

A

40-80%; only retrospective series

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30
Q

Causes of death in parathyroid cancer?

A

pulmonary mets

renal failure from hypercalcemia.

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31
Q

Absent thyroid lobe

A

check for ectopic thyroid tissue (tongue)

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32
Q

False negative rate of thyroid nodule < 4 cm

A

1-4%

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33
Q

False negative rate of a thyroid nodule > 4 cm

A

10%

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34
Q

Superior margin of a sistrunk procedure?

A

circumvali papillae in base of tongue

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35
Q

major complication of a sistrunk procedure?

A

airway injury

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36
Q

Can pyramidal lobe bifurcate?

A

yes

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37
Q

Chemo for ACC

A

Mitotane

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38
Q

Pheo lab work-up

A

plasma metanephrines first, then 24 hour urine for specificity

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39
Q

Aldosteronoma work-up

A

Lytes, Renin:Angiotensin ratio

IVC sampling is confirmatory and lateralizing test.

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40
Q

Malignancy risk of a 6 cm adrenal mass?

A

25%

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41
Q

When do you FNA an adrenal mass?

A

When suspicious for metastatic disease

Path cannot tell an ACC on needle biopsy.

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42
Q

Genetic syndrome associated with ACC

A

Lynch syndrome

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43
Q

How long to give mitotane for an ACC?

A

Can give for 5 years; most patients stop sooner due to side effects

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44
Q

Confirmatory test to evaluate for ectopic ACTH?

A

Ask IR to perform inferior petrossal sampling

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45
Q

ACTH work-up

A

low dose dex

high dose dex

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46
Q

Bilateral adrenalectomy for an unresectable ACTH secreting tumor?

A

never for MTC, SCC as survival is too poor. Maybe for a well selected PNET or thymic tumor.

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47
Q

What is mortality from Cushings refractory to medical management?

A

50% at 2 years

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48
Q

Get a frozen section on a thyroid nodule?

A

No, not useful

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49
Q

Thyroid nodule with decrease TSH, next step?

A

start propranolol
technicium uptake scan
only do lobectomy for a functional adenoma

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50
Q

FNA result shows suspicion for Hurthle cell neoplasm,
chance of cancer?
Management?

A

30% chance this is a Hurthle Cell cancer

would do lobectomy

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51
Q

Pancreatic enucleation is appropriate for?

A

hormonally functional PNETs

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52
Q

MEN I gene and function

A

mennin - transcription factor

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53
Q

Best palliative treatment for liver PNETs

A

TACE

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54
Q

Resect primary tumor in metastatic midgut carcinoid?

A

yes!

Good retrospective data it improves symptoms in 80% of patients.

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55
Q

Is there a survival benefit to removing primary tumor in metastatic midgut carcinoid?

A

Debatable. Some retrospective series say yes, but cannot account for selection bias.

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56
Q

Shave nodes off the SMA in metastatic carcinoid?

A

no.

Still good survival when leaving nodal disease behind.

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57
Q

Median survival after carcinoid resection with bulky nodal disease left behind?

A

> 6 years in some retrospective series.

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58
Q

HIPEC for carcinoid?

A

Has been done and abandoned in Europe

No difference in OS
Small PFS improvement, but high morbidity

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59
Q

First citation for 90% debulking of NETs

A

Sarmiento 2003. Retrospective study with suggesting good OS for these patients with no comparison arm, thus this threshold can be debated

PMID 12735141

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60
Q

What percent of patients recur after Liver debulking for carcinoid?

A

95%

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61
Q

Who should not get liver debulking for carcinoid?

A

comorbids that preclude liver surgery
over 50% replacement of hepatic parenchyma
disseminated mets outside the liver.

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62
Q

What percent of thyroid nodules are malignant?

A

5-10% all comers

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63
Q

US findings suggesting malignant thyroid nodule (6)

A
hypoechogenicity
calcifications
vascularity
taller than wide
ill defined margins
absent halo
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64
Q

FNA cutoff for thyroid nodules?

A

all > 1.5 cm

all > 1 cm with suspicious features

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65
Q

High risk patients for thyroid cancer

A

history of radiation
genetics
any PET avid node

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66
Q

MEN1 gene

A

mennin - autosomal dominant

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67
Q

MEN2 gene

A

RET - autosomal dominant

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68
Q

What percent of MTC are hereditary?

A

25%

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69
Q

What MTC patients get genetic testing?

A

all!

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70
Q

MTC with RLN involvement

A

take the RLN

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71
Q

Operation for MTC

A

always to total
do central LND if calcitonin >40
transplant all the paras into the forearm

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72
Q

MENIIB gene

A

RET mutation in codon M918T

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73
Q

MENIIA thyroid management

A

some data to tailor to specific mutation
follow calcitonin from birth
Perform total thyroidectomy when calcitonin level >150 or patient at age 5

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74
Q

MENIIB thyroid management

A

Remove thyroid before age 1

Start screening for pheo at age 11

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75
Q

What do you use to guide lymphadenectomy for MTC?

A

Some use calcitonin levels; (others rely on US)
<20: no nodal dissection
20-50: central and ipsilateral
>50: bilateral neck dissection
>500: add mediastinal lymph node dissection

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76
Q

Who should do thyroidectomies for MENIIB?

A

should send to quarternary center to manage the incredibly small parathyroids in infants.

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77
Q

Is ultrasound useful for neck nodes in MTC?

A

not sensitive enough, rely on calcitonin, but need to take ultrasound positive nodes.

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78
Q

Jaw Tumor Syndrome

A

High risk of parathyroid cancer

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79
Q

Best test to find a parathyroid gland in reoperative scenario?

A

4DCT

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80
Q

three tumors in VHL

A

Pheos
RCCs
PNETs

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81
Q

appearance of VHL pancreatic tumors?

A

Cystic with solid components

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82
Q

Workup for VHL pancreatic tumor?

A

get a pancreatic MRI look closely for the cystic component.

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83
Q

Most common MEN1 pancreatic tumor

A

non-hormonal PNET

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84
Q

Steps of a Thompson Procedure for MENI pancreas?

A
  1. Subtotal Distal Pancreatectomy
  2. Enucleate all Head tumors
  3. perform longitudinal duodenotomy and explore duodenum if gastrin is up.
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85
Q

Outcomes with the Michigan/Thompson Procedure

A

General Morbidity does not support and 30 of first 40 patients still required reoperation.

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86
Q

When to do a pancreas operation for MENI patient?

A

tumors >3cm
enlarging tumor
all insulinomas

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87
Q

Perioperative imaging for a MENI pancreas

A

octroscan preop

intraoperative ultrasound

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88
Q

When to do a duodenotomy for MENI pancreas?

A

for all elevated gastrin cases

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89
Q

Modern approach to MENI pancreas

A

Once in the OR do an enucleation for all detectable tumors

only do a whipple for gastrinoma of for recurrence.

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90
Q

rate of adrenal masses in autopsy studies?

A

7%

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91
Q

CT evaluation of adrenal masses

A

get a non-contrast CT benign if:
<10 Hounsfield units
well circumscribed
< 4cm

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92
Q

What else do you look for when looking at an adrenal mass?

A

check to make sure a contralateral adrenal is present!

93
Q

ACC risk factors for peritoneal recurrence?

A

numerous risk factors suggesting laparoscopic technique is associated with peritoneal recurrence.

94
Q

Node positivity rate of ACC?

A

10%

95
Q

Any benefit to lymph node dissection for ACC?

A

no evidence to prove it.

96
Q

FIRM ACT trial

A

ACC neoadjuvant trial for mitotane +/- EDT

Higher response rate with EDT but no OS benefit

97
Q

EDT chemotherapy

A

Etoposide
Doxorubicin
Cisplatin

98
Q

ACC with caval thrombus?

A

Give neoadjuvant EDT and mitotane

intraoperative ultrasound to evaluate extent of thrombus.

99
Q

tumor thrombectomy

A

Try to get distal control on IVC. Could guide a balloon from IJ to below the hepatic veins.

RCC <2cm from renal vein can be “milked back”

thrombus below hepatic veins, a simple thrombectomy.

thrombus above hepatic veins, cardiopulmonary bypass

100
Q

Advanced imaging for pheochromocytoma

A

PET/Dotatate - most sensitive test.

Would only do if you are still searching for a primary after a normal CT or MRI.

101
Q

what drugs mess up 24hr urine for metanephrines?

A

amphetamines, cocaine, MAO and tricyclic antidepressants.

102
Q

genetic testing for pheos?

A

test all positive patients and then all 1st degree relatives.

103
Q

three genetic conditions associated with pheos

A

Von Hippel Lindau
MENII
Neurofibromatosis I

104
Q

Observation of PNETs?

A

can observe low grade <1.5 cm

resect all >1.5 cm and all intermediate and high grade.

105
Q

Observation of PNETs in MENI patients?

A

resect all > 1 cm

106
Q

embryologic origin of PNET tumors

A

neural crest

107
Q

resect the primary in small bowel stage IV PNET?

A

usually should do given that symptomatic relief is well established and some retrospective studies suggest survival benefit

108
Q

pancreatic PNET management?

A

consider enucleation.

109
Q

Ultrasound follow-up of non-FNA thyroid nodules?

A

●6 to 12 months for subcentimeter nodules with suspicious characteristics

●12 to 24 months for nodules with low to intermediate suspicion on ultrasound

●2 to 3 years for very-low-risk nodules

110
Q

Suspicious findings for thyroid nodule?

A

Irregular margins

Microcalcifications

Taller than wide shape

Rim calcifications

111
Q

check a calcitonin for thyroid nodule?

A

no, especially if you cannot do a pentagastrin confirmatory test.

112
Q

FNA a PET avid thyroid nodule?

A

yes if >1cm

113
Q

Bethesda Classification

A

I Nondiagnostic (unsatisfactory) 5 to 10%
II Benign 0 to 3%
III Atypia of undetermined significance (AUS) or follicular lesion of undetermined significance (FLUS) 10 to 30%
IV Follicular neoplasm (or suspicious for follicular neoplasm) 25 to 40%
V Suspicious for malignancy 50 to 75%
VI Malignant 97 to 99%

114
Q

Thyroid FNA - no follicular tissue is obtained

A

Bethesda I nondiagnostic - need repeat FNA

115
Q

Thyroid FNA - dense lymphocytic infiltration and lymphoid germinal centers.
Plasma cell
Hürthle cell changes
fibrosis

A

Hashimotos Thyroiditis - correlate clinically do not operate.

116
Q

Thyroid FNA - mild nuclear atypia and lesions with extensive oncocytic (Hürthle cell) change but not enough to be classified as Hürthle cell neoplasm.

A

Atypia of undetermined significance - Bethesda III - repeat FNA v lobectomy

117
Q

Thyroid FNA - mixed macrofollicular and microfollicular pattern

A

Folicular lesion of undetermined significance - Bethesda III - repeat FNA v lobectomy

118
Q

Thyroid FNA - microfollicles, scant colloid, the absence of follicle formation, cells arranged in clusters and clumps, and varying nuclear atypia and cellular pleomorphism

A

Follicular neoplasm Bethesda IV - lobectomy

119
Q

Thyroid FNA - large polyclonal cells with abundant oxyphilic cytoplasm.

A

Hurthle Cells- bethesda IV - need lobectomy, diagnosis of cancer can only be made after excision

120
Q

Thyroid FNA - large cells, where cytoplasm has a “ground glass” appearance, nucleoli are prominent, and the nuclei demonstrate clefts and grooves and intranuclear cytoplasmic inclusions (“Orphan Annie eyes”)
Psammoma bodies
dense “sticky” colloid may also be seen

A

diagnostic for papillary thyroid cancer

121
Q

Thyroid FNA - eccentrically placed nuclei and cytoplasmic tails. The cytoplasm may be slightly granular and is usually configured as a tear drop or cytoplasmic tail

A

Medullary Thyroid cancer - may not always be picked up.

122
Q

immunostain positive for calcitonin

A

Medullary Thyroid cancer

123
Q

Thyroid FNA - marked pleomorphism, bizarre giant cells, and spindle cells

A

AnaplasticThyroid cancer

124
Q

Pathology of thyroid lymphoma

A

difficult to distinguish from hashimoto’s thyroiditis

125
Q

rate of distant mets with anaplastic thyroid cancer?

A

15-50%

126
Q

most common location for anapastic thyroid cancer to met?

A

extensive lung and mediastinum

127
Q

labs for an anaplastic thyroid cancer

A

Thyroid function tests

CBC/CMP

Serum calcium and phosphorus
hypercalcemia of malignancy
hypocalcemia due to compromise of the parathyroids

Serum thyroglobulin

128
Q

image based staging of anaplastic thyroid cancer

A

PET and brain MRI

129
Q

Metastatic disease seen with anaplastic TC primary

A

~30 percent of patients with ATC have coexisting differentiated thyroid cancer,

metastases may not originate from anaplastic thyroid cancer.

thyroglobulin level and/or PET scan may help distinguish between the two

130
Q

stage IV anaplastic TC management

A

There is no curative therapy for metastatic anaplastic thyroid cancer, and the disease is uniformly fatal.

median survival 4.2 months, compared with six months in those without metastases.

131
Q

best case scenario with anaplastic thyroid cancer

A

with complete R0 resection and post-op radiation, median OS is 2 years.

132
Q

chemo for anaplastic thyroid cancer

A

strongly consider clinical trials (basket trials)
doxorubicin
check for BRAF status for vemurafenib/trabetenib

133
Q

PTC is found in contralateral lobe in what percent?

A

80%

134
Q

PTC recurs clinically after what percent of thyroid lobectomies?

A

3-5%

135
Q

Offer genetic testing for Bethesda II-IV thyroid nodules?

A

Probably not ready for primetime/board answer.

Safety is generally established at high experience centers but NPV depends on local incidence.

Multiple validation studies have questioned NPV at different institutions.

136
Q

non-invasive follicular thyroid neoplasm with papillary like features. (NIFTP)

A

usually diagnosed at time of diagnostic lobectomy
No completion lobectomy
does need ultrasound surveilance of contralateral lobe.

137
Q

Ideal candidate for PTC observation?

A

young patient
small tumor (~<2cm?)
tumor not near capsule, airway or RLN

138
Q

Observe a Papillary TC?

A

Probably wouldn’t offer personally but offer referral to quarternary hospital that was following result prospectively.

Saftey seems established at MSKCC and Japanese studies.

139
Q

Patients who will eventually need PTC resection after o

A

tumor grows >3mm or >50% from baseline

140
Q

Evidence of stable persistent thyroid cancer after resection?

A

This is the norm, try to avoid overtreatment. Consider Tg doubling time.

141
Q

previous Papillary TC. Tg> 10 and I131 scan is negative, what next?

A

PET scan

142
Q

Local recurrence after resection of PTC?

A

make sure to read old op notes and path notes to gauge quality of previous operation

143
Q

What disease is RAI most effective for?

A

very effective for pulmonary mets

minimally effective for bone mets. (consider palliative RT?)

144
Q

TKIs for stage IV Papillary TC?

A

sorafenib
Lenvatinib (preferred)

(both dirty TKIs)

145
Q

what med screws up aldosteronoma work-up?

A

need to take patient off spirinolactone

146
Q

AUS/FLUS and benign molecular diagnostics

A

observation

147
Q

Goal TSH for high risk PTC patient?

A

< 0.1 mU/L

148
Q

Additional supplements when patient of TSH suppression Synthroid?

A

Calcium

Vitamin D

149
Q

First measurement of TSH and Tg after PTC resection?

A

6 - 12 weeks

150
Q

PTC CNS mets?

A

gamma knife RT

151
Q

Hoarsness is a presenting symptom for?

A

supraglottic or glottic cancer

152
Q

Treatment for glottic cancer?

A

T1-T2 definitive RT + laser surgery

153
Q

Neck node with occult primary?

A

FNA the node -> SSC
fine cut CT/MRI of the H&N
PET Scan

154
Q

Labs for occult SSC?

A

HPV/EBV
Thyroglobulin
Calcitonin
Pax 8

155
Q

Procedural work-up for SSC occult primary level IV or V node?

A

tripple endoscopy
laryngoscopy, consider tonsillectomy
bronchoscopy
esophagoscopy

156
Q

When to get ThyroSeq?

A

Bethesda 3-4 lesions

if low then <5% chance of cancer

157
Q

When is it an emergency to give RAI?

A

never

158
Q

Pre OR work-up for all thyroid cases?

A

TSH, T3/T4, thyroglobulin, calcium
laryngoscopy
Neck ultrasound

159
Q

What is the mortality in the MSKCC PTC observation series?

A

zero

160
Q

Risks of thyroidectomy?

A

3% RLN injury
3% permanent hypothyroid
1% risk of bleeding requiring reoperation

161
Q

hypoparathyroidism refractory to Ca and other supplements

A

NATPARA

recombinant PTH injections

162
Q

Black box warning on NATPARA

A

caused osteosarcomas in animal experiments

163
Q

Non-operative options for chyle leak

A

TPN/no fat diet

if fails, can get IR to sclerose the thoracic duct.

164
Q

Lambatinib

A

1st line in RAI non-responsive PTC

165
Q

Anaplastic Thyroid Cancer

A

Start with chemoradiation while sending of tumor sequencing
targeted therapy based on sequencing
basket trials

166
Q

What stage is Anaplastic Thyroid Cancer

A

Stage IV at presentation

167
Q

RAI for anaplastic thyroid cancer?

A

NO!

168
Q

When to trach anaplastic thyroid cancer?

A

only when symptomatic

tumor will often grow out of wound and cause bleeding issues.

169
Q

Gastrinoma management?

A

are mostly managed with PPIs now.

170
Q

what to do for a RLN injury?

A

call for backup
repair with 9-0 suture
Consult a laryngeal specialist after to help with tone.

171
Q

Bilateral RLN injury?

A

Need to do an emergent tracheostomy

172
Q

ACC tumor thrombus

A

usually small.
See if you can resect and reconstruct that segment
if you can’t go on veno-venous bypass, open vessel and tumor will usually “slide right out.”

173
Q

ATA 2016 guidelines for RAI?

A

give for:
extrathyroid extension
R2
distant mets of LN >3cm

selective use:
aggressive histologies
microscopic extension
LVI
>5 lymph nodes
174
Q

Post-op care for parathyroid cancer patient?

A

trend calcium levels q6hrs.
give standing calcitriol and tums
IV only if symptomatic

175
Q

dosage for calcitriol

A

half a microgram daily PO

176
Q

intense workup of functional adrenal tumors?

A

dexamethasone supression test
plasma metanephrines
renin/aldosterone levels

177
Q

hormonal work-up for an ACC?

A

yes, and not unusual to secrete more than one hormone

178
Q

Dosing of phenoxybenzamine for pheo?

A

stat at 10 mg daily

max 100 mg daily

179
Q

Timing of phenoxybenzamine for a pheo?

A

at leas two weeks preop

180
Q

Timing of b-blocker for a pheo?

A

3-5 days preop

181
Q

dosing of b-blocker for a pheo?

A

propranolol 10mg q 6

can change to extended release

182
Q

alternatives to a/b-blockade for a pheo?

A

calcium channel blocker

metyrosine (inhibits catacholamine synthesis)

183
Q

Side effects of metyrosine?

A
sedation/depression/anxiety
 nightmares
diarrhea
urolithiasis
galactorrhea
extrapyramidal signs
184
Q

Screening after pheo resection?

A

Don’t forget genetic testing
Imaging if malignant tumor
annual urine metanephrines

185
Q

immaging options for an occult Pheo?

A

standard PET

Dotatate scan

186
Q

Unresectable stage IV pheo?

A

XRT
Tace/RFA for liver
I-131 if MIGB scan positive

187
Q

How do you protect the thyroid if giving I-131 for a pheo?

A

give patient potassium-iodide

188
Q

Lutathera for Pheo?

A

not yet FDA approved, but promising;

could present at tumor board/look for trials

189
Q

Chemical surveillance of carcinoids/NETs?

A

chromogrannin level

urinary 5HIAA levels

190
Q

preparation before a procedure for carcinoids?

A

give preop octrotide 500 mcg IV

191
Q

hypotension during carcinoid case?

A

“carcinoid crisis”
start an octreotide gtt
generally refractory to volume/pressors

can happen during TACE too!

192
Q

medical therapy for aldosteronoma?

A

spirinolactone

193
Q

post-op management after removal of aldosteronoma

A

check an aldosterone level

Q6 hr lytes to watch for hyperkalemia

194
Q

Adrenal venous sampling pearls?

A

can give with cosyntropin infusion to increase sensitivity
need ratio of 1:5 in stimulated test
typically the unaffected side will be even less than the IVC since it is supressed.

195
Q

patient presents with hypokalemia and hypertension?

A

aldosteronoma

196
Q

young patient with unexplained weight change, facial plethora

A

cushings syndrome (exctopic corticosteroids)

197
Q

how to rule out Munchausen’s with cushings?

A

check urinary cortisol, if low then likely patient is taking pills.

198
Q

cushing’s work-up?

A

need at least 2 screening tests
low dose dexamethasone supression (1 mg)
urinary cortisol levels
late night salivary levels

199
Q

high dose dexamethasone supression test

A

4-8 mg dexamethasone
if plasma cortisol/ACTH supressed then pituitary source.
if no supression, then ectopic tumor

200
Q

patient presents with adrenal mass and virulazation?

A

think ACC

201
Q

what percent of virulizing tumors are cancer?

A

~50%

202
Q

What percent of ACC secrete hormones?

A

50% cortisol

25% androgens

203
Q

Blood tests for virulizing tumor

A

adrenal androgens

  • —-DHEAS
  • —-androstenedione
  • —-testosterone
  • —-17-hydroxyprogesterone
  • —-serum estradiol (men, old women)
204
Q

control of cortisol secreting ACC?

A

Mitotane 1 g four times a day
if refractory
metyrapone 250 mg four times a day

205
Q

control of androgen secreting ACC?

A

finasteride

tamoxifen

206
Q

who gets adjuvant mitotane?

A

high-grade disease (Ki67 >10 percent or mitotic rate greater than 20 per 50 high-power fields [HPF]), incompletely resected disease
intraoperative tumor spillage or fracture
large tumors
vascular or capsular invasion

207
Q

Any RCTs for ACC?

A

ADIUVO

RCT for mitotane for low grade resected ACCs

208
Q

How do you dose Mitotane?

A

Needs pharmacy serum monitoring

levels between 14 and 20 mcg/mL

209
Q

tumor markers for MTC?

A

calcitonin

CEA

210
Q

5 year recurrence of MTC if biochemical cure?

A

<5%

211
Q

follow-up MTC?

A

complete genetic testing if not done
follow calcitonin/CEA
Get a repeat neck ultrasound at year 1

212
Q

workup for elevated post-op calcitonin?

A

<150 start with neck ultrasound

>150 Neck ultrasound and CT c/a/p

213
Q

Mildly elevated post-op calcitonin and no radiologic evidence of disease?

A

suspect microscopic disease in the neck,

present at tumor board for consideration of neck irradiation

214
Q

Symptoms of metastatic MTC?

A

Diarrhea - usually from large calcitonin producing liver mets
Cushings - ectopic ACTH

215
Q

Palliation of MTC diarrhea?

A

imodium (check a c.diff)
octreotide
TACE
palliative debulking

216
Q

Palliation of MTC cushings?

A

Vandatanib - dirty TKI hits RET
Mitotane - inhibits synthesis
Bilateral adrenalectomy - select patients carefully

217
Q

Systemic therapies for stage IV MTC?

A

consider observation for Mets <1cm and asymptomatic
variety of TKIs (vandatanib 1st)
chemo has <10% response rate
LUTATHERA is emerging

218
Q

Resect a primary MTC in the setting of stage IV disease?

A

only for palliation of airway or esophageal compression.

219
Q

usefulness of postop PTH?

A

multiple studies show that PTH < 10 predicts symptomatic hypocalcemia and can be used to guide calcium replacement.

220
Q

critical error to avoid on a parathyroid cancer?

A

rupture caries a high risk of neck carcinomatosis.

221
Q

when to enucleate an insulinoma?

A

tumors < 2cm away from the main duct

Test for MEN I first so you don’t miss additional lesions

222
Q

malignancy rate of insulinomas?

A

10%

223
Q

most common non-adrenal location of a pheo?

A

organ of zuckerkandl at the aortic bifurcation.

224
Q

jaw tumor syndrome

A

hyperparathyroidism
mandible fibromas
kidney lesions

225
Q

patient presents with high PTH and calcium?

A

get genetic testing first before going into hyperparathyroid work-up!

226
Q

Lymph node dissection for follicular carcinoma?

A

NO! spreads hematogenously

remainder of treatment is as per PTC

227
Q

Initial work-up for an insulinoma?

A

fasting glucose and insulin level
c-peptide
get genetic testing or Brain MRI Ca++/PTH to rule our MEN I

228
Q

What to do before OR for an insulinoma?

A

Admit night before for D10 infusion while NPO