Endocrine Cancer

Question 1

What are the two types of differentiated thyroid carcinoma?

Answer 1

Papillary and follicular

Question 2

What is the standard of care treatment algorithm for localized differentiated thyroid cancer?

Answer 2

Surgery followed by RaI, then levothyroxine (TSH suppression)

Question 3

What is the cell of origin for medullary thyroid carcinoma?

Answer 3

Parafollicular C cells of thyroid, that produce calcitonin

Question 4

What is the clinical syndrome of MEN 2A

Answer 4

Medullary thyroid carcinoma (>95%)
Parathyroid hyperplasia (20%)
Pheochromocytoma (50%)

Question 5

What is the clinical syndrome of MEN 2B

Answer 5

Medullary thyroid carcinoma (100%)
Mucosal neuromas (GI ganglioneuromas >98%)
Pheochromocytoma (50%)

Question 6

What is the gene involved in MEN2A and MEN2B?

Answer 6

RET

Question 7

What are the two most common mutations seen in sporadic medullary thyroid carcinoma?

Answer 7

RET
RAS (HRAS, then KRAS, then NRAS)

Question 8

You see a patient with MEN2 prior to development of any malignancy. What do you do?

Answer 8

Rule out pheochromocytoma with labs or imaging, then do prophylactic thyroidectomy

Question 9

In a patient with medullary thyroid carcinoma, what clinical features puts them at high risk for residual disease after surgery?

Answer 9

LN+ (90% have residual disease vs 38% in LN-)

Question 10

In medullary thyroid carcinoma, what is a clue that there is residual disease after surgery?

Answer 10

Persistently high calcitonin levels

Question 11

What is a useful tumor marker for differentiated thyroid cancer?

Answer 11

Thyroglobulin
Makes sure you have reflex to TgAb because that can interfere with ability to measure thyroglobulin

Question 12

You see a patient with metastatic differentiated thyroid cancer. They have a low thyroglobulin. What should you suspect?

Answer 12

Poor differentiation and aggressive behavior

Question 13

What are useful tumor markers in MTC? (2)

Answer 13

Calcitonin
CEA

Question 14

What does a low Calcitonin:CEA ratio mean?

Answer 14

Poor differentiation, aggressive

Question 15

What does a high calcitonin:CEA ratio suggest?

Answer 15

Well differentiated disease

Question 16

What are two treatment options for advanced differentiated thyroid carcinoma?

Answer 16

Sorafenib and lenvatinib (preferred)

Question 17

What are two treatment options for 1st line advanced MTC?

Answer 17

Vandetanib
Cabozantinib

Question 18

What is the only FDA approved treatment for 2nd line advanced differentiated thyroid cancer?

Answer 18

Cabozantinib

Question 19

Treatment for advanced anaplastic thyroid carcinoma with BRAF mutation?

Answer 19

Dabrafenib + Trametinib

Question 20

Treatment for advanced papillary thyroid carcinoma with BRAFV600E mutation

Answer 20

Dabrafenib + trametinib

Question 21

Indications for dabrafenib + trametinib in thyroid cancer

Answer 21

Advanced anaplastic
Advanced papillary

Question 22

2 Treatment options for RET mutated/fusion advanced thyroid cancer?

Answer 22

Selpercatinib
Pralsetinib

Question 23

Treatment for advanced thyroid carcinoma with NTRK fusions (2 options)

Answer 23

Larotrectinib
Entrectinib

Question 24

What is the difference in treatment of localized differentiated thyroid carcinoma vs medullary or anaplastic?

Answer 24

Medullary or anaplastic doesn’t need RAI

Question 25

6 indications for total thyroidectomy in differentiated thyroid cancer?

Answer 25

prior radiation (2B) Extrathyroid extension poorly diff lateral cervical LN mets or gross central neck LN Tumor >4 cm Known distant mets

Question 26

RAI should not be offered to people with differentiated thyroid carcinoma who meet what 6 criteria?

Answer 26

Classic papillary small tumor <2 cm No extrathyroid extension No detectable Tg antibodies Postoperative thyroglobulin <1 ng/mL negative postoperative US, if done

Question 27

Indications for adjuvant RAI in differentiated thyroid cancer? 1 of 7

Answer 27

N1b disease extrathyroid extension Tumor >4 cm Postoperative thyroglobulin >10 >5 LNs Vascular invasion Known or suspected distant mets at presentation

Question 28

preferred management of locoregional recurrence of MTC?

Answer 28

Surgery

Question 29

For patients with carcinoid tumors of the appendix, what dictates how large of a surgery they get?

Answer 29

Tumors >2 cm (or those with incomplete resection or LN+), they should get scans for metastatic disease. If no mets, then they need a R hemicolectomy. Tumors <=2 cm, then appendectomy

Question 30

Clinical features of MEN1

Answer 30

Hyperparathyroidism Pancreatic NET (glucagonoma, insulinoma, VIPoma, gastrinoma)