Endocrine Cancer Flashcards
What are the two types of differentiated thyroid carcinoma?
Papillary and follicular
What is the standard of care treatment algorithm for localized differentiated thyroid cancer?
Surgery followed by RaI, then levothyroxine (TSH suppression)
What is the cell of origin for medullary thyroid carcinoma?
Parafollicular C cells of thyroid, that produce calcitonin
What is the clinical syndrome of MEN 2A
Medullary thyroid carcinoma (>95%)
Parathyroid hyperplasia (20%)
Pheochromocytoma (50%)
What is the clinical syndrome of MEN 2B
Medullary thyroid carcinoma (100%)
Mucosal neuromas (GI ganglioneuromas >98%)
Pheochromocytoma (50%)
What is the gene involved in MEN2A and MEN2B?
RET
What are the two most common mutations seen in sporadic medullary thyroid carcinoma?
RET
RAS (HRAS, then KRAS, then NRAS)
You see a patient with MEN2 prior to development of any malignancy. What do you do?
Rule out pheochromocytoma with labs or imaging, then do prophylactic thyroidectomy
In a patient with medullary thyroid carcinoma, what clinical features puts them at high risk for residual disease after surgery?
LN+ (90% have residual disease vs 38% in LN-)
In medullary thyroid carcinoma, what is a clue that there is residual disease after surgery?
Persistently high calcitonin levels
What is a useful tumor marker for differentiated thyroid cancer?
Thyroglobulin
Makes sure you have reflex to TgAb because that can interfere with ability to measure thyroglobulin
You see a patient with metastatic differentiated thyroid cancer. They have a low thyroglobulin. What should you suspect?
Poor differentiation and aggressive behavior
What are useful tumor markers in MTC? (2)
Calcitonin
CEA
What does a low Calcitonin:CEA ratio mean?
Poor differentiation, aggressive
What does a high calcitonin:CEA ratio suggest?
Well differentiated disease
What are two treatment options for advanced differentiated thyroid carcinoma?
Sorafenib and lenvatinib (preferred)
What are two treatment options for 1st line advanced MTC?
Vandetanib
Cabozantinib
What is the only FDA approved treatment for 2nd line advanced differentiated thyroid cancer?
Cabozantinib
Treatment for advanced anaplastic thyroid carcinoma with BRAF mutation?
Dabrafenib + Trametinib
Treatment for advanced papillary thyroid carcinoma with BRAFV600E mutation
Dabrafenib + trametinib
Indications for dabrafenib + trametinib in thyroid cancer
Advanced anaplastic
Advanced papillary
2 Treatment options for RET mutated/fusion advanced thyroid cancer?
Selpercatinib
Pralsetinib
Treatment for advanced thyroid carcinoma with NTRK fusions (2 options)
Larotrectinib
Entrectinib
What is the difference in treatment of localized differentiated thyroid carcinoma vs medullary or anaplastic?
Medullary or anaplastic doesn’t need RAI
6 indications for total thyroidectomy in differentiated thyroid cancer?
prior radiation (2B)
Extrathyroid extension
poorly diff
lateral cervical LN mets or gross central neck LN
Tumor >4 cm
Known distant mets
RAI should not be offered to people with differentiated thyroid carcinoma who meet what 6 criteria?
Classic papillary
small tumor <2 cm
No extrathyroid extension
No detectable Tg antibodies
Postoperative thyroglobulin <1 ng/mL
negative postoperative US, if done
Indications for adjuvant RAI in differentiated thyroid cancer? 1 of 7
N1b disease
extrathyroid extension
Tumor >4 cm
Postoperative thyroglobulin >10
>5 LNs
Vascular invasion
Known or suspected distant mets at presentation
preferred management of locoregional recurrence of MTC?
Surgery
For patients with carcinoid tumors of the appendix, what dictates how large of a surgery they get?
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
Clinical features of MEN1
Hyperparathyroidism
Pancreatic NET (glucagonoma, insulinoma, VIPoma, gastrinoma)