Neuroendocrine tumors

Neuroendocrine Tumors

Neuroendocrine Tumors Part 1

What is a rare but serious side effect of everolimus?

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Pneumonitis

Neuroendocrine Tumors Part 1
Neuroendocrine tumors

Which targeted therapy is good for insulinoma and why?

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Everolimus because of hyperglycemia

Neuroendocrine tumors
Neuroendocrine tumors

Which familial syndromes are neuroendocrine tumors associated with?

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MEN1, VHL, NF Type 1, Tuberous Sclerosis

Neuroendocrine tumors
Neuroendocrine tumors

What is the annual incidence of VIP, glucagon, somatostatin secreting neuroendocrine tumors?

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1/10 million

Neuroendocrine tumors
Neuroendocrine tumors

Where can carcinoid tumors arise?

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Appendix, stomach, duodenum, lung, rectum

Neuroendocrine tumors
Neuroendocrine tumors

How is the chromatin described for low and intermediate grade neuroendocrine tumor?

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Salt-and-pepper appearance

Neuroendocrine tumors
Neuroendocrine tumors

What are the 2 types of high-grade neuroendocrine tumor?

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Small cell carcinoma and large cell neuroendocrine carcinoma

Neuroendocrine tumors
Neuroendocrine tumors

What are the mitotic counts and Ki-67 indices for low, intermediate and high-grade neuroendocrine
tumors?

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Low grade <2/hpf, Ki-67 less than 3%Intermediate 2-20 /hpf, Ki-67 3-20%High-grade >20/hpf, Ki-67 >20%

Neuroendocrine tumors
Neuroendocrine tumors

What percentage of neuroendocrine tumors are non-secretory?

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75%

Neuroendocrine tumors
Neuroendocrine tumors

Which hormones can a neuroendocrine tumor secrete?

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VIP, glucagon, insulin, gastrin, somatostatin

Neuroendocrine tumors
Neuroendocrine tumors

Which neuroendocrine treatment offers a low side effect profile?

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Lanreotide

Neuroendocrine tumors
Neuroendocrine tumors

Which MEN is associated with gastrinoma?

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MEN1

Neuroendocrine tumors
Neuroendocrine tumors

Which second line chemo agent combination for neuroendocrine tumor has the best response rate ?

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Temozolamide plus capecitabine

Neuroendocrine tumors
Neuroendocrine tumors

How do you treat severe carcinoid syndrome during carcinoid flares?

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Intravenous octreotide

Neuroendocrine tumors
Neuroendocrine tumors

Which cardiac abnormality can occur with carcinoid?

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Right-sided pulmonic valve regurgitation

Neuroendocrine tumors
Neuroendocrine tumors

which vitamin deficiency can occur with carcinoid?

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Niacin deficiency

Neuroendocrine tumors
Neuroendocrine tumors

What treatments can be used for progressive low grade metastatic neuroendocrine tumor that are nonfunctional?

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Sunitinib and other oral VEGFR targeting TKIs

Neuroendocrine tumors
Neuroendocrine tumors

Which chemotherapy agents are used for poorly differentiated neuroendocrine tumors?

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Platinum-based

Neuroendocrine tumors
Neuroendocrine tumors

What are the main treatment option for insulinoma?

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Carbohydrates and octreotide

Neuroendocrine tumors
Neuroendocrine tumors

Which positron emission tomography tracers can be used to detect neuroendocrine tumors?

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18-FDG, 11 C- 5- HTP, 68-Ga-DOTATOC

Neuroendocrine tumors
Neuroendocrine tumors

What is an intervention technique that can diagnose neuroendocrine tumor from the pancreas?

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Arterial stimulation with venous sampling

Neuroendocrine tumors
Neuroendocrine tumors

Which treatments for neuroendocrine tumor require SRS imaging?

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Yttrium-90 octreotide and Lutetium – 177 octreotide

Neuroendocrine tumors
Neuroendocrine tumors

What are the only advantages of somatostatin receptor scanning?

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Detection of bone lesions and somatostatin receptor concentration correlates with treatment decision
making

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Neuroendocrine tumors

What type of image detector is used for octreotide scans?

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SPECT

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Neuroendocrine tumors

What isotype is used for somatostatin scans?

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Indium-111 tagged somatostatin

Neuroendocrine tumors
Neuroendocrine tumors

How do neuroendocrine tumors look on T1 and T2-weighted MRI?

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T1 is low intensity T2 is high intensity

Neuroendocrine tumors
Neuroendocrine tumors

What is the main challenge with imaging neuroendocrine tumors?

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Increased vascularity therefore arterial and venous phase contrast is required

Neuroendocrine tumors
Neuroendocrine tumors

Do the nonfunctioning neuroendocrine tumors secrete any hormones?

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Yes, chromogranin, neuron specific enolase

Neuroendocrine tumors
Neuroendocrine tumors

Which electrolyte abnormalities can be seen with VIP-oma?

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Hypokalemia and hypochloremia

Neuroendocrine tumors
Neuroendocrine tumors

What is a common and severe symptom of hypoglycemia from an insulinoma?

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Amnesia

Neuroendocrine tumors
Neuroendocrine tumors

What 2 major side effects of everolimus should be considered for neuroendocrine tumors?

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Stomatitis and hyperglycemia

Neuroendocrine tumors
Neuroendocrine tumors

Which family of drugs are effective for progressive neuroendocrine tumor?

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mTOR inhibitor, VEGF targeting antibody, VEGF inhibiting oral agents

Neuroendocrine tumors
Neuroendocrine tumors

Which genetic deletion is predictive of a temozolomide response?

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MGMT – if positive deletion then positive response, if WT- no response

Neuroendocrine tumors
Neuroendocrine tumors

What is a second chemotherapy regimen for symptomatic neuroendocrine tumor?

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Dacarbazine and Temozolamide

Neuroendocrine tumors
Neuroendocrine tumors

Which chemotherapy is ideal for a highly differentiated neuroendocrine tumor with signs / symptoms
related to metastases or growth?

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Streptozosin +/- doxorubicin +/- 5FU

Neuroendocrine tumors
Neuroendocrine tumors

What is the primary side effect of somatostatin octreotide analogs?

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Gallstones seen in 25%

Neuroendocrine tumors
Neuroendocrine tumors

What did the CLARINET trial show?

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Improved progression free survival for Lanreotide versus placebo in nonfunctioning neuroendocrine
tumor

Neuroendocrine tumors
Neuroendocrine tumors

How do you treat gastrinoma?

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PPI +/- octreotide

Neuroendocrine tumors
Neuroendocrine tumors

Which types of functioning neuroendocrine tumors benefit the most from somatostatin analogs?

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Insulinoma glucagonoma

Neuroendocrine tumors
Neuroendocrine Tumors Part 2

Has a PFS or OS been demonstrated with sunitinib in advanced NET ?

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Only PFS

Neuroendocrine Tumors Part 2
Neuroendocrine Tumors

Which agents have been combined with temozolomide for advanced NET ?

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Bevacizumab and lenalidomide

Neuroendocrine Tumors
Neuroendocrine Tumors

How often is octreotide administered based off the PROMID trial ?

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Every 4 weeks

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neuroendocrine tumors

What did the RADIANT-3 trial assess ?

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Everolimus monotherapy for NET

neuroendocrine tumors
Neuroendocrine tumors

Was the CLARINET trial limited to pancreatic NET only ?

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No

Neuroendocrine tumors
Neuroendocrine tumors

What was the Ki-67 cut-off for the CLARINET trial ?

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10%

Neuroendocrine tumors
Neuroendocrine tumors

What was the most common AE (all grade) reported in the RADIANT-3 trial ?

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Stomatitis

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Neuroendocrine tumors

Was there a statistically significant improvement in OS in the RADIANT-3 trial ?

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No, only a trend

Neuroendocrine tumors
Neuroendocrine tumors

Is lanreotide administered SC or IM ?

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SC

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Neuroendocrine tumors

What did the CLARINET trial assess in metastatic NET ?

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Lanreotide

Neuroendocrine tumors
Neuroendocrine tumors

What did the RADIANT-3 trial assess in metastatic pancreatic NET ?

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Everolimus vs placebo

Neuroendocrine tumors
Neuroendocrine tumors

Which cytotoxic agents are active in progressive NET ?

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Temozolamide, capecitabine, 5-FU, streptozocin, doxorubicin

Neuroendocrine tumors
neuroendocrine tumors

Which subtype of NET does temozolomide and lenolidmode appear to be most active in ?

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Pancreatic

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neuroendocrine tumors

What is the most common grade 3/4 AE seen with temozolomide when used in advanced NET ?

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Lymphopenia

neuroendocrine tumors
neuroendocrine tumors

Which subtype of NET appears to respond better to temozolomide and bevacizumab ?

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Pancreatic

neuroendocrine tumors
neuroendocrine tumors

Do hormonally active advanced NET respond better or worse to octreotide ?

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Similar

neuroendocrine tumors
neuroendocrine tumors

What predicts for improved responses to octreotide in advanced NET from the PROMID trial ?

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Resected primary tumor and low hepatic tumor load

neuroendocrine tumors
neuroendocrine tumors

What is the most common AE seen with lanreotide ?

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Diarrhea

neuroendocrine tumors
neuroendocrine tumors

What is the response rate of low grade NETs to PDL1 targeted therapies ?

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Very low, less than 10%

neuroendocrine tumors
Neuroendocrine tumors

Which 2 radionucleotide are used to treat neuroendocrine tumors?

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Yttrium-90 and Lutetium- 133- Octreotide

Neuroendocrine tumors
Neuroendocrine tumors

What mTOR and VEGF targeting agents have activity in NETs? ?

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Everolimus, tensirolimus and bevacizumab

Neuroendocrine tumors
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