Does Everolimus in Gastrointestinal, Lung NETs Delay Progression at Expense of Health-Related QOL?

Do you know the quality-of-life impact of everolimus in gastrointestinal or lung neuroendocrine tumors? Do you know the associations between neuroendocrine tumors and MEN1? Test your knowledge on these topics and more in our latest quiz on neuroendocrine tumors.

Question 1

Answer

B.  False. In the randomized phase III RADIANT-4 trial, HRQOL was maintained for patients with advanced, non-functional, gastrointestinal or lung NETs, with no relevant differences noted between the everolimus and placebo groups.

Reference

Pavel ME, Singh S, Strosberg JR, et al. Health-related quality of life for everolimus versus placebo in patients with advanced, non-functional, well-differentiated gastrointestinal or lung neuroendocrine tumours (RADIANT-4): a multicentre, randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Oncol. 2017;18:1411-22.

Question 2

Answer

B.  Patients with familial hyperparathyroidism including MEN1 are less likely to develop recurrent disease compared to sporadic hyperparathyroidism. Patients with familial hyperparathyroidism including MEN1 are more likely, not less likely, to develop recurrent disease compared to sporadic hyperparathyroidism. So Answer B is the false statement.

Reference

Montenegro FL, Lourenço DM Jr, Tavares MR, et al. Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center. Clinics (Sao Paulo). 2012;67(suppl 1):131-9.

Question 3

Answer

A.MEN1 is associated with pituitary gland and parathyroid tumors, pancreatic NETs, but not carcinoid or adrenal tumors, lipomas, or skin angiomas. MEN1, or Wermer syndrome, is characterized by tumors of the parathyroid and pituitary glands and pancreatic NETs, but may also be associated with carcinoid tumors of thymus, bronchial, gastric, adrenal tumors, and multiple lipomas and skin angiomas. So Answer A is the false statement.

Reference

Marx S, Spiegel AM, Skarulis MC, et al. Multiple endocrine neoplasia type 1: clinical and genetic topics. Ann Intern Med. 1998;129:484-94.

Question 4

Answer

D. D. Symptomatic functional tumors of any size. Symptomatic functional tumors of any size should not be removed unless they are refractory to medical treatment.

Reference

Yates CJ, Newey PJ, Thakker RV. Challenges and controversies in management of pancreatic neuroendocrine tumours in patients with MEN1. Lancet Diabetes Endocrinol. 2015;3:895-905.

Question 5

Answer

D. All of the above are true.

Reference

Wells SA Jr, Pacini F, Robinson BG, Santoro M. Multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma: an update. J Clin Endocrinol Metab. 2013;98:3149-64.