Test Your Knowledge of Neuroendocrine Tumors

Are you up to date on staging for neuroendocrine tumors (NETs)/carcinoid tumors? How about which follow-up tests are most appropriate for a patient with medullary thyroid carcinoma? Test your knowledge on these topics and more in our latest quiz.

Question 1

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D.All of the above. In a study that included over 35,000 patients with NETs, the researchers also found that "disease stage, primary tumor site, histologic grade, sex, race, age, and year of diagnosis were predictors of outcome" among patients with well-differentiated to moderately differentiated tumors.

Question 2

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Answer

D. Pancreatic NETs are staged differently than exocrine pancreatic carcinomas. Medullary thyroid carcinoma is a calcitonin-secreting tumor of the parafollicular C cells of the thyroid gland. About 25% of the cases are familial and associated with MEN2 syndrome and germline mutations of proto-oncogene RET. All of the tests are appropriate except radioactive iodine thyroid scan as this tumor does not originate from follicular or papillary cells.

Question 3

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B. Repeat surgical resection with right hemicolectomy. Repeat surgical resection with right hemicolectomy. Most appendiceal NETs that are 2 cm or smaller can be treated with simple appendectomy. However tumors larger than 2 cm with poor prognostic features such as lymphovascular invasion, mesoappendiceal invasion, or atypical histologic features require a more aggressive approach with right hemicolectomy.

Question 4

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B. Radioactive iodine scan. Medullary thyroid carcinoma is a calcitonin-secreting tumor of the parafollicular C cells of the thyroid gland. About 25% of the cases are familial and associated with MEN2 syndrome and germline mutations of proto-oncogene RET. All of the tests are appropriate except radioactive iodine thyroid scan as this tumor does not originate from follicular or papillary cells.

Question 5

A 54-year-old man with a history of peptic ulcer presents with epigastric pain, diarrhea, vomiting, and weight loss. He has been on proton pump inhibitors for his epigastric pain with no major improvement. An upper endoscopy reveals a partially obstructing duodenal ulcer and multiple smaller ulcers in the second part of the duodenum. Further work-up with CT abdomen pelvis shows thickening and enhancement of the small bowel and a vague pancreatic mass.

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C. Fasting serum gastrin levels. Fasting serum gastrin levels would be high due to the patient’s use of proton pump inhibitors, so they should be checked at least a week after discontinuation of proton pump inhibitors. The patient most likely has a gastrinoma in his pancreas. The rest of the work-up will be helpful to diagnose this tumor as well as searching for MEN1 syndrome-related tumors of pituitary and parathyroid glands.