MEN syndromes: Clinical

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MEN syndromes: Clinical

USMLE® Step 2 questions

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USMLE® Step 2 style questions USMLE

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A 50-year-old woman comes to the office because of episodic headaches and palpitations. She says her blood pressure during one of these episodes was 160/98 mm Hg. Currently, her temperature is 36.7°C (98°F), the pulse is 84/min, respirations are 14/min, and blood pressure is 110/84 mm Hg. A 24-hour urine metanephrine fractionation shows elevated levels of normetanephrine, metanephrine, and norepinephrine. An MRI study of the patient’s abdomen reveals a T2-hyperintense tumor just lateral to the abdominal aorta at the L1 level. Administration of which of the following medications is the most appropriate first step to prepare the patient for surgery?


The multiple endocrine neoplasias, or MEN for short, are a group of inherited diseases which cause tumors to grow in endocrine glands, like the pancreas, pituitary, thyroid, adrenals, and parathyroid glands.

So in multiple endocrine neoplasias there’s overproduction of various hormones.

Multiple endocrine neoplasias are caused by a dominant mutation in either the MEN 1 gene which is a tumor suppressor gene or the RET gene which is a proto oncogene.

A MEN1 mutation causes MEN type 1, and a RET mutation causes MEN type 2A and 2B.

In multiple endocrine neoplasia type 1, there are three types of tumors: pituitary, parathyroid, pancreatic - 3 “p’s”.

But the gastrinomas that occur in the pancreas, are actually more often found in the duodenum, so to keep things accurate 2 “p’s” and a “g” for gastrointestinal gastrinoma is better.

Now in MEN1, the most common tumors are parathyroid tumors which create excess parathyroid hormone. That leads to bone breakdown, which causes serum calcium levels to rise, and that allows calcium kidney stones to form. This is similar to what happens in an isolated parathyroid adenoma.

The key differences are that MEN1-associated hyperparathyroidism has an earlier age of onset, around 25 years, and tends to affect 3 or 4 of the parathyroid glands, rather than just one.

A silver lining is that MEN1 associated hyperparathyroidism almost never progresses to a malignant parathyroid cancer.

Management of hyperparathyroidism can be surgical or nonsurgical.


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