| Affiliation |
(AGL) Chairman, Department of Ophthalmology, The Methodist Hospital, Houston, Texas; Professor of Ophthalmology, Weill Cornell Medicine, New York City, New York; (PD) Class of 2021, Baylor College of Medicine, Houston, Texas |
| Transcript |
So we're going to talk about MEN today, which is Multiple Endocrine Neoplasia syndromes. There's the Number 1 and the Number 2. One of the fascinating things about the Number 1 is that they've often had a Whipple Procedure. And the Number 2 is 2a and 2b, and the 2a is sometimes called the Sipple. So you've got a Whipple and a Sipple, and then we've got this extra one, the 2b. The reason they've got the Wipple and the MEN1 is the characteristic features of pancreatic endocrine neoplasia, and that could be a gastrinoma or insulinoma, and it can produce endocrine manifestations, if it's secreting gastrin, it's the Zollinger-Ellison Syndrome, you might remember that from medical school, which can cause diarrhea. And so the Whipple goes with MEN1. The other two "P"s, and there are three of them in MEN1, is pituitary adenoma and parathyroid hormone. So the three "P"s of MEN1, pituitary adenoma, the parathyroid, and the pancreatic. And the way this comes to us, obviously, is by temporal hemianopsia or vision loss. This one [parathyroid] can come to us as hypercalcemia and band keratopathy, and this one [pancreatic] can cause diarrhea and lead to vitamin deficiencies B12 and folate, producing a central scotoma that is painless and progressive, thiamine deficiency producing Wernicke's [encephalopathy], vitamin A deficiency causing nyctalopia. So the vitamin deficiency things are occurring because patients with the gastrin secretion have diarrhea. In the MEN2a and MEN2b, the things that is linking those two together is medullary carcinoma of the thyroid. And since they almost always have that, they usually have a thyroidectomy. And so that comes to us in Ophthalmology as Horner's Syndrome, post-surgical from the thyroidectomy. And pheochromocytoma that comes to us as hypertension and what we would be looking for is hypertensive retinopathy. Either Grade 1 - arteriolar narrowing, Grade 2 - AV nicking, Grade 3 - hemorrhages, exudates, cottonwool spots, Grade 4- optic disc edema. So hypertension is how the pheo comes to us. And in the patients that have the MEN2b, they have enlarged cranial nerves, and they can have big nerves in other places, big nerves in the cornea, like multiple neuromas including conjunctival and other mucosal neuromas in their mouth and their lips, and they have a marfanoid body habitus. So these 2a and 2b do not have the pituitary adenoma and do not have the pancreatic lesions. So when we're dealing with MEN1, they've had a Whipple, pituitary, parathyroid, and pancreas [lesions]. 2a the Sipple, medullary carcinoma of the thyroid, Horner's Syndrome, pheochromocytoma, Sipple. And [2]b has no name, multiple neuromas, big nerves, mucosal neuromas, marfanoid body habitus, medullary carcinoma [of thyroid], and pheochromocytoma. So you should know how these MEN2 things come to us, and MEN1 pituitary, [parathyroid], and pancreas. |
