Sunday, March 6, 2016

How to Remember the MEN Syndromes

MEN1: Lots of "P"s and Zollinger ElliSON. (Say it this way to emphasize the rhyme) (Pituitary, Prolactinoma, Pancreatic and Zolinger Ellson Syndrome, explained below).

MEN2A: Tee Pee Phay (Thyroid, Parathyroid and Pheochromocytoma Neoplasias)

MEN2B: Tee Phay Gee (Thyroid, Pheochromocytoma, Ganglioneuroma Neoplasias)

Using this mnemonic, keep straight the Multiple Endocrine Neoplasia Syndromes and the major Organs they affect. There's also a figure most people use with a triangle or something, but I always found that confusing so I won't bother with it. Rhymes are easier for my mind at least. 

In detail, the syndromes per an amalgam of online resources:

Multiple endocrine neoplasia type 1 (MEN-1 syndrome) aka Wermer's syndrome is characterized by the following disorders:
  • Overactivity of the Parathyroid gland (hyperparathyroidism) is the most common sign of this disorder. Hyperparathyroidism disrupts the normal balance of calcium in the blood, which can lead to kidney stones, thinning of the bones (osteoporosis), high blood pressure (hypertension), loss of appetite, nausea, weakness, fatigue, and depression.
  • Neoplasia in the Pituitary gland can manifest as Prolactinomas whereby too much prolactin is secreted, suppressing the release of gonadotropins, causing a decrease in sex hormones such as testosterone. Pituitary tumor in MEN1 can be large and cause signs by compressing adjacent tissues.
  • Pancreatic tumors associated with MEN-1 usually form in the beta cells of the islets of Langerhans, causing over-secretion of insulin, resulting in low blood glucose levels (hypoglycemia). However, many other tumors of the pancreatic Islets of Langerhans can occur in MEN-1. One of these, involving the alpha cells, causes over-secretion of glucagon, resulting in a classic triad of high blood glucose levels (hyperglycemia), a rash called necrolytic migratory erythema, and weight loss. 
  • Another is a tumor of the non-beta islet cells, known as a gastrinoma, which causes the over-secretion of the hormone gastrin, resulting in the over-production of acid by the acid-producing cells of the stomach (parietal cells) and a constellation of sequelae known as Zollinger-Ellison syndrome. Zollinger-Ellison syndrome may include severe gastric ulcers, abdominal pain, loss of appetite, chronic diarrhea, malnutrition, and subsequent weight loss.
There are three subtypes of MEN 2: Familial Medullary Thyroid Cancer (FMTC), MEN 2A, and MEN 2B. ll are due to an autosomal dominant ret mutation.


Familial Medullary Thyroid Cancer (FMTC): 
In this form of MEN 2, patients present with only medullary thyroid carcinoma. (not covered by my mnemonic.)

Multiple endocrine neoplasia, type 2A (MEN 2A), aka Sipple Syndrome, is a hereditary syndrome characterized by
medullary carcinoma of the Thyroid,
hyperParathyroidism, 

pheochromocytoma,
and occasionally cutaneous lichen amyloidosis.


MEN 2B may have:
Medullary Thyroid cancer
Pheochromocytoma (generally benign tumor in the adrenal glands)
Mucosal Ganglioneuroma (benign tumor of nerve tissue on the tongue and lips)
Digestive problems
Muscle, joint, and spinal problems
Certain facial features typical of MEN 2B also include swollen lips and thick eyelids


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