ENDO NEOPLASM DISORDERS- MEN syndromes, Neoplastic ds

Question 1

MEN syndromes
- define
- affect what organs/glands

Answer 1

comprises 3 genetically distinct inherited fam ds to develop multiple endocrine tumors
- autosomal dom w incomp penetrance
- S/Sx at any age
- affect pituitary, thyroid, parathyroids, adrenals, pancreas

Question 2

3P’s for MEN 1 (wermer syndrome)

Answer 2

Parathyroid, Pancreatic, Pituitary
- parathyroid hyperplasia (hyperthyroid)
- pancreatic islet cell tumors–> ZES (zollinger ellison) or insulinoma
- pituitary tumors

Question 3

MEN type IIA (sipple syndrome)

Answer 3

Medullary, Pheochromo, parathyroid
- medullary thyroid carcinoma
- parathyroid hyperplasia (hyperparathyroid)
- pheochromocytoma

Question 4

MEN type IIB

Answer 4

tumors same as 2A minus parathyroids
- + multiple neuromas (tumor of nerve tissue skin dn mouth)
- + marfanoid

Question 5

MMMP for MEN IIB

Answer 5

• Mucosal neuromas (oropharynx, larynx, nasopharynx, conjunctiva)
• medullary thyroid carcinoma (more aggressive in 2a)
• marfanoid body habitus
• pheochromocytoma

Question 6

consequence of ca in body that releases hormones or cytokines

Answer 6

ectopic PTH like secretion— inc blood Calcium
cushings syndrome (ectopic ACTH)—-HTN, elevated gluc, cushing sx

SIADH— water retention, low NA, conc urine