Endo - Pathology (Malignancy) Flashcards
Pg. 329 in First Aid 2014 Sections include: -Carcinoid syndrome -Zollinger-Ellison syndrome -Multiple endocrine neoplasias
Is carcinoid syndrome common or rare? What causes/defines it, and what is secreted in it?
Rare syndrome caused by carcinoid tumors (neuroendocrine cells), especially metastatic small bowel tumors, which secrete high levels of serotonin (5-HT)
In what setting is carcinoid syndrome not seen, and why?
Not seen if tumor is limited to GI tract (5-HT undergoes first-pass metabolism in liver)
What are 4 symptoms/conditions that result from carcinoid syndrome?
Results in (1) recurrent diarrhea, (2) cutaneous flushing, (3) asthmatic wheezing, and (4) right-sided valvular disease.
What are 2 lab findings associated with carcinoid syndrome?
(1) High 5-hydroxyindoleacetic (5-HIAA) in urine (2) Niacin deficiency (pellagra)
What are 2 treatments for carcinoid syndrome?
Treatment: resection, somatostatin analog (e.g., octreotide)
What is the rule associated with carcinoid syndrome?
Rule of 1/3’s: 1/3 metastasize, 1/3 present with 2nd malignancy, 1/3 are multiple
What is the most common malignancy in the small intestine?
Carcinoid (tumor) syndrome
What is pathophysiology of Zollinger-Ellison syndrome? Where does it arise versus damage?
Gastrin-secreting tumor of pancreas or duodenum. Acid hypersecretion causes recurrent ulcers in distal duodenum and jejunum.
What are 2 symptoms with which Zollinger-Ellison syndrome presents?
Presents with (1) abdominal pain (peptic ulcer disease, distal ulcers), (2) Diarrhea (malabsorption).
With what condition may Zolinger-Ellison syndrome be associated?
May be associated with MEN 1
What does MEN stand for? What are the types of MEN?
Multiple endocrine neoplasias; (1) MEN 1 (Wermer syndrome) (2) MEN 2A (Sipple syndrome) (3) MEN 2B
What is another name for MEN 1? What 3 kinds of tumors characterize it?
MEN 1 (Wermer syndrome); (1) Parathyroid tumors (2) Pituitary tumors (prolactin or GH) (3) Pancreatic endocrine tumors - Zollinger-Ellison syndrome, Insulinomas, VIPomas, Glucagonomas (rare)
What are 4 examples of pancreatic endocrine tumors that may characterize MEN 1? Which of these is particularly rare?
Pancreatic endocrine tumors - Zollinger-Ellison syndrome, Insulinomas, VIPomas, Glucagonomas (rare)
Besides tumors, what are 2 additional conditions/symptoms with which MEN 1 commonly presents?
Commonly presents with kidney stones and stomach ulcers
What is another name for MEN 2A? What are 3 tumors associated with it?
MEN 2A (Sipple syndrome); (1) Medullary thyroid carcinoma (secretes calcitonin) (2) Pheochromocytoma (3) Parathyroid hyperplasia
What are 3 tumors associated with MEN 2B?
(1) Medullary thyroid carcinoma (secretes calcitonin) (2) Pheochromocytoma (3) Oral/intestinal ganglioneuromatosis (mucosal neuromas)
Besides tumors, with what condition is MEN 2B associated?
Associated with marfanoid habitus
What does medullary thyroid carcinoma secrete? With which type(s) of MEN is it associated?
Medullary thyroid carcinoma (secretes calcitonin); MEN 2A (Sipple syndrome) & 2B
What are the 2 kinds of pituitary tumors associated with a MEN? Which MEN is it?
Pituitary tumors (prolactin or GH); MEN 1 (Wermer syndrome)
What is the learning/memorization method for distinguishing the characteristic tumors that define each type of MEN?
(1) MEN 1 = 3 P’s (from cepahlad to caudad: Pituitary, Parathyroid, and Pancreas); remember by drawing a diamond (2) MEN 2A = 2 P’s (Parathyroids & Pheochromocytoma); remember by drawing a square (3) MEN 2B = 1 P (Pheochromocytoma); remember by drawing a triangle; See p. 329 in First Aid 2014 at bottom of page for visuals
What is the mode of inheritance for the MEN syndromes?
All MEN syndromes are autosomal dominant inheritance; Think: “All MEN are DOMINANT (or so they think)”
What is the gene mutation associated with 2 of the MEN syndromes? Which 2 are these?
Associated with ret gene mutation in 2A and 2B
