Study your flashcards anywhere!

Download the official Cram app for free >

  • Shuffle
    Toggle On
    Toggle Off
  • Alphabetize
    Toggle On
    Toggle Off
  • Front First
    Toggle On
    Toggle Off
  • Both Sides
    Toggle On
    Toggle Off
  • Read
    Toggle On
    Toggle Off

How to study your flashcards.

Right/Left arrow keys: Navigate between flashcards.right arrow keyleft arrow key

Up/Down arrow keys: Flip the card between the front and back.down keyup key

H key: Show hint (3rd side).h key

A key: Read text to speech.a key


Play button


Play button




Click to flip

86 Cards in this Set

  • Front
  • Back
what do cells in the APUD system (distributed throughout body) have in common (4)?
1) biochemical characteristics; 2) morphology; 3) function; 4) embryogenesis
what does APUD stand for?
amine precursor uptake and decarboxylation
what does this system use as biochemical precursor substrates (2)?
1) dopa; 2) 5-HT
what does this system secrete (2 types of molecules)?
1) LMW polypeptides; 2) bioactive amines
how is the morphology of APUD cells similar in LM?
similar cytology and pattern of growth
what stains can all APUD cells take up (2)?
10 silver; 2) chromogranin
how are APUD cells similar on EM?
all have cytoplasmic dense core (neurosecretory) granules
how are many APUD tumors identified?
due to excess secretion of biologically active substances, with resultant clinical syndromes (many APUD cells have endocrine function)
what cells do all APUD cells arise from embryologically (2)?
1) neuroectodermal; 2) gut-related endodermal
what is the incidence of carcinoid tumors?
1-2 / 10^5
what is the M:F ratio?
2 to 3
what is the most common age?
50, although any age is possible
77% of what tumor site are carcinoid tumors?
what is the most common site of carcinoid tumors, and what % come from this site?
lungs, bronchi, and trachea - 32.7 %
what is the next most common site?
ileum - 17.6 (followed by rectum - 10.1%, and appendix - 7.6%) - overall, more occur in the GIT than the respiratory system - other sites are the stomach, duodenum, jejunum, cecum, and colon
what does metastatic potential of carcinoid tumors correllate best with?
size and location (rather than histologic appearance)
what location has the highest % localized at diagnosis?
appendix - 98%
what % are localized in the small intestine, colon, rectum & sigmoid, and lungs?
SI - 40%, colon - 30%, rectum and sigmoid - 85%, lungs - 80%
what is the rate of metastasis if the tumor is <2cm, and >2cm?
if <2cm, mets unusual (<10%), if >2cm mets common (>80%)
what % of carcinoids are multiple?
up to 20%
what is the growth pattern of carcinoids?
slow growing - even with liver metastases, 5 year survival is >20%
what blood tests can be used to follow disease status (2)?
1) serum chromogranin levels; 2) urine 5-H1AA
what % of patients are positive for urine 5-H1AA?
what % of carcinoid tumors are associated with non-carcinoid tumors?
roughly 10%
what are carcinoid syndromes related to?
secretion of active substances (serotonin, etc.)
what substances were mentioned to be involved in carcinoid syndromes of midgut (2)?
1) serotonin; 2) kaliikrein
what problems were said to occur in carcinoid syndromes (4)?
1) flushing; 2) cramping abdominal pain/diarrhea; 3) asthma; 4) endocardial fibrosis
what % get asthma?
what % get endocardial fibrosis, and what is it (2 problems)?
2/3 of patients with carcinoid syndromes develop carcinoid heart disease: 1) right sided endocardial fibrosis; 2) tricuspid insufficiency
what GI origin was said to cause an atypical carcinoid syndrome?
foregut origin
what may effects of these foregut tumors be related to and why?
many effects may be histamine ralted, because these tumor cells lack decarboxylase activity, and they don't make serotonin, and patients don’t excrete 5-H1AA
what effects may be seen from histamine (4)?
1) prolonged dark flush; 2) facial edema and tearing; 3) profound vasodilation; 4) shock
what must tumors in the gut get past for carcinoid syndrome to take place?
bioactive substances must get past the liver - if the tumor is in the portal circulation, liver metastases must exist
when may carcinoid syndromes not be related to metastatic disease?
when they occur outside the portal circulation
what type of carcinoid was said not to cause carcinoid syndrome, and why?
hindgut carcinoid, because it does not secrete bioactive substances
what % of autopsies turn up islet cell tumors?
1.5% (but clinical syndromes are much less common)
why are most malignant and benign islet cell tumors found?
because of symptoms related to the secretion of bioactive substances (80% of malignant tumors and all benign tumors/hyperplasias are found this way)
what are four things that favor malignancy in islet cell tumors?
1) not an insulinoma; 2) >2cm; 3) angioinvasion; 4) Ki-67 more than 2%
what is the most common islet cell tumor?
what % are benign, what % are multiple or hyperplasia, and what % are malignant?
80% benign, 10% multiple or hyperplasia, 10% malignant
what is diagnosis of insulinoma related to (2)?
1) hypoglycemia; 2) resultant sympathetic stimulation
what is the name of the triad that helps diagnose insulinoma, and what are the three things this triad consists of?
Whipple's Triad - 1) attacks precipitated by fasting with; 2) blood sugards < 50mg/100ml; 3) relieved by glucose
what is the second most common islet cell tumor, and where do most occur?
gastrinoma - most (85%) are pancreatic, 13% are duodenal
what % are benign, what % are multiple or hyperplasia, and what % are malignant?
20% benign, 20% multiple or hyperplasia, 60% malignant
what syndrome is involved with gastrinomas?
Zollinger-Ellison syndrome
how can Z-E syndrome cause diarrhea, and what % get it?
damages intestinal mucosa by hypersecretion of gastric acid - 33%
what % of Z-E sufferers get peptic ulcer disease and what are ulcers like (severity, location)?
25% - fulminant, recurrent, resistant peptic ulceration - ulceration may be more distal than usual
what other problems occur in Z-E syndrome, in order of frequency (4)?
1) pain (95%); 2) G.I. bleeding (40%); 3) vomiting (26%); 4) perforation (18%)
what things are used to diagnose gastrinoma?
1) elevated serum gastrin (3-30x / nl); 2) elevated gastric secretion (both acid and volume) not affected by stimulation
what is the treatment for gastrinoma (4)?
1) remove tumors (if possible); 2) H2 blocker or related drugs; 3) antineoplastic drugs; 4) total gastrectomy
what is the mortality of gastrinoma, if untreated?
what % of patients died of neoplastic progression, in the past?
only 20%
what is the prognosis with aggressive medical/surgical management?
10-year survival may be greater than 90%
what are two rare types of islet cell tumors?
1) glucagonomas; 2) diarrheogenic
what problems can glucagonomas cause in the GIT (3)?
1) constipation; 2) ileus; 3) ascites
what other problems can glucagonomas cause (2)?
1) diabetes mellitus (usually not severe); 2) dermatitis (necrolytic migratory erythema)
what are diarrheogenic islet cell tumors?
non-B islet cell tumors
what syndrome is associated with diarrheogenic carcinoid tumors, and what else is it known as?
Verner-Morrison syndrome - WDHA
what does WDHA stand for?
Watery Diarrhea, Hypokalemia, Achlorhydria
what other tumors are there rare reports of?
islet cell tumors secreting other peptides such as CCK and somatostatin with resultant clinical manifestations
how are all MEN syndromes inherited?
autosomal dominant inheritence
what are alternate names for MEN I (2)?
1) pluriglandular; 2) Wermer's
what are alternate names for MEN II (2)?
1) Sipples; 2) IIA
what is an alternate name for MEN III?
what are the most common tumor sites of involvement in MEN-I (3)?
1) pituitary (65%); 2) parathyroid (88%); 3) pancreas (islet cell tumor - 81%)
what are the most common tumor sites of involvement in MEN-II (3)?
1) parathyroid (45%); 2) medullary CA (75-100%); 3) pheochromocytoma (40-75%)
what are the most common tumor sites of involvement of MEN-III?
1) medullary CA (94%); 2) pheochromocytoma (41%); 3) ganglioneuromatosis
what is the most common islet cell tumor in MEN-I?
what is parathyroid disease like in MEN-I (90 % of the time)?
four gland hyperplasia
what % of patients with Z-E syndrome have MEN-I?
where is the MEN-I gene located (chromosome)?
chromosome 11
what should be remembered about tumors in both II and III (2)?
1) pheochromocytoma often bilatera/extra-adrenal; 2) medullary carcinoma usually multifocal
what area is never involved in MEN-III?
what should be remembered about tumors that occur in III?
1) medullary carcinoma is very aggressive (more so than in sporadic or II); 2)
what else is common in MEN-III (not a tumor)?
skeletal anomalies are common (81%)
what gene is responsible for MEN II, II, and familial medullary carcinoma of the thyroid, and what chromosome is it located on?
RET proto-oncogene, located on chromosome 10
so, what tumors do II and III involve, that MEN-I does not (2)?
1) medullary CA; 2) adrenal medulla (pheochromocytoma)
what do MEN I and II involve, but not III?
parathyroid glands