Path carcinoid, GI stromal tumor, & lymphoma

Question 1

positive chromogranin stain

Answer 1

carcinoid

Question 2

another name for carcinoid

Answer 2

gastric neuroendocrine tumor

Question 3

carcinoid % association with hypergastrinemia, characteristics

% not associated with hypergastrinemia, characteristics

Answer 3

80% indolent, mets uncommon

10% aggressive, mets at diagnosis, carcinoid syndrome association

Question 4

targeted therapy for CD 117/C-KIT

Answer 4

imatinib

Question 5

immunostain that can identify liver met

Answer 5

gastrin stain

Question 6

zollinger-ellison syndrome dx criteria (2)

Answer 6

fasting gastrin level >1000 pg/ml, gastric pH 200 pg/ml (83% Sn, 100% Sp)

Question 7

zollinger-ellison syndrome tx

Answer 7

solitary, non-met disease: surg, high dose PPI, long acting somatostatin analog

met disease: streptozocin/doxorubicin or temozolomide

Question 8

intestinal neuroendocrine tumor presentation

Answer 8

late middle-aged pt
ileum & appendix
multiple
abdominal pain
intermittent obstruction
rectal tumors small, rarely met

Question 9

carcinoid syndrome

Answer 9

flushing (95%), diarrhea, wheezing, colicky abd pain, right heart endocardial fibrosis (50%)

10% of carconoid tumor pts, typically b/c of liver mets

high urine 5-hydroxyindole acetic acid (5-HIAA) (limited Sn/Sp)

Question 10

zollinger-ellison syndrome

Answer 10

gastrinoma of pancreas or duodenum

Question 11

colorectal neuroendocrine tumor

Answer 11

colon - large, right mass

rectum - found incidentally, carcinoid rare,

Question 12

GIST

Answer 12

gastrointestinal stromal tumors

Question 13

GIST epidemiology, location, molecular

Answer 13

uncommon, older adults

stomach, SI

derive from interstitial cells of Cajal, GI pacemaker cells.

mutation in KIT tm TK, activating it. positive immunostain CD117

regarded as malignant or possibly malignant

Question 14

GIST presentation, dx

Answer 14

overt GI bleeding (40%)
abdominal mass
abdominal pain (20%)

CT detection, biopsy false negative,
FINA (82% Sn, 100% Sp)

Question 15

tx of GIST

Answer 15

surg, imatinib mesylate

Question 16

PTLD

Answer 16

post-transplant lymphoproliferative disorder

Question 17

PTLD etiology

Answer 17

EBV-infected b cells proliferate.

Detection of EBV serology for DNA

Question 18

GI lymphoma

Answer 18

rare, older adults
70% in stomach, 30% small bowel
H pylori associated, autoimmune disease
50% low grade MALTomas, the rest are diffuse B-cell lymphomas

germinal centers in mucosa, lamina propria

Question 19

3 types of GI lymphoma

Answer 19

low grade MALToma
IPSIL malabsorption presentation
T-cell related increased sprue risk

Question 20

GI lymphoma presentation, dx, tx, prognosis

Answer 20

epigastric pain (93%), anorexia (47%), weight loss (25%), occult bleeding (19%)
Dx endoscopy & biopsy
Tx antibiotics for MALToma, rad/chemo for other

prognosis good for MALToma, bad for others.