Carcinoid Tumors, C46 P301-305 Flashcards

1
Q

What is a carcinoid tumor?

P301

A

Tumor arising from neuroendocrine cells
(APUDomas), a.k.a. Kulchitsky cells;
basically, a tumor that secretes serotonin

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2
Q

Why is it called “carcinoid”?

P301

A

Suffix “-oid” means “resembling”; thus,
carcinoid resembles a carcinoma but is
clinically and histologically less aggressive
than most GI carcinomas

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3
Q

How can you remember that
Kulchitsky cells are found in
carcinoid tumors?
P301

A

Think: “COOL CAR” or KULchitsky

CARcinoid

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4
Q

What is the incidence?

P301

A

Between 0.2% and 1.0% and about 25%

of all small bowel tumors

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5
Q

What are the common sites
of occurrence?
P301

A
“AIR”:
1. Appendix (most common)
2. Ileum
3. Rectum
4. Bronchus
Other sites: jejunum, stomach,
duodenum, colon, ovary, testicle,
pancreas, thymus
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6
Q

What are the signs/
symptoms?
P301

A
Depends on location; most cases are
asymptomatic; also SBO, abdominal
pain, bleeding, weight loss, diaphoresis,
pellagra skin changes, intussusception,
carcinoid syndrome, wheezing
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7
Q

Why SBO with carcinoid?

P301

A

Classically = severe mesenteric fibrosis

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8
Q

What are the pellagra-like
symptoms?
P302

A

Think “3-D”:

  1. Dermatitis
  2. Diarrhea
  3. Dementia
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9
Q

What causes pellagra in
carcinoid patients?
P302

A

Decreased niacin production

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10
Q

What is carcinoid syndrome?

P302

A

Syndrome of symptoms caused by release

of substances from a carcinoid tumor

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11
Q

What are the symptoms of
carcinoid syndrome?
P302

A

Remember the acronym “B FDR”:
Bronchospasm

Flushing (skin)
Diarrhea
Right-sided heart failure (from valve
   failure)
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12
Q

What is a complete memory
aid for carcinoid?
P302 (picture)

A

Think: B FDR = CARcinoid, or “Be FDR

in a cool CAR” (COOL= KULchitsky cells)

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13
Q

Why does right-sided heart
failure develop but not
left-sided heart failure?
P302

A

Lungs act as a filter (just like the liver);
thus, the left heart doesn’t see all the
vasoactive compounds

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14
Q
What is the incidence of
carcinoid SYNDROME in
patients who have a
carcinoid TUMOR?
P302
A

≈10%

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15
Q

What released substances
cause carcinoid syndrome?
P302

A

Serotonin and vasoactive peptides

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16
Q

What is the medical treatment
for carcinoid syndrome?
P302

A

Octreotide IV

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17
Q

What is the medical
treatment of diarrhea alone?
P302

A

Odansetron (Zofran®)—serotonin

antagonist

18
Q

How does the liver prevent
carcinoid syndrome?
P303

A

By degradation of serotonin and the
other vasoactive peptides when the
tumor drains into the portal vein

19
Q

Why does carcinoid
syndrome occur in some
tumors and not in others?
P303

A

Occurs when venous drainage from the
tumor gains access to the systemic circulation
by avoiding hepatic degradation
of the vasoactive substances

20
Q

What tumors can produce
carcinoid syndrome?
P303

A
Liver metastases
Retroperitoneal disease draining into
    paravertebral veins
Primary tumor outside the GI tract,
    portal venous drainage (e.g., ovary,
    testicular, bronchus), or both
21
Q

What does the liver break
down serotonin into?
P303

A

5-hydroxyindoleacetic acid (5-HIAA)

22
Q

What percentage of patients
with a carcinoid have an
elevated urine 5-HIAA level?
P303

23
Q

What are the associated
diagnostic lab findings?
P303

A

Elevated urine 5-HIAA as well as
elevated urine and blood serotonin
levels

24
Q

How do you remember
5-HIAA for carcinoid?
P303 (picture)

A

Think of a 5-HIGH CAR pile up =

5-HIAA CARcinoid

25
``` What stimulation test can often elevate serotonin levels and cause symptoms of carcinoid syndrome? P303 ```
Pentagastrin stimulation
26
How do you localize a GI carcinoid? P304
Barium enema, upper GI series with small bowel follow-through, colonoscopy, enteroscopy, enteroclysis, EGD, radiology tests
27
What are the special radiologic (scintigraphy) localization tests? P304
I-MIBG (131 metaiodobenzylguanidine) In-octreotide PET scan utilizing C-labeled HTP
28
What is the surgical treatment? P304
Excision of the primary tumor and single or feasible metastasis in the liver (liver transplant is an option with unresectable liver metastasis); chemotherapy for advanced disease
29
What is the medical treatment? P304
Medical therapy for palliation of the carcinoid syndrome (serotonin antagonists, somatostatin analogue [octreotide])
30
How effective is octreotide? | P304
It relieves diarrhea and flushing in more than 85% of cases and may shrink tumor in 10% to 20% of cases
31
What is a common antiserotonin drug? P304
Cyproheptadine
32
What is the overall prognosis? | P304
Two thirds of patients are alive at 5 years
33
What is the prognosis of patients with liver metastasis or carcinoid syndrome? P304
50% are alive at 3 years
34
What does carcinoid tumor look like? P304
Usually intramural bowel mass; appears | as yellowish tumor upon incision
35
``` For appendiceal carcinoid, when is a right hemicolectomy indicated versus an appendectomy? P304 ```
``` If the tumor is >1.5 cm, right hemicolectomy is indicated; if there are no signs of serosal or cecal involvement and tumor is <1.5 cm, appendectomy should be performed ```
36
Which primary site has the highest rate of metastasis? P304
Ileal primary tumor
37
Can a carcinoid tumor be confirmed malignant by looking at the histology? P304
No, metastasis must be present to | diagnose malignancy
38
What is the correlation between tumor size and malignancy potential? P305
Vast majority of tumors 2 cm, malignancy | potential is significant
39
``` What treatments might you use for the patient with unresectable liver metastasis that is refractory to medical treatment? P305 ```
Chemoembolization or radiofrequency | ablation
40
What are the overall survival rates for carcinoid tumors at 5 years? 10 years? P305
5 years = 70%; 10 years = 50%
41
What are the side effects of colorectal carcinoid? P305
Most common side effect is rectal bleeding | +/- vague abdominal pain/discomfort