Carcinoid Tumors Flashcards

1
Q

What is a carcinoid tumor?

A

Tumor arising from neuroendocrine cells (Kulchitsky cells).

Secretes serotonin.

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2
Q

Why is a carcinoid tumor called “carcinoid”?

A

A 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?

A

KUL CAR

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4
Q

What are the common sites for carcinoid tumors?

A

AIR:
Appendix, Ileum, Rectum
Also bronchus, jejunum, stomach, duodenum, colon, ovary, testicle, pancreas, thymus

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5
Q

What are the signs and symptoms of a carcinoid tumor?

A

Depends on the location (most cases are asymptomatic).
SBO, abdominal pain, bleeding, weight loss, diaphoresis, pellagra skin changes, intussusception, carcinoid syndrome, wheezing

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6
Q

Why SBO with carcinoid?

A

Severe mesenteric fibrosis

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7
Q

What are the pellagra-like symptoms of a carcinoid tumor?

A
  1. Dermatitis
  2. Diarrhea
  3. Dementia
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8
Q

What causes pellagra in carcinoid patients?

A

Decreased niacin production

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9
Q

What is carcinoid syndrome?

A

Syndrome of symptoms caused by release of substances from a carcinoid tumor

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10
Q

What are the symptoms of carcinoid syndrome?

A

B FDR:

Bronchospasm, Flushing (skin), Diarrhea, Right-sided heart failure (from valve failure)

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11
Q

Why does right-sided heart failure develop but not left-sided heart failure in carcinoid syndrome?

A

Lungs act as a filter, thus the left heart doesn’t see all the vasoactive compounds

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12
Q

What is the incidence of carcinoid syndrome in patients with a carcinoid tumor?

A

10%

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13
Q

What released substances cause carcinoid syndrome?

A

Serotonin and vasoactive peptides

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14
Q

What is the medical treatment for carcinoid syndrome?

A

Octreotide IV

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15
Q

What is the medical treatment of diarrhea alone in carcinoid syndrome?

A

Odansetron (Zofran)

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16
Q

How does the liver prevent carcinoid syndrome?

A

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

17
Q

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

A

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

18
Q

What tumors can produce carcinoid syndrome?

A

Liver metastases; retroperitoneal disease draining into paravertebral veins; primary tumor outside of GI tract, portal venous drainage, or both

19
Q

What does the liver break down serotonin into?

A

5-HIAA

20
Q

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

A

50%

21
Q

What are the associated diagnostic lab findings with carcinoid tumor?

A

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

22
Q

How do you remember 5-HIAA for carcinoid?

A

5-HIGH CAR pile up

23
Q

What stimulation test can often elevate serotonin levels and cause symptoms of carcinoid syndrome?

A

Pentagastrin stimulation

24
Q

How do you localize a GI carcinoid?

A

Barium enema; upper GI series with small bowel follow-through; colonoscopy; enteroscopy; enteroclysis; EGD; radiology tests

25
Q

What are the special radiologic (scintigraphy) localization tests for carcinoid tumors?

A

I-MIBG; In-octreotide; PET scan utilizing C-labeled HTP

26
Q

What is the surgical treatment for carcinoid tumor?

A

Excision of the primary tumor and single or feasible metastasis in the liver; chemotherapy for advanced disease

27
Q

What is the medical treatment for carcinoid tumor?

A

Palliation of the carcinoid syndrome (e.g. serotonin antagonists, somatostatin analog)

28
Q

How effective is octreotide in treating carcinoid syndrome?

A

It relieves diarrhea and flushing in more than 85% of cases and may shrink tumor in 10-20%

29
Q

What is a common anti-serotonin drug?

A

Cyproheptadine

30
Q

What is the overall prognosis for carcinoid tumor?

A

66% alive at 5 years

31
Q

What is the prognosis of patients with liver metastasis or carcinoid syndrome secondary to carcinoid tumor?

A

50% at 3 years

32
Q

What does a carcinoid tumor look like?

A

Usually intramural bowel mass.

Appears as yellowish tumor upon incision.

33
Q

For appendiceal carcinoid, when is a right hemicolectomy indicated vs. an appendectomy?

A

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.

34
Q

Which primary site of carcinoid tumor has the highest rate of metastasis?

A

Ileal primary tumor

35
Q

Can a carcinoid tumor be confirmed malignant by looking at the histology?

A

No, metastasis must be present to diagnose malignancy

36
Q

What is the correlation between carcinoid tumor size and malignancy potential?

A

Vast majority of tumors < 2 cm are benign.

In tumors > 2 cm, malignancy potential is significant.

37
Q

What treatments might you use for the patient with unresectable liver metastasis that is refractory to medical treatment?

A

Chemoembolization or radiofrequency ablation

38
Q

What are the overall survival rates for carcinoid tumors at 5 years? 10 years?

A

70%; 50%

39
Q

What are the side effects of colorectal carcinoid?

A

Most common side effect is rectal bleeding +/- vague abdominal pain or discomfort