Chapter 45: Carcinoid Tumors Flashcards

1
Q

What is a carcinoid tumor?

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”?

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

What are the common sites of occurrence?

A

Think “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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4
Q

What are the signs/symptoms?

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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5
Q

Why SBO with carcinoid?

A

Classically = severe mesenteric fibrosis

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6
Q

What are the pellagra-like symptoms?

A
  1. Dermatitis
  2. Diarrhea
  3. Dementia
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7
Q

What causes pellagra in carcinoid patients

A

Decreased niacin production

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8
Q

What is carcinoid syndrome?

A

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

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9
Q

What are the symptoms of carcinoid syndrome?

A

Remember the acronym “B FDR”:

  • Bronchospasm
  • Flushing (skin)
  • Diarrhea
  • Right-sided heart failure (from valve failure)
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10
Q

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

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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11
Q

Classic cardiac complication with carcinoid syndrome?

A

Tips:

  1. Tricuspid Insufficiency
  2. Pulmonary Stenosis
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12
Q

What is the incidence of carcinoid SYNDROME in patients who havea 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?

A

Odansetron (Zofran®)—serotonin antagonist

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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 the GI tract, portal venous drainage e.g.,
    • ovary
    • testicular
    • bronchus
19
Q

What does the liver break down serotonin into?

A

5-hydroxyindoleacetic acid (5-HIAA)

20
Q

What percentage of patients with a carcinoid have an elevated urine5-HIAA level?

21
Q

What are the associated diagnostic lab findings?

A

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

22
Q

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

A

Pentagastrin stimulation

23
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
24
Q

What are the special radiologic (scintigraphy) localization tests?

A
  • 131I-MIBG (131 metaiodobenzylguanidine)
  • 111In-octreotide
  • PET scan utilizing 11C-labeled HTP
25
What is the surgical treatment?
* Excision of the primary tumor and single or feasible metastasis in the liver (livertransplant is an option with unresectable liver metastasis) * chemotherapy for advanced disease
26
What is the medical treatment?
Medical therapy for palliation of the carcinoid syndrome (serotonin antagonists,somatostatin analogue [octreotide]
27
How effective is octreotide?
It relieves diarrhea and flushing in more than 85% of cases and may shrinktumor in 10% to 20% of cases
28
What is a common antiserotonin drug?
Cyproheptadine
29
What is the overall prognosis?
Two thirds of patients are alive at 5 years
30
What is the prognosis of patients with liver metastasis or carcinoidsyndrome?
50% are alive at 3 years
31
What does carcinoid tumor look like?
Usually intramural bowel mass; appears as yellowish tumor upon incision
32
For appendiceal carcinoid, when is a right hemicolectomy indicated versus an appendectomy?
* if the tumor is \>1.5 cm * right hemicolectomy is indicated * if there are no signsof serosal or cecal involvement and tumor is \<1.5 cm * appendectomy should be performed
33
Which primary site has the highest rate of metastasis?
Ileal primary tumo
34
Can a carcinoid tumor be confirmed malignant by looking at the histology?
No, metastasis must be present to diagnose malignancy
35
What is the correlation between tumor size and malignancypotential?
* Vast majority of tumors \<2 cm are benign * in tumors \>2 cm, malignancy potential is significant
36
What treatments might you use for the patient with unresectableliver metastasis that is refractory to medical treatment
Chemoembolization or radiofrequency ablation
37
Name the diagnostic modality: 50-year-old male with history of flushing, diarrhea, JVD with echorevealing right-sided heart failure
24-hour urinary 5-HIAA (5-hydroxyindoleacetic acid) level to work up carcinoid syndrome