Neuroendocrine tumors (NETs) can secrete functional hormones and commonly metastasize to the liver. Management depends on the specific tumor type and location. Carcinoid tumors of the appendix smaller than 1 cm require only appendectomy, while larger or higher grade tumors warrant right hemicolectomy. Multiple endocrine neoplasia (MEN) syndromes involve specific tumor patterns and genetic mutations. A woman with a 0.8 cm well-differentiated carcinoid tumor in her appendix requires no further intervention after appendectomy.

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2. Neuroendocine tumors (NETs)
MR 2/4/14

3. Facts
25% secrete a functional hormone
Aerodigestive tract = serotonin (carcinoid)
Pancreas = insulin, glucagon, gastrin, somatostatin
and vasoactive intestinal peptide
Nonfunctional tumors tend to be asymptomatic and
are discovered once disease is metastatic
Liver is the #1 site for metastatic disease

4. Management
• Carcinoid
– Most common site for carcinoid is the appendix
•
•
< 1 cm, appendectomy
> 2 cm or high grade features, hemicolectomy
• Since most are well-differentiated, indolent and
metastatic  expectant management and serial
images
–
–
–
Octreotide scan to identify somatostatin receptors
Hepatic artery embolization to decrease tumor bulk and
hormone production
Chemo has minimal activity, but it works better for pancreatic
NETs than for carcinoid

5. MEN syndromes
MEN1: “3Ps” or parathyroid > pancreatic > pituitary
 Tend to get lipomas, angiofibromas and collagenomas on the
back (see above left)
MEN2a: “TAP” or thyroid > adrenal > parathyroid
 Tend to have lichen amyloidosis and Hirschprungs (above
right)
MEN2b: “TAN” or thyroid > adrenal > neuroma
(mucosal or intestinal ganglio)

Tend to have developmental d/o like kyphoscoliosis or
lordosis), joint laxity, Marfanoid habitus
MEN2a, MEN2b and familial medullary thyroid
cancer (MTC) involve the RET proto-oncogene

6. MKSAP Question
 A 40-year-old woman is evaluated for a 36-hour history of fever and central abdominal
pain localized to the right lower quadrant followed by nausea and vomiting. Medical
history is unremarkable, and she takes no medications.
 On physical examination, temperature is 39.1 °C (102.4 °F), blood pressure is 110/75 mm
Hg, pulse rate is 92/min, and respiration rate is 16/min. No skin lesions are present. The
patient has right lower quadrant abdominal pain with tenderness, guarding, and rebound.
The chest is clear to auscultation and percussion. There are no heart murmurs, and no
palpable lymphadenopathy is noted.
 Contrast-enhanced CT scan of the abdomen and pelvis is consistent with acute
appendicitis. No other abnormalities are seen. The patient undergoes a laparoscopic
appendectomy without incident. Pathologic examination shows acute appendicitis with
the finding of a well-differentiated carcinoid tumor, 0.8 cm in maximal diameter. The
patient has fully recovered from her surgery and is back to full activities





A: Indium-111 pentotreotide scan
B: Octreotide
C: Right hemicolectomy
D: Streptozocin plus 5-FU
No further intervention

7. MKSAP Question
 A 40-year-old woman is evaluated for a 36-hour history of fever and central abdominal
pain localized to the right lower quadrant followed by nausea and vomiting. Medical
history is unremarkable, and she takes no medications.
 On physical examination, temperature is 39.1 °C (102.4 °F), blood pressure is 110/75 mm
Hg, pulse rate is 92/min, and respiration rate is 16/min. No skin lesions are present. The
patient has right lower quadrant abdominal pain with tenderness, guarding, and rebound.
The chest is clear to auscultation and percussion. There are no heart murmurs, and no
palpable lymphadenopathy is noted.
 Contrast-enhanced CT scan of the abdomen and pelvis is consistent with acute
appendicitis. No other abnormalities are seen. The patient undergoes a laparoscopic
appendectomy without incident. Pathologic examination shows acute appendicitis with
the finding of a well-differentiated carcinoid tumor, 0.8 cm in maximal diameter. The
patient has fully recovered from her surgery and is back to full activities





A: Indium-111 pentotreotide scan
B: Octreotide
C: Right hemicolectomy
D: Streptozocin plus 5-FU
No further intervention