Mr2414

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MR 2/4/14

Neuroendocine tumors (NETs)

Facts

25% secrete a functional hormoneAerodigestive 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

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

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

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