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1/12/2016 CharacteristicsofpancreaticneuroendocrinetumorsUpToDate

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Characteristicsofpancreaticneuroendocrinetumors

Tumor Pancreatic MEN1 Incidence


Clinical Malignancy
(secreted localization associated inMEN1
presentation (percent)
product) (percent) (percent) (percent)
Insulinoma Whipple'striad* >97 <10 510 21
(insulin)

Gastrinoma ZollingerEllison 2560 6090 2030 54


(gastrin) syndrome

VIPoma VernerMorrison >90 4070 6 17


(vasoactive syndrome
intestinal
polypeptide)

Glucagonoma Glucagonoma >95 5080 120 3


(glucagon) syndrome

Somatostatinoma Somatostatinoma 55 >70 4550 <5


(somatostatin) syndrome

*Hypoglycemicsymptoms,lowbloodglucoselevels,reversibleuponglucoseintake.
Diarrhea,hypergastrinemia,gastricacidhypersecretion,pepticulcerdiathesis.
WDHAsyndrome:waterydiarrhea,hypokalemia,achlorhydria.
4Dsyndrome:necrolyticmigratingerythematousdermatitis,diabetes,deepveinthrombosis,depression.
(Questionable)elevatedsomatostatinserumlevels,diabetes,hypochlorhydria,cholelithiasis,dia/steatorrhea,
anemia,weightloss.

Datafrom:
1.JensenRT.Pancreaticendocrinetumors:Recentadvances.AnnOncol199910:170.
2.EhehaltF,SaegerHD,SchmidtCM,GrtzmannR.Neuroendocrinetumorsofthepancreas.Oncologist
200914:456.

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