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==Symptoms==
==Staging==
Some PanNETs do not cause any symptoms, in which case they may be discovered incidentally on a CT scan performed for a different purpose. Symptoms such as abdominal or back pain or pressure, diarrhea, indigestion, or yellowing of the skin and whites of the eyes can arise from the effects of a larger PanNET tumor, either locally or at a [[metastasis]].<ref>Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®) National Cancer Institute [http://www.cancer.gov/cancertopics/pdq/treatment/isletcell/Patient/page1]</ref>{{medical citation needed|date=December 2014}}<!--add Burns etc--> About 40%{{medical citation needed|date=December 2014}}<!--NCCN page 66 states 40 to 91%, with their recent series = 22%--> of PanNETS have symptoms related to  excessive secretion of [[hormone]]s or active [[polypeptide]]s and are accordingly labeled as "functional"; the symptoms reflect the type of hormone secreted, as discussed below. Up to 60%{{medical citation needed|date=December 2014}} of PanNETs are nonsecretory or nonfunctional, in which there is no secretion, or the quantity or type of products, such as [[pancreatic polypeptide]] (PPoma), [[chromogranin]] A, and [[neurotensin]], do not cause a clinical syndrome although blood levels may be elevated. In total, 85% of PanNETs have an elevated blood marker.
The 2010 WHO classification of tumors of the digestive system grades all the [[neuroendocrine tumor]]s into three categories, based on their degree of [[Grading (tumors)|cellular differentiation]] (from well-differentiated "NET G1" through to poorly-differentiated "NET G3"). The NCCN recommends use of the same AJCC-UICC staging system as pancreatic adenocarcinoma. Using this scheme, the stage by stage outcomes for PanNETs are dissimilar to pancreatic exocrine cancers.<ref name="NCI_FIG1">National Cancer Institute. Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®) Incidence and Mortality [http://www.cancer.gov/cancertopics/pdq/treatment/isletcell/HealthProfessional/page1]</ref> A different TNM system for PanNETs has been proposed by The European Neuroendocrine Tumor Society.


Functional tumors are often classified by the hormone most strongly secreted, for example:
<gallery class="center" caption="Pancreatic neuroendocrine tumor staging ([[American Joint Committee on Cancer|AJCC]])">
* [[gastrinoma]]: the excessive [[gastrin]] causes [[Zollinger–Ellison syndrome]] (ZES) with [[peptic ulcer]]s and [[diarrhea]]
File:Pancrea2.png|Stage T1
* [[insulinoma]]: [[hypoglycemia]] occurs with concurrent elevations of [[insulin]], [[proinsulin]] and [[C peptide]]
File:Pancrea3.png|Stage T2
* [[glucagonoma]]: the symptoms are not all due to glucagon elevations, and include a [[Necrolytic migratory erythema|rash]], sore mouth, altered bowel habits, venous [[thrombosis]], and high blood glucose levels
File:Pancrea4.png|Stage T3
* [[VIPoma]], producing excessive [[vasoactive intestinal peptide]], which may cause profound chronic '''<u>w</u>'''atery <u>'''d'''</u>[[diarrhea|iarrhea]] and resultant [[dehydration]], <u>'''h'''</u>[[hypokalemia|ypokalemia]], and '''<u>a</u>'''[[achlorhydria|chlorhydria]] (WDHA or pancreatic cholera syndrome)
File:Pancrea5.png|Stage T4
* [[somatostatinoma]]: these rare tumors are associated with elevated blood glucose levels, [[achlorhydria]], [[cholelithiasis]], and [[diarrhea]]
File:Pancrea5.png|Involvement of nearby lymph nodes – Stage N1
* less common types include [[adrenocorticotropic hormone|ACTHoma]], [[Corticotropin-releasing hormone|CRH]]oma, [[calcitonin]]oma, [[GHRH]]oma, [[Growth hormone-releasing factor|GRFoma]], and [[parathyroid]] hormone–related peptide tumor
File:Pancrea7.png|Metastasis – stage M1
 
</gallery>
In these various types of functional tumors, the frequency of malignancy and the survival [[prognosis]] have been estimated dissimilarly, but a pertinent accessible summary is available.


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Revision as of 19:00, 17 August 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

Staging

The 2010 WHO classification of tumors of the digestive system grades all the neuroendocrine tumors into three categories, based on their degree of cellular differentiation (from well-differentiated "NET G1" through to poorly-differentiated "NET G3"). The NCCN recommends use of the same AJCC-UICC staging system as pancreatic adenocarcinoma. Using this scheme, the stage by stage outcomes for PanNETs are dissimilar to pancreatic exocrine cancers.[1] A different TNM system for PanNETs has been proposed by The European Neuroendocrine Tumor Society.

  • Pancreatic neuroendocrine tumor staging (AJCC)
  • Stage T1

    Stage T1

  • Stage T2

    Stage T2

  • Stage T3

    Stage T3

  • Stage T4

    Stage T4

  • Involvement of nearby lymph nodes – Stage N1

    Involvement of nearby lymph nodes – Stage N1

  • Metastasis – stage M1

    Metastasis – stage M1

References

  1. ↑ National Cancer Institute. Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®) Incidence and Mortality [1]
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