Pancreatic neuroendocrine tumor history and symptoms: Difference between revisions
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{{Pancreatic neuroendocrine tumor}} | {{Pancreatic neuroendocrine tumor}} | ||
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==Symptoms== | |||
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. | |||
Functional tumors are often classified by the hormone most strongly secreted, for example: | |||
* [[gastrinoma]]: the excessive [[gastrin]] causes [[Zollinger–Ellison syndrome]] (ZES) with [[peptic ulcer]]s and [[diarrhea]] | |||
* [[insulinoma]]: [[hypoglycemia]] occurs with concurrent elevations of [[insulin]], [[proinsulin]] and [[C peptide]] | |||
* [[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 | |||
* [[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) | |||
* [[somatostatinoma]]: these rare tumors are associated with elevated blood glucose levels, [[achlorhydria]], [[cholelithiasis]], and [[diarrhea]] | |||
* 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 | |||
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 18:58, 17 August 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]
Symptoms
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.[1][medical citation needed] About 40%[medical citation needed] of PanNETS have symptoms related to excessive secretion of hormones or active polypeptides and are accordingly labeled as "functional"; the symptoms reflect the type of hormone secreted, as discussed below. Up to 60%[medical citation needed] 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.
Functional tumors are often classified by the hormone most strongly secreted, for example:
- gastrinoma: the excessive gastrin causes Zollinger–Ellison syndrome (ZES) with peptic ulcers and diarrhea
- insulinoma: hypoglycemia occurs with concurrent elevations of insulin, proinsulin and C peptide
- glucagonoma: the symptoms are not all due to glucagon elevations, and include a rash, sore mouth, altered bowel habits, venous thrombosis, and high blood glucose levels
- VIPoma, producing excessive vasoactive intestinal peptide, which may cause profound chronic watery diarrhea and resultant dehydration, hypokalemia, and achlorhydria (WDHA or pancreatic cholera syndrome)
- somatostatinoma: these rare tumors are associated with elevated blood glucose levels, achlorhydria, cholelithiasis, and diarrhea
- less common types include ACTHoma, CRHoma, calcitoninoma, GHRHoma, GRFoma, and parathyroid hormone–related peptide tumor
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.