Functional pancreatic neuroendocrine tumors
This is a brief video on pancreatic neuroendocrine tumors that are functional and secreting excess hormones that have systemic effects.
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ADDITIONAL TAGS:
Functional pancreatic neuroendocrine tumors
“Islet cell tumorsâ€
Neoplasms that arise from the endocrine pancreas that actively secrete hormones to have systemic effects
Gastrinoma
Insulinoma
Glucagonoma
VIPoma
Somatostatinoma
Salt and pepper chromatin -- indicative of neuroendocrine differentiation
By Nephron - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/....w/index.php?curid=78
Gastrinoma
Second most common PNET
Gastrin secreting tumor of the endocrine pancreas (can also occur in the duodenum
Gastrin induces gastric acid secretion
Gastrin normally comes from G cells that are NOT found in endocrine pancreas
Causes recurrent ulcers in the stomach, duodenum, and jejunum (don’t usually get ulcers that far down)
Ulcers don't respond to conventional therapy
Presents with abd pain, diarrhea
Diagnose with failed suppression by secretin, which normally inhibits gastrin release; high blood gastrin levels
Treat with somatostatin (octreotide) or surgical resection
Causes Zollinger-Ellison syndrome
Gastrinoma
Insulinoma
Glucagonoma
VIPoma
Somatostatinoma
Insulinoma
Most common PNET
Insulin secreting tumor of the endocrine pancreas (beta cells)
Presents with hypoglycemia symptoms (hungry, nervous, sweating; progresses to lethargy, confusion, coma)
Whipple’s triad: hypoglycemia (sugar below 55), symptoms of hypoglycemia, symptoms corrected with administration of glucose
Bloodwork shows:
Low glucose
High insulin
High C-peptide
Unlike exogenous use
Pathology: usually small,
Treat with surgical resection
CC BY-SA 3.0, https://commons.wikimedia.org/....w/index.php?curid=50
Gastrinoma
Insulinoma
Glucagonoma
VIPoma
Somatostatinoma
Glucagonoma
Rare tumor; often malignant
Glucagon secreting tumor of the endocrine pancreas (alpha cells)
Bloodwork shows increased glucagon, often increased glucose
Presents with
mild diabetes,
dermatitis called necrolytic migratory erythema,
decreased weight,
decreased RBCs (anemia),
venous thrombosis (DVT),
Treat with somatostatin (octreotide) or surgical resection
Gastrinoma
Insulinoma
Glucagonoma
VIPoma
Somatostatinoma
VIPoma
AKA Verner-Morrison syndrome
Rare tumor, most are malignant
VIP secreting tumor of the endocrine pancreas (D1 cells)
Vasoactive intestinal peptide (VIP)
Normal role in gut is to stimulate secretion of water and electrolytes
Presents as profuse and explosive and watery diarrhea
Results in low HCl (hypochlorhydria), low K, and dehydration
Gastrinoma
Insulinoma
Glucagonoma
VIPoma
Somatostatinoma
Somatostatinoma
Rare tumor, most are malignant
Somatostatin secreting tumor of the endocrine pancreas (D or delta cells)
Somatostatin inhibits: Gastrin, Cholecystokinin (CCK), Secretin, Motilin, Vasoactive intestinal peptide (VIP), Gastric inhibitory polypeptide (GIP), Enteroglucagon
Presents with mild diabetes, gallstones, steatorrhea, hypochlorhydria
Treat with somatostatin (octreotide) or surgical resection
Less common PNETs:
ACTHoma, CRHoma, calcitoninoma, GHRHoma, GRFoma, and parathyroid hormone–related peptide tumor
Gastrinoma
Insulinoma
Glucagonoma
VIPoma
Somatostatinoma
Multiple endocrine neoplasia
Gastrinoma
Insulinoma
Glucagonoma
VIPoma
Somatostatinoma
By Mikael Häggström - Own work, CC0, https://commons.wikimedia.org/....w/index.php?curid=22
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