Endocrine Pancreas Flashcards
The pancreas develops from the (foregut/midgut/hindgut)
Foregut (duodenum)
The pancreas develops from the (endoderm/mesoderm/ectoderm)
Endoderm
The pancreas actually forms from the fusion of a Ventral and Dorsal bud (True or False)
True; failure to fuse causes Pancreatic Divisum
What ducts drain directly into the Main Pancreatic Duct (2 total)
Ampulla of Vater
Common Bile Duct
Rare condition in which the duodenum is surrounded by a ring of pancreatic tissue continuous with the head of the pancreas; can constrict the duodenum and block or impair the flow of food to the rest of the intestines
Annular Pancreas
Failure of the Ventral and Dorsal ducts of the pancreatic buds to fuse; can make prone to pancreatitis due to obstruction
Pancreatic Divisum
The pancreas is (intraperitoneal/retroperitoneal)
Retroperitoneal
The pancreas is (anterior/posterior) to the Aorta and IVC
Anterior
The Islets of Langerhans compose _____ % of the pancreatic mass
1-2
D1 cells of the pancreas secrete what hormone
Vasoactive Intestinal Polypeptide
Enterochromaffin cells of the Pancreas secrete what hormone
Serotonin
Epsilon cells of the Pancreas secrete what hormone
Ghrelin
Cells of the Islets of Langerhans; compose 20% of the islet; synthesize and secrete Glucagon; located along periphery of islet
Alpha
Cells that line the majority of the periphery of the Islets of Langerhan
Alpha (glucagon)
Cells of the Islets of Langerhans; compose 70% of the islet; synthesize and secrete Insulin; located in the center of islet
Beta
Cells that compose the majority of the center of the Islets of Langerhan
Beta (insulin)
Type (1/2) Diabetes Mellitus is hyperglycemia associated with the autoimmune destruction of Beta cells; little to no insulin; LEAST common; onset BEFORE age 20
Type 1
Type (1/2) is autoimmune
Type 1
Type (1/2) Diabetes Mellitus is hyperglycemia usually associated with obesity and genetics; combination of Insulin resistance; onset AFTER age 20
Type 2
Type (1/2) is usually associated with obesity
Type 2
Type (1/2) Diabetes Mellitus is more common
Type 2
Lymphocytic infiltrate in and around the Islets of Langerhan; seen in Type 1 Diabetes Mellitus
Insulitis
Type 1 Diabetes Mellitus manifests with symptoms when around _____% of the Beta cells are lost
90 (common trend with endocrine disorders, can compensate tremendously)
Type 2 Diabetes Mellitus is associated with the deposition of….
amyloid (composed of Amylin, the hormone secreted with Insulin)
The deposition of Amylin in the pancreatic Islets of Langerhan is diagnostic of Diabetes Mellitus (True or False)
False: can be seen in older people that don’t have Diabetes Mellitus
Group of genetic disorders featuring benign and malignant neoplasms of neuroendocrine glands; AD dominance
Multiple Endocrine Neoplasia (MEN)
The inheritance of MENs is…
Autosomal Dominance
Tumors seen with MEN1 (3 total)
Pituitary tumors
Parathyroid tumors
Pancreatic Neuroendocrine tumors
Type of mutation seen with MEN1
Loss of function for Tumor Suppressor
GENE: MEN 1
Tumors seen with MEN2A (3 total)
Parathyroid tumors
Pheochromocytoma
Medullary Thyroid Carcinoma
Type of mutation seen with MEN2A and MEN2B
Gain of Function for Proto-oncogene
GENE: RET
Tumors/findings seen with MEN2B (4 total)
Pheochromocytoma*
Medullary Thyroid Carcinoma*
Marfanoid Body Type
Mucosal Neuromas
*same as MEN2A
How to diagnose Familial Medullary Thyroid Carcinoma (which is different than MTC caused by MEN 2A or B)
1) find Medullary Thyroid Carcinoma
2) without other features of MEN2A/B
(that easy)
Pancreatic (Neuroendocrine/Adenocarcinoma) tumors are far more common
Adenocarcinoma (neuroendocrine tumors are less than 10%)
Pancreatic Neuroendocrine tumors are associated with which MEN
MEN1
Pancreatic (Microadenomas/Neuroendocrine) tumors are usually benign and less than 5mm in diameter
Microadenomas
Neuroendocrine tumors are usually large and metastatic
Pancreatic (Microadenomas/Neuroendocrine) tumors are usually metastatic and more than 5mm in diameter
Neuroendocrine
Microadenomas are usually small and benign
Neuroendocrine tumors are commonly described as having _____, ______, or _____ histologic pattern and __________ chromatin pattern.
- Solid/nested
- Trabecular
- Acinar
- Salt and pepper
How to diagnose malignancy of Pancreatic Neuroendocrine Tumors
Invasion into peripancreatic tissues
OR
Presence of metastatic disease
*cytologic atypia not enough
Examples of Functioning Pancreatic Neuroendocrine tumors (5 total)
Insulinoma Glucagonoma Gastrinoma Somatostatinoma VIPoma
Functioning Pancreatic Neuroendocrine tumor; insulin-secreting neoplasm of Beta-cells; MOST COMMON; signs and symptoms of Severe Hypoglycemia (confusion, lethargy, coma, etc.); usually SMALL (<3cm) and solitary; DOESN’T invade or metastasize
Insulinoma
Insulinomas are usually (small/big), (solitary/multiple), and (bening/malignant)
Small (<3cm)
Solitary
Benign
Most common Functioning Pancreatic Neuroendocrine tumor
Insulinoma
What causes the signs/symptoms of Insulinomas
Severe Hypoglycemia (confusion, lethargy, coma, etc.)
Insulinomas usually (do/don’t) metastasize and invade adjacent structures
Don’t
*only functioning pancreatic neuroendocrine tumor that doesn’t
Functioning Pancreatic Neuroendocrine tumor of “G cells”, secreting Gastrin; most commonly seen in Duodenum; NOT FOUND in NORMAL islets of langerhans; INDUCES ACID SECRETION from parietal cells; can cause Zollinger-Ellison Syndrome (severe ulcers that go beyond into jejunum due to high acid); most are MALIGNANT
Gastrinoma
Gastrinomas are usually (benign/malignant)
Malignant
Zollinger-Ellison Syndrome causes what changes in the stomach lining
Hypertrophic gastric folds caused by parietal cell hyperplasia (due to constant stimulation by Gastrin)
Gastrinomas can cause what peptic ulcer syndrome
Zollinger-Ellison Syndrome (peptic ulcers well beyond duodenum into jejunum)
Functioning Pancreatic Neuroendocrine tumor of Alpha cells, secreting Glucagon; RARE and MALIGNANT; results in hyperglycemia; symptoms include Diabetes, Necrolytic Migratory Erythema, Anemia
Glucagonoma
Glucagonomas are usually (benign/malignant)
Malignant
Special dermatologic finding for Glucagonomas
Necrolytic Migratory Erythema
Functioning Pancreatic Neuroendocrine tumor of “Delta (D) cells”, secreting Somatostatin; RARE and MALIGNANT; inhibits BOTH insulin and glucagon release; results in gallstones, steatorrhea (fatty stool) and hypochlorhydria (low stomach acid)
Somatostatinomas
Signs/Symptoms of Somatostatinomas
Gallstones (no motility)
Steatorrhea (no bile)
Hypochlorhydria (no acid)
Which Functioning Pancreatic Neuroendocrine tumors are MALIGNANT (4 total)
Gastrinomas
Glucagonomas
Somatostatinomas
VIPomas
Functioning Pancreatic Neuroendocrine tumor of D1 cells, secreting Vasoactive Intestinal Polypeptide; RARE and MALIGNANT; results in Verner-Morrison Syndrome (watery diarrhea, hypokalemia, and hypochlorhydria)
VIPoma
Signs/Symptoms of VIPoma
Verner-Morrison Syndrome:
- Explosive/watery diarrhea
- Hypokalemia
- Hypochlorhydria