Essentials: Pancreas Pathology (Summary Slides) Flashcards
Pancreatitis (2 types)
Acute Pancreatitis
Chronic Pancreatitis
Pancreatitis (definition + cause)
Inflammation of the pancreas caused by autodigestion of the pancreas by its own enzymes, due early enzyme activation
Key difference between acute and chronic pancreatitis
Acute: REVERSIBLE
Chronic: IRREVERSIBLE
Key features of acute pancreatitis (4)
- fat necrosis (saponification, calcium precipitation and hypocalcemia)
- parenchymal necrosis
- acute inflammation
- hemorrhage
Key features of chronic pancreatitis (3)
- Calcification
- Gland dropout (atrophy)
- Fibrosis
3 main causes of acute pancreatitis
- Alcohol (#1)
- Gallstones (#2)
- Drugs, vascular, infectious, genetic
Genetic mutations that can cause acute pancreatitis (2)
Mutations in genes PRSS, SPINK1
Complications of acute pancreatitis (6)
- DEATH (this is a medical emergency)
- Disseminated intravascular coagulopathy (DIC)
- Acute respiratory distress syndrome (ARDS)
- Hypocalcemia
- Peritonitis
- Pseudocyst
4 main causes of chronic pancreatitis
- Alcohol (#1)
- Autoimmune (IgG4)
- Genetic
3 genetic mutations that can cause chronic pancreatitis
Mutations in genes PRSS1, SPINK1
Cystic fibrosis
4 complications of chronic pancreatitis
- Weight loss
- malabsorption
- diabetes mellitus
- pancreatic cancer
Autoimmune pancreatitis responds well to…
steroids
2 types of non-neoplastic pancreatic cysts (i.e. no new growth, no malignant potential)
Congenital cysts
Pseudocysts
2 types of neoplastic pancreatic cysts without papillary structures
Serous cystadenoma
Mucinous cystic neoplasms
2 types of neoplastic pancreatic cysts WITH papillary structures
Intraductal papillary mucinous neoplasm (IPMN)
Solid pseudopapillary neoplasms
Congenital cystic lesions of the pancreas: important features
Benign
Developmental
Congenital cysts are associated with 2 diseases…
Polycystic kidney disease
Von Hippel-Lindau syndrome
Most common type of pancreatic cysts
Pseudocysts
What are pseudocysts (pancreas)?
Collections of necrotic material rich in pancreatic enzymes. They are not true cysts because they have no epithelial lining.
Why are pseudocysts not considered “real” cysts?
They do not have an epithelial lining
Pseudocysts of the pancreas usually develop secondary to…
pancreatitis
Serous cystadenoma (definition + key features)
Neoplastic cyst of the pancreas without papillary structures.
They have NO malignant potential (benign).
Key histological feature of serous cystadenoma
Multilocular (having many small compartments)
Serous cystadenomas usually affect…
females
Serous cystadenomas may be associated with a syndrome called…
Von Hippel-Lindau syndrome
Mucinous cystic neoplasms (definition + key features)
Neoplastic cysts of the pancreas without papillary structures.
They DO have malignant potential!
How do we manage mucinous cystic neoplasms?
Since they have malignant potential, they need to be resected.
Mucinous cystic neoplasms usually affect…
females
Key hisotlogical features of mucinous cystic neoplasms
They are made up of mucus-secreting cells and ovarian-like stroma
Mucinous cystic neoplasms have NO connection to…
the duct system
Intraductal papillary mucinous neoplasm (IPMN) (definition + key features)
Neoplastic cysts of the pancreas with papillary structures.
They have malignant potential!!!
How do we manage intraductal papillary mucinous neoplasms?
Since they have malignant potential, they need to be resected.
Intraductal papillary mucinous neoplasms (IPMN) usually affect…
males
Key histological feature of intraductal papillary mucinous neoplasms (IPMN)
Made of mucous cells
What type of pancreatic cyst has NO connection to ducts? What type of cyst is intimately connected with ducts?
No connection to duct system: Mucinous cystic neoplasms
Connection to duct system: Intraductal papillary mucinous neoplasm (IPMN)
Solid pseudopapillary neoplasm (definition + key features)
RARE neoplastic cyst of the pancreas with papillary structures. It has malignant potential (locally agressive).
How do we manage solid pseudopapillary neoplasms?
Since they have malignant potential (locally aggressive), they must be resected.
solid pseudopapillary neoplasms usually affect…
young females
Name 4 types of pancreatic cancer
- Ductal adenocarcinoma
- Neuroendocrine tumours
- Acinar carcinoma
- Pancreatoblastoma
Most (90%) of pancreatic cancers are…
ductal adenocarcinomas
Ductal adenocarcinomas are a cancer of…
a) exocrine pancreas
b) endocrine pancreas
a) exocrine pancreas
Ductal adenocarcinoma usually affects…
males
Key histological features of ductal adenocarcinomas (3)
- Haphazard glands
- Desmoplastic stroma (fibrosis)
- Perineural invasion (common)
Ductal adenocarcinoma is characterized by elevated serum markers…
CEA ad CA19.9
Precursor of ductal adenocarcinoma
Pancreatic intraepithelial neoplasia (PanIN)
Gene mutations that cause ductal adenocarcinomas (4)
Mutations in genes KRAS, TP53, SMAD4, BRCA2
Ductal adenocarcinoma risk factors (8)
- Chronic pancreatitis
- Smoking
- Hereditary pancreatitis (PRSS1, SPINK1)
- BRAC1 and BRCA2 genes
- Lynch syndrome (mismatch repair proteins are dysfunctional)
- FAP syndrome (APC gene)
- Peutz-Jeghers syndrome
- Familial melanoma syndrome
Which part of the pancreas does ductal adenocarcinoma usually present in?
Head of pancreas
Presentation of ductal adenocarcinoma at the head of pancreas vs body/tail
Head: Presents with jaundice & pruritus (itching)
Body/Tail: Pain, weight loss
Ductal adenocarcinomas are a cancer of…
a) exocrine pancreas
b) endocrine pancreas
b) endocrine pancreas
What cells of the pancreas are affected by neuroendocrine tumours?
Islet cells
Functional vs non-functional neuroendocrine pancreatic tumours
Functional: Mostly benign
Non-functional: Mostly malignant
What 3 criteria do neuroendocrine (non-functional) tumours have to meet to be considered malignant?
- Vascular invasion
- Local invasion
- Metastasis (lymph nodes, liver, distant)
4 types functional neuroendocrine pancreatic tumours
- insulinoma
- gastrinoma
- glucagonoma
- somatostatinoma
Most FUNCTIONAL neuroendocrine tumours of the pancreas are…
insulinomas
Insulinomas cause…
hypoglycemia
Neuroendocrine tumours of the pancreas are associated with a genetic disorder called…
MEN1 syndrome
Name 2 rare pancreatic cancer subtypes
Acinar carcinoma
Pancreatoblastoma (pediatric tumour)
Name a a rare pancreatic cancer/tumour affecting children
Pancreatoblastoma (pediatric)