Pancreas II: Cystic Fibrosis, Cystic Disorders, and Pancreatic Cancer: Marino

Question 1

CF related pancreatic pathology presents in adults or children?

Answer 1

Children, as it is congenital

Question 2

How do you Dx CF-related pancreatic abnormalities?

How do you tx it?

Answer 2

Sweat Cl- test, just like with lung-related CF

Tx: Abx and pancreatic enzymes

Question 3

Describe the histological presentation of CF involving the pancreas.

Answer 3

Dilated ducts filled with think, pink secretions.

Lots of fibrosis

Question 4

What genetic mutation is associated w/ benign serous cysts of the pancreas? 
Histological appearance?
Single cysts or multiple?
Tail or head of pancreas? 
Found predominately in men or women?

Answer 4

VHL
Cysts lined by simple cuboidal epith. Filled with straw colored fluid. 
Multiple cysts
Tail of pancreas
Found in women, predominately

Question 5

Mucinous cysts of the pancreas:
Histological appearance?
Men or women?
Head or tail of pancreas?
Gene mutations?
Precursor to invasive carcinoma?

Answer 5

Tall, columnar, mucin producing epith. Thick, ovarian-like stroma (found in women, who have ovaries…)
Women
Tail
KRAS, P53, RNF43
Can be precursor to invasive carcinoma (all the mucinous ones can become cancer)

Question 6

Intraductal papillary mucinous neoplasms:
Histological appearance?
Men or women?
Head or tail of pancreas?
Gene mutations?
Precursor to invasive carcinoma?

Answer 6

Papillae expanding ducts
Men
Head (think, men have big heads)
GNAS, KRAS, P53, SMAD4, RNF43 (big heads, more genetic muts)
Can be precursor to invasive carcinoma (all the mucinous ones can become cancer)

Question 7

Solid pseudopapillary neoplams:
How does it present?
Histological appearance?
Men or women?
Genetic associations?
Prognosis?

Answer 7

Presents as abdominal discomfort because of large size
Cyst with no epithelial lining. Solid sheets of discohesive cells.
Young women
Hyperactivation of Wnt signaling pathway due to acquired activating muts of CTNNB1 (B-catenin) oncogene.
Most pts cured following complete surgical resection of neoplasm

Question 8

Most common presentations of pancreatic cancer?

Prognosis?

Answer 8

Abd. pain and weight loss, jaundice.

Prog. is fucking terrible (

Question 9

Describe the surgical treatment options for cure of pancreatic cancer of the head and options for cancer of the tail.

Describe tx options for palliation.

Answer 9

Tail: distal pancreatectomy (not a lot of vital structures to damage)

Head: Whipple resection- complicated

Resection (when possible) provides best prognosis.

Palliation:
relieve obstructions
Splanchniectomy for pain reduction (not very helpful)

Question 10

Describe histological presentation of pancreatic carcinoma.

Answer 10

Desmoplasia (fibrosis), mucin producing. Highly anaplastic cells

Question 11

Pts w/ these two conditions are at incr. risk of developing pancreatic cancer:

Answer 11

Peutz-Jeghers syndrome- hamartomatous polyps in the gastrointestinal tract
Hereditary pancreatitis