Chapter 15: Pancreas Congenital Flashcards
Failure of the two embryonic pancreatic ducts to fuse - two separate ductal systems, each draining into the duodenum through the major and minor papillae, respectively.
Pancreas divisum.
Chronic pancreatitis in 25%
Pancreatic tissue discovered in the duodenum, stomach, and jejunum
Heterotopic pancreas. Smooth muscle abundant.
Pancreatic tumors may arise from this tissue - infiltrating ductal carcinoma.
Pancreatic head partly or completely surrounds the second portion of the duodenum
Annular pancreas. Commonly Down syndrome.
Some with duodenal atresia.
Can present late - 60s and 70s.
How do true pancreatic cysts form?
Faulty development of the pancreatic ducts.
Associated with renal tubular dysplasia, anorectal malformations, polydactyly, thoracic dystrophy.
What genes are involved in partial or complete pancreatic agenesis?
Homozygous mutations of homeodomain transcription factor IPF1 (PDX1).
A patient presents with severe epigastric pain that refers to the upper back. He also has nausea and vomiting. He has elevated serum amylase and lipase.
Biopsy of the pancreas shows acinar cell and fat necrosis.
The patient develops shock, ARDS, and acute renal failure and dies. At autopsy the pancreas has gray foci, with yellow-white areas of fat necrosis.
Acute pancreatitis. Due to aberrant release of pancreatic exocrine enzymes.
Due to acinar injury and duct obstruction.
Gallstone, anatomic anomalies, ethanol, viruses, azathioprine, blunt trauma, ischemia, hyperlipidemia, hypercalcemia, obesity, parasites.
A chronic alcoholic presents with a gradual onset of epigastric pain, new onset diabetes, and weight loss. Radiological examination shows areas of enhancement on the pancreas. It is removed and looks like below.
Histology shows atrophic lobular of acinar cells surrounded by dense fibrous tissue infiltrated by lymphocytes.
Chronic pancreatitis.
Chronic alcoholism, obstruction of the pancreatic duct, groove pancreatitis. Chronic injury to acinar cells (hemochromatosis). Renal failure. Autoimmune (IgG4). Cystic fibrosis. Hereditary pancreatitis (AD) increasing trypsin levels (PRSS1, SPINK1).
A patient presents with weight loss and constant epigastric pain. Biopsy of the pancreas shows loss of acinar tissue and a dense lymphoplasmacytic inflammatory infiltrate.
Autoimmune chronic pancreatitis. (Lymphoplasmacytic sclerosing pancreatitis, duct-destructive chronic pancreatitis).
Occurs with other autoimmune conditions - chronic sclerosing sialadenitis, retroperitoneal fibrosis.
IgG4 elevated. Hypergammaglobulinemia and ANA ab, rheumatoid factor, antilactoferrin, anti-carbonic anhydrase.
A patient presents with anorexia, weight loss, and gnawing epigastric pain radiating in the back. He also has painless jaundice.
Serum levels of CA 19-9 are elevated.
Positive Couvoisier sign - painless gallbladder dilation accompanied by jaundice
Positive Troussea syndrome - migratory thrombophlebitis.
Infiltrating ductal adenocarcinoma of the pancreas.
Smoking, high meat, fat, nitrate diet, diabetes mellitus, chronic pancreatitis, coal gas, metal, dry cleaning agents, leather tanning <- risk factors.
Hereditary factors. Peutz-Jegher syndrome especially.
Pancreatic intraductal neoplasia (PanIN - dysplasia of ducts. Mucinous epithelium replacing normal lining of ducts. KRAS oncogene.
A 65 year old patient presents with subcutaneous fat necrosis, polyarthralgia, and periopheral eosinophilia. His lipase is massively elevated.
A large, well circumscribed pancreatic mass is found and looks like below.
Acinar Cell Carcinoma.
Uniform cells arranged in small acini and nests. Immunohistochemistry demonstrates production of exocrine enzymes.
A 6 year old child presents with elevated alpha-fetoprotein and an enlarged pancreas. A biopsy is taken and shows polygonal cells in solid islands and acinar structures with intersperced squamoid nests.
The cells are producing exocrine enzymes.
Pancreatoblastoma.
May occur in setting of Beckwith-Wiedemann syndrome.
A patient presents with an enlarged pancreatic mass. Biopsy shows enlarged spaces lined by glycogen-rich cuboidal epithelium.
Serous cystic neoplasm of the pancreas.
Nearly always benign - VHL syndrome risk factor. Sunburst pattern on imaging studies. Sometimes large stellate central scar.
A patient with a history of chronic pancreatitis presents with a pancreatic mass. Biopsy shows dilated pancreatic ducts lined by neoplasic mucinous epithelium and filled with mucus. It takes on the below form.
Intraductal papillary mucinous tumor of the pancreas.
Often may harbor malignant, invasive carcinoma. Removed.
A middle-aged woman presents with a mass in the tail of the pancreas. Biopsy shows a mucin-rich epithelial lining a large, mucin-filled space.
It rests on an ovarian-type stroma.
Mucinous Cystic Neoplasm of the Pancreas
Sometimes associated with invasive carcinoma.
A girl presents with a pancreatic mass. Biopsy shows a solid and circumscried mass with cystic areas filled with blood and necrotic debris.
The tumor cells are monomorphic forming structures as seen below.
Solid pseudopapillary neopalsm of the pancreas
Pseudopapillary structures and loose sheets. Indolent, curable by resection.
Beta-catenin gene.
