Makindo Medical Notes.com

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Its deep position and vague clinical symptoms do not lend itself to early diagnosis unless a periampullary lesion causes jaundice. A normal abdominal USS does not exclude it.

About

• Most patients present with an incurable disease
• Cancer of the ampulla presents earlier with obstructive jaundice
• Periampullary and endocrine tumour do best

Risks

• Smoking, Alcohol, Coffee intake

Genetic - small number have genetic component

• BRCA2 mutation
• Familial atypical multiple mole melanoma (CDKN2A)
• Peutz-Jeghers syndrome, Von Hippel Lindau
• HNPCC and FAP, MEN syndrome

Pathology

• Head or neck of the pancreas (periampullary) 70%
• Body of pancreas 20% and Tail of pancreas 10%
• Spread: Invasive locally along the pancreatic duct. To Duodenum, local lymph nodes, portal vein, nerve sheaths and spreads to the liver

Histology

• Ductal adenocarcinomas
• Adenosquamous
• Mucinous cystadenocarcinomas
• Insulinomas, Glucagonomas, Gastrinomas

Clinical

• Anorexia, Painless jaundice, Weight loss, Ascites
• Jaundice and possibly a distended gallbladder (Courvoisier's law)
• Pancreatitis presentation in younger patients
• Venous thrombosis - DVT , PE
• Migratory Thrombophlebitis (Trousseau's sign)
• Secondary Diabetes
• Upper abdominal pain and backache with no gallstones (normal USS) and normal OGD
• Unexplained weight loss
• Unexplained pancreatitis
• New diabetes with Upper GI symptoms

Investigations

• FBC - anaemia U&E
• LFT - ↑ ALP ↑ Bilirubin, possibly ↑ Prothrombin time
• ↑ CA19-9 and ↑ CEA (non-specific)
• USS abdomen - biliary obstruction, mass at the head of the pancreas but in many causes is poor at viewing retroperitoneal pancreas and cannot be relied upon to exclude the diagnosis
• ERCP is the investigation of choice which allows brushing and biopsy and stenting if jaundiced
• Contrast enhanced CT can help determine operability.
• Percutaneous radiological guided biopsies may be required but can lead to seeding of the biopsy tract in some cases
• Histology is always important to exclude lymphoma or neuroendocrine tumour which have an entirely different treatment and prognosis

Complications

• Obstructive jaundice and pruritis (stent/surgery)
• Duodenal obstruction (stent/surgery)
• Venous thromboembolism
• Significant Pain control
• Splenic vein thrombosis
• Cholangitis
• Malignant Ascites
• Secondary diabetes
• Complications of surgery - haemorrhage, infection etc

Management

• Surgical resection in a few with limited disease and is a Pancreaticoduodenectomy or Whipple's procedure. Removal of the head of the pancreas, first 3 parts of duodenum and distal gastric antrum and common bile duct followed by a gastrojejunostomy with common bile duct and pancreas anastomosed to the small bowel. Obstruction should be managed prior to surgery as a raised bilirubin increases surgical risks.
• Tumours of the body and tail present late and the resection rate is very low.
• Vitamin K should be given to try to correct any clotting abnormality.
• LMWH is indicated to reduce Venous thromboembolism
• Palliative chaemotherapy can prolong survival in advanced disease
• Palliation - ERCP and stenting or choledochojejunostomy
• Stenting or Gastrojejunostomy for duodenal obstruction
• Radiotherapy or Coeliac plexus block for pain
• Prognosis is very poor with typical survival of 12 months or less