Ampullary Carcinoma

  • – It belongs to the family of periampullary carcinoma
  • – This family includes
  •               • Carcinoma of the duodenum
  •               • Carcinoma of the ampulla of vater
  •               • Carcinoma of the distal bile duct
  •               • Carcinoma of the pancreas
  • – Ampullary carcinomas are the second most common form of periampullary carcinoma (after pancreatic head cancer)
  • – Ampullary carcinomas are often diagnosed earlier than the others and therefore at a resectable stage, thus resulting in a better prognosis

 

  •    Epidemiology:
  • – 1% of all GI cancer
  • – 4-8% of periampullary cancer
  • – Slight male predominance (1.48:1)

 

  •          Etiology:
  • – Unknown in majority cases
  • Risk factors for ampullary carcinoma
  •                  • FAP (Periampullary carcinoma is the 2nd most common cause of death in FAP after colon cancer)
  •                  • 90% FAP patients develop ampullary/periampullary adenoma
  •                  • Gardner’s syndrome – variant of FAP
  •                  • Neurofibromatosis type 1
  •                  • Muir-Torre syndrome
  •                  • Cholangiocarcinoma
  •                  • Chronic liver fluke infection
  • – HNPCC is not a risk factor for ampullary carcinoma
  •  
  •              
  •             Pathology:

 

  • Normal ampulla of vater consists of
  •                • Papilla of duodenum
  •                • Common pancreaticobiliary channel
  •               • Distal bile duct
  •               • Distal main pancreatic duct
  • Macroscopic types of ampullary carcinoma
  •                • Intramural protruding (intra-ampullary)
  •                • Extramural protruding (periampullary)
  •                • Ulcerating (diagnosed in advanced stage and highest rate of LN metastasis)
  • – Histopathlogical types of ampullary carcinoma
  •          1. Adenocarcinoma- 90%
  •                           – Pancreticobiliary type (cytokeratin 7 positive)
  •                           – Intestinal type (cytokeratin 20 positive + MUC 2 positive)
  •          2. Mucinous type
  •          3. Signet-ring cell type
  •          4. Undifferentiated type
  •                          
  •                    Pathogenesis:

 

  • – The tumor may arise on the duodenal surface of the ampulla or its inner epithelial lining
  • – The majority of ampullary carcinomas follow an adenoma carcinoma sequence
  • – Carcinomas of the intestinal type typically develop from adenomas, whereas pancreaticobiliary and ulcerating carcinomas often lack a precursor lesion

 

 

  • Clinical features:
  • – Obstructive jaundice: Fluctuating jaundice, often progressive (other malignant biliary obstruction has progressive jaundice)
  • – Anictric patients may present with bacterial cholangitis
  • – Siver stool (due to bleeding of the tumor and acholic stool)
  • – Anemia (due to ulceration of the tumor)
  • – Hepatomegaly
  • – Palpable GB

 

  •         Investigations:
  • Tumor markers: CA 19-9, CEA
  • Endoscopy of upper GIT and biopsy from the lesion
  • Immunohistochemitry of the tumor : CEA, CA 19-9
  • – Subsequents tests are for staging
  •         → CT scan/ MRI
  •                            • Discrete hypodense mass on T2 weighted image
  •                            • Irregular thickening around the bile duct or bulging into the duodenum
  •                            • Double duct sign (dilatation of both pancreatic/ bile duct)
  •                            • Dilatation of only bile duct
  •                            • Rarely only pancreatic dilatation
  •            → EUS: To detect local invasion
  •                              TNM staging of ampullary carcinoma
  • – 42% to 60% of patients have LN metastases at the time of surgery
  •                  5 year survival:
  •     – Node positive patient: 9-47%
  •     – Node negative patient: 59-63%
  •            
  •  Treatment:
  • Surgery: Pancreaticoduodenectomy
  • Limited surgical/ endoscopic ampullectomy
  •                 • Not recommended
  •                 • High recurrence rate
  • Adjuvant chemoradiation therapy
  •                 • For patients with LN positive disease
  • Role of chemotherapy in unresectable tomor
  •                 • Undefined
  •            
  •          Palliative treatment
  •  – Biliary drainage with stenting
  •                     Endoscopic
  •                     • Percutaneous
  •   – Surgical bypass
  •                         Prognosis: 
  • – Improved prognosis may be due to morphology of the tumor that is more similar to intestinal tumor than biliary or pancreatic tumor
  • – Median survival: 30-50 months for all patients
  • – 5 year survival for surgically resected patients: 30-50%

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