| What are ampullomas? | Periampullary carcinomas include carcinomas of duodenum, distal bile duct, pancreas and ampulla of Vater (ampullomas)
It is the second most common periampullary carcinoma (after pancreatic head cancer), it is important to distinguish these cancers as it will give different prognosis (ampulloma has good prognosis and resectable stage detection usually) |
| What are the risk factors for developing amupllomas? | Familial adenomatous polyposis (FAP) relative risk is 124
Gardner's syndrome, Lynch syndrome, neurofibromatosis type I and Muir-Torre syndrome |
| What are clinical presentations of ampullomas? | Initially presents w/obstructive jaundice presenting at an earlier stage than biliary malignancies/ periampullary malignancies (other than pancreaticobiliary ampullary carcinoma)
Anicteric pt may present w/bacterial cholangitis, elevated CEA and CA19-9 (in 11-30% and 41-63 % respectively) |
| How is the dx of ampulloma? | Dx by endoscopy based on biopsy appearance, Imaging like CT and MRI show resectability and metastasis.
EUS used in preop evaluation, detects invasion of adjacent organs 80-90% of cases, and sensitivity and specificity are 73% and 90% respectively |
| How is the tx of ampulloma? | Surgical resection (only 77-93% are resectable at time of dx)
Std approach is pacreaticoduodenectomy.
Post op if no lymph node metastasis is good w/5 year survival rate 59- 78%, if nodes involved 15-25% only.
Obstructive cholestasis treated paliatively by endoscopic/ percutaneous stent placement
Benefit from chemotherapy adjuvant and chemoradiation. |
