Syed S. Karim, DO1, Pranay Srivastava, MD1, Jarin Prasa, MS2, Bobby Jacob, MD3, Charudatta Wankhade, MD1, Shino Prasandhan, MD1, Kaleem Rizvon, MD, FACG1; 1Nassau University Medical Center, East Meadow, NY; 2Touro College of Osteopathic Medicine, East Meadow, NY; 3Nassau University Medical Center, Lake Grove, NY
Introduction: Periampullary tumors are responsible for >30,000 deaths in the US per year. They are a group of tumors found in the vicinity of the ampulla of vater, but can arise from any source such as intestinal, pancreatic or biliary origin. Primary ampullary carcinomas (PAC) are distinct in that they originate in the ampullary cortex, distal to the bifurcation of the pancreatic duct and the common bile duct. They account for only 6% of periampullary tumors, and 4-6 cases per million in the population. We present a patient with PAC who was treated with pancreaticoduodenectomy.
Methods: 35 year old male with no medical history presented with fatigue, generalized pruritus, intermittent fevers, pale stools and 30 lb weight loss. ALP and total bilirubin was 1383 and 7 respectively. Initial work up demonstrated negative hepatitis and autoimmune panels. MRCP suggested diffuse biliary tree dilatation. ERCP revealed periampullary mass with biliary obstruction and he was decompressed with stent placement. Biliary brushing and biopsy showed poorly to moderately differentiated adenocarcinoma. CT showed soft tissue 2.1x1.6 cm mass at the ampulla of vater and a few enlarged mesenteric lymph nodes. CEA and CA-19 were both elevated at 6.3 and 60. The patient was then referred to a tertiary care center for EUS for staging and surgery. Discussion: PAC can be classified primarily between intestinal (47%), pancreatobiliary (24%) and poorly differentiated adenocarcinoma (13%) from histology. Some studies claim that the pancreatobiliary type has the worst prognosis while the other 2 fair significantly better. Like this case, most patients with PAC present with symptoms of obstructive jaundice (80%), steatorrhea, weight loss, abdominal pain, fever, and fatigue. MRCP may show biliary dilatation. CT is needed to exclude pancreatic cancer and to evaluate for any metastasis. Our patient had obstructive jaundice due to periampullary mass and underwent ERCP with biliary stent placement which is essential for diagnosis and treatment. CT showed that the mass was localized to the ampulla of vater, without any metastasis. Pathology confirmed adenocarcinoma. Unlike pancreatic cancer or distal cholangiocarcinomas, PAC with pancreaticoduodenectomyhave a favorable 5 year survival rate as high as 45-50%. Our patient is status post Wipple’s procedure and is currently asymptomatic. PACs are very rare, however, it is very important to distinguish from other periampullary tumors because of the comparatively favorable outcomes.
Disclosures: Syed Karim indicated no relevant financial relationships. Pranay Srivastava indicated no relevant financial relationships. Jarin Prasa indicated no relevant financial relationships. Bobby Jacob indicated no relevant financial relationships. Charudatta Wankhade indicated no relevant financial relationships. Shino Prasandhan indicated no relevant financial relationships. Kaleem Rizvon indicated no relevant financial relationships.