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Periampullary and Pancreatic Tumors. Rod L. Flynn, M.D. Surgical Oncologist Mary Washington Hospital Fredericksburg, VA. Defined as those that arise within 2 cm of the major papilla in the duodenum Classified on the basis of their tissue of origin
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Periampullary and Pancreatic Tumors Rod L. Flynn, M.D. Surgical Oncologist Mary Washington Hospital Fredericksburg, VA
Defined as those that arise within 2 cm of the major papilla in the duodenum Classified on the basis of their tissue of origin Often difficult or impossible to differentiate from pancreatic head adenocarcinoma before performing a resection Periampullary Tumors
Encompass tumors of the: ampulla of Vater distal common bile duct (intrapancreatic distal common bile duct), duodenal tumors (usually the second part) involving the papilla tumors of the pancreatic head involving in close proximity to the ampulla Pancreatic head adenocarcinoma accounts for most (approximately 80%) tumors in the periampullary region Periampullary Tumors
Non-pancreatic periampullary cancers tend to have a better prognosis than does pancreatic adenocarcinoma because they are often more resectable i.e. 90% of ampullary cancers vs 15-20% of pancreatic head cancers In general, the more distal the tumor is along the pancreatobiliary tree, the better the prognosis Hilar Cholangiocarcinomas(Klatskin Tumors) 15-20% 5-year survival Distal bile duct cancers 20-30% 5-year survival Ampullary carcinomas 40-60% 5-year survival Duodenal carcinomas 60% -70% 5-year survival Pancreatic head adenocarcinoma 15-20% 5-year survival Periampullary Tumors
Pancreatic Head AdenocarcinomaDemographics • Treatment poses diagnostic and therapeutic challenge • Second most common GI malignancy in U.S. (colorectal is the most common) • In 2006, 33,730 new cases were diagnosed in U.S. • Accounted for about 32,300 deaths • Fourth leading cause of cancer-related deaths (following lung, colon, breast/prostate)
Pancreatic Head AdenocarcinomaDemographics • Responsible for 5% of all cancer-related deaths • Surgical resection provides the only chance for cure • 80% of patients present with advanced disease not amenable to resection
Pancreatic Cancer Risk Factors • Exact cause is unknown • Environmental exposure • Smoking (main risk factor) • Risk increases with dose and exposure • Other tobacco carcinogens likely involved • Organic and nickel-containing solvents • Chlorinated compounds • High BMI • Diet -- low in vegetables and fruits, high in animal fats and meat products Risk higher in obese individuals • Risk higher in obese individuals • Decreases with weight loss and exercise
Pancreatic Cancer Risk Factors (cont’d) • Comorbid conditions • Chronic pancreatitis • Diabetes mellitus, type II • Risk doubles with > 5-year history of diabetes mellitus, type II • Genetic factors • Account for 15% to 20% of cases • 1 family member affected: 18 times risk • 3 family members affected: 57 times risk • Familial syndromes
Pancreatic Head AdenocarcinomaClinical Presentation • Most patients with periampullary cancer present with at least one of the following symptoms: • Weight loss • Jaundice (75% of patients) • Vague epigastric/ back pain (retroperitoneal plexus invasion) • Fatigue • Intestinal malabsorption • New onset diabetes (15%) • Symptom complex is vague, which often delays presentation and diagnosis • As a result about 80% of all patients present with unresectable disease
The presence of clinical signs usually means advanced disease Courvoisier’s sign Painless jaundice Palpable abdominal mass Large tumor or omental cake Ascites Umbillical nodule (Sister Mary Joseph’s node) Blumer’s shelf (rectovaginal/vescicle mass) Virchow’s node (left supraclavicular) Pancreatic Head AdenocarcinomaClinical Signs
TUMOR Tis: in situ carcinoma T1: < 2 cm T2: > 2 cm T3: beyond pancreas T4: involves celiac axis or superior mesenteric artery (unresectable) NODE N0: no lymph node metastases N1: regional lymph node metastases METASTASES M0: no distant metastases M1: distant metastases present AJCC STAGINGPancreatic Cancer
The goals of evaluating patients with periampullary cancers is to obtain diagnosis and clinical stage Based on these determinations the patient can be triaged into a treatment category (operative or non-operative) At time of initial diagnosis, approx 50% of patients will have metastatic disease 30% will have locally-advanced disease not amenable to surgical resection The superior mesenteric vein is involved with the large pancreatic head tumor Pancreatic CancerDiagnosis
Blood tests including CBC, LFTs, amylase/lipase, CEA, CA 19-9 Abdominal ultrasound A common initial test to evaluate jaundice Abdominal CT scan Gives better anatomical information on the source of the biliary obstruction Can give information about extrapancreatic sites of spread (liver, peritoneal/omental surfaces, ascites, extensive nodal involvement, adjacent organ involvement) Can assess involvent of major blood vessels (SMA/portal vein) Angiography ? PET scan Pancreatic CancerDiagnostic Tests
ERCP Brush cytology Stenting if necessary Look for dilated pancreatic duct Look for filling defect within bile duct Pancreatic CancerDiagnostic Tests
Endoscopic ultrasound Can detect very small tumors (<2cm) Can assist in staging by assessing mesenteric vascular involvement FNA biopsies are relatively easy to do Operator dependant Pancreatic CancerDiagnostic Tests
Laparoscopy for staging • Looking for • Local involvement of adjacent organs • Loco-regional extension (lymph nodes, soft tissue) • Small liver metastases • Peritoneal nodules Peritoneal Nodule
Treatment Available • Chemotherapy • Radiation therapy • Chemoradiation followed by resection • Resection • Resection + Adjuvant Therapy • Palliation • Stents • Bypass • Feeding tubes
Treatment Available • Only 10-20% of patients are eligible for surgery • Most have advanced disease at time of diagnosis • Most common chemo 5-FU & Gemcitabine
Whipple Procedure Pancreaticoduodenectomy • The Whipple operation was first described in the 1930’s by Allan Whipple • In the 1960’s and 1970’s the mortality rate for the Whipple operation was very high (Up to 25% of patients died from the surgery) • This experience of the 1970’s is still remembered by some physicians who are reluctant to recommend the Whipple operation • Today the Whipple operation has become an extremely safe operation in the USA - At tertiary care centers where large numbers of these procedures are performed by selected surgeons, the mortality rate is less than 4%.
Most common Diagnosis of patients undergoing Whipple • Peripancreatic Cancer (jaundice) • Pancreatic head • Ampulla • Bile duct • Duodenal wall • Pancreatitis • Cystic neoplasm • Carcinoid • Islet cell tumors
Surgery • Incisions • Omentum • Resectability (Portal Vein/SMA) • Gall Bladder and Porta Hepatis • Gastrectomy • Pancreas Transection • Reconstruction
Surgery • Incisions • Omentum • Resectability (Portal Vein/SMA) • Gall Bladder and Porta Hepatis • Gastrectomy • Pancreas Transection • Reconstruction
Surgery (cont.) • Pylorus Preserving • Extended Nodal Dissection • Gastric Inversion
Exposure of SMV Surgery (cont.)
Portal Triad Hepatic artery Portal vein Common bile duct Surgery (cont.)
Division of pancreas Surgery (cont.)
Resected specimen Surgery (cont.)
Plumbing restored Surgery (cont.)
Outcomes Possible complications44% in modern series out of Johns Hopkins; <5% 30-day mortality; 17day LOS v. 28 for complicated • Pancreatic fistula (Leak-8%) • Gastro paresis • Nutritional deficiencies • Malabsorption • Early satiety • Weight loss • Diabetes
(Johns Hopkins study, con’t) Outcomes • N= 201 patients • The mean age of the patients was 63 years, with a slight male predominance (108 men and 93 women). • There were no differences in survival based on age, gender, or race. • The actuarial one, three and five-year survival rates for all 201 patients were 57%, 26%, and 21% respectively, with a median survival of 15.5 months. • 11 five-year survivors, • 7 six-year survivors • one fifteen-year survivor.
Summary • Periampullary cancers include bile duct, ampulla of vater, duodenal, pancreatic head • Prognoses depend on relative location • Pancreatic head adenocarcinoma carries worst prognosis • Surgery is the only chance of cure, although a majority of patients are unresectable at the time of diagnosis • Better preoperative evaluation can reduce the number of unnecessary operations • Preoperative diagnosis is often very difficult if not impossible to make despite a myriad of diagnostic modalities at our disposal • In this group of patients we surgeons sometime have to tell the patient after a lengthy Whipple operation: “…I have good news, you don’t have cancer…”