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Pancreatic carcinoma and periampulary tumors •MH Emami • Poursina Hakim Research Institute • IUMS • Isfahan, Iran • 5.10 1392 Liver, Biliary System, and Pancreas Pancreatic masses • • • • • • • • Pancreatitis/cyst/Pseudocyst Autoimmune pancreatitis(AP) Neuroendocrine Tumors Lymphoma Distal CBD cholangiocarcinoma Pancreatic metastasis Periampulary tumors Exocrine pancreatic neoplasms" includes all tumors that are related to the pancreatic ductal and acinar cells and their stem cells (including pancreatoblastoma) Epidemiology • Cancer of the exocrine pancreas is a highly lethal malignancy. • It is the fourth leading cause of cancer-related death in the United States and second only to colorectal cancer as a cause of digestive cancerrelated death Pancreatic CA risk factors • • • • • • • • • • • • • Heritage Age Sex Oxidized fats Cigarettes Alcohol opium Low exercise, sedentary life obesity Chronic Inflammation ( pancreatitis) Low intake of vegetable and Fibers DM Immunosuppressants • Surgical resection is the only potentially curative treatment. • Unfortunately, because of the late presentation, only 15 to 20 percent of patients are candidates for pancreatectomy. • Furthermore, prognosis is poor, even after a complete resection. • Five-year survival after pancreaticoduodenectomy is about 25 to 30 percent for node-negative and 10 percent for node-positive disease. • Of the several subtypes of ductal adenocarcinoma, most share a similar poor long-term prognosis, with the exception of colloid carcinomas, which have a somewhat better prognosis. The most frequent symptoms of pancreatic CA at the time of diagnosis • • • • • • • • • • • • • Asthenia – 86 percent Weight loss – 85 percent Anorexia – 83 percent Abdominal pain – 79 percent Epigastric pain – 71 percent Dark urine – 59 percent Jaundice – 56 percent Nausea – 51 percent Back pain – 49 percent Diarrhea- 44 percent Vomiting – 33 percent Steatorrhea – 25 percent Thrombophlebitis – 3 percent The most frequent signs of pancreatic CA at the time of diagnosis • • • • • Jaundice – 55 percent Hepatomegaly- 39 percent Right upper quadrant mass – 15 percent Cachexia – 13 percent Courvoisier’s sign (nontender but palpable distended gallbladder at the right costal margin) – 13 percent • Epigastric mass – 9 percent • Ascites – 5 percent Signs of advanced, incurable disease include: • An abdominal mass or ascites • Left supraclavicular lymphadenopathy (Virchow's node) • A palpable periumbilical mass (Sister Mary Joseph’s node) or a palpable rectal shelf are present in some patients with widespread disease. Pancreatic cancer is the origin of a cutaneous metastasis to the umbilicus in 7 to 9 percent of cases Clues suggesting the possibility of a primary pancreatic lymphoma include • Lack of jaundice, • Constitutional symptoms (weight loss, fever, and night sweats), • Elevated serum lactate dehydrogenase (LDH) or beta-2 microglobulin level, • Normal serum CA 19–9 • An endoscopic ultrasound (EUS)-guided biopsy may be recommended if a diagnosis of chronic or autoimmune pancreatitis is suspected on the basis of history (eg, extreme young age, prolonged ethanol abuse, history of other autoimmune diseases), particularly if further imaging studies (either EUS, endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiopancreatography) reveal multifocal biliary strictures (suggestive of autoimmune pancreatitis) or diffuse pancreatic ductal changes (suggestive of chronic pancreatitis). • The rates of unresectable disease among all patients with a CA 19-9 level ≥130 units/mL versus <130 units/mL were 26 and 11 percent, respectively. Among patients with tumors in the body/tail of the pancreas, more than onethird of those who had a CA 19-9 level ≥130 units/mL had unresectable disease. • The addition of molecular genetic analysis (eg, assay for K-ras or p53 gene mutations by RTPCR) to cytologic examination may improve sensitivity, especially in patients with small primary tumors. Screening for Extra-Colonic Cancers in FAP Exira-colonic cancer Risk Screening Recommendations Duodenal or periampullary cancer 5%-10% Upper Gl endoscopy (including side-viewing exam) every 1 to 3 years, start at age 20-25 years Pancreatic cancer About 2% Possibly periodic abdominal ultrasound after age 20 years Thyroid cancer About 2% Annual thyroid examination. start age 10-12 years Gastric cancer About 0.5% Same as for duodenal CNS cancer, usually cerebellar meduloblastoma (Turcot syndrome) <1% but RR=92 Annual physical examination. possibly periodic head CT in affected families Hepatoblastoma 1 .6% of children <5 years of age Possibly liver palpation hepatic ultrasound AFP annually , during first decade of life Screening for Extra-Colonic Cancers in HNPCC(1) Cancer Cancer Risk Endometrial cancer 43%-60% Ovarian cancer 9%-12% Gastric cancer 13%-19% Screening Recommendations Pelvic exam, transvaginal ultrasound, and/or endometrial aspirate every 1-2 years, starting at age 25-35 years Upper Gl endoscopy every 1-2 years, starting at age 30-35 years Screening for Extra-Colonic Cancers in HNPCC (2) Urinary tract cancer 4.0%-10% Ultrasound and urinalysis (urine cytology) every 1-2 years, starting at age 30-35 years Renal cell adenocarcinoma 3.3% Biliary tract and gallbladder cancer 2.0%-18% Uncertain, possibly LFTs annually after age 30 years Central nervous system (usually glioblastoma) 3.7% Uncertain, possibly annual physical examination and periodic head CT in affected families Small bowel cancer 1%-4% Uncertain, at least small bowel x-ray if symptoms occur Screening for Extra-Colonic Cancers in Peutz-Jeghers Syndrome (1) Cancer Cancer Risk stomach duodenum 2%-13% Upper Gl Endoscopy every 2 RR=1 3 years, start at age 10 years (includes colon cancer risk) Annual hemoglobin, small bowel x-ray every 2 years, both start at age 10 years Small bowel Screening Recommendations Breast: RR=8.8 Annual breast exam and mammography every 2-3 years, both start at age 25 years Pancreatic RR=100 Endoscopic or abdominal ultrasound every 1-2 years, start at age 30 years Screening for Extra-Colonic Cancers in Peutz-Jeghers Syndrome(2) Uterine RR=8,0 Ovarian RR=13 Adenoma malignum (cervix) Rare Sex cord tumor with annular tubules (SCTAT), in almost all women 20% become malignant Sertoli cell tumor (males), 10%-20% unusual become malignant Annual pelvic exam with pap smear; and annual pelvic or vaginal ultrasound and/or uterine washings, both start at age 20 years Annual testicular exam, start at age 10 years; testicular ultrasound if feminizing features occur