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 cancer-related 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 one-third 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 RT-PCR) 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 RecommendationsDuodenal or peri-ampullary 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

Screening Recommendations

Endometrial cancer 43%-60% Pelvic exam, transvaginal ultrasound, and/or endometrial aspirate every 1-2 years, starting at age 25-35 yearsOvarian cancer 9%-12%

Gastric cancer 13%-19% 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 Screening Recommendations

stomach duodenum

2%-13%RR=1 3 (includes colon cancer risk)

Upper Gl Endoscopy every 2 years, start at age 10 years

Small bowel Annual hemoglobin, small bowel x-ray every 2 years, both start at age 10 years

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 Annual pelvic exam with pap smear; and annual pelvic or vaginal ultrasound and/or uterine washings, both start at age 20 years

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), unusual

10%-20% become malignant

Annual testicular exam, start at age 10 years; testicular ultrasound if feminizing features occur