GI Cancers

Mutations

• PTEN - Cowden's syndrome

• STK11 - Peutz-Jeghers

• MLH1, MSH2, MSH6, PMS2 - mismatch repair genes associated with Lynch Syndrome

Signs and Symptoms

• congenital hypertrophy of the retinal pigment epithelium (CHRPE) or "bear tracks"

  • multiple hyperpigmented round/oval-shaped spots on the retina, typically bilaterally

  • typically asymptomatic

  • 90% positive predictive value for FAP

  • proceed to EGD/colonoscopy if found 

    • high frequency of duodenal adenomas and fundic gland polyps in FAP

• ampullary adenocarcinoma

  • second leading cause of cancer death in FAP

Endoscopic Findings

• Numerous fundic gland polyps in the stomach

• Colorectal and duodenal adenomas

Treatment

• Thyroid ultrasound

  • papillary thyroid cancer is associated with FAP

• Exam with side-viewing duodenoscope to rule out ampullary neoplasm

Definition

• a recessive condition 

• due to mutations in base excision repair pathway gene MutYH

Signs and Symptoms

• colonic adenomas numbering 20-100

• increased risk of duodenal adenomas and cancer

Screening and Surveillance

• Colonoscopy every 1-2 y start at age 25-30

  • proceed to colectomy if polyp burden is not manageable endoscopically

• EGD with duodenoscopy with visualization of the ampulla

• No recommendations for endometrial biopsy unless symptomatic

• CT and EUS are not recommended for screening in MAP

Definition

• large gastric hamartomatous polyps

• due to germline mutations in BMPR1A and SMAD4

• Those with SMAD 4 mutations are more likely to have...

  • polyps and cancer in the stomach

  • hereditary hemorrhagic telangiectasias (HHT)

• Incidence of gastric cancer in JPS is ~21%

Signs and Symptoms

• Family history with gastric and colon cancer

Pathology Findings

• hamartomas - glandular dilation with inflammatory infiltrate

Definition

• a non-hereditary condition

Endoscopy Findings

• thickened gastric folds

Pathology findings

• cystically dilated foveolar glands

Definition

• a non-hereditary syndrome

Signs and Symptoms

• hamartomas in the GI tract with...

  • protein losing enteropathy

  • hair loss

  • nail growth problems

Definition

• Criteria for diagnosis

  • 5 or more serrated polyps proximal to the rectum all 5 mm or larger (2 or more being 10 mm or larger)

  • more than 20 serrated polyps of any size through the large intestine with 5 or more proximal to the rectum

• A clear genetic basis for this syndrome has not been identified

• 80% of SSPs are located in the proximal colon

• SSPs with dysplasia have high malignant potential

• Traditional SSPs  are more often in the distal colon, also with high malignant potential

Surveillance

• First degree relative (whichever is earliest)

  • age 40

  • at age of diagnosis of youngest affected relative

  • 10 years prior to earliest CRC diagnosis in affected relatives 

    • If no polyps are found proceed with surveillance every 5 years

    • If many adenomas or proximal SSPs are found proceed with surveillance every 1-3 years

• Colectomy for those where endoscopic surveillance is not possible

• No role for FIT-DNA stool testing

Definition

• Caused by a mutation in CDKN2A (also known as p16)

• multiple atypical moles with increased risk of melanoma and pancreatic cancer

• risk of pancreatic cancer is ~13-39 fold increased over the general population

  • vs BRCA1 mutation carriers, risk increased by 2 fold

Definition

• Amsterdam criteria

  • three relatives affected with Lynch related tumors

    • cancers of the colon, rectum, small bowel, stomach, pancreas, biliary tract, endometrium, ovary, ureter, renal pelvis, brain, sebaceous glands, and keratocanthomas

  • two successive generations 

    • one must be a 1st degree relative of the other two

    • one ore more cancers must be diagnosed under 50 yo

• mutations in mismatch repair genes increases risk of pancreatic cancer by 11 fold

Risk Factors

• microsatellite instability on pathology (personal or specimen from family member)

Treatment

• Genetic counseling

• Test for Lynch related mutations in mismatch repair genes

  • MLH1, MSH2, MSH6, PMS2 genes

  • Absent MSH2 and MSH6 most likely consistent with germline MSH2 mutation

  • test one affected family member first to see which gene mutation is responsible

• EGD

• EUS of pancreas

Surveillance

• Colonoscopy every 1-2y starting at 20-25yo

• EGD with antral biopsies every 2-3y starting at 30-35yo

• Annual pelvic exam with endometrial sampling and transvaginal ultrasound starting at 30-35yo

• Annual urine analysis starting at 30-35yo

Definition

• two or more hamartomatous polyps in the small bowel or colon

• due to mutation in STK11 gene

• associated with increased risk of certain cancers

  • colorectal, small bowel, stomach

  • pancreatic - risk increased by 132-fold

  • breast, ovarian, endometrial, cervical

  • testicular

  • lung cancers

Signs and Symptoms

• mucocutaneous pigmentation of the mouth/lips and fingers

• freckling of the mouth and buccal mucosa

• hamartomas, especially of the small intestine

Pathology Findings

• polyp - arborizing pattern of smooth muscle proliferation

Surveillance

• Every 1-3 yrs or more frequently of polyps found - colonoscopy, EGD, small bowel imaging

• Every 1-2 yrs starting at age 35 - MRI/MRCP to screen for pancreatic cancer 

• Annual breast self exams and breast imaging

• Annual transvaginal ultrasounds

• Annual pelvic exams and pap smears

Definition

• Due to a mutation in the PTEN gene

• Cumulative colorectal cancer risk is 9-16%

Signs and Symptoms

• hamartomas and ganglioneuromas in the GI tract

• glycogenic acanthosis of the esophagus

  • benign

  • multifocal plaques of hyperplastic squamous epithelium with abundant intracellular glycogen deposits

• macrocephaly

• trichilemmomas

  • a benign tumor originating from the outer root sheath of the hair follicle

• increased risk for certain cancers

  • breast, endometrial  

  • thyroid

  • renal cell 

  • melanoma

Definition

• Pathogenic variant in CDH1 gene

• lifetime risk of diffuse gastric cancer in HDGC is 70% in men and 56% in women

• risk of lobular breast cancer in women is 42%

Signs and Symptoms

• Family history

  • diffuse gastric cancer

  • lobular breast cancer under age 50

Treatment

• Total gastrectomy recommended in CDH1 mutation carriers to reduce risk

  • optimal age for surgery not established

  • should be done in young adulthood

  • if gastrectomy not done, should have surveillance with Cambridge protocol (at least 30 gastric biopsies)

Risk Factors

• caustic ingestion, particularly strong bases (ex. lye ingestion)

  • markedly increases risk by 1000 fold

  • surveillance with upper endoscopy recommended beginning 20 y after ingestion

    • include mucosal inspection with narrow band imaging or chromoendoscopy

  • majority associated with lye ingestion are located in the proximal esophagus at the area of the bronchial bifurcation 

• tobacco and EtOH use (more strongly associated with esophageal SCC than adenocarcinoma)

Treatment

• Carcinoma in situ without deep invasion or metastases to lymph nodes or other areas

  • risk of lymph node metastasis in this case is small

  • survival rate is excellent

  • Fist line: endoscopic mucosal resection (EMR) of any visible nodularity within the Barrett's segment as well as radiofrequency ablation

    • If EMR specimens do not show invasive cancer, this is curative

    • resection of the entire Barrett's segment is not necessary

  • Endoscopic submucosal dissection is associated with a higher stricture rate

Definition

• incidence has increased significantly over the last several decades

• least common GI cancer in women

• in developed countries, esophageal adenocarcinoma is more common than esophageal SCC

  • in the US and Western Europe, rates of adenocarcinoma are increasing and rates of SCC are decreasing 

• Tumor length of ≤ 2 cm is a predictor of long-term survival

Risk Factors

• Male sex

• obesity (more strongly associated with esophageal adenocarcinoma)

• Chronic GERD

• Barrett's esophagus (more strongly associated with esophageal adenocarcinoma)

• tobacco  (more strongly associated with esophageal SCC, moderate risk for adenocarcinoma)

• More common in Whites than in Blacks

Treatment

• EUS with FNA is more sensitive for nodal staging of esophageal cancer than CT

Definition

• Helicobacter pylori infection causes almost 90% of non-cardia cancers (the predominant type)

• rates highest in... 

  • hispanics (from Central and South American in particular)

  • East Asian populations (particularly recent immigrants)

  • African Americans

• Non-hispanic Whites have the lowest rate of gastric cancer

• Selenium and high consumption of fruits/vegetables may have a protective effect

Risk Factors

• H pylori infection

• Lynch syndrome

• Peutz-Jeghers syndrome

• Menetrier's disease

• germline mutations in CDH gene, which encodes E-cadherin

Endoscopic Findings

• Routine repeat EGD not recommended to confirm ulcer healing in PUD unless there is concern for associated cancer

• Sydney protocol - sampling from specific ares in the anturm and corpus for H pylori detection

Pathology Findings

Laboratory Findings

• serum E-cadherin levels are NOT used for gastric cancer detection

Screening

• Screening for gastric cancer is not recommended outside of certain high risk populations...

  • strong family history of gastric cancer

  • individuals with familial adenomatous polyposis or Lynch syndrome

  • those with extensive intestinal metaplasia

• Screening for gastric cancer does take place in Japan

  • incidence of gastric cancer is much higher here and results in detection of more early tumors in asymptomatic patients 

Endoscopic Findings

• Gastric mapping - used for surveillance of gastric intestinal metaplasia

  • sampling of specific areas in the antrum, incisura, and corpus (all in separate jars)

Definition

• derived from B cell clones

• ~80% of gastric lymphomas may regress with H pylori eradication 

• carry a more favorable prognosis than gastric adenocarcinomas

• MALT lymphomas

  • presence of 11:18 chromosome translocation, among others

Risk Factors

• H pylori infection

Definition

• small intestine cancer is overall rare in the US

• the most common tumor type is adenocarcinoma 

  • accounts for 30-40% of small bowel cancers

• most commonly found in the duodenum

Risk Factors

• Patients with FAP who have undergone colectomy

  • duodenal adenocarcinoma is an important cause of death

    • 50-90% lifetime risk of duodenal adenomas

    • 3-10% of these will develop adenocarcinoma

  • these patients merit surveillance endoscopy with a side viewing duodenoscope

• Lynch syndrome - 100 fold increased risk

• Crohn's disease - relative risk ~30

• Celiac disease - relative risk 60-80 fold

  • also associated with enteropathy associated T cell lymphomas

Definition

• carries a poor prognosis

Risk Factors

• associated with celiac disease

Definition

• most common mesenchymal tumor

• found most commonly in the stomach

  • gastric GISTs have a better prognosis than those in the small intestine

  • can cause bleeding due to ulceration

• malignant potential and decreased survival associated with size >2cm  and high mitotic index (more than 5/50 high power field)

• can occur in the small intestine but are less common than adenocarcinoma

• EUS with biopsy is preferred for diagnosis

Pathology Findings

• spindle cells

• CD117 positive

• KIT mutations

• High risk features

  • lobulated or irregular borders

  • invasion into adjacent structures

  • heterogeneity 

• FNA may be suboptimal, core biopsy is acceptable 

Treatment

• Resection for lesions that are

  • symptomatic

  • more than 2 cm in size

  • have high risk features on EUS

• Lesions <2cm

  • surveillance with EUS annually

• Metastatic or recurrent lesions

  • imatinib

  • sunitinib for lesions unresponsive to imatinib

  • regorafenib for lesions unresponsive to imatinib and sunitinib

Definition

• most common site is the ileum

• non-metastatic lesions do not causes symptoms

  • inactivates serotonin delivered via the portal vein

• often metastasized at the time of diagnosis

  • hepatic metastasis allows for serotonin to be delivered into the systemic circulation and lead to symptoms of carcinoid syndrome

  • Vs. hindgut carcinoids which are not typically metastasized

Endoscopic Findings

• yellowish submucosal nodule with overlying ulceration in the terminal ileum

Pathology Findings

• keyhole biopsies

• uniform small round cells with rare mitotic figures

• dense desmoplastic reaction

• immunohistochemistry staining postivie for...

  • synaptophysin 

  • neuron-specific enolase

  • chromogranins A, B, and C

Definition

• Most common GI cancer in the US

Risk Factors

• Alaskan natives have the highest incidence and mortality for colorectal cancer in the US

Endoscopic Findings

• Sessile serrated polyps, or sessile serrated adenomas

  • sessile or flat

  • few or no blood vessels

  • sometimes a mucus cap

  • more difficult to detect with colonoscopy

Pathology Findings

• Malignant polyp

  • polyp with cancer invading through the muscularis mucosa and into the submucosa

  • Unfavorable histologic features

    • poorly differentiated

    • presence of angiolymphatic invasion

    • positive margin of resection - tumor less than 1-2mm from the transected margin or tumor cells present at the margin

• Serrated lesions

  • account for ~30% of colorectal cancers

  • Types:

    • hyperplastic

      • not premalignant 

    • Sessile serrated polyps (SSP), also know as sessile serrated adenomas (SSA), with or without dysplasia

      • more commonly found in the proximal colon

      • BRAF mutations and CpG island methylation

      • do NOT have KRAS mutations

    • Traditional serrated adenomas

Treatment

• Malignant polyp with unfavorable histologic features

  • surgical resection 

• Malignant polyp with favorable features

  • endoscopic resection is curative 

  • there is controversy in the case of sessile malignant polyps (surgical vs endoscopic resection with favorable histology)

Screening 

• General population

  • start screening at age 45

• Family history of colon cancer in a first degree relative diagnosed < 60 yo

  • start screening at 40 yo or 10 y prior to earliest age of diagnosis

  • surveillance every 5 y thereafter

• Family history of colon cancer in a first degree relative diagnosed > 60 yo

  • Start screening at age 40 using options/intervals for average risk individuals

• African Americans

  • 2017 US MSTF guidelines - start screening at age 45 

  • higher incidence rates, earlier mean age of onset, worse survival and late-stage presentation, and higher proportion of presentation before age 50

• FIT-fecal DNA has a higher sensitivity and lower specificity compared to FIT testing alone

  • One time FIT-fecal DNA: sensitivity 92%, specificity 89.8% with normal colonoscopy 

  • One time FIT: sensitivity 74%, specificity 95% with FIT alone

• FIT testing annually

• FIT-Fecal DNA every three years

• Adenoma detection rate in average risk screening patients should be 

  • 25% or more overall

  • 30% or more in men

  • 20% or more in women

Surveillance 

• IBD with primary sclerosing cholangitis (PSC)

  • colonoscopy at diagnosis of PSC then annually with chromoendoscopy

• Family history of colon cancer

  • surveillance interval of at least 5 y

• After resection for colon cancer

  • colonoscopy at 1 yr and 3 yr to decrease the risk of metachronous colorectal cancer

  • FIT is not recommended for surveillance after colon cancer resection

• After resection of obstructing colon cancer precluding complete colonoscopy at diagnosis

  • colonoscopy at 3-6 mo

• See additional guidelines from ASGE

Definition

• prevalence is ~4-7% 

• most found incidentally 

• can be benign or neoplastic

• can be pseudopolyps including...

  • cholesterol polyps

  • inflammatory polyps

  • adenomyoma

    • benign or true polyps including adenocarcinomas or adenomas which have malignant potential

• risk of malignancy associated with...

  • age >50

  • polyp size >1 cm

  • coexisting gallbladder stones

  • solitary polyp 

  • lower polyp number

  • symptomatic polyp

  • PSC

  • polyp growth over time

Treatment

• Cholecystectomy in cases with...

  • polyps ≥1cm

  • polyps <1cm with symptoms 

  • symptoms

  • presence of gallstones

Surveillance

• Ultrasound surveillance for polyps <1cm in size without symptoms or other high risk features

Definition

• pancreatic cancer is the second most common GI cancer in the US

• 90% of pancreatic cancers are adenocarcinomas

• poor prognosis is primary due to diagnosis at a disease stage that is not resectable

  • local invasion

  • vascular involvement

  • metastasis

• Diagnose on CT, MRCP, EUS

Signs and Symptoms

• weight loss

• vague gastrointestinal symptoms

• elevated blood sugar

  • strong association between new onset non-insulin dependent diabetes  and pancreatic cancer, especially in...

    • elderly

    • with low BMI

    • weight loss

    • without family history  of diabetes

• Trousseau's syndrome

  • paraneoplastic condition characterized by migratory superficial thrombophlebitis

  • associated with pancreatic, gastric, and lung cancer

Risk Factors

• male sex

• smoking

  • in patients with a family history, smoking increases incidence and is associated with earlier presentation 

• strong family history of pancreatic cancer

• African American race

• obesity

• older age

• history of chronic pancreatitis

• Lynch syndrome

• Peutz-Jeghers syndrome

• Familial atypical multiple mole melanoma syndrome 

Signs and Symptoms

• Jaundice, scleral icterus

• anorexia

• weight loss

• Hemosuccus pancreaticus

  • rare complication of locally invasive pancreatic cancer

Imaging Findings

• CT: biliary dilation and heterogeneous mass in the head of the pancreas

Lab Findings

• elevated direct bilirubin and alkaline phosphatase 

• CA 19-9

  • limited value for screening

  • can be useful as a biomarker for recurrence in those with known disease

Treatment

• Pancreaticoduodenecotmy (Whipple) procedure

  • only 15-20% of patients are candidates

  • offers potential of cure

• Chemotherapy 

  • offers survival benefit against some forms of pancreatic adenocarcinoma

  • also has a role in adjuvant and palliative treatment

  • gemcitabine

  • capecitabine

Screening

• familial pancreatic cancer, 1st degree relative

  • annual EUS or MRCP should be offered at age 50 or 10 y prior to youngest affected family member

Diagnosis

• surgical laparoscopy for staging and possible resection  if anatomy is conducive and patient is a good surgical candidate

• ERCP with cholangioscopy and stenting is an alternative for tissue diagnosis and biliary decompression

• percutaneous or EUS guided FNB carries risk of seeding

  • do not proceed was this will preclude possible liver transplantation

• EUS for FNB of abnormal lymph nodes is reasonable 

Risk Factors

• PSC

  • 6-30% lifetime risk of cholangiocarcinoma

• anatomical changes of the bile duct 

  • choledochal cysts

  • Caroli's disease (multiple cystic dilations of the biliary tree)

• infection with liver flukes increases risk by 2-5 fold

  • Clonorchis sinensis - primarily found in Korea

  • Opisthorcis viverrini - primarily found in Thailand

• tobacco use

• asbestos exposure 

• thorium dioxide or "Thorotrast" exposure

  • previously used as a radiologic contrast agent

• Lynch syndrome

  • lifetime risk of biliary cancer is ~1-4% in those with MLH1 and MSH2 mutations

• Obesity

• Chronic liver disease, cirrhosis

Definition

• arises in the setting of cirrhosis

Imaging Findings

• CT - heterogeneous liver mass with portal venous enhancement 

Treatment

• surgical resection is potentially curative for iCCA

  • if cirrhosis and portal hypertension are present, there is high surgical risk

  • controversy regarding whether iCCA is a contraindication for liver transplantation due to high risk of recurrence 

  • recent studies suggests good survival with cirrhosis and in highly selected patients with early iCCA

    • without vascular invasion

    • without lymph node invasion

    • without extrahepatic spread

    • no history of prior attempted resection which might violate the tumor plane

    • single lesion ≤ 3 cm

• locoregional therapies (TACE and radiofrequency ablation) are reasonable in more advanced iCCA who are not candidates for resection or transplant

  • while awaiting transplant, locoregional therapies may be employed as a bridge to transplant

• Gemcitabine and cisplatin - first line chemotherapy in those with advanced CCA who are not candidates for locoregional therapy or surgery

Pathology Findings

• cytology from ERCP brushing of stricture may be negative

  • sensitivity of conventional cytology in diagnosis of malignant biliary strictures is 15-38%

  • supplementation of FISH studies increases the sensitivity of routine cytology

• polysomy on FISH studies in the setting of dominant structure

  • if no other definitive evidence of malignancy, further evaluation with ERCP with biliary brushings and cytology in 3-4 mo is required

  • polysomy on serial exams in patients with PSC is associated with an increased risk of CCA compared with those with PSC who do not have serial polysomy (69% vs 18%)

• percutaneous or endoscopic sampling of suspicious biliary strictures is not recommended due to risk of peritoneal seeding

Lab Findings

• in patients with PSC, CA 19-9 (cutoff 129 U/mL) has a sensitivity of 79-98% in diagnosis of CCA

  • however, ~1/3 of those with sustained elevation in CA 19-9 do not develop CCA

  • ~7% of the population are unable to express CA 19-9 due to lack of activity of fucosyltransferase 2 and 3

Treatment

• liver transplantation preceded by neoadjuvant chemoradiation preferred over surgical resection

Definition

• hepatitis B drives much of the global burden of HCC

  • chronic HBV infection via vertical transmission is the predominant mechanism for HCC in developing nations, increasing risk of HCC by ~100 fold

  • HBV (DNA virus) integrates into the genome of the host, exerting a direct carcinogenic effect

• Aflatoxin B1 is a fungal toxin produced by certain Aspergillus species 

  • more common in developing countries due to mold contamination of stored food

  • co-exposure to aflatoxin and HBV dramatically increases risk of HCC

• in the US, HCV is the most common etiology of cirrhosis and risk factor for HCC

  • EtOH is responsible for ~20% of HCC cases

  • NAFLD is responsible for ~10% of HCC cases (though this is a rising cause of cirrhosis)

Imaging Findings

• on triple phase CT or MRI, hepatoma has...

  • arterial enhancement

  • washout on late images

  • enhanced capsule

  • Versus: hemangioma which appears 

    • well demarcated with peripheral enhancement

    • homogeneous more often than heterogenous on later phases

    • delayed washout

    • hyperechoic

  • Versus: Focal nodular hyperplasia which has

    • arterial filling with a central scar

    • can be iso- or hypodense on delayed phases

  • Versus: hepatocellular adenoma

    • benign but at risk of degeneration when it reaches 5 cm in diameter or more

    • arterial enhancement 

    • hypodense on hepatobiliary phase relative to the liver tissue

Treatment

• evaluation for liver transplant regardless of decompensation

  • liver transplant is effective for those within Milan criteria

    • 3 lesions each < 3 cm or

    • 1 lesion < 5 cm

    • without evidence of vascular invasion or extrahepatic disease

• Bridge to liver transplantation to control tumor burden

  • Ablation with/without transarterial chemoembolization (TACE)

  • TACE recommended for nonsurgical candidates with

    • reasonable liver function

    • multinodular tumors beyond Milan criteria

• Surgical resection for those with a single mass

  • < 5 cm

  • in the setting of good liver function and absence of clinically significant portal hypertension

• Sorafenib

  • multi-kinase inhibitor with antiproliferative properties 

  • reserved for those with advanced HCC who...

    • are not candidates for locoregional treatment

    • have good liver function

Screening

• All patients with advanced liver fibrosis or cirrhosis should be offered HCC screening

  • cirrhotics have a 5% risk annually for developing hepatoma

• Liver imaging (ex. ultrasound) every 6 mo with or without serum AFP 

• Screen in...

  • African immigrants with HBV after age 20

  • patients with eradicated HCV as they continue to be at risk for HCC

• Stop screening in those with limited life expectancy 

Definition

• related to occupational exposure to vinyl chloride, an agent used in chemical manufacturing

• may be misdiagnosed on imaging as cavernous hepatic hemangiomas, but these would not be expected to be associated with LFT elevation, ascites, or splenic nodules

Signs and Symptoms

• Intraperitoneal bleeding - risk is ~25% and can complicated needle biopsy attempts

Treatment

• Local excision 

• Transarterial embolization, particularly in the case of intraperitoneal bleeding

• May be a contraindication to liver transplant given poor outcomes

Definition

• Type 1 neuroendocrine tumors

  • well-differentiated

  • often multifocal

  • associated with chronic atrophic gastritis

• Type 2 neuroendocrine tumors

  • associated with Zollinger-Ellison syndrome and multiple endocrine neoplasia 1 (MEN1)

• Type 3 and 4 neuroendocrine tumor

  • poor prognosis

Signs and Symptoms

• Associated conditions

  • MEN1 - nonfunctioning NET (80-100%), gastrinoma (50-60%), insulinoma (20%)

    • MEN 2A and 2B are NOT associated with NET 

  • VHL

  • NF1

Pathology Findings

• solid nests of monomorphic endocrine cells

Treatment

• Type 3 and 4

  • surgical resectio

Treatment

• pancreaticoduodenectomy

  • high rate of metastases seen even with small lesions

  • transduodenal ampullectomy offers an inadequate oncological resection, generally not recommended as first line

  • endoscopic resection not recommended given high rate of metastases and inability to assess lymph nodes. Reserved for those who are not surgical candidates

Treatment

• endoscopic resection via ampullectomy

• pancreaticoduodenectomy or surgical ampullectomy if cannot be removed endoscopically (ex. invasive carcinoma involving the pancreaticobiliary axis)

Definition

• Now called marginal zone B cell lymphoma of MALT type

• test and treat for H pylori in those with low grade MALT lymphoma or history of endoscopic resection of early gastric cancer

  • insufficient evidence to support screening family members