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Tumors of the small and large intestine

- Epithelial tumors – Major cause of morbidity and mortality

- Colon: More primary neoplasms than any other organ in the body

Tumors of the small intestine

- Only 3-6% of intestinal tumors
- Benign
- Most common: Adenomas and mesenchymal tumors
- Remainder: Lipomas, angiomas, rare hamartomatous mucosal lesions

- 25% of benign small intestinal tumors
- Next in order:
o Benign mesenchymal tumors (Leiomyomas)
o Lipoma
o Neuromatous lesions
- Most occur at the ampulla of Vater
- Usual presentation:
o 30-60 yo
o Occult blood loss rarely with obstruction or intusussception
- Patients with familial polyposis coli are particularly prone to developing
periampullary adenomas
- Macroscopic: Ampulla of vater
o Enlarged, exhibits a velvety surface
o Microscopic: Extension of adenomatous tissue into the ampullary orifice,
rendering surgical excision difficult, short of a pancreatoduodenectomy to
the clon
- Small intestinal adenoma: A premalignant condition
- Patients with adenoma: (+) Fatigue from occult blood loss may be the only sign

- Occur in the duodenum usually
- 40-70 yo
- Grow in a napkin ring encircling pattern or as polypoid exophytic masses in a
manner similar to colonic cancers
- Duodenal tumors involving the ampulla of Vater, may cause obstructive jaundice
early in the course
- Presenting agent: Intestinal obstruction
- Symptoms:
o Crampy pain
o Nausea
o Vomiting
o Weight loss
- Rarely, the tumoroid mass is a lead point for intusussception
- Major risk for factor: Chronic inflammation associated with CD
o Celiac disease
o Familial adenomatous polyposis
o Hereditary non polyposis colorectal cancer syndrome (HNPCC) aka Lynch
 Devt. Of colorectal CA, endometrial CA, Small intestine CA, ureter,
o Peutz-Jegher’s syndrome
o Alcohol, and smoking
- At the time of diagnosis, most tumors have already penetrated the bowel wall,
invaded the mesentery or other segments of the gut, spread to regional nodes and
sometimes mets to the liver and even more widely.
- Wide en bloc excision yields about a 70% 5 year survival rate
Tumors of the colon and rectum
- Colorectal CA
- Polyps: Of the colorectal mucosa are extraordinarily common in the older adult.
- Definition of terms:
o Polyp: A tumorous mass that protrudes into the lumen of the gut.
 Usually start as small, sessile lesions without a definable stalk
 Pedunculated due to traction
o Polyp formation: Abnormal mucosal maturation, inflammation, or
architecture, they are non neoplastic and do not have malignant potential
per se. Ex: Hyperplastic polyp
o Adenomatous polyp: Epithelial polyps that arise as a result of proliferation
and dysplasis.
 True neoplastic lesions and are precursors of CA
- Non neoplastic polyps:
o Hyperplastic polyp – 90%, 6th and 7th decade
 Due to decreased epithelial cell turnover and accumulation of mature
cells on the surface
 Morphology: Small, (<5mm dm), nipple like, hemispheric, smooth,
moist protrusions of the mucosa usually positioned on the tops of the
mucosal folds.
 Occur singly, more often multiple
 Often found in the rectosigmoid colon
o Histologic: Composed of well formed glands and crypts lined by non
neoplastic epithelial cells, most of which show differentiation into mature
goblet or absorptive cells.
 The delayed shedding of surface epithelial cells leads to infoldings of
the crowded epithelial cells and fission of the crypts creating a serrated
epithelial profile and irregular crypt architecture
 NO MALIGNANT potential
o Hamartomatous / Juvenile polyp – Malformations of the glands and the
stroma, occur usually in genetic syndromes.
 Occurs in children younger than 5yo
 Aka retention polyps
 80% in the rectum, but may also be scattered throughout the entire
 Large 1-3cm dm
 Inflammation is common, surface may be congested or ulcerated
 No malignant potential
 Rare: Juvenile polyposis syndrome  Multiple (50-100) juvenile polyps
• Increase risk for adenoma formation, precursor for adenoCA
• Gene mutation of SMAD/DPC4 gene
o Inflammatory polyp (Pseudopolyp) – Represents islands of inflamed
regenerating mucosa surrounded by ulceration.
 Seen in active IBD
o Lymphoid polyp – Normal variant of the mucosal bumps containing
intramucosal lymphoid tissue
o Peutz-Jeghers polyps: Hamartomatous polyps involving the mucosal
epithelium, LP, Muscularis mucosa
 Multiple hamartomatous polyps scattered throughout the entire GIT
and melanotic mucosal and cutaneous pigmentation around the lips,
oral mucosa, and face, genitalia, palmar surface of the hands.
 High risk for intusussception (Common cause of moratlity)
 Large and pedunculated
 Firm lobular contour
 No malignant potential but has increased risk of other CA’s like breast,
lung, ovarian
 Gene mutation STK11 (LKB1) on chromosome 19
- Two other hamartomatous polyps:
o Cowden syndrome – Autosomal dominant genetic syndrome
 Multiple hamartomatous involving organs derived from all three
germinal layers
 Common sites: GIT and mucocutaneous locations
 Characteristic: Intestinal hamartomatous polyps, facial
trichilemmomas, acral keratosis, and oral papillomas
 Predisposes the development of thyroid and breast CA
 Genetic abnormality: PTEN on chromosome 10
o Cronkhit-Canada syndrome
 A non hereditary d/o
 (+) GI hamartomatous polyposis and ectodermal abnormalities (eg nail
atrophy, skin pigmentation, and alopecia)
 Etiology unknown

Adenomas (Adenomatous polyp)

- Intraepithelial neoplasms that range from small, often pedunculated lesions to
large neoplasma that are usually sessile.
- Prevalence: 20-30% before age 40, rising 40-50% after age 60.
- Equal M-F ratio
- With a well defined familial predisposition to sporadic adenomas, four fold greater
risk among first degree relatives
- Four fold risk of colorectal CA
- 3 subtypes:
o Tubular adenomas
 75% tubular architecture
 Small and pedunculated
 Most pedunculated polyps
 Morphology:
• 90% found in the colon, may occur in the stomach and small intestine
(ampulla of Vater)
• Single, or at random
• Composed of neoplastic (dysplastic) epithelium which lines glands as
a tall, hyperchromatic somewhat disordered epithelium that may or
may not show mucin vacuoles.
• Intramucosal CA
o Villous adenomas
 50% villous structure
 Only 1% of adenomas
 If greater than 4cm  very worrisome.
 Larger and more ominous of the epithelial polyps
 Older persons
 Usually in the rectum and rectosigmoid colon
 Sessile, up to 10cm dm
 Histology: Frondlike viliform extensions of the mucosa covered by
dysplastic, sometimes very disorderly columnar epithelium
o Tubulovillous adenoma (Mixture)
 25-50% villous architecture
 Only 5-10% of adenomas
 The risk of harboring in situ or invasive CA generally correlates with the
proportion of the lesion that is villous.
- All adenomatous lesions arise as the result of epithelial proliferative dysplasia
o May range from low to high grade dysplasia (CA in situ)
- Precursor lesions for invasive colorectal adeno CA
- Period of development to CA: 10 years
- Malignancy of an adenomatous polyp depends on:
o Polyp size
o Histologic architecture
o Severity of epithelial dysplasia
 CA is rare in tubular adenomas < 1cm
 The risk of CA is high in sessile villous adenomas more than 4cm in dm
 Severe dysplasia, when present is often found in villous areas
- Clinical features:
o Colorectal tubular and tubulovillous
 Asymptomatic
 Discovered during evaluation of anemia or occult bleeding
o Villous adenomas
 Symptomatic
 Often discovered because of overt rectal bleeding
 Rarely, they may hypersecrete copious amounts of mucoid material
rich in protein and potassium that leads to hypproteinemia or

Familial syndromes
- Familial polyposis syndrome
- Autosomal dominant
- Familial Adenomatous Polyposis syndrome (FAP)
o The archetype of the adenomatous polyposis syndrome
o Mutation of Adenomatous polyposis coli (APC) gene on the chromosome
o Classification:
 Classic FAP – development of 500-2500 colonic adenomas that carpet the
mucosal surface
• Minimum of 100 polyps is necessary for a diagnosis of classic FAP
• Multiple adenomas may also present elsewhere in the alimentary tract
even in the egion of ampulla of vater.
• Histologic: Vast majority are tubular adenomas
• Some patients already have Colon CA or rectum at the time of dx
• May also have polyps in the stomach (Adenomas or fundic gland polyps)
and small intestine (around ampulla of vater)
 Attenuated FAP
• Fewer polyps (average is 30)
• Located on the proximal colon
• Cancer risk development of 50%
 Gardner syndrome
• Exhibit intestinal polyps identical to those in classic FAP
• (+) Multiple osteomas (Mandible, skull, and long bones) epidermal
cysts, and fibromatosis
• Less frequent are abnormalities of dentition like unerupted and
supernumerary teeth and a higher frequency of duodenal and thyroid
 Turcot syndrome
• Combination of adenomatous colonic polyposis and CNS tumors
• 2/3 of patients have APC mutations and develop brain
• 1/3 have mutations in one of the genes associated with HNPCC and
develop brain gliastomas
- Hereditary Nonpolyposis Colorectal Ca (HNPCC)
o Autosomal dominant familial syndrome
o A.k.a. Lynch syndrome
o Increased risk of colorectal CA and extraintestinal CA (Endometrium)
o Often multiple and are not usually associated with pre existing adenomas
o Hallmark: Mutations in DNA repair genes leading to microsatellite instability

Colorectal Carcinogenesis
- Pathologic basis: Adenoma-Carcinoma sequence
- The occurrence of colorectal CA without evidence of adenomatous precursors
suggests that some dysplastic lesions can degenerate into malignancy without
passing through a polypoid stage

Molecular Carcinogenesis
- First pathway: APC / B-caterin pathway
o Characteristics
 Chromosomal instability that results in stepwise accumulation of
mutations in a series of oncogenes and tumor suppressor genes
 Stages: (Adenoma-Carcinoma sequence)
• Localized colon epithelial proliferation
• Formation of small adenomas
• Progressively enlarge
• Becomes more dysplastic
• Development of invasive cancers
 The genetic correlates of this pathway:
• Loss of adenomatous Polyposis Coli (APC) Gene
o Mapped to 5q21
o Earliest event in the formation of adenomas
o A tumor suppressor gene
o Regulates B catenin, and important mediator of the Wnt/Beta
catenin signaling pathway (For normal epithelial development)

• K-RAS mutation
o Most frequently observed activated oncogene in adenomas and colon
• SMADs mutation
o A common allelic loss in colon CA is on 18q21
o Involves TGF-Beta signaling located on 18q21
o Deficient of SMAD4 increase GIT tumorigenesis
• p53 loss
o Loss at chromosome 3p which affect p53 gene
• Telomerase activation
o Plays a role in stabilizing the chromosome; they shorten with
each cell division until cell senescence develops
o Its activity is required to maintain telomere stability and hence cell
immortality, a prerequisite for CA cells
- 2nd pathway: Microsatellite Instability Pathway
o Genetic lesions in the DNA mismatch repair genes
o HNMPCC syndrome
o As in the APC/Beta catenin schema, there is accumulation of mutations but
the involved genes are different
o No clearly identifiable morphologic correlates
o Human mismatch repair genes:
 hMSH2 (Chromosome 2p22) -- majority
 gMLH1 (chromosome 3p21) -- majority
 MSH6 (chromosome 2p21)
 hPMS1 (chromosome 2q31-33)
 hPMS2 (chromosome 7p22)
o Mismatch of the former 2 cause alteration of microsatellites, leading to
microsatellite instability.

Colorectal CA
- 98% are adeno CA’s
- Usually arise in polyps and produce symptoms relatively early and at a stage
generally curable by resection.
- Epidemiology, Etiology and Pathogenesis
o Peak incidence 60 and 79 yo
o If found in a young person, pre existing ulcerative colitis or one of the
polyposis syndromes must be suspected
o M-F ratio: 1.2:1
o WW distribution
o Environmental factors: Dietary practices
 Excess dietary caloric intake relative to requirements
 A low content of unabsorbable vegetable fiber
 A corresponding high content of refined carbohydrates
 Intake of red meat
 Decreased intake of protective micronutrients
o Aspirin use or other NSAID’s
- Morphology:
o Distribution:
 Cecum / Ascending colon 22%
 Transverse colon 11%
 Descending colon 6%
 Rectosigmoid colon 55%
 Other sites 6%
o Right sided colon CA tend to have a greater microsatellite instability
o 99% single occurrence
o Although colorectal CA’s begin as in situ lesions, they evolve into different
morphologic patterns.
o Tumors in the proximal colon:
 Polypoid, exophytic masses that extend along one wall of the
capacious cecum and ascending colon
 Obstruction uncommon
 When CA in the distal colon are discovered, they tend to be annular,
encircling lesions that produce the so-called napkin-ring constrictions
of the bowel.
 Histologic: Right and left sided colonic adenoCA are similar.
o Invasive tumor incites a strong desmoplastic stromal response, leading to a
characteristic, firm, hard consistency of most colonic carcinomas.
o (+) Mucin production, (+) signet ring appearance.
- Clinical Features
o Asymptomatic for years
o Insidious
o Cecal and right colonic CA: Fatigue, weakness, iron deficiency anemia.
 They bleed readily and may be discovered at an early stage
o Left sided lesions: Pccult bleeding, changes in bowel habit, crampy left lower
quadrant discomfort.
 Have prominent bowel disturbances in bowel function like melena,
diarrhea, and constipation
o CA’s of rectum and sigmoid tend to be more infiltrative at the time of
diagnosis than proximal lesions  poorer prognosis
o Mets: Lymphatics and blood vessels, favored sites: Regional LN, liver, lungs,
bones followed by serosal membrane of peritoneal cavity, brain, and others.
o The single most important prognostic indicator of colorectal carcinoma is the
extent of the tumor at the time of diagnosis, the so called stage (TNM)

Carcinoid Tumors
- A carcinoma like lesion but with a much more indolent clinical course
- From resident endocrine cells with GIT and lung as the predominant sites of
- Peak at 6th decade
- May be confined to the mucosa and submucosa or may be malignant in behavior
with deep invasion and metastatic spread to regional lymph nodes and the liver.
- No reliable histologic difference between seemingly benign and malignant
carcinoid tumors.
- Tendency for aggressive behavior depends on:
o Site of origin
o Depth of local penetration
o Size of the tumor
o Histologic features of necrosis and mitosis.
- Appendiceal and rectal carcinoids infrequently metastasize, even though they may
show extensive local spread.
- 90% of ileal, gastric, and colonic carcinoids that have penetrated the muscle wall
have spread to lymph nodes and distant sites like the liver especially if they are
greater than 2cm.
- Morphology:
o Appendix: most common site, next is the small intestine (ileum), rectum,
stomach, colon.
o In the appendix they appear as:
 Bulbous swellings of the tip, which frequently obliterate the lumen.

- Clinical features:
o Vasomotor disturbances (Cutaneous flushes and apparent cyanosis [most
o Intestinal hypermotility (Diarrhea, cramps, nausea, vomiting)
o Asthmatic bronchoconstrictive attacks (Couth?, wheezing, dyspnea)
o Hepatomegaly (Nodular liver owing to hepatic mets
o Systemic fibrosis
 Cardiac involvement
• Pulmonic and tricuspid valve thickening and stenosis
• Endocardial fibrosis, principally in the R ventricle (bronchial
carcinoids affect the left side)
 Retroperitoneal and pelvic fibrosis
 Collagenous pleural and intimal aortic plaques

Gastrointestinal Lymphoma
- Dissemination of Hodgkin’s Lymphoma
- Exhibit no evidence of liver, spleen, mediastinal lymph node, or bone marrow
involvement at the time of diagnosis
- Usually occur as sporadic neoplasms
- Classification: T cell and B cell lymphoma
- B cell lymphoma:
o MALT lymphoma
 A sporadic lymphoma which arises from the B cells of Mucosa associated
Lymphoid tissue (MALT)
 Most common in the western hemisphere
 Behave as focal tumors in their early stages and are amenable to surgical
 Relapse may occur exclusively in the GIT
 Genotype changes are different than those observed in nodal lymphomas
[ t(11:18) translocation ]
 The cells are usually CD5 and CD10 negative
 Affects usually the adults with no gender predilection.
 Stomach 66-60%
 Small intestine 25-30%
 Proximal colon 10-15%
 Distal colon 10%
 Helicobacter associated chronic gastritis has been proposed as a
driving force for the development of gastric MALT lymphoma
o Immunoproliferative small intestinal disease (IPSID)
 A.k.a. Mediterranean lymphoma
 An unusual intestinal B cell lymphoma arising in patients with
Mediterranean ancestry having a background of chronic diffuse
mucosal plasmacytosis
 Plasma synthesize and abnormal Ig alpha heavy chain in which the
variable portion has been deleted.
 High incidence of weight loss and malabsorption
 Common in children and young adults, equal M-F ratio
o Burkitt’s Lymphoma (Intestinal T cell lymphoma)
 Associated with celiac disease (malabsorption syndrome)
 Occurs 30-40yo with a 10-20 year history of symptomatic
 Arises most often in the proximal small bowel
 Poor prognosis
- Morphology:
o Most lymphomas of the gut are B cell type, 95%
o T cell lymphomas are high grade lesions
o Both mucosal and submucosal
o Early: Plaque like expansions of the mucosa and submucosa.
o Diffusely infiltrating lesions: Full thickness mural thickening with effacement
of the overlying mucosal folds and focal ulceration
o Others: Polypoid, protruding into the lumen, or form large, fungating,
ulcerated masses
o Infiltration of muscle fibers
o Advanced lesions frequently cause motility problems with secondary
o Large tumors sometime perforate because of lack of stromal support
o Earliest histologic lesions: (+) Atypical lymphoid cells are seen infiltrating the
mucosa with effacement and loss of glands and massive expansion of
lymphoid tissue.
- Clinical features:
o Better prognosis

Tumors of the Hepatobiliary Tree

May generate epigastric fullness or discomfort

Nodular hyperplasia are not neoplasms, 2 kinds:

o Focal nodular hyperplasia

 A spontaneous mass lesion

 Young to middle age adults, more on females

 A well demarcated but poorly encapsulated nodule

 A central grey-white, depressed stellate scar from which fibrous septa

radiate to the periphery

 Fibromuscular hyperplasia

o Nodular regenerative hyperplasia

 Affects the entire liver with roughly spherical nodules in the absence of

 Microscopic: Plump hepatocytes are surrounded by rims of atrophic


 Associated with the development of portal hypertension with its

attendant clinical manifestations.

 Associated conditions:

• Solid organ (Renal) transplantation

• Bone marrow transplant

• Vasculitic conditions
* Common among the 2 kinds: Heterogeneity in hepatic blood supply, arising
from focal obliteration of portal vein radicles with compensatory
augmentation of arterial blood supply.

Benign Neoplasms
Most common: Cavernous Hemangiomas:

o Discrete, red blue, soft nodules usually less than 2 cm in diameter and often
occur beneath the capsule.

o Chief clinical significance: Not mistakened for metastatic tumors

o Blind percutaneous biopsies not be performed on them

Liver cell adenomas: Benign neoplasms developing from hepatocytes.

o Young women, taking PO contraceptives

o Reversible once PO contraceptives is stopped.

o 3 clinical significances:

 If presentations is an intrahepatic mass  mistaken for ominous

hepatocellular CA

 Subscapular adenomas have a tendency to rupture, partially during

pregnancy (Under estrogen stimulation) which causes severe
intraperitoneal damage

 Rarely, they may harbor hepatocellular CA

o Morphology:

 Pale, yellow tan, bile stained nodules found anywhere in the hepatic
substance but often beneath the capsule

 Well demarcated, encapsulation may not be present.

 Microscopic:

• (+) Sheets and cords of cells that may resemble normal

hepatocytes or have some variation in cell nuclear size

• Abundant glycogen may generate a clear cytoplasm

• (--) Portal tracts

• (+) Very prominent arterial vessels and draining veins are
distributed through the substance in the tumor.
Malignant Tumors
Hepatocellular CA (From hepatocytes)

Bile duct origin: Cholangiocarcinoma

Two rare forms of primary liver malignancy:

o Hepatoblastoma

 The most common liver tumor of young childhood, fatal within a few

 2 anatomic variants:

• Epithelial type – Small, polygonal fetal cells forming acini,

tubules, or papillary structures vaguely recapitulating liver

• Mixed epithelial and mesenchymal – Primitive mesenchyme,

osteoid, cartilage, or striated muscle

 Frequent activation of Wnt/beta-catenin signaling pathway  stabilizes

Beta catenin  Contributes to process of carcinogenesis

o Angiosarcoma – Occurring elsewehere.

 Due to exposure to: Vinyl chloride, arsenic, or Thorotrast

 Metastasizes widely, and generally kill within a year

Hepatocellular CA’s
5.4% of CA’s WW

Asia 76%, followed by Africa

Predominance of males

85% occur in countries with HBV infection which begins in infancy

o Cirrhosis may be absent ½ of these patients, often occurring at 20-40yo.

An increase in the prevalence of viral induced cirrhosis especially from Hep C virus
is the likely explanation.

Pathogenesis: HBV, HCV, chronic alcoholism, food contaminants (Aflatoxin),

tyrosinemia (40%) despite adequate diet control, hereditary hemochromatosis.

 Point mutations in selected cellular genes

 Loss of heterozygosity in tumor suppressor genes

 DNA methylation changes

 Constitutive expression of hepatocyte growth factor (HGF) and

Transforming growth factor (TGF)

o X protein: A transcriptional activator of many genes and is present in most

tumors with integrated HBV DNA, disrupts normal growth by activation of
host-cell proto-oncogens and disruption of cell cycle control

o Aflatoxins from Aspergillus flavus  mutation of p53 (Tumor suppressor

Morphology: Hepatocellular CA

o Grossly:

 3 kinds:

• Unifocal – Large mass

• Mutlifocal – Widely distributed nodules of variable size

• Diffuse infiltrative – Entire liver

 All 3 cause liver enlargement, and a strong propensity for invasion of

vascular channels.

 Fibrolamellar CA:

• Young, male and female 20-40yo

• No association with HBV pr cirrhosis risk factors

• Better prognosis

• A single large schirrous tumor with fibrous bands coursing

through it.

o Spreads extensively within the liver by obvious contiguous growth and

development of satellite nodules.

o Mets outside the liver is through venous.

Clinical Features

o Ill defined upper abdominal pain

o Malaise

o Fatigue

o Weight loss

o Awareness of an abdominal mass

o Jaundice

o Fever

o GI / Esophageal variceal bleeding

o Elevated alpha feto protein

o Death is due to

 Cachexia

 GI / Esophageal varcieal bleeding

 Liver failure with hepatic coma

 Rupture of the tumor with fatal hemorrhage

A malignancy of the biliary tree arising from bile ducts within and outside the liver.

Risk factors for development: Primary sclerosing cholangitis, congenital

fibropolycystic disease of the biliary system (Caroli’s disease and choledochal
cyst), previous exposure to Thorotrast, Orient: Opisthorchis sinensis

o Occur in the non cirrhotic liver and may track along intrahepatic portal

o Lymphatic and vascular invasion is prominent.  Intrahepatic mets.

o Most are sclerosing adenocarcinomas

o Rarely bile stained because differentiated bile duct epithelium does not
synthesize bile.

o Mixed variants (Infrequent)

 Types

• Separate tumor masses of HCC and cholangiosarcoma within the

same liver.

• Collision tumors – HCC and cholangiocarcinoma co-mingle at an

identifiable interface

• HCC and cholangicarcinoma mixed at the microscopic level.

 Hematogenic mets: Lungs, adrenals, brain, elsewehere.

Clinical features

o Not detected until late in its course

o 1 or 2 year survival rate,

o Median time from diagnosis to death: 6 months

Metastatic Tumors
More common than primary neoplasia

Multiple nodular mets are found that often cause striking hepatomegaly and may
replace over 8-% of existent hepatic parenchyma.

The liver weight can exceed several kilograms with a tendency to outgrow their
blood supply producing central necrosis and umbilication when viewed from the
surface of the liver.

TUMORS of the Biliary Tract

Epithelium lining the biliary tree


o Tubular

o Papillary

o Tubulopapillary

Inflammatory polyps: Sessile mucosal projections with a surface stroma infiltrated

with chronic inflammatory cells and lipid laden macrophages

Adenomyosis of the gallbladder: Hyperplasia of the muscularis, containing

intramural hyperplastic glands
Carcinoma of the gallbladder
Women, 7th decade

o 2 patterns of growth

 Infiltrating

• More common

• A poorly defined area of diffuse thickening and induration of the


• Schirrous

• Common sites of involvement: Fundus and neck, lateral walls


 Exophytic

o Most carcinomas are Adenocarcinomas

o By the time of discovery most have invaded the liver centrifugally

Clinical features:

o Insidious

o Indistinguishable from those associated with cholelithiasis: Abdominal pain,

jaundice, anorexia, N/V
CA of the extrahepatic bile ducts
Uncommon tumors

Extremely insidious

Painless, progressive deepening jaundice.

Risk is increased in patients with primary sclerosing cholangitis, ulcerative colitis,

cystic liver disease (esp Caroli disease and choldochal cysts, fluke infections with
Clonorchis sinensis

Periampullary carcinomas - arising in the immediate vicinity of the ampulla of

Vater; treated by surgical resection.


o Small lesions

o Firm, grey nodules within the bile duct wall, some diffusely infiltrative, others
papillary, polypoid lesions

o Mostly adenocarcinomas, may or not be mucin secreting.

o Squamous features are present uncommonly.

o Klatskin tumors: Tumors arising from the part of the common bile duct
between the cystic duct junction and the confluence of the Right and Left
hepatic duct at the liver hilus.

 Slow growing, sclerosing, infrequent occurrence of distal mets.

Clinical features

o Jaundice (Due to obstruction)

o Stool decolorization, nausea/vomiting, weight loss.

o Palpable gallbladder.

o Increased serum alk phosp. And amino transferase and bile stained urine.

o The majority of ductal cancers are not surgically resectable at the time of
diagnosis, despite their small size.

o Mean survival: 6-18 months.

Tumor stage Histologic features of the neoplasm

Tis CA in situ (High grade dysplasia or
intramucosal carcinoma (Lamina
propria invasion)
T1 Tumor invades the submucosa
T2 Extending into the muscularis propria
but not penetrating through it
T3 Penetrating through the musculariis
propria into the subserosa
T4 Tumor penetrates through serosa and
may directly invade other organs or
Nx Regional LN cannot be assessed
N0 None
N1 Mets in 1-3 LN
N2 Mets in 4 or more LN
Mx Distant mets not assessed
M0 No distant mets
M1 Distant mets

TNM Classification of Carcinoma of the Colon and Rectum