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BY Prof Dr. Ahmed Al_Gebaly Case 1

 54 Male Admitted with distended abdomen and vomiting.

Case 2

 Patient Data

 Male 47 years complaining Difficulty in swallowing liquids.

Case 3  C/O: Recurrent chest infection Case 4

 Axial contrast enhanced CT image of child with jaundice. Case 5

 73 year old male with Jaundice

Case 1

 54 Male Admitted with distended abdomen and vomiting.

 Grossly distended stomach, containing a large volume of debris. Thickening and enhancement of the anturm/pylorus of the stomach, suggesting of a tumour  Stenosing tumour, which caused the gastric outlet obstruction. Biopsy - proven adenocarcinoma.  Gastric outlet obstruction is a syndrome resulting from mechanical obstruction of stomach emptying.  an be due to malignant or benign causes.  Malignant  adenocarcinoma (second most common)  GIST  lymphoma (less commonly than other malignancies as it is a "soft" tumour)  metastases  Benign  duodenal or gastric peptic ulcers (most common)  pancreatic pseudocysts  gastric varices  granulomatous disease, e.g. Crohn disease, sarcoidosis, tuberculosis  gallstones (Bouveret's syndrome): rare  strictures, e.g. from caustic substance ingestion

 Gastric adenocarcinoma, commonly referred to as gastric cancer, refers to a primary malignancy arising from the gastric epithelium. It is the most common gastric malignancy (over 95% of malignant tumours of the stomach).

 Gastric cancer is rare before the age of 40

 It often produces no specific symptoms such as dyspepsia. Patients may present with anorexia and weight loss (95%) as well as abdominal pain that is vague and insidious in nature.

 Nausea and vomiting, may occur (with bulky tumours that obstruct the gastrointestinal lumen or infiltrative lesions that impair stomach distension); late signs.  CT is currently the staging modality of choice because it can help identify the primary tumour, assess for the local spread, and detect nodal involvement and distant metastases .  Demonstration of lesions facilitated by negative contrast agents (water or gas):  a polypoid mass with or without ulceration  focal wall thickening with mucosal irregularity or focal infiltration of the wall  ulceration: gas-filled ulcer crater within the mass  infiltrating carcinoma: wall thickening and loss of normal rugal fold pattern 4  Calcifications are rare but when present, they are usually mucinous adenocarcinoma.  There are several nodal metastases with eponymous names associated with gastric cancer has been described:  sister Mary Joseph’s node  Virchow’s node  Krukenberg’s node

 Sister Mary Joseph (born Julia Dempsey, 1856-1939) was the surgical assistant to William Mayo in the early days of the Mayo ClinicA Sister Mary Joseph nodule is a metastatic lesion involving the umbilicus. The most common primary source is an intra-abdominal adenocarcinoma (include the stomach, ovary, colon and pancreas).

 solid subcutaneous or dermal nodule or focal umbilical thickening, generally in the context of widespread malignancy.

 For a lesion involving the umbilicus consider  paraumbilical hernia  surgical scar (e.g. laparoscopy)  endometriosis  granuloma  primary umbilical tumour

 Metastasis to the umbilicus, known as a Sister Mary Joseph nodule, in a patient with metastatic ovarian cancer

 T of the abdomen and pelvis obtained with oral and intravenous contrast demonstrates a focal thickening of the sigmoid colon with stenosis of the lumen but no evidence of an established obstruction. No clear fat plane between the mass and the rectus muscle is identified although there is no convincing evidence of direct extension.

 A number of enlarged local lymph nodes are seen in the adjacent mesentery. No convincingly enlarged retroperitoneal nodes are noted, however an umbilical nodule is present.

 The liver contains a number of low attenuation ill-defined lesion, the largest in segment 5.

 Simple left renal cyst noted. Lungs and mediastinum appear unremarkable (although lung windows should of course be reviewed for small deposits).

 This case unfortunately demonstrates advanced colorectal carcinoma (pathologically proven well differentiated adenocarcinoma) in a young patient. It should act as a reminder that although typical age-range is a great way of limiting a differential diagnosis, most pathologies are sometimes encountered well outside the expected age groups and as such age alone is not enough to discount a diagnosis.

 Double-contrast barium enema demonstrates a focal narrowing in the sigmoid colon. It has rolled, heaped up shoulders and results in a severe stenosis. Features are typical of the so-called apple-core stenosis. The remainder of the imaged colon appears normal in calibre, and lined by normal mucosa.

Troisier sign

 Troisier sign is the clinical finding of a hard and enlarged left supraclavicular node (Virchow node), and is considered a sign of metastatic abdominal malignancy.

 It is sometimes referred to as Virchow node, which is the name given by Dr Rudolf Virchow (1821-1902), a German pathologist,

 The left supraclavicular lymph node drains via the thoracic duct, the abdomen, and thorax. It is the junction where incoming lymph is introduced back in the venous circulation through the left subclavian vein. Hence, any malignancy arising in these territories can be responsible for Troisier sign.

 Krukenberg tumour, also known as carcinoma mucocellulare, refers to the "signet ring" subtype of metastatic tumour to the ovary. The colon and stomach are the most common primary tumours to result in ovarian metastases, followed by the breast, lung, and contralateral ovary.  The tumours represent 5-10% of all ovarian tumours and up to 50% of all metastatic tumours to the ovary.  Krukenberg tumours are metastatic tumours to the ovary that contain well defined histological characteristics - mucin-secreting “signet ring” cells and usually originate in the gastrointestinal tract 1  They can originate from :  stomach cancer (signet-ring cells): most common  colorectal carcinoma: second most common  breast cancer  lung cancer  contralateral ovarian carcinoma  pancreatic carcinoma  cholangiocarcionoma/gallbladder carcinoma  Most imaging features are non-specific, consisting of predominantly solid components or a mixture of cystic and solid areas. It is often difficult to differentiate from other ovarian neoplasms 4-5. There are a variety of metastatic carcinomas to the ovary that can mimic primary ovarian tumour  These tumour are typically seen sonographically as bilateral, solid ovarian masses, with clear well defined margins.  CT appearances can be indistinguishable from primary ovarian carcinoma 2. Features will favour towards a Krukenberg tumour if a concurrent gastric or colic mural lesion is seen.  MR: The great majority of Krukenberg tumours are signet-ring cell carcinomas arising in the stomach. Signet- ring cells scatter in the ovarian stroma with abundant collagen formation or marked oedema. Therefore, Krukenberg tumours can occasionally show low or high signal intensity on T2-weighted images. internal hyperintensity (mucin) on T1 and T2 weighted MR images. Strong contrast enhancement is usually seen in the solid component or the wall of the intratumoural cys  Follow-up CT in known peritoneal carcinosis from resected colon cancer.

 Solid mass of the left ovary.  Coronal T2

 Axial T1  Axial T2  Both ovaries are replaced by lobulated, well defined, heterogeneously high T2, enhancing lesions. Moderate volume ascites see in the pelvis.  pathologically proven Krukenberg tumour of gastric origin.  Axial T1, contrast fat sat

 Sag T2

 Irregular stenosis with rigidity of the greater curvature of the stomach at prepyloric gastric antrum  66 years-old man who presented with general decline in health & epigastric heaviness  an intra-luminal hypodense and heteregeneous mass. . Also, there is a triangular hypodensity involving the upper pole of the spleen. (splenic artery thrombosis secondary to this infiltration)

Case 2

 Patient Data

 Male 47 years complaining Difficulty in swallowing liquids.

 Mediastinal widening. Trace amount of gastric air bubble seen. No air-fluid level in the mediastinal widening can be appreciated.  Dysphagia and recurrent chest infection.  Male, 40 years  Chest x-ray is largely unremarkabl e. Minor patchy opacities in the left base are noted. A small gastric air bubble is visible.  Upper GI study reveals uniform dilatation of the esophagus to the level of the gastroesophageal junction, where fixed narrowing is noted (bird peak sign or rat-tail sign)

Case 3  C/O: Recurrent chest infection  Note oesophageal dilatation  CT scan of the abdomen showed uniform dilatation of esophagus with air-fluid level. Pulmonary infiltrates in the upper segment of the right lower lobe likely due to aspiration  Achalasia (primary achalasia) refers to a failure of organised oesophageal peristalsis with an impaired relaxation of the lower oesophageal sphincter (LOS), resulting in often marked dilatation of the oesophagus and food stasis. Obstruction of the distal oesophagus (often due to tumour) has been termed "secondary achalasia" or "pseudoachalasia".

 Primary achalasia is most frequently seen in middle and late adulthood (30-70 years of age) with no gender predilection 6. In most cases achalasia is idiopathic; however, an identical appearance is seen in patients with Chagas disease. Authors differ as to whether they reserve the term achalasia for idiopathic cases, or whether they include Chagas disease.  Typically patients present with dysphagia (which is for both solids and liquids, in comparison to dysphagia for solids only in cases of esophageal carcinoma 7), chest pain/discomfort and eventual regurgitation. Initially, symptoms are intermittent. Patients may also present with a complication of long-standing achalasia:

 Peristalsis in the distal smooth muscle segment of the oesophagus may be lost due to an abnormality of Auerbach plexus (responsible for smooth muscle relaxation), resulting in weak contractions that are uncoordinated and therefore non-propulsive. The abnormality may also occur in the vagus nerve or the dorsal motor nucleus of the vagus nerve.

 The LOS eventually fails to relax, either partially or completely, with elevated pressures demonstrated manometrically 4. Early in the course of achalasia, this LOS tone may be normal or changes may be subtle.

 Radiographic features

 Achalasia characteristically involves a short segment (less than 3.5 cm) of the distal oesophagus

 Chest x-ray findings include:

 right convex opacity behind right cardiac border; occasionally left convex opacity if thoracic aorta tortuous

 air-fluid level due to stasis in thoracic oesophagus filled with retained secretions and food

 small/absent gastric air bubble

 anterior displacement and bowing of trachea on the lateral view

 A barium swallow is able to not only confirm that the oesophagus is dilated but is also able to assess for mucosal abnormalities. Findings include:  bird beak sign  oesophageal dilatation  incomplete LOS relaxation that is not coordinated with oesophageal contraction  pooling or stasis of barium in the oesophagus when the oesophagus has become atonic or non-contractile ( a late feature in the disease)  uncoordinated, non-propulsive, tertiary contractions (see case 1)  failure of normal peristalsis to clear the oesophagus of barium when the patient is in the recumbent position, with no primary waves identified  when barium column is high enough (patient standing) the hydrostatic pressure can overcome the LOS pressure allowing passage of oesophageal content  A hot or carbonated drink during the exam may help visualize sphincter relaxation and barium emptying.

 CT

 Patients with uncomplicated achalasia demonstrate a dilated thin walled oesophagus filled with fluid/food debris.

 Overall, CT has little role in directly assessing patients with achalasia, but is useful in assessing common complications. Careful assessment of the wall of the oesophagus should be undertaken to identify any focal regions of thickening which may indicate malignancy. The lungs should be inspected for evidence of aspiration.

 A number of entities may mimic achalasia, forming the so called 'achalasia pattern'.  achalasia: distal segment of narrowing is <3.5 cm  central and peripheral neuropathy  scleroderma: gastro-oesophageal junction (GOJ) will be open; less severe dilatation  oesophageal malignancy or gastric carcinoma: commonly referred as pseudoachalasia  oesophageal stricture  Chagas disease  anti-Hu antibodies from lung cancer  Other oesophageal disorders should also be considered:  non-specific oesophageal motility disorder  diffuse oesophageal spasm  presbyoesophagus

 The bird-beak sign is used to refer to the tapering of the inferior oesophagus in achalasia. The same appearance (although it is difficult to see the similarity) is also referred to as the rat-tail sign.  30 YO female, with Dysphagia more to fluids than solids.  The appearances although classically occurring in primary achalasia, can also occur in pseudoachalasia due to a gastric tumour involving the gastro-oseophageal junction.  The rat-tail sign is used to refer to the tapering of the inferior oesophagus in achalasia.

Case 4

 Axial contrast enhanced CT image of the #abdomen shows markedly dilated #bile ducts with a central hyperdense dot typical of #Caroli disease. The central dot represent the portal radicle surrounded by the dilated bile duct. Choledochal cyst

 A choledochal cyst is a congenital dilatation of the extrahepatic bile duct

 These patients do not have a ductal plate abnormality.

 The most common theory for the development of a choledochal cyst is that the dilatation is due to an underlying anomalous pancreaticobiliary ductal union (APBDU) .

 In all reported series, most of the patients with types I and IV choledochal cysts have been female with a ratio of four females to one male, but the number of males to number of females in types II, III and V has been noted to be equal.  One of the strongly suggested etiologies is reflux of pancreatic juice into the common bile duct and anomalous pancreaticobiliary ductal union (APBDU) triggers the reflux of pancreatic juice

 The mixing of pancreatic juice and bile as well as stasis results in cystic duct ectasia.  there are two distinct groups of patients.

 The first group is younger than six months of age and the choledochal cyst usually is found in the first month of age. These infants present with a clinical picture of complete bile duct blockage and jaundice.

 Numerous infants have been noted to have a choledochal cyst in utero on prenatal ultrasound, but following birth it appears that jaundice takes one to three weeks to become evident.

 The so-called adult form of choledochal cyst presents (usually older than two years). the most common form of presentation has been abdominal pain and jaundice, (the cause of abdominal pain in these patients is related to stretching of the dilated duct or to cholangitis Todani Classification

 Type I is a true choledichal cyst with focal dilatation of the extrahepatic duct. This is the most frequent type (90-95% of the cases). The right and left bile ducts and the ducts within the liver are usually normal in size in these.

 Type IV is also a true choledichal cyst with dilatation of the entire extrahepatic duct with involvement of portions of the intrahepatic ducts.

 Type II (about 2% of cases) and consists of a true diverticulum arising from the common duct.

 Type III (about 1-5% of cases) is a choledochocele, where there is dilatation of the distal part of the bile duct. ( supra-duodenal or may be within the duodenum or pancreas )

 Type V is Caroli disease.

 Type I cyst should be differentiated from type IVA cysts which involve both intra- and extra-hepatic ducts.

 Type I subtypes:

 type 1A - cystic dilatation of the common duct

 type 1B - focal segmental common duct dilatation

 type 1C - fusiform dilatation.  US is the primary imaging tool for evaluation, however, this modality has its limitations. Evaluating the entire biliary tree is difficult due to limited field of view and poor overview when compared with cross- sectional imaging modalities.

 MDCT can obtain volumetric data sets and multiplanar reformations (MPR) are readily available.

 Furthermore, MRCP is the preferred imaging modality for the correct diagnosis of biliary anomalies. MRCP is non-invasive and can be obtained without the use of contrast agents. 2D or 3D MRCP images clearly delineate biliary anomalies.

 Therefore, MDCT might be the primary imaging modality for huge cystic mass in the abdomen and MRCP can be a problem-solving tool.  Type 1, fusiform dilation (arrows) of common bile duct.

 Percutaneous transhepatic cholangiogram of same patient shows large type 1 bile duct cyst of extrahepatic bile duct containing contrast material (arrows). Slightly distended left and right hepatic ducts are noted (open arrows).  Type 1 Sonogram shows aneurysmal dilation of extrahepatic bile duct (arrows).

 Coronal oblique, thin-slab MR cholangiopancreatography shows marked dilation of extrahepatic bile duct (solid arrows) and left main intrahepatic duct (open arrow).

 Giant Choledochal Cyst Mimicking Massive Gallbladder Hydrops . Type 1 giant choledochal cyst. (b) CT scan, axial view of portal venous phase demonstrates dilated intrahepatic ducts  Type 1: There is fusiform dilatation of the common hepatic and common bile ducts. Dilatation is up to the junction with main pancreatic duct .  Type 2, diverticulum (arrows) originating from common bile duct.

 Type II cysts are supraduodenal diverticula  Type II  choledochocele : Multiplanar reconstruction (a) and axial image (b) shows a cystic lesion within the duodenum (arrow) which communicates with the common bile duct (CBD). Notice also the dilatation of the CBD (arrowhead)  Type 3, choledochocele (arrows).

 ERCP image shows contrast-filled and oval-shaped structure (arrows) of distal common bile duct (CBD) protruding into duodenum (D), which represents choledochocele Type 4A Type 4B

 Type 4A, fusiform dilation of extrahepatic bile duct (arrows) and cystic dilation of intrahepatic biliary ducts (open arrows).

 Type 4B, multiple cystic dilations involving only extrahepatic bile duct (arrows). (multiple extrahepatic cysts, which are much less common).  Multiple intrahepatic cysts and a large extrahepatic cyst related with biliary tree compatible with choledochal cysts ( type IVa ).

 Type 4A : multiple intrahepatic cysts and an extrahepatic cyst, (which accounts for 19% of cases.  Type 5, cystic dilation (arrows) of the intrahepatic bile ducts only (Caroli's disease).

 Coronal oblique, thin-slab MR cholangiopancreatography shows multiple cystic dilations of intrahepatic bile ducts (arrows) consistent with Caroli's disease.  Multiple hypodense cystic areas are seen in right lobe of liver, which appear to be of similar attenuation to few linear dilated intrahepatic biliary radicals. Some of the lesions show a central enhancing focus ("central dot sign"), suggesting Caroli's disease.

 Splenomegaly (portal hypertension ) seen

Caroli Disease

 Caroli disease is an autosomal recessive disease secondary to the ductal plate malformation.

 fibropolycystic diseases involve multiple organs. It is associated with polycystic kidney disease, medullary sponge kidney and medullary cystic disease.

 The hallmark of Caroli disease is intrahepatic duct dilatation. The dilatation can be very large and saccular or it can be very linear

 The duct dilatation in Caroli disease is due to a congenital malformation of the ductal plate, which is the precursor of the intrahepatic bile ducts

 Embryologically each bile duct begins as a single layer of cells that surrounds a portal vein. This layer then duplicates.

 So in the normal situation each portal vein is surrounded by interconnecting bile ducts  if the patient has ductal plate malformation, the bile ducts are too numerous and they are ectatic. The portal vein radicle is usually seen within the dilated duct

 If the large ducts are involved, we see this as Caroli disease. However if only the very small ducts are involved, the result is congenital hepatic fibrosis.

 If all ducts are involved, then there is a combination of fibrosis and Caroli disease, which is also known as the Caroli syndrome. Caroli’s syndrome belongs to a family of polycystic diseases, involving other organs; renal involvement is seen in 60% of cases. (renal tubular ectasia, adult recessive polycystic disease, and rarely adult dominant polycystic kidney disease.

Imaging

 Transabdominal imaging may reveal intrahepatic cystic anechoic areas in which fibrovascular bundles, stones, and linear bridging may be seen

 Doppler sonogram may be useful by demonstrating flow within fibrovascular bundles composed of the portal vein radicles (central dot sign),

 ERCP remains the gold standard for the evaluation of the biliary tree. ERCP may show communication between sacculi and bile ducts and diverticulum-like sacculi of the intrahepatic biliary tree CT

 Computed tomography may be helpful. Although nonspecific, it may show saccular and fusiform dilatations of intrahepatic bile ducts.  Fibrovascular bundles are seen as enhancing central dots (dot sign), septa, and nodules.  Renal cysts may also be seen.  A cirrhotic liver with signs of marked portal hypertension, which include ascites, splenomegaly, esophageal and splenic varices, and patent paraumbilical vein may be seen  Sonogram of duct with saccular dilatation shows echogenic central dot (arrow)  Central dot sign: a dot within the dilated ducts.

 The central dot corresponds to the portal vein (contain blood flow )that is surrounded by dilated bile ducts. (no color flow) .  ERCP shows multiple communicating sacculi of intrahepatic biliary tree.  Saccular bile duct dilatation in Caroli’s disease  Congenital hepatic fibrosis associated with Caroli’s disease. Contrast-enhanced computed tomography scans show fusiform dilatation of intrahepatic bile ducts (arrows).  Central dot sign in Caroli disease  Choledochal Cyst (Todani Type V)

 Contrast enhanced T1W acquisition demonstrates "central dot sign". “Central dot sign” represents the portal radicles surrounded by dilated cystic biliary space.

Choledocholithiasis

 At least 90 % of bile duct stones are stones that have passed from the gallbladder, so-called secondary stones.  Primary stones are those that arise in the bile duct and these are pigment stones  US should be performed first, as it is simple, and if positive carries a high predictive value  If the likelihood of stones is very high on clinical grounds, especially if the patient has cholangitis, early ERCP should be performed for potential sphincterotomy and stone extraction CT Vs MRCP

 Due to the measurement of attenuation, CT scans can depict the composition and nature of biliary calculi.  CT scans were performed with 5mm slices, thus being unable to identify very small biliary stones (of less than 3mm)  All the calculi never enhance in CECT.  High calcium stones are the easiest to identify in non-enhanced CT scans; they appear as hyper-attenuating CBD filling defects.  a cholesterol dominated stone will have a similar density as the bile.

 MRCP demonstrates calculi, with no contrast used, as the same filling defects, hypointense to the surrounding bile.  The effectiveness of MRCP is the greatest especially in very small calculi compared to CT

 MRCP - a very specific investigation for biliary pathology – precisely depicts the location of the obstruction and thereafter the real distance to the papilla. Also, MRCP precisely shows the calculi number, their size (starting from 1mm); anyway MRCP cannot depict the composition of the stones

 MRCP is a flow MRI sequence and it does not require intravenous contrast; it can fully assess biliary obstruction, but cannot demonstrate its precise nature.

 biliary calculi are shown within the dilated distal common bile duct  Unenhanced CT: hyper attenuation calculi Terminal CBD;

 US should be performed first, as it is simple, and if positive carries a high predictive value.

 MRCP  MRCP Multiple stones with dilation  MR studies revealed multiple calculi in the gallbladder as well as a large calculus in distal common bile duct.

 The double duct sign and meniscus sign were well shown.

Case 5

 73 year old male with Jaundice  73 year old male with Jaundice  Large mass-forming intrahepatic cholangiocarcinoma. (a) CT scan in the portal venous phase shows a large heterogeneous hypoattenuating mass in the liver. (b) Capsular retraction (arrow) is seen along the surface of the mass at a lower level  Large mass-forming intrahepatic cholangiocarcinoma. (a) CT scan in the portal venous phase shows a large heterogeneous hypoattenuating mass in the liver. (b) Capsular retraction (arrow) is seen along the surface of the mass at a lower level Cholangiocarcinoma  In Asian countries the incidence is ten times greater due to more chronic biliary infection

 High risk groups are patients with:

 Autoimmune diseases  PSC, ulcerative colitis, primary biliary cirrhosis

 Congenital anatomic anomalies  Caroli, choledochal cyst, anomalous pancreaticobiliary junction  Abnormal tumor suppressor genes, FAP, NF1

 Infection  Biliary parasites, recurrent pyogenic cholangitis  Cholangiocarcinoma usually presents as a mass of 5-20cm. In 65% there are satellite nodules and in some cases punctate calcifications are seen.

 The diagnosis of a cholangiocarcinoma is often difficult to make for a radiologist and even a pathologist.

 That is because cholangiocarcinoma has a varied morphology and histology. It can be a constricting or an expanding lesion, because it can have a fibrous or a glandular stroma.

 It can be located anywhere in the intrahepatic bile ducts or common bile duct. Cholangiocarcinoma

The most common primary neoplasm of the bile ducts

 Basic patterns of chlangiocarcinoma  Intrahepatic cholangiocarcinoma  Intraductal Cholangiocarcinoma  Klatskin Tumor - Hilar Cholangiocarcinoma  periductal infiltrating  Klatskin Tumor - Hilar Cholangiocarcinoma  at or near the confluence of the right and left hepatic ducts  have an aggressive biologic behavior  Imaging features:  Duct dilatation  Ill-defined mass  Lobar atrophy  Vascular invasion  On CT these tumors can be very difficult to visualize  cholangiography and is based on the finding of mass effect (shouldering), irregular margins and abrupt tapering at the obstruction.  ERCP : demonstrates the shouldering at the hilum

 CT: the tumor at the confluence of the left and right hepatic duct.  Bismuth-Corlette type I A type I tumor is a lesion limited of the common hepatic duct, i.e. below the confluence  Bismuth-Corlette type II A type II tumor is a lesion that extends to the confluence. These tumors are potentially resectable  Bismuth-Corlette type IIIa and IIIb A IIIa-tumor extends into the right and a IIIb-tumor extends into the left hepatic duct.  Bismuth-Corlette type IV : extension into the right and left duct. A type IV tumor is unresectable.

 Klatskin tumor with a mass in the liver hilum obstructing the bile ducts Intrahepatic cholangiocarcinoma

 is the second most prevalent intrahepatic primary cancer. (20- 25% of cholangiocarcinomas)

 is an aggressive malignancy with poor prognosis. (rapid growth, tendency to invade adjacent organs and metastasize)

 unlike hepatocellular carcinoma, is not usually related to liver cirrhosis and is sometimes accompanied by severe fibrosis.

 Chemotherapy, radiotherapy, and immunotherapy show little benefits

 On ultrasound imaging, there are no specific features to distinguish them from other intrahepatic tumors.  Intrahepatic cholangiocarcinoma (ICC) is a primary malignant tumor arising peripherally to the secondary bifurcation of the left or right hepatic duct. ICC is a relatively rare liver tumor when compared to hepatocellular carcinoma (HCC). ICC accounts for about 5–30% of primary liver tumors

 Lymph node metastasis (LNM) has been reported to be a significant prognostic factor in ICC

 If the absence of nodal metastasis is accurately detected, there would be no need for an extended irradiation field

 Therefore, identification of clinicopathologic factors that predict the status of lymph nodes in ICC could have potential clinical benefits.

 Unlike HCC, ICC is commonly associated with LNM. (23.8%)

 Patients with elevated CA19-9 levels are more likely to develop recurrent disease and survive for a shorter time than patients with normal CA19-9 level

 if a patient with a small tumor size (less than 5 cm), distinct tumor boundary and low-grade differentiation, the chance for LNM is very low (5%)

 Large mass-forming intrahepatic cholangiocarcinoma with hilar invasion. (a) CT scan in the portal venous phase shows a large markedly hypoattenuating mass which invades the hepatic hilum and obstructs hilar bile duct confluence (arrow). (b) There are multiple rim-enhancing enlarged lymph nodes (arrows) in the hepatoduodenal ligament, in keeping with metastases

 Large mass-forming intrahepatic cholangiocarcinoma. (a–c) CT scans in the arterial (a), portal venous (b), and delayed (c) phases show a large heterogeneous mass in the dome of the right lobe and a small metastatic nodule (arrow in (a)) in the left lobe. The large mass shows minimal peripheral enhancement in the arterial phase and substantial enhancement in the central portion of the mass in the delayed phase (arrows in (c))

 Large mass-forming intrahepatic cholangiocarcinoma. (a) CT scan in the portal venous phase shows a large heterogeneous hypoattenuating mass in the liver. (b) Capsular retraction (arrow) is seen along the surface of the mass at a lower level. (c) T2-weighted MR image shows a mildly hyperintense mass with central hypointense area due to dense fibrosis. (d) The mass is hypointense on T1- weighted image. (e) There is subtle peripheral enhancement of the mass on the arterial-phase contrast-enhanced T1-weighted MR image. (f) The entire mass shows hyperenhancement relative to the liver in the delayed phase (5 min after injection of contrast)

 Intrahepatic cholangiocarcinoma, CT image. The quadrate robe contains a mass. Peripheral enhancement of the tumor and peripheral bile duct dilatation are shown.  Defined as a malignant tumor arising from the intrahepatic bile duct epithelium. Cholangiocarcinoma arising from the right and left hepatic ducts at or near their junction (hilar cholangiocarcinoma) are considered as carcinoma of the extrahepatic bile ducts.  Intrahepatic cholangiocarcinoma is an aggressive malignancy with poor prognosis. The causes of this disease lethality are not only its rapid growth but also its tendency to invade adjacent organs and metastasize.

 Intrahepatic cholangiocarcinoma, unlike hepatocellular carcinoma, is not usually related to liver cirrhosis and is sometimes accompanied by severe fibrosis. This suggests that hepatocellular and cholangiocarcinoma might originate from hepatic precursor cells.  Intrahepatic cholangiocarcinoma is the second most prevalent intrahepatic primary cancer. It occurs in the middle-aged and elderly with no obvious sex differences. Its incidence reveals wide geographic variations

 Cholangiocarcinoma may arise at any portion of the bile duct epithelium, from terminal ductules (canals of Hering) to the ampulla of Vater, as well as the peribiliary glands. Intrahepatic cholangiocarcinoma is subdivided into peripheral or hilar disease (Klatskin tumor), on the basis of its site of origin  Perihilar tumors occur where the right and left hepatic ducts bifurcate, and are the most common type of cholangiocarcinoma. Intrahepatic tumors (located peripheral to the secondary confluence) are the least common. Extrahepatic tumors are located from the upper border of the pancreas to the ampulla. The tumor is located distal to the cystic duct takeoff, leading to augmentation of the gallbladder  Many different multislice CT protocols can be used in the diagnosis of cholangiocarcinoma. We use a combination of oral contrast (750 to 1000 mL) and intravenous contrast (120 to 150 mL, delivered at 3 to 5 mL/sec). Imaging is performed at 120 kVp with a tube current of 200 to 250 mA in the late arterial phase after IV contrast administration (40 sec), the venous phase (60 to 70 sec), and the parenchymal phase (five to 10 min). Collimation is 1.25 to 2.5 mm for arterial imaging, and 2.5 to 5 mm when visualizing venous anatomy.

 Contrast-enhanced CT can detect intrahepatic bile duct tumors, the level of biliary obstruction, and the presence of liver atrophy with good sensitivity. CT may also visualize nodal metastasis.5 A triple-phase spiral CT scan will detect all cholangiocarcinomas that are greater than 1 cm in diameter.6,7

 Dynamic CT can establish resectability in only about 60% of patients. Dynamic CT may still, however, provide more information on resectability than MRI. 8 Both imaging methods are similarly capable of showing tumor enhancement and biliary ductal dilatation, though the relationship of the tumor to vessels and surrounding organs is evaluated more easily using CT.

 Mass-forming cholangiocarcinoma. This kind of macroscopic growth is the most common among intrahepatic cholangiocarcinomas. Masses are generally large, lobulated, and well-defined, but they can also be irregular. The mass is typically hypodense on MSCT with stippled or punctate hyperattenuating foci within the tumor

 Mass-forming cholangiocarcinoma. This kind of macroscopic growth is the most common among intrahepatic cholangiocarcinomas. Masses are generally large, lobulated, and well-defined, but they can also be irregular. The mass is typically hypodense on MSCT with stippled or punctate hyperattenuating foci within the tumor

 Three phase CT of hemangioma of the liver.

 Three phase CT of hepatocellular carcinoma; Note the early enhancement and early washout.  Axial CT of the abdomen with intravenous contrast reveals an enhancing mass near the gallbladder neck (yellow arrow). This is compatible with a Klatskin tumor (hilar cholangiocarcinoma).  Axial CT of the abdomen and pelvis in the same patient at a more superior level reveals significant intrahepatic biliary dilatation (yellow arrows) compatible with bile duct obstruction by the Klatskin tumor seen in the first image above.  This is a Klatskin tumor causing bile duct obstruction and biliary dilatation. A Klatskin tumor is a cholangiocarcinoma that arises at near the confluence of the right and left hepatic ducts and causes obstruction of bile duct outflow, leading to biliary dilatation as seen in the second image above. Other causes of biliary dilatation may include pancreatic carcinoma, choledocholithiasis, chronic pancreatitis, primary sclerosing cholangitis, and others. Read about mimics of cholangiocarcinoma here.

 Cholangiocarcinoma is most common in patients older than 60, has a slight male predominance, and is more common in Asia. The five year survival unfortunately is very poor for extrahepatic cholangiocarcinoma, on the order of 1-2%.

  Klatskin's tumour

 Discussion:

 73 year old male with Jaundice and pruritus.

 Gradually increasing enhancement through the portovenous and delayed phase (approx. 15 mins post contrast) are features of a cholangiocarcinoma.

 Similar pattern of enhancement is seen in a mass at the confluence of the right and left hepatic ducts (Klatskin's Tumour) in this case.

 The cholangiogram shows a filling defect at the right and left hepatic duct confluence. An Internal - external type of a percutaneous drainage is performed in this case (Image4).

 Intrahepatic cholangiocarcinoma can be classified into three types on the basis of gross morphologic features:  mass-forming (the most common),  periductal infiltrating, and  intraductal growth.  Morphological types of cholangiocarcinoma:

 (A) mass-forming;

 (B) intraductal -growing;

 (C) periductal-infiltrating

 Cholangiocarcinoma can develop in any part of the extrahepatic duct,  involving the upper third of the duct including the hilum in 50-75% of the reported cases,  the middle third in 10-25% cases,  and the lower duct in 10-25% cases  Axial T2-weighted MR image shows a dilated peripheral intrahepatic duct with a slightly hyperintense lesion around the duct (arrow).

 Contrast-enhanced equilibrium phase MR image shows periductal enhancement around the dilated intrahepatic duct (arrowheads).

 Periductal infiltrating cholangiocarcinoma is characterized by growth along a dilated or narrowed bile duct without mass formation and manifests as an elongated, spiculated, or branch-like abnormality  periductal-infiltrating hilar cholangiocarcinoma.

 (A) Contrast-enhanced coronal CT image showing enhancing tumor (white single arrow) in common hepatic duct.

 (D) coronal T2W image show

 concentric thickening of common hepatic duct (white single arrow).  Intraductal cholangiocarcinoma.

 Localized or diffuse ductectasia with or without an echo-genic intraductal polypoid lesion

 (a) CT scan shows a soft-tissue component filling a mildly dilated duct (arrow). (b) MR cholangioancreatogram shows the mildly dilated duct with irregularities that mimic impacted stones (arrow-heads)  Intraductal papillary cholangiocarcinoma. (a) MR cholangiopancreatogram shows an intraductal polypoid mass with localized ductal dilatation (arrow)

 Intraductal-growing hilar cholangiocarcinoma  an intraductal polypoid mass with localized ductal dilatation Intrahepatic cholangiocarcinoma

 The typical CT features of a mass-forming cholangiocarcinoma include :  homogeneous attenuation,  irregular peripheral enhancement  with gradual centripetal enhancement,  capsular retraction, the presence of satellite nodules, and  vascular encasement without the formation of a grossly visible tumor thrombus  (a) Arterial & portal venous phase CT phases : centripetal enhancement of the tumor (black arrow). A satellite nodule is also seen (white arrow).

 (c) Three-minute delayed phase CT scan shows gradual centripetal enhancement with tumor encasement of the posterior branch of the right portal vein (arrowhead). without formation of a grossly visible tumor thrombus.  Intrahepatic cholangiocarcinoma

 Peripheral cholangiocarcinoma appears as either a well-defined or an irregular mass along the course of dilated intrahepatic ducts  Mass-forming cholangiocarcinoma

 a hyperintense mass, with marked intrahepatic biliary dilatation in the mass and the adjacent liver.  Contrast material–enhanced computed tomographic (CT) scan shows bile duct dilatation with a poorly enhancing lesion in the adjacent liver (arrows). There is significant parenchymal atrophy with capsular retraction peripheral to the more centrally located cholangiocarcinoma  Intrahepatic cholangiocarcinoma:  arise in the very small peripheral ducts  have abundant fibrous stroma that can cause retraction of the liver capsule.  The tumor typically enhances in the equilibrium and delayed phases (5-10 minutes).

 Intrapancreatic cholangiocarcinoma  Marked dilation of the intrahepatic biliary tract  the obstructing periampullary neoplasm (arrow )  Intrahepatic cholangiocarcinoma

 Capsule retraction  Cholangiocarcinoma: large hilar hypoechoic mass with right and left intrahepatic bile ducts dilatation.  The key findings to look for are:  Delayed enhancement  Peripheral biliary dilatation (one segment or lobe)  Capsular contraction

 Notice the capsular retraction (blue arrow) and the late enhancement (yellow arrow).  The finding of an infiltrating mass with capsular retraction and delayed persistent enhancement is very typical for a cholangiocarcinoma.

 the fibrous tissue will contract and cause retraction of the liver capsule (a pseudo-cirrhosis appearance)

 Some cholangiocarcinomas have a glandular stroma.