I- INTRODUCTION :
Cancers of VBEH, still called extrahepatic cholangiocarcinoma include the & rsquo; all malignant tumors that develop on & rsquo; biliary tree from the liver hilum up & rsquo; their drainage into the duodenum.
These tumors are rare. They represent less than 1 % all digestive cancers.
It s & rsquo; is in 95 % cases of & rsquo; adenocarcinoma. Early diagnosis is exceptional because of the clinical latency of these tumors.
Everything must be done to diagnosis can be worn early to consider surgical excision, only chance to cure these cancers.
II- EPIDEMIOLOGY :
A- Frequency :
Less of 1 % of all cancers of the digestive tract of the & rsquo; adult
B- Age- sex :
Occurs primarily in the & rsquo; adult beyond 50 years, usually between 60-70ans, with a slight male predominance If cancer occurs before < 40 years must be sought sclerosing cholangitis and / or chronic colitis associated.
C- Risk factors and precancerous lesion :
Two circumstances predisposing to stress
1- La rectocolite hémorragique (RCH) : risk of occurring the CBD cancer is multiplied by 9 at 21 compared to the general population. The RCH may be isolated or associated with sclerosing cholangitis.
2- Some malformations of bile ducts :
a- congenital cystic dilatation of the CBD:15 at 28 % of these malformations are complicated cancers. In the & rsquo; lack of preventive surgical resection.
b- The anomalies of the biliopancreatic junction, that & rsquo; it / or not a cystic dilation associated bile.
c- cystic disease or liver as fibrocystic disease Caroli
III- ANATOMOPATHOLOGIQUE :
A- Macroscopie :
These tumors are in a form or nodular sclerosing, rarely burgeoning.
B- histology :
These tumors are generally well-differentiated adenocarcinoma.
They are mostly invasive and associated with significant fibrosis.
C- Extension :
Is most often locally, source of recurrences.
L & rsquo; invasion can be done step by step along the bile duct walls, the other elements of the hepatic pedicle (portal vein, hepatic artery) are gradually invaded.
Finally, the occurrence of lymph node and liver metastases can s & rsquo; watch
D- Classifications :
1- anatomical :
The largest. It guides the surgical indications.
- tumor 1/3 higher "or hilar Tm Klatskin" :
- the most common 56 % and s & rsquo; extends from the channels intrahepatic up & rsquo; the confluence cysticohépatique.
- . tumor 1/ 3 way :
- , 20 % and s & rsquo; extends from the confluence cysticohépatique the upper edge of the pancreas.
- tumor 1/3 inferior : (3), 15 % and s & rsquo; extends from the upper edge of the pancreas in the ampulla of Vater.
- The diffuse forms (4), are not uncommon 5 at 10 %
Classification of cancers of the CBD
1. upper third ; 2. middle third ; 3. lower third ; 4. Diffuse
The tumors 1/3 sup can be classified into :
Type 1 : reaching convergence without the & rsquo; clog
Type 2 : reaching and obstructing the convergence
Type3 : Achieving the right or left side convergence
Type 4 : Achieving the right and left secondary convergence
Classification of cancers 1/3 sup VBP
2- TNM classification (UICC 2002) :
Tis : intra epithelial T : Tm limited to the VBP T2 : Tm exceeding the wall of the VBP T3 : Achievement of adjacent organs (foie, VB, pancreas, a right or left branch of the portal vein or the & rsquo; hepatic artery)
T4 : Reaching the door vein trunk, l & rsquo; artery5common hepatic, duodenum, stomach, le colon, wall
Nx : not EVALUE NO : no & rsquo; nodal NI : regional nodes involved (cystic, peri-biliary, hilar) N2 : Remote nodes involved M:
MO : no metastasis Ml : distant metastases or lymph nodes plus left clavicular
IV- POSITIVE DIAGNOSIS :
A- clinical manifestations :
1- The history : seeks ie primary telltale sign is :
a- l & rsquo; progressive obstructive jaundice, evolving d & rsquo; one piece, unremittingly, to become intense with dark urine and pale stools, sans fièvre douleurs ni
b- pruritus, may precede the & rsquo; jaundice,
c- l & rsquo; impaired general condition is more or less pronounced. L & rsquo; all of these signs is characteristic of & rsquo; neoplastic obstruction in the bile duct.
During the & rsquo; changes may occur : pain of & rsquo; right upper quadrant or cholangitis with febrile seizures and chills.
2- Clinical examination :
a- Jaundice mucocutaneous frank
c- A big bladder can be palpated as d & rsquo; a sub liver mass, piriforme, elastic.
The presence of & rsquo; a big bladder advocates d & rsquo; a downstream obstacle of & rsquo; stoma cystic duct.
His absence is in favor of & rsquo; an upstream obstacle cystic confluence.
d- A big liver choiéstase can be found.
B- Additional tests :
1- Laboratory tests :
a- the choiéstase is important: elevated alkaline phosphatase, GGT, and bilirubin,
b- decrease in prothrombin (TP) ; correcting by binding injections of vitamin K.
c- Liver pain is late: transaminase elevation.
L & rsquo; hypo albumin reflects the & rsquo; malnourished, d- tumor markers (ACE, THAT 19-9, AFP) usually remain negative but can be high especially CA 19-9.
However, the latter loses its specificity in the presence of & rsquo; jaundice.
2- radiological :
Nature specify, exact and & rsquo seat; extension of the tumor
a- Ultrasound :
- C & rsquo; is the first line examination.
- indirect signs
+ shows the bile duct dilatation (VBIH ± VBEH) • Research any big vesicle
+ defines the level of & rsquo; obstruction
- direct sign : by identifying the tumor
- allows searching liver metastases, ganglion, ascites, carcinose,
b- Scanner :
- confirms the data of & rsquo; Ultrasound o MRI surpassed by the Bill and & rsquo; echo-endoscopy)
- better visualize the & rsquo; liver parenchymal damage o information on & rsquo; & rsquo existence; liver atrophy
c- Echo-endoscopy and & rsquo; Doppler ultrasound : improved detection of tumors of the bile ducts and the search for the & rsquo; vascular invasion.
L & rsquo; echoendoscopy allows a fine needle aspiration.
d- They-IRM :
C & rsquo; is the best morphological examination in case of & rsquo; jaundice.
It is non-invasive and requires no & rsquo; contrast agent injection and quality reconstruction images of & rsquo; biliary tree is excellent
e- L & rsquo; opacification of bile ducts :
- These are invasive procedures that are required when MRI is not available or insufficient o Allows the diagnosis of & rsquo; hurdle,
- Specifies the exact seat and & rsquo; tumor extension along the & rsquo; biliary tree, o Allows biliary drainage (rarely useful) Two methods can be used:
Endoscopic retrograde cholangiopancreatography (CPRE)
- Visualize an image of & rsquo; o stop or stenosis often shows that the distal edge of the lesion, o risky d & rsquo; cholangitis and pancreatitis Cholangiography transmural o shows the upper pole of the tumor
- says its possible extension to hepatic ducts rights and / or left, o risk of bile leakage, bleeding or d & rsquo; infection
V- DIAGNOSTIC DIFFERENTIEL :
A- Whatever the seat to be discussed :
- medical jaundice
- extrinsic compression : adénopathies, Neighborhood cancers
- non-neoplastic obstacle : calculates enclosed in the VBP, tm benign, postoperative stenosis, KHF broken in the CBD, sclerosing cholangitis.
It is difficult to & rsquo; state nature benign or malignant d & rsquo; a localized stenosis. L & rsquo; cytologic analysis of bile collected by endoscopic catheterization can be useful to & rsquo; establishment of definitive diagnosis.
1- According seat :
If the stenosis is low :
- Cancer of the pancreas head but treatment is the same, o Cancer ampullary region
- benign strictures of the bile stolen: postoperative or chronic pancreatitis.
If the stenosis is high :
- Primary sclerosing cholangitis +++, However the occurrence of & rsquo; cholangiocarcinoma on lesions sclerosing cholangitis is frequent and unpredictable.
- Cancer of the gallbladder invading the pedicle and / or hepatic hilum
WE- TREATMENT :
The therapeutic management of cancers of the biliary tract is complex and poorly codified.
It should always be discussed in d & rsquo; CPR. The choice of treatment depends on the seat and the & rsquo; extent of the tumor, s & rsquo; general condition of the patient and the technical platform.
A- Means :
1- Surgery :
a- Surgery has referred healing : It s & rsquo; d & rsquo acts; surgical resection including the type and the & rsquo; extent depend on the tumor site
cancers 1/3 inferior : DPC is the & rsquo; intervention of choice.
cancers 1/3 way :
Excision off the VBP with lymphadenectomy and cholecystectomy.
Extemporaneous biopsies slices high and low biliary section. If & rsquo; downward extension, the pancreaticoduodenectomy indicated
If & rsquo; upward extension, resection of the biliary convergence (or an associated hepatectomy) is necessary.
The restoration of biliary continuity is achieved in & rsquo; d & rsquo assistance; jejunum mounted Y.
– cancers 1/3 superior : pose the most difficult problems. §0 resection of the bile duct and gall convergence associated with a more or less wide hepatic resection:
- Resection of segments I and IV is systematically for their bile duct drains directly into the biliary confluence,
- Hepatectomy enlarged left segment I for cancers extended to the left liver (III left), o enlarged right hepatectomy to segments I and IV for cancers extended to the right liver.
However, liver volume remaining (left lobe) is often reduced and exposes the patient to potentially fatal risk & rsquo; postoperative liver failure.
So, l & rsquo; action must be preceded by a biliary drainage of the left lobe and d & rsquo; a right portal embolization for its hypertrophy, o The restoration of biliary continuity is achieved in & rsquo; d & rsquo assistance; jejunum Y.
Liver transplantation can be offered to selected patients with unresectable hilar cancer as part of & rsquo; a protocol including radiation therapy and exploratory laparotomy and the & rsquo; absence of any particular node spread extrahepatic,
b- Chirurgie palliative The bilio-digestive derivations
cancers 1/3 inferior : anastomoses cholédoco-digestîves, covering either the duodenum or a jejunum Y.
Cancers of the upper third and the middle third : anastomoses concern dilated intrahepatic bile ducts (channel segment III or, more rarely the V channel or VI).
External transtumoral intubation using a drain T (de transport)
Intubation internal transtumoral lost to drains.
2- Nonsurgical Treatment :
a- stents : In inoperable patients stents are implemented endoscopic or percutaneous radiologic way. Biliary drainage is best achieved retrograde endoscopic to & rsquo; d & rsquo assistance; a metal prosthesis, longer permeable that & rsquo; a plastic prosthesis but more costly and irreversible. For hilar tumors, biliary drainage can s & rsquo; & rsquo accompanied; photodynamic therapy whose effects on the quality of life and survival were recently demonstrated.
b- Chemoradiotherapy is used in the forms locally advanced non-metastatic but n & rsquo; has never really been evaluated.
c- chemotherapy l & rsquo; improved survival with chemotherapy n & rsquo; is not demonstrated.
B- Indications :
1- Sick unresectable and resectable tumors :
only surgical resection. C & rsquo; is the only treatment that allows prolonged survival. It should always be discussed in d & rsquo; CPR
2- unresectable tumor :
Patient operable and having a survival expectancy >6 month : biliodigestive if technically feasible or endoscopic metal stent shunt
Patient inoperable or operable with an expectation of survival < 6 month : The stent endoscopic complementary treatment to be discussed according to the & rsquo; age and the & rsquo; condition (chemotherapy, radio- chemotherapy or supportive treatment)
3- metastatic tumor :
L & rsquo; indication is based on the & rsquo; age and the & rsquo; condition.
- If elderly or impaired general condition : abstention.
- If about young and good condition : chemotherapy
C- Results :
1- Surgery :
Operative mortality depends on the complexity of the surgical procedure performed and varies from 7% at 20 %.
It may even exceed 20% if d & rsquo; hepatectomy associated with DP.C Surgical resection n & rsquo; is possible that in 20% cases.
Morbidity depends on the type of resection performed and exceeds 30% if d & rsquo; hepatectomy and / or CPD.
It is dominated by the biliary fistulas from the bracket & rsquo; hepatectomy and / or the & rsquo; biliary anastomosis and may warrant postoperative drainage Remote Control.
Even if complete resection, the risk of recurrence, including locoregional, is high.
The median disease-free survival is 6 at 12 month.
The overall median survival is o 16 at 24 months in case of & rsquo; o removal 9 months for palliative treatment
2- Chemotherapy :
With gemcitabine alone or 5FU-cisplatin median survivals rates associations are lower 10 month.
La gemcitabine, combined with cisplatin or the & rsquo; oxaliplatin, allows tumor control by more & rsquo; one in two patients with median survival of relatives 12 month.
VII- CONCLUSION :
Cancers of the extrahepatic bile ducts have a poor prognosis.
Complete surgical resection is the only curative option but n & rsquo; is possible only in a minority of patients.
L & rsquo; pre treatment assessment, decision and implementation of the treatment of these cancers are complex and raise d & rsquo; a specialized multidisciplinary care.
Course of Dr A. BENHAMADA – Faculty of Constantine