Cholangiocarcinoma / Mirizzi / Choledochal cyst Flashcards

Question

Princinple of pCCA Resection

Answer 1

- Aim is tumor-free margin of >5 mm - If the tumor is deemed unresectable, local excision of the bile duct should be completed at this time (if feasible) with creation of a Roux-en-Y hepaticojejunostomy for palliation. - However, if the tumor is deemed resectable, the surgeon should proceed with hilar resection in the context of hepatectomy. - The choice of hepatectomy (right vs. left, standard vs. extended) is informed by preoperative staging but ultimately dictated by intraoperative findings. (Cameron 2024) - Caudate resection is typically performed for all patients with hilar disease. - Segment I, which drains into the ductal bifurcation, where the cancer lies, has to be removed in any curative procedure. - Liver transplantation is a potentially curative option for perihilar CCA * Staging laparoscopy should be performed prior to resection as 10% has undetected peritoneal metastasis * Definitive surgery- partial hepatectomy with en-bloc resection of extrahepatic BD, portal lymphadenectomy and biliary reconstruction with Roux-en-Y hepaticojejunostomy * Caudate lobe resected in all left sided and most of right sided tumor because of its anatomy of biliary drainage * Frozen section of distal BD usually performed to exclude distal spread and aid in R0 resection * En-bloc resection of portal vein and hepatic artery is required to obtain negative margin * Limited portal vein resection with reconstruction can be performed without jeopardizing oncological outcome * **Involvement of contralateral hepatic artery is considered non resectable worldwide** - **Bismuth** classification: - For types I and II: en bloc resection of the extrahepatic bile ducts and gall bladder, regional lymphadenectomy and Roux-en-Y hepaticojejunostomy. - For type III: as above plus right or left hepatectomy. - For type IV: not usually resectable but extended right or left hepatectomy may be feasible, dependent on biliary anatomy. Cameron 2024 - The bile duct margin should be assessed by frozen section if there is an option to excise additional tissue in an attempt to achieve negative margins. - There are reports of extrahepatic biliary tree resection without hepatectomy (or with limited liver resection), although this is for select cases of hilar cholangiocarcinoma without vascular involvement and tumor extension into the secondary biliary radicals. - When such an attempt at limited resection is performed, both the right and left hepatic duct margins should be assessed by frozen section to ensure complete resection of the patient's tumor before leaving the operating room as data suggest an increased risk of incomplete resection (higher R1 resection rate) with this approach.

Answer 2

Role of adjuvant therapy is not well defined but generally should be offered to margin or nodal positive patients * Adjuvant chemoradiation with fluorouracil chemotherapy in this group demonstrate better survival outcomes

Answer 3

* Historically not consider part of the treatment modality in pCCA * Advancement of neoadjuvant and careful selection of the patients leads to new evidence to support LT * Neoadjuvant chemoradiotherapy followed by LT associated with recurrence rate of 20% * 5 years recurrence free survival is acceptable at 70% * Patient selection criteria are stringent and 25-30% develop disease progression while waiting for LT * Availability of liver donor making LT less ideal treatment for pCCA

Answer 4

Americas HPB association criteria for non resectable pCCA 1. Bilateral segmental duct extension 2. Unilateral atrophy with either contralateral segmental ductal or vascular inflow involvement 3. Unilateral segmental ductal extension with contralateral vascular inflow involvement 4. Encasement or occlusion of main portal vein proximal to its bifurcation 5. Significant comorbid conditions, chronic liver disease and/or portal hypertension generally precludes resection. * 50% are deemed unresectable at diagnosis and those resectable patients, only 80% achieve R0 resection

Answer 5

* Since 2010, dCCA has been classified as distinct entity separate from the traditional extrahepatic CCA * **Majority exhibit papillary growth with well-defined stalk- less likely to invade adjacent structure, good prognosis** * AJCC TNM is the only classification available but it lacks correlation between tumor and its outcome * **Jaundice is the commonest presentation** but sometimes it exhibits **intermittent in nature** * This is **especially in papillary tumor breaking off from the main tumor and causing BD obstruction** * Signs are hepatomegaly and palpable gallbladder as well as constitutional symptoms * LFT usually abnormal with predominantly direct hyperbilirubinemia and high ALP (obstructive picture)

Answer 6

- Proximal and middle 3rd: Resection + Hepaticojejunostomy - **Distal 1/3rd: Pacreatico-duodenectomy (Whipple’s procedure)** * Surgical management of dCCA similar to patients with ampullary and periampullary tumor * Pancreatoduodenectomy offer the best chance of curative in resectable disease * Goal of surgery should be R0 resection and assessment of BD margin can be done with frozen section * Borderline resectable tumor can be subjected to neoadjuvant therapy * PV and SMV resection and reconstruction can be performed for minimal or short segment involvement * **Adjuvant therapy has no added benefits in margin negative and nodal negative disease** * **It can be considered in case of R1 resection or positive lymph nodes on pathological examination** **Unresectable** - Unresectable or metastatic disease can be treated with palliative systemic chemotherapy * Gemcitabine and cisplatin combination is the commonly used combination

Answer 7

**Endoscopic biliary stent** - Life expectancy of >3 months, metal stent is preferred - Initial stent insertion for biliary obstruction should be plastic or covered SEMS, particularly if diagnosis and resectability are undecided. - If the initial plastic stent becomes blocked, replacement with a metal stent is favored if the estimated survival is expected to be >4 months. - Covered stents not recommended for routine use based on current evidence. - Surgical bypass should be reconsidered in patients with a good estimated life expectancy where stenting has failed. ( Hepaticojejunostomy + JJ +- GJ) **Percutaneous biliary drainage** - if ERCP not available or unable to drain **Photodynamic therapy** - not recommended for routine use based on recent data. **Chemotherapy** - Chemotherapy for PS 0-1 (Cisplatin/gemcitabine (together median survival 1yr than gemcitabine alone 8mths) - oxaliplatin may be substituted for cisplatin where there is a concern about renal function) - phase III UK ABC-02 study, - Monotherapy (Gemcitabine) for PS 2 patients - No established second-line chemotherapy regimen; patients should be encouraged to participate in clinical trials. **Radiotherapy -** may be considered in patients with localized disease, after first-line chemotherapy **Radio-embolization** - may be considered in patients with inoperable iCCA, usually after first-line chemotherapy.

Answer 8

- 5-YS for R0-resected tumors - intrahepatic - 63%, - perihilar - 30%, - distal - 27%, - Median survival for palliated patients < 12 months

Answer 9

After potentially curative treatment – - **3-monthly first 2 years** after therapy including clinical examination, laboratory investigation (including LFTs and LDH), tumor markers (CEA or CA19-9 if one/both were known to be elevated before surgery) and CT-TAP. - **Extended to 6-monthly thereafter** and prolonged to **yearly** visits **after 5 years** of follow-up.

Answer 10

**Definition:** Obstructive jaundice due to CHD obstruction caused by extrinsic compression from an impacted stone in CD or Hartmann’s pouch of gallbladder. **Clinical features** - Painless obstructive jaundice (27.8–100%) - Charcot triad (jaundice, fever and RHC pain) - Reynaud pentad (confusion, hypotension) - Constitutional symptoms - fever, nausea, vomiting, diarrhea, and constipation - Rarely gallstone ileus. **Blood Investigation** - FBC, RP, LFT, PT/PTT, Septic workup - Elevated liver enzymes (AST/ALT) **USG** - Diagnostic accuracy 29%, sensitivity between 8.3% and 27%. Dilatation of biliary system above gallbladder neck, stone impacted in gallbladder neck, abrupt change in normal width of CBD below stone. **CT scan** - Not significantly add to USG. Can assess for malignancy – enlarge porta hepatis LN, liver metastasis. **PTC percutaneous transhepatic cholangiography** - for diagnosis and relief of obstructive symptoms preoperatively, especially when endoscopic treatment fails. **MRCP** - 50% diagnostic accuracy, as good as cholangiography but non therapeutic and is not efficient at localizing a cholecysto-choledochol fistula. **ERCP** - gold standard diagnostic tool, sensitivity of 76.2%. Confirm diagnosis, assess presence of fistula, rule out ampullary pathology - malignancy. Therapeutic - Stenting or nasal bile drainage (NBD) can be achieved. **Classification** Csendes (modified original type II to include a type III - IV) & Beltran (included type V) modification of McSherry's (type I & II only) Classification

Answer 11

Definition: A congenital malformation involving both local dilatation of intrahepatic, extrahepatic bile duct, common bile duct, and pancreaticobiliary maljunction (PBM). - 60% diagnosed during 1st decade of life; 20% diagnosed in adults - ↑ risk of cholangio Cancer (20-fold increase). - Cancer risk increased – Age, Cyst Type I & IV (20%), Type II & III (5%), Type V (15%) Clinical presentation: - 60% diagnosed in childhood. Incidental findings on U/S or ERCP - Triad of jaundice, RUQ pain and abdominal mass (15%); seen mainly in children. [DOI:10.5923/j.surgery.20130204.02] - Adult present with abdominal pain, jaundice, fever & complications - Frequency of acute pancreatitis occurring is 10.5%–56% for adults and 23% for children. Investigation: **Liver Function Test -** often normal unless obstructed which then shows, ↑ LFT - ↑bilirubin, ↑AP, ↑γ-GT, ↑AST, ALT **US** - recommended in children, **CT Scan** - recommended in adults **MRCP -** depicts overall biliary system including enhanced image of intrahepatic/ extrahepatic bile ducts as well as depiction of PBM, **ERCP -** useful in diagnosis of dilatation of extrahepatic bile duct and PBM or PTC **Follow Up** - 3mthly 1st yr then 6mthly 2nd year then, yearly - MRCP or CT abdomen with contrast, LFT **Prognosis** - Late complications of cholangitis and anastomotic stricture – 25-35% - Intrahepatic and bile duct stones – more common in type IV cysts - After excision, slight decrease in Ca risk (6.1%). - Cyst remnants lead to malignant transformation of the cyst wall. A surgical procedure without cyst excision does not diminish malignant potential

Answer 12

Common channel theory is that there is a common pancreatic and biliary duct, and then only controlled by common channel sphincter, with increased reflux of pancreatic juice into bile duct resulting in cystic dilatation and increased ductal pressure, and abnormal sphincter of Oddi function.

Cholangiocarcinoma / Mirizzi / Choledochal cyst Flashcards

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