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Hilar and distal cholangiocarcinoma

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Segmental obstruction can cause fulminant cholangitis

A hilar or distal malignant stricture may become infected spontaneously or after instrumentation, producing rigors, hypotension, confusion and kidney injury despite incomplete classical features.

Action: Begin sepsis resuscitation and an appropriate intravenous biliary antimicrobial, collect blood and bile cultures, and obtain urgent source-control drainage directed by the known duct anatomy and specialist expertise.

Synopsis

Distinguish perihilar from distal bile-duct cancer, sequence staging and tissue acquisition, plan segment-specific drainage, and connect resectability or molecular findings to specialist treatment.

  • Perihilar cholangiocarcinoma lies between second-order ducts and the cystic-duct insertion; distal cholangiocarcinoma lies below the cystic-duct insertion and is treated surgically like a pancreatic-head region cancer.
  • Obtain multiphasic CT of chest, abdomen and pelvis for all suspected cholangiocarcinoma; add contrast MRI/MRCP to map proximal duct and satellite liver disease in perihilar presentations.
  • For operable distal malignant obstruction, BSG recommends EUS and ERCP to seek histology; if jaundice is absent, perform EUS first and avoid ERCP-related complications unless drainage is needed.

Key red flags

Do not undertake endotherapy for a suspected perihilar cholangiocarcinoma before discussion with the regional HPB centre unless emergency source control makes delay unsafe.

Contrast injected into a hilar segment that is not subsequently drained can precipitate cholangitis; define which liver sectors require decompression before ERCP or PTC.

A negative brushing does not exclude cholangiocarcinoma, and a resectable distal or perihilar lesion should not undergo an unplanned percutaneous tumour biopsy.

Drainage-related infection

Fever and rigors after contrast or stent placement can represent an infected undrained segment and requires urgent re-imaging and source control.

Investigation priorities

01
Multiphasic CT chest, abdomen and pelvisFirst step

Stage the primary lesion, vascular involvement, nodes, liver, peritoneum and distant organs before endoscopic intervention.

Management branches

Resectable perihilar diseasePlan the future liver remnant

CT and MRCP show a hilar tumour extending into the right duct, with a potentially preservable left liver and no metastasis.

  1. Review ductal, portal and arterial anatomy in the HPB MDT before elective ERCP or percutaneous access. Avoid transperitoneal needle biopsy of a potentially operable hilar primary; if EUS sampling is needed, distinguish suspicious nodes from the primary and agree the target with the resection/transplant team.
  2. Estimate future left-liver volume and function and decide whether portal-vein embolisation or selective drainage is needed before surgery.
Distal obstructing lesionCouple tissue with necessary drainage

A stable jaundiced adult has a distal common-bile-duct stricture on CT and appears operable.

Key medicines

Durvalumab with gemcitabine and cisplatin for advanced biliary tract cancerFor first-line, locally advanced unresectable or metastatic biliary tract cancer, an eligible adult over 36 kg receives durvalumab 1,500 mg IV over 60 minutes on day 1, BEFORE chemotherapy. Give gemcitabine 1,000 mg/m² IV over 30 minutes and cisplatin 25 mg/m² IV on days 1 and 8, repeating every 21 days for up to eight cycles; continue durvalumab 1,500 mg IV every 28 days until progression or unacceptable toxicity. At 36 kg or less, durvalumab is 20 mg/kg on the same three-week combination/four-week maintenance intervals until weight exceeds 36 kg. Durvalumab’s label licenses the combination; the cited Hospira gemcitabine/cisplatin labels do not individually list BTC. The following delivery is the SWAG local BTC protocol, including off-label short cisplatin infusion, not the general cisplatin SmPC schedule: gemcitabine in 250–500 mL sodium chloride 0.9%; cisplatin in 500 mL sodium chloride 0.9% over 60 minutes. For a patient tolerating this fluid plan with normal potassium/magnesium, prehydrate with 1 litre sodium chloride 0.9% plus magnesium sulfate 2 g and potassium chloride 20 mmol over one hour, verify urine output above 100 mL/hour before cisplatin, then give 500 mL sodium chloride 0.9% over 30 minutes afterward. If output is inadequate, withhold cisplatin and assess volume status/renal function. If potassium or magnesium is low, SWAG instead uses a 1-litre post-hydration bag containing magnesium sulfate 2 g and potassium chloride 20 mmol over two hours. Reassess for overload and plan adequate oral fluid afterward; the standard SWAG oral target is at least 2 litres over 24 hours for someone able to tolerate it.Specialist oncology/pharmacy supervision is essential. Check FBC, creatinine/CrCl, liver tests, magnesium, calcium, glucose, thyroid function and baseline cortisol, plus hearing, neuropathy, volume status and performance status. Repeat counts before each treatment day, with day-8 FBC within 24 hours; check renal/liver/electrolyte results and symptoms before dosing. SWAG local full-dose limits are neutrophils >1.0 ×10^9/L, platelets >100 ×10^9/L, bilirubin <1.5×ULN, ALT/AST <3×ULN and CrCl ≥45 mL/min; the gemcitabine label’s general cycle-initiation neutrophil threshold is ≥1.5, so a lower starting count requires explicit specialist protocol authorisation. Outside these limits, hold for prescriber review. If neutrophils are <0.5 or platelets <50, omit both cytotoxics. Otherwise, at neutrophils 0.5–1.0 or platelets 50–100, SWAG uses gemcitabine at 75% and unchanged cisplatin if its other eligibility criteria are met. At CrCl 30–44 omit cisplatin; below 30 omit cisplatin and reconsider gemcitabine dose. Bilirubin >1.5×ULN prompts consultant consideration of gemcitabine at 80%. The cited cisplatin label contraindicates pre-existing renal impairment: a local protocol decision to treat outside that restriction must be documented by the responsible oncologist and pharmacist as an off-label departure, not inferred automatically from CrCl ≥45. Stop cisplatin for grade ≥2 hearing or neurological toxicity. Review nephrotoxic drugs, platinum allergy, dehydration and marrow suppression; avoid live vaccines. SWAG advises replacing warfarin through the anticoagulation team; if it continues pending that review, check INR closely. Before durvalumab, systemic immunosuppression is not recommended apart from physiological corticosteroid doses (≤10 mg/day prednisone-equivalent); obtain oncology review rather than abruptly stopping necessary treatment. Steroids used to treat an immune reaction are a separate indication. Durvalumab needs no adjustment for mild/moderate renal impairment; severe renal data are limited. Its label recommends no hepatic adjustment but severe hepatic experience remains limited. New breathlessness, diarrhoea, marked fatigue, headache or glucose disturbance needs urgent acute-oncology assessment for immune toxicity. Do not reduce its dose for immune toxicity: for grade 2 immune pneumonitis, withhold durvalumab, investigate competing causes and start systemic corticosteroid treatment at prednisone-equivalent 1–2 mg/kg/day under the acute oncology team; after improvement to grade ≤1, taper over at least a month. Grade 3–4 pneumonitis requires permanent discontinuation and urgent specialist treatment. In a jaundiced patient with a biliary stent, investigate cholangitis and obstruction urgently before attributing the change to immune hepatitis or giving reflex steroids. Pregnancy requires urgent specialist assessment: this combination is not a routine pregnancy treatment. Discuss fertility preservation. Effective contraception continues through treatment and the applicable component intervals: women at least 3 months after durvalumab, 6 months after gemcitabine and 29 weeks (at least 7 months) after the cited cisplatin; men 3 months after gemcitabine and 17 weeks (at least 4 months) after cisplatin. Calculate these from each drug’s last dose. No breastfeeding during cytotoxic treatment or for four weeks after cisplatin; continuing durvalumab requires a separate breastfeeding-versus-treatment decision.
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Sources and review status9 sources · checked 8 Sept 2026 · clinical review pending
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 8 Sept 2026; clinical approval remains outstanding.

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom