Rak dróg żółciowych w okolicy wątrobowo-dwunastniczej (cholangiocarcinoma okolicy wątrobowo-dwunastniczej)
Leczenie

Rak dróg żółciowych w okolicy wątrobowo-dwunastniczej (hilar cholangiocarcinoma) to agresywny nowotwór o złym rokowaniu, najczęściej diagnozowany w 6. dekadzie życia. Podstawą leczenia jest resekcja chirurgiczna z uzyskaniem marginesów wolnych od komórek nowotworowych (R0), obejmująca wycięcie zewnątrzwątrobowych dróg żółciowych, częściową hepatektomię oraz limfadenektomię en bloc. W zaawansowanych przypadkach stosuje się rozległe resekcje, takie jak trisekcjonektomia czy hepatopankreatoduodenektomia, często poprzedzone procedurami usprawniającymi drenaż żółciowy i zwiększającymi przyszłą pozostałość wątroby (FLR), np. embolizacją żyły wrotnej (PVE) lub metodą ALPPS. U pacjentów nieresekcyjnych rozważany jest przeszczep wątroby, poprzedzony chemioterapią i radioterapią, co może poprawić wyniki leczenia. Standardowa chemioterapia opiera się na pochodnych platyny i gemcytabinie, a w nowo zdiagnozowanych przypadkach rozważa się dodanie immunoterapii (durvalumab, pembrolizumab). Radioterapia (EBRT, brachyterapia, SBRT, protonoterapia) oraz terapia fotodynamiczna (PDT) pełnią rolę uzupełniającą i paliatywną, poprawiając kontrolę lokalną i jakość życia.

Rak dróg żółciowych w okolicy wątrobowo-dwunastniczej (cholangiocarcinoma okolicy wątrobowo-dwunastniczej) – Leczenie

Rak dróg żółciowych w okolicy wątrobowo-dwunastniczej (hilar cholangiocarcinoma) stanowi rzadki, ale wysoce agresywny nowotwór, który charakteryzuje się niekorzystnym rokowaniem. Nowotwór ten typowo pojawia się w 6. dekadzie życia i wymaga kompleksowego, wielodyscyplinarnego podejścia terapeutycznego. Strategie leczenia obejmują chirurgię, radioterapię, chemioterapię i terapię fotodynamiczną, a ich dobór zależy od zaawansowania choroby, stanu ogólnego pacjenta oraz możliwości przeprowadzenia zabiegu resekcyjnego.12

Leczenie chirurgiczne

Resekcja chirurgiczna z uzyskaniem marginesów wolnych od komórek nowotworowych (resekcja R0) pozostaje jedyną metodą leczenia dającą szansę na długoterminowe przeżycie. Standardowa terapia polega na resekcji z ujemnym marginesem chirurgicznym (R0), obejmującej wycięcie zewnątrzwątrobowych dróg żółciowych, częściową hepatektomię oraz en bloc limfadenektomię.12 Rozległe resekcje guzów rozwidlenia przewodów wątrobowych mogą obejmować przyległą wątrobę, w formie lobektomii lub usunięcia części segmentów 4 i 5 wątroby.3

Duża hepatektomia w połączeniu z resekcją dróg żółciowych zewnątrzwątrobowych zwiększyła odsetek resekcji R0, a także długoterminowe przeżycie i powinna być uznawana za standardowe postępowanie terapeutyczne.4 W przypadkach zaawansowanych guzów wnęki wątroby, konieczne może być zastosowanie rozszerzonej resekcji, obejmującej trisekcjonektomię wątroby dla guzów typu IV wg klasyfikacji Bismutha, hepatopankreatoduodenektomię dla guzów z rozległym rozprzestrzenianiem podłużnym oraz resekcję naczyń z rekonstrukcją w przypadku guzów naciekających struktury naczyniowe wątroby.5

Przed operacją często konieczne jest wykonanie procedur usprawniających drenaż żółciowy przyszłej pozostałości wątroby, co zmniejsza poziom bilirubiny i ułatwia przyszłą hipertrofię wątroby.6 W złożonych przypadkach można zastosować embolizację żyły wrotnej (PVE) w celu zwiększenia przyszłej pozostałości wątroby (FLR) przed wykonaniem rozległej resekcji.7

W niektórych ośrodkach dla wysoce wyselekcjonowanych pacjentów stosuje się również metodę ALPPS (Associating Liver Partition and Portal Vein Ligation with Staged hepatectomy), choć zaleca się, aby PVE była stosowana jako pierwsza metoda powiększania objętości wątroby, a ALPPS była zarezerwowana dla przypadków niepowodzenia standardowych metod.8

Przeszczep wątroby

W przypadkach, gdy resekcja radykalna nie jest możliwa, przeszczep wątroby wykazał akceptowalne wyniki u wyselekcjonowanych pacjentów.9 Dla wielu chorych przeszczep wątroby może stanowić metodę wyleczenia raka dróg żółciowych wnęki wątroby, choć istnieje ryzyko nawrotu nowotworu po transplantacji.10

Przeszczepienie wątroby może być rozważane w przypadku niektórych nieresekcyjnych nowotworów ograniczonych miejscowo, podczas gdy paliatywna chemioradioterapia (CRT) jest opcją dla tych z rozprzestrzeniającą się chorobą.11 Według zaleceń, przeszczep wątroby powinien być opcją dla nieresekcyjnych, bez przerzutów odległych przypadków raka wnęki wątroby, a resekcja powinna być wykonywana, jeśli można osiągnąć ujemny margines z powodu ograniczonej dostępności narządów.12

Przeszczep wątroby jest szczególnie ważną opcją terapeutyczną u pacjentów z wczesnym stadium nieresekcyjnego wewnątrzwątrobowego lub okołownękowego raka dróg żółciowych. Procedura ta często poprzedzona jest chemioterapią i napromienianiem, aby zniszczyć jak najwięcej komórek rakowych przed zabiegiem chirurgicznym.13

Chemioterapia

Pomimo ograniczonych danych, chemioterapia jest wskazana u pacjentów z guzami nieresekcyjnymi i odpowiednim stanem ogólnym.14 Aktualnie standardowe schematy chemioterapii dla nieresekcyjnego raka wnęki wątroby oparte są na pochodnych platyny w połączeniu z gemcytabiną.15

Chemioterapia może być stosowana przed przeszczepieniem wątroby lub jako opcja dla pacjentów z zaawansowanym rakiem dróg żółciowych, aby spowolnić postęp choroby i złagodzić objawy. Leki chemioterapeutyczne mogą być podawane dożylnie, tak aby trafiały do całego organizmu, lub mogą być podawane w sposób umożliwiający ich bezpośrednie dostarczenie do komórek nowotworowych.16

U pacjentów z zaawansowaną chorobą, schematy fazowe II oceniały chemioradioterapię w celu poprawy lokalnej kontroli i potencjalnego obniżenia stopnia zaawansowania w celu resekcji chirurgicznej.17 W badaniach klinicznych pojawiają się również nowe podejścia, takie jak połączenie chemioterapii z immunoterapią. Gemcytabina z cisplatyną oraz durvalumabem lub pembrolizumabem są obecnie rozważane jako nowe opcje pierwszej linii dla pacjentów z nowo zdiagnozowanym rakiem dróg żółciowych.1819

Radioterapia

Radioterapia wykorzystuje promienie lub wiązki energii o wysokiej mocy ze źródeł takich jak promienie rentgenowskie i protony do niszczenia komórek nowotworowych. Może obejmować maszynę, która kieruje wiązki promieniowania na ciało (radioterapia wiązką zewnętrzną) lub umieszczenie materiału radioaktywnego wewnątrz ciała w pobliżu miejsca nowotworu (brachyterapia).20

Radioterapia wiązką zewnętrzną (EBRT) i brachyterapia są stosowane u pacjentów z nieresekcyjnym rakiem dróg żółciowych i mogą odgrywać rolę w łagodzeniu żółtaczki i bólu. W niektórych ośrodkach stosuje się również nowsze techniki, takie jak stereotaktyczna radioterapia ciała (SBRT) oraz protonoterapia.2122

Radioterapia może być stosowana przed operacją (tzw. terapia neoadjuwantowa) w celu zmniejszenia guza i ułatwienia jego usunięcia. Radioterapia zewnętrzna może być również stosowana po operacji (terapia adjuwantowa), co może zmniejszyć ryzyko nawrotu raka.23

Terapia fotodynamiczna

Terapia fotodynamiczna (PDT) polega na dożylnym podaniu środków światłoczułych, które gromadzą się w szybko rosnących komórkach nowotworowych. Światło laserowe skierowane na nowotwór powoduje reakcję chemiczną w komórkach nowotworowych, zabijając je. Zazwyczaj potrzebnych jest wiele zabiegów. PDT może pomóc złagodzić objawy i może również spowolnić wzrost nowotworu.24

W terapii fotodynamicznej substancja światłoczuła jest wprowadzana do organizmu dożylnie lub miejscowo. Po aktywacji światłem o określonej długości fali, substancja ta wyzwala reakcję fotochemiczną, która niszczy komórki nowotworowe. Niedawno dwa randomizowane badania kliniczne z grupą kontrolną wykazały znaczącą korzyść dla przeżycia pacjentów z nieresekcyjnym rakiem dróg żółciowych leczonych PDT.2526

Terapie celowane i immunoterapia

Leczenie celowane koncentruje się na specyficznych nieprawidłowościach obecnych w komórkach nowotworowych. Blokując te nieprawidłowości, leczenie celowane może powodować śmierć komórek nowotworowych. Lekarz może przeprowadzić badanie komórek nowotworowych, aby sprawdzić, czy terapia celowana może być skuteczna w leczeniu raka dróg żółciowych wnęki wątroby.27

Immunoterapia wykorzystuje układ odpornościowy do walki z rakiem. Organizm może nie atakować nowotworu, ponieważ komórki nowotworowe wytwarzają białka, które pomagają im ukryć się przed komórkami układu odpornościowego. Immunoterapia działa poprzez zakłócanie tego procesu. W przypadku raka dróg żółciowych wnęki wątroby, immunoterapia może być opcją dla zaawansowanego nowotworu, gdy inne metody leczenia nie przyniosły poprawy.28

Dwie nowsze metody leczenia przerzutowego raka wnęki wątroby to:

  • Immunoterapia, jak inhibitory punktów kontrolnych układu immunologicznego, które działają poprzez pomoc układowi odpornościemu w walce z rakiem.
  • Terapia celowana, jak inhibitory receptora czynnika wzrostu fibroblastów (FGFR) lub inhibitory IDH. Inhibitory FGFR blokują wzrost komórek nowotworowych, powodując ich śmierć. Inhibitory IDH blokują nieprawidłowe białko, które wpływa na normalny wzrost komórek.

29

Drenaż żółciowy i terapie paliatywne

Drenaż żółciowy jest procedurą przywracającą przepływ żółci. Może obejmować umieszczenie cienkiej rurki w przewodzie żółciowym w celu drenażu żółci. Inne strategie obejmują operację omijającą w celu przekierowania żółci wokół nowotworu oraz stenty utrzymujące otwarty przewód żółciowy zapadnięty przez nowotwór. Drenaż żółciowy pomaga złagodzić objawy raka dróg żółciowych.30

Stenty mogą być umieszczane za pomocą ERCP (endoskopowej cholangiopankreatografii wstecznej) lub PTC (przezskórnej cholangiografii przezwątrobowej) w celu złagodzenia niedrożności żółciowej. Stentowanie może złagodzić świąd i poprawić jakość życia.31

W przypadku pacjentów z nieresekcyjnym rakiem dróg żółciowych, postępowanie jest ukierunkowane na leczenie paliatywne. Leczenie paliatywne jest terapią wspomagającą, której celem jest zapobieganie i leczenie objawów lub problemów spowodowanych przez nowotwór.32

Terapie lokoregionalne

Ogrzewanie komórek nowotworowych (ablacja prądem o częstotliwości radiowej) wykorzystuje prąd elektryczny do ogrzewania i niszczenia komórek nowotworowych. Korzystając z badania obrazowego jako przewodnika, takiego jak USG, lekarz wprowadza jedną lub więcej cienkich igieł do małych nacięć w jamie brzusznej. Gdy igły docierają do nowotworu, są ogrzewane prądem elektrycznym, niszcząc komórki nowotworowe.33

Inne techniki lokoregionalne obejmują:

  • Radioembolizację, która jest bardzo podobna do chemoembolizacji, ale z wykorzystaniem radioaktywnych mikrosfer.
  • Terapie oparte na tętnicy wątrobowej, które mogą obejmować podawanie chemioterapii bezpośrednio do wątroby.
  • Brachyterapię i ablację prądem o częstotliwości radiowej, które są wykorzystywane jako inne endoskopowe formy leczenia paliatywnego.

3435

Wybór optymalnej strategii leczenia

Ze względu na złożoność tej choroby zaleca się wielodyscyplinarne podejście z leczeniem multimodalnym.3637 Optymalne postępowanie wymaga współpracy specjalistów z różnych dziedzin, w tym chirurgii, onkologii medycznej, radioterapii, diagnostyki obrazowej, radiologii interwencyjnej, gastroenterologii i patomorfologii.38

Decyzja o wyborze metody leczenia powinna być podejmowana indywidualnie dla każdego pacjenta, biorąc pod uwagę:

  • Stopień zaawansowania choroby i możliwość wykonania resekcji
  • Lokalizację guza i jego związek z kluczowymi strukturami anatomicznymi
  • Stan ogólny pacjenta i współistniejące choroby
  • Funkcję wątroby i ryzyko pooperacyjnej niewydolności wątroby
  • Dostępność doświadczonego zespołu chirurgicznego

3940

W przypadku pacjentów z zaawansowaną chorobą, należy rozważyć udział w badaniach klinicznych. Wszyscy pacjenci z nieresekcyjną, przerzutową lub nawrotową chorobą, którzy nie otrzymali wcześniej inhibitora punktów kontrolnych, powinni mieć wykonane badania molekularne w kierunku niedoboru mechanizmów naprawy nieprawidłowo sparowanych zasad (dMMR) lub niestabilności mikrosatelitarnej (MSI-H).41

Leczenie skojarzone

Coraz więcej danych wskazuje na korzyści z leczenia skojarzonego, które może obejmować:

  • Terapię neoadiuwantową przed resekcją chirurgiczną w celu zmniejszenia guza i zwiększenia szansy na resekcję R0
  • Terapię adiuwantową po resekcji chirurgicznej, zazwyczaj z zastosowaniem kapecytabiny przez 24 tygodnie
  • Połączenie chemioterapii z immunoterapią w pierwszej linii leczenia u pacjentów z chorobą nieresekcyjną
  • Zastosowanie terapii celowanych u pacjentów z określonymi zmianami molekularnymi

4243

Najnowsze badania wykazały, że dodanie durvalumabu lub pembrolizumabu do standardowej chemioterapii gemcytabiną i cisplatyną wydłuża przeżycie całkowite u pacjentów z zaawansowanym rakiem dróg żółciowych.4445

W przypadku pacjentów z określonymi zmianami molekularnymi dostępne są również terapie celowane, takie jak inhibitory FGFR2 (pemigatinib, futibatinib) dla pacjentów z fuzjami lub rearanżacjami FGFR2, oraz iwosidenib dla pacjentów z mutacją IDH1.46

Rokowanie i dalsze perspektywy leczenia

Rak dróg żółciowych w okolicy wątrobowo-dwunastniczej jest agresywnym nowotworem o niekorzystnym długoterminowym rokowaniu. Inwazja węzłów chłonnych, stopień zróżnicowania guza i ujemne marginesy są ważnymi wskaźnikami prognostycznymi. Resekcja R0 stanowi jedyną szansę na długoterminowe przeżycie.47

Pomimo postępów w leczeniu, większość przypadków raka dróg żółciowych wnęki wątroby jest nieresekcyjna w momencie rozpoznania, co podkreśla znaczenie wczesnej diagnostyki i skierowania do ośrodków specjalistycznych.48

Trwające badania kliniczne koncentrują się na nowych strategiach leczenia, w tym:

  • Kombinacji immunoterapii z chemioterapią lub terapiami celowanymi
  • Nowych inhibitorach FGFR drugiej generacji o poprawionym profilu bezpieczeństwa
  • Terapiach opartych na przeciwciałach monoklonalnych dla pacjentów z amplifikacjami HER2
  • Nowatorskich metodach zwiększania przyszłej pozostałości wątroby przed rozległą resekcją

49

Rak dróg żółciowych w okolicy wątrobowo-dwunastniczej jest niewątpliwie trudnym i poważnym schorzeniem. Dane dotyczące przeżycia nie mówią jednak całej historii o tej chorobie. Jest ona wysoko podatna na leczenie, a badacze prowadzą badania kliniczne nad nowymi metodami terapii. W przypadku pacjentów z tym schorzeniem, zespół medyczny pomoże w zarządzaniu objawami i skutkami ubocznymi leczenia, a także chętnie zaproponuje badania kliniczne do rozważenia.50

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition
    https://hbsn.amegroups.org/article/view/3374/html
    Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6th decade of life. […] Treatment consists of surgery, radiation, chemotherapy and photodynamic therapy. […] Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile duct resection, hepatectomy and en bloc lymphadenectomy. […] In instances where curative resection is not possible, liver transplantation has demonstrated acceptable outcomes in highly selected patients. […] Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. […] Due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended for this complex disease. […] Margin negative (R0) resection remains the only treatment that offers the chance at long-term survival.
  • #2 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – PubMed
    https://pubmed.ncbi.nlm.nih.gov/24696835/
    Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6(th) decade of life. […] Treatment consists of surgery, radiation, chemotherapy and photodynamic therapy. Biliary drainage of the future liver remnant should be performed to decrease bilirubin levels thereby facilitating future liver hypertrophy. Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile duct resection, hepatectomy and en bloc lymphadenectomy. […] In instances where curative resection is not possible, liver transplantation has demonstrated acceptable outcomes in highly selected patients. Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. […] Due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended for this complex disease.
  • #2 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition
    https://hbsn.amegroups.org/article/view/3374/html
    Major hepatectomy combined with extra hepatic bile duct resection has increased R0 resection rates as well as long term survival and should be considered standard therapy. […] The role of minimally invasive HC resections also remains unclear. […] The current standard chemotherapy regimens for unresectable HC are platinum based in combination with gemcitabine. […] Photodynamic therapy (PDT) involves the intravenous administration of photosensitizing agents which accumulate within cancer cells. […] In conclusion, HC is a rare but aggressive disease with a dismal long-term prognosis. Lymph node invasion, tumor grade and negative margins are important prognostic indicators. R0 resection represents the only chance for long-term survival.
  • #3 Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq
    Localized intrahepatic and extrahepatic bile duct cancer may be completely removed by surgery. […] Extended resections of hepatic duct bifurcation tumors (Klatskin tumors, also known as hilar tumors) to include adjacent liver, either by lobectomy or removal of portions of segments 4 and 5 of the liver, may be performed. […] Most cases of intrahepatic, distal, and perihilar bile duct cancer are unresectable and cannot be completely removed. […] In locally advanced disease, phase II trials have evaluated chemoradiotherapy with the goal of improved local control and potential downstaging for surgical resection. […] For patients with unresectable bile duct cancer, management is directed at palliation. […] Treatment options for bile duct cancer are described in Table 16. […] Complete surgical resection with negative surgical margins offers the only chance of cure for distal bile duct cancers.
  • #4 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition
    https://hbsn.amegroups.org/article/view/3374/html
    Major hepatectomy combined with extra hepatic bile duct resection has increased R0 resection rates as well as long term survival and should be considered standard therapy. […] The role of minimally invasive HC resections also remains unclear. […] The current standard chemotherapy regimens for unresectable HC are platinum based in combination with gemcitabine. […] Photodynamic therapy (PDT) involves the intravenous administration of photosensitizing agents which accumulate within cancer cells. […] In conclusion, HC is a rare but aggressive disease with a dismal long-term prognosis. Lymph node invasion, tumor grade and negative margins are important prognostic indicators. R0 resection represents the only chance for long-term survival.
  • #5 Surgical management of hilar cholangiocarcinoma: Controversies and recommendations
    https://www.ahbps.org/view.html?uid=2537&&vmd=Full
    Surgical management of hilar cholangiocarcinoma: Controversies and recommendations […] Hilar cholangiocarcinomas are highly aggressive malignancies. They are usually at an advanced stage at initial presentation. Surgical resection with negative margins is the standard of management. It provides the only chance of cure. Liver transplantation has increased the number of curative procedures for cases previously considered to be unresectable. […] Meticulous and thorough preoperative planning is required to prevent fatal post-operative complications. Extended resection procedures, including hepatic trisectionectomy for Bismuth type IV tumors, hepatopancreaticoduodenectomy for tumors with extensive longitudinal spread, and combined vascular resection with reconstruction for tumors involving hepatic vascular structures, are challenging procedures with surgical indications expanded.
  • #6 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – PubMed
    https://pubmed.ncbi.nlm.nih.gov/24696835/
    Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6(th) decade of life. […] Treatment consists of surgery, radiation, chemotherapy and photodynamic therapy. Biliary drainage of the future liver remnant should be performed to decrease bilirubin levels thereby facilitating future liver hypertrophy. Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile duct resection, hepatectomy and en bloc lymphadenectomy. […] In instances where curative resection is not possible, liver transplantation has demonstrated acceptable outcomes in highly selected patients. Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. […] Due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended for this complex disease.
  • #7 Surgical management of hilar cholangiocarcinoma: Controversies and recommendations
    https://www.ahbps.org/journal/view.html?doi=10.14701/ahbps.23-028
    Complete R0 surgical resection is the only curative option, as the survival rates of R1 and R2 are low. […] Transplantation is the treatment modality for certain locally unresectable tumors, while palliative chemoradiotherapy (CRT) is the option for those that are widespread. […] Advanced HCs require some form of liver resection, and the ability to perform safe major resections depends upon the necessary future liver remnant (FLR) to maintain the normal function of the liver. […] Various methods are available to augment the FLR, like PVE, Associating Liver Partition and Portal Vein Ligation with Staged hepatectomy (ALPPS), portal vein ligation, Assessment of Liver remnant volume using ICG clearance Intraoperatively during Vascular Exclusion (ALIIVE), and Associated Portal vein Embolization and Artery Ligation (APEAL), the first two are mostly used in HC.
  • #8 Surgical management of hilar cholangiocarcinoma: Controversies and recommendations
    https://www.ahbps.org/journal/view.html?doi=10.14701/ahbps.23-028
    PVE is a good option to improve the FLR. However, there are still many areas of controversies. […] Recommendation: PVE should be used as the initial method for volume augmentation, while ALPPS should be reserved for failed cases. […] R0 resection is the mainstay of treatment of HC, but because of the locally advanced nature of the disease, R0 resections cannot usually be achieved. LT is a good option for such patients who have locally advanced but nonmetastatic disease that is not amenable to resection. […] Recommendation: LT should be an option for unresectable non-metastatic HCs, and resection should be done if a negative margin can be achieved because of organ scarcity. […] HC is an aggressive tumor, and an R0 resection remains the main treatment modality. Major hepatic resection is almost always required to achieve R0 with a risk of post-hepatectomy liver failure, which can be mitigated by improving the FLR by PVE and preoperative biliary drainage of the FLR.
  • #9 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition
    https://hbsn.amegroups.org/article/view/3374/html
    Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6th decade of life. […] Treatment consists of surgery, radiation, chemotherapy and photodynamic therapy. […] Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile duct resection, hepatectomy and en bloc lymphadenectomy. […] In instances where curative resection is not possible, liver transplantation has demonstrated acceptable outcomes in highly selected patients. […] Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. […] Due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended for this complex disease. […] Margin negative (R0) resection remains the only treatment that offers the chance at long-term survival.
  • #10 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413
    Treatments for cholangiocarcinoma (bile duct cancer) may include: […] Surgery. When possible, surgeons try to remove as much of the cancer as they can. For very small bile duct cancers, this involves removing part of the bile duct and joining the cut ends. For more-advanced bile duct cancers, nearby liver tissue, pancreas tissue or lymph nodes may be removed as well. […] Liver transplant. Surgery to remove your liver and replace it with one from a donor (liver transplant) may be an option in certain situations for people with hilar cholangiocarcinoma. For many, a liver transplant can be a cure for hilar cholangiocarcinoma, but there is a risk that the cancer will recur after a liver transplant. […] Chemotherapy. Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be used before a liver transplant. It may also be an option for people with advanced cholangiocarcinoma to help slow the disease and relieve signs and symptoms. Chemotherapy drugs can be infused into a vein so that they travel throughout the body. Or the drugs can be administered in a way so that they are delivered directly to the cancer cells.
  • #11 Surgical management of hilar cholangiocarcinoma: Controversies and recommendations
    https://www.ahbps.org/view.html?uid=2537&&vmd=Full
    Complete R0 surgical resection is the only curative option, as the survival rates of R1 and R2 are low. Traditionally, the aim of surgical resection was the relief of jaundice, but over the last few decades, there has been a paradigm shift from local excision to cone excision of the liver at the hilum, and more extensive major liver resections, along with vascular reconstruction, which increase the survival rate. […] Transplantation is the treatment modality for certain locally unresectable tumors, while palliative chemoradiotherapy (CRT) is the option for those that are widespread. […] Advanced HCs require some form of liver resection, and the ability to perform safe major resections depends upon the necessary future liver remnant (FLR) to maintain the normal function of the liver. […] Various studies have shown that regeneration is stimulated by growth factors released by the liver, as well as from the extrahepatic organs.
  • #12 Surgical management of hilar cholangiocarcinoma: Controversies and recommendations
    https://www.ahbps.org/view.html?uid=2537&&vmd=Full
    Recommendation: Portal vein resection helps to achieve better R0 resections with improved overall survival and acceptable complications, and should be offered to patients with portal vein invasion. […] Recommendation: LT should be an option for unresectable non-metastatic HCs, and resection should be done if a negative margin can be achieved because of organ scarcity. […] HC is an aggressive tumor, and an R0 resection remains the main treatment modality. Major hepatic resection is almost always required to achieve R0 with a risk of post-hepatectomy liver failure, which can be mitigated by improving the FLR by PVE and preoperative biliary drainage of the FLR.
  • #13 Hilar cholangiocarcinoma – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube
    https://www.augustahealth.com/disease/hilar-cholangiocarcinoma/
    Hilar cholangiocarcinoma is a type of bile duct cancer that occurs in the bile ducts that lead out of the liver (hepatic ducts) and join with the gallbladder. Hilar cholangiocarcinomas are also known as Klatskin tumors. […] Which treatment is best for you will depend on the location and extent of your hilar cholangiocarcinoma. Treatment typically involves surgery, liver transplant or supportive treatments to reduce your pain and other symptoms. […] Surgery for hilar cholangiocarcinoma involves removing: The cancer, Surrounding bile ducts, Portions of the liver, Nearby lymph nodes. […] Some people with early-stage hilar cholangiocarcinoma may consider liver transplant. Chemotherapy and radiation are typically used before the liver transplant to kill as many cancer cells as possible before surgery. […] People with advanced hilar cholangiocarcinoma who aren’t able to undergo surgery or liver transplant may receive chemotherapy to slow the growth of the cancer.
  • #14 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition
    https://hbsn.amegroups.org/article/view/3374/html
    Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6th decade of life. […] Treatment consists of surgery, radiation, chemotherapy and photodynamic therapy. […] Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile duct resection, hepatectomy and en bloc lymphadenectomy. […] In instances where curative resection is not possible, liver transplantation has demonstrated acceptable outcomes in highly selected patients. […] Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. […] Due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended for this complex disease. […] Margin negative (R0) resection remains the only treatment that offers the chance at long-term survival.
  • #15 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition
    https://hbsn.amegroups.org/article/view/3374/html
    Major hepatectomy combined with extra hepatic bile duct resection has increased R0 resection rates as well as long term survival and should be considered standard therapy. […] The role of minimally invasive HC resections also remains unclear. […] The current standard chemotherapy regimens for unresectable HC are platinum based in combination with gemcitabine. […] Photodynamic therapy (PDT) involves the intravenous administration of photosensitizing agents which accumulate within cancer cells. […] In conclusion, HC is a rare but aggressive disease with a dismal long-term prognosis. Lymph node invasion, tumor grade and negative margins are important prognostic indicators. R0 resection represents the only chance for long-term survival.
  • #16 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413
    Treatments for cholangiocarcinoma (bile duct cancer) may include: […] Surgery. When possible, surgeons try to remove as much of the cancer as they can. For very small bile duct cancers, this involves removing part of the bile duct and joining the cut ends. For more-advanced bile duct cancers, nearby liver tissue, pancreas tissue or lymph nodes may be removed as well. […] Liver transplant. Surgery to remove your liver and replace it with one from a donor (liver transplant) may be an option in certain situations for people with hilar cholangiocarcinoma. For many, a liver transplant can be a cure for hilar cholangiocarcinoma, but there is a risk that the cancer will recur after a liver transplant. […] Chemotherapy. Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be used before a liver transplant. It may also be an option for people with advanced cholangiocarcinoma to help slow the disease and relieve signs and symptoms. Chemotherapy drugs can be infused into a vein so that they travel throughout the body. Or the drugs can be administered in a way so that they are delivered directly to the cancer cells.
  • #17 Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq
    Localized intrahepatic and extrahepatic bile duct cancer may be completely removed by surgery. […] Extended resections of hepatic duct bifurcation tumors (Klatskin tumors, also known as hilar tumors) to include adjacent liver, either by lobectomy or removal of portions of segments 4 and 5 of the liver, may be performed. […] Most cases of intrahepatic, distal, and perihilar bile duct cancer are unresectable and cannot be completely removed. […] In locally advanced disease, phase II trials have evaluated chemoradiotherapy with the goal of improved local control and potential downstaging for surgical resection. […] For patients with unresectable bile duct cancer, management is directed at palliation. […] Treatment options for bile duct cancer are described in Table 16. […] Complete surgical resection with negative surgical margins offers the only chance of cure for distal bile duct cancers.
  • #18 New Therapies to Improve Outcomes for Patients With Intrahepatic Cholangiocarcinoma
    https://www.targetedonc.com/view/new-therapies-to-improve-outcomes-for-patients-with-intrahepatic-cholangiocarcinoma
    Treatment of intrahepatic cholangiocarcinoma (iCCA) expands each year, with the most recent advance being the addition of immune checkpoint inhibitors to the gemcitabine/cisplatin backbone, according to Rachna T. Shroff, MD, MS, FASCO. […] My presentation focused on 2 big buckets. One was frontline treatments for newly diagnosed, advanced biliary tract cancers, including intrahepatic cholangiocarcinoma. Chemotherapy plus immunotherapy is a new kind of platform for building on newly diagnosed patients therapies, based on TOPAZ-1 [NCT03875235]. […] Now, for our newly diagnosed patients, the standard treatment is to give them gemcitabine/cisplatin with the addition of a checkpoint inhibitor. This is being integrated into the National Comprehensive Cancer Network guidelines and is the new base to try to topple or to try to beat.
  • #19 New Therapies to Improve Outcomes for Patients With Intrahepatic Cholangiocarcinoma
    https://www.targetedonc.com/view/new-therapies-to-improve-outcomes-for-patients-with-intrahepatic-cholangiocarcinoma
    With these new combinations of gemcitabine/cisplatin plus immunotherapy, the median overall survival is still in the range of 12 to 13 months, so we still have a lot of work to do. […] It does seem that all comers with newly diagnosed intrahepatic cholangiocarcinoma should be getting gemcitabine/cisplatin with durvalumab, and possibly pembrolizumab. […] The second bucket that I sort of focused on was targeted therapies because in the space of intrahepatic cholangiocarcinoma, genomic alterations that we see in this disease are molecularly diverse, complex, quite interesting, and potentially targetable. […] The big areas in which we have drugs approved include the IDH1 mutation with the oral IDH1 inhibitor, ivosidenib [Tibsovo], based on the CLARITY data [NCT05174650], as well as oral FGFR inhibitors such as pemigatinib [Pemazyre] and futibatinib [Lytgobi].
  • #20 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413
    Radiation therapy. Radiation therapy uses high-powered energy beams from sources such as X-rays and protons to kill cancer cells. Radiation therapy can involve a machine that directs radiation beams at your body (external beam radiation). Or it can involve placing radioactive material inside your body near the site of your cancer (brachytherapy). […] Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die. Your doctor may test your cancer cells to see if targeted therapy may be effective against your cholangiocarcinoma. […] Immunotherapy. Immunotherapy uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that help them hide from the immune system cells. Immunotherapy works by interfering with that process. For cholangiocarcinoma, immunotherapy might be an option for advanced cancer when other treatments haven’t helped.
  • #21 Comprehensive management of cholangiocarcinoma: Part II. Treatment
    https://www.ijgii.org/journal/view.html?doi=10.18528/gii1500342
    Surgery for intrahepatic cholangiocarcinoma (ICC) is usually not indicated in tumors that invade the vasculature, multiple tumors, bilobar disease, and metastases. […] Surgery is the only way to achieve cure in PCC. […] At this time, liver transplantation (LT) for ICC should be considered within research trials along with neoadjuvant and/or adjuvant therapy. […] The role of neo-adjuvant chemotherapy for CC is not established and has an unclear benefit. […] The NCCN recommends biliary drainage in patients with jaundice if chemotherapy is planned. […] External-beam radiotherapy (EBRT) and brachytherapy are used in patients with unresectable CC and may have a role in relieving jaundice and pain. […] Hepatic artery infusion (HAI) delivers the drug to the tumor after implantation of a pump or port. […] Radiofrequency ablation (RFA) is a minimally invasive modality for inoperable CC. […] PDT may be applied endoscopically or percutaneously in patients with unresectable PCC and DCC, patients with anticipated R1 or R2 resection, or poor surgical candidates.
  • #22 7 Innovative Bile Duct Cancer (Cholangiocarcinoma) Treatment Options | MD Anderson Cancer Center
    https://www.mdanderson.org/cancer-types/bile-duct-cancer/bile-duct-cancer-treatment.html
    We offer clinical trials combining chemotherapy with surgery to remove biliary tumors. Chemotherapy may be offered before surgery to shrink tumors or after surgery to decrease the risk of cancer returning. […] Common radiation approaches for biliary cancer treatment include intensity modulated radiation therapy (IMRT) or stereotactic body radiation therapy (SBRT). These photon-based approaches use image guidance to send high dose radiation directly to the tumor. […] Proton therapy, which utilizes larger radiation particles and has different dose distribution properties compared to photon-based approaches, may also be used to treat biliary cancers. […] Oncologists decide which type of radiation to use on a case-by-case basis that considers each patients anatomy, liver function and tumor location. […] New advances in radiation therapy for biliary cancers include everything from the types of subatomic particles to the machines used to deliver radiation. New forms of radiation are being explored in clinical trials, dose-escalation continues to advance, and equipment continues to improve.
  • #23 Cholangiocarcinoma (Bile Duct Cancer): Staging and Treatment | OncoLink
    https://www.oncolink.org/cancers/gastrointestinal/cholangiocarcinoma/cholangiocarcinoma-bile-duct-cancer-staging-and-treatment
    Radiation treatment is the use of high-energy x-rays to kill cancer cells. It can be given with external beams of radiation, proton beam radiation, or internal radiation called brachytherapy. […] Radiation may be given before surgery (called neoadjuvant therapy) to make the tumor smaller and easier to take out. External radiation may be given after surgery (adjuvant therapy). This can lower the chance of the cancer coming back (recurrence). […] Chemotherapy is the use of medications that go throughout the whole body to kill cancer cells. It can be used alone or in with surgery, radiation, or immunotherapy. […] Chemotherapy alone will not cure this cancer, but it can help you live longer. […] Targeted therapy medications attack certain genes and proteins found on cancer cells. […] Immunotherapy medications help the immune system to find and destroy cancer cells.
  • #24 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413
    Heating cancer cells. Radiofrequency ablation uses electric current to heat and destroy cancer cells. Using an imaging test as a guide, such as ultrasound, the doctor inserts one or more thin needles into small incisions in your abdomen. When the needles reach the cancer, they’re heated with an electric current, destroying the cancer cells. […] Photodynamic therapy. In photodynamic therapy, a light-sensitive chemical is injected into a vein and accumulates in the fast-growing cancer cells. Laser light directed at the cancer causes a chemical reaction in the cancer cells, killing them. You’ll typically need multiple treatments. Photodynamic therapy can help relieve your signs and symptoms, and it may also slow cancer growth. You’ll need to avoid sun exposure after treatments. […] Biliary drainage. Biliary drainage is a procedure to restore the flow of bile. It might involve placing a thin tube into the bile duct in order to drain the bile. Other strategies include bypass surgery to reroute the bile around the cancer and stents to hold open a bile duct being collapsed by cancer. Biliary drainage helps relieve signs and symptoms of cholangiocarcinoma. […] Because cholangiocarcinoma is a very difficult type of cancer to treat, don’t hesitate to ask about your doctor’s experience with treating the condition. If you have any doubts, get a second opinion.
  • #25 The Korean Journal of Internal Medicine
    https://www.kjim.org/m/journal/view.php?number=162444
    The prognosis for hilar cholangiocarcinoma is limited by tumor spread along the biliary tree leading to refractory obstructive cholestasis, cholangitis, and liver failure. […] Photodynamic therapy (PDT) is a local photochemical tumor treatment consisting of a photosensitizing agent combined with laser irradiation of a distinct wavelength. Tumor ablation with PDT combined with biliary stenting reduces cholestasis and significantly improves median survival time. […] Optimum control of tumor spread along the bile ducts and control of cholestasis and cholangitis will prolong survival in one to two thirds of patients, and renders them suitable for other antitumor therapies. […] Recently, two randomized controlled trials have shown a significant survival benefit in patients with unresectable CC treated with PDT.
  • #26 The Korean Journal of Internal Medicine
    https://www.kjim.org/m/journal/view.php?number=162444
    PDT resulted in survival prolongation (group A: n = 20; median, 493 days; group B: n = 19; median, 98 days; p 0.0001). […] PDT also improved biliary drainage and quality of life. […] PDT improves survival, jaundice, quality of life, is well tolerated, and can be repeated without losing efficacy. […] PDT seems to be a promising therapeutic approach for nonresectable CC. […] If results are confirmed, new photosensitizers with greater penetration depth and shorter photosensitivity should be used, or better drug targeting or combination therapies should be implemented to induce additional tumor necrosis.
  • #27 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413
    Radiation therapy. Radiation therapy uses high-powered energy beams from sources such as X-rays and protons to kill cancer cells. Radiation therapy can involve a machine that directs radiation beams at your body (external beam radiation). Or it can involve placing radioactive material inside your body near the site of your cancer (brachytherapy). […] Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die. Your doctor may test your cancer cells to see if targeted therapy may be effective against your cholangiocarcinoma. […] Immunotherapy. Immunotherapy uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that help them hide from the immune system cells. Immunotherapy works by interfering with that process. For cholangiocarcinoma, immunotherapy might be an option for advanced cancer when other treatments haven’t helped.
  • #28 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413
    Radiation therapy. Radiation therapy uses high-powered energy beams from sources such as X-rays and protons to kill cancer cells. Radiation therapy can involve a machine that directs radiation beams at your body (external beam radiation). Or it can involve placing radioactive material inside your body near the site of your cancer (brachytherapy). […] Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die. Your doctor may test your cancer cells to see if targeted therapy may be effective against your cholangiocarcinoma. […] Immunotherapy. Immunotherapy uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that help them hide from the immune system cells. Immunotherapy works by interfering with that process. For cholangiocarcinoma, immunotherapy might be an option for advanced cancer when other treatments haven’t helped.
  • #29 Klatskin Tumors (Hilar Cholangiocarcinoma)
    https://my.clevelandclinic.org/health/diseases/hilar-cholangiocarcinoma
    Treatments will vary depending on your situation, but surgery is a common front-line or initial treatment for this condition. Surgery may be: […] Liver transplantation: The only cure for this condition is removing the cancerous tumor(s), which may mean removing your liver and having a liver transplant. […] Providers may use chemoradiation, which combines chemotherapy and radiation therapy, along with surgery or when surgery isn’t an option. Two newer treatments for metastatic Klatskin tumor are: […] Immunotherapy, like immune checkpoint inhibitors that work by helping your immune system to fight cancer. […] Targeted therapy, like fibroblast growth factor receptor (FGFR) inhibitors or IDH inhibitors. FGFR inhibitors block cancerous cell growth so the cells die. IDH inhibitors block an abnormal protein that affects normal cell growth.
  • #30 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413
    Heating cancer cells. Radiofrequency ablation uses electric current to heat and destroy cancer cells. Using an imaging test as a guide, such as ultrasound, the doctor inserts one or more thin needles into small incisions in your abdomen. When the needles reach the cancer, they’re heated with an electric current, destroying the cancer cells. […] Photodynamic therapy. In photodynamic therapy, a light-sensitive chemical is injected into a vein and accumulates in the fast-growing cancer cells. Laser light directed at the cancer causes a chemical reaction in the cancer cells, killing them. You’ll typically need multiple treatments. Photodynamic therapy can help relieve your signs and symptoms, and it may also slow cancer growth. You’ll need to avoid sun exposure after treatments. […] Biliary drainage. Biliary drainage is a procedure to restore the flow of bile. It might involve placing a thin tube into the bile duct in order to drain the bile. Other strategies include bypass surgery to reroute the bile around the cancer and stents to hold open a bile duct being collapsed by cancer. Biliary drainage helps relieve signs and symptoms of cholangiocarcinoma. […] Because cholangiocarcinoma is a very difficult type of cancer to treat, don’t hesitate to ask about your doctor’s experience with treating the condition. If you have any doubts, get a second opinion.
  • #31 Cholangiocarcinoma Treatment & Management: Approach Considerations, Stent Placement, Photodynamic Therapy
    https://emedicine.medscape.com/article/277393-treatment
    Complete surgical resection is the only therapy to afford a chance of cure for cholangiocarcinoma. Unfortunately, many patients present with unresectable disease. Additional treatment measures in cholangiocarcinoma may include the following: Stenting, Photodynamic therapy (PDT), Radiation therapy, Pharmacotherapy. […] For palliative treatment, celiac-plexus block via regional injection of alcohol or other sclerosing agent can relieve pain in the mid back from retroperitoneal tumor growth. In addition, other endoscopic forms of palliation, such as brachytherapy and radiofrequency ablation, have been used. […] Stents can be placed via endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous transhepatic cholangiography (PTC) to relieve biliary obstruction. Stenting may relieve pruritus and improve quality of life.
  • #32 Bile Duct Cancer Stage and Treatment Options | Cholangiocarcinoma Management | American Cancer Society
    https://www.cancer.org/cancer/types/bile-duct-cancer/treating/based-on-situation.html
    For some unresectable intrahepatic or perihilar bile duct cancers, a liver transplant may be an option. Chemo and radiation may be given first. […] For most bile duct cancers, it’s clear from imaging tests and/or laparoscopy when they’re not resectable. For these cancers, treatment is aimed at trying to control the growth of the cancer for as long as possible and to relieve any symptoms it’s causing. […] Radiation therapy and/or chemo may shrink or slow the growth of the cancer for a time. […] For advanced bile duct cancers that can’t be removed completely, another option might be chemo, plus the immunotherapy such as pembrolizumab or Durvalumab. […] For bile duct cancers within the liver, ablation using extreme heat or cold or alcohol might help control the tumors. […] Much of the focus of treating people with unresectable cancers is on relieving symptoms from the cancer. […] Palliative care is supportive care. It’s aimed at preventing and treating symptoms or problems caused by the cancer. […] In rare cases, if the cancer comes back where it started, surgery to try to remove the cancer may be an option.
  • #33 Cholangiocarcinoma (bile duct cancer) – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/diagnosis-treatment/drc-20352413
    Heating cancer cells. Radiofrequency ablation uses electric current to heat and destroy cancer cells. Using an imaging test as a guide, such as ultrasound, the doctor inserts one or more thin needles into small incisions in your abdomen. When the needles reach the cancer, they’re heated with an electric current, destroying the cancer cells. […] Photodynamic therapy. In photodynamic therapy, a light-sensitive chemical is injected into a vein and accumulates in the fast-growing cancer cells. Laser light directed at the cancer causes a chemical reaction in the cancer cells, killing them. You’ll typically need multiple treatments. Photodynamic therapy can help relieve your signs and symptoms, and it may also slow cancer growth. You’ll need to avoid sun exposure after treatments. […] Biliary drainage. Biliary drainage is a procedure to restore the flow of bile. It might involve placing a thin tube into the bile duct in order to drain the bile. Other strategies include bypass surgery to reroute the bile around the cancer and stents to hold open a bile duct being collapsed by cancer. Biliary drainage helps relieve signs and symptoms of cholangiocarcinoma. […] Because cholangiocarcinoma is a very difficult type of cancer to treat, don’t hesitate to ask about your doctor’s experience with treating the condition. If you have any doubts, get a second opinion.
  • #34 Treatment Options – Cholangiocarcinoma Australia
    https://cholangiocarcinomaaustralia.org/treatment-options/
    Radioembolization is very similar to chemoembolization but with the use of radioactive microspheres. […] Because of the poor response to regular (systemic) chemo, doctors have tried giving the drugs directly into the hepatic artery. […] For this technique, a light-activated drug is injected into a vein. […] Chemotherapy (chemo) is anti-cancer drugs given through a vein or by mouth. […] Unfortunately, chemo has not been very effective against bile duct cancer, so its use has been somewhat limited. […] As researchers have learned more about the gene and protein changes in cells that cause cancer, theyve developed newer drugs known as Targeted Therapy to specifically target these changes. […] Immunotherapy is treatment that helps a persons immune system better recognize and destroy cancer cells. […] This is treatment given to help control or reduce symptoms caused by advanced cholangiocarcinoma.
  • #35 Cholangiocarcinoma Treatment & Management: Approach Considerations, Stent Placement, Photodynamic Therapy
    https://emedicine.medscape.com/article/277393-treatment
    Complete surgical resection is the only therapy to afford a chance of cure for cholangiocarcinoma. Unfortunately, many patients present with unresectable disease. Additional treatment measures in cholangiocarcinoma may include the following: Stenting, Photodynamic therapy (PDT), Radiation therapy, Pharmacotherapy. […] For palliative treatment, celiac-plexus block via regional injection of alcohol or other sclerosing agent can relieve pain in the mid back from retroperitoneal tumor growth. In addition, other endoscopic forms of palliation, such as brachytherapy and radiofrequency ablation, have been used. […] Stents can be placed via endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous transhepatic cholangiography (PTC) to relieve biliary obstruction. Stenting may relieve pruritus and improve quality of life.
  • #36 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition
    https://hbsn.amegroups.org/article/view/3374/html
    Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6th decade of life. […] Treatment consists of surgery, radiation, chemotherapy and photodynamic therapy. […] Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile duct resection, hepatectomy and en bloc lymphadenectomy. […] In instances where curative resection is not possible, liver transplantation has demonstrated acceptable outcomes in highly selected patients. […] Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. […] Due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended for this complex disease. […] Margin negative (R0) resection remains the only treatment that offers the chance at long-term survival.
  • #37 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – PubMed
    https://pubmed.ncbi.nlm.nih.gov/24696835/
    Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6(th) decade of life. […] Treatment consists of surgery, radiation, chemotherapy and photodynamic therapy. Biliary drainage of the future liver remnant should be performed to decrease bilirubin levels thereby facilitating future liver hypertrophy. Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile duct resection, hepatectomy and en bloc lymphadenectomy. […] In instances where curative resection is not possible, liver transplantation has demonstrated acceptable outcomes in highly selected patients. Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. […] Due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended for this complex disease.
  • #38 Non-surgical treatment of hilar cholangiocarcinoma
    https://www.wjgnet.com/1948-5204/full/v13/i11/1696.htm
    The role of chemotherapy is sometimes associated with transplantation in the unresectable disease limited-stage; since 2005 some experience is described to investigate the role of liver transplantation after chemoradiation in stage I and II HCs. […] New target therapies have demonstrated a potential role in the intrahepatic cholangiocarcinoma treatment with isocitrate dehydrogenase (IDH) 1-IDH2 mutation and FGFR2 fusion. […] The future direction of the medical treatment of HC it might be a combination of therapies involving immunotherapy plus chemotherapy, immunotherapy and radiotherapy. […] Over the past two decades, several options for the management of unresectable HC have emerged. Due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended, including surgery, medical oncology, radiation oncology, diagnostic radiology, interventional radiology, gastroenterology, and pathology. Recent studies suggest an improvement in OS, better response rates, and tumor control in patients with unresectable HC can be achieved by combining chemotherapy and ablatives strategies.
  • #39 7 Innovative Bile Duct Cancer (Cholangiocarcinoma) Treatment Options | MD Anderson Cancer Center
    https://www.mdanderson.org/cancer-types/bile-duct-cancer/bile-duct-cancer-treatment.html
    MD Anderson has experts with years of experience treating bile duct and gallbladder cancer with surgery, radiation and chemotherapy. Choosing a treatment depends on the disease stage, a patients physical condition and tumor characteristics. MD Andersons treatment approach is individualized for every patient. […] Complete removal of the tumor is the most effective biliary cancer treatment. This may be possible depending on the location of the tumor along the bile duct. This surgery is most often offered to patients with early-stage disease who are in good physical condition. […] MD Anderson is a leader in using portal vein embolization (PVE) to spur liver growth. This involves the injection of tiny plastic pellets through a needle into the blood vessel closest to the tumor. […] Radiation therapy uses focused, high-energy radiation beams to destroy cancer cells. It can improve survival and provide a chance of a cure or prolonged disease control in patients who dont receive surgery.
  • #40 7 Innovative Bile Duct Cancer (Cholangiocarcinoma) Treatment Options | MD Anderson Cancer Center
    https://www.mdanderson.org/cancer-types/bile-duct-cancer/bile-duct-cancer-treatment.html
    Depending on a patients anatomy and liver function, MD Anderson radiation oncology experts will develop a personalized treatment plan that may include the following options: […] Most biliary cancers are discovered at an advanced stage when surgical treatment options are limited. Chemotherapy works by killing fast growing cells, including cancer cells, all over the body. It is typically used to control the spread of the cancer, alleviate symptoms and improve overall survival. […] Targeted therapy is an important area of biliary cancer research. Several genetic mutations are treatment targets for biliary cancer. Targeted therapies seek out and destroy these abnormalities within cancer cells. […] MD Anderson is developing new treatments and clinical trials to improve the prognosis and survival of patients with biliary cancers.
  • #41 Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente
    https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.bile-duct-cancer-cholangiocarcinoma-treatment-pdq%C2%AE-treatment-health-professional-information-nci.ncicdr0000062905
    All patients with unresectable, metastatic, or recurrent disease who have not already received a checkpoint inhibitor should have molecular testing for deficient mismatch repair (dMMR) or microsatellite instability-high (MSI-H) tumors. […] Clinical trials of investigational therapies should be considered for patients with targetable mutations. […] Patients with FGFR2 fusion-positive disease should be encouraged to enroll in a clinical trial. […] All patients are encouraged to enroll in clinical trials for adjuvant therapies.
  • #42 British Society of Gastroenterology guidelines for the diagnosis and management of cholangiocarcinoma | Gut
    https://gut.bmj.com/content/73/1/16
    Recommendation 32: R0 resection is the only curative treatment available. […] Recommendation 34: Liver transplantation for selected patients with perihilar CCA (pCCA) in the presence of chronic liver disease (most commonly primary sclerosing cholangitis), less than 3 cm in size with no evidence of extrahepatic disease, results in long-term disease-free survival. […] Recommendation 37: Patients who have undergone surgical resection for CCA should be considered for 24 weeks of adjuvant chemotherapy (currently capecitabine). […] Recommendation 39: Cisplatin plus gemcitabine (CisGem) chemotherapy is recommended as the first-line treatment in patients with advanced biliary tract cancer (BTC). […] Recommendation 41: CCA should be subjected to molecular profiling at the earliest opportunity, and results and treatment options should be reviewed by clinicians with appropriate expertise.
  • #43 Multi-Disciplinary Care of Hilar Cholangiocarcinoma: Review of Guidelines and Recent Advancements
    https://www.mdpi.com/2072-6694/16/1/30
    Upfront surgery with adjuvant capecitabine is the only curative treatment for hilar cholangiocarcinoma. […] Unfortunately, most patients do not present with resectable disease and are treated with a combination of locoregional therapy and systemic therapy. […] For patients with resectable disease, the standard of care is upfront surgery with adjuvant capecitabine. […] While surgery confers the greatest survival advantage, most patients will develop recurrent or metastatic disease afterwards. […] Systemic therapy options for these patients are limited and do little to improve long-term survival. […] Given the tumor heterogeneity associated with CCA, research has focused on developing effective targeted therapies. […] Targeted therapy carries a better side effect profile than cytotoxic chemotherapy and can be personalized based on the tumor’s genomic landscape.
  • #44 New systemic treatment options for advanced cholangiocarcinoma
    https://www.e-jlc.org/journal/view.php?doi=10.17998/jlc.2024.08.07
    Cholangiocarcinoma (CCA) is a rare and aggressive cancer, mostly diagnosed at advanced or metastatic stage, at which point systemic treatment represents the only therapeutic option. Chemotherapy has been the backbone of advanced CCA treatment. More recently, immunotherapy has changed the therapeutic landscape, as immune checkpoint inhibitors have yielded the first improvement in survival and currently, the addition of either durvalumab or pembrolizumab to standard of care cisplatin plus gemcitabine represents the new first-line treatment option. […] However, the use of immunotherapy in subsequent lines has not demonstrated its efficacy and therefore, it is not approved, except for pembrolizumab in the selected microsatellite instability-high population. In addition, advances in comprehensive genomic profiling have led to the identification of targetable genetic alterations, such as isocitrate dehydrogenase 1 (IDH1), fibroblast growth factor receptor 2 (FGFR2), human epidermal growth factor receptor 2 (HER2), proto-oncogene B-Raf (BRAF), neurotrophic tropomyosin receptor kinase (NTRK), rearranged during transfection (RET), Kirsten rat sarcoma virus (KRAS), and mouse double minute 2 homolog (MDM2), thus favoring the development of a precision medicine approach in previously treated patients. […] This review aims to provide an overview of the newly approved systemic therapies, the ongoing studies, and future research challenges in advanced CCA management.
  • #45 New systemic treatment options for advanced cholangiocarcinoma
    https://www.e-jlc.org/journal/view.php?doi=10.17998/jlc.2024.08.07
    Immunotherapy has recently changed the treatment landscape of advanced CCA, as immune checkpoint inhibitors (ICIs) have yielded the first improvement in OS in the first-line setting after over a decade of chemotherapy-based systemic treatment. […] Pembrolizumab, an anti-programmed death-1 (PD-1) antibody, was the first ICI evaluated in advanced CCA. […] Moreover, pembrolizumab was the first ICI approved for patients with pretreated microsatellite instability-high (MSI-H) advanced CCA, based on the results of the KEYNOTE-158 phase II basket trial. […] These promising results, coupled with a manageable safety profile, represented the basis for the TOPAZ-1 trial, which was the first phase III study that demonstrated a benefit in survival by adding durvalumab to standard of care chemotherapy with cisplatin and gemcitabine. […] New systemic treatment options including immunotherapy and targeted agents have demonstrated clinical efficacy and manageable safety profiles, surpassing chemotherapy-based regimens, and constitute the foundation of a novel treatment algorithm for the management of advanced CCA.
  • #46 First- and Second-Line Treatment Options for Intrahepatic Cholangiocarcinoma With FGFR2 Fusions or Alterations
    https://www.onclive.com/view/first–and-second-line-treatment-options-for-intrahepatic-cholangiocarcinoma-with-fgfr2-fusions-or-alterations
    Kelley stated first-line treatment for advanced cholangiocarcinoma is chemotherapy (gemcitabine/cisplatin) plus immunotherapy (durvalumab/pembrolizumab), regardless of mutation status, based on improved survival over chemotherapy alone. […] If patients have chemotherapy contraindications, Kelley would consider off-label FGFR blockade upfront. […] For FGFR2-altered cholangiocarcinoma progressing after first-line therapy, Goyal discussed FDA-approved FGFR inhibitors pemigatinib and futibatinib. The pemigatinib trial reported a 35.5% response rate and 7-month progression-free survival in previously treated patients. Since historical second-line chemotherapy response rates are only 5%, this efficacy is encouraging. Futibatinib demonstrated a 42% response rate and 9 months progression-free survival. Both drugs showed favorable safety profiles. […] In summary, first-line chemoimmunotherapy remains standard for advanced cholangiocarcinoma. However, 2 FGFR inhibitors have now shown marked efficacy vs historical treatments. Further trials explore these agents in additional lines of therapy and combination regimens.
  • #47 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition
    https://hbsn.amegroups.org/article/view/3374/html
    Major hepatectomy combined with extra hepatic bile duct resection has increased R0 resection rates as well as long term survival and should be considered standard therapy. […] The role of minimally invasive HC resections also remains unclear. […] The current standard chemotherapy regimens for unresectable HC are platinum based in combination with gemcitabine. […] Photodynamic therapy (PDT) involves the intravenous administration of photosensitizing agents which accumulate within cancer cells. […] In conclusion, HC is a rare but aggressive disease with a dismal long-term prognosis. Lymph node invasion, tumor grade and negative margins are important prognostic indicators. R0 resection represents the only chance for long-term survival.
  • #48 Surgery for Cholangiocarcinoma | CCA News Online
    https://www.ccanewsonline.com/web-exclusives/2521:surgery-for-cholangiocarcinoma
    Surgery for hilar CCA, or Klatskin tumor, is dependent on the extent of the disease. The goal of resection in patients with hilar CCA is a margin-negative resection, leaving at least 2 contiguous liver segments with adequate perfusion and biliary drainage. The surgery typically starts with a diagnostic laparoscopy, followed by a portal lymphadenectomy for staging, and then the removal of the bile duct, involved liver, and the caudate lobe, depending on the tumor location. […] In a series of 380 patients at Memorial Sloan Kettering Cancer Center in New York City, fewer than half of all individuals who were staged underwent curative resection. Most of these patients were found to be inoperable or with advanced disease. […] The use of minimally invasive surgery in patients with perihilar CCA has not been well-studied.
  • #49 New Therapies to Improve Outcomes for Patients With Intrahepatic Cholangiocarcinoma
    https://www.targetedonc.com/view/new-therapies-to-improve-outcomes-for-patients-with-intrahepatic-cholangiocarcinoma
    These FGFR inhibitors all have accelerated approvals based on single-arm, phase 2 trials that looked at patients with FGFR2 gene fusions and rearrangements, who had progressed on standard therapy and were started on 1 of these oral FGFR inhibitors. […] I also emphasized the fact that there is now a second generation of these drugs that are exciting, like RLY-4008. […] The other big area that’s really exciting in the targeted therapy space is in patients with HER2 amplifications and alterations, which is a little less common in intrahepatic cholangiocarcinoma. […] There was some exciting data that came out of ASCO with the monoclonal antibody zanidatamab [ZW25], as well as an oral tyrosine kinase inhibitor tucatinib [Tukysa] in combination with pembrolizumab. […] One of the greatest unmet needs is that 40% of patients have alterations, which means about 60% of patients don’t necessarily have a targeted therapy that we can think through. […] We have a lot of work to do in terms of refractory cholangiocarcinoma and thinking through novel drugs. […] And then of course, the other obvious question is, what can we do to build on the immunotherapy world?
  • #50 Klatskin Tumors (Hilar Cholangiocarcinoma)
    https://my.clevelandclinic.org/health/diseases/hilar-cholangiocarcinoma
    Klatskin tumor (hilar cholangiocarcinoma) is an aggressive type of bile duct cancer. There’s no question that it’s a challenging and serious illness. But data like survival rates don’t tell the whole story about this condition. It’s highly treatable, and researchers are doing clinical trials for new treatments. If you have this condition, your healthcare team will help you manage symptoms and treatment side effects. They’ll also be glad to suggest clinical trials you may want to consider.