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Case Report Open Access

Multimodal treatment and tumour biology‑driven long‑term survival in PSC‑associated hilar cholangiocarcinoma: A case report

  • Authors:
    • Evangelia Florou
    • Michael Heneghan
    • Debashis Sarker
    • Parthi Srinivasan
    • Andreas Prachalias
  • View Affiliations / Copyright

    Affiliations: Institute of Liver Studies, Department of Hepato‑Pancreato‑Biliary Surgery, King's College Hospital, London SE5 9RS, UK, Institute of Liver Studies, Department of General Hepatology and Liver Transplantation, King's College Hospital London, London SE5 9RS, UK;, Institute of Liver Studies, Department of Oncology, King's College Hospital London, London SE5 9RS, UK, Hepato‑Pancreato‑Biliary Surgery and Liver Transplantation, London Bridge Hospital, London SE1 2PR, UK
    Copyright: © Florou et al. This is an open access article distributed under the terms of Creative Commons Attribution License [CC BY 4.0].
  • Article Number: 11
    |
    Published online on: January 9, 2026
       https://doi.org/10.3892/mi.2026.295
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Abstract

Primary sclerosing cholangitis (PSC) is a recognised risk factor for hilar cholangiocarcinoma (hCCA). In selected patients, neoadjuvant chemoradiotherapy followed by liver transplantation provides the optimal chance of long‑term survival. However, for the patient described in the present case report, at the time of the patient's treatment, the UK did not have an approved transplant programme for cholangiocarcinoma, and access to liver transplantation was limited, often necessitating upfront surgical resection despite its complexity and limited curative potential. The present study describes the case of a 52‑year‑old male patient with PSC who was diagnosed with hCCA and underwent an extended right hepatectomy. After 26 months, progressive liver dysfunction due to PSC‑related cirrhosis prompted liver transplantation, which was approved following a lengthy appeals process. Over the following years, the patient developed metastases in the bowel, lungs and abdominal wall, all of which were successfully managed with surgical resections. He remained disease‑free for 8 years following his initial diagnosis before developing intrahepatic recurrence. The tumour was HER2‑positive, and the compassionate use of zanidatamab was initiated following progression on standard therapies. At the time of the writing of the present case report, the patient remained alive 101 months following this initial diagnosis. On the whole, the present case report highlights the potential impact of tumour biology and multimodal treatment in PSC‑associated hCCA. The prolonged survival of the patient despite delayed transplant and metastatic recurrence suggests that PSC‑related hCCA may follow a more indolent course compared to de novo cases. Future efforts are required to focus on tumour profiling and stratified therapeutic approaches to better guide treatment in this complex disease.
View Figures

Figure 1

Axial computed tomography image (left
panel) demonstrating bilateral intrahepatic biliary dilatation
secondary to a hilar cholangiocarcinoma (yellow arrow). Coronal
fluorodeoxyglucose positron emission tomography image (right panel)
illustrating intense metabolic activity at the liver hilum
corresponding to the hilar mass (yellow arrow). The image was
obtained in May, 2017.

Figure 2

Axial computed tomography image
demonstrating hypertrophy of the left lateral liver segment
following portal vein embolization, performed in preparation for an
extended right hepatectomy.

Figure 3

Axial computed tomography image
illustrating the post-operative liver remnant following extended
right hepatectomy.

Figure 4

Haematoxylin and eosin staining
demonstrating a moderately differentiated tubular adenocarcinoma,
consistent with perihilar cholangiocarcinoma (original
magnification, x400; indicative scale bar, ~50 µm).

Figure 5

Orthotopic liver transplantation using
a whole-liver graft from a brain-dead donor in 2019. The figure
depicts a post-operative computed tomography scan performed in
January, 2020, prior to discharge, after a long recovery from
surgery.

Figure 6

MRCP illustrating a new hilar
stricture at the hepaticojejunostomy (yellow arrow), consistent
with ischemic cholangiopathy in the liver graft (March, 2021). This
was treated with balloon dilation.

Figure 7

Axial contrast-enhanced computed
tomography scan demonstrating a solitary pulmonary nodule in the
left lower lobe, identified on routine surveillance in June,
2022.

Figure 8

Axial computed tomography scan
demonstrating a right upper lobe lung metastasis (left panel),
subsequently treated with image-guided ablation (right panel).

Figure 9

Axial computed tomography
demonstrating an FDG-avid soft tissue lesion in the anterior rectus
sheath, consistent with metastatic cholangiocarcinoma (white
arrow).

Figure 10

Haematoxylin and eosin staining of the
rectus sheath metastasis, demonstrating moderately differentiated
adenocarcinoma (magnification, x100; no calibrated scale bar
available at source).

Figure 11

Axial computed tomography imaging
illustrating multiple liver metastases in 2025, occurring 6 years
following liver transplantation and 8 years following the initial
diagnosis of PSC-related hilar cholangiocarcinoma.

Figure 12

HER2 immunohistochemical staining
illustrating membranous staining (score 2) (magnification, x200; no
calibrated scale bar available at source). Slides were reviewed
using a standard light microscope.
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Copy and paste a formatted citation
Spandidos Publications style
Florou E, Heneghan M, Sarker D, Srinivasan P and Prachalias A: Multimodal treatment and tumour biology‑driven long‑term survival in PSC‑associated hilar cholangiocarcinoma: A case report. Med Int 6: 11, 2026.
APA
Florou, E., Heneghan, M., Sarker, D., Srinivasan, P., & Prachalias, A. (2026). Multimodal treatment and tumour biology‑driven long‑term survival in PSC‑associated hilar cholangiocarcinoma: A case report. Medicine International, 6, 11. https://doi.org/10.3892/mi.2026.295
MLA
Florou, E., Heneghan, M., Sarker, D., Srinivasan, P., Prachalias, A."Multimodal treatment and tumour biology‑driven long‑term survival in PSC‑associated hilar cholangiocarcinoma: A case report". Medicine International 6.1 (2026): 11.
Chicago
Florou, E., Heneghan, M., Sarker, D., Srinivasan, P., Prachalias, A."Multimodal treatment and tumour biology‑driven long‑term survival in PSC‑associated hilar cholangiocarcinoma: A case report". Medicine International 6, no. 1 (2026): 11. https://doi.org/10.3892/mi.2026.295
Copy and paste a formatted citation
x
Spandidos Publications style
Florou E, Heneghan M, Sarker D, Srinivasan P and Prachalias A: Multimodal treatment and tumour biology‑driven long‑term survival in PSC‑associated hilar cholangiocarcinoma: A case report. Med Int 6: 11, 2026.
APA
Florou, E., Heneghan, M., Sarker, D., Srinivasan, P., & Prachalias, A. (2026). Multimodal treatment and tumour biology‑driven long‑term survival in PSC‑associated hilar cholangiocarcinoma: A case report. Medicine International, 6, 11. https://doi.org/10.3892/mi.2026.295
MLA
Florou, E., Heneghan, M., Sarker, D., Srinivasan, P., Prachalias, A."Multimodal treatment and tumour biology‑driven long‑term survival in PSC‑associated hilar cholangiocarcinoma: A case report". Medicine International 6.1 (2026): 11.
Chicago
Florou, E., Heneghan, M., Sarker, D., Srinivasan, P., Prachalias, A."Multimodal treatment and tumour biology‑driven long‑term survival in PSC‑associated hilar cholangiocarcinoma: A case report". Medicine International 6, no. 1 (2026): 11. https://doi.org/10.3892/mi.2026.295
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