NEJM Evidence June 01, 2022

Durvalumab plus Gemcitabine and Cisplatin in Advanced Biliary Tract Cancer (TOPAZ-1)

Do-Youn Oh, Aiwu Ruth He, Shukui Qin, Li-Tzong Chen, Takuji Okusaka, Arndt Vogel, et al.

Bottom Line

The phase 3 TOPAZ-1 trial demonstrated that adding the PD-L1 inhibitor durvalumab to standard gemcitabine and cisplatin significantly improves overall survival in previously untreated patients with advanced biliary tract cancer.

Key Findings

1. Durvalumab plus chemotherapy significantly improved overall survival compared to placebo plus chemotherapy (median OS 12.8 vs. 11.5 months; HR 0.80, 95% CI 0.66–0.97; P=0.021) [3.1.2].
2. The estimated 24-month overall survival rate was substantially higher in the durvalumab arm (24.9%) compared to the placebo arm (10.4%), indicating a durable long-term benefit for a subset of patients.
3. Progression-free survival was significantly prolonged with the addition of durvalumab (HR 0.75, 95% CI 0.63–0.89; P=0.001).
4. The objective response rate was higher with durvalumab plus chemotherapy (26.7%) versus placebo plus chemotherapy (18.7%).
5. The incidence of grade 3 or 4 treatment-related adverse events was similar between the two groups (62.7% with durvalumab vs. 64.9% with placebo), demonstrating that the addition of durvalumab did not compromise the safety profile of the chemotherapy backbone.

Study Design

Design
RCT
Double-Blind
Sample
685
Patients
Duration
16.8 mo
Median
Setting
Global, multicenter
Population Patients with previously untreated, unresectable locally advanced, recurrent, or metastatic biliary tract cancer with an ECOG performance status of 0 or 1
Intervention Durvalumab (1500 mg every 3 weeks) plus gemcitabine and cisplatin for up to 8 cycles, followed by durvalumab maintenance (1500 mg every 4 weeks) until disease progression or unacceptable toxicity
Comparator Placebo plus gemcitabine and cisplatin for up to 8 cycles, followed by placebo maintenance every 4 weeks
Outcome Overall survival

Study Limitations

The absolute improvement in median overall survival was relatively modest (1.3 months), reflecting the aggressive and treatment-refractory nature of advanced biliary tract cancers.
Biliary tract cancers are highly heterogeneous (encompassing intrahepatic, extrahepatic, and gallbladder cancers), and the trial's subgroup analyses may be underpowered to fully characterize differential efficacy based on primary tumor anatomical location.
The study lacked a prospectively validated predictive biomarker (such as a PD-L1 expression threshold) to help clinicians identify which specific patients are most likely to derive the long-term survival benefit.
Patients with poor performance status (ECOG > 1) were excluded, which may limit the generalizability of the findings to real-world patients who often present with a significant disease burden and functional decline.

Clinical Significance

TOPAZ-1 was a landmark, practice-changing study that established durvalumab plus gemcitabine and cisplatin as the new global first-line standard of care for patients with advanced biliary tract cancer. It represented the first major survival breakthrough utilizing an immune checkpoint inhibitor for an unselected biliary tract cancer population.

Historical Context

For over a decade following the 2010 ABC-02 trial, the gemcitabine and cisplatin chemotherapy doublet remained the undisputed first-line standard of care for advanced biliary tract cancer. Despite numerous phase 3 trials attempting to build upon this backbone, progress had stalled for the general patient population. While precision oncology recently advanced for biomarker-selected subgroups (e.g., FGFR2 fusions, IDH1 mutations), TOPAZ-1 was the first successful global phase 3 study to incorporate immunotherapy into the frontline setting, breaking a decade-long plateau in unselected biliary tract cancer survival.

Guided Discussion

High-yield insights from every perspective

Med Student
Medical Student

How does the addition of a cytotoxic chemotherapy regimen like gemcitabine and cisplatin theoretically enhance the efficacy of an immune checkpoint inhibitor like durvalumab in biliary tract cancer?

Key Response

Cytotoxic chemotherapy induces immunogenic cell death, releasing tumor neoantigens into the tumor microenvironment. Furthermore, it can deplete immunosuppressive cells like regulatory T cells and myeloid-derived suppressor cells. This converts a 'cold' tumor into a 'hot' tumor, synergizing with PD-L1 blockade (durvalumab) which then prevents the tumor from suppressing the newly primed T-cell response.

Resident
Resident

A 65-year-old patient is newly diagnosed with metastatic intrahepatic cholangiocarcinoma. Based on the TOPAZ-1 trial, what is the preferred first-line systemic therapy, and is baseline PD-L1 expression testing required to initiate this specific triplet regimen?

Key Response

The preferred first-line therapy is Gemcitabine plus Cisplatin combined with Durvalumab. Notably, the TOPAZ-1 trial demonstrated a survival benefit regardless of baseline PD-L1 expression. Therefore, while molecular profiling (including MMR/MSI status) is standard practice, PD-L1 testing is not a mandatory prerequisite to initiate this regimen.

Fellow
Fellow

The Kaplan-Meier overall survival curves in the TOPAZ-1 trial demonstrate a delayed separation, diverging around the 6-month mark and forming a distinct 'tail'. How does this phenomenon influence our interpretation of the hazard ratio, and how does it impact patient counseling regarding early versus late treatment benefits?

Key Response

Delayed separation is classic for immunotherapy, reflecting the time required for immune activation, which may violate the proportional hazards assumption (meaning the HR of 0.80 is an average over time rather than a constant risk reduction). Clinically, patients should be counseled that initial disease control is largely driven by the chemotherapy backbone, while the immunotherapy component provides the potential for durable, long-term survival (the 'tail') in a subset of responders.

Attending
Attending

Given the relatively modest median overall survival improvement of roughly 1.3 months in the TOPAZ-1 trial, how do you clinically justify the incorporation of durvalumab into routine practice considering the added financial toxicity, and which efficacy metric provides the strongest argument for its use?

Key Response

While the median OS improvement is modest, the justification for adoption lies in the landmark survival rates (e.g., estimated 24-month OS of 24.9% with durvalumab vs 10.4% with placebo). Immunotherapy's true clinical value in advanced solid tumors is often found in the tail of the curve, offering a clinically meaningful chance of long-term survival to a subset of patients without significantly increasing the rate of severe chemotherapy-related adverse events.

Scholarly Review

Critical appraisal through the lens of expert reviewers and guideline development

PhD
PhD

The TOPAZ-1 trial utilized a hierarchical testing strategy for its primary endpoint (Overall Survival) and secondary endpoints. How does this alpha-spending approach protect against Type I error in oncology trials, and what are the statistical limitations when heavily weighting the 'tail' of a Kaplan-Meier curve where the number at risk is small?

Key Response

Hierarchical testing strictly controls the family-wise error rate by ensuring secondary endpoints are only formally tested for significance if the primary endpoint crosses the prespecified efficacy boundary. However, relying on the 'tail' of the KM curve is statistically precarious; heavy censoring and low 'number at risk' at late time points widen confidence intervals, making survival estimates highly unstable and overly sensitive to individual patient events, which can limit generalizability.

Journal Editor
Journal Editor

As a reviewer evaluating the TOPAZ-1 manuscript, how would you scrutinize the potential unmeasured confounding arising from post-progression crossover therapies, and how might the lack of mandatory crossover to immune checkpoint inhibitors in the control arm bias the overall survival results?

Key Response

A critical reviewer would heavily scrutinize the supplementary data for subsequent therapies. If a significant portion of patients in the control (placebo) arm did not have access to off-protocol checkpoint inhibitors post-progression, the overall survival benefit observed might conflate the efficacy of adding durvalumab upfront versus simply having access to immunotherapy sequentially. Determining whether the OS benefit is truly synergistic or merely additive requires careful analysis of post-study drug availability.

Guideline Committee
Guideline Committee

In updating the NCCN and ESMO guidelines for advanced biliary tract cancer post-TOPAZ-1, should Gemcitabine plus Cisplatin plus Durvalumab be granted a Category 1 recommendation across all anatomical subtypes, and how does this paradigm shift compare to the historic ABC-02 standard of care?

Key Response

Yes, it should be (and was) granted a Category 1, preferred recommendation for all previously untreated locally advanced or metastatic biliary tract cancers. The TOPAZ-1 trial included intrahepatic, extrahepatic, and gallbladder cancers, showing consistent benefit across these subgroups in forest plots. This marks a major paradigm shift, officially replacing the ABC-02 Gemcitabine/Cisplatin doublet, which had been the undisputed standard of care for over a decade, as the new first-line benchmark.

Clinical Landscape

Noteworthy Related Trials

2010

ABC-02 Trial

n = 410 · NEJM

Tested

Cisplatin plus gemcitabine

Population

Patients with locally advanced or metastatic biliary tract cancer

Comparator

Gemcitabine alone

Endpoint

Overall survival

Key result: Cisplatin plus gemcitabine significantly improved overall survival compared to gemcitabine alone with a median OS of 11.7 months versus 8.1 months.
2021

ABC-06 Trial

n = 162 · Lancet Oncol

Tested

FOLFOX chemotherapy plus active symptom control

Population

Patients with advanced biliary tract cancer progressing on first-line cisplatin and gemcitabine

Comparator

Active symptom control alone

Endpoint

Overall survival

Key result: The addition of FOLFOX to active symptom control significantly improved overall survival in the second-line setting with a median OS of 6.2 months versus 5.3 months.
2023

KEYNOTE-966 Trial

n = 1069 · Lancet

Tested

Pembrolizumab plus gemcitabine and cisplatin

Population

Patients with previously untreated metastatic or unresectable biliary tract cancer

Comparator

Placebo plus gemcitabine and cisplatin

Endpoint

Overall survival

Key result: Adding pembrolizumab to chemotherapy significantly prolonged overall survival compared to chemotherapy alone with a median OS of 12.7 months versus 10.9 months.

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