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Single-Agent Checkpoint Blockade in ACC

Immunotherapy

Single-agent checkpoint blockade in adrenocortical carcinoma (ACC) refers primarily to PD-1 or PD-L1 inhibitor monotherapy used in recurrent, unresectable, or metastatic disease. Within ACC management, it is a systemic treatment option for advanced cancer rather than a substitute for surgery in localized tumors, and it has generally been studied after progression on or alongside mitotane-based and cytotoxic regimens.123 The available literature indicates that ACC is not uniformly refractory to immune checkpoint inhibition, but responses are uncommon and population-level benefit appears limited.45678910

Across prospective phase II studies, basket cohorts, and retrospective series, objective responses occur in a minority of treated patients, while stable disease is more frequent than marked tumor regression.4679 Median progression-free survival is typically short, although a small subset of patients appears to achieve durable benefit that is clinically meaningful in a disease with otherwise limited systemic options.5810 This pattern distinguishes checkpoint monotherapy from a reliably effective standard therapy and supports its use mainly as a selective or later-line approach.

The evidence base is constrained by the rarity of ACC and by substantial heterogeneity in the treated populations. Most reports involve small, nonrandomized cohorts, heavily pretreated patients, variable mitotane exposure, and inconsistent biomarker assessment, which limits cross-study comparison and weakens confidence in apparent subgroup effects.1121213 Proposed predictors such as PD-L1 expression, microsatellite instability, mismatch repair deficiency, tumor mutational burden, and cortisol secretion may influence response, but none has been validated as a consistently reliable selector in routine practice.471214

Biologic and diagnostic context

ACC has several biologic features that may help explain the modest activity of checkpoint monotherapy. Reviews and translational analyses describe many tumors as relatively immune-poor, with variable PD-L1 expression, limited effector T-cell infiltration, and tumor-intrinsic pathways such as WNT/beta-catenin and TP53 alterations that may contribute to immune exclusion.151612 Earlier immunotherapy work also identified plausible adrenal or steroidogenic antigens, but these concepts have not translated into broadly effective monotherapy.17

Hypercortisolism is a recurring concern because endogenous glucocorticoid excess may suppress antitumor immunity and alter the tumor microenvironment.151418 Available evidence supports this as a biologically plausible resistance mechanism, but the magnitude of its effect on checkpoint outcomes remains uncertain in clinical cohorts. The practical implication is that hormone secretion should inform interpretation of response expectations, even though it is not a validated exclusion criterion.

This biologic context helps frame the central clinical observation: ACC may occasionally respond well to PD-1 or PD-L1 blockade, but the disease as a whole appears relatively resistant. What is reliable is the existence of rare durable responders; what remains unreliable is prediction of who those patients will be before treatment begins.6710

Patterns of clinical activity

Pembrolizumab

Pembrolizumab is the best-studied single-agent checkpoint inhibitor in ACC. Across phase II studies and basket cohorts, it has shown modest but reproducible activity, with low-to-moderate response rates, disease control in a larger subset, and occasional prolonged responses despite short median progression-free survival for most patients.67810 The most dependable conclusion is that pembrolizumab can benefit selected patients, but it does not produce durable control for the majority.

Some responses have occurred despite microsatellite-stable disease or absent PD-L1 expression, indicating that conventional biomarkers do not fully capture sensitivity.6719 Clinically, pembrolizumab is a reasonable later-line option, especially when noncytotoxic therapy is preferred, but expectations should remain guarded.

Nivolumab

Prospective experience with nivolumab monotherapy suggests limited activity overall, while retrospective real-world data indicate that some patients may still achieve disease control.59 These data are less extensive than for pembrolizumab and are difficult to interpret because of small sample sizes and treatment heterogeneity.

Accordingly, nivolumab may be viewed as a class-consistent option rather than a clearly superior agent. What is reliable is that activity can occur; what is not reliable is any strong inference that nivolumab outperforms other PD-1 inhibitors in ACC.59

Avelumab

Avelumab has prospective data showing low objective response rates, modest disease control, and short progression-free survival, with toxicity that is generally manageable.4 Its main significance is that it demonstrates biologic activity of PD-L1 blockade in ACC, but not strong efficacy in unselected patients.

Indirect comparisons between avelumab and PD-1 inhibitors are not robust because the available studies differ in design and patient characteristics.412 In practice, avelumab supports the broader conclusion that single-agent PD-1/PD-L1 pathway inhibition has limited but genuine activity as a drug class.

Pediatric experience

Pediatric evidence is sparse and largely derived from small basket-trial experience and review-level summaries.2021 Early signals suggest activity is possible in some pediatric ACC cases, but the numbers are too small to define efficacy, sequencing, or biomarker selection with confidence.

The reliable conclusion is only that checkpoint sensitivity is not restricted to adults. The clinical implication is that pediatric use remains individualized and heavily dependent on extrapolation from limited evidence.2021

Biomarkers and response modifiers

No biomarker has been validated for routine prediction of response to single-agent checkpoint blockade in ACC. PD-L1 expression has shown inconsistent associations with outcome, and responses have been documented in PD-L1-negative tumors, limiting its utility as a stand-alone selection tool.4679 PD-L2 has also been proposed as a potentially relevant immune marker, but this remains exploratory rather than practice-defining.22

Mismatch repair deficiency, microsatellite instability-high status, and Lynch syndrome may enrich for benefit, consistent with broader immunotherapy experience, but these features are neither necessary nor sufficient for response.23714 Conversely, occasional responses in microsatellite-stable tumors underscore substantial biologic heterogeneity.619 The practical implication is that biomarker-positive disease may strengthen the rationale for treatment, whereas biomarker-negative disease should not automatically preclude consideration.

Emerging correlative work on immune-cell composition and intratumoral or circulating T-cell features is hypothesis-generating rather than validated for care decisions.18 At present, biomarkers mainly refine expectations and trial eligibility rather than reliably directing routine monotherapy selection.

Outcomes, safety, and major limitations

Taken together, the literature suggests that single-agent checkpoint blockade produces objective responses in only a minority of patients with advanced ACC, while disease control rates exceed response rates and median progression-free survival is commonly measured in months rather than years.4567910 This overall pattern is consistent across datasets even though exact estimates vary.

Compared with standard systemic approaches such as platinum-based chemotherapy plus mitotane, monotherapy checkpoint inhibition has not shown clearly superior population-level control in nonrandomized evidence.23 Its value lies more in the possibility of durable benefit for a subset of patients and in providing a noncytotoxic option after progression on conventional therapy. Because randomized comparative data are lacking, treatment decisions remain context dependent.

Adverse effects generally resemble those seen in other solid tumors treated with checkpoint inhibitors, with mostly low-grade events but the potential for serious immune-related toxicities including hepatitis, pneumonitis, and endocrinopathies.45610 In ACC, baseline endocrine dysfunction, glucocorticoid exposure, and the need to control hormone excess may complicate toxicity recognition and management. ACC-specific safety evidence is still limited, and some risks are inferred from broader oncology populations rather than disease-specific datasets.24

Role in management and implications for research

In current practice, single-agent checkpoint blockade occupies a selective role in advanced ACC, usually after standard therapies or when biomarker or clinical features suggest possible sensitivity.12 It is not established as preferred first-line therapy for metastatic disease and does not alter the central role of surgery for localized tumors.

The modest but real activity of monotherapy has also shaped subsequent research. Combination strategies with CTLA-4 blockade, antiangiogenic therapy, radiotherapy, chemotherapy, mitotane, or approaches aimed at glucocorticoid-mediated immune suppression are being developed in part because single-agent results suggest proof of principle but insufficient efficacy for most patients.25262728 Thus, single-agent checkpoint blockade currently serves both as an occasional treatment option and as the benchmark against which combination immunotherapy approaches in ACC are judged.

Included Articles

  • PMID 12931278: This review outlines early immunotherapy concepts for ACC, emphasizing dendritic cell vaccination and tissue-specific steroidogenic targets such as 21-hydroxylase, P450scc, StAR, and DAX-1. It presents preclinical rationale that these antigens may elicit cytotoxic antitumor responses despite the absence of well-defined ACC-specific tumor antigens.17
  • PMID 28893836: This review frames immune checkpoint inhibition as a potential treatment strategy for adrenocortical carcinoma within endocrine malignancies, emphasizing general CTLA-4 and PD-1/PD-L1 mechanisms, approved agents, and the challenge that endocrine tumors often have low mutational burden despite aggressive biology.1
  • PMID 30348224: In previously treated metastatic ACC, single-agent avelumab produced a 6% objective response rate, 48% disease control rate, median progression-free survival of 2.6 months, and median overall survival of 10.6 months, with manageable toxicity. Exploratory analyses suggested numerically higher activity in PD-L1-positive tumors, but the association was not statistically significant.4
  • PMID 30400026: This editorial summarizes early evidence that PD-L1 blockade with avelumab had limited activity in previously treated advanced ACC and discusses plausible resistance mechanisms, including low PD-L1 expression, glucocorticoid-mediated immunosuppression, and tumor-intrinsic WNT/beta-catenin or TP53-driven exclusion of effector immune cells.15
  • PMID 30527004: This discussion highlights that PD-L2 is expressed in nearly half of adrenocortical carcinomas and may be relevant to PD-1 blockade, because PD-1 inhibitors can block signaling from both PD-L1 and PD-L2. It also underscores that survival correlations and biomarker validation remain limited by small cohorts and incomplete follow-up.22
  • PMID 30593126: Two metastatic ACC case reports describe pembrolizumab given after multiple prior therapies in tumors with high mutational burden. A durable complete response occurred in the patient with an MSH2 mutation, whereas another high-TMB but mismatch-repair-proficient case had no response, suggesting MMR-related alterations may be more informative than TMB alone.23
  • PMID 30910391: This review summarizes early immunotherapy evidence in metastatic ACC, noting PD-L1 expression in a minority of tumor cells and many tumor-infiltrating mononuclear cells, plus modest activity of avelumab after platinum therapy with low response rates, short progression-free survival, and ongoing trials of PD-1/PD-L1 blockade alone or with CTLA-4 inhibition.11
  • PMID 31057613: This review summarizes early evidence that checkpoint blockade has limited activity in ACC and outlines proposed resistance mechanisms, including low PD-L1 positivity, glucocorticoid-driven immunosuppression, and tumor-intrinsic CTNNB1 and TP53 alterations linked to dendritic-cell exclusion and poor T-cell infiltration.16
  • PMID 31276163: A multicenter single-arm phase 2 trial found nivolumab had modest activity in metastatic ACC after prior platinum-based chemotherapy and/or mitotane, with median progression-free survival of 1.8 months and no confirmed responses in the first 10 evaluable patients. Toxicity was mostly grade 1 or 2, but immune-related hepatitis, pneumonitis, and other grade 3 or 4 adverse events occurred.5
  • PMID 31533818: A single-center phase II study of pembrolizumab monotherapy in previously treated metastatic ACC found modest activity, with a 27-week non-progression rate of 36%, objective response rate of 14%, and clinical benefit rate of 57%. Benefit was observed despite most tumors being microsatellite stable and all tested specimens lacking PD-L1 expression, with generally manageable toxicity.6
  • PMID 31644329: In a phase II single-center study of 39 patients with advanced ACC, pembrolizumab produced a 23% objective response rate and 52% disease control rate, with some durable responses despite a median progression-free survival of 2.1 months. Responses occurred in both MSI-H/MMR-deficient and microsatellite-stable tumors, while PD-L1 expression and MSI-H/MMR-deficient status were not associated with objective response.7
  • PMID 31812554: In the interim KEYNOTE-051 pediatric phase 1-2 trial, pembrolizumab at 2 mg/kg every 3 weeks was well tolerated but showed low overall activity across PD-L1-positive pediatric solid tumors; among 136 solid tumors and other lymphomas, partial responses occurred in two patients with adrenocortical carcinoma. The study also reported that PD-L1 positivity alone did not predict response.20
  • PMID 32188704: In a phase 2 basket trial of pembrolizumab for advanced rare cancers, the adrenocortical carcinoma cohort met interim non-futility criteria, with a 27-week non-progression rate of 31%, objective response rate of 15%, and clinical benefit rate of 54%. These findings suggest modest antitumor activity with a generally manageable toxicity profile in previously treated advanced ACC.8
  • PMID 32737143: A retrospective case series of eight heavily pretreated patients with recurrent or metastatic ACC found that lenvatinib plus pembrolizumab produced partial responses in two patients and stable disease in one, with median progression-free survival of 5.5 months and no treatment discontinuations for toxicity. The report highlights possible activity of checkpoint blockade when combined with a multikinase inhibitor after prior CPI or MKI failure, while emphasizing the need for prospective biomarker-driven trials.25
  • PMID 33498467: This review summarizes immune checkpoint inhibitor experience in ACC, emphasizing generally modest single-agent activity, signals of benefit with pembrolizumab in some patients including MSI-H/MMR-deficient disease, and ongoing combination strategies such as nivolumab plus ipilimumab or pembrolizumab plus glucocorticoid receptor antagonism.2
  • PMID 33809752: This review summarizes immune checkpoint inhibitor experience in advanced ACC, noting generally low response rates and short progression-free survival across five trials. It highlights unvalidated candidate predictors such as PD-1/PD-L1 expression, microsatellite instability, and tumor mutational burden, while emphasizing glucocorticoid-driven immunosuppression and other resistance mechanisms.12
  • PMID 33889043: A case report describes recurrent metastatic ACC with microsatellite-stable, PD-L1-negative disease achieving complete radiological response after four cycles of pembrolizumab added to ongoing mitotane. The report highlights potential activity of PD-1 blockade despite adverse biomarker features and notes the need for formal trials of the pembrolizumab-mitotane combination.19
  • PMID 33962475: This review summarizes early trial experience with immune checkpoint inhibitors in advanced ACC, noting modest overall activity but relatively more promising single-agent pembrolizumab results than avelumab or nivolumab. It highlights microsatellite instability or mismatch repair deficiency and Lynch syndrome as potential response biomarkers, while hypercortisolism may be linked to poorer immunotherapy responsiveness.14
  • PMID 34071333: This review summarizes immunotherapy experience in advanced adrenocortical carcinoma, including pembrolizumab, nivolumab, avelumab, vaccine approaches, dendritic cell strategies, and IGF-1 receptor-directed antibodies. Reported activity is generally modest but nonzero for checkpoint inhibitors, with ongoing trials testing combinations to improve outcomes.3
  • PMID 34261605: Computational analysis of TCGA ACC tumors found that higher mast cell infiltration and predominance of an antitumor mast cell signaling signature were associated with longer disease-free survival, while protumor mast cell gene signatures predicted worse survival. Cortisol-secreting ACC showed reduced expression of several favorable mast cell genes, suggesting a tumor microenvironment mechanism relevant to limited immunotherapy responsiveness.18
  • PMID 35464460: A review of IL-13Rα2-directed immunotherapy notes that IL-13Rα2 is overexpressed in adrenocortical carcinoma and associated with poor prognosis, supporting its candidacy as a targetable antigen. In metastatic ACC, intravenous IL13-PE immunotoxin showed dose-limiting toxicity at higher dosing and universal neutralizing antibody formation, highlighting feasibility and translational limitations.29
  • PMID 35836542: This case report describes metastatic ACC with lung involvement achieving repeated partial response and temporary disease control on EDP plus sintilimab, with mitotane added after cycle 1 and continued with sintilimab maintenance. The report also summarizes small phase II data suggesting limited but possible activity of PD-1 blockade in advanced ACC.30
  • PMID 37260092: This multicenter retrospective cohort of 54 patients with advanced ACC found modest real-world activity of immune checkpoint inhibitors, with an objective response rate of 13.5%, median progression-free survival of 3.0 months, and median overall survival of 10.4 months alongside manageable toxicity. PD-L1 positivity and treatment-related adverse events were associated with longer progression-free survival, while nivolumab showed more favorable outcomes than pembrolizumab after adjustment for concomitant mitotane use.9
  • PMID 37745701: A metastatic ACC case with MSI-H status, an MLH1 mutation, and high tumor mutational burden achieved durable stable disease after progression on mitotane plus etoposide-paraplatin, following addition of the PD-1 inhibitor sintilimab. The report also links cortisol control with mitotane to a potentially more favorable immunotherapy setting.31
  • PMID 37748508: This single-center retrospective case series describes three women with metastatic ACC treated with pembrolizumab plus high-dose-rate brachytherapy after progression on EDP-M. Local control of irradiated metastases was achieved, two patients had systemic complete or partial remissions despite lacking established checkpoint response markers, and severe immune-related toxicities occurred.26
  • PMID 39067873: A prospective phase II ACC cohort of the DART trial found dual checkpoint blockade with ipilimumab plus nivolumab showed activity in refractory metastatic disease, with confirmed partial responses in 14% and immune-related clinical benefit in 33%, including some notably durable responses despite short median progression-free survival.27
  • PMID 39220648: A case of metastatic ACC with lung metastases achieved a durable partial response and approximately 31 months of progression-free survival with etoposide-cisplatin plus sintilimab, with anlotinib added after cycle three, followed by sintilimab-anlotinib maintenance despite microsatellite-stable, low-TMB disease.32
  • PMID 39609453: A single-arm phase 2 trial in previously treated advanced ACC reported promising activity for camrelizumab plus apatinib, with objective response rate 52%, disease control rate 95%, median progression-free survival 13.3 months, and manageable toxicity. Exploratory analyses suggested response may correlate with higher peripheral CXCR3-positive CD8-positive T-cell abundance, lower immunosuppressive CD4-positive T-cell abundance, and greater overlap between circulating and tumor-infiltrating T-cell clonotypes.28
  • PMID 41143139: A single-center phase 2 trial of pembrolizumab monotherapy in previously treated advanced ACC found a 20% partial response rate, 30% nonprogression at 27 weeks, median progression-free survival of 4.0 months, and median overall survival of 15.5 months, with some durable responses and limited grade 3 or higher toxicity.10
  • PMID 41322192: This review notes that pediatric ACC is extremely rare and has poor outcomes when metastatic, highlighting early signals for immune checkpoint blockade. In cited data, pembrolizumab produced modest activity in adults with advanced ACC and objective responses in 2 of 4 pediatric ACC patients in KEYNOTE-051, supporting further study.21
  • PMID 41531268: A retrospective single-center study in previously treated metastatic ACC evaluated second-line cisplatin plus nivolumab, reporting partial responses in 34.8%, clinical benefit in 52.2%, median progression-free survival of 4.3 months, median overall survival of 18.9 months, and mostly low-grade toxicity.33
  • PMID 35924703: A retrospective urologic oncology series including one MSI-high ACC case described immune-related kidney injury after checkpoint inhibitor therapy, with higher risk in patients with advanced baseline CKD and with combination ICI regimens. Because ACC representation was limited to a single patient, the article is most informative as indirect safety context rather than disease-specific evidence.24
  • PMID 34680214: A 2021 genitourinary oncology review describes ACC as a rare tumor with comparatively little clinical immunotherapy data and notes that checkpoint inhibitor activity appears heterogeneous and limited overall.13

References

Footnotes

  1. Immunotherapy against endocrine malignancies: immune checkpoint inhibitors lead the way.. Endocr Relat Cancer. 2017. PMID: 28893836. Local full text: 28893836.md ↩ ↩2 ↩3

  2. The Role of Immunotherapy in the Treatment of Adrenocortical Carcinoma.. Biomedicines. 2021. PMID: 33498467. Local full text: 33498467.md ↩ ↩2 ↩3 ↩4 ↩5

  3. The Immunotherapy Landscape in Adrenocortical Cancer.. Cancers (Basel). 2021. PMID: 34071333. Local full text: 34071333.md ↩ ↩2 ↩3

  4. Avelumab in patients with previously treated metastatic adrenocortical carcinoma: phase 1b results from the JAVELIN solid tumor trial.. J Immunother Cancer. 2018. PMID: 30348224. Local full text: 30348224.md ↩ ↩2 ↩3 ↩4 ↩5 ↩6 ↩7 ↩8 ↩9

  5. Nivolumab in Metastatic Adrenocortical Carcinoma: Results of a Phase 2 Trial.. J Clin Endocrinol Metab. 2019. PMID: 31276163. Local full text: 31276163.md ↩ ↩2 ↩3 ↩4 ↩5 ↩6 ↩7

  6. Phase II clinical trial of pembrolizumab efficacy and safety in advanced adrenocortical carcinoma.. J Immunother Cancer. 2019. PMID: 31533818. Local full text: 31533818.md ↩ ↩2 ↩3 ↩4 ↩5 ↩6 ↩7 ↩8 ↩9 ↩10

  7. PD-1 Blockade in Advanced Adrenocortical Carcinoma.. J Clin Oncol. 2020. PMID: 31644329. Local full text: 31644329.md ↩ ↩2 ↩3 ↩4 ↩5 ↩6 ↩7 ↩8 ↩9 ↩10

  8. Phase 2 study of pembrolizumab in patients with advanced rare cancers.. J Immunother Cancer. 2020. PMID: 32188704. Local full text: 32188704.md ↩ ↩2 ↩3 ↩4

  9. Outcome of immunotherapy in adrenocortical carcinoma: a retrospective cohort study.. Eur J Endocrinol. 2023. PMID: 37260092. Local full text: 37260092.md ↩ ↩2 ↩3 ↩4 ↩5 ↩6 ↩7

  10. Phase 2 Study of Monotherapy with Pembrolizumab for Advanced Adrenocortical Carcinoma.. J Immunother Precis Oncol. 2025. PMID: 41143139. Local full text: 41143139.md ↩ ↩2 ↩3 ↩4 ↩5 ↩6 ↩7

  11. Rare Genitourinary Malignancies: Current Status and Future Directions of Immunotherapy.. Eur Urol Focus. 2020. PMID: 30910391. Local full text: 30910391.md ↩ ↩2

  12. Immunotherapy in Adrenocortical Carcinoma: Predictors of Response, Efficacy, Safety, and Mechanisms of Resistance.. Biomedicines. 2021. PMID: 33809752. Local full text: 33809752.md ↩ ↩2 ↩3 ↩4 ↩5

  13. Immunotherapies in Genitourinary Oncology: Where Are We Now? Where Are We Going?. Cancers (Basel). 2021. PMID: 34680214. Local full text: 34680214.md ↩ ↩2

  14. Advanced Adrenocortical Carcinoma: Current Perspectives on Medical Treatment.. Horm Metab Res. 2021. PMID: 33962475. Local full text: 33962475.md ↩ ↩2 ↩3 ↩4

  15. Immunotherapy failure in adrenocortical cancer: where next?. Endocr Connect. 2018. PMID: 30400026. Local full text: 30400026.md ↩ ↩2 ↩3

  16. Molecular Drivers of Potential Immunotherapy Failure in Adrenocortical Carcinoma.. J Oncol. 2019. PMID: 31057613. Local full text: 31057613.md ↩ ↩2

  17. Immunotherapy: new strategies for the treatment of adrenocortical carcinoma.. Horm Metab Res. 2003. PMID: 12931278. Local full text: 12931278.md ↩ ↩2

  18. Multiplatform computational analysis of mast cells in adrenocortical carcinoma tumor microenvironment.. Surgery. 2022. PMID: 34261605. Local full text: 34261605.md ↩ ↩2 ↩3

  19. Complete Radiological Response of Recurrent Metastatic Adrenocortical Carcinoma to Pembrolizumab and Mitotane.. Clin Med Insights Oncol. 2021. PMID: 33889043. Local full text: 33889043.md ↩ ↩2 ↩3

  20. Pembrolizumab in paediatric patients with advanced melanoma or a PD-L1-positive, advanced, relapsed, or refractory solid tumour or lymphoma (KEYNOTE-051): interim analysis of an open-label, single-arm, phase 1-2 trial.. Lancet Oncol. 2020. PMID: 31812554. Local full text: 31812554.md ↩ ↩2 ↩3

  21. Rare but not forgotten: Therapeutic advancements for rare childhood cancers.. Mol Ther Oncol. 2025. PMID: 41322192. Local full text: 41322192.md ↩ ↩2 ↩3

  22. Discussion.. Surgery. 2019. PMID: 30527004. Local full text: 30527004.md ↩ ↩2

  23. Pembrolizumab for metastatic adrenocortical carcinoma with high mutational burden: Two case reports.. Medicine (Baltimore). 2018. PMID: 30593126. Local full text: 30593126.md ↩ ↩2

  24. [Clinical Investigation of Immune Checkpoint Inhibitor-Associated Kidney Injury in Patients with Urologic Cancers].. Hinyokika Kiyo. 2022. PMID: 35924703. Local full text: 35924703.md ↩ ↩2

  25. Combined lenvatinib and pembrolizumab as salvage therapy in advanced adrenal cortical carcinoma.. J Immunother Cancer. 2020. PMID: 32737143. Local full text: 32737143.md ↩ ↩2

  26. High-Dose Rate Brachytherapy Combined with PD-1 Blockade as a Treatment for Metastatic Adrenocortical Carcinoma - A Single Center Case Series.. Horm Metab Res. 2024. PMID: 37748508. Local full text: 37748508.md ↩ ↩2

  27. Phase II basket trial of Dual Anti-CTLA-4 and Anti-PD-1 blockade in Rare Tumors (DART) SWOG S1609: adrenocortical carcinoma cohort.. J Immunother Cancer. 2024. PMID: 39067873. Local full text: 39067873.md ↩ ↩2

  28. Camrelizumab plus apatinib for previously treated advanced adrenocortical carcinoma: a single-arm phase 2 trial.. Nat Commun. 2024. PMID: 39609453. Local full text: 39609453.md ↩ ↩2

  29. Recent Advances in IL-13Rα2-Directed Cancer Immunotherapy.. Front Immunol. 2022. PMID: 35464460. Local full text: 35464460.md ↩

  30. EDP-M plus sintilimab in the treatment of adrenocortical carcinoma: a case report.. Transl Cancer Res. 2022. PMID: 35836542. Local full text: 35836542.md ↩

  31. Case report: Remarkable response to a novel combination of mitotane, etoposide, paraplatin, and sintilimab in a patient with metastatic adrenocortical carcinoma.. Front Endocrinol (Lausanne). 2023. PMID: 37745701. Local full text: 37745701.md ↩

  32. Etoposide, cisplatin, and sintilimab combined with anlotinib in successful treatment of adrenocortical carcinoma with lung metastasis: a case report.. Front Oncol. 2024. PMID: 39220648. Local full text: 39220648.md ↩

  33. Chemo-immunotherapy with cisplatin and nivolumab as second-line approach in metastatic adrenocortical carcinoma.. Eur J Endocrinol. 2026. PMID: 41531268. Local full text: 41531268.md ↩

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