Neoadjuvant therapy has long been established as a critical component in the management of locally advanced esophageal cancer, traditionally involving chemotherapy or chemoradiotherapy to downstage tumors prior to surgery (5). In this report, we present a novel approach using neoadjuvant immunotherapy in a patient with esophageal squamous cell carcinoma (ESCC) complicated by a right aortic arch (RAA), an exceedingly rare vascular anomaly. The use of immune checkpoint inhibitors (ICIs), specifically anti-PD-1 agents such as sintilimab, has demonstrated considerable efficacy in various malignancies, including esophageal cancer (6). However, its application in cases complicated by anatomical anomalies like RAA has not been previously reported.

The mechanism by which immunotherapy aids in such complex anatomical cases may be related to its ability to enhance T-cell-mediated cytotoxicity against tumor cells, promoting both direct tumor regression and indirect reshaping of the tumor microenvironment (7). This reduction in tumor bulk not only decreases the physical space occupied by the malignancy but also potentially reduces inflammation and fibrosis around critical vascular structures, making them more accessible for surgical manipulation. In cases like ours, where radiotherapy could have induced fibrotic changes complicating surgical resection, the anti-fibrotic advantage of immunotherapy may represent a key factor in improving operative outcomes (8).

In this case, immunotherapy provided a safer alternative to chemoradiotherapy, particularly given the risk of fibrosis associated with radiotherapy, which could exacerbate surgical complexity. By enhancing the immune response against the tumor, immunotherapy allowed for significant tumor shrinkage without compromising the anatomical structures crucial for surgery. After four cycles of sintilimab, the tumor size was notably reduced, enabling a safer surgical approach in a challenging anatomical environment. This underscores the potential of immunotherapy not only in reducing tumor burden but also in minimizing perioperative complications in patients with complex vascular anomalies.

Following a systematic search of the PubMed database using the keywords “right aortic arch” and “esophageal cancer,” we identified a range of cases detailing the management of this rare condition. A review of the literature, as summarized in Table 1 (9–22), shows that neoadjuvant chemoradiotherapy has been widely used in managing esophageal cancer, but immunotherapy remains underexplored in this context. This case suggests that ICIs may offer a promising neoadjuvant strategy in anatomically complex cases, providing similar benefits to traditional approaches but without the added risks associated with radiation-induced tissue changes.

Preoperative 3D reconstruction is an emerging tool in the surgical management of esophageal cancer, particularly in patients with anatomical anomalies such as RAA. In this case, 3D reconstruction provided critical insights into the spatial relationships between the tumor, the right aortic arch, and surrounding vascular structures, including the left subclavian artery. By allowing detailed visualization of the aberrant vasculature, 3D modeling enabled the surgical team to meticulously plan the operation, anticipate potential complications, and navigate around high-risk areas intraoperatively.

The ability to map out the patient’s vascular anatomy with such precision proved invaluable, especially in this case, where the aberrant vascular structures posed a significant risk for intraoperative vascular injury. Traditional imaging modalities, such as CT and MRI, can provide detailed cross-sectional images but lack the spatial integration needed to fully appreciate complex three-dimensional relationships. The use of 3D reconstruction in this case allowed for a more comprehensive understanding of the patient’s anatomy and significantly reduced the risks associated with the surgical resection.

Our review of the literature highlights the limited use of 3D reconstruction in cases of esophageal cancer complicated by RAA. Most previous cases relied on traditional imaging techniques, with few reports utilizing 3D technology to aid in surgical planning. This suggests an underutilization of a powerful tool that can enhance surgical safety and efficacy, particularly in anatomically challenging cases.

The table summarizing previous reports on esophageal cancer with RAA provides important insights into the current treatment landscape. It highlights a clear trend toward left thoracotomy as the preferred surgical approach due to the anatomical configuration of RAA, which complicates right-sided approaches. However, the table also reveals significant variability in the use of neoadjuvant therapies, with most cases relying on traditional chemoradiotherapy and none incorporating immunotherapy or 3D reconstruction.

This analysis underscores the novelty of our approach. By combining neoadjuvant immunotherapy with advanced 3D reconstruction, we were able to safely and effectively manage a highly complex case that would have otherwise posed significant challenges. The literature review further suggests that there is a need for greater exploration of these modern modalities in the treatment of esophageal cancer, particularly in patients with vascular anomalies. The use of 3D reconstruction in preoperative planning, combined with the immunomodulatory effects of checkpoint inhibitors, may represent a significant advancement in the treatment of these complex cases.

This case report is, to our knowledge, the first to combine neoadjuvant immunotherapy with 3D reconstruction in the treatment of esophageal cancer with RAA. The successful outcome in this case demonstrates the feasibility and potential benefits of this approach. Immunotherapy, by reducing tumor size without increasing surgical complexity, allowed for a safer and more effective resection. Meanwhile, 3D reconstruction provided critical preoperative insights that minimized intraoperative risks and improved surgical precision.

To further substantiate these findings, prospective studies examining the specific effects of immunotherapy on the surgical feasibility of anatomically complex cases are crucial. Such studies could assess perioperative outcomes, complication rates, and long-term survival compared to conventional chemoradiotherapy approaches. Additionally, exploring the biological and histopathological changes induced by immune checkpoint inhibitors in the tumor microenvironment of patients with vascular anomalies could offer deeper mechanistic insights. Multi-center clinical trials would also be beneficial in establishing standardized protocols for combining neoadjuvant immunotherapy with advanced surgical techniques like 3D reconstruction.

Future studies, including multi-institutional trials and larger case series, are needed to validate the effectiveness of this strategy and to explore its broader applicability. Additionally, the integration of other advanced technologies, such as artificial intelligence and augmented reality into 3D reconstruction, could further enhance its utility in complex surgical cases.