Pulmonary Sarcomatoid Carcinoma Research Articles

Advances in immunotherapy for pulmonary sarcomatoid carcinoma

Pulmonary sarcomatoid carcinoma (PSC) is a rare, poorly differentiated non-small cell lung cancer (NSCLC) that contains sarcomatoid components or sarcomatoid differentiation, and accounts for less than 1% of all lung tumors. Compared to other types of NSCLC, PSC has more invasive biological behavior, is prone to metastasis, and has a higher recurrence rate after early surgery. Its greater resistance to traditional treatments leads to a poorer prognosis compared to other NSCLCs. Immunotherapy offers the possibility of long-term survival for PSC patients.

Integrative genomic and transcriptomic profiling of pulmonary sarcomatoid carcinoma identifies molecular subtypes associated with distinct immune features and clinical outcomes

AbstractBackgroundPulmonary sarcomatoid carcinoma (PSC) is a rare and aggressive subtype of non‐small cell lung cancer (NSCLC), characterized by the presence of epithelial and sarcoma‐like components. The molecular and immune landscape of PSC has not been well defined.MethodsMultiomics profiling of 21 pairs of PSCs with matched normal lung tissues was performed through targeted high‐depth DNA panel, whole‐exome, and RNA sequencing. We describe molecular and immune features that define subgroups of PSC with disparate genomic and immunogenic features as well as distinct clinical outcomes.ResultsIn total, 27 canonical cancer gene mutations were identified, with TP53 the most frequently mutated gene, followed by KRAS. Interestingly, most TP53 and KRAS mutations were earlier genomic events mapped to the trunks of the tumors, suggesting branching evolution in most PSC tumors. We identified two distinct molecular subtypes of PSC, driven primarily by immune infiltration and signaling. The Immune High (IM‐H) subtype was associated with superior survival, highlighting the impact of immune infiltration on the biological and clinical features of localized PSCs.ConclusionsWe provided detailed insight into the mutational landscape of PSC and identified two molecular subtypes associated with prognosis. IM‐H tumors were associated with favorable recurrence‐free survival and overall survival, highlighting the importance of tumor immune infiltration in the biological and clinical features of PSCs.

Clinical characteristics and prognosis analysis of pulmonary sarcomatoid carcinoma

Objective: To summarize the clinical characteristics, treatment, and outcomes of patients with pulmonary sarcomatoid carcinoma (PSC) in order to improve clinicians' understanding of this disease. Methods: The clinical data of patients diagnosed with PSC in our hospital from January 1, 2015 to November 30, 2023 were retrospectively analyzed. According to whether radical resection was performed, the patients were divided into resectable group and unresectable group. The characteristics and treatments of PSC in different groups were compared. The survival curves were drawn by Kaplan-Meier method to compare the prognosis of different groups of patients. Results: A total of 43 PSC patients were included, including 32 males, with an average age of (62.79±9.59) years, and 31 smokers. Peripheral-type tumors were more common, with imaging showing predominantly solid soft tissue masses, and the maximum diameter of the tumor was more than 5 cm in 14 patients. Among the 23 patients who underwent NGS gene testing, the KRAS mutation rate was 43.5%, the TP53 mutation rate was 30.4%, and the MET mutation rate was 8.7%, all of which were MET-14 exon skipping mutations. PD-L1 expression was detected in 13 patients, 10 of whom showed high expression. The median overall survival (mOS) of the 43 patients with PSC was 24.6 months (13.0-52.7 months). Among them, 22 patients underwent radical lobectomy plus mediastinal lymph node dissection, 13 patients had postoperative recurrence, and 7 patients died during follow-up. The median disease-free survival (mDFS) was 12.3 months, the mOS was not achieved and the 1-year OS rate was 77.3 %. Twenty-one patients had unresectable locally advanced or advanced stage, and 15 patients died. The mDFS was 2.5 months, the mOS was 6.2 months, and the 1-year OS rate was 42.9 %. Seventeen patients received immunotherapy, and 1 patient received targeted therapy with the MET inhibitor glumetinib. Conclusions: PSC has a higher incidence in the elderly, smokers, and males, is highly malignant and has a poor prognosis. Based on its molecular biological characteristics, PD-L1 expression and tumor molecular detection can be performed to guide treatment options.

Effect of histological subtype on the efficacy of perioperative chemotherapy in pulmonary sarcomatoid carcinoma: a retrospective study based on SEER population.

The efficacy of perioperative chemotherapy (PC) in pulmonary sarcomatoid carcinoma (PSC) is controversial. We conducted this study to investigate the effect of different histological subtypes on the efficacy of PC in PSC patients. Clinicopathological data of 811 PSC patients of different histological subtypes were collected from the Surveillance, Epidemiology, and End Results (SEER) database. Kaplan-Meier method and log-rank test were used to evaluate the effects of PC on the overall survival (OS) and cancer-specific survival (CSS) in different subtypes of PSC patients. Propensity score matching (PSM) was used to reduce potential confounding effects. Subgroup analyses were conducted to further investigate the efficacy of PC in patients with different characteristics. A total of 210 (25.89%) enrolled PSC patients received PC. PC was not associated with OS or CSS benefit in pleomorphic carcinoma, giant cell carcinoma, or spindle cell carcinoma patients, neither before nor after matching. But survival benefit of PC was observed in carcinosarcoma patients both before (5-year OS: 48.79% vs. 38.75%, P=0.01) and after (5-year OS: 51.29% vs. 17.54%, P=0.003) matching. Subgroup analyses showed that in patients whose tumor larger than 4 cm, PC was still associated with improved survival in carcinosarcoma, but not in the other histological subtypes of PSC. The efficacy of PC varies between different subtypes of PSC. Survival benefit of PC was only observed in carcinosarcoma patients, but not in pleomorphic carcinoma, giant cell carcinoma, or spindle cell carcinoma patients. Histological subtype should be considered when treating PSC patients with PC.

Extended radical resection and chest wall reconstruction for a pulmonary sarcomatoid carcinoma: a case report

BackgroundPulmonary sarcomatoid carcinoma (PSC) is a rare and highly malignant type of non-small cell lung cancer (NSCLC), for which the treatment of choice is surgery. For peripheral PSC growing outward and invading the chest wall, a complete resection of the affected lung lobes and the invaded chest wall can improve long-term prognosis. However, when the extent of the resected chest wall is large, reconstruction is often required to reduce the risk of postoperative complications. Here, we present a case of PSC invading the chest wall treated with successful extended radical resection for lung cancer and chest wall reconstruction.Case presentationA 58-year-old male patient with a nodule in the right upper lobe that had been identified on physical examination 2 years before presentation presented to our hospital with a recent cough, expectoration, and chest pain. Imaging revealed a mass in the right upper lobe that had invaded the chest wall. Preoperative puncture pathology revealed poorly differentiated NSCLC. We performed extended radical resection for lung cancer under open surgery and reconstructed the chest wall using stainless steel wire and polypropylene meshes. The procedure was uneventful, and the patient was discharged 7 days postoperatively. Furthermore, the final pathology revealed PSC.ConclusionsThis case underscores the feasibility of surgical R0 resection in patients with PSC with chest wall invasion and no lymph node metastasis, potentially enhancing long-term outcomes. The novel aspect of this case lies in the individualized chest wall reconstruction for a large defect, using cost-effective materials that offered satisfactory structural support and postoperative recovery, thereby providing a valuable reference for similar future surgical interventions.

Biological characteristics and clinical treatment of pulmonary sarcomatoid carcinoma: a narrative review.

Pulmonary sarcomatoid carcinoma (PSC) is a subset of non-small cell lung cancer (NSCLC) with highly malignant, aggressive, and heterogeneous features. Patients with this disease account for approximately 0.1-0.4% of lung cancer cases. The absence of comprehensive summaries on the basic biology and clinical treatments for PSC means there is limited systematic awareness and understanding of this rare disease. This paper provides an overview of the biological characteristics of PSC and systematically summarizes various treatment strategies available for patients with this disease. For this narrative review, we have searched literature related to the basic biology and clinical treatment approaches of PSC by searching the PubMed database for articles published from July 16, 1990 to August 29, 2023. The following keywords were used: "pulmonary sarcomatoid carcinoma", "genetic mutations", "immune microenvironment", "hypoxia", "angiogenesis", "overall survival", "surgery", "radiotherapy", "chemotherapy", and "immune checkpoint inhibitors". Classical PSC comprises epithelial and sarcomatoid components, with most studies suggesting a common origin. PSC exhibits a higher tumor mutational burden (TMB) and mutation frequency than other types of NSCLC. The tumor microenvironment (TME) of PSC is characterized by hypoxia, hypermetabolism, elevated programmed cell death protein 1/programmed cell death-ligand 1 expression, and high immune cell infiltration. Treatment strategies for advanced PSC are mainly based on traditional NSCLC treatments, but PSC exhibits resistance to chemotherapy and radiotherapy. The advancement of genome sequencing has introduced targeted therapies as an option for mutation-positive PSC cases. Moreover, due to the characteristics of the immune microenvironment of PSC, many patients positively respond to immunotherapy, demonstrating its potential for the management of PSC. Although several studies have examined and assessed the TME of PSC, these are limited in quantity and quality, presenting challenges for research into the clinical treatment strategies for PSC. With the emergence of new technologies and the advancement of clinical research, for example, savolitinib's clinical study for MET exon 14 skipping mutations positive PSC patients have shown promising outcomes, more in-depth studies on PSC are eagerly anticipated.

18F-FDG PET/CT imaging in pulmonary sarcomatoid carcinoma.

Pulmonary sarcomatoid carcinoma (PSC) is a rare highly aggressive and poorly differentiated non-small cell carcinoma, and little is known about the information on the usefulness of 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT). We investigated the clinical and 18F-FDG PET/CT features of PSC. We retrospectively analyzed 25 consecutive PSC patients who had undergone 18F-FDG PET/CT. Demographic data, PET/CT findings before treatment, pathological features, and prognosis in these patients were investigated to define correlates between maximal standard uptake value (SUVmax) and clinicopathological parameters. From March 2017 to January 2023, twenty-five eligible patients with PSC were identified. There were 23 (92%) men, aged 68.5 ± 8.5 (range 56-90) years. Eighteen (72%) patients had a frequent smoking history. The mean size of PSCs was 59.3 ± 18.6 (range 29-97) mm, and 23 (92%) PSCs were Stage IV tumors. 20 (80%) lesions were located in the upper lung and 19 (76%) cases belonged to the peripheral type. Necrotic foci appeared in 21(84%) tumors. 11 (44%) PSCs invaded the pleura. All PSCs were FDG avid, and the mean of SUVmax was 11.8 ± 5.3 (range 4.8-25.5). Metastases were found on PET/CT in 24(96%) patients. The SUVmax of the lesions ≥ 5cm was higher than that of the lesions < 5cm (p=0.004), and the SUVmax of lesions with TTF-1 expression was higher than those of lesions without TTF-1 expression (p=0.009). All of the 25 primary lesions were considered malignant and confirmative, probable, and possible diagnosis of PSC was made in 2 (8%), 4 (16%), and 5(20%) patients, respectively on PET/CT. PSC was not considered in 14 (56%) patients, in PET/CT. The survival of patients with surgery didn't demonstrate a significantly good prognosis as compared with those without surgery (p=0.675). All PSCs had obvious FDG avidity. Although imaging diagnosis is still difficult, combined clinical and imaging features more than 40% of primary lesions were considered for the possibility of PSC in our group. Early histopathological diagnosis is necessary to help develop a reasonable regimen.

Pulmonary Sarcomatoid Carcinoma Presenting as Massive Pleural Effusion

Pulmonary Sarcomatoid Carcinoma Presenting as Massive Pleural Effusion

Role of immunotherapy in pulmonary sarcomatoid carcinoma: review of current approaches and related biomarkers.

Pulmonary sarcomatoid carcinoma (PSC), a rare subtype of non-small cell lung cancer, is highly malignant and has a poor prognosis. Treatments for PSC are presently limited. Traditional treatments provide fewer benefits to PSC patients and are associated with early recurrence and metastasis. Surgical intervention is the preferred option for early-stage PSC patients, whereas chemotherapy, radiotherapy, immunotherapy, and other targeted therapies are recommended for advanced PSC patients. Targeted therapy is only effective in a small number of PSC patients. The initial efficacy of immune checkpoint inhibitors has been acceptable in patients with advanced PSC; therefore, much attention on related biomarkers has been sought. This article aimed to review the research progress of PSC immunotherapy and related diagnostic and prognostic biomarkers in recent years.

73P Camrelizumab plus famitinib as first-line treatment in patient with locally advanced or metastatic pulmonary sarcomatoid carcinomas (CAPSTONE): A multi-center, single-arm, phase II study

73P Camrelizumab plus famitinib as first-line treatment in patient with locally advanced or metastatic pulmonary sarcomatoid carcinomas (CAPSTONE): A multi-center, single-arm, phase II study

Effectiveness and Safety of Immune Checkpoint Inhibitors Alone or in Combination With Chemotherapy in Pulmonary Sarcomatoid Carcinoma

IntroductionPulmonary sarcomatoid carcinoma (PSC) is a rare subtype of lung cancer associated with poor prognosis and resistance to conventional chemotherapy. Immune checkpoint inhibitors (ICIs), alone or in combination with chemotherapy, were found to have clinical benefits in PSC in recent studies. Nevertheless, because these studies included a small number of patients owing to disease rarity, larger studies are needed to evaluate the effectiveness and safety of ICI-based therapy for PSC. MethodsThis multicenter retrospective study evaluated patients with ICI-naive advanced or metastatic PSC who were treated with ICI-based therapy at 25 hospitals in Japan. ResultsA total of 124 patients were evaluated. The overall response rate, median progression-free survival (PFS), and median overall survival (OS) were 59.0%, 10.5 months, and 32.8 months, respectively. The PFS and OS rates at 24 months were 35.3% and 51.5%, respectively. Programmed death-ligand 1 expression, concomitant chemotherapy, and the treatment line were not significantly associated with PFS or OS. Immune-related adverse events (irAEs) were observed in 70 patients (56.5%), including 30 (24.2%) with grade 3 to 5 events. Patients with mild irAEs (grades 1–2) had longer PFS and OS than did those with severe (grades 3–5) or no irAEs. In a multivariate analysis, any-grade irAEs and the absence of liver metastases were independently associated with PFS, whereas any-grade irAEs and Eastern Cooperative Oncology Group performance status less than or equal to 1 were independently associated with OS. ConclusionsICI-based therapy was found to have promising effectiveness in patients with advanced or metastatic PSC, regardless of programmed death-ligand 1 expression, concomitant chemotherapy, or treatment line.

Genomic Landscape of Pulmonary Sarcomatoid Carcinoma.

Pulmonary sarcomatoid carcinoma (PSC) is a rare aggressive subtype of non-small cell lung cancer (NSCLC) with limited therapeutic strategies. We attempted to elucidate the evolutionary trajectories of PSC using multiregional and longitudinal tumor samples. A total of 31 patients were enrolled in this study and 11 longitudinal samples were available from them. Using whole exome sequencing data, we analyzed the mutational signatures in both carcinomatous and sarcomatous areas in primary tumors of the 31 patients and longitudinal samples obtained from 11 patients. Furthermore, digital droplet polymerase chain reaction (ddPCR), and programmed death-ligand 1 (PD-L1) immunohistochemistry using the Ventana SP263 assay were performed. TP53 was identified as the most frequently altered gene in the primary (74%) and metastatic (73%) samples. MET exon 14 skipping mutations, confirmed by ddPCR, and TP53 mutations were mutually exclusive; whereas, MET exon 14 skipping mutations frequently co-occurred with MDM2 amplification. Metastatic tumors showed dissimilar genetic profiles from either primary component. During metastasis, the signatures of APOBEC decreased in metastatic lesions compared with that in primary lesions. PSC showed higher MET and KEAP1 mutations and stronger PD-L1 protein expression compared with that recorded in other NSCLCs. Decreased APOBEC signatures and subclonal diversity were detected during malignant progression in PSC. Frequent MET mutations and strong PD-L1 expression distinguished PSC from other NSCLCs. The aggressiveness and therapeutic difficulties of PSC were possibly attributable to profound intratumoral and intertumoral genetic diversity. Next-generation sequencing could suggest the appropriate treatment strategy for PSC.

Recurrence of pulmonary sarcomatoid carcinoma presenting as haemothorax: a case report

Pulmonary sarcomatoid carcinoma (PSC) is a rare and highly invasive malignant tumour. It has similar clinical manifestations and imaging features to ordinary lung cancer. This current case report describes a 65-year-old male patient who had a mass in the apicoposterior segment of the left upper lobe with haemoptysis. Imaging studies revealed a central parenchymal mass surrounded by areas of ground-glass opacity, strongly indicating the presence of a pulmonary malignancy. Intraoperatively, the tumour was discovered to have invaded the chest wall and exhibited a significant propensity for bleeding. Consequently, a left upper lobe resection accompanied by extensive pleural debridement were performed. Subsequent postoperative histopathological examination confirmed the diagnosis of PSC. Unfortunately, 1 month after the surgery, the patient presented with left-sided haemothorax. Despite employing various haemostatic interventions, the patient eventually succumbed to haemorrhagic shock. This study provides a treatment strategy reference for patients with PSC presenting as haemothorax.

P2.22-01 Single-nuclei RNA Sequencing Reveals Heterogeneity and Malignancy in Pulmonary Sarcomatoid Carcinoma

P2.22-01 Single-nuclei RNA Sequencing Reveals Heterogeneity and Malignancy in Pulmonary Sarcomatoid Carcinoma

Survival analysis and nomogram for pulmonary sarcomatoid carcinoma: an SEER analysis and external validation

ObjectiveUncommon and particularly deadly, pulmonary sarcomatoid carcinoma (PSC) is an aggressive type of lung cancer. This research aimed to create a risk categorisation and nomogram to forecast the overall survival...

A rare case of pulmonary endobronchial spindle cell carcinoma with liver metastasis along with concurrent cutaneous neurofibroma

Spindle cell carcinoma is one of the histological variants of pulmonary sarcomatoid carcinoma. It is a rare, poorly differentiated, highly malignant, and rapidly growing non-small cell carcinoma of the lung. Information about pulmonary spindle carcinoma incidence, clinical features, and immunohistochemical markers are not well known compared to other variants of sarcomatoid carcinoma. Most sarcomatoid carcinoma features mimic non-small cell carcinoma, so it is easily misdiagnosed. Cutaneous neurofibroma with primary lung spindle cell carcinoma has not been reported in any previous literature. Here we report a rare endobronchial sarcoma with liver metastasis and concurrent skin neurofibroma.

Clinical outcomes of radical radiotherapy for pulmonary sarcomatoid carcinoma.

Pulmonary sarcomatoid carcinoma (PSC) is recognized for its aggressiveness and poor prognosis. The role of radical radiotherapy in PSC remains uncertain due to its scarcity and limited data. In the absence of an effective systemic agent, this study aims to explore the possibility of cure and to investigate potential prognostic factors and treatment outcomes. From January 2005 to December 2021, 149 PSC patients were identified. Among 62 patients who received radiotherapy for lung lesions, 25 who underwent palliative radiotherapy and 16 who underwent surgery were excluded. The median patient age was 71 years. The majority were male, and 17 patients (81.0%) were diagnosed at an advanced stage. After radical radiotherapy, distant metastasis (47.6%) was the most common site of failure, while the local recurrence rate was quite low (9.5%). Eventually, five patients (26.3%) demonstrated either a partial response or complete remission, including three complete remissions with durable responses. The median progression-free survival (PFS) and overall survival were 4.6 months and 7.9 months, respectively. Univariate and multivariate analyses revealed that a tumor size >5 cm was associated with a worse prognosis (p = 0.045), while a radiation dose >58 GyEQD2 was significantly associated with better PFS (p = 0.038). This study demonstrates clinical outcomes after radical radiotherapy in managing PSC, suggesting tumor size and radiation dose could be a predictor of a systemic response. Given the known bad prognosis but complete remission could be achieved in certain subgroups, future research should explore the potential strategies using radical radiotherapy for this challenging patient population.

Preoperative CT findings and prognosis of pulmonary sarcomatoid carcinoma: comparison with conventional NSCLC of similar tumor size

BackgroundPulmonary sarcomatoid carcinoma (PSC) is a rare subtype of non-small cell lung cancer (NSCLC) but differs in terms of treatment strategies compared with conventional-NSCLC (c-NSCLC). However, preoperative CT differentiation between PSC and c-NSCLC remains a challenge. This study aimed to explore the CT findings and prognosis of PSC compared with c-NSCLC of similar tumor size.MethodsClinical data and CT findings of 31 patients with PSC and 87 patients with c-NSCLC were retrospectively analyzed. Clinical data included sex, age, and smoking history. CT findings included tumor size, tumor location, calcification, vacuole/cavity, pleural invasion, mean CT value, and low-attenuation area (LAA) ratio. Kaplan‒Meier curves and log-rank tests were used for survival analysis. A Cox regression model was constructed to evaluate prognostic risk factors associated with overall survival (OS). The Spearman correlation among clinicoradiological outcomes were analyzed.ResultsThe mean tumor size of PSC and c-NSCLC were both 5.1 cm. The median survival times of PSC and c-NSCLC were 8 months and 34 months, respectively (P < 0.001). Calcification and vacuoles/cavities were rarely present in PSC. Pleural invasion occurred in both PSC and c-NSCLC (P = 0.285). The mean CT values of PSC and c-NSCLC on plain scan (PS), arterial phase (AP), and venous phase (VP) were 30.48 ± 1.59 vs. 36.25 ± 0.64 Hu (P = 0.002), 43.26 ± 2.96 vs. 58.71 ± 1.65 Hu (P < 0.001) and 50.26 ± 3.28 vs. 64.24 ± 1.86 Hu (P < 0.001), the AUCs were 0.685, 0.757 and 0.710, respectively. Compared to c-NSCLC, PSC had a larger LAA ratio, and the AUC was 0.802, with an optimal cutoff value of 20.6%, and the sensitivity and specificity were 0.645 and 0.862, respectively. Combined with the mean CT value and LAA ratio, AP + VP + LAA yielded the largest AUC of 0.826. The LAA ratio were not independent risk factors for PSC in this study. LAA ratio was negatively correlated with PS (r = -0.29), AP (r = -0.58), and VP (r = -0.66). LAA showed a weak positive correlation with tumor size(r = 0.27).ConclusionsPSC has a poorer prognosis than c-NSCLC of similar tumor size. The mean CT value and LAA ratio contributes to preoperative CT differentiation of PSC and c-NSCLC.

The use of Anatomage table for metastases to the oral region

IntroductionThe Anatomage Table is a modern device characterized by virtual reality functionality that may be used to enhance the teaching of human anatomy to medical and allied health students. The purpose of the present study was to use the virtual dissection table (3D Anatomage) as an additional tool for education and information in cases of metastases to the oral region. Materials and methodsThe hospital database of Vercelli Hospital, Vercelli, Italy, was searched for metastases to the oral region. DICOM data of Computed tomography scans were retrieved and uploaded in the Anatomage Table device. The workstation was used to obtain images of the body surface, “dissection cuts,” or “vascular reconstructions,” modifying the filters. ResultsThe reconstructed images of three cases of metastases to the oral region from pulmonary adenocarcinoma, pulmonary sarcomatoid carcinoma, and breast ductal infiltrative adenocarcinoma were produced and presented. Different filters were used. DiscussionThe use of Anatomage Table, a touch interactive anatomy virtual dissection table used in anatomy education, thanks to the application of different filters, may represent a promising resource both for patients and students.

Novel model for cancer-specific survival of patients with pulmonary sarcomatoid carcinoma: A population-based analysis and external validation

Background/ObjectiveWe aimed to develop a comprehensive and effective nomogram for predicting cancer-specific survival (CSS) in patients with pulmonary sarcomatoid carcinoma (PSC). MethodsData for patients diagnosed with PSC between 2004 and 2018 from the Surveillance, Epidemiology, and End Results database were retrospectively collected and randomly divided into training and internal validation sets. We then retrospectively recruited patients diagnosed with PSC to construct an external validation cohort from the Southwest Hospital. A prognostic nomogram for CSS was established using independent prognostic factors that were screened from the multivariate Cox regression analysis. The performance of the nomogram was evaluated using area under the receiver operating characteristic (ROC) curves, Harrell's concordance index (C-index), calibration diagrams, and decision curve analysis (DCA). The clinical value of the nomogram and tumor, nodes, and metastases (TNM) staging system was compared using the C-index and net reclassification index (NRI). ResultsOverall, 1356 patients with PSC were enrolled, including 876, 377, and 103 in the training, internal validation, and external validation sets, respectively. The C-index and ROC curves, calibration, and DCA demonstrated satisfactory nomogram performance for CSS in patients with PSC. In addition, the C-index and NRI of the nomogram suggested a significantly higher nomogram value than that of the TNM staging system. Subsequently, a web-based predictor was developed to help clinicians obtain this model easily. ConclusionsThe prognostic nomogram developed in this study can conveniently and precisely estimate the prognosis of patients with PSC and individualize treatment, thereby assisting clinicians in their shared decision-making with patients.