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Background Prediction of open–close and long-term outcome is challenging in patients undergoing cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Prognostic scores often include factors not known at baseline. Therefore, we aimed to analyze whether patterns of preoperative tumor markers could aid in prediction of open–close surgery and outcome in patients with pseudomyxoma peritonei (PMP) or colorectal peritoneal metastases (PM). Patients and Methods All patients accepted for CRS and HIPEC for PMP or colorectal PM at Uppsala University Hospital in 2013–2021 were included. The tumor markers CEA, CA19-9, CA125, CA72-4, and CA15-3 were clustered using the k -means algorithm; the average silhouette width determined the optimal numbers of clusters. Results Clustering of patients with PMP ( n = 138) and colorectal PM ( n = 213) resulted in two clusters each. PMPCluster-1 ( n = 124) had a 5-year overall survival (OS) of 77% (95% CI 69−85%), 11 (9%) open–close surgeries, and a median peritoneal cancer index (PCI) of 17. PMPCluster-2 ( n = 14) patients had poorer prognosis (36%, 95% CI 15–85%, p = 0.003), more often open–close ( n = 6, 43%, p = 0.002), and higher PCI (median 36, p < 0.001). ColorectalCluster-1 ( n = 191) had a 5-year OS of 28% (95% CI 21–37%), median PCI of 11, and 38 (20%) open–close surgeries. ColorectalCluster-2 ( n = 22) had poorer prognosis (10%, 95% CI 3–36%, p = 0.02), higher PCI (median 26, p < 0.001), higher completeness of cytoreduction score ( p = 0.005), but no difference in open–close surgery ( n = 6, 27%, p = 0.411). PMPCluster-2 and ColorectalCluster-2 were characterized by markedly elevated tumor markers. Open–close surgery was unusual in cases of normal CA72-4. Conclusions Elevation of several preoperative tumor markers is associated with poor prognosis and increased risk of open–close. CA72-4 deserves increased attention.

Pseudomyxoma peritonei (PMP) is a rare condition characterized by diffuse spread of mucinous tumors within the peritoneal cavity. Traditional treatment modalities, such as cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) are challenging in cases of recurrent disease, owing to anatomical complexities and increasing morbidity and mortality risk. BromAc has emerged as a novel, targeted therapy for PMP with evidence for intra-tumoral administration to break down mucin deposits. We present a 70-year-old female with confirmed diagnosis of symptomatic appendiceal PMP situated behind the stomach, refractory to prior CRS and HIPEC. Transhepatic intra-tumor injection of BromAc was performed, guided by imaging, with catheter placement into the posterior gastric mucinous tumor. The procedure was well-tolerated, and post-treatment imaging revealed a significant 40% reduction in tumor burden. The patient had fever on cycle days two and three, which self-resolved and septic screen performed was negative. Following BromAc administration, the patient demonstrated improvement in symptoms and quality of life. This case highlights the potential efficacy and safety of transhepatic administration of BromAc for the treatment of recurrent PMP behind the stomach. The targeted delivery of BromAc directly into a mucinous tumor via the transhepatic route offers a minimally invasive alternative for cases where traditional surgical interventions pose challenges. However, further research and clinical trials are warranted to validate the broader applicability of this novel approach, assess long-term outcomes, and optimize procedural parameters for enhanced therapeutic outcomes in PMP treatment.

Background Pseudomyxoma peritonei (PMP) is a rare peritoneal malignancy primarily originating from appendiceal mucinous neoplasms. Prognosis is influenced by histologic subtype, tumor markers, and the completeness of cytoreduction. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is the standard treatment. This study aims to identify key factors affecting long-term prognosis in appendiceal PMP patients and develop a dynamic nomogram for postoperative survival prediction. Methods This study retrospectively analyzed the clinical data of 153 patients with appendiceal-origin PMP confirmed by postoperative pathology from Jinan Central Hospital between October 2017 and December 2024. The primary endpoint was postoperative overall survival (OS). Patients were randomly divided into modeling (107 cases) and validation (46 cases) groups. Nonlinear relationships between continuous variables and survival time were assessed using restricted cubic splines (RCS) and ANOVA. Univariate Cox regression identified risk factors for postoperative OS, while Lasso regression generated a new variable, the LASSO-derived inflammation–nutrition score (LINS), from selected hematological indices. Multivariate Cox regression analysis identified independent prognostic factors, including histological type, preoperative peritoneal cancer index (PCI), CA125, LINS, and Completeness of Cytoreduction (CC) score ( P  < 0.05). A nomogram was developed to predict 1-, 3-, and 5-year postoperative OS. Model performance was validated using time-dependent ROC curves and calibration curves. Additionally, a dynamic nomogram was created using the Shiny application framework to predict survival probabilities in real-time. Results The 1-, 3-, and 5-year survival rates for the 153 patients were 82.54%, 66.00%, and 56.76%, respectively. The multivariate Cox regression identified LINS, CA125, preoperative PCI, histological type, and CC score as independent prognostic factors ( P  < 0.05). The nomogram demonstrated good predictive ability, with time-dependent ROC AUC values for the modeling group of 0.945, 0.823, and 0.736 at 1, 3, and 5 years. Calibration curves showed good agreement between predicted and observed survival. A dynamic survival prediction tool was developed and is accessible via a Shiny app ( https://lq1999.shinyapps.io/Dynamic/ ). Conclusion Histological type, preoperative PCI, CA125, LINS, and CC score are independent prognostic factors in appendiceal PMP. The developed nomogram offers accurate survival predictions and can be updated with new data for improved clinical decision-making.

Background PMP is a rare clinical syndrome characterized by the accumulation of mucinous material in the peritoneal cavity. The combination of CRS and HIPEC is the standard treatment, known to improve survival outcomes. However, follow-up adherence is critical for early detection of recurrence and extending survival. This study is the first to specifically investigate postoperative follow-up adherence in PMP patients treated with CRS and HIPEC. Aims To explore the socio-demographic and clinical determinants of follow-up adherence and its impact on survival in PMP patients treated with CRS and HIPEC. Methods This single-center, six-year retrospective study included 111 PMP patients who underwent CRS and HIPEC. Patients were categorized into compliant (control) and non-compliant (study) groups. Follow-up was initiated by the physician, with patients returning for regular visits as recommended. Kaplan-Meier analysis was used to evaluate survival outcomes, while univariate analysis identified factors influencing follow-up adherence. Results Among 111 patients, 32 died by August 2024, with 1-, 3-, and 5-year survival rates of 88%, 66%, and 53%, respectively. High PCI scores ( P  < 0.001) and poorer histopathological types ( P  = 0.002) were significant predictors of reduced survival. Non-compliance with follow-up showed a trend toward decreased survival ( P  = 0.07). Significant risk factors for non-compliance included lower ASA scores ( P  = 0.020), middle- and low-income levels ( P  = 0.034), and parental survival status ( P  = 0.003). Middle- and low-income patients also experienced longer delays in seeking medical advice and had fewer cases detected through routine health screenings ( P  = 0.019). Conclusions Follow-up compliance has a potential impact on survival outcomes in PMP patients treated with CRS and HIPEC. Socio-demographic factors significantly influence adherence, highlighting the need for tailored interventions to enhance patient management.

Background Pseudomyxoma peritonei (PMP) is a rare neoplastic disease that can occasionally present with pulmonary metastases as an even rarer manifestation. Although cytoreductive surgery represents the primary treatment option for PMP, surgical management of pulmonary metastases presents unique challenges owing to their mucinous nature. The aim of this case series was to present key considerations for the surgical management of PMP lung metastases, particularly regarding solid-appearing nodules that are impalpable during surgical resection, requiring more extensive resection owing to the risk of recurrence. Case presentation Between November 2013 and May 2023, we performed a total of 13 surgical procedures for pulmonary metastases in seven patients with PMP at our institution. During these procedures, 18 pulmonary lesions were resected. Some patients underwent multiple surgeries; multiple lesions were resected in a single procedure in certain cases. Notable characteristics of PMP pulmonary metastases, attributed to their mucinous dissemination pattern, included cases wherein subpleural solid nodules that would typically be palpable in patients with conventional solid tumour metastases were non-palpable and cases requiring right upper lobectomy following repeated surgical margin recurrence. Median follow-up results showed a progression-free survival of 20.0 months and overall survival of 40.3 months. Three of the seven patients experienced postoperative recurrence, with two patients having intrapulmonary metastatic recurrence involving low-grade tumours. Conclusions Surgical resection can be an effective treatment option for PMP pulmonary metastases; however, careful patient selection and appropriate surgical planning are essential. The unique characteristics of PMP metastases necessitate specific surgical strategies. Since computed tomography-apparent solid nodules may be non-palpable intraoperatively and surgical margin recurrence should be avoided, selecting surgical approaches that minimise unnecessary lung palpation, such as preoperative marking or anatomical lung resection, is crucial. The mechanisms of PMP pulmonary metastasis development and progression remain unclear, warranting further research into blood-borne dissemination patterns and optimal surgical techniques for non-palpable lesions.

Background and Aim Pseudomyxoma peritonei (PMP) is an unusual condition with unique behaviour caused by a mucinous neoplasm, usually arising from the appendix. The aim of this study was to evaluate the prevalence of genomic alterations in clinical specimens of PMP using a targeted assay and correlate the findings with clinical, pathological and outcome data. Sequencing data from 223 patients were analysed. Results The median follow‐up interval was 48 months. The primary neoplasm was appendiceal in 216 patients, ovarian in 4, urachal in 2 and renal in one. We confirmed common mutations in GNAS and KRAS (42% each) with significant co‐occurrence of variants in these genes. TP53 mutations were found in 8%. Other mutations were rare but included novel mutations in BAP1 and ERBB4. Of 17 patients with acellular peritoneal mucin, 6 (35%) were positive for DNA mutations. The non‐appendiceal cases generally showed a similar mutational landscape to the appendiceal lesions with GNAS and KRAS commonly mutated, although one urachal lesion showed multi‐hit TP53 mutation without variants in either GNAS or KRAS. Survival was significantly associated with the grade of the primary neoplasm, the grade of the peritoneal disease, the completeness of cytoreduction score and with mutation in either GNAS, KRAS or both. The hazard ratio (HR) associated with mutation in GNAS and/or KRAS was 1.87 (p = 0.004). Conclusions Survival outcome was more closely associated with the grade of the peritoneal disease than with the grade of the primary neoplasm. Our findings support the developing concept that mutational analysis may provide prognostic information in patients with PMP.

Pseudomyxoma peritonei (PMP) is a rare, progressive, slowly growing neoplastic condition which is poorly understood, with a 5-year progression-free survival rate as low as 48%. PMP is most commonly caused by appendiceal mucinous neoplasms (AMN), and understanding their genetic biology and pathogenicity may allow for the development of better novel systemic treatments to target key deleterious mutations and the implicated pathways. The primary aim of this systematic review was to identify the genetic profile of histologically confirmed human PMP or AMN samples. The secondary aim was to identify whether genetic marks could be used to predict patient survival. Ovid EMBASE, Ovid MEDLINE, PubMed, and Web of Science were searched to identify studies investigating the genetic profile of histologically-confirmed human PMP or AMN samples. We review findings of 46 studies totalling 2181 tumour samples. The most frequently identified somatic gene mutations in patients with PMP included KRAS (38–100%), GNAS (17–100%), and TP53 (5–23%); however, there were conflicting results of their effect on survival. Three studies identified molecular subtypes based on gene expression profiles classifying patients into oncogene-enriched, immune-enriched, and mixed molecular subtypes with prognostic value. This review summarises the current literature surrounding genetic aberrations in PMP and AMNs and their potential utility for targeted therapy. Given the recent advances in clinical trials to directly target KRAS and GNAS mutations in other cancers, we propose a rationale to explore these mutations in future pre-clinical studies in PMP with a view for a future clinical trial.

Background To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 199 (CA199) and CA125 in serum and ascites of appendiceal pseudomyxoma peritonei (PMP) patients relative to their diagnostic and predictive value. Methods The study comprised 183 patients with pathologically confirmed appendiceal PMP, enrolled from May 2012 to June 2020, in Aerospace Center Hospital. Serum and ascites tumor markers were obtained, and their diagnostic values were compared by receiver operating characteristic (ROC) curves. The prognostic factors of appendiceal PMP with different pathologic subgroups were calculated by univariate and multivariate Cox proportional hazard regression models. Results There were significant differences between the numbers of patients with positive CEA and CA199 in serum vs. ascites: p  = 0.034 in CEA and p  = 0.006 in CA199, respectively. The sensitivities with optimal cut-off values for ascites markers of CEA, CA199 and CA125 were 83.5%, 88.9% and 72.6%, respectively. CEA in ascites showed significant difference in the diagnosis of appendiceal PMP ( p  = 0.000); the areas under the ROC curves (AUROCs) and specificity were 0.725, 70.7%, respectively. Univariate analysis showed that the higher the ascites tumor markers, the poorer the survival ( p  = 0.014). Multivariate analysis indicated that completeness of cytoreduction (CCR), ascites CEA and pathological grade were independent risk factors for overall survival (OS). Conclusion CEA in ascites can be used to help specify the origin of PMP. Furthermore, elevation of ascites CEA, high pathological grade and incomplete cytoreduction predicted poor prognosis of appendiceal PMP.

The mechanisms by which neoplastic cells disseminate from the primary tumor to metastatic sites, so-called metastatic organotropism, remain poorly understood. Epithelial–mesenchymal transition (EMT) plays a role in cancer development and progression by converting static epithelial cells into the migratory and microenvironment-interacting mesenchymal cells, and by the modulation of chemoresistance and stemness of tumor cells. Several findings highlight that pathways involved in EMT and its reverse process (mesenchymal–epithelial transition, MET), now collectively called epithelial–mesenchymal plasticity (EMP), play a role in peritoneal metastases. So far, the relevance of factors linked to EMP in a unique peritoneal malignancy such as pseudomyxoma peritonei (PMP) has not been fully elucidated. In this review, we focus on the role of epithelial–mesenchymal dynamics in the metastatic process involving mucinous neoplastic dissemination in the peritoneum. In particular, we discuss the role of expression profiles and phenotypic transitions found in PMP in light of the recent concept of EMP. A better understanding of EMP-associated mechanisms driving peritoneal metastasis will help to provide a more targeted approach for PMP patients selected for locoregional interventions involving cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

Objectives This study aimed to identify the diagnostic accuracy of combined ultrasonography (US) and computed tomography (CT) in evaluating the tumor burden of pseudomyxoma peritonei (PMP). Besides, we assessed the ability of this combination to predict the likelihood of complete resection. Methods This retrospective study involved 504 patients diagnosed with PMP and scheduled for cytoreduction surgery. We compared tumor burden—quantified as peritoneal cancer index (PCI) by preoperative US and CT (US-CT-PCI)—with surgical findings. Next, we assessed the prognostic value of US-CT PCI and imaging features in determining the completeness of cytoreduction (CCR) score using multivariate analysis. Results US-CT PCI demonstrated a high PCI evaluation accuracy under moderate tumor burden. Higher US-CT PCI could predict incomplete resection. In addition, we identified imaging features such as mesenteric involvement as an independent predictor of incomplete resection (hazard ratio (HR) = 2.006; p = 0.007). Conclusions US-CT PCI allowed us to predict the completeness of cytoreductive surgery in patients with PMP. Moreover, the combined US and CT imaging detected several features indicating incomplete cytoreduction. Key Points • Ultrasonography (US) can act as a complementary diagnostic modality in peritoneal cancer index (PCI) evaluation by combining CT in the small bowel area and US in the abdominal area. • A modified peritoneal cancer index (US-CT PCI) helps preoperatively evaluate tumor burden with high accuracy and allows to predict incomplete resection. • US-CT PCI of 20 or above and the involvement of particular structures such as mesentery, independently indicate incomplete resection.