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Objective Currently, the primary surgical treatment for pseudomyxoma peritonei (PMP) is cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC). The perioperative period is frequently accompanied by severe pain. Erector spinae plane block (ESPB) can enhance analgesia for abdominal surgery. The purpose of this study was to compare the analgesic effects of bilateral multiple-injection ESPB in patients with PMP. Methods Fifty patients with PMP were randomly divided into two groups: the ESPB combined with general anesthesia group (Group E) and the general anesthesia alone group (Group C). Prior to the induction, patients in Group E underwent ESPB at the T7 and T11 levels. The primary outcome was the visual analog scale (VAS) scores during rest at 6 h post-extubation. Secondary outcomes included intraoperative and postoperative opioid consumption, time for first rescue analgesia, frequency distribution of rescue analgesia, incidence of nausea and vomiting, adverse events associated with ESPB. Results The Visual Analogue Scale (VAS) scores in Group E were significantly lower compared to Group C at immediate post-extubation (1.6 ± 0.9 vs. 2.4 ± 1.2, P  = 0.008), and at 2 (1.9 ± 1.2 vs. 3.2 ± 1.1, P  < 0.001), 4 (2.4 ± 1.5 vs. 3.7 ± 1.0, P  = 0.001), and 6 h (2.7 ± 1.1 vs. 3.8 ± 1.4, P  = 0.004) post-extubation during rest. Similarly, the VAS scores in Group E were significantly lower than those in Group C at immediate post-extubation (3.0 ± 1.4 vs. 4.6 ± 1.2, P  < 0.001), and at 2 (3.8 ± 1.7 vs. 4.9 ± 1.4, P  = 0.019), 4 (3.5 ± 1.3 vs. 5.3 ± 1.5, P  < 0.001), and 6 h (3.9 ± 1.8 vs. 4.9 ± 1.3, P  = 0.004) post-extubation during movement. In Group E, the intraoperative remifentanil administration (2319.3 ± 1089.5 vs. 2984.6 ± 796.1, P  = 0.017) and the amount of rescue analgesia within 2 h post-extubation (0 vs. 4, P  = 0.037) were significantly less than in Group C, and the first rescue analgesia time was shorter as well (231.4 ± 147.5 vs. 668.8 ± 416.7, P  < 0.001). Conclusion Compared to general anesthesia alone, bilateral multiple-injection ESPB with 0.2% ropivacaine can enhance analgesia and reduce opioid administration in patients with PMP. However, the duration of analgesia with ESPB is relatively short due to the low concentration of the local anesthetic used. Trial registration Chinese Clinical Trial Registry, ChiCTR2300069504, 20/03/2023.

Objective To investigate the course of health-related quality of life (HQL) over time in patients with peritoneal carcinomatosis (PC) after complete cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Methods Prospective, single-center, nonrandomized cohort study using the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire. Results Ninety patients who underwent CRS and HIPEC for PC in our institution were enrolled in the study. Mean age was 56 years (range 27–77 years) (61 % female). Primary tumor was colorectal in 21 %, ovarian in 19 %, pseudomyxoma peritonei in 16 %, an appendix tumor in 16 %, gastric cancer in 10 %, and peritoneal mesothelioma in 13 % of cases. Mean peritoneal carcinomatosis index was 22 (range 2–39). Mean global health status score was 69 ± 25 preoperatively and 55 ± 20, 66 ± 22, 66 ± 23, 71 ± 23, and 78 ± 21 at months 1, 6, 12, 24, and 36, respectively. Physical and role function recovered significantly at 6 months and were close to baseline at the 24-month measurement. Emotional function starting from a low baseline recovered to baseline by month 12. Cognitive and social function had slow recovery on follow-up. Fatigue, diarrhea, dyspnea, and sleep disturbance were symptoms persistent at 6-month follow-up, improving later on in survivors. Conclusions Survivors after CRS and HIPEC have postoperative quality of life similar to preoperatively, with most of the reduced elements recovering after 6–12 months. We conclude that reduced quality of life of patients after CRS and HIPEC should not be used as an argument to deny surgical therapy to these patients.

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.

Background The role of gastrectomy to achieve complete cytoreduction (CCR) for pseudomyxoma peritonei (PMP) is controversial due to uncertain risk/benefit ratio. The outcomes of patients who gastrectomy over a twenty-year period in a high-volume unit are reported. Methods All patients requiring gastrectomy to achieve CCR for appendiceal PMP between 2000 and 2020 were reviewed. Demographics, disease, operative, complication, and survival data were analysed. The first and second decades were compared. Results A total of 2148 patients underwent CRS and HIPEC, of which 78% had CCR. Gastrectomy was performed in 7.1%. Median age was 55 years, and 52% were female. Among gastrectomy patients, 94.2% had ≥1 elevated tumour marker, and 18% had high-grade disease. Median PCI was 30, and 30% required subtotal colectomy. Clavien–Dindo III-IV complications occurred in 32%, and 90-day mortality was 1.75%. Median survival was 104 months, and 10-year OS was 47%. Comparing the two decades, total CRS cases almost tripled, with a greater proportion achieving CCR (82.2% vs. 67.8%) but fewer requiring gastrectomy (5.3% vs. 13.5%). In those who had gastrectomy, disease was more advanced (higher PCI, more high-grade disease, more colectomies) in the later period. However, on multivariable analysis, there was no difference in survival between decades. High-grade histology was the only predictor of survival. Conclusions Gastrectomy can achieve good long-term survival with low mortality and acceptable morbidity and should not deter surgeons from achieving CCR. However, increasing experience shows CCR can be achieved, preserving the stomach in the majority of cases through careful consideration of the anatomy and gastric blood supply.

Appendiceal mucinous neoplasms (AMNs), characterized by expansile or “pushing” growth of neoplastic epithelium through the appendix wall, are sometimes accompanied by peritoneal involvement, the extent and grade of which largely determine clinical presentation and long-term outcomes. However, the prognosis of tumors entirely confined to the appendix is still debated and confusion remains regarding their biologic behavior and, consequently, their clinical management and even diagnostic nomenclature. We evaluated AMNs limited to the appendix from 337 patients (median age: 58 years, interquartile range (IQR): 47–67), 194 (57.6%) of whom were women and 143 (42.4%) men. The most common clinical indication for surgery was mass or mucocele, in 163 (48.4%) cases. Most cases (N = 322, 95.5%) comprised low-grade epithelium, but there were also 15 (4.5%) cases with high-grade dysplasia. Lymph nodes had been harvested in 102 (30.3%) cases with a median 6.5 lymph nodes (IQR: 2–14) per specimen for a total of 910 lymph nodes examined, all of which were negative for metastatic disease. Histologic slide review in 279 cases revealed 77 (27.6%) tumors extending to the mucosa, 101 (36.2%) to submucosa, 33 (11.8%) to muscularis propria, and 68 (24.4%) to subserosal tissues. In multivariate analysis, deeper tumor extension was associated with older age (p = 0.032; odds ratio (OR): 1.02, 95% confidence intervals (CI): 1.00–1.03), indication of mass/mucocele (p < 0.001; OR: 2.09, CI: 1.41–3.11), and wider appendiceal diameter, grossly (p < 0.001; OR: 1.61, CI: 1.28–2.02). Importantly, among 194 cases with at least 6 months of follow-up (median: 56.1 months, IQR: 24.4–98.5), including 9 high-grade, there was no disease recurrence/progression, peritoneal involvement (pseudomyxoma peritonei), or disease-specific mortality. These data reinforce the conclusion that AMNs confined to the appendix are characterized by benign biologic behavior and excellent clinical prognosis and accordingly suggest that revisions to their nomenclature and staging would be appropriate, including reverting to the diagnostic term mucinous adenoma in order to accurately describe a subset of them.

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.

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.

Peritoneal cancer index (PCI) is the surgical variable most commonly used to quantify the extent of peritoneal metastases for pseudomyxoma peritonei (PMP) patients. The present study aimed to investigate the agreement between CT predicted and surgical PCI by the Bland–Altman method for PMP of appendiceal origin. A total of 167 PMP patients of appendiceal origin were included between 2016 and 2021. Bland–Altman analysis was performed for both total PCI and selected PCI (regions 2 + 9–12). After the Bland–Altman plot was drawn, the mean bias and its 95% limit of agreements (LoAs) was quantified. Besides, the correlation coefficients between CT-PCI and surgical PCI were also been calculated. The Bland–Altman plot showed the mean bias ± SD between total CT-PCI and surgical PCI as 0.431 ± 3.005, with the LoAs from − 5.459 to 6.321. There were nine points of difference in total PCI exceeded the 95% LoAs, with the rate of 5.39% (9/167). As for selected CT-PCI, Bland–Altman plot showed the mean bias ± SD between selected CT-PCI and surgical PCI as − 0.287 ± 1.955, with the LoAs from − 4.118 to 3.544. There were ten points of difference in selected PCI exceeded the 95% LoAs, with the rate of 5.99% (10/167). The Spearman's rank correlation coefficient between total CT-PCI and surgical PCI was 0.911, P  < 0.001, as for selected CT-PCI and surgical PCI, the coefficient was 0.909, P  < 0.001. Although there was a strong correlation for both total and selected CT-PCI with surgical PCI, however, the agreement is still not good in Bland–Altman analysis, which suggested that CT-PCI cannot predict surgical PCI accurately even in professional PMP treatment centers. In brief explanation, CT makes it difficult to distinguish the borderline between tumor tissue and mucus and to detect tumor lesions in the small intestine regions, which caused overestimation or underestimation by CT-PCI. In the future, a multiple linear regression model based on CT-PCI might accurately predict surgical PCI preoperatively.

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.

Background Appendiceal mixed neuroendocrine-non-neuroendocrine neoplasms (a-MiNENs) combined with peritoneal pseudomyxoma (PMP) represent an exceptionally rare and clinically challenging entity. This study aims to elucidate the clinical characteristics of this unique tumor subtype and identify critical prognostic factors, thereby providing insights into the management and outcomes of affected patients. Methods We conducted a retrospective analysis of 14 patients diagnosed with a-MiNEN and PMP at the Department of Myxoma, Aerospace Center Hospital, between January 2014 and September 2022. Data included demographics, symptoms, tumor grading, treatments, and outcomes. Results Pathological evaluation revealed a diverse spectrum of tumor grades and PMP subtypes: 5 cases were classified as G1 neuroendocrine tumors (NET), 4 as G2 NET, and 5 as G3 neuroendocrine carcinoma (NEC). Regarding PMP subtypes, 5 cases had disseminated peritoneal adenomucinosis (DPAM), 4 had peritoneal mucinous carcinomatosis (PMCA), and 5 had peritoneal mucinous carcinomatosis with signet ring cell carcinoma (PMCA-S). The median postoperative survival time was 28.55 months, with 1-year and 2-year survival rates of 80.0% and 66.7%, respectively. Patients aged < 60 years, male sex, absence of right hemicolectomy, postoperative peritoneal cancer index (PCI) ≥ 25, and perineural invasion demonstrated significantly lower 1-year and 2-year survival rates compared to their counterparts, with distinct separation observed in the Kaplan–Meier curves. These factors likely reflect biological differences in disease progression, inadequate surgical debulking, and aggressive tumor behavior. Conclusions a-MiNEN with PMP progresses rapidly. Young patients, male patients, those with absence of right hemicolectomy, postoperative PCI ≥ 25 and perineural invasion may face poorer outcomes. This study, one of the few to analyze this rare condition, underscores the need for targeted treatments and further validation in larger cohorts.