Rui-Qing Ma

The management of incidental appendiceal mucinous neoplasm (IAMN) was confusing with difficult decision-making. This study was to investigate the association of variables from the initial operation to cytoreductive surgery with overall survival (OS). One hundred thirty patients of IAMN treated at Aerospace Center Hospital were retrospectively analyzed from January 2014 to December 2020. A total of 82 IAMN patients who underwent reoperation with cytoreductive surgery (CRS) and had available data on the date of their initial operation was included. Of these 82 IAMN patients, the mean time from the initial operation to CRS was 19 months (1–146 months). The 5-year OS rate was 69.8%, and the median OS was 39 months (1–73 months). The patients who underwent CRS within 6 months had a higher radical rate (63.3% vs 36.7%) with lower tumor burden and better survival. No significant difference was found in patients with right hemicolectomy or prior systemic chemotherapy. Multivariate analysis identified the completeness of cytoreduction score and the pathologic subtype of peritoneal pseudomyxoma (PMP) as independent prognostic factors. Extended surgery and systematic chemotherapy are not suitable for IAMN patients. It is more important to identify the pathology and accept standard therapy in academic medical centers within 6 months.

Background: Pseudomyxoma peritonei (PMP) is a rare peritoneal malignancy with limited genomic data across different biospecimen types. The extent to which ascites and plasma reflect tissue-detected genomic alterations remains incompletely characterized. This study compared genomic profiles across tumor tissue, ascites, and plasma and explored KRAS-defined molecular features in PMP. Methods: Sixty patients with pathologically confirmed PMP were retrospectively included. Next-generation sequencing (NGS) data were available from tumor tissue in 57 patients, ascites in 40, and plasma in 28, with overlapping specimen availability. Cross-specimen genomic concordance was evaluated in 37 patients with matched tissue–ascites samples, including 28 patients with matched tissue, ascites, and plasma. Exploratory analyses were performed to characterize genomic and pathway-level features across KRAS-defined subgroups. Results: In tumor tissue, KRAS (66.7%), GNAS (31.6%), and TP53 (21.1%) were the most frequently altered genes. Among 28 patients with matched tissue, ascites, and plasma, patient-level concordance was 35.7%. At the mutation level, ascites showed higher tissue-referenced positive percentage agreement (PPA) than plasma (93.1% vs. 36.2%). In the expanded cohort of 37 matched tissue–ascites pairs, ascites achieved a tissue-referenced PPA of 94.2% and revealed additional alterations not detected in the corresponding tissue samples. Exploratory KRAS mutation stratification identified G12D (35.0%), G12X (32.5%), and KRAS-wild-type (wt) (17.5%) tumors as the predominant subgroups. KRAS-mutant tumors harbored more detected genomic alterations and potentially clinically actionable alterations than KRAS-wt tumors. Wnt and PI3K pathway alterations were more commonly observed in the G12D and G12X subgroups, respectively, whereas canonical oncogenic pathway alterations were less frequent in KRAS-wt tumors. Conclusions: PMP exhibits substantial genomic heterogeneity across patients and biospecimen types. Ascites cfDNA showed high concordance with tissue and captured additional genomic alterations, suggesting that ascitic fluid may serve as a complementary source for molecular profiling in PMP. KRAS-defined subgroups exhibited distinct genomic characteristics, although their clinical relevance requires further validation in larger, well-characterized cohorts.

Pseudomyxoma peritonei (PMP) is a rare peritoneal malignancy. Its typical clinical manifestations include abdominal distension, increased abdominal girth, or abdominopelvic masses. It is extremely rare for an umbilical hernia to be the initial manifestation, so this can easily lead to clinical misdiagnosis. A 60-year-old woman presented with a reducible umbilical mass for 10 months. Physical examination revealed marked abdominal distension and a 6-cm firm, irreducible umbilical mass. Serum tumor markers were normal. Computed tomography (CT) showed diffuse low-density abdominopelvic masses consistent with PMP, an appendiceal cystic lesion suggestive of mucinous neoplasm, omental thickening, and periumbilical tumor extension. Ultrasound revealed 9.4-cm ascites with mucin particles and septations. She underwent cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. Intraoperatively, extensive gelatinous mucin, a ruptured cystic appendix, and periumbilical tumor involvement were noted. Postoperative pathology confirmed a high-grade appendiceal mucinous tumor with PMP. The immunohistochemistry results were as follows: cytokeratin 20 positive, caudal type homeobox 2 positive, and Ki-67 labeling index of 30%. Telephone follow-up 3 months after surgery indicated that the patient was doing well. PMP presenting with umbilical hernia as the initial symptom is relatively rare and is often misdiagnosed as a simple umbilical hernia. For middle-aged and elderly female patients with umbilical hernia who have concurrent ascites, thickened peritoneum, or a hard and fixed hernia mass, abdominopelvic CT and ultrasound should be performed to rule out PMP to avoid blindly performing hernia repair and delaying diagnosis and treatment.

Background: Pseudomyxoma peritonei (PMP) prognosis remains heterogeneous. Systemic inflammation and nutrition are linked to cancer survival, but their role in PMP is unclear. We aimed to evaluate the prognostic impact of inflammation–nutrition status in appendiceal high‐grade PMP and to develop and internally validate a perioperative prognostic model. Methods: We retrospectively included patients with pathologically confirmed appendiceal high‐grade PMP who underwent cytoreductive surgery (CRS) with or without hyperthermic intraperitoneal chemotherapy (HIPEC) between January 2015 and December 2024. Baseline variables included demographics, body mass index (BMI), tumor markers (CEA, CA19‐9, CA125), inflammation–nutrition indices (advanced lung cancer inflammation index [ALI], prognostic nutritional index [PNI], neutrophil‐to‐lymphocyte ratio [NLR], platelet‐to‐lymphocyte ratio [PLR], lymphocyte‐to‐monocyte ratio [LMR], systemic inflammation score [SIS], systemic immune–inflammation index [SII]), prior surgical score (PSS), peritoneal cancer index (PCI), completeness of cytoreduction (CC), HIPEC, pathological subtype, and Ki‐67. Overall survival (OS) was the primary endpoint. Prognostic factors were identified by univariable and multivariable Cox models, and a nomogram was developed and internally validated. Results: Among 223 patients (146 females, 65.5%), median age was 59 years, and median OS was 21 months. In the multivariable Cox regression model, independent risk factors for worse overall survival (OS) were high‐grade with signet‐ring cells (hazard ratio [HR] = 2.575, 95% CI 1.749–3.790; p < 0.001), elevated CA19‐9 (HR = 2.189, 95% CI 1.501–3.192; p < 0.001), elevated CA125 (HR = 1.495, 95% CI 1.050–2.127; p = 0.026), high Ki‐67 (HR = 1.976, 95% CI 1.238–3.154; p = 0.004), and an unfavorable CC (CC1–3) (HR = 2.705, 95% CI 1.295–5.648; p = 0.008), while receipt of hyperthermic intraperitoneal chemotherapy (HIPEC) (HR = 0.611, 95% CI 0.377–0.991; p = 0.046) and a high ALI (HR = 0.576, 95% CI 0.398–0.834; p = 0.003) were independent protective factors. The model achieved a C‐index of 0.753, with 1‐, 2‐, and 3‐year AUCs of 0.810, 0.839, and 0.775, respectively. Calibration error was low (≈3%–6%) and decision‐curve analysis showed sustained net benefit across broad threshold ranges. Conclusions: The ALI is an independent prognostic marker in appendiceal high‐grade PMP. A nomogram incorporating ALI with tumor and pathological variables enabled reliable perioperative survival prediction with strong discrimination, calibration, and clinical utility.

In this study, we examined the effect of postoperative anemia on the prognosis of 721 patients with pseudomyxoma peritonei (PMP). Based on the initial hemoglobin (Hb) levels measured after surgery, the patients were categorized into the normal Hb (n = 65) and anemia groups (n = 656 patients, 91%), and the anemic patients were further divided into four subgroups based on their Hb levels. Patients with normal postoperative Hb levels had a significantly higher 5-year survival rate (80%) than those with anemia (67.4%). In addition, patients with Hb levels of 81–90 g/L had the shortest median survival duration of 36.3 ± 1.4 months. Tumor recurrence rates were consistent across the groups, whereas plasma and red blood cell transfusion volumes differed among the anemia subgroups. The postoperative cytoreduction rate, peritoneal carcinomatosis index, tumor grade, and Hb level were identified as factors significantly affecting postoperative survival. In conclusion, anemia developing within 24 h after surgery significantly affects the 5-year survival rate in patients with PMP, and the severity of anemia is a crucial risk factor.

Background: Acute kidney injury (AKI) is common after cytoreductive surgery (CRS) and hyperthermia intraperitoneal chemotherapy (HIPEC). Urine-guided hydration is found effective in preventing AKI in other high-risk patient populations. This study tested whether targeted intraoperative urine output maintenance and simultaneous hydration can reduce AKI in patients after CRS-HIPEC. Methods: In this randomized trial, adult patients who were scheduled to undergo CRS and cisplatin-based HIPEC for pseudomyxoma peritonei were randomized to receive either urine-guided hydration (urine output greater than or equal to 3 ml ⋅ kg ⁻¹ ⋅ h ⁻¹ or greater than or equal to 200 ml ⋅ h ⁻¹ ) or routine hydration (urine output greater than or equal to 0.5 ml ⋅ kg ⁻¹ ⋅ h ⁻¹ ) during the procedure. The primary endpoint was the incidence of AKI within 7 days after surgery, diagnosed according to the Kidney Disease Improving Global Outcome criteria. Results: From July 24, 2023, to July 18, 2024, 168 patients (mean age, 58 yr; 66.1% female sex) were enrolled and randomized; all were included in the intension-to-treat analysis. AKI incidence within 7 days was lower with urine-guided hydration than with routine hydration (21.4% [18 of 84] vs. 39.3% [33 of 84]; relative risk [RR], 0.55; 95% CI, 0.33 to 0.89; P = 0.012). Subgroup analysis showed that the proportion of AKI diagnosed according to urine criteria (urine output less than 0.5 ml ⋅ kg ⁻¹ ⋅ h ⁻¹ for 6 h or longer) was less with urine-guided hydration than with routine hydration (21.4% [18 of 84] vs. 35.7% [30 of 84]; RR, 0.60; 95% CI, 0.36 to 0.99; P = 0.040). Patients with urine-guided hydration developed fewer major complications within 30 days (36.9% [31 of 84] vs. 56.0% [47 of 84]; RR, 0.66; 95% CI, 0.47 to 0.92; P = 0.013). Adverse events did not differ between groups. Conclusions: In patients who underwent CRS and cisplatin-HIPEC for pseudomyxoma peritonei, intraoperative urine-guided hydration reduced postoperative AKI by more than 40% and was safe. A large trial is warranted to verify the results of this study.

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. 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. 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. 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.

Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is a leading treatment for advanced ovarian cancer, significantly improving overall survival and disease-free survival. This case involves a patient with peritoneal metastasis and ultra-high intra-abdominal pressure (36 mmHg). CRS + HIPEC induces extensive pathological and physiological changes affecting respiratory, circulatory, renal, coagulation, and metabolic systems. Effective perioperative anesthesia management, including the type and volume of fluids administered, is crucial for optimizing patient outcomes. The complexities of anesthesia management in such cases present significant challenges.

The necessity of prophylactic cytoreductive surgery (PCRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for low-grade appendiceal mucinous neoplasms (LAMN) after complete removal is still controversial. This study aims to determine the role of PCRS + HIPEC and identify optimal strategies for managing these patients. One hundred fifty-nine patients who sought medical advice at Aerospace Center Hospital were retrospectively analyzed from January 2011 to December 2021. All the patients were divided into the PCRS group and the observation group. The data of surgical specimens and pathology was collected, and the effect on recurrence-free survival (RFS) was analyzed. Of these 159 patients, 88 were in the PCRS group, and 71 were in the observation group. The median follow-up time was 38 months. Seven patients recurred and developed into pseudomyxoma peritonei, 1 in the PCRS group and 6 in the observation group. The analysis of RFS showed that patients who underwent PCRS (P = .01) and HIPEC (P = .01) had better survival. After multivariate analysis, the surgical specimen accompanied by disseminated peritoneal adenocarcinoma was identified as an independent prognostic factor for RFS. In the study of surgical resection content, patients with greater omentum (P = .01) and bilateral fallopian tubes and ovaries of women (P = .002) resection had a more prolonged RFS with statistical significance. The research indicated that PCRS + HIPEC could prevent recurrence. Therefore this treatment were necessary for LAMN after complete removal in patients with a high risk of recurrence. However, it was not proven to be an independent factor for RFS, and a multicenter, prospective, randomized trial was need to definitively address the role of PCRS + HIPEC for LAMN after complete resection.