The mutational landscape and prognostic indicators of pseudomyxoma peritonei originating from the ovary.

Pseudomyxoma peritonei (PMP) is a rare disorder with unique pathological and genetic changes. Although several studies have reported the clinical features and mutational changes of PMP that originates from the appendix, few studies on PMP originating from the ovary have been reported due to its extreme rarity. In order to characterize the somatic mutational landscape and to investigate the prognosis predicting factors of ovary-originating PMP, we examined 830 cases of PMP and identified 16 patients with PMP that originated from the ovary. Whole-exome sequencing (WES) was performed on 12 cases using formalin-fixed, paraffin-embedded (FFPE) tissue samples. We found that 25% (3/12) of the patients carried mutations in cancer driver genes, including TP53, ATM and SETD2, and 16.7% (2/12) of the patients carried mutations in cancer driver genes, including ATRX, EP300, FGFR2, KRAS, NOCR1 and RB1. The MUC16 (58.33%), BSN (41.67%), PCNT (41.67%), PPP2R5A (41.67%), PRSS36 (41.67%), PTPRK (41.67%) and SBF1 (41.67%) genes presented the highest mutational frequencies. The PI3K-Akt signaling pathway, human papillomavirus infection pathway, cell skeleton, cell adhesion, and extracellular matrix and membrane proteins were the major pathways or functions that were affected. Patients were followed up to 174 months (median: 48.26 months). The 5-year OS rate for all patients was 71.2% and the median OS was not reached. PTPRK mutations, presurgical CA199 level, completeness of cytoreduction (CCR) and peritoneal cancer index (PCI) were identified as potential predictive factors for patient survival. In conclusion, the mutational landscape for ovary-originating PMP was revealed and exhibited unique features distinct from appendix-originating PMP. PTPRK, CA199, CCR and PCI may predict patient survival.

Hyperthermic intraperitoneal chemotherapy in patients with incomplete cytoreduction for appendiceal pseudomyxoma peritonei: a 10-year treatment experience in China.

BACKGROUND: To explore the application value of hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with incomplete cytoreduction for appendiceal pseudomyxoma peritonei (PMP). METHODS: We retrospectively analyzed the clinical data of 526 patients with incomplete cytoreduction for appendiceal PMP to discover its prognostic factors, and the therapeutic value of HIPEC. RESULTS: The 5-year and 10-year overall survival rates of patients after cytoreductive surgery (CRS) treated with HIPEC were significantly higher than those without HIPEC (5y-OS: 58% vs. 48%, 10y-OS: 37% vs. 16%, P = 0.032). The median progression-free survival (PFS) following CRS was 20 months, with a 20% 3-year PFS. The median PFS following CRS + HIPEC was 33 months, with a 60% 3-year PFS (P = 0.000). Univariate analysis indicated that HIPEC, gender, completeness of cytoreduction (CCR) and pathological grade had statistical difference. Multivariate analysis showed that CRS without HIPEC and high pathological grade were independent risk factors for poor prognosis and rapid tumor progression. CONCLUSIONS: HIPEC may prolong the survival in patients with incomplete cytoreduction for low-grade appendiceal PMP. High pathological grade indicates poor survival and rapid tumor progression.

Significance of laparoscopic cytoreductive surgery for appendiceal pseudomyxoma peritonei with limited disease and low tumor burden.

OBJECTIVE: To investigate the clinical value of laparoscopic cytoreductive surgery (CRS) in treating of appendiceal pseudomyxoma peritonei with limited disease and low tumor burden. METHODS: The clinical data of patients with appendiceal pseudomyxoma peritonei treated by surgery with CRS at the Aerospace Center Hospital from January 2018 to December 2021 were retrospectively analyzed. The patients were divided into laparoscopic or open CRS groups according to the operation method. A propensity score-matched (PSM) analysis (1:1) was performed, the related clinical variables were compared between the two groups, and the effect on progression-free survival (PFS) was also analyzed. RESULTS: One hundred and eight patients were included in this study. After PSM, 33 patients were selected from each group and the age and peritoneal cancer index were matched between the two groups. There were significant differences in operation time (P < 0.001), intraoperative bleeding (P < 0.001), intraoperative blood transfusion (P = 0.007), hospital stay (P < 0.001). The analysis of PFS showed that there was no significant difference between the two operation methods. After multivariate analysis, the pathologic subtype (P = 0.012) was identified as an independent prognostic factor for PFS. CONCLUSION: The curative effect of laparoscopic CRS is like that of open operation, which can significantly shorten the operation time and hospital stay and reduce intraoperative bleeding and blood transfusion event. The laparoscopic CRS is safe and feasible in strictly selected patients. The pathologic subtype is an independent factor affecting the prognosis for PFS.

Prognostic prediction of preoperative nutritional status in low-grade appendiceal mucinous neoplasms.

To describe the preoperative nutritional status of Low-grade Appendiceal Mucinous Neoplasms (LAMNs) and identify prognostic factors for survival. Medical records from 165 patients with LAMNs who attended the Aerospace Center Hospital, Beijing, China between January 2017, and December 2018 were retrospectively reviewed. Survival analysis was performed with the Kaplan-Meier method, the log-rank test, and a Cox proportional hazards model. Among 165 patients, 59 (36%) were male and 106 (64%) were female. Patient's median age was 58 years (range 20 to 78 years). Univariate analysis indicated that gender, weight loss, prior surgical score (PSS), red blood cell, albumin, peritoneal cancer index (PCI), completeness of cytoreduction (CCR), and hyperthermic intraperitoneal chemotherapy (HIPEC) were related to prognosis. Multivariate analysis showed that PSS, CCR and HIPEC were independent predictors of prognosis. The preoperative nutritional status of patients plays an important role in predicting prognosis. Patients can benefit from a complete cytoreductive surgery (CCRS) and HIPEC in an experienced institution for the first medical treatment.

Complete Cytoreductive Surgery vs. Debulking Surgery for pseudomyxoma peritonei of appendiceal origin: A propensity score-matched study based on a single-center experience.

OBJECTIVE: This study aimed to report the prognostic predictors and compare the long-term outcomes of complete cytoreductive surgery (CCRS) vs. debulking surgery (DS) in patients with pseudomyxoma peritonei (PMP) of appendiceal origin. METHODS: A retrospective analysis of 1008 consecutive patients with PMP undergoing primary surgery from January 2008 to December 2019 was performed. A propensity score-matched (PSM) analysis (1:1) was performed, and oncologic outcomes were compared between the CCRS and DS groups. RESULTS: Out of 1008 patients, 258 patients were excluded. Baseline characteristics differed significantly between the CCRS and DS groups (total n = 750). After PSM, 106 patients were selected from each group and the baseline characteristics were matched between groups. There were significant differences between groups in operative time, the incidence of major complications (P = 0.017), and the numbers of organs removed. The median follow-up was 28 (1-131) months. Median overall survival (OS) for the 212 patients was 52.0 months (95% CI 40.2-63.8), and 10-year OS was 39.0%. Median OS could not be calculated for the CCRS group; in the DS group, this value was 41 months (P = 0.010). The 10-year OS rate was 54.2% in the CCRS group and 31.2% in the DS group. Multivariate analyses identified CCRS (P = 0.012) and histopathologic subtype (P < 0.001) as independent prognostic factors for OS. CONCLUSIONS: In this matched-pair analysis of patients with appendiceal PMP, CCRS was safe and associated with better prognosis than DS. The completeness of cytoreduction and histopathologic subtype were two independent prognostic factors for OS.

[Ultrasound guided percutaneous microwave ablation in the treatment of recurrent thyroid nodules].

OBJECTIVE: To explore the clinical effect of ultrasound guided microwave ablation in the treatment 01 recurrent thyroid nodules. METHOD: Seventy-five cases of recurrent thyroid nodules were treated with ultrasound guided microwave ablation(MWA) under local anesthesia. All the patients underwent biopsy puncture, then the microwave ablation needle puncted in the center site of nodule with energy of 25-35 W, and the center tempera- ture could reach to 70-95 °C. RESULT: MWA were successfully performed in all the patients without significant com- plications. Temporary hoarseness occurred in 5 cases and dispeared after 1-3 weeks, choking water occurred in 2 patients and recovered 3-5 days later. Ultrasound examination showed that the bloodstream of thyroid nodules disappeared in all the patients, and the nodules diminuted with varying degrees at 3-month and at 6-month follow- up Serum FT4 and TSH did not change significantly. CONCLUSION: Ultrasound guided MWA could be effective in the treatment of recurrent thyroid nodule with minimal invasive technology.