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Abstract 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

Abstract Introduction Pseudomyxoma peritonei (PMP) is a generalized term, usually known as “jelly belly” since 1884. Incidence is very low, 1–3 per million people per year. Because of its indolent nature, it is usually diagnosed at an advanced stage, thereby impacting the quality of life. The 5-year survival rate varies from 23 to 86% in world literature. Even 10 years and 20 years of survival have been described. With our experience, we like to propose rename of PMP as abdomino-peritoneal mucinous carcinoma (APM) as we strongly feel the time has come to specify the term and standardize the management strategy. Methodology In the premier institute of India and as a tertiary referral center, we experienced the maximum number of advanced cases of APM. From 2012 to 2021, we analyzed all the APM patients based on a prospectively maintained computerized database in the department of surgical oncology and found the reasons for renaming from this traditional one. Results We included a total of 87 patients who underwent surgical intervention. Thirty-five patients underwent cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), and 52 patients underwent debulking. In CRS-HIPEC patients, CC-0 was achieved in 28 patients (80%), CC-1 in 4 patients (11.4%), and CC-2 in 3 patients (8.6%). Palliative intent HIPEC was done in 3 patients (8.6%). Clavien-Dindo grade III and IV morbidity was observed in 18.8% of patients with 90 days mortality of 5.7%. Conclusion With our long-term experience and advancement of scientific evidence, we like to propose a new name for PMP as APM. We strongly believe this paper will give a clear picture of this rare disease and standard management outlines.

Fengcai Yan,1,2 Feng Shi,1,2 Xinbao Li,3 Chunkai Yu,1 Yulin Lin,3 Yan Li,1,3 Mulan Jin2 1Department of Pathology, Beijing Shijitan Hospital, Capital Medical University, Beijing, People’s Republic of China; 2Department of Pathology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, People’s Republic of China; 3Department of Peritoneal Cancer Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing, People’s Republic of ChinaCorrespondence: Mulan Jin Email kinmokuran@163.comObjective: This study aims to demonstrate clinicopathological characteristics and immunohistopathological phenotypes of pseudomyxoma peritonei (PMP) originated from ovaries.Methods: The primary origin of PMP was explored by reviewing H&E sections retrospectively and performing a series of immunohistochemical staining on CK7, CK20, CDX2, CEA, Villin, SATB2, CA125, ER, PR, and MUC.Results: Among 310 PMP patients, a few originated from extra-appendix, whereas eight cases were of ovarian origin (2.6%), including three teratoma-associated ovarian mucinous tumors and five primary ovarian mucinous tumors with spontaneous or iatrogenic rupture, respectively. Most peritoneal metastases were acellular mucin or low-grade mucinous carcinoma peritonei (6/8, 75%), while the rest were high-grade mucinous carcinoma peritonei (2/8, 25%). Tumors were positive for CK20, CDX2, CEA, and Villin. SATB2 was specifically diffuse positive in teratoma-associated ovarian mucinous tumors, and negative in primary ovarian mucinous tumors. Differential expression of MUC was observed in these tumors.Conclusion: PMP of ovarian origin is extremely rare. The precise diagnosis requires serial sections of the appendix or suspicious tissue to exclude appendiceal mucinous neoplasms, as well as comprehensive analysis of clinical features, surgical findings, histopathological characteristics, and immunohistochemistry on specific biomarkers.Keywords: pseudomyxoma peritonei, PMP, ovary, mucinous tumor

Background: Pseudomyxoma peritonei (PMP) is a rare clinical condition with fatal outcomes, which is characterized by the progressive accumulation of mucinous ascites and peritoneal implants. Some studies have reported the effect of PMP biology on patient outcome. The objective of this study was to analyze published articles focusing on the impact of pathology on the prognosis of PMP patients undergoing debulking.Methods: Data from all studies regarding the prognosis of patients, with different pathologies, who underwent debulking surgery were analyzed. We searched PubMed, the Wiley Online Library, Ovid, and the Cochrane Library (through January 2020). Studies were confined to those articles written in English. Five studies were identified, and the differences in 5-year survival rates were analyzed according to the Kaplan–Meier survival curves. The hazard ratios (HRs) of the 5-year survival rates were calculated.Results: The mean and median 5-year survival rates of all patients were 39 and 40%, respectively. The median overall survival was 49.3 months. The mean 5-year survival rates of low-grade PMP was 45.2%. The five studies had sufficient data to calculate HRs from the 5-year survival rates data, and three had HRs lower than 1. The total HRs was 0.54, with a 95% CI between 0.33 and 0.89 (P = 0.01).Conclusions: Among PMP patients receiving debulking surgery who are not able to undergo complete cytoreductive surgery, low-grade biological PMP had a better prognosis than high-grade PMP.