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Medicine Jul 2020The aim of study was to develop and validate nomograms for predicting overall survival (OS) and cancer-specific survival (CSS) of patients with pseudomyxoma peritonei...
BACKGROUND
The aim of study was to develop and validate nomograms for predicting overall survival (OS) and cancer-specific survival (CSS) of patients with pseudomyxoma peritonei (PMP) and compare the predictive accuracy with the American Joint Committee on Cancer (AJCC) staging system.
METHODS
Data of 4959 PMP patients who underwent surgical resection were collected between 2004 and 2015 from the Surveillance Epidemiology and End Results (SEER) database. All included patients were divided into training (n = 3307) and validation (n = 1652) cohorts. The Kaplan-Meier method and Cox proportional hazard model were applied. Nomograms were validated by discrimination and calibration. Finally, concordance index (C-index) was used to compare the predictive performance of nomograms with that of the AJCC staging system.
RESULTS
According to the univariate and multivariate analyses of training sets, both nomograms for predicting OS and CSS combining age, grade, location, N stage, M stage, and chemotherapy were identified. Nomograms predicting OS also incorporated T stage and the number of lymph nodes removed (LNR). The calibration curves showed good consistency between predicted and actual observed survival. Moreover, C-index values demonstrated that the nomograms predicting both OS and CSS were superior to the AJCC staging system in both cohorts.
CONCLUSION
We successfully developed and validated prognostic nomograms for predicting OS and CSS in PMP patients. Two nomograms were more accurate and applicable than the AJCC staging system for predicting patient survival, which may help clinicians stratify patients into different risk groups, tailor individualized treatment, and accurately predict patient survival in PMP.
Topics: Adult; Aged; Female; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Neoplasm Staging; Nomograms; Peritoneal Neoplasms; Prognosis; Progression-Free Survival; Proportional Hazards Models; Pseudomyxoma Peritonei; Reproducibility of Results; SEER Program; Survival Analysis; Young Adult
PubMed: 32756083
DOI: 10.1097/MD.0000000000020963 -
Indian Journal of Surgical Oncology Jun 2023Pleural spread occurs in pseudomyxoma peritonei (PMP) in less than 10% of the patients and is treated by thoracic cytoreductive surgery with or without hyperthermic...
Pleural spread occurs in pseudomyxoma peritonei (PMP) in less than 10% of the patients and is treated by thoracic cytoreductive surgery with or without hyperthermic intrathoracic chemotherapy (HITOC). It is performed both for symptom palliation and disease control and includes pleurectomy and decortication and wedge and segmental lung resections. So far, only unilateral spread treated with a thoracic cytoreductive surgery (CRS) has been reported in literature. We report a patient with bilateral thoracic PMP following a complete abdominal CRS and hyperthermic intraperitoneal chemotherapy (HIPEC) who was treated with bilateral staged thoracic CRS and subsequently had a 4th CRS for abdominal disease. The staged procedure was performed as she was symptomatic due to the thoracic disease and there was disease on all pleural surfaces. HITOC was not performed. Both procedures were uneventful with no major morbidity. The patient is currently disease free nearly 84 months after the first abdominal CRS and 60 months after the second thoracic CRS. Thus, an aggressive CRS in the thorax in patients with PMP can result in a prolongation of survival while preserving the quality of life if the abdominal disease is controlled. A thorough understanding of the disease biology and surgical expertise are both essential for selecting the right patients for these complex procedures and achieving good short- and long-term outcomes.
PubMed: 37359933
DOI: 10.1007/s13193-023-01745-5 -
BMJ Case Reports Jul 2010Mucoceles resulting from cystadenomas of the ovary are uncommon. Although rare, rupture of the mucoceles can occur without causing any abdominal issue. This report...
BACKGROUND
Mucoceles resulting from cystadenomas of the ovary are uncommon. Although rare, rupture of the mucoceles can occur without causing any abdominal issue. This report concerns an unusual and interesting case of the right ovary associated with liver, spleen and kidney cysts.
CASE PRESENTATION
A case of ruptured mucocele resulting from cystadenoma of the ovary occurred in a 63-year-old woman. The patient underwent x-ray, ultrasound and a CT scan. Localised pseudomyxoma peritonei associated with adenocarcinoma of the right ovary was diagnosed. The patient underwent resection of the ovary, uterus, uterine tube and appendix with excision of the mesenteric lymph nodes and removal of about 5000 ml of yellowish mucoid jelly. She was free of symptoms at 1-year follow-up.
CONCLUSIONS
Pseudomyxoma peritonei may occur in patients with ovarian cystadenocarcinoma. In such patients, the ovary should be investigated and transvaginal ultrasound can be carefully performed in cases of ruptured mucoceles and localised pseudomyxoma peritonei. Surgical intervention is the current choice of management.
Topics: Biomarkers, Tumor; Cystadenocarcinoma; Diagnosis, Differential; Diagnostic Imaging; Female; Humans; Lymph Node Excision; Middle Aged; Mucocele; Ovarian Neoplasms; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 22753161
DOI: 10.1136/bcr.06.2008.0137 -
Archives of Pathology & Laboratory... Dec 2022High-grade appendiceal mucinous neoplasm (HAMN) is a relatively recently introduced term describing a rare epithelial neoplasm of the appendix that demonstrates...
CONTEXT.—
High-grade appendiceal mucinous neoplasm (HAMN) is a relatively recently introduced term describing a rare epithelial neoplasm of the appendix that demonstrates pushing-type invasion but high-grade cytologic atypia. It remains understudied.
OBJECTIVE.—
To describe clinicopathologic features of HAMNs.
DESIGN.—
We identified 35 HAMNs in a multi-institutional retrospective study. Clinical and histologic features were reviewed in all cases, as well as molecular features in 8 cases.
RESULTS.—
Patients were 57 years of age on average and most commonly presented with abdominal/pelvic pain. Histologically, 57% of the tumors showed widespread high-grade features. Architectural patterns in high-grade areas included flat, undulating, or villous growth, and occasionally micropapillary, cribriform, or multilayered growth. Thirteen cases had intact serosa, and the remaining 22 perforated the serosa, including 7 with peritoneal acellular mucin beyond appendiceal serosa and 10 with grade 2 pseudomyxoma peritonei. Molecular abnormalities included KRAS mutations in 7 cases and TP53 mutations in 4. No tumor confined to the appendix recurred. Two patients without pseudomyxoma peritonei at initial presentation developed pseudomyxoma on follow-up. Among 11 patients who presented with pseudomyxoma peritonei, 5 died of disease and 3 were alive with disease at last follow-up.
CONCLUSIONS.—
HAMNs have a similar presentation to low-grade appendiceal mucinous neoplasm, and similar stage-based prognosis. When they spread to the peritoneum, they typically produce grade 2 pseudomyxoma peritonei, which may be associated with a worse prognosis than classical grade 1 pseudomyxoma peritonei.
Topics: Humans; Pseudomyxoma Peritonei; Retrospective Studies; Peritoneal Neoplasms; Neoplasm Recurrence, Local; Appendiceal Neoplasms
PubMed: 35472721
DOI: 10.5858/arpa.2021-0430-OA -
World Journal of Gastroenterology Oct 2006Pseudomyxoma peritonei (PMP) is a rare disease. It refers to a progressive disease process within the peritoneum which originates from the appendix or ovaries and is... (Review)
Review
Pseudomyxoma peritonei (PMP) is a rare disease. It refers to a progressive disease process within the peritoneum which originates from the appendix or ovaries and is characterised by the production of copious amounts of mucinous fluid resulting in a "jelly belly". If untreated the condition is fatal. The traditional approach to PMP is based on repeated surgical debulking procedures, often associated with intraperitoneal or systemic chemotherapy. The natural history of this disease has been drastically modified since the introduction of a new surgical approach defined as a peritonectomy procedure. This paper is to review the literature on this treatment strategy.
Topics: Antineoplastic Agents; Cisplatin; Combined Modality Therapy; Doxorubicin; Humans; Hyperthermia, Induced; Mitomycin; Peritoneal Neoplasms; Peritoneum; Pseudomyxoma Peritonei
PubMed: 17036382
DOI: 10.3748/wjg.v12.i38.6124 -
The Oncologist Mar 2015The authors are currently conducting molecular and genetic research to identify new targets for therapeutic interventions and predictive biomarkers in pseudomyxoma...
The authors are currently conducting molecular and genetic research to identify new targets for therapeutic interventions and predictive biomarkers in pseudomyxoma peritonei. The final aim of these studies is to rationalize the choice of treatment, which remains an unmet clinical need.
Topics: Antineoplastic Combined Chemotherapy Protocols; Female; Humans; Male; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 25745055
DOI: 10.1634/theoncologist.2014-423 -
Frontiers in Surgery 2022To evaluate the correlation between pain intensity and comfort level in patients with pseudomyxoma peritoneum (PMP) before and after hyperthermic intraperitoneal...
OBJECTIVE
To evaluate the correlation between pain intensity and comfort level in patients with pseudomyxoma peritoneum (PMP) before and after hyperthermic intraperitoneal chemotherapy (HIPEC).
METHODS
From June 2018 to June 2019, patients who underwent HIPEC for PMP after surgical treatment in our institute were selected. The intra-abdominal pressure (IAP) and pain intensity (PI) before and after HIPEC were recorded, and the correlation between them was analyzed.
RESULTS
Seventy-four patients received HIPEC 253 times. IAP and PI were significantly higher after perfusion than before perfusion ( < 0.05). When IAP < 12 cmHO, the change of PI was not correlated to the increase of IAP, and the patient tolerated the treatment. However, when IAP > 12 cmHO, the increase of PI was significantly associated with IAP and cause significant discomfort during the treatment.
CONCLUSION
IAP may be a monitoring index for the comfort of PMP patients during the postoperative HIPEC treatment.
PubMed: 35284489
DOI: 10.3389/fsurg.2022.797811 -
Pleura and Peritoneum Sep 2020
PubMed: 33364339
DOI: 10.1515/pp-2020-0119 -
Cancers Sep 2022Pseudomyxoma peritonei (PMP) is ideally treated by cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), leading to significant morbidity....
Pseudomyxoma peritonei (PMP) is ideally treated by cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), leading to significant morbidity. Beyond the histologic grade, the prognosis lies in the completeness of cytoreduction (CC-score of 0/1 vs. 2/3) and the severe complication rate. The mucinous nature of the peritoneal implants sometimes induces liver and/or spleen scalloping on imaging. The predictive value of scalloping was assessed regarding resectability, grade, survival and severe morbidity. This monocentric, retrospective analysis compared CC-0/1 with CC-2/3 groups regarding liver and spleen scalloping parameters, assessed on pre-operative computed tomography (CT) scan, reviewed for the study. In addition, prognostic factors of severe complications and recurrence-free and overall survivals were explored in the CC-0/1 population. Overall, 129 patients were included (109 CC-0/1, 20 CC-2/3), with 58 (45%) exhibiting scalloping. All patients with splenic scalloping also had a liver one. Scalloping was more frequent (75% vs. 39%), with greater median maximal depth (21 vs. 11 mm) and higher PCI (32 vs. 14) in the CC-2/3 population, but was not predictive of either grade or survival. In CC-0/1 patients, survivals and postoperative complications were not affected by scalloping parameters. Scalloping appeared as a marker of advanced PMP, but was not predictive of grade, severe complications, or long-term outcomes.
PubMed: 36139595
DOI: 10.3390/cancers14184434 -
International Journal of Molecular... Nov 2020The mechanisms by which neoplastic cells disseminate from the primary tumor to metastatic sites, so-called metastatic organotropism, remain poorly understood.... (Review)
Review
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.
Topics: Animals; Biomarkers; Cell Plasticity; Disease Management; Disease Susceptibility; Epithelial-Mesenchymal Transition; Humans; Osteogenesis; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 33266161
DOI: 10.3390/ijms21239120