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European Journal of Surgical Oncology :... Jan 2021Pseudomyxoma Peritonei (PMP) and Peritoneal Mesothelioma (PM) are both rare peritoneal malignancies. Currently, affected patients may be treated with Cytoreductive... (Review)
Review
Pseudomyxoma Peritonei (PMP) and Peritoneal Mesothelioma (PM) are both rare peritoneal malignancies. Currently, affected patients may be treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy offering long-term survival or even cure in selected patients. However, many issues regarding the optimal treatment strategy are currently under debate. To aid physicians involved in the treatment of these patients in clinical decision making, the PSOGI executive committee proposed to create a consensus statement on PMP and PM. This manuscript describes the methodology of the consensus process. The Delphi technique is a reliable method for attaining consensus on a topic that lacks scientific evidence through multiple voting rounds which feeds back responses to the participants in between rounds. The GRADE system provides a structured framework for presenting and grading the available evidence. Separate questionnaires were created for PMP and PM and sent during two voting rounds to 80 and 38 experts, respectively. A consensus threshold of 51.0% was chosen. After the second round, consensus was reached on 92.9%-100.0% of the questions. The results were presented and discussed in the plenary session at the PSOGI 2018 international meeting in Paris. A third round for the remaining issues is currently in progress. In conclusion, using the Delphi technique and GRADE methodology, consensus was reached in many issues regarding the treatment of PM and PMP amongst an international panel of experts. The main results will be published in the near future.
Topics: Consensus; Cytoreduction Surgical Procedures; Delphi Technique; Humans; Hyperthermic Intraperitoneal Chemotherapy; Mesothelioma; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Terminology as Topic
PubMed: 30954350
DOI: 10.1016/j.ejso.2019.03.012 -
Journal of Gastrointestinal Cancer Dec 2019
Review
Topics: Appendiceal Neoplasms; Appendix; Biopsy; Colectomy; Combined Modality Therapy; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Male; Middle Aged; Mitomycin; Peritoneal Neoplasms; Peritoneum; Pseudomyxoma Peritonei; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 30618002
DOI: 10.1007/s12029-018-00192-8 -
International Cancer Conference Journal Oct 2023Both pseudomyxoma peritonei and Morgagni hernias in adults are rare clinical conditions. A 70-year-old woman who was diagnosed with pseudomyxoma peritonei with Morgagni...
Both pseudomyxoma peritonei and Morgagni hernias in adults are rare clinical conditions. A 70-year-old woman who was diagnosed with pseudomyxoma peritonei with Morgagni hernia underwent cytoreductive surgery and primary repair. Pseudomyxoma peritonei causes increased intra-abdominal pressure that may lead to acquired congenital diaphragmatic hernia when there is a local fragility in the diaphragmatic musculature. Parietal peritonectomy of the right diaphragmatic peritoneum can safely remove the hernia sac. The high rate of infections associated with cytoreductive surgery causes hesitation for concurrent mesh repair for Morgagni hernia. This is the first report of pseudomyxoma peritonei with Morgagni hernia. Cytoreductive surgery including parietal peritonectomy of the right diaphragmatic peritoneum plus primary repair of hernial defect was performed safely and successfully, which achieved positive short-term results for patients with pseudomyxoma peritonei-associated Morgagni hernia.
PubMed: 37577349
DOI: 10.1007/s13691-023-00614-w -
European Journal of Surgical Oncology :... Oct 2022Pseudomyxoma peritonei (PMP) is an extremely rare condition. Information regarding the disease burden of PMP in developing countries is limited. This study aimed to...
BACKGROUND
Pseudomyxoma peritonei (PMP) is an extremely rare condition. Information regarding the disease burden of PMP in developing countries is limited. This study aimed to determine the epidemiology of PMP in China.
METHODS
PMP data were extracted from the national databases of Urban Basic Medical Insurance. All cases were identified using the International Classification of Diseases (ICD) codes and Chinese diagnostic terms. The national prevalence from 2012 to 2016 and incidence in 2016 were estimated.
RESULTS
In total, 153 patients with PMP were identified. The crude prevalence of PMP in 2016 was 2.47 (95% confidence interval [CI] 1.71 to 3.23) per million person-year, with a higher prevalence in females than males. Prevalence increased with age, with the first peak in those aged 15-29 years and the highest in those aged >80 years. The crude incidence of PMP in 2016 was 1.19 (95% CI 0.59 to 1.78) per million person-years. Similar to the prevalence, the rates were higher in women than in men. The incidence also increased with age, with the highest prevalence in those aged >80 years. Besides, the most frequent comorbidities before and after the first diagnosis of PMP were unspecified secondary malignancies and malignancies of unspecified sites, followed by abdominal malignant tumours.
CONCLUSIONS
The rate of PMP was lower in mainland China than in European countries and increased with advancing age. Women were more likely to have PMP than men. Furthermore, an insufficient understanding of this rare disease presents a major challenge in accurately evaluating the disease burden.
Topics: Male; Humans; Female; Adolescent; Young Adult; Adult; Aged, 80 and over; Pseudomyxoma Peritonei; Incidence; Prevalence; Retrospective Studies; China; Peritoneal Neoplasms
PubMed: 35764460
DOI: 10.1016/j.ejso.2022.06.013 -
Journal of Visualized Experiments : JoVE Dec 2022Pseudomyxoma peritonei (PMP) is a rare condition that results from the dissemination of a mucinous primary tumor and the resultant accumulation of mucin-secreting tumor...
Pseudomyxoma peritonei (PMP) is a rare condition that results from the dissemination of a mucinous primary tumor and the resultant accumulation of mucin-secreting tumor cells in the peritoneal cavity. PMP can arise from various types of cancers, including appendiceal, ovarian, and colorectal, though appendiceal neoplasms are by far the most common etiology. PMP is challenging to study due to its (1) rarity, (2) limited murine models, and (3) mucinous, acellular histology. The method presented here allows real-time visualization and interrogation of these tumor types using patient-derived ex vivo organotypic slices in a preparation where the tumor microenvironment (TME) remains intact. In this protocol, we first describe the preparation of tumor slices using a vibratome and subsequent long-term culture. Second, we describe confocal imaging of tumor slices and how to monitor functional readouts of viability, calcium imaging, and local proliferation. In short, slices are loaded with imaging dyes and are placed in an imaging chamber that can be mounted onto a confocal microscope. Time-lapse videos and confocal images are used to assess the initial viability and cellular functionality. This procedure also explores translational cellular movement, and paracrine signaling interactions in the TME. Lastly, we describe a dissociation protocol for tumor slices to be used for flow cytometry analysis. Quantitative flow cytometry analysis can be used for bench-to-bedside therapeutic testing to determine changes occurring within the immune landscape and epithelial cell content.
Topics: Female; Humans; Animals; Mice; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Appendiceal Neoplasms; Ovary; Tumor Microenvironment
PubMed: 36571414
DOI: 10.3791/64620 -
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 -
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 -
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 -
BMC Cancer Jan 2023To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 199 (CA199) and CA125 in serum and ascites of appendiceal pseudomyxoma peritonei (PMP)...
BACKGROUND
To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 199 (CA199) and CA125 in serum and ascites of appendiceal pseudomyxoma peritonei (PMP) patients relative to their diagnostic and predictive value.
METHODS
The study comprised 183 patients with pathologically confirmed appendiceal PMP, enrolled from May 2012 to June 2020, in Aerospace Center Hospital. Serum and ascites tumor markers were obtained, and their diagnostic values were compared by receiver operating characteristic (ROC) curves. The prognostic factors of appendiceal PMP with different pathologic subgroups were calculated by univariate and multivariate Cox proportional hazard regression models.
RESULTS
There were significant differences between the numbers of patients with positive CEA and CA199 in serum vs. ascites: p = 0.034 in CEA and p = 0.006 in CA199, respectively. The sensitivities with optimal cut-off values for ascites markers of CEA, CA199 and CA125 were 83.5%, 88.9% and 72.6%, respectively. CEA in ascites showed significant difference in the diagnosis of appendiceal PMP (p = 0.000); the areas under the ROC curves (AUROCs) and specificity were 0.725, 70.7%, respectively. Univariate analysis showed that the higher the ascites tumor markers, the poorer the survival (p = 0.014). Multivariate analysis indicated that completeness of cytoreduction (CCR), ascites CEA and pathological grade were independent risk factors for overall survival (OS).
CONCLUSION
CEA in ascites can be used to help specify the origin of PMP. Furthermore, elevation of ascites CEA, high pathological grade and incomplete cytoreduction predicted poor prognosis of appendiceal PMP.
Topics: Humans; Pseudomyxoma Peritonei; Prognosis; Carcinoembryonic Antigen; Biomarkers, Tumor; Peritoneal Neoplasms; Ascites; Appendiceal Neoplasms; CA-125 Antigen
PubMed: 36703100
DOI: 10.1186/s12885-023-10545-7 -
Journal of Nepal Health Research Council Dec 2021A 53-year-old lady presented with post-menopausal bleeding (spotting) for two days and abdominal pain for three months. CT scan revealed bilateral adnexal mass and...
A 53-year-old lady presented with post-menopausal bleeding (spotting) for two days and abdominal pain for three months. CT scan revealed bilateral adnexal mass and ascites; and CEA and CA19.9 was raised. Histopathology of the resected tissues revealed mucinous carcinoma of bilateral ovaries and colon with omental metastasis. Generally, pseudomyxoma peritonei arises from primary ovarian or appendiceal adenomas or adenocarcinomas. Keywords: Adenocarcinomas; ascites; metastases; mucinous carcinoma; pseudomyxoma peritonei.
Topics: Adenocarcinoma, Mucinous; Appendiceal Neoplasms; Female; Humans; Middle Aged; Nepal; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 35140447
DOI: 10.33314/jnhrc.v19i3.3540