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Colorectal Disease : the Official... Sep 2021Appendiceal pseudomyxoma peritonei (PMP) is a rare entity, with recurrence rates up to 26% despite optimal cytoreductive surgery (CRS) and hyperthermic intraperitoneal...
AIM
Appendiceal pseudomyxoma peritonei (PMP) is a rare entity, with recurrence rates up to 26% despite optimal cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Evidence specific to PMP originating from non-infiltrative appendiceal mucinous neoplasms (low grade - LAMN and high grade - HAMN) is lacking. The aim of this study was to identify patterns of recurrence and predictive factors for patients appropriate for iterative surgery.
METHOD
A bi-institutional retrospective analysis was performed on patients undergoing complete cytoreduction and HIPEC for PMP derived from perforated LAMN or HAMN. Multivariate logistic regression was performed to identify independent predictors for re-do CRS. Five-year overall survival (OS) was stratified according to surgical intervention, and 5-year disease-free survival (DFS) was stratified according to histological PMP grade. Cox regression analysis was performed to identify independent predictors for OS and DFS.
RESULTS
Sixty of 239 (25.1%) patients developed peritoneal recurrence between 2007 and 2020. The median time to recurrence was 20.7 months. The risk of disease recurrence was highest with high-grade PMP (P <0.001) and increasing PCI (P <0.001). Patients with high-grade histology from their index procedure and aged over 60 years were less likely to be offered iterative surgery on multivariate analysis. Patients who underwent iterative CRS and HIPEC had a 5-year survival of 100%.
CONCLUSION
Iterative CRS and HIPEC is feasible in selected patients with recurrent PMP, displaying good oncological outcomes. Age, index histology and level of abdominal quadrant involvement are predictive of proceeding to re-do surgery.
Topics: Aged; Appendiceal Neoplasms; Combined Modality Therapy; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Neoplasm Recurrence, Local; Percutaneous Coronary Intervention; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Retrospective Studies
PubMed: 34157209
DOI: 10.1111/codi.15778 -
Abdominal Radiology (New York) Jul 2017
Review
Topics: Contrast Media; Diagnosis, Differential; Humans; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Tomography, X-Ray Computed
PubMed: 28255672
DOI: 10.1007/s00261-017-1092-9 -
Journal of Medical Imaging and... Oct 2018
Review
Topics: Humans; Liver; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Sensitivity and Specificity; Tomography, X-Ray Computed; Ultrasonography
PubMed: 30309123
DOI: 10.1111/1754-9485.47_12784 -
Chirurgie (Heidelberg, Germany) Dec 2022The term pseudomyxoma peritonei (PMP) describes a clinical syndrome characterized by the presence of gelatinous intraperitoneal accumulation of mucus. It mostly... (Review)
Review
BACKGROUND
The term pseudomyxoma peritonei (PMP) describes a clinical syndrome characterized by the presence of gelatinous intraperitoneal accumulation of mucus. It mostly originates from a mucocele of the vermiform appendix. Affected patients are often asymptomatic for a long time. Because of its indolent nature it is usually diagnosed at an advanced stage. Clinical presentation is determined by the dissemination of the tumor.
METHOD
A search and analysis of the current literature were carried out.
RESULTS
Based on the morphological characteristics PMP subtypes with various malignant potential can be differentiated. The prognosis depends on the histopathological differentiation and the clinical stage. The treatment spectrum varies from laparoscopic appendectomy to complete cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC).
CONCLUSION
Due to the rarity of PMP there are no prospective randomized studies. Therefore, there is still controversy regarding the best stage-dependent treatment strategy. This review article attempts to clarify the optimal management of mucinous neoplasms of the appendix and PMP taking the clinical presentation and the histological differentiation into consideration.
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Cytoreduction Surgical Procedures; Hyperthermia, Induced; Neoplasms, Glandular and Epithelial
PubMed: 36097078
DOI: 10.1007/s00104-022-01696-7 -
American Journal of Clinical Oncology May 2022Pseudomyxoma peritonei is an infrequent solid tumor in clinical practice. The low morbidity and deficient understanding of this mucus-secreting malignant disease... (Review)
Review
Pseudomyxoma peritonei is an infrequent solid tumor in clinical practice. The low morbidity and deficient understanding of this mucus-secreting malignant disease increase the risks of delayed identification or uncontrollable deterioration. In quite a lot cases, patients go through complete cytoreduction surgery and hyperthermic intraperitoneal chemotherapy could receive a long time survival over 5 years. But the recurrence rate is also hard to overlook. Unlike other types of cancer, the standard treatment for this considerable groups has not been confirmed yet. With the advanced medical progression, studies have been carrying out based on pathogenesis, biological characters, and mutated gene location. All but a few get statistical survival benefits, let alone the breaking progress on research or therapeutic practice in the field. We try to give a comprehensive exposition of pseudomyxoma peritonei around the epidemiology, radiologic features, clinical manifestation, present treatment and promising schemes, hoping to arise much attention and reflection on the feasible solutions, especially for the recrudescent part.
Topics: Combined Modality Therapy; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Neoplasm Recurrence, Local; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Retrospective Studies
PubMed: 35446281
DOI: 10.1097/COC.0000000000000911 -
Abdominal Radiology (New York) May 2019Recent advances, specifically cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), offer advantages compared to the traditional therapeutic... (Review)
Review
Recent advances, specifically cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), offer advantages compared to the traditional therapeutic approach of systemic chemotherapy in the treatment of peritoneal carcinomatosis from mucinous appendiceal neoplasms (MAN). This review provides an up-to-date, comprehensive summary of the histologic classification of MAN, reviews common imaging findings of mucoceles and pseudomyxoma peritonei, and describes the radiologist's role in the multidisciplinary care team in quantifying disease and in helping select patients for definitive surgery.
Topics: Adenocarcinoma, Mucinous; Appendectomy; Appendiceal Neoplasms; Cytoreduction Surgical Procedures; Disease Progression; Humans; Hyperthermia, Induced; Neoplasm Grading; Neoplasm Staging; Peritoneal Neoplasms; Prognosis; Pseudomyxoma Peritonei
PubMed: 30610247
DOI: 10.1007/s00261-018-01888-y -
Surgical Pathology Clinics Jun 2022This review provides an overview of the pathology of selected benign and malignant lesions of the female peritoneum and their often-encountered differential diagnoses.... (Review)
Review
This review provides an overview of the pathology of selected benign and malignant lesions of the female peritoneum and their often-encountered differential diagnoses. It includes endometriosis and its related lesions, endosalpingiosis, pseudomyxoma peritonei (PMP) and related ovarian/appendiceal pathology, and malignant and benign mesothelial tumors. The current terminology associated with PMP is also discussed.
Topics: Appendiceal Neoplasms; Female; Humans; Ovarian Neoplasms; Peritoneal Neoplasms; Peritoneum; Pseudomyxoma Peritonei
PubMed: 35715161
DOI: 10.1016/j.path.2022.02.005 -
Current Oncology (Toronto, Ont.) May 2022Pseudomyxoma peritonei (PMP) is a rare clinical syndrome. It originates from neoplasms of the appendix and leads to the formation of peritoneal implants and the... (Review)
Review
Pseudomyxoma peritonei (PMP) is a rare clinical syndrome. It originates from neoplasms of the appendix and leads to the formation of peritoneal implants and the accumulation of mucinous ascites. PMP represents a spectrum of low to high-grade disease. Despite aggressive management, many PMP patients recur, leading to debilitating symptoms and few treatment options. Therefore, scientists have continued to look for ways to improve treatment and further understand disease pathogenesis. Microorganisms were previously hypothesized to play a role in PMP progression and development. Hence, antibacterial treatment was suggested by some authors, but the data were limited. In this paper, we review the current data on the role of bacteria in PMP, discuss the significance, and suggest possible solutions to the inherent challenges in these studies. Given the limitations of the discussed studies, we remain skeptical about introducing novel antibacterial treatment into clinical practice at this time; however, the available data are valuable and indicate that more research into the molecular mechanisms of PMP is needed.
Topics: Appendiceal Neoplasms; Appendix; Humans; Neoplasm Recurrence, Local; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 35621679
DOI: 10.3390/curroncol29050289 -
Cancer Management and Research 2021Excessive mucus secretion is the most prominent feature of pseudomyxoma peritonei (PMP), which often leads to significant increase in abdominal circumference,... (Review)
Review
Excessive mucus secretion is the most prominent feature of pseudomyxoma peritonei (PMP), which often leads to significant increase in abdominal circumference, intractable abdominal pain, progressive intestinal obstruction, abdominal organ adhesions, and cachexia. Excessive mucus secretion is also the main cause of death. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is the recommended treatment for PMP. However, recurrence is frequently observed even after CRS and HIPEC, presenting similar clinical manifestations. Mucin 2 (MUC2) is the main type of mucin in PMP and plays a key role in the progressive sclerosis of mucus. To comprehensively demonstrate the biosynthetic process and molecular features of MUC2 and to provide new directions for the development of PMP mucolytic strategies, this review systematically summarizes the molecular biology of MUC2, including gene structure, transcription, translation, post-translational modification, tertiary structure, and factors regulating mucus viscoelasticity. The results show that MUC2 is a highly glycosylated protein, with glycan accounts for 80% to 90% of the dry weight. The assembly pattern of MUC2 is highly complicated, presenting a bead-like filament. Salt concentration, pH, mucin concentration and trefoil factor family may contribute to the increase in mucus viscoelasticity and sclerosis, which could be used to develop drugs to soften or even dissolve mucus in the future.
PubMed: 34703312
DOI: 10.2147/CMAR.S324982 -
Indian Journal of Pathology &... 2022Low-grade appendiceal mucinous neoplasms (LAMN) of the appendix have bland cytological features and well-differentiated morphology. Despite this, they may cause a...
BACKGROUND
Low-grade appendiceal mucinous neoplasms (LAMN) of the appendix have bland cytological features and well-differentiated morphology. Despite this, they may cause a pseudomyxoma peritonei (PMP) disease characterized by mucinous deposits in the peritoneal cavity and may exhibit malignant behavior.
AIMS AND OBJECTIVES
In this study, we evaluated the clinical course and histopathological findings of LAMN. The rate of progression to PMP, factors affecting its development, and the clinical course of cases with PMP were investigated.
MATERIALS AND METHODS
Twelve thousand and forty-seven cases who underwent appendectomy were reviewed, and 71 mucinous lesions cases whose slides are in our archive were included in the study. Histopathological findings were re-evaluated. Morphological findings that guide the differential diagnosis, the clinical course of the patients, and factors affecting PMP development were determined.
RESULTS
The cases were divided into group 1 non-neoplastic (9.9%), group 2 benign (18.3%), group 3 LAMN (60.6), and group 4 malignant neoplasms (11.3%). Age, gender, appendix diameter, gross appearance, epithelial characteristics, and presence of microcalcification were significantly different between the patient groups. The presence of mucin in the ileocecal region was found to be significant in the development of PMP. It was observed that the additional procedure performed after the appendectomy did not prevent a recurrence.
CONCLUSION
LAMNs are lesions with characteristic findings and different behaviors. The only method to distinguish from the lesions included in the differential diagnosis is to interpret the histopathological findings correctly. Additional operations after appendectomy do not contribute to preventing recurrence.
Topics: Appendiceal Neoplasms; Diagnosis, Differential; Humans; Neoplasms, Glandular and Epithelial; Peritoneal Neoplasms; Prognosis; Pseudomyxoma Peritonei
PubMed: 35900483
DOI: 10.4103/ijpm.ijpm_827_21