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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 -
JAAPA : Official Journal of the... Aug 2021Hyperthermic intraperitoneal chemotherapy (HIPEC) has been used for decades as a treatment for disseminated abdominal malignancies, including pseudomyxoma peritonei and... (Review)
Review
Hyperthermic intraperitoneal chemotherapy (HIPEC) has been used for decades as a treatment for disseminated abdominal malignancies, including pseudomyxoma peritonei and peritoneal mesothelioma. HIPEC had been used to treat recurrent ovarian cancer only when curative options were lacking, but new data indicate that it may be suitable as a primary treatment for patients with late-stage epithelial ovarian cancer, increasing the recurrence-free time interval and improving quality of life. However, treatment can be challenging because of patient trauma from surgery, combined with the toxicity and high temperature of the chemotherapeutic agents. This article reviews HIPEC and its targeted intraoperative and postoperative management.
Topics: Carcinoma, Ovarian Epithelial; Combined Modality Therapy; Cytoreduction Surgical Procedures; Female; Humans; Hyperthermia, Induced; Hyperthermic Intraperitoneal Chemotherapy; Neoplasm Recurrence, Local; Ovarian Neoplasms; Quality of Life
PubMed: 34320542
DOI: 10.1097/01.JAA.0000735800.98948.bb -
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 -
Gastroenterology Clinics of North... Dec 2019"The intestinal transplantation is reserved for patients with life-threatening complications of permanent intestinal failure or underlying gastrointestinal disease. The... (Review)
Review
"The intestinal transplantation is reserved for patients with life-threatening complications of permanent intestinal failure or underlying gastrointestinal disease. The choice of the allograft for a particular patient depends on several factors and the presence of concurrent organ failure, and availability of the donor organs, and specialized care. Combined liver and intestinal transplant allows for patients who have parenteral nutrition-associated liver disease a possibility of improved quality of life and nutrition as well as survival. Intestinal transplantation has made giant strides over the past few decades to the present era where current graft survivals are comparable with other solid organ transplants."
Topics: Abdominal Wall; Allografts; Humans; Intestines; Liver Transplantation; Malabsorption Syndromes; Parenteral Nutrition; Pseudomyxoma Peritonei; Quality of Life; Viscera
PubMed: 31668184
DOI: 10.1016/j.gtc.2019.08.010 -
Cancers Apr 2024Pseudomyxoma peritonei (PMP) is a rare, progressive, slowly growing, inadequately understood neoplasm with a 5-year progression-free survival rate of as low as 48%. It... (Review)
Review
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare, progressive, slowly growing, inadequately understood neoplasm with a 5-year progression-free survival rate of as low as 48%. It is characterized by varying degrees of malignancy and the production of mucinous and gelatinous structures. Typically, the development of pseudomyxoma peritonei is associated with the rupture of appendiceal mucinous tumors and other gastrointestinal or ovarian mucinous tumors. The goal of our literature review was to identify various aspects that characterize the ovarian causes of pseudomyxoma peritonei.
MATERIALS AND METHODS
The authors performed an extensive literature search between 1 February 2024 and 2 March 2024 on the following databases: Pubmed, Scopus, Oxford Journals, and Reaxys, and the findings were summarized into seven main clinical and paraclinical situations.
RESULTS
According to our research, the main instances in which pseudomyxoma peritonei can be triggered by an ovarian cause are the following: (1) mucinous cystadenoma; (2) mucinous ovarian cancer; (3) colon cancer with ovarian metastasis; (4) malignant transformation of an ovarian primary mature cystic teratoma; (5) appendiceal mucocele with peritoneal dissemination mimicking an ovarian tumor with peritoneal carcinomatosis; (6) mucinous borderline tumor developing inside an ovarian teratoma; and (7) the association between a mucinous bilateral ovarian cancer and a colonic tumor.
CONCLUSIONS
In our study, we aimed to provide a comprehensive overview of the ovarian causes of pseudomyxoma peritonei, including its epidemiology, imagery characteristics, symptoms, current treatment, and promising future therapies, in the hopes of finding feasible solutions, as a lack of understanding of this mucus-secreting malignant disease increases the risk of delayed diagnosis or uncontrolled deterioration.
PubMed: 38672528
DOI: 10.3390/cancers16081446 -
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 -
Cancers Apr 2024Pseudomyxoma peritonei (PMP) is a rare disease characterized by extensive peritoneal implantation and mass secretion of mucus after primary mucinous tumors of the... (Review)
Review
Pseudomyxoma peritonei (PMP) is a rare disease characterized by extensive peritoneal implantation and mass secretion of mucus after primary mucinous tumors of the appendix or other organ ruptures. Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is currently the preferred treatment, with excellent efficacy and safety, and is associated with breakthrough progress in long-term disease control and prolonged survival. However, the high recurrence rate of PMP is the key challenge in its treatment, which limits the clinical application of multiple rounds of CRS-HIPEC and does not benefit from conventional systemic chemotherapy. Therefore, the development of alternative therapies for patients with refractory or relapsing PMP is critical. The literature related to PMP research progress and treatment was searched in the Web of Science, PubMed, and Google Scholar databases, and a literature review was conducted. The overview of the biological research, treatment status, potential therapeutic strategies, current research limitations, and future directions associated with PMP are presented, focuses on CRS-HIPEC therapy and alternative or combination therapy strategies, and emphasizes the clinical transformation prospects of potential therapeutic strategies such as mucolytic agents and targeted therapy. It provides a theoretical reference for the treatment of PMP and the main directions for future research.
PubMed: 38611084
DOI: 10.3390/cancers16071406 -
Chirurgie (Heidelberg, Germany) Oct 2023Disseminated peritoneal seeding of mucin-forming tumors is a rare malignant disease with variable prognosis. Histomorphological criteria are instrumental in the... (Review)
Review
INTRODUCTION
Disseminated peritoneal seeding of mucin-forming tumors is a rare malignant disease with variable prognosis. Histomorphological criteria are instrumental in the prognostic assessment. The past 10 years have led to a standardization of nomenclature and subsequently to the establishment of therapeutic standards. This article aims to provide the current status of the pathological classification, staging, and grading.
METHOD AND MATERIAL
Selective literature search in PubMed and Medline RESULTS: The vast majority of disseminated peritoneal mucinous diseases that correspond to the clinical presentation of pseudomyxoma peritonei (PMP) arise from mucinous tumors of the vermiform appendix. Here are to be distinguished: 1) low-grade appendiceal mucinous neoplasms (LAMN), 2) (very rare) high-grade appendiceal mucinous neoplasms (HAMN), 3) mucinous adenocarcinoma without signet ring cells (G2) and 4) mucinous adenocarcinoma with signet ring cells or signet ring cell carcinoma (G3). Other primary tumors only rarely induce PMP. Terms such as mucocele or mucinous cystadenoma of the appendix correspond to LAMN and should no longer be used. Prognostic distinctions are further made between low-grade PMP, which usually arises from LAMN, and the prognostically less favorable high-grade PMP, which usually arises from mucinous/signet ring cell adenocarcinoma or the rare HAMN. Disseminated peritoneal mucinous disease/PMP must then be further distinguished from prognostically excellent local mucin formation of the peri-appendix region.
DISCUSSION
The currently valid nomenclature, as it has emerged from consensus meetings and in parts has also found its way into the current WHO 2019, has significantly contributed to the fact that the prognosis of patients today can be better estimated and effective forms of treatment could be developed.
PubMed: 37418023
DOI: 10.1007/s00104-023-01926-6 -
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 -
The Journal of Surgical Research Jun 2022The pathologic classification of pseudomyxoma peritonei is controversial. This study aimed to standardize the histopathological evaluation of pseudomyxoma peritonei and...
INTRODUCTION
The pathologic classification of pseudomyxoma peritonei is controversial. This study aimed to standardize the histopathological evaluation of pseudomyxoma peritonei and identify the clinicopathological factors associated with survival.
METHODS
A pathologic review was performed to systematize the pathology report and verify the relationship between clinical features and survival. Terminology was based on the World Health Organization and Peritoneal Surface Oncology Group International definitions. Preoperative serum levels of carcinoembryonic antigen, CA19-9, and CA-125 were evaluated to determine their association with overall survival (OS) and ability to predict CC0-1 cytoreduction.
RESULTS
Among 109 patients with carcinomas resulting from primary appendiceal neoplasms, 72 had pseudomyxoma peritonei of appendiceal origin and underwent debulking surgery. CC0-1 cytoreduction and CC2-3 cytoreduction were achieved in 61% and 39% of patients, respectively. Patients in the CC0-1 and CC2-3 groups had an OS of 122.80 and 32.92 mo, respectively. The histologic grade was associated with CC0-1 cytoreduction; however, it did not influence OS. Patients with CC0-1 cytoreduction, acellular mucin, and low-grade lesions had better disease-free survival. Higher preoperative CA19-9 levels were associated with poor OS. Normal carcinoembryonic antigen values were associated with 100% sensitivity for predicting CC0-1. CA19-9 levels of 625 U/mL were associated with a low possibility of predicting CC0-1.
CONCLUSIONS
Histologic grades are associated with disease-free survival when CC0-1 cytoreduction is achieved. Normal preoperative CA19-9 levels were associated with a better OS. CC0-1 cytoreduction is the main determinant of longer survival.
Topics: Appendiceal Neoplasms; Biomarkers, Tumor; CA-19-9 Antigen; Carcinoembryonic Antigen; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Peritoneal Neoplasms; Prognosis; Pseudomyxoma Peritonei
PubMed: 35123285
DOI: 10.1016/j.jss.2022.01.001