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Cleveland Clinic Journal of Medicine Sep 2022
Topics: Humans; Pseudomyxoma Peritonei; Ascites; Peritoneal Neoplasms
PubMed: 37907443
DOI: 10.3949/ccjm.89a.21086 -
American Journal of Cancer Research 2023Mucins are a significant extracellular component of neoplastic entities such as pseudomyxoma peritonei and several gastrointestinal adenocarcinomas. Mucinous tumours... (Review)
Review
Mucins are a significant extracellular component of neoplastic entities such as pseudomyxoma peritonei and several gastrointestinal adenocarcinomas. Mucinous tumours present a challenge for systemic treatments due to poor drug penetrance and increased resistance. Therefore, the development of an effective mucolytic therapy has significant therapeutic implications for these tumour types. BromAc is a novel mucolytic agent consisting of bromelain and acetylcysteine. It has demonstrated significant mucolysis and antitumour effects and for several mucinous tumours. It has also exhibited a synergistic potentiation of the effect of several cytotoxic agents on mucinous tumours in preclinical studies. Furthermore, it demonstrates locoregional safety and efficacy in animal and clinical studies. This literature review will summarise the history of BromAc for mucinous tumours, including its conception, preclinical development and , and clinical evidence. The implications of current data and directions for future research are then discussed.
PubMed: 37168359
DOI: No ID Found -
Journal of Family Medicine and Primary... Dec 2020Mucocele of the appendix is a very rare disease entity that often discovered incidentally during surgery. It can result from both non-neoplastic and neoplastic lesions... (Review)
Review
Mucocele of the appendix is a very rare disease entity that often discovered incidentally during surgery. It can result from both non-neoplastic and neoplastic lesions and histopathological examination is needed for confirmation. Failure to make an early preoperative diagnosis may results in its rapture and spillage of mucin contents into the peritoneal cavity leading to a disastrous complication of pseudomyxoma peritonei (PMP) that has a very bad prognosis. A clear pathological terminology and management strategies of appendiceal mucocele (AM) is lacking. This literature review aims to derive detailed information related to clinical significance of AM to avoid complication of PMP and plan appropriately during surgery according to the current evidence. The relevant articles from scientific databases such as Medline, PubMed, Google Scholar were searched and extracted using the keywords "mucocele appendix" "cystadenoma%". Data based on epidemiology, clinical manifestations, complications, pathology, diagnostic work up and management were analyzed and summarized. A meticulous surgical excision is the mainstay of treatment and open surgical approach is still preferred over laparoscopy. Preoperative diagnosis of AM is very imperative as it may harbour neoplasm and can be made utilising the imaging tools like computed tomography and ultrasonography. Primary care physicians can have a crucial role in making early detection and timely referral for appropriate management in order to avoid complications. After appendectomy, 5-year survival rate for the simple AM is 91%-100% but it reduces to 25% for the malignant AM.
PubMed: 33681010
DOI: 10.4103/jfmpc.jfmpc_1547_20 -
Radiologia Brasileira 2022Mucoceles of the appendix are rare and can have quite variable imaging and clinical presentations, sometimes mimicking an adnexal mass. The underlying cause can be...
Mucoceles of the appendix are rare and can have quite variable imaging and clinical presentations, sometimes mimicking an adnexal mass. The underlying cause can be neoplastic or non-neoplastic. The typical imaging appearance of a mucocele of the appendix is that of a cystic structure with a tubular morphology. This structure is defined by having a blind-ending and being contiguous with the cecum. Radiologists should be familiar with key anatomical landmarks and with the various imaging features of mucoceles of the appendix, in order to provide a meaningful differential diagnosis of a lesion in the right lower abdominal quadrant. In addition, a neoplastic mucocele can rupture, resulting in pseudomyxoma peritonei, which will change the prognosis dramatically. Therefore, prompt diagnostic imaging is crucial.
PubMed: 35795599
DOI: 10.1590/0100-3984.2021.0075 -
Indian Journal of Surgical Oncology Jun 2016Neoplasms of the appendix are rare, but because of their unusual presentation and unpredictable biologic behavior, it is important to diagnose them correctly. Mucinous... (Review)
Review
Neoplasms of the appendix are rare, but because of their unusual presentation and unpredictable biologic behavior, it is important to diagnose them correctly. Mucinous tumors account for 58 % of malignant tumors of appendix in SEER database and the remaining are carcinoids. The mucinous appendiceal tumors have a potential to spread to the peritoneum and viscera in the form of gelatinous material with or without neoplastic cells resulting in Pseudomyxoma peritonei. (PMP) PMP is a clinical entity that has a unique biological behavior and can arise from seemingly benign tumors to frankly malignant ones. Several classifications exist for PMP of which Ronnet's classification has been the most popular. In 2010, the WHO proposed a 2 tier classification that classified PMP as either low grade or high grade based on the presence of mucin, cytological and architectural features. According to this classification when the underlying cause for PMP is an appendiceal tumor it is always a mucinous adenocarcinoma rather than a mucocoele or adenoma and these terms should no longer be used. This system of classification helps in predicting the behavior of the tumor and proper treatment strategies. The understanding of the pathogenesis of the disease has also improved with identification of newer biomarkers and molecular genetic alterations. IHC markers CK 20, CDX2 and MUC2 are found to be positive in these tumors in addition to KRAS mutation and loss of heterozygosity in some gene loci. Proper histopathologic classification and predicting the tumor behavior requires a close interaction between the pathologist and the surgeon. The use of the combined modality treatment of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) has led to a 5-year survival ranging from 62.5 % to 100 % for low grade, and 0 %-65 % for high grade disease. This article focuses on the etiopathogenesis, clinical behavior, diagnosis and classification of mucinous tumors of the appendix and pseudomyxoma peritonei.
PubMed: 27065718
DOI: 10.1007/s13193-016-0516-2 -
Surgical Oncology Clinics of North... Jul 2018The early symptoms of appendiceal cancer may mimic the clinical picture of appendicitis. Most patients are diagnosed incidentally during surgical exploration or late... (Review)
Review
The early symptoms of appendiceal cancer may mimic the clinical picture of appendicitis. Most patients are diagnosed incidentally during surgical exploration or late when peritoneal or systemic dissemination has already occurred, as colonoscopy rarely will diagnose an appendiceal cancer. Systemic/extraperitoneal metastases are distinctly unusual for appendiceal mucinous lesions.
Topics: Appendiceal Neoplasms; Combined Modality Therapy; Humans; Peritoneal Neoplasms; Treatment Outcome
PubMed: 29935689
DOI: 10.1016/j.soc.2018.02.007 -
Journal of Obstetrics and Gynaecology... Jun 2022
PubMed: 33679016
DOI: 10.1007/s13224-021-01460-7 -
Orphanet Journal of Rare Diseases Feb 2021Pseudomyxoma peritonei (PMP) is a clinical malignant syndrome mainly originating from the appendix, with an incidence of 2-4 per million people. As a rare disease, an... (Review)
Review
BACKGROUND
Pseudomyxoma peritonei (PMP) is a clinical malignant syndrome mainly originating from the appendix, with an incidence of 2-4 per million people. As a rare disease, an early and accurate diagnosis of PMP is difficult. It was not until the 1980s that the systematic study of this disease was started.
MAIN BODY
As a result of clinical and basic research progress over the last 4 decades, a comprehensive strategy based on cytoreductive surgery (CRS) + hyperthermic intraperitoneal chemotherapy (HIPEC) has been established and proved to be an effective treatment for PMP. Currently, CRS + HIPEC was recommended as the standard treatment for PMP worldwide. There are several consensuses on PMP management, playing an important role in the standardization of CRS + HIPEC. However, controversies exist among consensuses published worldwide. A systematic evaluation of PMP consensuses helps not only to standardize PMP treatment but also to identify existing controversies and point to possible solutions in the future. The controversy underlying the consensus and vice versa promotes the continuous refinement and updating of consensuses and continue to improve PMP management through a gradual and continuous process. In this traditional narrative review, we systemically evaluated the consensuses published by major national and international academic organizations, aiming to get a timely update on the treatment strategies of CRS + HIPEC on PMP.
CONCLUSION
Currently, consensuses have been reached on the following aspects: pathological classification, terminology, preoperative evaluation, eligibility for surgical treatment, maximal tumor debulking, CRS technical details, and severe adverse event classification system. However, controversies still exist regarding the HIPEC regimen, systemic chemotherapy, and early postoperative intraperitoneal chemotherapy.
Topics: Consensus; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 33581733
DOI: 10.1186/s13023-021-01723-6 -
International Journal of Surgery... Jun 2015Mucocoele of the appendix is rarely encountered but consultant general surgical and trainees must be aware of it as a differential diagnosis, due to the nuances... (Review)
Review
INTRODUCTION
Mucocoele of the appendix is rarely encountered but consultant general surgical and trainees must be aware of it as a differential diagnosis, due to the nuances associated with its management. We aimed to provide a comprehensive review of the current literature concerning this rare surgical pathology.
METHODS
Search terms "appendi*", "tumour", "malignancy", "mucino*" and "cystadenoma" were used in combination to identify papers from PubMed. Abstracts and full text were manually reviewed to identify suitable papers.
RESULTS
Full search results included 311 articles. Review of titles and abstracts led to further full text review of 46 articles. Of these 30 were selected for inclusion based on relevance, adequate sample size and recent publication date.
DISCUSSION
Mucocoele of the appendix describes dilatation with associated luminal mucin and can result from benign and malignant processes. It contributes 0.2-0.7% of all appendiceal pathologies. The most common presenting symptoms are abdominal pain and a palpable mass in the right iliac fossa. Computed tomography of the abdomen and pelvis is key in facilitating diagnosis, although CEA and CA19-9 also have a role. The major complication of malignant causes of mucocoele is progression to pseudomyxoma peritonei. Treatment is surgical with or without chemotherapy depending on the underlying cause. Prognosis depends on aetiology.
CONCLUSION
Mucocoele of the appendix is a rare diagnosis. However, given the possibility of neoplastic peritoneal dissemination, it should be considered as a diagnosis, especially in older females with non-specific symptoms similar to appendicitis.
Topics: Abdominal Pain; Appendiceal Neoplasms; Appendicitis; Cecal Diseases; Cystadenoma, Mucinous; Diagnosis, Differential; Female; Humans; Mucocele; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Tomography, X-Ray Computed
PubMed: 25917270
DOI: 10.1016/j.ijsu.2015.04.052 -
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