-
Cancers Jun 2023A combination of bromelain and acetylcysteine, BromAc, is an efficient intraperitoneal mucolytic for thick mucus secreted in pseudomyxoma peritonei (PMP). Patients with...
A combination of bromelain and acetylcysteine, BromAc, is an efficient intraperitoneal mucolytic for thick mucus secreted in pseudomyxoma peritonei (PMP). Patients with PMP quite often undergo colon anastomosis. Hence, we investigated the effect of the intraperitoneal delivery of BromAc on colon-anastomosis healing in a rat model. Sixteen Wistar rats were divided into two groups (N = 8). The controls received intraperitoneal saline after anastomosis, whilst the other group received BromAc. They were monitored for body-weight and general health parameters. Half the rats in each group (N = 4) were culled at 4 or 13 days post-surgery for assessment. The healing process of the tissues was assessed by burst pressure and collagen density with histology to assess the integrity of the internal organs. The results indicated that there was a similar pattern of weight fluctuation during the experiment, although the rats treated with the BromAc showed slightly greater weight loss during the first 4 days. Although the burst pressure was similar in both groups, the BromAc group at day 13 showed a slightly higher burst pressure, which was complemented by a higher collagen density (albeit not statistically significant). The histology of the internal organs was comparable to those of the controls. This study indicates that the intraperitoneal delivery of BromAc in a rat model does not interfere with the healing process of colonic anastomosis.
PubMed: 37444430
DOI: 10.3390/cancers15133321 -
Cureus Jun 2023An appendiceal mucocele is a rare disease characterized by the dilation of the appendix lumen with mucus accumulation. Although this disease is often found incidentally...
An appendiceal mucocele is a rare disease characterized by the dilation of the appendix lumen with mucus accumulation. Although this disease is often found incidentally during appendectomy, it is crucial to differentiate it from acute appendicitis preoperatively to select adequate surgical management. We present a case of a 31-year-old male, medically free, with right-sided abdominal pain associated with nausea and vomiting. He was diagnosed with appendiceal mucocele and underwent laparoscopic appendectomy. The absence of a distinct clinical presentation and biochemical parameters necessitates a comprehensive and collaborative diagnostic approach for mucocele of the appendix. Achieving an accurate diagnosis prior to surgery is imperative to ensure the appropriate surgical technique is chosen, thereby minimizing the risk of serious intraoperative and postoperative complications such as pseudomyxoma peritonei.
PubMed: 37431343
DOI: 10.7759/cureus.40168 -
World Journal of Gastrointestinal... Jun 2023Pseudomyxoma peritonei (PMP) is a rare peritoneal malignant tumor syndrome. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is its standard...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare peritoneal malignant tumor syndrome. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is its standard treatment. However, there are few studies and insufficient evidence regarding systemic chemotherapy of advanced PMP. Regimens for colorectal cancer are often used clinically, but there is no uniform standard for late-stage treatment.
AIM
To determine if bevacizumab combined with cyclophosphamide and oxaliplatin (Bev+CTX+OXA) is effective for treatment of advanced PMP. The primary study endpoint was progression-free survival (PFS).
METHODS
Retrospective analysis was conducted on the clinical data of patients with advanced PMP who received Bev+CTX+OXA regimen (bevacizumab 7.5 mg/kg ivgtt d1, oxaliplatin 130 mg/m ivgtt d1 and cyclophosphamide 500 mg/m ivgtt d1, q3w) in our center from December 2015 to December 2020. Objective response rate (ORR), disease control rate (DCR) and incidence of adverse events were evaluated. PFS was followed up. Kaplan-Meier method was used to draw survival curve, and log-rank test was used for comparison between groups. Multivariate Cox proportional hazards regression model was used to analyze the independent influencing factors of PFS.
RESULTS
A total of 32 patients were enrolled. After 2 cycles, the ORR and DCR were 3.1% and 93.7%, respectively. The median follow-up time was 7.5 mo. During the follow-up period, 14 patients (43.8%) had disease progression, and the median PFS was 8.9 mo. Stratified analysis showed that the PFS of patients with a preoperative increase in CA125 (8.9 2.1, = 0.022) and a completeness of cytoreduction score of 2-3 (8.9 5.0, = 0.043) was significantly longer than that of the control group. Multivariate analysis showed that a preoperative increase in CA125 was an independent prognostic factor for PFS (HR = 0.245, 95%CI: 0.066-0.904, = 0.035).
CONCLUSION
Our retrospective assessment confirmed that the Bev+CTX+OXA regimen is effective in second- or posterior-line treatment of advanced PMP and that adverse reactions can be tolerated. A preoperative increase in CA125 is an independent prognostic factor of PFS.
PubMed: 37405093
DOI: 10.4240/wjgs.v15.i6.1149 -
Insights Into Imaging Jul 2023Peritoneal malignancies represent a diagnostic challenge for abdominal radiologists, oncologists, surgeons and pathologists in multidisciplinary teams, who must address... (Review)
Review
Peritoneal malignancies represent a diagnostic challenge for abdominal radiologists, oncologists, surgeons and pathologists in multidisciplinary teams, who must address their differential diagnosis, staging and treatment. In this article, we explain the pathophysiology of these processes and lay out the role of different imaging techniques in their evaluation. Then, we review the clinical and epidemiological aspects, the main radiological features and the therapeutic approaches for each primary and secondary peritoneal neoplasm, with surgical and pathological correlation. We further describe other rare peritoneal tumors of uncertain origin and a variety of entities that may mimic peritoneal malignancy. Finally, we summarize the key imaging findings of each peritoneal neoplasm to facilitate an accurate differential diagnosis that may impact patient management.Clinical relevance statementImaging plays an essential role in the evaluation of peritoneal malignancies, assessing their extension, detecting unfavorable sites of involvement and facilitating an accurate differential diagnosis, helping to choose the best therapeutic approach.
PubMed: 37395913
DOI: 10.1186/s13244-023-01417-6 -
Radiology Case Reports Aug 2023We report a case of ruptured low-grade appendiceal mucinous neoplasm with an impressive toy puffer ball-like appearance on magnetic resonance imaging. A 79-year-old...
We report a case of ruptured low-grade appendiceal mucinous neoplasm with an impressive toy puffer ball-like appearance on magnetic resonance imaging. A 79-year-old woman with lower abdominal pain underwent computed tomography scanning, revealing a 6-cm mass in the right lower abdomen. T2-weighted images showed a radial low-signal structure in the central area of the mass, which was presumed to be fibrotic. Pathology confirmed ruptured low-grade appendiceal mucinous neoplasm. The rupture point was at the tip of the appendix, coinciding with the center of radial fibrosis. The unique morphology of the puffer ball-like appearance in this case may be a characteristic of low-grade appendiceal mucinous neoplasms.
PubMed: 37388265
DOI: 10.1016/j.radcr.2023.05.006 -
Journal of Medical Case Reports Jun 2023Pseudomyxoma Peritonei (PMP) is a severe neoplastic clinical syndrome characterised by secretion of mucin from tumors often originating in the appendix. The standard...
BACKGROUND
Pseudomyxoma Peritonei (PMP) is a severe neoplastic clinical syndrome characterised by secretion of mucin from tumors often originating in the appendix. The standard treatment includes cytoreductive surgery (CRS) combined with heated intraperitoneal chemotherapy (HIPEC). A new perspective in PMP treatment aims at the mucins themselves as a therapeutic target.
CASE PRESENTATION
Here we report the first case of PMP with peritoneal dissemination of mucinous implants caused by low-grade appendiceal mucinous neoplasm (LAMN) in a 58-year-old white male exclusively treated by appendectomy and oral administration of bromelain and acetylcysteine in the context of a medical self-experimentation (by co-author T.R.). Observation so far covers a period of 48 months including regular magnetic resonance imaging (MRI) with stable findings.
CONCLUSIONS
Oral administration of bromelain and acetylcysteine can be used in the treatment of PMP caused by LAMN without relevant clinical side effects.
Topics: Male; Humans; Middle Aged; Pseudomyxoma Peritonei; Appendiceal Neoplasms; Administration, Oral; Peritoneal Neoplasms
PubMed: 37381056
DOI: 10.1186/s13256-023-04024-7 -
Journal of Clinical Medicine Jun 2023Pseudomyxoma peritonei (PMP) is a rare malignant growth characterized by the production of mucin and the potential for peritoneal relapse. This study aimed to...
Pseudomyxoma peritonei (PMP) is a rare malignant growth characterized by the production of mucin and the potential for peritoneal relapse. This study aimed to investigate the immunohistochemical and biological characteristics of mucin in patients with cellular and acellular PMP. We prospectively analyzed mucin specimens obtained from our patient cohort and described the composition and type of mucin present in each sample. A metagenomic analysis of the samples was performed to investigate the bacterial composition of the PMP microbiome. Secreted mucins 2 and 5AC and membrane-associated mucin-1 were the primary components of mucin in both cellular and acellular tumor specimens. The metagenomic study revealed a predominance of the phylum and the genus . Notably, , a species not previously reported in the human microbiome, was found to be the most abundant organism in the mucin of pseudomyxoma peritonei. Our findings suggest that the presence of MUC-2 and mucin colonization by Pseudomonas are characteristic features of both cellular and acellular disease. These results may have significant implications for the diagnosis and treatment of this rare entity.
PubMed: 37373701
DOI: 10.3390/jcm12124007 -
Annals of the Royal College of Surgeons... Mar 2024John Hunter is regarded as the father of scientific surgery. His principles involved reasoning, observation and experimentation. His most powerful saying was: "Why not...
John Hunter is regarded as the father of scientific surgery. His principles involved reasoning, observation and experimentation. His most powerful saying was: "Why not try the experiment?" This manuscript charts a career in abdominal surgery ranging from the treatment of appendicitis to the development of the largest appendiceal tumour centre in the world. The journey has led to the first report of a successful multivisceral and abdominal wall transplant for patients with recurrent non-resectable pseudomyxoma peritonei. We all stand on the shoulders of giants and surgery progresses by learning from the past while being prepared to experiment into the future.
Topics: Humans; Appendiceal Neoplasms; Appendicitis; Transplants; Abdominal Wall
PubMed: 37367485
DOI: 10.1308/rcsann.2023.0013 -
Indian Journal of Surgical Oncology Jun 2023To report a case series of patients with pseudomyxoma peritonei (PMP) from urachal mucinous neoplasm (UMN) treated with CRS and HIPEC at a high-volume referral centre,...
UNLABELLED
To report a case series of patients with pseudomyxoma peritonei (PMP) from urachal mucinous neoplasm (UMN) treated with CRS and HIPEC at a high-volume referral centre, along with an updated literature review. Retrospective review of cases treated between 2000 and 2021. A literature review using MEDLINE and Google Scholar databases was performed. Clinical presentation of PMP from UMN is heterogeneous, and common symptoms are abdominal distension, weight loss, fatigue and haematuria. At least one tumour marker among CEA, CA 19.9, and CA 125 was elevated in the six cases reported, and 5/6 had a preoperative working diagnosis of urachal mucinous neoplasm suspected on detailed cross-sectional imaging. Complete cytoreduction was achieved in five cases, while one patient underwent maximal tumour debulking. Histological findings mirrored the findings of PMP from appendiceal mucinous neoplasms (AMN). Overall survival ranged between 43 and 141 months after complete cytoreduction. On literature review, 76 cases have been reported to date. Complete cytoreduction is associated with good prognosis for patients with PMP from UMN. A definitive classification system is still not available.
SUPPLEMENTARY INFORMATION
The online version contains supplementary material available at 10.1007/s13193-022-01694-5.
PubMed: 37359937
DOI: 10.1007/s13193-022-01694-5 -
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