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Biological Procedures Online May 2024Pseudomyxoma peritonei (PMP) is a rare peritoneal mucinous carcinomatosis with largely unknown underlying molecular mechanisms. Cytoreductive surgery combined with...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare peritoneal mucinous carcinomatosis with largely unknown underlying molecular mechanisms. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is the only therapeutic option; however, despite its use, recurrence with a fatal outcome is common. The lack of molecular characterisation of PMP and other mucinous tumours is mainly due to the physicochemical properties of mucin.
RESULTS
This manuscript describes the first protocol capable of breaking the mucin barrier and isolating proteins from mucinous tumours. Briefly, mucinous tumour samples were homogenised and subjected to liquid chromatography using two specific columns to reduce mainly glycoproteins, albumins and immunoglobulin G. The protein fractions were then subjected to mass spectrometry analysis and the proteomic profile obtained was analysed using various bioinformatic tools. Thus, we present here the first proteome analysed in PMP and identified a distinct mucin isoform profile in soft compared to hard mucin tumour tissues as well as key biological processes/pathways altered in mucinous tumours. Importantly, this protocol also allowed us to identify MUC13 as a potential tumour cell marker in PMP.
CONCLUSIONS
In sum, our results demonstrate that this protein isolation protocol from mucin will have a high impact, allowing the oncology research community to more rapidly advance in the knowledge of PMP and other mucinous neoplasms, as well as develop new and effective therapeutic strategies.
PubMed: 38750435
DOI: 10.1186/s12575-024-00239-0 -
Radiology Case Reports Aug 2024Pseudomyxoma peritonei (PMP) is a relatively uncommon condition primarily associated with neoplasms of the appendiceal epithelium. It is characterized by non-specific...
Pseudomyxoma peritonei (PMP) is a relatively uncommon condition primarily associated with neoplasms of the appendiceal epithelium. It is characterized by non-specific clinical manifestations, leading to a high rate of misdiagnosis. This report describes the case of a 62-year-old male patient with recurrent and metastatic PMP. The patient first experienced unexplained epigastric pain and paroxysmal abdominal pain accompanied by distension over 8 years ago. He underwent surgical interventions for the condition in other hospitals in 2015 and 2018, respectively.
PubMed: 38745977
DOI: 10.1016/j.radcr.2024.04.034 -
Cureus Apr 2024Pseudomyxoma peritonei (PMP) is a rare intra-abdominal malignancy characterized by diffuse dissemination of mucinous tumor cells, leading to mucinous ascites. Accurate...
Pseudomyxoma peritonei (PMP) is a rare intra-abdominal malignancy characterized by diffuse dissemination of mucinous tumor cells, leading to mucinous ascites. Accurate diagnosis is crucial for appropriate management. This report presents a case of a 55-year-old Lebanese male farmer initially misdiagnosed with liver cirrhosis who presented with progressive abdominal distension refractory to diuretics and dietary modifications. Paracentesis revealed a mucinous exudate, with subsequent clinical and histopathological examination confirming PMP. The patient was referred for further evaluation at a specialized center equipped for cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). This case highlights the diagnostic challenges of PMP due to its non-specific presentation, emphasizing the importance of prompt and accurate diagnosis to facilitate optimal therapeutic intervention.
PubMed: 38721212
DOI: 10.7759/cureus.57857 -
International Journal of Surgery Case... Jun 2024Appendiceal tumors are rare neoplasms detected in about 2 % of appendicectomies. The clinical presentation is often unspecific, varying from unspecific abdominal pain...
INTRODUCTION
Appendiceal tumors are rare neoplasms detected in about 2 % of appendicectomies. The clinical presentation is often unspecific, varying from unspecific abdominal pain or presenting as an acute appendicitis or being asymptomatic.
CASE PRESENTATION
We present a case of a patient presenting as an acute appendicitis with a mucocele, and then classified as HAMN. The patient was treated with initial laparoscopic approach and then conversion in laparotomy with appendectomy. Histology demonstrated a high grade appendiceal mucinous neoplasm limited to submucosa (pT3), with concomitant acute phlegmonous appendicitis. The patient was subsequently sent to a referral center where a right hemicolectomy with HIPEC was performed.
DISCUSSION
HAMN is a rare entity, only recently classified as a new kind of appendiceal mucinous neoplasm. Due to the supposed higher aggressivity, HAMN must be treated as an appendiceal adenocarcinoma. The treatment of this rare entity is not yet well standardized, because of the rarity of this disease.
CONCLUSION
HAMN is a very rare tumor. In the emergency setting, it is mandatory to avoid rupture of the appendix, to minimize the risk of developing pseudomyxoma peritonei. Pathology is essential for further decisions in these patients and plays a very important role in treatment and prognosis.
PubMed: 38688155
DOI: 10.1016/j.ijscr.2024.109716 -
International Journal of Surgery Case... Jun 2024Pseudomyxoma peritonei (PMP) is characterized by the accumulation of mucinous fluid in the abdominal cavity, typically originating from mucin-producing tumors, most...
Successful treatment of pseudomyxoma peritonei (PMP) through cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC): A case report and literature review.
INTRODUCTION AND IMPORTANCE
Pseudomyxoma peritonei (PMP) is characterized by the accumulation of mucinous fluid in the abdominal cavity, typically originating from mucin-producing tumors, most commonly arising in the appendix.
CASE PRESENTATION
A 63-year-old male patient presented to the hospital with complaints of abdominal pain, significant weight loss, a palpable mass in the right iliac fossa, and evidence of ascites. A diagnosis of PMP was proposed based on findings from a computed tomography (CT) scan, which was subsequently confirmed through histopathological examination of a biopsy. The patient underwent successful treatment with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS-HIPEC).
CLINICAL DISCUSSION
Diagnosing PMP presents challenges due to its rarity and the potential for extensive spread throughout the peritoneal cavity, necessitating a multidisciplinary approach for successful treatment.
CONCLUSION
Pseudomyxoma peritonei is a rare yet medically significant condition. Documenting a case of this ailment in Palestine has the potential to advance medical understanding, raise awareness, and improve patient care standards within the local healthcare system. By documenting this uncommon condition, healthcare practitioners in Palestine can gain valuable insights into its manifestations, diagnostic processes, and treatment modalities. This contribution not only enriches the global medical literature but also promotes collaboration in addressing the challenges associated with rare diseases.
PubMed: 38677258
DOI: 10.1016/j.ijscr.2024.109656 -
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 -
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 -
Transfusion Medicine and Hemotherapy :... Apr 2024DEL is known to be one of the weakest D variants, which can be detected by the adsorption-elution technique or by molecular study. Currently, in Thailand, we do not...
INTRODUCTION
DEL is known to be one of the weakest D variants, which can be detected by the adsorption-elution technique or by molecular study. Currently, in Thailand, we do not routinely test for DEL variants serologically or genetically among serologic RhD-negative blood donors.
CASE PRESENTATION
We reported 2 cases of alloimmunization after transfused with Rh DEL, allele, in the Thai population. The first case was a 73-year-old male with anemia who presented with post-cardiac arrest and septic shock. The patient was group B, RhD-negative, and was transfused with RhD-negative red blood cells (RBCs). Antibody screening and identification found that the patient developed anti-D and anti-Mi during the admission course. The second case was a 38-year-old woman with pseudomyxoma peritonei who developed anti-D after receiving four units of RhD-negative RBCs during cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. Both patients did not receive anti-D immunoglobulin and had no previous history of anti-D detection. We retrospectively investigated and found two units of among the RBCs transfused to these patients.
DISCUSSION
Previous reports of several cases of anti-D alloimmunization in RhD-negative recipients transfused by , an Asian-type DEL, are limited only to East Asia. We first identified 2 patients with anti-D alloimmunization after receiving the RBCs in the Thai population. This raises concern about Rh DEL screening among D-negative Thai blood donors and whether to remove DEL units from the D-negative inventory to improve patient safety.
PubMed: 38584693
DOI: 10.1159/000533625 -
World Journal of Gastrointestinal... Mar 2024Appendiceal mucinous neoplasms (AMNs), although not classified as rare, are relatively uncommon tumors most often discovered incidentally during colorectal surgery....
BACKGROUND
Appendiceal mucinous neoplasms (AMNs), although not classified as rare, are relatively uncommon tumors most often discovered incidentally during colorectal surgery. Accurate identification of AMNs is difficult due to non-specific symptoms, overlapping tumor markers with other conditions, and the potential for misdiagnosis. This underscores the urgent need for precision in diagnosis to prevent severe complications.
CASE SUMMARY
This case report describes the unexpected discovery and treatment of a low-grade AMN (LAMN) in a 74-year-old man undergoing laparoscopic hemicolectomy for transverse colon adenocarcinoma (AC). Preoperatively, non-specific gastrointestinal symptoms and elevated tumor markers masked the presence of AMN. The tumor, presumed to be an AMN peritoneal cyst intraoperatively, was confirmed as LAMN through histopathological examination. The neoplasm exhibited mucin accumulation and a distinct immunohistochemical profile: Positive for Homeobox protein , Cytokeratin 20, special AT-rich sequence-binding protein 2, and Mucin 2 but negative for cytokeratin 7 and Paired box gene 8. This profile aids in distinguishing appendiceal and ovarian mucinous tumors. Postoperative recovery was uncomplicated, and the patient initiated adjuvant chemotherapy for the colon AC.
CONCLUSION
This case highlights the diagnostic complexity of AMNs, emphasizing the need for vigilant identification to avert potential complications, such as pseudomyxoma peritonei.
PubMed: 38577069
DOI: 10.4240/wjgs.v16.i3.944 -
Journal of Minimal Access Surgery Mar 2024Pseudomyxoma peritonei (PMP) is a condition characterised by the presence of gelatinous tumour-like growth within the peritoneal cavity. Combined cytoreductive surgery...
INTRODUCTION
Pseudomyxoma peritonei (PMP) is a condition characterised by the presence of gelatinous tumour-like growth within the peritoneal cavity. Combined cytoreductive surgery and intraperitoneal chemotherapy have shown to improve the survival rate in PMP patients. However, post-operative complications such as cognitive dysfunction, respiratory insufficiency and acute renal failure are still observed. This retrospective study aims to explore the risk factors associated with major post-operative complications and specifically investigate the correlation with intraoperative hypotension.
PATIENTS AND METHODS
This retrospective cohort study included PMP patients treated at Beijing Aerospace Center Hospital from 1 June, 2014 to 30 December, 2020. The primary outcome measures were major post-operative complications, including neurological, pulmonary, cardiovascular, surgical complications, acute hepatic injury and acute kidney injuries. The secondary outcome measures included infection, fever and deep venous thrombosis. Statistical analysis was conducted using EmpowerStats and R software.
RESULTS
A total of 782 patients were screened, and 668 patients were included in the statistical analysis. Amongst them, 234 (35.03%) individuals experienced major post-operative complications. Factors such as pre-operative American Society of Anaesthesiologists grading, age, haemoglobin and albumin levels, intraoperative mean arterial pressure, blood loss, fluid replacement volume, method of intraperitoneal hyperthermic perfusion and post-operative requirement for intensive care unit mechanical ventilation were found to be correlated with major complications.
CONCLUSION
The identified risk factors provide valuable insights for improving clinical pathways in the management of PMP. Further prospective studies are warranted to establish the association between these factors and patient outcomes.
PubMed: 38557560
DOI: 10.4103/jmas.jmas_299_23