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The British Journal of Surgery May 2018The prognostic value of the primary neoplasm responsible for pseudomyxoma peritonei (PMP) remains poorly studied. The aim of this study was to determine the prognosis...
BACKGROUND
The prognostic value of the primary neoplasm responsible for pseudomyxoma peritonei (PMP) remains poorly studied. The aim of this study was to determine the prognosis for patients with extra-appendicular PMP (EA-PMP) treated optimally with complete cytoreductive surgery (CCRS) and hyperthermic intraperitoneal chemotherapy (HIPEC).
METHODS
All patients treated for PMP with CCRS and HIPEC between 1994 and 2016 were selected retrospectively from a French multicentre database. Patients with EA-PMP had pathologically confirmed non-neoplastic appendices and were matched in a 1 : 4 ratio with patients treated for appendicular PMP (A-PMP), based on a propensity score.
RESULTS
Some 726 patients were identified, of which 61 (EA-PMP group) were matched with 244 patients (A-PMP group). The origins of primary tumours in the EA-PMP group included the ovary (45 patients), colon (4), urachus (4), small bowel (1), pancreas (1) and unknown (6). The median peritoneal carcinomatosis index was comparable in EA-PMP and A-PMP groups (15·5 versus 18 respectively; P = 0·315). In-hospital mortality (3 versus 2·9 per cent; P = 1·000) and major morbidity 26 versus 25·0 per cent; P = 0·869) were also similar between the two groups. Median follow-up was 66·9 months. The 5-year overall survival rate was 87·8 (95 per cent c.i. 83·2 to 92·5) per cent in the A-PMP group and 87 (77 to 96) per cent in the EA-PMP group. The 5-year disease-free survival rate was 66·0 (58·7 to 73·4) per cent and 70 (53 to 83) per cent respectively.
CONCLUSION
Overall and disease-free survival following treatment with CCRS and HIPEC is similar in patients with pseudomyxoma peritonei of appendicular or extra-appendicular origin.
Topics: Appendiceal Neoplasms; Cytoreduction Surgical Procedures; Disease-Free Survival; Female; Humans; Hyperthermia, Induced; Male; Middle Aged; Peritoneal Neoplasms; Prognosis; Pseudomyxoma Peritonei; Retrospective Studies; Survival Analysis
PubMed: 29412465
DOI: 10.1002/bjs.10716 -
Rozhledy V Chirurgii : Mesicnik... 2021Appendiceal mucocele is an obstructive dilatation of the appendix, which results from the filling of its lumen with mucus. This is a rare condition that is asymptomatic...
Appendiceal mucocele is an obstructive dilatation of the appendix, which results from the filling of its lumen with mucus. This is a rare condition that is asymptomatic in half of the patients. Its severity depends on the cause of appendiceal dilatation. In a small percentage of cases, the dilated appendix ruptures, leading to the development of serious complication; this is termed as pseudomyxoma peritonei. Due to the possibility of malignant etiology of the mucocele, surgical resection remains an essential part of the treatment.
Topics: Appendiceal Neoplasms; Appendix; Humans; Mucocele; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Radiologists
PubMed: 34465115
DOI: 10.33699/PIS.2021.100.6.266-270 -
Pleura and Peritoneum Sep 2020
PubMed: 33364339
DOI: 10.1515/pp-2020-0119 -
Annals of Surgical Oncology May 2024Ovarian pseudomyxoma peritonei (OPMP) are rare, without well-defined therapeutic guidelines. We aimed to evaluate cytoreductive surgery (CRS) and hyperthermic...
BACKGROUND
Ovarian pseudomyxoma peritonei (OPMP) are rare, without well-defined therapeutic guidelines. We aimed to evaluate cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) to treat OPMP.
METHODS
Patients from the French National Network for Rare Peritoneal Tumors (RENAPE) database with proven OPMP treated by CRS/HIPEC and with histologically normal appendix and digestive endoscopy were retrospectively included. Clinical and follow-up data were collected. Histopathological and immunohistochemical features were reviewed.
RESULTS
Fifteen patients with a median age of 56 years were included. The median Peritoneal Cancer Index was 16. Following CRS, the completeness of cytoreduction (CC) score was CC-0 for 9/15 (60%) patients, CC-1 for 5/15 (33.3%) patients, and CC-2 for 1/15 (6.7%) patients. The median tumor size was 22.5 cm. After pathological review and immunohistochemical studies, tumors were classified as Group 1 (mucinous ovarian epithelial neoplasms) in 3/15 (20%) patients; Group 2 (mucinous neoplasm in ovarian teratoma) in 4/15 (26.7%) patients; Group 3 (mucinous neoplasm probably arising in ovarian teratoma) in 5/15 (33.3%) patients; and Group 4 (non-specific group) in 3/15 (20%) patients. Peritoneal lesions were OPMP pM1a/acellular, pM1b/grade 1 (hypocellular) and pM1b/grade 3 (signet-ring cells) in 13/15 (86.7%), 1/15 (6.7%) and 1/15 (6.7%) patients, respectively. Disease-free survival analysis showed a difference (p = 0.0463) between OPMP with teratoma/likely-teratoma origin (groups 2 and 3; 100% at 1, 5, and 10 years), and other groups (groups 1 and 4; 100%, 66.6%, and 50% at 1, 5, and 10 years, respectively).
CONCLUSION
These results suggested that a primary therapeutic strategy using complete CRS/HIPEC for patients with OPMP led to favorable long-term outcomes.
Topics: Female; Humans; Middle Aged; Pseudomyxoma Peritonei; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; Retrospective Studies; Hyperthermia, Induced; Neoplasms, Cystic, Mucinous, and Serous; Teratoma; Appendiceal Neoplasms; Combined Modality Therapy; Survival Rate
PubMed: 38341381
DOI: 10.1245/s10434-023-14850-0 -
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 -
International Journal of Molecular... Nov 2020The mechanisms by which neoplastic cells disseminate from the primary tumor to metastatic sites, so-called metastatic organotropism, remain poorly understood.... (Review)
Review
The mechanisms by which neoplastic cells disseminate from the primary tumor to metastatic sites, so-called metastatic organotropism, remain poorly understood. Epithelial-mesenchymal transition (EMT) plays a role in cancer development and progression by converting static epithelial cells into the migratory and microenvironment-interacting mesenchymal cells, and by the modulation of chemoresistance and stemness of tumor cells. Several findings highlight that pathways involved in EMT and its reverse process (mesenchymal-epithelial transition, MET), now collectively called epithelial-mesenchymal plasticity (EMP), play a role in peritoneal metastases. So far, the relevance of factors linked to EMP in a unique peritoneal malignancy such as pseudomyxoma peritonei (PMP) has not been fully elucidated. In this review, we focus on the role of epithelial-mesenchymal dynamics in the metastatic process involving mucinous neoplastic dissemination in the peritoneum. In particular, we discuss the role of expression profiles and phenotypic transitions found in PMP in light of the recent concept of EMP. A better understanding of EMP-associated mechanisms driving peritoneal metastasis will help to provide a more targeted approach for PMP patients selected for locoregional interventions involving cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.
Topics: Animals; Biomarkers; Cell Plasticity; Disease Management; Disease Susceptibility; Epithelial-Mesenchymal Transition; Humans; Osteogenesis; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 33266161
DOI: 10.3390/ijms21239120 -
Minerva Chirurgica Jun 2015Peritoneal surface malignancies (PSM) include peritoneal metastases from gastrointestinal and gynecological tumor and rare primary peritoneal malignancies. PSM have been... (Review)
Review
Peritoneal surface malignancies (PSM) include peritoneal metastases from gastrointestinal and gynecological tumor and rare primary peritoneal malignancies. PSM have been historically considered as end-stage metastatic conditions only amenable to palliative options. Only in recent years, better knowledge of their natural history and pattern of disease-progression has evolved into the concept that PSM represent a local-regional disease stage. A novel treatment approach aiming at definitive disease eradication combines aggressive cytoreductive surgery (CRS) and perioperative local-regional chemotherapy, either in the form of hyperthermic intraperitoneal chemotherapy (HIPEC), or normothermic early postoperative chemotherapy. Such a combined treatment approach has reportedly resulted in a survival improvement over historical controls, and it is gaining an increasing acceptance as standard of care for selected patients with PSM. This article reviews the most recent literature data on the surgical and comprehensive management of PSM. Epidemiology and natural history of the different disease entities are briefly discussed. Cytoreductive surgical procedures and intraperitoneal chemotherapy administration techniques are described, focusing on the technical variants adopted in our institution. Indications for combined treatment, and outcomes following CRS/HIPEC, are addressed, including peritoneal metastases from appendiceal tumors (pseudomyxoma peritonei), colorectal cancer, gastric cancer, epithelial ovarian cancer, and rare primary peritoneum based neoplasms, such as diffuse malignant peritoneal mesothelioma, and primary peritoneal (extra-ovarian) serous papillary carcinoma.
Topics: Antineoplastic Combined Chemotherapy Protocols; Carcinoma, Ovarian Epithelial; Chemotherapy, Adjuvant; Cytoreduction Surgical Procedures; Evidence-Based Medicine; Gastrointestinal Neoplasms; Humans; Hyperthermia, Induced; Infusions, Parenteral; Meta-Analysis as Topic; Neoplasms, Glandular and Epithelial; Ovarian Neoplasms; Patient Selection; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Randomized Controlled Trials as Topic; Risk Factors; Treatment Outcome
PubMed: 25752673
DOI: No ID Found -
Clinics (Sao Paulo, Brazil) 2022The objective of this systematic review is to provide efficacy and safety data in the application of Intra-Abdominal Hyperthermia Chemotherapy (HIPEC) and Cytoreductive... (Review)
Review
Efficacy and safety in the use of intraperitoneal hyperthermia chemotherapy and peritoneal cytoreductive surgery for pseudomyxoma peritonei from appendiceal neoplasm: A systematic review.
The objective of this systematic review is to provide efficacy and safety data in the application of Intra-Abdominal Hyperthermia Chemotherapy (HIPEC) and Cytoreductive Surgery (CRS) in patients with Peritoneal Pseudomyxoma (PMP) of origin in the cecal appendix. The databases Medline and Central Cochrane were consulted. Patients with PMP of origin in the cecal appendix, classified as low grade, high or indeterminate, submitted to HIPEC and CRS. The results were meta-analyzed using the Comprehensive Metanalysis software. Twenty-six studies were selected to support this review. For low-grade PMP outcome, 60-month risk of mortality, Disease-Free Survival (DFS), and adverse events was 28.8% (95% CI 25.9 to 32), 43% (95% CI 36.4 and 49.8), and 46.7% (95% CI 40.7 to 52.8); for high-grade PMP, 60-month risk of mortality, Disease-Free Survival (DFS) and adverse events was 55.9% (95% CI 51.9 to 59.6), 20.1% (95% CI 15.5 to 25.7) and 30% (95% CI 25.2 to 35.3); PMP indeterminate degree, 60-month risk of mortality, Disease-Free Survival (DFS) and adverse events was 32.6% (95% CI 30.5 to 34.7), 61.8% (95% CI 58.8 to 64.7) and 32.9% (95% CI 30.5 to 35.4). The authors conclude that the HIPEC technique and cytoreductive surgery can be applied to selected cases of patients with PMP of peritoneal origin with satisfactory results.
Topics: Appendiceal Neoplasms; Combined Modality Therapy; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Hyperthermic Intraperitoneal Chemotherapy; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Retrospective Studies
PubMed: 35576869
DOI: 10.1016/j.clinsp.2022.100039 -
Pseudomyxoma peritonei leading to "jelly belly" abdomen: a case report and review of the literature.Journal of Medical Case Reports Jun 2024Pseudomyxoma peritonei is an infrequent condition with a global annual incidence of only one to two cases per million people. Mucinous neoplasms, widespread... (Review)
Review
BACKGROUND
Pseudomyxoma peritonei is an infrequent condition with a global annual incidence of only one to two cases per million people. Mucinous neoplasms, widespread intraperitoneal implants, and mucinous ascites characterize it. Currently, most clinicians misdiagnose this condition, which leads to delayed management.
CASE PRESENTATION
A 44-year-old North Indian female presented with a 1.5-month history of an abdominal lump. Physical examination revealed a sizeable abdominopelvic mass at 36 weeks. Contrast-enhanced computed tomography showed a massive multiloculated right ovarian cystic mass measuring 28 × 23 × 13 cm with mild ascites and elevated carcinoembryonic antigen levels (113.75 ng/ml). A provisional diagnosis of ovarian mucinous neoplasm was made, for which the patient underwent laparotomy. Intraoperatively, there were gross mucinous ascites, along with a large, circumscribed, ruptured right ovarian tumor filled with gelatinous material. The appendicular lump was also filled with mucinous material along with the omentum, ascending colon, right lateral aspect of the rectum, splenic surface, and small bowel mesentery. Cytoreductive surgery was performed along with an oncosurgeon, including total abdominal hysterectomy with bilateral salpingoophorectomy, omentectomy, right hemicolectomy, lower anterior resection, ileo-transverse stapled anastomosis with proximal ileal loop diversion stoma, excision of multiple peritoneal gelatinous implants, and peritoneal lavage. Histopathology and immunohistochemistry confirmed the presence of intestinal-type mucinous carcinoma. Postoperatively, the patient was given six cycles of chemotherapy. She tolerated it without any specific morbidity and had an uneventful recovery. Postoperative follow-up at 15 months revealed normal tumor marker levels and abdominal computed tomography findings and no signs suggestive of local recurrence or distal metastases.
CONCLUSIONS
Pseudomyxoma peritonei is a rare disease that is frequently misdiagnosed in the preoperative phase. Therefore, radiologists and clinicians should maintain a high index of suspicion for accurate diagnosis and multidisciplinary management.
Topics: Humans; Female; Pseudomyxoma Peritonei; Adult; Peritoneal Neoplasms; Tomography, X-Ray Computed; Cytoreduction Surgical Procedures; Ovarian Neoplasms; Ascites; Hysterectomy; Treatment Outcome
PubMed: 38937808
DOI: 10.1186/s13256-024-04612-1 -
International Journal of Cancer Mar 2015Pseudomyxoma peritonei (PMP) is a relatively rare clinical syndrome characterized by neoplastic epithelial cells growing in the peritoneal cavity and secreting mucinous...
Pseudomyxoma peritonei (PMP) is a relatively rare clinical syndrome characterized by neoplastic epithelial cells growing in the peritoneal cavity and secreting mucinous ascites. Our aim was to explore the molecular events behind this fatal but under-investigated disease. We extracted DNA from 19 appendix-derived PMP tumors and nine corresponding normal tissues, and analyzed the mutational hotspot areas of 48 cancer-related genes by amplicon-based next-generation sequencing (NGS). Further, we analyzed the protein expression of V600E mutated BRAF, MLH1, MSH2, MSH6 and p53 from a larger set of PMP tumors (n = 74) using immunohistochemistry. With NGS, we detected activating somatic KRAS mutations in all of the tumors studied. GNAS was mutated in 63% of the tumors with no marked difference between low-grade and high-grade tumors. Only one (5.3%) tumor showed oncogenic PIK3CA mutation, one showed oncogenic AKT1 mutation, three (15.8%) showed SMAD4 mutations and none showed an APC mutation. P53 protein was aberrantly expressed in higher proportion of high-grade tumors as compared with low-grade ones (31.3 vs. 7.1%, respectively; p = 0.012) and aberrant expression was an independent factor for reduced overall survival (p = 0.002). BRAF V600E mutation was only found in one (1.4%) high-grade tumor by immunohistochemistry (n = 74). All the studied tumors expressed mismatch repair proteins MLH1, MSH2 and MSH6. Our results indicate that KRAS mutations are evident in all and GNAS mutations in most of the PMPs, but BRAF V600E, PIK3CA and APC mutations are rare. Aberrantly expressed p53 is associated with high-grade histology and reduced survival.
Topics: Aged; Biomarkers, Tumor; DNA Mutational Analysis; Female; Gene Expression Profiling; Genomics; High-Throughput Nucleotide Sequencing; Humans; Immunoenzyme Techniques; Male; Mutation; Neoplasm Grading; Peritoneal Neoplasms; Prognosis; Pseudomyxoma Peritonei; Survival Rate
PubMed: 25274248
DOI: 10.1002/ijc.29245