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Rozhledy V Chirurgii : Mesicnik... 2020Pseudomyxoma peritonei (PMP) is a rare malignant disease with various grades of malignancy, producing mucinous and gelatinous masses. The origin of PMP is usually...
INTRODUCTION
Pseudomyxoma peritonei (PMP) is a rare malignant disease with various grades of malignancy, producing mucinous and gelatinous masses. The origin of PMP is usually connected with the rupture of appendiceal mucinous tumours, other mucinous tumours of the gastrointestinal tract or of the ovary. The staging of this disease is determined by the PCI score (peritoneal cancer index), and the efficiency of surgical procedure by the CC score. Clinical presentation is very variable and depends on the stage of the disease. Many patients are asymptomatic with a minimal clinical finding, presented only with abdominal discomfort. A typical finding of the “jelly belly“ syndrome expands with progression of the disease. The diagnosis consists in preoperative determination of the tumour characteristics and PCI based on imaging methods, especially CT imaging.
METHODS
The Sugarbaker technique of complete tumour removal or the so-called cytoreductive surgery (CRS) was used, including hyperthermic intraperitoneal chemotherapy (HIPEC) or alternatively early postoperative intraperitoneal chemotherapy (EPIC). We performed retrospective evalu-ation of 73 patients with pseudomyxoma peritonei undergoing surgery, 39 males and 34 females, mean age 50.6 and 56.4 years, respectively. Surgical revision was performed in 18 patients, 14 males and 4 females. The mean age of this group was 48.8 for the males and 47 for the females. The surgical procedures were performed between 1999 and 2018. Survival rates, median survival, complications based on Clavien-Dindo classification, lethality rates, and PCI and CC scores were assessed in the patient group.
RESULTS
96 surgeries were performed in 73 patients with pseudomyxoma peritonei at our surgical department between 19992018. The surgery had to be repeated in 18 patients (24.6%). High grade (HG) pseudomyxoma was diagnosed in 29 patients (39.7%), and low grade (LG) pseudomyxoma in 44 patients (60.3%). Overall morbidity was 27.3%, and the mortality rate was 5.4%. The mean overall survival (OS) was 139.5 months in the LG pseudomyxoma group and 71.5 months in the HG pseudomyxoma group. Median survival was 86 months in the entire group and 72 in the HG pseudomyxoma group; the median was not reached in the LG pseudomyxoma group.
CONCLUSIONS
Results in the literature and our results are comparable, confirming the high efficiency of this method both in the world and in the Czech republic. The results indicate a highly statistically significant improvement of the OS with acceptable mortality and morbidity. These results confirm this method as a gold standard therapy for selected patients.
Topics: Combined Modality Therapy; Czech Republic; Female; Humans; Male; Middle Aged; Percutaneous Coronary Intervention; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Retrospective Studies
PubMed: 32545978
DOI: 10.33699/PIS.2020.99.4.159-166 -
ANZ Journal of Surgery Oct 2020Mucinous appendiceal neoplasms (MANs) are rare tumours and the primary cause of pseudomyxoma peritonei. These tumours have a much more benign course than typical... (Review)
Review
Mucinous appendiceal neoplasms (MANs) are rare tumours and the primary cause of pseudomyxoma peritonei. These tumours have a much more benign course than typical colorectal cancers, generally growing for many years before giving any clinical signs. The spectrum of presentations, tumour stages and the underlying cytology is very wide, warranting from the simplest operation like an appendicectomy to the most complicated operation like a complete cytoreduction surgery and hyperthermic intraperitoneal chemotherapy. Fortunately, most patients can be offered a curative treatment, but limiting operative morbidity without compromising oncologic outcomes is the biggest challenge in managing these patients. Histopathology is the cornerstone of decision making for MANs, but is also subject to ongoing debate because of a lack of terminology consensus amongst pathologists. Combined with the rarity of this disease, the multiple histopathologic classification updates of MANs explain the ongoing confusion amongst clinicians in regard to individual optimal treatment. This review will cover the most recent histological classification of MANs and attempt to clarify optimal management of patients with different clinical presentation and histologic combinations.
Topics: Appendiceal Neoplasms; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Mitomycin; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 32761790
DOI: 10.1111/ans.16185 -
Chirurgie (Heidelberg, Germany) Oct 2023The gold standard in the treatment of mucinous intra-abdominal neoplasms is cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC).... (Review)
Review
BACKGROUND
The gold standard in the treatment of mucinous intra-abdominal neoplasms is cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC). Despite complete cytoreduction up to 45% of patients develop recurrences.
METHOD
A search and analysis of the current literature were carried out.
RESULTS
There is still controversy regarding the best treatment strategy for patients with recurrent pseudomyxoma peritonei (PMP) after CRS and HIPEC. The clinical management of these patients depends on many factors, such as the site and volume of recurrence, histological subtype and symptoms. Treatment options range from repeated surgery with curative intent with or without HIPEC to watch and wait strategies. In selected patients redo surgery is feasible and safe with low morbidity and mortality. Iterative complete CRS can result in a median 5‑year overall survival of more than 80%. Debulking surgery leads to a prolonged survival and to symptom control fora period with of nearly 2 years.
CONCLUSION
Repeated complete cytoreduction of recurrent PMP can result in long-term survival. Tumor debulking surgery may be particularly beneficial for symptomatic patients.
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Hyperthermia, Induced; Appendiceal Neoplasms; Neoplasms, Cystic, Mucinous, and Serous
PubMed: 37432477
DOI: 10.1007/s00104-023-01925-7 -
Cureus May 2024Pseudomyxoma peritonei (PMP) is a rare and complex clinical syndrome characterized by the accumulation of mucinous ascites within the peritoneal cavity, typically... (Review)
Review
Pseudomyxoma peritonei (PMP) is a rare and complex clinical syndrome characterized by the accumulation of mucinous ascites within the peritoneal cavity, typically associated with mucinous tumours of appendiceal origin. Despite its rarity, PMP poses significant challenges in diagnosis and management due to its indolent yet locally aggressive nature. This comprehensive review provides insights into the diagnosis, management, and prognosis of PMP, synthesizing current evidence and emerging trends in the field. Challenges and opportunities in PMP management are discussed, along with recommendations for clinical practice emphasizing the importance of a multidisciplinary approach and specialized care. Despite ongoing challenges, advances in surgical techniques, perioperative chemotherapy, and emerging therapies offer hope for improved outcomes and quality of life for PMP patients.
PubMed: 38939264
DOI: 10.7759/cureus.61244 -
The Journal of International Medical... Jun 2021To determine the levels of carcinoembryonic antigen (CEA), proliferating nuclear antigen Ki67 and p53 in pseudomyxoma peritonei (PMP) of appendiceal origin and to...
OBJECTIVE
To determine the levels of carcinoembryonic antigen (CEA), proliferating nuclear antigen Ki67 and p53 in pseudomyxoma peritonei (PMP) of appendiceal origin and to correlate the levels with clinicopathological characteristics and overall survival.
METHODS
This retrospective study collected data on clinicopathological features and immunohistochemical staining of CEA, Ki67 and p53 in patients with PMP of appendiceal origin. Overall survival was evaluated using Kaplan-Meier plots. Median survival time was estimated by Log-rank tests. Potential prognostic factors were evaluated by Cox proportional hazards regression models.
RESULTS
A total of 141 patients with PMP of appendiceal origin were enrolled in the study with a median age of 54 years. Of these, 93 (66.0%) were diagnosed with low-grade mucinous carcinoma, 43 (30.5%) with high-grade mucinous carcinoma and five (3.5%) with high-grade with signet ring cells. CEA exhibited ubiquitous immunopositivity in most cases and was not associated with overall survival. Ki67 labelling index (LI) and p53 status were related to histological grade and overall survival. The main pathological indicators affecting survival included histological grade, lymph node involvement, angiolymphatic invasion, Ki67 LI and p53.
CONCLUSION
Combined analysis of high Ki67 LI and aberrant p53 may provide the basis for evaluating the biological behaviour of PMP and predicting clinical outcome.
Topics: Carcinoembryonic Antigen; Humans; Ki-67 Antigen; Middle Aged; Peritoneal Neoplasms; Prognosis; Pseudomyxoma Peritonei; Retrospective Studies; Tumor Suppressor Protein p53
PubMed: 34187207
DOI: 10.1177/03000605211022297 -
International Journal of Medical... 2017Pseudomyxoma peritonei (PMP) is a rare disease with excess intraperitoneal mucin secretion. Treatment involves laparotomy, cytoreduction and chemotherapy that is very...
Pseudomyxoma peritonei (PMP) is a rare disease with excess intraperitoneal mucin secretion. Treatment involves laparotomy, cytoreduction and chemotherapy that is very invasive with patients often acquiring numerous compromises. Hence a mucolytic comprising of bromelain and N-acetyl cystein has been developed to solubilise mucin in situ for removal by catherization. Owing to differences in mucin appearance and hardness, dissolution varies. Therefore the current study investigates the inter-mucin physical and chemical characteristics, in order to reformulate an effective mucolytic for all mucin. PMP mucin, from the three categories (soft, semi hard and hard mucin) was solubilised and then various physical characteristics such as turbidity, density, kinematic viscosity were measured. The water content and the density of solid mucin were also determined. This was followed by the determination of sialic acid, glucose, lipid, Thiol (S-S and S-H) content of the samples. Lastly, the distribution of MUC2, MUC5B and MUC5AC was determined using western blot technique. Both turbidity and kinematic viscosity and sialic acid content increased linearly as the hardness of mucin increased. However, density, hydration, protein, glucose, lipid and sulfhydryl and disulphide content decreased linearly as hardness of mucin increased. The distribution ratio of mucins (MUC2:MUC5B:MUC5AC) in soft mucin is 2.25:1.5:1.0, semi hard mucin is 1:1:1 and hard mucin is 3:2:1. The difference in texture and hardness of mucin may be due to cellular content, hydration, glucose, protein, lipids, thiol and MUC distribution. Soft mucin is solely made of glycoprotein whilst the others contained cellular materials.
Topics: Glucose; Humans; Lipids; Mucin 5AC; Mucin-2; Mucin-5B; Mucins; Mucus; N-Acetylneuraminic Acid; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Sulfhydryl Compounds; Viscosity
PubMed: 28138305
DOI: 10.7150/ijms.16422 -
Journal of Gastrointestinal Surgery :... Apr 2020Pseudomyxoma peritonei (PMP) is a rare clinical presentation, with considerable morbidity and mortality if left untreated. In recent decades, there is growing acceptance...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare clinical presentation, with considerable morbidity and mortality if left untreated. In recent decades, there is growing acceptance for the use of cytoreductive surgery (CRS) with heated intraperitoneal chemotherapy (HIPEC). The aim of this study was to report on our 10-year single-center experience on outcomes following CRS and HIPEC for PMP of appendiceal origin.
METHODS
A retrospective analysis of a prospectively maintained database of all patients undergoing CRS and HIPEC for PMP of appendiceal origin over a 10-year period at a statewide referral center was conducted.
RESULTS
One hundred and seventy-five cytoreductive procedures were undertaken in 140 patients. The mean patient age was 57.4 years, with a female preponderance (56%). The median PCI was 16, with 73.1% of cases having a complete cytoreduction. Grade III/IV complications occurred in 36 (20.6%) cases, with no mortalities. The median overall and disease-free survival was 100 months and 40 months, respectively, with a 71% 5-year survival. High-grade histology was the main factor identified as an independent predictor of worse overall survival.
CONCLUSION
CRS and HIPEC are safe with acceptable rates of morbidity. It can provide very favorable survival in patients with PMP. High-grade histology is a key prognostic factor associated with a worse overall survival.
Topics: Appendiceal Neoplasms; Combined Modality Therapy; Cytoreduction Surgical Procedures; Female; Humans; Hyperthermia, Induced; Middle Aged; Percutaneous Coronary Intervention; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Retrospective Studies
PubMed: 31090036
DOI: 10.1007/s11605-019-04239-4 -
Journal of Surgical Oncology Sep 2021Pseudomyxoma peritonei is a disease caused by the widespread distribution of mucinous tumor into the peritoneal space from a perforated appendiceal neoplasm.
BACKGROUND
Pseudomyxoma peritonei is a disease caused by the widespread distribution of mucinous tumor into the peritoneal space from a perforated appendiceal neoplasm.
METHODS
All patients in this study had cytoreductive surgery with total gastrectomy plus perioperative intraperitoneal chemotherapy. A high diverting jejunostomy was used on all patients. Patient characteristics, adverse events, and survival were accumulated prospectively.
RESULTS
Fifty-eight patients were available for long-term follow-up. In the univariate analysis, features associated with a less favorable outcome included female gender (p = 0.0127), intestinal obstruction before treatment (p = 0.00791), and prior surgical score (PSS) (p = 0.0054). In the multivariate analysis, the two significant variables were grade (p = 0.0458) and PSS (p = 0.0041). Median survival was 12 years with a 5-, 10-, and 20-year survival of 76%, 58%, and 37%, respectively. There were two postoperative deaths (3.4%) and Grades 3 and 4 adverse events in 20 (34.5%) patients.
CONCLUSIONS
A 10-year survival after cytoreductive surgery, total gastrectomy with temporary high diverting jejunostomy, and perioperative chemotherapy occurred in 58% of these patients with advanced pseudomyxoma peritonei. High-grade disease and extensive prior surgery with a high PSS were associated with reduced benefit.
Topics: Adenocarcinoma, Mucinous; Adult; Aged; Appendiceal Neoplasms; Cytoreduction Surgical Procedures; Female; Gastrectomy; Humans; Jejunostomy; Male; Middle Aged; Perioperative Care; Pseudomyxoma Peritonei; Retrospective Studies; Survival Rate
PubMed: 33914912
DOI: 10.1002/jso.26506 -
World Journal of Gastrointestinal... Sep 2019Pseudomyxoma peritonei (PMP) is a disease surrounded by misunderstanding and controversies. Knowledge about the etymology of pseudomyxoma is useful to remove the... (Review)
Review
Pseudomyxoma peritonei (PMP) is a disease surrounded by misunderstanding and controversies. Knowledge about the etymology of pseudomyxoma is useful to remove the ambiguity around that term. The word pseudomyxoma derives from pseudomucin, a type of mucin. PMP was first described in a case of a woman alleged to have a ruptured pseudomucinous cystadenoma of the ovary, a term that has disappeared from today's classifications of cystic ovarian neoplasms. It is known today that in the majority of cases, the origin for PMP is an appendiceal neoplasm, often of low histological grade. Currently, ovarian tumors are wrongly being considered a significant recognized etiology of PMP. PMP classification continues to be under discussion, and experts' panels strive for consensus. Malignancy is also under discussion, and it is shown in this review that there is a long-standing historical reason for that. Surgery is the main tool in the treatment armamentarium for PMP, and the only therapy with potential curative option.
PubMed: 31572561
DOI: 10.4240/wjgs.v11.i9.358 -
Chirurgie (Heidelberg, Germany) Oct 2023Pseudomyxoma peritonei syndrome (PMP) is an orphan disease. Surgery is the fundament of treatment. (Review)
Review
BACKGROUND
Pseudomyxoma peritonei syndrome (PMP) is an orphan disease. Surgery is the fundament of treatment.
METHOD
Short review summarizing the state of the art treatment.
RESULTS
Cytoreductive surgery (CRS) in combination with hyperthermic intraperitoneal chemotherapy (HIPEC) form the foundations of treatment for PMP. The peritoneal cancer index should be preoperatively determined based on imaging and/or laparoscopy, intraoperatively validated and both should be documented. An extraperitoneal surgical preparation technique leads to effective en bloc resection of the peritoneum and the affected abdominal area. The HIPEC technique should be performed with mitomycin C for 60-90 min. Complete CRS (CC = 0, CC = 1) and the histological subtype are relevant for the prognosis. Structured educational programs and mentoring can optimize the learning curve. The aftercare should be performed at the surgical center. After follow-up imaging at 3 months after CRS, in the first 2 years a control should be carried out every 6 months. Thereafter, the intervals can be extended to 1 year.
CONCLUSION
Standardized surgical treatment and HIPEC, optimized specific surgical training and structured follow-up at the center lead to an excellent long-term prognosis for patients with PMP.
Topics: Humans; Pseudomyxoma Peritonei; Hyperthermic Intraperitoneal Chemotherapy; Peritoneal Neoplasms; Cytoreduction Surgical Procedures; Combined Modality Therapy; Hyperthermia, Induced
PubMed: 37578542
DOI: 10.1007/s00104-023-01937-3