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Innovative Surgical Sciences Mar 2024Treatment of peritoneal surface malignancies makes physicians face demanding and new-fangled problems, as there are many uncertain aspects considering the outcomes of... (Review)
Review
Treatment of peritoneal surface malignancies makes physicians face demanding and new-fangled problems, as there are many uncertain aspects considering the outcomes of affected patients' prognoses. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) are associated with favorable long-term outcomes in carefully selected patients with peritoneal metastases (PM). We aim to summarize the current results about the initial malignancies and their peritoneal spreads. The current literature has been scrutinized, and studies between 2016 and 2022 were included wherein long-term, progression-free (PFS), and overall survival (OS) data were considered relevant information. Medline, Embase, and Google Scholar have been the main sources. Hereby, we cover all the primer malignancies: gastric, ovarian, and colorectal cancers with peritoneal metastases (PM), malignant peritoneal mesothelioma, and pseudomyxoma peritonei. Examining the advances in the current peer-reviewed literature about the indications of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), target groups, risk factors, and other influencing elements, we intend to provide a complex state-of-the-art report, establishing the relevant aspects of that emerging treatment method.
PubMed: 38826635
DOI: 10.1515/iss-2023-0055 -
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 -
Scandinavian Journal of Surgery : SJS :... Jun 2024Pseudomyxoma peritonei (PMP) is a rare disease characterized by progressive build-up of mucinous deposits inside the abdominal cavity. The aim of this study was to...
BACKGROUND AND AIMS
Pseudomyxoma peritonei (PMP) is a rare disease characterized by progressive build-up of mucinous deposits inside the abdominal cavity. The aim of this study was to investigate the effect of disease recurrence on overall survival in patients with PMP after cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC).
METHODS
One-hundred thirty-two consecutive PMP patients treated with CRS + HIPEC at Helsinki University Hospital between 2008 and 2017 were included. The impact of clinicopathological and treatment-related characteristics on recurrence and overall survival was evaluated.
RESULTS
The median follow-up time in the study was 5.04 (range = 0.05-11.60) years. In 121 (91.7%) patients, the disease was classified as low grade and 11 (8.3%) had high-grade disease. In the low-grade group, 26 (21.5%) patients developed a recurrence during follow-up compared to 6 (54.5%) patients in the high-grade group. In the low-grade group, cumulative survival was 98.2%, 91.4%, and 91.4% at 3, 6, and 8 years, respectively. In the high-grade group, cumulative survival was 90.0% and 78.8% at 3 and 6 years, respectively. In patients with recurrent disease, the cumulative survival was 100%, 84.6%, and 84.6% at 3, 6, and 8 years in the low-grade category and 80.0% and 60.0% at 3 and 6 years in the high-grade category, respectively. In the low-grade group, a statistically significant correlation with recurrence but not with overall survival was identified with peritoneal cancer index (PCI), carcinoembryonic antigen (CEA), and the number of affected regions.
CONCLUSION
The recurrence of low-grade PMP does not significantly affect overall survival of patients. Disease extent may not be a prognostic indicator after curative CRS and HIPEC in low-grade PMP.
Topics: Humans; Pseudomyxoma Peritonei; Cytoreduction Surgical Procedures; Female; Male; Middle Aged; Retrospective Studies; Peritoneal Neoplasms; Hyperthermic Intraperitoneal Chemotherapy; Aged; Neoplasm Recurrence, Local; Adult; Combined Modality Therapy; Survival Rate; Finland
PubMed: 37828760
DOI: 10.1177/14574969231200653 -
Cureus Oct 2023Low-grade appendiceal mucinous neoplasm (LAMN) is an uncommon tumor of the appendix that is usually diagnosed incidentally after surgery. Although LAMN may be...
BACKGROUND
Low-grade appendiceal mucinous neoplasm (LAMN) is an uncommon tumor of the appendix that is usually diagnosed incidentally after surgery. Although LAMN may be asymptomatic, it can rupture and seed mucin and neoplastic epithelium into the peritoneum, causing pseudomyxoma peritonei (PMP).
MATERIALS AND METHODS
Data from 53 patients were retrospectively analyzed. Age, sex, tumor size, margin status, peritoneal carcinomatosis index, surgical procedures, postoperative results with histologic diagnosis, T stage, recurrence, and mortality of the patients were evaluated.
RESULTS
Appendectomy was performed in 37 patients, right hemicolectomy in nine patients, cytoreductive surgery in one patient, and cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in six patients. Recurrence occurred in four patients. Of the patients who developed recurrence, one patient had stage T4a disease, and the other three patients had T4b disease (p<0.001). Eighteen patients had acellular mucin in the serosa and four of these patients developed recurrence (p=0.004). Twelve patients had appendix perforation, and three of these patients had PMP on exploration (p<0.001). The mean survival time was 93.3 months in patients without recurrence and 32 months in patients with recurrence (p=0.021).
CONCLUSIONS
Low-grade appendiceal mucinous neoplasms are rare appendiceal tumors. The appropriate management of this tumor is controversial. T stage, appendix perforation, presence of acellular mucin on the serosa, and surgical margins are risk factors for the development of PMP. Appendectomy is considered sufficient when there are no risk factors for Tis (LAMN) and T3 disease. Right hemicolectomy may be sufficient if there are no risk factors for T4a disease, but cytoreductive surgery and hyperthermic intraperitoneal chemotherapy seem to be the most appropriate treatments in the presence of the stated risk factors for T4b disease.
PubMed: 37808597
DOI: 10.7759/cureus.46591 -
World Journal of Surgical Oncology Oct 2023As the standard treatment for pseudomyxoma peritonei (PMP), cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) can significantly prolong...
BACKGROUND
As the standard treatment for pseudomyxoma peritonei (PMP), cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) can significantly prolong the survival of PMP patients, and some patients can even achieve long-term survival (LTS) or clinical cure. The purpose of this study was to analyze the clinicopathological and treatment features of PMP patients with LTS and to explore the survival benefit factors of PMP patients.
METHODS
The clinicopathological and prognostic data of PMP patients who received CRS + HIPEC at our center from December 2004 to May 2023 were retrospectively analyzed. PMP patients were divided into LTS group (≥ 10 years) and short-term survival (STS) group (< 5 years) according to the length of natural history. Univariate and multivariate analyses were performed to explore the beneficial factors of PMP patients with LTS.
RESULTS
A total of 609 patients with PMP received CRS + HIPEC treatment at our center. Two-hundred one patients with PMP were included in the study after screening, including 39 patients (19.4%) in the LTS group and 162 patients (80.6%) in the STS group. In STS group and LTS group, median overall survival based on natural history was 29.2 (2.4-59.9) vs. 138.9 (120.3-416.7) months. Univariate analysis revealed 8 factors (P < 0.05) with statistically significant differences between the two groups: gender, chemotherapy history, previous surgical score, Karnofsky Performance Status score, pathological diagnosis, lymphatic metastasis, peritoneal cancer index, and completeness of cytoreduction (CC). Multivariate analysis identified only two factors independently associated with LTS of PMP patients: CC and pathological diagnosis.
CONCLUSION
Complete CRS and pathological features are two key factors affecting LTS in PMP patients.
Topics: Humans; Pseudomyxoma Peritonei; Retrospective Studies; Peritoneal Neoplasms; Combined Modality Therapy; Hyperthermia, Induced; Cytoreduction Surgical Procedures; China; Survival Rate
PubMed: 37891655
DOI: 10.1186/s12957-023-03232-1 -
Cancer Diagnosis & Prognosis 2024Pseudomyxoma peritonei (PMP) is a clinical entity of subtle onset abdominal pain, ascites, and distention associated with characteristic imaging. In most cases,...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a clinical entity of subtle onset abdominal pain, ascites, and distention associated with characteristic imaging. In most cases, laparoscopic exploration will give the definitive diagnosis and histopathologic verification. However, usually there are difficulties in the diagnosis of this disease.
CASE REPORT
Herein, we present a case of a 51-year-old female who developed ascites over 5 months. An investigational laparotomy established the diagnosis of PMP, after the discovery of a mucinous, grey-brown tumor that was CK20 positive and CK7 negative. Subsequently, chemotherapy with oxaliplatin combined with 5-FU (FOLFOX4 regimen), was initiated and the patient survived for 30 months. We also present a comprehensive review of the English literature concerning the different symptoms and radiological findings of this rare entity. According to the literature review, 35 cases of PMP with different clinical and radiological findings have been described. In the majority of the cases, ultrasound, computed tomography or magnetic resonance imaging was orientating towards a proper diagnosis before a diagnostic laparotomy.
CONCLUSION
The combination of a clinical picture with the characteristic imaging findings enables a prompt diagnosis of PMP, making prognosis more favorable.
PubMed: 38434922
DOI: 10.21873/cdp.10308 -
Annals of Surgery Open : Perspectives... Sep 2023To describe the long-term survival and clinical- and treatment-related variables that determine the outcome of repeat cytoreductive surgery (CRS) for mucinous... (Review)
Review
OBJECTIVE
To describe the long-term survival and clinical- and treatment-related variables that determine the outcome of repeat cytoreductive surgery (CRS) for mucinous appendiceal neoplasms with peritoneal dissemination.
SUMMARY BACKGROUND
After patients with peritoneal dissemination of an appendiceal mucinous neoplasm have a CRS, disease progression may require secondary cytoreductive surgery (SCRS) and other treatments performed in a timely manner to prolong survival and help preserve an optimal quality of life.
METHODS
The clinical- and treatment-related variables associated with the index CRS and the SCRS were statistically assessed for their impact on survival.
RESULTS
One hundred eighty-six of 687 complete CRS patients (27.1%) had SCRS. The median follow-up was 10 years and the median survival was 12 years. There were 95 males (51%) and the median age was 45.0 years. Survival benefit was associated with the index CRS by use of early postoperative intraperitoneal chemotherapy (EPIC) with 5-fluorouracil [Hazard ratio (HR), 0.4; = 0.0004]. Also, survival of low-grade mucinous appendiceal neoplasms versus mucinous appendiceal adenocarcinoma (HR, 2.8; < 0.0001) was improved. The interval between index CRS and SCRS was significant at ≤12 months versus 12-36 months versus >36 months ( < 0.0001). Change in peritoneal cancer index and disease distribution as focal or diffuse was significant by univariant and multivariant analyses.
CONCLUSIONS
If the CRS was complete, the use of EPIC 5-fluorouracil, the interval between the index CRS and the SCRS, the histologic grade of the mucinous neoplasm, and the extent of recurrent disease were prognostic variables that should be used to help select patients for SCRS.
PubMed: 37746617
DOI: 10.1097/AS9.0000000000000335 -
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 -
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