-
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 -
Current Oncology (Toronto, Ont.) Nov 2023Patients with low-grade appendiceal mucinous carcinomas (LAMNs) treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have a... (Review)
Review
Patients with low-grade appendiceal mucinous carcinomas (LAMNs) treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have a favorable prognosis. However, a subgroup of patients presents a clinically aggressive course with disease progression despite receiving treatment. The purpose of this study is to report the experience of clinically aggressive LAMN patients treated by the same team, and to present a review of the literature. The cases of four patients with clinically aggressive LAMNs were reviewed. Clinical and histopathological characteristics were re-examined. Recurrences and the time of recurrence, as well as the survival time, were recorded. These patients were four men with clinically aggressive LAMNs treated with CRS plus HIPEC. One of them underwent CC-0 surgery, two underwent CC-1 surgery, and one underwent CC-3 surgery. All patients received systemic chemotherapy after surgery. Recurrence was recorded in three of the patients within 4-23 months after the initial treatment. Two of the patients underwent secondary CRS. Three patients died of disease recurrence within 13-23 months, and one is alive with a disease relapse at 49 months after his initial surgery. LAMNs were identified in both the initial specimens and the specimens obtained during reoperation. The prognosis of LAMN patients treated with CRS plus HIPEC is favorable. A small number of patients present a clinically aggressive course that is unresponsive to any treatment. Molecular and genetic studies are required to identify this group of LAMN patients who have an unfavorable prognosis.
Topics: Humans; Male; Appendiceal Neoplasms; Combined Modality Therapy; Hyperthermia, Induced; Neoplasm Recurrence, Local; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 37999146
DOI: 10.3390/curroncol30110726 -
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 -
Anticancer Research Oct 2023Pseudomyxoma peritonei (PMP) is a rare clinical condition of progressive peritoneal mucin accumulation. PMP has a reasonable survivability but with a notable risk of...
BACKGROUND/AIM
Pseudomyxoma peritonei (PMP) is a rare clinical condition of progressive peritoneal mucin accumulation. PMP has a reasonable survivability but with a notable risk of tumour recurrence. Standard treatment, including for tumour relapse, aims for a cure with complete cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. In the case of tumour recurrence, surgery becomes progressively complex, and some patients are not suitable for surgery either due to patient preference or morbidity and mortality risk. BromAc is an emerging, novel mucolytic combination therapy composed of bromelain and acetylcysteine which can be administered intratumorally via radiologically guided drains. It represents a minimally invasive treatment for patients who have symptomatic tumour deposits but are not surgical candidates.
CASE REPORT
We report the case of a 64-year-old male with a background of appendiceal PMP who presented with a gastric outlet obstruction from a perigastric tumour deposit. This was managed with BromAc administration, following which the patient's symptoms resolved. This corresponded with an 80% reduction in the tumour volume radiologically.
CONCLUSION
BromAc is an emerging minimally invasive treatment for PMP tumour deposits that may be considered as adjunctive or alternative treatment in patients who are not surgical candidates to reduce tumour burden and improve symptomatology and quality of life.
PubMed: 37772592
DOI: 10.21873/anticanres.16670 -
Scientific Reports Dec 2023Peritoneal cancer index (PCI) is the surgical variable most commonly used to quantify the extent of peritoneal metastases for pseudomyxoma peritonei (PMP) patients. The...
Peritoneal cancer index (PCI) is the surgical variable most commonly used to quantify the extent of peritoneal metastases for pseudomyxoma peritonei (PMP) patients. The present study aimed to investigate the agreement between CT predicted and surgical PCI by the Bland-Altman method for PMP of appendiceal origin. A total of 167 PMP patients of appendiceal origin were included between 2016 and 2021. Bland-Altman analysis was performed for both total PCI and selected PCI (regions 2 + 9-12). After the Bland-Altman plot was drawn, the mean bias and its 95% limit of agreements (LoAs) was quantified. Besides, the correlation coefficients between CT-PCI and surgical PCI were also been calculated. The Bland-Altman plot showed the mean bias ± SD between total CT-PCI and surgical PCI as 0.431 ± 3.005, with the LoAs from - 5.459 to 6.321. There were nine points of difference in total PCI exceeded the 95% LoAs, with the rate of 5.39% (9/167). As for selected CT-PCI, Bland-Altman plot showed the mean bias ± SD between selected CT-PCI and surgical PCI as - 0.287 ± 1.955, with the LoAs from - 4.118 to 3.544. There were ten points of difference in selected PCI exceeded the 95% LoAs, with the rate of 5.99% (10/167). The Spearman's rank correlation coefficient between total CT-PCI and surgical PCI was 0.911, P < 0.001, as for selected CT-PCI and surgical PCI, the coefficient was 0.909, P < 0.001. Although there was a strong correlation for both total and selected CT-PCI with surgical PCI, however, the agreement is still not good in Bland-Altman analysis, which suggested that CT-PCI cannot predict surgical PCI accurately even in professional PMP treatment centers. In brief explanation, CT makes it difficult to distinguish the borderline between tumor tissue and mucus and to detect tumor lesions in the small intestine regions, which caused overestimation or underestimation by CT-PCI. In the future, a multiple linear regression model based on CT-PCI might accurately predict surgical PCI preoperatively.
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Appendix; Peritoneum; Tomography, X-Ray Computed; Retrospective Studies
PubMed: 38057378
DOI: 10.1038/s41598-023-48975-9 -
Human Cell Mar 2024Pseudomyxoma peritonei (PMP) is a rare phenomenon, characterized by accumulation of mucus in the abdominal cavity due to a mucinous neoplasm. Histologically, PMP is...
Pseudomyxoma peritonei (PMP) is a rare phenomenon, characterized by accumulation of mucus in the abdominal cavity due to a mucinous neoplasm. Histologically, PMP is divided into three prognostic classes, namely low-grade mucinous carcinoma peritonei (LGMCP), high-grade mucinous carcinoma peritonei (HGMCP), and high-grade mucinous carcinoma peritonei with signet ring cells (HGMCP-S); HGMCP-S exhibits the worst prognosis. Complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy have been established as the standard therapy for PMP. However, 50% of patients with PMP experience a recurrence, and 30-40% are unable to receive the standard treatment due to invasive diseases. Therefore, novel therapies are required for their treatment. Although patient-derived cell lines are important tools for basic and pre-clinical research, PMP cell lines derived from patients with HGMCP-S have never been reported. Thus, we established a novel PMP cell line NCC-PMP2-C1, using surgically resected tumor tissue from a patient with HGMCP-S. NCC-PMP2-C1 cells were maintained for more than five months and passaged 30 times under culture conditions. NCC-PMP2-C1 cells exhibited multiple deletions and somatic mutations, slow growth, histological features, and dissemination of tumor cells in nude mice. Screening for the anti-proliferative effects of anti-cancer drugs on cells revealed that bortezomib, mubritinib, and romidepsin had a significant response against NCC-PMP2-C1 cells. Thus, the NCC-PMP2-C1 cell line is the first PMP cell line harboring signet ring cells and will be a valuable resource for basic and preclinical studies of HGMCP-S.
Topics: Animals; Mice; Humans; Pseudomyxoma Peritonei; Mice, Nude; Peritoneal Neoplasms; Adenocarcinoma, Mucinous; Carcinoma, Signet Ring Cell; Myelin P2 Protein
PubMed: 38143259
DOI: 10.1007/s13577-023-01015-0 -
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 -
Anticancer Research May 2024Pseudomyxoma peritonei (PMP) is a rare condition characterized by diffuse spread of mucinous tumors within the peritoneal cavity. Traditional treatment modalities, such...
BACKGROUND/AIM
Pseudomyxoma peritonei (PMP) is a rare condition characterized by diffuse spread of mucinous tumors within the peritoneal cavity. Traditional treatment modalities, such as cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) are challenging in cases of recurrent disease, owing to anatomical complexities and increasing morbidity and mortality risk. BromAc has emerged as a novel, targeted therapy for PMP with evidence for intra-tumoral administration to break down mucin deposits.
CASE REPORT
We present a 70-year-old female with confirmed diagnosis of symptomatic appendiceal PMP situated behind the stomach, refractory to prior CRS and HIPEC. Transhepatic intra-tumor injection of BromAc was performed, guided by imaging, with catheter placement into the posterior gastric mucinous tumor. The procedure was well-tolerated, and post-treatment imaging revealed a significant 40% reduction in tumor burden. The patient had fever on cycle days two and three, which self-resolved and septic screen performed was negative. Following BromAc administration, the patient demonstrated improvement in symptoms and quality of life.
CONCLUSION
This case highlights the potential efficacy and safety of transhepatic administration of BromAc for the treatment of recurrent PMP behind the stomach. The targeted delivery of BromAc directly into a mucinous tumor via the transhepatic route offers a minimally invasive alternative for cases where traditional surgical interventions pose challenges. However, further research and clinical trials are warranted to validate the broader applicability of this novel approach, assess long-term outcomes, and optimize procedural parameters for enhanced therapeutic outcomes in PMP treatment.
Topics: Humans; Female; Aged; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Stomach Neoplasms
PubMed: 38677761
DOI: 10.21873/anticanres.17031 -
The British Journal of Surgery Mar 2024
Topics: Humans; Pseudomyxoma Peritonei; Acetylcysteine; Bromelains; Peritoneal Neoplasms; Adenocarcinoma, Mucinous
PubMed: 38477594
DOI: 10.1093/bjs/znae045