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Frontiers in Oncology 2024Pseudomyxoma peritonei (PMP) was a complex disease that had attracted increasing attention. However, there had been no bibliometric analysis of this disease so far. This...
OBJECTIVE
Pseudomyxoma peritonei (PMP) was a complex disease that had attracted increasing attention. However, there had been no bibliometric analysis of this disease so far. This study aimed to explore the current situation and frontier trend of PMP through bibliometric and visualization analysis, and to indicate new directions for future research.
METHODS
The original research articles and reviews related to the PMP research were downloaded from Web of Science Core Collection on September 11, 2023. CiteSpace (6.2.R4) and VOSviewer(1.6.18) were used to perform bibliometric analysis of the publications, and establish the knowledge map. The data collected was analyzed using the Online Analysis Platform of Bibliometric to evaluate the cooperation of countries in this field.
RESULTS
We identified 1449 original articles and reviews on PMP published between 1998 and 2023. The number of publications on PMP increased continuously. The United States, the United Kingdom and China were the top contributors. The most productive organization was the MedStar Washington Hospital Center. Sugarbaker, Paul H. was the most prolific author and the most cited. Keyword analysis showed that "Pseudomyxoma peritonei", "cancer", "cytoreductive surgery", and "hyperthermic intraperitoneal chemotherapy" were the most common keywords. The earliest and latest used keywords were "mucinous tumors" and "impact", respectively. "classification", "cytoreductive surgery", "appendiceal" were the top 3 strongest citation bursts. The reference "Carr NJ, 2016, " had the highest co-citations.
CONCLUSION
This bibliometric analysis showed an increasing trend in literature related to PMP. The research trends and hotspots identified in this study could guide the future research directions in this field, in order to promote the development of PMP.
PubMed: 38390264
DOI: 10.3389/fonc.2024.1323796 -
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 -
World Journal of Clinical Cases Jan 2024Low-grade appendiceal neoplasms (LAMN) are characterized by low incidence and atypical clinical presentations, often leading to misdiagnosis as acute or chronic...
BACKGROUND
Low-grade appendiceal neoplasms (LAMN) are characterized by low incidence and atypical clinical presentations, often leading to misdiagnosis as acute or chronic appendicitis before surgery. The primary diagnostic tool for LAMN is abdominal computed tomography (CT) imaging. Surgical resection remains the cornerstone of LAMN management, necessitating en bloc tumor excision to minimize the risk of iatrogenic rupture. Laparoscopy, known for its minimal invasiveness, reduced postoperative discomfort, and expedited recovery, is a safe and reliable approach for LAMN treatment. Despite the possibility of pseudomyxoma peritonei development, appendectomy and partial appendectomy generally result in negative tumor margins and favorable outcomes, which can be attributed to the disease's slow growth and lower malignancy.
CASE SUMMARY
A 71-year-old male patient was admitted to our hospital with a pelvic space-occupying lesion detected 1 mo prior. Physical examination showed a soft abdomen without tenderness or rebound and no palpable masses. No shifting dullness was noted, and digital rectal examination revealed no palpable mass. Enteroscopy revealed a raised, smooth-surfaced mass measuring 3.0 cm in the cecum. Abdominal contrast-enhanced CT showed a markedly thickened and dilated appendix with visible cystic shadows. Laparoscopic surgery was performed and revealed a significantly dilated appendix, leading to laparoscopic resection of the appendix and part of the cecum. Post-surgical pathologic analysis confirmed LAMN. The patient received symptomatic and supportive post-operative care and was discharged on postoperative day 4 without complications such as abdominal bleeding, intestinal obstruction, or incision infection. No tumor recurrence was observed during a 7-mo follow-up period.
CONCLUSION
LAMN is a rare disease that lacks specific clinical manifestations. Abdominal CT plays a crucial role in diagnosing LAMN, and laparoscopic surgery is a safe and effective diagnostic and therapeutic approach.
PubMed: 38313642
DOI: 10.12998/wjcc.v12.i2.361 -
World Journal of Surgical Oncology Jan 2024The peritoneal cancer index (PCI) has been used to predict surgical outcomes for pseudomyxoma peritonei (PMP). The present study aimed to establish the optimal cutoff...
BACKGROUND
The peritoneal cancer index (PCI) has been used to predict surgical outcomes for pseudomyxoma peritonei (PMP). The present study aimed to establish the optimal cutoff point for PCI to predict surgical resectability of PMP.
METHODS
A total of 366 PMP patients were included. The patients were divided into low-grade and high-grade groups. Based on the completeness of the cytoreduction (CC) score, both low-grade and high-grade PMP patients were further divided into complete cytoreductive surgery (CRS) and maximal tumor debulking (MTD) subgroups. The ability to predict surgical resectability of total and selected PCI (regions 2 + 9 to 12) was analyzed through receiver operating characteristic (ROC) curves.
RESULTS
Both total and selected PCI demonstrated excellent discriminative ability in predicting surgical resectability for low-grade PMP patients (n = 266), with the ROC-AUC of 0.940 (95% CI: 0.904-0.965) and 0.927 (95% CI: 0.889-0.955). The corresponding optimal cutoff point was 21 and 5, respectively. For high-grade PMP patients (n = 100), both total and selected PCI exhibited good performance in predicting surgical resectability, with the ROC-AUC of 0.894 (95% CI: 0.816-0.946) and 0.888 (95% CI: 0.810-0.943); correspondingly, the optimal cutoff point was 25 and 8, respectively. The discriminative ability between total and selected PCI in predicting surgical resectability did not show a statistical difference.
CONCLUSIONS
Both total and selected PCI exhibited good performance and similarity in predicting complete surgical resection for both low-grade and high-grade PMP patients. However, the selected PCI was simpler and time-saving in clinical practice. In the future, new imaging techniques or predictive models may be developed to better predict PCI preoperatively, which might assist in confirming whether complete surgical resection can be achieved.
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Combined Modality Therapy; Hyperthermia, Induced; Cytoreduction Surgical Procedures; Retrospective Studies
PubMed: 38297355
DOI: 10.1186/s12957-024-03318-4 -
Journal of Cancer Research and... Jan 2024Pseudomyxoma peritonei (PMP) is an unusual clinical condition typically presenting with widespread mucinous neoplastic lesions within the peritoneum resulting in gelatin...
BACKGROUND
Pseudomyxoma peritonei (PMP) is an unusual clinical condition typically presenting with widespread mucinous neoplastic lesions within the peritoneum resulting in gelatin material-rich ascites. It was first described by Werth in 1884. Ever since, its clinical presentation, definition, site of origin, and prognosis have been a subject of debate. However, many histopathologic, immunohistochemical, and genetic studies have attempted to locate the primary lesion in the appendix in both genders.
OBJECTIVES
To analyze the histological origin and survival outcomes of pseudomyxoma peritonei in patients treated at a regional cancer center.
MATERIALS AND METHODS
Fifteen cases of PMP were diagnosed during the five-year study period. The demographic and clinicopathological details were retrieved; the slides were reviewed and histological parameters reassessed. Descriptive statistics were used to express proportions. Continuous variables were recorded as mean (SD) or median (IQR). Kaplan-Meier (KM) curve was used to estimate overall survival.
RESULTS
Mean age for PMP was found to be 47.5 years for low grade Mucinous Carcinoma Peritonei (MCP), 54.2 years for high grade MCP, and 58 years for high grade MCP with signet ring cells. Most common overall presentation was abdominal distension in 53.3% (8/15) of cases, followed by acute appendicitis in 20% (3/15) cases. PMP was detected synchronous with the primary tumor in 9/15 cases (60%). Primary lesion in the appendix was grossly identified in 7/15 cases, while it was not explored in the remaining eight cases. Yet, by combined clinical, radiological, histopathological, and immunohistochemical analysis, we identified that most of the cases (14/15) had an appendiceal origin (93.3%). The overall survival for 12 months was 50% and for 18 months was 37%.
CONCLUSION
The surgeon and radiologist may well bear in mind the most common possibility of an appendiceal origin for PMP and resect the appendix, irrespective of the presence of a grossly or radiologically detectable lesions. We emphasize that immunohistochemistry helped to detect the site of origin even when the primary was occult.
PubMed: 38261429
DOI: 10.4103/jcrt.jcrt_191_23 -
Pathology, Research and Practice Feb 2024Epithelial-mesenchymal transition (EMT), angiogenesis, cell adhesion and extracellular matrix (ECM) interaction are essential for colorectal cancer (CRC) metastasis. Low...
Epithelial-mesenchymal transition (EMT), angiogenesis, cell adhesion and extracellular matrix (ECM) interaction are essential for colorectal cancer (CRC) metastasis. Low grade mucinous neoplasia of the appendix (LAMN) and its advanced state low grade pseudomyxoma peritonei (lgPMP) show local aggressiveness with very limited metastatic potential as opposed to CRC. To better understand the underlying processes that foster or impede metastatic spread, we compared LAMN, lgPMP, and CRC with respect to their molecular profile with subsequent pathway analysis. LAMN, lgPMP and (mucinous) CRC cases were subjected to transcriptomic analysis utilizing Poly(A) RNA sequencing. Successfully sequenced cases (LAMN n = 10, 77%, lgPMP n = 13, 100% and CRC n = 8, 100%) were investigated using bioinformatic and statistical tests (differential expression analysis, hierarchical clustering, principal component analysis and gene set enrichment analysis). We identified a gene signature of 28 genes distinguishing LAMN, lgPMP and CRC neoplasias. Ontology analyses revealed that multiple pathways including EMT, ECM interaction and angiogenesis are differentially regulated. Fifty-three significantly differentially regulated gene sets were identified between lgPMP and CRC followed by CRC vs. LAMN (n = 21) and lgPMP vs. LAMN (n = 16). Unexpectedly, a substantial enrichment of the EMT gene set was observed in lgPMP vs. LAMN (FDR=0.011) and CRC (FDR=0.004). Typical EMT markers were significantly upregulated (Vimentin, TWIST1, N-Cadherin) or downregulated (E-Cadherin) in lgPMP. However, MMP1 and MMP3 levels, associated with EMT, ECM and metastasis, were considerably higher in CRC. We show that the different tumor biological behaviour and metastatic spread pattern of midgut malignancies is reflected in a different gene expression profile. We revealed a strong activation of the EMT program in non-metastasizing lgPMP vs. CRC. Hence, although EMT is considered a key step in hematogenous spread, successful EMT does not necessarily lead to hematogenous dissemination. This emphasizes the need for further pathway analyses and forms the basis for mechanistic and therapy-targeting research.
Topics: Humans; Pseudomyxoma Peritonei; Transcriptome; Colorectal Neoplasms; Epithelial-Mesenchymal Transition; Peritoneal Neoplasms; Gene Expression Profiling; Cell Line, Tumor; Cell Movement
PubMed: 38232629
DOI: 10.1016/j.prp.2024.155129 -
Asian Journal of Surgery Apr 2024
Topics: Humans; Pseudomyxoma Peritonei; Appendiceal Neoplasms; Peritoneal Neoplasms
PubMed: 38182506
DOI: 10.1016/j.asjsur.2023.12.114 -
Orphanet Journal of Rare Diseases Jan 2024To explore the application value of hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with incomplete cytoreduction for appendiceal pseudomyxoma peritonei...
BACKGROUND
To explore the application value of hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with incomplete cytoreduction for appendiceal pseudomyxoma peritonei (PMP).
METHODS
We retrospectively analyzed the clinical data of 526 patients with incomplete cytoreduction for appendiceal PMP to discover its prognostic factors, and the therapeutic value of HIPEC.
RESULTS
The 5-year and 10-year overall survival rates of patients after cytoreductive surgery (CRS) treated with HIPEC were significantly higher than those without HIPEC (5y-OS: 58% vs. 48%, 10y-OS: 37% vs. 16%, P = 0.032). The median progression-free survival (PFS) following CRS was 20 months, with a 20% 3-year PFS. The median PFS following CRS + HIPEC was 33 months, with a 60% 3-year PFS (P = 0.000). Univariate analysis indicated that HIPEC, gender, completeness of cytoreduction (CCR) and pathological grade had statistical difference. Multivariate analysis showed that CRS without HIPEC and high pathological grade were independent risk factors for poor prognosis and rapid tumor progression.
CONCLUSIONS
HIPEC may prolong the survival in patients with incomplete cytoreduction for low-grade appendiceal PMP. High pathological grade indicates poor survival and rapid tumor progression.
Topics: Humans; Pseudomyxoma Peritonei; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; Peritoneal Neoplasms; Appendiceal Neoplasms; Retrospective Studies; Hyperthermia, Induced; Survival Rate
PubMed: 38178189
DOI: 10.1186/s13023-023-02995-w -
In Vivo (Athens, Greece) 2024Appendiceal mucinous neoplasms (AMNs) are tumors with dysplastic mucinous epithelium, a pushing growth pattern but no infiltrative invasion to the appendiceal wall. Some...
BACKGROUND/AIM
Appendiceal mucinous neoplasms (AMNs) are tumors with dysplastic mucinous epithelium, a pushing growth pattern but no infiltrative invasion to the appendiceal wall. Some AMNs are associated with pseudomyxoma peritonei, characterized by intraperitoneal mucinous involvement. Recent studies have demonstrated that LAMNs confined to the appendix have low or no risk for disease recurrence, progression, and peritoneal involvement during follow up.
CASE REPORT
Here, we present two extremely rare cases with confined low grade appendiceal mucinous neoplasm (pTis and pT3) and simultaneous extraperitoneal subcutaneous or ovary involvement at the time of diagnosis.
CONCLUSION
Our cases demonstrate that although the primary tumor is limited to the appendix, coexisting distant metastasis may occur on very rare occasions.
Topics: Female; Humans; Peritoneal Neoplasms; Neoplasm Recurrence, Local; Appendiceal Neoplasms; Pseudomyxoma Peritonei; Ovary
PubMed: 38148061
DOI: 10.21873/invivo.13438 -
Journal of Investigative Medicine High... 2023Pseudomyxoma peritonei (PMP) is a rare clinical entity characterized by widespread mucinous implants in the peritoneal cavity. Commonly seen in females in their 50s, PMP...
Pseudomyxoma peritonei (PMP) is a rare clinical entity characterized by widespread mucinous implants in the peritoneal cavity. Commonly seen in females in their 50s, PMP typically originates from ruptured appendiceal mucoceles that find refuge in the peritoneal space. Rarely, PMP may originate from the ovary, stomach, colon, or pancreas. Pseudomyxoma peritonei of colorectal origin is more malignant and has a lower survival rate. We report a case of a 59-year-old Hispanic woman with PMP who presented to the emergency room with a 3-month history of progressive abdominal distention. Pseudomyxoma peritonei was confirmed by computed tomography (CT) scan of the abdomen and pelvis and histopathology, and the patient underwent partial cytoreductive surgery. Given her Eastern Cooperative Oncology Group (ECOG) performance status of 1 despite extensive carcinomatosis, our patient may benefit from hyperthermic intraperitoneal chemotherapy (HIPEC) in the future.
Topics: Female; Humans; Middle Aged; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Mucocele; Appendiceal Neoplasms
PubMed: 38102072
DOI: 10.1177/23247096231217822