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Cureus Feb 2022Pseudomyxoma peritonei (PMP) is any mucin-producing tumor in the abdomen. If left untreated, it can compress vital abdominal organs. We describe a misleading...
Pseudomyxoma peritonei (PMP) is any mucin-producing tumor in the abdomen. If left untreated, it can compress vital abdominal organs. We describe a misleading presentation of disseminated peritoneal adenomucinosis (DPAM) of appendiceal origin. Treatment strategies for PMP vary from watchful waiting to cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) or early postoperative intraperitoneal chemotherapy (EPIC). The rarity of PMP has precluded any randomized studies, and few institutions see sufficient cases to report a series. The purpose of this case report is to contribute to reaching a common consensus on the guidelines of pseudomyxoma peritonei. Recent studies support that cytoreduction followed by HIPEC improves survival in patients with peritoneal carcinomatosis of colorectal origin. Our case report introduced a patient with low-grade appendiceal mucinous neoplasm with visceral peritoneal penetration who underwent surgical debulking with promising results that supports the practice. Our patient has also responded well to adjuvant systemic chemotherapy without hyperthermic intraperitoneal chemotherapy.
PubMed: 35340503
DOI: 10.7759/cureus.22255 -
Journal of Gastrointestinal Oncology Apr 2021High-throughput "-omics" analysis may provide a broader and deeper understanding of cancer biology to define prognostic and predictive biomarkers and identify novel... (Review)
Review
High-throughput "-omics" analysis may provide a broader and deeper understanding of cancer biology to define prognostic and predictive biomarkers and identify novel therapy targets. In this review we provide an overview of studies where the peritoneal tumor component of peritoneal metastases from colorectal cancer (PM-CRC) and pseudomyxoma peritonei (PMP) were analyzed. Most of the available data was derived from DNA mutation analysis, but a brief review of findings from transcriptomic and protein expression analysis was also performed. Studies reporting genomic analysis of peritoneal tumor samples from 1,779 PM-CRC and 623 PMP cases were identified. The most frequently mutated genes in PM-CRC were , , , , and , while in PMP , , , , and mutations were most commonly identified. Analyses were performed by single-gene analyses and to some extent targeted next-generation sequencing, and a very limited amount of broad explorative data exists. The investigated cohorts were typically small and heterogeneous with respect to the methods used and to the reporting of clinical data. This was even more apparent regarding transcriptomic and protein data, as the low number of cases examined and quality of clinical data would not support firm conclusions. Even for the most frequently mutated genes, the results varied greatly; for instance, mutations were reported at frequencies between 20-57% in PM-CRC and 38-100% in PMP. Such variation could be caused by random effects in small cohorts, heterogeneity in patient selection, or sensitivity of applied technology. Although a large number of samples have been subjected to analysis, cross-study comparisons are difficult to perform, and combined with small cohorts and varying quality and detail of clinical information, the observed variation precludes useful interpretation in a clinical context. Although omics data in theory could answer questions to aid management decisions in PM-CRC and PMP, the existing data does not presently support clinical implementation. With the necessary technologies being generally available, the main challenge will be to obtain sufficiently large, representative cohorts with adequate clinical data and standardized reporting of results. Importantly, studies where the focus is specifically on peritoneal disease are needed, where the study designs are aligned with clearly defined research questions to allow robust conclusions. Such studies are highly warranted if patients with PM-CRC and PMP are to derive benefit from recent advances in precision cancer medicine.
PubMed: 33968437
DOI: 10.21037/jgo-20-136 -
Cancer Management and Research 2020This study aims to demonstrate clinicopathological characteristics and immunohistopathological phenotypes of pseudomyxoma peritonei (PMP) originated from ovaries.
OBJECTIVE
This study aims to demonstrate clinicopathological characteristics and immunohistopathological phenotypes of pseudomyxoma peritonei (PMP) originated from ovaries.
METHODS
The primary origin of PMP was explored by reviewing H&E sections retrospectively and performing a series of immunohistochemical staining on CK7, CK20, CDX2, CEA, Villin, SATB2, CA125, ER, PR, and MUC.
RESULTS
Among 310 PMP patients, a few originated from extra-appendix, whereas eight cases were of ovarian origin (2.6%), including three teratoma-associated ovarian mucinous tumors and five primary ovarian mucinous tumors with spontaneous or iatrogenic rupture, respectively. Most peritoneal metastases were acellular mucin or low-grade mucinous carcinoma peritonei (6/8, 75%), while the rest were high-grade mucinous carcinoma peritonei (2/8, 25%). Tumors were positive for CK20, CDX2, CEA, and Villin. SATB2 was specifically diffuse positive in teratoma-associated ovarian mucinous tumors, and negative in primary ovarian mucinous tumors. Differential expression of MUC was observed in these tumors.
CONCLUSION
PMP of ovarian origin is extremely rare. The precise diagnosis requires serial sections of the appendix or suspicious tissue to exclude appendiceal mucinous neoplasms, as well as comprehensive analysis of clinical features, surgical findings, histopathological characteristics, and immunohistochemistry on specific biomarkers.
PubMed: 32904568
DOI: 10.2147/CMAR.S264474 -
European Radiology Apr 2023This study aimed to identify the diagnostic accuracy of combined ultrasonography (US) and computed tomography (CT) in evaluating the tumor burden of pseudomyxoma...
OBJECTIVES
This study aimed to identify the diagnostic accuracy of combined ultrasonography (US) and computed tomography (CT) in evaluating the tumor burden of pseudomyxoma peritonei (PMP). Besides, we assessed the ability of this combination to predict the likelihood of complete resection.
METHODS
This retrospective study involved 504 patients diagnosed with PMP and scheduled for cytoreduction surgery. We compared tumor burden-quantified as peritoneal cancer index (PCI) by preoperative US and CT (US-CT-PCI)-with surgical findings. Next, we assessed the prognostic value of US-CT PCI and imaging features in determining the completeness of cytoreduction (CCR) score using multivariate analysis.
RESULTS
US-CT PCI demonstrated a high PCI evaluation accuracy under moderate tumor burden. Higher US-CT PCI could predict incomplete resection. In addition, we identified imaging features such as mesenteric involvement as an independent predictor of incomplete resection (hazard ratio (HR) = 2.006; p = 0.007).
CONCLUSIONS
US-CT PCI allowed us to predict the completeness of cytoreductive surgery in patients with PMP. Moreover, the combined US and CT imaging detected several features indicating incomplete cytoreduction.
KEY POINTS
• Ultrasonography (US) can act as a complementary diagnostic modality in peritoneal cancer index (PCI) evaluation by combining CT in the small bowel area and US in the abdominal area. • A modified peritoneal cancer index (US-CT PCI) helps preoperatively evaluate tumor burden with high accuracy and allows to predict incomplete resection. • US-CT PCI of 20 or above and the involvement of particular structures such as mesentery, independently indicate incomplete resection.
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Retrospective Studies; Prognosis; Tomography, X-Ray Computed; Ultrasonography; Cytoreduction Surgical Procedures; Hyperthermia, Induced; Combined Modality Therapy
PubMed: 36418618
DOI: 10.1007/s00330-022-09242-z -
Cureus May 2022Introduction The appendix is considered an appendage of little value and is often treated disdainfully, be it as part of evolutionary process, on a grossing table, under...
Introduction The appendix is considered an appendage of little value and is often treated disdainfully, be it as part of evolutionary process, on a grossing table, under a microscope or while archiving specimens and slides. It is only recently, with data indicating its importance in gut immunity and as the origin of pseudomyxoma, that its space in a human body appears vindicated. Aim Our aim was to screen the histopathologic spectrum of appendix lesions observed in our hospital for rare, incidental or clinico-radiologically uncertain lesions that would help emphasize a necessary seriousness in its sampling. Method All appendectomy specimens over ten years were screened for diagnosis other than acute/chronic/resolving appendicitis and pseudomyxoma peritonei. Among the recorded rare diagnoses, one representative case each, based on interesting history or pathology, was selected for discussion. Observation Forty-three lesions were found to meet inclusion criteria comprising 12 varied etiologies. Among these, 25 had a normal-appearing appendix and 27 were not suspected on radiology or on clinical/surgical assessment. Histopathology comprised, among others, neoplastic entities such as (Diffuse large B-cell) lymphoma, metastasis, carcinoid as well as interesting non-neoplastic diagnoses such as pinworm infestation (in the elderly) and (post-menopausal) endometriosis. Conclusion Sampling and histopathologic assessment of the appendix should be compulsory, careful and representative. Each specimen must be treated as harboring a potential pathology, until microscopically proven otherwise because missed "rare" diagnoses could delay therapy or alter key management decisions as cancer staging.
PubMed: 35719826
DOI: 10.7759/cureus.25055 -
Frontiers in Surgery 2022To determine prognosis for young female patients with peritoneal pseudomyxoma (PMP) of appendiceal origin and unilateral or bilateral ovaries preserved during...
OBJECTIVE
To determine prognosis for young female patients with peritoneal pseudomyxoma (PMP) of appendiceal origin and unilateral or bilateral ovaries preserved during cytoreductive surgery (CRS).
METHODS
Clinical data of female patients treated with CRS with or without hyperthermic intraperitoneal chemotherapy (HIPEC) at the Aerospace Center Hospital, Beijing between January, 2009 and December, 2019 were retrospectively reviewed. Patients had no changes in the bilateral ovaries on gross pathological observations or biopsy during CRS, and normal ovarian function. The demographic and clinical characteristics and prognosis of women with ovaries preserved (ovarian preservation group) or resected (ovarian resection group) during CRS were compared. Independent prognostic factors for survival were identified using univariate and multivariate analysis.
RESULTS
40 patients were included in the final analysis. 19 patients chose ovarian preservation while 21 patients underwent ovarian resection. Completeness of cytoreduction (CCR) scores were CCR-0/1. There were significant differences in age (<40 vs. ≥40), symptoms, intraoperative HIPEC (Y vs. N), and histopathologic subtype of PMP (low-grade vs. high-grade) ( < 0.001) between patients in the ovarian preservation and ovarian resection groups. In the ovarian preservation group, median overall survival (OS) was 59 months (range, 53-65 months), and the 5-year survival rate was 37.9%. Median disease-free survival (DFS) was 13 months (range, 9-17 months), and the 5-year recurrence rate was 87.4%. In the ovarian resection group, the 5-year survival rate was 87.7%, and the 5-year recurrence rate was 18.3%. Median OS and median DFS were not reached. In patients with low-grade PMP, median DFS was significantly longer in patients with ovarian resection compared to ovarian preservation ( < 0.001). Univariate analysis showed histopathologic subtype of PMP (low-grade vs. high-grade, < 0.001) was significantly associated with OS and DFS. On multivariate analysis, high-grade histopathologic subtype of PMP was an independent predictor of poor prognosis (OS and DFS).
CONCLUSION
Histopathologic subtype of PMP represents an independent predictor of prognosis in female patients with PMP of appendiceal origin and unilateral or bilateral ovaries preserved during CRS. These findings imply that ovarian preservation is a more suitable option for young females with low-grade PMP compared to high-grade PMP. Further prospective studies should be done investigating the role of resection of uninvolved ovaries in PMP.
PubMed: 36034395
DOI: 10.3389/fsurg.2022.881510 -
Pleura and Peritoneum Mar 2017Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is the gold standard treatment for patients with pseudomyxoma peritonei (PMP) but...
BACKGROUND
Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is the gold standard treatment for patients with pseudomyxoma peritonei (PMP) but involves routine bilateral salpingo-oophorectomy. Young women wishing to maintain fertility may be reluctant to pursue this. An alternative strategy in women with low-grade PMP has been explored in the form of laparoscopic evacuation of pelvic and ovarian mucin with resection of the appendiceal tumour.
METHODS
Between January 2012 and January 2015, four young women (aged 28-35 years) with PMP seeking to maintain fertility underwent laparoscopy, appendicectomy and pelvic mucinous evacuation and washout. Data regarding intra-operative and histopathological findings were collected. Endpoints were fertility-related outcomes and oncological follow-up.
RESULTS
Infertility was a presenting symptom in three of the four women. All four had significant pelvic mucinous disease on radiological imaging and were offered CRS and HIPEC as definitive treatment, but chose laparoscopy with appendicectomy and copious irrigation and washout of the pelvis with stripping of mucinous disease off the ovarian surfaces. Postoperative histology demonstrated a low-grade appendiceal mucinous neoplasm (LAMN) in all patients with acellular mucin or low-grade mucinous carcinoma peritonei in the peritoneal cavity. All patients successfully conceived subsequently and gave birth to healthy babies. After 12-29 months follow-up, all women are well with no radiological or laparoscopic evidence of disease recurrence.
CONCLUSIONS
In patients with low-grade PMP, initial therapeutic laparoscopy can restore fertility, whilst providing short- to medium-term disease control. This modality in young women wishing to have children appears to be a feasible alternative to immediate CRS and HIPEC.
PubMed: 30911630
DOI: 10.1515/pp-2016-0024 -
Cancer Medicine Dec 2015Pseudomyxoma peritonei (PMP) is a rare disease exhibiting a distinct clinical feature caused by cancerous cells that produce mucinous fluid in the abdominal cavity. PMPs...
Pseudomyxoma peritonei (PMP) is a rare disease exhibiting a distinct clinical feature caused by cancerous cells that produce mucinous fluid in the abdominal cavity. PMPs originate most frequently from the appendix and less frequently from the ovary. This disease can range from benign to malignant, and histologically, PMP is classified into two types: disseminated peritoneal adenomucinosis (DPAM) representing the milder phenotype, and peritoneal mucinous adenocarcinomas (PMCA) representing the aggressive phenotype. Although histological classification is clinically useful, the pathogenesis of PMP remains largely unknown. To elucidate the molecular mechanisms underlying PMP, we analyzed 18 PMP tumors comprising 10 DPAMs and 8 PMCAs. DNA was extracted from tumor and matched non-tumorous tissues, and was sequenced using Ion AmpliSeq Cancer Panel containing 50 cancer-related genes. Analysis of the data identified a total of 35 somatic mutations in 10 genes, and all mutations were judged as pathological mutations. Mutations were frequently identified in KRAS (14/18) and GNAS (8/18). Interestingly, TP53 mutations were found in three of the eight PMCAs, but not in the DPAMs. PIK3CA and AKT1 mutations were also identified in two PMCAs, but not in the DPAMs. These results suggested that KRAS and/or GNAS mutations are common genetic features of PMP, and that mutations in TP53 and/or genes related to the PI3K-AKT pathway may render malignant properties to PMP. These findings may be useful for the understanding of tumor characteristics, and facilitate the development of therapeutic strategies.
Topics: Aged; Aged, 80 and over; Biomarkers; Combined Modality Therapy; DNA Mutational Analysis; Female; Gene Expression Profiling; Humans; Immunohistochemistry; Male; Middle Aged; Mutation; Neoplasm Grading; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Transcriptome; Tumor Suppressor Protein p53
PubMed: 26475379
DOI: 10.1002/cam4.542 -
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 -
Asian Journal of Surgery Mar 2023To investigate the clinical value of laparoscopic cytoreductive surgery (CRS) in treating of appendiceal pseudomyxoma peritonei with limited disease and low tumor burden.
OBJECTIVE
To investigate the clinical value of laparoscopic cytoreductive surgery (CRS) in treating of appendiceal pseudomyxoma peritonei with limited disease and low tumor burden.
METHODS
The clinical data of patients with appendiceal pseudomyxoma peritonei treated by surgery with CRS at the Aerospace Center Hospital from January 2018 to December 2021 were retrospectively analyzed. The patients were divided into laparoscopic or open CRS groups according to the operation method. A propensity score-matched (PSM) analysis (1:1) was performed, the related clinical variables were compared between the two groups, and the effect on progression-free survival (PFS) was also analyzed.
RESULTS
One hundred and eight patients were included in this study. After PSM, 33 patients were selected from each group and the age and peritoneal cancer index were matched between the two groups. There were significant differences in operation time (P < 0.001), intraoperative bleeding (P < 0.001), intraoperative blood transfusion (P = 0.007), hospital stay (P < 0.001). The analysis of PFS showed that there was no significant difference between the two operation methods. After multivariate analysis, the pathologic subtype (P = 0.012) was identified as an independent prognostic factor for PFS.
CONCLUSION
The curative effect of laparoscopic CRS is like that of open operation, which can significantly shorten the operation time and hospital stay and reduce intraoperative bleeding and blood transfusion event. The laparoscopic CRS is safe and feasible in strictly selected patients. The pathologic subtype is an independent factor affecting the prognosis for PFS.
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Retrospective Studies; Cytoreduction Surgical Procedures; Tumor Burden; Laparoscopy; Hyperthermia, Induced; Combined Modality Therapy; Survival Rate
PubMed: 36064480
DOI: 10.1016/j.asjsur.2022.08.052