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Cureus Sep 2022Low-grade appendiceal mucinous neoplasm (LAMN) is a lesion of the appendix with potentially fatal consequences if untreated. Though LAMN can be asymptomatic and stable,...
Low-grade appendiceal mucinous neoplasm (LAMN) is a lesion of the appendix with potentially fatal consequences if untreated. Though LAMN can be asymptomatic and stable, it can rupture and seed mucin and neoplastic epithelium into the peritoneum, leading to pseudomyxoma peritonei (PMP), a serious complication characterized by intraperitoneal accumulation of mucinous tumors and ascites with a high morbidity and mortality rate. Therefore, timely identification and treatment of LAMN are crucial for reducing PMP risk and improving prognosis and outcome. This case series sought to examine five LAMN cases and delineate the strategies for managing LAMN and progression to rupture and PMP.
PubMed: 36211097
DOI: 10.7759/cureus.28755 -
The British Journal of Radiology Feb 2022The peritoneum is a unique serosal membrane, which can be the site of primary tumors and, more commonly, secondary pathologic processes. Peritoneal carcinomatosis is the... (Review)
Review
The peritoneum is a unique serosal membrane, which can be the site of primary tumors and, more commonly, secondary pathologic processes. Peritoneal carcinomatosis is the most common malignant condition to affect the peritoneal cavity, and the radiologist plays an important role in making the diagnosis and assessing the extent of disease, especially in sites that may hinder surgery. In this review, we address the role of the radiologist in the setting of peritoneal pathology, focusing on peritoneal carcinomatosis as this is the predominant malignant process, followed by revising typical imaging findings that can guide the differential diagnosis.We review the most frequent primary and secondary peritoneal tumor and tumor-like lesions, proposing a systemic approach based on clinical history and morphological appearance, namely distinguishing predominantly cystic from solid lesions, both solitary and multiple.
Topics: Ascites; Ascitic Fluid; Carcinoma; Desmoplastic Small Round Cell Tumor; Diagnosis, Differential; Echinococcosis; Endometriosis; Female; Humans; Lymphangioma; Lymphoma; Magnetic Resonance Imaging; Mesothelioma; Neoplasms, Neuroepithelial; Peritoneal Neoplasms; Peritoneum; Peritonitis; Positron Emission Tomography Computed Tomography; Pseudomyxoma Peritonei; Splenosis; Tomography, X-Ray Computed
PubMed: 34767464
DOI: 10.1259/bjr.20210346 -
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 -
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 -
Pseudomyxoma peritonei leading to "jelly belly" abdomen: a case report and review of the literature.Journal of Medical Case Reports Jun 2024Pseudomyxoma peritonei is an infrequent condition with a global annual incidence of only one to two cases per million people. Mucinous neoplasms, widespread... (Review)
Review
BACKGROUND
Pseudomyxoma peritonei is an infrequent condition with a global annual incidence of only one to two cases per million people. Mucinous neoplasms, widespread intraperitoneal implants, and mucinous ascites characterize it. Currently, most clinicians misdiagnose this condition, which leads to delayed management.
CASE PRESENTATION
A 44-year-old North Indian female presented with a 1.5-month history of an abdominal lump. Physical examination revealed a sizeable abdominopelvic mass at 36 weeks. Contrast-enhanced computed tomography showed a massive multiloculated right ovarian cystic mass measuring 28 × 23 × 13 cm with mild ascites and elevated carcinoembryonic antigen levels (113.75 ng/ml). A provisional diagnosis of ovarian mucinous neoplasm was made, for which the patient underwent laparotomy. Intraoperatively, there were gross mucinous ascites, along with a large, circumscribed, ruptured right ovarian tumor filled with gelatinous material. The appendicular lump was also filled with mucinous material along with the omentum, ascending colon, right lateral aspect of the rectum, splenic surface, and small bowel mesentery. Cytoreductive surgery was performed along with an oncosurgeon, including total abdominal hysterectomy with bilateral salpingoophorectomy, omentectomy, right hemicolectomy, lower anterior resection, ileo-transverse stapled anastomosis with proximal ileal loop diversion stoma, excision of multiple peritoneal gelatinous implants, and peritoneal lavage. Histopathology and immunohistochemistry confirmed the presence of intestinal-type mucinous carcinoma. Postoperatively, the patient was given six cycles of chemotherapy. She tolerated it without any specific morbidity and had an uneventful recovery. Postoperative follow-up at 15 months revealed normal tumor marker levels and abdominal computed tomography findings and no signs suggestive of local recurrence or distal metastases.
CONCLUSIONS
Pseudomyxoma peritonei is a rare disease that is frequently misdiagnosed in the preoperative phase. Therefore, radiologists and clinicians should maintain a high index of suspicion for accurate diagnosis and multidisciplinary management.
Topics: Humans; Female; Pseudomyxoma Peritonei; Adult; Peritoneal Neoplasms; Tomography, X-Ray Computed; Cytoreduction Surgical Procedures; Ovarian Neoplasms; Ascites; Hysterectomy; Treatment Outcome
PubMed: 38937808
DOI: 10.1186/s13256-024-04612-1