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Cancers Nov 2021Pseudomyxoma Peritonei (PMP) is an anatomo-clinical condition characterized by the implantation of neoplastic cells on peritoneal surfaces with the production of a large... (Review)
Review
Pseudomyxoma Peritonei (PMP) is an anatomo-clinical condition characterized by the implantation of neoplastic cells on peritoneal surfaces with the production of a large amount of mucin. The rarity of the disease precludes the evaluation of treatment strategies within randomized controlled trials. Cytoreductive Surgery (CRS) combined with Hyperthermic Intraperitoneal Chemotherapy (HIPEC) has proven to be the only therapeutic option with potential chances of cure and long-term disease control. The present review discusses the epidemiology, pathogenesis, clinical presentation and treatment of PMP, focusing on the molecular factors involved in tumor progression and mucin production that could be used, in the upcoming future, to improve patient selection for surgery and to expand the therapeutic armamentarium.
PubMed: 34885075
DOI: 10.3390/cancers13235965 -
The Surgical Clinics of North America Jun 2020Cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy is an aggressive, potentially curative approach used to treat locoregional disease associated... (Review)
Review
Cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy is an aggressive, potentially curative approach used to treat locoregional disease associated with primary and secondary malignancies of the peritoneum. It involves resection of all macroscopic disease larger than 2.5 mm, followed by instillation of hyperthermic chemotherapy directly into the peritoneum for higher drug exposure to microscopic disease. In select patients with primary peritoneal mesothelioma, pseudomyxoma peritonei, colorectal adenocarcinoma, appendiceal adenocarcinoma, or ovarian cancer, with no extra-abdominal metastasis and limited involvement of the peritoneum, the procedure can be performed to increase overall survival.
Topics: Colorectal Neoplasms; Combined Modality Therapy; Cytoreduction Surgical Procedures; Female; Humans; Hyperthermia, Induced; Mesothelioma; Ovarian Neoplasms; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 32402303
DOI: 10.1016/j.suc.2020.02.009 -
European Journal of Surgical Oncology :... Dec 2022Appendiceal mucinous neoplasms are routinely accompanied by peritoneal metastases at the time of diagnosis of the primary appendiceal tumor. In contrast, liver... (Review)
Review
BACKGROUND
Appendiceal mucinous neoplasms are routinely accompanied by peritoneal metastases at the time of diagnosis of the primary appendiceal tumor. In contrast, liver metastases and lymph node metastases are unusual.
METHODS
From an extensive database, patients with lymph node metastases identified at the time of primary appendiceal cancer resection were selected for special study. The clinical, treatment-related and histologic variables of this group of patients were statistically analyzed for their impact on overall survival.
RESULTS
From a prospectively maintained database of 685 patients with a complete cytoreduction of a mucinous appendiceal neoplasm with peritoneal dissemination, 39 patients (5.6%) had lymph node metastases at the time of primary diagnosis. The median follow-up was 5.0 years and overall median survival was 6.0 years. Histologically, 6 of these patients (15.4%) had an appendiceal mucinous neoplasm - Intermediate type (MACA-Int). In 5 patients, the involved lymph nodes were not within the ileocolic lymph node group. The 7 patients (17.9%) who had a complete or near complete response to neoadjuvant chemotherapy prior to definite cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) showed prolonged survival (HR 4.8 (1.1, 20.5) p = 0.0323). A prior right colon resection required repeat resection in 87% of patients.
CONCLUSION
Long-term survival is unusual but occasionally seen in this group of patients. Response to neoadjuvant chemotherapy is an important determinant of a favorable outcome.
Topics: Humans; Peritoneal Neoplasms; Lymphatic Metastasis; Hyperthermia, Induced; Adenocarcinoma, Mucinous; Pseudomyxoma Peritonei; Appendiceal Neoplasms; Cytoreduction Surgical Procedures; Retrospective Studies; Combined Modality Therapy; Survival Rate
PubMed: 35941031
DOI: 10.1016/j.ejso.2022.07.018 -
Journal of Obstetrics and Gynaecology... Jun 2022
PubMed: 33679016
DOI: 10.1007/s13224-021-01460-7 -
Cleveland Clinic Journal of Medicine Sep 2022
Topics: Humans; Pseudomyxoma Peritonei; Ascites; Peritoneal Neoplasms
PubMed: 37907443
DOI: 10.3949/ccjm.89a.21086 -
Orphanet Journal of Rare Diseases Feb 2021Pseudomyxoma peritonei (PMP) is a clinical malignant syndrome mainly originating from the appendix, with an incidence of 2-4 per million people. As a rare disease, an... (Review)
Review
BACKGROUND
Pseudomyxoma peritonei (PMP) is a clinical malignant syndrome mainly originating from the appendix, with an incidence of 2-4 per million people. As a rare disease, an early and accurate diagnosis of PMP is difficult. It was not until the 1980s that the systematic study of this disease was started.
MAIN BODY
As a result of clinical and basic research progress over the last 4 decades, a comprehensive strategy based on cytoreductive surgery (CRS) + hyperthermic intraperitoneal chemotherapy (HIPEC) has been established and proved to be an effective treatment for PMP. Currently, CRS + HIPEC was recommended as the standard treatment for PMP worldwide. There are several consensuses on PMP management, playing an important role in the standardization of CRS + HIPEC. However, controversies exist among consensuses published worldwide. A systematic evaluation of PMP consensuses helps not only to standardize PMP treatment but also to identify existing controversies and point to possible solutions in the future. The controversy underlying the consensus and vice versa promotes the continuous refinement and updating of consensuses and continue to improve PMP management through a gradual and continuous process. In this traditional narrative review, we systemically evaluated the consensuses published by major national and international academic organizations, aiming to get a timely update on the treatment strategies of CRS + HIPEC on PMP.
CONCLUSION
Currently, consensuses have been reached on the following aspects: pathological classification, terminology, preoperative evaluation, eligibility for surgical treatment, maximal tumor debulking, CRS technical details, and severe adverse event classification system. However, controversies still exist regarding the HIPEC regimen, systemic chemotherapy, and early postoperative intraperitoneal chemotherapy.
Topics: Consensus; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 33581733
DOI: 10.1186/s13023-021-01723-6 -
Colorectal Disease : the Official... Sep 2021Appendiceal pseudomyxoma peritonei (PMP) is a rare entity, with recurrence rates up to 26% despite optimal cytoreductive surgery (CRS) and hyperthermic intraperitoneal...
AIM
Appendiceal pseudomyxoma peritonei (PMP) is a rare entity, with recurrence rates up to 26% despite optimal cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Evidence specific to PMP originating from non-infiltrative appendiceal mucinous neoplasms (low grade - LAMN and high grade - HAMN) is lacking. The aim of this study was to identify patterns of recurrence and predictive factors for patients appropriate for iterative surgery.
METHOD
A bi-institutional retrospective analysis was performed on patients undergoing complete cytoreduction and HIPEC for PMP derived from perforated LAMN or HAMN. Multivariate logistic regression was performed to identify independent predictors for re-do CRS. Five-year overall survival (OS) was stratified according to surgical intervention, and 5-year disease-free survival (DFS) was stratified according to histological PMP grade. Cox regression analysis was performed to identify independent predictors for OS and DFS.
RESULTS
Sixty of 239 (25.1%) patients developed peritoneal recurrence between 2007 and 2020. The median time to recurrence was 20.7 months. The risk of disease recurrence was highest with high-grade PMP (P <0.001) and increasing PCI (P <0.001). Patients with high-grade histology from their index procedure and aged over 60 years were less likely to be offered iterative surgery on multivariate analysis. Patients who underwent iterative CRS and HIPEC had a 5-year survival of 100%.
CONCLUSION
Iterative CRS and HIPEC is feasible in selected patients with recurrent PMP, displaying good oncological outcomes. Age, index histology and level of abdominal quadrant involvement are predictive of proceeding to re-do surgery.
Topics: Aged; Appendiceal Neoplasms; Combined Modality Therapy; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Neoplasm Recurrence, Local; Percutaneous Coronary Intervention; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Retrospective Studies
PubMed: 34157209
DOI: 10.1111/codi.15778 -
Journal of Family Medicine and Primary... Dec 2020Mucocele of the appendix is a very rare disease entity that often discovered incidentally during surgery. It can result from both non-neoplastic and neoplastic lesions... (Review)
Review
Mucocele of the appendix is a very rare disease entity that often discovered incidentally during surgery. It can result from both non-neoplastic and neoplastic lesions and histopathological examination is needed for confirmation. Failure to make an early preoperative diagnosis may results in its rapture and spillage of mucin contents into the peritoneal cavity leading to a disastrous complication of pseudomyxoma peritonei (PMP) that has a very bad prognosis. A clear pathological terminology and management strategies of appendiceal mucocele (AM) is lacking. This literature review aims to derive detailed information related to clinical significance of AM to avoid complication of PMP and plan appropriately during surgery according to the current evidence. The relevant articles from scientific databases such as Medline, PubMed, Google Scholar were searched and extracted using the keywords "mucocele appendix" "cystadenoma%". Data based on epidemiology, clinical manifestations, complications, pathology, diagnostic work up and management were analyzed and summarized. A meticulous surgical excision is the mainstay of treatment and open surgical approach is still preferred over laparoscopy. Preoperative diagnosis of AM is very imperative as it may harbour neoplasm and can be made utilising the imaging tools like computed tomography and ultrasonography. Primary care physicians can have a crucial role in making early detection and timely referral for appropriate management in order to avoid complications. After appendectomy, 5-year survival rate for the simple AM is 91%-100% but it reduces to 25% for the malignant AM.
PubMed: 33681010
DOI: 10.4103/jfmpc.jfmpc_1547_20 -
Radiologia Brasileira 2022Mucoceles of the appendix are rare and can have quite variable imaging and clinical presentations, sometimes mimicking an adnexal mass. The underlying cause can be...
Mucoceles of the appendix are rare and can have quite variable imaging and clinical presentations, sometimes mimicking an adnexal mass. The underlying cause can be neoplastic or non-neoplastic. The typical imaging appearance of a mucocele of the appendix is that of a cystic structure with a tubular morphology. This structure is defined by having a blind-ending and being contiguous with the cecum. Radiologists should be familiar with key anatomical landmarks and with the various imaging features of mucoceles of the appendix, in order to provide a meaningful differential diagnosis of a lesion in the right lower abdominal quadrant. In addition, a neoplastic mucocele can rupture, resulting in pseudomyxoma peritonei, which will change the prognosis dramatically. Therefore, prompt diagnostic imaging is crucial.
PubMed: 35795599
DOI: 10.1590/0100-3984.2021.0075 -
Biomedicines Jul 2023In pseudomyxoma peritonei (PMP), and mutations are frequent. We hypothesized that these mutations may contribute to the suppression of antitumor immunity: may induce...
In pseudomyxoma peritonei (PMP), and mutations are frequent. We hypothesized that these mutations may contribute to the suppression of antitumor immunity: may induce GMCSF expression, while may enhance the expression of cyclic adenosine monophosphate and A2AR signaling. This study aimed to explore possible mechanisms facilitated by and mutations for escaping immune surveillance. Additionally, we looked for new potential therapeutic and prognostic targets in this rare disease which is poorly characterized at the molecular level. GM-CSF, A2AR, CD73, CD39, and PD-L1 expression was investigated by immunohistochemistry in 40 PMPs characterized for and mutational status. Immune cell populations were studied by immunohistochemistry and nanostring nCounter. Following the criteria of a prognostic nomogram reported for PMP, we stratified the patients into two different risk groups, with 28 "low-risk" and 12 "high-risk" patients. We observed the expression of GM-CSF (74%); CD39 (37%); CD73 (53%); A2AR (74%); and PD-L1 (16%) which was unrelated to or status. The tumor microenvironment showed the presence of CD4+ T cells (86%); CD8+ T cells (27%); CD20+ B (67%); CD15+ cells (86%); and CD163+ M2 macrophages (67%), while CD56+ NK cells were absent. CD163 expression (27%) in PMP tumor cells was associated with poor prognosis. mutation and A2AR expression were not associated with a specific immune transcriptional signature. However, the expression assay revealed 21 genes associated with prognosis. The "high-risk" patients exhibited worse progression-free survival (HR = 2.3, CI 95%: 1.1-5.1, = 0.034) and significant downregulation of , , , , , and . In conclusion, we documented the presence of immunosuppressive factors such as GM-CSF, A2AR, and PD-L1 in PMP. These factors were not associated with and status and could be explored as therapeutic molecular targets. Additionally, a set of potential prognostic biomarkers, including CD163 expression in tumor cells, deserve further investigation.
PubMed: 37509688
DOI: 10.3390/biomedicines11072049