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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 -
Medicina (Kaunas, Lithuania) Jan 2023The peritoneum is a common site for the dissemination of digestive malignancies, particularly gastric, colorectal, appendix, or pancreatic cancer. Other tumors such as... (Review)
Review
The peritoneum is a common site for the dissemination of digestive malignancies, particularly gastric, colorectal, appendix, or pancreatic cancer. Other tumors such as cholangiocarcinomas, digestive neuroendocrine tumors, or gastrointestinal stromal tumors (GIST) may also associate with peritoneal surface metastases (PSM). Peritoneal dissemination is proven to worsen the prognosis of these patients. Cytoreductive surgery (CRS), along with systemic chemotherapy, have been shown to constitute a survival benefit in selected patients with PSM. Furthermore, the association of CRS with hyperthermic intraperitoneal chemotherapy (HIPEC) seems to significantly improve the prognosis of patients with certain types of digestive malignancies associated with PSM. However, the benefit of CRS with HIPEC is still controversial, especially due to the significant morbidity associated with this procedure. According to the results of the PRODIGE 7 trial, CRS for PSM from colorectal cancer (CRC) achieved overall survival (OS) rates higher than 40 months, but the addition of oxaliplatin-based HIPEC failed to improve the long-term outcomes. Furthermore, the PROPHYLOCHIP and COLOPEC trials failed to demonstrate the effectiveness of oxaliplatin-based HIPEC for preventing peritoneal metastases development in high-risk patients operated for CRC. In this review, we discuss the limitations of these studies and the reasons why these results are not sufficient to refute this technique, until future well-designed trials evaluate the impact of different HIPEC regimens. In contrast, in pseudomyxoma peritonei, CRS plus HIPEC represents the gold standard therapy, which is able to achieve 10-year OS rates ranging between 70 and 80%. For patients with PSM from gastric carcinoma, CRS plus HIPEC achieved median OS rates higher than 40 months after complete cytoreduction in patients with a peritoneal cancer index (PCI) ≤6. However, the data have not yet been validated in randomized clinical trials. In this review, we discuss the controversies regarding the most efficient drugs that should be used for HIPEC and the duration of the procedure. We also discuss the current evidence and controversies related to the benefit of CRS (and HIPEC) in patients with PSM from other digestive malignancies. Although it is a palliative treatment, pressurized intraperitoneal aerosolized chemotherapy (PIPAC) significantly increases OS in patients with unresectable PSM from gastric cancer and represents a promising approach for patients with PSM from other digestive cancers.
Topics: Humans; Oxaliplatin; Peritoneal Neoplasms; Antineoplastic Combined Chemotherapy Protocols; Peritoneum; Stomach Neoplasms; Hyperthermia, Induced; Combined Modality Therapy; Survival Rate; Retrospective Studies; Colorectal Neoplasms
PubMed: 36837456
DOI: 10.3390/medicina59020255 -
Cancers Feb 2023Pseudomyxoma peritonei (PMP) is a rare peritoneal condition where mucus-secreting tumorous cells progressively produce a thick, gelatin-like substance. The prognosis of...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare peritoneal condition where mucus-secreting tumorous cells progressively produce a thick, gelatin-like substance. The prognosis of patients with PMP is determined by the degree of cellularity within the mucin (low-grade (LAMN) vs. high-grade (HAMN) histologic features) and by the extent of the disease.
METHODS
Prognostic relevance of tumor markers CA19-9 and CEA, gender, Peritoneal Cancer Index (PCI), and completeness of cytoreduction (CC) after cytoreductive surgery were evaluated on 193 consecutive PMP patients, based on a retrospective analysis of prospectively gathered data from a German tertial referral center.
RESULTS
We demonstrated that low PCI, CC0 status, low-grade histology, and female gender were independent positive prognostic factors for both overall survival (OS) and progression-free survival (PFS). Furthermore, LAMN patients with achieved CC0 status show significantly better OS and PFS compared to those with CC1 status ( = 0.0353 and = 0.0026 respectively). In contrast, the duration and drug of hyperthermic intraperitoneal chemotherapy (HIPEC) were not prognostic in any comparison. Increased CA19-9 and CEA levels were significantly associated with HAMN cases, but also predicted recurrence in patients with low-grade histologies.
CONCLUSION
Our study confirmed the prognostic role of tumor markers and emphasized the importance of CC status and PCI in a large cohort of PMP- and LAMN patients.
PubMed: 36831667
DOI: 10.3390/cancers15041326 -
Journal of Personalized Medicine Feb 2022Pseudomyxoma peritonei (PMP) is the intraperitoneal accumulation of mucus due to a mucinous tumor. PMP predominantly occurs in low-grade carcinomas. The incidence rate...
Pseudomyxoma peritonei (PMP) is the intraperitoneal accumulation of mucus due to a mucinous tumor. PMP predominantly occurs in low-grade carcinomas. The incidence rate of PMP is one to two cases per million people per year. The standard therapy of PMP comprises complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. PMP recurs in about 50% of patients, and 30-40% are unable to receive the standard treatment because of its invasiveness. Therefore, novel therapies are of the utmost necessity. For basic and pre-clinical research, patient-derived cell lines are essential resources. However, only two PMP cell lines have been reported. Thus, we established a novel PMP cell line from resected metastatic PMP tissue. The cell line, named NCC-PMP1-C1, was maintained for more than 5 months and was passaged 25 times. NCC-PMP1-C1 cells demonstrated multiple amplifications and deletions, slow growth, tumorigenic ability, and dissemination of tumor cells in nude mice. We also used NCC-PMP1-C1 cells to screen drugs, which demonstrated a significant response to daunorubicin HCl, homoharringtonine, mitomycin C, and ponatinib. The NCC-PMP1-C1 cell line is the first PMP cell line derived from metastasized tissue and will be a potential resource for basic and pre-clinical research of metastasized PMP.
PubMed: 35207746
DOI: 10.3390/jpm12020258 -
Cureus Oct 2023Low-grade appendiceal mucinous neoplasm (LAMN) is an uncommon tumor of the appendix that is usually diagnosed incidentally after surgery. Although LAMN may be...
BACKGROUND
Low-grade appendiceal mucinous neoplasm (LAMN) is an uncommon tumor of the appendix that is usually diagnosed incidentally after surgery. Although LAMN may be asymptomatic, it can rupture and seed mucin and neoplastic epithelium into the peritoneum, causing pseudomyxoma peritonei (PMP).
MATERIALS AND METHODS
Data from 53 patients were retrospectively analyzed. Age, sex, tumor size, margin status, peritoneal carcinomatosis index, surgical procedures, postoperative results with histologic diagnosis, T stage, recurrence, and mortality of the patients were evaluated.
RESULTS
Appendectomy was performed in 37 patients, right hemicolectomy in nine patients, cytoreductive surgery in one patient, and cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in six patients. Recurrence occurred in four patients. Of the patients who developed recurrence, one patient had stage T4a disease, and the other three patients had T4b disease (p<0.001). Eighteen patients had acellular mucin in the serosa and four of these patients developed recurrence (p=0.004). Twelve patients had appendix perforation, and three of these patients had PMP on exploration (p<0.001). The mean survival time was 93.3 months in patients without recurrence and 32 months in patients with recurrence (p=0.021).
CONCLUSIONS
Low-grade appendiceal mucinous neoplasms are rare appendiceal tumors. The appropriate management of this tumor is controversial. T stage, appendix perforation, presence of acellular mucin on the serosa, and surgical margins are risk factors for the development of PMP. Appendectomy is considered sufficient when there are no risk factors for Tis (LAMN) and T3 disease. Right hemicolectomy may be sufficient if there are no risk factors for T4a disease, but cytoreductive surgery and hyperthermic intraperitoneal chemotherapy seem to be the most appropriate treatments in the presence of the stated risk factors for T4b disease.
PubMed: 37808597
DOI: 10.7759/cureus.46591 -
Annals of Oncology : Official Journal... Nov 2016Outcome of pseudomyxoma peritonei (PMP) after cytoreductive surgery (CRS) and hypertermic intraperitoneal chemotherapy (HIPEC) is heterogeneous even after adjusting for...
BACKGROUND
Outcome of pseudomyxoma peritonei (PMP) after cytoreductive surgery (CRS) and hypertermic intraperitoneal chemotherapy (HIPEC) is heterogeneous even after adjusting for clinico-pathological prognostic variables. The identification of additional prognostic or even predictive biomarkers is an unmet clinical need.
PATIENTS AND METHODS
Forty patients with mucinous appendiceal tumors and PMP were clinically eligible and had evaluable tumor samples obtained after CRS and HIPEC. We carried out next-generations sequencing (NGS) of 50 gene's hotspot regions contained in the Hotspot Cancer Panel v2 using the Ion Torrent Personal Genome Machine platform (Life Technologies).
RESULTS
KRAS and GNAS mutations were found in 72% and 52%, and their allelic frequency was below 10% in 55% and 43% of samples, respectively. KRAS and GNAS mutations were associated with worse progression-free survival (PFS) at univariate analysis (P = 0.006 and 0.011, respectively). At multivariate analysis, only KRAS mutations were independently associated with PFS (P = 0.012); GNAS mutations were not-being significantly associated with other poor prognostic features such as incomplete cytoreduction or KRAS mutations. Validation of results was carried out in an independent bi-institutional cohort of 25 patients and the prognostic effect of KRAS mutations was again confirmed in the multivariate model (P = 0.029). NGS approach allowed the discovery of other potentially druggable mutations such as those in PI3K, AKT, LKB1, FGFR3 and PDGFRA.
CONCLUSIONS
Given the homogeneity of this series and the sensitivity of NGS in this low-cellularity tumor, we demonstrated for the first time a poor prognostic role of KRAS mutations.
Topics: Adult; Aged; Biomarkers, Tumor; Chromogranins; Cisplatin; Combined Modality Therapy; Cytoreduction Surgical Procedures; Disease-Free Survival; Female; GTP-Binding Protein alpha Subunits, Gs; High-Throughput Nucleotide Sequencing; Humans; Hyperthermia, Induced; Male; Middle Aged; Mutation; Prognosis; Proto-Oncogene Proteins p21(ras); Pseudomyxoma Peritonei
PubMed: 27502722
DOI: 10.1093/annonc/mdw314 -
Frontiers in Oncology 2021To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 125 (CA125), CA19-9, CA724, and CA242 in serum and ascites of pseudomyxoma peritonei...
PURPOSE
To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 125 (CA125), CA19-9, CA724, and CA242 in serum and ascites of pseudomyxoma peritonei (PMP) patients and evaluate the predictive value of these elevated biomarkers in pathological grade, completeness of cytoreduction (CC), and survival.
METHODS
From May 2009 to October 2019, a total of 512 patients diagnosed with PMP through pathology in Aerospace Center Hospital were enrolled. The serum and ascites tumor biomarkers were obtained. The diagnostic values between serum and ascites biomarkers in pathology and CC were compared by the receiver operating characteristic (ROC) curves. The correlation between pathology, cytoreduction, and biomarkers was calculated by univariate and multivariate logistic regression. The associations between different numbers of elevated biomarkers and survival status were examined using univariate and multivariate backward Cox proportional hazard regression models.
RESULTS
The results showed that the areas under the ROC curves (AUROC) in the diagnosis of CC were 0.798 (95% CI: 0.760-0.836) and 0.632 (95% CI: 0.588-0.676) in serum and ascites biomarkers, respectively. The elevated serum and ascites biomarkers were independent risk factors for both pathology and CC. The 1-year, 3-year, and 5-year survival rates were 89.07%, 73.22%, and 66.94%, respectively. Longer survival was observed in patients who had less than two elevated serum biomarkers compared with those with 2-3 and 4-5 elevated serum biomarkers (p < 0.001).
CONCLUSION
CEA, CA125, CA19-9, CA724, and CA242 in serum and ascites can be used to judge the severity and predict the resectability. Furthermore, different numbers of elevated biomarkers can help determine the prognosis of PMP.
PubMed: 34733775
DOI: 10.3389/fonc.2021.594763 -
BJS Open Apr 2019Pseudomyxoma peritonei (PMP) is a rare clinical condition characterized by mucinous ascites, typically related to appendiceal or ovarian tumours. Current standard... (Observational Study)
Observational Study
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare clinical condition characterized by mucinous ascites, typically related to appendiceal or ovarian tumours. Current standard treatment involves cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), but recurrences occur in 20-30 per cent of patients. The aim of this study was to define the timing and patterns of recurrence to provide a basis for modifying follow-up of these patients.
METHODS
This observational study examined a prospectively developed multicentre national database (RENAPE working group) to identify patients with recurrence after optimal CRS and HIPEC for PMP. Postoperative complications, long-term outcomes and potential prognostic factors were evaluated.
RESULTS
Of 1411 patients with proven PMP, 948 were identified who had undergone curative CRS and HIPEC. Among these patients, 229 first recurrences (24·2 per cent) were identified: 196 (20·7 per cent) occurred within the first 5 years (early recurrence) and 30 (3·2 per cent) occurred between 5 and 10 years. Three patients developed a first recurrence more than 10 years after the original treatment. The mean(s.d.) time to first recurrence was 2·36(2·21) years. Preoperative chemotherapy and high-grade pathology were significant factors for early recurrence. Overall survival for the entire group was 77·9 and 63·1 per cent at 5 and 10 years respectively. The principal site of recurrence was the peritoneum.
CONCLUSION
Recurrence of PMP was rare after 5 years and exceptional after 10 years.
Topics: Adult; Aged; Combined Modality Therapy; Cytoreduction Surgical Procedures; Disease-Free Survival; Female; Follow-Up Studies; Humans; Hyperthermia, Induced; Kaplan-Meier Estimate; Male; Middle Aged; Neoplasm Recurrence, Local; Peritoneal Neoplasms; Peritoneum; Prognosis; Prospective Studies; Pseudomyxoma Peritonei; Retrospective Studies; Time Factors
PubMed: 30957067
DOI: 10.1002/bjs5.97 -
Indian Journal of Surgical Oncology Jun 2016Pseudomyxoma peritonei (PMP) is the intra-peritoneal accumulation of mucus due to mucinous neoplasia, most often from a ruptured mucinous appendiceal neoplasm. A similar...
Pseudomyxoma peritonei (PMP) is the intra-peritoneal accumulation of mucus due to mucinous neoplasia, most often from a ruptured mucinous appendiceal neoplasm. A similar syndrome is caused by appendix cancer and other gastrointestinal malignancies. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) provides long-term survival in selected patients with these conditions. The management of the appendiceal neoplasm prior to development of peritoneal involvement is initially discussed. This is followed by an overview of the management of peritoneal disease caused by appendiceal neoplasms. The principles and basic techniques of CRS and intraperitoneal chemotherapy (both intraoperative and post operative) are then discussed. Survival outcomes from several large studies are summarised. Prognostic factors are also discussed. We report our basic outcome data for the 345 patients with PMP or appendix cancer treated at our institution. Finally, the promising upcoming treatment of mucolytic therapy is discussed. We conclude that appendiceal neoplasms, although rare can cause significant morbidity and mortality. With optimal management long-term survival is possible in the majority of patients. The key to treatment is complete cytoreduction and use of hyperthermic intraperitoneal chemotherapy.
PubMed: 27065707
DOI: 10.1007/s13193-015-0478-9 -
Current Opinion and Practice on Peritoneal Carcinomatosis Management: The North African Perspective.Frontiers in Surgery 2022The status of peritoneal surface malignancy (PSM) management in North Africa is undetermined. The aim of this study was to assess and compare current practice and...
BACKGROUND
The status of peritoneal surface malignancy (PSM) management in North Africa is undetermined. The aim of this study was to assess and compare current practice and knowledge regarding PSM and examine satisfaction with available treatment options and need for alternative therapies in North Africa.
METHODS
This is a qualitative study involving specialists participating in PSM management in North Africa. The survey analyzed demographic characteristics and current knowledge and opinions regarding PSM management in different institutions. We also looked at goals and priorities, satisfaction with treatment modalities and heated intraperitoneal chemotherapy (HIPEC) usefulness according to specialty, country, years of experience, and activity sector.
RESULTS
One-hundred and three participants responded to the survey (response rate of 57%), including oncologists and surgeons. 59.2% of respondents had more than 10 years experience and 45.6% treated 20-50 PSM cases annually. Participants satisfaction with PSM treatment modalities was mild for gastric cancer (3/10 [IQR 2-3]) and moderate for colorectal (5/10 [IQR 3-5]), ovarian (5/10 [IQR 3-5]), and pseudomyxoma peritonei (5/10 [IQR 3-5]) type of malignancies. Good quality of life and symptom relief were rated as main priorities for treatment and the need for new treatment modalities was rated 9/10 [IQR 8-9]. The perceived usefulness of systemic chemotherapy in first intention was described as high by 42.7 and 39.8% of respondents for PSM of colorectal and gastric origins, while HIPEC was described as highly useful for ovarian (49.5%) and PMP (73.8) malignancies.
CONCLUSIONS
The management of PSM in the North African region has distinct differences in knowledge, treatments availability and priorities. Disparities are also noted according to specialty, country, years of expertise, and activity sector. The creation of referral structures and PSM networks could be a step forward to standardized PSM management in the region.
PubMed: 35350143
DOI: 10.3389/fsurg.2022.798523