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International Journal of Surgery Case... Aug 2023Incidence of low grade appendiceal mucinous neoplasm is increasing. Preoperatively, it may present similarly to primary ovarian malignancy. This case report describes a...
INTRODUCTION
Incidence of low grade appendiceal mucinous neoplasm is increasing. Preoperatively, it may present similarly to primary ovarian malignancy. This case report describes a case of presumed ovarian malignancy with final pathologic diagnosis of low grade appendiceal mucinous neoplasm. We also propose several surgical strategies to approach this conundrum.
PRESENTATION OF CASE
A postmenopausal woman with abdominal pain was found to have a 30 cm abdominopelvic mass with elevated CA-125 and CEA presumably a primary ovarian malignancy. During surgical staging, intraoperative findings were notable for an appendiceal mass. Intraoperative surgical oncology consultation recommended appendectomy for diagnostic purposes. Following primary surgery and final pathologic diagnosis, she underwent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.
DISCUSSION
Low grade appendiceal mucinous neoplasm should be within the differential diagnosis of gynecologic surgeons when presented with a patient with large volume ascites and biopsy of acellular mucin. Intraoperatively, an abnormal appearing appendix with normal appearing gynecologic structures should trigger suspicion for appendiceal rather than ovarian origin. Preoperative symptoms, imaging studies, tumor markers, and frozen section pathology may not be able to differentiate between appendiceal and epithelial ovarian malignancies.
CONCLUSION
A recognition of mucinous material and abnormal appearing appendix should prompt the surgeon to consider performing an appendectomy to obtain primary pathologic diagnosis. A high level of suspicion could better optimize the patient for a joint case with the appropriate surgeons. Given the documented disguise of low grade appendiceal mucinous neoplasm as primary ovarian cancer and its increasing incidence, diagnosis and general understanding of treatment should be understood.
PubMed: 37524024
DOI: 10.1016/j.ijscr.2023.108563 -
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 -
Cancers Jun 2023A combination of bromelain and acetylcysteine, BromAc, is an efficient intraperitoneal mucolytic for thick mucus secreted in pseudomyxoma peritonei (PMP). Patients with...
A combination of bromelain and acetylcysteine, BromAc, is an efficient intraperitoneal mucolytic for thick mucus secreted in pseudomyxoma peritonei (PMP). Patients with PMP quite often undergo colon anastomosis. Hence, we investigated the effect of the intraperitoneal delivery of BromAc on colon-anastomosis healing in a rat model. Sixteen Wistar rats were divided into two groups (N = 8). The controls received intraperitoneal saline after anastomosis, whilst the other group received BromAc. They were monitored for body-weight and general health parameters. Half the rats in each group (N = 4) were culled at 4 or 13 days post-surgery for assessment. The healing process of the tissues was assessed by burst pressure and collagen density with histology to assess the integrity of the internal organs. The results indicated that there was a similar pattern of weight fluctuation during the experiment, although the rats treated with the BromAc showed slightly greater weight loss during the first 4 days. Although the burst pressure was similar in both groups, the BromAc group at day 13 showed a slightly higher burst pressure, which was complemented by a higher collagen density (albeit not statistically significant). The histology of the internal organs was comparable to those of the controls. This study indicates that the intraperitoneal delivery of BromAc in a rat model does not interfere with the healing process of colonic anastomosis.
PubMed: 37444430
DOI: 10.3390/cancers15133321 -
Cureus Jun 2023An appendiceal mucocele is a rare disease characterized by the dilation of the appendix lumen with mucus accumulation. Although this disease is often found incidentally...
An appendiceal mucocele is a rare disease characterized by the dilation of the appendix lumen with mucus accumulation. Although this disease is often found incidentally during appendectomy, it is crucial to differentiate it from acute appendicitis preoperatively to select adequate surgical management. We present a case of a 31-year-old male, medically free, with right-sided abdominal pain associated with nausea and vomiting. He was diagnosed with appendiceal mucocele and underwent laparoscopic appendectomy. The absence of a distinct clinical presentation and biochemical parameters necessitates a comprehensive and collaborative diagnostic approach for mucocele of the appendix. Achieving an accurate diagnosis prior to surgery is imperative to ensure the appropriate surgical technique is chosen, thereby minimizing the risk of serious intraoperative and postoperative complications such as pseudomyxoma peritonei.
PubMed: 37431343
DOI: 10.7759/cureus.40168 -
World Journal of Gastrointestinal... Jun 2023Pseudomyxoma peritonei (PMP) is a rare peritoneal malignant tumor syndrome. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is its standard...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare peritoneal malignant tumor syndrome. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is its standard treatment. However, there are few studies and insufficient evidence regarding systemic chemotherapy of advanced PMP. Regimens for colorectal cancer are often used clinically, but there is no uniform standard for late-stage treatment.
AIM
To determine if bevacizumab combined with cyclophosphamide and oxaliplatin (Bev+CTX+OXA) is effective for treatment of advanced PMP. The primary study endpoint was progression-free survival (PFS).
METHODS
Retrospective analysis was conducted on the clinical data of patients with advanced PMP who received Bev+CTX+OXA regimen (bevacizumab 7.5 mg/kg ivgtt d1, oxaliplatin 130 mg/m ivgtt d1 and cyclophosphamide 500 mg/m ivgtt d1, q3w) in our center from December 2015 to December 2020. Objective response rate (ORR), disease control rate (DCR) and incidence of adverse events were evaluated. PFS was followed up. Kaplan-Meier method was used to draw survival curve, and log-rank test was used for comparison between groups. Multivariate Cox proportional hazards regression model was used to analyze the independent influencing factors of PFS.
RESULTS
A total of 32 patients were enrolled. After 2 cycles, the ORR and DCR were 3.1% and 93.7%, respectively. The median follow-up time was 7.5 mo. During the follow-up period, 14 patients (43.8%) had disease progression, and the median PFS was 8.9 mo. Stratified analysis showed that the PFS of patients with a preoperative increase in CA125 (8.9 2.1, = 0.022) and a completeness of cytoreduction score of 2-3 (8.9 5.0, = 0.043) was significantly longer than that of the control group. Multivariate analysis showed that a preoperative increase in CA125 was an independent prognostic factor for PFS (HR = 0.245, 95%CI: 0.066-0.904, = 0.035).
CONCLUSION
Our retrospective assessment confirmed that the Bev+CTX+OXA regimen is effective in second- or posterior-line treatment of advanced PMP and that adverse reactions can be tolerated. A preoperative increase in CA125 is an independent prognostic factor of PFS.
PubMed: 37405093
DOI: 10.4240/wjgs.v15.i6.1149 -
Insights Into Imaging Jul 2023Peritoneal malignancies represent a diagnostic challenge for abdominal radiologists, oncologists, surgeons and pathologists in multidisciplinary teams, who must address... (Review)
Review
Peritoneal malignancies represent a diagnostic challenge for abdominal radiologists, oncologists, surgeons and pathologists in multidisciplinary teams, who must address their differential diagnosis, staging and treatment. In this article, we explain the pathophysiology of these processes and lay out the role of different imaging techniques in their evaluation. Then, we review the clinical and epidemiological aspects, the main radiological features and the therapeutic approaches for each primary and secondary peritoneal neoplasm, with surgical and pathological correlation. We further describe other rare peritoneal tumors of uncertain origin and a variety of entities that may mimic peritoneal malignancy. Finally, we summarize the key imaging findings of each peritoneal neoplasm to facilitate an accurate differential diagnosis that may impact patient management.Clinical relevance statementImaging plays an essential role in the evaluation of peritoneal malignancies, assessing their extension, detecting unfavorable sites of involvement and facilitating an accurate differential diagnosis, helping to choose the best therapeutic approach.
PubMed: 37395913
DOI: 10.1186/s13244-023-01417-6 -
Radiology Case Reports Aug 2023We report a case of ruptured low-grade appendiceal mucinous neoplasm with an impressive toy puffer ball-like appearance on magnetic resonance imaging. A 79-year-old...
We report a case of ruptured low-grade appendiceal mucinous neoplasm with an impressive toy puffer ball-like appearance on magnetic resonance imaging. A 79-year-old woman with lower abdominal pain underwent computed tomography scanning, revealing a 6-cm mass in the right lower abdomen. T2-weighted images showed a radial low-signal structure in the central area of the mass, which was presumed to be fibrotic. Pathology confirmed ruptured low-grade appendiceal mucinous neoplasm. The rupture point was at the tip of the appendix, coinciding with the center of radial fibrosis. The unique morphology of the puffer ball-like appearance in this case may be a characteristic of low-grade appendiceal mucinous neoplasms.
PubMed: 37388265
DOI: 10.1016/j.radcr.2023.05.006 -
Journal of Medical Case Reports Jun 2023Pseudomyxoma Peritonei (PMP) is a severe neoplastic clinical syndrome characterised by secretion of mucin from tumors often originating in the appendix. The standard...
BACKGROUND
Pseudomyxoma Peritonei (PMP) is a severe neoplastic clinical syndrome characterised by secretion of mucin from tumors often originating in the appendix. The standard treatment includes cytoreductive surgery (CRS) combined with heated intraperitoneal chemotherapy (HIPEC). A new perspective in PMP treatment aims at the mucins themselves as a therapeutic target.
CASE PRESENTATION
Here we report the first case of PMP with peritoneal dissemination of mucinous implants caused by low-grade appendiceal mucinous neoplasm (LAMN) in a 58-year-old white male exclusively treated by appendectomy and oral administration of bromelain and acetylcysteine in the context of a medical self-experimentation (by co-author T.R.). Observation so far covers a period of 48 months including regular magnetic resonance imaging (MRI) with stable findings.
CONCLUSIONS
Oral administration of bromelain and acetylcysteine can be used in the treatment of PMP caused by LAMN without relevant clinical side effects.
Topics: Male; Humans; Middle Aged; Pseudomyxoma Peritonei; Appendiceal Neoplasms; Administration, Oral; Peritoneal Neoplasms
PubMed: 37381056
DOI: 10.1186/s13256-023-04024-7 -
Journal of Clinical Medicine Jun 2023Pseudomyxoma peritonei (PMP) is a rare malignant growth characterized by the production of mucin and the potential for peritoneal relapse. This study aimed to...
Pseudomyxoma peritonei (PMP) is a rare malignant growth characterized by the production of mucin and the potential for peritoneal relapse. This study aimed to investigate the immunohistochemical and biological characteristics of mucin in patients with cellular and acellular PMP. We prospectively analyzed mucin specimens obtained from our patient cohort and described the composition and type of mucin present in each sample. A metagenomic analysis of the samples was performed to investigate the bacterial composition of the PMP microbiome. Secreted mucins 2 and 5AC and membrane-associated mucin-1 were the primary components of mucin in both cellular and acellular tumor specimens. The metagenomic study revealed a predominance of the phylum and the genus . Notably, , a species not previously reported in the human microbiome, was found to be the most abundant organism in the mucin of pseudomyxoma peritonei. Our findings suggest that the presence of MUC-2 and mucin colonization by Pseudomonas are characteristic features of both cellular and acellular disease. These results may have significant implications for the diagnosis and treatment of this rare entity.
PubMed: 37373701
DOI: 10.3390/jcm12124007 -
Annals of the Royal College of Surgeons... Mar 2024John Hunter is regarded as the father of scientific surgery. His principles involved reasoning, observation and experimentation. His most powerful saying was: "Why not...
John Hunter is regarded as the father of scientific surgery. His principles involved reasoning, observation and experimentation. His most powerful saying was: "Why not try the experiment?" This manuscript charts a career in abdominal surgery ranging from the treatment of appendicitis to the development of the largest appendiceal tumour centre in the world. The journey has led to the first report of a successful multivisceral and abdominal wall transplant for patients with recurrent non-resectable pseudomyxoma peritonei. We all stand on the shoulders of giants and surgery progresses by learning from the past while being prepared to experiment into the future.
Topics: Humans; Appendiceal Neoplasms; Appendicitis; Transplants; Abdominal Wall
PubMed: 37367485
DOI: 10.1308/rcsann.2023.0013