Low-Grade Pseudomyxoma Peritonei Behaving as a High-Grade Disease: A Case Series and Literature Review

Fetched: November 25th, 2023, 5:02am GMT by Petros Bangeas

Curr Oncol. 2023 Nov 18;30(11):9996-10006. doi: 10.3390/curroncol30110726.

ABSTRACT

Patients with low-grade appendiceal mucinous carcinomas (LAMNs) treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have a favorable prognosis. However, a subgroup of patients presents a clinically aggressive course with disease progression despite receiving treatment. The purpose of this study is to report the experience of clinically aggressive LAMN patients treated by the same team, and to present a review of the literature. The cases of four patients with clinically aggressive LAMNs were reviewed. Clinical and histopathological characteristics were re-examined. Recurrences and the time of recurrence, as well as the survival time, were recorded. These patients were four men with clinically aggressive LAMNs treated with CRS plus HIPEC. One of them underwent CC-0 surgery, two underwent CC-1 surgery, and one underwent CC-3 surgery. All patients received systemic chemotherapy after surgery. Recurrence was recorded in three of the patients within 4-23 months after the initial treatment. Two of the patients underwent secondary CRS. Three patients died of disease recurrence within 13-23 months, and one is alive with a disease relapse at 49 months after his initial surgery. LAMNs were identified in both the initial specimens and the specimens obtained during reoperation. The prognosis of LAMN patients treated with CRS plus HIPEC is favorable. A small number of patients present a clinically aggressive course that is unresponsive to any treatment. Molecular and genetic studies are required to identify this group of LAMN patients who have an unfavorable prognosis.

PMID:37999146 | PMC:PMC10670747 | DOI:10.3390/curroncol30110726
Long-term survival following cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in Waikato, Aotearoa New Zealand: a 12-year experience

Fetched: November 24th, 2023, 5:02am GMT by Rennie Xinrui Qin

ANZ J Surg. 2023 Nov 22. doi: 10.1111/ans.18777. Online ahead of print.

ABSTRACT

BACKGROUNDS: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have improved survival for selected cases of peritoneal surface malignancy. In 2008, a CRS/HIPEC service was first established in Aotearoa New Zealand (AoNZ) at Waikato and Braemar Hospitals in the Waikato region.

METHODS: This is a retrospective review of a prospectively maintained database of all patients undergoing CRS/HIPEC from 1 January 2008 to 1 November 2020 at Waikato and Braemar Hospitals. We analysed long-term survival and predictors of survival for each tumour type.

RESULTS: 240 procedures were performed for 221 patients, including 22 re-do procedures. Cases had a median peritoneal cancer index of 16. Complete cytoreduction (CC0-1) was achieved in 196 cases (81.7%). All complete cytoreduction cases received HIPEC. There were 152 pseudomyxoma peritonei (PMP), 39 colorectal cancers (CRC), 29 appendiceal cancers, eight ovarian cancers, six peritoneal mesotheliomas, and six other cancers. The 5-year overall survival (OS) for PMP with acellular mucin, low-grade mucinous carcinoma peritonei, and high-grade mucinous carcinoma peritonei with or without signet cells were 91.6%, 80.5%, and 72.2%, respectively. 2- and 5-year OS in CRC were 56.7% and 40.4%. The achievement of complete cytoreduction improved the 5-year OS to 87.9% across all PMP and 45.1% in colorectal cancer. Incomplete cytoreduction predicted worse survival in appendiceal PMP. In colorectal cancer, worse survival was predicted in those who had incomplete cytoreduction, liver metastasis, and presentation with obstruction and perforation.

CONCLUSION: Favourable long-term outcomes following CRS/HIPEC for peritoneal surface malignancy have been achieved in AoNZ through the Waikato peritonectomy service.

PMID:37994292 | DOI:10.1111/ans.18777
A case of pseudomyxoma peritonei arising from a perforated intraductal papillary mucinous neoplasm that underwent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy

Fetched: November 20th, 2023, 5:02am GMT by Yuhi Yoshizaki

Clin J Gastroenterol. 2023 Nov 18. doi: 10.1007/s12328-023-01890-y. Online ahead of print.

ABSTRACT

Pseudomyxoma peritonei (PMP) of pancreatic origin arising from an intraductal papillary mucinous neoplasm (IPMN) is rare. Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has been established as the optimal treatment for PMP. However, the benefits and safety of CRS with HIPEC for treating PMP of pancreatic origin remain unclear. Herein, we describe a case of PMP of pancreatic origin that was treated with CRS and HIPEC without postoperative complications. A 75-year-old woman was referred to our department. Computed tomography (CT) revealed a multilocular cystic tumor in the pancreatic tail, notable mucinous ascites in the abdominal cavity, and scalloping of the liver and spleen. CT did not reveal the appendix, and the ovaries were normal in size. The patient was diagnosed with PMP of pancreatic origin, and CRS and HIPEC were performed. Intraoperatively, the pancreatic tumor was perforated, and there was a large amount of mucinous ascites. We performed distal pancreatectomy in addition to CRS and HIPEC, with no intraoperative complications. The postoperative course was uneventful, and the patient survived after 6 months without recurrence. CRS with HIPEC may be a feasible treatment option for PMP of pancreatic origin.

PMID:37980306 | DOI:10.1007/s12328-023-01890-y
Analysis of the characteristics and outcomes of patients with pseudomyxoma peritonei of appendiceal origin treated with curative-intent surgery

Fetched: November 19th, 2023, 5:02am GMT by Yasuyuki Kamada

Surg Oncol. 2023 Nov 5;51:102012. doi: 10.1016/j.suronc.2023.102012. Online ahead of print.

ABSTRACT

BACKGROUND: There are no previous studies on pseudomyxoma peritonei regarding the details of surgical procedures included in cytoreductive surgery and quantitative evaluation for peritoneal metastases by region in the abdominal cavity. This study aimed to describe the characteristics and procedural details involved in cytoreductive surgery, and survival outcomes of patients with pseudomyxoma peritonei originating from appendiceal mucinous neoplasm, and identify differences in the difficulty of cytoreductive surgery based on tumor location.

METHODS: Patient characteristics and survival outcomes were studied through a retrospective review. The complete cytoreduction rate (i), the 5-year survival rate for patients with complete cytoreduction (ii), and an index as a complement (i × ii × 100) were described for patients who had tumors larger than 50 mm in one of the 13 regions of the abdominal cavity.

RESULTS: A total of 989 patients were treated with curative-intent cytoreductive surgery. The median peritoneal cancer index was 18 (interquartile range, 6-29), with complete cytoreduction achieved in 702 patients (71%); the major complication rate was 17%. The median overall survival was 92.9 months, compared to 53.8 months for patients who underwent total gastrectomy and 30.4 months for those who underwent total colectomy. In the 13 abdominal regions, the index scores indicating cytoreduction difficulty were categorized into three risk groups: upper and mid-abdominal (>20), lateral abdominal (10-20), and small bowel (<10).

CONCLUSIONS: Cytoreductive surgery offered favorable survival outcomes, even in cases involving total gastrectomy. The difficulty of achieving complete cytoreduction varied across abdominal regions and was classified into three levels.

PMID:37972508 | DOI:10.1016/j.suronc.2023.102012
Rare urachal mucinous cystic tumor of low malignant potential with peritoneal pseudomyxoma: A case report

Fetched: November 10th, 2023, 5:02am GMT by Lijiang Chen

Exp Ther Med. 2023 Oct 17;26(6):555. doi: 10.3892/etm.2023.12254. eCollection 2023 Dec.

ABSTRACT

Mucinous cystic tumors of low malignant potential (MCTLMP) are rare urachal neoplasms. The morphological characteristics and clinical prognosis of MCTLMP is similar to that of mucinous cystic tumors occurring in the ovary and appendix. After complete resection, almost no cases of recurrence or metastasis have been reported. Because MCTLMP is rare, it may be missed in the clinic. MCTLMP can lead to the formation of pseudomyxoma peritonei (PMP), which manifests as the widespread production of mucus in the abdominal cavity and makes the disease complex or difficult to diagnose. At present, only 3 cases of MCTLMP with PMP have been reported in the literature. In the present study a fourth case of urachal MCTLMP in a 74-year-old male that resulted in widespread PMP is presented. Initially, a multilocular cystic lesion was revealed in the urachal duct area at the anterior upper margin of the bladder after a patient, experiencing lower abdominal pain, was imaged. As revealed using light microscopy, the cyst was lined with a mucous columnar epithelium, and part of the epithelium indicated pseudolamellar hyperplasia and papillary structures. The cells indicated mild atypia and low mitotic activity. There was no stromal infiltration of tumor cells, and a large amount of mucous exudate was observed. As preoperative computed tomography examination suggested the presence of a large amount of ascites and there were increased levels of blood tumor markers, carcinoembryonic antigen and carbohydrate antigen 125, clinicians considered that the diagnosis maybe a malignant tumor of the urachal gland with peripheral dissemination. However, the diagnosis of MCTLMP with PMP was confirmed by histopathological examination. The mass was completely removed, along with part of the peritoneum and bladder wall as these were within the tumor margin. The appendix appeared normal during surgery. A one off dose of intraperitoneal infusion chemotherapy with 1,000 mg 5-fluorouracil was performed after surgery. No recurrence was observed during the 8-month follow-up period.

PMID:37941591 | PMC:PMC10628641 | DOI:10.3892/etm.2023.12254
ASO Author Reflections: Contemporary Management of Low-Grade Appendiceal Mucinous Neoplasms

Fetched: November 9th, 2023, 5:02am GMT by Michael G White

Ann Surg Oncol. 2023 Dec;30(13):8168-8169. doi: 10.1245/s10434-023-13523-2. Epub 2023 May 25.

NO ABSTRACT

PMID:37231292 | DOI:10.1245/s10434-023-13523-2
Long-term survival in patients with PMP: a single-institutional retrospective study from China

Fetched: October 29th, 2023, 5:02am GMT by Rui Yang

World J Surg Oncol. 2023 Oct 28;21(1):347. doi: 10.1186/s12957-023-03232-1.

ABSTRACT

BACKGROUND: As the standard treatment for pseudomyxoma peritonei (PMP), cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) can significantly prolong the survival of PMP patients, and some patients can even achieve long-term survival (LTS) or clinical cure. The purpose of this study was to analyze the clinicopathological and treatment features of PMP patients with LTS and to explore the survival benefit factors of PMP patients.

METHODS: The clinicopathological and prognostic data of PMP patients who received CRS + HIPEC at our center from December 2004 to May 2023 were retrospectively analyzed. PMP patients were divided into LTS group (≥ 10 years) and short-term survival (STS) group (< 5 years) according to the length of natural history. Univariate and multivariate analyses were performed to explore the beneficial factors of PMP patients with LTS.

RESULTS: A total of 609 patients with PMP received CRS + HIPEC treatment at our center. Two-hundred one patients with PMP were included in the study after screening, including 39 patients (19.4%) in the LTS group and 162 patients (80.6%) in the STS group. In STS group and LTS group, median overall survival based on natural history was 29.2 (2.4-59.9) vs. 138.9 (120.3-416.7) months. Univariate analysis revealed 8 factors (P < 0.05) with statistically significant differences between the two groups: gender, chemotherapy history, previous surgical score, Karnofsky Performance Status score, pathological diagnosis, lymphatic metastasis, peritoneal cancer index, and completeness of cytoreduction (CC). Multivariate analysis identified only two factors independently associated with LTS of PMP patients: CC and pathological diagnosis.

CONCLUSION: Complete CRS and pathological features are two key factors affecting LTS in PMP patients.

PMID:37891655 | PMC:PMC10612327 | DOI:10.1186/s12957-023-03232-1
High-grade appendiceal mucinous neoplasm presenting as renal colic; a case report and review of literature

Fetched: October 20th, 2023, 5:02am GMT by Baraa Amir

J Surg Case Rep. 2023 Oct 17;2023(10):rjad567. doi: 10.1093/jscr/rjad567. eCollection 2023 Oct.

ABSTRACT

High-grade appendiceal mucinous neoplasms (HAMN) are rare mucinous appendiceal tumors that are mostly incidentally discovered and histologically show marked cytoplasmic atypia. We report a 62-year-old female patient who was diagnosed with acute right flank pain mimicking renal colic. Abdominal and pelvis CT scans showed no calculi or hydronephrosis. Incidentally, a markedly distended retrocecal appendix was identified and an appendectomy was performed. Histopathology revealed an HAMN of size 5.8 cm. HAMN are one of the rare and somewhat recently described epithelial appendiceal tumors. The latest 2019 classification is based on histologic appearance and on the consensus for classification and pathologic reporting of Pseudomyxoma peritonei and associated appendiceal neoplasia (Peritoneal Surface Pathology Group International, modified Delphi process). Histologically, appendiceal mucinous lesions are classified as nonneoplastic/mucocele and neoplastic lesions with separate subcategorization. Despite its rarity, it is crucial for clinicians to be aware of HAMN to distinguish it from other clinical differentials.

PMID:37854517 | PMC:PMC10581701 | DOI:10.1093/jscr/rjad567
Outcomes of laparoscopic single-incision caecal pole resection for low-grade appendiceal mucinous neoplasm

Fetched: October 17th, 2023, 5:02am GMT by Han Deok Kwak

J Minim Access Surg. 2023 Sep 14. doi: 10.4103/jmas.jmas_113_23. Online ahead of print.

ABSTRACT

INTRODUCTION: Low-grade appendiceal mucinous neoplasm (LAMN) is a rare disease, and its clinical course varies from an incidental finding without symptoms to pseudomyxoma peritonei. Furthermore, there are few established treatment guidelines. This study was designed to confirm the outcomes in patients diagnosed with LAMN who underwent single-incision laparoscopic caecal pole resection.

PATIENTS AND METHODS: This study was conducted on pathologically confirmed LAMNs from patients who underwent surgery at a single centre, a tertiary institution, from July 2016 to August 2022. Patients diagnosed with LAMN as a result of biopsy were included. All surgeries were performed with single-incision laparoscopic caecal pole resection by a single surgeon.

RESULTS: A total of 70 patients were included. The median age was 65.5 years and 36 (51.4%) patients were female. The baseline carcinoembryonic antigen (CEA) was 8.08 ng/mL (0.76-148.11). The mean maximum diameter was 29.4 mm (7-70) and calcification was seen in 22 cases on pre-operative computed tomography (CT). As a result of histological examination, all patients were marginally negative. The larger the tumour size, the higher the CEA was (P = 0.011), and it was often accompanied by calcification (P = 0.021). In addition, tumour size and CEA showed a positive partial correlation with each other (r = 0.318, P = 0.018). The overall median follow-up period was 22.7 months (2-60). One case had suspected of recurrence on CT scan performed at 33 months following the surgery, but there were no related symptoms.

CONCLUSION: Single-port laparoscopic caecal pole resection without margin involvement in LAMN was safe and feasible, and showed a favourable long-term outcome.

PMID:37843158 | DOI:10.4103/jmas.jmas_113_23
Recurrence after cytoreductive surgery and HIPEC for pseudomyxoma peritonei: A single-center retrospective cohort study

Fetched: October 14th, 2023, 5:02am GMT by Antti Yrjönen

Scand J Surg. 2023 Oct 12:14574969231200653. doi: 10.1177/14574969231200653. Online ahead of print.

ABSTRACT

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.

PMID:37828760 | DOI:10.1177/14574969231200653
Low-Grade Appendiceal Mucinous Neoplasm: What Is the Best Treatment?

Fetched: October 10th, 2023, 5:03am GMT by Murat Guner

Cureus. 2023 Oct 6;15(10):e46591. doi: 10.7759/cureus.46591. eCollection 2023 Oct.

ABSTRACT

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.

PMID:37808597 | PMC:PMC10560074 | DOI:10.7759/cureus.46591
Effective Use of Bromelain and Acetylcysteine (BromAc®) for Treatment of Perigastric Pseudomyxoma Peritonei: A Case Report

Fetched: September 30th, 2023, 5:02am GMT by Elizabeth Skalkos

Anticancer Res. 2023 Oct;43(10):4735-4738. doi: 10.21873/anticanres.16670.

ABSTRACT

BACKGROUND/AIM: Pseudomyxoma peritonei (PMP) is a rare clinical condition of progressive peritoneal mucin accumulation. PMP has a reasonable survivability but with a notable risk of tumour recurrence. Standard treatment, including for tumour relapse, aims for a cure with complete cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. In the case of tumour recurrence, surgery becomes progressively complex, and some patients are not suitable for surgery either due to patient preference or morbidity and mortality risk. BromAc^® is an emerging, novel mucolytic combination therapy composed of bromelain and acetylcysteine which can be administered intratumorally via radiologically guided drains. It represents a minimally invasive treatment for patients who have symptomatic tumour deposits but are not surgical candidates.

CASE REPORT: We report the case of a 64-year-old male with a background of appendiceal PMP who presented with a gastric outlet obstruction from a perigastric tumour deposit. This was managed with BromAc^® administration, following which the patient's symptoms resolved. This corresponded with an 80% reduction in the tumour volume radiologically.

CONCLUSION: BromAc^® is an emerging minimally invasive treatment for PMP tumour deposits that may be considered as adjunctive or alternative treatment in patients who are not surgical candidates to reduce tumour burden and improve symptomatology and quality of life.

PMID:37772592 | DOI:10.21873/anticanres.16670
Low-grade appendiceal mucinous neoplasm encountered during risk-reducing salpingo-oophorectomy: A case of laparoscopic surgery

Fetched: September 30th, 2023, 5:02am GMT by Katsuyuki Konnai

J Obstet Gynaecol Res. 2023 Dec;49(12):2975-2978. doi: 10.1111/jog.15802. Epub 2023 Sep 28.

ABSTRACT

Low-grade appendiceal mucinous neoplasm (LAMN) is a rare epithelial malignancy of the appendix. If it perforates the abdominal cavity, it can cause a serious clinical syndrome called pseudomyxoma peritonei. In the present case, we laparoscopically removed a LAMN encountered during risk-reducing salpingo-oophorectomy (RRSO). The patient was a 53-year-old woman who was diagnosed with hereditary breast and ovarian cancer syndrome. RRSO was planned, and magnetic resonance imaging revealed a large cystic tumor in the right lower abdomen. We expected an ovarian cyst; however, it was a primary tumor of the appendix. Partial cecal resection was performed laparoscopically by a surgical oncologist. The pathological diagnosis was LAMN. Gynecologists may encounter this disease incidentally. Mucinous appendiceal neoplasm (MAN) may be encountered during RRSO. If a right lower abdominal mass is found near a normal ovary preoperatively, gynecologists should consider MAN as well as paraovarian cyst.

PMID:37771102 | DOI:10.1111/jog.15802
Whole-Exome Sequencing Identifies Mutation Profile and Mutation Signature-Based Clustering Associated with Prognosis in Appendiceal Pseudomyxoma Peritonei

Fetched: September 29th, 2023, 5:02am GMT by Yu-Lin Lin

Mol Cancer Res. 2023 Sep 28. doi: 10.1158/1541-7786.MCR-22-0801. Online ahead of print.

ABSTRACT

Pseudomyxoma peritonei (PMP) is a rare malignant clinical syndrome with little known about the global mutation profile. In this study, whole-exome sequencing (WES) was performed in 49 appendiceal PMP to investigate mutation profiles and mutation signatures. A total of 4020 somatic mutations were detected, with a median mutation number of 56 (1 - 402). Tumor mutation burden (TMB) was generally low (median 1.55 mutations/Mb, 0.12 - 11.26 mutations/Mb). Mutations were mainly enriched in the function of cancer-related axonogenesis, extracellular matrix-related processes, calcium signaling pathway, and cAMP signaling pathway. Mutations in FCGBP, RBFOX1, SPEG, RTK-RAS, PI3K-AKT, and focal adhesion pathways were associated with high-grade mucinous carcinoma peritonei. These findings revealed distinct mutation profile in appendiceal PMP. Ten mutation signatures were identified, dividing patients into mutation signature cluster (MSC) 1 (N = 28, 57.1%) and MSC 2 (N = 21, 42.9%) groups. MSC (P = 0.007) was one of the four independent factors associated with 3-year survival. TMB (P = 0.003) and microsatellite instability (P = 0.002) were independent factors associated with MSC 2 grouping. Taken together, our findings provided a broader view in the understanding of molecular pathological mechanism in appendiceal PMP and may be critical to developing an individualized approach to appendiceal PMP treatment. Implications: This work describes exhaustive mutation profile of PMP based on WES data and derives ten mutation signatures, which divides patients into two clusters and serve as an independent prognostic factor associated with 3-year survival.

PMID:37768171 | DOI:10.1158/1541-7786.MCR-22-0801
Surgery of peritoneal surface malignancies - surgical cytoreduction and hyperthermic intraperitoneal chemotherapy

Fetched: September 29th, 2023, 5:02am GMT by Gergely Huszty

Magy Onkol. 2023 Sep 28;67(3):247-258. Epub 2023 Sep 12.

ABSTRACT

Peritoneal carcinosis has historically been considered as inoperable, although the technique of its resesection together with high dose intraperitoneal chemotherapy potentiated by heat has been described decades ago. It has not became a widely practiced routine except in specialized centers - the complex technique, weakly standardized but resource demanding chemotherapy, lacking financial background and the many times questionable clinical benefit at a cost of high surgical load might have been the key factors. Refined technology, developing chemotherapy protocols together with growing clinical evidence are now more sharply delineating the range of indications where the procedure might be beneficial, increases survival, or is the only curative therapy. These include tumors of the appendix and pseudomyxoma peritonei, mesothelioma, and selected cases of ovarian, colorectal and gastric cancer. In addition to technical description of the intervention, we summarize the currently valid indications and describe our institutional protocol for the treatment of appendiceal malignancies.

PMID:37768120
Comparison of coagulation parameters associated with fibrinogen concentrate and cryoprecipitate for treatment of bleeding in patients undergoing cytoreductive surgery for pseudomyxoma peritonei: Subanalysis from a randomized, controlled phase 2 study

Fetched: September 29th, 2023, 5:02am GMT by Ashok Roy

Health Sci Rep. 2023 Sep 26;6(9):e1558. doi: 10.1002/hsr2.1558. eCollection 2023 Sep.

ABSTRACT

BACKGROUND AND AIMS: The FORMA-05 study compared the efficacy and safety of human fibrinogen concentrate (HFC) versus cryoprecipitate for hemostasis in bleeding patients undergoing cytoreductive surgery for pseudomyxoma peritonei (PMP). This subanalysis explores coagulation parameters in the FORMA-05 patients, with a focus on the seven patients who developed thromboembolic events (TEEs).

METHODS: FORMA-05 was a prospective, randomized, controlled phase 2 study in which patients with predicted blood loss ≥2 L received HFC (4 g) or cryoprecipitate (two pools of five units), repeated as needed. Plasma fibrinogen, platelet count, factor (F) XIII, FVIII, von Willebrand Factor (VWF) antigen and ristocetin cofactor activity levels, EXTEM A20, FIBTEM A20, and endogenous thrombin potential (ETP) were measured perioperatively.

RESULTS: Fibrinogen, platelet count, EXTEM and FIBTEM A20, FXIII, FVIII, VWF levels, and ETP were maintained throughout surgery in both the HFC group (N = 21) and the cryoprecipitate group (N = 23). Seven TEEs were observed in the cryoprecipitate group. The two patients developing deep vein thromboses (DVT) appeared to have a procoagulant status preoperatively, with distinctively higher fibrinogen level, FIBTEM A20, and platelet levels, all of which persisted perioperatively. The five patients developing pulmonary embolism (PE) had slightly higher VWF levels preoperatively, with a disproportionate increase intraoperatively (postcryoprecipitate administration) and postoperatively.

CONCLUSIONS: Patients treated with HFC versus cryoprecipitate showed broad overlaps in coagulation parameters. Patients with PE experienced a disproportionate VWF rise following cryoprecipitate administration, whereas patients developing DVT displayed a procoagulant status before and following surgery. Preoperative testing may allow these patients to be identified.

PMID:37766781 | PMC:PMC10521228 | DOI:10.1002/hsr2.1558
Correction: Noguchi et al. Establishment and Characterization of NCC-PMP1-C1: A Novel Patient-Derived Cell Line of Metastatic Pseudomyxoma Peritonei. J. Pers. Med. 2022, 12, 258

Fetched: September 29th, 2023, 5:02am GMT by Rei Noguchi

J Pers Med. 2023 Sep 15;13(9):1383. doi: 10.3390/jpm13091383.

ABSTRACT

There was an error in the original publication [...].

PMID:37763192 | PMC:PMC10533008 | DOI:10.3390/jpm13091383
Etiological analysis of infection after CRS + HIPEC in patients with PMP

Fetched: September 27th, 2023, 5:02am GMT by Rui Yang

BMC Cancer. 2023 Sep 26;23(1):903. doi: 10.1186/s12885-023-11404-1.

ABSTRACT

BACKGROUND: Cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) is the standard treatment for pseudomyxoma peritonei (PMP). It can significantly prolong the survival of patients, but at the same time may increase the risk of postoperative infection.

METHOD: Patients with PMP who underwent CRS + HIPEC at our center were retrospectively analyzed. According to PMP patients, basic clinical data and relevant information of postoperative infection, we analyzed the common sites of postoperative infection, results of microbial culture and the antibiotics sensitivity. Univariate and multivariate analysis were performed to explore infection-related risk factors.

RESULT: Among the 482 patients with PMP, 82 (17.0%) patients were infected after CRS + HIPEC. The most common postoperative infection was central venous catheter (CVC) infection (8.1%), followed by abdominal-pelvic infection (5.2%). There were 29 kinds of microbes isolated from the culture (the most common was Staphylococcus epidermidis), including 13 kinds of Gram-positive bacteria, 12 kinds of Gram-negative bacteria, and 4 kinds of funguses. All the antibiotics sensitivity results showed that the most sensitive antibiotics were vancomycin to Gram-positive bacteria (98.4%), levofloxacin to Gram-negative bacteria (68.5%), and fluconazole to fungus (83.3%). Univariate and multivariate analysis revealed the infection independent risk factors as follow: intraoperative blood loss ≥ 350 mL (P = 0.019), ascites volume ≥ 300 mL (P = 0.008).

CONCLUSION: PMP patients may have increased infection risk after CRS + HIPEC, especially CVC, abdominal-pelvic and pulmonary infections. The microbial spectrum and antibiotics sensitivity results could help clinicians to take prompt prophylactic and therapeutic approaches against postoperative infection for PMP patients.

PMID:37752468 | PMC:PMC10521434 | DOI:10.1186/s12885-023-11404-1
Determinants of Outcome with Reoperative Surgery for Pseudomyxoma Peritonei in 186 Patients

Fetched: September 26th, 2023, 5:03am GMT by Paul H Sugarbaker

Ann Surg Open. 2023 Sep 15;4(3):e335. doi: 10.1097/AS9.0000000000000335. eCollection 2023 Sep.

ABSTRACT

OBJECTIVE: To describe the long-term survival and clinical- and treatment-related variables that determine the outcome of repeat cytoreductive surgery (CRS) for mucinous appendiceal neoplasms with peritoneal dissemination.

SUMMARY BACKGROUND: After patients with peritoneal dissemination of an appendiceal mucinous neoplasm have a CRS, disease progression may require secondary cytoreductive surgery (SCRS) and other treatments performed in a timely manner to prolong survival and help preserve an optimal quality of life.

METHODS: The clinical- and treatment-related variables associated with the index CRS and the SCRS were statistically assessed for their impact on survival.

RESULTS: One hundred eighty-six of 687 complete CRS patients (27.1%) had SCRS. The median follow-up was 10 years and the median survival was 12 years. There were 95 males (51%) and the median age was 45.0 years. Survival benefit was associated with the index CRS by use of early postoperative intraperitoneal chemotherapy (EPIC) with 5-fluorouracil [Hazard ratio (HR), 0.4; P = 0.0004]. Also, survival of low-grade mucinous appendiceal neoplasms versus mucinous appendiceal adenocarcinoma (HR, 2.8; P < 0.0001) was improved. The interval between index CRS and SCRS was significant at ≤12 months versus 12-36 months versus >36 months (P < 0.0001). Change in peritoneal cancer index and disease distribution as focal or diffuse was significant by univariant and multivariant analyses.

CONCLUSIONS: If the CRS was complete, the use of EPIC 5-fluorouracil, the interval between the index CRS and the SCRS, the histologic grade of the mucinous neoplasm, and the extent of recurrent disease were prognostic variables that should be used to help select patients for SCRS.

PMID:37746617 | PMC:PMC10513308 | DOI:10.1097/AS9.0000000000000335
Health related quality of life is excellent and sustained at two decades after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in survivors of pseudomyxoma peritonei of appendiceal origin

Fetched: September 8th, 2023, 5:02am GMT by Victor Kung

Eur J Surg Oncol. 2023 Nov;49(11):107045. doi: 10.1016/j.ejso.2023.107045. Epub 2023 Aug 29.

ABSTRACT

INTRODUCTION: Optimal management of pseudomyxoma peritonei (PMP) is by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), which can achieve 20-year disease-free, and overall survival. There is limited information on the health-related quality of life (HRQOL) of PMP survivors beyond five years. We report longitudinal HRQOL in patients with PMP of appendiceal origin up to 17-years after their CRS and HIPEC in 2003-2004.

METHODS: Patients had HRQOL assessed with EORTC QLQ-C30 questionnaires pre-operatively, and at 1-, 10- and 17-years post-operatively. Comparisons in global health-related QOL (global-HRQOL) measures were made with (1) an age- and sex-matched normal European population, (2) between patients who underwent complete cytoreduction (CRS CC0/1) versus maximal tumor debulking (MTD), and (3) between those with and without peritoneal recurrence.

RESULTS: Forty-six patients underwent CRS & HIPEC for appendiceal PMP. One patient withdrew from the study. Of the 45 patients, 23 patients were alive at ten and 15 patients at 17-years post-operatively. 21/23 (91%) and 14/15 patients (93%) completed questionnaires respectively. Pre-operatively, patients had significantly lower global-HRQOL compared with the reference population. Over follow-up, patients experienced improvements in their global-HRQOL. By post-operative year-10 and -17, there was no difference between the global-HRQOL of patients and reference population. As expected, patients with CC0/1 and without peritoneal tumor recurrence had better global-HRQOL at ten- and 17-years post-operatively compared with those with MTD or recurrence.

CONCLUSIONS: Optimal CRS and HIPEC is an effective treatment for appendiceal PMP that can achieve long-term survival. HRQOL is excellent and maintained, in those who have CC0/1 without recurrence.

PMID:37677915 | DOI:10.1016/j.ejso.2023.107045
A Case of Low-Grade Appendiceal Mucinous Neoplasm That Led to Surgery After 12 Years of No Treatment

Fetched: September 8th, 2023, 5:02am GMT by Akihiro Makino

Cureus. 2023 Aug 6;15(8):e43024. doi: 10.7759/cureus.43024. eCollection 2023 Aug.

ABSTRACT

Low-grade appendiceal mucinous neoplasm (LAMN) is a relatively rare, non-invasive appendiceal tumor. We experienced a case of LAMN that led to surgery after 12 years of no treatment. Until now, LAMN has been reported to progress more slowly than other tumors, but there have been no reports of long-term follow-up of appendiceal tumors without treatment. Although the tumor had grown over the course of 12 years, there was no mixing or migration of other histological types, and it did not lead to pseudomyxoma peritonei. As this course is considered to be relatively rare, we report it along with a literature review.

PMID:37674965 | PMC:PMC10478797 | DOI:10.7759/cureus.43024
Mytomicin-C, Metronomic Capecitabine, and Bevacizumab in Patients With Unresectable or Relapsed Pseudomyxoma Peritonei of Appendiceal Origin

Fetched: September 3rd, 2023, 5:02am GMT by Filippo Ghelardi

Clin Colorectal Cancer. 2023 Dec;22(4):450-456.e1. doi: 10.1016/j.clcc.2023.08.005. Epub 2023 Aug 11.

ABSTRACT

INTRODUCTION: Pseudomyxoma peritonei (PMP) is a rare, slow growing tumor, traditionally considered chemoresistant. The only curative approach is cytoreductive surgery (CRS) followed by hyperthermic intraperitoneal chemotherapy (HIPEC). At disease relapse, or in patients with inoperable disease at diagnosis, no standard treatment has been defined, though nonrandomized series showed promising results with fluoropyrimidine-based regimens.

PATIENTS AND METHODS: We conducted a prospective study in patients with relapsed or unresectable PMP and confirmed disease progression at baseline. Patients received MMC (7 mg/m² every 6 weeks, up to a maximum of 4 cycles) plus metronomic capecitabine (625 mg/sqm/day b.i.d.) and bevacizumab (7.5 mg/kg every 3 weeks) until disease progression, unacceptable toxicity, or consent withdrawal. Primary endpoint was progression-free survival (PFS); secondary endpoints were overall survival (OS), overall response rate according to RECIST v1.1 criteria, serum markers response and safety.

RESULTS: Fifteen patients were included. At a median follow-up of 26.1 months (IQR, 17.7-49.6), median PFS was 17.9 months (95% CI, 11.0-NE), with 1-year PFS and OS rates of 73% and 87%. Safety profile was manageable, with only 13% G3/G4 treatment-related adverse events.

CONCLUSION: Metronomic capecitabine, bevacizumab, and MMC are an active regimen in advanced and progressive PMP and favorably compares with historical series.

PMID:37657955 | DOI:10.1016/j.clcc.2023.08.005
A case of low-grade intestinal-type mucinous neoplasm of the fallopian tube with KRAS exon 2 mutation

Fetched: August 17th, 2023, 5:02am GMT by Kiyotaka Nagahama

Gynecol Oncol Rep. 2023 Aug 3;49:101252. doi: 10.1016/j.gore.2023.101252. eCollection 2023 Oct.

ABSTRACT

Several types of mucinous lesions of the fallopian tube have been reported, including metaplastic and neoplastic lesions, most of which exhibit gastric phenotypes. Here, we report a unique case of a mucinous tumor arising in the right fallopian tube of a 36-year-old female who presented with refractory abdominal pain for approximately one year. Abdominal CT and MRI found a cystic lesion leading to the diagnosis of hematosalpinx, thus right salpingo-oophorectomy and appendectomy were performed. Macroscopic findings included cystic dilatation of the distal portion of the right fallopian tube, filled with gelatinous mucin. Histologically, mucinous columnar cells proliferated in papillary configurations in the cystic region without invasion, resembling low-grade appendiceal mucinous neoplasms. Immunohistochemical analysis revealed that the neoplastic cells expressed CDX-2 and SATB2, but not WT-1, PAX8, ER, PgR, or claudin 18. Sanger sequencing of the mucinous lesion identified a KRAS exon 2 mutation (p.G12A), confirming similar pathologic and genetic characteristics to ovarian mucinous borderline tumors. This rare low grade intestinal-type mucinous tumor indicates the fallopian tube epithelium can give rise to tumors resembling low-grade appendiceal mucinous neoplasms and cause pseudomyxoma peritonei without appendiceal lesions.

PMID:37583589 | PMC:PMC10424131 | DOI:10.1016/j.gore.2023.101252
Surgical treatment of pseudomyxoma peritonei-Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy

Fetched: August 15th, 2023, 5:02am GMT by Roger Wahba

Chirurgie (Heidelb). 2023 Oct;94(10):840-844. doi: 10.1007/s00104-023-01937-3. Epub 2023 Aug 14.

ABSTRACT

BACKGROUND: Pseudomyxoma peritonei syndrome (PMP) is an orphan disease. Surgery is the fundament of treatment.

METHOD: Short review summarizing the state of the art treatment.

RESULTS: Cytoreductive surgery (CRS) in combination with hyperthermic intraperitoneal chemotherapy (HIPEC) form the foundations of treatment for PMP. The peritoneal cancer index should be preoperatively determined based on imaging and/or laparoscopy, intraoperatively validated and both should be documented. An extraperitoneal surgical preparation technique leads to effective en bloc resection of the peritoneum and the affected abdominal area. The HIPEC technique should be performed with mitomycin C for 60-90 min. Complete CRS (CC = 0, CC = 1) and the histological subtype are relevant for the prognosis. Structured educational programs and mentoring can optimize the learning curve. The aftercare should be performed at the surgical center. After follow-up imaging at 3 months after CRS, in the first 2 years a control should be carried out every 6 months. Thereafter, the intervals can be extended to 1 year.

CONCLUSION: Standardized surgical treatment and HIPEC, optimized specific surgical training and structured follow-up at the center lead to an excellent long-term prognosis for patients with PMP.

PMID:37578542 | DOI:10.1007/s00104-023-01937-3
A rare case of pseudomyxoma peritonei with Morgagni hernia

Fetched: August 15th, 2023, 5:02am GMT by Toru Imagami

Int Cancer Conf J. 2023 Jul 5;12(4):263-267. doi: 10.1007/s13691-023-00614-w. eCollection 2023 Oct.

ABSTRACT

Both pseudomyxoma peritonei and Morgagni hernias in adults are rare clinical conditions. A 70-year-old woman who was diagnosed with pseudomyxoma peritonei with Morgagni hernia underwent cytoreductive surgery and primary repair. Pseudomyxoma peritonei causes increased intra-abdominal pressure that may lead to acquired congenital diaphragmatic hernia when there is a local fragility in the diaphragmatic musculature. Parietal peritonectomy of the right diaphragmatic peritoneum can safely remove the hernia sac. The high rate of infections associated with cytoreductive surgery causes hesitation for concurrent mesh repair for Morgagni hernia. This is the first report of pseudomyxoma peritonei with Morgagni hernia. Cytoreductive surgery including parietal peritonectomy of the right diaphragmatic peritoneum plus primary repair of hernial defect was performed safely and successfully, which achieved positive short-term results for patients with pseudomyxoma peritonei-associated Morgagni hernia.

PMID:37577349 | PMC:PMC10421794 | DOI:10.1007/s13691-023-00614-w
Is the measurement of sarcopenia associated with oncological disease in patients undergoing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy?

Fetched: August 2nd, 2023, 5:02am GMT by Adrian Hang Yue Siu

ANZ J Surg. 2023 Sep;93(9):2186-2191. doi: 10.1111/ans.18645. Epub 2023 Jul 31.

ABSTRACT

BACKGROUND: Peritoneal malignancies are challenging cancers to manage. While cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS and HIPEC), may offer a cure, it is a radical procedure associated with significant morbidity. Pre-emptive identification of deconditioned patients for optimization may mitigate surgical risk. However, the difficulty lies in identifying a cost-effective predictive tool. Recently, there has been interest in sarcopenia, which may occur due to malignancy. The purpose of this study was to assess the utility of sarcopenia at predicting post-operative outcomes.

METHODS: A quaternary-centre retrospective study of CRS and HIPEC patients (2017-2020), were conducted to determine the association between pre-operative sarcopenia on oncological (peritoneal carcinomatosis index (PCI)) and surgical outcomes (complications). Sarcopenia from lumbar CT-images were measured using Slice-o-matic™. Statistical differences were analysed using Mann-Whitney U and Chi-squared test.

RESULTS: Cohort analysis (n = 94) found 40% had sarcopenia, majority were female (53.2%), and average age of 55 years. The major pathologies was colorectal cancer (n = 39, 41.5%), appendix adenocarcinoma (n = 21, 22.3%), and pseudomyxoma peritonei (PMP) (n = 19, 20.2%). Sarcopenia was associated with decreased weight, 72.7 versus 82.2 kg (P = 0.014) and shorter survival, 1.4 versus 2.1 years (95% CI, 1.09-3.05, P = 0.032). Median PCI (excluding PMP) was 11 (6-18) and median PCI (only PMP) was 25 (11-32). Post-operatively, sarcopenia patients experienced more complications (72.5% vs. 64.8%, P = 0.001).

CONCLUSION: Pre-emptive identification of sarcopenia may be a useful prognostic indicator and predictor of post-operative outcomes in CRS and HIPEC. For oncological patients, sarcopenia may be an indicator of patients requiring targeted pre-operative rehabilitation, or advanced disease requiring further treatment.

PMID:37525364 | DOI:10.1111/ans.18645
Low grade appendiceal mucinous neoplasm metastatic to the ovary: A case report and intraoperative assessment guide

Fetched: August 1st, 2023, 5:02am GMT by Christine G T Nguyen

Int J Surg Case Rep. 2023 Aug;109:108563. doi: 10.1016/j.ijscr.2023.108563. Epub 2023 Jul 26.

ABSTRACT

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.

PMID:37524024 | PMC:PMC10400853 | DOI:10.1016/j.ijscr.2023.108563
A2AR Expression and Immunosuppressive Environment Independent of KRAS and GNAS Mutations in Pseudomyxoma Peritonei

Fetched: July 30th, 2023, 5:02am GMT by Shigeki Kusamura

Biomedicines. 2023 Jul 20;11(7):2049. doi: 10.3390/biomedicines11072049.

ABSTRACT

In pseudomyxoma peritonei (PMP), KRAS and GNAS mutations are frequent. We hypothesized that these mutations may contribute to the suppression of antitumor immunity: KRAS may induce GMCSF expression, while GNAS may enhance the expression of cyclic adenosine monophosphate and A2AR signaling. This study aimed to explore possible mechanisms facilitated by KRAS and GNAS 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 GNAS and KRAS 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 GNAS or KRAS 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. GNAS 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, p = 0.034) and significant downregulation of MET, IL8, PPARG, DTX4, HMGA1, ZIC2, WNT5B, and CCRL2. 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 GNAS and KRAS 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.

PMID:37509688 | PMC:PMC10377259 | DOI:10.3390/biomedicines11072049
Intraperitoneal BromAc® Does Not Interfere with the Healing of Colon Anastomosis

Fetched: July 15th, 2023, 5:02am GMT by Ahmed H Mekkawy

Cancers (Basel). 2023 Jun 24;15(13):3321. doi: 10.3390/cancers15133321.

ABSTRACT

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.

PMID:37444430 | PMC:PMC10340507 | DOI:10.3390/cancers15133321
Surgical treatment for recurrent intra-abdominal mucinous neoplasms

Fetched: July 12th, 2023, 5:02am GMT by H Leebmann

Chirurgie (Heidelb). 2023 Oct;94(10):845-849. doi: 10.1007/s00104-023-01925-7. Epub 2023 Jul 11.

ABSTRACT

BACKGROUND: The gold standard in the treatment of mucinous intra-abdominal neoplasms is cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC). Despite complete cytoreduction up to 45% of patients develop recurrences.

METHOD: A search and analysis of the current literature were carried out.

RESULTS: There is still controversy regarding the best treatment strategy for patients with recurrent pseudomyxoma peritonei (PMP) after CRS and HIPEC. The clinical management of these patients depends on many factors, such as the site and volume of recurrence, histological subtype and symptoms. Treatment options range from repeated surgery with curative intent with or without HIPEC to watch and wait strategies. In selected patients redo surgery is feasible and safe with low morbidity and mortality. Iterative complete CRS can result in a median 5‑year overall survival of more than 80%. Debulking surgery leads to a prolonged survival and to symptom control fora period with of nearly 2 years.

CONCLUSION: Repeated complete cytoreduction of recurrent PMP can result in long-term survival. Tumor debulking surgery may be particularly beneficial for symptomatic patients.

PMID:37432477 | DOI:10.1007/s00104-023-01925-7
Mucocele of the Appendix: A Case Report and Review of Literature

Fetched: July 12th, 2023, 5:02am GMT by Arshadullah Khan

Cureus. 2023 Jun 9;15(6):e40168. doi: 10.7759/cureus.40168. eCollection 2023 Jun.

ABSTRACT

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.

PMID:37431343 | PMC:PMC10329778 | DOI:10.7759/cureus.40168
Pseudomyxoma peritonei

Fetched: July 10th, 2023, 5:02am GMT by Nathaniel Juárez de la Garza

Med Clin (Barc). 2023 Dec 7;161(11):506. doi: 10.1016/j.medcli.2023.05.015. Epub 2023 Jul 7.

NO ABSTRACT

PMID:37423876 | DOI:10.1016/j.medcli.2023.05.015
Mucinous tumors of the peritoneum

Fetched: July 8th, 2023, 5:02am GMT by Anne Kristin Fischer

Chirurgie (Heidelb). 2023 Oct;94(10):823-831. doi: 10.1007/s00104-023-01926-6. Epub 2023 Jul 7.

ABSTRACT

INTRODUCTION: Disseminated peritoneal seeding of mucin-forming tumors is a rare malignant disease with variable prognosis. Histomorphological criteria are instrumental in the prognostic assessment. The past 10 years have led to a standardization of nomenclature and subsequently to the establishment of therapeutic standards. This article aims to provide the current status of the pathological classification, staging, and grading.

METHOD AND MATERIAL: Selective literature search in PubMed and Medline RESULTS: The vast majority of disseminated peritoneal mucinous diseases that correspond to the clinical presentation of pseudomyxoma peritonei (PMP) arise from mucinous tumors of the vermiform appendix. Here are to be distinguished: 1) low-grade appendiceal mucinous neoplasms (LAMN), 2) (very rare) high-grade appendiceal mucinous neoplasms (HAMN), 3) mucinous adenocarcinoma without signet ring cells (G2) and 4) mucinous adenocarcinoma with signet ring cells or signet ring cell carcinoma (G3). Other primary tumors only rarely induce PMP. Terms such as mucocele or mucinous cystadenoma of the appendix correspond to LAMN and should no longer be used. Prognostic distinctions are further made between low-grade PMP, which usually arises from LAMN, and the prognostically less favorable high-grade PMP, which usually arises from mucinous/signet ring cell adenocarcinoma or the rare HAMN. Disseminated peritoneal mucinous disease/PMP must then be further distinguished from prognostically excellent local mucin formation of the peri-appendix region.

DISCUSSION: The currently valid nomenclature, as it has emerged from consensus meetings and in parts has also found its way into the current WHO 2019, has significantly contributed to the fact that the prognosis of patients today can be better estimated and effective forms of treatment could be developed.

PMID:37418023 | DOI:10.1007/s00104-023-01926-6
Treatment outcome analysis of bevacizumab combined with cyclophosphamide and oxaliplatin in advanced pseudomyxoma peritonei

Fetched: July 6th, 2023, 5:02am GMT by Ying Zhang

World J Gastrointest Surg. 2023 Jun 27;15(6):1149-1158. doi: 10.4240/wjgs.v15.i6.1149.

ABSTRACT

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/m² ivgtt d1 and cyclophosphamide 500 mg/m² 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 vs 2.1, P = 0.022) and a completeness of cytoreduction score of 2-3 (8.9 vs 5.0, P = 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, P = 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.

PMID:37405093 | PMC:PMC10315110 | DOI:10.4240/wjgs.v15.i6.1149
Primary and secondary tumors of the peritoneum: key imaging features and differential diagnosis with surgical and pathological correlation

Fetched: July 4th, 2023, 5:02am GMT by Javier Miguez González

Insights Imaging. 2023 Jul 3;14(1):115. doi: 10.1186/s13244-023-01417-6.

ABSTRACT

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.

PMID:37395913 | PMC:PMC10317950 | DOI:10.1186/s13244-023-01417-6
Low-grade appendiceal mucinous neoplasm with puffer ball-like appearance: A case report

Fetched: July 1st, 2023, 5:02am GMT by Mihoko Yamazaki

Radiol Case Rep. 2023 Jun 6;18(8):2790-2795. doi: 10.1016/j.radcr.2023.05.006. eCollection 2023 Aug.

ABSTRACT

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.

PMID:37388265 | PMC:PMC10300464 | DOI:10.1016/j.radcr.2023.05.006
Oral administration of bromelain and acetylcysteine in pseudomyxoma peritonei (PMP) caused by low-grade appendiceal mucinous neoplasm (LAMN): a case report

Fetched: June 29th, 2023, 5:02am GMT by Dominik Geisel

J Med Case Rep. 2023 Jun 29;17(1):297. doi: 10.1186/s13256-023-04024-7.

ABSTRACT

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.

PMID:37381056 | PMC:PMC10308782 | DOI:10.1186/s13256-023-04024-7
ASO Visual Abstract: Targeted Next-Generation Sequencing Improves the Prognostication of Patients with Disseminated Appendiceal Mucinous Neoplasms (Pseudomyxoma Peritonei)

Fetched: June 29th, 2023, 5:02am GMT by Abigail I Wald

Ann Surg Oncol. 2023 Nov;30(12):7595-7596. doi: 10.1245/s10434-023-13795-8.

NO ABSTRACT

PMID:37378848 | DOI:10.1245/s10434-023-13795-8
Incidental finding of appendiceal mucinous neoplasms

Fetched: June 29th, 2023, 5:02am GMT by Franziska Köhler

Chirurgie (Heidelb). 2023 Oct;94(10):832-839. doi: 10.1007/s00104-023-01910-0. Epub 2023 Jun 28.

ABSTRACT

Low-grade appendiceal mucinous neoplasms (LAMN) are rare and are diagnosed in at least 0.13% of appendectomies in Germany, although significant underreporting is likely. Perforation of the tumors can result in abdominal mucinous collections, so-called pseudomyxoma peritonei (PMP). The challenge in the treatment of LAMN is the adequate approach to the incidental finding of these tumors. If a mucinous neoplasm is preoperatively suspected in cases of an acute condition, usually appendicitis, it must be weighed up whether a conservative approach is justifiable or whether immediate appendectomy is necessary. If this is the case, an intraoperative perforation of the appendix must be avoided and the complete abdominal cavity must be inspected for mucin deposits. If conservative treatment is possible, further treatment should take place at a specialized center. If the neoplasm is first found incidentally during surgery, perforation of the appendix should also be avoided and the entire abdominal cavity should be inspected for a PMP. If a PMP is present cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) should be performed in a specialized center. If LAMN are found during the postoperative histological work-up, it should be evaluated whether a perforation was present and mucin collections are noted in the surgical report. In the case of LAMN without evidence of a PMP, appendectomy is the adequate treatment. In cases of intra-abdominal mucinous collections, samples should be taken and further treatment should be performed at a center with sufficient expertise. An ileocecal resection or oncological hemicolectomy is not indicated. After adequate treatment, all patients should receive a follow-up using cross-sectional imaging (preferably magnetic resonance imaging, MRI) and determination of the tumor markers CEA, CA 19-9 and CA 125.

PMID:37378666 | DOI:10.1007/s00104-023-01910-0
Under the Hood: Understanding the Features of Mucin in Pseudomyxoma Peritonei

Fetched: June 29th, 2023, 5:02am GMT by Pedro Villarejo-Campos

J Clin Med. 2023 Jun 12;12(12):4007. doi: 10.3390/jcm12124007.

ABSTRACT

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 Proteobacteria and the genus Pseudomonas. Notably, Pseudomonas plecoglossicida, 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.

PMID:37373701 | PMC:PMC10298939 | DOI:10.3390/jcm12124007
Appendicitis to multivisceral transplantation: a career experience with appendiceal malignancy

Fetched: June 28th, 2023, 5:02am GMT by B J Moran

Ann R Coll Surg Engl. 2023 Jun 27. doi: 10.1308/rcsann.2023.0013. Online ahead of print.

ABSTRACT

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.

PMID:37367485 | DOI:10.1308/rcsann.2023.0013
Pseudomyxoma Peritonei Arising from Urachal Mucinous Neoplasms: a Case Series and Updated Literature Review

Fetched: June 27th, 2023, 5:02am GMT by Niccolo Allievi

Indian J Surg Oncol. 2023 Jun;14(Suppl 1):144-150. doi: 10.1007/s13193-022-01694-5. Epub 2023 Jan 18.

ABSTRACT

To report a case series of patients with pseudomyxoma peritonei (PMP) from urachal mucinous neoplasm (UMN) treated with CRS and HIPEC at a high-volume referral centre, along with an updated literature review. Retrospective review of cases treated between 2000 and 2021. A literature review using MEDLINE and Google Scholar databases was performed. Clinical presentation of PMP from UMN is heterogeneous, and common symptoms are abdominal distension, weight loss, fatigue and haematuria. At least one tumour marker among CEA, CA 19.9, and CA 125 was elevated in the six cases reported, and 5/6 had a preoperative working diagnosis of urachal mucinous neoplasm suspected on detailed cross-sectional imaging. Complete cytoreduction was achieved in five cases, while one patient underwent maximal tumour debulking. Histological findings mirrored the findings of PMP from appendiceal mucinous neoplasms (AMN). Overall survival ranged between 43 and 141 months after complete cytoreduction. On literature review, 76 cases have been reported to date. Complete cytoreduction is associated with good prognosis for patients with PMP from UMN. A definitive classification system is still not available.

SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s13193-022-01694-5.

PMID:37359937 | PMC:PMC10284775 | DOI:10.1007/s13193-022-01694-5
Bilateral Thoracic Cytoreductive Surgery for Pseudomyxoma Peritonei of Appendiceal Origin

Fetched: June 27th, 2023, 5:02am GMT by Sanket Mehta

Indian J Surg Oncol. 2023 Jun;14(Suppl 1):161-165. doi: 10.1007/s13193-023-01745-5. Epub 2023 Apr 26.

ABSTRACT

Pleural spread occurs in pseudomyxoma peritonei (PMP) in less than 10% of the patients and is treated by thoracic cytoreductive surgery with or without hyperthermic intrathoracic chemotherapy (HITOC). It is performed both for symptom palliation and disease control and includes pleurectomy and decortication and wedge and segmental lung resections. So far, only unilateral spread treated with a thoracic cytoreductive surgery (CRS) has been reported in literature. We report a patient with bilateral thoracic PMP following a complete abdominal CRS and hyperthermic intraperitoneal chemotherapy (HIPEC) who was treated with bilateral staged thoracic CRS and subsequently had a 4th CRS for abdominal disease. The staged procedure was performed as she was symptomatic due to the thoracic disease and there was disease on all pleural surfaces. HITOC was not performed. Both procedures were uneventful with no major morbidity. The patient is currently disease free nearly 84 months after the first abdominal CRS and 60 months after the second thoracic CRS. Thus, an aggressive CRS in the thorax in patients with PMP can result in a prolongation of survival while preserving the quality of life if the abdominal disease is controlled. A thorough understanding of the disease biology and surgical expertise are both essential for selecting the right patients for these complex procedures and achieving good short- and long-term outcomes.

PMID:37359933 | PMC:PMC10284746 | DOI:10.1007/s13193-023-01745-5
Long-Term Survival in Patients Treated by Cytoreductive Surgery with or Without HIPEC for Peritoneal Surface Malignancies-A report from the Indian HIPEC Registry

Fetched: June 27th, 2023, 5:02am GMT by Snita Sinukumar

Indian J Surg Oncol. 2023 Jun;14(Suppl 1):198-208. doi: 10.1007/s13193-023-01727-7. Epub 2023 Mar 9.

ABSTRACT

A previous report from the Indian HIPEC registry showed acceptable early survival and morbidity in patients undergoing cytoreductive surgery (CRS) + / - hyperthermic intraperitoneal chemotherapy (HIPEC). The goal of this retrospective study was to evaluate the long-term outcomes in these patients. Three hundred seventy-four patients treated from December 2010 to December 2016 and enrolled in the Indian HIPEC registry were included. All patients had completed 5 years from the date of surgery. The 1-, 3-, 5- and 7-year progression-free (PFS) and overall survival (OS) and factors affecting these were evaluated. The histology was epithelial ovarian cancer in 209 (46.5%), pseudomyxoma peritonei (PMP) in 65 (17.3%) and colorectal cancer in 46 (12.9%) patients. The peritoneal cancer index (PCI) was ≥ 15 in 160 (42.8%). A completeness of cytoreduction (CC) score of 0/1 resection was obtained in 83% (CC-0-65%; CC-1-18%). HIPEC was performed in 59.2%. At a median, follow-up of 77 months (6-120 months), 243 (64.9%) patients developed recurrence, and 236 (63%) died of any cause; 138 (36.9%) were lost to follow-up. The median OS was 56 months (95% CI 53.42-61.07), and the median PFS was 28 months (95% CI 37.5-44.4). The 1-, 3-, 5- and 7-year OS was 97.6%, 63%, 37.7% and 24% respectively. The 1-, 3-, 5- and 7-year PFS was 84.8%, 36.5%, 27.3% and 22% respectively. The use of HIPEC (p = 0.03) and PMP of appendiceal origin (p = 0.01) was independent predictors of a longer OS. CRS + / - /HIPEC may achieve long-term survival in patients with PM from different primary sites in the Indian scenario. More prospective studies are needed to confirm these findings and identify factors influencing long-term survival.

SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s13193-023-01727-7.

PMID:37359912 | PMC:PMC10284732 | DOI:10.1007/s13193-023-01727-7
Pseudomyxoma peritonei

Fetched: June 18th, 2023, 5:03am GMT by Jayendra Shukla

J R Coll Physicians Edinb. 2023 Sep;53(3):184-185. doi: 10.1177/14782715231179254. Epub 2023 Jun 17.

NO ABSTRACT

PMID:37329264 | DOI:10.1177/14782715231179254
Targeted Next-Generation Sequencing Improves the Prognostication of Patients with Disseminated Appendiceal Mucinous Neoplasms (Pseudomyxoma Peritonei)

Fetched: June 15th, 2023, 5:02am GMT by Abigail I Wald

Ann Surg Oncol. 2023 Nov;30(12):7517-7526. doi: 10.1245/s10434-023-13721-y. Epub 2023 Jun 14.

ABSTRACT

BACKGROUND: Appendiceal mucinous neoplasms (AMNs) with disseminated disease (pseudomyxoma peritonei) are heterogeneous tumors with variable clinicopathologic behavior. Despite the development of prognostic systems, objective biomarkers are needed to stratify patients. With the advent of next-generation sequencing (NGS), it remains unclear if molecular testing can improve the evaluation of disseminated AMN patients.

METHODS: Targeted NGS was performed for 183 patients and correlated with clinicopathologic features to include American Joint Committee on Cancer/World Health Organization (AJCC/WHO) histologic grade, peritoneal cancer index (PCI), completeness of cytoreduction (CC) score, and overall survival (OS).

RESULTS: Genomic alterations were identified for 179 (98%) disseminated AMNs. Excluding mitogen-activated protein kinase genes and GNAS due to their ubiquitous nature, collective genomic alterations in TP53, SMAD4, CDKN2A, and the mTOR genes were associated with older mean age, higher AJCC/WHO histologic grade, lymphovascular invasion, perineural invasion, regional lymph node metastasis, and lower mean PCI (p < 0.040). Patients harboring TP53, SMAD4, ATM, CDKN2A, and/or mTOR gene alterations were found to have lower OS rates of 55% at 5 years and 14% at 10 years, compared with 88% at 5 years and 88% at 10 years for patients without the aforementioned alterations (p < 0.001). Based on univariate and multivariate analyses, genomic alterations in TP53, SMAD4, ATM, CDKN2A, and/or the mTOR genes in disseminated AMNs were a negative prognostic factor for OS and independent of AJCC/WHO histologic grade, PCI, CC score, and hyperthermic intraperitoneal chemotherapy treatment (p = 0.006).

CONCLUSIONS: Targeted NGS improves the prognostic assessment of patients with disseminated AMNs and identifies patients who may require increased surveillance and/or aggressive management.

PMID:37314541 | DOI:10.1245/s10434-023-13721-y
Correlation between PSOGI pathological classification and survival outcomes of patients with pseudomyxoma peritonei treated using cytoreductive surgery and HIPEC: national referral centre experience and literature review

Fetched: June 14th, 2023, 5:02am GMT by Lorena Martín-Román

Pleura Peritoneum. 2023 May 1;8(2):65-74. doi: 10.1515/pp-2023-0001. eCollection 2023 Jun.

ABSTRACT

OBJECTIVES: The Peritoneal Surface Oncology Group International (PSOGI) consensus subdivided pseudomyxoma peritonei (PMP) into four groups according to histopathological features. The aim of this paper is to report survival outcomes after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) from a national referral centre and to correlate the PSOGI classification with survival.

METHODS: A retrospective study of a prospectively maintained database was performed. Consecutive patients treated with CRS + HIPEC for PMP of appendiceal origin were included (September-2013 to December-2021). Pathological features of the peritoneal disease were used to classify patients into the four groups proposed by PSOGI. Survival analysis was performed to evaluate the correlation of pathology on overall survival (OS) and disease-free survival (DFS).

RESULTS: Overall, 104 patients were identified; 29.6 % were reclassified as acellular mucin (AM), 43.9 % as low-grade mucinous carcinoma peritonei (LGMCP), 22.4 % as high-grade MCP (HGMCP) and 4.1 % as HGMCP with signet ring cells (HGMCP-SRC). Median PCI and rate of optimal cytoreduction were 19 and 82.7 %, respectively. Median OS and DFS were not reached, 5-year OS and DFS were 88.6(SD 0.04) % and 61.6(SD 0.06) %, respectively. Log-Rank test revealed significant differences in terms of OS and DFS across the different histological subgroups (p<0.001 in both cases). However, histology did not retain its significance in the multivariate analysis for OS or DFS (p=0.932 and p=0.872, respectively).

CONCLUSIONS: Survival outcomes after CRS + HIPEC for PMP are excellent. The PSOGI pathological classification correlates with OS and DFS, but differences were not significant at multivariate analysis when adjusted for other prognostic factors.

PMID:37304162 | PMC:PMC10249754 | DOI:10.1515/pp-2023-0001
Overall survival and morbidity are not associated with advanced age for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy: a single centre experience

Fetched: June 14th, 2023, 5:02am GMT by Ernest Cheng

Pleura Peritoneum. 2023 Apr 11;8(2):83-90. doi: 10.1515/pp-2022-0202. eCollection 2023 Jun.

ABSTRACT

OBJECTIVES: Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) has enabled better prognosis for patients with peritoneal surface malignancies. However, in older age groups, short -and long-term outcomes are still perceived as poor. We evaluated patients aged 70 and over and determine if age is a predictor of morbidity, mortality and overall survival (OS).

METHODS: A retrospective cohort analysis was performed on CRS/HIPEC patients and categorised by age. The primary outcome was overall survival. Secondary outcomes included morbidity, mortality, hospital and incentive care unit (ICU) stay and early postoperative intraperitoneal chemotherapy (EPIC).

RESULTS: A total of 1,129 patients were identified with 134 aged 70+ and 935 under 70. There was no difference in OS (p=0.175) or major morbidity (p=0.051). Advanced age was associated with higher mortality (4.48 vs. 1.11 %, p=0.010), longer ICU stay (p<0.001) and longer hospitalisation (p<0.001). The older group was less likely to achieve complete cytoreduction (61.2 vs. 73 %, p=0.004) and receive EPIC (23.9 vs. 32.7 %, p=0.040).

CONCLUSIONS: In patients undergoing CRS/HIPEC, age of 70 and above does not impact OS or major morbidity but is associated with increased mortality. Age alone should not be a limiting factor in selecting CRS/HIPEC patients. Careful multi-disciplinary approach is needed when considering those of advanced age.

PMID:37304160 | PMC:PMC10249755 | DOI:10.1515/pp-2022-0202
Treatment of Peritoneal Surface Malignancies by Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in Spain: Results of the National Registry of the Spanish Group of Peritoneal Oncologic Surgery (REGECOP)

Fetched: June 12th, 2023, 5:02am GMT by Israel Manzanedo

J Clin Med. 2023 May 31;12(11):3774. doi: 10.3390/jcm12113774.

ABSTRACT

INTRODUCTION: Treatment of Peritoneal Surface Malignancies (PSM) with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) has achieved results never seen before in these patients, which classically have a poor prognosis. The possibility of conducting clinical trials in these diseases is complicated, since some of them are rare, so the analysis of large databases provides very valuable scientific information. The aim of this study is to analyze the global results of the National Registry of the Spanish Group of Peritoneal Oncologic Surgery (REGECOP), whose objective is to register all patients scheduled for HIPEC nationwide.

METHODS: This is a retrospective analysis of the data recorded in the REGECOP from 36 Spanish hospitals from 2001 to 2021. There were 4159 surgical interventions in 3980 patients.

RESULTS: 66% are women and 34% are men with a median age of 59 years (range 17-86). 41.5% of the patients were treated for Peritoneal Metastases (PM) of colorectal cancer (CRC); 32.4% were women with ovarian cancer (OC) with PM; 12.8% were treated for pseudomyxoma peritonei (PMP); 6.2% had PM from gastric cancer (GC); 4.9% had PM of non-conventional origin; and, finally, 2.1% of cases were patients diagnosed with peritoneal mesothelioma. The median Peritoneal Cancer Index (PCI) was 9 (0-39), and complete cytoreduction was achieved in 81.7% of the procedures. Severe morbidity (Dindo-Clavien grade III-IV) was observed in 17.7% of surgeries, with 2.1% mortality. Median hospital stay was 11 days (0-259). Median overall survival (OS) was 41 months for CRC patients, 55 months for women with OC, was not reached in PMP patients, was 14 months for GC patients, and 66 months in mesothelioma patients.

CONCLUSIONS: large databases provide extremely useful data. CRS with HIPEC in referral centers is a safe treatment with encouraging oncologic results in PSM.

PMID:37297969 | PMC:PMC10253421 | DOI:10.3390/jcm12113774
Pseudomyxoma peritonei and an incidental low-grade appendiceal mucinous neoplasm and neuroendocrine appendiceal collision tumour: a case report

Fetched: May 31st, 2023, 5:02am GMT by Ankit Gupta

J Surg Case Rep. 2023 May 25;2023(5):rjad281. doi: 10.1093/jscr/rjad281. eCollection 2023 May.

ABSTRACT

Appendiceal collision tumours are extremely rare, with most reported cases describing tumours consisting of a mucinous component and a neuroendocrine component. Low-grade appendiceal mucinous neoplasms, in some cases, have a tendency to rupture and disseminate their mucin-producing cells throughout the abdominal cavity, leading to a clinical syndrome known as pseudomyxoma peritonei (PMP). We present the case of a 64-year-old male who initially presented with acute appendicitis and was subsequently found to have PMP and appendiceal malignancy. After several years of scans, surgical intervention and histological analysis, it became apparent that the appendiceal malignancy was comprised of distinct cell types. The patient underwent two rounds of cytoreductive surgery with hyperthermic intraperitoneal chemotherapy, which resulted in a 2-year disease-free period. Unfortunately, the PMP recurred, having morphological changes consistent with a more aggressive disease process.

PMID:37251256 | PMC:PMC10212664 | DOI:10.1093/jscr/rjad281
Bromelain and acetylcysteine (BromAc®): a novel approach to the treatment of mucinous tumours

Fetched: May 12th, 2023, 5:02am GMT by Henry K Wen

Am J Cancer Res. 2023 Apr 15;13(4):1522-1532. eCollection 2023.

ABSTRACT

Mucins are a significant extracellular component of neoplastic entities such as pseudomyxoma peritonei and several gastrointestinal adenocarcinomas. Mucinous tumours present a challenge for systemic treatments due to poor drug penetrance and increased resistance. Therefore, the development of an effective mucolytic therapy has significant therapeutic implications for these tumour types. BromAc^® is a novel mucolytic agent consisting of bromelain and acetylcysteine. It has demonstrated significant mucolysis and antitumour effects in vitro and in vivo for several mucinous tumours. It has also exhibited a synergistic potentiation of the effect of several cytotoxic agents on mucinous tumours in preclinical studies. Furthermore, it demonstrates locoregional safety and efficacy in animal and clinical studies. This literature review will summarise the history of BromAc^® for mucinous tumours, including its conception, preclinical development in vitro and in vivo, and clinical evidence. The implications of current data and directions for future research are then discussed.

PMID:37168359 | PMC:PMC10164791
A rare case report: low grade appendiceal mucinous neoplasm (LAMN) located under the left costal margin, the gastric level and the transverse colon

Fetched: May 9th, 2023, 5:02am GMT by Halit Maloku

J Surg Case Rep. 2023 May 3;2023(5):rjad234. doi: 10.1093/jscr/rjad234. eCollection 2023 May.

ABSTRACT

We report a case with a low-grade appendiceal mucinous neoplasm, located under the left costal margin, the gastric level and the transverse colon. The mucinous appendiceal neoplasm has caused intussusception of the appendix in the cecum, completely dislocating the cecum on the left side of the upper abdomen. In such cases, it is important to diagnose prior to surgical treatment to avoid mucocele perforation and intraoperative dissemination. The patient underwent a right hemicolectomy procedure, completely removing the mass according to oncological principles. The atypical localization of the cecum leads to difficulty in diagnosing the mucinous neoplasm of the appendix. It is important to know the diagnosis before the operation to plan the right course of treatment.

PMID:37153824 | PMC:PMC10156431 | DOI:10.1093/jscr/rjad234
A Novel Assessment of Metabolic Pathways in Peritoneal Metastases from Low-Grade Appendiceal Mucinous Neoplasms

Fetched: May 7th, 2023, 5:02am GMT by Eric A Hanse

Ann Surg Oncol. 2023 Aug;30(8):5132-5141. doi: 10.1245/s10434-023-13587-0. Epub 2023 May 6.

ABSTRACT

BACKGROUND: There is a paucity of targeted therapies for patients with pseudomyxoma peritonei (PMP) secondary to low-grade appendiceal mucinous neoplasms (LAMNs). Dysregulated metabolism has emerged as a hallmark of cancer, and the relationship of metabolomics and cancer is an area of active scientific exploration. We sought to characterize phenotypic differences found in peritoneal metastases (PM) derived from LAMN versus adenocarcinoma.

METHODS: Tumors were washed with phosphate-buffered saline (PBS), microdissected, then dissociated in ice-cold methanol dried and reconstituted in pyridine. Samples were derivatized in tert-butyldimethylsilyl (TBDMS) and subjected to gas chromatography-coupled mass spectrometry. Metabolites were assessed based on a standard library. RNA sequencing was performed, with pathway and network analyses on differentially expressed genes.

RESULTS: Eight peritoneal tumor samples were obtained and analyzed: LAMNs (4), and moderate to poorly differentiated adenocarcinoma (colon [1], appendix [3]). Decreases in pyroglutamate, fumarate, and cysteine in PM from LAMNs were found compared with adenocarcinoma. Analyses showed the differential gene expression was dominated by the prevalence of metabolic pathways, particularly lipid metabolism. The gene retinol saturase (RETSAT), downregulated by LAMN, was involved in the multiple metabolic pathways that involve lipids. Using network mapping, we found IL1B signaling to be a potential top-level modulation candidate.

CONCLUSIONS: Distinct metabolic signatures may exist for PM from LAMN versus adenocarcinoma. A multitude of genes are differentially regulated, many of which are involved in metabolic pathways. Additional research is needed to identify the significance and applicability of targeting metabolic pathways in the potential development of novel therapeutics for these challenging tumors.

PMID:37149550 | DOI:10.1245/s10434-023-13587-0
A Metastatic Breast Tumor of an Appendiceal Signet Ring Cell Carcinoma

Fetched: May 2nd, 2023, 5:02am GMT by Kenta Okumura

Case Rep Oncol. 2023 Apr 26;16(1):267-272. doi: 10.1159/000529672. eCollection 2023 Jan-Dec.

ABSTRACT

A 54-year-old woman with pseudomyxoma peritonei from an appendiceal signet ring cell carcinoma was referred to our hospital. Right massive effusion with cytology-proven malignant cells was controlled with thoracentesis. Pathological study after intraperitoneal (IP) chemotherapy, hyperthermic IP chemotherapy, and cytoreductive surgery showed no malignant cells in the abdomen except for the appendix and greater omentum. Although the patient noticed a right breast mass, mammography (MMG) showed no abnormality. Ultrasonography showed right breast masses consisting of hypo- and hyper-echoic areas without clear tumor margins. Magnetic resonance imaging (MRI) with contrast medium showed multiple lesions showing persistent enhancement pattern. Pathological study of the vacuum-assisted biopsy specimen showed signet ring cells growing in diffuse, trabecular, and linear fashions, leading to the diagnosis of metastatic breast tumors from the appendiceal signet ring cell carcinoma. Positron emission tomography (PET)/computed tomography (CT) showed no fluorodeoxyglucose uptake in the breasts. The patient was treated with simple mastectomy for local control. Pathological study of the resected breast showed predominant localization of the metastatic breast tumors deep in the mammary gland and lymphovascular invasion. Metastatic breast tumors from appendiceal signet ring cell carcinomas are extremely rare and can sometimes be difficult to detect with MMG and PET/CT. General surgeon should note that appendiceal signet ring cell carcinomas can metastasize to the breast, presumably through lymphatic permeation from malignant pleural effusion, without abnormal MMG and PET/CT findings.

PMID:37123607 | PMC:PMC10134057 | DOI:10.1159/000529672
Peritoneal inclusion cysts in a young male: A case report

Fetched: April 28th, 2023, 5:02am GMT by Callie Killoran

Int J Surg Case Rep. 2023 May;106:108248. doi: 10.1016/j.ijscr.2023.108248. Epub 2023 Apr 23.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Peritoneal inclusion cyst is a rare benign condition with low potential for malignant transformation but high recurrence rates. Debulking surgery is the recommended first line management for these patients, however, recurrence rates are up to 50 % (Padmanabhan et al., 2020; Chapel and Husain, 2021).

CASE PRESENTATION: A 26-year-old male being worked up for non-specific abdominal pain with cross-sectional imaging showing multiple multicystic lesions in the abdomen and pelvis. There was a pre-operative suspicion of Pseudomyxoma Peritonei and decision was made for diagnostic laparoscopy and biopsy. Mucin and an abnormal small bowel mesentery was found intraoperatively and sampled leading to the diagnosis of peritoneal inclusion cyst.

CLINICAL DISCUSSION: Treatment of peritoneal inclusion cyst range from surveillance to aggressive treatment with complete cytoreductive surgery with involved field peritonectomy and hyperthermic intra-peritoneal chemotherapy.

CONCLUSION: First line management of peritoneal inclusion cysts is for debulking surgery. Arguments for less invasive and more aggressive management has been proposed, however, further data needs to be collected to determine gold standard of treatment.

PMID:37105030 | PMC:PMC10164883 | DOI:10.1016/j.ijscr.2023.108248
Low-grade mucinous neoplasm arising from mature ovarian teratoma with pseudomyxoma peritonei

Fetched: April 9th, 2023, 5:02am GMT by Ridin Balakrishnan

Int J Gynecol Cancer. 2023 Jun 5;33(6):1005-1006. doi: 10.1136/ijgc-2022-004099.

NO ABSTRACT

PMID:37028808 | DOI:10.1136/ijgc-2022-004099
Survival benefits with EPIC in addition to HIPEC for low grade appendiceal neoplasms with pseudomyxoma peritonei: a propensity score matched study

Fetched: April 7th, 2023, 5:02am GMT by Raymond Hayler

Pleura Peritoneum. 2023 Mar 16;8(1):27-35. doi: 10.1515/pp-2022-0205. eCollection 2023 Mar.

ABSTRACT

OBJECTIVES: Appendiceal cancer is a rare malignancy, occurring in roughly 1.2 per 100,000 per year. Low grade appendiceal neoplasams (LAMN) in particular can lead to pseudomyxoma peritonei (PMP), and respond poorly to systemic chemotherapy. Standard treatment includes cytoreduction surgery (CRS) with addition of heated intraoperative peritoneal chemotherapy (HIPEC). Several centres include early postoperative intraperitoneal chemotherapy (EPIC) however; the literature is mixed on the benefits. We aim to examine the benefits of additional EPIC through a propensity-matched analysis.

METHODS: Patients with LAMN with PMP who underwent cytoreductive surgery at St George hospital between 1996 and 2020 were included in this retrospective analysis. Propensity score matching was performed with the following used to identify matched controls; sex, age, American Society of Anesthesiologists (ASA) grade, peritoneal cancer index (PCI) and morbidity grade. Outcomes measured included length of stay and survival.

RESULTS: A total of 224 patients were identified of which 52 received HIPEC alone. Propensity matching was performed to identify 52 matched patients who received HIPEC + EPIC. Those receiving HIPEC + EPIC were younger at 54.3 vs. 58.4 years (p=0.044). There was a median survival benefit of 34.3 months for HIPEC + EPIC (127.3 vs. 93.0 months, p=0.02). Median length of stay was higher in those who received EPIC (25.0 vs. 23.5 days, p=0.028).

CONCLUSIONS: In LAMN with PMP, the addition of EPIC to HIPEC with CRS improves overall survival in propensity score matched cases but results in prolonged hospitalisation. The use of EPIC should still be considered in selected patients.

PMID:37020474 | PMC:PMC10067551 | DOI:10.1515/pp-2022-0205
Determining a minimum data set for reporting clinical and radiologic data for pseudomyxoma peritonei

Fetched: April 7th, 2023, 5:02am GMT by Thale D J H Patrick-Brown

Pleura Peritoneum. 2023 Mar 21;8(1):1-9. doi: 10.1515/pp-2022-0200. eCollection 2023 Mar.

ABSTRACT

OBJECTIVES: Pseudomyxoma peritonei (PMP) is a rare cancer currently affecting over 11,736 patients across Europe. Since PMP is so uncommon, collaboration between scientific centers is key to discovering the mechanisms behind the disease, efficient treatments, and targets pointing to a cure. To date, no consensus has been reached on the minimum data that should be collected during PMP research studies. This issue has become more important as biobanking becomes the norm. This paper begins the discussion around a minimum data set that should be collected by researchers through a review of available clinical trial reports in order to facilitate collaborative efforts within the PMP research community.

CONTENT: A review of articles from PubMed, CenterWatch, ClinicalTrials.gov and MedRxiv was undertaken, and clinical trials reporting PMP results selected.

SUMMARY: There is a core set of data that researchers report, including age and sex, overall survival, peritoneal cancer index (PCI) score, and completeness of cytoreduction, but after this, reports become variable.

OUTLOOK: Since PMP is a rare disease, it is important that reports include as large of a number of standardised data points as possible. Our research indicates that there is still much ground to cover before this becomes a reality.

PMID:37020469 | PMC:PMC10067554 | DOI:10.1515/pp-2022-0200
A huge pseudomyxoma pleurii mimicking hydatid cyst: A case report

Fetched: April 6th, 2023, 5:02am GMT by Zekrullah Baset

Int J Surg Case Rep. 2023 Apr;105:108098. doi: 10.1016/j.ijscr.2023.108098. Epub 2023 Apr 1.

ABSTRACT

INTRODUCTION: Pseudomyxoma pleurii is a rare disease that is defined by the pleural extension of pseudomyxoma peritonei, usually secondary to a mucinous neoplasm of the appendix or ovary. It is characterized by diffuse mucinous deposits on the pleural surface.

CASE PRESENTATION: A 31-year-old woman presented to the hospital with dyspnea, an increased respiratory rate, and decreased oxygen saturation. Following an appendectomy for a perforated mucinous appendiceal tumor eight years ago, the patient underwent multiple surgeries for the resection of mass deposits in the peritoneal cavity. At presentation, her chest computed tomography with contrast revealed cystic mass deposits on the right-side pleura with a massive multi-locular pleural effusion mimicking hydatid cyst. Upon histopathologic examination, multiple small cystic structures lined by tall columnar epithelium with basally placed bland nuclei floating in the mucin pools were noted.

CLINICAL DISCUSSION: Pseudomyxoma peritonei often leads to abdominal distention, intestinal blockage, anorexia, cachexia, and eventually death. It rarely spreads outside the abdomen, and its extension to the pleura is extremely unusual, with only a small number of cases documented in the literature to date. Radiologically, pseudomyxoma pleurii may resemble hydatid cyst of the lung and pleura.

CONCLUSION: Pseudomyxoma pleurii is a rare entity with a poor prognosis that usually arises secondary to Pseudomyxoma peritonei. The risk of morbidity and mortality is reduced by early diagnosis and treatment. The present case places emphasis on the inclusion of pseudomyxoma pleurii in the differential diagnosis of pleural lesions in patients with the history of appendiceal or ovarian mucinous tumors.

PMID:37018950 | PMC:PMC10112193 | DOI:10.1016/j.ijscr.2023.108098
Four cases of pseudomyxoma peritonei with ovarian tumors at our hospital

Fetched: March 30th, 2023, 5:02am GMT by Riho Yazawa

Fukushima J Med Sci. 2023 Apr 5;69(1):57-65. doi: 10.5387/fms.2022-41. Epub 2023 Mar 30.

ABSTRACT

We describe four cases of pseudomyxoma peritonei (PMP) that were diagnosed and treated at our hospital.Case 1: A 26-year-old woman with a large multicystic ovarian tumor and massive ascites was diagnosed with PMP originating from a borderline mucinous ovarian tumor. She underwent fertility-preserving staging laparotomy and was treated with three courses of intraperitoneal chemotherapy. There has been no recurrence in the 15 years since her first operation. Case 2: A 72-year-old woman with a giant ovarian tumor and massive ascites was diagnosed with PMP originating from low-grade appendiceal mucinous neoplasm (LAMN). After laparotomy, the patient was managed conservatively because she did not want aggressive treatment. She has remained asymptomatic with a small amount of ascites for 3 years. Case 3: A 82-year-old woman with ovarian tumors, massive ascites, and suspected PMP underwent emergency laparotomy due to appendiceal perforation and pan-peritonitis. She was diagnosed with PMP originating from LAMN. She has remained asymptomatic with a small amount of ascites for 2 years. Case 4: A 42-year-old woman with multicystic ovarian tumors and massive ascites underwent laparotomy. She was diagnosed with PMP originating from LAMN. Since multidisciplinary treatment was indicated and desired, the patient was referred to a specialized facility where cytoreductive surgery and hyperthermic intraperitoneal chemotherapy was performed. The patient has done well since the treatment.Although most cases of PMP originate from mucinous tumors of the appendix, female patients with PMP often present with ovarian tumors and are commonly referred to gynecology clinics. It is therefore important for gynecologists to be familiar with PMP and to be able to diagnose it accurately and select the most suitable management including multidisciplinary treatments.

PMID:36990788 | PMC:PMC10122967 | DOI:10.5387/fms.2022-41
Re: an unusual life-threatening case of appendiceal pseudomyxoma peritonei (PMP) mimicking sigmoid volvulus

Fetched: March 24th, 2023, 5:02am GMT by Sabri Selcuk Atamanalp

ANZ J Surg. 2023 May;93(5):1449. doi: 10.1111/ans.18274. Epub 2023 Mar 23.

NO ABSTRACT

PMID:36951048 | DOI:10.1111/ans.18274
Pseudomyxoma peritonei induced by a well-differentiated appendicular mucinous adenocarcinoma: An uncommon cause of isolated ascites in a young man patient

Fetched: March 21st, 2023, 5:02am GMT by Nitah Harivony Randriamifidy

Clin Case Rep. 2023 Mar 14;11(3):e7103. doi: 10.1002/ccr3.7103. eCollection 2023 Mar.

ABSTRACT

Pseudomyxoma peritonei (PMP) remains difficult to diagnose and has a reserved prognosis. Pseudomyxoma peritonei is a rare entity, of appendicular origin in the majority of cases. Its clinical symptomatology is not specific, and the diagnosis is evoked by imaging and surgery and confirmed by histology.

PMID:36937631 | PMC:PMC10014516 | DOI:10.1002/ccr3.7103
External multicentre validation of pseudomyxoma peritonei PSOGI-Ki67 classification

Fetched: March 20th, 2023, 5:02am GMT by A Arjona-Sanchez

Eur J Surg Oncol. 2023 Aug;49(8):1481-1488. doi: 10.1016/j.ejso.2023.03.206. Epub 2023 Mar 15.

ABSTRACT

BACKGROUND: Pseudomyxoma peritonei (PMP) is a rare malignant disease. Adding of the Ki67 proliferation index to the PSOGI PMP classification provided two different subcategories of the extensive HG-PMP group (HG-PMP ≤15% and HG-PMP >15%) with different survival in a previous unicentric study. This study aims to carry out an external and multicentre validation of this new proposed classification.

METHOD: It was a prospective analysis of samples from a historical and international cohort of patients. A representative area with higher cellular density was used to determine the Ki67%. The Ki67 proliferation index (%) was determined in all the HG-PMP patients. A Cox proportional hazard models and multivariable COX models were used. The Kaplan-Meier method and the two-tailed log-rank test were used to analyse the effect of different PSOGI-Ki67 categories on OS and DFS. Its predictive accuracy was analysed using Harrel's C-index and the ROC curve. The calibration was performed using the calibration plots matching.

RESULTS: After exclusions, 349 patients were available for analysis. The 5-years OS were 86% for LG-PMP, 59% for HG-PMP≤15, 38% for HG-PMP>15 and 42% for SRC-PMP (p = 0.0001). The 5-years DFS were 49% for LG-PMP, 35% for HG-PMP≤15, 16% for HG-PMP>15 and 18% SRC-PMP (p = 0.0001). The discrimination capability of PSOGI-Ki67 was validated.

CONCLUSION: the PSOGI-Ki67 classification discriminates and predicts the OS and DFS in patients with PMP dividing the HG-PMP category into two well-defined sub-categories. The Ki67 proliferation index should be incorporated routinely in the pathology report for these patients.

PMID:36935222 | DOI:10.1016/j.ejso.2023.03.206
Development and Validation of Nomograms to Predict Survival in Patients Undergoing Complete Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei of Appendiceal Origin

Fetched: March 16th, 2023, 5:02am GMT by Kandiah Chandrakumaran

JAMA Surg. 2023 May 1;158(5):522-530. doi: 10.1001/jamasurg.2023.0112.

ABSTRACT

IMPORTANCE: Pseudomyxoma peritoni, a rare condition characterized by mucinous ascites and peritoneal deposits, mainly originates from a ruptured mucinous appendix tumor and is considered an indolent disease but can progress and become fatal. Optimal treatment to improve cure and survival rates involves complete cytoreductive surgery (CCRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Accurate predictive models are useful in supporting and informing treatment strategies and stratifying patient follow-up.

OBJECTIVE: To evaluate the prognostic significance of clinically important variables and generate validated nomograms to predict overall (OS) and disease-free survival (DFS) following CCRS and hyperthermic intraperitoneal HIPEC for pseudomyxoma peritonei (PMP) of appendiceal origin.

DESIGN, SETTING, AND PARTICIPANTS: This retrospective study used prospectively collected data on patients who had cytoreductive surgery (CRS) and HIPEC in a single institution between 1994 and 2018. The cohort was randomly allocated into development (70%) and validation (30%) sets. Univariate and multivariate analyses were performed with Cox proportional hazards regression.

MAIN OUTCOMES AND MEASURES: A prediction model was developed with significant prognostic factors identified by multivariate analysis. The model's prognostic performance was evaluated with the concordance index (C index). The nomogram was calibrated by comparing the predicted and observed probabilities.

RESULTS: Of 2637 CRS and HIPEC operations, 1102 patients (female, 64.4%; median age [IQR], 57.0 [48.0-66.0] years) (41.8%) had CCRS for PMP of appendiceal origin. Elevated tumor markers, peritoneal carcinomatosis index, gastrectomy, and tumor grade were independent predictive factors for DFS. Gender, age, elevated tumor makers, peritoneal carcinomatosis index, and tumor grade influenced OS. The nomograms were generated with respective prognostic factors. The nomograms showed good performance in predicting survival. Median OS of the cohort was 16.5 years (95% CI, 13.7-19.2) with a 5-year probability of survival of 80.2%. The median DFS was 10.3 years (95% CI, 7.2- 13.3) and the 5-year probability of recurrence-free survival was 60.5%.

CONCLUSIONS AND RELEVANCE: Clinically important independent predictors for survival and recurrence were selected to develop the nomograms for OS and DFS. These 2 nomograms are user friendly and useful tools for patient management with clinical trial design applications.

PMID:36920381 | PMC:PMC10018403 | DOI:10.1001/jamasurg.2023.0112
Is a Prognostic Nomogram for Complete Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei Useful?

Fetched: March 16th, 2023, 5:02am GMT by Edward A Levine

JAMA Surg. 2023 May 1;158(5):531. doi: 10.1001/jamasurg.2023.0119.

NO ABSTRACT

PMID:36920379 | DOI:10.1001/jamasurg.2023.0119
Effect of standardized fluid management on cardiac function after CRS + HIPEC in patients with PMP: a single-center case-control study

Fetched: March 9th, 2023, 5:04am GMT by Rui Yang

Int J Hyperthermia. 2023;40(1):2182749. doi: 10.1080/02656736.2023.2182749.

ABSTRACT

OBJECTIVE: To investigate the effects of standardized fluid management (SFM) on cardiac function in patients with pseudomyxoma peritonei (PMP) after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC).

METHOD: Patients with PMP who underwent CRS + HIPEC at our center were retrospectively analyzed. The patients were divided into control and study groups according to whether SFM was applied after CRS + HIPEC. We compared the preoperative and postoperative cardiac and renal function parameters, daily fluid volume three days after CRS, and cardiovascular-related adverse events. Univariate and multivariate analyses were performed to identify the indicators affecting clinical prognosis.

RESULT: Among the 104 patients, 42 (40.4%) were in the control group and 62 (59.6%) in the study group. There were no statistically significant differences between the two groups in the main clinicopathological characteristics, preoperative cardiac and renal function parameters, and CRS + HIPEC-related indicators. The incidences of cardiac troponin I (CTNI) > upper limit of normal (ULN), >2 × ULN, >3 × ULN, serum creatinine > ULN, and blood urea nitrogen > ULN were higher in the control group than in the study group (p < 0.05). The median daily fluid volume of the control group was higher than that of the study group 3 days after CRS (p < 0.05). Postoperative CTNI > 2 × ULN was an independent risk factor for serious circulatory adverse events. Survival analysis revealed pathological grading, completeness of cytoreduction score, and postoperative CTNI > ULN as independent prognostic factors.

CONCLUSIONS: SFM after CRS + HIPEC in patients with PMP may reduce cardiovascular adverse events risk and improve clinical outcomes.

PMID:36889694 | DOI:10.1080/02656736.2023.2182749
Benign multicystic peritoneal mesothelioma mimicking mucinous ovarian neoplasm with pseudomyxoma peritonei

Fetched: March 8th, 2023, 5:02am GMT by Ceylan Altintas Taslicay

BMJ Case Rep. 2023 Mar 7;16(3):e254116. doi: 10.1136/bcr-2022-254116.

ABSTRACT

Cystic mesothelioma, also known as benign multicystic peritoneal mesothelioma (BMPM), is a rare neoplasm and one of the five main histological types of peritoneal mesothelioma. Although it is usually considered benign histologically, it is increasingly accepted as a 'borderline malignancy' because the local recurrence rate is high. It is more common in middle-aged women and is generally asymptomatic. Considering that BMPM is often found in the pelvis, it is difficult to differentiate from other pelvic and abdominal lesions, such as cystic ovarian masses, especially mucinous cystadenoma-adenocarcinoma, pseudomyxoma peritonei, etc. Preoperative diagnosis is very difficult due to its rarity and lack of specific diagnostic imaging and clinical findings. Definitive diagnosis is made only by pathological evaluation. In this article, we aimed to present the imaging findings of a BMPM case in a woman who was diagnosed with mucinous ovarian neoplasm with pseudomyxoma peritonei preoperatively and underwent cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.

PMID:36882266 | PMC:PMC10008379 | DOI:10.1136/bcr-2022-254116
Clinicopathological features of non-carcinoid malignant epithelial appendix tumors and risk factors for recurrence, single-center experience

Fetched: March 3rd, 2023, 5:02am GMT by Ozturk Ates

Indian J Cancer. 2022 Oct 12. doi: 10.4103/ijc.IJC_395_20. Online ahead of print.

ABSTRACT

BACKGROUND: Non-carcinoid appendix epithelial tumors are rare. These tumors include low-grade and high-grade mucinous neoplasm also adenocarcinomas. We aimed to investigate the clinicopathological features, treatment, and risk factors of recurrence.

METHODS: Patients diagnosed between 2008 and 2019 were retrospectively analyzed. Categorical variables were expressed as percentages and compared using the Chi-square test or Fisher's exact tests. Overall survival and Disease-free survival of the groups were calculated by the Kaplan-Meier method, and the log-rank test was used to compare the survival rates.

RESULTS: A total of 35 patients were included in the study. Of the patients, 19 (54%) were women and the median diagnosis age of patients was 50.4 years (19-76). As for pathological types, a total of 14 (40%) patients were mucinous adenocarcinoma and 14 (40%) patients were Low-Grade Mucinous Neoplasm (LGMN). Lymph node excision and lymph node involvement were 23 (65%) and 9 (25%) patients respectively. The majority of patients were stage 4 (27, 79%) and 25 (71%) of these patients had peritoneal metastasis. A total of 48.6% patients had been treated with cytoreductive surgery and hyper-thermic intraperitoneal chemotherapy. Median Peritoneal cancer index value was 12 (2-36). The median follow-up time was 20 (1-142) months. Recurrence developed in 12 (34%) of patients. When risk factors for recurrence are considered, there was a statistically significant difference in appendix tumors with high-grade, adenocarcinoma pathology, ones with peritoneal cancer index ≥12 and not having pseudomyxoma peritonei. Median disease-free survival was 18 (13-22, 95% CI) months. Median overall survival could not be reached while the 3-year survival rate was 79%.

CONCLUSION: The risk of recurrence is higher in high-grade appendix tumors, having peritoneal cancer index ≥ 12, not having pseudomyxoma peritonei and adenocarcinoma pathology. High-grade appendix adenocarcinoma patients should be followed closely for recurrence.

PMID:36861708 | DOI:10.4103/ijc.IJC_395_20
Ultrasound-guided day-case wide-bore percutaneous mucin aspiration in advanced pseudomyxoma peritonei

Fetched: February 27th, 2023, 5:02am GMT by P Borg

Clin Radiol. 2023 May;78(5):e458-e462. doi: 10.1016/j.crad.2023.01.016. Epub 2023 Feb 16.

ABSTRACT

AIM: To evaluate percutaneous ultrasound-guided day-case mucin aspiration in advanced pseudomyxoma peritonei (PMP) using a wide-bore drain with regards to its safety and efficacy.

MATERIALS AND METHODS: All patients who underwent percutaneous mucin aspiration for PMP between 2019-2021 at a single national peritoneal tumour service were included in this study. Under local anaesthesia, a suction-enabled 28-32 F catheter was used for drainage following wire-guided track dilatation. The volume drained and difference in abdominal girth pre- and post-procedure were measured. Patients graded difficulty in breathing and abdominal discomfort pre- and post-procedure. Histology reports were reviewed.

RESULTS: Sixteen patients received 56 percutaneous mucin aspirations between 2019-2021. The aetiology was a low-grade appendiceal mucinous neoplasm (LAMN) in 50% of patients. The mean amount of mucin drained was 7,320 ± 3,000ml (range 300-13,500 ml). The mean reduction in abdominal girth post-procedure was 12.2 ± 5 cm (range 0-27 cm). Only grade 1 complications were observed.

CONCLUSION: Percutaneous ultrasound-guided day-case aspiration of mucin for advanced and recurrent PMP using a wide-bore drain is a safe and effective procedure. It may be used in the palliative setting or as a bridge to surgery in the very symptomatic patient or if there is a reversible contraindication to surgery.

PMID:36842914 | DOI:10.1016/j.crad.2023.01.016
Surgical Options for Peritoneal Surface Metastases from Digestive Malignancies-A Comprehensive Review

Fetched: February 26th, 2023, 5:01am GMT by Mihai Adrian Eftimie

Medicina (Kaunas). 2023 Jan 28;59(2):255. doi: 10.3390/medicina59020255.

ABSTRACT

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.

PMID:36837456 | PMC:PMC9960111 | DOI:10.3390/medicina59020255
Prognostic Factors in Pseudomyxoma Peritonei with Emphasis on the Predictive Role of Peritoneal Cancer Index and Tumor Markers

Fetched: February 26th, 2023, 5:01am GMT by Sebastian Blaj

Cancers (Basel). 2023 Feb 19;15(4):1326. doi: 10.3390/cancers15041326.

ABSTRACT

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 (p = 0.0353 and p = 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.

PMID:36831667 | PMC:PMC9954733 | DOI:10.3390/cancers15041326
10 Years of Pressurized Intraperitoneal Aerosol Chemotherapy (PIPAC): A Systematic Review and Meta-Analysis

Fetched: February 26th, 2023, 5:01am GMT by Andrea Di Giorgio

Cancers (Basel). 2023 Feb 9;15(4):1125. doi: 10.3390/cancers15041125.

ABSTRACT

BACKGROUND: Pressurized intraperitoneal aerosol chemotherapy (PIPAC) is a novel intraperitoneal drug delivery method of low-dose chemotherapy as a pressurized aerosol in patients affected by peritoneal cancer of primary or secondary origin. We performed a systematic review and meta-analysis with the aim of assessing the feasibility, safety, and efficacy of PIPAC.

METHODS: A systematic literature search was performed using Medline and Web of Science databases from 1 January 2011, to inception, to 31 December 2021. Data were independently extracted by two authors. The Newcastle-Ottawa Scale was used to assess the quality and risk of bias of studies. Meta-analysis was performed for pathological response, radiological response, PCI variation along treatment, and for patients undergoing three or more PIPAC. Pooled analyses were performed using the Freeman-Tukey double arcsine transformation, and 95% CIs were calculated using Clopper-Pearson exact CIs in all instances.

RESULTS: A total of 414 papers on PIPAC were identified, and 53 studies considering 4719 PIPAC procedure in 1990 patients were included for analysis. The non-access rate or inability to perform PIPAC pooled rate was 4% of the procedures performed. The overall proportion of patients who completed 3 or more cycles of PIPAC was 39%. Severe toxicities considering CTCAE 3-4 were 4% (0% to 38.5%). In total, 50 studies evaluated deaths within the first 30 postoperative days. In the included 1936 patients were registered 26 deaths (1.3%). The pooled analysis of all the studies reporting a pathological response was 68% (95% CI 0.61-0.73), with an acceptable heterogeneity (I² 28.41%, p = 0.09). In total, 10 papers reported data regarding the radiological response, with high heterogeneity and a weighted means of 15% (0% to 77.8%). PCI variation along PIPAC cycles were reported in 14 studies. PCI diminished, increased, or remained stable in eight, one and five studies, respectively, with high heterogeneity at pooled analysis. Regarding survival, there was high heterogeneity. The 12-month estimated survival from first PIPAC for colorectal cancer, gastric cancer, gynecological cancer and hepatobiliary/pancreatic cancer were, respectively, 53%, 25%, 59% and 37%.

CONCLUSIONS: PIPAC may be a useful treatment option for selected patients with PM, with acceptable grade 3 and 4 toxicity and promising survival benefit. Meta-analysis showed high heterogeneity of data among up-to-date available studies. In a subset analysis per primary tumor origin, pathological tumor regression was documented in 68% of the studies with acceptable heterogeneity. Pathological regression seems, therefore, a reliable outcome for PIPAC activity and a potential surrogate endpoint of treatment response. We recommend uniform selection criteria for patients entering a PIPAC program and highlight the urgent need to standardize items for PIPAC reports and datasets.

PMID:36831468 | PMC:PMC9954579 | DOI:10.3390/cancers15041125
Palliation of mucinous appendiceal neoplasms with peritoneal metastases with total abdominal colectomy

Fetched: February 25th, 2023, 5:02am GMT by Paul H Sugarbaker

J Surg Oncol. 2023 May;127(6):1019-1027. doi: 10.1002/jso.27220. Epub 2023 Feb 24.

ABSTRACT

BACKGROUND: Incomplete cytoreduction for mucinous appendiceal neoplasms is often required to temporarily alleviate symptoms. The surgical technology for this intervention may be complex and result in extensive morbidity and even mortality. Knowledgeable patient selection for this surgery is mandatory.

METHODS: A database was used to identify patients who had palliative surgery that included total abdominal colectomy (TAC). Clinical- and treatment-related variables were assessed for their impact on overall survival.

RESULTS: Seventy-eight patients had an incomplete cytoreductive surgery (CRS) that included a TAC. The median survival was 2.5 years and the median follow-up was 2 years. Patients with symptoms of abdominal distention and ascites showed a reduced prognosis (p = 0.0254). The low-grade appendiceal mucinous neoplasms (LAMN) and mucinous appendiceal adenocarcinoma intermediate type (MACA-Int) grouped together showed a prolonged survival (p = 0.0003). MACA with positive lymph nodes showed a reduced survival (p = 0.0009) when compared to MACA patients without positive lymph nodes. A peritoneal cancer index of 1-30 versus >30 and completeness of cytoreduction score of 2 versus 3 were not significant.

CONCLUSION: TAC with end ileostomy was used as a treatment option to provide palliation of patients having an incomplete CRS for appendiceal mucinous neoplasms. With LAMN or MACA-Int, median survival was 5.0 years. The surgery can be performed with a morbidity of 9.0% and mortality of 2.6%. Although not considered a palliative option in the past, these data suggest TAC may be used with acceptable results in this group of patients.

PMID:36825427 | DOI:10.1002/jso.27220
Appendiceal tumors and pseudomyxoma peritonei: current recommendations for clinical practice

Fetched: February 23rd, 2023, 5:02am GMT by D Klos

Rozhl Chir. 2023 Winter;102(1):5-10. doi: 10.33699/PIS.2023.102.1.5-10.

ABSTRACT

Appendiceal tumors and pseudomyxoma peritonei (PMP) are rare tumors. Perforated epithelial tumors of the appendix are the most common source of PMP. This disease is characterized by the presence of mucin of varying degrees of consistency, partially adherent to the surfaces. Appendiceal mucoceles themselves are also very rare and usually their treatment involves only a simple appendectomy. The aim of this study was to provide an up-to-date review of the recommendations for the diagnosis and treatment of these malignancies according to the current guidelines of The Peritoneal Surface Oncology Group International (PSOGI) and the Blue Book of the Czech Society for Oncology of the Czech Medical Association of J. E. Purkyně (ČOS ČLS JEP).

PMID:36809888 | DOI:10.33699/PIS.2023.102.1.5-10
Total abdominal colectomy to facilitate complete cytoreduction in 56 patients with mucinous appendiceal neoplasms with peritoneal metastases

Fetched: February 15th, 2023, 5:02am GMT by Paul H Sugarbaker

J Surg Oncol. 2023 May;127(6):1011-1018. doi: 10.1002/jso.27211. Epub 2023 Feb 13.

ABSTRACT

BACKGROUND: Cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy are currently the standard of care for management of appendiceal mucinous neoplasms with peritoneal metastases. The goal of the CRS is complete removal of all visible disease through the use of peritonectomy procedures and visceral resections. One of the major resections that may be required is total abdominal colectomy (TAC).

METHODS: From a database and secured files of patients having a complete CRS, all patients who had TAC were identified. The clinical and histologic variables associated with these patients were identified and assessed for their impact on overall survival.

RESULTS: The 450 complete CRS with low-grade appendiceal mucinous neoplasms had 26 TAC (5.8%) with a 16.0-year median survival. The mucinous adenocarcinoma (MACA)-Intermediate (MACA-Int) group consisted of 37 patients with 8 patients (21.6%) having TAC that resulted in a median survival of 11.5 years. The 159 complete CRS with MACA had 22 TAC (13.8%) with a median survival of 7.5 years. There was a single mortality with a class 4 adverse event in 5 patients (10.7%). With a class 4 adverse event, survival decreased significantly (p = 0.0006, hazard ratio: 6.2).

CONCLUSION: Complete CRS required TAC in 56 of 646 patients (8.7%) with appendiceal mucinous neoplasms. With TAC, median survival was 12.0 years. A class 4 adverse event markedly reduced survival.

PMID:36785941 | DOI:10.1002/jso.27211
Intraoperative oxygen tension and redox homeostasis in Pseudomyxoma peritonei: A short case series

Fetched: February 14th, 2023, 5:02am GMT by Francisca Valenzuela-Molina

Front Oncol. 2023 Jan 26;13:1076500. doi: 10.3389/fonc.2023.1076500. eCollection 2023.

ABSTRACT

INTRODUCTION: Pseudomyxoma peritonei (PMP) is a rare malignant disease characterized by a massive multifocal accumulation of mucin within the peritoneal cavity. The current treatment option is based on complete cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy. However, the recurrence is frequent with subsequent progression and death. To date, most of the studies published in PMP are related to histological and genomic analyses. Thus, the need for further studies unveiling the underlying PMP molecular mechanisms is urgent. In this regard, hypoxia and oxidative stress have been extensively related to tumoral pathologies, although their contribution to PMP has not been elucidated.

METHODS: In this manuscript, we have evaluated, for the first time, the intratumoral real-time oxygen microtension (pO2mt) in the tumor (soft and hard mucin) and surrounding healthy tissue from five PMP patients during surgery. In addition, we measured hypoxia (Hypoxia Inducible Factor-1a; HIF-1α) and oxidative stress (catalase; CAT) markers in soft and hard mucin from the same five PMP patient samples and in five control samples.

RESULTS: The results showed low intratumoral oxygen levels, which were associated with increased HIF-1α protein levels, suggesting the presence of a hypoxic environment in these tumors. We also found a significant reduction in CAT activity levels in soft and hard mucin compared with healthy tissue samples.

DISCUSSION: In conclusion, our study provides the first evidence of low intratumoral oxygen levels in PMP patients associated with hypoxia and oxidative stress markers. However, further investigation is required to understand the potential role of oxidative stress in PMP in order to find new therapeutic strategies.

PMID:36776312 | PMC:PMC9909963 | DOI:10.3389/fonc.2023.1076500
Diagnostic and Therapeutic Algorithm for Appendiceal Tumors and Pseudomyxoma Peritonei: A Consensus of the Peritoneal Malignancies Oncoteam of the Italian Society of Surgical Oncology (SICO)

Fetched: February 12th, 2023, 5:02am GMT by Marco Vaira

Cancers (Basel). 2023 Jan 24;15(3):728. doi: 10.3390/cancers15030728.

ABSTRACT

Aim: Pseudomyxoma peritonei (PMP) is an uncommon pathology, and its rarity causes a lack of scientific evidence, precluding the design of a prospective trial. A diagnostic and therapeutic algorithm (DTA) is necessary in order to standardize the disease treatment while balancing optimal patient management and the correct use of resources. The Consensus of the Italian Society of Surgical Oncology (SICO) Oncoteam aims at defining a diagnostic and therapeutic pathway for PMP and appendiceal primary tumors applicable in Italian healthcare. Method: The consensus panel included 10 delegated representatives of oncological referral centers for Peritoneal Surface Malignancies (PSM) affiliated to the SICO PSM Oncoteam. A list of statements regarding the DTA of patients with PMP was prepared according to recommendations based on the review of the literature and expert opinion. Results: A consensus was obtained on 33 of the 34 statements linked to the DTA; two flowcharts regarding the management of primary appendiceal cancer and peritoneal disease were approved. Conclusion: Currently, consensus has been reached on pathological classification, preoperative evaluation, cytoreductive surgery technical detail, and systemic treatment; some controversies still exist regarding the exclusion criteria for HIPEC treatment. A shared Italian model of DTA is an essential tool to ensure the appropriateness and equity of treatment for these patients.

PMID:36765686 | PMC:PMC9913318 | DOI:10.3390/cancers15030728
Repeated Cytoreduction Combined with Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in Selected Patients Affected by Peritoneal Metastases: Italian PSM Oncoteam Evidence

Fetched: February 12th, 2023, 5:02am GMT by Enrico Maria Pasqual

Cancers (Basel). 2023 Jan 18;15(3):607. doi: 10.3390/cancers15030607.

ABSTRACT

The reiteration of surgical cytoreduction (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in patients affected by recurrent peritoneal metastases is still questioned regarding safety and effectiveness. This study evaluates the safety, efficacy, and associated factors of iterative CRS combined with HIPEC. This multicentric retrospective study collected data from four surgical oncology centers, on iterative HIPEC. We gathered data on patient and cancer characteristics, the peritoneal cancer index (PCI), completeness of cytoreduction (CC), postoperative complications, and overall survival (OS). In the study period, 141 CRS-plus-HIPECs were performed on 65 patients. Nine patients underwent three iterative procedures, and one underwent five. No increased incidence of complications after the second or third procedure was observed. Furthermore, operative time and hospitalization stay were significantly shorter after the second than after the first procedure (p < 0.05). Optimal cytoreduction was achieved in more than 90% of cases in each procedure, whether first, second, or third. A five-year (5 y) OS represented 100% of the cases of diffuse malignant-peritoneal-mesotheliomas, 81.39% of pseudomyxoma peritonei, 34.67% of colorectal cancer (CRC), and 52.50% of ovarian cancer. During the second CRS combined with HIPEC, we observed a lower rate of complete cytoreduction and a non-significantly better survival in cases with complete cytoreduction (5 y-OS CC-0 56.51% vs. 37.82%, p = 0.061). Concomitant hepatic-CRC-metastasis did not compromise the CRS-plus-HIPEC safety and efficacy. This multicentric experience encourages repeated CRS-plus-HIPEC, showing promising results.

PMID:36765565 | PMC:PMC9913411 | DOI:10.3390/cancers15030607
Rare Mucinous Adenocarcinoma of the Appendix Undergoing Multiple Recurrent Surgical Interventions

Fetched: February 7th, 2023, 5:02am GMT by Usman Ilyas

Cureus. 2023 Jan 3;15(1):e33294. doi: 10.7759/cureus.33294. eCollection 2023 Jan.

ABSTRACT

Primary appendiceal carcinomas are rare and often found incidentally when the appendix is surgically removed. Adenocarcinoma predominates the histological types of malignancies, with mucinous adenocarcinoma being the most prevalent of the various subtypes. Pseudomyxoma peritonei (PMP), a complication seen in mucinous adenocarcinoma of the appendix (MAA), is the collection of mucinous ascites in the intra-abdominal cavity and the thickening of the surrounding viscera by mucin-producing tumor cells. PMP initially presents with increased abdominal discomfort and girth and, in later stages, presents with obstructive abdomen symptoms. These symptoms are nonspecific and can be a challenge to pinpoint. Such was the case for our patient, in this case report, who initially presented with dyspepsia and later demonstrated compressive symptoms and weight loss, raising concern for malignancy. An appendiceal pathology was of concern when his right lower quadrant pain acutely worsened during an abdominal ultrasound, and imaging and biopsy confirmed MAA with PMP. The aim of this report is to shed light on the management of recurrent MAA. Our patient's recurrent MAA was managed with debulking procedures and three rounds of hyperthermic intraperitoneal chemotherapy (HIPEC) and was managed postoperatively with folinic acid, fluorouracil, and irinotecan (FOLFIRI) and bevacizumab, which in its totality helped achieve a progression-free survival of more than two years. We believe that cytoreduction and intraoperative chemotherapy prolong survival in patients with recurrent disease, as was the case with our patients. Our patient also demonstrated benefit as his disease stabilized after starting bevacizumab; however, more studies need to be performed at a larger scale to show a consistent relationship.

PMID:36741631 | PMC:PMC9894632 | DOI:10.7759/cureus.33294

Trial - Pseudomyxoma Peritonei

Intestinal & Multivisceral Transplantation for Unresectable Mucinous Carcinoma Peritonei (TRANSCAPE)

Posted: October 16th, 2023, 2:00am GMT

Conditions: Secondary Malignant Neoplasm of Retroperitoneum; Secondary Malignant Neoplasm of Peritoneum; Pseudomyxoma Peritonei
Interventions: Procedure: Intestinal, Multivisceral or Modified Multivisceral Transplantation; Drug: Alemtuzumab; Drug: Tacrolimus; Drug: Sirolimus
Sponsors: Case Comprehensive Cancer Center
Not yet recruiting
Effect of Urine-guided Hydration on Acute Kidney Injury After CRS-HIPEC

Posted: July 11th, 2023, 2:00pm GMT

Conditions: Cytoreductive Surgery; Hyperthermic Intraperitoneal Chemotherapy; Hydration; Diuresis; Acute Kidney Injury; Postoperative Complications
Interventions: Procedure: Urine-guided hydration; Procedure: Routine hydration; Drug: Forced administration of furosemide; Drug: Routine administration of furosemide
Sponsors: Peking University First Hospital; Aerospace Center Hospital
Recruiting
Gastrointestinal Stimulation as a Treatment of Postoperative Ileus Following Extensive Surgery

Posted: March 2nd, 2023, 3:00pm GMT

Conditions: Postoperative Ileus; Bowel Paralysis
Intervention: Device: Gastric electric pacemaker
Sponsors: University of Aarhus; Danish Cancer Society
Recruiting
Flat Dose vs. Weight-based IP Chemotherapy for CRS/HIPEC

Posted: March 3rd, 2021, 3:00pm GMT

Condition: Peritoneal Carcinomatosis
Interventions: Drug: Mitomycin C, flat dose 40 mg; Drug: Mitomycin C, weight-based dose 12.5 mg/m2
Sponsor: Prakash Pandalai
Recruiting
A Study of SGN-STNV in Advanced Solid Tumors

Posted: December 14th, 2020, 3:00pm GMT

Conditions: Carcinoma, Non-Small Cell Lung; HER2 Negative Breast Neoplasms; Ovarian Neoplasms; Uterine Cervical Neoplasms; Endometrial Neoplasms; Esophageal Neoplasms; Gastroesophageal Junction Carcinoma; Stomach Neoplasms; Colorectal Neoplasms; Exocrine Pancreatic Adenocarcinoma; Appendiceal Adenocarcinoma; Pseudomyxoma Peritonei
Interventions: Drug: SGN-STNV
Sponsors: Seagen Inc.
Active, not recruiting
Impact of Cardiac Coherence on Anxiety in Patients Operated on for a Peritoneal Carcinosis

Posted: July 18th, 2019, 2:00am GMT

Conditions: Peritoneal Carcinomatosis; Pseudomyxoma Peritonei; Mesothelioma Peritoneum
Interventions: Other: Cardiac coherence; Other: Standard care
Sponsors: Institut du Cancer de Montpellier - Val d'Aurelle
Recruiting
Gastrointestinal Microbiome Study of Appendiceal Cancer

Posted: November 6th, 2015, 3:00pm GMT

Conditions: Pseudomyxoma Peritonei; Appendiceal Neoplasm; Cancer, Appendiceal
Interventions: Other: Bio-specimen collection; Other: Questionnaire
Sponsors: Mercy Medical Center; University of California, San Diego
Recruiting
Crossover Trial of Systemic Chemotherapy in Patients With Metastatic Well-Differentiated Mucinous Appendiceal Adenocarcinomas With Pseudomyxoma Peritonei

Posted: September 20th, 2013, 2:00am GMT

Conditions: Gastrointestinal Neoplasms
Interventions: Behavioral: Questionnaire
Sponsors: M.D. Anderson Cancer Center
Active, not recruiting
Register With Patients in Which Hyperthermic Intra-Peritoneal Chemotherapy (HIPEC) Was Performed

Posted: June 12th, 2012, 2:00pm GMT

Conditions: Peritoneal Carcinomatosis; Pseudomyxoma Peritonei; Peritoneal Mesothelioma
Intervention:
Sponsor: Universitaire Ziekenhuizen KU Leuven
Recruiting

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