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ANZ Journal of Surgery Apr 2024Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have improved survival for selected cases of peritoneal surface malignancy. In 2008, a...
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.
Topics: Female; Humans; Peritoneal Neoplasms; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; New Zealand; Hyperthermia, Induced; Pseudomyxoma Peritonei; Appendiceal Neoplasms; Adenocarcinoma, Mucinous; Colorectal Neoplasms; Retrospective Studies; Survival Rate; Combined Modality Therapy
PubMed: 37994292
DOI: 10.1111/ans.18777 -
Clinical Journal of Gastroenterology Feb 2024Pseudomyxoma peritonei (PMP) of pancreatic origin arising from an intraductal papillary mucinous neoplasm (IPMN) is rare. Cytoreductive surgery (CRS) with hyperthermic...
A case of pseudomyxoma peritonei arising from a perforated intraductal papillary mucinous neoplasm that underwent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.
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.
Topics: Female; Humans; Aged; Pseudomyxoma Peritonei; Hyperthermic Intraperitoneal Chemotherapy; Peritoneal Neoplasms; Ascites; Cytoreduction Surgical Procedures; Hyperthermia, Induced; Pancreatic Neoplasms; Retrospective Studies
PubMed: 37980306
DOI: 10.1007/s12328-023-01890-y -
Surgical Oncology Dec 2023There are no previous studies on pseudomyxoma peritonei regarding the details of surgical procedures included in cytoreductive surgery and quantitative evaluation for...
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.
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Peritoneum; Gastrectomy; Colectomy; Retrospective Studies; Appendiceal Neoplasms; Cytoreduction Surgical Procedures; Hyperthermia, Induced; Combined Modality Therapy
PubMed: 37972508
DOI: 10.1016/j.suronc.2023.102012 -
Experimental and Therapeutic Medicine Dec 2023Mucinous cystic tumors of low malignant potential (MCTLMP) are rare urachal neoplasms. The morphological characteristics and clinical prognosis of MCTLMP is similar to...
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.
PubMed: 37941591
DOI: 10.3892/etm.2023.12254 -
World Journal of Surgical Oncology Oct 2023As the standard treatment for pseudomyxoma peritonei (PMP), cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) can significantly prolong...
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.
Topics: Humans; Pseudomyxoma Peritonei; Retrospective Studies; Peritoneal Neoplasms; Combined Modality Therapy; Hyperthermia, Induced; Cytoreduction Surgical Procedures; China; Survival Rate
PubMed: 37891655
DOI: 10.1186/s12957-023-03232-1 -
Journal of Surgical Case Reports Oct 2023High-grade appendiceal mucinous neoplasms (HAMN) are rare mucinous appendiceal tumors that are mostly incidentally discovered and histologically show marked cytoplasmic...
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 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.
PubMed: 37854517
DOI: 10.1093/jscr/rjad567 -
Journal of Minimal Access Surgery Sep 2023Low-grade appendiceal mucinous neoplasm (LAMN) is a rare disease, and its clinical course varies from an incidental finding without symptoms to pseudomyxoma peritonei....
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.
PubMed: 37843158
DOI: 10.4103/jmas.jmas_113_23 -
Scandinavian Journal of Surgery : SJS :... Jun 2024Pseudomyxoma peritonei (PMP) is a rare disease characterized by progressive build-up of mucinous deposits inside the abdominal cavity. The aim of this study was to...
BACKGROUND AND AIMS
Pseudomyxoma peritonei (PMP) is a rare disease characterized by progressive build-up of mucinous deposits inside the abdominal cavity. The aim of this study was to investigate the effect of disease recurrence on overall survival in patients with PMP after cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC).
METHODS
One-hundred thirty-two consecutive PMP patients treated with CRS + HIPEC at Helsinki University Hospital between 2008 and 2017 were included. The impact of clinicopathological and treatment-related characteristics on recurrence and overall survival was evaluated.
RESULTS
The median follow-up time in the study was 5.04 (range = 0.05-11.60) years. In 121 (91.7%) patients, the disease was classified as low grade and 11 (8.3%) had high-grade disease. In the low-grade group, 26 (21.5%) patients developed a recurrence during follow-up compared to 6 (54.5%) patients in the high-grade group. In the low-grade group, cumulative survival was 98.2%, 91.4%, and 91.4% at 3, 6, and 8 years, respectively. In the high-grade group, cumulative survival was 90.0% and 78.8% at 3 and 6 years, respectively. In patients with recurrent disease, the cumulative survival was 100%, 84.6%, and 84.6% at 3, 6, and 8 years in the low-grade category and 80.0% and 60.0% at 3 and 6 years in the high-grade category, respectively. In the low-grade group, a statistically significant correlation with recurrence but not with overall survival was identified with peritoneal cancer index (PCI), carcinoembryonic antigen (CEA), and the number of affected regions.
CONCLUSION
The recurrence of low-grade PMP does not significantly affect overall survival of patients. Disease extent may not be a prognostic indicator after curative CRS and HIPEC in low-grade PMP.
Topics: Humans; Pseudomyxoma Peritonei; Cytoreduction Surgical Procedures; Female; Male; Middle Aged; Retrospective Studies; Peritoneal Neoplasms; Hyperthermic Intraperitoneal Chemotherapy; Aged; Neoplasm Recurrence, Local; Adult; Combined Modality Therapy; Survival Rate; Finland
PubMed: 37828760
DOI: 10.1177/14574969231200653 -
Cureus Oct 2023Low-grade appendiceal mucinous neoplasm (LAMN) is an uncommon tumor of the appendix that is usually diagnosed incidentally after surgery. Although LAMN may be...
BACKGROUND
Low-grade appendiceal mucinous neoplasm (LAMN) is an uncommon tumor of the appendix that is usually diagnosed incidentally after surgery. Although LAMN may be asymptomatic, it can rupture and seed mucin and neoplastic epithelium into the peritoneum, causing pseudomyxoma peritonei (PMP).
MATERIALS AND METHODS
Data from 53 patients were retrospectively analyzed. Age, sex, tumor size, margin status, peritoneal carcinomatosis index, surgical procedures, postoperative results with histologic diagnosis, T stage, recurrence, and mortality of the patients were evaluated.
RESULTS
Appendectomy was performed in 37 patients, right hemicolectomy in nine patients, cytoreductive surgery in one patient, and cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in six patients. Recurrence occurred in four patients. Of the patients who developed recurrence, one patient had stage T4a disease, and the other three patients had T4b disease (p<0.001). Eighteen patients had acellular mucin in the serosa and four of these patients developed recurrence (p=0.004). Twelve patients had appendix perforation, and three of these patients had PMP on exploration (p<0.001). The mean survival time was 93.3 months in patients without recurrence and 32 months in patients with recurrence (p=0.021).
CONCLUSIONS
Low-grade appendiceal mucinous neoplasms are rare appendiceal tumors. The appropriate management of this tumor is controversial. T stage, appendix perforation, presence of acellular mucin on the serosa, and surgical margins are risk factors for the development of PMP. Appendectomy is considered sufficient when there are no risk factors for Tis (LAMN) and T3 disease. Right hemicolectomy may be sufficient if there are no risk factors for T4a disease, but cytoreductive surgery and hyperthermic intraperitoneal chemotherapy seem to be the most appropriate treatments in the presence of the stated risk factors for T4b disease.
PubMed: 37808597
DOI: 10.7759/cureus.46591 -
Anticancer Research Oct 2023Pseudomyxoma peritonei (PMP) is a rare clinical condition of progressive peritoneal mucin accumulation. PMP has a reasonable survivability but with a notable risk of...
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.
PubMed: 37772592
DOI: 10.21873/anticanres.16670