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Indian Journal of Surgical Oncology Jun 2023To 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,...
UNLABELLED
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.
PubMed: 37359937
DOI: 10.1007/s13193-022-01694-5 -
Indian Journal of Surgical Oncology Jun 2023Pleural spread occurs in pseudomyxoma peritonei (PMP) in less than 10% of the patients and is treated by thoracic cytoreductive surgery with or without hyperthermic...
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.
PubMed: 37359933
DOI: 10.1007/s13193-023-01745-5 -
Indian Journal of Surgical Oncology Jun 2023A previous report from the Indian HIPEC registry showed acceptable early survival and morbidity in patients undergoing cytoreductive surgery...
UNLABELLED
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 ( = 0.03) and PMP of appendiceal origin ( = 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.
PubMed: 37359912
DOI: 10.1007/s13193-023-01727-7 -
Pleura and Peritoneum Jun 2023The Peritoneal Surface Oncology Group International (PSOGI) consensus subdivided pseudomyxoma peritonei (PMP) into four groups according to histopathological features....
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.
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.
PubMed: 37304162
DOI: 10.1515/pp-2023-0001 -
Pleura and Peritoneum Jun 2023Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) has enabled better prognosis for patients with peritoneal surface malignancies. However,...
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.
PubMed: 37304160
DOI: 10.1515/pp-2022-0202 -
Journal of Clinical Medicine May 2023Treatment of Peritoneal Surface Malignancies (PSM) with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) has achieved results never seen...
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).
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.
PubMed: 37297969
DOI: 10.3390/jcm12113774 -
Journal of Surgical Case Reports May 2023Appendiceal collision tumours are extremely rare, with most reported cases describing tumours consisting of a mucinous component and a neuroendocrine component....
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.
PubMed: 37251256
DOI: 10.1093/jscr/rjad281 -
American Journal of Cancer Research 2023Mucins are a significant extracellular component of neoplastic entities such as pseudomyxoma peritonei and several gastrointestinal adenocarcinomas. Mucinous tumours... (Review)
Review
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 and 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 and , and clinical evidence. The implications of current data and directions for future research are then discussed.
PubMed: 37168359
DOI: No ID Found -
Journal of Surgical Case Reports May 2023We 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...
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.
PubMed: 37153824
DOI: 10.1093/jscr/rjad234 -
Case Reports in Oncology 2023A 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...
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.
PubMed: 37123607
DOI: 10.1159/000529672