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Frontiers in Oncology 2023The role of cytoreductive surgery combined with hyperthermic intrathoracic chemotherapy (CRS+HITOC) for patients with secondary pleural metastases has scarcely been...
BACKGROUND
The role of cytoreductive surgery combined with hyperthermic intrathoracic chemotherapy (CRS+HITOC) for patients with secondary pleural metastases has scarcely been investigated.
PATIENTS AND METHODS
We conducted a retrospective, multicentre study investigating the outcome of CRS+HITOC for 31 patients with pleural metastases from different primary tumours in four high-volume departments of thoracic surgery in Germany. The primary endpoint was overall survival (OS). Secondary endpoints included postoperative complications and recurrence/progression-free survival (RFS/PFS).
RESULTS
The primary tumour was non-small cell lung cancer in 12 (39%), ovarian cancer in 5 (16%), sarcoma in 3 (10%), pseudomyxoma peritonei in 3 (10%), and others in 8 (26%) patients. A macroscopic complete resection (R/1) could be achieved in 28 (90%) patients. Major postoperative complications as classified by Clavien-Dindo (III-V) were observed in 11 (35%) patients. The postoperative mortality rate was 10% (n=3). A total of 13 patients received additive chemotherapy (42%). The median time of follow up was 30 months (95% CI = 17- 43). The median OS was 39 months (95% CI: 34-44 months) with 1-month, 3-month, 1-, 3-, and 5-year survival estimates of 97%, 89%, 77%, 66%, and 41%. There was a significantly prolonged OS in patients who received additive chemotherapy compared to patients with only CRS+HITOC (median OS 69 38 months; p= 0.048). The median RFS was 14 months (95% CI: 7-21 months).
CONCLUSIONS
We observed that CRS+HITOC is a feasible approach with reasonable complications and prolonged survival as a part of multimodal concept for highly selected patients with secondary pleural metastases.
PubMed: 38090507
DOI: 10.3389/fonc.2023.1259779 -
Case Reports in Oncology 2023Pseudomyxoma peritonei is a rare peritoneal malignancy characterized by the progressive accumulation of mucinous material and tumour within the abdomen and pelvis....
Pseudomyxoma peritonei is a rare peritoneal malignancy characterized by the progressive accumulation of mucinous material and tumour within the abdomen and pelvis. Percutaneous drainage of mucin may be a non-surgical option for relief of symptoms; however, it remains difficult due to the high viscosity of mucin, with numerous case reports reporting difficulty removing material through medium-bore catheters alone. BromAc is a therapy currently undergoing development which dissolves mucinous tumour masses and allows for extraction. This report describes the case of a patient who has had multiple treatments with BromAc over 4 years.
PubMed: 38074518
DOI: 10.1159/000534202 -
Experimental Hematology & Oncology Dec 2023Pseudomyxoma peritonei (PMP) is a rare disease characterized by a massive accumulation of mucus in the peritoneal cavity. The only effective treatment is aggressive...
Pseudomyxoma peritonei (PMP) is a rare disease characterized by a massive accumulation of mucus in the peritoneal cavity. The only effective treatment is aggressive surgery, aimed at removing all visible tumors. However, a high percentage of patients relapse, with subsequent progression and death. Recently, there has been an increase in therapies that target mutated oncogenic proteins. In this sense, KRAS has been reported to be highly mutated in PMP, with KRAS being the most common subtype. Here, we tested the efficacy of a small-molecule KRAS inhibitor, MRTX1133, in a high-grade PMP xenograft mouse model carrying a KRAS mutation. The results obtained in this work showed a profound inhibition of tumor growth, which was associated with a reduction in cell proliferation, an increase in apoptosis, and a reduction in the MAPK and PI3K/AKT/mTOR signaling pathways. In conclusion, these results demonstrate the high potency and efficacy of MRTX1133 in KRAS-PMP tumors and provide a rationale for clinical trials.
PubMed: 38066554
DOI: 10.1186/s40164-023-00465-4 -
Scientific Reports Dec 2023Peritoneal cancer index (PCI) is the surgical variable most commonly used to quantify the extent of peritoneal metastases for pseudomyxoma peritonei (PMP) patients. The...
Peritoneal cancer index (PCI) is the surgical variable most commonly used to quantify the extent of peritoneal metastases for pseudomyxoma peritonei (PMP) patients. The present study aimed to investigate the agreement between CT predicted and surgical PCI by the Bland-Altman method for PMP of appendiceal origin. A total of 167 PMP patients of appendiceal origin were included between 2016 and 2021. Bland-Altman analysis was performed for both total PCI and selected PCI (regions 2 + 9-12). After the Bland-Altman plot was drawn, the mean bias and its 95% limit of agreements (LoAs) was quantified. Besides, the correlation coefficients between CT-PCI and surgical PCI were also been calculated. The Bland-Altman plot showed the mean bias ± SD between total CT-PCI and surgical PCI as 0.431 ± 3.005, with the LoAs from - 5.459 to 6.321. There were nine points of difference in total PCI exceeded the 95% LoAs, with the rate of 5.39% (9/167). As for selected CT-PCI, Bland-Altman plot showed the mean bias ± SD between selected CT-PCI and surgical PCI as - 0.287 ± 1.955, with the LoAs from - 4.118 to 3.544. There were ten points of difference in selected PCI exceeded the 95% LoAs, with the rate of 5.99% (10/167). The Spearman's rank correlation coefficient between total CT-PCI and surgical PCI was 0.911, P < 0.001, as for selected CT-PCI and surgical PCI, the coefficient was 0.909, P < 0.001. Although there was a strong correlation for both total and selected CT-PCI with surgical PCI, however, the agreement is still not good in Bland-Altman analysis, which suggested that CT-PCI cannot predict surgical PCI accurately even in professional PMP treatment centers. In brief explanation, CT makes it difficult to distinguish the borderline between tumor tissue and mucus and to detect tumor lesions in the small intestine regions, which caused overestimation or underestimation by CT-PCI. In the future, a multiple linear regression model based on CT-PCI might accurately predict surgical PCI preoperatively.
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Appendix; Peritoneum; Tomography, X-Ray Computed; Retrospective Studies
PubMed: 38057378
DOI: 10.1038/s41598-023-48975-9 -
Current Oncology (Toronto, Ont.) Nov 2023Patients with low-grade appendiceal mucinous carcinomas (LAMNs) treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have a... (Review)
Review
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.
Topics: Humans; Male; Appendiceal Neoplasms; Combined Modality Therapy; Hyperthermia, Induced; Neoplasm Recurrence, Local; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 37999146
DOI: 10.3390/curroncol30110726 -
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