Cytoreductive Surgery In Combination Research Articles

THE ROLE OF SURGICAL STAGE IN COMBINATION THERAPY FOR PATIENTS WITH LOCALLY ADVANCED CERVICAL CANCER

Objective: to justify the expediency of the surgical stage as a part of the combination treatment for stage IIA-IIIB cervical cancer. Materials and methods. The study included 343 women with stage IIA-IIIB cervical cancer treated from 2013 to 2016 with mandatory follow-up for at least 2 years. Patients were divided into 2 groups. The first group included 214 patients who received a combination treatment. At the first stage, neoadjuvant chemoradiation therapy was performed (remote radiation therapy 5 days a week with radio modification with Cisplatin once a week at a dose of 40 mg/m2). After evaluating the effect, patients were subjected to surgical treatment or continued chemoradiotherapy. The second group (n = 129) received standard combined radiation therapy. Various schemes of combination and complex treatment and standard combined radiation therapy were evaluated using the indices of general and relapse-free survival. Results. The proposed scheme for the combination therapy for patients with locally advanced cervical cancer showed significantly higher survival rates at all the analyzed stages. For the combined treatment group with complete cytoreduction, the two-year overall and relapse-free survival with stage IIA is 94.1% vs. 82.4%, with IIB 90.8% vs. 80.3%, with IIB 87.5% vs. 75%, with IIB with metastatic lesion of regional lymph nodes 85% vs. 70%. For the second group, two-year overall and relapse-free survival with stage IIA 75% vs. 50%, with IIB 70.9% vs. 56.3%, with IIB 59.1% vs. 40.9%, with IIB with metastatic lesion of regional lymph nodes 62.2% and 40.5%. The advantages of this approach are most clearly seen within patients with metastatic lesions of regional lymph nodes (85% vs. 62% accordingly). Conclusion. Cytoreductive surgery in combination with the combination therapy allows to achieve a significant increase in overall and relapse-free survival for patients with locally advanced cervical cancer compared with standard treatment programs.

Anti-tumor efficacy of folate modified PLGA-based nanoparticles for the co-delivery of drugs in ovarian cancer.

Background: Ovarian cancer is a leading cause of death in gynecologic malignancies. The high mortality is mainly caused by advanced stage at presentation in most patients. Even after the combination of cytoreductive surgery and systemic platinum and taxane treatment, most patients relapse and eventually succumb to the disease. Therefore, there is an urgent need for new treatments. Purpose: A novel folate (FA)-targeted co-delivery of docetaxel (DTX) and gemcitabine (GEM) nanoparticles (NPs) was developed to overcome ovarian cancer. Materials and methods: Physicochemical characteristics of NPs such as size, morphology, and release profiles were explored. In vitro and in vivo studies were carried out to assess the efficacy of their antitumor activity in target cells. Results: FA modified DTX and GEM co-loaded NPs were prepared using the solvent evaporation method. The NPs with a particle size of ~120nm were stable in the observation period. The hemolysis results indicated that FA-PEG2000-PLGA was potentially feasible for targeted antitumor drug delivery through blood circulation. In vitro release study suggested that in comparison with the free drug, PLGA-DTX/GEM NPs and FA-PEG2000-PLGA-DTX/GEM NPs had sustained-release properties. However, there was no obvious difference between the two NPs with the same drug in the release profile. Ovarian cancer cells in vitro efficiently took up the non-targeted and FA-targeted NPs; improved cytotoxicity was observed in the FA-targeted NPs, showing a 3.59- fold drop in the IC50 in SKOV-3 cells as compared to DTX/GEM alone. Cellular uptake showed that through surface modification, more drugs entered the cell successfully. Pharmacodynamics results showed a statistically significant effect on the rate of reduction of tumor volume for FA-PEG2000-PLGA-DTX/GEM NPs than other groups and no toxicity of organs.Conclusion: The present study indicates that the FA-PEG2000-PLGA-DTX/GEM NPs provides a promising platform for the treatment of ovarian cancer.

Pressurized intraperitoneal aerosol chemotherapy and its effect on gastric-cancer-derived peritoneal metastases: an overview

This manuscript aspires to portray a review of the current literature focusing on manifest peritoneal metastasis (PM) derived from gastric cancer and its treatment options. Despite the development of chemotherapy and multimodal treatment options during the last decades, mortality remains high worldwide. After refreshing important epidemiological considerations, the molecular mechanisms currently accepted through which PM occurs are revised. Palliative chemotherapy is the only recommended treatment option for patients with PM of gastric cancer according to the National Comprehensive Cancer Network guidelines, although cytoreductive surgery in combination with hyperthermic intraperitoneal chemotherapy demonstrated promising results in selected patients with regional PM and localized intraabdominal tumor spread. A novel treatment named pressurized intraperitoneal aerosol chemotherapy may have a promising future in improving overall survival with an acceptable postoperative complication rate and stabilizing quality of life during treatment. Additionally, the procedure has been proved to be safe for the patient and medical personnel and a feasible, repeatable method to deter metastatic proliferation. This overview comprehensively addresses this novel and promising treatment in the context of a scientifically and clinically challenging disease.

Indications and selected aspects of cytoreductive surgery in combination with intra-peritoneal perfusion chemotherapy in hyperthermia (CRS + HIPEC) in the treatment of peritoneal tumors - 4 years of experience of the Lower Silesian Oncology Center (LSOC)

Indications and selected aspects of cytoreductive surgery in combination with intra-peritoneal perfusion chemotherapy in hyperthermia (CRS + HIPEC) in the treatment of peritoneal tumors - 4 years of experience of the Lower Silesian Oncology Center (LSOC)

Combined cytoreductive surgery and hyperthermic intraperitoneal chemotherapy as a treatment for recurrent epithelial ovarian cancer-National Cancer Institute experience

BackgroundComplete cytoreduction has been associated with survival benefit in the treatment of recurrent epithelial ovarian cancer (EOC). In this study, the aim is to investigate the role of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in the treatment of recurrent EOC. Patients and methodsThis is a descriptive (case series) study including 9 patients with recurrent EOC treated by CRS and HIPEC. They were treated and followed up between December 2011 and December 2017. The study was performed at The National Cancer Institute (NCI) – Cairo University (CU). ResultsPostoperative death occurred in 2 cases, while recurrence occurred in one case. Six cases had smooth postoperative course and free follow-up. Median follow-up period was 39 months, ranging from 29 to 47 months. Median overall survival was 42 months while median disease-free survival was not reached. ConclusionsTreatment of recurrent EOC by CRS and HIPEC appears to be promising. However, this line of treatment requires further evaluations and larger studies for better assessment of the potential survival benefits and possible complications.

A combination of electrochemotherapy, gene electrotransfer of plasmid encoding canine IL-12 and cytoreductive surgery in the treatment of canine oral malignant melanoma

The aim of this study was to evaluate the safety and efficacy of the combination of electrochemotherapy (ECT) with bleomycin and gene electrotransfer (GET) of plasmid encoding canine interleukin 12 (IL-12) for the treatment of canine oral malignant melanoma (OMM). Our focus was to determine the effect of the treatment on achieving local tumor control and stimulation of an antitumor immune response. Nine dogs with histologically confirmed OMM stage I to III were included in a prospective, non-randomized study. The dogs were treated with a combination of cytoreductive surgery, ECT and IL-12 GET, which was repeated up to five times, depending on the clinical response to the treatment, evaluated according to the follow-up protocol (7, 14 and 28 days after, the last treatment). One month after treatment, the objective response (OR) rate was 67% (6/9). Median survival time (MST) was 6 months and, even though the disease progressed in 8/9 patients at the end of the observation period (2 to 22 months), four animals were euthanized due to tumor-unrelated reasons. In addition, we observed a decline in the percentage of regulatory T cells (Treg) in the peripheral blood in the course of the treatment, which could be attributed to a systemic antitumor response to IL-12 GET. The results of this study suggest that a combination of ECT and IL-12 GET may be beneficial for dogs with OMM, especially when other treatment approaches are not acceptable due to their invasiveness or cost.

Cytoreductive Surgery and Hyperthermic Chemotherapy Role in Peritoneal Carcinomatosis Treatment

The aim of this article is to provide a review of some current therapeutic strategies for patients with peritoneal carcinomatosis (PC), which historically has been considered a terminal condition, only amenable to palliative care, and occasionally systemic chemotherapy alone with poor response and lower average survival. The combination of cytoreductive surgery (CRS), which involves peritonectomy and multivisceral resections and intraoperative hyperthermic intraperitoneal chemotherapy (HIPEC) is a relatively new intraperitoneal treatment of microscopic residual disease concept. The indication of this method has been extended progressively to highly selected patients with PC and it is based in several phase III studies, which concluded that it is possible to obtain sufficient cytoreduction. However, until now there is some consensus based on evidence, in which HIPEC would be indicated in patients with peritoneal PC originated in pseudomyxoma, peritoneal mesothelioma and colorectal cancer; and CP from another source, depending on each particularcase.

Radiation therapy for superficial inguinal lymph node metastases from ovarian clear cell carcinoma and associated inguinal hernia

About 3% of ovarian cancer (OC) metastases reportedly involve inguinal lymph nodes, but little published data on their radiosensitivity is available. Here, we report a 53-year-old woman with clear cell OC and left inguinal, supra-inguinal and obturator lymph node metastases (LNMs), and a huge, unresectable mass of adherent inguinal nodes. After standard hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and pelvic lymphadenectomy, she underwent various chemotherapy regimens over 12 months, as the inguinal mass enlarged. Radiotherapy was subsequently successful but complicated by an inguinal hernia. Combined cytoreductive surgery, inguinal metastasectomy, and radiotherapy may be appropriate for patients with OC with inguinal LNMs.

Pressurized Intraperitoneal Aerosol Chemotherapy (PIPAC) through a single port: alternative delivery for the control of peritoneal metastases.

Peritoneal carcinomatosis is the natural course of gastrointestinal, gynecologic, and primary peritoneal neoplasms. In recent years, our understanding of carcinomatosis has changed; it is no longer considered a disseminated condition, but rather a disease confined to the peritoneum. Thus, the combination of cytoreductive surgery and intraperitoneal chemotherapy has become the cornerstone of control of peritoneal metastases. Traditionally, intraperitoneal chemotherapy is delivered in the form of liquid solutions. However, a new mode of chemotherapy delivery to the abdominal cavity has arisen as an alternative to the conventional method. In Pressurized Intraperitoneal Aerosol Chemotherapy (PIPAC), the liquid solution is aerosolized into a spray, potentiating the distribution and penetration of the chemotherapeutic agent intraperitoneally. The present study aims to describe a novel form of this innovative surgical technique performed for the first time in Brazil, in a modification of the technique originally described for PIPAC: delivery through a single-port device.

Abstract CT155: Design of a dose escalation phase 1/2 trial for a novel combined cell and oncolytic viral therapy

Abstract Ovarian cancer is the sixth most common malignancy in women and the leading cause of death from all gynecological cancers in the United States with epithelial ovarian cancer are often diagnosed with advanced- stage disease. Although clinically complete remissions are obtained in the majority of patients through a combination of cyto-reductive surgery and chemotherapy, relapse is common with 70% of patients exhibiting recurring disease and thus represents a high unmet need. Single-agent therapies used to treat the subset of patients with chemotherapy- resistant/refractory ovarian cancer include paclitaxel, PLD (peglyated liposomal doxorubicin) and topotecan. The response rate is in the 10-15% range and overall survival is approximately 12 months. Our immunotherapy is comprised of cytokine-induced NK-T cells which stealthily delivers a tumor selective vaccinia virus to the tumor microenvironment, attracted by chemokines released by the tumor and binding to tumor stress ligands. The virus is released in the tumor site and is synchronously released to kill tumor cells, reducing or eradicating the tumor. This therapeutic approach is multi-mechanistic and delivery of the oncolytic agent by the immune effector cells together may result in a durable immune response to ovarian tumor- specific antigens through a cytotoxic T-cell mechanism. The two components, vaccinia virus vvDD-CDSR and CIK cells have been tested and investigated in multiple pre-clinical and clinical studies that have demonstrated their antitumor efficacy and overall safety. In our proposed Open Phase I/II clinical study will be a dose escalation to determine a MFD/MTD. The maximum-feasible dose (MFD) will be defined as the top dose level if no MTD is defined. It is expected that a maximum of 22 patients will be required to establish the MTD for one infusion. Once an MFD/and or MTD is determined, the study moves onto Phase II part of the protocol design allowing multiple cycles up to 4, treating 6-22 patients in Arm 1 safety and 20 patients in Arm 2 efficacy. Citation Format: Pamela R. Contag. Design of a dose escalation phase 1/2 trial for a novel combined cell and oncolytic viral therapy [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2018; 2018 Apr 14-18; Chicago, IL. Philadelphia (PA): AACR; Cancer Res 2018;78(13 Suppl):Abstract nr CT155.

PIPAC and HIPEC-competing or supplementary therapeutic procedures for peritoneal metastases

Peritoneal carcinomatosis remains atherapeutic challenge. The combination of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is currently the only potentially curative option with good results. For good oncological results acomplete macroscopic cytoreduction is essential. This mostly requires acomplex operative procedure with significant morbidity and mortality. Therefore, multimodal treatment is limited to a few highly selected patients. Pressurized intraperitoneal aerosol chemotherapy (PIPAC) is anew minimally invasive approach, the value and spectrum of applications of which are still under investigation; however, the articles on PIPAC published so far are encouraging and PIPAC is therefore a possible palliative therapy option for patients who are not eligible for CRS and HIPEC. The aim of this review is to present a summary of the recent data regarding CRS-HIPEC and PIPAC.

Clinical treatment progress of pseudomyxoma peritonei syndrome

Pseudomyxoma peritonei syndrome (PMP) is characterized by a gradual expansion of mucoid tumour and fluid at specific sites within abdominopelvic regions as a result of a perforated appendiceal adenoma. The standard therapy for PMP is combining cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy. Maximal tumor debulking surgery (MTD) may be beneficial in patients in whom complete cytoreductive surgery (CCRS) cannot be achieved. It is now recognized that CCRS is one of the strongest predictors of long term survival in patients with PMP. The 5-year survival rate in patients who underwent CCRS was 80.0%-87.4%, however the rate in patients who underwent MTD was only 24.0%-39.2%. This article reviews the pathological classification, diagnosis and treatment of PMP. Key words: Pseudomyxoma peritonei; Cytoreductive surgery; Maximal tumor debulking surgery; Hyperthermic intraperitoneal chemotherapy

Cytoreductive Surgery in Combination with HIPEC in the Treatment of Peritoneal Sarcomatosis.

Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is an effective treatment for peritoneal carcinomatosis, but it has been debated for peritoneal sarcomatosis. The purpose of the study is the presentation of perioperative and long-term results of CRS and hyperthermic intraoperative intraperitoneal chemotherapy in patients with peritoneal sarcomatosis. Retrospective study in a prospectively maintained database of 20 patientsthat underwent 29 CRS + HIPEC for peritoneal sarcomatosis. Clinical and histopathologic variables were correlated to survival. Complete cytoreduction was possible in 86.2% of the cases. The hospital mortality and morbidity rate were 0 and 20.7%, respectively. The median follow-up was 26months, and recurrence was recorded in 20 cases (69%). The median and 5-year survival was 55 ± 13 (34-58) months and 43%, respectively. Prior surgical score (PSS) was the single variable related to survival (p = 0.018). The histologic subtype of the tumor was related to recurrence (p < 0.001). CRS and HIPEC in peritoneal sarcomatosis may offer a survival benefit in selected patients with low hospital mortality. The variety of histologic types of sarcomatosis has not made possible the identification of subgroups of patients that may be offered significant benefit by CRS and HIPEC. Further studies are required.

Pharmacokinetic drug evaluation of niraparib for the treatment of ovarian cancer

ABSTRACTIntroduction: Ovarian cancer is a disease with a propensity to recur despite dramatic responses to initial treatment, which typically consists of a combination of cytoreductive surgery and platinum-based chemotherapy. A maintenance therapy, which may prevent or delay relapse while not negatively impacting quality of life, is critical to improving outcomes.Areas covered: This review discusses the pharmacologic properties, clinical efficacy, and safety profile of niraparib, a poly(ADP-ribose) polymerase (PARP) inhibitor indicated for the maintenance treatment of patients with recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in a complete or partial response to platinum-based chemotherapy.Expert opinion: Following presentation of ENGOT-OV16/NOVA at the European Society for Medical Oncology (ESMO) 2016 Congress, niraparib became the first PARP inhibitor to receive full approval by the U.S. Food and Drug Administration (FDA) for the maintenance treatment of recurrent ovarian cancer, regardless of a patient’s germline or somatic mutational status. This approval has had a sweeping impact on treatment strategies, moving the indication for a PARP inhibitor earlier in the treatment course and greatly expanding the population of patients who may benefit from this class of drugs. Active clinical trials suggest that new indications and novel treatment combinations are eagerly sought.

Inflammatory bowel disease drastically affects the prognosis of patients treated for peritoneal metastases with combined cytoreductive surgery and hyperthermic intraperitoneal chemotherapy: A multicenter study

BackgroundComplete cytoreductive surgery (CCRS) followed by hyperthermic intraperitoneal chemotherapy (HIPEC) is a validated treatment in selected patients with peritoneal metastases (PM) of intestinal origin. There is an increased risk of Colorectal Cancer (CRC) and Small Bowel Adenocarcinoma (SBA) in Inflammatory Bowel Disease (IBD). The feasibility and benefit of that surgical approach in IBD patients is unknown. MethodsIBD patients with operated PM complicating CRC or SBA were extracted from a French national multicenter prospective database of patients who underwent surgery for PM in HIPEC expert centers from 1995 to 2016. IBD patients who underwent CCRS plus HIPEC were compared with a cohort of 234 patients who had the same surgery for sporadic colon cancer. Results14 patients (male 57%, median age 40 years, 12 Crohn's disease) with CRC (n = 7) and SBA (n = 7) were included. CCRS followed by HIPEC (oxaliplatin 72.7%) was performed in 11 cases (median peritoneal cancer index 7; range 1–30). The control group had the same characteristics except an older age at HIPEC (56.52 vs 45.74; p = 0.003). Overall survival (HR = 4.47; 90% CI, 1.91 to 10.49), Relapse Free Survival (HR = 2.31; 90% CI, 1.17 to 4.56) and Peritoneal Recurrence Free Survival (HR = 3.30; 90% CI, 1.59 to 6.85) were significantly lower in IBD patients. Six of the 11 patients presented major surgical morbidity with no impact on post-operative treatment. ConclusionCCRS followed by HIPEC is less effective in IBD patients with resectable PM complicating CRC or SBA. More careful selection of those patients is needed.

Pseudomyxoma peritonei treated with cytoreductive surgery and hyperthermic chemotherapy: a 7-year single-center experience.

Pseudomyxoma peritonei (PMP) is a rare clinical entity characterized by diffuse intraabdominal gelatinous collections with mucinous implants on the peritoneal surfaces and omentum. This condition should be considered a borderline malignancy with disease progression over time. Encouraging treatment results have been recently reported with the combination of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). . From December 2003 to December 2010, 18 patients with PMP were referred to our institution. All patients underwent peritonectomy and CRS combined with HIPEC in accordance with Sugarbaker's procedure. The mean Peritoneal Cancer Index score was 27.6 (range, 5-39). Twelve (67%) patients had disseminated peritoneal adenomucinosis and 6 (33%) peritoneal mucinous carcinomatosis. Optimal cytoreduction with no visible residual disease or residual disease ≤2.5 mm in diameter was achieved in all patients. The mean duration of the surgical procedure including HIPEC was 9 hours and 30 minutes (range, 5-13 hours); major morbidity occurred in 30% of patients and the mortality was 11%. The mean follow-up was 27 months (range, 1-72) and the 5-year overall survival 66%. In line with the existing literature, our experience suggests that patients with PMP could benefit from CRS + HIPEC in terms of survival and locoregional disease control.

Effects of Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in the Treatment of Goblet Cell Carcinoma: A Prospective Cohort Study.

Goblet cell carcinoma (GCC) of the appendix is a rare disease. Treatment options vary according to disease staging. Cytoreductive surgery in combination with hyperthermic intraperitoneal chemotherapy (CRS+HIPEC) may improve survival in patients with peritoneal spreading. The aim of this study was to examine the prognosis of patients with appendiceal GCC treated per protocol, and to evaluate the results of CRS+HIPECin cases of peritoneal spreading. From 2009 to 2016, a total of 48 GCC patients were referred to the European Neuroendocrine Tumour Center of Excellence, Aarhus University Hospital. All patients received treatment per protocol according to disease staging. In patients with localized disease, the treatment was a right hemicolectomy. Patients with peritoneal spread who met the inclusion criteria for CRS+HIPEC, as well as patients with high-risk features of developing peritoneal spread, received CRS+HIPEC. If too-extensive disease was found, palliative chemotherapy was offered. Overall survival for patients with localized disease (n=6) or deemed at risk of peritoneal spread (n=8) was 100% after a median follow-up of 3.5years. In patients with peritoneal spread and eligible for CRS+HIPEC(n=27), the median survival was 3.2years [95% confidence interval (CI) 2.3-4.1] and the 5-year survival rate was 57%. In contrast, the median survival for patients with too-extensive intraperitoneal disease (n=7) was 1.3years (95% CI 0.6-2.0), with a 3-year survival rate of 20%. Long-term survival can be achieved in patients with peritoneal spread treated with CRS+HIPEC. CRS+HIPECwas associated with a favorable outcome in GCC patients at high-risk of developing peritoneal spread.

Cytoreductive Surgery and HIPEC in the First-Line and Interval Time Points of Advanced Epithelial Ovarian Cancer

The increasing popularity of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for epithelial ovarian cancer (EOC) has been focus of a heated debate. The objectives of this review are to discuss: (1) the main concerns related to radical surgery and the importance of the quality of the CRS in the final outcome of the patient and (2) the rationale and current status to propose trials on CRS and HIPEC in the first-line and interval settings. Although the standard treatment of EOC is the combination of CRS and platinum-based adjuvant chemotherapy, the potential benefits of radical surgery have been under exploited due to unrealistic and exaggerated concern regarding the prognostic limit imposed by the initial tumor burden. Radical surgery at first-line or interval setting could: (1) enable the early treatment of chemoresistant clones that are likely to be already present at diagnosis; (2) not delay the initiation of subsequent chemotherapy at a point to jeopardize the prognosis; (3) enable, by means of HIPEC, the treatment of microscopic residuals that would otherwise grow after surgery, according to the tumor cell entrapment theory. Data from observational studies on first-line CRS and HIPEC did not demonstrate a striking prognostic advantage over CRS alone. However, a recent Dutch randomized trial on interval CRS and HIPEC versus interval CRS demonstrated a clear-cut improvement of overall and progression-free survivals. More prospective trials on HIPEC are ongoing, but the main question at stake, which is the advisable limits of radical surgery, will still remain open.

Advances in surgery for peritoneal surface malignancies

Abstract Currently, patients with isolated peritoneal surface malignancies are treated with a combination of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). This combination should now be considered the standard of care for appendiceal cancers (including pseudomyxoma), colorectal cancer and peritoneal mesothelioma, while patients with peritoneal metastases from ovarian or gastric cancer may be treated within clinical trials. At present, 8 clinical centres in Poland perform CRS and HIPEC. The unanswered problems of combined intraperitoneal therapy were an impulse for the organization of the 4th International Conference “Advances in Surgical Oncology” that was held on November 23-24, 2017, in Lublin (Poland), and the Polish chapter of the Peritoneal Surface Oncology Group International (PSOGI) was established as a result of this meeting. This special issue of the Current Issues in Pharmacy and Medical Sciences is dedicated to the current therapeutic difficulties discussed during the two days of the conference – experiences that were exchanged to set in motion further directions for the improvement of intraperitoneal treatment.

Clinical presentation, diagnosis, classification and management of peritoneal mesothelioma: a review.

Peritoneal mesothelioma (PM) is an uncommon but a serious, and often, fatal primary peritoneal tumour, with increasing incidence worldwide. Conventional systemic chemotherapy, generally based on experience with pleural mesothelioma, usually has disappointing results considering PM as a terminal condition. Patients usually present with non-specific symptoms of abdominal distension and pain making the diagnosis challenging. As PM is confined to the abdomen for all, or much, of its clinical course, a multimodality treatment combining cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has emerged as a new standard of care, and has been reported to achieve promising survival outcomes and local disease control in selected patients with PM. This review updates the presentation, diagnosis, classification and treatment strategies for PM.