Malignant Peritoneal Mesothelioma Research Articles

Anaplastic lymphoma kinase (ALK) partners identified by next-generation sequencing in Chinese patients with solid tumors.

3555 Background: Anaplastic lymphoma kinase (ALK) rearrangement is a validated therapeutic driver gene in non-small cell lung cancer (NSCLC). More than 30 different fusion partner genes of ALK in NSCLC have been reportedand most of these ALK fusions respond well to ALK inhibitors crizotinib. With the development of next-generation sequencing (NGS), more novel partners for ALK rearrangement have been identified. Here, we aimed to report the landscape of ALK rearrangement in Chinese patients with solid tumors. Methods: Tissue or blood samples were subjected to NGS in a College of American Pathologists-certified and Clinical Laboratory Improvement Amendments-accredited lab for ALK arrangement. Results: In total, we profiled more than 40,000 patients, among which 72 cases with 52 ALK fusion partner, harboring 17 reported partners and 35 novel partners. The average ALK rearrangement patients' age was 53 years (range, 17-76 years). Among all the ALK fusion cases (n = 72), lung cancer were the largest proportion with 77.8% (n = 56), colorectal cancer accounted for, 5.5% (n = 4), liver cancer accounted for 4.2% (n = 3), biliary cancer, melanoma, carcinosarcoma and inflammatory myofibroblastic tumor accounted for 2.8% (n = 2) respectively, and only one case (n = 1) was malignant peritoneal mesothelioma. The most common ALK fusion partners were KIF5B (n = 6), DCTN1 (n = 5) and STRN (n = 5). In 38 cases, 35 novel ALK fusion partners were discovered. The novel CLIP4-ALK, EHBP1-ALK, PLB1-ALK occurred twice in 6 patients, which were two lung cancer patients with CLIP4-ALK fusion, two lung cancer patients with PLB1-ALK fusion, one hepatic cellular cancer patients with EHBP1-ALK, and one melanoma patients with EHBP1-ALK. There were two special lung cancer cases with two ALK fusions. One case detected the novel LRIG1-ALK fusion and novel PLB1-ALK fusion, the other case detected novel GLI3-ALK fusion and reported HIP1-ALK fusion. Conclusions: Novel ALK fusions are detected in patients with not only NSCLC but also other solid tumors. NGS fusion assay is an optional method for screening novel fusions.

Phase II study of olaparib in malignant mesothelioma (MM) to correlate efficacy with germline and somatic mutations in DNA repair genes.

9054 Background: BRCA1 associated protein 1 ( BAP1), a nuclear deubiquitinase involved in DNA double-strand break repair is frequently mutated in MM. Because poly(ADP-ribose) polymerase inhibitors (PARPIs) induce synthetic lethality in BRCA1/2 mutant cancers, we sought to evaluate efficacy of olaparib in patients with MM and correlate it with pathogenic germline and somatic mutations in DNA repair genes. Methods: Phase II single-center study (NCT03531840) enrolled patients with advanced pleural or peritoneal mesothelioma who had progressed on prior therapies, age >18 years, ECOG performance status <1, adequate organ and bone marrow function. Olaparib 300mg was given twice daily orally in 3 week cycles until disease progression or toxicity. Efficacy was assessed by CT scan every 6 weeks using RECIST criteria. Whole exome sequencing (WES) was performed on blood and tumor samples to identify pathogenic germline and somatic mutations in DNA repair genes. Primary objective was to determine response rate based on germline or somatic mutation status of DNA repair genes. Results: Between July 2018 to May 2019, 23 patients were enrolled, 15 pleural and 8 peritoneal MM [14 male; median age 63 (range 41-75 years); median number of prior treatments 3 (range 1-5)]. Median olaparib cycles received was 4 (2-21). WES to identify pathogenic mutations in the germline and tumor was performed in 23 and 17 patients respectively. Four patients had germline BAP1, 1 germline MRE11A, and 5 had somatic BAP1 mutations. Of 22 evaluable patients, 1(4%) had partial response (PR), 17 (77%) had stable disease at 6 weeks and 4 (18%) had progressive disease. Patient with PR had a germline mutation in MRE11A. Median progression free survival (PFS) and overall survival (OS) for all patients was 3.4 months (95% CI: 2.7 – 4.8 months) and 8.1 months (95% CI: 4.5 months – not estimable) respectively. Median PFS of germline BAP1 mutant patients (n = 4) was 2.3 months (95% CI: 1.3 – 3.6 months) compared to 4.1 months (95% CI: 2.7 – 5.5 months) for BAP1wild type patients (n = 18;P = 0.026). Median OS was 4.6 months (95% CI: 3.1 – 4.9 months) for patients with germline BAP1 mutation versus not reached for those without germline BAP1 mutation (P = 0.0058). The most common side effects of olaparib were anemia (16%), lymphopenia (24%), nausea (14%), and increased creatinine (9%). Conclusions: Olaparib has limited anti-tumor activity in previously treated MM patients including those with germline or somatic BAP1 mutations. Presence of germline BAP1 mutations was associated with decreased PFS and OS. Clinical trial information: NCT03531840.

Real-world outcomes of pembrolizumab in peritoneal mesothelioma.

e21094 Background: Clinical trials evaluating pembrolizumab (P) in malignant mesothelioma (MM) have included small numbers of peritoneal MM (MPM) patients (pts), but the efficacy of P in the MPM subpopulation remains unclear. We evaluated the efficacy of P in a cohort of MPM pts treated at our institution. Methods: This retrospective cohort study identified MPM pts via ICD9/10 codes seen at our institution between 1/1/2009 and 9/1/2019. Pts were included if they received P as treatment for MPM. Median progression free survival (PFS) and median overall survival (mOS) were estimated from Kaplan-Meier curves. PFS was defined as the start of P until radiologic or clinical progression. Best overall response rate (BOR) and disease control rate (DCR) were determined retrospectively using RECIST 1.1 criteria. Results: We identified 13 non-papillary MPM pts who received P. All pts had received prior chemotherapy (12 platinum/pemetrexed, 1 pemetrexed). Median age at diagnosis was 65.6 years; 77% were white; 46% never smokers; 62% with known asbestos exposure; 70% epithelioid/15% biphasic/ 7.7% sarcomatoid/ 7.7% desmoplastic. BOR to P was 18% (Partial response (PR): 2, Progressive disease (PD): 2, Stable disease (SD): 7, Lost to follow up: 2). DCR was 81% and mPFS 5.7mo. From the start of P mOS was 20.9 mo. Only 3 pts had known PDL1 positivity (1%, 2%, 80%), 4 were negative, 6 untested. Median PFS was not statistically different between PDL1 positive and negative pts (mPFS 5.1mo vs. 5.7 mo, log rank p = 0.73, respectively). Three pts experienced a PFS of > 2 years (PDL1/BOR/% tumor change: Unknown/SD/19%, Negative/SD/-21%, 80%/PR/-70%). The patient with PDL1 80%, biphasic histology and a PR experienced skin toxicity requiring a treatment break. Outcomes for epithelioid histology vs non-epithelioid after P did not differ statistically (mPFS 5 mo vs 39 mo, log rank p = 0.14; mOS 17.5 mo vs NA, log rank p = 0.31). Conclusions: In a real world setting, P has clinically meaningful activity in a PDL1 unselected cohort of MPM pts and should be considered a treatment option for this subpopulation of MM. We did not detect a difference in outcomes based on PDL1 level or histology.

Localized biphasic malignant mesothelioma presenting as a giant pelvic wall mass: a rare case report and literature review

BackgroundLocalized biphasic MPeM is rare in clinical practice, we reviewed 8 cases of localized biphasic MPeM (including our present case), and summarized the clinical and imaging features of the disease.Case presentationWe reported a 79-year-old man with chief complaint of a narrowing in the caliber of the stool for one year. A soft tissue shadow was occasionally found by CT examination in the right pelvic wall, and it was diagnosed as localized biphasic malignant peritoneal mesothelioma (MPeM) by postoperative pathology. Radical excision was performed and no radio-chemotherapy was applied. Nearly six years after surgery, the mass was significantly enlarged, and the neighboring tissues including rectum, prostate, seminal vesicle, and right ischial ramus were all infiltrated. The patient was in the end stage of cancer with poor prognosis.ConclusionsThe localized biphasic MPeM may show following characteristics: (1) with heterogeneous low-density and obscure margin; (2) with low incidence rate of ascites; (3) with few central hemorrhage and necrosis; (4) with few calcified structures; (5) with mild to moderate heterogeneous delayed enhancement on contrast-enhanced CT. The imaging characteristics can provide further information for the diagnosis of localized biphasic MPeM in the future.

Overexpression of miR-199/214 is a distinctive feature of iron-induced and asbestos-induced sarcomatoid mesothelioma in rats.

Malignant mesothelioma (MM) is one of the most lethal tumors in humans. The onset of MM is linked to exposure to asbestos, which generates reactive oxygen species (ROS). ROS are believed to be derived from the frustrated phagocytosis and the iron in asbestos. To explore the pathogenesis of MM, peritoneal MM was induced in rats by the repeated intraperitoneal injection of iron saccharate and nitrilotriacetate. In the present study, we used microarray techniques to screen the microRNA (miR) expression profiles of these MM. We observed that the histological subtype impacted the hierarchical clustering of miR expression profiles and determined that miR‐199/214 is a distinctive feature of iron saccharate‐induced sarcomatoid mesothelioma (SM). Twist1, a transcriptional regulator of the epithelial‐mesenchymal transition, has been shown to activate miR‐199/214 transcription; thus, the expression level of Twist1 was examined in iron‐induced and asbestos‐induced mesotheliomas in rats. Twist1 was exclusively expressed in iron saccharate‐induced SM but not in the epithelioid subtype. The Twist1‐miR‐199/214 axis is activated in iron saccharate‐induced and asbestos‐induced SM. The expression levels of miR‐214 and Twist1 were correlated in an asbestos‐induced MM cell line, suggesting that the Twist1‐miR‐199/214 axis is preserved. MeT5A, an immortalized human mesothelial cell line, was used for the functional analysis of miR. The overexpression of miR‐199/214 promoted cellular proliferation, mobility and phosphorylation of Akt and ERK in MeT5A cells. These results indicate that miR‐199/214 may affect the aggressive biological behavior of SM.

Prise en charge des tumeurs primitives et métastatiques du péritoine en période de pandémie CoViD-19. Pistes de réflexion et de priorisation du groupe RENAPE et BIG-RENAPE

RésuméLa pandémie de COVID-19 modifie profondément l’organisation et l’accès aux soins, en particulier pour les pathologies néoplasiques péritonéales, dont la prise en charge curative mobilise des moyens importants en personnel, bloc opératoire et réanimation. Les groupes BIG-RENAPE et RENAPE proposent des pistes de réflexion et de priorisation pour leur prise en charge. Un renforcement des critères habituels de sélection est nécessaire pour une prise en charge à visée curative : patients jeunes, avec peu de co-morbidités et une extension péritonéale limitée. Il est souhaitable de prioriser les pathologies pour lesquelles la chirurgie de cytoréduction associée ou non à une chimiohyperthermie intrapéritonéale (CHIP) est le traitement de référence et celles pour lesquelles la chimiothérapie systémique ne peut être une alternative temporaire ou prolongée : pseudomyxomes péritonéaux ; mésothéliomes péritonéaux malins résécables ; métastases péritonéales d’origine colorectale si résécables, non répondeuses à la chimiothérapie systémique et/ou après 12 cures, carcinoses ovariennes en 1re intention si résécables et limitées ou en situation intervallaire après un maximum de 6 cycles de chimiothérapie systémique. L’adjonction d’une CHIP devra être discutée au cas par cas, en centre expert. La priorisation des indications devra prendre en considération les conditions locales et la phase de la période épidémique pour permettre une prise en charge péri-opératoire optimale.

Treatment of primary and metastatic peritoneal tumors in the Covid-19 pandemic. Proposals for prioritization from the RENAPE and BIG-RENAPE groups.

SummaryThe Covid-19 pandemic is profoundly changing the organization of healthcare access. This is particularly so for peritoneal neoplastic diseases, for which curative treatment mobilizes substantial personnel, operating room and intensive care resources. The BIG-RENAPE and RENAPE groups have made tentative proposals for prioritizing care provision. A tightening of the usual selection criteria is needed for curative care: young patients with few or no comorbidities and limited peritoneal extension. It is desirable to prioritize disease conditions for which cytoreduction surgery with or without associated hyperthermic intraoperative peritoneal chemotherapy (HIPEC) is the gold-standard treatment, and for which systemic chemotherapy cannot be a temporary or long-term alternative: pseudomyxoma peritonei, resectable malignant peritoneal mesotheliomas, peritoneal metastases of colorectal origin if they are resectable and unresponsive to systemic chemotherapy after up to 12 courses, first-line ovarian carcinomatosis if resectable or in interval surgery after at most six courses of systemic chemotherapy. Addition of HIPEC must be discussed case by case in an expert center. The prioritization of indications must consider local conditions and the phase of the epidemic to allow optimal peri-operative care.

Multicystic and diffuse malignant peritoneal mesothelioma in children.

Malignant and multicystic peritoneal mesotheliomas are extremely rare tumors in children, developing from mesothelial cells. No specific guidelines are available at this age. We performed a retrospective analysis of all identified children(<18-year-old) treated in France from 1987 to 2017 for a diffuse malignant peritoneal mesothelioma (DMPM) or a multicystic peritoneal mesothelioma (MCPM). Fourteen patients (5 males and nine females), aged 2.2 to 17.5 years, were included. The most frequent presenting symptoms were abdominal pain, ascitis, and alteration in the general condition. Eight patients had epithelioid mesothelioma, three had biphasic mesothelioma, and three had MCPM. Eight patients with DMPM diagnosis received cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). Among them, six patients had neoadjuvant systemic chemotherapy, one patient, post-operative chemotherapy, and one patient CRS and HIPEC only. Three patients received only systemic chemotherapy. All patients with MCPM had only surgery. After a median follow-up of seven years (2-15), six patients (6/11; one death) with DMPM and two patients (two/three) with MCPM had a local and distant recurrences. Peritoneal mesothelioma in children is a rare condition with difficult diagnosis and high risk of recurrence. Worldwide interdisciplinary collaboration and networking are mandatory to help diagnosis and provide harmonious treatment guidelines.

ASO Author Reflections: Expert Surgical Evaluation is Critical for Patients with Diffuse Malignant Peritoneal Mesothelioma: Lessons Learned from Assessing Outcomes of 2000 Patients.

ASO Author Reflections: Expert Surgical Evaluation is Critical for Patients with Diffuse Malignant Peritoneal Mesothelioma: Lessons Learned from Assessing Outcomes of 2000 Patients.

Survivin expression as an independent predictor of overall survival in malignant peritoneal mesothelioma.

Malignant peritoneal mesothelioma (MPeM) is an incurable cancer strongly associated with asbestos exposure and characterised by poor prognosis. The aim of the present study was to elucidate the prognostic and predictive value of CD146 and survivin expression in MPeM. Diagnostic biopsies from 60 patients with MPeM were collected and analysed for CD146, survivin and Ki-67 expression using immunohistochemistry. Complete clinical and follow-up information was obtained from patients' records. CD146 was expressed in 31/60 MPeM specimens and survivin in 34/60 specimens, with both expression levels being significantly associated with the Ki-67 labelling index (Ki-67LI). Kaplan-Meier and univariate Cox regression analyses revealed that a lower peritoneal cancer index (PCI), tumour-directed treatment, stage I, lower Ki-67LI and lower CD146 and survivin expression had a statistically positive effect on overall survival (OS). Cox regression analysis revealed that PCI [hazard ratio (HR)=1.99; 95% CI, 1.04–3.83; P=0.038], survivin (HR=1.47; 95% CI, 1.03–2.10; P=0.034) and treatment protocol including intraperitoneal chemotherapy (HR=0.28; 95% CI, 0.14–0.57; P=0.013) and systemic chemotherapy (HR=0.13; 95% CI, 0.04–0.42; P=0.013) retained independent prognostic significance for OS. All of these were included in the nomogram. Calibration curves showed good agreement between nomogram-predicted and observed survival. The C-index of the nomogram for predicting OS was 0.77. A lower PCI, intraperitoneal chemotherapy, systemic chemotherapy and a lower level of survivin were powerful prognostic markers in patients with MPeM. The proposed nomogram provides individual survival prediction for patients with MPeM.

Retrospective Evaluation of Malignant Peritoneal Mesothelioma Patients

ilişkilendirilmiştir. Malign peritoneal mezotelyoma (MPM) plevral tutulumdan sonra ikinci sıklıkta görülür. Çalışmamızda MPM tanısı alan hastalarda demografik özelliklerin, kullanılan tedavi seçeneklerinin incelenmesi amaçlandı. Çalışmamızda 12 erkek, 4 kadın toplamda 16 hastanın medyan yaşı 66 (46-93) yıldı. 15 hasta epiteolid, 1 hasta bifazik histopatolojiye sahipti. 2 hastaya hipertermik intraperitoneal kemoterapi (HİPEK) yapılmıştı. Birinci seçim kemoterapi alan 16 hasta, ikinci seçim kemoterapi alan 13 hasta, üçüncü seçim kemoterapi alan 4 hasta, dördüncü seçim kemoterapi alan 1 hasta mevcuttu. Birinci seçim kemoterapi alan hastaların medyan progresyonsuz sağkalımı 14,4 ay (CI %95 7,4:21,4) saptandı. Hastaların medyan toplam sağkalımı 22,0 aydı (CI %95 16:27,9). Standart tedavi seçenekleri arasında olan sitoredüksiyon cerrahisi + HİPEK, sistemik kemoterapi ve immünoterapinin optimal kullanımı ve bu tedavilere uygun hasta seçimi için prospektif, multidispliner, daha fazla hasta sayısı içeren çalışmalara ihtiyaç vardır.

Frequent homozygous deletion of Cdkn2a/2b in tremolite-induced malignant mesothelioma in rats.

The onset of malignant mesothelioma (MM) is linked to exposure to asbestos fibers. Asbestos fibers are classified as serpentine (chrysotile) or amphibole, which includes the crocidolite, amosite, anthophyllite, tremolite, and actinolite types. Although few studies have been undertaken, anthophyllite has been shown to be associated with mesothelioma, and tremolite, a contaminant in talc and chrysotile, is a risk factor for carcinogenicity. Here, after characterizing the length and width of these fibers by scanning electron microscopy, we explored the cytotoxicity induced by tremolite and anthophyllite in cells from an immortalized human mesothelial cell line (MeT5A), murine macrophages (RAW264.7), and in a rat model. Tremolite and short anthophyllite fibers were phagocytosed and localized to vacuoles, whereas the long anthophyllite fibers were caught on the pseudopod of the MeT5A and Raw 264.7 cells, according to transmission electron microscopy. The results from a 2‐day time‐lapse study revealed that tremolite was engulfed and damaged the MeT5A and RAW264.7 cells, but anthophyllite was not cytotoxic to these cells. Intraperitoneal injection of tremolite in rats induced diffuse serosal thickening, whereas anthophyllite formed focal fibrosis and granulomas on peritoneal serosal surfaces. Furthermore, the loss of Cdkn2a/2b, which are the most frequently lost foci in human MM, were observed in 8 cases of rat MM (homozygous deletion [5/8] and loss of heterozygosity [3/8]) by array‐based comparative genomic hybridization techniques. These results indicate that tremolite initiates mesothelial injury and persistently frustrates phagocytes, causing subsequent peritoneal fibrosis and MM. The possible mechanisms of carcinogenicity based on fiber diameter/length are discussed.

Peritoneal mesothelioma: PSOGI/EURACAN clinical practice guidelines for diagnosis, treatment and follow-up

Peritoneal mesothelioma (PM) is a rare and aggressive primary peritoneal malignancy characterized by widespread multiple metastatic tumour nodules originating from the peritoneum. The conventional classification distinguishes diffuse malignant peritoneal mesothelioma (DMPM) and border-line forms: multicystic peritoneal mesothelioma (MCPM) and well-differentiated papillary peritoneal mesothelioma (WDPPM). Despite the novel achievements in the management of PM, there is difficulty in conducting randomized trials due to its rarity and aggressive biology in many cases.

Establishment and characterization of patient derived xenograft model of malignant peritoneal mesothelioma in nude mice

Objective: To establish patient derived xenograft (PDX) model of malignant peritoneal mesothelioma (MPM), and to identify the key characteristics of tumor biology of the model, so as to provide an experiment platform for studying the pathologic mechanisms and new therapeutic strategies for MPM. Methods: Surgically excised MPM tumor tissues were inoculated subcutaneously in BALB/c-nu/nu mice for 3 stable passages. In the 4th passage, the subcutaneous tumors were harvested under aseptic conditions, cleaned and made into MPM tumor cell homogenate. Four nude mice (two males and two females) were selected and one male and one female nude mouse were inoculated in the abdominal cavity at the dose of 100 μL, others were inoculated at a dose of 200 μL. The PDX model of MPM was established. The changes of body mass in nude mice were measured regularly, the extent of abdominal and pelvic tumors was judged by experimental peritoneal cancer index (ePCI) score, and the pathologic characteristics of tumors were analyzed. Results: The subcutaneous and abdominal animal models of MPM were successfully established. The subcutaneous tumor model grew into tumor on the 20th day, followed by a slow growth stage between the 20th and 29th day, then a rapid growth stage between the 30th and 57th day. According to the dose of tumor cells (100, 200 μL) and timing (14th and 69th days after grafting), the abdominal tumor model successfully simulated the early and late clinical stages of MPM. The HE staining results of the MPM nude mice model showed that the tumor was epithelial mesothelioma and invaded most of the organs, including liver, spleen, pancreas, mesentery. Immunohistochemical staining for calretinin, cytokeratin 5/6, WT1 and Ki-67 were positive. Whole-genome exon sequencing identified 26 and 36 high frequency gene mutations in tumors derived from the PDX model and clinical sample from patients, including 21 common gene mutations. Conclusions: The PDX model of MPM is established. The model is characterized by highly malignant tumor with rapid growth and high invasiveness.

Peritoneal mesothelioma.

Malignant peritoneal mesothelioma (MPM) is a rare and lethal disease of the peritoneal lining, with high variability in biologic aggressiveness. Morbidity and mortality of the disease are related to progressive locoregional effects within the abdominal cavity, such as distention, pain, early satiety, and decreased oral intake that can ultimately lead to bowel obstruction and cachexia. The standard of care for patients with resectable disease remains cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC), with potential survival outcomes greater than 5 years in appropriately selected patients. Patients with inoperable MPM can be offered systemic treatment, although the disease is usually refractory to standard chemotherapic regimens. Patients with MPM should be treated at high volume centers with strong consideration for inclusion in tumor registries and clinical trials. In 2020, research will continue to explore promising genetic and immunologic targets and focus on refinement of surgical methods to optimize CRS-HIPEC approaches.

Diffuse malignant peritoneal mesothelioma (DMPM) - a rare diagnosis

Diffuse malignant peritoneal mesothelioma (DMPM) is a rare diagnosis, found more frequently in men than in women. Symptoms are unspecific abdominal disorders making that diagnosis difficult to set. Causes of DMPM are yet to be discovered in entirety. Asbestos exposure is the reason for approximately 7 % of all peritoneal mesotheliomas. Until the evaluation of systematic cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) DMPM was a fatal diagnosis with a median overall survival (OS) of 4-13months. The prognosis of DMPM dramatically improved with implementation of CRS and HIPEC to an OS of 30-92month nowadys. CRS and HIPEC were performed in this case.

Predictors and Outcomes of Surgery in Peritoneal Mesothelioma: an Analysis of 2000 Patients from the National Cancer Database.

Diffuse malignant peritoneal mesothelioma (DMPM) is a rare malignancy associated with poor outcomes. Recent reports have shown longer survival with radical surgery, usually combined with intraperitoneal chemotherapy. However, surgical interventions in these patients have not been extensively studied at a population level. The objective of this retrospective cohort study is to assess the prevalence of surgical and nonsurgical interventions for DMPM patients, the influence of surgery on survival outcomes, and the associations between demographic and clinical factors with treatments and outcomes. This study included adult patients diagnosed with DMPM from 2003 to 2014 and registered in the National Cancer Database (NCDB). The primary outcome was overall survival. Histologically confirmed mesothelioma was defined using International Classification of Diseases (ICD)-3 codes 9050/3, 9051/3, 90523, and 9053/3 and peritoneum as primary affected organ using ICD codes C17-19, C22-24, C26, C42, C48, and C76. Relationships between demographic and clinical variables, surgical treatments, and survival outcomes were evaluated using logistic and Cox modeling and log-rank tests. A total of 2062 patients were identified, of whom 1055 (51%) did not receive any surgery while 701 (34%) received radical surgery. Patients receiving radical surgery had overall survival of 38.4months compared with 7.1months for patients without surgery (p < 0.001) and 41.8months in patients who received both radical surgery and systemic chemotherapy. Patients selected for and treated with radical surgery had significantly better overall survival compared with those receiving nonsurgical treatment. Patients newly diagnosed with DMPM should be evaluated for the possibility of receiving radical surgery.

Malignant Peritoneal Mesothelioma: Patterns of Care and Survival in The Netherlands. A Population Based Study.

Malignant Peritoneal Mesothelioma: Patterns of Care and Survival in The Netherlands. A Population Based Study.

Unusually favorable outcome of 6 consecutive patients with diffuse malignant peritoneal mesothelioma treated with repeated doses of intraperitoneal paclitaxel. A case series

BackgroundDiffuse malignant peritoneal mesothelioma (DMPM) is a disease in transition. Two decades ago this cancer had a median survival of 1 year. Recent reports in selected patients show a 5-year survival of 70% and median survival not reached at 10 years. MethodsConsecutive patients with a biopsy-confirmed diagnosis of DMPM, epithelial type, were treated by a consistent treatment plan. Cytoreductive surgery using peritonectomy procedures was always the first intervention. In the operating theater after all resections were complete but prior to intestinal reconstruction, hyperthermic intraperitoneal chemotherapy (HIPEC) using 2 cytotoxic agents was given. An intraperitoneal port was placed for normothermic intraperitoneal chemotherapy (NIPEC). Prospective data regarding pharmacokinetics, survival and adverse events were accumulated. ResultsSix consecutive patients were treated. Three to 6 one-week long cycles of normothermic intraperitoneal paclitaxel were administered through an intraperitoneal port. No systemic chemotherapy was given. Pharmacokinetic studies were performed on 5 patients. Four patients are free of disease at 8, 13, 18, and 19 years and two patients died of disease 15 years after definitive treatment. Three of 6 patients had a grade III/IV adverse event related to intraperitoneal chemotherapy administration through a port. ConclusionsThe small number of patients treated with repeated doses of intraperitoneal paclitaxel cause these data to be interpreted with caution. However, the long-term benefit and favorable pharmacology of these patients suggests the need for further study. Adverse events from this intraperitoneal port were recorded in half of the patients.

Massive Ascites Secondary to Malignant Peritoneal Mesothelioma

Massive Ascites Secondary to Malignant Peritoneal Mesothelioma