• Research

    New Vaccine Treatment for GNAS

    Researchers from Oslo, Norway have released a new paper outlining the potential for use of a peptide vaccine to help patients with PMP exhibiting mutation in GNAS. They write: “Patients with GNAS-mutated pseudomyxoma peritonei (PMP) have a pre-existing immunity against mutated Gsa. Inhibition of anti-tumor T-cells by upregulation of immune checkpoint molecules results in insufficient immune response. The results suggest exporint peptide vaccination with Gsa peptides in combination with immune checkpoint inhibition as a possible curative treatment for PMP and other GNAS-mutated cancers.”     You can read this new paper here.

  • Events,  News

    Upcoming talks

    Due to the COVID-19 crisis, meetings of the EuroPMP COST Action have had to be postponed. In order to continue the work of the Action, and to help all our members get to know the work that other researchers are focusing on across Europe and around the world, EuroPMP will be offering short (~1 hour) lunchtime seminars on the Zoom platform once per month beginning in September. Links to join the meeting will be sent to members via email before the seminar date. All talks take place at 2pm on the stated date. The next talk will be on the 22nd October 2021, and will be presented by Dr Andreas…

  • News

    Are you our new Virtual Network Support Manager?

    COST has made it possible for us to appoint a Virtual Network Support manager to help us in delivering digital content to our participants. This new voluntary role will be in charge of creating a virtual network strategy, coordinating the calls for expression of interest for the new Virtual Mobility Grants, and for creating a report of virtual networking activity undertaken by EuroPMP as an Action. The person who takes on this role will be required to: Prepare and distribute a detailed virtual networking strategy for the entire Action Support planning, preparation for and execution of collaborative activities including online and/or blended meetings Support the selection of hosts for virtual…

  • Research

    Ki-67 shows great potential as a stratification biomarker

    The identifiation of patients who may require a specific treatment for PMP has long been one of the largest challenges facing doctors. Researchers from Spain’s Reina Sofia University Hospital and Maimonides Institute for Biomedical Research of Cordoba (IMIBIC) have published a new paper looking at the potential for use of the tumour proliferation protein Ki-67 as a potential biomarker of disease severity. EuroPMP members Alvaro Arjona-Sanchez and Antiono Romero-Ruiz noted that patients with high grade PMP associated with a high proliferative index in Ki-67 (over 15%) were more likely to suffer from a worse prognosis overall than patients with lower levels of this key proliferation protein. The inclusion of this…

  • Research

    Antiproliferative activity of vorinostat

    Researchers from the EuroPMP COST Action have recently published a new article looking at the antiproliferative activity of vorinostat in PMP-like cell lines. Members from Greece (Christiana Mantzourani and George Kokotos) and Finland (Pirjo Nummela and Ari Ristimäki) collaborated on this project to shed new light on the potential use of antitumor agents to treat pseudomyxoma peritonei. George notes: “We have shown that one of these new analogs showed antiproliferative activity against two colon cancer cell lines genetically resembling pseudomyxoma peritonei (PMP), namely HCT116 GNAS R201C/+ and LS174T (IC50 0.6 and 1.4 μМ, respectively) with potency slightly better than vorinostat (IC50 1.1 and 2.1 μМ, respectively).” We congratulate Finland and…

  • Research

    New paper suggests the value of anti-angiogenic treatments

    There is a lot of evidence to suggest anti-angiogenic treatment (treatments that stop new blood vessels from growing) might be helpful in treatment for Pseudomyxoma peritonei. A new paper from the Oslo team suggests that angiogenesis may be a larger factor in PMP progression than previously thought. Although research is still in the early stages, this research suggests that the potential for anti-angiogenic treatment warrants further study. The new paper may be read here. (Image: Wikimedia Commons)

  • Research

    The Role of EM Plasticity in PMP

    EuroPMP members from Venice, Italy, Maria Luisa Calabrò and Antonio Sommariva, have recently published a new paper on the role of epithelial-mesenchymal plasticity in PMP. This is a very interesting paper that looks at a part of the PMP picture that is still not well-understood, namely the way in which cells move from the first site they grow in to other sites around the abdominal cavity. Dr Calabrò states: “This review focuses on the role of epithelial-mesenchymal dynamics leading to the dissemination and growth of appendix mucinous neoplasms in the peritoneum. We mainly discuss factors and pathways involved in the pathogenesis of PMP highlighting the plasticity of PMP cells, a…

  • Information


    **请在此处访问临床指南共识文档。 腹膜假黏液瘤是一种较为罕见的疾病,其患病率约为百万分之二。在您的整个职业生涯中,或许只有一两名患者被确诊为该病。如果没有经验丰富的病理学家进行专业的组织病理学检查,通常很难将腹膜假黏液瘤与其他常见黏液性肿瘤进行区分,例如卵巢癌。 腹膜假黏液瘤是发生在腹膜腔内的广泛分布的粘液性瘤变,通常由阑尾肿瘤破裂引起。通常表现为由腹腔胶胨样黏蛋白的积聚引起的胶质状腹水,称为“胶腹”或“果冻腹”。随着黏蛋白的蓄积,其对腹腔产生的压力逐渐压迫腹腔器官,从而引起肠梗阻和腹部胀痛。亦可出现女性卵巢增大或新发疝等临床表现。 大多腹膜假黏液瘤生长缓慢,黏液性腹水呈渐进性生长。亦有患者因突发肠梗阻,或女性患者因卵巢黏液性肿瘤或卵巢克鲁根勃氏瘤而导致腹膜假黏液瘤在数月内快速生长。 需与结肠、胰腺、胆囊或卵巢的原发性黏液性肿瘤,以及腹膜间皮瘤进行鉴别诊断。经验丰富的病理医生将是协助您获得明确诊断的关键。需要注意的是,真正的腹膜假黏液瘤很少起源于卵巢。多数伴有卵巢囊腺瘤的病例发现其具有临床隐匿性阑尾原发灶。 通常使用影像学检查进行疑似腹膜假黏液瘤的诊断。腹部成像提示黏液性腹水,网膜饼和卵巢增大等特征。CT检查可见肝脏与脾脏边缘呈特征性扇贝样凹陷。部分病例影像可见阑尾原发性肿瘤,阑尾黏膜膨出最为常见。腹膜假黏液瘤也会在其他外科手术中被诊断出。 明确的鉴别诊断需要对阑尾原发肿瘤和腹膜疾病进行组织病理学评估。阑尾中最常见的病变是低级别阑尾黏液性肿瘤,其他可能的阑尾原发肿瘤包括黏液腺癌和罕见的高级别阑尾黏液性肿瘤。腹膜假黏液瘤在病理学上分为三大类:低级别粘液性腹膜癌(以前称为弥漫性腹膜腺黏液腺瘤病),高级别腹膜黏液癌和腹膜高级别黏液癌伴印戒细胞。疾病的病理分类可能会影响治疗方案的选择,因此病理学诊断是并不可少的。 一经确诊,可以通过肿瘤减灭术和腹腔热灌注化疗联合治疗进行较为彻底地治疗,也可以采取姑息性治疗减缓症状。最为有效的治疗方案,需要针对患病个体进行选择。由于腹膜假黏液瘤通常进展缓慢,因此,对于身体条件不允许或因其他疾病无法满足手术指征的个体,可采取非手术干预措施以维持其生存质量。 肿瘤减灭术是一项手术范围广且具有侵入性的治疗方法,但其预后较好。肿瘤减灭术的目的是从腹腔中清除所有可见的肿瘤。除了腹前壁腹膜和偏侧膈切除外,还可以包括结肠切除术,脾切除术,网膜切除术和胆囊切除术,以及女性的子宫切除术和双侧输卵管卵巢切除术。肿瘤减灭术通常与腹腔热灌注化疗进行联合治疗,利用热效应及化疗液灌洗以消除可能残留的肿瘤细胞,从而降低复发风险。 腹膜假黏液瘤是一种“播种”型癌症,因此,在手术期间必须格外小心。如果在手术过程中遇到黏液性肿瘤或腹水,应考虑其为腹膜假黏液瘤。可进一步检查阑尾是否受累,如果在该处发现肿瘤,那么应立即与消化内科医生进行会诊。 值得注意的是,此时需要避免在手术中扩大范围。 手术创面的开放使得邻近组织易被脱落的肿瘤细胞侵犯,从而提升后续治疗的难度。 如果您需要有关腹膜假黏液瘤的更多详细信息,请与您所在地区的EuroPMP代表取得联系,以得到该地区可提供专业帮助的外科医生,病理学家和研究人员。 英国克里斯蒂NHS基金会信托已为医生提供了腹膜假黏液瘤相关文档,并为英国患者提供了姑息治疗。 [Information for Doctors] [Palliation of PMP]  请注意,此信息仅供参考。请与您当地的相关部门联系,以获取有关您所在国家/地区的腹膜假黏液瘤治疗方案的更多信息。 在这里可以找到有关欧洲腹膜假黏液瘤患病率和发病率的最新数据。    

  • Information

    Pseudomyxoma peritonei (na hrvatskom)

    Pseudomyxoma peritonei (PMP) je rijetka bolest sa stopom incidencije od oko 2 osobe na milijun stanovnika. To je dijagnoza koju liječnik tijekom svoje karijere može postaviti kod jednog ili dva pacijenta, ukoliko se uopće sretne s tim problemom. Često je teško razlikovati PMP od drugih češćih vrsta mucinoznog tumora, poput raka jajnika, bez temeljitog histološkog pregleda iskusnog patologa. PMP je široko diseminirana mucinozna neoplazma koja se javlja u peritonealnoj šupljini i obično nastaje zbog puknuća tumora slijepog crijeva. Može se prikazati kao generalizirana nakupina mucina u trbušnoj šupljini zbog čega dolazi do znatnijeg povećanja abdominalne šupljine, kolokvijalno nazvanog “žele trbuh”. Akumulacija tekućine postaje problem za pacijenta, jer pritisak sve veće…

  • Information

    Informazioni per i medici

    È possibile accedere qui alla documentazione sul consensus delle linee guida cliniche attuali. (linee guida attuali / palliazione) Lo pseudomixoma peritoneale (PMP) è una malattia rara con un indice di incidenza di circa 2 persone per milione. Durante la tua carriere potresti diagnosticare al massimo uno o due pazienti affetti da questa malattia. Spesso è difficile la diagnosi differenziale tra lo PMP e un più comune tipo di tumore mucinoso, come il cancro ovarico, senza l’ausilio di un profondo esame istologico condotto da un patologo esperto. Lo PMP è una neoplasia mucinosa ad ampia diffusione che si verifica nella cavità peritoneale e, generalmente, originato da un tumore appendicolare che porta…