Dartigues Peggy, Isaac Sylvie, Villeneuve Laurent, Glehen Olivier, Capovilla Mathieu, Chevallier Anne, Croce Sabrina, Kaci Rachid, Lang-Averous Gerlinde, Laverriere Marie-Hélène, Leroux-Broussier Agnès, Mery Éliane, Poizat Flora, Valmary-Degano Séverine, Verriele-Beurrier Véronique, Gilly François-Noël, Bibeau Frédéric
Département d'anatomie pathologique, institut Gustave-Roussy, 94805 Villejuif, France.
Service d'anatomie pathologique, centre hospitalier Lyon-Sud, hospices civils de Lyon, 69495 Pierre-Bénite, France.
Ann Pathol. 2014 Feb;34(1):14-25. doi: 10.1016/j.annpat.2014.01.012. Epub 2014 Feb 25.
Pseudomyxoma peritonei is a clinical entity characterized by a gelatinous ascite associated with mucinous tumor deposits spreading on peritoneal surface and potentially invading abdominal organs. It is considered as a tumor process linked, in most of cases, to a mucinous appendiceal neoplasm. Pseudomyxoma peritonei may benefit from a therapeutic strategy combining cytoreductive surgery and intra-peritoneal chemotherapy, which has led to a major prognosis improvement. Different classifications are available and the last one corresponds to the WHO 2010 version, which individualizes pseudomyxoma peritonei in two classes: low grade and high grade mucinous carcinoma. The very low frequency of this entity and its specific therapeutic strategy need specific health care centres, as well as physicians and pathologists collaborating through dedicated networks. The aim of this article is to summarize the pathology, causes, mechanisms and therapeutic approaches of pseudomyxoma peritonei, as well as their interfaces with dedicated networks.
腹膜假黏液瘤是一种临床病症，其特征为伴有黏液性肿瘤沉积物的胶冻样腹水，这些沉积物散布于腹膜表面并可能侵犯腹部器官。在大多数情况下，它被认为是一种与黏液性阑尾肿瘤相关的肿瘤病变。腹膜假黏液瘤可能受益于减瘤手术和腹腔内化疗相结合的治疗策略，这已使预后得到显著改善。目前有不同的分类方法，最新的一种对应于世界卫生组织2010年版，该版本将腹膜假黏液瘤分为两类：低级别和高级别黏液性癌。这种病症极为罕见，其特定的治疗策略需要专门的医疗保健中心，以及通过专门网络协作的医生和病理学家。本文旨在总结腹膜假黏液瘤的病理学、病因、机制和治疗方法，以及它们与专门网络的联系。
