Galani E, Marx G M, Steer C B, Culora G, Harper P G
Medical Oncology Department, Guy's Hospital, London, UK.
Int J Gynecol Cancer. 2003 Jul-Aug;13(4):413-8. doi: 10.1046/j.1525-1438.2003.13318.x.
Pseudomyxoma peritonei (PMP) is a rare disease that is characterized by a large amount of mucinous ascites with peritoneal and omental implants. The etiology of the disease remains unclear. Histologically, two main categories have been described: disseminated peritoneal adenomucinosis (DPAM) and peritoneal mucinous carcinomatosis (PMCA). It is commonly diagnosed incidentally at laparotomy. Most investigators agree that radical surgical debulking and appendectomy are the cornerstone of treatment, but the optimal management of the disease remains controversial. The role of intraoperative and intraperitoneal chemotherapy has been evaluated by a number of authors. The clinical outcomes vary widely between the benign and the malignant forms and between the different treatment modalities. We discuss the pathology, origin, clinical presentation, diagnosis, treatment, and prognosis of PMP.
腹膜假黏液瘤（PMP）是一种罕见疾病，其特征为大量黏液性腹水伴腹膜和网膜种植。该疾病的病因仍不清楚。在组织学上，已描述了两种主要类型：播散性腹膜腺黏液瘤（DPAM）和腹膜黏液性癌（PMCA）。它通常在剖腹手术时偶然被诊断出来。大多数研究者认为，根治性手术减瘤和阑尾切除术是治疗的基石，但该疾病的最佳管理仍存在争议。许多作者对术中及腹腔内化疗的作用进行了评估。良性和恶性形式以及不同治疗方式之间的临床结果差异很大。我们讨论了PMP的病理学、起源、临床表现、诊断、治疗和预后。
