Hinson F L, Ambrose N S
Department of General Surgery, St James's University Hospital, Leeds, UK.
Br J Surg. 1998 Oct;85(10):1332-9. doi: 10.1046/j.1365-2168.1998.00882.x.
Pseudomyxoma peritonei is an unusual condition in which gelatinous fluid collections are associated with mucinous implants on the peritoneal surfaces and omentum. The pathological origin and ideal treatment of the condition are subjects of debate.
An unrestricted Medline search over 1986-1997 was performed for pseudomyxoma peritonei.
There is increasing evidence that pseudomyxoma peritonei is a neoplastic condition which usually arises from a primary adenoma or adenocarcinoma of the appendix. Reported series include a spectrum of pathological lesions, from entirely benign ruptured mucocele to advanced carcinoma. This, and the rarity of the condition, limit the conclusions that can be drawn regarding its treatment and prognosis. Most authorities agree that a thorough surgical debulking should be made. In most cases this will be a difficult and time-consuming undertaking, possibly requiring cooperation between two or more specialists and consideration of delivering intraperitoneal adjuvant therapy during or immediately after surgery. Treatment therefore requires a planned approach with accurate preoperative assessment of the diagnosis and the extent of the condition. There is some largely anecdotal evidence in favour of intraperitoneal chemotherapy and radioisotope treatment. Ultraradical surgery, with heated intraoperative and further postoperative chemotherapy, is strongly advocated by one group but remains contentious. The majority of patients will eventually suffer recurrence. The 5-year survival rate ranges from 53 to 75 per cent, but outcomes vary widely between relatively benign and malignant subgroups.
腹膜假黏液瘤是一种罕见病症，其中胶冻样液体聚集与腹膜表面和大网膜上的黏液性种植体相关。该病症的病理起源和理想治疗方法存在争议。
对1986年至1997年期间的腹膜假黏液瘤进行了无限制的医学文献数据库检索。
越来越多的证据表明，腹膜假黏液瘤是一种肿瘤性疾病，通常起源于阑尾的原发性腺瘤或腺癌。报道的病例系列包括一系列病理病变，从完全良性的破裂黏液囊肿到晚期癌。这一点以及该病症的罕见性，限制了关于其治疗和预后所能得出的结论。大多数权威人士一致认为应进行彻底的手术减瘤。在大多数情况下，这将是一项困难且耗时的工作，可能需要两个或更多专科医生的合作，并考虑在手术期间或手术后立即进行腹腔内辅助治疗。因此，治疗需要一种有计划的方法，对诊断和病情范围进行准确的术前评估。有一些主要是传闻性的证据支持腹腔内化疗和放射性同位素治疗。一组强烈主张采用超根治性手术，术中加热并术后进一步化疗，但仍存在争议。大多数患者最终会复发。5年生存率在53%至75%之间，但相对良性和恶性亚组的结果差异很大。
