腹膜假黏液瘤的临床诊治进展

Clinical treatment progress of pseudomyxoma peritonei syndrome

腹膜假黏液瘤（PMP）是指由于产生黏液蛋白的肿瘤破裂导致腹腔内广泛黏液性腹腔积液的临床综合征，主要来源于阑尾黏液性肿瘤。目前PMP的标准治疗方案是肿瘤细胞减灭术（CRS）联合腹腔热灌注化疗（HIPEC），然而对于肿瘤负荷大且无法达到肿瘤完全减灭的患者，可采用最大限度减瘤术（MTD）。肿瘤减灭程度是影响预后的最关键因素，肿瘤完全减灭的患者5年生存率为80.0%-87.4%，而无法达到肿瘤完全减灭的患者5年生存率仅为24.0%-39.2%。文章从PMP的病理分型及诊疗现状等方面作一综述。

Pseudomyxoma peritonei syndrome （PMP） is characterized by a gradual expansion of mucoid tumour and fluid at specific sites within abdominopelvic regions as a result of a perforated appendiceal adenoma. The standard therapy for PMP is combining cytoreductive surgery （CRS） plus hyperthermic intraperitoneal chemotherapy. Maximal tumor debulking surgery （MTD） may be beneficial in patients in whom complete cytoreductive surgery （CCRS） cannot be achieved. It is now recognized that CCRS is one of the strongest predictors of long term survival in patients with PMP. The 5-year survival rate in patients who underwent CCRS was 80.0 %-87.4 %, however the rate in patients who underwent MTD was only 24.0 %- 39,2 %. This article reviews the pathological classification, diagnosis and treatment of PMP.