Mohamed Faheez, Gething Sue, Haiba Moutaz, Brun Erwin A, Sugarbaker Paul H
The Washington Cancer Institute, Washington Hospital Center, Washington, District of Columbia 20010, USA.
J Surg Oncol. 2004 Apr 1;86(1):10-5. doi: 10.1002/jso.20038.
Three distinct morphologic types of pseudomyxoma peritonei syndrome have been defined: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinoma (PMCA), and a hybrid morphologic type. Prognosis is best in patients with DPAM; unfortunately, some patients with DPAM succumb to a rapidly progressive disease process.
We identified a subset of 11 patients with a histopathology of DPAM but a clinical course showing an invasive disease process. As a comparison group, from a database of 501 patients with pseudomyxoma peritonei, we selected 22 age- and sex-matched controls with a DPAM histology who are alive with no evidence of disease. Clinical factors were identified for comparison of case and control groups. Expression of mucin antigens, mucin (MUC)1 and MUC2, were evaluated using immunohistochemistry.
The study group consisted of 11 patients (five men and six women), with a median survival of 52.2 months (SD 7.46) and a 31% 5-year survival. All 22 matched control cases (10 men and 12 women) are alive and disease-free. Clinical data on the study and control groups including co-morbidity were similar. No significant difference in the expression of MUC1 (P = 0.74, Fisher's exact test) or MUC2 (P = 0.34, Fisher's exact test) was demonstrated between groups.
Further investigation of pseudomyxoma peritonei at a molecular and genetic level may help to formulate a more comprehensive classification.
腹膜假黏液瘤综合征已被定义为三种不同的形态学类型：播散性腹膜腺黏液瘤（DPAM）、腹膜黏液腺癌（PMCA）以及一种混合形态学类型。DPAM患者的预后最佳；不幸的是，一些DPAM患者会死于快速进展的疾病过程。
我们确定了11例组织病理学为DPAM但临床病程显示为侵袭性疾病过程的患者亚组。作为对照组，我们从501例腹膜假黏液瘤患者的数据库中，选择了22例年龄和性别匹配、组织学为DPAM且无疾病证据存活的对照。确定临床因素以比较病例组和对照组。使用免疫组织化学评估黏蛋白抗原、黏蛋白（MUC）1和MUC2的表达。
研究组由11例患者（5名男性和6名女性）组成，中位生存期为52.2个月（标准差7.46），5年生存率为31%。所有22例匹配的对照病例（10名男性和12名女性）均存活且无疾病。研究组和对照组包括合并症在内的临床数据相似。两组之间MUC1（P = 0.74，Fisher精确检验）或MUC2（P = 0.34，Fisher精确检验）的表达无显著差异。
在分子和基因水平上对腹膜假黏液瘤进行进一步研究可能有助于制定更全面的分类。
