摘要目的 分析腹膜黏液性肿瘤的临床病理特征及其与预后的关系,并对其起源进行探讨.方法 按照WHO 2010消化系统肿瘤分类标准对34例曾诊断为“腹膜假黏液瘤”的病例重新分类,分为低级别和高级别两类,分析低级别和高级别黏液性肿瘤的临床病理特征及其与预后的关系.采用免疫组织化学EnVision法检测特异性AT序列结合蛋白2(special AT-rich sequence-binding protein 2,SATB2)在腹膜黏液性肿瘤中的表达,并对未明确原发病灶的病例进行CK7、CK20及CDX-2染色对其起源加以探讨.结果 34例腹膜黏液性肿瘤中,低级别黏液性肿瘤25例(其中2例为无细胞型),高级别黏液性肿瘤9例,两者与患者年龄、性别、是否复发及累及周围器官均无相关性(P＞0.05).对30例患者的随访发现,低级别和高级别黏液性肿瘤的总体生存比例分别为13/21(61.9％)和3/9,中位生存时间分别为74和24个月,两者统计学具有相关性,高级别黏液性肿瘤的生存率显著低于低级别黏液性肿瘤(P =0.002).对除去2例为无细胞型病例共32例行免疫组织化学染色,CDX-2、CK20及CK7总的表达比例分别为93.8％ (30/32)、96.9％ (31/32)和3/16;在已明确来源的16例中,表达CDX-2、CK20、CK7的分别为15、16和1例;在未明确原发病灶的16例中,表达CDX-2、CK20、CK7的分别为15、15和2例;表达CDX-2、CK20、CK7的在已明确来源的和未明确来源的肿瘤之间差异无统计学意义(P＞0.05).SATB2在肿瘤中总的表达率为56.3％ (18/32),在低级别和高级别黏液性肿瘤的表达比例分别为15/23 (65.2％)和3/9,二者差异无统计学意义(P=0.102);SATB2的阳性表达与预后也无明显相关性(P =0.786).结论 腹膜黏液性肿瘤其WHO最新分级与预后相关,其中高级别肿瘤患者生存率显著低于低级别肿瘤患者;大部分腹膜黏液性肿瘤起源于阑尾.

abstractsObjective To investigate the clinicopathologic characteristics,prognosis and histologic origin of the mucinous tumor of the peritoneum.Methods According to 2010 WHO classification of tumours of the digestive system,34 cases diagnosed as "pseudomyxoma peritonei (PMP)" were reevaluated and divided into low grade and high grade.Immunohistochemistry was applied to investigate the expression of SATB2 and the histologic origin of the mucinous tumor of the peritoneum,using antibodies against SATB2,CK7,CK20 and CDX-2.The relationship between clinicopathologic characteristics and prognosis of the low grade and high grade tumors were analyzed.Results Twenty five patients had low grade mucinous tumors (two of them were no cell type),nine patients had high grade mucinous tumors.There was no significant difference between low grade and high grade mucinous tumors in age,sex,recurrence and organs involvement (P ＞ 0.05).Thirty patients were followed up,the overall survival rates of patients with low grade and high grade mucinous tumors were 13/21 (61.9％) and 3/9,respectively.The median survival time was 74 and 24 months in low and high grade patients,and the difference was statistically significant (P =0.002).Immunohistochemistry showed the expression rates of CDX-2,CK20,and CK7 in totally 32 cases (excluding 2 cases of no cell type) were 30/32 (93.8％),31/32 (96.9％),and 3/16,respectively; the expression rates of CDX-2,CK20,and CK7 in 16 cases with distinct primary site were 15,16,and 1,respectively; fifteen of 16 cases of tumors of unknown primary site were positive for CDX-2 and CK20,two of the them were positive for CK7.There was no difference in the expression of CDX-2,CK20 and CK7 between tumors with distinct primary site and tumors with unknown primary site (P ＞0.05).The expression rate of SATB2 in the cases was 56.3％ (18/32),excluding 2 cases of no cell type.There was no significant difference between low grade and high grade tumors in the expression of SATB2 [15/23 (65.2％) vs 3/9,P =0.102],also SATB2 was not related to the prognosis of the tumor (P =0.786).Conclusion The prognosis of the mucinous tumor of the peritoneum was significantly different between low grade and high grade according to WHO 2010 classification,and most mucinous tumor of the peritoneum originated from the appendix.