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目的探讨高级别阑尾黏液性肿瘤(HAMN)及相关性腹膜假黏液瘤(PMP)的临床病理学特征。方法回顾性分析2008-01-2016-06间在航天中心医院接受手术切除的9例HAMN的临床病理资料,对具有代表性的切片进行CK7、CK20、villin、CDX2、MUC-2免疫组化染色。结果男性5例,女性4例,年龄55~66岁,平均年龄61岁。随访6~81个月,4例死亡,4例生存,1例失访。形态学上,HAMN内衬绒毛状/乳头状/波浪状、单层平坦、筛状黏液性上皮,部分区域上皮可脱失,7例局灶见高级别异型增生,2例内衬上皮几乎均呈高级别异型增生,均未见侵袭性浸润。9例均伴有PMP,其中6例为高级别PMP,1例为低级别PMP,2例为无细胞性黏液池。9例免疫组化结果:CK20、villin、CDX2和MUC-2均(+),CK7(-)。结论 HAMN具有独特的形态学特征,并常伴有高级别PMP,生物学行为较LAMN更具侵袭性,在诊断过程中尤其需注意将具有局灶性高级别异型增生的AMN报告为HAMN,以提示临床预后。
Objective To investigate the clinicopathological features of high grade appendix mucinous neoplasm (HAMN) and related peritoneal pseudomyxoma (PMP). Methods The clinicopathological data of 9 HAMN patients who underwent surgical resection at the Space Central Hospital from January 2008 to June 2016 were retrospectively analyzed. Immunohistochemical staining for CK7, CK20, villin, CDX2 and MUC-2 was performed on representative sections . Results 5 males and 4 females, aged 55 to 66 years, mean age 61 years. All cases were followed up for 6 ~ 81 months, 4 died, 4 survived and 1 lost follow-up. Morphologically, HAMN was lined with villi / papillae / wavy, flat monolayer, and smegmatous myxoepithelial epithelium. Some regions of the epithelium could be exfoliated, while 7 cases showed high-grade dysplasia. High-grade dysplasia, no invasive invasion. Nine cases were accompanied by PMP, of which 6 cases of high-grade PMP, 1 case of low-grade PMP, 2 cases of acellular mucus pool. Nine cases of immunohistochemistry results: CK20, villin, CDX2 and MUC-2 (+), CK7 (-). Conclusions HAMN has unique morphological features, often associated with high-grade PMP, and its biological behavior is more aggressive than LAMN. In particular, attention should be paid in the diagnosis process to AMN with focal high-grade dysplasia as HAMN, Prompted clinical prognosis.