阑尾黏液性肿瘤病理学特征及预后分析

目的探讨基于2010版WHO分类的各类阑尾黏液性肿瘤的病理学特征及预后。方法收集并复习2003年1月至2012年6月间在南京大学医学院附属鼓楼医院接受手术切除的70例阑尾黏液性肿瘤术后病理切片，按照2010年消化系统肿瘤WHO新分类，将阑尾黏液性肿瘤分为5种类型：阑尾黏液性腺瘤／囊腺瘤（MA）、低级别黏液性肿瘤（LAMN）、起源于阑尾的低级别腹膜假黏液瘤（PMP．L）、浸润性黏液腺癌（MAC）以及起源于阑尾的高级别腹膜假黏液瘤（PMP．H）。结果11例MA肿瘤上皮和黏液仅局限于黏膜肌层以内；术后无复发或死亡病例。41例LAMN黏液均可出现于黏膜肌层外阑尾壁或阑尾表面，其中39黏液湖内没有或仅见少数黏液上皮，上皮具有轻度异型性；有3例复发或进展，元死亡病例。7例PMP—L上皮具有轻度异型性，其中有4例黏液湖内上皮数量稀少或缺乏，其余3例上皮数量中等至较多：术后复发和死亡各1例。7例MAC和4例PMP—H均为浸润性肿瘤，至少局部出现高级别细胞学特征；术后复发4例，死亡3例（含2例复发后死亡）。MA和LAMN在组织学上表现为非浸润性肿瘤。而PMP-L、MAC和PMP-H则在生物学行为上表现为腺癌特征。结论阑尾黏液性肿瘤是一系列肿瘤谱系，其生物学行为取决于肿瘤性质和类型，统一、规范而准确的病理诊断对于治疗至关重要。

Analysis on pathology features and prognosis of appendiceal mucinous neoplasm

Objective To explore the pathological features and prognosis of appendiceal mucinous neoplasms （AMN） based on WHO classification 2010. Methods Seventy consecutive cases of AMN were classified into 5 groups according to WHO classification of digestive system tumors in 2010 induding mucinous adenomas/cystadenoma （MA）, low grade appendiceal mucinous neoplasms （LAMN）, low grade pseudomyxoma peritoneum originated from appendix （PMP-L）, invasive mucinous adenocarcinomas （MAC） and high grade pseudomyxoma peritoneum originated from appendix （PMP-H）. Clinicopathological features, classification, treatment and prognosis of AMN were investigated retrospectively. Results There were 11 cases of MA with neoplastic epithelium and mucin being defined in lumen and mucosa but without invasive lesions, and no relapse or death was found. In 41 LAMN cases, mucin was found in submucosa, muscularis proparis, or serosa of appendix, no or only scant mucinous epithelium with low grade dysplasia presented in mucinous pools in most cases（39/41）. Among 41 LAMN cases, 3 developed relapse or PMP-L, and no death was observed. In 7 PMP-L cases, low grade dysplastic mucinous epithelium in mucinous pools could be found easily in 3 cases and was very scanty in 4 cases, with 1 relapse and 1 death. Eleven invasive carcinomas were found,including 7 MAC cases and 4 PMP-H cases, with local high grade dysplastic epithelium at least. In these invasive lesions, 4 cases recurred and 3 case died （including 2 recurred cases above）. MA and LAMN were both non-invasive neoplasms histologically, however, PMP-L, MAC and PMP-H were regarded as adenocarcinomas according to their biological behavior. Conclusion AMN displays a relatively homogeneous group of neoplasms that pursues a predictable clinical course based on their nature, so it is necessary to diagnose and administrate accurately with consistently standards for these neoplastic entities.