原发性阑尾黏液性肿瘤的临床特点和生存分析

Clinical Features and Survival Analysis of Primary Appendiceal Mucinous Neoplasm

背景:原发性阑尾黏液性肿瘤(AMN)临床少见,病理上分为黏液性腺瘤/囊腺瘤(MA)、低级别阑尾黏液性肿瘤(LAMN)、低级别腹膜假黏液瘤(PMP-L)、阑尾黏液腺癌(MAC)、高级别腹膜假黏液瘤(PMP-H)5种类型,后三者在临床上被认定为恶性肿瘤。目的:探讨原发性AMN的临床特点和预后因素。方法:回顾性分析2004年1月—2015年12月上海交通大学医学院附属仁济医院收治的23例原发性AMN患者的临床病理资料,采用Kaplan-Meier法行生存分析,分别采用单因素和多因素分析评估影响患者预后的因素。结果:23例AMN患者的中位生存率(OS)为56.13个月,中位无进展生存率(PFS)为53.79个月。单因素分析显示,病理分类、局部淋巴结转移、疾病进展是影响患者OS的因素(P<0.05);病理分类、局部淋巴结和远处转移、实质脏器累及、腹膜假黏液瘤(PMP)形成是影响患者PFS的因素(P<0.05)。多因素分析显示,局部淋巴结转移是影响患者PFS的独立因素(P=0.042)。在15例恶性AMN患者中,单因素分析显示局部淋巴结转移是影响患者OS的因素(P=0.014)。多因素分析显示,局部淋巴结转移(P=0.010)、实质脏器累及(P=0.012)、PMP形成(P=0.042)、术后腹腔化疗(P=0.037)是影响患者PFS的独立因素。结论:AMN患者伴有局部淋巴结转移易发生疾病进展;病理为恶性肿瘤且伴有局部淋巴结转移或疾病进展者的预后差。局部淋巴结转移、实质脏器累及、PMP形成、术后未行腹腔化疗的恶性AMN患者易发生疾病进展,局部淋巴结转移的恶性AMN患者预后可能较差。

Background：Primary appendiceal mucinous neoplasm （AMN ） is rare in clinic and is classified into 5 groups including mucinous adenoma （MA ）,low grade appendiceal mucinous neoplasm （LAMN ）,low grade pseudomyxoma peritoneum （PMP-L）,mucinous adenocarcinoma （MAC）and high grade pseudomyxoma peritoneum （PMP-H）based on pathology. PMP-L,MAC and PMP-H are identified as malignant tumors in clinic. Aims：To investigate the clinical characteristics and prognostic factors of primary AMN. Methods：The clinical data of 23 patients with primary AMN treated at Ren Ji Hospital of Shanghai Jiao Tong University School of Medicine from January 2004 to December 2015 were retrospectively analyzed. The survival was analyzed by Kaplan-Meier method. The influencing factors were analyzed by univariate and multivariate models. Results：The median overall survival （OS ） and median progression-free survival （PFS）of 23 patients were 56. 13 months and 53. 79 months,respectively. Univariate analysis revealed that pathological categorization,regional lymph node metastasis and disease progression were the influencing factors of OS （P 〈0. 05）. Pathological categorization,regional lymph node and distant metastasis,organ involvement,pseudomyxoma peritoneum （PMP）formation were the influencing factors of PFS （P〈0. 05）. Multivariate analysis showed that regional lymph node metastasis was the independent influencing factor of PFS （P =0. 042）. In 15 patients with malignant AMN,univariate analysis revealed that regional lymph node metastasis was the influencing factor of OS （P=0. 014）. Multivariate analysis showed that regional lymph node metastasis （P=0. 010）,organ involvement （P=0. 012）,PMP formation （P=0. 042） and intraperitoneal chemotherapy （P=0. 037）were independent influencing factors of PFS. Conclusions：AMN patients with regional lymph node metastasis are easily to have disease progression. The prognosis of pathologically malignant tumor patient with regional lymph node metastasis or progression of disease is poor. Malignant AMN patients with regional lymph＆amp;nbsp;node metastasis,organ involvement,PMP formation,not receiving intraperitoneal chemotherapy postoperatively are likely to have disease progression,and malignant AMN patients with regional lymph node metastasis have poor prognosis.