女性腹膜假黏液瘤的临床病理特征及预后影响因素分析

Prognostic value of clinical and pathological characteristics in 48 women with pseudomyxoma peritonei

目的探讨女性腹膜假黏液瘤的临床病理特征及预后影响因素。方法回顾性分析解放军总医院1982年1月至2011年12月间收治的48例女性腹膜假黏液瘤患者的临床病理及随访资料，探讨腹膜假黏液瘤的临床病理特征。采用Kaplan—Meier法计算患者生存率，并采用log—rank检验、Cox风险比例模型分别对预后影响因素进行单因素及多因素分析。结果（1）临床病理特征：48例患者的平均年龄为58．8岁（24～79岁）；症状：以腹胀、腹部不适为主；影像学检查：多数提示腹盆腔有非特异性占位性病变。治疗：48例患者均行剖腹探查及外科肿瘤细胞减灭术（CRS），其中肿瘤切除的完整性（CCR）分级为CCR－1者15例（31％）、CCR－2者24例（50％）、CCR－3者9例（19％）；6例（12％）患者术中行顺铂腹腔热灌注化疗（HIPEC），20例（42％）术后行不同方案的辅助化疗。病理类型：分为3型，即腹膜弥漫性黏液性腺瘤病、腹膜黏液性腺癌病以及伴有中间型形态的腹膜黏液性腺癌病，分别为22例（46％）、9例（19％）、17例（35％）；阑尾肿瘤类型：44例（92％）患者行阑尾切除术，其中阑尾肿瘤38例（包括低级别阑尾黏液性腺瘤20例、阑尾黏液性腺癌18例）、阑尾炎2例、不详4例，另4例（8％）未行阑尾切除术的患者，分别于首次手术后1、11、32和85个月出现腹腔肿瘤复发并发现阑尾黏液性肿瘤；实质脏器累及：卵巢累及者34例（71％，其中右侧累及者15例、双侧均累及者13例、左侧累及者6例），卵巢以外的其他实质脏器累及者10例（21％）。（2）预后影响因素：48例患者中随访期内（随访1～479个月），11例死亡、31例存活、6例失访，患者总生存时间为1～312个月，中位总生存时间为99个月，3、5和10年总生存率分别为73．3％、68．0％和46．6％。单因素分析显示，年龄、病理类型及其他实质脏器累及与患者的预后有关（P〈0．05）；而手术次数、阑尾肿瘤类型、卵巢累及、CCR分级、术中HIPEC及术后辅助化疗均与患者的预后无关（P〉0．05）。多因素分析显示，病理类型与年龄是影响患者预后的独立因素（P〈0．05）。结论腹膜假黏液瘤的临床表现无特异性，治疗宜行CRS联合HIPEC，并应常规探查双侧卵巢、行阑尾切除术。腹膜假黏液瘤的10年总生存率低，年龄、病理类型及卵巢以外的其他实质脏器累及与患者预后相关，且病理类型和年龄为影响患者预后的独立因素。

Objective To investigate the clinic-pathological characteristics and prognosis of 48 female cases with peritoneal pseudomyxoma（PMP）. Methods The clinicopathologic features and follow-up data of 48 female patients with PMP diagnosed in the General Hospital of People＇s Liberation Army from Jan. 1982 to Dec. 2011 were retrospectively reviewed. The relationship between clinic-pathological characteristics and prognosis were analyzed using log-rank test and Cox proportional hazards model. Results （ 1 ） Clinicopathologic features： the mean age of the 48 cases was 58.8 years （range from 24 to 79 years）. Symptoms： abdominal distention and abdominal discomfort were the main symptoms. Imaging examinations showed nonspecific abdominal and pelvic lesions in most cases. Treatment： all the 48 patients underwentlaparotomy and cytoreductive surgery （CRS）, in which 15 （31%） patients with completeness of the cancer resection （CCR） -1, 24（50% ） cases with CCR-2, and CCR-3 in 9（ 19% ） cases. Six （ 12% ） cases were treated by intraoperative hyperthermic intraperitoneal chemotherapy （HIPEC） with cisplatin, 20 （42%） patients were treated with different options postoperative adjuvant chemotherapy. Pathological types： the cases were histologically classified into 3 subcategories： disseminated peritoneal adenomucinosis （DPAM）, peritoneal mucinous carcinomatosis （PMCA）, and PMCA with intermediate or discordant features （PMCA-I/D） , which were 22 （46%） cases, 9 （ 19% ） cases and 17 （ 35% ） cases, respectively. Appendiceal tumors： 44 （92%） cases underwent appendectomy, in which 38 cases presented appendiceal tumors （including 20 cases of low-grade appendiceal nmcinous adenoma and 18 cases of appendiceal mucinous adenocarcinoma） , 2 cases were diagnosed as appendicitis, 4 cases with unknown pathologic diagnosis. And the other 4（8% ） cases, who didn＇t undergo appendectomy at the first operation, presented peritoneal tumor recurrence and appendiceal mucinous tumors 1, 11,32 and 85 months after surgel＇y, respectively. Parenchymal organs involved： ovarian involving was happened in 34 （71% ）patients including 15 cases with the right ovary involving, 13 cases in both sides, and 6 cases involving the left side. The other parenchymal organs in l0 （21% ） cases. （2） Prognostic factors ： 11 patients died, 31 survived and 6 cases were lost to follow-up. The mean survival time was 99 months（ranged from 1 to 312 months）. The 3-year, 5-year and 10-year survival rates were 73.3% , 68.0% and 46. 6% , respectively. Univariate statistical analysis showed that age, pathological type and parenchymal involvement were significantly relationship with the survival time （ all P 〈 0. 05）. But the operation times, appendiceal tumor type, ovarian involvement, CCR, intraperitoneal HIPEC and post-operative adjuvant chemotherapy were not significantly correlate with survival time （ all P 〉 0. 05 ）. Muhivariate analysis showed that age and pathologic type were independent prognostic factors （ P 〈 0. 05 ）. Conclusions No specific clinical features presented in PMP. CRS with HIPEC should the recommended treatment. Both ovaries exploration and appendectomy should be carried out routinely in CRS. The 10-year overall survival of PMP is low. Age, pathological type and parenchymal organs involvement other than ovarian are correlated with the prognosis. And the pathological type and age are independent prognostic factors of PMP.