Guo Ai-tao, Wei Li-xin, Song Xin
Department of Pathology, The General Hospital of PLA, Beijing 100853, China.
Zhonghua Bing Li Xue Za Zhi. 2007 Jul;36(7):474-9.
To clarify the various diagnostic connotations of pseudomyxoma peritonei (PMP) and to study their prognostic implications.
Clinicopathologic features and follow-up data of 40 patients with PMP diagnosed in The General Hospital of PLA were retrospectively reviewed. 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). The survival rate was calculated using Kaplan-Meier method and the difference was statistically analyzed.
Twelve of the 40 patients died on follow up. The duration of survival ranged from 2 to 348 months (medium = 37.5 months). In general, the 3-year, 5-year and 10-year survival rates were 79.0%, 69.4% and 53.0%, respectively. The mean age of the patients at the time of diagnosis was 50.3 years (age range = 22 to 76 years). The male-to-female ratio was 1:1. The age and sex of patients, frequency of operation and presence of ovarian involvement did not correlate with duration of survival. On the other hand, the presence of appendiceal tumor, parenchymal invasion of abdominal viscera, cellularity, architecture, nuclear atypia and mitotic activity of the peritoneal lesion significantly correlated with survival. There was also significant difference in survival between DPAM, PMCA-I/D and PMCA subcategories (P = 0.018). The difference in survival rate between PMCA-I/D and PMCA subgroups however was not statistically significant (P = 0.096). The outcome of DPAM was significantly better when compared with the combined group of PMCA-I/D and PMCA (P = 0.006).
In general, the 10-year survival rate of PMP was low, despite the relatively benign-looking or low-grade pathologic appearance. Peritoneal lesions with higher cellularity, conspicuous nuclear atypia and higher mitotic activity are associated with a lower survival rate. The prognosis was even worse in the presence of appendiceal carcinoma or parenchymal invasion of abdominal viscera. It is thus advisable to subclassify PMP into DPAM, PMCA and PMCA-I/D, due to the difference in prognostic implication.
阐明腹膜假黏液瘤（PMP）的各种诊断内涵，并研究其对预后的影响。
回顾性分析解放军总医院确诊的40例PMP患者的临床病理特征及随访资料。病例在组织学上分为3个亚类：播散性腹膜腺黏液瘤（DPAM）、腹膜黏液性癌（PMCA）以及具有中间或不一致特征的PMCA（PMCA-I/D）。采用Kaplan-Meier法计算生存率并进行统计学差异分析。
40例患者中有12例在随访期间死亡。生存时间为2至348个月（中位时间 = 37.5个月）。总体而言，3年、5年和10年生存率分别为79.0%、69.4%和53.0%。患者诊断时的平均年龄为50.3岁（年龄范围 = 22至76岁）。男女比例为1:1。患者的年龄和性别、手术频率以及卵巢受累情况与生存时间无关。另一方面，阑尾肿瘤的存在、腹部脏器的实质侵犯、腹膜病变的细胞密度、结构、核异型性和有丝分裂活性与生存显著相关。DPAM、PMCA-I/D和PMCA亚类之间的生存也存在显著差异（P = 0.018）。然而，PMCA-I/D和PMCA亚组之间的生存率差异无统计学意义（P = 0.096）。与PMCA-I/D和PMCA联合组相比，DPAM的预后明显更好（P = 0.006）。
总体而言，尽管PMP的病理表现相对良性或低级别，但10年生存率较低。细胞密度较高、明显核异型性和较高有丝分裂活性的腹膜病变与较低的生存率相关。存在阑尾癌或腹部脏器实质侵犯时预后更差。因此，鉴于预后意义的差异，建议将PMP细分为DPAM、PMCA和PMCA-I/D。
