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阑尾黏液性肿瘤进展为腹膜假黏液瘤的预测因素。

Predictors of Progression of Appendiceal Mucinous Neoplasm to Pseudomyxoma Peritonei.

机构信息

Kaiser Permanente Los Angeles Medical Center, Los Angeles, USA.

出版信息

Am Surg. 2020 Oct;86(10):1379-1384. doi: 10.1177/0003134820964464.

Abstract

Appendiceal mucinous neoplasm (AMN) can present with a spectrum of disease. Predicting factors in development of pseudomyxoma peritonei (PMP) from AMN could aid in management and treatment. The aim of this study was to determine factors predictive of PMP from AMN. This was a retrospective multicenter study of all patients diagnosed with AMN from 2006-2017. Diagnosis of PMP was compared by (1) patient demographics, (2) tumor characteristics, and (3) surgery. Secondary end points were disease-specific survival (DSS) and overall survival (OS).One-hundred thirty-eight patients with AMN were identified. Thirty-six patients (26.1%) had a ruptured appendix at presentation, and 12 patients (8.7%) were diagnosed with PMP during the study period. Eight patients presented with PMP at the time of surgery. No demographic factors were predictive of PMP. Operative approach and extent of initial resection did not correlate with PMP. Tumor rupture at presentation was the only factor associated with PMP, though only 14% of patients who presented with simple rupture eventually progressed to PMP.OS was not different between those who were diagnosed with PMP and those who were not. DSS was significantly lower for the group diagnosed with PMP ( = .007). Tumor rupture at presentation did not influence OS or DSS. The only factor found to be significantly associated with PMP was tumor rupture at presentation. Diagnosis of PMP did not affect OS but did lead to decreased DSS.In conclusion, though a majority of patients who presented with rupture did not go on to develop PMP, tumor rupture at presentation was the only factor significantly associated with PMP. Diagnosis of PMP did not affect OS at 5 years. In patients with AMN who present with a ruptured appendix on final pathology, we recommended continued surveillance, though overall risk of PMP is relatively low.

摘要

阑尾黏液性肿瘤(AMN)可表现为多种疾病。预测 AMN 发生假性黏液瘤腹膜(PMP)的因素有助于管理和治疗。本研究旨在确定 AMN 发生 PMP 的预测因素。这是一项对 2006 年至 2017 年间所有诊断为 AMN 的患者进行的回顾性多中心研究。通过(1)患者人口统计学特征,(2)肿瘤特征和(3)手术来比较 PMP 的诊断。次要终点是疾病特异性生存率(DSS)和总体生存率(OS)。

共确定了 138 例 AMN 患者。36 例(26.1%)患者就诊时阑尾破裂,12 例(8.7%)患者在研究期间诊断为 PMP。8 例患者在手术时出现 PMP。没有人口统计学因素可以预测 PMP。手术方式和初始切除范围与 PMP 不相关。就诊时肿瘤破裂是唯一与 PMP 相关的因素,尽管只有 14%就诊时表现为单纯破裂的患者最终进展为 PMP。

诊断为 PMP 与未诊断为 PMP 的患者的 OS 无差异。诊断为 PMP 的患者的 DSS 显著低于未诊断为 PMP 的患者(P=0.007)。就诊时肿瘤破裂对 OS 或 DSS 没有影响。唯一与 PMP 显著相关的因素是就诊时肿瘤破裂。PMP 的诊断并未影响 OS,但确实导致 DSS 降低。

总之,尽管大多数就诊时破裂的患者未发展为 PMP,但就诊时肿瘤破裂是唯一与 PMP 显著相关的因素。诊断为 PMP 并不影响 5 年 OS。对于最终病理表现为破裂阑尾的 AMN 患者,我们建议继续监测,尽管 PMP 的总体风险相对较低。

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