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基于印度三级肿瘤中心的长期探索和经验,提出将假性黏液瘤腹膜转移(pseudomyxoma peritonei,PMP)更名为腹膜黏液腺癌(abdomino-peritoneal mucinous carcinoma,APM)的建议。

A proposal for changing nomenclature from pseudomyxoma peritonei (PMP) to abdomino-peritoneal mucinous carcinoma (APM) based on its long journey and experience from tertiary oncology center in India.

机构信息

Department of Surgical Oncology, DR BRA-IRCH, All India Institute of Medical Sciences, New Delhi, India.

出版信息

World J Surg Oncol. 2022 Jun 1;20(1):171. doi: 10.1186/s12957-022-02639-6.

Abstract

INTRODUCTION

Pseudomyxoma peritonei (PMP) is a generalized term, usually known as "jelly belly" since 1884. Incidence is very low, 1-3 per million people per year. Because of its indolent nature, it is usually diagnosed at an advanced stage, thereby impacting the quality of life. The 5-year survival rate varies from 23 to 86% in world literature. Even 10 years and 20 years of survival have been described. With our experience, we like to propose rename of PMP as abdomino-peritoneal mucinous carcinoma (APM) as we strongly feel the time has come to specify the term and standardize the management strategy.

METHODOLOGY

In the premier institute of India and as a tertiary referral center, we experienced the maximum number of advanced cases of APM. From 2012 to 2021, we analyzed all the APM patients based on a prospectively maintained computerized database in the department of surgical oncology and found the reasons for renaming from this traditional one.

RESULTS

We included a total of 87 patients who underwent surgical intervention. Thirty-five patients underwent cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), and 52 patients underwent debulking. In CRS-HIPEC patients, CC-0 was achieved in 28 patients (80%), CC-1 in 4 patients (11.4%), and CC-2 in 3 patients (8.6%). Palliative intent HIPEC was done in 3 patients (8.6%). Clavien-Dindo grade III and IV morbidity was observed in 18.8% of patients with 90 days mortality of 5.7%.

CONCLUSION

With our long-term experience and advancement of scientific evidence, we like to propose a new name for PMP as APM. We strongly believe this paper will give a clear picture of this rare disease and standard management outlines.

摘要

简介

假性黏液瘤(PMP)是一个通用术语,通常被称为“果冻肚子”,自 1884 年以来就已经为人所知。其发病率非常低,每年每百万人中有 1-3 人。由于其惰性特征,通常在晚期才被诊断出来,从而影响生活质量。在世界文献中,5 年生存率从 23%到 86%不等。甚至有 10 年和 20 年的生存时间也被描述过。根据我们的经验,我们建议将 PMP 重新命名为腹膜黏液腺癌(APM),因为我们强烈感觉到现在是时候指定这个术语并规范管理策略了。

方法

在印度的一流机构和作为三级转诊中心,我们在外科肿瘤学系的一个前瞻性维护的计算机数据库中分析了 2012 年至 2021 年期间所有的 APM 患者,并从传统术语中找到了重新命名的原因。

结果

我们共纳入 87 名接受手术干预的患者。35 名患者接受了细胞减灭术(CRS)联合腹腔内热灌注化疗(HIPEC),52 名患者接受了减瘤术。在 CRS-HIPEC 患者中,28 名患者(80%)达到了 CC-0,4 名患者(11.4%)达到了 CC-1,3 名患者(8.6%)达到了 CC-2。3 名患者(8.6%)接受了姑息性 HIPEC。36.1%的患者出现了 3 级和 4 级并发症,90 天死亡率为 5.7%。

结论

根据我们的长期经验和科学证据的进展,我们建议将 PMP 重新命名为 APM。我们坚信,本文将为这种罕见疾病提供清晰的描述和标准的管理概述。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c82a/9158346/2b8ac876639f/12957_2022_2639_Fig1_HTML.jpg

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