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多囊性腹膜间皮瘤:简短综述

Multicystic peritoneal mesothelioma: A short review.

作者信息

Zhang Chi-Hao, Yu Ji-Wei, Luo Meng

机构信息

Department of General Surgery, Shanghai Ninth People׳s Hospital, School of Medicine, Shanghai Jiao Tong University, Baoshan, Shanghai, China.

Department of General Surgery, Shanghai Ninth People׳s Hospital, School of Medicine, Shanghai Jiao Tong University, Huangpu, Shanghai, China.

出版信息

Curr Probl Cancer. 2017 Sep-Oct;41(5):340-348. doi: 10.1016/j.currproblcancer.2017.03.002. Epub 2017 Mar 31.

Abstract

Multicystic peritoneal mesothelioma (MCPM) is a rare neoplasm, predominantly affecting female patients during their reproductive years. The lesion is usually distributed diffusely in the abdomen and pelvis, but the peritoneum of the pelvic organs is the most common site. MCPM is composed of fluid-filled translucent cysts, connected by varying amounts of fibrous tissue, and lined by a layer of mesothelial cells. Because of the rarity of this disease, the pathogenesis and natural history of MCPM remain poorly understood and continuously debated. Some authors consider it to be a reactive process for its association with prior surgery or abdominal inflammation. But its high rate of local-regional recurrence, as well as its malignant potential, suggests a neoplastic etiology. Preoperative diagnosis is often very difficult. Imaging methods, such as ultrasound, computed tomography, and magnetic resonance imaging, are of little value for an accurate diagnosis of MCPM. The definitive diagnosis relies on histologic examination of target lesions combined with immunohistochemical stains. There is no consensus on the clinical management of MCPM, although surgical removal remains the first-line treatment of choice. But no standards have been reached concerning which surgical options-traditional debulking surgery or more aggressive one-should be chosen. Alternative therapeutic approaches include hand-off treatment, hormonal supplementation, laser vaporization, and sclerotherapy, and they all come with uncertain results. Moreover, the lesions show no response to adjuvant chemotherapy and radiotherapy. This article aimed to focus on those controversial problems in pathogenesis, natural history, diagnosis, and treatment strategies to help medical workers to better understand this rare disease.

摘要

多囊性腹膜间皮瘤(MCPM)是一种罕见的肿瘤,主要影响处于生育年龄的女性患者。病变通常弥漫分布于腹部和盆腔,但盆腔器官的腹膜是最常见的部位。MCPM由充满液体的半透明囊肿组成,囊肿由不同量的纤维组织相连,并内衬一层间皮细胞。由于这种疾病罕见,MCPM的发病机制和自然史仍知之甚少且不断受到争议。一些作者认为它是一种反应性过程,因为它与既往手术或腹部炎症有关。但其局部区域复发率高以及具有恶性潜能,提示其肿瘤性病因。术前诊断往往非常困难。超声、计算机断层扫描和磁共振成像等影像学方法对MCPM的准确诊断价值不大。明确诊断依赖于对目标病变进行组织学检查并结合免疫组化染色。尽管手术切除仍然是首选的一线治疗方法,但对于MCPM的临床管理尚无共识。对于应选择哪种手术方式——传统的减瘤手术还是更积极的手术方式,尚未达成标准。其他治疗方法包括姑息治疗、激素补充、激光汽化和硬化治疗,但其结果均不确定。此外,病变对辅助化疗和放疗无反应。本文旨在关注发病机制、自然史、诊断和治疗策略等方面的争议问题,以帮助医务工作者更好地了解这种罕见疾病。

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