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恶性腹膜间皮瘤作为消化不良症状和腹水的罕见病因:一项诊断挑战。

Malignant Peritoneal Mesothelioma as a Rare Cause of Dyspeptic Complaints and Ascites: A Diagnostic Challenge.

作者信息

Marques de Sousa Susana, Pereira Filipa, Duarte Maria, Marques Marta, Vázquez Dolores, Marques Cristina

机构信息

Internal Medicine Department, Centro Hospitalar Póvoa de Varzim/Vila do Conde, Póvoa de Varzim, Portugal.

Oncology Department, Instituto Português de Oncologia do Porto, Porto, Portugal.

出版信息

GE Port J Gastroenterol. 2020 Apr;27(3):197-202. doi: 10.1159/000503075. Epub 2019 Oct 8.

Abstract

INTRODUCTION

Malignant peritoneal mesothelioma (MPM) is a rare malignancy of the mesothelial cells in the peritoneum. The best-defined risk factor is asbestos exposure, but germline mutations in BAP1 also increase susceptibility to this tumor. The diagnosis of MPM is challenging since clinical manifestations are often nonspecific.

CASE PRESENTATION

We describe a case of MPM in a 53-year-old former construction worker with prior asbestos exposure. The clinical presentation was a 3-month history of dyspeptic complaints. As initial workup, abdominal ultrasound and upper gastrointestinal endoscopy were performed. Chronic gastritis due to was detected, which was promptly treated but without symptom relief. Abdominal ultrasound showed small volume ascites with hyperechogenic foci, which was later confirmed on computed tomography scan showing the presence of peritoneal nodules in the greater omentum and mesentery. A thorough investigation was conducted based on the suspicion of peritoneal carcinomatosis. A non-peritoneal primary tumor was not found. Ascitic cytology and immunocytochemical studies were suggestive of mesothelioma. He underwent exploratory laparotomy and inoperable peritoneal disease was observed. Peritoneal biopsy confirmed epithelioid-type MPM. Systemic therapy was initiated with platinum plus pemetrexed with good response. The last follow-up was 38 months after the diagnosis.

DISCUSSION/CONCLUSION: The diagnosis of MPM is challenging since it requires a high degree of suspicion. MPM has a poor prognosis. The standard of treatment recommended is cytoreductive surgery with hyperthermic intraperitoneal chemotherapy. For those who are inoperable, systemic therapy with pemetrexed-cisplatin combination is the alternative. Given the infrequency of disease, it is imperative to ensure patient participation in clinical trials with the purpose of treatment standardization.

摘要

引言

恶性腹膜间皮瘤(MPM)是一种罕见的腹膜间皮细胞恶性肿瘤。最明确的危险因素是石棉暴露,但BAP1基因的种系突变也会增加患这种肿瘤的易感性。MPM的诊断具有挑战性,因为临床表现往往不具有特异性。

病例介绍

我们描述了一例MPM病例,患者为一名53岁的前建筑工人,曾接触过石棉。临床表现为3个月的消化不良症状史。作为初步检查,进行了腹部超声和上消化道内镜检查。检测到由……引起的慢性胃炎,立即进行了治疗,但症状未缓解。腹部超声显示少量腹水伴高回声灶,后来的计算机断层扫描证实,大网膜和肠系膜存在腹膜结节。基于对腹膜癌病的怀疑进行了全面检查。未发现非腹膜原发性肿瘤。腹水细胞学和免疫细胞化学研究提示为间皮瘤。他接受了剖腹探查术,观察到无法手术切除的腹膜疾病。腹膜活检证实为上皮样型MPM。开始采用铂类加培美曲塞进行全身治疗,反应良好。最后一次随访是在诊断后38个月。

讨论/结论:MPM的诊断具有挑战性,因为它需要高度怀疑。MPM预后较差。推荐的标准治疗方法是细胞减灭术联合腹腔内热化疗。对于无法手术的患者,培美曲塞 - 顺铂联合全身治疗是替代方案。鉴于该病发病率低,必须确保患者参与临床试验以实现治疗标准化。

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本文引用的文献

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Update on the management of malignant peritoneal mesothelioma.恶性腹膜间皮瘤管理的最新进展
Transl Lung Cancer Res. 2018 Oct;7(5):599-608. doi: 10.21037/tlcr.2018.08.03.
2
Malignant peritoneal mesothelioma: a review.恶性腹膜间皮瘤:综述
Transl Lung Cancer Res. 2018 Oct;7(5):537-542. doi: 10.21037/tlcr.2018.10.04.
3
Malignant peritoneal mesothelioma: clinical aspects, and therapeutic perspectives.恶性腹膜间皮瘤:临床特点及治疗前景
Ann Gastroenterol. 2018 Nov-Dec;31(6):659-669. doi: 10.20524/aog.2018.0305. Epub 2018 Sep 14.
6
Malignant peritoneal mesothelioma: a review.恶性腹膜间皮瘤:综述
Ann Transl Med. 2017 Jun;5(11):236. doi: 10.21037/atm.2017.03.96.

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