Primary Intrahepatic Mesothelioma: Case Series and Systematic Review of Literature.

IF 1.6 Q4 ONCOLOGY Journal of Gastrointestinal Cancer Pub Date : 2024-12-01 Epub Date: 2024-08-14 DOI:10.1007/s12029-024-01075-x
Junjun Jia, Xinyue Tan, Feng Gao, Zhou Shao, Min Zhang
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Abstract

Background: Primary intrahepatic mesothelioma (PIHMM) has been rarely reported. Its typical clinical presentation, radiological features and pathology have not been defined. Here, we aimed to summarize its diagnosis and treatment.

Methods: We conducted a retrospective analysis of three cases of PIHMM in the First Affiliated Hospital of Zhejiang University School of Medicine and reviewed the current literature to investigate the clinical and pathological characteristics and prognosis of PIHMM.

Results: Based on our case series and the literature, the mean age of PIHMM was 59.7 (41-83) years. Most patients present with nonspecific symptoms such as abdominal pain, fever, weight loss and weakness. On imaging, PIHMM usually presented as a solid, heterogeneous soft tissue mass with irregular margins and significant enhancement of the margins in the arterial phase. Immunohistochemical markers such as calretinin, cytokeratin (CK)5/6, D2-40, WT-1, mesothelin CK and vimentin may be useful for diagnosis. The 3-year relapse-free survival rate (RFS) was 51.85%, the 3-year overall survival (OS) rate was 83.33% and the 3-year postoperative overall survival rate was 100%.

Conclusion: PIHMM can only be diagnosed by careful postoperative pathology, because of its nonspecific clinical presentations, serological indicators or imaging features. Immunohistochemical staining is very useful to distinguish this tumor from other liver tumors. Surgery is the mainstay of treatment.

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原发性肝内间皮瘤:病例系列和文献系统回顾。
背景:原发性肝内间皮瘤(PIHMM)鲜有报道。其典型的临床表现、放射学特征和病理学尚未明确。在此,我们旨在总结其诊断和治疗方法:我们对浙江大学医学院附属第一医院的 3 例 PIHMM 进行了回顾性分析,并查阅了现有文献,以研究 PIHMM 的临床、病理特征和预后:根据我们的病例系列和文献,PIHMM 的平均年龄为 59.7(41-83)岁。大多数患者表现为非特异性症状,如腹痛、发热、体重减轻和乏力。在影像学检查中,PIHMM 通常表现为实性、异型软组织肿块,边缘不规则,动脉期边缘明显强化。免疫组化标记物,如钙网蛋白、细胞角蛋白(CK)5/6、D2-40、WT-1、间皮素 CK 和波形蛋白可能有助于诊断。3年无复发生存率(RFS)为51.85%,3年总生存率(OS)为83.33%,术后3年总生存率为100%:结论:PIHMM由于其临床表现、血清学指标或影像学特征不具特异性,只能通过术后仔细的病理检查才能确诊。免疫组化染色对于区分这种肿瘤和其他肝脏肿瘤非常有用。手术是治疗的主要手段。
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来源期刊
CiteScore
3.80
自引率
0.00%
发文量
121
期刊介绍: The Journal of Gastrointestinal Cancer is a multidisciplinary medium for the publication of novel research pertaining to cancers arising from the gastrointestinal tract.The journal is dedicated to the most rapid publication possible.The journal publishes papers in all relevant fields, emphasizing those studies that are helpful in understanding and treating cancers affecting the esophagus, stomach, liver, gallbladder and biliary tree, pancreas, small bowel, large bowel, rectum, and anus. In addition, the Journal of Gastrointestinal Cancer publishes basic and translational scientific information from studies providing insight into the etiology and progression of cancers affecting these organs. New insights are provided from diverse areas of research such as studies exploring pre-neoplastic states, risk factors, epidemiology, genetics, preclinical therapeutics, surgery, radiation therapy, novel medical therapeutics, clinical trials, and outcome studies.In addition to reports of original clinical and experimental studies, the journal also publishes: case reports, state-of-the-art reviews on topics of immediate interest or importance; invited articles analyzing particular areas of pancreatic research and knowledge; perspectives in which critical evaluation and conflicting opinions about current topics may be expressed; meeting highlights that summarize important points presented at recent meetings; abstracts of symposia and conferences; book reviews; hypotheses; Letters to the Editors; and other items of special interest, including:Complex Cases in GI Oncology:  This is a new initiative to provide a forum to review and discuss the history and management of complex and involved gastrointestinal oncology cases. The format will be similar to a teaching case conference where a case vignette is presented and is followed by a series of questions and discussion points. A brief reference list supporting the points made in discussion would be expected.
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