罕少疾病杂志2025,Vol.32Issue(1) :4-5.DOI:10.3969/j.issn.1009-3257.2025.1.002

腹膜高分化乳头状间皮瘤一例并文献回顾

Well-differentiated Papillary Mesothelioma of the Peritoneum:A Case Report and Literature Review

王粤培 郑炜 梁楚琳 黄国庆 刘序友
罕少疾病杂志2025,Vol.32Issue(1) :4-5.DOI:10.3969/j.issn.1009-3257.2025.1.002

腹膜高分化乳头状间皮瘤一例并文献回顾

Well-differentiated Papillary Mesothelioma of the Peritoneum:A Case Report and Literature Review

王粤培 1郑炜 1梁楚琳 1黄国庆 1刘序友1
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作者信息

  • 1. 暨南大学附属广州红十字会医院(广东 广州 510220)
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摘要

目的 探讨高分化乳头状间皮瘤(well-differentiated papillary mesothelioma,WDPM)的主要临床症状、诊断和治疗.方法 分析1例腹膜高分化乳头状间皮瘤患者资料并复习相关文献.结果 患者主要表现为肠梗阻.为解除肠梗阻行腹腔镜探查术,术中未见明显梗阻点,小肠系膜表面可见多个小结节,结节在镜下所见:由衬覆单层立方形间皮细胞的乳头组成,乳头核心见纤维血管,间皮细胞形态一致,核圆形,可见小核仁,核分裂罕见.免疫组化结果:CR(+)、D2-40(+)、CK5/6(弱+)、CK7(+)、CK19(+)、Ki-67(约 1%+)、P53(约50%+)、EMA(-)、CEA(-).初步诊断为腹膜高分化乳头状间皮瘤,予患者保守治疗并随访.结论 WDPM是一种罕见的间皮来源的具有低度恶性潜能的肿瘤,无特异性症状.诊断主要依赖于术中探查、病理活组织检查和免疫组化分析.WDPM的标准化治疗尚无共识.

Abstract

Objective To comprehend the primary clinical symptoms,prognosis,and therapy of well-differentiated papillary mesothelioma(WDPM).Methods We reviewed the pertinent research and the patient's peritoneal hyperdifferentiated papillary mesothelioma data.Results The patient mainly presented with intestinal obstruction.On the mesenteric surface of the small intestine,several tiny nodules can be recognized.Inside the nodules,which were observed under a microscope,were papillae bordered with a single layer of rectangular mesothelial cells,each having a fibrovascular core and a consistent mesothelial cell shape,including round nuclei and short nucleoli.CR(+),D2-40(+),CK5/6(weak+),CK7(+),CK19(+),Ki-67(1%+),P53(50%+),EMA(-),and CEA(-)were the immunohistochemical findings.The patient had conservative treatment and was monitored after receiving a preliminary diagnosis of peritoneal highly differentiated papillary mesothelioma.Conclusion The WDPM is an uncommon mesothelial tumor with a low-grade malignant potential and no distinguishing symptoms.There is no agreement on the conventional treatment of WDPM,and the diagnosis primarily relies on operative exploration,pathological biopsies,and immunohistochemical analysis.

关键词

高分化乳头状间皮瘤/腹膜/临床特征/病例报告

Key words

Well-differentiated Papillary Mesothelioma/Peritoneum/Clinical Features/Case Report

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出版年

2025
罕少疾病杂志
深圳市卫生局

罕少疾病杂志

影响因子:0.583
ISSN:1009-3257
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